TY - JOUR AB - Purpose:Rare, recurrent chromosome 1q21.1 duplications have been associated with developmental delay, congenital anomalies, and macrocephaly in children. Data on adult clinical expression would help to inform genetic counseling.Methods:A systematic review of 22 studies reporting 107 individuals (59 children and 48 adults) with 1q21.1 duplications was conducted. We compiled the available phenotypic data to attempt to identify the most highly associated clinical features and to determine expression in adults. We also report on seven adult cases newly identified in the studies of schizophrenia and tetralogy of Fallot at our center.Results:Five cases were ascertained as controls, 32 as relatives of probands, and 70 as having clinical features: autism spectrum disorder (n = 15), congenital heart disease (n = 12), schizophrenia (n = 10), or other, mostly developmental, features (n = 33). The 1q21.1 duplication was significantly enriched in the cohorts with schizophrenia (P = 0.0155) and tetralogy of Fallot (P = 0.0040) at our center as compared with controls. There was a paucity of clinical data for adults; the most common features, other than those used for ascertainment, included macrocephaly and abnormalities of possible connective tissue origin (e.g., carpal tunnel syndrome).Conclusion: Further data are needed to characterize lifetime expression of 1q21.1 duplications. These initial results, however, suggest that anticipatory care should include attention to later-onset conditions such as schizophrenia. AD - A.S. Bassett, Clinical Genetics Research Program, Centre for Addiction and Mental Health, Toronto, ON, Canada AU - Dolcetti, A. AU - Silversides, C. K. AU - Marshall, C. R. AU - Lionel, A. C. AU - Stavropoulos, D. J. AU - Scherer, S. W. AU - Bassett, A. S. DB - Embase Medline DO - 10.1038/gim.2012.129 IS - 4 KW - article attention deficit disorder autism bipolar disorder carpal tunnel syndrome cataract cholelithiasis chromosome 1q chromosome duplication congenital heart disease congenital malformation craniofacial malformation deep vein thrombosis depression developmental disorder Fallot tetralogy gene expression head circumference heart disease heart single ventricle heart ventricle septum defect human hypercholesterolemia intellectual impairment macrocephaly non insulin dependent diabetes mellitus schizophrenia sex ratio systematic review LA - English M3 - Article N1 - L368683711 2013-04-17 PY - 2013 SN - 1098-3600 1530-0366 SP - 282-283 ST - 1q21.1 Microduplication expression in adults T2 - Genetics in Medicine TI - 1q21.1 Microduplication expression in adults UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L368683711 http://dx.doi.org/10.1038/gim.2012.129 VL - 15 ID - 1005 ER - TY - JOUR AB - Background: The 8p23.1 duplication syndrome and copy number variation of the 8p23.1 defensin gene cluster are cytogenetically indistinguishable but distinct at the molecular level. To our knowledge, the 8p23.1 duplication syndrome has been described at prenatal diagnosis only once and we report our experience with four further apparent duplications ascertained at prenatal diagnosis. Methods. Additional material at band 8p23.1 was detected using conventional G-banded cytogenetics in each case. Multiplex Ligation-dependent Probe Amplification (MLPA) or Fluorescence In Situ Hybridisation (FISH) were used depending on whether only DNA (Cases 1 and 4) or cytogenetic preparations (Cases 2 and 3) were available from the laboratory of origin. The extent of the duplication in Case 1 was retrospectively determined using array Comparative Genomic Hybridisation (array CGH). Results. Three cases of 8p23.1 duplication syndrome were found (Cases 1 to 3). Two were de novo and continued to term and the third, a paternally transmitted duplication, was terminated because of a previous child with psychomotor delay and 8p23.1 duplication syndrome. Case 1 was ascertained with a hypoplastic left heart but the ventricular septal and interventricular defects, in Cases 2 and 3 respectively, were found after ascertainment for advanced maternal age. By contrast, case 4 was a maternally transmitted copy number variation of the defensin cluster with normal outcome. Conclusions. Our data underline the need to differentiate 8p23.1 duplications from copy number variation of the defensin cluster using FISH, MLPA or array CGH. Cardiac defects were ascertained by ultrasound in only one of the three duplication 8p23.1 pregnancies but were visible in two of the three at 21 to 22 weeks gestation. Our results provide further evidence that both deletion and duplication of the GATA4 transcription factor can give rise to a variety of conotruncal heart defects with variable penetrance and expressivity. © 2010 Barber et al; licensee BioMed Central Ltd. AD - J. C. Barber, Wessex Regional Genetics Laboratory, Salisbury NHS Foundation Trust, Salisbury, SP2 8BJ, United Kingdom AU - Barber, J. C. AU - Bunyan, D. AU - Curtis, M. AU - Robinson, D. AU - Morlot, S. AU - Dermitzel, A. AU - Liehr, T. AU - Alves, C. AU - Trindade, J. AU - Paramos, A. I. AU - Cooper, C. AU - Ocraft, K. AU - Taylor, E. J. AU - Maloney, V. K. DB - Embase DO - 10.1186/1755-8166-3-3 IS - 1 KW - defensin transcription factor GATA 4 8p23.1 duplication syndrome adult amniocentesis article chromosome 8p chromosome analysis chromosome band chromosome duplication chromosome number chromosome variant comparative genomic hybridization copy number variation female fluorescence in situ hybridization gene cluster gestational age heart catheterization heart ventricle septum defect human hypoplastic left heart syndrome maternal age pregnancy outcome prenatal diagnosis priority journal psychomotor disorder LA - English M3 - Article N1 - L50843680 2010-04-13 2010-06-22 PY - 2010 SN - 1755-8166 ST - 8p23.1 duplication syndrome differentiated from copy number variation of the defensin cluster at prenatal diagnosis in four new families T2 - Molecular Cytogenetics TI - 8p23.1 duplication syndrome differentiated from copy number variation of the defensin cluster at prenatal diagnosis in four new families UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50843680 http://dx.doi.org/10.1186/1755-8166-3-3 VL - 3 ID - 1141 ER - TY - JOUR AB - Between 1994 and 2011, we performed 133 Fontan conversions with arrhythmia surgery. Most patients had tricuspid atresia or double-inlet left ventricle with prior atriopulmonary connection. Operative mortality was 1.5%, and mean length of stay was 14 days. A total of eight patients (6%) have had late cardiac transplantation. Freedom from arrhythmia recurrence is 85% at 10 years. For properly selected patients with a functionally univentricular heart who have had an atriopulmonary Fontan procedure, Fontan conversion with arrhythmia surgery significantly improves quality of life. © 2011 Cambridge University Press. AD - Division of Cardiovascular-Thoracic Surgery, Children's Memorial Hospital, 2300 Childrens Plaza, Chicago, IL 60614, United States AU - Backer, C. L. DB - Scopus DO - 10.1017/S1047951111001764 IS - SUPPL. 2 KW - arrhythmia surgery cavopulmonary connection congenital heart disease Cox-maze III Fontan procedure Functionally univentricular heart M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 2011 SP - 169-176 ST - 12th Annual C. Walton Lillehei Memorial Lecture in Cardiovascular Surgery: Fontan conversion - The Chicago experience T2 - Cardiology in the Young TI - 12th Annual C. Walton Lillehei Memorial Lecture in Cardiovascular Surgery: Fontan conversion - The Chicago experience UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-83755229179&doi=10.1017%2fS1047951111001764&partnerID=40&md5=356ccd83ae489ed32b7d5444d956edf8 VL - 21 ID - 2106 ER - TY - JOUR AB - Between 1994 and 2011, we performed 133 Fontan conversions with arrhythmia surgery. Most patients had tricuspid atresia or double-inlet left ventricle with prior atriopulmonary connection. Operative mortality was 1.5%, and mean length of stay was 14 days. A total of eight patients (6%) have had late cardiac transplantation. Freedom from arrhythmia recurrence is 85% at 10 years. For properly selected patients with a functionally univentricular heart who have had an atriopulmonary Fontan procedure, Fontan conversion with arrhythmia surgery significantly improves quality of life. AD - Division of Cardiovascular-Thoracic Surgery, Children's Memorial Hospital, 2300 Children’s Plaza, Chicago, Illinois 60614, USA. cbacker@childrensmemorial.org AN - 22152545 AU - Backer, C. L. DA - Dec DB - PubMed DO - 10.1017/s1047951111001764 DP - NLM ET - 2011/12/14 KW - Chicago Child Fontan Procedure/*methods Heart Defects, Congenital/*surgery Humans LA - eng N1 - 1467-1107 Backer, Carl L Lecture England Cardiol Young. 2011 Dec;21 Suppl 2:169-76. doi: 10.1017/S1047951111001764. PY - 2011 SN - 1047-9511 SP - 169-76 ST - 12th Annual C. Walton Lillehei Memorial Lecture in Cardiovascular Surgery: Fontan conversion--the Chicago experience T2 - Cardiol Young TI - 12th Annual C. Walton Lillehei Memorial Lecture in Cardiovascular Surgery: Fontan conversion--the Chicago experience VL - 21 Suppl 2 ID - 470 ER - TY - JOUR AB - The 22q11.2 deletion syndrome is characterized by multiple congenital anomalies including conotruncal cardiac defects. Identifying the patient with a 22q11.2 deletion (22q11del) can be challenging because many extracardiac features become apparent later in life. We sought to better define the cardiac phenotype associated with a 22q11del to help direct genetic testing. 1,610 patients with conotruncal defects were sequentially tested for a 22q11del. The counts and frequencies of primary lesions and cardiac features were tabulated for those with and those without a 22q11del. Logistic regression models investigated cardiac features that predicted deletion status in tetralogy of Fallot (TOF). Deletion frequency varied by primary anatomic phenotype. Regardless of the cardiac diagnosis, a concurrent aortic arch anomaly (AAA) was strongly associated with deletion status [odds ratio (OR), 5.07; 95 % confidence interval (CI), 3.66-7.04]. In the TOF subset, the strongest predictor of deletion status was an AAA (OR, 3.14; 95 % CI 1.87-5.27; p < 0.001), followed by pulmonary valve atresia (OR, 2.03; 95 % CI 1.02-4.02; p = 0.04). Among those with double-outlet right ventricle and transposition of the great arteries, only those with an AAA had a 22q11del. However, 5 % of the patients with an isolated conoventricular ventricular septal defect and normal aortic arch anatomy had a 22q11del, whereas no one with an interrupted aortic arch type A had a 22q11del. A subset of patients with conotruncal defects are at risk for a 22q11del. A concurrent AAA increases the risk regardless of the intracardiac anatomy. These findings help to direct genetic screening for the 22q11.2 deletion syndrome in the cardiac patient. © 2013 Springer Science+Business Media New York. AD - S. Peyvandi, Department of Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA, United States AU - Peyvandi, S. AU - Lupo, P. J. AU - Garbarini, J. AU - Woyciechowski, S. AU - Edman, S. AU - Emanuel, B. S. AU - Mitchell, L. E. AU - Goldmuntz, E. DB - Embase Medline DO - 10.1007/s00246-013-0694-4 IS - 7 KW - 22q11.2 deletion syndrome aortic arch anomaly arterial trunk article child chromosome 22q clinical feature congenital heart disease conotruncal cardiac defect Fallot tetralogy female fluorescence in situ hybridization gene deletion genetic association genetic screening heart right ventricle double outlet heart single ventricle heart ventricle septum defect human infant learning disorder major clinical study male multiplex ligation dependent probe amplification palatopharyngeal incompetence phenotype predictive value preschool child pulmonary valve atresia pulmonary valve stenosis school child LA - English M3 - Article N1 - L52548099 2013-04-23 2013-10-22 PY - 2013 SN - 0172-0643 1432-1971 SP - 1687-1694 ST - 22q11.2 deletions in patients with conotruncal defects: Data from 1,610 consecutive cases T2 - Pediatric Cardiology TI - 22q11.2 deletions in patients with conotruncal defects: Data from 1,610 consecutive cases UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52548099 http://dx.doi.org/10.1007/s00246-013-0694-4 VL - 34 ID - 988 ER - TY - JOUR AU - Gidding, S. S. DB - Embase Medline DO - 10.1016/j.jpeds.2012.03.017 IS - 3 KW - article congenital heart disease dysphagia growth disorder heart single ventricle hemodynamics human priority journal prognosis quality of life vocal cord paralysis LA - English M3 - Article N1 - L619859634 2017-12-25 2018-01-02 PY - 2012 SN - 1097-6833 0022-3476 SP - 389 ST - 50 Years Ago in The Journal of Pediatrics: Growth Disturbance in Congenital Heart Disease T2 - Journal of Pediatrics TI - 50 Years Ago in The Journal of Pediatrics: Growth Disturbance in Congenital Heart Disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L619859634 http://dx.doi.org/10.1016/j.jpeds.2012.03.017 VL - 161 ID - 1047 ER - TY - JOUR AB - This study aimed to assess critically the role of 64-slice multidetector-row computed tomographic (MDCT) angiography for evaluating congenital heart disease. The study enrolled 60 consecutive patients (median age, 4.7 years; median weight, 16.5 kg) with congenital heart disease who underwent 64-slice MDCT angiography during the period June 2006 through September 2007. The results were classified as diagnostic categories, and the impact of the procedure on strategizing management was critically analyzed. In each of the groups, the current technique offered a clear advantage over conventional imaging and provided specific clues for surgical/interventional management. A management algorithm was evolved based on questions frequently asked about pulmonary artery anatomy. The correlation with surgical anatomy in all cases that involved surgery was excellent. Early results suggest that 64-slice MDCT angiography is a major breakthrough in cardiovascular imaging with an important diagnostic and decision-aiding role. Diagnostic cardiac catheterization, especially for evaluating great vessel anomalies, could be largely replaced by the described technique for congenital heart disease. © 2008 Springer Science+Business Media, LLC. AD - R. S. Kumar, Department of Pediatric Cardiology, Institute of Cardiovascular Diseases, Madras Medical Mission, 4A, Dr. J.J. Nagar, Mogappair, Chennai 600 037, India AU - Khatri, S. AU - Varma, S. K. AU - Khatri, P. AU - Kumar, R. S. DB - Embase Medline DO - 10.1007/s00246-008-9196-1 IS - 4 KW - adolescent adult algorithm aortopulmonary shunt article cardiovascular system child clinical decision making computed tomographic angiography congenital heart disease controlled study diagnostic imaging diagnostic value female Fontan procedure heart catheterization human infant major clinical study male multidetector computed tomography pulmonary artery surgical anatomy Williams Beuren syndrome LA - English M3 - Article N1 - L50063101 2008-08-05 PY - 2008 SN - 0172-0643 SP - 755-762 ST - 64-Slice multidetector-row computed tomographic angiography for evaluating congenital heart disease T2 - Pediatric Cardiology TI - 64-Slice multidetector-row computed tomographic angiography for evaluating congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50063101 http://dx.doi.org/10.1007/s00246-008-9196-1 VL - 29 ID - 1204 ER - TY - JOUR AB - Patients with a functionally univentricular heart who have had an atriopulmonary Fontan are at risk for atrial dilatation, atrial arrhythmias, and progressive circulatory failure. Between 1994 and 2018, we performed 149 Fontan conversions with arrhythmia surgery and epicardial pacemaker placement at Ann & Robert H. Lurie Children's Hospital of Chicago. This operation converts the atriopulmonary Fontan to an extracardiac Fontan that improves hemodynamics and controls the atrial arrhythmias. Operative mortality during that time was 2%, and freedom from death or heart transplant at 10 years is 84%. For properly selected patients, Fontan conversion improves both survival and quality of life. Patients with an atriopulmonary Fontan constitute an eroding population, as they face many comorbidities and have a decreased life expectancy without treatment; therefore, all patients with an atriopulmonary Fontan should be evaluated for this procedure. AD - Ann & robert h. Lurie children's hospital of chicago, northwestern university feinberg school of medicine, chicago, illinois. Florida hospital for children, orlando, florida. Johns hopkins university school of medicine, baltimore, maryland. AN - 31384373 AU - Backer, C. L. AU - Mavroudis, C. C2 - PMC6668738 DA - Apr-Jun DB - PubMed DO - 10.14797/mdcj-15-2-105 DP - NLM ET - 2019/08/07 IS - 2 KW - Adolescent Adult Arrhythmias, Cardiac/etiology/mortality/physiopathology/*surgery Cardiomegaly/etiology/mortality/physiopathology/*surgery Child Child, Preschool Clinical Decision-Making Female *Fontan Procedure/adverse effects/mortality Heart Defects, Congenital/complications/mortality/physiopathology/*surgery Humans Male Middle Aged Patient Selection Progression-Free Survival Reoperation Risk Assessment Risk Factors Time Factors Young Adult Fontan conversion arrhythmia atrial fibrillation atriopulmonary Fontan congenital heart surgery cryoablation epicardial pacemaker protein-losing enteropathy tricuspid atresia Methodist DeBakey Cardiovascular Journal Conflict of Interest Statement and none were reported. LA - eng N1 - 1947-6108 Backer, Carl L Mavroudis, Constantine Journal Article Methodist Debakey Cardiovasc J. 2019 Apr-Jun;15(2):105-110. doi: 10.14797/mdcj-15-2-105. PY - 2019 SN - 1947-6094 (Print) 1947-6108 SP - 105-110 ST - 149 Fontan Conversions T2 - Methodist Debakey Cardiovasc J TI - 149 Fontan Conversions VL - 15 ID - 8 ER - TY - JOUR AU - Stout, K. K. AU - Daniels, C. J. AU - Aboulhosn, J. A. AU - Bozkurt, B. AU - Broberg, C. S. AU - Colman, J. M. AU - Crumb, S. R. AU - Dearani, J. A. AU - Fuller, S. AU - Gurvitz, M. AU - Khairy, P. AU - Landzberg, M. J. AU - Saidi, A. AU - Valente, A. M. AU - Van Hare, G. F. AU - Levine, G. N. AU - O’Gara, P. T. AU - Halperin, J. L. AU - Albert, N. M. AU - Al-Khatib, S. M. AU - Beckman, J. A. AU - Birtcher, K. K. AU - Bozkurt, B. AU - Brindis, R. G. AU - Cigarroa, J. E. AU - Curtis, L. H. AU - Deswal, A. AU - Fleisher, L. A. AU - Gentile, F. AU - Gidding, S. S. AU - Goldberger, Z. D. AU - Hlatky, M. A. AU - Ikonomidis, J. AU - Joglar, J. AU - Kovacs, R. J. AU - Mauri, L. AU - Magnus Ohman, E. AU - Piano, M. R. AU - Pressler, S. J. AU - Riegel, B. AU - Sellke, F. W. AU - Shen, W. K. AU - Wijeysundera, D. N. AU - Michael Valentine, C. AU - Gates, C. C. AU - Oetgen, W. J. AU - Elma, M. AU - Scholtz, A. AU - Sheehan, K. A. AU - Abdullah, A. R. AU - Benjamin, I. AU - Brown, N. AU - Robertson, R. M. AU - Whitman, G. R. AU - Nedungadi, P. AU - Hundley, J. AU - Force, Acc Aha Task DB - Scopus DO - 10.1161/CIR.0000000000000602 IS - 14 KW - AHA scientific statements Arrhythmias Cardiac catheterization Cardiac defects Congenital heart disease Congenital heart surgery Unoperated/repaired heart defect M3 - Article N1 - Cited By :34 Export Date: 15 June 2020 PY - 2019 SP - E637-E697 ST - 2018 AHA/ACC guideline for the management of adults with congenital heart disease: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on clinical practice guidelines T2 - Circulation TI - 2018 AHA/ACC guideline for the management of adults with congenital heart disease: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on clinical practice guidelines UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85064196356&doi=10.1161%2fCIR.0000000000000602&partnerID=40&md5=dce1c997e7c3c513c79b492958ff6dae VL - 139 ID - 1752 ER - TY - JOUR AB - OBJECTIVES: To determine whether fetuses with an isolated aberrant course of the right subclavian artery (ARSA) have increased risk for chromosomal abnormalities, including trisomy 21 or 22q11 deletion. METHODS: We performed a retrospective chart review of all fetuses with antenatally diagnosed ARSA. Data were collected from fetal anatomic surveys, fetal echocardiograms, noninvasive trisomy 21 screening programs, invasive genetic studies, and neonatal records. RESULTS: Seventy-nine fetuses with ARSA were identified at 20.3 ± 3.8 weeks' gestation. Forty-eight fetuses underwent chromosomal evaluation. Of those, seven had trisomy 21. Four other fetuses had unusual karyotype abnormalities. All fetuses with genetic anomalies had additional aberrant ultrasound findings. There were three spontaneous fetal deaths (trisomy 21-2 and Wolf-Hirshhorn-1). Nine pregnancies were terminated because of abnormalities and one died as a result of hypoplastic left heart syndrome. No neonate was found or suspected to have 22q11.2 deletion. The ARSA was isolated in 43 fetuses; all had unremarkable neonatal outcomes, and none were readmitted within 6 months after discharge. CONCLUSIONS: As an apparently isolated finding, ARSA is benign and not associated with trisomy 21 or 22q11.2 deletion. The finding of ARSA, however, warrants a detailed fetal ultrasound. All fetuses with ARSA and genetic anomalies had additional ultrasound findings. AU - Ranzini, A. C. AU - Hyman, F. AU - Jamaer, E. AU - van Mieghem, T. DB - Medline DO - 10.7863/ultra.16.05028 IS - 4 KW - abnormalities aneurysm cardiovascular malformation complication diagnostic imaging Down syndrome echocardiography embryology female fetus echography genetic screening human male newborn pregnancy procedures retrospective study subclavian artery LA - English M3 - Article N1 - L618708006 2017-10-16 PY - 2017 SN - 1550-9613 SP - 785-790 ST - Aberrant Right Subclavian Artery: Correlation Between Fetal and Neonatal Abnormalities and Abnormal Genetic Screening or Testing T2 - Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine TI - Aberrant Right Subclavian Artery: Correlation Between Fetal and Neonatal Abnormalities and Abnormal Genetic Screening or Testing UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L618708006 http://dx.doi.org/10.7863/ultra.16.05028 VL - 36 ID - 762 ER - TY - JOUR AB - OBJECTIVES: Novel protocols were used to focus on dynamic cardiorespiratory function during submaximal exercise and on the recovery from 1-min pulses of exercise in children who had undergone Fontan corrections for single-ventricle lesions. BACKGROUND: Particularly in children, maximal oxygen uptake (VO2max), which is commonly used to assess the functional capability of patients after the Fontan procedure, is highly effort dependent and not physiologic and leads to uncomfortable metabolic and cardiorespiratory stress. Alternative approaches include the measurement of dynamic responses during progressive exercise and recovery after short bursts of exercise. These strategies yield mechanistic insight into cardiorespiratory impairment and can be used to gauge limitations in daily life activity. METHODS: Sixteen patients (mean [+/-SD] age 12.2 +/- 2.4 years; 9 boys) and 10 age-matched control subjects (mean age 12.2 +/- 2.4 years; 6 boys) performed two separate cycle ergometer tests in which gas exchange was measured on a breath by breath basis: 1) Progressive exercise was used to determine the dynamic relation among VO2, carbon dioxide production (VCO2), ventilation (VE), heart rate (HR) and work rate (WR). 2) A 1-min constant WR test was used to determine the recovery time for gas exchange and HR. RESULTS: Peak VO2 and anaerobic threshold were reduced in patients who underwent the Fontan procedure compared with control subjects by 57% and 52%, respectively (p < 0.001). Dynamic relations during progressive exercise--deltaVO2/deltaHR and deltaVO2/deltaWR--were decreased (p < 0.001) and deltaVE/deltaVCO2 was increased (p < 0.005) in the Fontan group patients. Recovery times for HR and VO2 were prolonged in the Fontan group patients by 154% and 69%, respectively (p < 0.01). CONCLUSIONS: The results demonstrate that submaximal gas exchange responses to progressive exercise and recovery times after brief high intensity exercise are abnormal in patients after the Fontan procedure. These observations complement the findings of reduced VO2max observed here and by others. We speculate that the mechanisms for these responses are related to 1) a pervasive reduction in stroke volume for both low and high intensity exercise, 2) an abnormal linkage of ventilation to tissue carbon dioxide production, and 3) increased dependence on anaerobic metabolism in skeletal muscles. The prolonged recovery of HR and VO2 provides a possible mechanism for reduced physical activity. AD - Department of Pediatrics, School of Medicine, University of California Los Angeles, Westwood, USA. WilliamvBvMaj.Troutman@KAFBVA.Mednet.AF.MIL AN - 9502651 AU - Troutman, W. B. AU - Barstow, T. J. AU - Galindo, A. J. AU - Cooper, D. M. DA - Mar 1 DB - PubMed DO - 10.1016/s0735-1097(97)00545-7 DP - NLM ET - 1998/03/21 IS - 3 KW - Adolescent Carbon Dioxide/metabolism Child *Exercise Exercise Test Female *Fontan Procedure Heart/*physiopathology Heart Function Tests Heart Rate Humans Lung/*physiopathology Male Oxygen/blood Respiration Respiratory Function Tests LA - eng N1 - Troutman, W B Barstow, T J Galindo, A J Cooper, D M HD 269239/HD/NICHD NIH HHS/United States Journal Article Research Support, U.S. Gov't, P.H.S. United States J Am Coll Cardiol. 1998 Mar 1;31(3):668-73. doi: 10.1016/s0735-1097(97)00545-7. PY - 1998 SN - 0735-1097 (Print) 0735-1097 SP - 668-73 ST - Abnormal dynamic cardiorespiratory responses to exercise in pediatric patients after Fontan procedure T2 - J Am Coll Cardiol TI - Abnormal dynamic cardiorespiratory responses to exercise in pediatric patients after Fontan procedure VL - 31 ID - 437 ER - TY - JOUR AB - Objectives: Novel protocols were used to focus on dynamic cardiorespiratory function during submaximal exercise and on the recovery from 1-min pulses of exercise in children who had undergone Fontan corrections for single-ventricle lesions.Background: Particularly in children, maximal oxygen uptake (VO2max), which is commonly used to assess the functional capability of patients after the Fontan procedure, is highly effort dependent and not physiologic and leads to uncomfortable metabolic and cardiorespiratory stress. Alternative approaches include the measurement of dynamic responses during progressive exercise and recovery after short bursts of exercise. These strategies yield mechanistic insight into cardiorespiratory impairment and can be used to gauge limitations in daily life activity.Methods: Sixteen patients (mean [+/-SD] age 12.2 +/- 2.4 years; 9 boys) and 10 age-matched control subjects (mean age 12.2 +/- 2.4 years; 6 boys) performed two separate cycle ergometer tests in which gas exchange was measured on a breath by breath basis: 1) Progressive exercise was used to determine the dynamic relation among VO2, carbon dioxide production (VCO2), ventilation (VE), heart rate (HR) and work rate (WR). 2) A 1-min constant WR test was used to determine the recovery time for gas exchange and HR.Results: Peak VO2 and anaerobic threshold were reduced in patients who underwent the Fontan procedure compared with control subjects by 57% and 52%, respectively (p < 0.001). Dynamic relations during progressive exercise--deltaVO2/deltaHR and deltaVO2/deltaWR--were decreased (p < 0.001) and deltaVE/deltaVCO2 was increased (p < 0.005) in the Fontan group patients. Recovery times for HR and VO2 were prolonged in the Fontan group patients by 154% and 69%, respectively (p < 0.01).Conclusions: The results demonstrate that submaximal gas exchange responses to progressive exercise and recovery times after brief high intensity exercise are abnormal in patients after the Fontan procedure. These observations complement the findings of reduced VO2max observed here and by others. We speculate that the mechanisms for these responses are related to 1) a pervasive reduction in stroke volume for both low and high intensity exercise, 2) an abnormal linkage of ventilation to tissue carbon dioxide production, and 3) increased dependence on anaerobic metabolism in skeletal muscles. The prolonged recovery of HR and VO2 provides a possible mechanism for reduced physical activity. AD - Division of Pediatric Cardiology, Department of Pediatrics, School of Medicine, University of California Los Angeles, Westwood, California, USA; Division of Respiratory and Critical Care, Department of Pediatrics, Harbor-UCLA Medical Center, Torrance, California, USA. Department of Pediatrics, School of Medicine, University of California Los Angeles, Westwood, USA AN - 116983157. Language: English. Entry Date: 20161127. Revision Date: 20190812. Publication Type: journal article AU - Troutman, William Brad AU - Barstow, Thomas J. AU - Galindo, Alvaro J. AU - Cooper, Dan M. AU - Troutman, W. B. AU - Barstow, T. J. AU - Galindo, A. J. AU - Cooper, D. M. DB - ccm DO - 10.1016/S0735-1097(97)00545-7 DP - EBSCOhost IS - 3 KW - Lung -- Physiopathology Heart -- Physiopathology Exercise Cardiopulmonary Bypass Male Exercise Test Female Oxygen -- Blood Respiratory Function Tests Adolescence Carbon Dioxide -- Metabolism Respiration Child Human Heart Function Tests Heart Rate Validation Studies Comparative Studies Evaluation Research Multicenter Studies Clinical Assessment Tools Exercise of Self-Care Agency Scale Scales Short Portable Mental Status Questionnaire N1 - research. Journal Subset: Biomedical; Peer Reviewed; USA. Instrumentation: Short Portable Mental Status Questionnaire (SPMSQ) (Pfeiffer); Longitudinal Interval Follow-Up Evaluation (LIFE); Work Environment Scale (WES) (Moos et al); Exercise of Self-Care Agency Scale (ESCA) (Kearney and Fleischer); Functional Living Index: Cancer (FLIC) (Schipper et al); Global Assessment Scale (GAS); Behavior Rating Inventory of Executive Function (BRIEF); Constant and Murley Score. Grant Information: HD 269239/HD/NICHD NIH HHS/United States. NLM UID: 8301365. PMID: NLM9502651. PY - 1998 SN - 0735-1097 SP - 668-673 ST - Abnormal dynamic cardiorespiratory responses to exercise in pediatric patients after Fontan procedure T2 - Journal of the American College of Cardiology (JACC) TI - Abnormal dynamic cardiorespiratory responses to exercise in pediatric patients after Fontan procedure UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=116983157&site=ehost-live&scope=site VL - 31 ID - 1601 ER - TY - JOUR AB - Objectives. Novel protocols were used to focus on dynamic cardiorespiratory function during submaximal exercise and on the recovery from 1-min pulses of exercise in children who had undergone Fontan corrections for single-ventricle lesions. Background. Particularly in children, maximal oxygen uptake (Vo2max), which is commonly used to assess the functional capability of patients after the Fontan procedure, is highly effort dependent and not physiologic and leads to uncomfortable metabolic and cardiorespiratory stress. Alternative approaches include the measurement of dynamic responses during progressive exercise and recovery after short bursts of exercise. These strategies yield mechanistic insight into cardiorespiratory impairment and can be used to gauge limitations in daily life activity. Methods. Sixteen patients (mean (±SD) age 12.2.± 2.4 years; 9 boys) and 10 age-matched control subjects (mean age 12.2 ± 2.4 years; 6 boys) performed two separate cycle ergometer test in which gas exchange was measured on a breath by basis:1) Progressive exercise was used to determine the dynamic relation among Vo2, carbon dioxide production (VCO2), ventilation (VE), heart rate (HR) and work rate (WR).2) A 1-min constant WR test was used to determine the recovery time for gas exchange and HR. Results. Peak VO2 and anaerobic threshold were reduced in patients who underwent the Fontan procedure compared with control subjects by 57% and 52%, respectively (p < 0.001). Dynamic relations during progressive exercise - ΔVO2/ΔHR and ΔVO2/ΔWR - were decreased (p < 0.001) and ΔE/ΔVCO2 was increased (p < 0.005) in the Fontan group patients. Recovery times for HR VO2 were prolonged in the Fontan group patients by 154% and 69%, respectively (p < 0.01). Conclusions. The results demonstrate that submaximal gas exchange response to progressive exercise and recovery times after brief intensity exercise are abnormal in patients after the Fontan procedure. These observations complement the findings of reduced VO2 max observed here and by others. We speculate that the mechanisms for these responses are related to 1) a pervasive reduction in stroke volume for both low and high intensity exercise, 2) an abnormal linkage of ventilation to tissue carbon dioxide production, and 3) increased dependence on anaerobic metabolism in skeletal muscles. The prolonged recovery of HR and VO2 provides a possible mechanism for reduced physical activity. AD - W.B. Troutman, 81 MDOS/SGOC, 301 Fisher Street, Air Force Base, MS 39534-2519, United States AU - Troutman, W. B. AU - Barstow, T. J. AU - Galindo, A. J. AU - Cooper, D. M. DB - Embase Medline DO - 10.1016/S0735-1097(97)00545-7 IS - 3 KW - anaerobic threshold article bicycle ergometry clinical article exercise test female follow up Fontan procedure heart single ventricle human lung gas exchange male oxygen tension priority journal school child treatment outcome LA - English M3 - Article N1 - L28144136 1998-05-05 PY - 1998 SN - 0735-1097 SP - 668-673 ST - Abnormal dynamic cardiorespiratory responses to exercise in pediatric patients after fonton procudure T2 - Journal of the American College of Cardiology TI - Abnormal dynamic cardiorespiratory responses to exercise in pediatric patients after fonton procudure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L28144136 http://dx.doi.org/10.1016/S0735-1097(97)00545-7 VL - 31 ID - 1352 ER - TY - JOUR AB - Background: Fontan patients exhibit a high prevalence of abnormal glucose metabolism (AGM). We aimed to characterize AGM and clarify its association with Fontan pathophysiology.Methods: We prospectively evaluated AGM with plasma glucose dynamics [mg/dL; fasting glucose (FPG), and maximum glucose increase (PG-spike)] during oral glucose tolerance test and hemoglobin A1c (HbA1c) in 276 consecutive Fontan patients (aged 19 ± 7 years). Of these, 176 patients had serial AGM assessments with a mean interval of 6.5 years.Results: Initial analysis revealed a high prevalence of impaired glucose tolerance (38.4%) and diabetes mellitus (DM) (4.7%), and positive family history, high HbA1c, and high central venous pressure independently predicted presence of DM. HbA1c was independently determined by hypersplenism and presence of DM (P < .05). Serial assessments revealed an increased PG-spike and a decreased HbA1c (P < .001 for both). Prevalence of DM increased (6.3% to 10.3%), and positive family history, high liver enzymes, and AGM predicted new onset of DM (P < .05 for all). Twenty-one patients died during 7.1-year follow-up. FPG (P < .01) and PG-spike (P < .05) independently predicted all-cause mortality. Particularly, patients with FPG ≤ 74 and/or PG-spike ≥85 had a mortality rate 8.7 times higher than those without (P = .0129).Conclusions: AGM progressed even in young adult Fontan patients, and HbA1c showed limited predictive value for progression. Oral glucose tolerance test plays important roles in uncovering unique Fontan AGM as well as predicting all-cause mortality. AD - Department of Pediatric Cardiology, Center for Cerebral and Cardiovascular Center, National Cerebral and Cardiovascular Center, Osaka, Japan Adult Congenital Heart Disease, Center for Cerebral and Cardiovascular Center, National Cerebral and Cardiovascular Center, Osaka, Japan Preventive Medicine and Epidemiologic Informatics, Center for Cerebral and Cardiovascular Center, National Cerebral and Cardiovascular Center, Osaka, Japan AN - 139526769. Language: English. Entry Date: In Process. Revision Date: 20200314. Publication Type: journal article. Journal Subset: Biomedical AU - Ohuchi, Hideo AU - Negishi, Jun AU - Hayama, Yosuke AU - Miike, Hikari AU - Suzuki, Dai AU - Nakajima, Kimiko AU - Konagai, Nao AU - Iwasa, Toru AU - Sakaguchi, Heima AU - Kurosaki, Kenichi AU - Nakai, Michikazu DB - ccm DO - 10.1016/j.ahj.2019.07.013 DP - EBSCOhost KW - Diabetes Mellitus -- Metabolism Glucose -- Metabolism Fasting Cardiopulmonary Bypass Young Adult Female Heart Defects, Congenital -- Metabolism Blood Glucose -- Metabolism Adolescence Hypersplenism -- Metabolism Prospective Studies Diabetes Mellitus -- Mortality Child Middle Age Liver Diseases -- Metabolism Adult Diabetes Mellitus -- Epidemiology Cause of Death Male Glucose Tolerance Test Prevalence Cross Sectional Studies Case Control Studies Predictive Value of Tests Disease Progression N1 - USA. NLM UID: 0370465. PMID: NLM31425899. PY - 2019 SN - 0002-8703 SP - 125-135 ST - Abnormal glucose metabolism in patients with Fontan circulation: Unique characteristics and associations with Fontan pathophysiology T2 - American Heart Journal TI - Abnormal glucose metabolism in patients with Fontan circulation: Unique characteristics and associations with Fontan pathophysiology UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=139526769&site=ehost-live&scope=site VL - 216 ID - 1442 ER - TY - JOUR AB - BackgroundFontan survivors have depressed cardiac index that worsens over time. Serum biomarker measurement is minimally invasive, rapid, widely available, and may be useful for serial monitoring. The purpose of this study was to identify biomarkers that correlate with lower cardiac index in Fontan patients.Methods and resultsThis study was a multi-centre case series assessing the correlations between biomarkers and cardiac magnetic resonance-derived cardiac index in Fontan patients ⩾6 years of age with biochemical and haematopoietic biomarkers obtained ±12 months from cardiac magnetic resonance. Medical history and biomarker values were obtained by chart review. Spearman’s Rank correlation assessed associations between biomarker z-scores and cardiac index. Biomarkers with significant correlations had receiver operating characteristic curves and area under the curve estimated. In total, 97 cardiac magnetic resonances in 87 patients met inclusion criteria: median age at cardiac magnetic resonance was 15 (6–33) years. Significant correlations were found between cardiac index and total alkaline phosphatase (−0.26, p=0.04), estimated creatinine clearance (0.26, p=0.02), and mean corpuscular volume (−0.32, p<0.01). Area under the curve for the three individual biomarkers was 0.63–0.69. Area under the curve for the three-biomarker panel was 0.75. Comparison of cardiac index above and below the receiver operating characteristic curve-identified cut-off points revealed significant differences for each biomarker (p<0.01) and for the composite panel [median cardiac index for higher-risk group=2.17 L/minute/m2 versus lower-risk group=2.96 L/minute/m2, (p<0.01)].ConclusionsHigher total alkaline phosphatase and mean corpuscular volume as well as lower estimated creatinine clearance identify Fontan patients with lower cardiac index. Using biomarkers to monitor haemodynamics and organ-specific effects warrants prospective investigation. AD - Department of Pediatrics, Division of Cardiology, Division of Critical Care Medicine, Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, United States of America Department of Pediatrics, Division of Cardiology, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania United States of America Department of Pediatrics, Division of Pediatric Cardiology, University of Michigan C.S. Mott Children’s Hospital, Ann Arbor, Michigan, United States of America Department of Pediatrics, Division of Biostatistics & Epidemiology, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, United States of America Department of Pediatrics, Division of General and Community Pediatrics, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, United States of America Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America Department of Pediatrics, Division of Pediatric Cardiology, Children’s Hospital of Wisconsin, Milwaukee, Wisconsin, United States of America Department of Pediatrics, Division of Cardiology, Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, Illinois United States of America Department of Pediatrics, Division of Cardiology, The Mayo Clinic, Rochester, Minnesota, United States of America Department of Pediatrics, Division of Cardiology, Nationwide Children’s Hospital, Columbus Ohio, United States of America Department of Pediatric and Congenital Heart Surgery, Cleveland Clinic, Cleveland, Ohio, United States of America Department of Pediatrics, Division of Pediatric Cardiology, University of Minnesota Amplatz Children’s Hospital, Minneapolis, Minnesota, United States of America Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, United States of America AN - 121745179. Language: English. Entry Date: 20170324. Revision Date: 20180101. Publication Type: journal article AU - Marino, Bradley S. AU - Goldberg, David J. AU - Dorfman, Adam L. AU - King, Eileen AU - Kalkwarf, Heidi AU - Zemel, Babette S. AU - Smith, Margaret AU - Pratt, Jesse AU - Fogel, Mark A. AU - Shillingford, Amanda J. AU - Deal, Barbara J. AU - John, Anitha S. AU - Goldberg, Caren S. AU - Hoffman, Timothy M. AU - Jacobs, Marshall L. AU - Lisec, Asher AU - Finan, Susan AU - Kochilas, Lazaros K. AU - Pawlowski, Thomas W. AU - Campbell, Kathleen DB - ccm DO - 10.1017/S1047951116000093 DP - EBSCOhost IS - 1 KW - Monitoring, Physiologic -- Methods Cardiac Output -- Physiology Cardiopulmonary Bypass -- Methods Heart Defects, Congenital -- Blood Prognosis Heart Defects, Congenital -- Surgery Adult Male Prospective Studies Heart Defects, Congenital -- Physiopathology Female Adolescence Young Adult Child ROC Curve Human Validation Studies Comparative Studies Evaluation Research Multicenter Studies N1 - research. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Instrumentation: Craig Handicap Assessment and Reporting Technique (CHART). NLM UID: 9200019. PMID: NLM28281411. PY - 2017 SN - 1047-9511 SP - 59-68 ST - Abnormalities in serum biomarkers correlate with lower cardiac index in the Fontan population T2 - Cardiology in the Young TI - Abnormalities in serum biomarkers correlate with lower cardiac index in the Fontan population UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=121745179&site=ehost-live&scope=site VL - 27 ID - 1496 ER - TY - JOUR AB - BACKGROUND: Fontan survivors have depressed cardiac index that worsens over time. Serum biomarker measurement is minimally invasive, rapid, widely available, and may be useful for serial monitoring. The purpose of this study was to identify biomarkers that correlate with lower cardiac index in Fontan patients. Methods and results This study was a multi-centre case series assessing the correlations between biomarkers and cardiac magnetic resonance-derived cardiac index in Fontan patients ⩾6 years of age with biochemical and haematopoietic biomarkers obtained ±12 months from cardiac magnetic resonance. Medical history and biomarker values were obtained by chart review. Spearman's Rank correlation assessed associations between biomarker z-scores and cardiac index. Biomarkers with significant correlations had receiver operating characteristic curves and area under the curve estimated. In total, 97 cardiac magnetic resonances in 87 patients met inclusion criteria: median age at cardiac magnetic resonance was 15 (6-33) years. Significant correlations were found between cardiac index and total alkaline phosphatase (-0.26, p=0.04), estimated creatinine clearance (0.26, p=0.02), and mean corpuscular volume (-0.32, p<0.01). Area under the curve for the three individual biomarkers was 0.63-0.69. Area under the curve for the three-biomarker panel was 0.75. Comparison of cardiac index above and below the receiver operating characteristic curve-identified cut-off points revealed significant differences for each biomarker (p<0.01) and for the composite panel [median cardiac index for higher-risk group=2.17 L/minute/m2 versus lower-risk group=2.96 L/minute/m2, (p<0.01)]. CONCLUSIONS: Higher total alkaline phosphatase and mean corpuscular volume as well as lower estimated creatinine clearance identify Fontan patients with lower cardiac index. Using biomarkers to monitor haemodynamics and organ-specific effects warrants prospective investigation. AD - 1Department of Pediatrics,Division of Cardiology,Division of Critical Care Medicine,Heart Institute,Cincinnati Children's Hospital Medical Center,Cincinnati,Ohio,United States of America. 7Department of Pediatrics,Division of Cardiology,The Children's Hospital of Philadelphia,Philadelphia,PennsylvaniaUnited States of America. 9Department of Pediatrics,Division of Pediatric Cardiology,University of Michigan C.S. Mott Children's Hospital,Ann Arbor,Michigan,United States of America. 2Department of Pediatrics,Division of Biostatistics & Epidemiology,Cincinnati Children's Hospital Medical Center,Cincinnati,Ohio,United States of America. 3Department of Pediatrics,Division of General and Community Pediatrics,Cincinnati Children's Hospital Medical Center,Cincinnati,Ohio,United States of America. 8Department of Pediatrics,Division of Gastroenterology, Hepatology and Nutrition,The Children's Hospital of Philadelphia,Philadelphia,Pennsylvania,United States of America. 10Department of Pediatrics,Division of Pediatric Cardiology,Children's Hospital of Wisconsin,Milwaukee,Wisconsin,United States of America. 11Department of Pediatrics,Division of Cardiology,Ann & Robert H. Lurie Children's Hospital of Chicago,Chicago,IllinoisUnited States of America. 12Department of Pediatrics,Division of Cardiology,The Mayo Clinic,Rochester,Minnesota,United States of America. 13Department of Pediatrics,Division of Cardiology,Nationwide Children's Hospital,ColumbusOhio,United States of America. 14Department of Pediatric and Congenital Heart Surgery,Cleveland Clinic,Cleveland,Ohio,United States of America. 15Department of Pediatrics,Division of Pediatric Cardiology,University of Minnesota Amplatz Children's Hospital,Minneapolis,Minnesota,United States of America. 4Department of Pediatrics,Division of Gastroenterology, Hepatology and Nutrition,Cincinnati Children's Hospital Medical Center,Cincinnati,Ohio,United States of America. 5Department of Pediatrics,Division of Hematology/Oncology,Cincinnati Children's Hospital Medical Center,Cincinnati,Ohio,United States of America. 6Department of Pediatrics,Division of Nephrology and Hypertension,Cincinnati Children's Hospital Medical Center,Cincinnati,Ohio,United States of America. AN - 28281411 AU - Marino, B. S. AU - Goldberg, D. J. AU - Dorfman, A. L. AU - King, E. AU - Kalkwarf, H. AU - Zemel, B. S. AU - Smith, M. AU - Pratt, J. AU - Fogel, M. A. AU - Shillingford, A. J. AU - Deal, B. J. AU - John, A. S. AU - Goldberg, C. S. AU - Hoffman, T. M. AU - Jacobs, M. L. AU - Lisec, A. AU - Finan, S. AU - Kochilas, L. K. AU - Pawlowski, T. W. AU - Campbell, K. AU - Joiner, C. AU - Goldstein, S. L. AU - Stephens, P. AU - Chin, A. J. DA - Jan DB - PubMed DO - 10.1017/s1047951116000093 DP - NLM ET - 2017/03/11 IS - 1 KW - Adolescent Adult Biomarkers/*blood Cardiac Output/*physiology Child Female Follow-Up Studies Fontan Procedure/*methods Heart Defects, Congenital/*blood/physiopathology/surgery Humans Male Monitoring, Physiologic/*methods Prognosis Prospective Studies ROC Curve Young Adult *Fontan *biomarkers *cardiac MRI *cardiac index LA - eng N1 - 1467-1107 Marino, Bradley S Goldberg, David J Dorfman, Adam L King, Eileen Kalkwarf, Heidi Zemel, Babette S Smith, Margaret Pratt, Jesse Fogel, Mark A Shillingford, Amanda J Deal, Barbara J John, Anitha S Goldberg, Caren S Hoffman, Timothy M Jacobs, Marshall L Lisec, Asher Finan, Susan Kochilas, Lazaros K Pawlowski, Thomas W Campbell, Kathleen Joiner, Clinton Goldstein, Stuart L Stephens, Paul Chin, Alvin J Journal Article Multicenter Study England Cardiol Young. 2017 Jan;27(1):59-68. doi: 10.1017/S1047951116000093. PY - 2017 SN - 1047-9511 SP - 59-68 ST - Abnormalities in serum biomarkers correlate with lower cardiac index in the Fontan population T2 - Cardiol Young TI - Abnormalities in serum biomarkers correlate with lower cardiac index in the Fontan population VL - 27 ID - 217 ER - TY - JOUR AD - Sch of Nursing, UCLA, Los Angeles, CA Sch of Medicine - Div of Anesthesiology, UCLA, Los Angeles, CA AN - 135764627. Language: English. Entry Date: In Process. Revision Date: 20190909. Publication Type: Article. Supplement Title: 2018 Supplement. Journal Subset: Biomedical AU - Moye, Stefanie AU - Woo, Mary A. AU - Roy, Bhaswati AU - Singh, Sadhana AU - Kumar, Rajesh AU - Pike, Nancy A. DB - ccm DP - EBSCOhost N1 - Peer Reviewed; USA. NLM UID: 0147763. PY - 2018 SN - 0009-7322 SP - A12726-A12726 ST - Abstract 12726: Hippocampal Brain Volume, Single Ventricle Diagnosis, and Anxiety Predict Cognitive Deficits in Adolescents T2 - Circulation TI - Abstract 12726: Hippocampal Brain Volume, Single Ventricle Diagnosis, and Anxiety Predict Cognitive Deficits in Adolescents UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=135764627&site=ehost-live&scope=site VL - 138 ID - 1526 ER - TY - JOUR AD - DEPARTMENT OF PEDIATRICS, Childrens Hosp Philadelphia, Philadelphia, PA CHPS, Childrens Hosp Philadelphia, Philadelphia, PA AN - 135764863. Language: English. Entry Date: In Process. Revision Date: 20190909. Publication Type: Article. Supplement Title: 2018 Supplement. Journal Subset: Biomedical AU - Bhatt, Shivani M. AU - Decost, Grace AU - Wang, Yan AU - Kim, Ji Young AU - Goldberg, David J. AU - McBride, Michael AU - Paridon, Stephen AU - Mercer-Rosa, Laura DB - ccm DP - EBSCOhost N1 - Peer Reviewed; USA. NLM UID: 0147763. PY - 2018 SN - 0009-7322 SP - A13093-A13093 ST - Abstract 13093: Exercise Stress Echocardiography Demonstrates Impaired Contractile Reserve in Youth With Fontan Circulation and Predicts Exercise Performance T2 - Circulation TI - Abstract 13093: Exercise Stress Echocardiography Demonstrates Impaired Contractile Reserve in Youth With Fontan Circulation and Predicts Exercise Performance UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=135764863&site=ehost-live&scope=site VL - 138 ID - 1527 ER - TY - JOUR AB - Background - Most studies evaluating neurocognitive outcomes in children with congenital heart defects (CHD) have focused on high-risk patients or used specialized, resource-intensive testing. To determine the association of CHD with academic outcomes and compare outcomes according to the severity of CHD, we linked state educational records with a birth defects registry and birth certificates. Methods and Results - We performed a retrospective cohort study using data from the North Carolina Birth Defects Monitoring Program, North Carolina Department of Public Instruction, and North Carolina Department of Health and Human Services vital records. We performed logistic regression, adjusting for maternal education, race/ethnicity, enrollment in public pre-Kindergarten, and gestational age, to determine the association of CHD with not meeting standards on reading and math end-of-grade examinations in third grade in 2006 to 2012. Of 5624 subjects with CHD and 10 832 with no structural birth defects, 2807 (50%) and 6355 (59%) were linked, respectively. Children with CHD had 1.24× the odds of not meeting standards in either reading or math (95% confidence interval, 1.12-1.37), with 44.6% of children with CHD not meeting standards in at least one of these areas compared with 37.5% without CHD. Although children with both critical and noncritical CHD had poorer outcomes, those with critical CHD were significantly more likely to receive exceptional services compared with the noncritical group (adjusted odds ratio, 1.46; 95% confidence interval, 1.15-1.86). Conclusions - Children with all types of CHD have poorer academic outcomes compared with their peers. Evaluation for exceptional services should be considered in children with any type of CHD. © 2017 American Heart Association, Inc. AD - Department of Pediatrics, Emory University, School of Medicine, Atlanta, GA, United States Department of Cardiology, Children's Healthcare of Atlanta, Atlanta, GA, United States Department of Epidemiology, Emory University, Rollins School of Public Health, Atlanta, GA, United States Department of Physical Therapy, Methodist University, Fayetteville, NC, United States Department of Pediatrics, Duke University Medical Center, Duke Clinical Research Institute, Durham, NC, United States Birth Defects Monitoring Branch, State Center for Health Statistics, North Carolina Division of Public Health, Raleigh, NC, United States AU - Oster, M. E. AU - Watkins, S. AU - Hill, K. D. AU - Knight, J. H. AU - Meyer, R. E. C7 - e003074 DB - Scopus DO - 10.1161/CIRCOUTCOMES.116.003074 IS - 2 KW - birth certificates child education gestational age heart defects, congenital registries M3 - Article N1 - Cited By :13 Export Date: 15 June 2020 PY - 2017 ST - Academic outcomes in children with congenital heart defects T2 - Circulation: Cardiovascular Quality and Outcomes TI - Academic outcomes in children with congenital heart defects UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85014657045&doi=10.1161%2fCIRCOUTCOMES.116.003074&partnerID=40&md5=62cd7124474337b52c13d377387bfc4e VL - 10 ID - 1874 ER - TY - JOUR AB - Although advances in medical and surgical arenas have allowed the ability to "mend" children born with congenital heart disease, the increasing number of survivors has created a growing cohort of children with potential academic difficulties. This article reviews the current understanding of the midterm neurologic outcomes of children who underwent neonatal and infant cardiac surgery. Lesions including transposition of great arteries, tetralogy of Fallot, hypoplastic left heart syndrome, total anomalous pulmonary venous connection, and single ventricle lesions requiring Fontan palliation or cardiac transplantation are discussed. AD - Division of Pediatric Cardiology, Department of Pediatrics, The Cardiac Center, The Children's Hospital of Philadelphia, 34th & Civic Center Boulevard, Philadelphia, PA 19104, USA. shillingford@email.chop.edu AN - 15561177 AU - Shillingford, A. J. AU - Wernovsky, G. DA - Dec DB - PubMed DO - 10.1016/j.pcl.2004.07.007 DP - NLM ET - 2004/11/25 IS - 6 KW - Child Behavior Disorders/*etiology Heart Defects, Congenital/complications/*surgery Humans Infant Infant, Newborn Learning Disabilities/*etiology *Postoperative Complications Risk Factors Treatment Outcome LA - eng N1 - Shillingford, Amanda J Wernovsky, Gil Journal Article Review United States Pediatr Clin North Am. 2004 Dec;51(6):1625-39, ix. doi: 10.1016/j.pcl.2004.07.007. PY - 2004 SN - 0031-3955 (Print) 0031-3955 SP - 1625-39, ix ST - Academic performance and behavioral difficulties after neonatal and infant heart surgery T2 - Pediatr Clin North Am TI - Academic performance and behavioral difficulties after neonatal and infant heart surgery VL - 51 ID - 189 ER - TY - JOUR AB - In the modern era, it is possible to achieve programmatic balance in the approach to the treatment of hypoplastic left heart syndrome by offering staged palliation, transplantation, and in suitable cases, biventricular repair. Strategies for optimal selection should continue to improve and evolve. Overall, the current hope for survival, and excellent quality of life, for patients with hypoplastic left heart syndrome and other forms of functionally univentricular heart is better than ever before. Pioneering efforts allowed what we achieve today. Our challenge is to continue to improve the surgical and medical care of these patients towards normalization of survival and quality of life for future generations. These goals will be achieved by capitalizing on current advances in the field, and continuing to "push the envelope", with research into new advances. AD - Congenital Heart Institute of Florida, University of South Florida College of Medicine, All Children's Hospital, St. Petersburg, Florida 33701, USA. AN - 15244153 AU - Quintessenza, J. A. AU - Morell, V. O. AU - Jacobs, J. P. DA - Feb DB - PubMed DO - 10.1017/s1047951104006456 DP - NLM ET - 2004/07/13 KW - Adolescent Cardiac Surgical Procedures/*methods/trends Child Clinical Trials as Topic Forecasting Heart Transplantation/*methods/trends Humans Hypoplastic Left Heart Syndrome/*surgery Palliative Care/trends Reconstructive Surgical Procedures/*methods/trends LA - eng N1 - Quintessenza, James A Morell, Victor O Jacobs, Jeffrey P Journal Article Review England Cardiol Young. 2004 Feb;14 Suppl 1:127-30. doi: 10.1017/s1047951104006456. PY - 2004 SN - 1047-9511 (Print) 1047-9511 SP - 127-30 ST - Achieving a balance in the current approach to the surgical treatment of hypoplastic left heart syndrome T2 - Cardiol Young TI - Achieving a balance in the current approach to the surgical treatment of hypoplastic left heart syndrome VL - 14 Suppl 1 ID - 347 ER - TY - JOUR AN - 18095980 AU - Weber, M. D. AU - Thammasitboon, S. AU - Rosen, D. A. DA - Jan DB - PubMed DO - 10.1111/j.1460-9592.2007.02377.x DP - NLM ET - 2007/12/22 IS - 1 KW - Analgesia, Epidural Blood Pressure/drug effects Cardiac Catheterization Cardiovascular Diseases/chemically induced/physiopathology Child, Preschool Dexmedetomidine/*adverse effects Fontan Procedure Heart Rate/drug effects Humans Hypnotics and Sedatives/*adverse effects Hypoplastic Left Heart Syndrome/complications/surgery Male Midazolam Substance Withdrawal Syndrome/*physiopathology LA - eng N1 - Weber, Mark D Thammasitboon, Satid Rosen, David A Case Reports Letter France Paediatr Anaesth. 2008 Jan;18(1):87-8. doi: 10.1111/j.1460-9592.2007.02377.x. PY - 2008 SN - 1155-5645 (Print) 1155-5645 SP - 87-8 ST - Acute discontinuation syndrome from dexmedetomidine after protracted use in a pediatric patient T2 - Paediatr Anaesth TI - Acute discontinuation syndrome from dexmedetomidine after protracted use in a pediatric patient VL - 18 ID - 497 ER - TY - JOUR AB - INTRODUCTION: Cardiopulmonary compromises are infrequent but high-acuity events that occur at pediatric hospitals. Simulation is a powerful modality to teach fellows in pediatric critical care, cardiology, and neonatology important clinical skills in managing complex cardiorespiratory pathophysiology in infants with cyanotic heart disease. METHODS: We developed three simulation cases of hypoxemia involving differing complex cardiorespiratory pathophysiology in neonates/infants with cyanotic heart disease. Through teamwork, the participants were expected to recognize hypoxemia, work through a differential diagnosis, and implement the medical intervention needed to temporize while awaiting further procedures. Assessment of the participants' performance was via direct observation during the simulated activity. Debriefing occurred immediately using a formal debriefing framework. RESULTS: In 10 years, these three cases have been utilized approximately 48 times. Participants subjectively increased their confidence in managing cardiopulmonary events and improved their teamwork and communication skills in similar high-stress events. DISCUSSION: This unique module advanced learners' knowledge by building on their Pediatric Advanced Life Support and Neonatal Resuscitation Program foundation, identified management deficits in the care of patients with complex cardiorespiratory pathophysiology, and taught effective teamwork with role assignment and closed-loop communication. AD - Assistant Professor of Clinical Anesthesiology and Critical Care, Department of Anesthesiology and Critical Care, Perelman School of Medicine at the University of Pennsylvania. Cardiac Intensivist, Division of Cardiac Critical Care Medicine, Children's Hospital of Philadelphia. AN - 30800906 AU - Peddy, S. B. C2 - PMC6342404 DA - Apr 17 DB - PubMed DO - 10.15766/mep_2374-8265.10706 DP - NLM ET - 2019/02/26 KW - Curriculum Cyanosis/etiology/physiopathology Fellowships and Scholarships/methods Female Heart/*anatomy & histology/physiopathology Heart Defects, Congenital/complications/therapy Humans Hypoxia/etiology/*therapy Infant Infant, Newborn Male Patient Simulation Pediatrics/*education Resuscitation/education/methods *Hypoplastic Left Heart Syndrome *Hypoxemia *Hypoxia *Tetralogy of Fallot *Transposition of Great Arteries *Transposition of Great Vessels LA - eng N1 - 2374-8265 Peddy, Stacie B Journal Article MedEdPORTAL. 2018 Apr 17;14:10706. doi: 10.15766/mep_2374-8265.10706. PY - 2018 SN - 2374-8265 SP - 10706 ST - Acute Hypoxemia in Infants With Cyanotic Complex Cardiac Anatomy: Simulation Cases for Pediatric Fellows T2 - MedEdPORTAL TI - Acute Hypoxemia in Infants With Cyanotic Complex Cardiac Anatomy: Simulation Cases for Pediatric Fellows VL - 14 ID - 196 ER - TY - JOUR AB - In hypoplastic left heart syndrome, thrombosis of the native ascending aorta is rare and often fatal; there are no previously reported cases presenting with acute heart block. We review a case of native ascending aorta thrombosis in a 2-year-old boy with hypoplastic left heart syndrome, presenting with acute heart block. This case highlights the benefit of multi-modality imaging in complex cases. © Cambridge University Press 2014. AD - Department of Pediatrics, Division of Cardiolgy, University of Colorado, Aurora, CO, United States Department of Surgery; Children's Hospital Colorado, University of Colorado, Aurora, CO, United States AU - Burkett, D. A. AU - Wilson, N. AU - Mitchell, M. B. AU - Younoszai, A. K. DB - Scopus DO - 10.1017/S1047951114002558 IS - 1 KW - complete heart block congenital heart disease Echocardiography myocardial infarction M3 - Article N1 - Export Date: 15 June 2020 PY - 2014 SP - 151-154 ST - Acute ischaemic heart block in hypoplastic left heart syndrome T2 - Cardiology in the Young TI - Acute ischaemic heart block in hypoplastic left heart syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84954197451&doi=10.1017%2fS1047951114002558&partnerID=40&md5=8ad85a4bbe17289ebbba4c647b628f61 VL - 26 ID - 1979 ER - TY - JOUR AB - Ischemic strokes in childhood are rare. Thrombolytic therapy with intravenous (IV) tissue plasminogen activator (tPA) has been the main intervention for the management of pediatric stroke patients, but safety data are lacking and efficacy has been questioned. Recently, successful endovascular treatments for acute ischemic stroke in children have been reported with increasing frequency, suggesting that mechanical thrombectomy can be a safe and effective treatment. We present the case of a 22-month-old child with acute ischemic stroke due to basilar artery occlusion that was successfully treated with a stent retriever. AD - J.J. Gemmete, Departmentof Radiology and Neurosurgery, University of Michigan Health System, 1500 E Medical Center Dr, UH B1 D328, Ann Arbor, MI, United States AU - Savastano, L. AU - Gemmete, J. J. AU - Pandey, A. S. AU - Roark, C. AU - Chaudhary, N. DB - Embase Medline DO - 10.1136/bcr-2015-011821 KW - acetylsalicylic acid biotinidase nicardipine warfarin artery puncture article ataxia basilar artery obstruction biotinidase deficiency brain circulus arteriosus brain infarction brain ischemia case report child computer assisted tomography echocardiography female follow up Fontan procedure gait disorder guiding catheter heart atrium septum defect heart ventricle septum defect human intracardiac thrombosis lung artery banding magnetic resonance angiography medical history mental disease mesencephalon microcatheter muscle tone nuclear magnetic resonance imaging physical examination pons postoperative period priority journal school child stent retriever thalamus tricuspid valve atresia unsteadiness vertebral arteriography Cello Rebar Solitaire LA - English M3 - Article N1 - L605648609 2015-08-25 2015-08-28 PY - 2015 SN - 1757-790X ST - Acute ischemic stroke in a child due to basilar artery occlusion treated successfully with a stent retriever T2 - BMJ Case Reports TI - Acute ischemic stroke in a child due to basilar artery occlusion treated successfully with a stent retriever UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L605648609 http://dx.doi.org/10.1136/bcr-2015-011821 VL - 2015 ID - 860 ER - TY - JOUR AB - Ischemic strokes in childhood are rare. Thrombolytic therapy with intravenous (IV) tissue plasminogen activator (tPA) has been the main intervention for the management of pediatric stroke patients, but safety data are lacking and efficacy has been questioned. Recently, successful endovascular treatments for acute ischemic stroke in children have been reported with increasing frequency, suggesting that mechanical thrombectomy can be a safe and effective treatment. We present the case of a 22-month-old child with acute ischemic stroke due to basilar artery occlusion that was successfully treated with a stent retriever. AD - J.J. Gemmete, Departments of Radiology and Neurosurgery, 1500 E Medical Center Dr, UH B1 D328, University of Michigan Health System, Ann Arbor, MI, United States AU - Savastano, L. AU - Gemmete, J. J. AU - Pandey, A. S. AU - Roark, C. AU - Chaudhary, N. DB - Embase Medline DO - 10.1136/bcr-2015-011821 IS - 8 KW - acetylsalicylic acid biotinidase nicardipine warfarin artery puncture article ataxia basilar artery obstruction biotinidase deficiency brain circulus arteriosus brain infarction brain ischemia case report child computer assisted tomography echocardiography female follow up Fontan procedure gait disorder guiding catheter heart atrium septum defect heart ventricle septum defect human intracardiac thrombosis lung artery banding magnetic resonance angiography medical history mental disease mesencephalon microcatheter muscle tone nuclear magnetic resonance imaging physical examination pons postoperative period priority journal school child stent retriever thalamus tricuspid valve atresia unsteadiness vertebral arteriography Cello Rebar Solitaire LA - English M3 - Article N1 - L611567936 2016-08-10 2016-08-22 PY - 2016 SN - 1759-8486 1759-8478 SP - e33 ST - Acute ischemic stroke in a child due to basilar artery occlusion treated successfully with a stent retriever T2 - Journal of NeuroInterventional Surgery TI - Acute ischemic stroke in a child due to basilar artery occlusion treated successfully with a stent retriever UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L611567936 http://dx.doi.org/10.1136/bcr-2015-011821 VL - 8 ID - 800 ER - TY - JOUR AB - BACKGROUND: Stroke is a common presentation in geriatric patients in emergency department but rarely seen in pediatric patients. In case of acute ischemic stroke in pediatric age group, management is different from that of adult ischemic stroke where thrombolysis is a good option. METHODS: We report a case of a 17-year-old male child presenting in emergency with an episode of acute ischemic stroke causing left hemiparesis with left facial weakness and asymmetry. The patient suffered from cyanotic congenital heart disease for which he had undergone Fontan operation previously. He had a history of missing his daily dose of warfarin for last 3 days prior to the stroke. RESULTS: The patient recovered from acute ischemic stroke without being thrombolyzed. CONCLUSION: In pediatric patients, acute ischemic stroke usually is evolving and may not require thrombolysis. © 2012 World Journal of Emergency Medicine. AD - M. Mohammad, Department of Emergency Medicine, Max Super Speciality Hospital, New Delhi, India AU - Mohammad, M. AU - James, A. F. AU - Qureshi, R. S. AU - Saraf, S. AU - Ahluwalia, T. AU - Mukherji, J. D. AU - Kole, T. DB - Embase DO - 10.5847/wjem.j.1920-8642.2012.02.014 IS - 2 KW - enalapril low molecular weight heparin warfarin adolescent anticoagulation article brain ischemia case report congenital heart disease cyanotic heart disease echocardiography emergency ward face asymmetry face muscle Fontan procedure hemiparesis hospital patient human image analysis limb weakness male medical history muscle weakness nuclear magnetic resonance imaging treatment refusal LA - English M3 - Article N1 - L365171219 2012-07-12 2012-07-26 PY - 2012 SN - 1920-8642 SP - 154-156 ST - Acute ischemic stroke in a child with cyanotic congenital heart disease due to non-compliance of anticoagulation T2 - World Journal of Emergency Medicine TI - Acute ischemic stroke in a child with cyanotic congenital heart disease due to non-compliance of anticoagulation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L365171219 http://dx.doi.org/10.5847/wjem.j.1920-8642.2012.02.014 VL - 3 ID - 1056 ER - TY - JOUR AB - Background Successful transition to adult healthcare is particularly important for congenital heart disease (CHD) patients who have undergone palliative surgery, as they risk adverse events if not followed closely. This study examines young people at the worst end of the CHD spectrum who are born with a single ventricle (pumping heart chamber), and who undergo a series of operations that culminate in the Fontan surgical procedure. Purpose To explore adolescents with a Fontan circulation, and their parents', readiness for transition to adult care and pre-implementation of a transition program. Methods Seventeen adolescents (15-18 years) and 15 of their parents completed questionnaires at the start of their first transition clinic. Results Adolescents reported poor knowledge about their Fontan circulation, and 41% had a poor understanding of the purpose of their medications/treatments. Over half of the adolescents had poor knowledge around medical help-seeking (when, who, how). Most reported feeling comfortable with discussing their medical issues with their cardiologist, but considerably less so about sensitive adolescent issues, in particular, emotional wellbeing. Parents reported high levels of anxiety around transition to adult care services. Conclusion Findings pre-program indicate poor adolescent health knowledge, a lack of focus in health services on emotional wellbeing and high parental anxiety. These findings highlight the need for dedicated programs that focus on early preparation, parental involvement and acknowledgment, transition as a process, strong integration and prioritisation in the health system with a youth-friendly and holistic focus, in particular, around emotional wellbeing. AD - Heart Research Group, Murdoch Children's Research Institute, Melbourne, Victoria, 50 Flemington Road, Parkville, Melbourne, Victoria, 3052, Australia, Phone: +61 393456161. Transition Support Service, The Royal Children's Hospital, Melbourne, Victoria, Australia. Heart Research Group, Murdoch Children's Research Institute, Melbourne, Victoria, Australia. Department of Cardiac Surgery, The Royal Children's Hospital, Melbourne, Victoria, Australia. AN - 28599384 AU - Du Plessis, K. AU - Culnane, E. AU - Peters, R. AU - d'Udekem, Y. DA - May 24 DB - PubMed DO - 10.1515/ijamh-2017-0021 DP - NLM ET - 2017/06/11 IS - 4 KW - Fontan circulation transition from paediatric to adult care transition support programs LA - eng N1 - 2191-0278 Du Plessis, Karin Culnane, Evelyn Peters, Rebecca d'Udekem, Yves Journal Article Germany Int J Adolesc Med Health. 2017 May 24;31(4):/j/ijamh.2019.31.issue-4/ijamh-2017-0021/ijamh-2017-0021.xml. doi: 10.1515/ijamh-2017-0021. PY - 2017 SN - 0334-0139 ST - Adolescent and parent perspectives prior to involvement in a Fontan transition program T2 - Int J Adolesc Med Health TI - Adolescent and parent perspectives prior to involvement in a Fontan transition program VL - 31 ID - 87 ER - TY - JOUR AB - Background Successful transition to adult healthcare is particularly important for congenital heart disease (CHD) patients who have undergone palliative surgery, as they risk adverse events if not followed closely. This study examines young people at the worst end of the CHD spectrum who are born with a single ventricle (pumping heart chamber), and who undergo a series of operations that culminate in the Fontan surgical procedure. Purpose To explore adolescents with a Fontan circulation, and their parents', readiness for transition to adult care and pre-implementation of a transition program. Methods Seventeen adolescents (15-18 years) and 15 of their parents completed questionnaires at the start of their first transition clinic. Results Adolescents reported poor knowledge about their Fontan circulation, and 41% had a poor understanding of the purpose of their medications/treatments. Over half of the adolescents had poor knowledge around medical help-seeking (when, who, how). Most reported feeling comfortable with discussing their medical issues with their cardiologist, but considerably less so about sensitive adolescent issues, in particular, emotional wellbeing. Parents reported high levels of anxiety around transition to adult care services. Conclusion Findings pre-program indicate poor adolescent health knowledge, a lack of focus in health services on emotional wellbeing and high parental anxiety. These findings highlight the need for dedicated programs that focus on early preparation, parental involvement and acknowledgment, transition as a process, strong integration and prioritisation in the health system with a youth-friendly and holistic focus, in particular, around emotional wellbeing. AD - Heart Research Group, Murdoch Children's Research Institute, Melbourne, Victoria, 50 Flemington Road, Parkville, Melbourne, Victoria, 3052, Australia, Phone: +61 393456161 Transition Support Service, The Royal Children's Hospital, Melbourne, Victoria, Australia Heart Research Group, Murdoch Children's Research Institute, Melbourne, Victoria, Australia Department of Cardiac Surgery, The Royal Children's Hospital, Melbourne, Victoria, Australia AN - 138503595. Language: English. Entry Date: 20200222. Revision Date: 20190912. Publication Type: journal article. Journal Subset: Biomedical AU - Du Plessis, Karin AU - Culnane, Evelyn AU - Peters, Rebecca AU - d'Udekem, Yves DB - ccm DO - 10.1515/ijamh-2017-0021 DP - EBSCOhost IS - 4 N1 - Middle East; Peer Reviewed. NLM UID: 8506960. PMID: NLM28599384. PY - 2019 SN - 0334-0139 SP - N.PAG-N.PAG ST - Adolescent and parent perspectives prior to involvement in a Fontan transition program T2 - International Journal of Adolescent Medicine & Health TI - Adolescent and parent perspectives prior to involvement in a Fontan transition program UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=138503595&site=ehost-live&scope=site VL - 31 ID - 1443 ER - TY - JOUR AB - Continued advances in the understanding and management of congenital heart disease (CHD) mean that over 90% of children born with CHD now survive to adulthood. This in turn results in greater numbers of adult patients presenting for medical and surgical care at non-specialist centres. A simple classification of adult congenital heart disease (ACHD) according to complexity can help clinicians to understand the implications of the specific cardiac anomaly encountered. Issues relating to the conduct of anaesthesia in ACHD patient include careful attention to euvolaemia, the preservation of sinus rhythm and cardiac output, and in complex patients manipulating the balance between systemic and pulmonary blood flows. Additionally, effective antibiotic prophylaxis and the prevention of either excessive bleeding or thromboembolism are vitally important. It should not be forgotten that although many patients with simple or repaired cardiac lesions may be very well managed in a non-specialist unit, those with Eisenmenger's syndrome or severe pulmonary hypertension have an extremely high risk of death in the perioperative period, and in all but life-threatening situations should always be managed within specialist centres. AU - Weale, J. AU - Kelleher, A. A. DB - Embase DO - 10.1016/j.mpaic.2015.07.007 IS - 10 KW - anesthetic agent adult congenital heart disease anesthesia anesthesia level antibiotic prophylaxis article atrioventricular septal defect bleeding congenital heart disease disease classification Ebstein anomaly Eisenmenger complex Fallot tetralogy heart arrhythmia heart atrium septum defect heart failure heart injury heart ventricle septum defect human hypoplastic left heart syndrome postoperative care pregnancy priority journal psychological aspect pulmonary hypertension pulmonary valve atresia regional anesthesia thromboembolism LA - English M3 - Article N1 - L605937011 2015-09-14 2015-10-20 PY - 2015 SN - 1878-7584 1472-0299 SP - 528-534 ST - Adult congenital heart disease T2 - Anaesthesia and Intensive Care Medicine TI - Adult congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L605937011 http://dx.doi.org/10.1016/j.mpaic.2015.07.007 VL - 16 ID - 893 ER - TY - JOUR AB - The population of adults with congenital heart disease (CHD) (commonly called grown-ups with congenital heart disease or GUCH) is increasing steadily and exceeds the population of children with CHD already. The specificities of GUCH surgery are multiple and include (1) variety of the anatomo-clinical situations (defects repaired during childhood, malformations either nonoperated or palliated, nonreparable defects), (2) usual multiorgan involvement, and (3) many technical differences related to cardiopulmonary bypass, myocardial protection, and surgical technique. The surgical indications should be taken after a precise evaluation of the risk/benefit ratio on an individual basis; a balanced attitude should be kept between unwise interventionism and excessive waiting policy. It is now agreed that GUCH surgery should be performed in specialized centers with large patient volumes and expertise of both surgical and medical disciplines. Much remains to be done to implement these recommendations and to accumulate experience and evidence-based information to provide optimal outcome. © 2011 Elsevier Inc. AD - P.R. Vouhé, Chirurgie Cardiaque Pédiatrique, Hôpital Necker, Enfants Malades, 149 Rue de Sèvres, 75015 Paris, France AU - Vouhé, P. R. DB - Embase Medline DO - 10.1053/j.semtcvs.2011.09.003 IS - 3 KW - antiarrhythmic agent anticoagulant agent antithrombocytic agent blood clotting factor erythropoietin fresh frozen plasma heparin adult congenital heart surgery air embolism aortic clamping article biventricular malformation bleeding blood vessel occlusion cardiac graft rejection cardiopulmonary bypass cerebrovascular accident chromosome disorder clinical competence clinical decision making comorbidity computer assisted tomography congenital heart disease conservative treatment coronary artery disease cyanosis echocardiography embolism evidence based medicine Fontan procedure functional status graft failure grown ups with congenital heart disease heart catheterization heart disease heart muscle fibrosis heart protection heart surgery heart transplantation heart ventricle hypertrophy human long term care lung blood vessel lung vascular resistance nuclear magnetic resonance imaging palliative therapy phlebotomy plasma transfusion postoperative complication postoperative hemorrhage prognosis prosthesis pulmonary vascular disease quality of life recurrent disease reoperation risk benefit analysis risk factor silent myocardial ischemia sternotomy surgical training survival rate teamwork thoracotomy thrombocyte transfusion treatment indication ultrafiltration vascular access LA - English M3 - Article N1 - L363091126 2011-12-21 2012-02-07 PY - 2011 SN - 1043-0679 1532-9488 SP - 209-215 ST - Adult congenital surgery: Current management T2 - Seminars in Thoracic and Cardiovascular Surgery TI - Adult congenital surgery: Current management UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L363091126 http://dx.doi.org/10.1053/j.semtcvs.2011.09.003 VL - 23 ID - 1076 ER - TY - JOUR AB - BACKGROUND: Limited data exist regarding the perioperative morbidity profile of adults who have cardiac operations at pediatric facilities. METHODS: A retrospective review (January 2000 to December 2004) of patients (aged 18 years or older) who underwent cardiac surgery at our pediatric institution was performed. RESULTS: There were 149 cardiac operations performed in 135 patients. There were 2 early deaths. There were 70 preoperative noncardiac morbidities in 49 patients (36%) and 140 preoperative cardiac morbidities in 78 patients (58%). Preoperative arrhythmia (n = 76) and moderate or greater ventricular systolic dysfunction (n = 24) were most common. There were 51 postoperative adverse noncardiac events in 32 patients (24%). Renal insufficiency (> 0.5 mg/dL baseline change; n = 8) was most common. There were 53 postoperative adverse cardiac events in 44 patients (33%). Ventricular tachycardia (n = 13) was most common. Risk factors for postoperative adverse noncardiac events included preoperative histories of New York Heart Association (NYHA) class III or greater (p < 0.001), seizure (p = 0.04), and psychiatric disorder (p = 0.002). Risk factors for postoperative adverse cardiac events included older patient age (p = 0.001), preoperative functional single ventricle (p = 0.006), NHYA class III or greater (p = 0.003), atrial fibrillation/flutter (p < 0.001), and ventricular tachycardia or fibrillation (p = 0.04). CONCLUSIONS: Postoperative adverse events occur frequently when adults undergo cardiac operations at children's hospitals. Older patient age, preoperative arrhythmias, and preoperative NHYA class are predictors of postoperative adverse cardiac events. AD - Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas 77030, USA. jrossano@bcm.tmc.edu AN - 17257995 AU - Rossano, J. W. AU - Smith, E. O. AU - Fraser, C. D., Jr. AU - McKenzie, E. D. AU - Chang, A. C. AU - Hemingway, A. AU - Price, J. F. AU - Dickerson, H. A. AU - Mott, A. R. DA - Feb DB - PubMed DO - 10.1016/j.athoracsur.2006.08.058 DP - NLM ET - 2007/01/30 IS - 2 KW - Adult Age Factors Arrhythmias, Cardiac/complications Cardiac Surgical Procedures/*adverse effects Cohort Studies Female Heart Diseases/complications/physiopathology/surgery Hospitals, Pediatric/*statistics & numerical data Humans Incidence Intraoperative Complications/*epidemiology Male Medical Records Mental Disorders/complications Middle Aged Postoperative Complications/*epidemiology Prognosis Retrospective Studies Risk Factors Seizures/complications Severity of Illness Index LA - eng N1 - 1552-6259 Rossano, Joseph W Smith, E O'Brian Fraser, Charles D Jr McKenzie, E Dean Chang, Anthony C Hemingway, Amy Price, Jack F Dickerson, Heather A Mott, Antonio R Journal Article Netherlands Ann Thorac Surg. 2007 Feb;83(2):606-12. doi: 10.1016/j.athoracsur.2006.08.058. PY - 2007 SN - 0003-4975 SP - 606-12 ST - Adults undergoing cardiac surgery at a children's hospital: an analysis of perioperative morbidity T2 - Ann Thorac Surg TI - Adults undergoing cardiac surgery at a children's hospital: an analysis of perioperative morbidity VL - 83 ID - 443 ER - TY - JOUR AB - Background: Limited data exist regarding the perioperative morbidity profile of adults who have cardiac operations at pediatric facilities. Methods: A retrospective review (January 2000 to December 2004) of patients (aged 18 years or older) who underwent cardiac surgery at our pediatric institution was performed. Results: There were 149 cardiac operations performed in 135 patients. There were 2 early deaths. There were 70 preoperative noncardiac morbidities in 49 patients (36%) and 140 preoperative cardiac morbidities in 78 patients (58%). Preoperative arrhythmia (n = 76) and moderate or greater ventricular systolic dysfunction (n = 24) were most common. There were 51 postoperative adverse noncardiac events in 32 patients (24%). Renal insufficiency (> 0.5 mg/dL baseline change; n = 8) was most common. There were 53 postoperative adverse cardiac events in 44 patients (33%). Ventricular tachycardia (n = 13) was most common. Risk factors for postoperative adverse noncardiac events included preoperative histories of New York Heart Association (NYHA) class III or greater (p < 0.001), seizure (p = 0.04), and psychiatric disorder (p = 0.002). Risk factors for postoperative adverse cardiac events included older patient age (p = 0.001), preoperative functional single ventricle (p = 0.006), NHYA class III or greater (p = 0.003), atrial fibrillation/flutter (p < 0.001), and ventricular tachycardia or fibrillation (p = 0.04). Conclusions: Postoperative adverse events occur frequently when adults undergo cardiac operations at children's hospitals. Older patient age, preoperative arrhythmias, and preoperative NHYA class are predictors of postoperative adverse cardiac events. © 2007 The Society of Thoracic Surgeons. AD - J.W. Rossano, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, TX, United States AU - Rossano, J. W. AU - Smith, E. O. AU - Fraser Jr, C. D. AU - McKenzie, E. D. AU - Chang, A. C. AU - Hemingway, A. AU - Price, J. F. AU - Dickerson, H. A. AU - Mott, A. R. DB - Embase Medline DO - 10.1016/j.athoracsur.2006.08.058 IS - 2 KW - adult article bleeding brachial plexus injury cholelithiasis congenital heart disease controlled study cyanosis decubitus depression endocrine disease epistaxis female genetic disorder heart arrest heart arrhythmia atrial fibrillation heart atrium flutter heart single ventricle heart surgery heart ventricle fibrillation heart ventricle tachycardia human hypertension infection kidney disease kidney failure liver dysfunction lung disease lung embolism major clinical study male mental disease morbidity neurologic disease cardiac rhythm management device pericardial effusion pleura effusion pneumonia pneumothorax postoperative complication predictor variable priority journal psychosis risk factor seizure sepsis sleep disordered breathing cerebrovascular accident supraventricular tachycardia systolic dysfunction tracheostomy urinary tract infection urine retention vein thrombosis LA - English M3 - Article N1 - L46135443 2007-02-27 PY - 2007 SN - 0003-4975 SP - 606-612 ST - Adults Undergoing Cardiac Surgery at a Children's Hospital: An Analysis of Perioperative Morbidity T2 - Annals of Thoracic Surgery TI - Adults Undergoing Cardiac Surgery at a Children's Hospital: An Analysis of Perioperative Morbidity UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L46135443 http://dx.doi.org/10.1016/j.athoracsur.2006.08.058 VL - 83 ID - 1240 ER - TY - JOUR AB - Improvement in pre-, peri-, and postoperative management of congenital heart disease (CHD) has significantly increased long-term survival in children with CHD. However, there is a subset of patients with CHD who are either poor candidates for surgical palliation or in whom surgical intervention has failed and require advanced cardiac support. Heart transplant (HT) as a therapy for CHD has undergone tremendous evolution. Though transplantation remains the standard of care to improve survival and quality of life when conventional medical and surgical therapies have failed, it remains limited by the scarcity and unpredictability of donor organ availability. As such, the use of ventricular assist devices (VADs) as a bridge to transplant is gaining increasing popularity. Because of improvement in device technology, and reduced rate of VAD-related complications, the use of these devices is expanding to smaller children and more complex congenital anatomy. Challenges with VAD support and HT in the congenital heart population will be addressed in this review with focus on: (1) reasons for VAD implantation; (2) VAD support in Fontan circulation; (3) challenges with human leukocyte antigen (HLA) sensitization in HT; and (4) effect of VAD support on HT in CHD. AD - Department of Cardiology, Children's Hospital Boston, and the Department of Pediatrics, Harvard Medical School, Boston, MA, USA. AN - 23683470 AU - VanderPluym, C. AU - Urschel, S. AU - Buchholz, H. DA - Jul DB - PubMed DO - 10.1016/j.cjca.2013.02.008 DP - NLM ET - 2013/05/21 IS - 7 KW - Child Fontan Procedure HLA Antigens/immunology Heart Defects, Congenital/*surgery *Heart Transplantation *Heart-Assist Devices Humans LA - eng N1 - 1916-7075 VanderPluym, Christina Urschel, Simon Buchholz, Holger Journal Article Review England Can J Cardiol. 2013 Jul;29(7):796-802. doi: 10.1016/j.cjca.2013.02.008. Epub 2013 May 16. PY - 2013 SN - 0828-282x SP - 796-802 ST - Advanced therapies for congenital heart disease: ventricular assist devices and heart transplantation T2 - Can J Cardiol TI - Advanced therapies for congenital heart disease: ventricular assist devices and heart transplantation VL - 29 ID - 272 ER - TY - JOUR AB - As we enter the next millennium, we are encouraged by the progress that has been made in the care of neonates, infants, and children with heart disease. Surgical repair can be offered at an earlier age with excellent results. Diseases that were uniformly fatal in the past have improved outcomes. Research continues in the area of interventional devices such that surgical repair might be eliminated or delayed. We continue to look forward to advances in the next several years that will allow for future improvement in outcome, better quality-of-life and better long-term results. AD - Department of Pediatric Cardiology, Medical College of Wisconsin, Children's Hospital of Wisconsin, Milwaukee, USA. AN - 9775754 AU - Fedderly, R. T. AU - Berger, S. AU - Frommelt, P. C. AU - Lewis, D. A. AU - Dhala, A. AU - Pelech, A. N. AU - Frommelt, M. A. AU - Friedberg, D. Z. AU - Thomas, J. P., Jr. AU - Tweddell, J. S. AU - Litwin, S. B. DA - Sep DB - PubMed DP - NLM ET - 1998/10/17 IS - 8 KW - Child Heart Diseases/*diagnosis/physiopathology/*surgery Humans Hypoplastic Left Heart Syndrome/surgery Transposition of Great Vessels/surgery LA - eng N1 - Fedderly, R T Berger, S Frommelt, P C Lewis, D A Dhala, A Pelech, A N Frommelt, M A Friedberg, D Z Thomas, J P Jr Tweddell, J S Litwin, S B Journal Article United States WMJ. 1998 Sep;97(8):47-50. PY - 1998 SN - 1098-1861 (Print) 1098-1861 SP - 47-50 ST - Advances in the care of children with heart disease T2 - Wmj TI - Advances in the care of children with heart disease VL - 97 ID - 476 ER - TY - JOUR AB - As we enter the next millennium, we are encouraged by the progress that has been made in the care of neonates, infants, and children with heart disease. Surgical repair can be offered at an earlier age with excellent results. Diseases that were uniformly fatal in the past have improved outcomes. Research continues in the area of interventional devices such that surgical repair might be eliminated or delayed. We continue to look forward to advances in the next several years that will allow for future improvement in outcome, better quality-of-life and better long-term results. AD - Department of Pediatric Cardiology, Medical College of Wisconsin, Children's Hospital of Wisconsin, Milwaukee, WI, United States Department of Surgery, Medical College of Wisconsin, Children's Hospital of Wisconsin, Milwaukee, WI, United States Dept. of Pediatric Cardiology, Children's Hospital of Wisconsin, 9000 W. Wisconsin Ave., Milwaukee, WI 53226, United States AU - Fedderly, R. T. AU - Berger, S. AU - Frommelt, P. C. AU - Lewis, D. A. AU - Dhala, A. AU - Pelech, A. N. AU - Frommelt, M. A. AU - Friedberg, D. Z. AU - Thomas Jr, J. P. AU - Tweddell, J. S. AU - Litwin, S. B. DB - Scopus IS - 8 M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 1998 SP - 47-50 ST - Advances in the care of children with heart disease T2 - Wisconsin Medical Journal TI - Advances in the care of children with heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-15144346186&partnerID=40&md5=d5c877bf5529d29d13c6ba7b4b4f0e9b VL - 97 ID - 2286 ER - TY - JOUR AB - Scoliosis is common in children with congenital heart disease (CHD) and may have deleterious effects on quality of life and hemodynamics. Relatively little is known about the outcomes of spinal fusion for scoliosis repair in children with complex CHD. We reviewed all cases of children with CHD undergoing first time spinal fusion excluding those with minor CHD between 1995 and 2015. Seventy-eight patients were identified and included in the study. 97.4% of patients included had undergone prior cardiac surgery and sixteen patients had single ventricle circulations. 17.9% of patients experienced a significant perioperative event defined as an aggregate of the presence of any of the following: need for early unanticipated reoperation, neurologic deficit, postoperative bleeding requiring intervention, end organ dysfunction, or death. There were no deaths in our cohort. 38.5% of patients experienced any adverse event, the majority of which were related to perioperative fluid shifts. Larger preoperative Cobb angle and longer length of spinal fusion were associated with increased risk of significant perioperative event while larger preoperative Cobb angle and longer length of spinal fusion, older age at time of surgery, single ventricle circulation, cyanosis and patients taking cardiac medications at the time of surgery were more likely to experience any adverse event. Operative repair of scoliosis in children with complex CHD has been performed without mortality over a 20-year period in a single institution, albeit with a higher rate of perioperative complication than is seen in the general pediatric population. Patients with large preoperative Cobb angles and cyanotic single ventricle circulations appear to be at the highest risk for perioperative complications. AD - Department of Cardiology, Boston Children's Hospital, Boston, MA, USA. robert.przybylski@cardio.chboston.org. Department of Orthopedic Surgery, Boston Children's Hospital, Boston, MA, USA. Division of Cardiac Anesthesia, Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Boston, MA, USA. Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Boston, MA, USA. Department of Cardiology, Boston Children's Hospital, Boston, MA, USA. AN - 31350568 AU - Przybylski, R. AU - Hedequist, D. J. AU - Nasr, V. G. AU - McCann, M. E. AU - Brustowicz, R. M. AU - Emans, J. B. AU - Marshall, A. C. AU - Brown, D. W. DA - Oct DB - PubMed DO - 10.1007/s00246-019-02169-1 DP - NLM ET - 2019/07/28 IS - 7 KW - Adolescent Aged Case-Control Studies Child Female Heart Defects, Congenital/complications/*surgery Humans Male Postoperative Complications/*epidemiology Retrospective Studies Scoliosis/complications/*surgery Spinal Fusion/*adverse effects/statistics & numerical data Treatment Outcome Congenital heart disease Scoliosis Spinal fusion LA - eng N1 - 1432-1971 Przybylski, Robert Orcid: 0000-0001-7938-0998 Hedequist, Daniel J Nasr, Viviane G McCann, Mary Ellen Brustowicz, Robert M Emans, John B Marshall, Audrey C Brown, David W Journal Article United States Pediatr Cardiol. 2019 Oct;40(7):1468-1475. doi: 10.1007/s00246-019-02169-1. Epub 2019 Jul 26. PY - 2019 SN - 0172-0643 SP - 1468-1475 ST - Adverse Perioperative Events in Children with Complex Congenital Heart Disease Undergoing Operative Scoliosis Repair in the Contemporary Era T2 - Pediatr Cardiol TI - Adverse Perioperative Events in Children with Complex Congenital Heart Disease Undergoing Operative Scoliosis Repair in the Contemporary Era VL - 40 ID - 32 ER - TY - JOUR AB - BACKGROUND: The Fontan circulation is a palliation for patients with a functionally univentricular heart. It is characterized by gradual attrition over time. An increase in pulmonary vascular resistance could be a key factor in the long-term failure of the Fontan circulation. In this study we aimed to identify pulmonary vascular remodeling in patients with a Fontan circulation. METHODS: Pulmonary vascular histomorphometric analysis and immunohistochemistry were performed in lung tissue obtained at autopsy from 12 Fontan patients. These patients had died either peri-operatively (Group A: death during or <15 days after Fontan completion; n = 5) or in mid to long-term follow-up (Group B: death >5 years after Fontan completion; n = 7). Two age-matched control groups (n = 10 and n = 14, respectively) were included. RESULTS: Intra-acinar pulmonary vessels in the Fontan Group B patients showed decreased medial thickness (p = 0.028) compared with age-matched controls, whereas intimal thickness was increased (p = 0.002). Intimal thickness in the Fontan Group B patients correlated with age at death (r = 0.964, p < 0.001) and with the length of time that the Fontan circulation had been in place (r = 0.714, p = 0.036). Immunohistochemistry revealed a reduction of vascular smooth muscles cells in the medial layer of the intra-acinar pulmonary vessels. The eccentric intimal thickening was composed of mainly acellular fibrosis with collagen deposition. CONCLUSIONS: We observed a unique pattern of adverse pulmonary vascular remodeling in patients with a long-standing Fontan circulation who had died during follow-up. This remodeling pattern may play a major role in long-term attrition of the Fontan circulation. AD - Department of Pediatric Cardiology, Beatrix Children's Hospital. Department of Pathology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. Department of Cardiology. Department of Cardiothoracic Surgery, Center for Congenital Heart Diseases, University Medical Center Groningen. Department of Pediatric Cardiology, Beatrix Children's Hospital. Electronic address: r.m.f.berger@umcg.nl. AN - 25813767 AU - Ridderbos, F. J. AU - Wolff, D. AU - Timmer, A. AU - van Melle, J. P. AU - Ebels, T. AU - Dickinson, M. G. AU - Timens, W. AU - Berger, R. M. DA - Mar DB - PubMed DO - 10.1016/j.healun.2015.01.005 DP - NLM ET - 2015/03/31 IS - 3 KW - Adolescent Adult Child Child, Preschool Female *Fontan Procedure Heart Defects, Congenital/mortality/physiopathology/*surgery Heart Ventricles/*physiopathology Humans Infant Male Netherlands/epidemiology Survival Rate/trends *Vascular Remodeling Vascular Resistance/*physiology Young Adult Fontan operation congenital heart disease failing Fontan pulmonary vascular remodeling pulmonary vascular resistance shear stress LA - eng N1 - 1557-3117 Ridderbos, Floris-Jan S Wolff, Djoeke Timmer, Albertus van Melle, Joost P Ebels, Tjark Dickinson, Michael G Timens, Wim Berger, Rolf M F Journal Article United States J Heart Lung Transplant. 2015 Mar;34(3):404-13. doi: 10.1016/j.healun.2015.01.005. Epub 2015 Jan 16. PY - 2015 SN - 1053-2498 SP - 404-13 ST - Adverse pulmonary vascular remodeling in the Fontan circulation T2 - J Heart Lung Transplant TI - Adverse pulmonary vascular remodeling in the Fontan circulation VL - 34 ID - 276 ER - TY - JOUR AB - Background The Fontan circulation is a palliation for patients with a functionally univentricular heart. It is characterized by gradual attrition over time. An increase in pulmonary vascular resistance could be a key factor in the long-term failure of the Fontan circulation. In this study we aimed to identify pulmonary vascular remodeling in patients with a Fontan circulation. Methods Pulmonary vascular histomorphometric analysis and immunohistochemistry were performed in lung tissue obtained at autopsy from 12 Fontan patients. These patients had died either peri-operatively (Group A: death during or <15 days after Fontan completion; n = 5) or in mid to long-term follow-up (Group B: death >5 years after Fontan completion; n = 7). Two age-matched control groups (n = 10 and n = 14, respectively) were included. Results Intra-acinar pulmonary vessels in the Fontan Group B patients showed decreased medial thickness (p = 0.028) compared with age-matched controls, whereas intimal thickness was increased (p = 0.002). Intimal thickness in the Fontan Group B patients correlated with age at death (r = 0.964, p < 0.001) and with the length of time that the Fontan circulation had been in place (r = 0.714, p = 0.036). Immunohistochemistry revealed a reduction of vascular smooth muscles cells in the medial layer of the intra-acinar pulmonary vessels. The eccentric intimal thickening was composed of mainly acellular fibrosis with collagen deposition. Conclusions We observed a unique pattern of adverse pulmonary vascular remodeling in patients with a long-standing Fontan circulation who had died during follow-up. This remodeling pattern may play a major role in long-term attrition of the Fontan circulation. © 2015 International Society for Heart and Lung Transplantation. All rights reserved. AD - Department of Pediatric Cardiology, Beatrix Children's Hospital, Groningen, Netherlands Department of Cardiology, Groningen, Netherlands Department of Cardiothoracic Surgery, Center for Congenital Heart Diseases, University Medical Center Groningen, Groningen, Netherlands Department of Pathology, University Medical Center Groningen, University of Groningen, Groningen, Netherlands AU - Ridderbos, F. J. S. AU - Wolff, D. AU - Timmer, A. AU - Van Melle, J. P. AU - Ebels, T. AU - Dickinson, M. G. AU - Timens, W. AU - Berger, R. M. F. DB - Scopus DO - 10.1016/j.healun.2015.01.005 IS - 3 KW - congenital heart disease failing Fontan Fontan operation pulmonary vascular remodeling pulmonary vascular resistance shear stress M3 - Article N1 - Cited By :45 Export Date: 15 June 2020 PY - 2015 SP - 404-413 ST - Adverse pulmonary vascular remodeling in the Fontan circulation T2 - Journal of Heart and Lung Transplantation TI - Adverse pulmonary vascular remodeling in the Fontan circulation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84925303163&doi=10.1016%2fj.healun.2015.01.005&partnerID=40&md5=c7be485674c5a3f0a129e52bc24311b5 VL - 34 ID - 1950 ER - TY - JOUR AB - PURPOSE: To evaluate effects of an exercise program on health-related quality of life (HRQoL) in children and adolescents with tetralogy of Fallot (ToF) or a Fontan circulation. METHODS: Stratified, randomized, controlled intervention study conducted in five participating centers of pediatric cardiology in The Netherlands. In total, 93 patients, aged 10-25 years, with surgical repair for tetralogy of Fallot or with a Fontan circulation for single-ventricle physiology were included. They were randomly allocated with a ratio of 2:1 to: (1) a 12-week period with an exercise program for 3 times per week or (2) to a control group. Randomization was stratified by age, gender, and cardiac diagnosis. At baseline and follow-up after 12 weeks, all participants completed Web-based age-appropriate HRQoL questionnaires. Primary analyses involved change in HRQoL during follow-up. Secondary analyses concerned influence of cardiac diagnosis and comparison with normative data. RESULTS: Forty-eight (86%) and 32 (86%) patients in the exercise-group and control-group respectively completed all questionnaires at baseline and follow-up. Compared with the control-group, children, aged 10-15 years, in the exercise-group improved significantly on self-reported cognitive functioning, p < .05, r = .30, and parent-reported social functioning, p < .05, r = .30. Youngsters aged 16-25 years did not change their HRQoL. Cardiac diagnosis had no influence on pre/post changes. Children and youngsters in this study reported comparable or better HRQoL than norm groups. CONCLUSIONS: Participation in an exercise program improved HRQoL of children with ToF or a Fontan circulation, especially in those with low baseline QoL. AD - Department of Child and Adolescent Psychiatry/Psychology, Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands. Department of Paediatrics, Division of Cardiology, Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands. Department of Pediatric Cardiology, Academic Medical Center, Amsterdam, The Netherlands. Department of Pediatric Cardiology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands. Department of Cardiology-Thoraxcenter, Erasmus Medical Center, Rotterdam, The Netherlands. Department of Child and Adolescent Psychiatry/Psychology, Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands. Electronic address: e.utens@erasmusmc.nl. AN - 24518533 AU - Dulfer, K. AU - Duppen, N. AU - Kuipers, I. M. AU - Schokking, M. AU - van Domburg, R. T. AU - Verhulst, F. C. AU - Helbing, W. A. AU - Utens, E. M. DA - Jul DB - PubMed DO - 10.1016/j.jadohealth.2013.12.010 DP - NLM ET - 2014/02/13 IS - 1 KW - Adolescent Adult Child Cognition/physiology Exercise/physiology/*psychology Exercise Tolerance/physiology Female Fontan Procedure/psychology/*rehabilitation Humans Male Netherlands Prospective Studies *Quality of Life Social Behavior Tetralogy of Fallot/physiopathology/psychology/*rehabilitation/surgery Young Adult Adolescents Aerobic exercise Children Congenital heart disease Quality of life Randomized controlled trial Young adults LA - eng N1 - 1879-1972 Dulfer, Karolijn Duppen, Nienke Kuipers, Irene M Schokking, Michiel van Domburg, Ron T Verhulst, Frank C Helbing, Willem A Utens, Elisabeth M W J Journal Article Multicenter Study Randomized Controlled Trial Research Support, Non-U.S. Gov't United States J Adolesc Health. 2014 Jul;55(1):65-72. doi: 10.1016/j.jadohealth.2013.12.010. Epub 2014 Feb 8. PY - 2014 SN - 1054-139x SP - 65-72 ST - Aerobic exercise influences quality of life of children and youngsters with congenital heart disease: a randomized controlled trial T2 - J Adolesc Health TI - Aerobic exercise influences quality of life of children and youngsters with congenital heart disease: a randomized controlled trial VL - 55 ID - 169 ER - TY - JOUR AB - Purpose To evaluate effects of an exercise program on health-related quality of life (HRQoL) in children and adolescents with tetralogy of Fallot (ToF) or a Fontan circulation. Methods Stratified, randomized, controlled intervention study conducted in five participating centers of pediatric cardiology in The Netherlands. In total, 93 patients, aged 10-25 years, with surgical repair for tetralogy of Fallot or with a Fontan circulation for single-ventricle physiology were included. They were randomly allocated with a ratio of 2:1 to: (1) a 12-week period with an exercise program for 3 times per week or (2) to a control group. Randomization was stratified by age, gender, and cardiac diagnosis. At baseline and follow-up after 12 weeks, all participants completed Web-based age-appropriate HRQoL questionnaires. Primary analyses involved change in HRQoL during follow-up. Secondary analyses concerned influence of cardiac diagnosis and comparison with normative data. Results Forty-eight (86%) and 32 (86%) patients in the exercise-group and control-group respectively completed all questionnaires at baseline and follow-up. Compared with the control-group, children, aged 10-15 years, in the exercise-group improved significantly on self-reported cognitive functioning, p <.05, r =.30, and parent-reported social functioning, p <.05, r =.30. Youngsters aged 16-25 years did not change their HRQoL. Cardiac diagnosis had no influence on pre/post changes. Children and youngsters in this study reported comparable or better HRQoL than norm groups. Conclusions Participation in an exercise program improved HRQoL of children with ToF or a Fontan circulation, especially in those with low baseline QoL. © 2014 Society for Adolescent Health and Medicine. All rights reserved. AD - E.M.W.J. Utens, Department of Child and Adolescent Psychiatry/Psychology, Erasmus Medical Center, Sophia Children's Hospital, P.O. Box 2060, 3000 CB, Rotterdam, Netherlands AU - Dulfer, K. AU - Duppen, N. AU - Kuipers, I. M. AU - Schokking, M. AU - Van Domburg, R. T. AU - Verhulst, F. C. AU - Helbing, W. A. AU - Utens, E. M. W. J. DB - Embase Medline DO - 10.1016/j.jadohealth.2013.12.010 IS - 1 KW - adolescent adolescent disease adult aerobic exercise article child childhood disease cognition comparative study congenital heart disease controlled study diagnosis related group Fallot tetralogy female follow up Fontan circulation groups by age Health Related Quality of Life Questionnaire heart single ventricle heart surgery human intervention study major clinical study male multicenter study assessment of humans pediatric cardiology physical activity priority journal quality of life randomized controlled trial self report sex difference Short Form 36 single blind procedure social interaction TNO AZL Child Quality of Life Questionnaire LA - English M3 - Article N1 - L52997185 2014-02-18 2014-07-08 PY - 2014 SN - 1879-1972 1054-139X SP - 65-72 ST - Aerobic exercise influences quality of life of children and youngsters with congenital heart disease: A randomized controlled trial T2 - Journal of Adolescent Health TI - Aerobic exercise influences quality of life of children and youngsters with congenital heart disease: A randomized controlled trial UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52997185 http://dx.doi.org/10.1016/j.jadohealth.2013.12.010 VL - 55 ID - 950 ER - TY - JOUR AB - BACKGROUND AND OBJECTIVE: Newborn pulse oximetry screening is recommended to promote early referral of neonates with critical congenital heart disease (CCHD) and reduce mortality; however, the impact of late referral on mortality is not well defined. The purpose of this population-based study was to describe the association between timing of referral to a cardiac center and mortality in 2360 liveborn neonates with CCHD. METHODS: Neonates with CCHD born before pulse oximetry screening (1996-2007) were selected from the Texas Birth Defects Registry and linked to state birth and death records. Age at referral was ascertained from date of first cardiac procedure at a cardiac center. Logistic and Cox proportional hazards regression models were used to estimate factors associated with late referral and mortality; the Kaplan-Meier method was used to estimate 3-month survival. RESULTS: Median age at referral was 1 day (25th-75th percentile: 0-6 days). Overall, 27.5% (649 of 2360) were referred after age 4 days and 7.5% (178 of 2360) had no record of referral. Neonatal mortality was 18.1% (277 of 1533) for those referred at 0 to 4 days of age, 9.0% (34 of 379) for those referred at 5 to 27 days of age, and 38.8% (69 of 178) for those with no referral. No improvement in age at referral was found across the 2 eras within 1996-2007. CONCLUSIONS: A significant proportion of neonates with CCHD experienced late or no referral to cardiac specialty centers, accounting for a significant number of the deaths. Future population-based studies are needed to determine the benefit of pulse oximetry screening on mortality and morbidity. Copyright © 2014 by the American Academy of Pediatrics. AD - D.E. Fixler, Pediatric Cardiology, Children's Medical Center, 1935 Medical District Dr, Dallas, TX 75235, United States AU - Fixler, D. E. AU - Xu, P. AU - Nembhard, W. N. AU - Ethen, M. K. AU - Canfield, M. A. DB - Embase Medline DO - 10.1542/peds.2013-2895 IS - 1 KW - age article congenital malformation cardiovascular mortality cardiovascular system examination cause of death congenital heart disease controlled study critically ill patient cyanosis early childhood intervention ethnicity Fallot tetralogy female great vessels transposition heart failure high risk infant human hypoplastic left heart syndrome infant lung vein drainage anomaly major clinical study male newborn newborn death newborn intensive care newborn morbidity newborn mortality newborn screening patient referral priority journal pulmonary valve atresia pulse oximetry risk reduction survival time United States LA - English M3 - Article N1 - L373517881 2014-07-22 2014-07-23 PY - 2014 SN - 1098-4275 0031-4005 SP - e98-e105 ST - Age at referral and mortality from critical congenital heart disease T2 - Pediatrics TI - Age at referral and mortality from critical congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L373517881 http://dx.doi.org/10.1542/peds.2013-2895 VL - 134 ID - 934 ER - TY - JOUR AB - Plastic bronchitis or " Bronchitis Plastica" is a rare disease characterized by the formation of thick, tenacious, arborizing mucofibrinous tracheobronchial casts that result in life-threatening airway obstruction and pulmonary failure. We review three children who developed recurrent plastic bronchitis after undergoing a Fontan procedure for single ventricle physiology. Case series of three patients with plastic bronchitis at a tertiary referral children's hospital. All patients required repeated bronchoscopies, one requiring four separate ones over a week's period, for removal of the rigid casts. Extra-corporal membrane oxygenation (ECMO) was needed in two children because of severe respiratory failure. All were also managed with adjunctive intensive medical support. Pulmonary function returned to normal in all children, but recurred 2 months later in one who subsequently expired due to pulmonary failure. Plastic bronchitis is an unusual condition of unknown cause that occurs in multiple clinical settings, but especially in those children who have undergone a Fontan operation. Management of this distressing situation is difficult and early diagnosis and aggressive measures to remove rigid casts combined with intensive medical care are necessary. The intrinsic cardiopulmonary physiology of children with Fontan procedures, including the risk of arrhythmias, hypo-oxygenation, and pulmonary hypertension make this condition even more complex. © 2010 Elsevier Ireland Ltd. AD - Division of Pediatric Otolaryngology-Head and Neck Surgery, Children's National Medical Center, George Washington University, 111 Michigan Ave NW, Washington, DC 20010, United States Division of Anesthesiology, Children's National Medical Center, George Washington University, Washington, DC, United States AU - Preciado, D. AU - Verghese, S. AU - Choi, S. DB - Scopus DO - 10.1016/j.ijporl.2010.02.005 IS - 7 KW - Bronchial casts Fontan Plastic bronchitis M3 - Article N1 - Cited By :14 Export Date: 15 June 2020 PY - 2010 SP - 820-822 ST - Aggressive bronchoscopic management of plastic bronchitis T2 - International Journal of Pediatric Otorhinolaryngology TI - Aggressive bronchoscopic management of plastic bronchitis UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-79952028354&doi=10.1016%2fj.ijporl.2010.02.005&partnerID=40&md5=dbe47e00b67271a8d49b9ac8fdb6bba4 VL - 74 ID - 2149 ER - TY - JOUR AB - We report a male patient born at 37-weeks gestation, weighing 1.885 kg (<0.4th centile). Shortly after birth, he was diagnosed with hypoplastic left heart syndrome for which he underwent a Norwood procedure. Subsequently, he developed problems with failure to thrive and developmental delay. At the age of 4 years his delay in growth and development led to further investigations, which revealed a small de-novo interstitial deletion of chromosome 20p12.2. JAGGED1 haploinsufficiency was confirmed by fluorescence in situ hybridization. Array-comparative genomic hybridization analysis confirmed and quantified an approximate 5.4 Mb interstitial deletion involving the chromosomal region 20p12.2-p12.3. This precise interstitial deletion has not been previously reported. Further clinical evaluation revealed posterior embryotoxon and butterfly vertebrae. He has normal liver function tests, facial features consistent with Alagille syndrome, and mild learning difficulties. To our knowledge this is the first report of Alagille syndrome associated with hypoplastic left heart syndrome. © 2007 Lippincott Williams & Wilkins, Inc. AD - M.L.P. Robert, Peninsula Clinical Genetics Service, Royal Devon and Exeter Hospital, Gladstone Road, Exeter EX1 2ED, United Kingdom AU - Robert, M. L. P. AU - Lopez, T. AU - Crolla, J. AU - Huang, S. AU - Owen, C. AU - Burvill-Holmes, L. AU - Stumper, O. AU - Turnpenny, P. D. DB - Embase Medline DO - 10.1097/MCD.0b013e3282358d21 IS - 4 KW - Alagille syndrome article case report chromosome 20p chromosome deletion comparative genomic hybridization developmental disorder fluorescence in situ hybridization haplotype human hypoplastic left heart syndrome infant learning disorder liver function test male priority journal LA - English M3 - Article N1 - L47360229 2007-10-01 PY - 2007 SN - 0962-8827 SP - 241-246 ST - Alagille syndrome with deletion 20p12.2-p12.3 and hypoplastic left heart T2 - Clinical Dysmorphology TI - Alagille syndrome with deletion 20p12.2-p12.3 and hypoplastic left heart UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L47360229 http://dx.doi.org/10.1097/MCD.0b013e3282358d21 VL - 16 ID - 1227 ER - TY - JOUR AB - Abnormal water metabolism is frequently observed after Fontan surgery. We hypothesized that patients' adrenal hormones show unique responses immediately after Fontan operation and that such a hormonal profile is related to postoperative hemodynamics and water imbalance. Twenty-eight patients who underwent a Fontan operation (n = 16) or a non-Fontan type operation (n = 12; controls) under cardiopulmonary bypass were studied. Postoperative urine cortisol and aldosterone levels were measured daily to minimize the influence of circadian rhythms and temporal hemodynamic variations. Cortisol excretion was markedly elevated on postoperative day (POD) 0 in controls, consistent with a stress-induced cortisol response. Cortisol excretion was not high on POD 0 in Fontan patients and was markedly lower than that in the controls (444 ± 150 vs 34 ± 6 μg/m(2)/day, p <0.05), indicating an adrenal insufficiency status. Conversely, aldosterone levels were significantly higher in Fontan patients than in controls immediately after surgery and remained so thereafter. The cortisol-to-aldosterone ratio was significantly lower in Fontan patients on POD 0 (p <0.05 vs controls); low cortisol-to-aldosterone ratios were associated with a longer pleural drainage duration and intensive care unit stay. Daily cortisol and aldosterone levels were significantly associated with postoperative hemodynamics; low cortisol levels correlated with low cardiac and urine outputs, whereas high aldosterone levels correlated with low cardiac output and increased blood pressure and central venous pressure. Thus, aldosterone-to-cortisol imbalance occurred specifically after the Fontan operation. This unique hormonal profile significantly affected patients' postoperative water balance and hemodynamics. Modulation of the adrenal hormone could be useful for reducing postoperative complications after the Fontan operation. AD - Division of Pediatric Cardiology, Saitama Medical Center, Saitama Medical University, Saitama, Japan. Division of Pediatric Cardiology, Saitama Medical Center, Saitama Medical University, Saitama, Japan. Electronic address: hsenzaki@saitama-med.ac.jp. AN - 25261875 AU - Saiki, H. AU - Kuwata, S. AU - Kurishima, C. AU - Iwamoto, Y. AU - Ishido, H. AU - Masutani, S. AU - Senzaki, H. DA - Nov 15 DB - PubMed DO - 10.1016/j.amjcard.2014.08.030 DP - NLM ET - 2014/09/30 IS - 10 KW - Aldosterone/*blood Blood Pressure Child, Preschool Female Follow-Up Studies *Fontan Procedure Heart Defects, Congenital/*metabolism/physiopathology/surgery Homeostasis/*physiology Humans Hydrocortisone/*blood Male Postoperative Complications/*metabolism Retrospective Studies Time Factors LA - eng N1 - 1879-1913 Saiki, Hirofumi Kuwata, Seiko Kurishima, Clara Iwamoto, Yoichi Ishido, Hirotaka Masutani, Satoshi Senzaki, Hideaki Comparative Study Journal Article United States Am J Cardiol. 2014 Nov 15;114(10):1578-83. doi: 10.1016/j.amjcard.2014.08.030. Epub 2014 Aug 27. PY - 2014 SN - 0002-9149 SP - 1578-83 ST - Aldosterone-cortisol imbalance immediately after fontan operation with implications for abnormal fluid homeostasis T2 - Am J Cardiol TI - Aldosterone-cortisol imbalance immediately after fontan operation with implications for abnormal fluid homeostasis VL - 114 ID - 500 ER - TY - JOUR AB - OBJECTIVE: This study compares in vivo pulmonary blood flow patterns and shear stresses in patients with either the direct atrium-pulmonary artery connection or the bicaval tunnel connection of the Fontan procedure to those in normal volunteers. Comparisons were made with the use of three-dimensional phase contrast magnetic resonance imaging. METHODS: Three-dimensional velocities, flows, and pulmonary artery cross-sectional areas were measured in both pulmonary arteries of each subject. Axial, circumferential, and radial shear stresses were calculated with the use of velocities and estimates of viscosity. RESULTS: The axial velocities were not significantly different between subject groups. However, the flows and cross-sectional areas were higher in the normal group than in the two patient groups in both pulmonary arteries. The group with the bicaval connection had circular swirling in the cross section of both pulmonary arteries, causing higher shear stresses than in the controls. The disorder caused by the connection of the atrium to the pulmonary artery caused an increase in some shear stresses over the controls, but not higher than those found in the group having a bicaval tunnel. CONCLUSIONS: We found that pulmonary flow was equally reduced compared with normal flow in both patient groups. This reduction in flow can be attributed in part to the reduced size of the pulmonary arteries in both patient groups without change in axial velocity. We also found higher shear stress acting on the wall of the vessels in the patients having a bicaval tunnel, which may alter endothelial function and affect the longevity of the repair. AD - Department of Biomedical Engineering, Vanderbilt University, Nashville, Tenn, USA. AN - 9699583 AU - Morgan, V. L. AU - Graham, T. P., Jr. AU - Roselli, R. J. AU - Lorenz, C. H. DA - Aug DB - PubMed DO - 10.1016/s0022-5223(98)70130-8 DP - NLM ET - 1998/08/12 IS - 2 KW - Adolescent Adult Anastomosis, Surgical Blood Flow Velocity Child Child, Preschool Female *Fontan Procedure Heart Atria/pathology/physiopathology Humans *Image Processing, Computer-Assisted *Magnetic Resonance Imaging Male Pulmonary Artery/pathology/*physiopathology Pulmonary Atresia/diagnosis/surgery Stress, Physiological/physiopathology Systole Tricuspid Valve/abnormalities/surgery Venae Cavae/*surgery LA - eng N1 - Morgan, V L Graham, T P Jr Roselli, R J Lorenz, C H HL 07411-16/HL/NHLBI NIH HHS/United States Comparative Study Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S. United States J Thorac Cardiovasc Surg. 1998 Aug;116(2):294-304. doi: 10.1016/s0022-5223(98)70130-8. PY - 1998 SN - 0022-5223 (Print) 0022-5223 SP - 294-304 ST - Alterations in pulmonary artery flow patterns and shear stress determined with three-dimensional phase-contrast magnetic resonance imaging in Fontan patients T2 - J Thorac Cardiovasc Surg TI - Alterations in pulmonary artery flow patterns and shear stress determined with three-dimensional phase-contrast magnetic resonance imaging in Fontan patients VL - 116 ID - 452 ER - TY - JOUR AB - PURPOSE: Single ventricle heart disease (SVHD) patients show injury in brain sites that regulate autonomic, mood, and cognitive functions. However, the nature (acute or chronic changes) and extent of brain injury in SVHD are unclear. Our aim was to examine regional brain tissue damage in SVHD over controls using DTI-based mean diffusivity (MD), axial diffusivity (AD), radial diffusivity (RD), and fractional anisotropy (FA) procedures. METHODS: We collected two DTI series (3.0-T MRI), mood and cognitive data, from 27 SVHD and 35 control adolescents. Whole-brain MD, AD, RD, and FA maps were calculated from each series, realigned and averaged, normalized to a common space, smoothed, and compared between groups using ANCOVA (covariates, age and sex; false discovery rate, p < 0.05). Region-of-interest analyses were performed to calculate MD, AD, RD, and FA values for magnitude assessment between groups. RESULTS: SVHD patients showed impaired mood and cognitive functions over healthy adolescents. Multiple brain sites in SVHD showed increased MD values, including the insula, caudate, cingulate, hypothalamus, thalamus, medial prefrontal and frontal cortices, parahippocampal gyrus, hippocampus, precentral gyrus, amygdala, cerebellum, corpus callosum, basal forebrain, mammillary bodies, internal capsule, midbrain, fornix, and occipital, parietal, and temporal cortices, indicating chronic tissue changes. Similar areas showed either increased AD or RD values, with RD changes more enhanced over AD in SVHD compared to controls. Few brain regions emerged with increased or decreased FA values in SVHD patients over controls. CONCLUSION: SVHD adolescents, more than a decade from their last surgical procedure, show widespread brain abnormalities in autonomic, mood, and cognitive regulatory areas. These findings indicate that brain injury is in a chronic stage in SVHD with predominantly myelin changes that may result from previous hypoxia/ischemia- or developmental-induced processes. AD - Department of Anesthesiology, David Geffen School of Medicine at UCLA, University of California at Los Angeles, 56-141 CHS, 10833 Le Conte Aves, Los Angeles, CA, 90095-1763, USA. UCLA School of Nursing, University of California Los Angeles, Los Angeles, CA, USA. Division of Cardiology, Children's Hospital, Los Angeles, CA, USA. Division of Pediatric Cardiology, University of California Los Angeles, Los Angeles, CA, USA. Department of Anesthesiology, David Geffen School of Medicine at UCLA, University of California at Los Angeles, 56-141 CHS, 10833 Le Conte Aves, Los Angeles, CA, 90095-1763, USA. rkumar@mednet.ucla.edu. Department of Radiological Sciences, University of California Los Angeles, Los Angeles, CA, USA. rkumar@mednet.ucla.edu. AN - 31041457 AU - Singh, S. AU - Roy, B. AU - Pike, N. AU - Daniel, E. AU - Ehlert, L. AU - Lewis, A. B. AU - Halnon, N. AU - Woo, M. A. AU - Kumar, R. C2 - PMC6582969 C6 - NIHMS1528246 DA - Jul DB - PubMed DO - 10.1007/s00234-019-02208-x DP - NLM ET - 2019/05/02 IS - 7 KW - Adolescent Anisotropy Brain Diseases/*diagnostic imaging Diffusion Tensor Imaging/*methods Female *Fontan Procedure Humans Male Surveys and Questionnaires Brain injury Cognition Diffusion tensor imaging Single ventricle heart disease LA - eng N1 - 1432-1920 Singh, Sadhana Roy, Bhaswati Pike, Nancy Daniel, Ebenezer Ehlert, Luke Lewis, Alan B Halnon, Nancy Woo, Mary A Kumar, Rajesh R01 NR016463/NR/NINR NIH HHS/United States R01NR016463/National Institute of Health/ Journal Article Neuroradiology. 2019 Jul;61(7):811-824. doi: 10.1007/s00234-019-02208-x. Epub 2019 Apr 30. PY - 2019 SN - 0028-3940 (Print) 0028-3940 SP - 811-824 ST - Altered brain diffusion tensor imaging indices in adolescents with the Fontan palliation T2 - Neuroradiology TI - Altered brain diffusion tensor imaging indices in adolescents with the Fontan palliation VL - 61 ID - 43 ER - TY - JOUR AB - Children and adolescents with congenital heart disease (CHD) are at risk for mild to moderate cognitive impairments. In particular, impaired working memory performance has been found in CHD patients of all ages. Working memory is an important domain of higher order cognitive function and is crucial for everyday activities, with emerging importance in adolescence. However, the underlying neural correlate of working memory impairments in CHD is not yet fully understood. Diffusion tensor imaging and tract based spatial statistics analyses were conducted in 47 adolescent survivors of childhood cardiopulmonary bypass surgery (24 females) and in 44 healthy controls (24 females) between 11 and 16 years of age (mean age = 13.9, SD = 1.6). Fractional anisotropy (FA) of white matter diffusion was compared between groups and was correlated with working memory performance, derived from the Wechsler Intelligence Scale for Children-IV. CHD patients had significantly poorer working memory compared to controls (p = 0.001). Widespread bilateral reduction in FA was observed in CHD patients compared to healthy controls (threshold-free cluster enhancement (TFCE) corrected p < 0.05). This reduction in FA was present both in cyanotic and acyanotic CHD patients compared to healthy controls (both p < 0.001). The FA reduction in the frontal lobe, mainly in the forceps minor, was associated with poorer working memory performance in both patients with CHD and healthy controls (TFCE corrected p < 0.05). The current findings underline that in CHD patients, irrespective of disease severity, disrupted or delayed maturation of white matter may persist into adolescence and is associated with working memory impairments, particularly if present in the frontal lobe. Adolescence, which is a crucial period for prefrontal brain maturation, may offer a window of opportunity for intervention in order to support the maturation of frontal brain regions and therefore improve higher order cognitive function in patients with CHD. AD - R. O'Gorman Tuura, Center for MR Research, University Children's Hospital Zurich, 75 Steinwiesstrasse, Zurich, Switzerland AU - Ehrler, M. AU - Latal, B. AU - Kretschmar, O. AU - von Rhein, M. AU - O'Gorman Tuura, R. DB - Embase Medline DO - 10.1016/j.nicl.2019.102123 KW - adolescent aortic coarctation aortic stenosis article brain fornix capsula interna cardiopulmonary bypass cerebral peduncle child cingulum (brain) clinical article congenital heart disease controlled study corona radiata (brain) corpus callosum cyanotic heart disease diffusion tensor imaging double inlet left ventricle external capsule Fallot tetralogy female fractional anisotropy frontal lobe genu (corpus callosum) great vessels transposition heart atrium septum defect heart ventricle septum defect human image processing inferior longitudinal fasciculus intelligence quotient left hemisphere lung vein drainage anomaly male memory disorder neuroanatomy neuroimaging occipitofrontal fasciculus outcome assessment priority journal pulmonary valve atresia pulmonary valve stenosis pyramidal tract right hemisphere splenium superior longitudinal fasciculus thalamus tricuspid valve atresia uncinate fasciculus Wechsler intelligence scale for children white matter working memory LA - English M3 - Article N1 - L2004323034 2019-12-30 2020-01-08 PY - 2020 SN - 2213-1582 ST - Altered frontal white matter microstructure is associated with working memory impairments in adolescents with congenital heart disease: A diffusion tensor imaging study T2 - NeuroImage: Clinical TI - Altered frontal white matter microstructure is associated with working memory impairments in adolescents with congenital heart disease: A diffusion tensor imaging study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2004323034 http://dx.doi.org/10.1016/j.nicl.2019.102123 VL - 25 ID - 570 ER - TY - JOUR AB - Background: The Fontan circulation is a unique palliation procedure for several congenital heart defects. Impaired exercise capacity has previously been demonstrated in these patients and also a higher risk for cardiopulmonary mortality. Hemorheology was shown to affect cardiopulmonary capacity and in turn to be affected by regular exercise and hypoxia but none of these have been investigated in Fontan patients so far. The aim of this study was to detect general differences in hemorheology in normoxia as well as possible altered hemorheological responses to hypoxia exposure and hypoxic exercise between Fontan patients and healthy controls. Methods and Findings: 26 Fontan patients and 20 healthy controls performed an acute exercise test (AET) on a bicycle ergometer under hypoxia with ambient 15.2% oxygen saturation (sO2). Blood samples were taken at rest in normoxia (T0), at rest in hypoxia (T1), after maximum exhaustion in hypoxia (T2), and after 50 min recovery in normoxia (T3). Hemorheological and blood parameters were investigated. Additionally, arterial stiffness was tested at T0. Red blood cell (RBC) deformability, NOx, erythropoietin (EPO) concentration, RBC count, hemoglobin (Hb) concentration and hematocrit (hct) were significantly increased in Fontan patients compared to controls. Same was observed for arterial stiffness. No changes were observed for RBC aggregation, fibrinogen concentration, free radical levels and vascular endothelial growth factor (VEGF). Hypoxia exposure did not change parameters, whereas exercise in hypoxia increased aggregation and hct significantly in both groups. Fontan patients showed significantly increased aggregation-disaggregation balance compared to controls. Conclusion: Acute hypoxia exposure and exercise under hypoxia might have similar impact on hemorheology in Fontan patients and controls and was clinically well tolerated. Nevertheless, exercise alters aggregation and possibly hemodynamics which requires special attention in Fontan patients. © Copyright © 2019 Härtel, Müller, Herberg, Breuer, Bizjak, Bloch and Grau. AD - Department of Molecular and Cellular Sports Medicine, German Sport University Cologne, Cologne, Germany Department for Pediatric Cardiology, University Hospital Bonn, Bonn, Germany AU - Härtel, J. A. AU - Müller, N. AU - Herberg, U. AU - Breuer, J. AU - Bizjak, D. A. AU - Bloch, W. AU - Grau, M. C7 - 1443 DB - Scopus DO - 10.3389/fphys.2019.01443 KW - exercise Fontan circulation hypoxia red blood cells rheology M3 - Article N1 - Export Date: 15 June 2020 PY - 2019 ST - Altered Hemorheology in Fontan Patients in Normoxia and After Acute Hypoxic Exercise T2 - Frontiers in Physiology TI - Altered Hemorheology in Fontan Patients in Normoxia and After Acute Hypoxic Exercise UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85076687445&doi=10.3389%2ffphys.2019.01443&partnerID=40&md5=0d9fdcae44273320767a5d90e0fb453e VL - 10 ID - 1724 ER - TY - JOUR AB - OBJECTIVE: To compare white matter microstructure in children and adolescents with single ventricle who underwent the Fontan procedure with healthy controls, and to explore the association of white matter injury with cognitive performance as well as patient and medical factors. STUDY DESIGN: Fontan (n = 102) and control subjects (n = 47) underwent diffusion tensor imaging (DTI) at ages 10-19 years. Mean DTI measures (fractional anisotropy, radial diffusivity, axial diffusivity, and mean diffusivity) were calculated for 33 fiber tracts from standard white matter atlases. Voxel-wise group differences in DTI measures were assessed using Tract-Based Spatial Statistics. Associations of regional fractional anisotropy with IQ and processing speed as well as medical characteristics were examined. RESULTS: Subjects with Fontan, compared with controls, had reduced bilateral regional and voxel-wise fractional anisotropy in multiple white matter tracts along with increased regional radial diffusivity in several overlapping tracts; regional mean diffusivity differed in 2 tracts. The groups did not differ in voxel-wise radial diffusivity or mean diffusivity. Among subjects with Fontan, fractional anisotropy in many tracts correlated positively with Full-Scale Intelligence Quotient and processing speed, although similar findings were absent in controls. Lower mean fractional anisotropy in various tracts was associated with more complications in the first operation, a greater number of total operations, and history of neurologic event. CONCLUSIONS: Children and adolescents who have undergone the Fontan procedure have widespread abnormalities in white matter microstructure. Furthermore, white matter microstructure in several tracts is associated with cognitive performance and operative and medical history characteristics. AD - Graduate Program for Neuroscience, Boston University, Boston, MA; Department of Neurology, Boston Children's Hospital, Boston, MA. Department of Cardiology, Boston Children's Hospital, Boston, MA. Department of Cardiology, Boston Children's Hospital, Boston, MA; Department of Biostatistics, Harvard T.H. Chan School of Public Health, Boston, MA; Department of Pediatrics, Harvard Medical School, Boston, MA. Department of Neurology, Boston Children's Hospital, Boston, MA; Department of Psychiatry, Boston Children's Hospital, Boston, MA; Department of Psychiatry, Harvard Medical School, Boston, MA; Department of Neurology, Harvard Medical School, Boston, MA. Department of Cardiology, Boston Children's Hospital, Boston, MA; Department of Pediatrics, Harvard Medical School, Boston, MA. Department of Neurology, Boston Children's Hospital, Boston, MA; Department of Psychiatry, Boston Children's Hospital, Boston, MA; Department of Neurology, Harvard Medical School, Boston, MA; Department of Radiology, Boston Children's Hospital, Boston, MA. Electronic address: michael.rivkin@childrens.harvard.edu. AN - 29934026 AU - Watson, C. G. AU - Stopp, C. AU - Wypij, D. AU - Bellinger, D. C. AU - Newburger, J. W. AU - Rivkin, M. J. DA - Sep DB - PubMed DO - 10.1016/j.jpeds.2018.04.022 DP - NLM ET - 2018/06/24 KW - Adolescent Child Cognition/*physiology Diffusion Magnetic Resonance Imaging/*methods Female *Fontan Procedure Heart Defects, Congenital/*psychology/surgery Humans *Image Processing, Computer-Assisted Male Postoperative Period Retrospective Studies White Matter/*diagnostic imaging/growth & development Young Adult *dti *Fontan *mri *brain *cognition *congenital heart disease *single ventricle LA - eng N1 - 1097-6833 Watson, Christopher G Stopp, Christian Wypij, David Bellinger, David C Newburger, Jane W Rivkin, Michael J Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't United States J Pediatr. 2018 Sep;200:140-149.e4. doi: 10.1016/j.jpeds.2018.04.022. Epub 2018 Jun 19. PY - 2018 SN - 0022-3476 SP - 140-149.e4 ST - Altered White Matter Microstructure Correlates with IQ and Processing Speed in Children and Adolescents Post-Fontan T2 - J Pediatr TI - Altered White Matter Microstructure Correlates with IQ and Processing Speed in Children and Adolescents Post-Fontan VL - 200 ID - 71 ER - TY - JOUR AU - Snyder, C. AU - Moodie, D. DB - Embase Medline DO - 10.1111/chd.12275 IS - 3 KW - aortic dilation article artificial embolization body mass body surface body weight brain ventricle dilatation cardiovascular response central venous pressure congenital heart disease Down syndrome echocardiography electrocardiogram Fontan procedure Glenn shunt heart dilatation heart pacing heart single ventricle heart ventricle septum defect hemodynamics human obesity patent ductus arteriosus physical examination postoperative period predictive value preoperative period pressure transducer priority journal screening test sensitivity and specificity survival rate thorax radiography LA - English M3 - Article N1 - L605058367 2015-07-06 2015-07-07 PY - 2015 SN - 1747-0803 1747-079X SP - 288-290 ST - American Academy of Pediatrics Section on Cardiology Meeting T2 - Congenital Heart Disease TI - American Academy of Pediatrics Section on Cardiology Meeting UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L605058367 http://dx.doi.org/10.1111/chd.12275 VL - 10 ID - 868 ER - TY - JOUR AB - Amiodarone is a class III antiarrhythmic medication known for its efficacy in treating tachyarrhythmias. The pulmonary complications including acute lung toxicity are well documented in the adult population, however much less so in pediatrics. We describe two pediatric congenital heart disease patients with suspected amiodarone induced pulmonary toxicity in the post-operative period. Case 1 presented with acute respiratory distress syndrome accompanied by periods of pulmonary hemorrhage post-operatively; after multiple courses of methylprednisolone and discontinuation of amiodarone, she showed significant clinical improvement with normalization of her CXR. Case 2 presented as hypoxemic with acute respiratory distress syndrome, and had notable clinical improvement after cessation of amiodarone with pulse dose methylprednisolone and inhaled budesonide. AD - S. Covi, Division of Pediatric Cardiology, St. John Hospital and Medical Center, PB II Suite 275, Detroit, MI, United States AU - Covi, S. AU - Clark, J. AU - Delius, R. AU - Chauhan, M. DB - Embase DO - 10.1016/j.ppedcard.2018.08.001 KW - amiodarone budesonide hydrocortisone methylprednisolone prednisone propranolol adolescent adult respiratory distress syndrome article bronchoscopy case report clinical article drug dose reduction drug withdrawal Ebstein anomaly echocardiography extubation female follow up Glenn shunt heart ventricle tachycardia human hypoplastic left heart syndrome hypoxemia loading drug dose lung hemorrhage lung lavage lung toxicity priority journal thorax radiography tricuspid annuloplasty tricuspid valve replacement LA - English M3 - Article N1 - L2001010275 2018-08-10 2019-03-14 PY - 2019 SN - 1558-1519 1058-9813 SP - 46-49 ST - Amiodarone toxicity in two post-operative congenital heart disease patients T2 - Progress in Pediatric Cardiology TI - Amiodarone toxicity in two post-operative congenital heart disease patients UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2001010275 http://dx.doi.org/10.1016/j.ppedcard.2018.08.001 VL - 52 ID - 622 ER - TY - JOUR AB - Computational fluid dynamic (CFD) simulations are widely utilized to assess Fontan hemodynamics that are related to long-term complications. No previous studies have systemically investigated the effects of using different inlet velocity profiles in Fontan simulations. This study implements real, patient-specific velocity profiles for numerical assessment of Fontan hemodynamics using CFD simulations. Four additional, artificial velocity profiles were used for comparison: (1) flat, (2) parabolic, (3) Womersley, and (4) parabolic with inlet extensions [to develop flow before entering the total cavopulmonary connection (TCPC)]. The differences arising from the five velocity profiles, as well as discrepancies between the real and each of the artificial velocity profiles, were quantified by examining clinically important metrics in TCPC hemodynamics: power loss (PL), viscous dissipation rate (VDR), hepatic flow distribution, and regions of low wall shear stress. Statistically significant differences were observed in PL and VDR between simulations using real and flat velocity profiles, but differences between those using real velocity profiles and the other three artificial profiles did not reach statistical significance. These conclusions suggest that the artificial velocity profiles (2)-(4) are acceptable surrogates for real velocity profiles in Fontan simulations, but parabolic profiles are recommended because of their low computational demands and prevalent applicability. AD - Wallace H. Coulter School of Biomedical Engineering, Georgia Institute of Technology, 387 Technology Circle, Suite 232, Atlanta, GA, 30313-2412, USA. School of Chemistry and Biochemistry, Georgia Institute of Technology, Atlanta, GA, USA. Department of Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA. Department of Mathematics, Department of Computer Science, Emory University, Atlanta, GA, USA. Wallace H. Coulter School of Biomedical Engineering, Georgia Institute of Technology, 387 Technology Circle, Suite 232, Atlanta, GA, 30313-2412, USA. ajit.yoganathan@bme.gatech.edu. AN - 31236791 AU - Wei, Z. A. AU - Huddleston, C. AU - Trusty, P. M. AU - Singh-Gryzbon, S. AU - Fogel, M. A. AU - Veneziani, A. AU - Yoganathan, A. P. C2 - PMC6842101 C6 - NIHMS1532766 DA - Nov DB - PubMed DO - 10.1007/s10439-019-02307-z DP - NLM ET - 2019/06/27 IS - 11 KW - Adolescent Child *Computer Simulation Female *Fontan Procedure *Hemodynamics Humans Hydrodynamics Male *Models, Cardiovascular Pulmonary Artery Computational fluid dynamics Fontan hemodynamics Inlet velocity profiles LA - eng N1 - 1573-9686 Wei, Zhenglun Alan Huddleston, Connor Trusty, Phillip M Singh-Gryzbon, Shelly Fogel, Mark A Veneziani, Alessandro Yoganathan, Ajit P Orcid: 0000-0001-5818-1316 R01 HL098252/HL/NHLBI NIH HHS/United States R01 HL067622/HL/NHLBI NIH HHS/United States Petit Undergraduate Research Scholarship/Georgia Institute of Technology/ HL67622/HL/NHLBI NIH HHS/United States HL098252/HL/NHLBI NIH HHS/United States Journal Article Ann Biomed Eng. 2019 Nov;47(11):2258-2270. doi: 10.1007/s10439-019-02307-z. Epub 2019 Jun 24. PY - 2019 SN - 0090-6964 (Print) 0090-6964 SP - 2258-2270 ST - Analysis of Inlet Velocity Profiles in Numerical Assessment of Fontan Hemodynamics T2 - Ann Biomed Eng TI - Analysis of Inlet Velocity Profiles in Numerical Assessment of Fontan Hemodynamics VL - 47 ID - 118 ER - TY - JOUR AB - Twenty-four patients with single ventricle, six with single left (SLV) and 18 with single right (SRV) ventricle, who received a Blalock-Taussig (BT) shunt at an average age of 3.2 years were studied. Ventricular function was assessed angiographically by end-diastolic volume index (EDVI) and ejection fraction (EF), and attempts were made to measure ventricular mass index (VMI) and VM/EDV. In 14 patients, the preoperative and postoperative results (average 2.4 years after placement of BT shunt) were compared in SLV (n = 5) and SRV (n = 9) groups. The SLV group showed significant increases in EDVI, VMI, and VM/EDV without a significant change in EF. The SRV group showed significant increases in EDVI and VMI, while EF decreased and VM/EDV was unchanged. Late death from congestive heart failure occurred in five patients with SRV. Three patients with atrioventricular valve regurgitation suffered late death. Among the patients with SRV, the late death group had significantly lower preoperative EF and VM/EDV compared with the survivors (n = 13). All of those with a preoperative EF of less than 0.50 and a VM/EDV of less than 0.35 g/ml suffered late death. In summary, patients with SRV appear to fail to develop adaptational hypertrophy to volume loading after the BT shunt procedure, with concomitant depression in ventricular pump function. Also, late cardiac failure seems likely to develop when low EF and VM/EDV are present preoperatively. AD - First Department of Surgery, Osaka University Medical School, Fukushima-ku, Osaka 553 AU - Kuroda, O. AU - Sano, T. AU - Matsuda, H. DB - Embase Medline IS - 3 II SUPPl. KW - Blalock Taussig shunt child clinical article congenital disorder great blood vessel heart heart single ventricle heart ventricle function human priority journal therapy ventriculography LA - English M3 - Article N1 - L17133457 1987-11-09 PY - 1987 SN - 0009-7322 SP - 24-28 ST - Analysis of the effects of Blalock-Taussig shunt on ventricular function and the prognosis in patients with single ventricle T2 - Circulation TI - Analysis of the effects of Blalock-Taussig shunt on ventricular function and the prognosis in patients with single ventricle UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L17133457 VL - 76 ID - 1389 ER - TY - JOUR AB - Twenty-four patients with single ventricle, six with single left (SLV) and 18 with single right (SRV) ventricle, who received a Blalock-Taussig (BT) shunt at an average age of 3.2 years were studied. Ventricular function was assessed angiographically by end-diastolic volume index (EDVI) and ejection fraction (EF), and attempts were made to measure ventricular mass index (VMI) and VM/EDV. In 14 patients, the preoperative and postoperative results (average 2.4 years after placement of BT shunt) were compared in SLV (n = 5) and SRV (n = 9) groups. The SLV group showed significant increases in EDVI, VMI, and VM/EDV without a significant change in EF. The SRV group showed significant increases in EDVI and VMI, while EF decreased and VM/EDV was unchanged. Late death from congestive heart failure occurred in five patients with SRV. Three patients with atrioventricular valve regurgitation suffered late death. Among the patients with SRV, the late death group had significantly lower preoperative EF and VM/EDV compared with the survivors (n = 13). All of those with a preoperative EF of less than 0.50 and a VM/EDV of less than 0.35 g/ml suffered late death. In summary, patients with SRV appear to fail to develop adaptational hypertrophy to volume loading after the BT shunt procedure, with concomitant depression in ventricular pump function. Also, late cardiac failure seems likely to develop when low EF and VM/EDV are present preoperatively. AN - 2441894 AU - Kuroda, O. AU - Sano, T. AU - Matsuda, H. AU - Nakano, S. AU - Hirose, H. AU - Shimazaki, Y. AU - Kato, H. AU - Taniguchi, K. AU - Ogawa, M. AU - Kawashima, Y. DA - Sep DB - PubMed DP - NLM ET - 1987/09/01 IS - 3 Pt 2 KW - Child, Preschool Female Follow-Up Studies Heart Defects, Congenital/mortality/*surgery Heart Ventricles/*abnormalities Humans Male Myocardial Contraction Palliative Care/*methods Postoperative Complications/epidemiology Prognosis Stroke Volume LA - eng N1 - Kuroda, O Sano, T Matsuda, H Nakano, S Hirose, H Shimazaki, Y Kato, H Taniguchi, K Ogawa, M Kawashima, Y Journal Article United States Circulation. 1987 Sep;76(3 Pt 2):III24-8. PY - 1987 SN - 0009-7322 (Print) 0009-7322 SP - Iii24-8 ST - Analysis of the effects of the Blalock-Taussig shunt on ventricular function and the prognosis in patients with single ventricle T2 - Circulation TI - Analysis of the effects of the Blalock-Taussig shunt on ventricular function and the prognosis in patients with single ventricle VL - 76 ID - 432 ER - TY - JOUR AB - The purposes of this study were to characterize the morphologic, imaging, and clinical characteristics of double-inlet, double-outlet right ventricle (DI-DORV), a rare congenital heart disease, and to compare these findings to those in patients with double-inlet left ventricle (DILV). The clinical records, imaging studies, and heart specimens of 22 consecutive patients with DI-DORV were reviewed. A comparison group of 44 patients with DILV was matched by age at latest follow-up. Among the 22 patients with DI-DORV, 14 had clinical data, 5 had clinical and autopsy data, and 3 had only autopsy data. Abdominal and atrial situs were normal in all, and heart position was levocardia in 91%. The morphology of the atrioventricular valves was variable, with 15 patients showing neither a typical tricuspid nor mitral valve. In the remaining 7 patients, 1 or both atrioventricular valves resembled a tricuspid or a mitral valve. Myocardial architecture was characterized by atypical muscle bundles of varying degrees of hypertrophy and orientation. Most patients had bilateral conus, and 82% had either aortic or pulmonary outflow tract obstructions. The rate of overall mortality or heart transplantation was 36%. Significant differences between DI-DORV and DILV included the type of ventricular loop, the type of conus, and a higher mortality rate in patients with DI-DORV. In conclusion, DI-DORV is a distinct type of functional single ventricle congenital heart disease with variable atrioventricular valve morphology and myocardial architecture. Accurate diagnosis using noninvasive imaging techniques is of paramount importance for optimal management. © 2010 Elsevier Inc. All rights reserved. AD - Department of Cardiology, Children's Hospital Boston, Boston, MA, United States Department of Pathology, Children's Hospital Boston, Boston, MA, United States Department of Pediatrics, Harvard Medical School, Boston, MA, United States Department of Pathology, Harvard Medical School, Boston, MA, United States AU - Saleeb, S. F. AU - Juraszek, A. AU - Geva, T. DB - Scopus DO - 10.1016/j.amjcard.2009.09.056 IS - 4 M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 2010 SP - 542-549 ST - Anatomic, Imaging, and Clinical Characteristics of Double-Inlet, Double-Outlet Right Ventricle T2 - American Journal of Cardiology TI - Anatomic, Imaging, and Clinical Characteristics of Double-Inlet, Double-Outlet Right Ventricle UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-75349096342&doi=10.1016%2fj.amjcard.2009.09.056&partnerID=40&md5=9c01b9397ab4cde55a26ff07fcab97a4 VL - 105 ID - 2158 ER - TY - JOUR AB - In all, 67 patients with complete transposition of the great vessels were seen at the Children's Hospital, Columbus, Ohio during 7 yr. The type and number of communications were confirmed by necropsy and/or angiocardiogram in 40 patients. A single shunt was found in 37.5% and was due to a ventricular septal defect in 22.5%, a patent ductus arteriosus in 10%, and an atrial septal defect in 5%. A combination of 2 or 3 shunts was encountered in 21 patients (52.5%). Six patients (15%) had a single ventricle, and there was one with no apparent shunt at necropsy. Complete measurements of the free walls of the ventricles were recorded in 28 cases. Anatomical ventricular hypertrophy patterns were analysed in relation to the type and number of shunts. The hypertrophy patterns that were evolved from this study have been set out. AD - H.B. Calleja, Amerman Heart Clin., Philadelphia, PA, United States AU - Calleja, H. B. AU - Hosier, D. M. AU - Grajo, M. Z. DB - Embase Classic Medline IS - 5 KW - angiocardiography autopsy great blood vessel heart atrium septum defect heart single ventricle heart ventricle hypertrophy heart ventricle septum defect hypertrophy interpersonal communication patent ductus arteriosus patient pediatric hospital transfusion United States LA - English M3 - Article N1 - L281135951 1964-12-01 PY - 1964 SN - 0007-0769 SP - 642-648 ST - Anatomical ventricular hypertrophy patterns in complete transposition of the great vessels T2 - British Heart Journal TI - Anatomical ventricular hypertrophy patterns in complete transposition of the great vessels UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L281135951 VL - 26 ID - 1415 ER - TY - JOUR AB - Adult congenital heart disease, previously considered a rare comorbidity, is increasingly becoming a reality for today's anesthesia providers. Improvements in prenatal diagnosis, sophisticated surgical techniques and equipment, advances in pediatric critical care, enhanced efficacy of cardiovascular pharmacologic agents, and an overall increase in postrepair survival rates have resulted in an estimated population of approximately 800,000 adults with congenital heart disease. Despite successful surgical repair or palliation, these individuals present the anesthesia provider with a multitude of challenges. Individualized care of these fragile patients should be approached with a keen understanding of the patient's underlying cardiac anomaly. This case report chronicles the anesthetic care of a 36-year-old woman presenting for left-sided ureteroscopy with laser lithotripsy and stent placement. Her medical history was remarkable for the presence of complex congenital heart disease consisting of multiple anomalies: a double-outlet right ventricle, transposition of the great arteries, pulmonary stenosis, atrial septal defect, and a hypoplastic left ventricle with concomitant mitral valve atresia. General anesthesia was successfully administered, with meticulous attention given to maintenance of systemic vascular resistance to minimize shunting, oxygenation, administration of preprocedure antibiotics, and judicious replacement of intravenous fluids via air-filtered tubing. AD - M.S. Cosgrove, Hospital of St Raphael, School of Nurse Anesthesia, New Haven, CT, United States AU - Cosgrove, M. S. DB - Embase Medline IS - 3 KW - acetylsalicylic acid epinephrine amiodarone ampicillin analgesic agent aspartate aminotransferase bilirubin creatinine digoxin enalapril fentanyl ferrous sulfate gentamicin glycopyrronium infusion fluid ketamine levothyroxine midazolam morphine sulfate neostigmine ondansetron rocuronium sevoflurane ablation therapy adult anesthesia induction antibiotic prophylaxis article cardiomegaly case report cleft lip congestive heart failure conservative treatment cyanotic heart disease cystoscopy drug hypersensitivity female general anesthesia great vessels transposition heart arrest heart atrium flutter heart atrium septum defect heart left anterior bundle branch block heart left ventricle hypertrophy heart right bundle branch block heart right left shunt heart right ventricle double outlet heart ventricle fibrillation human hypoplastic left heart syndrome hypothyroidism laser lithotripsy leukocyte count lymphocyte count mean corpuscular volume mitral valve atresia muscle relaxation neutrophil count oxygen saturation oxygen therapy postoperative nausea and vomiting proteinuria pulmonary valve stenosis self report shunting stent systemic vascular resistance systolic heart murmur thorax radiography ureter stone ureteroscopy LA - English M3 - Article N1 - L365386207 2012-08-14 2012-08-28 http://www.aana.com/newsandjournal/Documents/congenital-cyanotic-heart-defect-0612-p197-203.pdf PY - 2012 SN - 0094-6354 SP - 197-203 ST - Anesthesia for the adult patient with an unrepaired congenital cyanotic heart defect: A case report T2 - AANA Journal TI - Anesthesia for the adult patient with an unrepaired congenital cyanotic heart defect: A case report UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L365386207 VL - 80 ID - 1058 ER - TY - JOUR AB - Anomalous left coronary artery connected to the pulmonary artery (ALCAPA) can be associated rarely with other congenital heart defects. The preoperative joint diagnosis is challenging. From 1987 to 2012, a retrospective bicentric assessment of 12 patients with ALCAPA related to other cardiac defects focused on the associated heart defect, the moment of complete diagnosis related to surgery, and outcome. Coarctation was the most frequently associated heart defect (n = 5) followed by tetralogy of Fallot with or without pulmonary atresia (n = 3). The study group comprised one case of hypoplastic left heart syndrome, one right aortic arch, one congenital mitral malformation, and one infant with divided left atrium and anomalous pulmonary venous return. Only four patients had a complete diagnosis of both the cardiac defect and the coronary abnormality before surgery. In two cases, the coronary anomaly was discovered during surgery performed for another cardiac defect and treated at the same time. The diagnosis of the six remaining patients was determined after cardiac repair. Of the 12 patients, 7 (58 %) died after surgery. Half of these patients died within the first 30 days after repair. At this writing, the remaining patients are in good health after a median follow-up period of 5.4 years (range, 2.1–8.5 years). This study confirmed that ALCAPA associated with other cardiac defects often is misdiagnosed before surgery, mostly due to specific hemodynamics masking myocardial ischemia preoperatively. Survival was compromised due to the unrecognized diagnosis of an associated coronary abnormality but also because of midterm complications related to the other cardiac defects. AD - D. Laux, Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C-CCML, Department of Congenital Cardiac Surgery, Centre Chirurgical Marie Lannelongue, 133 Avenue de la Résistance, Le Plessis Robinson, France AU - Laux, D. AU - Bertail, C. AU - Bajolle, F. AU - Houyel, L. AU - Boudjemline, Y. AU - Bonnet, D. DB - Embase Medline DO - 10.1007/s00246-014-0916-4 IS - 7 KW - aortic arch aortic coarctation congenital heart disease congenital heart malformation congenital malformation coronary artery anomaly diagnosis Fallot tetralogy follow up health heart heart left atrium heart muscle ischemia hemodynamics human hypoplastic left heart syndrome infant left coronary artery lung vein drainage anomaly patient pulmonary artery pulmonary valve atresia surgery survival writing LA - English M3 - Article N1 - L53181004 2014-06-13 PY - 2014 SN - 1432-1971 0172-0643 SP - 1198-1205 ST - Anomalous Left Coronary Artery Connected to the Pulmonary Artery Associated With Other Cardiac Defects: A Difficult Joint Diagnosis T2 - Pediatric Cardiology TI - Anomalous Left Coronary Artery Connected to the Pulmonary Artery Associated With Other Cardiac Defects: A Difficult Joint Diagnosis UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L53181004 http://dx.doi.org/10.1007/s00246-014-0916-4 VL - 35 ID - 958 ER - TY - JOUR AB - We report a case of anomalous origin of the left anterior descending coronary artery (LAD) from the main pulmonary artery in a child with hypoplastic left heart syndrome (mitral atresia/aortic atresia). Mechanical circulatory support was necessary because of the inability to wean from cardiopulmonary bypass after the Norwood procedure. The patient died at 4 months of age having continued depressed right ventricular function. The diagnosis was made during a catheterization performed 6 weeks after surgery because of concern for stenosis of Blalock-Taussig shunt. We believe his prolonged postoperative recovery and eventual demise can partially be attributed to lack of cardioplegia to the anomalous LAD territory during surgery. AD - Divisions of Pediatric Cardiology and Pediatric Cardiovascular Surgery, University of Texas Health Science Center, Houston, Texas. Divisions of Pediatric Cardiology and Pediatric Cardiovascular Surgery, University of Texas Health Science Center, Houston, Texas. Electronic address: duraisamy.balaguru@uth.tmc.edu. AN - 26652530 AU - Turiy, Y. AU - Douglas, W. AU - Balaguru, D. DA - Dec DB - PubMed DO - 10.1016/j.athoracsur.2015.01.077 DP - NLM ET - 2015/12/15 IS - 6 KW - Coronary Vessel Anomalies/complications/*diagnosis/surgery Fatal Outcome Humans Hypoplastic Left Heart Syndrome/complications/*diagnosis/surgery Infant, Newborn Male Pulmonary Artery/*abnormalities LA - eng N1 - 1552-6259 Turiy, Yuliya Douglas, William Balaguru, Duraisamy Case Reports Journal Article Netherlands Ann Thorac Surg. 2015 Dec;100(6):2346-8. doi: 10.1016/j.athoracsur.2015.01.077. PY - 2015 SN - 0003-4975 SP - 2346-8 ST - Anomalous Origin of Coronary Artery From Main Pulmonary Artery in Hypoplastic Left Heart Syndrome T2 - Ann Thorac Surg TI - Anomalous Origin of Coronary Artery From Main Pulmonary Artery in Hypoplastic Left Heart Syndrome VL - 100 ID - 286 ER - TY - JOUR AB - The article discusses pregnancy and childbirth by referring to early identification and timely interventions to prevent mortality from childbirth. Topics discussed include antenatal care such as counseling couples with recurrent pregnancy loss and prediction of fetuses at risk of perinatal morbidity, an understanding of the molecular control of gametogenesis, pre-implantation genetic diagnosis, pregnancy loss rate, and intervention to improve prognosis in the congenital diaphragmatic hernia. AD - Executive Chairman Women’s Clinical Services Management Group (WCMG), Sidra Medicine, Professor of Obstetrics and Gynaecology, Weill Cornell Medical School, Doha, Qatar Emeritus Professor University of Leicester, Robert Kilpatrick Clinical Sciences Building, Leicester Royal Infirmary, Leicester LE2 7LX, UK AN - 131732974. Language: English. Entry Date: 20180920. Revision Date: 20191106. Publication Type: Editorial AU - Konje, Justin C. DB - ccm DO - 10.1515/jpm-2018-0275 DP - EBSCOhost IS - 7 KW - Prenatal Care Prepregnancy Care Infant Mortality -- Prevention and Control Morbidity -- Prevention and Control -- In Infancy and Childhood Pregnancy Female Infant Serial Publications Childbirth Risk Management Aneuploidy Pre-Eclampsia Abortion, Spontaneous Pregnancy Trimester, First Decision Making, Clinical Pregnancy, Twin Hypoplastic Left Heart Syndrome -- Surgery Balloon Dilatation -- Methods Ventricular Ejection Fraction Extracorporeal Membrane Oxygenation Respiration, Artificial Fetal Abnormalities -- Psychosocial Factors Maternal Attitudes N1 - editorial. Journal Subset: Biomedical; Continental Europe; Double Blind Peer Reviewed; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed. NLM UID: 0361031. PY - 2018 SN - 0300-5577 SP - 697-700 ST - Antenatal and prepregnancy care – prevention of perinatal morbidity and mortality T2 - Journal of Perinatal Medicine TI - Antenatal and prepregnancy care – prevention of perinatal morbidity and mortality UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=131732974&site=ehost-live&scope=site VL - 46 ID - 1473 ER - TY - JOUR AB - OBJECTIVE: Prenatal exposures are known to alter fetal neurodevelopment and autonomic control. We aimed to explore the correlation between fetal autonomic activity, measured by fetal heart rate variability, and 18-month developmental outcome in subjects with congenital heart disease. STUDY DESIGN: From 2010 to 2013, 5 fetuses with hypoplastic left heart syndrome, 9 with transposition of the great arteries and 9 with tetralogy of Fallot were included in this prospective cohort study. A maternal abdominal fetal electrocardiogram monitor recorded fetal heart rate at 34 to 38 weeks gestational age. We assessed associations between fetal heart rate parameters including interquartile range and s.d. of the fetal RR intervals and 18-month Bayley Scales of Infant Development-III scores using Pearson's correlation coefficient. Multivariable regression modeling identified predictors of neurodevelopmental scores. RESULTS: Fetal heart rate variability parameters at 34 to 38 weeks gestational age correlated with 18-month Cognition (r=0.47, P=0.03) and Motor scores (r=0.66, P=0.001). The interquartile range of the fetal RR intervals predicted Cognition (β=0.462, P=0.028, R(2)=0.282) and Motor (β=0.637, P<0.001, R(2)=0.542) scores. CONCLUSIONS: In fetuses with congenital heart disease, low heart rate variability at 34 to 38 weeks gestational age predicts diminished 18-month Cognitive and Motor performance. Prenatal autonomic activity may serve as a marker of early childhood development in these high-risk patients. AD - Department of Pediatrics, Columbia University Medical Center, New York, NY, USA. Department of Psychiatry, Columbia University Medical Center, New York, NY, USA. Division of Developmental Neuroscience, New York State Psychiatric Institute, New York, NY, USA. AN - 28471442 AU - Siddiqui, S. AU - Fifer, W. P. AU - Ordonez-Retamar, M. AU - Nugent, J. D. AU - Williams, I. A. C2 - PMC5578909 C6 - NIHMS864887 article to disclose. DA - Aug DB - PubMed DO - 10.1038/jp.2017.59 DP - NLM ET - 2017/05/05 IS - 8 KW - Cardiotocography/*methods Child Development Cohort Studies Female Gestational Age *Heart Rate, Fetal Humans *Hypoplastic Left Heart Syndrome/complications/diagnosis/psychology Infant Infant, Newborn Male Mental Status and Dementia Tests *Neurocognitive Disorders/diagnosis/etiology Prenatal Care/methods Prenatal Diagnosis/methods Prospective Studies Statistics as Topic *Tetralogy of Fallot/complications/diagnosis/psychology *Transposition of Great Vessels/complications/diagnosis/psychology United States LA - eng N1 - 1476-5543 Siddiqui, S Fifer, W P Ordonez-Retamar, M Nugent, J D Williams, I A K23 HD061601/HD/NICHD NIH HHS/United States R37 HD032774/HD/NICHD NIH HHS/United States Journal Article Research Support, N.I.H., Extramural J Perinatol. 2017 Aug;37(8):953-957. doi: 10.1038/jp.2017.59. Epub 2017 May 4. PY - 2017 SN - 0743-8346 (Print) 0743-8346 SP - 953-957 ST - An antenatal marker of neurodevelopmental outcomes in infants with congenital heart disease T2 - J Perinatol TI - An antenatal marker of neurodevelopmental outcomes in infants with congenital heart disease VL - 37 ID - 144 ER - TY - JOUR AB - BACKGROUND: Abnormal height and adiposity are observed after the Fontan operation. These abnormalities may be associated with worse functional outcome. METHODS: We analyzed data from the National Heart, Lung, and Blood Institute Pediatric Heart Network cross-sectional study of Fontan patients. Groups were defined by height (z-score<-1.5 or≥-1.5) and body mass index (body mass index [BMI] z-score<-1.5 or -1.5 to 1.5 or≥1.5). Associations of anthropometric measures with measurements from clinical testing (exercise, echocardiography, magnetic resonance imaging) were determined adjusting for demographics, anatomy, and pre-Fontan status. Relationships between anthropometric measures and functional health status (FHS) were assessed using the Child Health Questionnaire. RESULTS: Mean age of the cohort (n=544) was 11.9±3.4 years. Lower height-z patients (n=124, 23%) were more likely to have pre-Fontan atrioventricular valve regurgitation (P=.029), as well as orthopedic and developmental problems (both P<.001). Lower height-z patients also had lower physical and psychosocial FHS summary scores (both P<.01). Higher BMI-z patients (n=45, 8%) and lower BMI-z patients (n=53, 10%) did not have worse FHS compared to midrange BMI-z patients (n=446, 82%). However, higher BMI-z patients had higher ventricular mass-to-volume ratio (P=.03) and lower % predicted maximum work (P=.004) compared to midrange and lower BMI-z patients. CONCLUSIONS: Abnormal anthropometry is common in Fontan patients. Shorter stature is associated with poorer FHS and non-cardiac problems. Increased adiposity is associated with more ventricular hypertrophy and poorer exercise performance, which may have significant long-term implications in this at-risk population. AD - The Children's Hospital of Philadelphia, Philadelphia, PA. AN - 21146663 AU - Cohen, M. S. AU - Zak, V. AU - Atz, A. M. AU - Printz, B. F. AU - Pinto, N. AU - Lambert, L. AU - Pemberton, V. AU - Li, J. S. AU - Margossian, R. AU - Dunbar-Masterson, C. AU - McCrindle, B. W. C2 - PMC3058397 C6 - NIHMS231442 DA - Dec DB - PubMed DO - 10.1016/j.ahj.2010.07.039 DP - NLM ET - 2010/12/15 IS - 6 KW - *Body Height *Body Weight Child Cross-Sectional Studies Disease Progression Female Fontan Procedure/*methods Heart Defects, Congenital/diagnosis/physiopathology/*surgery Humans Hypertrophy, Left Ventricular/diagnosis/*etiology/physiopathology Magnetic Resonance Imaging Male Overweight/*complications/physiopathology Postoperative Period Risk Factors Surveys and Questionnaires LA - eng N1 - 1097-6744 Cohen, Meryl S Zak, Victor Atz, Andrew M Printz, Beth F Pinto, Nelangi Lambert, Linda Pemberton, Victoria Li, Jennifer S Margossian, Renee Dunbar-Masterson, Carolyn McCrindle, Brian W U01 HL068279-01/HL/NHLBI NIH HHS/United States HL068288/HL/NHLBI NIH HHS/United States HL068285/HL/NHLBI NIH HHS/United States U01 HL068269/HL/NHLBI NIH HHS/United States U01 HL068279/HL/NHLBI NIH HHS/United States U01 HL068290/HL/NHLBI NIH HHS/United States HL068279/HL/NHLBI NIH HHS/United States U01 HL068288/HL/NHLBI NIH HHS/United States U10 HL068270/HL/NHLBI NIH HHS/United States U01 HL068281/HL/NHLBI NIH HHS/United States U01 HL068270/HL/NHLBI NIH HHS/United States HL068281/HL/NHLBI NIH HHS/United States U01 HL068292/HL/NHLBI NIH HHS/United States HL068269/HL/NHLBI NIH HHS/United States HL068270/HL/NHLBI NIH HHS/United States HL068290/HL/NHLBI NIH HHS/United States U01 HL068285/HL/NHLBI NIH HHS/United States HL068292/HL/NHLBI NIH HHS/United States Comparative Study Journal Article Multicenter Study Randomized Controlled Trial Research Support, N.I.H., Extramural Am Heart J. 2010 Dec;160(6):1092-8, 1098.e1. doi: 10.1016/j.ahj.2010.07.039. PY - 2010 SN - 0002-8703 (Print) 0002-8703 SP - 1092-8, 1098.e1 ST - Anthropometric measures after Fontan procedure: implications for suboptimal functional outcome T2 - Am Heart J TI - Anthropometric measures after Fontan procedure: implications for suboptimal functional outcome VL - 160 ID - 358 ER - TY - JOUR AB - Background: Abnormal height and adiposity are observed after the Fontan operation. These abnormalities may be associated with worse functional outcome.Methods: We analyzed data from the National Heart, Lung, and Blood Institute Pediatric Heart Network cross-sectional study of Fontan patients. Groups were defined by height (z-score<-1.5 or≥-1.5) and body mass index (body mass index [BMI] z-score<-1.5 or -1.5 to 1.5 or≥1.5). Associations of anthropometric measures with measurements from clinical testing (exercise, echocardiography, magnetic resonance imaging) were determined adjusting for demographics, anatomy, and pre-Fontan status. Relationships between anthropometric measures and functional health status (FHS) were assessed using the Child Health Questionnaire.Results: Mean age of the cohort (n=544) was 11.9±3.4 years. Lower height-z patients (n=124, 23%) were more likely to have pre-Fontan atrioventricular valve regurgitation (P=.029), as well as orthopedic and developmental problems (both P<.001). Lower height-z patients also had lower physical and psychosocial FHS summary scores (both P<.01). Higher BMI-z patients (n=45, 8%) and lower BMI-z patients (n=53, 10%) did not have worse FHS compared to midrange BMI-z patients (n=446, 82%). However, higher BMI-z patients had higher ventricular mass-to-volume ratio (P=.03) and lower % predicted maximum work (P=.004) compared to midrange and lower BMI-z patients.Conclusions: Abnormal anthropometry is common in Fontan patients. Shorter stature is associated with poorer FHS and non-cardiac problems. Increased adiposity is associated with more ventricular hypertrophy and poorer exercise performance, which may have significant long-term implications in this at-risk population. AD - The Children's Hospital of Philadelphia, Philadelphia, PA AN - 104966543. Language: English. Entry Date: 20110218. Revision Date: 20161222. Publication Type: journal article AU - Cohen, M. S. AU - Zak, V. AU - Atz, A. M. AU - Printz, B. F. AU - Pinto, N. AU - Lambert, L. AU - Pemberton, V. AU - Li, J. S. AU - Margossian, R. AU - Dunbar-Masterson, C. AU - McCrindle, B. W. AU - Cohen, Meryl S. AU - Zak, Victor AU - Atz, Andrew M. AU - Printz, Beth F. AU - Pinto, Nelangi AU - Lambert, Linda AU - Pemberton, Victoria AU - Li, Jennifer S. AU - Margossian, Renee DB - ccm DO - 10.1016/j.ahj.2010.07.039 DP - EBSCOhost IS - 6 KW - Body Height Body Weight Cardiopulmonary Bypass -- Methods Heart Defects, Congenital -- Surgery Hypertrophy, Left Ventricular -- Etiology Obesity -- Complications Child Cross Sectional Studies Disease Progression Female Heart Defects, Congenital -- Diagnosis Heart Defects, Congenital -- Physiopathology Human Hypertrophy, Left Ventricular -- Diagnosis Hypertrophy, Left Ventricular -- Physiopathology Magnetic Resonance Imaging Male Obesity -- Physiopathology Postoperative Period Questionnaires Risk Factors N1 - research. Journal Subset: Biomedical; USA. Grant Information: HL068292/HL/NHLBI NIH HHS/United States. NLM UID: 0370465. PMID: NLM21146663. PY - 2010 SN - 0002-8703 SP - 1092-1098.e1 ST - Anthropometric measures after Fontan procedure: implications for suboptimal functional outcome T2 - American Heart Journal TI - Anthropometric measures after Fontan procedure: implications for suboptimal functional outcome UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104966543&site=ehost-live&scope=site VL - 160 ID - 1636 ER - TY - JOUR AB - OBJECTIVES: Caring for children with congenital heart disease places significant stress on caregivers. Minimal data exist evaluating stress levels in caregivers of children with hypoplastic left heart syndrome (HLHS). The goal of this study was to obtain baseline stress scores for caregivers of children with HLHS and determine if associations exist between scores and specific caregiver factors. STUDY DESIGN: A cross-sectional study using a web-based survey targeted towards caregivers of children with HLHS was performed. Baseline demographics of the caregiver and child with HLHS were obtained. Caregivers completed three validated questionnaires including the Pediatric Quality of Life Inventory (PedsQL), Parenting Stress Index- Short Form (PSI-SF) and the Pediatric Inventory for Parents (PIP). RESULTS: Four hundred fifty-nine caregivers completed at least one questionnaire. PSI-SF total score was 80.5 ± 23.1 (> 86 = significant stress), PIP frequency total score was 119.0 ± 37.2, and PIP difficulty total score was 118.1 ± 35.7. Lower quality of life was significantly correlated with higher scores on the PSI-SF (r = -0.6), the presence of a developmental issue in the child (r = 0.3) as well as higher scores on the PIP frequency (r = -0.5) and difficulty scales (r = 0.4). Other demographic values for the caregiver and child did not significantly correlate with PSI or PIP total scores. CONCLUSIONS: Anxiety/stress scores of caregivers with children with HLHS are correlated with how well the child is perceived to be doing physically and developmentally by the caregivers. Caregivers with physical and/or developmental concerns may need added psychosocial support. AD - Nationwide Children's Hospital, Columbus, Ohio. Nicklaus Children's Hospital, Columbus, Ohio. AN - 27320439 AU - Caris, E. C. AU - Dempster, N. AU - Wernovsky, G. AU - Butz, C. AU - Neely, T. AU - Allen, R. AU - Stewart, J. AU - Miller-Tate, H. AU - Fonseca, R. AU - Texter, K. AU - Nicholson, L. AU - Cua, C. L. DA - Dec DB - PubMed DO - 10.1111/chd.12387 DP - NLM ET - 2016/06/21 IS - 6 KW - Adaptation, Psychological Adult Anxiety/diagnosis/*etiology/psychology Caregivers/*psychology Cost of Illness Cross-Sectional Studies Female Health Surveys Humans Hypoplastic Left Heart Syndrome/diagnosis/psychology/*therapy Male Mental Health Prospective Studies *Quality of Life Risk Factors Stress, Psychological/diagnosis/*etiology/psychology *Congenital Heart Disease *Hypoplastic Left Heart *Parenting Stress *Survey LA - eng N1 - 1747-0803 Caris, Elizabeth C Dempster, Nicole Wernovsky, Gil Butz, Catherine Neely, Trent Allen, Robin Stewart, Jamie Miller-Tate, Holly Fonseca, Rachel Texter, Karen Nicholson, Lisa Cua, Clifford L Journal Article United States Congenit Heart Dis. 2016 Dec;11(6):727-732. doi: 10.1111/chd.12387. Epub 2016 Jun 20. PY - 2016 SN - 1747-079x SP - 727-732 ST - Anxiety Scores in Caregivers of Children with Hypoplastic Left Heart Syndrome T2 - Congenit Heart Dis TI - Anxiety Scores in Caregivers of Children with Hypoplastic Left Heart Syndrome VL - 11 ID - 81 ER - TY - JOUR AB - Objective: Despite the improvement in life-expectancy of adults with congenital heart disease, they may experience unique medical and social challenges that could impact on their psychological functioning. The aims of this study were to address the experience of anxiety, depressive and somatic symptoms among adults with congenital heart disease in comparison with that of non-heart diseased persons considering the role of various factors (e.g. socio-economic). Methods: In cross-sectional case-control study, the participants consisted of 347 patients with congenital heart disease (18-64. years, 52.2% female) and 353 matched (by sex/age) non-heart diseased persons. The participants completed a questionnaire. The data were analyzed with bivariate and multivariate methods. Results: In bivariate analyses, scores in anxiety and somatic symptoms were higher among patients than the healthy controls (both at p≤ 0.001), whereas the groups did not differ in depressive symptoms. Following multiple-linear-regression-analyses, only the association between congenital heart disease and somatic symptoms was confirmed. Among the patients, perceived financial strain was significantly related to anxiety, depressive and somatic symptoms; lower perceived social support to anxiety and depression; and low annual income to somatic symptoms. Additionally, somatic symptoms were associated with anxiety and depressive symptoms, and vice versa. And no medical variables were related to anxiety, depressive and somatic symptoms. Conclusions: Congenital heart disease was only independently associated with somatic symptoms. Financial strain, social support and co-existence of emotional distress with somatic symptoms should be considered in developing appropriate interventions to improve the well-being of patients with congenital heart disease. However, longitudinal research is warranted to clarify causality. © 2012 Elsevier Inc.. AD - Institution of Health Sciences, Department of Public Health Sciences, Mid Sweden University, Sundsvall, Sweden Department of Research, Tehran Heart Center, Tehran University of Medical Sciences, Tehran, Iran Institution for Social Sciences, Department of Psychology, Mid Sweden University, Östersund, Sweden Department of Occupational and Public Health Sciences, University of Gävle, Gävle, Sweden Institution of Social Medicine, Department of Public Health Sciences, Karolinska Institutet, Stockholm, Sweden Department of Nursing, University of Social Welfare and Rehabilitation Sciences, Tehran, Iran AU - Eslami, B. AU - Sundin, T. AU - Macassa, G. AU - Khankeh, H. R. AU - Soares, J. J. F. DB - Scopus DO - 10.1016/j.jpsychores.2012.10.006 IS - 1 KW - Congenital heart disease Grown-up Mental health Psychosomatic Social functioning M3 - Article N1 - Cited By :31 Export Date: 15 June 2020 PY - 2013 SP - 49-56 ST - Anxiety, depressive and somatic symptoms in adults with congenital heart disease T2 - Journal of Psychosomatic Research TI - Anxiety, depressive and somatic symptoms in adults with congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84871686549&doi=10.1016%2fj.jpsychores.2012.10.006&partnerID=40&md5=9e5c9a712f1198d62242a39302f80972 VL - 74 ID - 2069 ER - TY - JOUR AB - OBJECTIVE: To describe the incidence, characteristics, and outcomes of hospitalized children and young adults with aortic dissection (AD). DESIGN: Retrospective review. SETTING: The Pediatric Health Information System database, a multiinstitutional administrative database. PATIENTS: All index cases of patients <30 years old hospitalized with AD between January 2004 and June 2011. OUTCOME MEASURES: The primary outcome measure was inpatient mortality. RESULTS: Of 3 800 964 hospitalizations, AD was identified in 124 (<1%), accounting for 110 patients (69% male, P = .003) at a median age of 12.9 (interquartile range 3.9-16.8) years with a bimodal distribution in infancy and late adolescence. Associated diagnoses included congenital heart disease (CHD) (38%), trauma (24%), connective tissue disease (CTD) (16%), and isolated hypertension (HTN) (8%). Common CHD diagnoses included aortic arch (24%) and valve (21%) disease, hypoplastic left heart syndrome (10%), and transposition of the great arteries (10%). CHD patients were younger and more likely to undergo inpatient non-AD-related cardiovascular procedures compared with other diagnostic groups (P < .001 for both). Marfan and Ehlers-Danlos syndrome were present in 72% and 11% of CTD patients, respectively. Overall in-hospital mortality in patients with AD was 13% compared with 1% in the database population (odds ratio 12.0, 95% confidence interval 6.9-21.1). By diagnostic category, mortality was 22% in HTN, 22% in CTD, 12% in CHD, and 4% in trauma. CONCLUSIONS: AD is rare in children and young adults but most commonly occurs in CHD and CTD and in males. AD is associated with high inpatient mortality. AD - Lillie Frank Abercrombie Section of Cardiology, Department of Pediatrics, Texas Children's Hospital, Baylor College of Medicine, Houston, Tex, USA. AN - 23682693 AU - Shamszad, P. AU - Barnes, J. N. AU - Morris, S. A. DA - Jan-Feb DB - PubMed DO - 10.1111/chd.12090 DP - NLM ET - 2013/05/21 IS - 1 KW - Adolescent Adult Age Factors Aneurysm, Dissecting/diagnosis/*mortality/therapy Aortic Aneurysm/diagnosis/*mortality/therapy Chi-Square Distribution Child Child, Preschool Connective Tissue Diseases/mortality Female Heart Defects, Congenital/mortality *Hospital Mortality Humans Hypertension/mortality Incidence *Inpatients Male Odds Ratio Retrospective Studies Risk Assessment Risk Factors United States/epidemiology Wounds and Injuries/mortality Young Adult Aortic Disease Hospital Utilization Mortality Pediatrics LA - eng N1 - 1747-0803 Shamszad, Pirouz Barnes, Jeff N Morris, Shaine A Journal Article Multicenter Study Observational Study United States Congenit Heart Dis. 2014 Jan-Feb;9(1):54-62. doi: 10.1111/chd.12090. Epub 2013 May 20. PY - 2014 SN - 1747-079x SP - 54-62 ST - Aortic dissection in hospitalized children and young adults: a multiinstitutional study T2 - Congenit Heart Dis TI - Aortic dissection in hospitalized children and young adults: a multiinstitutional study VL - 9 ID - 188 ER - TY - JOUR AB - Percutaneous cardiac intervention is an invasive diagnostic and therapeutic technique which carries a significant complication rate. Although the usefulness of EFAST protocol is widely recognised, this paper will attempt to explore a modified approach involving a focused examination on the retroperitoneal (r-EFAST). We have provided examples of 3 cases where r-EFAST was used to detect retroperitoneal bleeding in critical situations. © 2017, The Author(s). AD - Pediatric Intensive Care Unit, Ramón y Cajal University Hospital, Madrid, Spain Pediatric Intensive Care Unit, Crta Colmenar Viejo 9100, Madrid, 28034, Spain Emergency Department, Ramón y Cajal University Hospital, Madrid, Spain Pediatric Cardiology Department, Ramón y Cajal University Hospital, Madrid, Spain AU - Vázquez Martínez, J. L. AU - Quiñones Coneo, K. L. AU - Villegas, T. V. AU - Sánchez Porras, M. AU - Macarrón, C. P. C. AU - Pérez, A. C. AU - Pineda, L. F. C7 - 12 DB - Scopus DO - 10.1186/s13089-017-0070-3 IS - 1 KW - Cardiac catheterization Congenital heart defect Hematoma Retroperitoneal space M3 - Article N1 - Export Date: 15 June 2020 PY - 2017 ST - Applicability of a modified EFAST protocol (r-EFAST) to evaluate hemodynamically unstable patients after percutaneous cardiac intervention T2 - Critical Ultrasound Journal TI - Applicability of a modified EFAST protocol (r-EFAST) to evaluate hemodynamically unstable patients after percutaneous cardiac intervention UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85020787194&doi=10.1186%2fs13089-017-0070-3&partnerID=40&md5=d74c856d260e06cba287e3afd67f0f79 VL - 9 ID - 1839 ER - TY - JOUR AB - Laboratory and clinical data have confirmed the deleterious effects of deep hypothermic circulatory arrest. Long-term data and rigorous neuropsychiatric data are currently sparse, but that which are available show adverse outcomes following circulatory arrest, and therefore support the use of continuous perfusion techniques. There are no available long-term data on continuous perfusion techniques with respect to neurologic outcomes, but using these techniques the incidence of postoperative seizures or other neurologic events is rare. Currently available bypass systems and microsurgical techniques have allowed continuous flow and regional perfusion to become practical alternatives. Several innovative techniques for avoiding circulatory arrest during neonatal aortic arch reconstruction for univentricular and biventricular hearts are described. It would appear prudent and desirable to provide continuous perfusion now that long-term survival after repair of even the most complex cardiac anomalies including single ventricle defects is commonplace. Copyright © 2002 by W.B. Saunders Company. AD - F.L. Hanley, Pediatric Heart Center, Cardiovascular Research Center, 300 Pasteur Dr, Stanford, CA 94305-5407, United States AU - MacDonald, M. J. AU - Hanley, F. L. AU - Reddy, V. M. DB - Embase Medline DO - 10.1053/pcsu.2002.31503 KW - aortic arch aortic reconstruction article brain blood flow brain perfusion cardiopulmonary bypass child heart arrest heart disease heart single ventricle human infant mental disease microsurgery neurologic disease newborn outcomes research postoperative complication profound induced hypothermia regional perfusion seizure surgical technique survival rate LA - English M3 - Article N1 - L38196205 2004-02-25 PY - 2002 SN - 1092-9126 SP - 95-103 ST - Arch reconstruction without circulatory arrest: Current clinical applications and results of therapy T2 - Pediatric Cardiac Surgery Annual TI - Arch reconstruction without circulatory arrest: Current clinical applications and results of therapy UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L38196205 http://dx.doi.org/10.1053/pcsu.2002.31503 VL - 5 ID - 1310 ER - TY - JOUR AB - BACKGROUND: Literature on the risk of birth defects among foreign- versus U.S.-born Hispanics is limited or inconsistent. We examined the association between country of birth, immigration patterns, and birth defects among Hispanic mothers. METHODS: We used data from the National Birth Defects Prevention Study and calculated odds ratios (ORs) and 95% confidence intervals and assessed the relationship between mothers' country of birth, years lived in the United States, and birth defects among 575 foreign-born compared to 539 U.S.-born Hispanic mothers. RESULTS: Hispanic mothers born in Mexico/Central America were more likely to deliver babies with spina bifida (OR = 1.53) than their U.S.-born counterparts. Also, mothers born in Mexico/Central America or who were recent United States immigrants (≤5 years) were less likely to deliver babies with all atrial septal defects combined, all septal defects combined, or atrial septal defect, secundum type. However, Hispanic foreign-born mothers who lived in the United States for >5 years were more likely to deliver babies with all neural tube defects combined (OR = 1.42), spina bifida (OR = 1.89), and longitudinal limb defects (OR = 2.34). Foreign-born mothers, regardless of their number of years lived in the United States, were more likely to deliver babies with anotia or microtia. CONCLUSIONS: Depending on the type of birth defect, foreign-born Hispanic mothers might be at higher or lower risk of delivering babies with the defects. The differences might reflect variations in predisposition, cultural norms, behavioral characteristics, and/or ascertainment of the birth defects. © 2009 Wiley-Liss, Inc. AD - T. Ramadhani, Birth Defects Epidemiology and Surveillance Branch, Texas Department of State Health Services, 1100 West 49th Street, Austin, TX, United States AU - Ramadhani, T. AU - Short, V. AU - Canfield, M. A. AU - Waller, D. K. AU - Correa, A. AU - Royle, M. AU - Scheuerle, A. DB - Embase Medline DO - 10.1002/bdra.20584 IS - 9 KW - adult anencephalus anus atresia aortic coarctation aortic stenosis article ataxia baby behavior congenital malformation Central America cleft lip cleft palate comparative study controlled study craniofacial synostosis obstetric delivery diaphragm hernia disease association disease predisposition esophagus atresia Fallot tetralogy female gastroschisis genetic variability gestational age great vessels transposition heart atrium septum defect heart left ventricle outflow tract obstruction heart right ventricle outflow tract obstruction heart septum defect heart ventricle septum defect Hispanic human hypoplastic left heart syndrome hypospadias immigration infant limb defect longitudinal study lung vein drainage anomaly major clinical study male maternal age Mexico microtia mother neural tube defect omphalocele priority journal pulmonary valve stenosis risk spinal dysraphism United States LA - English M3 - Article N1 - L355278366 2009-10-27 http://www3.interscience.wiley.com/cgi-bin/fulltext/122307129/PDFSTART PY - 2009 SN - 1542-0752 1542-0760 SP - 755-763 ST - Are birth defects among hispanics related to maternal nativity or number of years lived in the United States? T2 - Birth Defects Research Part A - Clinical and Molecular Teratology TI - Are birth defects among hispanics related to maternal nativity or number of years lived in the United States? UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L355278366 http://dx.doi.org/10.1002/bdra.20584 VL - 85 ID - 1177 ER - TY - JOUR AB - Background: CHD is the leading cause of mortality due to birth defects. Array comparative genomic hybridisation (aCGH) detects submicroscopic copy number changes and may improve identification of the genetic basis of CHD. Methods: This is a retrospective analysis of 1252 patients from a regional referral centre who had undergone aCGH. Of the patients, 173 had CHD. A whole-genome custom-designed oligonucleotide array with >44,000 probes was used to detect copy number changes. Results: Of the 1252 patients, 335 (26.76%) had abnormal aCGH results. Of the 173 patients with CHD, 50 (28.9%) had abnormal aCGH results versus 284 (26.3%) of 1079 non-cardiac patients. There were six patients with CHD who had well-described syndromes such as Wolf-Hirschhorn, trisomy 13, DiGeorge, and Williams. Of the patients with CHD, those with left-sided heart disease had the highest proportion (14/31; 45.13%) of abnormal aCGH results, followed by those with conotruncal heart disease (10/29; 34.48%), endocardial cushion defects (13/50; 26%), complex/other heart disease (12/52; 23.08%), and patent ductus arteriosus (1/11; 9.09%). Conclusions: Patients with CHD are at a substantial risk of having microdeletions and microduplications. The incidence of abnormalities on aCGH analysis is higher than identified with karyotype, and identification of copy number changes may help identify the genetic basis of the specific heart defects. However, aCGH may not have a significant diagnostic yield in those with isolated CHD. Further research using larger data sets may help identify candidate genes associated with CHD. AD - N. Ambalavanan, Department of Pediatrics, University of Alabama at Birmingham, Women and Infants Center, 619 South 19th Street, Birmingham, AL, United States AU - Hightower, H. B. AU - Robin, N. H. AU - Mikhail, F. M. AU - Ambalavanan, N. DB - Embase Medline DO - 10.1017/S1047951114001838 IS - 6 KW - left right determination factor protein Nodal transcription factor GATA 4 transcription factor GATA 6 aortic arch interruption aortic coarctation aortic valve disease array comparative genomic hybridization article chromosome deletion chromosome deletion 15q11 chromosome deletion 22q11 comparative genomic hybridization congenital heart malformation controlled study copy number variation CRELD1 gene DiGeorge syndrome DNA microarray endocardial cushion defect Fallot tetralogy GATA4 gene GATA6 gene gene great vessels transposition heart atrium septum defect heart right ventricle double outlet human hypoplastic left heart syndrome infant karyotype LEFTY gene lung vein drainage anomaly major clinical study male NKX25 gene NODAL gene patent ductus arteriosus pulmonary valve atresia retrospective study TBX20 gene trisomy 13 Williams Beuren syndrome Wolf Hirschhorn syndrome ZIC3 gene LA - English M3 - Article N1 - L600644008 2014-12-09 2015-11-06 PY - 2015 SN - 1467-1107 1047-9511 SP - 1155-1172 ST - Array comparative genomic hybridisation testing in CHD T2 - Cardiology in the Young TI - Array comparative genomic hybridisation testing in CHD UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L600644008 http://dx.doi.org/10.1017/S1047951114001838 VL - 25 ID - 915 ER - TY - JOUR AB - Background and Objectives: The purpose of the present study was to investigate the advantages and disadvantages of verifying genetic abnormalities using array comparative genomic hybridization (a-CGH) immediately afer diagnosis of congenital heart disease (CHD). Methods: Among neonates under the age of 28 days who underwent echocardiography from January 1, 2014 to April 30, 2016, neonates whose chromosomal and genomic abnormalities were tested using a-CGH in cases of an abnormal fnding on echocardiography were enrolled. Results: Of the 166 patients diagnosed with CHD, 81 underwent a-CGH and 11 patients (11/81, 13.5%) had abnormal fndings on a-CGH. 22q11.2 deletion syndrome was the most common (4/11, 36.4%). On the frst a-CGH, 4 patients were negative (4/81, 5%). Three of them were fnally diagnosed with Williams syndrome using fluorescent in situ hybridization (FISH), 1 patient was diagnosed with Noonan syndrome through exome sequencing. All of them exhibited difluse pulmonary artery branch hypoplasia, as well as increased velocity of blood flow, on repeated echocardiography. Five patients started rehabilitation therapy at mean 6 months old age in outpatient clinics and epilepsy was diagnosed in 2 patients. Parents of 2 patients (22q11.2 deletion syndrome and Patau syndrome) refused treatment due to the anticipated prognosis. Conclusions: Screening tests for genetic abnormalities using a-CGH in neonates with CHD has the advantage of early diagnosis of genetic abnormality during the neonatal period in which there is no obvious symptom of genetic abnormality. However, there are disadvantages that some genetic abnormalities cannot be identifed on a-CGH. AD - Y.H. Kim, Department of Pediatrics, Kyungpook National University Children's Hospital, Kyungpook National University School of Medicine, 130 Dongdeokro, Jung-gu, Daegu, South Korea AU - Choi, B. G. AU - Hwang, S. K. AU - Kwon, J. E. AU - Kim, Y. H. DB - Embase DO - 10.4070/kcj.2017.0166 IS - 3 KW - aortic coarctation artery dysplasia article blood flow velocity chromosome aberration chromosome deletion 22q11 comparative genomic hybridization congenital heart disease controlled study echocardiography epilepsy Fallot tetralogy fluorescence in situ hybridization genetic disorder heart ventricle septum defect human hypoplastic left heart syndrome major clinical study newborn Noonan syndrome outpatient department prognosis pulmonary artery pulmonary valve atresia rehabilitation care tertiary care center trisomy 13 whole exome sequencing Williams Beuren syndrome LA - English M3 - Article N1 - L621417664 2018-04-02 2018-04-12 PY - 2018 SN - 1738-5555 1738-5520 SP - 209-216 ST - Array comparative genomic hybridization as the first-line investigation for neonates with congenital heart disease: Experience in a single tertiary center T2 - Korean Circulation Journal TI - Array comparative genomic hybridization as the first-line investigation for neonates with congenital heart disease: Experience in a single tertiary center UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L621417664 http://dx.doi.org/10.4070/kcj.2017.0166 VL - 48 ID - 703 ER - TY - JOUR AB - The paper presents the findings on the prevalence rate and structure of arrhythmias and conduction disorder in children with chronic heart defect (CHD) and a functionally univentricular heart (FUH) at the stages of hemodynamic correction. The article also analyses factors determining the risk of arrhythmia development. Seventy patients with CHD and FUH who underwent all the stages of hemodynamic correction were examined. At each stage of surgical correction all children had an examination, including cardiac ultrasound, pulse oximetry, cardiac catheterization and angiopneumography, ECG, and 24-hour ECG monitoring before and after surgery, in order to reveal arrhythmia and conduction disorder. Absence of rhythm disturbances at the first stage of hemodynamic correction was 98.1%, at the second - 80%, at the third - 48.6% and at the fourth stage - 41.5%. Starting from the second stage of hemodynamic correction, the most frequent rhythm disturbance was dysfunction of the sinus node. Statistical analysis showed that there was a significant decrease in oxygen saturation in capillary blood (73 ± 12% and 81 ± 4%, respectively, p <0.05) and an increase in the average pressure in the pulmonary artery after total cavopulmonary connection (TCPC) (9.6 ± 4.2% and 11.5 ± 5.8%, respectively, p <0.05) in the group of children with heart rhythm disturbances as compared to children without arrhythmias. The average age of patients with arrhythmias in the group of patients having TCPC and, respectively, after the closure of fenestration, was statistically significantly higher than in the group of children without arrhythmias (72 ± 43 months and 52 ± 27 months, respectively, p <0.05). The study of arrhythmias in patients with a functionally univentricular heart requires prospective observation, since they significantly affect the prognosis of the disease and the quality of life. © 2018 Siberian State Medical University. All rights reserved. AD - Research Institute Cardiology, Tomsk National Research Medical Center (TNRMC), Russian Academy of Sciences (RAS), Department of Pediatric Cardiology, 111a, Kievskaya Str., Tomsk, 634012, Russian Federation Research and Clinical Institute for Pediatrics, Department of Pediatric Cardiology, 2, Taldomskaya Str., Moscow, 125412, Russian Federation AU - Plotnikova, I. V. AU - Dzhaffarova, O. Yu AU - Svintsova, L. I. AU - Saushkin, V. V. AU - Kovalev, I. A. DB - Scopus DO - 10.20538/1682-0363-2018-2-60-70 IS - 2 KW - Children Postoperative arrhythmias Stages of hemodynamic correction of congenital heart defect Univentricular heart M3 - Article N1 - Export Date: 15 June 2020 PY - 2018 SP - 60-70 ST - Arrhythmias in children with a single ventricle T2 - Bulletin of Siberian Medicine TI - Arrhythmias in children with a single ventricle UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85053029941&doi=10.20538%2f1682-0363-2018-2-60-70&partnerID=40&md5=2d9cc0dfb428f0a81c7764abdee0a01e VL - 17 ID - 1831 ER - TY - JOUR AB - While exercise stress testing (EST) is an important tool, little is known about its use for determining ischemia in patients with hypoplastic left heart syndrome (HLHS) and Fontan palliation. We sought to determine the frequency of ST segment changes during EST in HLHS patients after Fontan and examine results of further testing performed in response to ST changes. A single-center chart review of HLHS patients post-Fontan from January 1995 to December 2012 was performed. Data collected included demographics, indications for EST, resting electrocardiogram findings, EST and echocardiogram results and outcomes. ESTs were evaluated for ST segment changes concerning for ischemia. Results of additional testing performed based on concerning EST findings were collected. Twenty-seven patients underwent 64 ESTs (mean 2.4 ESTs/patient). Median age at first EST was 9.6 years (range 6.2-16.4). EST was concerning for ischemia in 13 patients (48 %) on 25 (39 %) ESTs. Based on EST results, two patients had stress sestamibi testing, two underwent coronary angiography, and one had both. No reversible perfusion defects or coronary artery obstructions were demonstrated. No patient who underwent EST has died. ST segment depression was not associated with ventricular dysfunction prior to EST or at the end of follow-up (p > 0.05). In patients with HLHS post-Fontan palliation, ST segment depression on EST is common. In patients who underwent further testing, no evidence of ischemia or coronary abnormalities was found. Additional testing may not be necessary in all patients. AD - Division of Cardiology, Department of Pediatrics, Texas Children's Hospital, Baylor College of Medicine, 6621 Fannin, MC# 19345-C, Houston, TX, 77030, USA. Department of Pediatrics, Arkansas Children's Hospital, University of Arkansas Medical School, Little Rock, AR, USA. Division of Cardiology, Department of Pediatrics, Texas Children's Hospital, Baylor College of Medicine, 6621 Fannin, MC# 19345-C, Houston, TX, 77030, USA. knlopez@bcm.edu. AN - 26725480 AU - Kyle, W. B. AU - Denfield, S. W. AU - Valdes, S. O. AU - Penny, D. J. AU - Bolin, E. H. AU - Lopez, K. N. DA - Mar DB - PubMed DO - 10.1007/s00246-015-1312-4 DP - NLM ET - 2016/01/05 IS - 3 KW - Adolescent Child *Coronary Angiography *Electrocardiography *Exercise Test Female Follow-Up Studies *Fontan Procedure Hospitals, Pediatric Humans Hypoplastic Left Heart Syndrome/*surgery Male Myocardial Ischemia/*diagnostic imaging Texas Congenital heart disease Exercise stress testing Fontan Hypoplastic left heart syndrome Ischemia ST segment abnormality LA - eng N1 - 1432-1971 Kyle, William Buck Denfield, Susan W Valdes, Santiago O Penny, Daniel J Bolin, Elijah H Lopez, Keila N Journal Article United States Pediatr Cardiol. 2016 Mar;37(3):545-51. doi: 10.1007/s00246-015-1312-4. Epub 2016 Jan 4. PY - 2016 SN - 0172-0643 SP - 545-51 ST - Assessing ST Segment Changes and Ischemia During Exercise Stress Testing in Patients with Hypoplastic Left Heart Syndrome and Fontan Palliation T2 - Pediatr Cardiol TI - Assessing ST Segment Changes and Ischemia During Exercise Stress Testing in Patients with Hypoplastic Left Heart Syndrome and Fontan Palliation VL - 37 ID - 139 ER - TY - JOUR AB - PURPOSE OF REVIEW: The purpose of this review is to describe the challenges associated with the diagnosis and treatment of children with borderline ventricles. A borderline ventricle is one in which there is concern that it will not be able to support its circulation. If a biventricular repair is attempted and fails, outcome is often poor. Thus, this early decision is important. RECENT FINDINGS: For the borderline right ventricle, options to add an additional source of pulmonary blood flow make the surgical strategy a bit more flexible than for patients with a borderline left ventricle. In general, outcome for a so-called one and one-half ventricle repair are generally good, though the long-term outcome and the effects of this physiology on lifelong exercise performance and quality of life remain to be seen. For the small left ventricle, often multiple surgeries are required to 'force' blood into the left ventricle and potentially help it grow. Though this strategy is successful in some, in others it results in significant residual cardiac issues including pulmonary hypertension. SUMMARY: Determining whether a patient will be better off in the long term with a marginal biventricular repair versus a Fontan circulation remains one of the most difficult problems in the field of pediatric cardiology and cardiac surgery. AD - Division of Cardiology, The Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, USA. AN - 29084001 AU - Cohen, M. S. DA - Jan DB - PubMed DO - 10.1097/hco.0000000000000466 DP - NLM ET - 2017/10/31 IS - 1 KW - Aorta, Thoracic/abnormalities/diagnostic imaging/physiopathology Aortic Valve Stenosis/diagnostic imaging/physiopathology/therapy Clinical Decision-Making Ebstein Anomaly/diagnostic imaging/physiopathology/therapy Fontan Procedure Heart Defects, Congenital/diagnostic imaging/physiopathology/*therapy Heart Septal Defects/diagnostic imaging/physiopathology/therapy Heart Valve Diseases/diagnostic imaging/physiopathology/therapy Heart Ventricles/abnormalities/diagnostic imaging/physiopathology/*surgery Humans Infant Infant, Newborn Mitral Valve Pulmonary Atresia/diagnostic imaging/physiopathology/therapy Ventricular Outflow Obstruction/diagnostic imaging/physiopathology/therapy LA - eng N1 - 1531-7080 Cohen, Meryl S Journal Article Review United States Curr Opin Cardiol. 2018 Jan;33(1):95-100. doi: 10.1097/HCO.0000000000000466. PY - 2018 SN - 0268-4705 SP - 95-100 ST - Assessing the borderline ventricle in a term infant: combining imaging and physiology to establish the right course T2 - Curr Opin Cardiol TI - Assessing the borderline ventricle in a term infant: combining imaging and physiology to establish the right course VL - 33 ID - 513 ER - TY - JOUR AB - Introduction: Anti-platelet therapies, like aspirin, are routinely used following many surgical repairs for congenital heart disease to prevent life-threatening thrombotic complications. Optimal dosing is not well studied and determination of real-time dose response to prophylactic aspirin is not routinely performed due to blood volume requirements and poor predictive value of existing platelet function assays (PFAs). Our objective was to study platelet function and thrombosis in post-operative cardiac patients under physiologic shear conditions using a novel collagen-coated microfluidic thrombosis assay (MTA) compared to a standard whole blood aggregometry (WBA) assay before and after clinically and experimentally administered aspirin therapy. Methods: We recruited congenital heart surgical patients with post-operative indications for aspirin therapy at the Children's Hospital of Michigan. Samples of 1.8 mL blood were drawn within 24 h of initiating aspirin (D0) and 24 to 48 h after initiating aspirin (D1). Blood samples were split for use in the MTA and the WBA. The MTA sample was run through collagen coated microfluidic channels at shear stress of 10 dyne/cm2 and pulse frequency of 1.67 Hz to simulate physiologic flow conditions. Each sample was run under untreated conditions (reflects the study subject's endogenous platelet function, D0, D1) and following exogenous aspirin administration (reflects maximum theoretical platelet response to aspirin, +eASA). The kinetics of thrombus formation was assessed as area percent of signal intensity (total fluorescent intensity, TFI) of thrombosis at 6 &10 min and as area under curve (AUC) for MTA and as AUC and impedance at 6 min for WBA. Results: Results from MTA and WBA on pre- and post-aspirin blood samples collected from 18 subjects, with and without exogenously added aspirin, were analyzed in this study. Fold changes for AUC were calculated for each test and were compared using Wilcoxon signed rank test; this showed no statistically significant difference. Three of the patients were found to have no response to endogenous or exogenous aspirin. Conclusions: We observed that measured platelet function is variable and potential sources of variability could be sample source, timing of sample from bypass, platelet count, transfusions and others. Standard aspirin doses might not be adequate for patients who are at high risk of thrombosis. In this study, we demonstrate the feasibility of employing microfluidic thrombosis assay to assess real time clinical platelet function in the cardiac ICU setting. Microfluidic assays provide real time assessment of platelet function under physiologic flow conditions and might be helpful with optimizing aspirin doses. Further studies on larger cohorts are required to validate this possibility. AD - Y. Kapileshwarkar, Children's Hospital Of Illinois, Peoria, IL, United States AU - Kapileshwarkar, Y. AU - Gelmini, L. AU - Tseng, Y. S. AU - Jackson, T. AU - Gao, X. AU - Richards, K. AU - Dokas, M. AU - Walters, H. AU - Hines, P. C. DB - Embase DO - 10.1016/j.ppedcard.2019.101191 KW - acetylsalicylic acid collagen ketorolac adolescent anticoagulant therapy aortic stenosis article blood aggregometry assay blood clotting blood examination blood sampling cardiac patient child clinical article controlled study coronary care unit drug indication Fallot tetralogy female heart single ventricle heart surgery human in vitro study infant male microfluidic analysis newborn pediatric patient platelet count postoperative thrombosis priority journal prospective study pulse rate shear rate shear stress thrombocyte activation thrombocyte function treatment response LA - English M3 - Article N1 - L2005025112 2020-02-27 2020-04-20 PY - 2020 SN - 1558-1519 1058-9813 ST - Assessment of antiplatelet therapy response in pediatric patients following cardiac surgery by microfluidic assay T2 - Progress in Pediatric Cardiology TI - Assessment of antiplatelet therapy response in pediatric patients following cardiac surgery by microfluidic assay UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2005025112 http://dx.doi.org/10.1016/j.ppedcard.2019.101191 VL - 56 ID - 563 ER - TY - JOUR AB - Background: Patients with functional single ventricles after the Fontan procedure have abnormal cardiac mechanics. The aims of this study were to determine factors that influence diastolic function and to describe associations of diastolic function with current clinical status.Methods: Echocardiograms were obtained as part of the Pediatric Heart Network Fontan Cross-Sectional Study. Diastolic function grade (DFG) was assessed as normal (grade 0), impaired relaxation (grade 1), pseudonymization (grade 2), or restrictive (grade 3). Studies were also classified dichotomously (restrictive pattern present or absent). Relationships between DFG and pre-Fontan variables (e.g., ventricular morphology, age at Fontan, history of volume-unloading surgery) and current status (e.g., systolic function, valvar regurgitation, exercise performance) were explored.Results: DFG was calculable in 326 of 546 subjects (60%) (mean age, 11.7 ± 3.3 years). Overall, 32% of patients had grade 0, 9% grade 1, 37% grade 2, and 22% grade 3 diastolic function. Although there was no association between ventricular morphology and DFG, there was an association between ventricular morphology and E', which was lowest in those with right ventricular morphology (P < .001); this association remained significant when using Z scores adjusted for age (P < .001). DFG was associated with achieving maximal effort on exercise testing (P = .004); the majority (64%) of those not achieving maximal effort had DFG 2 or 3. No additional significant associations of DFG with laboratory or clinical measures were identified.Conclusions: Assessment of diastolic function by current algorithms results in a high percentage of patients with abnormal DFG, but few clinically or statistically significant associations were found. This may imply a lack of impact of abnormal diastolic function on clinical outcomes in this cohort, or it may indicate that the methodology may not be applicable to pediatric patients with functional single ventricles. AD - Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts New England Research Institutes, Watertown, Massachusetts National Heart, Lung, and Blood Institute, Bethesda, Maryland Duke University Medical Center, Durham, North Carolina The Hospital for Sick Children, Toronto, Ontario, Canada The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania University of Utah, Salt Lake City, Utah Medical University of South Carolina, Charleston, South Carolina AN - 119595125. Corporate Author: Pediatric Heart Network Investigators. Language: English. Entry Date: 20171231. Revision Date: 20190515. Publication Type: journal article AU - Margossian, Renee AU - Sleeper, Lynn A. AU - Pearson, Gail D. AU - Barker, Piers C. AU - Mertens, Luc AU - Quartermain, Michael D. AU - Su, Jason T. AU - Shirali, Girish AU - Chen, Shan AU - Colan, Steven D. DB - ccm DO - 10.1016/j.echo.2016.07.016 DP - EBSCOhost IS - 11 KW - Heart Defects, Congenital -- Surgery Ventricular Dysfunction, Left Cardiopulmonary Bypass -- Statistics and Numerical Data Ventricular Dysfunction, Left -- Epidemiology Heart Defects, Congenital -- Epidemiology Stroke Volume Treatment Outcomes Risk Factors Postoperative Complications Incidence Heart Defects, Congenital Female North America Male Adolescence Echocardiography -- Methods Causal Attribution Human Ventricular Dysfunction, Left -- Prevention and Control Child Echocardiography -- Statistics and Numerical Data Validation Studies Comparative Studies Evaluation Research Multicenter Studies Funding Source N1 - research. Journal Subset: Allied Health; Biomedical; Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. Grant Information: KL2 TR001115/TR/NCATS NIH HHS/United States. NLM UID: 8801388. PMID: NLM27624592. PY - 2016 SN - 0894-7317 SP - 1066-1073 ST - Assessment of Diastolic Function in Single-Ventricle Patients After the Fontan Procedure T2 - Journal of the American Society of Echocardiography TI - Assessment of Diastolic Function in Single-Ventricle Patients After the Fontan Procedure UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=119595125&site=ehost-live&scope=site VL - 29 ID - 1475 ER - TY - JOUR AB - Objective: To test the hypothesis that surgeons are more directive than pediatricians in discussions with parents of critically ill children. Study Design: We designed a novel tool to assess physician directiveness in discussions with parents of children with hypoplastic left heart syndrome. We sent surveys containing the assessment tool as well as demographic questions to all attending physicians in Neonatology, Pediatric Critical Care, Pediatric Cardiology, and Congenital Cardiac Surgery at 14 of the largest pediatric cardiac surgery centers in the United States. Responses to the tool were used to determine a "directiveness score" for each physician. We then performed univariate and multivariable linear regression analyses to assess the association of physician specialty, race/ethnicity, religion, gender, and years in practice with the directiveness score. Results: Responses from 138 physicians were analyzed. In univariate analyses, with neonatologists as the reference group, surgeons were most directive (regression coefficient 3.02, 95% confidence interval 0.69 to 5.35, P=0.01), followed by cardiologists (regression coefficient 1.67, 95% confidence interval 0.13 to 3.20, P=0.03). No other variable was associated with physician directiveness score. These results were similar in the multivariable analysis. Conclusions: These results suggest that physician specialty is associated with directiveness. It is unclear, however, what amount of direction parents of critically ill children prefer. © 2004 Nature Publishing Group. All rights reserved. AD - A.A. Kon, Department of Pediatrics, University of California Davis, 2516 Stockton Blvd., Sacramento, CA 95817, United States AU - Kon, A. A. DB - Embase Medline DO - 10.1038/sj.jp.7211132 IS - 8 KW - article controlled study critical illness demography ethnology female gender human hypoplastic left heart syndrome linear regression analysis male medical practice parent parent counseling pediatrician physician attitude race religion scoring system surgeon United States LA - English M3 - Article N1 - L39149368 2004-09-12 PY - 2004 SN - 0743-8346 SP - 500-504 ST - Assessment of physician directiveness: Using hypoplastic left heart syndrome as a model T2 - Journal of Perinatology TI - Assessment of physician directiveness: Using hypoplastic left heart syndrome as a model UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L39149368 http://dx.doi.org/10.1038/sj.jp.7211132 VL - 24 ID - 1292 ER - TY - JOUR AB - OBJECTIVES: To assess self-reported quality of life (QOL) in a large multicenter cohort of adolescent and young adults surviving Fontan. STUDY DESIGN: Cross-sectional. The Pediatric Quality of Life Inventory (PedsQL) was administered to 408 survivors of Fontan ages 13-25 years enrolled in the Pediatric Heart Network Fontan Follow-up Study. Subjects also completed either the Child Health Questionnaire (age <19 years) or Short Form Health Survey (age ≥ 19 years). PedsQL data were compared with matched controls without a chronic health condition. Correlations between the measures were examined. RESULTS: Mean PedsQL scores for subjects receiving Fontan were significantly lower than those for the control group for physical and psychosocial QOL (P < .001). Overall, 45% of subjects receiving Fontan had scores in the clinically significant impaired range for physical QOL with 30% in the impaired range for psychosocial QOL. For each 1 year increase in age, the physical functioning score decreased by an average of 0.76 points (P = .004) and the emotional functioning score decreased by an average of 0.64 points (P = .03). Among subjects ≥19 years of age, the physical functioning score decreased by an average of 2 points for each year increase in age (P = .02). PedsQL scale scores were significantly correlated with conceptually related Child Health Questionnaire (P < .001) and Short Form Health Survey scores (P < .001). CONCLUSIONS: Survivors of Fontan are at risk for significantly impaired QOL which may decline with advancing age. Routine assessment of QOL is essential to inform interventions to improve health outcomes. The PedsQL allowed QOL assessment from pediatrics to young adulthood. TRIAL REGISTRATION: ClinicalTrials.gov: NCT00132782. AD - University of Michigan Mott Children's Hospital, Ann Arbor, MI. Electronic address: karenu@med.umich.edu. New England Research Institutes, Watertown, MA. University of Toronto Hospital for Sick Children, Toronto, Ontario, Canada. Texas A & M University, College Station, TX. Children's Hospital of Philadelphia, Philadelphia, PA. Boston Children's Hospital, Boston, MA. Duke University Medical Center, Durham, NC. Primary Children's Hospital, Salt Lake City, UT. National Heart, Lung, and Blood Institute, Bethesda, MD. Columbia University Medical Center, New York, NY. Medical University of South Carolina, Charleston, SC. AN - 26685073 AU - Uzark, K. AU - Zak, V. AU - Shrader, P. AU - McCrindle, B. W. AU - Radojewski, E. AU - Varni, J. W. AU - Daniels, K. AU - Handisides, J. AU - Hill, K. D. AU - Lambert, L. M. AU - Margossian, R. AU - Pemberton, V. L. AU - Lai, W. W. AU - Atz, A. M. C2 - PMC4769899 C6 - NIHMS737968 DA - Mar DB - PubMed DO - 10.1016/j.jpeds.2015.11.016 DP - NLM ET - 2015/12/20 KW - Adolescent Adult Age Factors Cross-Sectional Studies Female Follow-Up Studies Fontan Procedure/*psychology Humans Male *Quality of Life Surveys and Questionnaires Survivors United States/epidemiology Young Adult LA - eng N1 - 1097-6833 Uzark, Karen Zak, Victor Shrader, Peter McCrindle, Brian W Radojewski, Elizabeth Varni, James W Daniels, Kaitlyn Handisides, Jill Hill, Kevin D Lambert, Linda M Margossian, Renee Pemberton, Victoria L Lai, Wyman W Atz, Andrew M Pediatric Heart Network Investigators HL068288/HL/NHLBI NIH HHS/United States HL068285/HL/NHLBI NIH HHS/United States U01 HL068269/HL/NHLBI NIH HHS/United States U01 HL068279/HL/NHLBI NIH HHS/United States U10 HL109743/HL/NHLBI NIH HHS/United States U01 HL068290/HL/NHLBI NIH HHS/United States HL068279/HL/NHLBI NIH HHS/United States U01 HL068288/HL/NHLBI NIH HHS/United States U10 HL068270/HL/NHLBI NIH HHS/United States U01 HL068281/HL/NHLBI NIH HHS/United States U10 HL109737/HL/NHLBI NIH HHS/United States HL068281/HL/NHLBI NIH HHS/United States U01 HL068292/HL/NHLBI NIH HHS/United States HL068269/HL/NHLBI NIH HHS/United States HL068270/HL/NHLBI NIH HHS/United States HL068290/HL/NHLBI NIH HHS/United States U01 HL068285/HL/NHLBI NIH HHS/United States HL068292/HL/NHLBI NIH HHS/United States Journal Article Multicenter Study Observational Study Research Support, N.I.H., Extramural J Pediatr. 2016 Mar;170:166-72.e1. doi: 10.1016/j.jpeds.2015.11.016. Epub 2015 Dec 10. PY - 2016 SN - 0022-3476 (Print) 0022-3476 SP - 166-72.e1 ST - Assessment of Quality of Life in Young Patients with Single Ventricle after the Fontan Operation T2 - J Pediatr TI - Assessment of Quality of Life in Young Patients with Single Ventricle after the Fontan Operation VL - 170 ID - 88 ER - TY - JOUR AB - Objectives To assess self-reported quality of life (QOL) in a large multicenter cohort of adolescent and young adults surviving Fontan. Study design Cross-sectional. The Pediatric Quality of Life Inventory (PedsQL) was administered to 408 survivors of Fontan ages 13-25 years enrolled in the Pediatric Heart Network Fontan Follow-up Study. Subjects also completed either the Child Health Questionnaire (age <19 years) or Short Form Health Survey (age ≥19 years). PedsQL data were compared with matched controls without a chronic health condition. Correlations between the measures were examined. Results Mean PedsQL scores for subjects receiving Fontan were significantly lower than those for the control group for physical and psychosocial QOL (P <.001). Overall, 45% of subjects receiving Fontan had scores in the clinically significant impaired range for physical QOL with 30% in the impaired range for psychosocial QOL. For each 1 year increase in age, the physical functioning score decreased by an average of 0.76 points (P =.004) and the emotional functioning score decreased by an average of 0.64 points (P =.03). Among subjects ≥19 years of age, the physical functioning score decreased by an average of 2 points for each year increase in age (P =.02). PedsQL scale scores were significantly correlated with conceptually related Child Health Questionnaire (P <.001) and Short Form Health Survey scores (P <.001). Conclusions Survivors of Fontan are at risk for significantly impaired QOL which may decline with advancing age. Routine assessment of QOL is essential to inform interventions to improve health outcomes. The PedsQL allowed QOL assessment from pediatrics to young adulthood. Trial registration ClinicalTrials.gov: NCT00132782. © 2016 Elsevier Inc. AD - University of Michigan Mott Children's Hospital, 1500 E Medical Center Dr, Ann Arbor, MI 48109-5202, United States New England Research Institutes, Watertown, MA, United States University of Toronto Hospital for Sick Children, Toronto, ON, Canada Texas A and M University, College Station, TX, United States Children's Hospital of Philadelphia, Philadelphia, PA, United States Boston Children's Hospital, Boston, MA, United States Duke University Medical Center, Durham, NC, United States Primary Children's Hospital, Salt Lake City, UT, United States National Heart, Lung, and Blood Institute, Bethesda, MD, United States Columbia University Medical Center, New York, NY, United States Medical University of South Carolina, Charleston, SC, United States AU - Uzark, K. AU - Zak, V. AU - Shrader, P. AU - McCrindle, B. W. AU - Radojewski, E. AU - Varni, J. W. AU - Daniels, K. AU - Handisides, J. AU - Hill, K. D. AU - Lambert, L. M. AU - Margossian, R. AU - Pemberton, V. L. AU - Lai, W. W. AU - Atz, A. M. AU - Pediatric Heart Network, Investigators DB - Scopus DO - 10.1016/j.jpeds.2015.11.016 M3 - Article N1 - Cited By :44 Export Date: 15 June 2020 PY - 2016 SP - 166-172.e1 ST - Assessment of Quality of Life in Young Patients with Single Ventricle after the Fontan Operation T2 - Journal of Pediatrics TI - Assessment of Quality of Life in Young Patients with Single Ventricle after the Fontan Operation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84960154012&doi=10.1016%2fj.jpeds.2015.11.016&partnerID=40&md5=f01b501dd39a5ece38026b7105ed08b0 VL - 170 ID - 1916 ER - TY - JOUR AB - Objective To delineate risk factors for failure of extubation in the operating room among pediatric cardiac surgery patients. Design Prospective, observational study. Setting Single center, tertiary care, teaching hospital. Participants The study comprised 448 congenital cardiac surgery patients who were enrolled for intended extubation in the pediatric cardiac operating room over 5 years. Interventions The airways of enrolled patients were extubated in the operating room if predetermined suitability criteria were met. If the criteria were not met, patients were transferred to the intensive care unit with an endotracheal tube in situ. Patients whose airways were extubated successfully were followed up to determine specifically whether reintubation or use of noninvasive ventilation was necessary post-procedure. Measurements and Main Results The airways of 92% (412) patients were extubated in the operating room. Incidence of reintubation in the intensive care unit was 2.4%. There were 4 mortalities in the whole group. A 100% success rate for operating room extubation was achieved for patients in Risk Adjusted Congenital Heart Surgery category 1, and patients undergoing adult congenital cardiac disease surgery and redo sternotomy. The airways of 85% of patients with preoperative pulmonary hypertension were extubated in the operating room. Statistical analysis was applied to identify risk factors present in the group that made extubation in the operating room unachievable. Conclusions Extubation in the operating room was successful in a majority of patients undergoing cardiac surgery. Multivariate analysis identified weight<5 kg, age<1 year, cardiopulmonary bypass time>120 minutes, and presence of significant noncardiac structural anomalies as significant factors affecting extubation in the operating room, with an adjusted odds ratio (95% confidence interval) of 10 (2.7-37), 7.2 (2-22), 5.5 (1.7-17.7), and 3.3 (1.2-9.3), respectively. Pulmonary hypertension, redo sternotomy, higher Risk Adjusted Congenital Heart Surgery category, and aortic clamp time>60 minutes did not achieve significance in the multivariate analysis as risk factors for extubation in the operating room. AD - R.K. Joshi, Department of Pediatric Cardiac Sciences, Institute of Child Health & Dharamvira Heart Center, Sir Ganga Ram Hospital, New Delhi, India AU - Joshi, R. K. AU - Aggarwal, N. AU - Agarwal, M. AU - Dinand, V. AU - Joshi, R. DB - Embase Medline DO - 10.1053/j.jvca.2016.06.017 IS - 6 KW - aortopulmonary septal defect article child childhood mortality congenital heart disease cor triatriatum coronary artery anomaly Down syndrome endotracheal tube extubation female follow up Fontan procedure Glenn shunt heart atrium septum defect heart right ventricle double outlet heart right ventricle outflow tract obstruction heart surgery heart tumor heart ventricle septum defect human infant intensive care unit left coronary artery lung vein drainage anomaly major clinical study male noninvasive ventilation observational study operating room patent ductus arteriosus pediatric surgery priority journal prospective study pulmonary hypertension reoperation retreatment sternotomy surgical mortality surgical patient surgical risk vascular ring LA - English M3 - Article N1 - L613245725 2016-11-22 2016-12-12 PY - 2016 SN - 1532-8422 1053-0770 SP - 1530-1538 ST - Assessment of Risk Factors for a Sustainable “On-Table Extubation” Program in Pediatric Congenital Cardiac Surgery: 5-Year Experience T2 - Journal of Cardiothoracic and Vascular Anesthesia TI - Assessment of Risk Factors for a Sustainable “On-Table Extubation” Program in Pediatric Congenital Cardiac Surgery: 5-Year Experience UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L613245725 http://dx.doi.org/10.1053/j.jvca.2016.06.017 VL - 30 ID - 786 ER - TY - JOUR AB - To elucidate the ventricular contractile state and function in patients with univentricular heart, the ventricular volume, mass, ejection phase index, and wall stress were evaluated with biplane ventriculography and pressure measurement in 41 patients: 18 with left ventricular (LV) type (age, 6.4 +/- 6.1 years) and 23 with right ventricular (RV) type (age, 5.7 +/- 4.1 years), and data from patients with univentricular heart were compared with data from 19 normal control subjects (age, 7.2 +/- 4.3 years). Although the end-diastolic and end-systolic volumes were significantly greater in both types of univentricular heart than in the normal control group, the volumes for the LV and RV type patients did not differ from each other. The ejection fraction (EF) was depressed in both patient types of univentricular heart and was significantly (p less than 0.005) lower in the RV type than in the LV type patients (0.56 +/- 0.05 for LV type, 0.50 +/- 0.07 for RV type, and 0.64 +/- 0.03 for the control group). The ventricular mass was larger in both patient types of univentricular heart than in that of the control group, whereas the ratio of ventricular mass to end-diastolic volume was significantly (p less than 0.001) lower in the RV type patients than in the LV type patients and the control group (0.79 +/- 0.18 g/ml for LV type, 0.51 +/- 0.10 for RV type, and 0.82 +/- 0.13 for control group). End-systolic stress was significantly elevated in both types of univentricular heart (241 +/- 45 for LV type, 328 +/- 52 for RV type, and 205 +/- 26 kdynes/cm2 for the control group) and significantly (p less than 0.001) greater in the RV type than in the LV type patients. There was a significant inverse correlation (p less than 0.001) between end-systolic stress and the ratio of mass to end-diastolic volume in all the patients. In 27 patients (12 patients for LV type, 15 for RV type) the mean normalized systolic ejection rate corrected for heart rate (MNSERc) clearly fell below the 95% confidence limit of the normal end-systolic stress-MNSERc relation. The end-systolic stress:end-systolic volume ratio was also significantly depressed in both patient types of univentricular heart (3.49 +/- 1.77 for LV type, 4.07 +/- 2.13 for RV type, and 7.20 +/- 1.32 for the control group). In these variables, however, there were no significant differences between LV and RV type patients of univentricular heart.(ABSTRACT TRUNCATED AT 400 WORDS) AD - Department of Pediatrics, Osaka University Medical School, Japan. AN - 2720926 AU - Sano, T. AU - Ogawa, M. AU - Taniguchi, K. AU - Matsuda, H. AU - Nakajima, T. AU - Arisawa, J. AU - Shimazaki, Y. AU - Nakano, S. AU - Kawashima, Y. DA - Jun DB - PubMed DO - 10.1161/01.cir.79.6.1247 DP - NLM ET - 1989/06/01 IS - 6 KW - Angiocardiography Cardiac Catheterization Child Cineangiography Heart Defects, Congenital/*physiopathology Heart Ventricles/abnormalities Humans *Myocardial Contraction Stroke Volume LA - eng N1 - Sano, T Ogawa, M Taniguchi, K Matsuda, H Nakajima, T Arisawa, J Shimazaki, Y Nakano, S Kawashima, Y Comparative Study Journal Article United States Circulation. 1989 Jun;79(6):1247-56. doi: 10.1161/01.cir.79.6.1247. PY - 1989 SN - 0009-7322 (Print) 0009-7322 SP - 1247-56 ST - Assessment of ventricular contractile state and function in patients with univentricular heart T2 - Circulation TI - Assessment of ventricular contractile state and function in patients with univentricular heart VL - 79 ID - 36 ER - TY - JOUR AB - IMPORTANCE: Use of assisted reproductive technology (ART) has been associated with increased risks for birth defects. Variations in birth defect risks according to type of ART procedure have been noted, but findings are inconsistent. OBJECTIVES: To examine the prevalence of birth defects among liveborn infants conceived with and without ART and to evaluate risks associated with certain ART procedures among ART-conceived infants. DESIGN, SETTING, AND PARTICIPANTS: Used linked ART surveillance, birth certificates, and birth defects registry data for 3 states (Florida, Massachusetts, and Michigan). Methods for ascertaining birth defect cases varied by state. Resident live births during 2000 to 2010 were included, and the analysis was conducted between Feburary 2015 and August 2015. EXPOSURES: Use of ART among all live births and use of certain ART procedures among ART births. MAINOUTCOMEANDMEASURES: Prevalenceofselected chromosomal and nonchromosomal birth defects that are usually diagnosed at or immediately after birth. RESULTS: Of the 4618 076 liveborn infants between 2000 and 2010, 64861 (1.4%) were conceived using ART. Overall, the prevalence of 1 or more of the selected nonchromosomal defects was 58.59 per10000 for ART infants (n = 389) vs 47.50 per 10000 for non-ART infants (n = 22 036). The association remained significant after adjusting for maternal characteristics and year of birth (adjusted risk ratio [aRR], 1.28; 95% CI, 1.15-1.42). Similar differences were observed for singleton ART births vs their non-ART counterparts (63.69 per 10 000 [n = 218] vs 47.17 per 10 000 [n = 21 251]; aRR, 1.38; 95% CI, 1.21-1.59). Among multiple births, the prevalence of rectal and large intestinal atresia/stenosis was higher for ART births compared with non-ART births (aRR, 2.39; 95% CI, 1.38-4.12). Among ART births conceived after fresh embryo transfer, infants born to mothers with ovulation disorders had a higher prevalence of nonchromosomal birth defects (aRR, 1.53; 95% CI, 1.13-2.06) than those born to mothers without the diagnosis, and use of assisted hatching was associated with birth defects among singleton births (aRR, 1.55; 95% CI, 1.10-2.19). Multiplicity-adjusted P values for these associations were greater than.05. CONCLUSIONS AND RELEVANCE: Infants conceived after ART had a higher prevalence of certain birth defects. Assisted hatching and diagnosis of ovulation disorder were marginally associated with increased risks for nonchromosomal birth defects; however, these associations may be caused by other underlying factors. AD - S.L. Boulet, National Center for Chronic Disease Prevention and Health Promotion, Centers for Disease Control and Prevention, 4770 Buford Hwy, MS F-74, Atlanta, GA, United States AU - Boulet, S. L. AU - Kirby, R. S. AU - Reefhuis, J. AU - Zhang, Y. AU - Sunderam, S. AU - Cohen, B. AU - Bernson, D. AU - Copeland, G. AU - Bailey, M. A. AU - Jamieson, D. J. AU - Kissin, D. M. AU - Backus, K. AU - Mueller, L. AU - Stone, C. AU - Diop, H. AU - McKane, P. AU - Mersol-Barg, M. AU - Sappenfield, W. AU - Chang, J. AU - Crawford, S. AU - Kulkarni, A. DB - Embase Medline DO - 10.1001/jamapediatrics.2015.4934 IS - 6 KW - adult anencephalus anophthalmia article atrioventricular septal defect cleft lip cleft lip palate cleft palate congenital malformation controlled study diaphragm hernia disease association Down syndrome embryo transfer encephalocele esophagus atresia Fallot tetralogy female Florida gastroschisis great vessels transposition human hypoplastic left heart syndrome infant infant disease infertility therapy intestine atresia intestine stenosis limb deformity live birth major clinical study male Massachusetts Michigan microphthalmia mother omphalocele ovary insufficiency prevalence priority journal spinal dysraphism tracheoesophageal fistula trisomy 13 trisomy 18 trisomy 21 LA - English M3 - Article N1 - L610778594 2016-06-17 2019-10-31 PY - 2016 SN - 2168-6203 ST - Assisted reproductive technology and birth defects among liveborn infants in Florida, Massachusetts, and Michigan, 2000-2010 T2 - JAMA Pediatrics TI - Assisted reproductive technology and birth defects among liveborn infants in Florida, Massachusetts, and Michigan, 2000-2010 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L610778594 http://dx.doi.org/10.1001/jamapediatrics.2015.4934 VL - 170 ID - 807 ER - TY - JOUR AB - OBJECTIVE: To assess the association between early anthropometric measurements, device-assisted feeding, and early neurodevelopment in infants with complex congenital heart diseases (CHDs). STUDY DESIGN: Bayley Scales of Infant Development II were used to assess cognitive and motor skills in 72 infants with CHD at 6 and 12 months of age. Linear regression models were used to assess the association between mode of feeding and anthropometric measurements with neurodevelopment at 6 and 12 months of age. RESULTS: Of the 72 infants enrolled in the study, 34 (47%) had single-ventricle physiology. The mean Mental Developmental Index (MDI) and Psychomotor Developmental Index (PDI) scores at 6 months of age were 92 ± 10 and 81 ± 14, respectively. At 12 months of age, the mean MDI and PDI scores were 94 ± 12 and 80 ± 16, respectively. Lower length-for-age z score (P < .01) and head circumference-for-age z score (P < .05) were independently associated with lower MDI at 6 months, and both increased hospital length of stay (P < .01) and lower length-for-age z score (P = .04) were associated independently with lower MDI at 12 months. Device-assisted feeding at 3 months (P = .04) and lower length-for-age z score (P < .05) were independently associated with lower PDI at 6 months. Both lower weight-for-age z score (P = .04) and lower length-for-age z score (P = .04) were associated independently with PDI at 12 months. CONCLUSION: Neonates with complex CHD who required device-assisted feeding and those with lower weight and length and head circumference z scores at 3 months were at risk for neurodevelopmental delay at 6 and 12 months of age. AD - University of Pennsylvania School of Nursing, Philadelphia, PA; The Children's Hospital of Philadelphia, Philadelphia, PA. Electronic address: medoff@nursing.upenn.edu. University of Pennsylvania School of Nursing, Philadelphia, PA; The Children's Hospital of Philadelphia, Philadelphia, PA. University of Pennsylvania School of Nursing, Philadelphia, PA. The Children's Hospital of Philadelphia, Philadelphia, PA. University of Pennsylvania Perelman School of Medicine; Department of Gastroenterology, The Children's Hospital of Philadelphia, Philadelphia, PA. Northwestern University Feinberg School of Medicine; Department of Cardiology, Anne and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL. Department of Cardiology, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA. AN - 26585995 AU - Medoff-Cooper, B. AU - Irving, S. Y. AU - Hanlon, A. L. AU - Golfenshtein, N. AU - Radcliffe, J. AU - Stallings, V. A. AU - Marino, B. S. AU - Ravishankar, C. C2 - PMC4729590 C6 - NIHMS729667 DA - Feb DB - PubMed DO - 10.1016/j.jpeds.2015.10.017 DP - NLM ET - 2015/11/21 KW - *Child Development *Feeding Methods/instrumentation Female *Growth Heart Defects, Congenital/complications/*physiopathology Humans Infant Male Neurodevelopmental Disorders/etiology Prospective Studies LA - eng N1 - 1097-6833 Medoff-Cooper, Barbara Irving, Sharon Y Hanlon, Alexandra L Golfenshtein, Nadya Radcliffe, Jerilynn Stallings, Virginia A Marino, Bradley S Ravishankar, Chitra R01 NR002093/NR/NINR NIH HHS/United States UL1-RR-024134/RR/NCRR NIH HHS/United States M01 RR000240/RR/NCRR NIH HHS/United States MO1-RR00240/RR/NCRR NIH HHS/United States UL1 RR024134/RR/NCRR NIH HHS/United States Journal Article Research Support, N.I.H., Extramural J Pediatr. 2016 Feb;169:154-9.e1. doi: 10.1016/j.jpeds.2015.10.017. Epub 2015 Nov 13. PY - 2016 SN - 0022-3476 (Print) 0022-3476 SP - 154-9.e1 ST - The Association among Feeding Mode, Growth, and Developmental Outcomes in Infants with Complex Congenital Heart Disease at 6 and 12 Months of Age T2 - J Pediatr TI - The Association among Feeding Mode, Growth, and Developmental Outcomes in Infants with Complex Congenital Heart Disease at 6 and 12 Months of Age VL - 169 ID - 259 ER - TY - JOUR AB - Previous reports suggest that adverse events during childhood could be related to an array of psychiatric problems. Here, we question the relationship between childhood traumatic experiences and the sensory complexity of hallucinations in a cohort of 75 children and adolescents. We evidence a positive link between the number of sensory modalities involved in hallucinations and history of childhood trauma, even after controlling for the co-occurrence of suicidal ideation or the number of ICD-10 diagnoses. These findings support initiatives in which a routine exploration of traumatic events in childhood is performed when multimodal hallucinations are present. (PsycInfo Database Record (c) 2020 APA, all rights reserved) AD - Jardri, Renaud AN - 2020-01113-001 AU - Medjkane, François AU - Notredame, Charles-Edouard AU - Sharkey, Lucie AU - D'Hondt, Fabien AU - Vaiva, Guillaume AU - Jardri, Renaud DB - psyh DO - 10.1192/bjp.2019.266 DP - EBSCOhost IS - 3 KW - Hallucinations voice hearing childhood trauma multisensory phenomenology adolescents children early onset Mental Disorders Onset (Disorders) Trauma Childhood Adversity Adolescent Psychiatry Auditory Hallucinations Early Experience N1 - The Asylum Journal; The Asylum Journal of Mental Science. Partial author list: First Author & Affiliation: Medjkane, François; Centre Lille Neuroscience & Cognition, INSERM U1172, Univ. Lille, Lille, France. Other Publishers: Royal College of Psychiatrists. Release Date: 20200109. Correction Date: 20200604. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Hallucinations; Mental Disorders; Onset (Disorders); Trauma; Childhood Adversity. Minor Descriptor: Adolescent Psychiatry; Auditory Hallucinations; Early Experience. Classification: Psychological Disorders (3210). Population: Human (10); Male (30); Female (40). Location: France. Age Group: Childhood (birth-12 yrs) (100); Adolescence (13-17 yrs) (200); Adulthood (18 yrs & older) (300); Young Adulthood (18-29 yrs) (320). Tests & Measures: Multisensory Hallucinations Scale for Children. Methodology: Empirical Study; Quantitative Study. Page Count: 3. Issue Publication Date: Mar, 2020. Publication History: Accepted Date: Nov 22, 2019; Revised Date: Sep 27, 2019; First Submitted Date: Jul 25, 2019. Copyright Statement: This is an Open Access article, distributed under the terms of the Creative Commons Attribution licence (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted re-use, distribution, and reproduction in any medium, provided the original work is properly cited. The Author(s). 2020. PY - 2020 SN - 0007-1250 1472-1465 SP - 156-158 ST - Association between childhood trauma and multimodal early-onset hallucinations T2 - The British Journal of Psychiatry TI - Association between childhood trauma and multimodal early-onset hallucinations UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2020-01113-001&site=ehost-live&scope=site ORCID: 0000-0003-4596-1502 ORCID: 0000-0003-2462-008X ORCID: 0000-0001-5683-0490 renaud.jardri@chru-lille.fr VL - 216 ID - 1656 ER - TY - JOUR AB - OBJECTIVE: To investigate the association of fetal growth and cerebrovascular resistance at different periods in gestation with neurodevelopment (ND) at 14 months in the univentricular subject. METHODS: We reviewed serial prenatal ultrasound (US) examinations from 133 infants enrolled in the Pediatric Heart Network's Single Ventricle Reconstruction or Infants with Single Ventricle trials, including a subset of 82 infants in whom ND was assessed at 14 months using mental (MDI) and psychomotor (PDI) developmental indices. US examinations were assigned to one of four gestational time periods: (1) 20-23 weeks, (2) 24-29 weeks, (3) 30-33 weeks and (4) ≥ 34 weeks. Middle cerebral artery (MCA) flow velocity was measured and pulsatility index (PI), a measure of downstream resistance, was calculated. Data on fetal head circumference (HC), femur length, abdominal circumference (AC) and estimated fetal weight (EFW) were collected and their Z-scores were calculated. We evaluated the rate of change of these parameters over time within individuals, tested correlations between fetal growth and ND and assessed predictors of ND using linear regression. RESULTS: The mean prenatal HC Z-score was < 0 at each gestational-age period and became more negative later in pregnancy. There was less growth in HC from time period 3 to period 4 compared with from period 2 to 3 (Δ HC Z-score, -0.07 ± 0.1 vs 0.11 ± 0.22, P = 0.03). Though ND did not correlate with HC, HC Z-score or MCA-PI Z-score, HC growth from period 2 to period 3 correlated with MDI (r = 0.45, P = 0.047). AC Z-score in period 4 predicted MDI (β = 4.02, P = 0.04). EFW Z-score and AC Z-score in period 2 predicted PDI (β = 10.6, P = 0.04 and β = 3.29, P = 0.047, respectively). Lower MCA-PI at initial US predicted higher PDI (β = -14.7, P = 0.03). CONCLUSION: In univentricular fetuses, lower cerebrovascular resistance may be protective for ND. Decreased fetal somatic growth may predict developmental abnormalities. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd. AD - The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA. The Cardiac Center at the Children's Hospital of Philadelphia, Philadelphia, PA, USA. Department of Cardiology, Boston Children's Hospital, Boston, MA, USA. The Congenital Heart Center at C.S. Mott Children's Hospital, Ann Arbor, MI, USA. Labatt Family Heart Center, The Hospital for Sick Children, Toronto, ON, Canada. Department of Biostatistics, Mailman School of Public Health, Columbia University, New York, NY, USA. Division of Cardiology, Department of Pediatrics, Columbia University Medical Center, New York, NY, USA. AN - 25900850 AU - Hahn, E. AU - Szwast, A. AU - Cnota, J., 2nd AU - Levine, J. C. AU - Fifer, C. G. AU - Jaeggi, E. AU - Andrews, H. AU - Williams, I. A. DA - Apr DB - PubMed DO - 10.1002/uog.14881 DP - NLM ET - 2015/04/23 IS - 4 KW - Cerebrovascular Circulation/*physiology Female Fetal Development/*physiology Fetus/*physiopathology Gestational Age Heart Ventricles/*abnormalities/physiopathology Humans Male Middle Cerebral Artery/physiopathology Neurodevelopmental Disorders/*etiology/physiopathology Pregnancy Pulsatile Flow/physiology Retrospective Studies Ultrasonography, Prenatal/methods cerebral blood flow fetuses growth neurodevelopment single ventricle LA - eng N1 - 1469-0705 Hahn, E Szwast, A Cnota, J 2nd Levine, J C Fifer, C G Jaeggi, E Andrews, H Williams, I A HL068269/HL/NHLBI NIH HHS/United States HL068270/HL/NHLBI NIH HHS/United States HL068279/HL/NHLBI NIH HHS/United States HL068281/HL/NHLBI NIH HHS/United States HL068285/HL/NHLBI NIH HHS/United States HL068288/HL/NHLBI NIH HHS/United States HL068290/HL/NHLBI NIH HHS/United States HL068292/HL/NHLBI NIH HHS/United States HL085057/HL/NHLBI NIH HHS/United States Journal Article Research Support, N.I.H., Extramural England Ultrasound Obstet Gynecol. 2016 Apr;47(4):460-5. doi: 10.1002/uog.14881. Epub 2016 Feb 18. PY - 2016 SN - 0960-7692 SP - 460-5 ST - Association between fetal growth, cerebral blood flow and neurodevelopmental outcome in univentricular fetuses T2 - Ultrasound Obstet Gynecol TI - Association between fetal growth, cerebral blood flow and neurodevelopmental outcome in univentricular fetuses VL - 47 ID - 519 ER - TY - JOUR AB - OBJECTIVES: Technical Performance Score (TPS) has been shown to have a strong association with early and late outcomes after congenital cardiac surgery, with greater morbidity and reintervention in children with major residual lesions (TPS class 3). We sought to explore the effect of TPS on the neurodevelopmental outcomes. METHODS: All infants undergoing cardiac surgery, excluding those with trisomy 21, were offered neurodevelopmental testing at 1 year of age using the Bayley Scales of Infant Development, 3rd edition. TPSs from the discharge echocardiograms were graded as class 1 (optimal), class 2 (minor residual), or class 3 (major residual). Multivariate regression analysis was performed using patient characteristics and preoperative variables. RESULTS: Neurodevelopmental testing was performed in 140 patients at a median age of 16 months. Of these, 28 (20%) had single ventricle palliation; 39 (28%) were in Risk Adjustment for Congenital Heart Surgery category 4 to 6. Significant differences between the groups were found in the cognitive (P = .01) and motor (P = .05) domains, with subjects in TPS class 3 having significantly lower cognitive and motor composite scores. The scores did not vary significantly according to single ventricle versus biventricular repair or Risk Adjustment for Congenital Heart Surgery categorization. In multivariate modeling, class 3 TPS remained significantly associated with a lower Bayley cognitive score (P = .02), with a trend toward a lower Bayley motor score (P = .08). CONCLUSIONS: We found that TPS is an independent predictor of neurodevelopmental outcomes after infant heart surgery. Future research should explore whether a structured program of intraoperative recognition and intervention on residual lesions can improve the TPS and neurodevelopmental outcomes. AD - Department of Cardiac Surgery, Boston Children's Hospital and Harvard Medical School, Boston, Mass. Electronic address: meena.nathan@cardio.chboston.org. Cardiac Neurodevelopmental Program, Boston Children's Hospital and Harvard Medical School, Boston, Mass. Department of Cardiology, Boston Children's Hospital and Harvard Medical School, Boston, Mass. Medicine Critical Care Program, Boston Children's Hospital and Harvard Medical School, Boston, Mass. Cardiac Neurodevelopmental Program, Boston Children's Hospital and Harvard Medical School, Boston, Mass; Department of Cardiology, Boston Children's Hospital and Harvard Medical School, Boston, Mass. Department of Cardiac Surgery, Boston Children's Hospital and Harvard Medical School, Boston, Mass; Cardiac Neurodevelopmental Program, Boston Children's Hospital and Harvard Medical School, Boston, Mass. AN - 24084277 AU - Nathan, M. AU - Sadhwani, A. AU - Gauvreau, K. AU - Agus, M. AU - Ware, J. AU - Newburger, J. W. AU - Pigula, F. DA - Jul DB - PubMed DO - 10.1016/j.jtcvs.2013.08.032 DP - NLM ET - 2013/10/03 IS - 1 KW - Age Factors Cardiac Surgical Procedures/*adverse effects *Child Development Cognition Developmental Disabilities/diagnosis/*etiology/physiopathology/psychology Heart Defects, Congenital/diagnosis/*surgery Humans Infant Infant Behavior Infant, Newborn Linear Models Male Motor Activity Multivariate Analysis Nervous System/*growth & development *Neuropsychological Tests Predictive Value of Tests Retrospective Studies Risk Factors LA - eng N1 - 1097-685x Nathan, Meena Sadhwani, Anjali Gauvreau, Kimberlee Agus, Michael Ware, Janice Newburger, Jane W Pigula, Frank Journal Article United States J Thorac Cardiovasc Surg. 2014 Jul;148(1):232-237.e3. doi: 10.1016/j.jtcvs.2013.08.032. Epub 2013 Sep 29. PY - 2014 SN - 0022-5223 SP - 232-237.e3 ST - Association between Technical Performance Scores and neurodevelopmental outcomes after congenital cardiac surgery T2 - J Thorac Cardiovasc Surg TI - Association between Technical Performance Scores and neurodevelopmental outcomes after congenital cardiac surgery VL - 148 ID - 431 ER - TY - JOUR AB - A heart specimen is presented which showed a rare combination of atrioventricular valve atresia with single ventricle, truncus arteriosus communis with an anteriorly placed trunk, ie transposition. Because of the problems involved in the classification of this heart, the types of single ventricle are revised with particular attention to the rare cases of atrioventricular valve atresia and single ventricle reported in the literature. Also the rare type of truncus arteriosus communis with truncoatrioventricular valve discontinuity is excluded from the so-called transposition complexes, and properly classified as a form of truncus. The literal meaning of transposition is stressed and it is urged to approach congenital heart defects in a purely anatomic sense with definition of each segment as accurately as possible. Thus the present confusion in terminology and further plethora of new terms are avoided. AN - 691270 AU - Shakibi, J. G. AU - Aryanpur, I. AU - Nazarian, I. AU - Siassi, B. DA - May DB - PubMed DO - 10.1536/ihj.19.346 DP - NLM ET - 1978/05/01 IS - 3 KW - Child Heart Defects, Congenital Heart Ventricles/*abnormalities/pathology Humans Male Mitral Valve/*abnormalities/pathology Terminology as Topic Transposition of Great Vessels/*complications/pathology Truncus Arteriosus/pathology Truncus Arteriosus, Persistent/*complications LA - eng N1 - Shakibi, J G Aryanpur, I Nazarian, I Siassi, B Case Reports Journal Article Japan Jpn Heart J. 1978 May;19(3):346-57. doi: 10.1536/ihj.19.346. PY - 1978 SN - 0021-4868 (Print) 0021-4868 SP - 346-57 ST - Association of atrioventricular valve atresia with single ventricle, truncus arteriosus communis and transposition. A basic reorientation in the approach to the definition of congenital heart defects T2 - Jpn Heart J TI - Association of atrioventricular valve atresia with single ventricle, truncus arteriosus communis and transposition. A basic reorientation in the approach to the definition of congenital heart defects VL - 19 ID - 461 ER - TY - JOUR AB - OBJECTIVE: Previous epidemiological studies have evaluated cases with all congenital heart defects (CHDs), rather than analysing different types of CHD. The objective of this study was to evaluate the possible association of certain chronic maternal diseases with the risk of different types of CHD, because the role of possible environmental factors in the origin of CHDs is unclear in the vast majority of patients. STUDY DESIGN: Different types of CHD, diagnosed after lethal outcome (autopsy report) or after surgical intervention (catheter or correction), were evaluated in order to estimate the possible role of chronic maternal diseases in their origin. This analysis was based on the rates of medically recorded chronic maternal diseases in 3562 live-born cases with CHDs, 38,151 population controls without any birth defects, and 16,602 malformed controls with other isolated congenital abnormalities, using the data set of the population-based Hungarian Case-Control Surveillance of Congenital Abnormalities (1980-1996). RESULTS: Maternal epilepsy treated with carbamazepine and migraine were found to be associated with higher risk of ventricular septal defect; panic disorders were associated with higher risk of hypoplastic left heart; type I diabetes mellitus was associated with higher risk of coarctation of the aorta; chronic hypertension was associated with higher risk of ventricular septal defect, common atrioventricular canal and common truncus; and paroxysmal supraventricular tachycardia was associated with higher risk of atrial septal defect secundum, common atrioventricular canal and ventricular septal defect. CONCLUSION: In conclusion, certain chronic maternal diseases were found to be associated with higher risk of specific CHDs. Appropriate treatment of these diseases may help to prevent these CHDs. AD - Versys Clinics, Human Reproduction Institute, Budapest, Hungary. Electronic address: attila.vereczkey@versysclinics.com. Alfréd Rényi Institute of Mathematics, Hungarian Academy of Sciences, Budapest, Hungary. Foundation for the Community Control of Hereditary Diseases, Budapest, Hungary. Department of Obstetrics and Gynaecology, University of Pécs, Pécs, Hungary. AN - 25216447 AU - Vereczkey, A. AU - Gerencsér, B. AU - Czeizel, A. E. AU - Szabó, I. DA - Nov DB - PubMed DO - 10.1016/j.ejogrb.2014.08.022 DP - NLM ET - 2014/09/13 KW - Adult Anticonvulsants/therapeutic use Aortic Coarctation/epidemiology Carbamazepine/therapeutic use Case-Control Studies Chronic Disease Diabetes Mellitus, Type 1/epidemiology Epilepsy/drug therapy/epidemiology Female Heart Defects, Congenital/*epidemiology Heart Septal Defects Heart Septal Defects, Atrial/epidemiology Heart Septal Defects, Ventricular/epidemiology Humans Hungary/epidemiology Hypertension/epidemiology Hypoplastic Left Heart Syndrome/epidemiology Migraine Disorders/epidemiology Mitral Valve Insufficiency/epidemiology Panic Disorder/epidemiology Pregnancy Pregnancy Complications/*epidemiology Risk Factors Tachycardia, Paroxysmal/epidemiology Tachycardia, Supraventricular/epidemiology Young Adult Congenital heart defects Diabetes mellitus Epilepsy Migraine Population-based case–control study LA - eng N1 - 1872-7654 Vereczkey, A Gerencsér, B Czeizel, A E Szabó, I Journal Article Research Support, Non-U.S. Gov't Ireland Eur J Obstet Gynecol Reprod Biol. 2014 Nov;182:1-6. doi: 10.1016/j.ejogrb.2014.08.022. Epub 2014 Aug 27. PY - 2014 SN - 0301-2115 SP - 1-6 ST - Association of certain chronic maternal diseases with the risk of specific congenital heart defects: a population-based study T2 - Eur J Obstet Gynecol Reprod Biol TI - Association of certain chronic maternal diseases with the risk of specific congenital heart defects: a population-based study VL - 182 ID - 314 ER - TY - JOUR AB - BACKGROUND: Obesity is associated with increased lifelong morbidity and reduced life span and is increasingly prevalent in the congenital heart disease population. Habitual exercise is an important aspect of a healthy lifestyle and primary prevention of obesity in the general population. The association between habitual activity and body mass index (BMI) has not been studied in children with congenital heart disease. METHODS: A cross-sectional analysis of two previously collected cohorts was performed, including participants 8 to 18 years old with tetralogy of Fallot, transposition of the great arteries, and single ventricle heart disease after a Fontan operation. The association between BMI and duration of habitual exercise (measured by questionnaire) was studied. Secondary analyses assessing the effect of other possible factors for BMI were performed. RESULTS: In total, 172 participants were studied (45% Tetralogy of Fallot, 12% transposition of the great arteries, and 43% Fontan). Median BMI was 18.2, and 29% of the participants were obese or overweight. Median habitual exercise was 5.9 h/wk. Thirty-eight percent of participants reported having their activity restricted by their cardiologist. Increasing exercise duration was associated with lower BMI ( P = .01) in univariate analysis. In secondary analyses, restriction to mild exertion and participation in low-intensity exercise were both associated with increased BMI. CONCLUSION: Increased habitual activity was associated with lower BMI, emphasizing the potential role of recreational sport in the health of children with congenital heart disease. AD - 1 Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA. 2 Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA. 3 Center for Pediatric Clinical Effectiveness, Children's Hospital of Philadelphia, Philadelphia, PA, USA. 4 Leonard Davis Institute, University of Pennsylvania, Philadelphia, PA, USA. AN - 29544424 AU - O'Byrne, M. L. AU - McBride, M. G. AU - Paridon, S. AU - Goldmuntz, E. C2 - PMC6154798 C6 - NIHMS987085 DA - Mar DB - PubMed DO - 10.1177/2150135117752122 DP - NLM ET - 2018/03/17 IS - 2 KW - Adolescent *Body Mass Index Child Cross-Sectional Studies *Exercise Female *Fontan Procedure Health Behavior Humans Male Pediatric Obesity/diagnosis/*etiology/psychology Postoperative Complications/diagnosis/*etiology/psychology Retrospective Studies Risk Factors Surveys and Questionnaires Tetralogy of Fallot/*surgery Transposition of Great Vessels/*surgery *exercise physiology *hypoplastic left heart syndrome *outcomes research *pediatric cardiology *transposition of the great arteries LA - eng N1 - 2150-136x O'Byrne, Michael L McBride, Michael G Paridon, Stephen Goldmuntz, Elizabeth K23 HL130420/HL/NHLBI NIH HHS/United States P50 HL074731/HL/NHLBI NIH HHS/United States T32 HL007915/HL/NHLBI NIH HHS/United States UL1 TR000003/TR/NCATS NIH HHS/United States Journal Article Research Support, N.I.H., Extramural World J Pediatr Congenit Heart Surg. 2018 Mar;9(2):177-184. doi: 10.1177/2150135117752122. PY - 2018 SN - 2150-1351 (Print) 2150-1351 SP - 177-184 ST - Association of Habitual Activity and Body Mass Index in Survivors of Congenital Heart Surgery: A Study of Children and Adolescents With Tetralogy of Fallot, Transposition of the Great Arteries, and Fontan Palliation T2 - World J Pediatr Congenit Heart Surg TI - Association of Habitual Activity and Body Mass Index in Survivors of Congenital Heart Surgery: A Study of Children and Adolescents With Tetralogy of Fallot, Transposition of the Great Arteries, and Fontan Palliation VL - 9 ID - 73 ER - TY - JOUR AB - OBJECTIVES: To describe neurodevelopmental outcomes in infants with single ventricle (SV) physiology and determine factors associated with worse outcomes. STUDY DESIGN: Neurodevelopmental outcomes for infants with SV enrolled in a multicenter drug trial were assessed at 14 months of age using the Bayley Scales of Infant Development-II. Multivariable regression analysis was used to identify factors associated with worse outcomes. RESULTS: Neurodevelopmental testing was performed at 14 ± 1 months in 170/185 subjects in the trial. Hypoplastic left heart syndrome was present in 59% and 75% had undergone the Norwood operation. Mean Psychomotor Developmental Index (PDI) and mental developmental index (MDI) were 80 ± 18 and 96 ± 14, respectively, (normal 100 ± 15, P < .001 for each). Group-based trajectory analysis provided a 2-group model ("high" and "low") for height z-score trajectory and brain type natriuretic peptide (BNP) trajectory. The predicted PDI scores were 15 points higher in the "high" height z-score trajectory compared with the "low" cluster (P < .001). A higher number of serious adverse events during the trial was associated with lower PDI scores (P = .02). The predicted MDI scores were 13-17 points lower in "low height trajectory-high BNP trajectory" group compared with the other 3 groups (P < .001). MDI scores were also lower in subjects who required extracorporeal membrane oxygenation during the neonatal hospitalization (P = .01) or supplemental oxygen at discharge (P = .01). CONCLUSIONS: Neurodevelopmental outcome at 14 months of age is impaired in infants with SV physiology. Low height trajectory and high BNP trajectory were associated with worse neurodevelopmental outcomes. Efforts to improve nutritional status alone may not improve neurodevelopmental outcomes. AD - Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA. Ravishankar@email.chop.edu AN - 22939929 AU - Ravishankar, C. AU - Zak, V. AU - Williams, I. A. AU - Bellinger, D. C. AU - Gaynor, J. W. AU - Ghanayem, N. S. AU - Krawczeski, C. D. AU - Licht, D. J. AU - Mahony, L. AU - Newburger, J. W. AU - Pemberton, V. L. AU - Williams, R. V. AU - Sananes, R. AU - Cook, A. L. AU - Atz, T. AU - Khaikin, S. AU - Hsu, D. T. C2 - PMC3547153 C6 - NIHMS404926 NCT00113087 DA - Feb DB - PubMed DO - 10.1016/j.jpeds.2012.07.048 DP - NLM ET - 2012/09/04 IS - 2 KW - *Child Development Developmental Disabilities/drug therapy/epidemiology/*etiology/*physiopathology Double-Blind Method Female *Growth Heart Ventricles/*abnormalities Humans Infant Male Prospective Studies Treatment Outcome LA - eng N1 - 1097-6833 Ravishankar, Chitra Zak, Victor Williams, Ismee A Bellinger, David C Gaynor, J William Ghanayem, Nancy S Krawczeski, Catherine D Licht, Daniel J Mahony, Lynn Newburger, Jane W Pemberton, Victoria L Williams, Richard V Sananes, Renee Cook, Amanda L Atz, Teresa Khaikin, Svetlana Hsu, Daphne T Pediatric Heart Network Investigators HL068288/HL/NHLBI NIH HHS/United States HL068285/HL/NHLBI NIH HHS/United States U01 HL068269/HL/NHLBI NIH HHS/United States U10 HL109816/HL/NHLBI NIH HHS/United States P30 HD018655/HD/NICHD NIH HHS/United States U01 HL068279/HL/NHLBI NIH HHS/United States U01 HL068290/HL/NHLBI NIH HHS/United States HL068279/HL/NHLBI NIH HHS/United States U01 HL068288/HL/NHLBI NIH HHS/United States U10 HL068270/HL/NHLBI NIH HHS/United States U01 HL068281/HL/NHLBI NIH HHS/United States U01 HL068270/HL/NHLBI NIH HHS/United States HL085057/HL/NHLBI NIH HHS/United States UL1 TR000055/TR/NCATS NIH HHS/United States HL068281/HL/NHLBI NIH HHS/United States U01 HL068292/HL/NHLBI NIH HHS/United States HL068269/HL/NHLBI NIH HHS/United States HL068270/HL/NHLBI NIH HHS/United States U01 HL085057/HL/NHLBI NIH HHS/United States HL068290/HL/NHLBI NIH HHS/United States U01 HL068285/HL/NHLBI NIH HHS/United States HL068292/HL/NHLBI NIH HHS/United States Journal Article Multicenter Study Randomized Controlled Trial Research Support, N.I.H., Extramural Research Support, U.S. Gov't, P.H.S. J Pediatr. 2013 Feb;162(2):250-6.e2. doi: 10.1016/j.jpeds.2012.07.048. Epub 2012 Aug 30. PY - 2013 SN - 0022-3476 (Print) 0022-3476 SP - 250-6.e2 ST - Association of impaired linear growth and worse neurodevelopmental outcome in infants with single ventricle physiology: a report from the pediatric heart network infant single ventricle trial T2 - J Pediatr TI - Association of impaired linear growth and worse neurodevelopmental outcome in infants with single ventricle physiology: a report from the pediatric heart network infant single ventricle trial VL - 162 ID - 328 ER - TY - JOUR AB - Objectives: To describe neurodevelopmental outcomes in infants with single ventricle (SV) physiology and determine factors associated with worse outcomes.Study Design: Neurodevelopmental outcomes for infants with SV enrolled in a multicenter drug trial were assessed at 14 months of age using the Bayley Scales of Infant Development-II. Multivariable regression analysis was used to identify factors associated with worse outcomes.Results: Neurodevelopmental testing was performed at 14 ± 1 months in 170/185 subjects in the trial. Hypoplastic left heart syndrome was present in 59% and 75% had undergone the Norwood operation. Mean Psychomotor Developmental Index (PDI) and mental developmental index (MDI) were 80 ± 18 and 96 ± 14, respectively, (normal 100 ± 15, P < .001 for each). Group-based trajectory analysis provided a 2-group model ("high" and "low") for height z-score trajectory and brain type natriuretic peptide (BNP) trajectory. The predicted PDI scores were 15 points higher in the "high" height z-score trajectory compared with the "low" cluster (P < .001). A higher number of serious adverse events during the trial was associated with lower PDI scores (P = .02). The predicted MDI scores were 13-17 points lower in "low height trajectory-high BNP trajectory" group compared with the other 3 groups (P < .001). MDI scores were also lower in subjects who required extracorporeal membrane oxygenation during the neonatal hospitalization (P = .01) or supplemental oxygen at discharge (P = .01).Conclusions: Neurodevelopmental outcome at 14 months of age is impaired in infants with SV physiology. Low height trajectory and high BNP trajectory were associated with worse neurodevelopmental outcomes. Efforts to improve nutritional status alone may not improve neurodevelopmental outcomes. AD - Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA AN - 104294524. Corporate Author: Pediatric Heart Network Investigators. Language: English. Entry Date: 20130322. Revision Date: 20180806. Publication Type: journal article AU - Ravishankar, C. AU - Zak, V. AU - Williams, I. A. AU - Bellinger, D. C. AU - Gaynor, J. W. AU - Ghanayem, N. S. AU - Krawczeski, C. D. AU - Licht, D. J. AU - Mahony, L. AU - Newburger, J. W. AU - Pemberton, V. L. AU - Williams, R. V. AU - Sananes, R. AU - Cook, A. L. AU - Atz, T. AU - Khaikin, S. AU - Hsu, D. T. AU - Ravishankar, Chitra AU - Zak, Victor AU - Williams, Ismee A. DB - ccm DO - 10.1016/j.jpeds.2012.07.048 DP - EBSCOhost IS - 2 KW - Child Development Developmental Disabilities -- Etiology Developmental Disabilities -- Physiopathology Growth Heart Ventricle -- Abnormalities Developmental Disabilities -- Drug Therapy Developmental Disabilities -- Epidemiology Double-Blind Studies Female Human Infant Male Prospective Studies Scales Treatment Outcomes Funding Source Neuropsychological Tests N1 - research. Journal Subset: Biomedical; Peer Reviewed; USA. Special Interest: Pediatric Care. Instrumentation: Bayley Scales of Infant Development-Second Edition (BSID-II); Psychomotor Developmental Index (PDI). Grant Information: HL068269/HL/NHLBI NIH HHS/United States. NLM UID: 0375410. PMID: NLM22939929. PY - 2013 SN - 0022-3476 SP - 250-256.e2 ST - Association of impaired linear growth and worse neurodevelopmental outcome in infants with single ventricle physiology: a report from the pediatric heart network infant single ventricle trial T2 - Journal of Pediatrics TI - Association of impaired linear growth and worse neurodevelopmental outcome in infants with single ventricle physiology: a report from the pediatric heart network infant single ventricle trial UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104294524&site=ehost-live&scope=site VL - 162 ID - 1635 ER - TY - JOUR AB - Background-Daily home monitoring of oxygen saturation and weight has been reported to improve outcomes for patients with single-ventricle heart disease during the period between stage I palliation and stage II palliation. However, these studies have been limited to single institutions and used historical control subjects. Our objective was to determine the association of various interstage home monitoring strategies with outcomes using a multicenter cohort with contemporary control subjects. Methods and Results-We performed a retrospective cohort study using prospectively collected data from the National Pediatric Cardiology Quality Improvement Collaborative from 2008 to 2012. We compared interstage mortality, unscheduled readmissions, and change in weight-for-age Z score for various home monitoring strategies of oxygen saturation (n=494) or weight (n=472), adjusting for sex, syndrome, tricuspid regurgitation, arch obstruction, and shunt type. Overall interstage mortality was 8.1%, and 47% had ≥1 unscheduled readmission. We did not find any associations of home oxygen saturation or weight monitoring with mortality or readmission. Although there was no difference in weight-for-age Z score for daily (0.33±0.12) versus weekly (0.34±0.18, P=0.98) weight monitoring, daily home weight monitoring was superior to no home weight monitoring (-0.15±0.18; P<0.01). Conclusions-Home weight monitoring is associated with improved weight gain during the interstage period, but we did not find any benefits in other clinical outcomes for either home oxygen saturation monitoring or home weight monitoring. AD - M.E. Oster, Emory University, School of Medicine, 2835 Brandywine Rd, Atlanta, GA, United States AU - Oster, M. E. AU - Ehrlich, A. AU - King, E. AU - Petit, C. J. AU - Clabby, M. AU - Smith, S. AU - Glanville, M. AU - Anderson, J. AU - Darbie, L. AU - Beekman, R. H. DB - Embase Medline DO - 10.1161/CIRCULATIONAHA.114.014107 IS - 6 KW - aortic arch anomaly article Blalock Taussig shunt cardiovascular mortality cohort analysis controlled study echocardiography female heart ventricle function home monitoring hospital readmission human interstage home monitoring major clinical study male multicenter study outcome assessment oxygen saturation priority journal prospective study quality of life retrospective study sex tricuspid valve regurgitation body weight gain LA - English M3 - Article N1 - L605657747 2015-08-24 2015-08-25 PY - 2015 SN - 1524-4539 0009-7322 SP - 502-508 ST - Association of Interstage Home Monitoring with Mortality, Readmissions, and Weight Gain: A Multicenter Study from the National Pediatric Cardiology Quality Improvement Collaborative T2 - Circulation TI - Association of Interstage Home Monitoring with Mortality, Readmissions, and Weight Gain: A Multicenter Study from the National Pediatric Cardiology Quality Improvement Collaborative UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L605657747 http://dx.doi.org/10.1161/CIRCULATIONAHA.114.014107 VL - 132 ID - 891 ER - TY - JOUR AB - IMPORTANCE: Prenatal maternal psychological distress can result in detrimental mother and child outcomes. Maternal stress increases with receipt of a prenatal diagnosis of fetal congenital heart disease (CHD); however, the association between maternal stress and the developing brain in fetuses with CHD is unknown. OBJECTIVE: To determine the association of maternal psychological distress with brain development in fetuses with CHD. DESIGN, SETTING, AND PARTICIPANTS: This longitudinal, prospective, case-control study consecutively recruited 48 pregnant women carrying fetuses with CHD and 92 healthy volunteers with low-risk pregnancies from the Children's National Health System between January 2016 and September 2018. Data were analyzed between January 2016 and June 2019. EXPOSURES: Fetal CHD and maternal stress, anxiety, and depression. MAIN OUTCOMES AND MEASURES: Maternal stress, anxiety, and depression were measured using the Perceived Stress Scale, Spielberger State-Trait Anxiety Inventory, and Edinburgh Postnatal Depression Scale, respectively. Volumes of fetal total brain, cerebrum, left and right hippocampus, cerebellum, and brainstem were determined from 3-dimensionally reconstructed T2-weighted magnetic resonance imaging (MRI) scans. RESULTS: This study included 223 MRI scans from 140 fetuses (74 MRIs from 48 fetuses with CHD and 149 MRIs from 92 healthy fetuses) between 21 and 40 weeks' gestation. Among 48 women carrying fetuses with CHD, 31 (65%) tested positive for stress, 21 (44%) for anxiety, and 14 (29%) for depression. Among 92 pregnant women carrying healthy fetuses, 25 (27%) tested positive for stress, 24 (26%) for anxiety, and 8 (9%) for depression. Depression scores were higher among 17 women carrying fetuses with single-ventricle CHD vs 31 women carrying fetuses with 2-ventricle CHD (3.8; 95% CI, 0.3 to 7.3). Maternal stress and anxiety were associated with smaller left hippocampal (stress: -0.003 cm3; 95% CI, -0.005 to -0.001 cm3), right hippocampal (stress: -0.004; 95% CI, -0.007 to -0.002; trait anxiety: -0.003; 95% CI, -0.005 to -0.001), and cerebellar (stress: -0.06; 95% CI, -0.09 to -0.02) volumes only among women with fetal CHD. Impaired hippocampal regions were noted in the medial aspect of left hippocampal head and inferior aspect of right hippocampal head and body. Impaired cerebellar regions were noted in the anterior superior aspect of vermal and paravermal regions and the left cerebellar lobe. CONCLUSIONS AND RELEVANCE: These findings suggested that psychological distress among women carrying fetuses with CHD is prevalent and is associated with impaired fetal cerebellar and hippocampal development. These data underscore the importance of universal screening for maternal psychological distress, integrated prenatal mental health support, and targeted early cognitive-behavioral interventions given that stress is a potentially modifiable risk factor in this high-risk population. AD - Center for the Developing Brain, Children's National Health System, Washington, DC. Division of Biostatistics and Study Methodology, Children's Research Institute, Children's National Health System, Washington, DC. Division of Neonatology, Children's National Health System, Washington, DC. Division of Cardiology, Children's National Health System, Washington, DC. Department of Diagnostic Imaging and Radiology, Children's National Health System, Washington, DC. Hospital and Specialty Services, Children's National Health System, Washington, DC. Fetal Medicine Institute, Children's National Health System, Washington, DC. AN - 31930365 AU - Wu, Y. AU - Kapse, K. AU - Jacobs, M. AU - Niforatos-Andescavage, N. AU - Donofrio, M. T. AU - Krishnan, A. AU - Vezina, G. AU - Wessel, D. AU - du Plessis, A. AU - Limperopoulos, C. C2 - PMC6990726 DA - Jan 13 DB - PubMed DO - 10.1001/jamapediatrics.2019.5316 DP - NLM ET - 2020/01/14 IS - 3 LA - eng N1 - 2168-6211 Wu, Yao Kapse, Kushal Jacobs, Marni Niforatos-Andescavage, Nickie Donofrio, Mary T Krishnan, Anita Vezina, Gilbert Wessel, David du Plessis, Adré Limperopoulos, Catherine U54 HD090257/HD/NICHD NIH HHS/United States Journal Article JAMA Pediatr. 2020 Jan 13;174(3):e195316. doi: 10.1001/jamapediatrics.2019.5316. PY - 2020 SN - 2168-6203 (Print) 2168-6203 SP - e195316 ST - Association of Maternal Psychological Distress With In Utero Brain Development in Fetuses With Congenital Heart Disease T2 - JAMA Pediatr TI - Association of Maternal Psychological Distress With In Utero Brain Development in Fetuses With Congenital Heart Disease VL - 174 ID - 95 ER - TY - JOUR AB - Objective: To examine associations between maternal reports of prenatal fever or influenza and congenital heart defects (CHDs), and to evaluate whether those associations varied with antipyretic use. Study design: We analyzed case infants with CHD (n = 2361) and control infants without CHD (n = 3435) from the Baltimore-Washington Infant Study (1981-1989). Participating mothers were asked whether they experienced a "fever of 101°F or higher," had "influenza (flu)," or used an antipyretic agent (ie, acetaminophen, salicylate, or nonsteroidal anti-inflammatory drug) during the period extending from 3 months before pregnancy through the end of the third month of pregnancy. We used logistic regression to compute ORs and 95% CIs while controlling for potential confounders. Results: There were significant associations between fever and influenza and specific CHDs, namely right-sided obstructive defects (fever: OR, 2.04; 95% CI, 1.27 to 3.27; influenza: OR, 1.75; 95% CI, 1.16 to 2.62) and atrioventricular septal defects in infants with Down syndrome (fever: OR, 1.92; 95% CI, 1.10 to 3.38; influenza: OR, 1.66; 95% CI, 1.04 to 2.63). Maternal antipyretic use in the setting of fever or influenza tended to decrease these associations. Conclusions: Prenatal maternal fever or influenza may be associated with right-sided obstructive lesions in all infants and with atrioventricular septal defects in infants with Down syndrome. The use of antipyretics might attenuate such associations. Copyright © 2011 Mosby Inc. All rights reserved. AD - M. E. Oster, Division of Pediatric Cardiology, Children's Healthcare of Atlanta, 1405 Clifton Road NE, Atlanta, GA 30322, United States AU - Oster, M. E. AU - Riehle-Colarusso, T. AU - Alverson, C. J. AU - Correa, A. DB - Embase Medline DO - 10.1016/j.jpeds.2010.11.058 IS - 6 KW - antipyretic agent nonsteroid antiinflammatory agent paracetamol salicylic acid aortic coarctation aortic stenosis article congenital heart malformation controlled study disease association Down syndrome Ebstein anomaly Fallot tetralogy female fever great vessels transposition heart atrium septum defect heart outflow tract obstruction heart ventricle septum defect human hypoplastic left heart syndrome infant influenza major clinical study male maternal disease prenatal disorder prenatal drug exposure priority journal pulmonary valve stenosis risk assessment third trimester pregnancy tricuspid valve atresia upper respiratory tract infection urinary tract infection LA - English M3 - Article N1 - L51240926 2011-01-28 2011-05-30 PY - 2011 SN - 0022-3476 1097-6833 SP - 990-995 ST - Associations between maternal fever and influenza and congenital heart defects T2 - Journal of Pediatrics TI - Associations between maternal fever and influenza and congenital heart defects UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51240926 http://dx.doi.org/10.1016/j.jpeds.2010.11.058 VL - 158 ID - 1103 ER - TY - JOUR AB - OBJECTIVE: Echocardiography was used to study electromechanical atrial and ventricular function in adult patients with a 1-ventricle heart who were in sinus rhythm to better understand the recurrence of atrial flutter in these conditions. Patients who had recent atrial flutter, with and without the Fontan procedure, were compared with those who had no arrhythmia. METHODS: This was a prospective study that used M-mode and 2-dimensional Doppler echocardiography and electrocardiography. Conventional measurements were used to evaluate ventricular long-axis function. Basic data were drawn from case notes. The setting was a designated quaternary service for grown-up congenital heart patients (GUCH) in a tertiary referral center for cardiology and cardiac surgery. From January 1997 to February 1998, 26 consecutive adult patients (aged >16 years) with a heart with one functioning ventricle and a history of atrial flutter were studied: group 1, with non-Fontan palliative surgery or no surgery (10 patients), and group 2, with Fontan-type repair (16 patients). Also studied were 20 patients with a 1-ventricle heart but no history of atrial flutter. These 20 patients were divided into 2 groups: control 1, which comprised 14 patients with previous shunts or no surgery, and control 2, which consisted of 6 patients with Fontan repair. RESULTS: P-wave duration on the electrocardiogram was similar in the 4 patient groups, but the amplitude was reduced in group 2 and control 2 (patients with Fontan surgery) (P <.016). Bifid P wave was seen in 5 (50%) of 10 patients in group 1 and in 6 (43%) of 14 patients in control 1, but it was not seen in patients with Fontan (P <.01). Ventricular systolic and diastolic dimensions and fractional shortening were not different between patients and controls. Right atrial transverse dimensions were greater in group 2 patients compared with those in controls. Significant atrioventricular valve regurgitation was seen in 9 of 10 group 1 patients but in none of the others. Right-sided total long-axis excursion and atrial A-wave amplitude were depressed in group 2 patients compared with the values in the others. The onset of right atrial shortening was delayed by 50 ms in group 2 compared with control 2, whereas the left atrial shortening was delayed by 30 ms in group 1 compared with control 1. This particular disturbance remained 6 months after cardioversion. CONCLUSION: In 1-ventricle hearts, significant atrioventricular valve regurgitation is commonly associated with atrial flutter in patients who did not undergo the Fontan procedure, and with electromechanical disturbances in those who did. Recognition of disturbances in ventricular long-axis function may thus assist in the identification of patients with a 1-ventricle heart who are prone to atrial flutter. AD - Jane Somerville GUCH Unit and Department of Echocardiography, Royal Brompton Hospital and Imperial College, London University, United Kingdom. AN - 11241014 AU - Li, W. AU - Sarubbi, B. AU - Sutton, R. AU - Somerville, J. AU - Gibson, D. AU - Henein, M. Y. DA - Mar DB - PubMed DO - 10.1067/mje.2001.110141 DP - NLM ET - 2001/03/10 IS - 3 KW - Adolescent Adult Atrial Flutter/*diagnostic imaging/*etiology/physiopathology Chi-Square Distribution Echocardiography, Doppler/*methods Electrocardiography Female *Fontan Procedure Heart Conduction System/physiopathology Heart Ventricles/*abnormalities/surgery Humans Male Middle Aged Palliative Care Prospective Studies LA - eng N1 - Li, W Sarubbi, B Sutton, R Somerville, J Gibson, D Henein, M Y Journal Article Research Support, Non-U.S. Gov't United States J Am Soc Echocardiogr. 2001 Mar;14(3):186-93. doi: 10.1067/mje.2001.110141. PY - 2001 SN - 0894-7317 (Print) 0894-7317 SP - 186-93 ST - Atrial and ventricular electromechanical function in 1-ventricle hearts: influence of atrial flutter and Fontan procedure T2 - J Am Soc Echocardiogr TI - Atrial and ventricular electromechanical function in 1-ventricle hearts: influence of atrial flutter and Fontan procedure VL - 14 ID - 503 ER - TY - JOUR AB - Fontan operation and its modifications are used for the physiological correction of complex congenital heart malformations with functionally single ventricle. Atrial natriuretic peptide (ANP), a physiological diuretic and vasodilator that-together with the effects of cardiopulmonary bypass-plays an important role in the augmentation of capillary permeability in Fontan patients. The rise in right atrial pressure and wall stress is an important stimulus for the release of ANP.ANP levels were measured before and early after surgery in Fontan group (n = 20) and control group (n = 20, patients with simple cardiac defects) to study its influence on and association with mean right atrial pressure, pulmonary vascular resistance (PVR), systemic vascular resistance, amount of drainage during early and late postoperative period, duration of intensive care unit and hospital stay, and need for colloid supplement.Early postoperative ANP values showed a negative correlation with PVR (r = -0.55) and total drainage (r = -0.88). There was no significant change in ANP during surgery or in the postoperative period in control patients.Reduction of PVR and maintenance of efficient urine output are important in the management of Fontan circulation. We conclude that high levels of ANP measured early after Fontan operation can be used as a marker for the successful establishment of Fontan circulation in patients with complex congenital heart defects. Copyright © American Society of Artificial Internal Organs. AD - T. Alkan, V.K.V. American Hospital, Department of Cardiovascular Surgery, Guzelbahce sk. No. 20 Nisantasi, 34365, Istanbul, Turkey AU - Alkan, T. AU - Sarioǧlu, A. AU - Samanli Ü, B. AU - Sarioǧlu, T. AU - Akçevin, A. AU - Türkoǧlu, H. AU - Paker, T. AU - Aytaç, A. DB - Embase Medline DO - 10.1097/01.mat.0000235275.65027.1d IS - 5 KW - atrial natriuretic factor adolescent article capillary permeability child clinical article colloid congenital heart malformation controlled study effusion female Fontan procedure heart atrium pressure hospitalization human intensive care unit lung vascular resistance male surgical drainage systemic vascular resistance LA - English M3 - Article N1 - L44394265 2006-10-01 PY - 2006 SN - 1058-2916 SP - 543-548 ST - Atrial natriuretic peptide: Could it be a marker for postoperative recurrent effusions after fontan circulation in complex congenital heart defects? T2 - ASAIO Journal TI - Atrial natriuretic peptide: Could it be a marker for postoperative recurrent effusions after fontan circulation in complex congenital heart defects? UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L44394265 http://dx.doi.org/10.1097/01.mat.0000235275.65027.1d VL - 52 ID - 1255 ER - TY - JOUR AB - Fontan operation and its modifications are used for the physiological correction of complex congenital heart malformations with functionally single ventricle. Atrial natriuretic peptide (ANP), a physiological diuretic and vasodilator that--together with the effects of cardiopulmonary bypass--plays an important role in the augmentation of capillary permeability in Fontan patients. The rise in right atrial pressure and wall stress is an important stimulus for the release of ANP. ANP levels were measured before and early after surgery in Fontan group (n = 20) and control group (n = 20, patients with simple cardiac defects) to study its influence on and association with mean right atrial pressure, pulmonary vascular resistance (PVR), systemic vascular resistance, amount of drainage during early and late postoperative period, duration of intensive care unit and hospital stay, and need for colloid supplement. Early postoperative ANP values showed a negative correlation with PVR (r = -0.55) and total drainage (r = -0.88). There was no significant change in ANP during surgery or in the postoperative period in control patients. Reduction of PVR and maintenance of efficient urine output are important in the management of Fontan circulation. We conclude that high levels of ANP measured early after Fontan operation can be used as a marker for the successful establishment of Fontan circulation in patients with complex congenital heart defects. AD - Department of Cardiovascular Surgery, V.K.V. American Bristol Hospital, Turkey. AN - 16966855 AU - Alkan, T. AU - Sarioğlu, A. AU - Samanli, U. B. AU - Sarioğlu, T. AU - Akçevin, A. AU - Türkoğlu, H. AU - Paker, T. AU - Aytaç, A. DA - Sep-Oct DB - PubMed DO - 10.1097/01.mat.0000235275.65027.1d DP - NLM ET - 2006/09/13 IS - 5 KW - Adolescent Atrial Function, Right Atrial Natriuretic Factor/*blood Biomarkers/blood *Cardiovascular Physiological Phenomena Child Child, Preschool Drainage Fontan Procedure/*adverse effects Heart Defects, Congenital/*surgery Humans Postoperative Care Recurrence Vascular Resistance LA - eng N1 - Alkan, T Sarioğlu, A Samanli, U B Sarioğlu, T Akçevin, A Türkoğlu, H Paker, T Aytaç, A Journal Article United States ASAIO J. 2006 Sep-Oct;52(5):543-8. doi: 10.1097/01.mat.0000235275.65027.1d. PY - 2006 SN - 1058-2916 (Print) 1058-2916 SP - 543-8 ST - Atrial natriuretic peptide: could it be a marker for postoperative recurrent effusions after Fontan circulation in complex congenital heart defects? T2 - Asaio j TI - Atrial natriuretic peptide: could it be a marker for postoperative recurrent effusions after Fontan circulation in complex congenital heart defects? VL - 52 ID - 410 ER - TY - JOUR AB - The hemodynamics after Fontan surgery are notable for hypertension and dilation of the right atrium (RA). The effect of this stress on atrial cytoarchitecture has not been systematically studied and might be relevant to arrhythmias and their treatment. Morphologic and histopathologic analyses were performed on tissue from the RA and left atrium (LA) from autopsy specimens of Fontan hearts (n = 47). The findings were compared to those from control samples from young patients with normal atrial hemodynamics (n = 15). Most Fontan specimens were from young patients who died after a relatively short duration of Fontan physiology. The tissues were analyzed for wall thickness, fibrosis content, and fibrosis pattern. The mean wall thickness for the RA (3.0 +/- 1.0 mm) and LA (2.3 +/- 0.6 mm) in the Fontan hearts was significantly greater than that in the control hearts (RA, 1.8 +/- 0.4 mm; LA, 1.8 +/- 0.5 mm; p <0.001 and p = 0.024, respectively). The predictors for thickening of the RA included (1) older age at Fontan surgery, (2) older age at death, and (3) longer duration of Fontan circulation. The Fontan hearts and control hearts exhibited nearly identical fibrosis patterns in the RA and LA. Neither wall thickness nor fibrosis varied with the underlying heart defect or style of Fontan connection. In conclusion, atrial remodeling after Fontan surgery for univentricular heart physiology involves increased wall thickness in both the RA and LA. Interstitial fibrosis was also observed in the Fontan atria; however, because a similar pattern was present in the control tissue, this likely represented normal fibroelastic atrial structure, rather than a specific response to Fontan hemodynamics. The degree of wall thickening observed in the Fontan atria was not so excessive as to preclude transmural lesions during catheter or surgical ablation of reentrant arrhythmias. AD - Department of Pediatrics, Harvard Medical School, Boston, MA, USA. AN - 19962486 AU - Wolf, C. M. AU - Seslar, S. P. AU - den Boer, K. AU - Juraszek, A. L. AU - McGowan, F. X. AU - Cowan, D. B. AU - Del Nido, P. AU - Triedman, J. K. AU - Berul, C. I. AU - Walsh, E. P. C2 - PMC3399422 C6 - NIHMS232497 DA - Dec 15 DB - PubMed DO - 10.1016/j.amjcard.2009.07.061 DP - NLM ET - 2009/12/08 IS - 12 KW - Child Child, Preschool Female Fontan Procedure/*adverse effects Heart Atria/*pathology/*physiopathology Hemodynamics Humans Male LA - eng N1 - 1879-1913 Wolf, Cordula M Seslar, Stephen P den Boer, Karen Juraszek, Amy L McGowan, Francis X Cowan, Douglas B Del Nido, Pedro Triedman, John K Berul, Charles I Walsh, Edward P R01 HL068915/HL/NHLBI NIH HHS/United States R01 HL068915-08/HL/NHLBI NIH HHS/United States R01 HL088206/HL/NHLBI NIH HHS/United States R01 HL088206-04/HL/NHLBI NIH HHS/United States Comparative Study Journal Article Am J Cardiol. 2009 Dec 15;104(12):1737-42. doi: 10.1016/j.amjcard.2009.07.061. PY - 2009 SN - 0002-9149 (Print) 0002-9149 SP - 1737-42 ST - Atrial remodeling after the Fontan operation T2 - Am J Cardiol TI - Atrial remodeling after the Fontan operation VL - 104 ID - 343 ER - TY - JOUR AB - The hemodynamics after Fontan surgery are notable for hypertension and dilation of the right atrium (RA). The effect of this stress on atrial cytoarchitecture has not been systematically studied and might be relevant to arrhythmias and their treatment. Morphologic and histopathologic analyses were performed on tissue from the RA and left atrium (LA) from autopsy specimens of Fontan hearts (n = 47). The findings were compared to those from control samples from young patients with normal atrial hemodynamics (n = 15). Most Fontan specimens were from young patients who died after a relatively short duration of Fontan physiology. The tissues were analyzed for wall thickness, fibrosis content, and fibrosis pattern. The mean wall thickness for the RA (3.0 +/- 1.0 mm) and LA (2.3 +/- 0.6 mm) in the Fontan hearts was significantly greater than that in the control hearts (RA, 1.8 +/- 0.4 mm; LA, 1.8 +/- 0.5 mm; p <0.001 and p = 0.024, respectively). The predictors for thickening of the RA included (1) older age at Fontan surgery, (2) older age at death, and (3) longer duration of Fontan circulation. The Fontan hearts and control hearts exhibited nearly identical fibrosis patterns in the RA and LA. Neither wall thickness nor fibrosis varied with the underlying heart defect or style of Fontan connection. In conclusion, atrial remodeling after Fontan surgery for univentricular heart physiology involves increased wall thickness in both the RA and LA. Interstitial fibrosis was also observed in the Fontan atria; however, because a similar pattern was present in the control tissue, this likely represented normal fibroelastic atrial structure, rather than a specific response to Fontan hemodynamics. The degree of wall thickening observed in the Fontan atria was not so excessive as to preclude transmural lesions during catheter or surgical ablation of reentrant arrhythmias. AD - Department of Pediatrics, Harvard Medical School, Boston, MA, USA AN - 105259995. Language: English. Entry Date: 20100312. Revision Date: 20161124. Publication Type: journal article AU - Wolf, C. M. AU - Seslar, S. P. AU - den Boer, K. AU - Juraszek, A. L. AU - McGowan, F. X. AU - Cowan, D. B. AU - Del Nido, P. AU - Triedman, J. K. AU - Berul, C. I. AU - Walsh, E. P. AU - Wolf, Cordula M. AU - Seslar, Stephen P. AU - den Boer, Karen AU - Juraszek, Amy L. AU - McGowan, Francis X. AU - Cowan, Douglas B. AU - Del Nido, Pedro AU - Triedman, John K. AU - Berul, Charles I. AU - Walsh, Edward P. DB - ccm DO - 10.1016/j.amjcard.2009.07.061 DP - EBSCOhost IS - 12 KW - Cardiopulmonary Bypass -- Adverse Effects Heart Atrium -- Pathology Heart Atrium -- Physiopathology Child Child, Preschool Female Hemodynamics Male N1 - research. Journal Subset: Biomedical; Peer Reviewed; USA. Grant Information: R01 HL088206-04/HL/NHLBI NIH HHS/United States. NLM UID: 0207277. PMID: NLM19962486. PY - 2009 SN - 0002-9149 SP - 1737-1742 ST - Atrial remodeling after the Fontan operation T2 - American Journal of Cardiology TI - Atrial remodeling after the Fontan operation UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105259995&site=ehost-live&scope=site VL - 104 ID - 1593 ER - TY - JOUR AB - RATIONALE: Atrioventricular reentrant tachycardia (AVRT) is the most common supraventricular tachycardia occurring in children. However, in complex congenital heart disease patients with a different heart anatomy and conduction system morphology, accessory pathway modification may be particularly challenging because of distortion of typical anatomic landmarks. PATIENT CONCERNS: A 10-year-old boy with tricuspid atresia and history of bidirectional Glenn operation had recurrent chest distress and palpitation for 3 months. He had multiple hospitalizations for narrow-QRS tachycardia with poor hemodynamic tolerance, despite the use of adenosine and amiodarone. DIAGNOSES: AVRT. Tricuspid atresia with secundum atrial septal defect, large ventricular septal defect, and right ventricular outflow tract stenosis. INTERVENTIONS: Cardiac catheterization, electrophysiological examination, and ablation. OUTCOMES: The child has not had a recurrent AVRT during 6 months of follow-up and is waiting for Fontan operation. LESSONS: Since there is an increased risk of accessory pathways in patients with tricuspid atresia, all these patients should be checked before the Fontan operation to exclude congenital accessory pathways. AD - Department of Cardiology, Hunan Children's Hospital. Department of Cardiology, Second Xiangya Hospital, Central South University. Department of Cardiothoracic Surgery, Hunan Children's Hospital, Changsha, Hunan, China. AN - 30732151 AU - Wang, Y. AU - Liu, Q. AU - Deng, X. AU - Xiao, Y. AU - Chen, Z. C2 - PMC6380756 DA - Feb DB - PubMed DO - 10.1097/md.0000000000014320 DP - NLM ET - 2019/02/09 IS - 6 KW - *Catheter Ablation Child Humans Male Tachycardia, Atrioventricular Nodal Reentry/complications/diagnosis/*surgery Tricuspid Atresia/complications/diagnosis/*surgery LA - eng N1 - 1536-5964 Wang, Yefeng Liu, Qiming Deng, Xicheng Xiao, Yunbin Chen, Zhi Case Reports Journal Article Medicine (Baltimore). 2019 Feb;98(6):e14320. doi: 10.1097/MD.0000000000014320. PY - 2019 SN - 0025-7974 (Print) 0025-7974 SP - e14320 ST - Atrioventricular reentrant tachycardia in a child with tricuspid atresia: A case report of catheter ablation T2 - Medicine (Baltimore) TI - Atrioventricular reentrant tachycardia in a child with tricuspid atresia: A case report of catheter ablation VL - 98 ID - 113 ER - TY - JOUR AB - Rationale: Atrioventricular reentrant tachycardia (AVRT) is the most common supraventricular tachycardia occurring in children. However, in complex congenital heart disease patients with a different heart anatomy and conduction system morphology, accessory pathway modification may be particularly challenging because of distortion of typical anatomic landmarks.Patient Concerns: A 10-year-old boy with tricuspid atresia and history of bidirectional Glenn operation had recurrent chest distress and palpitation for 3 months. He had multiple hospitalizations for narrow-QRS tachycardia with poor hemodynamic tolerance, despite the use of adenosine and amiodarone.Diagnoses: AVRT. Tricuspid atresia with secundum atrial septal defect, large ventricular septal defect, and right ventricular outflow tract stenosis.Interventions: Cardiac catheterization, electrophysiological examination, and ablation.Outcomes: The child has not had a recurrent AVRT during 6 months of follow-up and is waiting for Fontan operation.Lessons: Since there is an increased risk of accessory pathways in patients with tricuspid atresia, all these patients should be checked before the Fontan operation to exclude congenital accessory pathways. AD - Department of Cardiology, Hunan Children's Hospital Department of Cardiology, Second Xiangya Hospital, Central South University Department of Cardiothoracic Surgery, Hunan Children's Hospital, Changsha, Hunan, China. AN - 135999102. Language: English. Entry Date: 20190427. Revision Date: 20190427. Publication Type: journal article AU - Wang, Yefeng AU - Liu, Qiming AU - Deng, Xicheng AU - Xiao, Yunbin AU - Chen, Zhi AU - Na DB - ccm DO - 10.1097/MD.0000000000014320 DP - EBSCOhost IS - 6 KW - Catheter Ablation Tricuspid Atresia -- Surgery Tachycardia, Supraventricular -- Surgery Tachycardia, Supraventricular -- Diagnosis Tricuspid Atresia -- Diagnosis Tricuspid Atresia -- Complications Child Tachycardia, Supraventricular -- Complications Male N1 - case study. Journal Subset: Biomedical; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. NLM UID: 2985248R. PMID: NLM30732151. PY - 2019 SN - 0025-7974 SP - e14320-e14320 ST - Atrioventricular reentrant tachycardia in a child with tricuspid atresia: A case report of catheter ablation T2 - Medicine TI - Atrioventricular reentrant tachycardia in a child with tricuspid atresia: A case report of catheter ablation UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=135999102&site=ehost-live&scope=site VL - 98 ID - 1515 ER - TY - JOUR AB - OBJECTIVE: We conducted a survey to determine which management options pediatric cardiologists and cardiac surgeons in North America discuss and recommend when counseling parents after the diagnosis of hypoplastic left heart syndrome (HLHS). METHODS: Pediatric cardiologists and cardiac surgeons across North America were asked to complete an anonymous, Internet-based survey about their attitudes and practices regarding the management of HLHS. RESULTS: We contacted 1621 pediatric cardiologists and surgeons, of whom 749 (46%) completed the survey. When counseling parents of newborns with HLHS, 99.7% of respondents discussed staged palliative surgery, 67% discussed cardiac transplantation, and 62.2% discussed compassionate care without surgery. Only a minority (14.9%) discussed all of those options. Staged palliative surgery was recommended over cardiac transplantation or compassionate care without surgery by 76.2% of respondents. When counseling parents after prenatal diagnosis of HLHS, 98.8% of respondents discussed continuation of pregnancy with staged palliative surgery after birth, 53.5% discussed continuation of pregnancy with cardiac transplantation after birth, 56.9% discussed continuation of pregnancy with compassionate care after birth, and 74.3% discussed termination of pregnancy. Only 36.5% discussed all of those options. Continuation of pregnancy with staged palliative surgery after birth was recommended over the other options by 56% of respondents. CONCLUSIONS: Virtually all North American pediatric cardiologists and cardiac surgeons surveyed discuss a surgical intervention when counseling parents about the care of their child or fetus with HLHS. However, only a minority discuss all options. Most physicians recommend staged palliative surgery for management of HLHS. Copyright © 2010 by the American Academy of Pediatrics. AD - C. V. Rohlicek, Division of Cardiology, Montreal Children's Hospital, McGill University Health Centre, Montreal, QC, Canada AU - Prsa, M. AU - Holly, C. D. AU - Carnevale, F. A. AU - Justino, H. AU - Rohlicek, C. V. DB - Embase Medline DO - 10.1542/peds.2009-1678 IS - 3 KW - article birth cardiologist compassionate emotion health survey heart transplantation human hypoplastic left heart syndrome Internet medical practice medical specialist North America palliative therapy parent counseling pediatrics physician attitude pregnancy pregnancy termination priority journal LA - English M3 - Article N1 - L358375971 2010-03-10 2010-03-23 http://pediatrics.aappublications.org/cgi/reprint/125/3/e625 PY - 2010 SN - 0031-4005 1098-4275 SP - e625-e630 ST - Attitudes and practices of cardiologists and surgeons who manage HLHS T2 - Pediatrics TI - Attitudes and practices of cardiologists and surgeons who manage HLHS UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L358375971 http://dx.doi.org/10.1542/peds.2009-1678 VL - 125 ID - 1148 ER - TY - JOUR AB - BACKGROUND: Recent studies showed that children with univentricular heart have elevated plasma B-type natriuretic peptide (BNP) levels prior to bidirectional cavopulmonary anastomosis (BDCPA). However, it remains to be established whether BNP levels reflect the degree of hemodynamic overload and relate to long-term outcome in univentricular circulation. METHODS: Fifty one consecutive children with functionally univentricular heart prior to BDCPA were studied. All patients underwent cardiac catheterization and BNP measurement. Ventricular end-systolic wall stress (ESWS) and end-diastolic wall stress (EDWS) were calculated from cardiac catheterization data. RESULTS: Median age was 1.1 years and 34% were female. Median BNP concentration was 90.4 pg/ml. Patients with high BNP (≥ 100 pg/ml) had higher pulmonary to systemic flow ratio (p = 0.014), a greater end-diastolic volume (p=0.009), more severe atrioventricular valve regurgitation (p= 0.02) and lower ventricular mass to end-diastolic volume ratio (p=0.006). BNP levels strongly related to EDWS (r = 0.75, p< 0.0001) and ESWS (r = 0.63, p < 0.0001). During median follow-up period of 3.2 years, 15 patients died and one underwent heart transplantation for refractory heart failure. On multivariate Cox regression analysis, high BNP concentration was an independent predictor of death or transplantation (HR 3.05, CI: 1.06-8.83, p = 0.04). CONCLUSIONS: High BNP concentration at the first palliative stage towards Fontan circulation reflects high wall stress due to high volume load and insufficient ventricular hypertrophy. Moreover, high BNP levels at this stage were independently related to long-term outcome. BNP may be used as a guide to identify patients with high workload due to inadequate adaptation to hemodynamic load, who are at high risk. AD - The Department of Pediatric Cardiology, Chiba Children's Hospital, Chiba-city, Chiba, Japan. inuzukar-tky@umin.ac.jp AN - 21397962 AU - Inuzuka, R. AU - Tatebe, S. AU - Wakiguchi, S. AU - Nakajima, H. AU - Ohtsu, H. AU - Dimopoulos, K. AU - Aotsuka, H. DA - Aug 23 DB - PubMed DO - 10.1016/j.ijcard.2011.02.023 DP - NLM ET - 2011/03/15 IS - 2 KW - Adaptation, Physiological/*physiology Biomarkers/blood Female Follow-Up Studies Fontan Procedure Heart Failure/*blood/diagnosis Humans Infant Male Natriuretic Peptide, Brain/*blood Stroke Volume/*physiology Ventricular Function, Left/physiology LA - eng N1 - 1874-1754 Inuzuka, Ryo Tatebe, Shunsuke Wakiguchi, Sadae Nakajima, Hiromichi Ohtsu, Hiroshi Dimopoulos, Konstantinos Aotsuka, Hiroyuki Journal Article Research Support, Non-U.S. Gov't Netherlands Int J Cardiol. 2012 Aug 23;159(2):88-93. doi: 10.1016/j.ijcard.2011.02.023. Epub 2011 Mar 12. PY - 2012 SN - 0167-5273 SP - 88-93 ST - B-type natriuretic peptide at the early stage of univentricular circulation reflects inadequate adaptation to volume overload T2 - Int J Cardiol TI - B-type natriuretic peptide at the early stage of univentricular circulation reflects inadequate adaptation to volume overload VL - 159 ID - 372 ER - TY - JOUR AB - Background: The bedside two-dimensional echocardiography (2-D ECHO) has been successfully used to guide the balloon atrial septostomy, speeding up the procedure and preventing the risks of transportation to the hemodynamics laboratory. Objective: To assess the results of the bedside balloon atrial septostomy in cyanotic neonates. Methods: Between January/1997 and July/2008, 102 atrioseptostomies by balloon catheter guided by echocardiography were carried out and saturation levels, defect diameter, oxygen saturation, clinical-laboratory response and complications related to the procedure were evaluated. Results: Of 102 cases of bedside Rashkind septostomy, 98 met the inclusion criteria, with 90 neonates in Group A (Rashkind procedure in the preoperative phase) and 8 in Group B (procedure at the postoperative phase). There was a predominance of the male sex (75%), mean age was 8.3 ± 9.3 days and the mean weight was 3,100 ± 1,100 g; the transposition of the great arteries was the most frequent congenital heart disease (n = 74). When comparing the levels of saturation pre and post-procedure (65.9 ± 19.5% and 86 ± 9.7%) and the diameter of the interatrial septal defect pre and post-procedure (2.3 ± 1.0 mm and 5.5 ± 1.3 mm) there was a statistically significant difference (p < 0.001). When comparing the levels of saturation and the diameter of the interatrial septal defect between the group of survivors vs non-survivor, there was no statistically significant difference (p < 0.05). Conclusion: The balloon atrial septostomy guided by the echocardiography reduces the risks by allowing the effective palliative procedure to be carried out promptly in cyanotic neonates, in addition to presenting lower hospital costs. AD - L. M. Lopes, Al. Santos, 211, conj 704, Cerqueira César - 01419-000 - São Paulo, SP, Brazil AU - Lopes, L. M. AU - Kawano, C. AU - Cristóvão, S. A. B. AU - Nagamatsu, C. T. AU - Da Fonseca, L. AU - Furlanetto, B. H. S. AU - Furlanetto, G. AU - Da Silva, J. P. AU - Mangione, J. A. DB - Embase Medline DO - 10.1590/S0066-782X2010005000076 IS - 2 KW - article balloon atrial septostomy balloon catheter fatality female great vessels transposition atrial fibrillation interatrial septum heart atrium septum defect heart surgery heart tamponade heart ventricle tachycardia human hypoplastic left heart syndrome major clinical study male newborn newborn intensive care outcome assessment oxygen saturation palliative therapy risk reduction surgical technique two dimensional echocardiography LA - Portuguese English M3 - Article N1 - L359687625 2010-10-11 2010-10-20 http://www.scielo.br/pdf/abc/v95n2/en_aop07110.pdf PY - 2010 SN - 0066-782X 1678-4170 SP - 153-158 ST - Balloon atrial septostomy guided by echocardiography in a neonatal intensive care unit T2 - Arquivos Brasileiros de Cardiologia TI - Balloon atrial septostomy guided by echocardiography in a neonatal intensive care unit UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L359687625 http://dx.doi.org/10.1590/S0066-782X2010005000076 VL - 95 ID - 1138 ER - TY - JOUR AB - BACKGROUND: The pulsatile nature of pulmonary blood flow is important for shear stress-mediated release of endothelium-derived nitric oxide (NO) and lowering pulmonary vascular resistance (PVR) by passive recruitment of capillaries. Normal pulsatile flow is lost or markedly attenuated after Fontan-type operations, but to date, there are no data on basal pulmonary vascular resistance and its responsiveness to exogenous NO at late follow-up in these patients. METHODS AND RESULTS: We measured indexed PVR (PVRI) using Fick principle to calculate pulmonary blood flow, with respiratory mass spectrometry to measure oxygen consumption, in 15 patients (median age, 12 years; range, 7 to 17 years; 12 male, 3 female) at a median of 9 years after a Fontan-type operation (6 atriopulmonary connections, 7 lateral tunnels, 2 extracardiac conduits). The basal PVRI was 2.11+/-0.79 Wood unit (WU) times m2 (mean+/-SD) and showed a significant reduction to 1.61+/-0.48 (P=0.016) after 20 ppm of NO for 10 minutes. The patients with nonpulsatile group in the pulmonary circulation dropped the PVRI from 2.18+/-0.34 to 1.82+/-0.55 (P<0.05) after NO inhalation. CONCLUSIONS: PVR falls with exogenous NO late after Fontan-type operation. These data suggest pulmonary endothelial dysfunction, related in some part to lack of pulsatility in the pulmonary circulation because of altered flow characteristics. Therapeutic strategies to enhance pulmonary endothelial NO release may have a role in these patients. AD - Cardiothoracic Unit, Great Ormond Street Hospital for Children NHS Trust, London WC1N 3JH, UK. s.khambadkone@ich.ucl.ac.uk AN - 12821557 AU - Khambadkone, S. AU - Li, J. AU - de Leval, M. R. AU - Cullen, S. AU - Deanfield, J. E. AU - Redington, A. N. DA - Jul 1 DB - PubMed DO - 10.1161/01.Cir.0000074210.49434.40 DP - NLM ET - 2003/06/25 IS - 25 KW - Administration, Inhalation Adolescent Age Factors Angiography Blood Flow Velocity/drug effects/physiology Cardiac Catheterization Child Endothelium, Vascular/drug effects/*physiology Female *Fontan Procedure Heart Defects, Congenital/surgery Hemodynamics/drug effects/physiology Humans Male Nitric Oxide/*administration & dosage Oxygen Consumption/physiology Pulmonary Circulation/drug effects/*physiology Pulsatile Flow/physiology Time Vascular Resistance/drug effects/*physiology LA - eng N1 - 1524-4539 Khambadkone, S Li, J de Leval, M R Cullen, S Deanfield, J E Redington, A N Clinical Trial Journal Article Research Support, Non-U.S. Gov't United States Circulation. 2003 Jul 1;107(25):3204-8. doi: 10.1161/01.CIR.0000074210.49434.40. Epub 2003 Jun 23. PY - 2003 SN - 0009-7322 SP - 3204-8 ST - Basal pulmonary vascular resistance and nitric oxide responsiveness late after Fontan-type operation T2 - Circulation TI - Basal pulmonary vascular resistance and nitric oxide responsiveness late after Fontan-type operation VL - 107 ID - 468 ER - TY - JOUR AB - Objective: To assess the utility of bedside ultrasound combining B-and M-mode in the diagnosis of abnormal diaphragmatic motion in children after heart surgery. Design: Prospective post hoc blinded comparison of ultrasound performed by two different intensivists and fluoroscopy results with electromyography. Setting: Tertiary university hospital. Subjects: Children with suspected abnormal diaphragmatic motion after heart surgery. Interventions: None. Measurements and Main Results: Abnormal diaphragmatic motion was suspected in 26 children. Electromyography confirmed the diagnosis in 20 of 24 children (83.3%). The overall occurrence rate of abnormal diaphragmatic motion during the study period was 7.5%. Median patient age was 5 months (range, 16 d to 14 yr). Sensitivity and specificity of chest ultrasound performed at the bedside by the two intensivists (91% and 92% and 92% and 95%, respectively) were higher than those obtained by fluoroscopy (87% and 83%). Interobserver agreement (k) between both intensivists was 0.957 (95% CI, 0.87-100). Conclusions: Chest ultrasound performed by intensivists is a valid tool for the diagnosis of diaphragmatic paralysis, presenting greater sensitivity and specificity than fluoroscopy. Chest ultrasound should be routinely used after pediatric heart surgery given its reliability, reproducibility, availability, and safety. © 2017 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies. AD - Pediatric Intensive Care Unit, Department of Pediatrics, Hospital Universitari Vall d'Hebron, Barcelona, Spain Department of Clinical Neurophysiology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain Department of Neonatology, Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain Department of Pediatric Radiology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain Cardiac Intensive Care Unit, Department of Critical Care Medicine, Children's Hospital of Pittsburgh, Pittsburgh, PA, United States Unit of Clinical Research Support, Vall d'Hebron Research Institute, Universitat Autònoma de Barcelona, Barcelona, Spain Department of Pediatric Cardiothoracic Surgery, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain Department of Pediatric Cardiology, Sant Joan de Deu Barcelona Children's, University of Barcelona, Barcelona, Spain AU - Gil-Juanmiquel, L. AU - Gratacós, M. AU - Castilla-Fernández, Y. AU - Piqueras, J. AU - Baust, T. AU - Raguer, N. AU - Balcells, J. AU - Perez-Hoyos, S. AU - Abella, R. F. AU - Sanchez-De-Toledo, J. DB - Scopus DO - 10.1097/PCC.0000000000001015 IS - 2 KW - Diaphragmatic paralysis electromyography fluoroscopy pediatric heart surgery ultrasound M3 - Article N1 - Cited By :6 Export Date: 15 June 2020 PY - 2017 SP - 159-164 ST - Bedside Ultrasound for the Diagnosis of Abnormal Diaphragmatic Motion in Children after Heart Surgery T2 - Pediatric Critical Care Medicine TI - Bedside Ultrasound for the Diagnosis of Abnormal Diaphragmatic Motion in Children after Heart Surgery UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84994168629&doi=10.1097%2fPCC.0000000000001015&partnerID=40&md5=1fa077603c242afcc23e421ef1301d71 VL - 18 ID - 1875 ER - TY - JOUR AB - OBJECTIVES: We measured behavioral, quality of life (QoL), and functional status outcomes for 6-year-old children with hypoplastic left heart syndrome enrolled in the Single Ventricle Reconstruction Trial. We sought to compare these outcomes with those in the normative population and to analyze risk factors for worse outcomes within the single-ventricle group. METHODS: Parent-response instruments included the Vineland Adaptive Behavior Scales, Second Edition (Vineland-II) (primary outcome), Behavior Assessment System for Children 2, Pediatric Quality of Life Inventory 4.0, and other measures of QoL and functional status. We compared subjects with those in the normative sample using 1-sample Wilcoxon rank tests and assessed outcome predictors using multivariable regression. RESULTS: Of 325 eligible patients, 250 (77%) participated. Compared with population norms, participants had lower scores on the Vineland-II motor skills domain (90 ± 17 vs 100 ± 15; P < .001), with 11% scoring >2 SDs below the normative mean. On nearly all major domains, more study subjects (3.3%-19.7%) scored outside the normal range than anticipated for the general population. Independent risk factors for lower Vineland-II scores included perioperative extracorporeal membrane oxygenation, male sex, use of regional cerebral perfusion, catheterization after stage 2 operation, visual problems, seizure history, and more complications after 2 years (R (2) = 0.32). Independent predictors of worse Behavior Assessment System for Children 2 (R (2) = 0.07-0.20) and Pediatric Quality of Life Inventory 4.0 (R (2) = 0.17-0.25) domain scores also included sociodemographic factors and measures of morbidity and/or greater course complexity. CONCLUSIONS: At 6 years, children with hypoplastic left heart syndrome had difficulty in areas of adaptive behavior, behavioral symptoms, QoL, and functional status. Principal risks for adverse outcomes include sociodemographic factors and measures of greater course complexity. However, models reveal less than one-third of outcome variance. AD - Department of Pediatrics, Medical School, University of Michigan and Michigan Medicine, Ann Arbor, Michigan; cgoldber@med.umich.edu. New England Research Institutes, Watertown, Massachusetts. Department of Pediatrics, Medical College of Wisconsin and Children's Hospital of Wisconsin, Milwaukee, Wisconsin. Pediatric Cardiothoracic Surgery, School of Medicine, University of Pennsylvania and Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. Department of Pediatrics, Emory University and Children's Healthcare of Atlanta, Atlanta, Georgia. Department of Pediatrics, and The University of Utah and Primary Children's Hospital, Salt Lake City, Utah. Labatt Family Heart Center, Hospital for Sick Children, Toronto, Canada. Department of Pediatrics, Medical School, University of Michigan and Michigan Medicine, Ann Arbor, Michigan. Department of Surgery, Nemours Cardiac Center, Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware. National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland. Department of Pediatrics, Children's Hospital Los Angeles, Los Angeles, California. Department of Pediatrics, Duke University, Durham, North Carolina. Department of Pediatrics, East Carolina University, Greenville, North Carolina. Department of Pediatrics, Wake Forest University, Winston-Salem, North Carolina. Congenital Heart Institute of Florida, St Petersburg, Florida. Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Department of Pediatrics, Medical University of South Carolina, Charleston, South Carolina. Department of Pediatrics, NewYork-Presbyterian Morgan Stanley Children's Hospital, New York, New York. Department of Neurology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts. Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts; and. AN - 31628208 AU - Goldberg, C. S. AU - Hu, C. AU - Brosig, C. AU - Gaynor, J. W. AU - Mahle, W. T. AU - Miller, T. AU - Mussatto, K. A. AU - Sananes, R. AU - Uzark, K. AU - Trachtenberg, F. AU - Pizarro, C. AU - Pemberton, V. L. AU - Lewis, A. B. AU - Li, J. S. AU - Jacobs, J. P. AU - Cnota, J. AU - Atz, A. M. AU - Lai, W. W. AU - Bellinger, D. AU - Newburger, J. W. C2 - PMC6856798 conflicts of interest to disclose. DA - Nov DB - PubMed DO - 10.1542/peds.2019-1010 DP - NLM ET - 2019/10/20 IS - 5 KW - Child Child Behavior Child Behavior Disorders/*etiology Female Humans *Hypoplastic Left Heart Syndrome/complications/psychology Male Prospective Studies *Quality of Life Surveys and Questionnaires LA - eng N1 - 1098-4275 Goldberg, Caren S Hu, Chenwei Brosig, Cheryl Gaynor, J William Mahle, William T Miller, Thomas Mussatto, Kathleen A Sananes, Renee Uzark, Karen Trachtenberg, Felicia Pizarro, Christian Pemberton, Victoria L Lewis, Alan B Li, Jennifer S Jacobs, Jeffrey P Cnota, James Atz, Andrew M Lai, Wyman W Bellinger, David Newburger, Jane W Phn investigators U10 HL109818/HL/NHLBI NIH HHS/United States U10 HL109741/HL/NHLBI NIH HHS/United States U10 HL109781/HL/NHLBI NIH HHS/United States U10 HL109816/HL/NHLBI NIH HHS/United States U10 HL109743/HL/NHLBI NIH HHS/United States U01 HL068290/HL/NHLBI NIH HHS/United States U10 HL068270/HL/NHLBI NIH HHS/United States U01 HL068270/HL/NHLBI NIH HHS/United States U10 HL109777/HL/NHLBI NIH HHS/United States U10 HL109778/HL/NHLBI NIH HHS/United States U10 HL109673/HL/NHLBI NIH HHS/United States Journal Article Research Support, N.I.H., Extramural Pediatrics. 2019 Nov;144(5):e20191010. doi: 10.1542/peds.2019-1010. Epub 2019 Oct 18. PY - 2019 SN - 0031-4005 (Print) 0031-4005 ST - Behavior and Quality of Life at 6 Years for Children With Hypoplastic Left Heart Syndrome T2 - Pediatrics TI - Behavior and Quality of Life at 6 Years for Children With Hypoplastic Left Heart Syndrome VL - 144 ID - 20 ER - TY - JOUR AB - The use of VADs as a BTT in children with heart failure has increased due to enhanced device design and reliability, leading to improved survival, functional capacity, and quality of life. However, the use of VADs in patients with Fontan physiology as a BTT is rare with few case reports. Here, we describe a case of implantation of the Berlin VAD as a BTT for 179 days, one of the longest reported periods of mechanical support, in a child with failing single ventricle. AD - Section of Pediatric Cardiovascular Surgery, Department of Cardiac Surgery, University of Michigan, Ann Arbor, MI, USA. AN - 26408232 AU - Halaweish, I. AU - Ohye, R. G. AU - Si, M. S. DA - Dec DB - PubMed DO - 10.1111/petr.12607 DP - NLM ET - 2015/09/27 IS - 8 KW - Adolescent *Fontan Procedure Heart Failure/physiopathology/*surgery *Heart Transplantation Heart Ventricles/physiopathology *Heart-Assist Devices Humans Male Fontan single ventricle transplant ventricular assist device LA - eng N1 - 1399-3046 Halaweish, I Ohye, R G Si, M S Case Reports Journal Article Denmark Pediatr Transplant. 2015 Dec;19(8):E193-5. doi: 10.1111/petr.12607. Epub 2015 Sep 26. PY - 2015 SN - 1397-3142 SP - E193-5 ST - Berlin heart ventricular assist device as a long-term bridge to transplantation in a Fontan patient with failing single ventricle T2 - Pediatr Transplant TI - Berlin heart ventricular assist device as a long-term bridge to transplantation in a Fontan patient with failing single ventricle VL - 19 ID - 421 ER - TY - JOUR AB - Catel-Manzke syndrome is characterized by the combination of Pierre Robin sequence and radial deviation, shortening as well as clinodactyly of the index fingers, due to an accessory ossification center. Mutations in TGDS have been identified as one cause of Catel-Manzke syndrome, but cannot be found as causative in every patient with the clinical diagnosis. We performed a chromosome microarray and/or exome sequencing in three patients with hand hyperphalangism, heart defect, short stature, and mild to severe developmental delay, all of whom were initially given a clinical diagnosis of Catel-Manzke syndrome. In one patient, we detected a large deletion of exons 1–8 and the missense variant c.1282C > T (p.Arg428Trp) in KYNU (NM_003937.2), whereas homozygous missense variants in KYNU were found in the other two patients (c.989G > A (p.Arg330Gln) and c.326G > C (p.Trp109Ser)). Plasma and urine metabolomic analysis of two patients indicated a block along the tryptophan catabolic pathway and urine organic acid analysis showed excretion of xanthurenic acid. Biallelic loss-of-function mutations in KYNU were recently described as a cause of NAD deficiency with vertebral, cardiac, renal and limb defects; however, no hand hyperphalangism was described in those patients, and Catel-Manzke syndrome was not discussed as a differential diagnosis. In conclusion, we present unrelated patients identified with biallelic variants in KYNU leading to kynureninase deficiency and xanthurenic aciduria as a very likely cause of their hyperphalangism, heart defect, short stature, and developmental delay. We suggest performance of urine organic acid analysis in patients with suspected Catel-Manzke syndrome, particularly in those with cardiac or vertebral defects or without mutations in TGDS. AD - N. Ehmke, Institute for Human Genetics and Medical Genetics, Charité-Universitaetsmedizin Berlin, Augustenburger Platz 1, Berlin, Germany AU - Ehmke, N. AU - Cusmano-Ozog, K. AU - Koenig, R. AU - Holtgrewe, M. AU - Nur, B. AU - Mihci, E. AU - Babcock, H. AU - Gonzaga-Jauregui, C. AU - Overton, J. D. AU - Xiao, J. AU - Martinez, A. F. AU - Muenke, M. AU - Balzer, A. AU - Jochim, J. AU - El Choubassi, N. AU - Fischer-Zirnsak, B. AU - Huber, C. AU - Kornak, U. AU - Elsea, S. H. AU - Cormier-Daire, V. AU - Ferreira, C. R. DB - Embase Medline DO - 10.1016/j.bone.2019.115219 KW - computed tomography scanner extracorporeal membrane oxygenation device percutaneous endoscopic gastrostomy tube carboxylic acid kynureninase levetiracetam tryptophan valproic acid xanthurenic acid aciduria adolescent amniocentesis article attention deficit disorder blood level bradycardia brain radiography Caribbean (person) case report catabolism Catel Manzke syndrome child chromosome analysis clinical article clinical feature clinodactyly cochlea hypoplasia cognitive defect complete heart block computer assisted tomography developmental delay disease severity dysostosis echography electroencephalogram enzyme deficiency evoked brain stem auditory response exon extracorporeal oxygenation face dysmorphia Fallot tetralogy family history feeding difficulty female Fontan procedure gene deletion genetic variability gestational age hand hyperphalangism hand malformation hand radiography heart atrium septum defect heart disease heart failure heart ventricle septum defect hepatomegaly homozygosity human hypoplasia hypoplastic left heart syndrome joint laxity kidney agenesis KYNU gene learning disorder Lennox Gastaut syndrome loss of function mutation male medical history mental performance metabolome metabolomics microarray analysis microcephaly missense mutation motor dysfunction muscle hypotonia occupational therapy patient referral perception deafness physical examination physiotherapy renography retrognathia scoliosis short stature speech delay speech therapy systemic disease tonic seizure Turk (people) urine level vaginal delivery whole exome sequencing Wide Range Achievement Test X ray xanthurenic aciduria LA - English M3 - Article N1 - L2004748692 2020-01-31 2020-02-11 PY - 2020 SN - 8756-3282 ST - Biallelic variants in KYNU cause a multisystemic syndrome with hand hyperphalangism T2 - Bone TI - Biallelic variants in KYNU cause a multisystemic syndrome with hand hyperphalangism UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2004748692 http://dx.doi.org/10.1016/j.bone.2019.115219 VL - 133 ID - 557 ER - TY - JOUR AB - The Fontan operation is considered a palliative procedure for the treatment of complex congenital heart diseases with a morbidity and mortality that are not negligible, especially in high-risk patients. In these cases, a bidirectional cavopulmonary anastomosis with additional source of blood flow to the lungs can represent a valuable surgical option. Between November 1992 and December 1995, 21 patients with univentricular heart physiology were submitted to bidirectional cavopulmonary anastomosis with additional blood flow the additional flow consisted of an aortopulmonary shunt in 13 cases, pulmonary artery banding in seven cases, and pulmonary artery stenosis in one case. Mean age at operation was 35.5 months (range 4 months to 12 years) and mean body weight 10.9 (range 4-24.4) kg. Hospital mortality was zero. Morbidity (pleuropericardial effusions) was significant in one case. There were three late deaths (14%) caused by worsening atrioventricular valve regurgitation: two of these occurred in patients with right isomerism. Late haemodynamic evaluation documented a significant increase in cardiac output (P < 0.01) and oxygen saturation (P < 0.05) during pharmacological stress testing. Angiographic characterization of blood flow distribution to the lungs showed better results when the additional blood flow was represented by an anterograde source (pulmonary artery banding or native pulmonary artery stenosis). In conclusion, bidrectional cavopulmonary anastomosis with additional blood flow is a good alternative to classic or fenestrated Fontan technique in high-risk patients. Secondly, the diagnosis of isomerism could be a contraindication to this type of surgery, because the persistent volume overload could worsen atrioventricular valve regurgitation. Thirdly, the pharmacological stress testing shows a favourable behaviour of cardiac output and arterial oxygen saturation. Fourth, the type of additional blood flow seems to condition both pulsatility and distribution of pulmonary blood flow. AD - Department of Cardiology, Gaslini Pediatric Institute, Genoa, Italy. AN - 9423944 AU - Borini, I. AU - Marasini, M. AU - Dalmonte, P. AU - Bava, G. L. AU - Lerzo, F. AU - Cervo, G. AU - Moretti, R. AU - Bertolini, A. AU - Zannini, L. DA - Dec DB - PubMed DO - 10.1016/s0967-2109(97)00077-x DP - NLM ET - 1998/01/10 IS - 6 KW - Child Child, Preschool Heart Bypass, Right/*methods Heart Defects, Congenital/physiopathology/*surgery Hemodynamics Humans Infant Lung/*blood supply Regional Blood Flow Retrospective Studies Treatment Outcome LA - eng N1 - Borini, I Marasini, M Dalmonte, P Bava, G L Lerzo, F Cervo, G Moretti, R Bertolini, A Zannini, L Journal Article England Cardiovasc Surg. 1997 Dec;5(6):588-92. doi: 10.1016/s0967-2109(97)00077-x. PY - 1997 SN - 0967-2109 (Print) 0967-2109 SP - 588-92 ST - Bidirectional cavo-pulmonary anastomosis with an additional flow source to the lungs: clinical experience in 21 cases T2 - Cardiovasc Surg TI - Bidirectional cavo-pulmonary anastomosis with an additional flow source to the lungs: clinical experience in 21 cases VL - 5 ID - 464 ER - TY - JOUR AB - The bidirectional cavopulmonary anastomosis is a surgical procedure suitable for patients with cyanotic congenital heart disease and univentricular physiology. This operation is able to increase the effective pulmonary blood flow without any additional load on the cardiac work and without any further distortion on the pulmonary artery branches. The cavopulmonary anastomosis can represent the first stage for patients destined for Fontan repair or a definitive palliative operation in high risk Fontan candidates. In order to test the hypothesis of a definitive palliation by cavopulmonary anastomosis in this kind of patients, we evaluated the hemodynamic data before and after this surgical approach and compared these data with their clinical and functional outcome. We evaluated 74 patients submitted to bidirectional cavopulmonary anastomosis by either hemodynamic or functional evaluation. End-diastolic and end-systolic ventricular volumes were significantly reduced by bidirectional cavopulmonary anastomosis (p less than 0.0005). Despite these data and a normal ambulatory ECG, spirometry and echocardiographic analysis, the stress test showed discouraging results. In fact, mean work time and peak heart rate were significantly different from normal values showing an impaired functional capacity of these children. In conclusion we think that bidirectional cavopulmonary anastomosis can not be considered an adequate definitive palliation but it represents a very good stage to preserve the pulmonary arteries and to prepare the systemic ventricle towards the Fontan repair. AD - Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesu' Hospital, Rome, Italy. AN - 1383112 AU - Giannico, S. AU - Santoro, G. AU - Marino, B. AU - Cicini, M. P. AU - Marcelletti, C. DA - Aug DB - PubMed DP - NLM ET - 1992/08/01 IS - 4 KW - Adolescent Anastomosis, Surgical/*methods Aorta/surgery Blood Vessel Prosthesis Child Child, Preschool Echocardiography Exercise Test Female Follow-Up Studies Heart Defects, Congenital/physiopathology/*surgery Hemodynamics/*physiology Humans Infant Infant, Newborn Male Myocardial Contraction/physiology Palliative Care/*methods Postoperative Complications/*physiopathology Pulmonary Artery/*surgery Pulmonary Circulation/physiology Pulmonary Wedge Pressure/physiology Vena Cava, Superior/*surgery LA - eng N1 - Giannico, S Santoro, G Marino, B Cicini, M P Marcelletti, C Journal Article Germany Herz. 1992 Aug;17(4):234-41. PY - 1992 SN - 0340-9937 (Print) 0340-9937 SP - 234-41 ST - Bidirectional cavopulmonary anastomosis in congenital heart disease. Functional and clinical outcome T2 - Herz TI - Bidirectional cavopulmonary anastomosis in congenital heart disease. Functional and clinical outcome VL - 17 ID - 482 ER - TY - JOUR AB - We present our experience with bilateral branch pulmonary artery banding as a bridge to decision/optimization of hemodynamics, followed by standard Norwood stage I palliation in very high-risk infants with hypoplastic left heart syndrome. © The Author(s) 2012. AD - P. Sinha, Department of Cardiac Surgery, Children's National Medical Center, 111 Michigan Avenue NW., Washington, DC 20010, United States AU - Kurkluoglu, M. AU - Jonas, R. A. AU - Sinha, P. DB - Embase Medline DO - 10.1177/2150135112473615 IS - 2 KW - angioplasty article bilateral branch pulmonary artery banding blalock hanlon operation cardiovascular stent cardiovascular surgery case report clinical decision making clinical feature congestive heart failure extracorporeal oxygenation female high risk patient human hypoplastic left heart syndrome infant lung artery banding male Norwood procedure priority journal resuscitation risk reduction treatment outcome LA - English M3 - Article N1 - L369378081 2013-07-30 2020-04-07 PY - 2013 SN - 2150-136X 2150-1351 SP - 227-228 ST - Bilateral branch pulmonary artery banding as a bridge to decision/ preoperative optimization of high-risk neonates with hypoplastic left heart syndrome T2 - World Journal for Pediatric and Congenital Hearth Surgery TI - Bilateral branch pulmonary artery banding as a bridge to decision/ preoperative optimization of high-risk neonates with hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L369378081 http://dx.doi.org/10.1177/2150135112473615 VL - 4 ID - 1027 ER - TY - JOUR AB - Background/Purpose: Birth defects (BDs) are main causes of mortality and disability in infants and children. The aims of this study were to analyze the prevalence, types and risk factors of BDs in Taiwan. Methods: Data of all births (including live and stillbirths), types, characteristics, and associated risk factors of BDs were obtained from the National Birth Registry and National Health Insurance Research Data base in Taiwan between 2005 and 2014. Birth defects were coded according to International Classification of Diseases 9th Revision-Clinical Modification codes 740–759. Results: A total of 55,299 infants were diagnosed as having BDs among 2,033,004 births. The prevalence of BDs was 271.66 per 10,000 births. The prevalence of BDs did not change significantly between 2005 and 2014, there was a higher birth rate and lower BDs in 2012 (year of dragon) in Taiwan. The most common type of BDs was cardiovascular abnormalities, and ventricular septal defect was the most common disease. Extreme maternal age (<18 years or ≧30 years), preterm, and low birth weight were associated with BDs. Maternal diseases associated with BDs included hypertension, cardiovascular diseases, renal diseases, genitourinary infections, anemia, mental disorders, and diabetes mellitus. Conclusion: The prevalence of BDs was 271.66 per 10,000 births. The most common types of BDs were cardiovascular abnormalities. If we can reduce maternal chronic diseases, we will decrease the prevalence of BDs. AD - J.-Y. Chen, Division of Neonatology, Changhua Christian Children's Hospital, No. 320, Xuguang Road, Changhua City, Changhua County, Taiwan AU - Chen, L. J. AU - Chiou, J. Y. AU - Huang, J. Y. AU - Su, P. H. AU - Chen, J. Y. DB - Embase Medline DO - 10.1016/j.jfma.2019.08.006 IS - 1P3 KW - achondroplasia anemia anencephalus anophthalmia anus atresia aortic arch interruption aortic coarctation aortic valve stenosis article atrioventricular septal defect attributable risk bile duct atresia birth weight bladder exstrophy cardiovascular disease cardiovascular malformation choana atresia chromosome aberration chronic disease cleft lip with or without cleft palate cleft palate clubfoot cohort analysis common bile duct cyst congenital cataract congenital diaphragm hernia congenital heart disease congenital hip dislocation congenital hydrocephalus congenital malformation congenital skin disease cryptorchism cystic adenomatoid malformation encephalocele epispadias esophagus atresia Fallot tetralogy female gastroschisis gestational age great vessels transposition heart atrium septum defect heart right ventricle double outlet heart single ventricle heart ventricle septum defect hermaphroditism Hirschsprung disease holoprosencephaly human hydronephrosis hypoplastic left heart syndrome hypospadias ICD-9-CM intestine atresia karyotype 47,XXY kidney agenesis kidney disease kidney polycystic disease laryngomalacia limb defect live birth low birth weight lung agenesis lung sequestration lung vein drainage anomaly major clinical study male maternal age maternal diabetes mellitus maternal disease maternal hypertension meningomyelocele mental disease microcephaly microphthalmia multicystic dysplastic kidney newborn omphalocele patent ductus arteriosus polydactyly population research prematurity prevalence pulmonary valve atresia pulmonary valve stenosis pylorus stenosis risk factor spinal dysraphism stillbirth syndactyly Taiwan tracheoesophageal fistula tracheomalacia tricuspid valve atresia tricuspid valve stenosis trisomy 13 trisomy 18 trisomy 21 Turner syndrome urinary tract obstruction urogenital tract infection LA - English M3 - Article N1 - L2002740551 2019-09-04 2020-02-04 PY - 2020 SN - 1876-0821 0929-6646 SP - 553-559 ST - Birth defects in Taiwan: A 10-year nationwide population-based, cohort study T2 - Journal of the Formosan Medical Association TI - Birth defects in Taiwan: A 10-year nationwide population-based, cohort study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2002740551 http://dx.doi.org/10.1016/j.jfma.2019.08.006 VL - 119 ID - 568 ER - TY - JOUR AB - Surgical decision in mild forms of hypoplastic left heart syndrome can be challenging. Once a univentricular pathway has been chosen, it can be difficult to reconsider a biventricular repair. A commitment to a palliative pathway is usually considered irreversible after initial univentricular repair. We present this case as an example in which the primary surgical palliation pathway was altered, and eventually a successful biventricular repair was performed in a mild variant of hypoplastic left heart syndrome, despite the fact that maneuvers to promote left ventricular growth were not recruited at the time of initial surgery. © 2013 by The Society of Thoracic Published by Elsevier Inc. AD - Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, United States Division of Cardiovascular Surgery, Mayo Clinic, Rochester, MN, United States AU - Qureshi, M. Y. AU - Burkhart, H. M. AU - Hagler, D. J. DB - Scopus DO - 10.1016/j.athoracsur.2013.04.138 IS - 5 M3 - Article N1 - Export Date: 15 June 2020 PY - 2013 SP - e119-e120 ST - Biventricular repair after stage II univentricular surgery: Palliation is not a one-way path T2 - Annals of Thoracic Surgery TI - Biventricular repair after stage II univentricular surgery: Palliation is not a one-way path UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84887054047&doi=10.1016%2fj.athoracsur.2013.04.138&partnerID=40&md5=95be316c2e5f8acdac53839af6f0159f VL - 96 ID - 2031 ER - TY - JOUR AB - There is a rapid growth of interest in heart transplantation therapy during early infancy. From 10% to 25% of the infants who are listed for transplantation annually have died while awaiting a donor heart. There has been no significant trend in this variable. Since November 1985, 140 consecutive orthotopic transplantation procedures were performed in 139 infants who were from 3 hours to 12 months of age. Indications for transplantation included hypoplastic left heart syndrome (63%), other complex structural anomalies (29%), myopathy (6.5%), and tumors (1.5%). Most recipients had ductus-dependent circulation and received continuous infusion of prostaglandin E1. Heart donors were usually victims of trauma, sudden infant death, or birth asphyxia. A donor-recipient weight ratio of 4.0 or less was found to be acceptable. The amount of time the graft underwent cold ischemia, ranged from 64 to 576 minutes. The procurement process was facilitated by a single dose of cold crystalloid cardioplegic solution and cold immersion transport. Profound hypothermic circulatory arrest was used for graft implantation. One hundred twenty-four (89%) recipients survived transplantation and were discharged from the hospital. There were 9 late deaths, which resulted in an 83% overall survival. The 5-year actuarial survival is 80%. The survival among newborn recipients (n = 60) at 5 years is 84%. Chronic immunomodulation was cyclosporine-based and steroid-free. Surveillance was noninvasive and relied heavily on echocardiography, electrocardiography, and clinical intuition. There was one documented late lethal infection, tumor was not encountered, and coronary occlusive disease was known to exist in only one long-term survivor. We concluded that transplantation results in excellent life quality and is a highly effective and durable therapy when applied during early infancy. AD - L.L. Bailey, Division of Cardiothoracic Surgery, Loma Linda University Medical Center, Loma Linda, CA 92354, United States AU - Bailey, L. L. AU - Gundry, S. R. AU - Razzouk, A. J. AU - Wang, N. AU - Sciolaro, C. M. AU - Chiavarelli, M. AU - Branson, B. AU - Zorn, E. AU - Eby, W. AU - Allard, M. AU - Applegate, R. AU - Brauer, S. AU - Lau, C. AU - Martin, R. AU - Lebeck, L. AU - Nehlsen-Cannarella, S. AU - Boucek, M. AU - Kanakriyeh, M. AU - Larson, R. DB - Embase Medline IS - 5 KW - cyclosporine prostaglandin E1 article graft rejection heart transplantation human hypoplastic left heart syndrome infant major clinical study newborn organ donor pediatric surgery priority journal quality of life survival time LA - English M3 - Article N1 - L23151811 1993-06-07 PY - 1993 SN - 0022-5223 SP - 805-815 ST - Bless the babies: One hundred fifteen late survivors of heart transplantation during the first year of life T2 - Journal of Thoracic and Cardiovascular Surgery TI - Bless the babies: One hundred fifteen late survivors of heart transplantation during the first year of life UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L23151811 VL - 105 ID - 1379 ER - TY - JOUR AB - There is a rapid growth of interest in heart transplantation therapy during early infancy. From 10% to 25% of the infants who are listed for transplantation annually have died while awaiting a donor heart. There has been no significant trend in this variable. Since November 1985, 140 consecutive orthotopic transplantation procedures were performed in 139 infants who were from 3 hours to 12 months of age. Indications for transplantation included hypoplastic left heart syndrome (63%), other complex structural anomalies (29%), myopathy (6.5%), and tumors (1.5%). Most recipients had ductus-dependent circulation and received continuous infusion of prostaglandin E1. Heart donors were usually victims of trauma, sudden infant death, or birth asphyxia. A donor-recipient weight ratio of 4.0 or less was found to be acceptable. The amount of time the graft underwent cold ischemia, ranged from 64 to 576 minutes. The procurement process was facilitated by a single dose of cold crystalloid cardioplegic solution and cold immersion transport. Profound hypothermic circulatory arrest was used for graft implantation. One hundred twenty-four (89%) recipients survived transplantation and were discharged from the hospital. There were 9 late deaths, which resulted in an 83% overall survival. The 5-year actuarial survival is 80%. The survival among newborn recipients (n = 60) at 5 years is 84%. Chronic immunomodulation was cyclosporine-based and steroid-free. Surveillance was noninvasive and relied heavily on echocardiography, electrocardiography, and clinical intuition. There was one documented late lethal infection, tumor was not encountered, and coronary occlusive disease was known to exist in only one long-term survivor. We concluded that transplantation results in excellent life quality and is a highly effective and durable therapy when applied during early infancy. AD - Department of Surgery, Loma Linda University and Medical Center, CA 92354. AN - 8487560 AU - Bailey, L. L. AU - Gundry, S. R. AU - Razzouk, A. J. AU - Wang, N. AU - Sciolaro, C. M. AU - Chiavarelli, M. DA - May DB - PubMed DP - NLM ET - 1993/05/01 IS - 5 KW - Actuarial Analysis Female Graft Rejection/epidemiology/prevention & control Heart Defects, Congenital/*surgery Heart Transplantation/methods/*mortality Humans Immunosuppression/methods Immunosuppressive Agents/therapeutic use Infant Infant, Newborn Male Survival Rate Time Factors Tissue and Organ Procurement LA - eng N1 - Bailey, L L Gundry, S R Razzouk, A J Wang, N Sciolaro, C M Chiavarelli, M Journal Article United States J Thorac Cardiovasc Surg. 1993 May;105(5):805-14; discussion 814-5. PY - 1993 SN - 0022-5223 (Print) 0022-5223 SP - 805-14; discussion 814-5 ST - Bless the babies: one hundred fifteen late survivors of heart transplantation during the first year of life. The Loma Linda University Pediatric Heart Transplant Group T2 - J Thorac Cardiovasc Surg TI - Bless the babies: one hundred fifteen late survivors of heart transplantation during the first year of life. The Loma Linda University Pediatric Heart Transplant Group VL - 105 ID - 535 ER - TY - JOUR AB - Cyanosis is observed in patients with complex congenital heart disease (CHD) and pulmonary hypertension, heart failure represents an important clinical problem in such patients. The aim of this study was to evaluate the exercise capacity in patients with cyanotic CHDs using cardiopulmonary exercise test, measuring serum BNP levels as well as to seek correlation between BNP levels and cardiopulmonary exercise test parameters and identify the effects of blood oxygen desaturation and pulmonary hypertension on these indices. The study group consisted of 53 patients (21 males) at the mean age of 39.4±14.3 years, of whom 19 were operated on at the mean age of 9.6±8.6 years. Mean blood oxygen saturation (SO2) in patients was 81.2±6.2%. Twenty four patients presented with Eisenmenger syndrome, 16-univentricular hearts, 4-transposition of the great arteries, 6-Fallot's tetralogy, and 3-Ebstein anomaly. The control group comprised 32 healthy individuals (16 males) at the mean age of 40.7±9.9 years. Cardiopulmonary stress test showed significantly lower exercise capacity in patients with cyanosis than in controls: maximal oxygen uptake (VO2max) 15.5±4.9 vs. 31.6±7.1 ml/kg/min (p=0.00001), maximum heart rate at peak exercise (HR max): 139.5±22.5 bpm vs. 176.6±12.1 (p=0.0001), VE/VCO2 slope: 46.4±10.1 vs. 27.3±2.9 (p=0.00001), forced vital capacity FVC: 3.1±1.1 l vs. 4.4±0.8 l (p=0.00001). Subjects with the evidence of pulmonary hypertension (PH+) had lower exercise capacity than those without (PH-): VO2max: 17.2±4.2 vs. 12.8±4.8 ml/kg/min (p=0.002), VE/VCO2: 43.7±11.1 vs. 50.9±6.4 (p=0.01), FVC: 3.46±1.05 l vs. 2.37±0.91 l (p=0.0002). Plasma BNP levels in the study group were higher than in controls: 122.4±106.7 vs. 21.1±20.2 pg/ml p=0.00001 and did not differ between PH+ and PH- groups (115.7±99.0 vs. 127.9±114.1 pg/ ml p=0.78). Negative correlations between BNP levels and VO2max (r=-0.389, p=0.006), FVC (r=-0.395 p=0.005), FEV1 (r=-0.386 p=0.006), SO2 (r=-0.445 p=0.00001), and positive correlation between BNP level and VE/VCO2 (r=0.369 p=0.009) were found. © 2008 Published by Elsevier Ireland Ltd. AD - Department of Cardiology, University of Medical Science, Poznan, Poland Department of Cardiac Surgery, University of Medical Science, Poznan, Poland Department of Clinical Pharmacology, University of Medical Science, Poznan, Poland Department of Computer Sciences and Statistics, University of Medical Science, Poznan, Poland AU - Trojnarska, O. AU - Gwizdala, A. AU - Katarzynski, S. AU - Katarzynska, A. AU - Oko-Sarnowska, Z. AU - Grajek, S. AU - Kramer, L. DB - Scopus DO - 10.1016/j.ijcard.2008.10.025 IS - 3 KW - BNP level in adult patients with congenital heart disease Cardiopulmonary exercise test Heart failure M3 - Article N1 - Cited By :21 Export Date: 15 June 2020 PY - 2010 SP - 241-247 ST - The BNP concentrations and exercise capacity assessment with cardiopulmonary stress test in cyanotic adult patients with congenital heart diseases T2 - International Journal of Cardiology TI - The BNP concentrations and exercise capacity assessment with cardiopulmonary stress test in cyanotic adult patients with congenital heart diseases UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-80052525864&doi=10.1016%2fj.ijcard.2008.10.025&partnerID=40&md5=a6d7abd02eaf08d35b9d82638bee4dc1 VL - 139 ID - 2155 ER - TY - JOUR AB - BACKGROUND: The Fontan procedure has improved survival in children with functionally univentricular hearts. With time, however, complications such as reduced exercise capacity are seen more frequently. Exercise intolerance is multifactorial, but pulmonary vascular resistance probably plays a crucial role. Elevated pulmonary vascular resistance has been associated with raised levels of endothelin-1, which are common both before and after Fontan operations. Treatment with endothelin-1 receptor antagonists could theoretically improve cardiopulmonary hemodynamics and exercise capacity. The aim of this study was therefore to examine the efficacy and safety of bosentan in Fontan patients. METHODS AND RESULTS: Seventy-five adolescents and adults were randomized 1:1 to 14 weeks of treatment with bosentan or placebo. Cardiopulmonary exercise test, functional class, blood samples, and quality-of-life questionnaires were evaluated at baseline and at the end of treatment. Sixty-nine patients (92%) completed the study. Peak oxygen consumption increased 2.0 mL·kg(-1)·min(-1) (from 28.7 to 30.7 mL·kg(-1)·min(-1)) in the bosentan group compared with 0.6 mL·kg(-1)·min(-1) (from 28.4 to 29.0 mL·kg(-1)·min(-1)) in the placebo group (P=0.02). Cardiopulmonary exercise test time increased by 0.48 minute (from 6.79 to 7.27 minutes) versus 0.08 minute (from 6.94 to 7.02 minutes; P=0.04). Nine bosentan-treated patients improved 1 functional class, whereas none improved in the placebo group (P=0.0085). Side effects were mild and occurred equally in both groups. No serious adverse effects were seen, and no patients had liver enzyme levels above the 3-fold upper limit. CONCLUSIONS: Bosentan improves exercise capacity, exercise time, and functional class in Fontan patients without serious adverse events or hepatotoxicity. CLINICAL TRIAL REGISTRATION URL: http://www.clinicaltrials.gov. Unique identifier: NCT01292551. AD - From the Department of Cardiology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark (A.H., L.I., A.S.J., L.S.); Institute of Sports Medicine, Department of Orthopedic Surgery, Bispebjerg Hospital and Center for Healthy Ageing, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark (U.R.M.); Departments of Cardiology (U.T.) and Pediatric Cardiology (K.H.), Lund University Hospital, Lund, Sweden; Department of Cardiology/Department of Medicine, Karolinska University Hospital, Karolinska Institutet, Stockholm, Sweden (S.N.); and Department of Cardiology, Skejby, Aarhus University Hospital, Aarhus, Denmark (K.E.S.). andershebert@gmail.com. From the Department of Cardiology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark (A.H., L.I., A.S.J., L.S.); Institute of Sports Medicine, Department of Orthopedic Surgery, Bispebjerg Hospital and Center for Healthy Ageing, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark (U.R.M.); Departments of Cardiology (U.T.) and Pediatric Cardiology (K.H.), Lund University Hospital, Lund, Sweden; Department of Cardiology/Department of Medicine, Karolinska University Hospital, Karolinska Institutet, Stockholm, Sweden (S.N.); and Department of Cardiology, Skejby, Aarhus University Hospital, Aarhus, Denmark (K.E.S.). AN - 25446057 AU - Hebert, A. AU - Mikkelsen, U. R. AU - Thilen, U. AU - Idorn, L. AU - Jensen, A. S. AU - Nagy, E. AU - Hanseus, K. AU - Sørensen, K. E. AU - Søndergaard, L. DA - Dec 2 DB - PubMed DO - 10.1161/circulationaha.113.008441 DP - NLM ET - 2014/12/03 IS - 23 KW - Adolescent Adult Bosentan Child Child, Preschool Double-Blind Method Endothelin Receptor Antagonists/*administration & dosage/adverse effects Exercise Tolerance/*drug effects Female Fontan Procedure/*adverse effects Hemodynamics Humans Infant Male Oxygen Consumption/*drug effects Placebos Postoperative Complications/*drug therapy Receptor, Endothelin A/blood Statistics, Nonparametric Sulfonamides/*administration & dosage/adverse effects Treatment Outcome Young Adult Fontan procedure exercise test heart defects, congenital vasodilation LA - eng N1 - 1524-4539 Hebert, Anders Mikkelsen, Ulla R Thilen, Ulf Idorn, Lars Jensen, Annette S Nagy, Edit Hanseus, Katarina Sørensen, Keld E Søndergaard, Lars Journal Article Randomized Controlled Trial Research Support, Non-U.S. Gov't United States Circulation. 2014 Dec 2;130(23):2021-30. doi: 10.1161/CIRCULATIONAHA.113.008441. Epub 2014 Oct 20. PY - 2014 SN - 0009-7322 SP - 2021-30 ST - Bosentan improves exercise capacity in adolescents and adults after Fontan operation: the TEMPO (Treatment With Endothelin Receptor Antagonist in Fontan Patients, a Randomized, Placebo-Controlled, Double-Blind Study Measuring Peak Oxygen Consumption) study T2 - Circulation TI - Bosentan improves exercise capacity in adolescents and adults after Fontan operation: the TEMPO (Treatment With Endothelin Receptor Antagonist in Fontan Patients, a Randomized, Placebo-Controlled, Double-Blind Study Measuring Peak Oxygen Consumption) study VL - 130 ID - 325 ER - TY - JOUR AB - BACKGROUND: Single ventricle heart disease (SVHD) adolescents show cognitive impairments and anxiety and depressive symptoms, indicating the possibility of brain injury in regions that control these functions. However, brain tissue integrity in cognition, anxiety, and depression regulatory sites in SVHD remains unclear. We examined brain tissue changes in SVHD compared to controls using T2-relaxometry procedures, which measure free water content and show tissue injury. METHODS: Proton-density and T2-weighted images, using a 3.0-Tesla MRI, as well as anxiety (Beck anxiety inventory [BAI]), depressive symptoms (patient health questionnaire-9 [PHQ-9]), and cognition (wide range assessment of memory and learning 2 [WRAML2] and Montreal cognitive assessment [MoCA]) data were collected from 20 SVHD (age: 15.8 ± 1.1 years, male/female: 11/9) and 36 controls (age: 16.0 ± 1.1 years, male/female: 19/17). Whole-brain T2-relaxation maps were calculated, normalized to a common space, smoothed, and compared between groups and sexes (analysis of covariance; covariates: age, sex; p < 0.001). RESULTS: SVHD subjects showed significantly increased BAI and PHQ-9 and reduced MoCA and WRAML2 scores over controls. Several brain regions in SVHD showed increased T2-relaxation values (chronic injury), including the cingulate, and insula, hippocampus/para-hippocampal gyrus, thalamus, hypothalamus, amygdala, frontal white matter, corpus callosum, brainstem, and cerebellar areas. Decreased T2-relaxation values (acute injury) emerged in a few regions, including the prefrontal and cerebellar cortices in SVHD over controls. In addition, male SVHD showed more brain changes over female SVHD. CONCLUSIONS: Adolescents with SVHD showed significant brain injury with variable male-female differences in areas that control cognition, anxiety, and depression, which may contribute to functional deficits found in the condition. AD - UCLA School of Nursing, University of California, Los Angeles, CA, 90095, USA. Department of Anesthesiology, University of California, Los Angeles, CA, USA. Division of Pediatric Cardiology, University of California, Los Angeles, CA, 90095, USA. Division of Pediatric Cardiology, Children's Hospital Los Angeles, CA, 90027, USA. Department of Radiological Sciences, University of California, Los Angeles, CA, USA. Department of Bioengineering, University of California, Los Angeles, CA, USA. Brain Research Institute, University of California, Los Angeles, CA, USA. AN - 29315714 AU - Pike, N. A. AU - Roy, B. AU - Gupta, R. AU - Singh, S. AU - Woo, M. A. AU - Halnon, N. J. AU - Lewis, A. B. AU - Kumar, R. C2 - PMC6103299 C6 - NIHMS984925 DA - Jun DB - PubMed DO - 10.1002/jnr.24215 DP - NLM ET - 2018/01/10 IS - 6 KW - Adolescent Anxiety/diagnostic imaging/*etiology Brain Injuries/*diagnostic imaging/*etiology Cognitive Dysfunction/diagnostic imaging/*etiology Cross-Sectional Studies Depression/diagnostic imaging/*etiology Female Heart Diseases/*pathology/*psychology Humans Male Sex Factors *T2-relaxometry *cingulate *congenital heart disease *hippocampus *insula *magnetic resonance imaging LA - eng N1 - 1097-4547 Pike, Nancy A Roy, Bhaswati Gupta, Ritika Singh, Sadhana Woo, Mary A Halnon, Nancy J Lewis, Alan B Kumar, Rajesh Orcid: 0000-0001-5355-9586 R01 NR013930/NR/NINR NIH HHS/United States R01 NR016463/NR/NINR NIH HHS/United States Comparative Study Journal Article Research Support, N.I.H., Extramural J Neurosci Res. 2018 Jun;96(6):1104-1118. doi: 10.1002/jnr.24215. Epub 2018 Jan 6. PY - 2018 SN - 0360-4012 (Print) 0360-4012 SP - 1104-1118 ST - Brain abnormalities in cognition, anxiety, and depression regulatory regions in adolescents with single ventricle heart disease T2 - J Neurosci Res TI - Brain abnormalities in cognition, anxiety, and depression regulatory regions in adolescents with single ventricle heart disease VL - 96 ID - 66 ER - TY - JOUR AB - Objectives: To evaluate the financial burden and outcome of the children with brain abscess and underlying congenital cyanotic heart disease. To emphasize the need of early diagnosis and surgery of congenital cyanotic heart disease. Study Design: Cross Sectional Descriptive Study. Place and Duration of Study: This study was conducted at the department of paediatric cardiology The Children's Hospital and The Institute of Child Health, Multan from February 2010 to January 2012. Patients and Methods: All the patients presenting with brain abscess and having underlying congenital heart disease were included in the study. All the patients underwent transthoracic echocardiography and computed tomography (CT) scan of the brain. Data was taken on a written proforma after proper consent. Results: A total of 58 patients with cyanotic congenital heart disease presented with CNS manifestations including headache, fever, focal neurological signs, fits or altered sensorium during the study period. Out of these 24 turned out to have brain abscess. Multiple brain abscesses were found in 7 patients. All the patients were given broad spectrum IV antibiotics. 19 of the patients required surgical intervention. Repeat CT scan was performed in all the patients. Only 5 patients recovered without any neurological sequalae.7 patients expired during hospital stay. All of the remaining 12 patients survived but with some permanent neurological deficit.IV antibiotics, supportive treatment, CT scan, other laboratory investigations and surgery, all have a lot of cost. Around 1500 to 2000 US $, on an average, were spent by the government, on one patient during hospital stay. Conclusion: The only way to avoid CNS complications of CCHD is early diagnosis and early surgical treatment. But unfortunately, in this country because of lake of paediatric cardiology services and deficiency of paediatric cardiac surgery centers, corrective surgery of congenital cyanotic heart disease is delayed. It increases the risk of brain abscess formation which in turn leads to excessive financial burden on government resources and also increases the risk of long term neurological sequalae. Establishment of more and more paediatric cardiac surgery centers in this country is urgently required. AD - M.S. Arshad, Department of Paediatric Cardiology, Children's Hospital, Institute of Child Health, Abdali Road, Multan, Pakistan AU - Arshad, M. S. AU - Aslam, M. AU - Rubab, S. DB - Embase IS - 9 KW - ceftriaxone metronidazole vancomycin adolescent antibiotic therapy aphasia article blindness brain abscess child computer assisted tomography craniotomy cross-sectional study cyanotic heart disease descriptive research early diagnosis facial nerve paralysis Fallot tetralogy fever finance government great vessels transposition headache health care cost heart ventricle septum defect hemiplegia hospitalization human major clinical study neuroimaging outcome assessment preschool child pulmonary valve atresia pulmonary valve stenosis school child sensory system transthoracic echocardiography treatment response tricuspid valve atresia LA - English M3 - Article N1 - L365895250 2012-10-31 2012-11-08 PY - 2012 SN - 1029-385X SP - 65-68 ST - Brain abscess in cyanotic congenital heart disease: A preventable complication with heavy financial burden and poor outcome T2 - Medical Forum Monthly TI - Brain abscess in cyanotic congenital heart disease: A preventable complication with heavy financial burden and poor outcome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L365895250 VL - 23 ID - 1042 ER - TY - JOUR AB - It is every parent's worst nightmare. You have just given birth, and doctors tell you that your child has been born with a major congenital abnormality. A midwife tells her story. AD - Lecturer in Midwifery, School of Nursing and Midwifery, University of East Anglia AN - 106655266. Language: English. Entry Date: 20041029. Revision Date: 20150818. Publication Type: Journal Article AU - Williamson, A. DB - ccm DP - EBSCOhost IS - 4 KW - Communication Professional-Family Relations Truth Disclosure Infant, Newborn Parental Attitudes Parents -- Psychosocial Factors Tricuspid Atresia N1 - anecdote. Journal Subset: Double Blind Peer Reviewed; Europe; Expert Peer Reviewed; Nursing; Peer Reviewed; UK & Ireland. NLM UID: 101189530. PY - 2004 SN - 1479-2915 SP - 170-171 ST - Breaking bad news: a parent's perspective T2 - RCM Midwives TI - Breaking bad news: a parent's perspective UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106655266&site=ehost-live&scope=site VL - 7 ID - 1653 ER - TY - JOUR AB - A personal narrative is presented which explores the author's experience of spending time on British National Health Service (NHS) children's wards while breastfeeding her son who had hypoplastic left heart syndrome in September 2013. AN - 99878683. Language: English. Entry Date: 20141211. Revision Date: 20190926. Publication Type: Article AU - Calvert, Helen DB - ccm DO - 10.7748/ncyp.26.10.15.s23 DP - EBSCOhost IS - 10 KW - Breast Feeding Social Attitudes Perception Lactation Consultants N1 - pictorial. Journal Subset: Core Nursing; Double Blind Peer Reviewed; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Nursing; Peer Reviewed; UK & Ireland. Special Interest: Nutrition; Obstetric Care. NLM UID: 101554473. PY - 2014 SN - 2046-2336 SP - 15-15 ST - ‘Breast isn’t best, it’s just normal’ T2 - Nursing Children & Young People TI - ‘Breast isn’t best, it’s just normal’ UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=99878683&site=ehost-live&scope=site VL - 26 ID - 1634 ER - TY - JOUR AB - Background: The Coping Health Inventory for Parents (CHIP) has demonstrated good psychometric properties in several language forms and has been used to assess the coping behaviors of families facing disease. However, the CHIP has not been validated in Mexico among families of children with chronic conditions, where it could be useful for research and intervention. The objectives of this instrumental study were to obtain a version of the CHIP for the Spanish language in Mexico, establish the factor structure of the Mexican version of the CHIP, probe its internal consistency reliability, and assess its concurrent construct validity. Methods: A nonprobability sample of 405 family caregivers of children with chronic diseases responded to a battery of measurement instruments that included the CHIP, the Beck Anxiety Inventory, and the Beck Depression Inventory. The sample was randomly divided into two parts. In one subsample (190 participants), an exploratory factor analysis was performed using a principal component analysis and oblique rotation. In the second subsample (215 participants), a confirmatory factor analysis was performed using maximum likelihood estimation. Results: The scale was reduced to 16 items (CHIP-16) with factorial loads greater than.50. The empirical criteria used to determine the number of factors converged on the following five factors: belief and trust (McDonald ω =.85), spouse/partner relationship (ω =.79), home care (ω =.77), family involvement (ω =.75), and security/stability (ω =.79). The overall internal consistency was good (ω =.88). The five-factor model showed acceptable fit indices and high parsimony. The mean CHIP-16 scores and the Spouse/partner relationship scores among the caregivers with anxiety were greater than those among the caregivers without anxiety. The mean home-care scores among the women were greater than those among men. Conclusions: The 16-item version of the CHIP showed good internal consistency and construct validity; thus, the CHIP-16 is a useful instrument for measuring and assessing coping in family caregivers of children with chronic diseases. AD - F. Toledano-Toledano, Unidad de Investigación en Medicina Basada en Evidencias, Hospital Infantil de México Federico Gómez, National Institute of Health, Dr. Márquez 162, Doctores, Cuauhtémoc, México City, Mexico AU - Toledano-Toledano, F. AU - Moral De La Rubia, J. AU - McCubbin, L. D. AU - Cauley, B. AU - Luna, D. DB - Embase Medline DO - 10.1186/s12955-020-01357-5 IS - 1 KW - adult article asthma Beck Anxiety Inventory Beck Depression Inventory caregiver child chronic disease concurrent validity confirmatory factor analysis congenital heart malformation construct validity controlled study Coping Health Inventory for Parents cystic fibrosis Down syndrome end stage renal disease exploratory factor analysis female human Human immunodeficiency virus infection internal consistency major clinical study male malignant neoplasm maximum likelihood method nephrotic syndrome oblique rotation pediatric patient principal component analysis psychometry reliability school child Spanish (language) tricuspid valve atresia LA - English M3 - Article N1 - L631627209 2020-05-13 2020-05-22 PY - 2020 SN - 1477-7525 ST - Brief version of the coping health inventory for parents (CHIP) among family caregivers of children with chronic diseases T2 - Health and Quality of Life Outcomes TI - Brief version of the coping health inventory for parents (CHIP) among family caregivers of children with chronic diseases UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L631627209 http://dx.doi.org/10.1186/s12955-020-01357-5 VL - 18 ID - 552 ER - TY - JOUR AB - OBJECTIVE: Bronchial compression as a result of pulmonary artery and aortic arch stenting may cause significant respiratory distress. We set out to limit airway narrowing by endovascular stenting, by using simultaneous flexible bronchoscopy and graduated balloon stent dilatation, or balloon angioplasty to determine maximum safe stent diameter. METHODS: Between August 2010 and August 2013, patients with suspected airway compression by adjacent vascular structures, underwent CT or a 3D rotational angiogram to evaluate the relationship between the airway and the blood vessels. If these studies showed close proximity of the stenosed vessel and the airway, simultaneous bronchoscopy and graduated stent re-dilation or graduated balloon angioplasty were performed. RESULTS: Five simultaneous bronchoscopy and interventional catheterization procedures were performed in four patients. Median age/weight was 33 (range 9-49) months and 14 (range 7.6-24) kg, respectively. Three had hypoplastic left heart syndrome, and one had coarctation of the aorta (CoA). All had confirmed or suspected left main stem bronchial compression. In three procedures, serial balloon dilatation of a previously placed stent in the CoA was performed and bronchoscopy was used to determine the safest largest diameter. In the other two procedures, balloon testing with simultaneous bronchoscopy was performed to determine the stent size that would limit compression of the adjacent airway. In all cases, simultaneous bronchoscopy allowed selection of an ideal caliber of the stent that optimized vessel diameter while minimizing compression of the adjacent airway. CONCLUSION: In cases at risk for airway compromise, flexible bronchoscopy is a useful tool to guide endovascular stenting. Maximum safe stent diameter can be determined without risking catastrophic airway compression. AD - Division of Cardiology, UCSD School of Medicine & Rady Children's Hospital, San Diego, California. AN - 25504498 AU - Ebrahim, M. AU - Hagood, J. AU - Moore, J. AU - El-Said, H. DA - Apr DB - PubMed DO - 10.1002/ccd.25772 DP - NLM ET - 2014/12/17 IS - 5 KW - Airway Obstruction/diagnosis/etiology/*prevention & control Angiography Angioplasty, Balloon/methods Aortic Coarctation/*complications/diagnosis/surgery Bronchoscopy/*methods Cardiac Surgical Procedures Child, Preschool Endovascular Procedures/*methods Female Humans Hypoplastic Left Heart Syndrome/*complications/diagnosis/surgery Imaging, Three-Dimensional Infant Male Retrospective Studies *Stents Surgery, Computer-Assisted/*methods Lpa branch pulmonary arteries bronchial compression bronchoscopy stent LA - eng N1 - 1522-726x Ebrahim, Mohammad Hagood, James Moore, John El-Said, Howaida Journal Article United States Catheter Cardiovasc Interv. 2015 Apr;85(5):832-6. doi: 10.1002/ccd.25772. Epub 2014 Dec 27. PY - 2015 SN - 1522-1946 SP - 832-6 ST - Bronchoscopic guidance of endovascular stenting limits airway compression T2 - Catheter Cardiovasc Interv TI - Bronchoscopic guidance of endovascular stenting limits airway compression VL - 85 ID - 295 ER - TY - JOUR AB - Objective: To describe the radiographic appearance of subclinical calcified brown fat necrosis and the associated clinical and laboratory findings. Materials and Methods: Picture Archiving and Communications Sytem (PACS) was searched using keywords 'soft tissue calcification' and 'chest.' The clinical record was searched for prior cardiac surgery, bypass, Extracorporeal Membrane Oxygentation (ECMO) and prostaglandin use. Age when calcifications were first detected, location, resolution, and associated laboratory abnormalities were recorded. Results: Nine patients were identified. None had skin lesions. All patients had congenital heart disease and had experienced cardiac/respiratory arrest and/or severe hypotension 1-6 weeks before soft tissue calcifications occurred. Calcifications resolved by 9 weeks to 5 months in 3 patients. The remaining were either deceased or lacked follow-up imaging. Renal ultrasound was performed in all but 1 patient. Nephrocalcinosis was only seen in 1 patient. Conclusion: Brown fat necrosis is subclinical, diagnosed on plain film, and likely self-limited. It occurs in term and preterm infants who have undergone significant systemic stress and carries a poor prognosis. AD - E. Golden, Department of Radiology and Imaging Sciences, Children's Healthcare of Atlanta and Emory University, 5775 Glendridge Dr #525, Atlanta, GA, United States AU - Golden, E. AU - Dickson, P. AU - Simoneaux, S. DB - Embase DO - 10.4103/ijri.IJRI_67_17 IS - 1 KW - prostaglandin article atrioventricular canal bicuspid aortic valve brown adipose tissue bypass surgery clinical article clinical feature congenital heart disease disease course drug use extracorporeal oxygenation fat necrosis female follow up heart failure heart right ventricle double outlet heart surgery heart ventricle septum defect human hypoplastic left heart syndrome hypotension induced hypothermia infant kidney calcification male neuroimaging newborn newborn disease patent ductus arteriosus patient identification pediatric radiologist picture archiving and communication system prematurity renography respiratory arrest respiratory failure risk factor skin defect soft tissue calcification tricuspid valve atresia trisomy 18 LA - English M3 - Article N1 - L621510053 2018-04-06 2018-04-12 PY - 2018 SN - 1998-3808 0971-3026 SP - 107-110 ST - Brown fat necrosis with calcifications in the newborn: Risk factors, radiographic findings, and clinical course T2 - Indian Journal of Radiology and Imaging TI - Brown fat necrosis with calcifications in the newborn: Risk factors, radiographic findings, and clinical course UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L621510053 http://dx.doi.org/10.4103/ijri.IJRI_67_17 VL - 28 ID - 723 ER - TY - JOUR AB - INTRODUCTION: With increasing survival of children with HLHS and other single ventricle lesions, the complexity of medical care for these patients is substantial. Establishing and adhering to best practice models may improve outcome, but requires careful coordination and monitoring. METHODS: In 2013 our Heart Center began a process to build a comprehensive Single Ventricle Team designed to target these difficult issues. RESULTS: Comprehensive Single Ventricle Team in 2014 was begun, to standardize care for children with single ventricle heart defects from diagnosis to adulthood within our institution. The team is a multidisciplinary group of providers committed to improving outcomes and quality of life for children with single ventricle heart defects, all functioning within the medical home of our heart center. Standards of care were developed and implemented in five target areas to standardize medical management and patient and family support. Under the team 100 patients have been cared for. Since 2014 a decrease in interstage mortality for HLHS were seen. Using a team approach and the tools of Quality Improvement they have been successful in reaching high protocol compliance for each of these areas. CONCLUSIONS: This article describes the process of building a successful Single Ventricle team, our initial results, and lessons learned. Additional study is ongoing to demonstrate the effects of these interventions on patient outcomes. AD - Division of Cardiology, Nationwide Children's Hospital, Columbus, Ohio, USA. Department of Pediatrics, The Ohio State University, Columbus, Ohio, USA. Division of Cardiothoracic Surgery, Nationwide Children's Hospital, Columbus, Ohio, USA. AN - 28744980 AU - Texter, K. AU - Davis, J. A. M. AU - Phelps, C. AU - Cheatham, S. AU - Cheatham, J. AU - Galantowicz, M. AU - Feltes, T. F. DA - Jul DB - PubMed DO - 10.1111/chd.12459 DP - NLM ET - 2017/07/27 IS - 4 KW - Child Female Follow-Up Studies Heart Ventricles/*abnormalities/surgery Humans Hypoplastic Left Heart Syndrome/*surgery Male Norwood Procedures/*standards Palliative Care/*organization & administration Patient Care Team/*organization & administration *Quality Improvement Quality of Life Retrospective Studies care coordination hypoplastic left heart quality improvement LA - eng N1 - 1747-0803 Texter, Karen Orcid: 0000-0002-7875-9266 Davis, Jo Ann M Phelps, Christina Cheatham, Sharon Cheatham, John Galantowicz, Mark Feltes, Timothy F Journal Article United States Congenit Heart Dis. 2017 Jul;12(4):403-410. doi: 10.1111/chd.12459. Epub 2017 Jul 26. PY - 2017 SN - 1747-079x SP - 403-410 ST - Building a comprehensive team for the longitudinal care of single ventricle heart defects: Building blocks and initial results T2 - Congenit Heart Dis TI - Building a comprehensive team for the longitudinal care of single ventricle heart defects: Building blocks and initial results VL - 12 ID - 111 ER - TY - JOUR AB - • Bupropion, an amphetamine, is authorised as an aid to smoking cessation, despite its negative harm-benefit balance. • In 2004, data from a registry of pregnancies exposed to bupropion drew attention to an increased risk of congenital heart defects. • In 2010, a case-control study including more than 10 000 children showed a higher incidence of in utero exposure to bupropion among children who had congenital left heart defects. Other, less reliable studies did not show such a link. • In practice, this risk of congenital heart defects is yet another reason to avoid using bupropion, including during pregnancy, and to monitor the cardiac status of exposed fetuses and newborns. ©Prescrire. DB - Embase Medline IS - 126 KW - amfebutamone aortic coarctation aortic stenosis congenital malformation congenital heart malformation human hypoplastic left heart syndrome patient monitoring patient safety pregnancy short survey smoking cessation teratogenicity LA - English M3 - Short Survey N1 - L364764122 2012-05-16 2012-05-18 http://english.prescrire.org/en/20338B806CD5CA10A1DF6E78CC35577F/Download.aspx PY - 2012 SN - 1167-7422 SP - 97 ST - Bupropion: Congenital heart defects (continued) T2 - Prescrire International TI - Bupropion: Congenital heart defects (continued) UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L364764122 VL - 21 ID - 1063 ER - TY - JOUR AB - Objective: Fontan survivors report reduced health-related quality of life (HRQOL) and have decreased exercise tolerance compared to healthy peers. We recently demonstrated that a 12-week home-based physical activity program was safe, feasible, and improved parent proxy-reported HRQOL and objective measures of exercise capacity in patients with Fontan circulation. We sought to determine if these improvements in HRQOL and exercise capacity are sustained at 6-month follow-up. Design: Patients, 8–12 years old, with Fontan circulation that completed a 12-week moderate-to-vigorous intensity home-based physical activity program were invited to attend a 6-month follow-up session to complete objective assessments of HRQOL and exercise capacity. HRQOL was measured with validated questionnaires. The 20-meter Shuttle Run (PACER Test) was used to measure exercise capacity. Results: Of the 13 patients who completed the original 12-week physical activity program, 11 (85%) attended the 6-month follow-up. There were no adverse events during the follow-up period. Improvements in parent proxy-report for HRQOL at completion of the 12-week program were sustained at 6-month follow-up. Patients reported a significant decrease in HRQOL assessed by PedsQL at 6-month follow-up. However, there was a non-significant trend of improvement in patient PCQLI total and psychosocial impact scores at completion of the 12-week program, which was sustained at 6-month follow-up. The significant improvements in objective measures of exercise capacity seen from baseline to completion of the 12-week program were sustained at 6-month follow-up. Conclusion: Improvements in parent-proxy report HRQOL and objective measures of exercise capacity after completion of a home-based 12-week physical activity program were sustained at 6-month follow-up. Despite improvements in exercise capacity, patients did not report improved HRQOL. A larger, controlled study of this home-based physical activity program is needed to assess the impact of physical activity and improved exercise capacity on HRQOL in children with Fontan circulation. AD - R. Jacobsen, 13123 E 16th Street, B100, Aurora, CO, United States AU - Jacobsen, R. AU - Danduran, M. AU - Mussatto, K. AU - Hill, G. D. AU - Ginde, S. DB - Embase DO - 10.1016/j.ppedcard.2018.06.003 KW - article child clinical article exercise exercise intensity exercise test exercise tolerance female follow up Fontan procedure functional status home physiotherapy human male physical activity priority journal quality of life quality of life assessment questionnaire school child social psychology survivor treatment duration Twenty Meter Shuttle Run LA - English M3 - Article N1 - L2001002345 2018-08-07 2018-09-21 PY - 2018 SN - 1558-1519 1058-9813 SP - 12-16 ST - Can a home-based cardiac physical activity program improve and sustain quality of life and exercise capacity in children with Fontan circulation? T2 - Progress in Pediatric Cardiology TI - Can a home-based cardiac physical activity program improve and sustain quality of life and exercise capacity in children with Fontan circulation? UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2001002345 http://dx.doi.org/10.1016/j.ppedcard.2018.06.003 VL - 50 ID - 667 ER - TY - JOUR AB - OBJECTIVE: Patients after Fontan operation for complex congenital heart disease (CHD) have decreased exercise capacity and report reduced health-related quality of life (HRQOL). Studies suggest hospital-based cardiac physical activity programs can improve HRQOL and exercise capacity in patients with CHD; however, these programs have variable adherence rates. The impact of a home-based cardiac physical activity program in Fontan survivors is unclear. This pilot study evaluated the safety, feasibility, and benefits of an innovative home-based physical activity program on HRQOL in Fontan patients. METHODS: A total of 14 children, 8-12 years, with Fontan circulation enrolled in a 12-week moderate/high intensity home-based cardiac physical activity program, which included a home exercise routine and 3 formalized in-person exercise sessions at 0, 6, and 12 weeks. Subjects and parents completed validated questionnaires to assess HRQOL. The Shuttle Test Run was used to measure exercise capacity. A Fitbit Flex Activity Monitor was used to assess adherence to the home activity program. RESULTS: Of the 14 patients, 57% were male and 36% had a dominant left ventricle. Overall, 93% completed the program. There were no adverse events. Parents reported significant improvement in their child's overall HRQOL (P < .01), physical function (P < .01), school function (P = .01), and psychosocial function (P < .01). Patients reported no improvement in HRQOL. Exercise capacity, measured by total shuttles and exercise time in the Shuttle Test Run and calculated VO2 max, improved progressively from baseline to the 6 and 12 week follow up sessions. Monthly Fitbit data suggested adherence to the program. CONCLUSION: This 12-week home-based cardiac physical activity program is safe and feasible in preteen Fontan patients. Parent proxy-reported HRQOL and objective measures of exercise capacity significantly improved. A 6-month follow up session is scheduled to assess sustainability. A larger study is needed to determine the applicability and reproducibility of these findings in other age groups and forms of complex CHD. AD - Division of Pediatric Cardiology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wis, USA. Division of Cardiovascular Medicine, Department of Internal Medicine, Medical College of Wisconsin, Milwaukee, Wis, USA. Division of Pediatric Cardiology, Department of Pediatrics, Children's Hospital of Wisconsin, Milwaukee, Wis, USA. Program in Exercise Science, College of Health Sciences, Marquette University, Milwaukee, Wis, USA. AN - 26879633 AU - Jacobsen, R. M. AU - Ginde, S. AU - Mussatto, K. AU - Neubauer, J. AU - Earing, M. AU - Danduran, M. DA - Mar-Apr DB - PubMed DO - 10.1111/chd.12330 DP - NLM ET - 2016/02/18 IS - 2 KW - Child Exercise/*physiology Exercise Test Exercise Therapy/*methods Female Follow-Up Studies *Fontan Procedure Heart Defects, Congenital/physiopathology/*rehabilitation/surgery *Home Care Services Humans Male *Quality of Life Reproducibility of Results Surveys and Questionnaires Time Factors Congenital Heart Disease Exercise Fontan Physical Activity Quality of Life Rehabilitation LA - eng N1 - 1747-0803 Jacobsen, Roni M Ginde, Salil Mussatto, Kathleen Neubauer, Jennifer Earing, Michael Danduran, Michael Journal Article Research Support, Non-U.S. Gov't United States Congenit Heart Dis. 2016 Mar-Apr;11(2):175-82. doi: 10.1111/chd.12330. Epub 2016 Feb 16. PY - 2016 SN - 1747-079x SP - 175-82 ST - Can a Home-based Cardiac Physical Activity Program Improve the Physical Function Quality of Life in Children with Fontan Circulation? T2 - Congenit Heart Dis TI - Can a Home-based Cardiac Physical Activity Program Improve the Physical Function Quality of Life in Children with Fontan Circulation? VL - 11 ID - 149 ER - TY - JOUR AB - OBJECTIVE: Children after Fontan palliation have reduced exercise capacity and quality of life. Our aim was to study whether endurance training could improve physical capacity and quality of life in Fontan patients. METHODS: Fontan patients (n=30) and healthy age- and gender-matched control subjects (n=25) performed a 6-minute walk test at submaximal capacity and a maximal cycle ergometer test. Quality of life was assessed with Pediatric Quality of Life Inventory Version 4.0 questionnaires for children and parents. All tests were repeated after a 12-week endurance training programme and after 1 year. RESULTS: Patients had decreased submaximal and maximal exercise capacity (maximal oxygen uptake 35.0±5.1 ml/minute per·kg versus 43.7±8.4 ml/minute·per·kg, p<0.001) and reported a lower quality of life score (70.9±9.9 versus 85.7±8.0, p<0.001) than controls. After training, patients improved their submaximal exercise capacity in a 6-minute walk test (from 590.7±65.5 m to 611.8±70.9 m, p<0.05) and reported a higher quality of life (p<0.01), but did not improve maximal exercise capacity. At follow-up, submaximal exercise capacity had increased further and improved quality of life was sustained. The controls improved their maximal exercise capacity (p<0.05), but not submaximal exercise capacity or quality of life after training. At follow-up, improvement of maximal exercise capacity was sustained. CONCLUSIONS: We believe that an individualised endurance training programme for Fontan patients improves submaximal exercise capacity and quality of life in Fontan patients and the effect on quality of life appears to be long-lasting. AD - Department of Women's and Children's Health,Karolinska Institutet,Stockholm,Sweden. AN - 29237518 AU - Hedlund, E. R. AU - Lundell, B. AU - Söderström, L. AU - Sjöberg, G. DA - Mar DB - PubMed DO - 10.1017/s1047951117002360 DP - NLM ET - 2017/12/15 IS - 3 KW - Adolescent Case-Control Studies Child Exercise Therapy/*methods *Exercise Tolerance Female Fontan Procedure/*rehabilitation Heart Rate Humans Male Physical Endurance *Quality of Life Regression Analysis Surveys and Questionnaires Sweden Walk Test Endurance training Fontan palliation physical capacity quality of life LA - eng N1 - 1467-1107 Hedlund, Eva R Lundell, Bo Söderström, Liselott Sjöberg, Gunnar Journal Article England Cardiol Young. 2018 Mar;28(3):438-446. doi: 10.1017/S1047951117002360. Epub 2017 Dec 14. PY - 2018 SN - 1047-9511 SP - 438-446 ST - Can endurance training improve physical capacity and quality of life in young Fontan patients? T2 - Cardiol Young TI - Can endurance training improve physical capacity and quality of life in young Fontan patients? VL - 28 ID - 55 ER - TY - JOUR AB - Objective: Children after Fontan palliation have reduced exercise capacity and quality of life. Our aim was to study whether endurance training could improve physical capacity and quality of life in Fontan patients.Methods: Fontan patients (n=30) and healthy age- and gender-matched control subjects (n=25) performed a 6-minute walk test at submaximal capacity and a maximal cycle ergometer test. Quality of life was assessed with Pediatric Quality of Life Inventory Version 4.0 questionnaires for children and parents. All tests were repeated after a 12-week endurance training programme and after 1 year.Results: Patients had decreased submaximal and maximal exercise capacity (maximal oxygen uptake 35.0±5.1 ml/minute per·kg versus 43.7±8.4 ml/minute·per·kg, p<0.001) and reported a lower quality of life score (70.9±9.9 versus 85.7±8.0, p<0.001) than controls. After training, patients improved their submaximal exercise capacity in a 6-minute walk test (from 590.7±65.5 m to 611.8±70.9 m, p<0.05) and reported a higher quality of life (p<0.01), but did not improve maximal exercise capacity. At follow-up, submaximal exercise capacity had increased further and improved quality of life was sustained. The controls improved their maximal exercise capacity (p<0.05), but not submaximal exercise capacity or quality of life after training. At follow-up, improvement of maximal exercise capacity was sustained.Conclusions: We believe that an individualised endurance training programme for Fontan patients improves submaximal exercise capacity and quality of life in Fontan patients and the effect on quality of life appears to be long-lasting. AD - Department of Women’s and Children’s Health, Karolinska Institutet, Stockholm, Sweden Department of Women's and Children's Health,Karolinska Institutet,Stockholm,Sweden AN - 128269771. Language: English. Entry Date: 20180522. Revision Date: 20190301. Publication Type: journal article. Journal Subset: Biomedical AU - Hedlund, Eva R. AU - Lundell, Bo AU - Söderström, Liselott AU - Sjöberg, Gunnar AU - Söderström, Liselott AU - Sjöberg, Gunnar DB - ccm DO - 10.1017/S1047951117002360 DP - EBSCOhost IS - 3 KW - Exercise Tolerance Cardiopulmonary Bypass -- Rehabilitation Quality of Life Therapeutic Exercise -- Methods Heart Rate Case Control Studies Physical Endurance Adolescence Male Child Female Regression Sweden N1 - Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Instrumentation: Pediatric Quality of Life Inventory (PEDSQL); Quality of Life Inventory (QOLI). NLM UID: 9200019. PMID: NLM29237518. PY - 2018 SN - 1047-9511 SP - 438-446 ST - Can endurance training improve physical capacity and quality of life in young Fontan patients? T2 - Cardiology in the Young TI - Can endurance training improve physical capacity and quality of life in young Fontan patients? UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=128269771&site=ehost-live&scope=site VL - 28 ID - 1456 ER - TY - JOUR AB - Aims: To investigate the prevalence of loss to follow-up, factors predisposing to loss to follow-up and the outcome of recall into specialist care among grown-ups with congenital heart disease (GUCH) of moderate or severe complexity prior to the introduction of formal transition in Malta. Methods: Medical documentation for all live patients with tetralogy of Fallot, aortic coarctation/interrupted aortic arch, partial and complete atrioventricular septal defect, Fontan-type circulation and transposition of the great arteries in our institutional database aged ≥16 years was analysed to determine follow-up status. Patients lost to follow-up were recalled through a postal appointment. Ordinal logistic regression was used to analyse the effect of gender, CHD complexity, consistency of paediatric cardiology follow-up during childhood, number of cardiac surgical/interventional procedures and use of longterm cardiac medications on loss to follow-up. Results: Forty-one of 187 patients (21.9%) (27 males; 34 with moderate disease) had been lost to follow-up. Limited paediatric cardiology input (OR, 5.08; 95% CI, 1.77-14.63) (p=0.003), ≤1 surgical/interventional procedures (OR, 3.34; 95% CI, 1.09-10.26) (p=0.035) and no long-term cardiac medications (OR 7.34; 95% CI, 1.74-31.02) (p=0.007) were associated with higher risk of loss to follow-up. A positive response to recall was obtained from 33/41 (80.5%) patients. Significant cardiac morbidity was found in 5/33 (15.2%) patients upon reassessment. Conclusions: Loss to specialist follow-up occurs even in health systems with little perceived barriers to medical care. Consistent specialist input during all stages and patient and family education through formal transition can help ensure a smoother transfer to GUCH care. AD - M. Caruana, Department of Cardiology, Mater Dei Hospital, Msida, Malta AU - Caruana, M. AU - Aquilina, O. AU - Grech, V. DB - Embase IS - 1 KW - adult aortic arch anomaly aortic coarctation article congenital heart disease Fallot tetralogy female follow up great vessels transposition human major clinical study male mitral valve regurgitation patient dropout prevalence prognosis pulmonary valve insufficiency pulmonary valve replacement pulmonary valve stenosis pulmonary valvuloplasty transition to adult care transluminal valvuloplasty LA - English M3 - Article N1 - L624643148 2018-11-02 2018-11-07 PY - 2018 SN - 1813-3339 SP - 13-21 ST - Can the inevitable be prevented? – An analysis of loss to follow-up among grown-ups with congenital heart disease in Malta T2 - Malta Medical Journal TI - Can the inevitable be prevented? – An analysis of loss to follow-up among grown-ups with congenital heart disease in Malta UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L624643148 VL - 30 ID - 673 ER - TY - JOUR AB - OBJECTIVE: Clinicians believe nutrition support is important; however, delivery of enteral nutrition may be delayed or interrupted due to a lack of guidelines or perceived contraindications to administration. The aim of this national survey was to examine the knowledge and perceived barriers among clinicians which prevent enteral nutrition administration to PICU patients. DESIGN: The survey consisted of 23 questions (19 primary and four branching). The survey was validated using a semistructured pilot test by three pediatric critical care intensivists and two pediatric critical care registered dietitians external to the study team. SETTING: The survey was electronically distributed to clinicians in all PICUs across Canada. POPULATION: One hundred sixty-two PICU clinicians, including 96 staff intensivists, eight clinical assistants, 36 fellows, and 22 registered dietitians from PICUs across Canada. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The survey was administered from January to March 2013. The response rate was 50% (55 staff intensivists, two clinical assistants, nine fellows, and 15 registered dietitians). There was high variability among clinicians regarding reasons to delay the onset of enteral nutrition or interrupt enteral nutrition administration. High variability (> 70% agreement and < 10% disagreement or vice versa) was found for some reasons to delay or interrupt enteral nutrition, including lactates (rising or > 2 or > 4 mmol/L), high gastric residual volumes, CT/MRI scans, and hypoplastic left heart syndrome. Sixty-eight percent of PICU clinicians reported no written feeding protocol to be in place. CONCLUSIONS: Overall, there is high variability among clinicians regarding acceptable procedural and clinical barriers to enteral nutrition administration; this may be improved by a standardized feeding protocol. Therefore, further research must be conducted to provide clinicians with evidence to support their practices for enteral nutrition administration. AD - 1Department of Agricultural, Food and Nutritional Sciences, University of Alberta, Edmonton, AB, Canada. 2Department of Pediatrics, University of Alberta, Stollery Children's Hospital, Edmonton, AB, Canada. 3Nutrition Services Alberta Health Services, Stollery Children's Hospital, Edmonton, AB, Canada. AN - 107884172. Language: English. Entry Date: 20141128. Revision Date: 20150712. Publication Type: Journal Article AU - Leong, Amanda Y. AU - Cartwright, Kristina R. AU - Guerra, Gonzalo Garcia AU - Joffe, Ari R. AU - Mazurak, Vera C. AU - Larsen, Bodil M. K. DB - ccm DO - 10.1097/PCC.0000000000000016 DP - EBSCOhost IS - 2 KW - Enteral Nutrition -- Methods Attitude to Health Health Services Accessibility Canada Child Enteral Nutrition -- Adverse Effects Female Health Personnel Surveys Human Intensive Care Units, Pediatric Male Questionnaires N1 - research. Journal Subset: Biomedical; Peer Reviewed; USA. Special Interest: Critical Care; Pediatric Care. NLM UID: 100954653. PMID: NLM24196008. PY - 2014 SN - 1529-7535 SP - e49-55 ST - A Canadian Survey of Perceived Barriers to Initiation and Continuation of Enteral Feeding in PICUs T2 - Pediatric Critical Care Medicine TI - A Canadian Survey of Perceived Barriers to Initiation and Continuation of Enteral Feeding in PICUs UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=107884172&site=ehost-live&scope=site VL - 15 ID - 1633 ER - TY - JOUR AB - A 3-year-old Japanese girl treated for hypoplastic left heart syndrome and Dandy-Walker syndrome was diagnosed with Kabuki syndrome (KS) with a mutation of KMT2D; c.13285C>T:p.Q4429∗. Concurrently, macrohematuria portended the diagnosis of Wilms tumor. Postoperative chemotherapy has achieved complete remission despite a prolonged and reduced regimen due to liver dysfunction and convulsions. Cancer predisposition has been suggested for KS due to oncogenic mutations in KMT2D or KDM6A. The first case of nephroblastoma exemplified the treatability of malignancies in KS patients, as shown in the 9 cases reviewed. Active screening and intervention are recommended for the cure of malignancy in KS children. AD - Y. Koga, Departments of Pediatrics, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, Japan AU - Teranishi, H. AU - Koga, Y. AU - Nakashima, K. AU - Morihana, E. AU - Ishii, K. AU - Sakai, Y. AU - Taguchi, T. AU - Oda, Y. AU - Miyake, N. AU - Matsumoto, N. AU - Ohga, S. DB - Embase Medline DO - 10.1097/MPH.0000000000001111 IS - 5 KW - dactinomycin DNA vincristine article brain ventricle peritoneum shunt cancer regression cancer surgery case report child clinical article computer assisted tomography convulsion Dandy Walker syndrome drug dose reduction female gene gene mutation Glenn shunt hematuria histopathology human human cell human tissue hypoplastic left heart syndrome infant Japanese (people) Kabuki makeup syndrome KMT2D gene liver dysfunction nephroblastoma Norwood procedure nuclear magnetic resonance imaging peripheral blood mononuclear cell preschool child priority journal Sanger sequencing seizure whole exome sequencing LA - English M3 - Article N1 - L622860336 2018-07-09 2018-07-11 PY - 2018 SN - 1536-3678 1077-4114 SP - 391-394 ST - Cancer management in kabuki syndrome: The first case of wilms tumor and a literature review T2 - Journal of Pediatric Hematology/Oncology TI - Cancer management in kabuki syndrome: The first case of wilms tumor and a literature review UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L622860336 http://dx.doi.org/10.1097/MPH.0000000000001111 VL - 40 ID - 674 ER - TY - JOUR AB - OBJECTIVE: We sought to determine the relationship between relative value units (RVUs) and intended measures of work in catheterization for congenital heart disease. METHODS: RVU was determined by matching RVU values to Current Procedural Terminology codes generated for cases performed at a single institution. Differences in median case duration, radiation exposure, adverse events, and RVU values by risk category and cases were assessed. Interventional case types were ranked from lowest to highest median RVU value, and correlations with case duration, radiation dose, and a cases-predicted probability of an adverse event were quantified with the Spearman rank correlation coefficient. RESULTS: Between January 2008 and December 2010, 3557 of 4011 cases were identified with an RVU and risk category designation, of which 2982 were assigned a case type. Median RVU values, radiation dose, and case duration increased with procedure risk category. Although all diagnostic cases had similar RVU values (median 10), adverse event rates ranged from 6% to 21% by age group (P , .001). Median RVU values ranged from 9 to 54 with the lowest in diagnostic and biopsy cases and increasing with isolated and then multiple interventions. Among interventional cases, no correlation existed between ranked RVU value and case duration, radiation dose, or adverse event probability (P = .13, P = .62, and P = .43, respectively). CONCLUSIONS: Time, skill, and stress inherent to performing catheterization procedures for congenital heart disease are not captured by measurement of RVU alone. Copyright © 2013 by the American Academy of Pediatrics. AD - L. Bergersen, Children's Hospital Boston, Harvard Medical School, 300 Longwood Ave, Boston, MA, United States AU - Bergersen, L. AU - Gauvreau, K. AU - McElhinney, D. AU - Fenwick, S. AU - Kirshner, D. AU - Harding, J. AU - Hickey, P. AU - Mayer, J. AU - Marshall, A. DB - Embase Medline DO - 10.1542/peds.2012-0043 IS - 2 KW - adolescent adult analytic method angiography article child congenital heart disease correlation analysis correlation coefficient Current Procedural Terminology data base disease severity female fluoroscopy Fontan procedure health care financing health care personnel heart atrium septum defect heart catheterization heart muscle biopsy heart transplantation hemodynamics human human tissue infant intervention study major clinical study male nomenclature patent ductus arteriosus percutaneous transluminal angioplasty preschool child priority journal probability prospective study radiation dose reimbursement relative value unit system school child stent validity LA - English M3 - Article N1 - L368281810 2013-02-14 2013-02-20 PY - 2013 SN - 1098-4275 0031-4005 SP - 258-267 ST - Capture of complexity of specialty care in pediatric cardiology by work RVU measures T2 - Pediatrics TI - Capture of complexity of specialty care in pediatric cardiology by work RVU measures UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L368281810 http://dx.doi.org/10.1542/peds.2012-0043 VL - 131 ID - 1025 ER - TY - JOUR AB - Cardiac abnormalities in birth asphyxia were first recognised in the 1970s. These include (i) transient tricuspid regurgitation which is the commonest cause of a systolic murmur in a newborn and tends to disappear without any treatment unless it is associated with transient myocardial ischemia or primary pulmonary hypertension of the newborn (ii) transient mitral regurgitation which is much less common and is often a part of transient myocardial ischemia, at times with reduced left ventricular function and, therefore, requires treatment in the form of inotropic and ventilatory support (iii) transient myocardial ischemia (TMI) of the newborn. This should be suspected in any baby with asphyxia, respiratory distress and poor pulses, especially if a murmur is audible. It is of five types (A to E) according to Rowe's classification. Type B is the most severe with respiratory distress, congestive heart failure and shock. Echocardiography helps to rule out critical left ventricular obstructive lesions like hypoplastic left heart syndrome or critical aortic stenosis. ECG is very important for diagnosis of TMI, and may show changes ranging from T wave inversion in one lead to a classical segmental infarction pattern with abnormal q waves. CPK-MB may rise and echocardiogram shows impaired left ventricular function, mitral and/or tricuspid regurgitation, and at times, wall motion abnormalities of left ventricle. Ejection fraction is often depressed and is a useful marker of severity and prognosis. Treatment includes fluid restriction, inotropic support, diuretics and ventilatory resistance if required (v) persistent pulmonary hypertension of the newborn (PPHN). Persistent hypoxia sometimes results in persistence of constricted fetal pulmonary vascular bed causing pulmonary arterial hypertension with consequent right to left shunt across patent ductus arteriosus and foramen ovale. This causes respiratory tension and right ventricular failure with systolic murmur of tricuspid, and at times, mitral regurgitation. Treatment consists of oxygen and general care for mild cases, ventilatory support, ECMO and nitric oxide for severe cases. Cardiac abnormalities in asphyxiated neonates are often underdiagnosed and require a high index of suspicion. ECG and Echo help in early recognition and hence better management of these cases. AD - M.S. Ranjit, Sri Ramachandra Medical College and Research Institute, Porur, Chennai, Tamil Nadu. AU - Ranjit, M. S. DB - Medline IS - 7 KW - article heart disease heart muscle ischemia human mitral valve regurgitation newborn newborn hypoxia persistent pulmonary hypertension time tricuspid valve regurgitation LA - English M3 - Article N1 - L31365467 2000-09-20 PY - 2000 SN - 0019-5456 SP - 529-532 ST - Cardiac abnormalities in birth asphyxia T2 - Indian journal of pediatrics TI - Cardiac abnormalities in birth asphyxia UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L31365467 VL - 67 ID - 1334 ER - TY - JOUR AB - Cardiac haemangiomas are exceedingly rare; however, they can cause significant haemodynamic impairment and disturbances in heart rhythm. Rarely, cardiac tumours may also coexist with congenital heart lesions. We present an extremely unusual case of a cardiac haemangioma in the setting of complex transposition of the great arteries that caused functional tricuspid atresia. To our knowledge, this is the first such case described in the literature. AD - S.A. Djordjevic, Department of Cardiology, University Children's Hospital, 10 Tirsova Street, Belgrade, Serbia AU - Djordjevic, S. A. AU - Glumac, S. AU - Kalanj, J. DB - Embase Medline DO - 10.1017/S1047951116002699 IS - 5 KW - alpha smooth muscle actin blood clotting factor 8 platelet endothelial cell adhesion molecule 1 oxygen prostaglandin E1 tumor protein unclassified drug WT 1 protein article autopsy bradycardia cardiac hemangioma cardiogenic shock case report congenital heart disease cyanosis duct dependent congenital heart disease dyspnea echocardiography first degree atrioventricular block great vessels transposition heart atrium septum defect heart right left shunt heart sound heart tumor heart ventricle tachycardia hemangioma histopathology human human tissue hypoxia immunohistochemistry infant intubation male metabolic acidosis pulmonary valve atresia pulse rate ST segment systolic heart murmur T wave thorax radiography tissue oxygenation treatment refusal tricuspid valve atresia LA - English M3 - Article N1 - L614085344 2017-01-20 2017-06-12 PY - 2017 SN - 1467-1107 1047-9511 SP - 990-992 ST - Cardiac haemangioma associated with a duct-dependent congenital heart disease in a newborn infant T2 - Cardiology in the Young TI - Cardiac haemangioma associated with a duct-dependent congenital heart disease in a newborn infant UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L614085344 http://dx.doi.org/10.1017/S1047951116002699 VL - 27 ID - 741 ER - TY - JOUR AB - Mechanical circulatory support (MCS) options are limited for patients with dysfunctional single ventricle physiology. To address this unmet clinical need, we are developing an axial-flow blood pump to provide mechanical assistance to the cavopulmonary circulation. In this study, we investigate the use of high-resolution cardiac magnetic resonance imaging (MRI) to visualize the complex fluid flow conditions of mechanical circulatory assist in two patient-specific Fontan anatomies. A three-bladed axial-flow impeller coupled to a supportive cage with a four-bladed diffuser was positioned in the inferior vena cava (IVC) of each Fontan anatomy. Cardiac magnetic resonance (CMR) imaging and power efficiency studies were conducted at physiologic relevant parameters with cardiac outputs of 2, 3, and 4 L/min with impeller rotational speeds of 2000 and 4000 rpm. The axial-flow impeller was able to generate improved flow in the total cavopulmonary connection (TCPC). The higher rotational speed was able to redistribute flow in the TCPC anastomosis aiding in removing stagnant blood. No retrograde flow was observed or measured in the superior vena cava (SVC). As an extension of the CMR data, a scalar stress analysis was performed on both models and found a maximum scalar stress of approximately 42 Pa for both patient anatomies. The power efficiency experiments demonstrated a maximum energy gain of 8.6 mW for TCPC Anatomy 1 and 12.58 mW for TCPC Anatomy 2 for a flow rate of 4 L/min and at 4000 rpm. These findings support the continued development of axial blood pumps for mechanical cavopulmonary assist. © 2019 by ASME. AD - School of Biomedical Engineering, Science and Health Systems, Bossone Research Enterprise Center, Drexel University, 3141 Chestnut Street, Philadelphia, PA 19104, United States Division of Cardiology, Department of Radiology, Children's Hospital of Philadelphia, 3401 Civic Center Boulevard, Philadelphia, PA 19104, United States AU - Chopski, S. G. AU - Whitehead, K. AU - Englehardt, G. J. AU - Throckmorton, A. C7 - 011001 DB - Scopus DO - 10.1115/1.4041414 IS - 1 KW - cardiac magnetic resonance CMR mechanical circulatory assistance PC-MRI pediatric circulatory support rotary blood pump M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2019 ST - Cardiac magnetic resonance imaging of mechanical cavopulmonary assistance T2 - Journal of Medical Devices, Transactions of the ASME TI - Cardiac magnetic resonance imaging of mechanical cavopulmonary assistance UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85056210234&doi=10.1115%2f1.4041414&partnerID=40&md5=bb1ea008c957a7153d91ebf12bbb7816 VL - 13 ID - 1758 ER - TY - JOUR AB - BACKGROUND: A superior cavopulmonary connection is commonly performed before the Fontan procedure in patients with a functionally univentricular heart. Data are limited regarding associations between a prior superior cavopulmonary connection and functional and ventricular performance late after the Fontan procedure. METHODS: We compared characteristics of those with and without prior superior cavopulmonary connection among 546 subjects enrolled in the Pediatric Heart Network Fontan Cross-Sectional Study. We further compared different superior cavopulmonary connection techniques: bidirectional cavopulmonary anastomosis (n equals 229), bilateral bidirectional cavopulmonary anastomosis (n equals 39), and hemi-Fontan (n equals 114). RESULTS: A prior superior cavopulmonary connection was performed in 408 subjects (75%); the proportion differed by year of Fontan surgery and centre (p-value less than 0.0001 for each). The average age at Fontan was similar, 3.5 years in those with superior cavopulmonary connection versus 3.2 years in those without (p-value equals 0.4). The type of superior cavopulmonary connection varied by site (p-value less than 0.001) and was related to the type of Fontan procedure. Exercise performance, echocardiographic variables, and predominant rhythm did not differ by superior cavopulmonary connection status or among superior cavopulmonary connection types. Using a test of interaction, findings did not vary according to an underlying diagnosis of hypoplastic left heart syndrome. CONCLUSIONS: After controlling for subject and era factors, most long-term outcomes in subjects with a prior superior cavopulmonary connection did not differ substantially from those without this procedure. The type of superior cavopulmonary connection varied significantly by centre, but late outcomes were similar. AD - Division of Cardiology, Department of Pediatrics, Medical University of South Carolina, Charleston, South Carolina 29425, USA. atzam@musc.edu AN - 22824161 AU - Atz, A. M. AU - Travison, T. G. AU - McCrindle, B. W. AU - Mahony, L. AU - Glatz, A. C. AU - Kaza, A. K. AU - Breitbart, R. E. AU - Colan, S. D. AU - Kaltman, J. R. AU - Margossian, R. AU - Pasquali, S. K. AU - Wang, Y. AU - Gersony, W. M. C2 - PMC3578173 C6 - NIHMS404182 DA - Jun DB - PubMed DO - 10.1017/s1047951112001175 DP - NLM ET - 2012/07/25 IS - 3 KW - Adolescent Child Child, Preschool Cross-Sectional Studies Female *Fontan Procedure Heart Bypass, Right/*methods Heart Defects, Congenital/physiopathology/*surgery Heart Function Tests Humans Male *Quality of Life Treatment Outcome LA - eng N1 - 1467-1107 Atz, Andrew M Travison, Thomas G McCrindle, Brian W Mahony, Lynn Glatz, Andrew C Kaza, Aditya K Breitbart, Roger E Colan, Steven D Kaltman, Jonathan R Margossian, Renee Pasquali, Sara K Wang, Yanli Gersony, Welton M Pediatric Heart Network Investigators K08 HL103631/HL/NHLBI NIH HHS/United States HL068288/HL/NHLBI NIH HHS/United States HL068285/HL/NHLBI NIH HHS/United States U01 HL068269/HL/NHLBI NIH HHS/United States U01 HL068279/HL/NHLBI NIH HHS/United States U01 HL068290/HL/NHLBI NIH HHS/United States HL068279/HL/NHLBI NIH HHS/United States U01 HL068288/HL/NHLBI NIH HHS/United States U10 HL068270/HL/NHLBI NIH HHS/United States U01 HL068281/HL/NHLBI NIH HHS/United States U01 HL068270/HL/NHLBI NIH HHS/United States HL068281/HL/NHLBI NIH HHS/United States U01 HL068292/HL/NHLBI NIH HHS/United States HL068270/HL/NHLBI NIH HHS/United States HL068290/HL/NHLBI NIH HHS/United States U01 HL068285/HL/NHLBI NIH HHS/United States HL068292/HL/NHLBI NIH HHS/United States Clinical Trial Comparative Study Journal Article Research Support, N.I.H., Extramural Cardiol Young. 2013 Jun;23(3):335-43. doi: 10.1017/S1047951112001175. Epub 2012 Jul 24. PY - 2013 SN - 1047-9511 (Print) 1047-9511 SP - 335-43 ST - Cardiac performance and quality of life in patients who have undergone the Fontan procedure with and without prior superior cavopulmonary connection T2 - Cardiol Young TI - Cardiac performance and quality of life in patients who have undergone the Fontan procedure with and without prior superior cavopulmonary connection VL - 23 ID - 213 ER - TY - JOUR AD - Department of Pediatric Cardiology and Cardiovascular Surgery, Saitama Medical School Hospital, Japan. hsenzaki@saitama-med.ac.jp AN - 14752452 AU - Senzaki, H. AU - Kyo, S. AU - Matsumoto, K. AU - Asano, H. AU - Masutani, S. AU - Ishido, H. AU - Matunaga, T. AU - Taketatu, M. AU - Kobayashi, T. AU - Sasaki, N. AU - Yokote, Y. DA - Jan DB - PubMed DO - 10.1016/j.jtcvs.2003.08.037 DP - NLM ET - 2004/01/31 IS - 1 KW - Abnormalities, Multiple/*diagnosis/*therapy Adolescent Cardiac Catheterization Cardiac Pacing, Artificial/*methods Echocardiography, Doppler Electrocardiography Follow-Up Studies Heart Failure/diagnosis/etiology/*therapy Heart Valve Diseases/diagnosis Heart Ventricles/*abnormalities Humans Male Pulmonary Valve Stenosis/diagnosis Quality of Life Risk Assessment Spleen/abnormalities Treatment Outcome Ventricular Remodeling LA - eng N1 - Senzaki, Hideaki Kyo, Shunei Matsumoto, Kazuo Asano, Haruhiko Masutani, Satoshi Ishido, Hirotaka Matunaga, Tamotu Taketatu, Mio Kobayashi, Toshiki Sasaki, Nozomu Yokote, Yuji Case Reports Journal Article United States J Thorac Cardiovasc Surg. 2004 Jan;127(1):287-8. doi: 10.1016/j.jtcvs.2003.08.037. PY - 2004 SN - 0022-5223 (Print) 0022-5223 SP - 287-8 ST - Cardiac resynchronization therapy in a patient with single ventricle and intracardiac conduction delay T2 - J Thorac Cardiovasc Surg TI - Cardiac resynchronization therapy in a patient with single ventricle and intracardiac conduction delay VL - 127 ID - 499 ER - TY - JOUR AB - PURPOSE OF REVIEW: Heart failure is an important problem after surgical correction of congenital heart disease. Timely recognition may be difficult. Recent developments in exercise testing and stress-imaging may change the management of patients with congenital heart disease. RECENT FINDINGS: Exercise tests are commonly used in the follow-up of patients with congenital heart disease. Maximal exercise studies are not always feasible in this patient population. Variables of submaximal exercise and ventilator efficiency have shown a good correlation with variables of maximal exercise and have been suggested to relate to long-term cardiac function.For evaluation of submaximal exercise, stress imaging may reveal abnormal responses unrecognized at rest. Both physical exercise as well as pharmacological stress may be used in combination with various imaging modalities. For practical reasons, dobutamine is most widely used to generate and mimic stress and is well tolerated in low doses. Particularly in lesions affecting the right ventricle and with single ventricular physiology after the Fontan operation, magnetic resonance stress imaging has provided additional insight into the cardiac function. SUMMARY: The abnormal stress responses can potentially be used for risk assessment in the follow-up of patients with congenital cardiac disease. Further studies are required to provide common protocols for stress imaging. AD - Division of Cardiology, Department of Paediatrics, Erasmus MC-Sophia Children's Hospital, and Department of Radiology, Erasmus MC, Rotterdam, The Netherlands. w.a.helbing@erasmusmc.nl AN - 105112614. Language: English. Entry Date: 20101105. Revision Date: 20150711. Publication Type: Journal Article AU - Helbing, W. A. AU - Luijnenburg, S. E. AU - Moelker, A. AU - Robbers-Visser, D. DB - ccm DO - 10.1097/MOP.0b013e32833e139e DP - EBSCOhost IS - 5 KW - Exercise Test, Cardiopulmonary -- Methods Heart Defects, Congenital -- Surgery Diagnostic Imaging -- Methods Dobutamine -- Therapeutic Use Drug Evaluation Echocardiography -- Methods Exercise Exercise Test, Cardiopulmonary -- Evaluation Heart Diseases -- Physiopathology Magnetic Resonance Imaging -- Methods Pediatrics Perfusion Imaging -- Methods Surgery, Operative -- Methods Survival N1 - diagnostic images; review; tables/charts. Journal Subset: Biomedical; Peer Reviewed; USA. Special Interest: Pediatric Care. NLM UID: 9000850. PMID: NLM20736836. PY - 2010 SN - 1040-8703 SP - 579-586 ST - Cardiac stress testing after surgery for congenital heart disease T2 - Current Opinion in Pediatrics TI - Cardiac stress testing after surgery for congenital heart disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105112614&site=ehost-live&scope=site VL - 22 ID - 1641 ER - TY - JOUR AB - Details are given of the results of surgical treatment for congenital heart disease performed during 4 yr. on 120 infants all under the age of one year. The majority is suffering from ventricular septal defect (29), Fallot's tetralogy (28), tricuspid atresia (10), transposition of the great vessels (17), coarctation of the aorta (14), or patent ductus arteriosus (9). The indication for operation in all cases was the serious condition of the infant, suggesting that it was unlikely to survive without urgent operative treatment. Eighty-three (69%) of the children survived the operation. Full details of the various operations performed and the anatomical problems encountered are given and the original paper should be consulted for these. The value of surgery in saving life in this age group is stressed. AD - H.A. Collins, Cora and Webb Mading Depts. of Surg. and Ped., Baylor Univ. Coll. of Med., Houston, TX, United States AU - Collins, H. A. AU - Harberg, F. J. AU - Soltero, L. R. AU - McNamara, D. G. AU - Cooley, D. A. DB - Embase Classic Medline IS - 3 KW - aorta child congenital heart disease Fallot tetralogy great blood vessel groups by age heart surgery heart ventricle septum defect infant newborn patent ductus arteriosus patient surgery tricuspid valve atresia LA - English M3 - Article N1 - L281053120 1959-12-01 PY - 1959 SN - 0039-6060 SP - 506-519 ST - Cardiac surgery in the newborn. Experience with 120 patients under one year of age T2 - Surgery TI - Cardiac surgery in the newborn. Experience with 120 patients under one year of age UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L281053120 VL - 45 ID - 1422 ER - TY - JOUR AB - Future improvements can be expected in cardiac transplantation in children. We continue to advance our understanding of the immune system, and to develop more specific immunosuppressive agents. Ultimately, the future for recipients may be improved by strategies such as induction therapy or donor-derived chimeric destined transfusions, designed to enhance the tolerance of the host to a human leukocyte antigen incompatible graft. Improvements in tolerance of the host would allow for reduction or elimination of many, if not all, of the immunosuppressive agents, and for longevity extending well into the adulthood. Survival, particularly for infants, has improved dramatically in the last decade. The most recent results from the registry of the International Society of Heart and Lung Transplantation/United Network for Organ Sharing show that recipients less than one year old at transplantation, who survive the first year, have greater than a 95% survival to four years (Fig. 1). As late outcomes continue to improve, transplantation will provide a better quality and duration of life for infants with hypoplastic left heart syndrome. It is possible, nonetheless, that some infants will require retransplantation, since the half life of a transplanted heart in children has been about 12 years. The alternative is conventional surgery with multiple palliative operations, and the need for later transplantation as end-stage cardiac function is reached. Efforts to increase potential donors and donor utilization can be supported by innovative schemes, such as ABO incompatible transplants. Additional efforts are made more urgent when the current data indicate excellent outcomes after transplantation, but a high mortality while waiting for transplantation. AD - R.J. Boucek, Department of Pediatric Cardiology, Congenital Heart Institute of Florida and University of South Florida/ All Children's Hospital, Saint Petersburg, Florida 33701-4823, USA. AU - Boucek Jr, R. J. AU - Chrisant, M. R. DB - Medline KW - article child child development heart transplantation human hypoplastic left heart syndrome infant kidney function test mortality newborn quality of life survival survival rate time treatment outcome LA - English M3 - Article N1 - L39351971 2004-10-24 PY - 2004 SN - 1047-9511 SP - 83-87 ST - Cardiac transplantation for hypoplastic left heart syndrome T2 - Cardiology in the young TI - Cardiac transplantation for hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L39351971 VL - 14 Suppl 1 ID - 1296 ER - TY - JOUR AB - Future improvements can be expected in cardiac transplantation in children. We continue to advance our understanding of the immune system, and to develop more specific immunosuppressive agents. Ultimately, the future for recipients may be improved by strategies such as induction therapy or donor-derived chimeric destined transfusions, designed to enhance the tolerance of the host to a human leukocyte antigen incompatible graft. Improvements in tolerance of the host would allow for reduction or elimination of many, if not all, of the immunosuppressive agents, and for longevity extending well into the adulthood. Survival, particularly for infants, has improved dramatically in the last decade. The most recent results from the registry of the International Society of Heart and Lung Transplantation/United Network for Organ Sharing show that recipients less than one year old at transplantation, who survive the first year, have greater than a 95% survival to four years (Fig. 1). As late outcomes continue to improve, transplantation will provide a better quality and duration of life for infants with hypoplastic left heart syndrome. It is possible, nonetheless, that some infants will require retransplantation, since the half life of a transplanted heart in children has been about 12 years. The alternative is conventional surgery with multiple palliative operations, and the need for later transplantation as end-stage cardiac function is reached. Efforts to increase potential donors and donor utilization can be supported by innovative schemes, such as ABO incompatible transplants. Additional efforts are made more urgent when the current data indicate excellent outcomes after transplantation, but a high mortality while waiting for transplantation. AD - Department of Pediatric Cardiology, Congenital Heart Institute of Florida and University of South Florida/ All Children's Hospital, Saint Petersburg, Florida 33701-4823, USA. BoucekR@allkids.org AN - 15244145 AU - Boucek, R. J., Jr. AU - Chrisant, M. R. DA - Feb DB - PubMed DO - 10.1017/s1047951104006353 DP - NLM ET - 2004/07/13 KW - Child Child Development *Heart Transplantation Humans Hypoplastic Left Heart Syndrome/mortality/*surgery Infant Infant, Newborn Kidney Function Tests Quality of Life Survival Analysis Survival Rate Time Factors Treatment Outcome LA - eng N1 - Boucek, Robert J Jr Chrisant, Maryanne R K Journal Article England Cardiol Young. 2004 Feb;14 Suppl 1:83-7. doi: 10.1017/s1047951104006353. PY - 2004 SN - 1047-9511 (Print) 1047-9511 SP - 83-7 ST - Cardiac transplantation for hypoplastic left heart syndrome T2 - Cardiol Young TI - Cardiac transplantation for hypoplastic left heart syndrome VL - 14 Suppl 1 ID - 307 ER - TY - JOUR AU - Gavotto, A. AU - Amedro, P. AU - Guillaumont, S. AU - Vincenti, M. AU - De La Villeon, G. AU - Soulatges, C. AU - Bredy, C. AU - Matecki, S. DB - Embase DO - 10.1016/S1878-6480(16)30570-5 IS - 5 KW - anaerobic threshold article cardiopulmonary exercise test child congenital heart disease controlled study follow up heart left ventricle ejection fraction heart left ventricle outflow tract obstruction heart rehabilitation heart right ventricle heart right ventricle outflow tract heart single ventricle heart valve regurgitation human lung function test major clinical study multicenter study (topic) oxygen consumption priority journal quality of life systolic blood pressure tertiary care center LA - English M3 - Article N1 - L613234179 2016-11-21 2016-12-13 PY - 2016 SN - 1878-6502 1878-6480 SP - 14 ST - Cardiopulmonary exercise test among children with congenital heart diseases: a multicenter study T2 - Archives of Cardiovascular Diseases Supplements TI - Cardiopulmonary exercise test among children with congenital heart diseases: a multicenter study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L613234179 http://dx.doi.org/10.1016/S1878-6480(16)30570-5 VL - 8 ID - 795 ER - TY - JOUR AB - Background: The prevalence of cardiovascular anomalies in Down's syndrome is well described, but there are few data on spectrum, management and outcome. The authors aimed to provide this information for infants with Down's syndrome in a defined population over a 22-year period. Methods: The regional paediatric cardiology database in Newcastle upon Tyne provided information on all cardiovascular anomalies, surgical treatment and outcome. Data was subdivided into two eras, 1985-1995 and 1996-2006, and surgical results and outcomes compared. Data on live births with Down's syndrome were obtained from the Northern Congenital Abnormality Survey (NorCAS). Denominator data on all live births in the region were obtained from UK Statistics. Results: In 1985-2006 there were 754,486 live births in the population. 821 infants were live-born with Down's syndrome (1.09 per 1000 live births). 342 (42%) infants with Down's syndrome had a cardiovascular anomaly. The commonest anomaly was complete atrioventricular septal defect in 125 (37%) infants. Three patients had univentricular physiology. In 1985-1995, 101/163 (62%) infants had surgery with 30% mortality; in 1996-2006, 129/180 (72%) had surgery with 5% mortality. One patient underwent Fontan completion. There were two cardiac transplants for cardiomyopathy. One-year survival in Down's syndrome with a cardiovascular anomaly improved from 82% in 1985-1995 to 94% in 1996-2006. Conclusions: The incidence of cardiovascular anomalies in Down's syndrome was 42%. There has been a significant reduction in postoperative mortality and improvement in 1-year survival. Treatment modalities such as single ventricle palliation and cardiac transplantation are now considered in these patients. AD - C.A. Irving, Department of Paediatric Cardiology, Freeman Hospital, Freeman Road, Newcastle upon Tyne NE7 7DN, United Kingdom AU - Irving, C. A. AU - Chaudhari, M. P. DB - Embase Medline DO - 10.1136/adc.2010.210534 IS - 4 KW - article cardiovascular malformation congestive cardiomyopathy disease association Down syndrome heart atrium septum defect heart transplantation heart ventricle septum defect human infant live birth major clinical study mortality patent ductus arteriosus prevalence priority journal survival time LA - English M3 - Article N1 - L51573575 2011-08-17 2012-04-09 http://adc.bmj.com/content/97/4/326.full.pdf+html PY - 2012 SN - 0003-9888 1468-2044 SP - 326-330 ST - Cardiovascular abnormalities in Down's syndrome: Spectrum, management and survival over 22 years T2 - Archives of Disease in Childhood TI - Cardiovascular abnormalities in Down's syndrome: Spectrum, management and survival over 22 years UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51573575 http://dx.doi.org/10.1136/adc.2010.210534 VL - 97 ID - 1065 ER - TY - GEN AB - Cardiovascular diseases are becoming increasingly prevalent in children, due at least in part to the rise in childhood obesity. Public awareness is also increasing, and treatment options are improving. Pharmacists have the responsibility of counseling children and their caregivers about pharmacologic and nonpharmacologic therapies and interventions that can improve long-term prognosis. Adherence to complex medication regimens is key to optimal management. In cases of CHDs, for example, pharmacists can provide education about the rationale for drug therapy, and in cases of arrhythmia, they can obtain a thorough medication history to assist in diagnosis or avoidance. More important, pharmacists can stress the possibility of preventing the onset of cardiovascular diseases and encourage lifestyle changes that can help reduce the occurrence of hypertension and obesity. AD - C. Tom-Revzon, Department of Pharmacy Practice, Arnold and Marie Schwartz College of Pharmacy and Health Sciences, Long Island University, Brooklyn, NY, United States AU - Tom-Revzon, C. C1 - aldactone hydrodiuril inderal lotensin DB - Embase J2 - U.S. Pharm. KW - adenosine epinephrine amiodarone antibiotic agent atropine benazepril beta adrenergic receptor stimulating agent calcium channel blocking agent central stimulant agent chlorothiazide cholinergic receptor blocking agent digoxin endothelin receptor antagonist flecainide furosemide hydrochlorothiazide lidocaine oxygen phenothiazine phosphodiesterase V inhibitor procainamide propranolol prostaglandin quinidine salicylic acid sotalol spironolactone theophylline tricyclic antidepressant agent unindexed drug aortic coarctation article bacterial endocarditis blood pressure regulation bradycardia cardiovascular disease cardiovascular surgery child health care childhood mortality congenital heart disease Fallot tetralogy great vessels transposition heart arrhythmia atrial fibrillation heart atrium septum defect heart failure heart ventricle fibrillation heart ventricle septum defect heart ventricle tachycardia hospitalization human hypertension hypoplastic left heart syndrome lifestyle modification medical decision making obesity patent ductus arteriosus patient counseling patient monitoring pharmaceutical care pharmacist attitude prevalence prognosis sinus tachycardia supraventricular tachycardia aldactone hydrodiuril inderal lotensin LA - English M1 - (Tom-Revzon C.) Department of Pharmacy Practice, Arnold and Marie Schwartz College of Pharmacy and Health Sciences, Long Island University, Brooklyn, NY, United States M3 - Article N1 - L352024597 2008-08-05 http://www.uspharmacist.com/index.asp?show=article&page=8_1979.htm PY - 2007 SN - 0148-4818 SP - 52-65 ST - Cardiovascular diseases in children TI - Cardiovascular diseases in children UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L352024597 VL - 32 ID - 1239 ER - TY - JOUR AB - The authors discuss an early hybrid cardiovascular intervention in a neonate with left-sided congenital diaphragmatic hernia and significant left heart hypoplasia. The operation included persistent ductus arteriosus stenting and right pulmonary artery calibrated banding (3.5 mm) to increase blood flow in the aorta and to decrease right ventricle overload and decrease blood pressure and overflow in pulmonary circulation. The operation improved the child's general condition and gained time for left ventricle growth and restoration of its function. © The Author 2012. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. AD - Department of Surgery and Urology for Children and Adolescents, Medical University of Gdańsk, Gdańsk, Poland Department of Paediatric Cardiac Surgery, Nicolaus Copernicus Pomeranian Centre of Traumatology, Gdańsk, Poland AU - Sroka, M. AU - Haponiuk, I. AU - Chojnicki, M. AU - Czauderna, P. C7 - ezs099 DB - Scopus DO - 10.1093/ejcts/ezs099 IS - 1 KW - Cardiovascular hybrid intervention Congenital diaphragmatic hernia Left heart hypoplasia M3 - Article N1 - Cited By :6 Export Date: 15 June 2020 PY - 2012 SP - 185-187 ST - Cardiovascular hybrid procedure in severe congenital diaphragmatic hernia with significant left heart hypoplasia T2 - European Journal of Cardio-thoracic Surgery TI - Cardiovascular hybrid procedure in severe congenital diaphragmatic hernia with significant left heart hypoplasia UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84871671863&doi=10.1093%2fejcts%2fezs099&partnerID=40&md5=4e1c0b9018a25b355180b42e93c42634 VL - 42 ID - 2091 ER - TY - JOUR AD - Assistant Professor of Pediatric Critical Care Medicine and Cardiology, St. Louis Children's Hospital, Washington University School of Medicine, St. Louis, MO, United States Professor of Surgery, Director of Pediatric Cardiothoracic Surgery, St. Louis Children's Hospital, Washington University School of Medicine, St. Louis, MO, United States Associate Professor of Anesthesiology and Pediatrics, St. Louis Children's Hospital, Washington University School of Medicine, St. Louis, MO, United States Retired Associate Professor of Cardiothoracic Surgery, University of Massachusetts Medical School, Worcester, MA, United States AU - Gazit, A. Z. AU - Huddleston, C. B. AU - Checchia, P. A. AU - Fehr, J. AU - Pezzella, A. T. DB - Scopus DO - 10.1067/j.cpsurg.2009.12.003 IS - 4 M3 - Article N1 - Cited By :8 Export Date: 15 June 2020 PY - 2010 SP - 261-376 ST - Care of the Pediatric Cardiac Surgery Patient-Part 2 T2 - Current Problems in Surgery TI - Care of the Pediatric Cardiac Surgery Patient-Part 2 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-77950363330&doi=10.1067%2fj.cpsurg.2009.12.003&partnerID=40&md5=94dba1934cfe0e2ef969e51b708eb46d VL - 47 ID - 2154 ER - TY - JOUR AB - INTRODUCTION: Improved weight gain during the interstage (IS) period has been shown to improve overall outcomes in patients with single ventricle physiology (SVP). This emphasis on nutrition may have untoward effects, such as increasing anxiety/stress levels in caregivers, who are already known to be at risk for increased anxiety/stress levels. The goal of this study was to evaluate anxiety/stress levels of caregivers as it pertains to feeding during the IS period and to determine if certain characteristics were associated with higher anxiety/stress scores. METHODS: Caregivers of children with SVP who completed the IS period, defined as the time between the first and second cardiac surgeries, were recruited. Baseline demographics were obtained. Anxiety/stress levels were measured via eight questions using a 0- to 10-point scale. Correlations were performed between demographic variables and anxiety/stress level scores. RESULTS: Fifty-six surveys were completed (39 males, 27 females) on 43 children. Fourteen children required tube feeds during the IS period. There were significant correlations between anxiety/stress scores and caregiver's gender, caregiver's age, caregiver's level of education, percent of time a caregiver spent feeding the child, if caregivers were taking medications for anxiety, and if the child was seen in the emergency room during the IS period. There were no correlation of anxiety/stress scores with caregiver's race, child's underlying cardiac diagnosis, age of child, route of feeding during the IS period, birth order of the child or number of children in the family, relationship status, or distance from the hospital. CONCLUSION: In general, caregivers of children with SVP experience anxiety/stress during the IS period specifically due to feeding concerns. Certain intrinsic and extrinsic characteristics were associated with higher anxiety/stress levels. Future studies are needed to determine how to minimize anxiety/stress levels during this stressful time period. AD - Heart Center, Nationwide Children's Hospital, Columbus, Ohio, USA. AN - 25876670 AU - Stewart, J. AU - Dempster, R. AU - Allen, R. AU - Miller-Tate, H. AU - Dickson, G. AU - Fichtner, S. AU - Principe, A. J. AU - Fonseca, R. AU - Nicholson, L. AU - Cua, C. L. DA - Mar-Apr DB - PubMed DO - 10.1111/chd.12257 DP - NLM ET - 2015/04/17 IS - 2 KW - Adult *Aftercare Anxiety/epidemiology/*etiology Caregivers/*psychology Child Child, Preschool Cross-Sectional Studies *Feeding Methods Female Humans Infant Male Patient Discharge Prospective Studies Stress, Psychological/epidemiology/*etiology Surveys and Questionnaires Anxiety Hypoplastic Left Heart Interstage Nutrition Single Ventricle LA - eng N1 - 1747-0803 Stewart, Jamie Dempster, Robert Allen, Robin Miller-Tate, Holly Dickson, Gabrielle Fichtner, Samantha Principe, Alex J Fonseca, Rachel Nicholson, Lisa Cua, Clifford L Journal Article United States Congenit Heart Dis. 2015 Mar-Apr;10(2):E98-106. doi: 10.1111/chd.12257. PY - 2015 SN - 1747-079x SP - E98-106 ST - Caregiver anxiety due to interstage feeding concerns T2 - Congenit Heart Dis TI - Caregiver anxiety due to interstage feeding concerns VL - 10 ID - 225 ER - TY - JOUR AB - Neonatal cardiac transplantation for hypoplastic left heart syndrome (HLHS) is associated with excellent long-term survival compared to older recipients. However, heart transplantation for neonates is greatly limited by the critical shortage of donor hearts, and by the associated mortality of the long pre-transplant waiting period. This led to the development of staged surgical palliation as the first-line surgical therapy for HLHS. Recent advances in genetic engineering and xenotransplantation have provided the potential to replicate the excellent results of neonatal cardiac allotransplantation while eliminating wait-list-associated mortality through genetically modified pig-to-human neonatal cardiac xenotransplantation. The elimination of the major pig antigens in addition to the immature B-cell response in neonates allows for the potential to induce B-cell tolerance. Additionally, the relatively mature neonatal T-cell response could be reduced by thymectomy at the time of operation combined with donor-specific pig thymus transplantation to “reprogram” the host’s T-cells to recognize the xenograft as host tissue. In light of the recent significantly increased graft survival of genetically-engineered pig-to-baboon cardiac xenotransplantation, we propose that now is the time to consider devoting research to advance the potential clinical application of cardiac xenotransplantation as a treatment option for patients with HLHS. Employing cardiac xenotransplantation could revolutionize therapy for complex congenital heart defects and open a new chapter in the field of pediatric cardiac transplantation. AD - D. Cleveland, Division of Pediatric Cardiovascular Surgery, University of Alabama at Birmingham, Birmingham, AL, United States AU - Cleveland, D. AU - Adam Banks, C. AU - Hara, H. AU - Carlo, W. F. AU - Mauchley, D. C. AU - Cooper, D. K. C. DB - Embase Medline DO - 10.1007/s00246-018-1998-1 IS - 2 KW - article health care cost heart single ventricle heart transplantation high risk population human hypoplastic left heart syndrome intermethod comparison length of stay long term care mortality rate newborn nonhuman palliative therapy quality of life reoperation survival rate thymus transplantation tolerance treatment outcome xenotransplantation LA - English M3 - Article N1 - L624434149 2018-10-24 2019-05-14 PY - 2019 SN - 1432-1971 0172-0643 SP - 437-444 ST - The Case for Cardiac Xenotransplantation in Neonates: Is Now the Time to Reconsider Xenotransplantation for Hypoplastic Left Heart Syndrome? T2 - Pediatric Cardiology TI - The Case for Cardiac Xenotransplantation in Neonates: Is Now the Time to Reconsider Xenotransplantation for Hypoplastic Left Heart Syndrome? UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L624434149 http://dx.doi.org/10.1007/s00246-018-1998-1 VL - 40 ID - 626 ER - TY - JOUR AB - The rate of extracardiac malformation (ECM) associated with congenital heart disease (CHD) is high in neonates. 108 cases of 212 neonates with CHD had ECM (50.9%). Main ECMs were digestive system anomalies (36.1%), chromosomal aberrations (26.8%), respiratory system anomalies (21.3%), CNS anomalies (13.0%), and other (2.7%). Single lesion of left to right shunt accounted for 77.2% (61/79) of CHD with ECM from other than chromosomal aberrations. The prognosis of neonates with CHD without ECM was also studied. Total anomalous pulmonary venous connection, pure pulmonary atresia (PPA), pulmonary stenosis (PS), hypoplastic left heart syndrome are not generally associated with ECM, but the prognosis is poor. Coarctation complex which is sometimes associated with ECM has a poor prognosis in neonates. There is an increase of the survival rate in the patients with hypoxemia, such as PPA or severe PS, extreme tetralogy of Fallot, and tricuspid atresia, which can be managed with prostaglandin E1. PDA associated with respiratory distress syndrome is ideally treated with indomethacin. In recent years, mortality from PDA has decreased in neonates. The mortality rate during the neonatal period was 46.8% (51/109): 37.5% (30/80) died before surgical interventions and 72.4% (21/29) died during or after surgery. Half of neonatal deaths from CHD occurred within 3 days of admission. Thus, early detection, early diagnosis, and early treatment of neonates with CHD is most important. AD - M. Naganuma AU - Naganuma, M. AU - Tsunemoto, M. AU - Naito, T. DB - Medline DO - 10.1253/jcj.45.215 IS - 2 KW - article central nervous system chromosome aberration chromosome disorder congenital heart malformation congenital malformation digestive system malformation female human male mortality multiple malformation syndrome newborn postoperative complication prognosis respiratory tract malformation LA - English M3 - Article N1 - L11602833 1981-07-08 PY - 1981 SN - 0047-1828 SP - 215-220 ST - Case histories of neonates with congenital heart disease T2 - Japanese circulation journal TI - Case histories of neonates with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L11602833 http://dx.doi.org/10.1253/jcj.45.215 VL - 45 ID - 1400 ER - TY - JOUR AB - The case of a girl, aged 5 1/2 years, with slight cyanosis and clinical signs of a pseudotruncus and single ventricle is discussed. An aberrant myocardial venous drainage opening into the common ventricle is supposed to be present. A catheter passed along a coronary vein showed penetration of its tip into the wall of the common ventricle. The depressed ventricular curve together with the very low oxygen saturation make it probable that there is no opening into the ventricle. The thin ventricular wall acts as a pressure membrane, and ventricular pressure is thus transferred. A similar case with an identical position of the catheter was reported in the scientific exhibition at the 6th International Pediatric Congress at Zurich in July 1950. The phenomenon is to be regarded as an effect opposite to the 'pulmonary capillary pressure' far out in the pulmonary artery (Hellems, Haynes and Dexter J. Applied Physiol. 1949, 2, 24). Here fully oxygenated blood is received together with a pressure curve of the pulmonary vein or left auricle. AD - A.J. Miller, Cardiovascular Dept., Med. Res. Inst., Michael Reese Hosp., Chicago AU - Miller, A. J. AU - Prec, O. AU - Akman, L. AU - Katz, L. N. AU - Gibson, S. DB - Embase Classic Medline DO - 10.1016/0002-8703(50)90258-4 IS - 4 KW - aged blood catheter congenital heart disease coronary artery coronary vein cyanosis girl heart single ventricle heart ventricle pressure heart ventricle wall lung capillary pressure membrane oxygen saturation pulmonary artery pulmonary vein trunk LA - English M3 - Article N1 - L280603552 1950-12-01 PY - 1950 SN - 0002-8703 SP - 607-614 ST - A case of congenital heart disease. Truncus aorticus solitarius, single ventricle and aberrant coronary drainage into the common ventricle T2 - American Heart Journal TI - A case of congenital heart disease. Truncus aorticus solitarius, single ventricle and aberrant coronary drainage into the common ventricle UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L280603552 http://dx.doi.org/10.1016/0002-8703(50)90258-4 VL - 39 ID - 1427 ER - TY - JOUR AB - Wolf-Hirschhorn Syndrome (WHS) is a genetic syndrome that includes a typical facial appearance, mental retardation, growth delay, seizures, and congenital cardiac defects. A deletion of the terminal band of the short arm of chromosome 4, with a breakpoint at the 4p15 to 4p16 region, is the most common genetic mutation causing WHS. Congenital heart disease associated with WHS typically includes atrial and ventricular septal defects, though there are a few case reports of associated complex congenital heart disease. Here we report a case of an infant with a large 4p deletion, with a breakpoint at the 4p12 region, and hypoplasic left heart syndrome. We discuss a possible link between the size of the chromosomal deletion in WHS and the severity of the cardiac defect. © 2012 Springer Science+Business Media, LLC (outside the USA). AD - K. Von Elten, Department of Pediatrics, MCHK-PE, Tripler Army Medical Center, 1 Jarrett White Road, Honolulu, HI 96859, United States AU - Von Elten, K. AU - Sawyer, T. AU - Lentz-Kapua, S. AU - Kanis, A. AU - Studer, M. DB - Embase Medline DO - 10.1007/s00246-012-0367-8 IS - 5 KW - article case report chromosome deletion 4 congenital heart disease disease severity gene mutation human hypoplastic left heart syndrome infant Wolf Hirschhorn syndrome LA - English M3 - Article N1 - L368953102 2013-05-29 2013-06-05 PY - 2013 SN - 0172-0643 1432-1971 SP - 1244-1246 ST - A case of wolf-hirschhorn syndrome and hypoplastic left heart syndrome T2 - Pediatric Cardiology TI - A case of wolf-hirschhorn syndrome and hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L368953102 http://dx.doi.org/10.1007/s00246-012-0367-8 VL - 34 ID - 995 ER - TY - JOUR AB - As a result of advances and modifications in surgical procedures and the development of drugs for pulmonary arterial hypertension, many patients who have undergone Fontan procedures are able to enjoy good quality of life, without pulmonary arterial hypertension and severe complications. In Shizuoka Children's Hospital, drugs for pulmonary arterial hypertension have long been given to Fontan candidates and patients with established Fontan circulation to maintain sufficient pulmonary blood flow and suppress pulmonary arterial hypertension. We present three typical cases that were treated with anti-pulmonary hypertensive drugs before or after Fontan procedure. The first case had asplenia syndrome, and a single ventricle with major aortopulmonary collateral arteries. Anti-pulmonary hypertensive therapy permitted a Fontan procedure and maintained a good long-term quality of life. The second case was a Down syndrome patient who had progressive cyanosis after a Fontan operation. Anti-pulmonary hypertensive therapy improved cyanosis. The third case suffered from protein-losing enteropathy, for which all procedures and medical therapies were ineffective. Fontan candidates and patients with Fontan circulation have varied anatomical backgrounds and pulmonary properties. We must identify the conditions that lead to successful Fontan procedure and Fontan circulation correction, as well as conditions that result in failed Fontan procedure and poorly-controlled Fontan circulation. AD - Department of Cardiology, Shizuoka Children's Hospital. AN - 25787796 AU - Ono, Y. AU - Mitsushita, N. DB - PubMed DO - 10.1536/ihj.14-353 DP - NLM ET - 2015/03/20 KW - Down Syndrome/complications *Fontan Procedure Heart Defects, Congenital/complications/surgery Heterotaxy Syndrome/complications Humans Hypertension, Pulmonary/*drug therapy/surgery Infant Postoperative Care *Preoperative Care Protein-Losing Enteropathies/complications LA - eng N1 - 1349-3299 Ono, Yasuo Mitsushita, Norie Case Reports Journal Article Research Support, Non-U.S. Gov't Japan Int Heart J. 2015;56 Suppl:S31-4. doi: 10.1536/ihj.14-353. PY - 2015 SN - 1349-2365 SP - S31-4 ST - Case studies of patients successfully and unsuccessfully managed pre- and post-Fontan procedure T2 - Int Heart J TI - Case studies of patients successfully and unsuccessfully managed pre- and post-Fontan procedure VL - 56 Suppl ID - 195 ER - TY - JOUR AB - The majority of infant heart transplants are for the correction of hypoplastic left heart syndrome (HLHS). This article will describe the preoperative, intraoperative, and postoperative care for perioperative and pediatric intensive care areas implemented for HLHS infant heart transplantation at Medical Center Hospital, San Antonio, TX. The surgical procedure is given. Dimensions of psychosocial care and education of the parents are briefly described. AN - 1301868 AU - Feliciano, S. M. AU - Girard, N. J. AU - Calhoon, J. H. DA - Jan DB - PubMed DP - NLM ET - 1992/01/01 IS - 1 KW - Cardiac Surgical Procedures/methods/*nursing Humans Infant Infant, Newborn Neonatal Nursing/*methods Operating Room Nursing/*methods Patient Care Planning LA - eng N1 - Feliciano, S M Girard, N J Calhoon, J H Journal Article United States Semin Perioper Nurs. 1992 Jan;1(1):37-50. PY - 1992 SN - 1056-8670 (Print) 1056-8670 SP - 37-50 ST - Case study: infant transplantation: a change of heart T2 - Semin Perioper Nurs TI - Case study: infant transplantation: a change of heart VL - 1 ID - 488 ER - TY - JOUR AB - The acutely failing Fontan circulation requires urgent management to decompress the systemic venous pressures and augment cardiac output. This may involve the use of extracorporeal membrane oxygenation support to preserve life. Catheter-based effective Fontan takedown is an alternative to surgery in these patients, who are acutely unstable, and offers the potential for an adjustable fenestration. © 2011 The Society of Thoracic Surgeons. AD - Heart Unit, Birmingham Children's Hospital, Birmingham, United Kingdom AU - Anderson, B. W. AU - Barron, D. J. AU - Jones, T. J. AU - Edwards, L. AU - Brawn, W. AU - Stumper, O. DB - Scopus DO - 10.1016/j.athoracsur.2011.01.040 IS - 1 M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2011 SP - 346-348 ST - Catheter takedown in the management of the acutely failing Fontan circulation T2 - Annals of Thoracic Surgery TI - Catheter takedown in the management of the acutely failing Fontan circulation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-79959803618&doi=10.1016%2fj.athoracsur.2011.01.040&partnerID=40&md5=b2e5b729c5f52bf9e6c19ca5f0e72490 VL - 92 ID - 2126 ER - TY - JOUR AB - Adolescents with single ventricle heart disease (SVHD) exhibit mood and cognitive deficits, which may result from injury to the basal ganglia structures, including the caudate nuclei. However, the integrity of the caudate in SVHD adolescents is unclear. Our aim was to examine the global and regional caudate volumes, and evaluate the relationships between caudate volumes and cognitive and mood scores in SVHD and healthy adolescents. We acquired two high-resolution T1-weighted images from 23 SVHD and 37 controls using a 3.0-Tesla MRI scanner, as well as assessed mood (Patient Health Questionnaire-9 [PHQ-9]; Beck Anxiety Inventory [BAI]) and cognition (Montreal Cognitive Assessment [MoCA]; Wide Range Assessment of Memory and Learning-2; General Memory Index [GMI]) functions. Both left and right caudate nuclei were outlined, which were then used to calculate and compare volumes between groups using ANCOVA (covariates: age, gender, and head-size), as well as perform 3D surface morphometry. Partial correlations (covariates: age, gender, and head-size) were used to examine associations between caudate volumes, cognition, and mood scores in SVHD and controls. SVHD subjects showed significantly higher PHQ-9 and BAI scores, indicating more depressive and anxiety symptoms, as well as reduced GMI scores, suggesting impaired cognition, compared to controls. SVHD patients showed significantly reduced caudate volumes (left, 3,198.8 ± 490.1 vs. 3,605.0 ± 480.4 mm(3) , p < 0.004; right, 3,162.1 ± 475.4 vs. 3,504.8 ± 465.9 mm(3) , p < 0.011) over controls, and changes were localized in the rostral, mid-dorsolateral, and caudal areas. Significant negative correlations emerged between caudate volumes with PHQ-9 and BAI scores and positive correlations with GMI and MoCA scores in SVHD and controls. SVHD adolescents show significantly reduced caudate volumes, especially in sites that have projections to regulate mood and cognition, which may result from developmental and/or hypoxia-/ischemia-induced processes. AD - Department of Anesthesiology, David Geffen School of Medicine at UCLA, University of California Los Angeles, Los Angeles, CA, USA. UCLA School of Nursing, University of California Los Angeles, Los Angeles, CA, USA. Division of Pediatric Cardiology, University of California Los Angeles, Los Angeles, CA, USA. Division of Pediatric Cardiology, Children's Hospital Los Angeles, Los Angeles, CA, USA. Department of Radiological Sciences, University of California Los Angeles, Los Angeles, CA, USA. Department of Bioengineering, University of California Los Angeles, Los Angeles, CA, USA. Brain Research Institute, University of California Los Angeles, Los Angeles, CA, USA. AN - 32530059 AU - Noorani, S. AU - Roy, B. AU - Sahib, A. K. AU - Cabrera-Mino, C. AU - Halnon, N. J. AU - Woo, M. A. AU - Lewis, A. B. AU - Pike, N. A. AU - Kumar, R. DA - Jun 12 DB - PubMed DO - 10.1002/jnr.24667 DP - NLM ET - 2020/06/13 KW - 3D surface morphometry anxiety basal ganglia congenital heart disease depression magnetic resonance imaging LA - eng N1 - 1097-4547 Noorani, Sarah Orcid: 0000-0002-3289-4120 Roy, Bhaswati Sahib, Ashish K Cabrera-Mino, Cristina Halnon, Nancy J Woo, Mary A Lewis, Alan B Pike, Nancy A Kumar, Rajesh Orcid: 0000-0001-5355-9586 R01 NR-016463/National Institute of Nursing Research/ Journal Article United States J Neurosci Res. 2020 Jun 12. doi: 10.1002/jnr.24667. PY - 2020 SN - 0360-4012 ST - Caudate nuclei volume alterations and cognition and mood dysfunctions in adolescents with single ventricle heart disease T2 - J Neurosci Res TI - Caudate nuclei volume alterations and cognition and mood dysfunctions in adolescents with single ventricle heart disease ID - 353 ER - TY - JOUR AB - All deceased infants were autopsied in a territory with a population of 1,220,000 inhabitants and a mean of 17,410 live births a year. During a period of 25 years (which preceded the introduction of advanced neonatal cardiac surgery), 393 neonates (6% of all deceased neonates) had died in whom a congenital heart malformation was found at autopsy. 118 (30%) of deaths were due to noncardiac causes. Lethal congenital noncardiac malformations caused death in 38 (10%) neonates. A perinatal disease (including birth trauma) was the cause in 65 (16%) neonates. An association of a noncardiac congenital malformation with a perinatal disease was involved in 6 (2%) neonates and a birth weight lower than 1000 g (together with a lethal perinatal disease or alone) was considered responsible in 9 (2%) neonates. Noncardiac but not unambiguously lethal malformations (mostly multiple) could at least have contributed to the fatal outcome in a further 86 (22%) neonates. A low birth weight could have contributed to death in 102 (26%) neonates, particularly in 42 (11%) neonates with a low birth weight associated with multiple noncardiac nonlethal malformations. A most probable or possible noncardiac cause of death was found in all cases of atrial septal defect, in 93% of tetralogies of Fallot, in 84% of ventricular septal defects, in 72% of hearts with double outlet right ventricle and in 57% of those with aortic coarctation. A congenital heart malformation was considered responsible for a fatal outcome in 82% of cases with discordant ventriculoarterial connexions ('transposition of the great arteries'), 79% of hypoplastic left hearts, 68% of pulmonary atresias and 53% of tricuspid atresias. The high proportion of noncardiac death noted to occur in the first postnatal day was found rapidly to decrease in the following days. AD - Centre of Paediatric Cardiology and Cardiovascular Surgery, University Hospital Motol, 150 18 Prague 5 AU - Samanek, M. AU - Goetzova, J. AU - Benesova, D. DB - Embase Medline DO - 10.1016/0167-5273(86)90200-7 IS - 1 KW - autopsy cause of death congenital disorder congenital heart disease fatality heart heart death human major clinical study newborn priority journal LA - English M3 - Article N1 - L16163031 1986-05-27 PY - 1986 SN - 0167-5273 SP - 63-74 ST - Causes of death in neonates born with a heart malformation T2 - International Journal of Cardiology TI - Causes of death in neonates born with a heart malformation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L16163031 http://dx.doi.org/10.1016/0167-5273(86)90200-7 VL - 11 ID - 1394 ER - TY - JOUR AB - OBJECTIVESThere is an increasing trend to perform the bidirectional superior cavopulmonary (Glenn) anastomosis without cardiopulmonary bypass. In this report, we present our results of off-pump bidirectional Glenn operation done without using a venoatrial shunt to decompress the randomized comparative study was superior vena cava during clamping.METHODSA prospective, non-randomized comparative study was conducted in 50 patients with functional single ventricle anomalies who underwent bidirectional Glenn anastomosis without cardiopulmonary bypass. The patients were divided into two groups: Group I (n = 25), where it was done without a veno-atrial shunt, and Group II (n = 25), where it was done with a veno-atrial shunt. Two patients in Group I and 4 patients in Group II had a bilateral bidirectional Glenn shunt. Five patients in Group I and three patients in Group II had a previous left modified Blalock-Taussig shunt. All patients underwent a complete neurological examination both preoperatively as well as postoperatively.RESULTSThe early hospital mortality was 4% (2/50), one in each group. The median follow-up was 14 months. The mean internal venous pressure on clamping the superior vena cava was 37.07 ± 7.12 mmHg in Group I and 24 ± 4.4 mmHg in Group II. The mean clamp time was 9.85 ± 3.52 min in Group I and 21.3 ± 4.4 min in Group II. The transcranial pressure gradient was 62.37 ± 15.01 mmHg in Group I, while 65.08 ± 13.89 in Group II. The mean intensive care unit stay was 2.57 ± 75 days in Group I, 3.3 ± 1.09 in Group II. There were no major neurological complications apart from treatable convulsions in one case in Group I (4%), 2 cases in Group II (8%), and delayed recovery in one case (4%) in Group I.CONCLUSIONSOff-pump bidirectional Glenn operation without caval decompression is a safe, simple and more economic procedure. © 2013 The Author 2013. AD - Department of Cardiovascular and Thoracic Surgery, Ain Shams University Hospitals, Ramses Str. Abbassia, 11588 Cairo, Egypt AU - Mostafa, E. A. AU - El Midany, A. A. H. AU - Zalat, M. M. AU - Helmy, A. DB - Scopus DO - 10.1093/icvts/ivs518 IS - 5 KW - Bidirectional Glenn shunt Coronary heart disease Off-pump surgery Univentricular heart M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2013 SP - 649-654 ST - Cavopulmonary anastomosis without cardiopulmonary bypass T2 - Interactive Cardiovascular and Thoracic Surgery TI - Cavopulmonary anastomosis without cardiopulmonary bypass UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84876563377&doi=10.1093%2ficvts%2fivs518&partnerID=40&md5=7460e5812489cacaf2929aed5cd4916e VL - 16 ID - 2051 ER - TY - JOUR AB - BACKGROUND: Congenital heart disease (CHD) is a leading birth defect in the United States, affecting about 40 000 neonates each year. Despite efforts to prevent developmental delays, many children with CHD have neurological deficits that last into adulthood, influencing employability, self-care, and quality of life. OBJECTIVE: To determine if neonates with CHD have impaired cerebral autoregulation and poorer neurodevelopmental outcomes compared with healthy controls. METHODS: A total of 44 full-term neonates, 28 with CHD and 16 without, were enrolled in the study. Inclusion criteria included confirmed diagnosis of CHD, stable hemodynamic status, and being no more than 12 days old. Exclusion criteria included intraventricular hemorrhage and intubation. Cerebral autoregulation was determined by measuring regional cerebral oxygenation during a postural change. The Einstein Neonatal Neurobehavioral Assessment Scale was used to measure overall neurodevelopmental outcomes (motor, visual, and auditory functions). RESULTS: Of the 28 neonates with CHD, 8 had single-ventricle physiology. A χ(2) analysis indicated no significant difference in impaired cerebral autoregulation between neonates with CHD and controls (P = .38). Neonates with CHD had lower regional cerebral oxygenation than did neonates without CHD (P < .001). Regression analyses with adjustments for cerebral autoregulation indicated that neonates with CHD had poorer total neurodevelopmental outcomes scores (β = 9.3; P = .02) and motor scores (β = 7.6; P = .04). CONCLUSION: Preoperative neonates with CHD have poorer developmental outcomes and more hypoxemia than do controls. AD - Nhu N. Tran is a clinical research nurse III, Department of Cardiothoracic Surgery, Children's Hospital Los Angeles, Los Angeles, California. Ram Kumar is an assistant professor of surgery, Keck School of Medicine, University of Southern California, Los Angeles, California. Felicia S. Hodge is a professor and Paul M. Macey is an associate professor, School of Nursing, University of California, Los Angeles. ntran@chla.usc.edu. Nhu N. Tran is a clinical research nurse III, Department of Cardiothoracic Surgery, Children's Hospital Los Angeles, Los Angeles, California. Ram Kumar is an assistant professor of surgery, Keck School of Medicine, University of Southern California, Los Angeles, California. Felicia S. Hodge is a professor and Paul M. Macey is an associate professor, School of Nursing, University of California, Los Angeles. AN - 30173174 AU - Tran, N. N. AU - Kumar, S. R. AU - Hodge, F. S. AU - Macey, P. M. DA - Sep DB - PubMed DO - 10.4037/ajcc2018672 DP - NLM ET - 2018/09/03 IS - 5 KW - Brain/*metabolism Case-Control Studies Cross-Sectional Studies Developmental Disabilities/etiology Female Heart Defects, Congenital/complications/*physiopathology Homeostasis/*physiology Humans Hypoxia/etiology Infant, Newborn Male Neurologic Examination Oxygen/*metabolism Prospective Studies Spectroscopy, Near-Infrared LA - eng N1 - 1937-710x Tran, Nhu N Kumar, S Ram Hodge, Felicia S Macey, Paul M Journal Article Research Support, Non-U.S. Gov't United States Am J Crit Care. 2018 Sep;27(5):410-416. doi: 10.4037/ajcc2018672. PY - 2018 SN - 1062-3264 SP - 410-416 ST - Cerebral Autoregulation in Neonates With and Without Congenital Heart Disease T2 - Am J Crit Care TI - Cerebral Autoregulation in Neonates With and Without Congenital Heart Disease VL - 27 ID - 538 ER - TY - JOUR AB - Background Congenital heart disease (CHD) is a leading birth defect in the United States, affecting about 40 000 neonates each year. Despite efforts to prevent developmental delays, many children with CHD have neurological deficits that last into adulthood, influencing employability, self-care, and quality of life. Objective To determine if neonates with CHD have impaired cerebral autoregulation and poorer neurodevelopmental outcomes compared with healthy controls. Methods A total of 44 full-term neonates, 28 with CHD and 16 without, were enrolled in the study. Inclusion criteria included confirmed diagnosis of CHD, stable hemodynamic status, and being no more than 12 days old. Exclusion criteria included intraventricular hemorrhage and intubation. Cerebral autoregulation was determined by measuring regional cerebral oxygenation during a postural change. The Einstein Neonatal Neurobehavioral Assessment Scale was used to measure overall neurodevelopmental outcomes (motor, visual, and auditory functions). Results Of the 28 neonates with CHD, 8 had single-ventricle physiology. A χ2 analysis indicated no significant difference in impaired cerebral autoregulation between neonates with CHD and controls (P = .38). Neonates with CHD had lower regional cerebral oxygenation than did neonates without CHD (P < .001). Regression analyses with adjustments for cerebral autoregulation indicated that neonates with CHD had poorer total neurodevelopmental outcomes scores (β = 9.3; P = .02) and motor scores (β = 7.6; P = .04). Conclusion Preoperative neonates with CHD have poorer developmental outcomes and more hypoxemia than do controls. © 2018 American Association of Critical-Care Nurses. AD - Department of Cardiothoracic Surgery, Children's Hospital Los Angeles, Los Angeles, CA, United States Keck School of Medicine, University of Southern California, Los Angeles, CA, United States School of Nursing, University of California, Los Angeles, United States AU - Tran, N. N. AU - Ram Kumar, S. AU - Hodge, F. S. AU - Macey, P. M. DB - Scopus DO - 10.4037/ajcc2018672 IS - 5 M3 - Article N1 - Export Date: 15 June 2020 PY - 2018 SP - 410-416 ST - Cerebral autoregulation in neonates with and without congenital heart disease T2 - American Journal of Critical Care TI - Cerebral autoregulation in neonates with and without congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85052832886&doi=10.4037%2fajcc2018672&partnerID=40&md5=56d80b20a44b380042f6b10d31f8013e VL - 27 ID - 1790 ER - TY - JOUR AB - Hypoplastic left heart syndrome (HLHS) palliation may result in altered cerebral blood flow with subsequent neurodevelopmental implications. The purpose of the study was to assess blood flow in the middle cerebral artery (MCA) and investigate the relationship with early neurodevelopmental outcomes in infants with HLHS after hybrid stage I. Transcranial Doppler (TCD) was performed to obtain peak systolic, end-diastolic, and mean velocities, as well as pulsatility index of the MCA in infants with HLHS (n = 18) at baseline and at 2, 4, and 6 months of age. Developmental assessment was performed at 6 months of age. Results of TCD and development were compared to healthy control subjects (n = 6) and normative data. Overall, peak systolic velocity (p = 0.0031), end-diastolic velocity (p < 0.0001), and mean velocity (p < 0.0001) were significantly lower and pulsatility index (p = 0.0011) significantly higher in the HLHS group compared to the control group. A significant increase in change over time was noted for peak systolic velocity (p < 0.0016) and mean velocity (p < 0.0046). There was no significant correlation between TCD variables and development scores. TCD values in 5–6 months old infants with HLHS who undergo hybrid stage I palliation had consistently lower blood flow velocities than control infants; however, pulsatility index was slightly higher. No correlation between TCD measurements and measures of cognitive, language, and motor skills were noted. AD - S.L. Cheatham, The Heart Center, Nationwide Children’s Hospital, 700 Children’s Drive, Columbus, OH, United States AU - Cheatham, S. L. AU - Chisolm, J. L. AU - O’Brien, N. DB - Embase Medline DO - 10.1007/s00246-018-1836-5 IS - 4 KW - Companion III ultrasound transducer artery blood flow article blood flow velocity brain blood flow brain perfusion child development clinical article cognitive development controlled study female gestational age head circumference heart surgery heart ventricle enddiastolic pressure human hypoplastic left heart syndrome language development longitudinal study male middle cerebral artery motor performance newborn palliative therapy prospective study pulsatility index systolic blood pressure transcranial Doppler ultrasonography treatment planning LA - English M3 - Article N1 - L621564804 2018-04-11 2018-08-09 PY - 2018 SN - 1432-1971 0172-0643 SP - 837-843 ST - Cerebral Blood Flow Following Hybrid Stage I Palliation in Infants with Hypoplastic Left Heart Syndrome T2 - Pediatric Cardiology TI - Cerebral Blood Flow Following Hybrid Stage I Palliation in Infants with Hypoplastic Left Heart Syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L621564804 http://dx.doi.org/10.1007/s00246-018-1836-5 VL - 39 ID - 693 ER - TY - JOUR AB - OBJECTIVES: Compared with normal fetuses, fetuses with hypoplastic left heart syndrome (HLHS) have smaller brain volumes and are at higher risk of brain injury, possibly due to diminished cerebral blood flow and oxygen content. By increasing cerebral oxygen delivery, maternal hyperoxygenation (MH) might improve brain development and reduce the risk of brain injury in these fetuses. This study investigated whether gestational age and baseline cerebrovascular resistance affect the response to MH in fetuses with HLHS. METHODS: The study population comprised 43 fetuses with HLHS or HLHS variant referred for fetal echocardiography between January 2004 and September 2008. Middle cerebral artery (MCA) pulsatility index (PI), a surrogate measure of cerebrovascular resistance, was assessed between 20 and 41 weeks' gestation at baseline in room air (RA) and after 10 min of MH. Z-scores of MCA-PI were generated. A mixed-effects model was used to determine whether change in MCA-PI depends upon gestational age and baseline MCA-PI. RESULTS: In RA and following MH, MCA-PI demonstrated a curvilinear relationship with gestational age in fetuses with HLHS, peaking at around 28 weeks and then falling more steeply near term. MCA-PI Z-score declined in a linear manner, such that it was 1.4 SD below that in normal fetuses at 38 weeks. Increase in MCA-PI Z-score after MH was first seen at ≥ 28 weeks. A baseline MCA-PI Z-score ≤ -0.96 was predictive of an increase in cerebrovascular resistance in response to MH. CONCLUSION: In fetuses with HLHS, MCA-PI first increases in response to MH at ≥ 28 weeks' gestation. A baseline MCA-PI Z-score ≤ -0.96 predicts an increase in cerebrovascular resistance in response to MH. These results may have implications for clinical trials utilizing MH as a neuroprotective agent. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd. AU - Szwast, A. AU - Putt, M. AU - Gaynor, J. W. AU - Licht, D. J. AU - Rychik, J. DB - Medline DO - 10.1002/uog.18919 IS - 4 KW - oxygen adult blood brain circulation diagnostic imaging echocardiography embryology female fetus echography fetus monitoring gestational age human hypoplastic left heart syndrome mental disease metabolism middle cerebral artery mother newborn oxygen therapy pathophysiology physiology placenta pregnancy pulsatile flow treatment outcome vascularization LA - English M3 - Article N1 - L624217167 2018-10-22 2019-02-18 PY - 2018 SN - 1469-0705 SP - 473-478 ST - Cerebrovascular response to maternal hyperoxygenation in fetuses with hypoplastic left heart syndrome depends on gestational age and baseline cerebrovascular resistance T2 - Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology TI - Cerebrovascular response to maternal hyperoxygenation in fetuses with hypoplastic left heart syndrome depends on gestational age and baseline cerebrovascular resistance UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L624217167 http://dx.doi.org/10.1002/uog.18919 VL - 52 ID - 663 ER - TY - JOUR AB - Congenital heart disease (CHD) is one of major categories of illness that, if treated, can restore health and improve quality of life. Unfortunately, in many developing countries, health professionals may not have the resources to replenish themselves with modern technology and/or surgery skills to treat a child with complex CHD. Until now, CHD remained a health challenge for many children and families. During the past few decades, due to limited medical resources and operative techniques, only a very small proportion of the children with CHD in our region were able to be operated for lesion repair. However, the situation in the last 20 years has changed dramatically. Treatment for pediatric CHDs has been greatly improved and developed in China. The collaboration with Project HOPE, an international health professional education organization, has promoted the development of our specialty. Specifically, Project HOPE has played an important role in facilitating the collaboration between Shangahi Xin Hua Hospital, Shanghai Children's Medical Center, and many world-leading pediatric health care institutions. With contributions from Project HOPE in the last 25 years, cardiovascular surgeons at Shanghai Children's Medical Center have achieved tremendous progress. The landmark of the cardiac center and successful clinical data in treating children with cardiac illness symbolizes the successful collaboration among a state-owned hospital, a non-profit organization-Project HOPE, and multiple international corporations, which have provided valuable resources to facilitate the growth of the hospital into a state-of-the-art tertiary hospital to serve children and families. © 2009 Elsevier Inc. All rights reserved. AD - J. Liu, Department of Thoracic and Cardiovascular Surgery, Shanghai Children's Medical Center, Shanghai, China AU - Liu, J. DB - Embase Medline DO - 10.1053/j.pcsu.2009.01.020 IS - 1 KW - article Blalock Taussig shunt child health care China clinical competence congenital heart disease endocardial cushion defect Fontan procedure Glenn shunt great vessels transposition health practitioner heart atrium septum defect heart right ventricle double outlet heart single ventricle heart surgery heart ventricle septum defect human international cooperation medical education patent ductus arteriosus pediatric hospital pediatric surgery surgical technique LA - English M3 - Article N1 - L354401222 2009-04-17 PY - 2009 SN - 1092-9126 SP - 12-18 ST - Challenges and Progress of the Pediatric Cardiac Surgery in Shanghai Children's Medical Center: A 25-year Solid Collaboration With Project HOPE T2 - Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual TI - Challenges and Progress of the Pediatric Cardiac Surgery in Shanghai Children's Medical Center: A 25-year Solid Collaboration With Project HOPE UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L354401222 http://dx.doi.org/10.1053/j.pcsu.2009.01.020 VL - 12 ID - 1184 ER - TY - JOUR AB - OBJECTIVE: Infants with "single ventricle" congenital heart disease are at high risk for sudden death following palliative surgical management. We developed a pilot telemedicine project to evaluate the feasibility of using Web-based daily reporting of clinical data with the goal of reducing unexpected admissions and sudden death. SUBJECTS AND METHODS: We enrolled 9 subjects (enrolled subjects [ES]) following surgical palliation over 12 months. Parents electronically transmitted ES daily weight and oxygen saturation and then completed an automated 10-point phone questionnaire on nutrition, activity, and distress. Subject enrollment continued until a second surgical palliative procedure (n=5), sudden death (n=2), or disenrollment (n=2). We collected clinical data on all ES and 9 historical controls (HC) from the preceding 18 months and analyzed clinical management, including outpatient telephone surveillance success, scheduled and unscheduled office and emergency department visits, hospitalizations, procedures, and adverse events, including death. RESULTS: Subject recruitment was more difficult than expected. Weight transmission success was high, but there was poor correlation between telemedicine system-measured oxygen saturation and a commercial monitor. The outpatient clinical telephone surveillance success rate for HC and ES was approximately 30%. After technical adjustments, parents of all ES (100%) were able to transmit questionnaire data. There were 9 emergency room visits for ES versus 11 unscheduled emergency room visits for HC. Sudden death occurred in 1 of 9 HC and 2 of 9 ES. CONCLUSIONS: Telemedicine monitoring for high-risk congenital heart disease patients is feasible but challenging, may reduce unscheduled visits, but may not impact the primary end point of preventing sudden death in this high-risk pediatric population. AD - 1 Kosair Charities Pediatric Heart Research Program, Cardiovascular Innovation Institute , Louisville, Kentucky. AN - 25083905 AU - Black, A. K. AU - Sadanala, U. K. AU - Mascio, C. E. AU - Hornung, C. A. AU - Keller, B. B. DA - Sep DB - PubMed DO - 10.1089/tmj.2013.0343 DP - NLM ET - 2014/08/02 IS - 9 KW - Body Weight Cardiology/*methods Death, Sudden, Cardiac Emergency Service, Hospital/statistics & numerical data Feasibility Studies Female Heart Defects, Congenital/mortality/*surgery Home Care Services, Hospital-Based/*organization & administration Humans Infant Infant, Newborn *Internet Male Monitoring, Physiologic/methods Oxygen/blood Palliative Care Pediatrics/*methods Pilot Projects Surveys and Questionnaires Telemedicine/*methods congenital heart disease home health monitoring hypoplastic left heart syndrome interstage sudden death pediatrics telemedicine LA - eng N1 - 1556-3669 Black, Allison K Sadanala, Usha K Mascio, Christopher E Hornung, Carlton A Keller, Bradley B Journal Article Research Support, Non-U.S. Gov't United States Telemed J E Health. 2014 Sep;20(9):858-67. doi: 10.1089/tmj.2013.0343. Epub 2014 Aug 1. PY - 2014 SN - 1530-5627 SP - 858-67 ST - Challenges in implementing a pediatric cardiovascular home telehealth project T2 - Telemed J E Health TI - Challenges in implementing a pediatric cardiovascular home telehealth project VL - 20 ID - 367 ER - TY - JOUR AB - Introduction: While in developed countries prenatal diagnosis is currently used to detect Congenital Heart Disease before (CHD) before birth, in developing countries only a minority of children with CHD is detected and few benefit from surgical treatment. Having created a referral unit for diagnosis and treatment of cardiovascular diseases in a resource-deprived country we designed a study aiming at describing the spectrum and characteristics of CHD and discuss the challenges of its management. Population and methods: We studied retrospectively all patients assisted at a referral unit between 2001 and 2007, collecting epidemiological, clinical, echocardiographic and surgical data from hospital files. Results: We studied 534 patients with median age at diagnosis of 4 years (range 0-79); 296 were females (55.4%). Only 282 (52.8%) patients were diagnosed under the age of two years, and complications were present in 155 (29.0%) at time of diagnosis. We operated 196 patients with mean age of 8 ± 10 years. Only 29 of the 111 complex defects were considered for surgery. The 30-days post-operative mortality was 8/196 (4.0%). The most important post-surgical complications were post pericardectomy syndrome (22). Discussion: A pattern of late presentation accompanied by high rate of complications was found. In resource-deprived settings the approach to the management of CHD emphasizes the treatment of "curable" malformations. Surgery for CHD in these settings must be linked to early detection and a referral system in which general practitioners, pediatricians, obstetricians and cardiologists interact in the design and implementation protocols for diagnosis, management and follow-up of patients. © 2009 Elsevier Ireland Ltd. AD - A. O. Mocumbi, Instituto Do Coração, Av Kenneth Kaunda 1111, Maputo, Mozambique AU - Mocumbi, A. O. AU - Lameira, E. AU - Yaksh, A. AU - Paul, L. AU - Ferreira, M. B. AU - Sidi, D. DB - Embase Medline DO - 10.1016/j.ijcard.2009.11.006 IS - 3 KW - adolescent adult age aged aortic aneurysm aortic valve disease arterial trunk article atrioventricular septal defect bacterial endocarditis bleeding cardiomyopathy child congenital heart disease cyanotic heart disease developing country disease severity Fallot tetralogy female great vessels transposition heart arrhythmia heart atrium septum defect heart failure heart left right shunt heart right ventricle double outlet heart right ventricle outflow tract obstruction heart single ventricle heart surgery heart ventricle septum defect human Human immunodeficiency virus infection kidney failure lung insufficiency major clinical study male malnutrition mitral valve regurgitation neurologic disease neurological complication palliative therapy patent ductus arteriosus polycythemia postoperative complication priority journal pulmonary hypertension pulmonary valve atresia pulmonary valve stenosis reoperation retrospective study situs inversus cerebrovascular accident surgical mortality treatment contraindication treatment refusal tricuspid valve atresia LA - English M3 - Article N1 - L50710522 2009-11-24 2011-05-06 PY - 2011 SN - 0167-5273 SP - 285-288 ST - Challenges on the management of congenital heart disease in developing countries T2 - International Journal of Cardiology TI - Challenges on the management of congenital heart disease in developing countries UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50710522 http://dx.doi.org/10.1016/j.ijcard.2009.11.006 VL - 148 ID - 1104 ER - TY - JOUR AB - BACKGROUND AND AIMS: Several years ago, one of us described the difference in attitude to patients with hypoplastic left heart syndrome in the United States of America and Europe as similar to that between Mars and Venus. Uncertainty remains with regard to the long-term prognosis for patients with hypoplastic left heart syndrome. This prognosis may be considered in terms of survival, functional performance, including exercise capacity and neurodevelopment, as well as psychosocial effects on the patient, family, and siblings. Counselling parents where either an antenatal or postnatal diagnosis of hypoplastic left heart syndrome has been made requires practitioners to give information on these prognostic aspects. We wanted to see how attitudes among European surgeons have changed over the last few years. METHODS: We performed a review of recent European data for hypoplastic left heart syndrome and conducted a survey among surgeons in major European centres to ascertain key aspects of their attitudes to the management of patients with hypoplastic left heart syndrome and how they counsel parents. RESULTS AND CONCLUSIONS: As of January, 2011, 2392 citations in the PubMed database were available for the search string "hypoplastic left heart". The majority of these were from the centres from the United States of America and Europe. The European Association for Cardio-Thoracic Surgery Congenital Heart Surgery Database shows an annual increase in the number of Norwood (Stage I) operations for hypoplastic left heart syndrome from 2003 to 2009, with a corresponding reduction in mortality. European rates of antenatal detection vary widely between centres, as do the rates of termination for a prenatal diagnosis of hypoplastic left heart syndrome. We observed a wide variation in the estimates of surgeons for survival and quality of life for surgical palliation of hypoplastic left heart syndrome, as well as in their estimates for actual rates of termination of pregnancy in their centres. Further, there was marked inconsistency in the information given to parents as part of the process of counselling. These issues remain to be resolved if parents are to make a fully informed decision for their child. AD - Department of Paediatric Cardiothoracic Surgery, The Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom. AN - 22152542 AU - Murtuza, B. AU - Elliott, M. J. DA - Dec DB - PubMed DO - 10.1017/s1047951111001739 DP - NLM ET - 2011/12/14 KW - *Attitude of Health Personnel Cardiac Surgical Procedures/*methods *Disease Management Europe Female Humans *Hypoplastic Left Heart Syndrome/diagnosis/embryology/surgery Infant, Newborn Pregnancy *Pregnancy Complications, Cardiovascular Prenatal Diagnosis LA - eng N1 - 1467-1107 Murtuza, Bari Elliott, Martin J Journal Article Review England Cardiol Young. 2011 Dec;21 Suppl 2:148-58. doi: 10.1017/S1047951111001739. PY - 2011 SN - 1047-9511 SP - 148-58 ST - Changing attitudes to the management of hypoplastic left heart syndrome: a European perspective T2 - Cardiol Young TI - Changing attitudes to the management of hypoplastic left heart syndrome: a European perspective VL - 21 Suppl 2 ID - 143 ER - TY - JOUR AB - The placenta is a complex organ that influences prenatal growth and development, and through fetal programming impacts postnatal health and well-being lifelong. Little information exists on placental pathology in the presence of congenital heart disease (CHD). Our objective is to characterize the placenta in CHD and investigate for distinctions based on type of malformation present. Placental pathology from singleton neonates prenatally diagnosed and delivered at > 37 weeks gestation was analyzed. Placental findings of absolute weight, placental weight-to-newborn birth weight ratio, chorangiosis, villus maturity, thrombosis, and infarction were recorded and analyzed based on four physiological categories of CHD: (1) single ventricle-aortic obstruction, (2) single ventricle-pulmonic obstruction, (3) two-ventricle anomalies, and (4) transposition of the great arteries (TGA). Associations between fetal Doppler assessments of middle cerebral/umbilical arterial flow and placental findings were investigated. A total of 120 cases of complex CHD were analyzed. Overall placental-to-birth weight ratios were < 10th percentile for 77% and < 3rd percentile for 49% with abnormalities of chorangiosis (18%), hypomature villi (15%), thrombosis (41%), and infarction (17%) common. There was no association between fetal Doppler flow measures and placental abnormalities. Newborns with TGA had the greatest degree of placental abnormality. Placentas of newborns with CHD are smaller than expected and manifest a number of vascular abnormalities, with TGA most prominent. Fetal Doppler does not correlate with these abnormalities. Studies investigating the relationship between placental abnormalities and postnatal outcomes may offer insight into the fetal origins of outcome variability in CHD. AD - J. Rychik, Fetal Heart Program, Cardiac Center, Children’s Hospital of Philadelphia, 3401 Civic Center Boulevard, Philadelphia, PA, United States AU - Rychik, J. AU - Goff, D. AU - McKay, E. AU - Mott, A. AU - Tian, Z. AU - Licht, D. J. AU - Gaynor, J. W. DB - Embase Medline DO - 10.1007/s00246-018-1876-x IS - 6 KW - aortic occlusion artery blood flow article birth weight cardiovascular malformation chorion villus cohort analysis congenital heart disease Doppler echocardiography fetus gestational age great vessels transposition human infarction major clinical study middle cerebral artery newborn obstetric delivery placenta placenta weight pulmonary valve stenosis thrombosis umbilical artery LA - English M3 - Article N1 - L622011690 2018-05-11 2018-11-21 PY - 2018 SN - 1432-1971 0172-0643 SP - 1165-1171 ST - Characterization of the Placenta in the Newborn with Congenital Heart Disease: Distinctions Based on Type of Cardiac Malformation T2 - Pediatric Cardiology TI - Characterization of the Placenta in the Newborn with Congenital Heart Disease: Distinctions Based on Type of Cardiac Malformation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L622011690 http://dx.doi.org/10.1007/s00246-018-1876-x VL - 39 ID - 672 ER - TY - JOUR AB - Performing chest CT angiography on pediatric patients on extracorporeal membrane oxygenation (ECMO) can be challenging. Successfully performing CT angiography in these children requires substantial communication and coordination between the radiologists and clinical care providers. Additionally, the radiologist must understand the child’s anatomy and disease pathophysiology, flow dynamics of the ECMO circuit, image acquisition timing, contrast injection site, and volume, rate and duration of contrast administration. In this article we highlight the vital factors the radiologist needs to consider to optimize the chest CT angiography in pediatric patients on ECMO. © 2017, Springer-Verlag GmbH Germany, part of Springer Nature. AD - Department of Radiology, Mayo Clinic, Division of Cardiovascular Radiology, 200 1st St. SW, Rochester, MN 55905, United States Department of Radiology, Duke Children’s Health Center, Duke University Medical Center, Durham, NC, United States AU - Hull, N. C. AU - Schooler, G. R. AU - Binkovitz, L. A. AU - Williamson, E. E. AU - Araoz, P. A. AU - Yu, L. AU - Young, P. M. DB - Scopus DO - 10.1007/s00247-017-4034-3 IS - 7 KW - Chest Children Computed tomography Computed tomography angiography Extracorporeal membrane oxygenation (ECMO) M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2018 SP - 1021-1030 ST - Chest computed tomography angiography in children on extracorporeal membrane oxygenation (ECMO) T2 - Pediatric Radiology TI - Chest computed tomography angiography in children on extracorporeal membrane oxygenation (ECMO) UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85037356966&doi=10.1007%2fs00247-017-4034-3&partnerID=40&md5=0bf15a3601bf175bfbd1b18201c9df81 VL - 48 ID - 1794 ER - TY - JOUR AB - The objective of this study was to evaluate the active lifestyle capacity (daily physical activity, strength, flexibility, body composition) of children after the Fontan procedure; hypothesized to be lower than healthy peers. Participants (n = 64, 25 females) were 9.0 ± 1.7 years of age (range 6.0–11.7 years). Fontan completion occurred at 3.3 ± 1.4 years of age (5.7 ± 2.0 years prior). Canadian Health Measures Survey protocols assessed aerobic endurance (paced walking up/down steps), strength (handgrip), flexibility (sit and reach), body composition (body mass index), and daily moderate-to-vigorous physical activity (7-day accelerometry). Difference scores compared participant data to published norms (t tests). Linear regression evaluated age/gender/demographic factor associations. Children after Fontan had strength scores similar (mean difference 1.1 kg) to their peers were less likely to be obese (mean difference of body mass index = 1.1 ± 2.5, p = 0.001) and performed 50 min of moderate-to-vigorous activity (MVPA) per day (12 ± 17 min/day below healthy peers, p < 0.001). Estimated peak endurance (61 % of expected) and flexibility (64 % of expected) were lower than peers (p < 0.001). Almost all (60/63) participants demonstrated the capacity to perform at least 20 min of MVPA per day. Difference from norms was smaller among children younger at Fontan completion (4 ± 2 min/year) and taking antithrombotic medication (7 ± 18 and 22 ± 17 min/day for taking/not taking, respectively). Children after Fontan demonstrate the capacity for the daily physical activity associated with optimal health. They have similar strength and good body composition. We recommend that children after Fontan be counselled that they can successfully participate in healthy, active lifestyles and physically active peer play. AD - P.E. Longmuir, Children’s Hospital of Eastern Ontario Research Institute, 401 Smyth Road, RI#1-214, Ottawa, ON, Canada AU - Longmuir, P. E. AU - Corey, M. AU - Faulkner, G. AU - Russell, J. L. AU - McCrindle, B. W. DB - Embase DO - 10.1007/s00246-014-1080-6 IS - 4 KW - anticoagulant agent accelerometry article body composition body mass Canadian child controlled study endurance exercise female Fontan procedure hand grip heart ventricle function human lifestyle major clinical study male outcome assessment oxygen consumption physical activity rural area strength tricuspid valve atresia LA - English M3 - Article N1 - L600783032 2014-12-22 2015-06-29 PY - 2015 SN - 1432-1971 0172-0643 SP - 759-767 ST - Children After Fontan have Strength and Body Composition Similar to Healthy Peers and Can Successfully Participate in Daily Moderate-to-Vigorous Physical Activity T2 - Pediatric Cardiology TI - Children After Fontan have Strength and Body Composition Similar to Healthy Peers and Can Successfully Participate in Daily Moderate-to-Vigorous Physical Activity UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L600783032 http://dx.doi.org/10.1007/s00246-014-1080-6 VL - 36 ID - 880 ER - TY - JOUR AB - OBJECTIVE: Previous studies suggest that children with congenital cardiac diagnoses report lower quality of life when compared with healthy norms. A few studies have evaluated quality of life specifically in children born with hypoplastic left heart syndrome, a condition requiring several surgeries before age three. The aim of this study was to use an empirically validated and standardised measure - the Pediatric Quality of Life Inventory - to evaluate quality of life in children with hypoplastic left heart syndrome and compare the findings with similar, medically complicated samples. METHODS: The parent-report Pediatric Quality of Life Inventory was administered, and demographic information was collected through an internet portal. A total of 121 caregivers of children with hypoplastic left heart syndrome responded. The sample included children aged 2-18 years (M=10.81 years). Independent sample t-tests were used to compare our sample with published norms of healthy children and children with acute or chronic illnesses. RESULTS: Children with hypoplastic left heart syndrome were rated as having significantly lower overall quality-of-life scores (M=59.69) compared with published norms of children without medical diagnoses (M=83.00) and those with acute (M=78.70) or chronic (M=77.19) illnesses (p<0.001). Children with hypoplastic left heart syndrome complicated by a stroke or seizure (15%) reported the lowest quality of life. The results held for all subscales (p<0.001). CONCLUSIONS: Children with hypoplastic left heart syndrome appear to be a significantly vulnerable population with difficulties in functioning across psychosocial domains and across the age span. Further research is required to facilitate early identification of the need for resources for these children and families, especially for children who experience additional medical complications. AD - 1Nationwide Children's Hospital Columbus,Ohio,United States of America. 2Miami,Florida,United States of America. AN - 28847316 AU - Dempster, N. AU - Cua, C. L. AU - Wernovsky, G. AU - Caris, E. AU - Neely, T. AU - Allen, R. AU - Butz, C. DA - Jan DB - PubMed DO - 10.1017/s1047951117001159 DP - NLM ET - 2017/08/30 IS - 1 KW - Adolescent Caregivers Case-Control Studies Child Child, Preschool Female Humans Hypoplastic Left Heart Syndrome/*physiopathology/surgery Male Psychiatric Status Rating Scales *Quality of Life Severity of Illness Index Surveys and Questionnaires United States Hypoplastic left heart syndrome adjustment quality of life LA - eng N1 - 1467-1107 Dempster, Nicole Cua, Clifford L Wernovsky, Gil Caris, Elizabeth Neely, Trent Allen, Robin Butz, Catherine Journal Article England Cardiol Young. 2018 Jan;28(1):21-26. doi: 10.1017/S1047951117001159. Epub 2017 Aug 29. PY - 2018 SN - 1047-9511 SP - 21-26 ST - Children with hypoplastic left heart syndrome have lower quality of life than healthy controls and children with other illnesses T2 - Cardiol Young TI - Children with hypoplastic left heart syndrome have lower quality of life than healthy controls and children with other illnesses VL - 28 ID - 47 ER - TY - JOUR AD - Section of Pediatric Critical Care Medicine, University of California-Davis, Ticon II, Room 228, 2516 Stockton Boulevard, Sacramento, CA 95817, USA. aakon@ucdavis.edu AN - 12804748 AU - Kon, A. A. AU - Ackerson, L. AU - Lo, B. DA - Jun 15 DB - PubMed DO - 10.1016/s0002-9149(03)00412-0 DP - NLM ET - 2003/06/14 IS - 12 KW - Attitude of Health Personnel Cardiac Surgical Procedures *Decision Making Female Health Knowledge, Attitudes, Practice Humans Hypoplastic Left Heart Syndrome/mortality/*therapy Male Multivariate Analysis Observer Variation Palliative Care *Parent-Child Relations Physician's Role/psychology Physicians/*psychology Predictive Value of Tests Retrospective Studies Severity of Illness Index Survival Analysis Treatment Outcome United States/epidemiology LA - eng N1 - Kon, Alexander A Ackerson, Lynn Lo, Bernard Journal Article Multicenter Study United States Am J Cardiol. 2003 Jun 15;91(12):1506-9, A9. doi: 10.1016/s0002-9149(03)00412-0. PY - 2003 SN - 0002-9149 (Print) 0002-9149 SP - 1506-9, a9 ST - Choices physicians would make if they were the parents of a child with hypoplastic left heart syndrome T2 - Am J Cardiol TI - Choices physicians would make if they were the parents of a child with hypoplastic left heart syndrome VL - 91 ID - 491 ER - TY - JOUR AB - Patients with hypoplastic left heart syndrome (HLHS) can have associated genetic abnormalities. This study evaluated the incidence of genetic abnormalities among infants with HLHS and the short-term outcomes of this population during the first hospitalization. This is a retrospective analysis of the multi-center Pediatric Heath Information System database of infants with HLHS who underwent Stage I Norwood, Hybrid, or heart transplant during their first hospitalization from 2004 through 2013. We compared clinical data between infants with and without genetic abnormality, among the three most common chromosomal abnormalities, and between survivors and non-survivors. Multivariable analysis was completed to evaluate predictors of mortality among patients with genetic abnormalities. A total of 5721 infants with HLHS were identified; 282 (5%) had associated genetic abnormalities. The three most common chromosomal abnormalities were Turner (25%), DiGeorge (22%), and Downs (12.7%) syndromes. Over the study period, the number of patients with genetic abnormalities undergoing cardiac operations increased without any significant increases in mortality. Infants with genetic abnormalities compared to those without abnormalities had longer hospital length of stay and higher morbidity and mortality. Variables associated with mortality were lower gestational age, longer duration of vasopressor therapy, need for dialysis, and cardiopulmonary resuscitation; and complicated clinical course as suggested by necrotizing enterocolitis, septicemia. Presence of any genetic abnormality in infants with HLHS undergoing cardiac surgery is associated with increased mortality and morbidity. Timely genetic testing, appropriate family counseling, and thorough preoperative case selection are suggested for these patients for any operative intervention. AD - D. Zakaria, Pediatric Cardiology, Arkansas Children’s Hospital, University of Arkansas for Medical Sciences, Little Rock, AR, United States AU - Zakaria, D. AU - Tang, X. AU - Bhakta, R. AU - ElHassan, N. O. AU - Prodhan, P. DB - Embase Medline DO - 10.1007/s00246-017-1717-3 IS - 1 KW - article chromosome aberration cohort analysis dialysis DiGeorge syndrome disease association disease course Down syndrome family counseling female genetic screening gestational age heart graft heart surgery human hypoplastic left heart syndrome infant infant mortality length of stay major clinical study male morbidity necrotizing enterocolitis Norwood procedure resuscitation retrospective study septicemia short term survival survival prediction survivor treatment duration treatment outcome Turner syndrome LA - English M3 - Article N1 - L618363885 2017-09-26 2018-08-22 PY - 2018 SN - 1432-1971 0172-0643 SP - 11-18 ST - Chromosomal Abnormalities Affect the Surgical Outcome in Infants with Hypoplastic Left Heart Syndrome: A Large Cohort Analysis T2 - Pediatric Cardiology TI - Chromosomal Abnormalities Affect the Surgical Outcome in Infants with Hypoplastic Left Heart Syndrome: A Large Cohort Analysis UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L618363885 http://dx.doi.org/10.1007/s00246-017-1717-3 VL - 39 ID - 712 ER - TY - JOUR AB - We describe a case of a baby girl born with hypoplastic left heart syndrome consisting of mitral atresia, aortic atresia, hypoplastic ascending aorta, and left ventricle. The pulmonary arteries were hypoplastic, measuring 3 mm. Fluorescence in situ hybridisation analysis demonstrated a microduplication of chromosome 22q11.2. Subsequent array comparative genomic hybridisation showed a gain of 2.3 Mb in one copy of chromosome 22q at band 22q11.21. The proband underwent a successful Norwood procedure with Sano shunt and subsequently underwent bi-directional Glenn shunt and Fontan procedure. This report highlights the association between hypoplastic left heart syndrome with hypoplastic pulmonary arteries and chromosome 22q11.21 microduplication. AD - C.J. McMahon, Department of Paediatric Cardiology, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland AU - McMahon, C. J. AU - Morgan, C. T. AU - Greally, M. T. DB - Embase DO - 10.1017/S104795111300231X IS - 1 KW - anamnesis aorta anomaly aortic atresia artery diameter article ascending aorta ascending aorta hypoplasia case report cesarean section chromosome 22q chromosome duplication chromosome microduplication 22q21 comparative genomic hybridization cyanosis disease association ECG abnormality face dysmorphia female fetus echography fluorescence in situ hybridization Fontan procedure gestational age Glenn shunt heart atrium septum defect heart catheterization heart left right shunt heart left ventricle heart murmur heart right ventricle hypertrophy human human tissue hypoplastic left heart syndrome hypoplastic pulmonary artery infant mitral valve atresia Norwood procedure oxygen saturation pulmonary artery malformation right ventricle to pulmonary artery conduit speech delay surgical approach surgical technique LA - English M3 - Article N1 - L601104085 2015-01-12 2015-01-13 PY - 2014 SN - 1467-1107 1047-9511 SP - 167-170 ST - Chromosome 22q11.21 microduplication in association with hypoplastic left heart syndrome with hypoplastic pulmonary arteries T2 - Cardiology in the Young TI - Chromosome 22q11.21 microduplication in association with hypoplastic left heart syndrome with hypoplastic pulmonary arteries UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L601104085 http://dx.doi.org/10.1017/S104795111300231X VL - 25 ID - 970 ER - TY - JOUR AB - BACKGROUND AND OBJECTIVES: Little is known about chronic neuromotor disability (CND) including cerebral palsy and motor impairments after acquired brain injury in children surviving early complex cardiac surgery (CCS). We sought to determine the frequency and presentation of CND in this population while exploring potentially modifiable acute care predictors. METHODS: This prospective follow-up study included 549 children after CCS requiring cardiopulmonary bypass at ≤6 weeks of age. Groups included those with only 1 CCS, mostly biventricular CHD, and those with .1 CCS, predominantly single ventricle defects. At 4.5 years of age, 420 (94.6%) children received multidisciplinary assessment. Frequency of CND is given as percentage of assessed survivors. Predictors of CND were analyzed using multiple logistic regression analysis. RESULTS: CND occurred in 6% (95% confidence interval [CI] 3.7%-8.2%) of 4.5-year survivors; for 1 CCS, 4.2% (CI 2.3%-6.1%) and >1, 9.8% (CI 7%-12.6%). CND presentation showed: hemiparesis, 72%; spasticity, 80%; ambulation, 72%; intellectual disability, 44%; autism, 16%; epilepsy, 12%; permanent vision and hearing impairment, 12% and 8%, respectively. Overall, 32% of presumed causative events happened before first CCS. Independent odds ratio for CND are age (days) at first CCS, 1.08 (CI 1.04-1.12; P < .001); highest plasma lactate before first CCS (mmol/L), 1.13 (CI 1.03-1.23; P = 0.008); and >1 CCS, 3.57 (CI 1.48-8.9; P = .005). CONCLUSIONS: CND is not uncommon among CCS survivors. The frequency of associated disabilities characterized in this study informs pediatricians caring for this vulnerable population. Shortening the waiting period and reducing preoperative plasma lactate levels at first CCS may assist in reducing the frequency of CND. AD - C.M.T. Robertson, Pediatric Rehabilitation, Glenrose Rehabilitation Hospital, 10230 111 Ave, T5G 0B7, Edmonton, AB, Canada AU - Ricci, M. F. AU - Andersen, J. C. AU - Joffe, A. R. AU - Watt, M. J. AU - Moez, E. K. AU - Dinu, I. A. AU - Guerra, G. G. AU - Ross, D. B. AU - Rebeyka, I. M. AU - Robertson, C. M. T. DB - Embase Medline DO - 10.1542/peds.2015-1879 IS - 4 KW - lactic acid acquired brain injury article autism cardiopulmonary bypass child chronic neuromotor disability cohort analysis complex cardiac surgery congenital heart disease epilepsy female follow up hearing impairment heart surgery hemiparesis human intellectual impairment lactate blood level major clinical study male motor dysfunction postoperative period preschool child priority journal prospective study spasticity survival rate visual impairment walking difficulty LA - English M3 - Article N1 - L606267880 2015-10-09 2015-10-14 PY - 2015 SN - 1098-4275 0031-4005 SP - e922-e933 ST - Chronic neuromotor disability after complex cardiac surgery in early life T2 - Pediatrics TI - Chronic neuromotor disability after complex cardiac surgery in early life UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L606267880 http://dx.doi.org/10.1542/peds.2015-1879 VL - 136 ID - 895 ER - TY - JOUR AB - Aim: To describe the incidence, patient profile, management strategies and outcome for infants and children who developed a chylothorax in the UK. Methods: A prospective study of infants and children ≥24 weeks' gestation - ≤16 years, who developed a chylothorax in the UK and were reported through the British Paediatric Surveillance Unit (BPSU). Clinicians completed a questionnaire on the presentation, diagnosis, management and outcome of these children. Three further data sources were accessed to confirm these data. Results: The incidence in children in the UK was 0.0014% (1.4 per 100 000) and 3.2% (3200 per 100 000) for those developing a chylothorax following a cardiac surgical procedure. The incidence was highest in infants ≤12 months at 16 per 100 000 (0.016%). A total of 219 questionnaires were returned with 172 cases meeting the eligibility criteria. Development of a chylothorax was most commonly associated with cardiac surgical procedure (65.1%) and was most frequently confirmed by laboratory verification of triglyceride content of the pleural fluid ≥1.1 mmol/L (66%). Although a variety of management strategies were employed, treatment with an intercostal pleural catheter (86.5%) and a medium chain triglyceride (MCT) diet (89%) was most commonly reported. The majority of the children had a prolonged hospital stay with a reported mortality of 12.2%. Conclusions: Development of a chylothorax in infants and children in the UK was not common. The primary association was with a cardiac surgical procedure. The child's hospital stay was lengthy and therefore the impact on the child, family and hospital resources were significant. Common management strategies existed but national guidance is required to optimise practice. This study allows for better information relating to this serious complication to be given to patients and families and provides the basis for future research and practice development. AD - C. Haines, Paediatric Intensive Care Unit, Bristol Royal Hospital for Children, Upper Maudlin Street, Bristol, BS2 8BJ, United Kingdom AU - Haines, C. AU - Walsh, B. AU - Fletcher, M. AU - Davis, P. J. DB - Embase Medline DO - 10.1136/archdischild-2013-304364 IS - 8 KW - medium chain triacylglycerol octreotide somatostatin triacylglycerol adolescent article child chylothorax Down syndrome Fallot tetralogy female Fontan procedure heart surgery human infant length of stay major clinical study male morbidity mortality newborn Noonan syndrome parenteral nutrition pleurodesis postoperative complication priority journal prospective study steroid therapy thoracocentesis Turner syndrome United Kingdom LA - English M3 - Article N1 - L53089285 2014-04-11 2014-08-18 PY - 2014 SN - 1468-2044 0003-9888 SP - 724-730 ST - Chylothorax development in infants and children in the UK T2 - Archives of Disease in Childhood TI - Chylothorax development in infants and children in the UK UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L53089285 http://dx.doi.org/10.1136/archdischild-2013-304364 VL - 99 ID - 956 ER - TY - JOUR AB - Traditionally, exercise echo has been used to evaluate pediatric heart disease, specifically, obtaining information regarding pressures, gradients, and function during increased cardiac demands. However, its use is limited because of poor acoustic windows and patient age. Pharmacologic stress echo is an attractive alternative in evaluating heart disease other than coronary disease but has not been explored in children. Methods: We performed pharmacologic stress echo in evaluating non-coronary diseases in 9 patients (3 to 19 y.o.). Diagnoses included aortic stenosis in 3 patients; and coarctation, hypertrophic cardiomyopathy, pulmonary stenosis, "healed" myocarditis, dilated cardiomyopathy and single ventricle in 1 each. Test indications were to assess stress gradient in obstructive lesions and stress function in the other diagnoses. Dobutamine (in 8) or isoproterenol (in 1) was given in increasing doses until one or more of the following endpoints: 1) symptoms (sx), 2) severe gradient, 3) decreasing function, and/or 4) maximum dose. Results: In all patients, tests were safe, and echo information was easily obtained. Gradients increased to significant levels in 5 of the 6 patients with obstruction. The remaining studies were normal. In 4 patients, stress results altered patient care: medical treatment in 3 patients and surgery in 1. Conclusions: Pharmacologic stress echo overcomes the limitations of exercise echo, and is safe and feasible in assessing pediatric heart disease other than coronary artery disease. Hemodynamics during stress may be more clinically relevant than resting echo data because they reflect physiology more closely resembling that which occurs during a child's typically active state. This may improve timing of medical and surgical intervention. AD - Noninvasive Cardiac Imaging and Hemodynamic Research Laboratory, Division of Cardiology, Children's Hospital Medical Center, Cincinnati, OH, United States AU - Kimball, T. R. AU - Witt, S. A. DB - Scopus IS - 4 M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 1997 SP - 398 ST - The clinical application of pediatric pharmacologic stress echocardiography in evaluating non-ischemic heart disease T2 - Journal of the American Society of Echocardiography TI - The clinical application of pediatric pharmacologic stress echocardiography in evaluating non-ischemic heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0008543150&partnerID=40&md5=afc1f16510b1c668b0d9c4296d396473 VL - 10 ID - 2293 ER - TY - JOUR AB - A 5-year-old girl with right atrial isomerism, complete atrioventricular septal defect, hypoplastic left ventricle, double outlet right ventricle, and mixed-type total anomalous pulmonary venous connection with totally occluded left pulmonary veins presented at our center for fenestrated total cavo-pulmonary connection with an extra cardiac conduit at the age of 3 years. Eleven months after the Fontan completion, she developed protein-losing enteropathy (PLE). Spontaneously closed fenestration was thought to be the cause of the PLE, and she underwent revision of fenestration at the age of 5 years. After the operation, PLE did not improve, and newly developed hypoxemia impaired her systemic ventricular function, leading to the initiation of veno-arterial extracorporeal membrane oxygenation (ECMO) with the Endumo® system 18 days after the operation to treat her hemodynamic instability. Although the ECMO circuit was changed three times during the first 8 days, the fourth circuit could be used for 74 days without hemolysis and serum leakage, until the patient unfortunately died 82 days after the operation due to multi-organ failure. © 2013 The Japanese Society for Artificial Organs. AD - Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka 565-8565, Japan Department of Clinical Engineering, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka 565-8565, Japan AU - Kusajima, K. AU - Hoashi, T. AU - Kagisaki, K. AU - Yoshida, K. AU - Nishigaki, T. AU - Hayashi, T. AU - Ichikawa, H. DB - Scopus DO - 10.1007/s10047-013-0747-8 IS - 1 KW - Endumo Extracorporeal membrane oxygenation Fontan Pediatric M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2014 SP - 99-102 ST - Clinical experience of more than 2 months usage of extracorporeal membrane oxygenation (Endumo® 4000) without circuit exchange T2 - Journal of Artificial Organs TI - Clinical experience of more than 2 months usage of extracorporeal membrane oxygenation (Endumo® 4000) without circuit exchange UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84897113780&doi=10.1007%2fs10047-013-0747-8&partnerID=40&md5=ac4ebcd030d498f44c1ab7f71c5643d2 VL - 17 ID - 2014 ER - TY - JOUR AB - Nine patients with a single ventricle and the great vessel arrangement of congenitally corrected transposition are reviewed to emphasize the different clinical pictures which may be seen. Physical findings are not characteristic. Cyanosis is not always clinically detectable. Variable systolic murmurs are always present, but the most striking auscultatory finding, not stressed previously, is a decrescendo diastolic murmur of pulmonary insufficiency. Electrocardiographic features characteristic of congenitally corrected transposition of the great vessels include some type of arrhythmia, abnormal P waves, a Qr or QS pattern in lead aVR, and reversal of the normal precordial QRS pattern with a Q wave in leads V3R or V1 and no Q wave in V6 or V7. These findings, however, do not allow a distinction between cases with a single ventricle and those with other associated cardiac defects. Characteristic roentgenologic features of congenitally corrected transposition include a narrow cardiac base, a straight or slightly convex left upper cardiac border with absence of a definite pulmonary artery segment, an unusually cephalad position of the right pulmonary hilar shadows, a "waterfall" configuration of the dilated and tortuous right pulmonary artery, a huge main pulmonary artery causing an indentation on the left side of the barium filled esophagus, and a prominent shadow in the right superior mediastinum representing the displaced superior vena cava. These findings, although present in the majority of patients in this series, are not different from those found in cases of congenitally corrected transposition and two ventricles and a large ventricular septal defect. Cardiac catheterization and angiocardiography confirm the great vessel arrangement of corrected transposition and a bidirectional shunt in a ventricle whose systolic pressure is at systemic levels. Selective ventricular or subaortic angiocardiography is highly desirable to distinguish patients with a single ventricle in which a subaortic infundibular chamber is present from those with congenitally corrected transposition and two ventricles with a large ventricular septal defect. Although complete surgical correction of a single ventricle is not yet possible, pulmonary artery banding in patients with a large pulmonary blood flow may be a feasible palliative procedure. © 1966. AD - Departments of Pediatrics, Medicine, Surgery and Pathology, College of Medicine, University of Florida, Gainesville, FL, United States AU - Morgan, A. D. AU - Krovetz, L. J. AU - Bartley, T. D. AU - Green Jr, J. R. AU - Shanklin, D. R. AU - Wheat Jr, M. W. AU - Schiebler, G. L. DB - Scopus DO - 10.1016/0002-9149(66)90220-7 IS - 3 M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 1966 SP - 379-388 ST - Clinical features of single ventricle with congenitally corrected transposition T2 - The American Journal of Cardiology TI - Clinical features of single ventricle with congenitally corrected transposition UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0013886369&doi=10.1016%2f0002-9149%2866%2990220-7&partnerID=40&md5=85b5291fefe69838ceccbc8464fc3d5d VL - 17 ID - 2338 ER - TY - JOUR AB - From July 1, 1989 to June 30, 1996, a total of six patients, four male and two female, with double aortic arch (DAA) were registered. They aged 16 days to 6.5 years. There were two patients with isolated DAA, and four complicated with intracardiac anomalies. All six patients presented respiratory distress and feeding problem early in life. However, cyanosis, owing to decreased pulmonary blood flow, predominated the clinical picture in each patient having DAA intricated with tetralogy of Fallot (TOF) and pulmonary stenosis, TOF with absent pulmonary valve syndrome, and type II-A tricuspid atresia. DiGeorge syndrome was documented in one patient with TOF and pulmonary stenosis. Expiratory wheezing was remarkable in the patient with TOF and absent pulmonary valve syndrome. Mild cyanosis and heart failure was noted in one patient with ventricular septal defect (VSD), and bicuspid pulmonary valve with mild valvular stenosis. Barium esophagogram showed indentations on esophagus in five patients. Echocardiography was yielding in three infants. Four patients, two isolated and two complex DAA, survived the surgical division of the smaller left aortic arch. One-stage and two-stage operation was performed in patients having DAA intricated with VSD and TOF, respectively. The other two patients with complex DAA remained in close observation owing to the tolerance of symptoms related to intracardiac anomalies. AD - J.-K. Wang, Department of Pediatrics, Division of Pediatric Cardiology, Department of Surgery, Chung-Shan South Road, Taipei 10002, Taiwan AU - Lee, M. L. AU - Wang, J. K. AU - Wu, M. H. AU - Lue, H. C. AU - Ing-Sh, Chiu AU - Chang, C. I. DB - Embase Medline DO - 10.1016/S0167-5273(97)00325-2 IS - 3 KW - aortic arch anomaly article child clinical article clinical feature congenital heart malformation cyanosis DiGeorge syndrome echocardiography esophagography Fallot tetralogy feeding disorder female heart failure heart ventricle septum defect human infant male newborn priority journal pulmonary valve stenosis respiratory distress tricuspid valve atresia wheezing LA - English M3 - Article N1 - L28149996 1998-04-21 PY - 1998 SN - 0167-5273 SP - 205-210 ST - Clinical implications of isolated double aortic arch and its complex with intracardiac anomalies T2 - International Journal of Cardiology TI - Clinical implications of isolated double aortic arch and its complex with intracardiac anomalies UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L28149996 http://dx.doi.org/10.1016/S0167-5273(97)00325-2 VL - 63 ID - 1353 ER - TY - JOUR AB - Background The main objective of this retrospective analysis in a large tertiary center was the clinical outcome of prenatally diagnosed cardiac rhabdomyomas as well as the identification of factors influencing fetal prognosis. Methods A total of 45 cases of fetuses with prenatally suspected rhabdomyoma and their clinical outcome were analyzed retrospectively. A review of the literature was also performed. Results In five cases, after a tuberous sclerosis complex (TSC) mutation had been confirmed, termination of pregnancy was chosen. In 30 cases postnatal data were available. In 93% TSC was confirmed clinically or by mutational analysis. Two thirds of fetuses presented with multiple tumor while one third presented with a solitary tumor. In two fetuses mild pericardial effusions were observed. Another three fetuses presented with extrasystoles prenatally. No hydrops fetalis or fetal perinatal demise were observed. After birth 41% of the children suffered from arrhythmia including supra- and ventricular tachycardia, Wolff-Parkinson-White syndrome and atrioventricular block. One child received a Fontan procedure with Glenn anastomosis. Another child with a dilatative cardiomyopathy and a left ventricular ejection fraction of 15% died. Fifty-two percent of the children with TSC suffered from epilepsy ranging from absence epilepsy and West syndrome to generalized seizures with a frequency of up to 40 per day. Two children underwent neurosurgery to remove the epileptogenic focus. One child suffered from TSC and Lesch-Nyhan disease. In another case Beckwith-Wiedemann syndrome was identified as the causative disorder. Conclusion Rhabdomyoma are rare, benign tumors. There is an association with TSC. In the majority of cases rhabdomyoma are not hemodynamically relevant and do not increase in size. The quality of life of affected patients is impaired particularly due to epilepsy and psychomotor retardation. AD - Department of Obstetrics, Charité - Universitätsmedizin Berlin, Berlin, Germany. Practice for Prenatal Diagnosis, Berlin, Germany. AN - 31811808 AU - Altmann, J. AU - Kiver, V. AU - Henrich, W. AU - Weichert, A. DA - Dec 18 DB - PubMed DO - 10.1515/jpm-2019-0246 DP - NLM ET - 2019/12/08 IS - 1 KW - Beckwith-Wiedemann syndrome Lesch-Nyhan disease cardiac rhabdomyoma cranial tuber tuberous sclerosis complex LA - eng N1 - 1619-3997 Altmann, Judith Orcid: 0000-0003-2065-5058 Kiver, Verena Orcid: 0000-0002-1356-9576 Henrich, Wolfgang Weichert, Alexander Journal Article Germany J Perinat Med. 2019 Dec 18;48(1):74-81. doi: 10.1515/jpm-2019-0246. PY - 2019 SN - 0300-5577 SP - 74-81 ST - Clinical outcome of prenatally suspected cardiac rhabdomyomas of the fetus T2 - J Perinat Med TI - Clinical outcome of prenatally suspected cardiac rhabdomyomas of the fetus VL - 48 ID - 6 ER - TY - JOUR AB - Objective: To characterize mortality and morbidity outcomes in patients undergoing surgical Fontan conversion. Background: Indications for and anticipated clinical outcomes associated with Fontan conversion are controversial. Methods: A retrospective single-center cohort study of consecutive patients undergoing Fontan conversion between 1990 and 2006 stratified according to concomitant arrhythmia surgery. Results: Forty patients underwent Fontan conversion at a median age of 19.0 years and were followed for 4.2 years. Six (15%) died, two perioperatively, three early postoperatively, and one following heart transplant. Older age was a univariate risk factor. Major perioperative complications occurred in 9 of 35 (26%) early survivors. Patients with concomitant arrhythmia surgery (N = 21) were older at conversion and had longer interoperative intervals. They experienced a reduction in prevalence (95% vs. 28%, P < 0.0001) and severity (severity score 7.3 vs. 3.3, P = 0.001) of atrial tachyarrhythmias, in contrast to patients without arrhythmia surgery (47% vs. 53%; 3.3 vs. 3.9, P = NS). NYHA functional class improved in both groups. In a subgroup (N = 14) with non-urgent late postoperative catheterization, filling pressures were unchanged from preoperative values. Conclusions: Mortality and morbidity after Fontan conversion are substantial, but survivors experience a subjective improvement in functional status. Concomitant arrhythmia surgery reduces the arrhythmia burden without a detectable increase in complication rates. © 2008 Elsevier Ireland Ltd. All rights reserved. AD - J.K. Triedman, Department of Cardiology, Children's Hospital Boston, Boston, MA, United States AU - Takahashi, K. AU - Fynn-Thompson, F. AU - Cecchin, F. AU - Khairy, P. AU - del Nido, P. AU - Triedman, J. K. DB - Embase Medline DO - 10.1016/j.ijcard.2008.06.074 IS - 3 KW - antiarrhythmic agent adolescent adult article brain hemorrhage cerebrovascular accident clinical article cohort analysis disease severity follow up Fontan procedure functional assessment gastrointestinal hemorrhage heart atrium arrhythmia heart atrium thrombosis heart catheterization heart surgery heart transplantation hematothorax human intracardiac thrombosis kidney dysfunction morbidity mortality multiple organ failure outcome assessment peroperative complication perioperative period peritoneal dialysis pleura effusion postoperative care postoperative hemorrhage postoperative period prevalence priority journal retrospective study risk factor risk reduction scoring system seizure supraventricular tachycardia survivor treatment outcome LA - English M3 - Article N1 - L50240271 2010-01-05 PY - 2009 SN - 0167-5273 SP - 260-266 ST - Clinical outcomes of Fontan conversion surgery with and without associated arrhythmia intervention T2 - International Journal of Cardiology TI - Clinical outcomes of Fontan conversion surgery with and without associated arrhythmia intervention UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50240271 http://dx.doi.org/10.1016/j.ijcard.2008.06.074 VL - 137 ID - 1171 ER - TY - JOUR AB - We report here the case of a hospitalized 11 year-old boy (YP) with a positive diagnosis of ‘Childhood Onset Schizophrenia’. YP experienced verbal-auditory hallucinations, a delusion of alien control and hetero-aggressive behavior. Antipsychotic drugs were unsuccessful and further more provoked severe acute dystonia. fMRI-guided rTMS applied over several cortical regions provided the means to reveal for the first time a functional dissociation between auditory-verbal hallucinations and agency. These results demonstrate the efficacy of rTMS for young patients suffering from drug-resistant hallucinations but they further more question the physiopathology of the hallucinatory process by suggesting that agency and hallucinations may be sub-served by different neural networks. (PsycINFO Database Record (c) 2018 APA, all rights reserved) AD - Jardri, Renaud, Universite Lille 2, Laboratoire de Neurosciences Fonctionnelles et Pathologies, CNRS UMR 8160 Explorations Fonctionnelles de la Vision, Hopital Roger Salengro, Centre Hospitalier Universitaire, 59037, Lille, France, Cedex AN - 2008-18443-015 AU - Jardri, Renaud AU - Delevoye-Turrell, Yvonne AU - Lucas, Bernard AU - Pins, Delphine AU - Bulot, Virginie AU - Delmaire, Christine AU - Thomas, Pierre AU - Delion, Pierre AU - Goeb, Jean-Louis DB - psyh DO - 10.1016/j.neuropsychologia.2008.08.006 DP - EBSCOhost IS - 1 KW - auditory hallucinations childhood onset schizophrenia verbal hallucinations aggressive behavior alienation antipsychotic drugs delusions transcranial magnetic stimulation self awareness agency motor control fMRI-guided rTMS Brain Mapping Child Dissociative Disorders Electric Stimulation Hallucinations Humans Image Processing, Computer-Assisted Magnetic Resonance Imaging Male Neuropsychological Tests Oxygen Schizophrenia, Childhood Schizophrenic Psychology Childhood Schizophrenia Neuroleptic Drugs Visual Hallucinations N1 - Universite Lille Nord de France, CNRS UMR-8160, CHRU de Lille, Lille, France. Release Date: 20090511. Correction Date: 20180607. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Childhood Schizophrenia; Delusions; Hallucinations; Neuroleptic Drugs; Transcranial Magnetic Stimulation. Minor Descriptor: Aggressive Behavior; Auditory Hallucinations; Visual Hallucinations; Agency. Classification: Health & Mental Health Treatment & Prevention (3300). Population: Human (10); Male (30); Inpatient (50). Location: US. Age Group: Childhood (birth-12 yrs) (100); School Age (6-12 yrs) (180). Tests & Measures: Auditory Hallucination Rating Scale; Bush-Francis Catatonia Rating Scale; Schedule for Affective Disorders and Schizophrenia for School-Age Children; Autism Diagnostic Interview-Revised DOI: 10.1037/t18128-000. Methodology: Clinical Case Study. References Available: Y. Page Count: 7. Issue Publication Date: Jan, 2009. PY - 2009 SN - 0028-3932 1873-3514 SP - 132-138 ST - Clinical practice of rTMS reveals a functional dissociation between agency and hallucinations in schizophrenia T2 - Neuropsychologia TI - Clinical practice of rTMS reveals a functional dissociation between agency and hallucinations in schizophrenia UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2008-18443-015&site=ehost-live&scope=site ORCID: 0000-0001-9158-8425 ORCID: 0000-0003-4596-1502 r-jardri@chru-lille.fr VL - 47 ID - 1696 ER - TY - JOUR AB - Increasingly, more patients with univentricular heart reach adulthood. Therefore, long-term psychological features are an important concern. The aim of this study was to evaluate the clinical and psychological profile of post-Fontan adult patients and to identify the most significant determinants of quality of life. In this retrospective cross-sectional study, we reviewed the surgical and medical history of post-Fontan adult patients. Patients underwent a 24-h electrocardiogram, echocardiography and exercise testing. Self-report questionnaires were used to assess the Work Ability Index, quality of life (Satisfaction with Life Scale), perceived health status (SF-36 questionnaire), coping strategies (Brief Cope questionnaire) and presence of mood disorders (Hospital Anxiety and Depression Scale). Thirty-nine patients aged between 18 and 48 years (mean 27.5 years) were enrolled. The mean follow-up was 21.5 years. Most patients were unmarried (82.9 %), had a high school diploma (62.9 %) and were employed (62.9 %). Twenty-nine patients (82.3 %) had at least one long-term complication. The median single ventricle ejection fraction was 57 %, and the median maximal oxygen consumption was 26.8 ml/min/kg. This population tended to be anxious and to use adaptive coping strategies. Quality of life was perceived as excellent or good in 57.2 % of cases and was not related to either cardiac function or exercise capacity. Both quality of life and SF-36 domains were related to the Work Ability Index. This cohort of post-Fontan adult patients enjoyed a good quality of life irrespective of disease severity. AD - Pediatric Cardiology Unit, Department of Women's and Children's Health, University of Padua, Via Giustiniani 3, 35128, Padua, Italy, giulia.bordin@gmail.com. AN - 25828147 AU - Bordin, G. AU - Padalino, M. A. AU - Perentaler, S. AU - Castaldi, B. AU - Maschietto, N. AU - Michieli, P. AU - Crepaz, R. AU - Frigo, A. C. AU - Vida, V. L. AU - Milanesi, O. DA - Aug DB - PubMed DO - 10.1007/s00246-015-1156-y DP - NLM ET - 2015/04/02 IS - 6 KW - *Adaptation, Psychological Adolescent Adult Anxiety/*psychology Cross-Sectional Studies Depression/psychology Echocardiography Electrocardiography Exercise Test/methods Female Fontan Procedure/adverse effects/*psychology Health Status Humans Male Middle Aged Postoperative Complications/epidemiology/*psychology Quality of Life/*psychology Retrospective Studies Stroke Volume/physiology Surveys and Questionnaires Young Adult LA - eng N1 - 1432-1971 Bordin, Giulia Padalino, Massimo Antonio Perentaler, Sonja Castaldi, Biagio Maschietto, Nicola Michieli, Pierantonio Crepaz, Roberto Frigo, Anna Chiara Vida, Vladimiro Lorenzo Milanesi, Ornella Journal Article United States Pediatr Cardiol. 2015 Aug;36(6):1261-9. doi: 10.1007/s00246-015-1156-y. Epub 2015 Apr 2. PY - 2015 SN - 0172-0643 SP - 1261-9 ST - Clinical Profile and Quality of Life of Adult Patients After the Fontan Procedure T2 - Pediatr Cardiol TI - Clinical Profile and Quality of Life of Adult Patients After the Fontan Procedure VL - 36 ID - 91 ER - TY - JOUR AB - Background Cyanotic congenital heart disease (CCHD) accounts for 25% of congenital heart defects and has a high morbidity and mortality in neonates. Objective The aim of this work was to study clinical profile and available therapeutic modalities used in the management of CCHD. Subjects and methods This was a prospective study carried out for a period of one year, starting from January 2011, and included 50 neonates admitted to neonatal intensive care unit (NICU) of Sohag University Hospital, Sohag, Upper Egypt. All included patients were subjected to thorough clinical history, full clinical examination, initial and frequent measurement of oxygen saturation by pulse oximetry, blood gas analysis, and echocardiography. Also, therapeutic modalities used in management of CCHD were noted. Results 50 neonates were diagnosed as having CCHD, out of them 39 (78%) were males with male to female ratio 3.55:1. The mean age of presentation was 11.78 ± 9.4 days. CCHD frequency was found to be 9.5% (50/524) in our NICU population. The most common type of CCHD was d-transposition of great arteries (D-TGA) (66%) followed by complex CCHD (12%) and hypoplastic left heart syndrome (HLHS) (12%), whereas the less common type was hypoplastic right ventricle (2%). All cases presented with central cyanosis and needed medical treatment whereas balloon atrial septostomy was performed in 26% of cases. Seventy-four percent of cases were improved and referred to higher centers while 26% were expired during hospital stay. Conclusion CCHD is a leading cause of neonatal morbidity and mortality. CCHD frequency was significant (9.5%) in our study population with D-TGA being the commonest type. Majority of neonates with CCHD showed survival with suitable management. Early diagnosis and referral to pediatric cardiac center for proper management will improve the outcome. Neonatologists and pediatric cardiologists should be familiar with diagnosis and management of CCHD. © 2016 Ain Shams University AD - Pediatric Department, Sohag University, Sohag, Egypt AU - Abou-Taleb, A. AU - Abdelhamid, M. A. AU - Bahkeet, M. A. M. DB - Scopus DO - 10.1016/j.ejmhg.2016.01.003 IS - 1 KW - Cyanotic congenital heart disease Echocardiography Transposition of great arteries M3 - Article N1 - Export Date: 15 June 2020 PY - 2017 SP - 47-51 ST - Clinical profile of cyanotic congenital heart disease in neonatal intensive care unit at Sohag University Hospital, Upper Egypt T2 - Egyptian Journal of Medical Human Genetics TI - Clinical profile of cyanotic congenital heart disease in neonatal intensive care unit at Sohag University Hospital, Upper Egypt UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84960154799&doi=10.1016%2fj.ejmhg.2016.01.003&partnerID=40&md5=5953732264e2c4429db8dcd5ca7e53dd VL - 18 ID - 1878 ER - TY - JOUR AB - OBJECTIVES: The study aims to describe the clinical profile of the adult Fontan survivor and identify the worries, symptoms, and the impact of cardiac surveillance most commonly experienced. DESIGN: A descriptive, cross-sectional design was used. SETTING: The study was performed in outpatient adult and pediatric cardiology clinics in university-affiliated and private practice offices. PATIENTS: Fifty-four adolescent and adult patients with single ventricle congenital heart disease who have undergone the Fontan procedure participated in the study. The mean age was 26 ± 9 years with 52% female and 63% Caucasian. OUTCOME MEASURES: Demographic and clinical data were obtained by a standard intake form and retrospective chart reviews. The Congenital Heart Disease TNO/AZL Adult Quality Of Life questionnaire was completed to assess worries, symptoms, and the impact of cardiac surveillance. RESULTS: The majority were single (73%), employed or full-time students (93%), with health insurance (94%), had a single left ventricle (78%), the diagnosis of tricuspid atresia or double inlet left ventricle (59%), lateral tunnel Fontan type (44%), history of arrhythmias (76%), left ventricle ejection fraction percentage >50 (66%), oxygen saturations >90% (70%), frequent headaches (50%), scoliosis (22%), varicose veins, ascites, and liver cirrhosis (46%), normal body mass index (59%), and New York Heart Association class I (48%) and II-III (52%). Primary worries related to current health (83%), job/employment (69%), ability to work, (61%) and living independently (54%). The most bothersome symptoms were shortness of breath with ambulation (69%), dizziness (61%), and palpitations (61%). CONCLUSIONS: Fontan survivors experience residua and sequelae from multiple surgical procedures and the natural disease course. Our results support the need for ongoing assessment of both physical symptoms and psychosocial concerns, and suggest the need for multiple risk factor intervention strategies that improve physical and emotional health in Fontan survivors. AD - School of Nursing, University of California, CA, USA. npike@sonnet.ucla.edu AN - 21269408 AU - Pike, N. A. AU - Evangelista, L. S. AU - Doering, L. V. AU - Koniak-Griffin, D. AU - Lewis, A. B. AU - Child, J. S. C2 - PMC3177559 C6 - NIHMS322636 DA - Jan-Feb DB - PubMed DO - 10.1111/j.1747-0803.2010.00475.x DP - NLM ET - 2011/01/29 IS - 1 KW - Adolescent Adult Cross-Sectional Studies Emotions Female *Fontan Procedure/adverse effects Heart Defects, Congenital/complications/physiopathology/psychology/*surgery Humans Los Angeles Male Mental Health Middle Aged Quality of Life Retrospective Studies Risk Assessment Risk Factors Surveys and Questionnaires *Survivors/psychology Time Factors Treatment Outcome Young Adult LA - eng N1 - 1747-0803 Pike, Nancy A Evangelista, Lorraine S Doering, Lynn V Koniak-Griffin, Deborah Lewis, Alan B Child, John S P30 AG021684/AG/NIA NIH HHS/United States P30 AG021684-10/AG/NIA NIH HHS/United States R01 HL093466/HL/NHLBI NIH HHS/United States R01 HL093466-02/HL/NHLBI NIH HHS/United States Journal Article Congenit Heart Dis. 2011 Jan-Feb;6(1):9-17. doi: 10.1111/j.1747-0803.2010.00475.x. PY - 2011 SN - 1747-079X (Print) 1747-079x SP - 9-17 ST - Clinical profile of the adolescent/adult Fontan survivor T2 - Congenit Heart Dis TI - Clinical profile of the adolescent/adult Fontan survivor VL - 6 ID - 99 ER - TY - JOUR AB - BACKGROUND: This study was performed to clarify the present global clinical status, including medication(s) and social abilities, of adult patients with single ventricular physiology (SVP). METHODS AND RESULTS: Clinical charts were retrospectively reviewed to evaluate the global clinical status, including functional capacity, medication(s), complications, and social activities of 68 adult patients with SVP aged 20-53 years (41 males); 50 had undergone the Fontan operation and they were divided into the young adult (25 patients) and adult Fontan groups. The others were cyanotic patients without the Fontan operation (non-Fontan group). Although the Fontan groups showed better functional capacity, higher arterial oxygen saturation and brain natriuretic peptide levels, and a better renal function, there was no difference in the cardiovascular events during follow-up between the 3 groups. The most frequent complication was arrhythmia without a significant group difference, although the non-Fontan group showed a high percentage of heart failure. Only 41 patients (60%) had a job and 8 (12%) were married. CONCLUSIONS: Although the Fontan groups had favorable pathophysiological conditions, the high rate of cardiovascular events, as well as unsatisfactory social situations, indicate the importance of meticulous life-long management of patients with SVP, regardless of the type of surgical intervention. AD - Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan. AN - 19638711 AU - Motoki, N. AU - Ohuchi, H. AU - Miyazaki, A. AU - Yamada, O. DA - Sep DB - PubMed DO - 10.1253/circj.cj-08-1155 DP - NLM ET - 2009/07/30 IS - 9 KW - Adult Age Factors Arrhythmias, Cardiac/etiology Biomarkers/blood Cardiovascular Agents/*therapeutic use Disease-Free Survival Employment Female *Fontan Procedure/adverse effects/mortality Heart Defects, Congenital/complications/mortality/physiopathology/psychology/*therapy Heart Failure/etiology Heart Ventricles/*abnormalities/physiopathology Hemodynamics Humans Japan Kaplan-Meier Estimate Male Marital Status Middle Aged Natriuretic Peptide, Brain/blood Palliative Care Quality of Life Retrospective Studies Risk Assessment *Survivors/psychology Time Factors Treatment Outcome Young Adult LA - eng N1 - 1347-4820 Motoki, Noriko Ohuchi, Hideo Miyazaki, Aya Yamada, Osamu Journal Article Japan Circ J. 2009 Sep;73(9):1711-6. doi: 10.1253/circj.cj-08-1155. Epub 2009 Jul 28. PY - 2009 SN - 1346-9843 SP - 1711-6 ST - Clinical profiles of adult patients with single ventricular physiology T2 - Circ J TI - Clinical profiles of adult patients with single ventricular physiology VL - 73 ID - 270 ER - TY - JOUR AB - Background: Exercise-induced ST-T wave depression rarely occurs in the pediatric exercise lab but its prevalence and significance are unknown. Methods: We reviewed 4400 graded exercise stress tests (EST) or chemical stress tests performed in our hospital from 2004-16. ST-T wave depression was defined as ≥2 mm ST-T wave depression in multiple leads. Myocardial perfusion imaging (MPI) with 99mTc Sestamibi injection before and at peak exercise and/or cardiac catheterization (CC) was performed when indicated. Myocardial ischemia was determined by positive MPI or coronary artery obstruction on CC or abnormal hemodynamics measured by echo or CC. Results: Eighteen patients with ST-T segment depression (0.4% incidence; Age 10.1+/-4 years; 72% male) were divided into 2 groups: Group A (12/18) with myocardial ischemia (true positives) and Group B (6/18) without myocardial ischemia (false positives). The cardiac diagnosis in Group A consisted of aortic stenosis (AS): 4, truncus arteriosus (TA): 2, hypertrophic cardiomyopathy (HCM): 2, transposition of great arteries s/p arterial switch operation (TGA, s/p ASO):1, hypertension: 1, Kawasaki disease: 1, restrictive cardiomyopathy (RCM):1. Group B included: patients s/p Fontan operation: 3, s/p Tetralogy of Fallot (TOF) repair: 2 and TGA s/p ASO: 1. In group A, 5 patients had surgery to correct the underlying abnormalities (3 AS, 1 TA, 1 TGA s/p ASO). Of these 5 patients, 4 showed resolved ST-T segment depression resolved. Of the other 7 patients, 1 patient with AS was lost to f/u. 1 patient with TA died awaiting transplant, 2 HCM patients and 1 hypertensive patient were placed on medication, 1 Kawasaki patient was scheduled for CC and 1 RCM patient had an AICD placed. In group B (6/18), no coronary abnormalities were found in the 1 TGA s/p ASO patient, 2 patients s/p TOF repair or 3 patients s/p Fontan. Discussion: In a pediatric exercise lab, ST-T wave depression is rare (0.4%) but can represent true myocardial ischemia and help management (66%). In 33% of the cases, ST-T wave depression was a non-specific finding unrelated to coronary artery disease in those s/p TOF or Fontan repair or TGA s/p ASO . AD - A.I. duPont Hospital for Children, Thomas Jefferson University AN - 120431794. Language: English. Entry Date: 20170106. Revision Date: 20170106. Publication Type: Article AU - Trivedi, Sohum AU - Temple, Joel AU - Octavio, Jenna AU - Thacker, Deepika AU - Kharouf, Rami AU - Tsuda, Takeshi AU - Gidding, Samuel S. AU - Robinson, Brad DB - ccm DP - EBSCOhost KW - Pharmacologic Stress Testing -- In Infancy and Childhood Exercise Test, Cardiopulmonary -- In Infancy and Childhood Exercise Test, Cardiopulmonary -- In Adolescence Pharmacologic Stress Testing -- In Adolescence Myocardial Ischemia -- Diagnosis -- In Infancy and Childhood Myocardial Ischemia -- Diagnosis -- In Adolescence Myocardial Ischemia -- Epidemiology -- In Infancy and Childhood Myocardial Ischemia -- Epidemiology -- In Adolescence Prevalence Perfusion Imaging Hemodynamics Incidence Prospective Studies Child Adolescence Female Male Human N1 - abstract; research. Supplement Title: 2016 Supplement. Journal Subset: Allied Health; Biomedical; Peer Reviewed; USA. NLM UID: 8909729. PY - 2016 SN - 0899-8493 SP - 37-37 ST - Clinical significance of ST- T wave changes in pediatric cardiac patients in the exercise lab...2016 North American Society for Pediatric Exercise Medicine (NASPEM) Biennial Meeting, Knoxville, Tennessee T2 - Pediatric Exercise Science TI - Clinical significance of ST- T wave changes in pediatric cardiac patients in the exercise lab...2016 North American Society for Pediatric Exercise Medicine (NASPEM) Biennial Meeting, Knoxville, Tennessee UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=120431794&site=ehost-live&scope=site VL - 28 ID - 1580 ER - TY - JOUR AB - Congenital pulmonary lymphangiectasis (CPL) is a rare, poorly documented disease, characterized by abnormal dilatation of pulmonary lymphatics without lymphatic proliferation. This disease is seen almost exclusively in infancy and early childhood. It can usually be divided into primary (congenital) and secondary forms. The primary form presents in neonates, and the patients mostly die due to the respiratory distress, shortly after birth. The authors experienced two cases of primary CPL in a 13-day-old male neonate and a one-day-old male neonate, showing prominent lymphatic dilatation in the septal, subpleural, and peri-bronchial tissue throughout both lungs. The latter case was associated with congenital cardiac anomaly including single ventricle. These are unique cases of CPL in Korea of which the diagnosis was established through post-mortem examination. Therefore, the authors report these two cases with primary CPL with a review of the literature. AD - Department of Forensic Medicine, Western District Office, National Institute of Scientific Investigation, Jangseong, Korea. AN - 17728521 AU - Eom, M. AU - Choi, Y. D. AU - Kim, Y. S. AU - Cho, M. Y. AU - Jung, S. H. AU - Lee, H. Y. C2 - PMC2693831 DA - Aug DB - PubMed DO - 10.3346/jkms.2007.22.4.740 DP - NLM ET - 2007/08/31 IS - 4 KW - Humans Infant, Newborn Lung/pathology Lung Diseases/congenital/*pathology Lymphangiectasis/congenital/*pathology Lymphatic System/pathology Male LA - eng N1 - 1598-6357 Eom, Minseob Choi, Yoo Duk Kim, Youn Shin Cho, Mee Yon Jung, Soon Hee Lee, Han Young Case Reports Journal Article J Korean Med Sci. 2007 Aug;22(4):740-5. doi: 10.3346/jkms.2007.22.4.740. PY - 2007 SN - 1011-8934 (Print) 1011-8934 SP - 740-5 ST - Clinico-pathological characteristics of congenital pulmonary lymphangiectasis: report of two cases T2 - J Korean Med Sci TI - Clinico-pathological characteristics of congenital pulmonary lymphangiectasis: report of two cases VL - 22 ID - 490 ER - TY - JOUR AB - Congenital pulmonary lymphangiectasis (CPL) is a rare, poorly documented disease, characterized by abnormal dilatation of pulmonary lymphatics without lymphatic proliferation. This disease is seen almost exclusively in infancy and early childhood. It can usually be divided into primary (congenital) and secondary forms. The primary form presents in neonates, and the patients mostly die due to the respiratory distress, shortly after birth. The authors experienced two cases of primary CPL in a 13-day-old male neonate and a one-day-old male neonate, showing prominent lymphatic dilatation in the septal, subpleural, and peri-bronchial tissue throughout both lungs. The latter case was associated with congenital cardiac anomaly including single ventricle. These are unique cases of CPL in Korea of which the diagnosis was established through post-mortem examination. Therefore, the authors report these two cases with primary CPL with a review of the literature. Copyright © The Korean Academy of Medical Sciences. AD - S.-H. Jung, Department of Pathology, Wonju College of Medicine, Yonsei University, 162 Ilsan-dong, Wonju 220-701, South Korea AU - Eom, M. AU - Yoo, D. C. AU - Youn, S. K. AU - Cho, M. Y. AU - Jung, S. H. AU - Han, Y. L. DB - Medline DO - 10.3346/jkms.2007.22.4.740 IS - 4 KW - article case report human lung lung disease lymphangiectasis lymphatic system male newborn pathology LA - English M3 - Article N1 - L47395324 2008-01-17 http://jkms.org/fulltext/pdf/jkms-22-740.pdf PY - 2007 SN - 1011-8934 1598-6357 SP - 740-745 ST - Clinico-pathological characteristics of congenital pulmonary lymphangiectasis: Report of two cases T2 - Journal of Korean Medical Science TI - Clinico-pathological characteristics of congenital pulmonary lymphangiectasis: Report of two cases UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L47395324 http://dx.doi.org/10.3346/jkms.2007.22.4.740 VL - 22 ID - 1231 ER - TY - JOUR AB - INTRODUCTION: Tracheocutaneous fistula is a complication of tracheostomy. Tracheocutaneous fistulectomy followed by primary closure carries a high possibility of complications. PRESENTATION OF CASE: An 11-year-old boy underwent surgery to repair a tracheocutaneous fistula, using skin and muscle flaps. A vertical incision was made around the fistula and 3 skin flaps were prepared: 2 hinge flaps, and 1 to cover the skin defect (advanced flap). The 2 hinged turnover flaps were invaginated by multiple layered sutures, and a strap muscle flap was placed over the resulting tracheal closure. An advanced skin flap was used to cover the area of the previous defect. The patient was extubated immediately after surgery. He was discharged on the sixth postoperative day without tracheal leakage or subcutaneous emphysema. The patient is currently doing well, with no respiratory symptoms and no recurrence at the postoperative 5 months. DISCUSSION: Our technique is minimally invasive and has a low risk of lumen stenosis, other complications, or recurrence. CONCLUSION: This technique demonstrates the multiple-layered closure of a tracheocutaneous fistula, using skin flaps and a muscle flap. © 2012 Surgical Associates Ltd. AD - Department of Pediatric Surgery, National Hospital Organization, Fukuyama Medical Center, 4-14-17, Okinogami-cho, Fukuyama, Hiroshima 720-8520, Japan AU - Tatekawa, Y. AU - Yamanaka, H. AU - Hasegawa, T. DB - Scopus DO - 10.1016/j.ijscr.2012.11.015 IS - 2 KW - Hinge flap Muscle flap Tracheocutaneous fistula M3 - Article N1 - Cited By :9 Export Date: 15 June 2020 PY - 2013 SP - 170-174 ST - Closure of a tracheocutaneous fistula by two hinged turnover skin flaps and a muscle flap: A case report T2 - International Journal of Surgery Case Reports TI - Closure of a tracheocutaneous fistula by two hinged turnover skin flaps and a muscle flap: A case report UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84871889573&doi=10.1016%2fj.ijscr.2012.11.015&partnerID=40&md5=9a7b1fd48856dc7ba2b3ac752eca8759 VL - 4 ID - 2066 ER - TY - JOUR AB - BACKGROUND: Patients with a single ventricle have multiple risk factors for central nervous system injury, both before and after the Fontan procedure. METHODS AND RESULTS: A geographically selected cohort was invited to undergo standardized testing, including age-appropriate measures of intelligence quotient (IQ) and achievement tests. Historical information was obtained by chart review and patient questionnaires. Of the 222 eligible patients, 133 (59.9%) participated. Median age at testing was 11.1 years (range, 3. 7 to 41.0 years), 6.0 years (range, 1.6 to 19.6 years) after surgery. Mean full-scale IQ was 95.7+/-17.4 (P<0.006 versus normal); 10 patients (7.8%) had full-scale IQ scores <70 (P=0.001). After adjustment for socioeconomic status, lower IQ was associated with the use of circulatory arrest before the Fontan operation (P=0.002), the anatomic diagnoses of hypoplastic left heart syndrome (P<0.001) and "other complex" (P=0.05), and prior placement of a pulmonary artery band (P=0.04). Mean composite achievement score was 91.6+/-15. 4 (P<0.001 versus normal); 14 patients (10.8%) scored <70 (P<0.001). After adjustment for socioeconomic status, independent risk factors for low achievement scores included the diagnoses of hypoplastic left heart syndrome (P=0.004) and "other complex" (P=0.003) or prior use of circulatory arrest (P=0.03), as well as a reoperation with cardiopulmonary bypass within 30 days of the Fontan (P=0.01). CONCLUSIONS: Most individual patients palliated with the Fontan procedure in the 1970s and 1980s have cognitive outcome and academic function within the normal range, but the performance of the cohort is lower than that of the general population. AD - Department of Cardiology, Children's Hospital, Harvard Medical School, Boston, MA, USA. wernovsky@email.chop.edu AN - 10952957 AU - Wernovsky, G. AU - Stiles, K. M. AU - Gauvreau, K. AU - Gentles, T. L. AU - duPlessis, A. J. AU - Bellinger, D. C. AU - Walsh, A. Z. AU - Burnett, J. AU - Jonas, R. A. AU - Mayer, J. E., Jr. AU - Newburger, J. W. DA - Aug 22 DB - PubMed DO - 10.1161/01.cir.102.8.883 DP - NLM ET - 2000/08/23 IS - 8 KW - Adolescent Adult Cardiopulmonary Bypass/adverse effects Child Child, Preschool Cognition Disorders/*etiology Educational Measurement/methods Follow-Up Studies Fontan Procedure/*adverse effects Heart Defects, Congenital/*complications/surgery Heart Ventricles/abnormalities/surgery Humans Intelligence Tests Learning Disabilities/etiology Multivariate Analysis Surveys and Questionnaires Treatment Outcome LA - eng N1 - 1524-4539 Wernovsky, G Stiles, K M Gauvreau, K Gentles, T L duPlessis, A J Bellinger, D C Walsh, A Z Burnett, J Jonas, R A Mayer, J E Jr Newburger, J W Journal Article Research Support, Non-U.S. Gov't United States Circulation. 2000 Aug 22;102(8):883-9. doi: 10.1161/01.cir.102.8.883. PY - 2000 SN - 0009-7322 SP - 883-9 ST - Cognitive development after the Fontan operation T2 - Circulation TI - Cognitive development after the Fontan operation VL - 102 ID - 379 ER - TY - JOUR AB - Neurological and radiologic research results show an abnormal cerebral microstructure as well as abnormal neurodevelopment in patients treated for hypoplastic left heart syndrome. The aim of this study was to assess the varying cognitive performance these children have developed in dependence upon prenatal diagnosis, surgical techniques, surgical learning effects, anatomy, perfusion techniques, gender, pedagogic, and sociodemographic parameters in comparison to age-adjusted normative values. School-age children (6.3-16.9 years) with hypoplastic left heart syndrome, who were treated at the Children's heart Center Linz between 1997 and 2009, (n = 74), were surveyed in reference to cognitive achievements. 43 patients were examined prospectively by psychologists using the Wechsler intelligence scale for children IV in order to determine the respective total intelligence quotient index for each child's developmental stage. The mean index was 84.5 (percentile rank 26.4). The statistical spread and standard deviation ranged from a minimum of 40 to a maximum of 134 ± 20.8. The results for verbal comprehension, perceptual reasoning, and processing speed corresponded with total index results and were thus lower than the mean value of the normative values. The assessment of working memory showed results in the average. Prenatal diagnosis, type of lung perfusion, anatomy, and various cerebral perfusion techniques did not significantly affect the cognitive results of the patients. The results show that hypoplastic left heart syndrome patients can be successfully tutored formally as well as personally in cognitive areas, although when compared to healthy children, they showed lower results for intellectual area parameters. AD - Children's Heart Center Linz, Kepler University Hospital, Krankenhausstrasse 26, 4020, Linz, Austria. r.oberhuber@eduhi.at. Department of Inclusive Education, University of Education Upper Austria, Kaplanhofstrasse 40, 4020, Linz, Austria. r.oberhuber@eduhi.at. Children's Heart Center Linz, Kepler University Hospital, Krankenhausstrasse 26, 4020, Linz, Austria. Department of Inclusive Education, University of Education Upper Austria, Kaplanhofstrasse 40, 4020, Linz, Austria. AN - 28508919 AU - Oberhuber, R. D. AU - Huemer, S. AU - Mair, R. AU - Sames-Dolzer, E. AU - Kreuzer, M. AU - Tulzer, G. DA - Aug DB - PubMed DO - 10.1007/s00246-017-1623-8 DP - NLM ET - 2017/05/17 IS - 6 KW - Adolescent Child Cognition Disorders/*diagnosis/etiology Female Humans Hypoplastic Left Heart Syndrome/*complications/surgery Intelligence Tests Male Neurodevelopmental Disorders/*diagnosis/etiology Neuropsychological Tests Norwood Procedures/methods Cognitive development Hypoplastic left heart syndrome Long-term follow-up LA - eng N1 - 1432-1971 Oberhuber, Raphael D Huemer, Sonja Mair, Rudolf Sames-Dolzer, Eva Kreuzer, Michaela Tulzer, Gerald Journal Article Review United States Pediatr Cardiol. 2017 Aug;38(6):1089-1096. doi: 10.1007/s00246-017-1623-8. Epub 2017 May 16. PY - 2017 SN - 0172-0643 SP - 1089-1096 ST - Cognitive Development of School-Age Hypoplastic Left Heart Syndrome Survivors: A Single Center Study T2 - Pediatr Cardiol TI - Cognitive Development of School-Age Hypoplastic Left Heart Syndrome Survivors: A Single Center Study VL - 38 ID - 79 ER - TY - JOUR AB - OBJECTIVE: We carried out a cross-sectional study to assess cognitive function in a sample of adult CHD patients, within the Functioning in Adult Congenital Heart Disease study London. The association between cognitive functioning and disease complexity was examined. METHODS: A total of 310 patients participated in this study. Patients were classified into four structural complexity groups - tetralogy of Fallot, transposition of the great arteries, single ventricle, and simple conditions. Each patient underwent neuropsychological assessment to evaluate cognitive function, including memory and executive function, and completed questionnaires to assess depression and anxiety. RESULTS: Among all, 41% of the sample showed impaired performance (>1.5 SD below the normative mean) on at least three tests of cognitive function compared with established normative data. This was higher than the 8% that was expected in a normal population. The sample exhibited significant deficits in divided attention, motor function, and executive functioning. There was a significant group difference in divided attention (F=5.01, p=0.002) and the mean total composite score (F=5.19, p=0.002) between different structural complexity groups, with the simple group displaying better cognitive function. CONCLUSION: The results indicate that many adult CHD patients display impaired cognitive function relative to a healthy population, which differs in relation to disease complexity. These findings may have implications for clinical decision making in this group of patients during childhood. Possible mechanisms underlying these deficits and how they may be reduced or prevented are discussed; however, further work is needed to draw conclusive judgements. AD - 1Centre for Health Services Research,School of Health Sciences,City University London,London,United Kingdom. 2GUCH Unit,The Heart Hospital,University College of London Hospitals NHS Foundation Trust,London,United Kingdom. AN - 27751192 AU - Tyagi, M. AU - Fteropoulli, T. AU - Hurt, C. S. AU - Hirani, S. P. AU - Rixon, L. AU - Davies, A. AU - Picaut, N. AU - Kennedy, F. AU - Deanfield, J. AU - Cullen, S. AU - Newman, S. P. DA - Jul DB - PubMed DO - 10.1017/s1047951116001396 DP - NLM ET - 2016/10/19 IS - 5 KW - Adolescent Adult Aged Anxiety/psychology Attention Cognition Cognitive Dysfunction/*epidemiology Cross-Sectional Studies Depression/psychology Executive Function Female Heart Defects, Congenital/*classification/*complications Humans London/epidemiology Male Middle Aged Neuropsychological Tests Psychiatric Status Rating Scales Self Report Young Adult Adult CHD mood neuropsychological assessment LA - eng N1 - 1467-1107 Tyagi, Manavi Fteropoulli, Theodora Hurt, Catherine S Hirani, Shashivadan P Rixon, Lorna Davies, Anna Picaut, Nathalie Kennedy, Fiona Deanfield, John Cullen, Shay Newman, Stanton P MR/K006584/1/Medical Research Council/United Kingdom Journal Article England Cardiol Young. 2017 Jul;27(5):851-859. doi: 10.1017/S1047951116001396. Epub 2016 Oct 18. PY - 2017 SN - 1047-9511 SP - 851-859 ST - Cognitive dysfunction in adult CHD with different structural complexity T2 - Cardiol Young TI - Cognitive dysfunction in adult CHD with different structural complexity VL - 27 ID - 262 ER - TY - JOUR AB - OBJECTIVES: Improvements in the management of complex congenital heart disease, including those with single ventricle physiology, have resulted in increased survival. As this population ages, the recognition of cognitive impairment is increasingly important. At present, little is known about the potential mechanisms of cognitive dysfunction. In this cross-sectional study, we aimed to characterize the nature of abnormalities in cerebral blood flow and the relationship to cognitive deficits in adults with single ventricular physiology. PATIENTS: Ten adults with single ventricular physiology (age 18-40 years) and 12 age- and gender-matched controls underwent transcranial Doppler ultrasound and accompanying cognitive assessment. OUTCOME MEASURES: Patients underwent neuropsychological testing that assessed differing cognitive domains, with subjective cognitive decline determined from a 24-question survey. Transcranial Doppler ultrasound was used to assess baseline cerebral blood flow as well as change in cerebral blood flow velocities from baseline and during cognitive testing. Age, ethnicity, individual, and parental education levels were considered in the multivariate analyses. RESULTS: On assessment of cognitive function, the patient group performed more poorly across each of the measured domains. The control group had a significantly greater increase in cerebral blood flow in response to cognitive stimuli compared to the patient cohort; these differences in response to cognitive stimuli were seen to a similar extent across each of the measured cognitive domains. CONCLUSION: Adults with Fontan physiology are underperforming in assessments of executive function with associated abnormalities in cerebral perfusion potentially contributing to cognitive deficits. AD - School of Biomedical Sciences and Pharmacy, University of Newcastle, Callaghan, Australia. Department of Medicine, John Hunter Hospital, New Lambton, Australia. AN - 30908884 AU - Wong, R. AU - Al-Omary, M. AU - Baker, D. AU - Spratt, N. AU - Boyle, A. AU - Baker, N. AU - Howe, P. AU - Collins, N. DA - Jul DB - PubMed DO - 10.1111/chd.12763 DP - NLM ET - 2019/03/26 IS - 4 KW - Adolescent Adult Cerebrovascular Circulation/*physiology Cognition/*physiology Cognitive Dysfunction/diagnosis/*etiology/physiopathology Cross-Sectional Studies Female Follow-Up Studies Heart Defects, Congenital/*complications/diagnosis/physiopathology Heart Ventricles/*abnormalities Humans Male Regional Blood Flow/*physiology Ultrasonography, Doppler, Transcranial/*methods Young Adult Fontan procedure cerebral blood flow cognitive function congenital heart defects LA - eng N1 - 1747-0803 Wong, Rachel Al-Omary, Mohammed Baker, David Spratt, Neil Boyle, Andrew Baker, Natasha Howe, Peter Collins, Nicholas Orcid: 0000-0003-4722-0214 Journal Article United States Congenit Heart Dis. 2019 Jul;14(4):638-644. doi: 10.1111/chd.12763. Epub 2019 Mar 25. PY - 2019 SN - 1747-079x SP - 638-644 ST - Cognitive dysfunction is associated with abnormal responses in cerebral blood flow in patients with single ventricular physiology: Novel insights from transcranial Doppler ultrasound T2 - Congenit Heart Dis TI - Cognitive dysfunction is associated with abnormal responses in cerebral blood flow in patients with single ventricular physiology: Novel insights from transcranial Doppler ultrasound VL - 14 ID - 502 ER - TY - JOUR AB - OBJECTIVE: Despite surgical palliation, children with hypoplastic left heart syndrome (HLHS) have compromised cardiac functioning and increased risk for cognitive deficits. We quantitatively reviewed the empirical data from this literature. METHODS: The present meta-analysis included 13 studies reporting cognitive function for children with HLHS between the ages of 2 years and 6 months and 17 years that used standardized assessments of Full Scale IQ (FSIQ), Verbal IQ (VIQ), and Performance IQ (PIQ). Differences in cognitive function were assessed relative to normative data, and we examined sample mean age and publication year as moderators. RESULTS: Large effects were found for FSIQ (g = -.87, 95% CI [-1.10, -.65], M = 86.88) and PIQ (g = -.89, 95% CI [-1.11, -.68], M = 86.56), and a medium effect was found for VIQ (g = -.61, 95% CI [-.84, -.38], M = 90.82). All models demonstrated significant heterogeneity. Meta-regression analyses of effect size via Hedges' g on child age revealed a significant effect on FSIQ (coefficient = -.07, 95% CI [-.12, -.01], p < .01, R2 = .40) indicating a loss of 1.1 FSIQ points across studies with each increased year of mean sample age. CONCLUSIONS: Deficits in FSIQ may reflect chronic brain injury or failure to make expected gains as children age. This review highlights the importance of early intervention in this population, and the need for longitudinal studies analyzing more specific domains of cognitive function and potential moderators. AU - Siciliano, R. E. AU - Prussien, K. V. AU - Lee, C. A. AU - Patel, N. J. AU - Murphy, L. K. AU - Compas, B. E. AU - Jordan, L. C. DB - Medline DO - 10.1093/jpepsy/jsz021 IS - 8 KW - article brain injury child cognitive defect controlled study early intervention effect size female human hypoplastic left heart syndrome longitudinal study male meta analysis publication regression analysis systematic review LA - English M3 - Article N1 - L627742416 2019-05-23 PY - 2019 SN - 1465-735X SP - 937-947 ST - Cognitive Function in Pediatric Hypoplastic Left Heart Syndrome: Systematic Review and Meta-Analysis T2 - Journal of pediatric psychology TI - Cognitive Function in Pediatric Hypoplastic Left Heart Syndrome: Systematic Review and Meta-Analysis UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L627742416 http://dx.doi.org/10.1093/jpepsy/jsz021 VL - 44 ID - 599 ER - TY - JOUR AB - Neurodevelopmental disabilities are common sequelae of congenital heart disease, particularly cognitive impairments that impede academic achievement. Cognitive training has been shown to be effective in improving working memory in other patient groups. Computerized cognitive training programs may hold potential for children and adolescents with congenital heart disease, but are untested in this population. In this single-center, single-blinded, randomized controlled trial, the feasibility and efficacy of Cogmed Working Memory Training (Cogmed) was compared with observation in children with hypoplastic left heart syndrome 8 to 16 years of age. Participants were randomized to either Cogmed at home for 5 weeks with coaching by a study team member or an observation control group. Baseline, 6–8 weeks later (post-intervention), and 6-month assessments of working memory were completed. Participants randomized to the Cogmed intervention (n = 10) demonstrated greater improvement than controls (n = 10) on measures of working memory, the Wechsler Intelligence Scale for Children-V Working Memory Index and the National Institutes of Health Toolbox Cognitive Battery post-training that were statistically significant (Cohen's d = 0.76, p < .001 and d = 0.43, p < .01) compared to controls over the same time period. Improvements were not maintained at 6-month follow-up. Three children withdrew from cognitive training, and one additional child did not complete at least 20 of 25 cognitive training sessions despite coaching. In conclusion, study findings show computerized cognitive training is feasible for most children with hypoplastic left heart syndrome, but shorter sessions may improve adherence. Training-related improvements in working memory occur, though continued reinforcement may be necessary for these gains to be maintained. AD - L.C. Jordan, Department of Pediatrics, Division of Pediatric Neurology, Vanderbilt University Medical Center, 2200 Children's Way DOT 11212, Nashville, TN, United States AU - Jordan, L. C. AU - Siciliano, R. E. AU - Cole, D. A. AU - Lee, C. A. AU - Patel, N. J. AU - Murphy, L. K. AU - Markham, L. W. AU - Prussien, K. V. AU - Gindville, M. C. AU - Compas, B. E. DB - Embase DO - 10.1016/j.ppedcard.2019.101185 KW - adolescent article child clinical article cognitive defect comparative effectiveness complication controlled study feasibility study female follow up human hypoplastic left heart syndrome male national health organization nervous system malformation randomized controlled trial reinforcement single blind procedure Wechsler intelligence scale for children working memory LA - English M3 - Article in Press N1 - L2004191225 2019-12-20 PY - 2019 SN - 1558-1519 1058-9813 ST - Cognitive training in children with hypoplastic left heart syndrome: A pilot randomized trial T2 - Progress in Pediatric Cardiology TI - Cognitive training in children with hypoplastic left heart syndrome: A pilot randomized trial UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2004191225 http://dx.doi.org/10.1016/j.ppedcard.2019.101185 ID - 636 ER - TY - JOUR AB - Objective To describe outcomes for women with a Fontan circulation attending preconception counselling (PCC). Exploring (1) impact of counselling on decision to become pregnant; (2) pregnancy rates in those opting for pregnancy and (3) short-term and long-term outcome of those who succeeded in becoming pregnant. Methods Retrospective review of women aged 16-45 years with a Fontan circulation from 1994 to 2014. Results 58 women were offered PCC, 3 declined and 55 received PCC. Following PCC, 15 opted against conception, 16 decided to delay pregnancy, 19 attempted pregnancy and 5 were lost to follow-up. Of the 19 women, 14 succeeded, becoming pregnant a total of 43 times (median 1, range 1-9). Of these, 6 miscarried all pregnancies. 8 carried 14 pregnancies to viability. Baseline hypoxaemia and cardiac disease in pregnancy (CARPREG) score was similar in those opting for and against pregnancy, but CARPREG score was better in those delaying conception. Women exclusively miscarrying or unable to conceive were more likely to have baseline hypoxaemia and greater CARPREG score. Cardiac complications included arrhythmia requiring treatment (n=4) and one thromboembolism. Obstetric complications were greater in women with a Fontan circulation, 10 preterm births (<37 weeks) and 8 small for gestational age babies (<10th centile). There was one neonatal death. At follow-up, there was no deterioration in clinical status as determined by echo. Conclusions Most women accept PCC and decided to pursue pregnancy; in some cases, this was despite being advised of a poor prognosis. Pregnancy outcome was related to baseline hypoxia and CARPREG scores. AD - Academic Department of Obstetrics and Gynaecology, Imperial College London, Chelsea and Westminster Hospital, 369 Fulham Road, London, SW10 9NH, United Kingdom Adult Congenital Heart Centre, National Heart and Lung Institute, Imperial College London, Royal Brompton Hospital, London, United Kingdom AU - Cauldwell, M. AU - Von Klemperer, K. AU - Uebing, A. AU - Swan, L. AU - Steer, P. J. AU - Babu-Narayan, S. V. AU - Gatzoulis, M. A. AU - Johnson, M. R. DB - Scopus DO - 10.1136/heartjnl-2015-308788 IS - 7 M3 - Article N1 - Cited By :22 Export Date: 15 June 2020 PY - 2016 SP - 534-540 ST - A cohort study of women with a Fontan circulation undergoing preconception counselling T2 - Heart TI - A cohort study of women with a Fontan circulation undergoing preconception counselling UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84956634273&doi=10.1136%2fheartjnl-2015-308788&partnerID=40&md5=1b6a1016e3e7fe9ccdf5730842ae22a4 VL - 102 ID - 1914 ER - TY - JOUR AB - Capillary leak syndrome is a critical condition occasionally occurring posttransplant and is characterized by acute endothelial hyperpermeability leading to systemic protein-rich fluid extravasation and consequent hypovolemia, hypoperfusion, and acute kidney injury. Treatment is merely supportive and is based on osmotic drugs, diuretics, continuous renal replacement therapy, and surgical drainage. However, removal of the underlying inflammatory cause is mandatory to achieve stable resolution. Herein, we report the first successful treatment with colchicine in 2 life-threatening pediatric cases of capillary leak syndrome with renal failure occurring after transplant (heart and bone marrow) and unresponsive to any other line of therapy. Both cases were only palliated by supportive therapy and revealed an impressively rapid response to colchicine both in terms of diuresis and clinical condition recovery, allowing for the cessation of renal replacement therapy in a few hours. In both patients, colchicine was temporarily discontinued for transient leukopenia (attributed to an additive effect with mycophenolate mofetil), resulting in extravasation, and renal failure recurrence was restored only after colchicine reintroduction. Although the association of colchicine with an immunosuppressive drug was formerly contraindicated, no other adverse events were noted when using a minimized dose. Both patients are now maintaining a good renal function without recurrence of extravasation after 6 months of follow-up. In conclusion, this strikingly positive experience forces physicians to consider this old and cost-effective drug as a new, powerful rescue tool in such critical cases. Copyright © 2019 by the American Academy of Pediatrics. AD - University of Turin, Turin, Italy Nephrology, Dialysis and Transplantation Unit, Regina Margherita Children’s Hospital, Turin, Italy Pediatric Cardiac Surgery Unit, Regina Margherita Children’s Hospital, Turin, Italy Pediatric Onco-Hematology Unit, Stem Cell Transplantation and Cellular Therapy Division, Regina Margherita Children’s Hospital, Turin, Italy AU - Cocchi, E. AU - Chiale, F. AU - Gianoglio, B. AU - Deorsola, L. AU - Napoleone, C. P. AU - Fagioli, F. AU - Peruzzi, L. C7 - Y DB - Scopus DO - 10.1542/peds.2018-2820 IS - 5 M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2019 ST - Colchicine: An impressive effect on posttransplant capillary leak syndrome and renal failure T2 - Pediatrics TI - Colchicine: An impressive effect on posttransplant capillary leak syndrome and renal failure UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85065508941&doi=10.1542%2fpeds.2018-2820&partnerID=40&md5=4235ad71be0819705435612801b19ff6 VL - 143 ID - 1751 ER - TY - JOUR AB - AIMS: During the last decade the cooperation between surgeons and cardiologists has further expanded by combining surgical and interventional techniques (CCBSI) performed in the operating room, without the use of fluoroscopy. We sought to evaluate the results of our experience with CCBSI. METHODS: All children with congenital heart disease (CHD) who underwent a CCBSI in the operating room between June 2007 and January 2014 were enrolled. RESULTS: Sixty-eight patients were included. Median age at CCBSI was five months (range 1-48 months). The three main diagnoses leading to surgery included: (1) tetralogy of Fallot (TOF) (n = 40), (2) muscular ventricular septal defects (VSD) (n = 12), (3) single ventricle with pulmonary artery branch stenosis (n = 4). There were 72 catheter-based procedures associated with surgical maneuvres, including: (1) transatrial balloon dilation (BD) of the pulmonary valve (n = 45), (2) transinfundibular BD of the main pulmonary artery trunk (n = 12), (3) perventricular VSD closure with septal occluder (n = 8), (4) BD of pulmonary artery branches (n = 5), and other less common procedures (n = 2). There were no procedure-related complications and no hospital deaths. Median follow-up time was four years (range 0.95-7.9 years). There was one late death for respiratory distress after transapical balloon dilation of the aortic valve. One patient required BD and stenting of the left pulmonary artery branch 3.6 years after intraoperative BD for residual stenosis. CONCLUSIONS: The CCBSI represents a safe and effective treatment for selected patients with complex CHD. It will be helpful in minimizing patients' surgical trauma and in shortening or avoiding the use of cardiopulmonary bypass. AD - Pediatric and Congenital Cardiac Surgery Unit, Department of Thoracic, Cardiac and Vascular Sciences, University of Padua, Padua, Italy. Pediatric Cardiology Unit, Department of Paediatrics, University of Padua, Padua, Italy. AN - 26174169 AU - Vida, V. L. AU - Guariento, A. AU - Zucchetta, F. AU - Padalino, M. A. AU - Milanesi, O. AU - Maschietto, N. AU - Stellin, G. DA - Sep DB - PubMed DO - 10.1111/jocs.12595 DP - NLM ET - 2015/07/16 IS - 9 KW - Cardiac Catheterization/methods Cardiac Surgical Procedures/*methods Cardiopulmonary Bypass Child, Preschool Combined Modality Therapy Dilatation/methods Escherichia coli Proteins Female Heart Defects, Congenital/*therapy Humans Infant Male Operative Time Pulmonary Artery Pulmonary Valve Septal Occluder Device Stents Tetrahydrofolate Dehydrogenase Time Factors Treatment Outcome LA - eng N1 - 1540-8191 Vida, Vladimiro L Guariento, Alvise Zucchetta, Fabio Padalino, Massimo A Milanesi, Ornella Maschietto, Nicola Stellin, Giovanni Journal Article United States J Card Surg. 2015 Sep;30(9):719-23. doi: 10.1111/jocs.12595. Epub 2015 Jul 14. PY - 2015 SN - 0886-0440 SP - 719-23 ST - Combined Surgical and Interventional Approaches for Treating Patients with Congenital Heart Disease T2 - J Card Surg TI - Combined Surgical and Interventional Approaches for Treating Patients with Congenital Heart Disease VL - 30 ID - 371 ER - TY - JOUR AB - Early recognition of congenital cardiac pathologies and their treatment by means of palliative or corrective surgery at birth or infancy has vital importance. Successful repair of congenital cardiac defects by surgical methods has gained importance especially during the last twenty years. As the scope of the surveillance increased so did the interest in the outcomes of these treatments when the patients had reached puberty and adulthood. The purpose of our research was to study the psychological framework of the adolescents who had experienced these surgeries by listening both the children and the parents talk about their feelings and experiences. Our data was accumulated through interviews with 17 adolescents and their families, using qualitative methods. The main theme at the end of the analysis was "to be strong and resistive". We reached the conclusion that this condition was not a pathological build up but an attitude of coping, as it did not cause loss of functionality. The defensive psychological mechanisms used by these adolescents consisted of repression, compensation and reaction formation. We believe that this information is important to understand the real meaning of the manners displayed when these adolescents and their families pursue their daily lives, communicate and make relationships with their environment and especially professionals in the health services. © 2011 Zahmacioglu et al; licensee BioMed Central Ltd. AD - Department of Child and Adolescent Psychiatry, Yeditepe University Medical Faculty, Istanbul, Turkey Department of Cardiovascular Surgery, Istanbul University Institute of Cardiology, Istanbul, Turkey Department of Pediatric Cardiology, Istanbul University Cerrahpasa Medical Faculty, Istanbul, Turkey Cardiovascular Surgery Clinic, Duzce Ataturk State Hospital, Duzce, Turkey AU - Zahmacioglu, O. AU - Yildiz, C. E. AU - Koca, B. AU - Ugurlucan, M. AU - Gokalp, S. AU - Cetin, G. AU - Guler Eroglu, A. AU - Oztunc, F. C7 - 155 DB - Scopus DO - 10.1186/1749-8090-6-155 IS - 1 KW - Congenital heart disease Coping styles Defense mechanisms experiences Fontan operation Single ventricle M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2011 ST - Coming from behind to win - A Qualitative research about psychological conditions of adolescents who have undergone open-heart surgery for single ventricle between the ages 0-5 T2 - Journal of Cardiothoracic Surgery TI - Coming from behind to win - A Qualitative research about psychological conditions of adolescents who have undergone open-heart surgery for single ventricle between the ages 0-5 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-81555230243&doi=10.1186%2f1749-8090-6-155&partnerID=40&md5=59e383c44cfd801aacad656b8d13b26e VL - 6 ID - 2111 ER - TY - JOUR AB - Early recognition of congenital cardiac pathologies and their treatment by means of palliative or corrective surgery at birth or infancy has vital importance. Successful repair of congenital cardiac defects by surgical methods has gained importance especially during the last twenty years. As the scope of the surveillance increased so did the interest in the outcomes of these treatments when the patients had reached puberty and adulthood. The purpose of our research was to study the psychological framework of the adolescents who had experienced these surgeries by listening both the children and the parents talk about their feelings and experiences. Our data was accumulated through interviews with 17 adolescents and their families, using qualitative methods. The main theme at the end of the analysis was "to be strong and resistive". We reached the conclusion that this condition was not a pathological build up but an attitude of coping, as it did not cause loss of functionality. The defensive psychological mechanisms used by these adolescents consisted of repression, compensation and reaction formation. We believe that this information is important to understand the real meaning of the manners displayed when these adolescents and their families pursue their daily lives, communicate and make relationships with their environment and especially professionals in the health services. AD - Department of Child and Adolescent Psychiatry, Yeditepe University Medical Faculty, Istanbul, Turkey. AN - 22112589 AU - Zahmacioglu, O. AU - Yildiz, C. E. AU - Koca, B. AU - Ugurlucan, M. AU - Gokalp, S. AU - Cetin, G. AU - Eroglu, A. G. AU - Oztunc, F. C2 - PMC3269384 DA - Nov 23 DB - PubMed DO - 10.1186/1749-8090-6-155 DP - NLM ET - 2011/11/25 KW - *Adaptation, Psychological Adolescent Cardiac Surgical Procedures/*psychology Child, Preschool Female Heart Defects, Congenital/psychology/*surgery Heart Ventricles/*abnormalities/surgery Humans Infant Infant, Newborn Male *Qualitative Research Surveys and Questionnaires LA - eng N1 - 1749-8090 Zahmacioglu, Oguzhan Yildiz, Cenk Eray Koca, Bulent Ugurlucan, Murat Gokalp, Selman Cetin, Gurkan Eroglu, Ayse Guler Oztunc, Funda Comparative Study Journal Article J Cardiothorac Surg. 2011 Nov 23;6:155. doi: 10.1186/1749-8090-6-155. PY - 2011 SN - 1749-8090 SP - 155 ST - Coming from behind to win--a qualitative research about psychological conditions of adolescents who have undergone open-heart surgery for single ventricle between the ages 0-5 T2 - J Cardiothorac Surg TI - Coming from behind to win--a qualitative research about psychological conditions of adolescents who have undergone open-heart surgery for single ventricle between the ages 0-5 VL - 6 ID - 274 ER - TY - JOUR AB - BACKGROUND: Single ventricle defects are among the most complex congenital heart defects and the development of advanced surgical procedures in recent decades has created the first generation of adolescents and young adults living with this condition. Yet little is known about how these individuals experience life and what impact the heart defect has on their life in general. OBJECTIVE: The aim was to illuminate and gain a deeper understanding of adolescents' and young adults' experiences of living with a surgically palliated univentricular heart. DESIGN: Seven open-ended in-depth interviews were conducted, transcribed, and analyzed according to the henomenological hermeneutical method. All adolescents and young adults operated before 1995 according to the Fontan procedure or the total cavo-pulmonary connection procedure at one pediatric cardiology unit were included in the study. They were 17-32 years of age (median age 22 years). RESULTS: The interpretation of the interview transcripts showed that the participants experienced living with a surgically palliated univentricular heart in terms of feeling exceptional, strong, and healthy. This was supported by two structural analyses, where three themes emerged: happiness over being me, focusing on possibilities, and being committed to life. CONCLUSION: Living with a Fontan circulation included negative experiences but the analyses clearly demonstrated a feeling of being strong and healthy. An appreciation of having survived and being committed to life was found to be an integral part of the development of the interviewees' existential growth. This probably strengthens them further in their ability to balance expectations and hurdles in life. This study provides valuable insights into the experience of patients after the Fontan procedure and the importance of a positive health care environment throughout their lives. AD - Institute of Medicine, Sahlgrenska Academy, Gothenburg, Sweden. Centre for Person-Centred Care, University of Gothenburg, Gothenburg, Sweden. Institute of Caring, Health and Culture, University West, Trollhättan, Sweden. Institute of Health and Care Sciences, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden. Department of Clinical Sciences, Pediatrics, University of Umeå, Umeå, Sweden. Adult Congenital Heart Unit, Sahlgrenska University Hospital/Östra, Gothenburg, Sweden. AN - 25564938 AU - Berghammer, M. C. AU - Brink, E. AU - Rydberg, A. M. AU - Dellborg, M. AU - Ekman, I. DA - Sep-Oct DB - PubMed DO - 10.1111/chd.12244 DP - NLM ET - 2015/01/08 IS - 5 KW - *Adaptation, Psychological Adolescent *Adolescent Behavior Adult *Cost of Illness Emotions Female Fontan Procedure/adverse effects/*psychology Heart Defects, Congenital/diagnosis/physiopathology/psychology/*surgery Humans Interviews as Topic Male Palliative Care *Quality of Life Self Concept Treatment Outcome Young Adult Adolescents and Young Adults Fontan Circulation In-Depth Interviews Lived Experience Phenomenological Hermeneutics Univentricular Heart LA - eng N1 - 1747-0803 Berghammer, Malin C Brink, Eva Rydberg, Annika M Dellborg, Mikael Ekman, Inger Journal Article United States Congenit Heart Dis. 2015 Sep-Oct;10(5):403-12. doi: 10.1111/chd.12244. Epub 2015 Jan 7. PY - 2015 SN - 1747-079x SP - 403-12 ST - Committed to Life: Adolescents' and Young Adults' Experiences of Living with Fontan Circulation T2 - Congenit Heart Dis TI - Committed to Life: Adolescents' and Young Adults' Experiences of Living with Fontan Circulation VL - 10 ID - 101 ER - TY - JOUR AB - Background The angiotensin-converting enzyme insertion/deletion (ACE I/D) polymorphism is described in association with numerous phenotypes, including arrhythmias, and may provide predictive value among pediatric patients undergoing congenital heart surgery. Objective The purpose of this study was to examine the role of a common polymorphism on postoperative tachyarrhythmias in a large cohort of pediatric patients undergoing congenital heart surgery with cardiopulmonary bypass (CPB). Methods Subjects undergoing congenital heart surgery with CPB at our institution were consecutively enrolled from September 2007 to December 2012. In addition to DNA, perioperative clinical data were obtained from subjects. Results Postoperative tachyarrhythmias were documented in 45% of 886 enrollees and were associated with prolonged mechanical ventilation (P <.001) and intensive care unit length of stay (P <.001). ACE I/D was in Hardy-Weinberg equilibrium (19% I/I, 49% I/D, 32% D/D). I/D or D/D genotypes were independently associated with a 60% increase in odds of new tachyarrhythmia (odds ratio [OR] 1.6, 95% confidence interval [CI] 1.1-2.3, P =.02). Preoperative ACE inhibitor administration was independently associated with a 47% reduction in odds of postoperative tachyarrhythmia in the entire cohort (OR 0.53, 95% CI 0.32-0.88, P =.01), driven by a 5-fold reduction in tachyarrhythmias among I/I genotype patients (OR 0.19, 95% CI 0.04-0.88, P =.02). Conclusion The risk of tachyarrhythmias after congenital heart surgery is independently affected by the ACE I/D polymorphism. Preoperative ACE inhibition is associated with a lower risk of postoperative tachyarrhythmias, an antiarrhythmic effect that appears genotype dependent. An understanding of genotype variation may play an important role in the perioperative management of congenital heart surgery. AD - A.H. Smith, Division of Pediatric Critical Care Medicine, Department of Pediatrics, Vanderbilt University, Monroe Carell Jr. Children's Hospital at Vanderbilt, School of Medicine, 2200 Children's Way, Nashville, TN, United States AU - Smith, A. H. AU - Flack, E. C. AU - Borgman, K. Y. AU - Owen, J. P. AU - Fish, F. A. AU - Bichell, D. P. AU - Kannankeril, P. J. DB - Embase Medline DO - 10.1016/j.hrthm.2014.01.005 IS - 4 KW - beta adrenergic receptor blocking agent digoxin dipeptidyl carboxypeptidase dipeptidyl carboxypeptidase inhibitor DNA age article artificial ventilation atrioventricular junction arrhythmia body weight cardiopulmonary bypass cardiovascular risk child cohort analysis comparative study controlled study disease association disease course endocardial cushion defect enzyme inhibition enzyme polymorphism Fallot tetralogy female gene deletion genotype heart atrium septum defect heart surgery heart ventricle septum defect heart ventricle tachycardia human hypoplastic left heart syndrome incidence infant intensive care length of stay major clinical study male medical history newborn Norwood procedure perioperative period pharmacogenetics postoperative complication postoperative period preoperative period priority journal prophylaxis risk reduction scoring system supraventricular tachycardia tachycardia LA - English M3 - Article N1 - L606267577 2015-10-09 2015-10-14 PY - 2014 SN - 1556-3871 1547-5271 SP - 637-643 ST - A common angiotensin-converting enzyme polymorphism and preoperative angiotensin-converting enzyme inhibition modify risk of tachyarrhythmias after congenital heart surgery T2 - Heart Rhythm TI - A common angiotensin-converting enzyme polymorphism and preoperative angiotensin-converting enzyme inhibition modify risk of tachyarrhythmias after congenital heart surgery UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L606267577 http://dx.doi.org/10.1016/j.hrthm.2014.01.005 VL - 11 ID - 978 ER - TY - JOUR AB - In the present report, we discuss a patient who developed persistent hypoxemia after an attempt at Fontan completion failed. As a bail-out procedure, a left common carotid artery to left internal jugular vein shunt was constructed, after which the hypoxemia was relieved. © 2012 The Society of Thoracic Surgeons. AD - S. Talwar, Cardiothoracic Centre, All India Institute of Medical Sciences, Department of Cardiothoracic and Vascular Surgery, Ansari Nagar, New Delhi 110029, India AU - Garg, P. AU - Talwar, S. AU - Rajashekar, P. AU - Kothari, S. S. AU - Gulati, G. S. AU - Airan, B. DB - Embase Medline DO - 10.1016/j.athoracsur.2012.01.050 IS - 3 KW - anticonvulsive agent adolescent agitation angiography arteriovenous fistula arteriovenous shunt artery catheterization article cardiopulmonary bypass case report cavopulmonary connection common carotid artery cyanosis dextrocardia disorientation Doppler echocardiography dyspnea echocardiography femoral artery focal epilepsy Fontan procedure Glenn shunt heart catheterization heart ventricle septum defect human hypoxemia internal jugular vein lung angiography male priority journal pulmonary valve stenosis sternotomy LA - English M3 - Article N1 - L365483696 2012-08-29 2012-08-31 PY - 2012 SN - 0003-4975 1552-6259 SP - 998-1001 ST - Common carotid artery to internal jugular vein shunt for managing hypoxemia after a cavopulmonary shunt T2 - Annals of Thoracic Surgery TI - Common carotid artery to internal jugular vein shunt for managing hypoxemia after a cavopulmonary shunt UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L365483696 http://dx.doi.org/10.1016/j.athoracsur.2012.01.050 VL - 94 ID - 1041 ER - TY - JOUR AB - Autism Spectrum Disorders (ASD) is a topic receiving great attention from researchers and clinicians in the field. However, many of these studies focus on children or adults, with research on infants and toddlers evincing ASD being virtually non-existent. Even more scant is information pertaining to the assessment of symptoms of comorbid psychopathology in this young population. Nonetheless, it is essential to identify comorbid conditions in addition to the symptoms associated with the core features of ASD. Building on the effectiveness of early intervention with children with ASD, comprehensive evaluations and individualized treatment goals are necessary and may enhance treatment efficacy. The Baby and Infant Screen for Children with aUtIsm Traits-Part 2 (BISCUIT-Part 2) is a new assessment, specifically designed to examine symptoms of psychopathology in infants and toddlers with ASD. The purpose of this study was twofold. First, the factor structure of the BISCUIT-Part 2 was established. Second, group differences in the endorsement of symptoms of psychopathology were examined between infants and toddlers with and without ASD. © 2011 Elsevier Ltd. AD - J. L. Matson, Department of Psychology, Louisiana State University, Baton Rouge, LA 70803, United States AU - Matson, J. L. AU - Boisjoli, J. A. AU - Hess, J. A. AU - Wilkins, J. DB - Embase DO - 10.1016/j.rasd.2010.06.005 IS - 1 KW - article asthma cerebral palsy child comorbidity controlled study corpus callosum agenesis diabetes mellitus Down syndrome epilepsy female fetal alcohol syndrome human hydrocephalus hypoplastic left heart syndrome autism major clinical study male meningitis mental disease preschool child priority journal screening seizure spinal dysraphism symptomatology traumatic brain injury Usher syndrome LA - English M3 - Article N1 - L50981952 2010-07-13 2010-10-20 PY - 2011 SN - 1750-9467 SP - 426-432 ST - Comorbid psychopathology factor structure on the Baby and Infant Screen for Children with aUtIsm Traits-Part 2 (BISCUIT-Part 2) T2 - Research in Autism Spectrum Disorders TI - Comorbid psychopathology factor structure on the Baby and Infant Screen for Children with aUtIsm Traits-Part 2 (BISCUIT-Part 2) UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50981952 http://dx.doi.org/10.1016/j.rasd.2010.06.005 VL - 5 ID - 1124 ER - TY - JOUR AB - Objective: We sought to determine whether the presence or absence of aortic obstruction impacts cerebrovascular resistance in fetuses with single-ventricle (SV) congenital heart disease (CHD). Methods: Pulsatility indices (PIs) were recorded for the middle cerebral artery (MCA) and the umbilical artery (UA) from 18 to 40 weeks' gestation in 59 fetuses (163 examinations) with SV-CHD with unobstructed aortic flow, yet decreased pulmonary flow, in 72 fetuses (170 examinations) with obstructed aortic flow and hypoplastic left heart syndrome (HLHS) and in 92 normal fetuses (92 examinations). The cerebral-to-placental resistance (CPR) was calculated as the MCA-PI/UA-PI. Z-scores were generated for the MCA-PI and the UA-PI in order to make comparisons independent of gestational age. Statistical analyses were performed using one-way ANOVA with post-hoc testing. Trends in these variables over the course of gestation were assessed using linear regression and univariate ANOVA. Results: The MCA-PI and the CPR were significantly lower in SV fetuses with aortic obstruction compared with SV fetuses with pulmonary obstruction and with normal fetuses. Moreover, the MCA-PI decreased significantly for SV fetuses with aortic obstruction over the course of gestation. In contrast, the MCA-PI was higher over the course of gestation in SV fetuses with pulmonary obstruction compared with normal fetuses. Conclusion: In fetuses with SV-CHD, cerebrovascular resistance varies substantially between fetuses with and without aortic obstruction. Compared with normal fetuses, cerebrovascular resistance is decreased in SV fetuses with aortic obstruction, yet increased in SV fetuses with pulmonary obstruction. In fetuses with SV physiology, inherent differences in cerebral blood flow may underlie postnatal neurodevelopmental outcomes. AD - The Fetal Heart Program at Cardiac Center, Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA The Fetal Heart Program at the Cardiac Center at the Children's Hospital of Philadelphia, Philadelphia, PA, USA; The University of Pennsylvania School of Medicine, Department of Pediatrics, Division of Cardiology, Philadelphia, PA, USA. szwast@email.chop.edu. AN - 104355958. Language: English. Entry Date: 20130329. Revision Date: 20170411. Publication Type: journal article AU - Szwast, A. AU - Tian, Z. AU - McCann, M. AU - Soffer, D. AU - Rychik, J. AU - Szwast, A. AU - Tian, Z. AU - McCann, M. AU - Soffer, D. AU - Rychik, J. DB - ccm DO - 10.1002/uog.11147 DP - EBSCOhost IS - 1 KW - Cerebrovascular Circulation Developmental Disabilities -- Physiopathology Heart Defects, Congenital -- Physiopathology Cerebral Arteries -- Physiopathology Physics Umbilical Arteries -- Physiopathology Analysis of Variance Blood Flow Velocity Developmental Disabilities -- Etiology Developmental Disabilities -- Ultrasonography Female Gestational Age Heart Defects, Congenital -- Complications Heart Defects, Congenital -- Embryology Heart Defects, Congenital -- Ultrasonography Human Infant Infant, Newborn Male Cerebral Arteries -- Embryology Cerebral Arteries -- Ultrasonography Pregnancy Ultrasonography, Prenatal Umbilical Arteries -- Embryology Umbilical Arteries -- Ultrasonography Vascular Resistance N1 - research. Journal Subset: Biomedical; Europe; Peer Reviewed; UK & Ireland. Special Interest: Obstetric Care. NLM UID: 9108340. PMID: NLM22407644. PY - 2012 SN - 0960-7692 SP - 62-67 ST - Comparative analysis of cerebrovascular resistance in fetuses with single-ventricle congenital heart disease T2 - Ultrasound in Obstetrics & Gynecology TI - Comparative analysis of cerebrovascular resistance in fetuses with single-ventricle congenital heart disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104355958&site=ehost-live&scope=site VL - 40 ID - 1561 ER - TY - JOUR AB - Purpose Echocardiographic evaluation of systolic function in patients with single right ventricles (SRV) is important but remains challenging. Minimal data exist correlating echocardiographic indices with catheterization data in this population. The goal of this study was to evaluate which echocardiographic measurement correlated best with dP/dt (max) obtained by cardiac catheterization in SRV patients. Methods Patients with SRV physiology who underwent simultaneous echocardiography and cardiac catheterization were evaluated. Echocardiographic data included fractional area change % (FAC), displacement, TDI s' wave, myocardial performance index (MPI), global systolic strain, and global SR s wave. Maximum positive rate of ventricular pressure change measured as dP/dt (max) was obtained from the cardiac catheterization report. Correlations of echocardiographic and catheterization variables were examined using the Pearson correlation. Results Twenty-seven SRV patients were studied. Median age at the time of the catheterization was 11.4 months (range 0 - 132 months). dP/dt (max) values ranged from 337-1860 mmHg/s with a median of 994 mmHg/s. Mean FAC was 27.15 ± 7.13%, displacement was 7.35 ± 2.88 mm, TDI s' was 4.98 ±1.93 cm/sec, MPI was 0.41 ± 0.17, global strain was -14.85 ± 4.32%, and global SR s wave was -1.03 ± 0.34 sec-1. There were no signifi cant correlations between dP/dt (max) and any of the echocardiographic measurements of systolic function in SRV patients. Conclusion In patients with SRV physiology, catheterization-derived dP/dt (max) did not correlate with echocardiographic measurements of systolic function. Larger studies are needed to determine which non-invasive parameter best describes systolic function in patients with SRV. AD - C. L. Cua, Heart Center, Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH 43205, United States AU - Husain, N. AU - Gokhale, J. AU - Nicholson, L. AU - Perkins, A. AU - Cooper, A. L. AU - Cheatham, J. P. AU - Holzer, R. J. AU - Cua, C. L. DB - Embase Medline DO - 10.2143/AC.69.3.3027831 IS - 3 KW - article atrioventricular septal defect cardiovascular parameters clinical article cross-sectional study diastolic blood pressure displacement behavior echocardiography female fractional area change Glenn shunt global systolic strain heart catheterization heart disease heart right ventricle double outlet heart single right ventricle heart ventricle pressure human hypoplastic left heart syndrome infant male myocardial performance index post fontan procedure prospective study status post hybrid procedure systolic blood pressure systolic function tissue doppler indices Sensis XP Vivid LA - English M3 - Article N1 - L373357080 2014-07-01 2014-07-11 PY - 2014 SN - 0373-7934 0001-5385 SP - 281-288 ST - Comparing echocardiographic assessment of systolic function with catheterization data in patients with single right ventricles T2 - Acta Cardiologica TI - Comparing echocardiographic assessment of systolic function with catheterization data in patients with single right ventricles UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L373357080 http://dx.doi.org/10.2143/AC.69.3.3027831 VL - 69 ID - 932 ER - TY - JOUR AB - BACKGROUND: There are several potential physiological differences between the atriopulmonary (AP) and the total cavopulmonary connection (TCPC) Fontan circulations. Studies suggest that the TCPC reduces energy loss due to turbulence and may have more dependence on respiratory movement for pulmonary blood flow. We compared cardiopulmonary physiology during rest and exercise in patients who had undergone the AP Fontan procedure with those who had undergone the TCPC Fontan procedure. METHODS AND RESULTS: Forty-three children were studied more than 6 months after undergoing a Fontan procedure (23 AP and 20 TCPC); 106 healthy children were also studied as a control group. Measurements of effective pulmonary blood flow, stroke volume, arteriovenous oxygen difference, minute ventilation, heart rate, and oxygen and carbon dioxide consumption were made with an Innovision quadrupole mass spectrometer. Data from the control group allowed calculation of z scores for the Fontan groups matched for age, sex, pubertal stage, and body surface area. Maximal exercise performance was equal in the two Fontan groups, but it was below normal. However, adaptation to exercise was different in the Fontan groups. After 9 minutes of exercise, pulmonary blood flow rose less in the AP group than in the TCPC group (P < .01), and the stroke volume in the AP group also tended to be lower (P = .057) and their arteriovenous oxygen difference was significantly greater (P < .01). Although minute ventilation per unit of carbon dioxide production was similar in the Fontan groups at this level of exercise, children in the TCPC group breathed faster by approximately 10 breaths per minute (P < .005). CONCLUSIONS: At submaximal exercise, children who had undergone the TCPC Fontan procedure had pulmonary hemodynamics superior to those of children who had undergone the AP procedure, largely because of respiratory adaptation that permitted blood to be "sucked" into the lungs. To achieve the same maximal exercise performance, children who had undergone the AP procedure had a superior metabolic adaptation to exercise stress. AD - Department of Paediatric Respiratory Medicine, Royal Brompton National Heart and Lung Hospital, London, U.K. AN - 7805240 AU - Rosenthal, M. AU - Bush, A. AU - Deanfield, J. AU - Redington, A. DA - Jan 15 DB - PubMed DO - 10.1161/01.cir.91.2.372 DP - NLM ET - 1995/01/15 IS - 2 KW - *Adaptation, Physiological Adolescent Child *Exercise/physiology Female *Fontan Procedure Heart/*physiology Hemodynamics Humans Lung/*physiology Male Oxygen Consumption Pulmonary Circulation Regional Blood Flow Respiration Stroke Volume LA - eng N1 - Rosenthal, M Bush, A Deanfield, J Redington, A Comparative Study Journal Article Research Support, Non-U.S. Gov't United States Circulation. 1995 Jan 15;91(2):372-8. doi: 10.1161/01.cir.91.2.372. PY - 1995 SN - 0009-7322 (Print) 0009-7322 SP - 372-8 ST - Comparison of cardiopulmonary adaptation during exercise in children after the atriopulmonary and total cavopulmonary connection Fontan procedures T2 - Circulation TI - Comparison of cardiopulmonary adaptation during exercise in children after the atriopulmonary and total cavopulmonary connection Fontan procedures VL - 91 ID - 380 ER - TY - JOUR AB - Background The last decade has seen a vast increase in the use of patient-reported outcomes. As patient-reported outcomes are used in order to capture patients' perspectives of their health and illness, it is a prerequisite for accurate patient-reported outcome evaluations to use representative samples. In order to evaluate representativeness, the present study focussed on the comparison between participants and non-participants in the Swedish branch of the international study APPROACH-IS (Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease-International Study), regarding demographic, clinical, and health status characteristics. Methods Eligible patients for APPROACH-IS were identified and selected from SWEDCON, the Swedish registry for congenital heart disease (CHD). Overall, 912 eligible patients were identified, of whom 471 participated, 398 did not participate, and 43 were either unreachable or declined to participate in APPROACH-IS. The participants and non-participants were compared in terms of statistical significance and effect sizes. Results Significant differences were observed between participants and non-participants for sex, age, primary diagnosis, number of cardiac operations, and fatigue; however, the effect sizes were in general small, except for the difference in primary diagnosis. No differences between the two groups were found in number of catheterisations, implanted device, the distribution of NYHA functional class, or health status and symptoms. Conclusions This study shows that participants and non-participants are relatively comparable groups, which confirms the representativeness of the participants. The Swedish data from APPROACH-IS can therefore be reliably generalised to the population of adults with CHD in Sweden. © Cambridge University Press 2016. AD - Institute of Medicine, Sahlgrenska Academy at University of Gothenburg, Gothenburg, S-41685, Sweden Centre for Person-Centred Care (GPCC), University of Gothenburg, Gothenburg, Sweden Department of Health Science, University West, Trollhättan, Sweden Department of Cardiology, Karolinska University Hospital, Stockholm, Sweden Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden Institute of Health and Caring Sciences, Sahlgrenska Academy at University of Gothenburg, Gothenburg, Sweden KU Leuven Department of Public Health and Primary Care, KU Leuven, University of Leuven, Leuven, Belgium Adult Congenital Heart Unit, Sahlgrenska University Hospital/Östra, Göteborg, Sweden AU - Berghammer, M. C. AU - Mattsson, E. AU - Johansson, B. AU - Moons, P. AU - Dellborg, M. DB - Scopus DO - 10.1017/S1047951116000676 IS - 3 KW - Adults comparison congenital heart defect multicentre study patient-reported outcome M3 - Article N1 - Cited By :8 Export Date: 15 June 2020 PY - 2017 SP - 427-434 ST - Comparison of participants and non-participants in patient-reported outcome surveys: The case of Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease-International Study T2 - Cardiology in the Young TI - Comparison of participants and non-participants in patient-reported outcome surveys: The case of Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease-International Study UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84966470677&doi=10.1017%2fS1047951116000676&partnerID=40&md5=b9ef8697e944275b4496e506d023fa93 VL - 27 ID - 1871 ER - TY - JOUR AB - Objectives We aimed to compare the procedural and mid-term performance of a specifically designed self-expanding stent with balloon-expandable stents in patients undergoing hybrid palliation for hypoplastic left heart syndrome and its variants. Background The lack of specifically designed stents has led to off-label use of coronary, biliary, or peripheral stents in the neonatal ductus arteriosus. Recently, a self-expanding stent, specifically designed for use in hypoplastic left heart syndrome, has become available. Methods We carried out a retrospective cohort comparison of 69 neonates who underwent hybrid ductal stenting with balloon-expandable and self-expanding stents from December, 2005 to July, 2014. Results In total, 43 balloon-expandable stents were implanted in 41 neonates and more recently 47 self-expanding stents in 28 neonates. In the balloon-expandable stents group, stent-related complications occurred in nine patients (22%), compared with one patient in the self-expanding stent group (4%). During follow-up, percutaneous re-intervention related to the ductal stent was performed in five patients (17%) in the balloon-expandable stent group and seven patients (28%) in self-expanding stents group. Conclusions Hybrid ductal stenting with self-expanding stents produced favourable results when compared with the results obtained with balloon-expandable stents. Immediate additional interventions and follow-up re-interventions were similar in both groups with complications more common in those with balloon-expandable stents. © Cambridge University Press 2016. AD - Department of Congenital Heart Disease, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, Westminster Bridge Rd, London, SE1 7EH, United Kingdom Department of Cardiology, Polish Mother's Memorial Hospital, Research Institute, Lodz, Poland AU - Goreczny, S. AU - Qureshi, S. A. AU - Rosenthal, E. AU - Krasemann, T. AU - Nassar, M. S. AU - Anderson, D. R. AU - Morgan, G. J. DB - Scopus DO - 10.1017/S1047951116001347 IS - 5 KW - ductal stenting hybrid procedure Hypoplastic left heart syndrome M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2017 SP - 837-845 ST - Comparison of self-expandable and balloon-expanding stents for hybrid ductal stenting in hypoplastic left heart complex T2 - Cardiology in the Young TI - Comparison of self-expandable and balloon-expanding stents for hybrid ductal stenting in hypoplastic left heart complex UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85020004209&doi=10.1017%2fS1047951116001347&partnerID=40&md5=7cd97175df41d8d3553299d9d5cfb53e VL - 27 ID - 1855 ER - TY - JOUR AB - OBJECTIVE: To evaluate the incidence of otherwise undiagnosed congenital heart disease (CHD) in a population of children born in a hospital with routine pulse oximetry (RPO) screening compared to children born at home. METHODS: We reviewed 15 years of births at 2 hospitals for incidence of undiagnosed CHD with RPO. The Health Department reviewed the same data for out of hospital births. RESULTS: A total of 50,545 hospital births were screened and 1,274 children were born outside the hospital. There were 28 hospital-born babies diagnosed with cyanotic CHD prior to nursery discharge. Only one of these babies would not have been diagnosed without RPO. Three children were missed and there were 3 false positives. Sensitivity and positive predictive value of RPO was 25%, specificity and negative predictive value of RPO exceed 99%. The incidence of CHD requiring RPO diagnosis was roughly one birth per 50,000. Two children born at home with undiagnosed CHD were missed. One of these children presented with neonatal demise. CONCLUSION: RPO screening is still valuable in diagnosing CHD only diagnosable with RPO. However, the incidence of CHD requiring RPO to diagnose is similar to other congenital diseases which are not mandated national screening tests. In our limited experience a patient is roughly 25 times more likely to have undiagnosed CHD if they are born outside of a hospital. AD - J.D. Reich, Watson Clinic LLP, 1600 Lakeland Hills Blvd., Lakeland, FL, United States AU - Reich, J. D. AU - Haight, D. AU - Reich, Z. S. DB - Embase Medline DO - 10.3233/NPM-1651 IS - 1 KW - article child comparative study congenital heart disease controlled study cyanotic heart disease diagnostic test accuracy study Ebstein anomaly Fallot tetralogy false positive result great vessels transposition heart single ventricle home delivery hospital care human incidence major clinical study newborn screening nursery predictive value priority journal pulmonary valve atresia pulse oximetry routine pulse oximetry sensitivity and specificity trisomy 21 Williams Beuren syndrome LA - English M3 - Article N1 - L615419101 2017-04-21 2017-04-25 PY - 2017 SN - 1878-4429 1934-5798 SP - 71-77 ST - A comparison of the incidence of undiagnosed congenital heart disease in hospital born and home born children T2 - Journal of Neonatal-Perinatal Medicine TI - A comparison of the incidence of undiagnosed congenital heart disease in hospital born and home born children UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L615419101 http://dx.doi.org/10.3233/NPM-1651 VL - 10 ID - 781 ER - TY - JOUR AB - OBJECTIVE: Major associated cardiac anomalies are known to increase the risk of repair of complete atrioventricular septal defects (CAVSDs). The purpose of this study was to examine the effects of such anomalies on the current surgical management of CAVSDs and their influence on outcome following repair. METHODS: We performed a retrospective review of a 100 consecutive non-isomeric patients undergoing repair of CAVSD at our institution, between January 1989 and December 1994; patients with partial or intermediate defects were excluded. Complete atrioventricular septal defect patients with other major cardiac abnormalities (complex) were then compared to those with isolated CAVSDs. RESULTS: There were 15 patients (15%) with associated anomalies; 3 had tetralogy of Fallot, 1 patient had pulmonary atresia, 6 had hypoplastic left or right ventricle, 1 had tetralogy of Fallot and hyperplastic right ventricle, 2 patients had double outlet right ventricle, 1 had hypoplastic aortic arch and 1 patient had aortic coarctation. The median age at operation was similar for both groups (4.2 months), while the median weight was not significantly different for isolated CAVSDs compared to complex (4.2 months vs 3.4 months, P = 0.89), but there was a higher incidence of trisomy 21 (70/85, 82% vs 8/15, 53.3%, P = 0.01). Two of the 85 isolated CAVSD patients (2.3%) had undergone palliative pulmonary artery banding, while 5 of the 15 complex patients (33.3%) had either banding or Blalock-Taussig shunts performed. The technique of CAVSD repair was identical in each group. All complex patients had standard repair of their associated anomalies. Hospital mortality was higher in the complex group (3/15, 20% vs 2/85, 2.3%, P = 0.004); all early deaths in the complex group occurred in patients with a hypoplastic ventricle. Reoperation for left atrioventricular valve regurgitation was required in six isolated CAVSD patients (7.1%) and in one complex (6.6%). CONCLUSIONS: In the absence of significant ventricular hypoplasia, the early results of surgical repair in patients with CAVSDs and associated cardiovascular anomalies are similar to those achieved in patients with isolated CAVSD. AD - J.M. Redmond, Heart Unit, Birmingham Children's Hospital, Ladywood, UK. AU - Redmond, J. M. AU - Silove, E. D. AU - De Giovanni, J. V. AU - Wright, J. G. AU - Sreeram, N. AU - Brawn, W. J. AU - Sethia, B. DB - Medline IS - 11 KW - aortic coarctation article child congenital malformation Down syndrome endocardial cushion defect Fallot tetralogy female heart right ventricle double outlet human hypoplastic left heart syndrome infant male mortality preschool child pulmonary artery reoperation retrospective study survival rate procedures thoracic aorta treatment outcome LA - English M3 - Article N1 - L127207570 1997-03-18 PY - 1996 SN - 1010-7940 SP - 991-995 ST - Complete atrioventricular septal defects: the influence of associated cardiac anomalies on surgical management and outcome T2 - European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery TI - Complete atrioventricular septal defects: the influence of associated cardiac anomalies on surgical management and outcome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L127207570 VL - 10 ID - 1360 ER - TY - JOUR AB - BACKGROUND: The high-flow nasal cannula (HFNC) system has been widely used for children in various clinical settings. However, the physiological and clinical impact of HFNC therapy on the pediatric patient with respiratory distress after cardiac surgery has not been thoroughly investigated. MAIN BODY OF THE ABSTRACT: It seems logical to use HFNC as a primary therapy for post-extubation respiratory failure after congenital heart surgery, in which low cardiac output syndrome and upper airway obstruction are commonly encountered; the HFNC therapy alleviates the work of breathing and large negative swings of intrathoracic pressure, which in turn helps to decrease the systemic ventricular afterload. When applying HFNC to patients after congenital heart surgery, however, consideration must be given to its diverse effects on hemodynamics because of the complex respiratory and cardiac pathophysiology in these patients. The positive pressure generated by HFNC can exert different effects on pulmonary vascular resistance depending on the lung condition, while its impact on cardiac output may also differ depending on the cardiac physiology. The hemodynamic effects of HFNC may become even more complex in a patient with a single ventricle. To better assess its physiologic effects in patients after cardiac surgery, future studies could utilize various modalities including esophageal balloon catheters, electrical impedance tomography, and near-infrared spectroscopy. At the same time, studies should focus on specific types of cardiac pathophysiology or surgery when evaluating the effects of HFNC, since it may exert various effects, depending on the cardiac physiology or preoperative pulmonary hemodynamics. Lastly, the optimal flow rate at which the benefit of HFNC is maximized through favorable cardiopulmonary interactions should be determined in future studies. SHORT CONCLUSION: Further studies are needed to better understand the effect of HFNC in different cardiac and respiratory physiologies, given their complexity in pediatric patients after cardiac surgery. AD - Department of Intensive Care Medicine, Osaka Women's and Children's Hospital, 840 Murodo-cho, Izumi, Osaka 594-1101 Japan. AN - 28572979 AU - Inata, Y. AU - Takeuchi, M. C2 - PMC5450202 DB - PubMed DO - 10.1186/s40560-017-0227-y DP - NLM ET - 2017/06/03 KW - Cardiopulmonary interactions Congenital heart disease High-flow nasal cannula LA - eng N1 - 2052-0492 Inata, Yu Orcid: 0000-0002-2180-9517 Takeuchi, Muneyuki Editorial J Intensive Care. 2017 May 30;5:30. doi: 10.1186/s40560-017-0227-y. eCollection 2017. PY - 2017 SN - 2052-0492 (Print) 2052-0492 SP - 30 ST - Complex effects of high-flow nasal cannula therapy on hemodynamics in the pediatric patient after cardiac surgery T2 - J Intensive Care TI - Complex effects of high-flow nasal cannula therapy on hemodynamics in the pediatric patient after cardiac surgery VL - 5 ID - 244 ER - TY - JOUR AB - Enormous progress has been made in the last decade toward decreasing the risk of the Fontan operation and optimizing early and intermediate outcomes. Heterotaxy syndrome patients, with their extensive constellation of anatomic abnormalities, constitute a challenging group to manage surgically. Palliative procedures early in life are directed at creation of a satisfactory balance between pulmonary and systemic blood flow and at the same time assurance of unobstructed pulmonary venous return. For heterotaxy patients who survive beyond the newborn period, and are considered to have nonseptatable hearts, early reduction of the volume load of the functional single ventricle is extremely important. Early conversion from parallel pulmonary and systemic circulations to a superior cavopulmonary anastomosis (bidirectional Glenn or hemi-Fontan) is the preferred strategy. Traditionally, those patients with heterotaxy who survive initial surgical management have been considered a very high-risk population with respect to an eventual Fontan procedure. In heterotaxy patients it is particularly important to understand the unique and variable anatomy of the sinus node and conduction system, the potential for occult pulmonary venous obstruction, the tendency for development of atrioventricular valve regurgitation in volume loaded ventricles, and the potential for recurrent or persistent cyanosis because of intrahepatic shunting or pulmonary arteriovenous malformations. Copyright © 2002 by W.B. Saunders Company. AD - M.L. Jacobs, Section of Cardiothoracic Surgery, St. Christopher's Hosp. for Children, Erie Ave. at Front St., Philadelphia, PA 19134, United States AU - Jacobs, M. L. DB - Embase Medline DO - 10.1053/pcsu.2002.31500 KW - article blood volume cyanosis Fontan procedure heart muscle conduction system heart single ventricle heterotaxy syndrome high risk patient human pulmonary arteriovenous fistula lung blood flow lung circulation newborn newborn period outcomes research palliative therapy pulmonary vein pulmonary vein obstruction risk reduction sinus node surgical patient surgical risk surgical technique survival time systemic circulation venous return LA - English M3 - Article N1 - L38202148 2004-02-25 PY - 2002 SN - 1092-9126 SP - 25-35 ST - Complications associated with heterotaxy syndrome in fontan patients T2 - Pediatric Cardiac Surgery Annual TI - Complications associated with heterotaxy syndrome in fontan patients UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L38202148 http://dx.doi.org/10.1053/pcsu.2002.31500 VL - 5 ID - 1311 ER - TY - JOUR AB - Stenting of vascular, extracardiac or lately intracardiac stenosis has become an established interventional treatment for a variety of problems in congenital or acquired heart disease. Most stent procedures are completed successfully and the long-term outcome is favorable in the majority of cases. Stent collapse or deformation is a well recognized entity in peripheral stents and can be attributed to insufficient radial force; it can also be attributed to excessive external forces, like deformation of stents in the right ventricular outflow tract, where external compression is combined with continuous movement caused by the beating heart. The protection of the thoracic cage may prove to be insufficient in extraordinary circumstances, such as chest compression in trauma or cardiopulmonary resuscitation (CPR). In this case series, we describe three patients in whom large endovascular stents were placed to treat significant stenosis of the aorta, the aortic arch or the venous system of the inferior vena cava close to the atrium. In all patients, CPR was necessary during their clinical course for various reasons; after adequate CPR, including appropriate chest compression all patients survived the initial resuscitation phase. Clinical, echocardiographic as well as radiologic re-evaluation after resuscitation revealed significant stent distortion, compression, displacement or additional vascular injury. The possibility of mechanical deformation of large endovascular stents needs to be considered and recognized when performing CPR; if CPR is successful, immediate re-evaluation of the implanted stents-if possible by biplane fluoroscopy-seems mandatory. © 2014 Springer-Verlag. AD - N.A. Haas, Department of Congenital Heart Defects, Heart and Diabetes Centre North Rhine Westphalia, Ruhr University Bochum, Georgstrasse 11, 32545 Bad Oeynhausen, Germany AU - Haas, N. A. AU - Happel, C. M. AU - Jategaonkar, S. AU - Moysich, A. AU - Hanslik, A. AU - Kececioglu, D. AU - Sandica, E. AU - Laser, K. T. DB - Embase Medline DO - 10.1007/s00392-014-0706-2 IS - 9 KW - adult angiography aortic stenosis aortic arch surgery article atrioventricular septal defect balloon dilatation bare metal stent case report cavopulmonary connection congenital heart malformation echocardiography endotracheal tube female fluoroscopy great vessels transposition heart catheterization human hypoplastic left heart syndrome infant inferior cava vein male medical device complication arterial switch operation Norwood procedure peripheral artery stent postoperative care restenosis resuscitation stent compression stent displacement stent distortion supraventricular tachycardia thorax radiography tracheobronchomalacia vascular stent Palmaz Genesis stent Valeo vascular stent LA - English M3 - Article N1 - L53089037 2014-04-11 2014-08-30 PY - 2014 SN - 1861-0692 1861-0684 SP - 719-725 ST - Compression, distortion and dislodgement of large caliber stents in congenital heart defects caused by cardiopulmonary resuscitation: A case series and review of the literature T2 - Clinical Research in Cardiology TI - Compression, distortion and dislodgement of large caliber stents in congenital heart defects caused by cardiopulmonary resuscitation: A case series and review of the literature UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L53089037 http://dx.doi.org/10.1007/s00392-014-0706-2 VL - 103 ID - 955 ER - TY - JOUR AB - Background Interactions between genetic and environmental factors, including modifiable maternal nutrition and lifestyle, play a significant role in the pathogenesis of most congenital heart defects (CHD). The aim of this study was to investigate associations between periconceptional maternal vitamin D status and the prevalence of CHD in offspring. Methods A case-control study was performed in 345 mothers of a child with CHD and 432 mothers of a child without CHD from four tertiary hospitals in the Netherlands between 2003 and 2005. Approximately 15 months after pregnancy mothers filled out questionnaires regarding general characteristics and periconceptional lifestyle. Maternal blood was obtained to determine serum 25-hydroxyvitamin D and lipid concentrations. The 25-hydroxyvitamin D concentration was stratified into a deficient < 50 nmol/l, moderate 50–75 nmol/l and adequate > 75 nmol/l status. Logistic regression was performed to study associations between vitamin D status and CHD risk, adjusted for maternal age, body mass index, ethnicity, smoking and total cholesterol concentration. Results Case mothers less often had an adequate vitamin D status compared with controls (27% vs. 38%; p = 0.002). The use of multivitamin supplements, ethnicity, season and body mass index were associated with vitamin D concentrations. A moderate (odds ratio 1.58, [95%CI 1.08, 2.32]) and deficient (odds ratio 2.15, [95%CI 1.44–3.19]) vitamin D status were associated with CHD in offspring. Conclusion A compromised maternal vitamin D status is associated with an approximately two-fold increased prevalence of CHD in offspring. Therefore, improvement of the periconceptional maternal vitamin D status is recommended. AD - R.P.M. Steegers-Theunissen, Departments of Obstetrics and Gynaecology and Pediatrics, Division of Neonatology, Erasmus MC, University Medical Centre, PO Box 2040, Rotterdam, Netherlands AU - Koster, M. P. H. AU - van Duijn, L. AU - Krul-Poel, Y. H. M. AU - Laven, J. S. AU - Helbing, W. A. AU - Simsek, S. AU - Steegers-Theunissen, R. P. M. DB - Embase Medline DO - 10.1016/j.earlhumdev.2017.12.011 KW - 25 hydroxyvitamin D apolipoprotein B cholesterol high density lipoprotein high density lipoprotein cholesterol low density lipoprotein low density lipoprotein cholesterol triacylglycerol vitamin D adult aortic coarctation aortic valve stenosis article association atrioventricular septal defect birth weight body mass case control study cholesterol blood level congenital heart malformation controlled study ethnicity Fallot tetralogy female gestational age great vessels transposition heart ventricle septum defect human hypoplastic left heart syndrome major clinical study maternal age maternal blood pathogenesis perimembranous ventricular septal defect pulmonary valve stenosis questionnaire smoking triacylglycerol blood level LA - English M3 - Article N1 - L619933927 2018-01-02 2018-11-06 PY - 2018 SN - 1872-6232 0378-3782 SP - 50-56 ST - A compromised maternal vitamin D status is associated with congenital heart defects in offspring T2 - Early Human Development TI - A compromised maternal vitamin D status is associated with congenital heart defects in offspring UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L619933927 http://dx.doi.org/10.1016/j.earlhumdev.2017.12.011 VL - 117 ID - 707 ER - TY - JOUR AB - Objectives: Adult patients who have undergone the Fontan procedure are highly vulnerable to gradual, progressive circulatory failure, and options to reverse this situation are few. A cavopulmonary assist device could decongest the venous and lymphatic systems, overcome elevated pulmonary vascular resistance, increase cardiac output, and support some of these patients to heart transplant. This study characterizes the performance and challenges of a novel multilumen cannula coupled to an external blood pump proposed as a potential Fontan cavopulmonary assist strategy. Methods: Computational fluid dynamic simulations were conducted for 3 extracardiac Fontan geometries consisting of 1 idealized model and 2 patient-specific models. A range of physiologic flow rates and pump assist levels were simulated to calculate the pressure gain provided by the multilumen cannula. Hemolysis index was estimated for the idealized model with Lagrangian particle tracking and 2 variations of the power-law. Wall shear stresses were also examined. Results: Pressure gains up to 4 and 9 mm Hg were achieved for the idealized and patient-specific models, respectively. Pressure gains increased with both higher cardiac output and larger pump intake through the external pump. Flow-weighted hemolysis show hemoglobin damage levels to be several times lower than the 2% threshold at the highest pump intake flow cases. Wall shear stress predictions depict elevated areas in the pulmonary vessels and regions of the cannula device. Conclusions: The cannula tested in this study shows promise as a percutaneous option to bridge support in some patients with a failing extracardiac Fontan. Limitations identified will be addressed in future design iterations and in ongoing experimental tests. AD - M.G. Doyle, 5 King's College Rd, Toronto, ON, Canada AU - Lin, W. C. P. AU - Doyle, M. G. AU - Roche, S. L. AU - Honjo, O. AU - Forbes, T. L. AU - Amon, C. H. DB - Embase Medline DO - 10.1016/j.jtcvs.2019.03.008 IS - 5 KW - blood pump cannula cavopulmonary assist device heart assist device mercury adult article assisted circulation cardiovascular magnetic resonance cavopulmonary connection circulation computational fluid dynamics computer simulation female flow rate Fontan procedure heart graft heart output heart ventricle enddiastolic pressure hemolysis human inferior cava vein left pulmonary artery lung blood vessel lung vascular resistance male priority journal right pulmonary artery shear stress superior cava vein young adult LA - English M3 - Article N1 - L2001832792 2019-05-14 2019-10-18 PY - 2019 SN - 1097-685X 0022-5223 SP - 1424-1433.e5 ST - Computational fluid dynamic simulations of a cavopulmonary assist device for failing Fontan circulation T2 - Journal of Thoracic and Cardiovascular Surgery TI - Computational fluid dynamic simulations of a cavopulmonary assist device for failing Fontan circulation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2001832792 http://dx.doi.org/10.1016/j.jtcvs.2019.03.008 VL - 158 ID - 587 ER - TY - JOUR AB - OBJECTIVE: Exercise intolerance is common in total cavopulmonary connection (TCPC) patients. It has been suggested that power loss (Ploss) inside the TCPC plays a role in reduced exercise performance. Our objective is to establish the role of Ploss inside the TCPC during increased flow, simulating exercise in a patient-specific way. METHODS: Cardiac MRI (CMR) was used to obtain flow rates from the caval veins during rest and increased flow, simulating exercise with dobutamine. A 3D reconstruction of the TCPC was created using CMR data. Computational fluid dynamics (CFD) simulations were performed to calculate Ploss inside the TCPC structure for rest and stress conditions. To reflect the flow distribution during exercise, a condition where inferior caval vein (IVC) flow was increased twofold compared with rest was added. 29 TCPC patients (15 intra-atrial lateral tunnel (ILT) and 14 extracardiac conduit (ECC)) were included. RESULTS: Mean Ploss at rest was 1.36 ± 0.94 (ILT) and 3.20 ± 1.26 (ECC) mW/m(2) (p<0.001), 2.84 ±1.95 (ILT) and 8.41 ± 3.77 (ECC) mW/m(2) (p<0.001) during dobutamine and 5.21 ± 3.50 (ILT) and 15.28 ± 8.30 (ECC) mW/m(2) (p=0.001) with twofold IVC flow. The correlation between cardiac index and Ploss was exponential (ILT: R(2)=0.811, p<0.001; ECC: R(2)=0.690, p<0.001). CONCLUSIONS: Ploss inside the TCPC structure is limited but increases with simulated exercise. This relates to the anatomy of TCPC and the surgical technique used. In all flow conditions, ILT patients have lower Ploss than ECC patients. We did not find a relationship between Ploss and exercise capacity. AD - Department of Paediatrics, Division of Cardiology, Erasmus Medical Centre-Sophia Children's Hospital, , Rotterdam, The Netherlands. AN - 24634021 AU - Bossers, S. S. AU - Cibis, M. AU - Gijsen, F. J. AU - Schokking, M. AU - Strengers, J. L. AU - Verhaart, R. F. AU - Moelker, A. AU - Wentzel, J. J. AU - Helbing, W. A. DA - May DB - PubMed DO - 10.1136/heartjnl-2013-304969 DP - NLM ET - 2014/03/19 IS - 9 KW - Blood Flow Velocity/*physiology Child Cross-Sectional Studies Electronic Data Processing/*methods Exercise Test/methods Exercise Tolerance/*physiology Female Follow-Up Studies Fontan Procedure/*methods/mortality Heart Defects, Congenital/mortality/physiopathology/*surgery Humans Imaging, Three-Dimensional Magnetic Resonance Imaging, Cine Male Netherlands/epidemiology Survival Rate/trends Treatment Outcome Venae Cavae/*physiopathology Computational Fluid Dynamics Congenital Fontan Procedure Single Ventricle Total Cavopulmonary Connection LA - eng N1 - 1468-201x Bossers, Sjoerd S M Cibis, Merih Gijsen, Frank J Schokking, Michiel Strengers, Jan L M Verhaart, René F Moelker, Adriaan Wentzel, Jolanda J Helbing, Willem A Evaluation Study Journal Article Multicenter Study Research Support, Non-U.S. Gov't England Heart. 2014 May;100(9):696-701. doi: 10.1136/heartjnl-2013-304969. Epub 2014 Mar 14. PY - 2014 SN - 1355-6037 SP - 696-701 ST - Computational fluid dynamics in Fontan patients to evaluate power loss during simulated exercise T2 - Heart TI - Computational fluid dynamics in Fontan patients to evaluate power loss during simulated exercise VL - 100 ID - 440 ER - TY - JOUR AB - Hypoplastic left heart syndrome (HLHS) is a congenital heart disease which should be treated at neonate. Even now, its operation is one of the greatest challenges. However, currently there are no quantitative standards to evaluate and predict the outcome of the therapy. In this study, computational fluid dynamics (CFD) was used to estimate the performance of first stage HLHS surgery, the Norwood operation. An image data transfer system was developed to convert clinical images into three-dimensional geometry. To confirm software applicability, a validation process was carried out to eliminate any influence of numerical procedures. The velocities derived from echocardiography measurements were used as boundary conditions, and pressure waves measured by a cardiac catheter simultaneous with an electrocardiogram (ECG) were employed to validate the results of CFD simulation. Calculated results were congruent with the in vivo measurement results. The blood flow circulations were successfully simulated and the distribution of blood flow in each vessel was estimated. Time-varying energy losses (EL), local pressure and wall shear stress (WSS) were analyzed to estimate clinical treatment. The results indicated that pulsatile simulation is essential in quantitative evaluation. Computational hemodynamics may be applied in the surgical optimization for the treatment of HLHS. AD - Y. Qian, Center for Advanced Biomedical Science, TWIns, Waseda University, Tokyo, Japan. AU - Qian, Y. AU - Liu, J. L. AU - Itatani, K. AU - Miyaji, K. AU - Umezu, M. DB - Medline IS - 7 KW - article cardiovascular surgery software computer simulation congenital heart malformation female heart disease hemodynamics human hypoplastic left heart syndrome mechanical stress newborn physiology LA - English M3 - Article N1 - L359491876 2010-09-15 PY - 2010 SN - 1521-6047 SP - 2302-2313 ST - Computational hemodynamic analysis in congenital heart disease: simulation of the Norwood procedure T2 - Annals of biomedical engineering TI - Computational hemodynamic analysis in congenital heart disease: simulation of the Norwood procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L359491876 VL - 38 ID - 1140 ER - TY - JOUR AB - Children born with anatomic or functional “single ventricle” must progress through two or more major operations to sustain life. This management sequence culminates in the total cavopulmonary connection, or “Fontan” operation. A consequence of the “Fontan circulation”, however, is elevated central venous pressure and inadequate ventricular preload, which contribute to continued morbidity. We propose a solution to these problems by increasing pulmonary blood flow using an “injection jet” (IJS) in which the source of blood flow and energy is the ventricle itself. The IJS has the unique property of lowering venous pressure while enhancing pulmonary blood flow and ventricular preload. We report preliminary results of an analysis of this circulation using a tightly-coupled, multi-scale computational fluid dynamics model. Our calculations show that, constraining the excess volume load to the ventricle at 50% (pulmonary to systemic flow ratio of 1.5), an optimally configured IJS can lower venous pressure by 3 mmHg while increasing systemic oxygen delivery. Even this small decrease in venous pressure may have substantial clinical impact on the Fontan patient. These findings support the potential for a straightforward surgical modification to decrease venous pressure, and perhaps improve clinical outcome in selected patients. © 2018, Biomedical Engineering Society. AD - Department of Mechanical and Aerospace Engineering, University of Central Florida, 4000 Central Florida Blvd, Orlando, FL 32816, United States College of Medicine, University of Central Florida, 4000 Central Florida Blvd, Orlando, FL, United States Department of Mechanical Engineering, Embry-Riddle Aeronautical University, 600 S Clyde Morris Blvd, Daytona Beach, FL, United States The Perelman School of Medicine at The University of Pennsylvania, Philadelphia, PA, United States Division of Cardiology/Department of Pediatrics and the Department of Radiology, The Children’s Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA, United States Arnold Palmer Hospital for Children, 92 W Miller St, Orlando, FL, United States AU - Ni, M. W. AU - Prather, R. O. AU - Rodriguez, G. AU - Quinn, R. AU - Divo, E. AU - Fogel, M. AU - Kassab, A. J. AU - DeCampli, W. M. DB - Scopus DO - 10.1007/s13239-018-0342-5 IS - 2 KW - Fontan palliation Hypoplastic left heart syndrome Injection jet Multi-scale Pulmonary vascular resistance Total cavopulmonary connection M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 2018 SP - 202-216 ST - Computational Investigation of a Self-Powered Fontan Circulation T2 - Cardiovascular Engineering and Technology TI - Computational Investigation of a Self-Powered Fontan Circulation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85048107327&doi=10.1007%2fs13239-018-0342-5&partnerID=40&md5=fad876ec386e80fda84f08f1d9eda72e VL - 9 ID - 1800 ER - TY - JOUR AD - Department of Pathology (Cardiovascular and Thoracic Division), Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India Department of Pediatrics, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India AU - Vaideeswar, P. AU - Karande, S. DB - Scopus DO - 10.4103/jpgm.JPGM_671_19 IS - 2 M3 - Short Survey N1 - Export Date: 15 June 2020 PY - 2020 SP - 108-109 ST - Congenital absence of the left atrial appendage T2 - Journal of Postgraduate Medicine TI - Congenital absence of the left atrial appendage UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85083101043&doi=10.4103%2fjpgm.JPGM_671_19&partnerID=40&md5=37f8943b04c5e07f6dd00d86f39681cb VL - 66 ID - 1707 ER - TY - JOUR AB - Objective: Firstly, to assess the completeness of ascertainment in the National Congenital Anomaly System (NCAS), the basis for congenital anomaly surveillance in England and Wales, and its variation by defect, geographical area, and socioeconomic deprivation. Secondly, to assess the impact of the lack of data on pregnancies terminated because of fetal anomaly. Design: Comparison of the NCAS with four local congenital anomaly registers in England. Setting: Four regions in England covering some 109 000 annual births. Participants: Cases of congenital anomalies registered in the NCAS (live births and stillbirths) and independently registered in the four local registers (live births, stillbirths, fetal losses from 20 weeks' gestation, and pregnancies terminated after prenatal diagnosis of fetal anomaly). Main outcome measure: The ratio of cases identified by the national register to those in local registry files, calculated for different specified anomalies, for whole registry areas, and for hospital catchment areas within registry boundaries. Results: Ascertainment by the NCAS (compared with data from local registers, from which terminations of pregnancy were removed) was 40% (34% for chromosomal anomalies and 42% for non-chromosomal anomalies) and varied markedly by defect, by local register, and by hospital catchment area, but not by area deprivation. When terminations of pregnancy were included in the register data, ascertainment by NCAS was 27% (19% for chromosomal anomalies and 31% for non-chromosomal anomalies), and the geographical variation was of a similar magnitude. Conclusion: The surveillance of congenital anomalies in England is currently inadequate because ascertainment to the national register is low and non-uniform and because no data exist on termination of pregnancy resulting from prenatal diagnosis of fetal anomaly. AD - P.A. Boyd, National Perinatal Epidemiology Unit, University of Oxford, Oxford OX3 7LF, United Kingdom AU - Boyd, P. A. AU - Armstrong, B. AU - Dolk, H. AU - Botting, B. AU - Pattenden, S. AU - Abramsky, L. AU - Rankin, J. AU - Vrijheid, M. AU - Wellesley, D. DB - Embase Medline IS - 7481 KW - article chromosome aberration cleft lip cleft palate congenital heart malformation congenital malformation diaphragm hernia digestive system malformation Down syndrome Fallot tetralogy fetus fetus malformation gastroschisis health care system human hypoplastic left heart syndrome infant kidney polycystic disease limb malformation major clinical study national health service neural tube defect newborn pregnancy termination prenatal diagnosis priority journal register spinal dysraphism stillbirth umbilical hernia United Kingdom LA - English M3 - Article N1 - L40070475 2005-01-20 PY - 2005 SN - 0959-8146 SP - 27-29 ST - Congenital anomaly surveillance in England - Ascertainment deficiencies in the national system T2 - British Medical Journal TI - Congenital anomaly surveillance in England - Ascertainment deficiencies in the national system UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L40070475 VL - 330 ID - 1286 ER - TY - JOUR AB - Background-Neurodevelopmental disorders are the most common and distressful comorbidities associated with congenital heart defects (CHD). Head circumference at birth (HC), a proxy for prenatal cerebral growth, is an established risk factor for neurodevelopmental disorders. Methods and Results-In a nationwide cohort, we included all 924 422 liveborn Danish singletons, 1997 to 2011. CHD was present in 5519. The association between CHD and growth indices was analyzed by multivariable linear regression, adjusted for potential confounders. We report mean differences in gestational age-specific z scores in comparison with the general population. CHD was associated with lower HC z scores,-0.10 (95% confidence interval [CI],-0.13 to-0.08). Several CHD subtypes were associated with smaller HC, eg, hypoplastic left heart syndrome,-0.39 (95% CI,-0.58 to-0.21); common arterial trunk,-0.41 (95% CI,-0.74 to-0.09); and major ventricular septal defects,-0.25 (95% CI,-0.35 to-0.15). Other single-ventricle defects, transposition of the great arteries, tetralogy of Fallot, and anomalous pulmonary venous return, were also associated with smaller HC. Transposition of the great arteries was associated with smaller HC relative to birth weight,-0.26 (95% CI,-0.39 to-0.13). Major ventricular septal defects were associated with larger HC relative to birth weight. The results were consistent under various conditions, eg, when siblings of infants with CHD (n=5311) or infants with other major malformations (n=24 974) were used as the reference. Conclusions-Several subtypes of CHD were associated with smaller HC. The associations with major ventricular septal defects, common arterial trunk, and anomalous pulmonary venous return have not previously been described. Only infants with transposition of the great arteries had smaller HC relative to birth weight. AD - N.B. Matthiesen, Department of Pediatrics, Aarhus University Hospital, Palle Juul-Jensens Blvd 99, Aarhus N, Denmark AU - Matthiesen, N. B. AU - Henriksen, T. B. AU - Gaynor, J. W. AU - Agergaard, P. AU - Bach, C. C. AU - Hjortdal, V. E. AU - Østergaard, J. R. DB - Embase Medline DO - 10.1161/CIRCULATIONAHA.115.019089 IS - 6 KW - aortic arch anomaly aortic coarctation aortic stenosis arterial trunk article atrioventricular septal defect brain development cohort analysis congenital heart malformation disease association Fallot tetralogy gestational age great vessels transposition head circumference heart right ventricle double outlet heart single ventricle heart ventricle septum defect human hypoplastic left heart syndrome live birth lung vein drainage anomaly major clinical study mental disease newborn priority journal pulmonary valve atresia pulmonary valve stenosis risk assessment risk factor LA - English M3 - Article N1 - L607842418 2016-01-26 2016-02-26 PY - 2016 SN - 1524-4539 0009-7322 SP - 566-575 ST - Congenital Heart Defects and Indices of Fetal Cerebral Growth in a Nationwide Cohort of 924 422 Liveborn Infants T2 - Circulation TI - Congenital Heart Defects and Indices of Fetal Cerebral Growth in a Nationwide Cohort of 924 422 Liveborn Infants UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L607842418 http://dx.doi.org/10.1161/CIRCULATIONAHA.115.019089 VL - 133 ID - 823 ER - TY - JOUR AB - Background- This study determines the prevalence of Congenital Heart Defects (CHD), diagnosed prenatally or in infancy, and fetal and perinatal mortality associated with CHD in Europe. Methods and Results- Data were extracted from the European Surveillance of Congenital Anomalies central database for 29 population-based congenital anomaly registries in 16 European countries covering 3.3 million births during the period 2000 to 2005. CHD cases (n=26 598) comprised live births, fetal deaths from 20 weeks gestation, and terminations of pregnancy for fetal anomaly (TOPFA). The average total prevalence of CHD was 8.0 per 1000 births, and live birth prevalence was 7.2 per 1000 births, varying between countries. The total prevalence of nonchromosomal CHD was 7.0 per 1000 births, of which 3.6% were perinatal deaths, 20% prenatally diagnosed, and 5.6% TOPFA. Severe nonchromosomal CHD (ie, excluding ventricular septal defects, atrial septal defects, and pulmonary valve stenosis) occurred in 2.0 per 1000 births, of which 8.1% were perinatal deaths, 40% were prenatally diagnosed, and 14% were TOPFA (TOPFA range between countries 0% to 32%). Live-born CHD associated with Down syndrome occurred in 0.5 per 1000 births, with >4-fold variation between countries. Conclusion- Annually in the European Union, we estimate 36 000 children are live born with CHD and 3000 who are diagnosed with CHD die as a TOFPA, late fetal death, or early neonatal death. Investing in primary prevention and pathogenetic research is essential to reduce this burden, as well as continuing to improve cardiac services from in utero to adulthood. Copyright © 2011 American Heart Association. All rights reserved. AD - H. Dolk, Department of Epidemiology and Health Services Research, University of Ulster, Jordanstown Campus, Shore Rd, BT370QB, United Kingdom AU - Dolk, H. AU - Loane, M. AU - Garne, E. DB - Embase Medline DO - 10.1161/CIRCULATIONAHA.110.958405 IS - 8 KW - aortic coarctation aortic valve stenosis arterial trunk article Austria Belgium birth rate congenital heart malformation Croatia Denmark Down syndrome Ebstein anomaly Europe Fallot tetralogy fetus fetus death fetus malformation fetus mortality France Germany gestational age great vessels transposition heart disease heart single ventricle human hypoplastic right heart infant Ireland Italy live birth major clinical study Malta Netherlands Norway perinatal mortality perinatal period Poland pregnancy outcome pregnancy termination prenatal diagnosis prevalence priority journal pulmonary valve atresia pulmonary valve stenosis Spain survival Switzerland tricuspid valve atresia trisomy 13 trisomy 18 Ukraine United Kingdom LA - English M3 - Article N1 - L51279082 2011-03-16 2011-03-21 PY - 2011 SN - 0009-7322 1524-4539 SP - 841-849 ST - Congenital heart defects in Europe: Prevalence and perinatal mortality, 2000 to 2005 T2 - Circulation TI - Congenital heart defects in Europe: Prevalence and perinatal mortality, 2000 to 2005 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51279082 http://dx.doi.org/10.1161/CIRCULATIONAHA.110.958405 VL - 123 ID - 1111 ER - TY - JOUR AB - The oculo-auriculo-vertebral spectrum (OAVS) is a non-random association of microtia, hemifacial microsomia with mandibular hypoplasia, ocular epibulbar dermoid, and cervical vertebral malformations. Congenital heart defects (CHDs) have been reported in 5-58% of the patients. We analyze the frequency and anatomic features of CHD in a series of 87 patients with OAVS examined between January 1990 and February 2007 with normal chromosomes, ranging in age between 0.1 and 16.8 years. A twin pregnancy occurred in eight cases (dizygotic in six cases and monozygotic in two). CHDs were diagnosed in 28/87 (32%) patients, and classified into categories of postulated developmental mechanisms including 9 (32%) atrial and ventricular septal defects, 11 (39%) conotruncal defects, 4 (14%) targeted growth defects, two (7%) with situs and looping defects, one (4%) with a left-sided obstructive lesion and one (4%) with patent ductus arteriosus. As noted in other series, the most common individual CHDs were ventricular septal defect (six patients) and tetralogy of Fallot (TOF) (classic or with pulmonary atresia) (six patients). Comparing the frequencies of CHDs groups observed in the OAVS patients with the findings of the Emilia-Romagna Registry which ascertained CHDs prevalence in the general population, conotaincal defects, targeted growth defects, and heterotaxia were significantly associated with OAVS. © 2008 Wiley-Liss, Inc. AD - M. C. Digilio, Medical Genetics, Bambino Gesù Hospital, Rome, Italy AU - Digilio, M. C. AU - Calzolari, F. AU - Capolino, R. AU - Toscano, A. AU - Sarkozy, A. AU - De Zorzi, A. AU - Dallapiccola, B. AU - Marino, B. DB - Embase Medline DO - 10.1002/ajmg.a.32407 IS - 14 KW - adolescent aortic coarctation article brain malformation cerebellum hypoplasia cervical spine child cleft lip palate clinical feature conduction deafness congenital heart malformation conotruncal heart defect dextrocardia disease association dizygotic twins face malformation Fallot tetralogy female Goldenhar syndrome heart atrium septum defect heart left right shunt heart right ventricle double outlet heart ventricle septum defect hemifacial microsomia heterotaxia human hydrocephalus infant kidney malformation lung hypoplasia lung vein drainage anomaly major clinical study male mandible hypoplasia mental deficiency microphthalmia microtia monozygotic twins patent ductus arteriosus perception deafness prevalence priority journal pulmonary valve atresia pulmonary valve stenosis register scimitar syndrome skeleton malformation spine malformation teratoma tricuspid valve atresia twin pregnancy vertebra malformation LA - English M3 - Article N1 - L352002776 2008-08-19 PY - 2008 SN - 1552-4825 1552-4833 SP - 1815-1819 ST - Congenital heart defects in patients with oculo-auriculo-vertebral spectrum (Goldenhar syndrome) T2 - American Journal of Medical Genetics, Part A TI - Congenital heart defects in patients with oculo-auriculo-vertebral spectrum (Goldenhar syndrome) UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L352002776 http://dx.doi.org/10.1002/ajmg.a.32407 VL - 146 ID - 1202 ER - TY - JOUR AB - 1. On the basis of clinical consensus and research observation, newborns with ductal-dependent congenital heart disease (CHD) may present with cyanosis or shock as the patent ductus arteriosus starts to close, indicating a need for prostaglandin to maintain output to the pulmonary or systemic circulation. (5)(7)(8) 2. On the basis of research evidence, as well as clinical consensus, children with acyanotic shunting lesions can present with features of congestive heart failure and require nutritional and diuretic optimization until the time of early surgical correction. (1)(2)(9) 3. On the basis of research evidence, as well as clinical consensus, children with cyanotic heart disease may undergo early corrective surgery or, in some instances, undergo palliation with a shunt (modified Blalock-Taussig-Thomas shunt) to supply pulmonary blood flow prior to their corrective surgery. (22)(32) 4. On the basis of strong clinical evidence, children who undergo single-ventricle palliation are at risk for developing complications at any stage of the palliation. (31) 5. On the basis of clinical evidence, as well as clinical consensus, although most children with corrected CHD have a normal quality of life, lifelong cardiology follow-up is needed for all of these patients to monitor the development of any long-term complications and/or sequelae. (13)(14)(20)(24)(27)(30)(39). AU - Puri, K. AU - Allen, H. D. AU - Qureshi, A. M. DB - Embase Medline DO - 10.1542/pir.2017-0032 IS - 10 KW - diuretic agent ibuprofen indometacin metoprolol phenylephrine spironolactone aortic valve stenosis arterial trunk article atrioventricular septal defect clinical feature congenital heart disease Fallot tetralogy general practitioner great vessels transposition health care heart atrium septum defect heart catheterization heart valve stenosis heart ventricle septum defect human immunization lung vein drainage anomaly newborn period patent ductus arteriosus pulmonic valve stenosis pulse oximetry subacute endocarditis transluminal valvuloplasty tricuspid valve atresia LA - English M3 - Article N1 - L618715119 2017-10-17 2018-07-20 PY - 2017 SN - 1526-3347 0191-9601 SP - 471-484 ST - Congenital heart disease T2 - Pediatrics in Review TI - Congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L618715119 http://dx.doi.org/10.1542/pir.2017-0032 VL - 38 ID - 732 ER - TY - JOUR AB - Congenital heart disease (CHD) is present in approximately 50% of patients with trisomy 21 (T21) and Turner syndrome (TS). According to the American Academy of Pediatrics, every patient with these genetic disorders should have a postnatal echocardiogram. T21 is usually associated with atrioventricular (30%–60%), atrial (16%-21%), or ventricular septal defects (14%–27%). TS is usually associated with left-sided heart disease. However, the spectrum of CHD in these genetic disorders is wider than those mentioned lesions. More cardiac surgical procedures are offered to these patients and that has influenced positively their life expectancy for some CHD conditions. Single ventricular anatomy is associated with high mortality in these genetic disorders (49% in T21 and 83%–91% in TS). The goal of this article is to describe the spectrum of CHD, screening guidelines, and cardiac surgical outcomes in patients with T21 or TS with CHD. AD - R. Morales-Demori, Department of Pediatrics, Division of Pediatric Cardiology, Baylor College of Medicine, Texas Children's Hospital, Houston, TX, United States AU - Morales-Demori, R. DB - Embase Medline DO - 10.1111/chd.12521 IS - 6 KW - antihypertensive agent beta adrenergic receptor blocking agent growth hormone hypocholesterolemic agent anomalous venous return aortic arch branching anomaly aortic coarctation aortic dilation aortic dissection article atrioventricular septal defect surgery bicuspid aortic valve body mass bronchomalacia cardiac surgical outcome cause of death chylothorax clinical outcome congenital heart disease coronary artery anomaly diabetes mellitus disease association disease severity electrocardiography electrophysiology extracorporeal circulation follow up Fontan procedure heart atrium arrhythmia heart single ventricle heart transplantation hormone substitution human hypertension intellectual impairment interpersonal communication language disability laryngomalacia lung infection maternal hypertension maternal smoking pacemaker placement pathogenesis pathophysiology physical examination postoperative complication practice guideline pregnancy prevalence priority journal pulmonary hypertension reoperation risk factor scoring system screening test sensitivity analysis sex difference single ventricular anatomy sinus node spectrum speech disorder subglottic stenosis surgical infection surgical mortality surgical technique thorax radiography tracheomalacia trisomy 21 Turner syndrome visuomotor coordination LA - English M3 - Article N1 - L619491803 2017-12-06 2017-12-12 PY - 2017 SN - 1747-0803 1747-079X SP - 820-827 ST - Congenital heart disease and cardiac procedural outcomes in patients with trisomy 21 and Turner syndrome T2 - Congenital Heart Disease TI - Congenital heart disease and cardiac procedural outcomes in patients with trisomy 21 and Turner syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L619491803 http://dx.doi.org/10.1111/chd.12521 VL - 12 ID - 728 ER - TY - JOUR AB - Background: Management of congenital heart disease (CHD) in developing countries is challenging because of limited access to health-care facilities and socioeconomic limitations. The aim of this study was to describe the recent experience with CHD at Children's Hospital, Vientiane, Laos, the only pediatric referral hospital in the country. Methods: From July 2013 to November 2015, 1009 echocardiograms were carried out in 797 individuals who visited Children's Hospital, in whom CHD was identified in 213. Demographic characteristics, echocardiography and age at first CHD diagnosis on echocardiogram were compared by residential area. Results: Among the 213 patients, the most frequent anomalies, in descending order, were ventricular septal defect, atrial septal defect, patent ductus arteriosus, and tetralogy of Fallot. Moderate or severe CHD requiring surgery or intervention was detected in 137 patients; median age at initial diagnosis was 6.0 months (IQR, 1.5–29.8). Among those with moderate or severe CHD, 89 patients were from rural areas and 48 from the capital area. The proportion of patients from rural areas older than 1 and 3 years at diagnosis was 46.1% and 32.6%, respectively. In contrast, patients from the capital area were diagnosed at a significantly earlier age: 16.7% at age >1 year and 6.2% at age >3 years (both P < 0.001 vs rural areas). Conclusions: Diagnosis of CHD requiring surgery is delayed in Laos, especially in rural areas. Better education and training of local medical staff, and greater access to medical advice are required to improve CHD care in Laos. AD - J.Y. Choi, Department of Pediatrics, Seoul National University Children's Hospital, Seoul, South Korea AU - Hwang, I. C. AU - Sisavanh, M. AU - Billamay, S. AU - Phangmanixay, S. AU - Oudavong, B. AU - Kang, J. AU - Kwon, B. S. AU - Kim, G. B. AU - Bae, E. J. AU - Noh, C. I. AU - Choi, J. Y. DB - Embase Medline DO - 10.1111/ped.13156 IS - 3 KW - age distribution aortic coarctation article atrioventricular septal defect cesarean section child congenital heart disease congestive heart failure controlled study cyanotic heart disease delayed diagnosis demography developing country Down syndrome echocardiography Fallot tetralogy female geographic distribution great vessels transposition heart atrium septum defect heart ventricle septum defect household income human hypoplastic left heart syndrome Laos major clinical study male maternal age mortality rate patent ductus arteriosus prenatal care pulmonary valve atresia pulmonary valve stenosis residential area rural area secondary care center vaginal delivery LA - English M3 - Article N1 - L614943219 2017-03-27 2017-04-11 PY - 2017 SN - 1442-200X 1328-8067 SP - 271-279 ST - Congenital heart disease at Laos Children's Hospital: Two year experience T2 - Pediatrics International TI - Congenital heart disease at Laos Children's Hospital: Two year experience UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L614943219 http://dx.doi.org/10.1111/ped.13156 VL - 59 ID - 769 ER - TY - JOUR AB - The Pediatric Cardiac Genomics Consortium (PCGC) designed the Congenital Heart Disease Genetic Network Study to provide phenotype and genotype data for a large congenital heart defects (CHDs) cohort. This article describes the PCGC cohort, overall and by major types of CHDs (e.g., conotruncal defects) and subtypes of conotrucal heart defects (e.g., tetralogy of Fallot) and left ventricular outflow tract obstructions (e.g., hypoplastic left heart syndrome). Cases with CHDs were recruited through ten sites, 2010–2014. Information on cases (N = 9,727) and their parents was collected through interviews and medical record abstraction. Four case characteristics, eleven parental characteristics, and thirteen parent-reported neurodevelopment outcomes were summarized using counts and frequencies and compared across CHD types and subtypes. Eleven percent of cases had a genetic diagnosis. Among cases without a genetic diagnosis, the majority had conotruncal heart defects (40%) or left ventricular outflow tract obstruction (21%). Across CHD types, there were significant differences (p<0.05) in the distribution of all four case characteristics (e.g., sex), four parental characteristics (e.g., maternal pregestational diabetes), and five neurodevelopmental outcomes (e.g., learning disabilities). Several characteristics (e.g., sex) were also significantly different across CHD subtypes. The PCGC cohort is one of the largest CHD cohorts available for the study of genetic determinants of risk and outcomes. The majority of cases do not have a genetic diagnosis. This description of the PCGC cohort, including differences across CHD types and subtypes, provides a reference work for investigators who are interested in collaborating with or using publically available resources from the PCGC. AD - A.J. Agopian, Department of Epidemiology, Human Genetics and Environmental Sciences, UTHealth School of Public Health, Houston, TX, United States AU - Hoang, T. T. AU - Goldmuntz, E. AU - Roberts, A. E. AU - Chung, W. K. AU - Kline, J. K. AU - Deanfield, J. E. AU - Giardini, A. AU - Aleman, A. AU - Gelb, B. D. AU - Neal, M. M. AU - Porter, G. A. AU - Kim, R. AU - Brueckner, M. AU - Lifton, R. P. AU - Edman, S. AU - Woyciechowski, S. AU - Mitchell, L. E. AU - Agopian, A. J. DB - Embase Medline DO - 10.1371/journal.pone.0191319 IS - 1 KW - article child cohort analysis congenital heart disease congenital heart malformation female genetic disorder genetics heart left ventricle outflow tract obstruction human infant learning disorder major clinical study male maternal diabetes mellitus mental disease LA - English M3 - Article N1 - L620255961 2018-01-26 2018-01-30 PY - 2018 SN - 1932-6203 ST - The congenital heart disease genetic network study: Cohort description T2 - PLoS ONE TI - The congenital heart disease genetic network study: Cohort description UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L620255961 http://dx.doi.org/10.1371/journal.pone.0191319 VL - 13 ID - 718 ER - TY - JOUR AB - Major advances in paediatric cardiac surgery over recent years have resulted in an increased number of children with congenital heart disease (CHD) surviving into adulthood. Currently in Europe, the adult CHD population is greater than that of the paediatric CHD population. This changing prevalence and ageing congenital population brings with it multiple comorbidities and new challenges in all areas of their medical, surgical and psychological management. This article gives an introduction to the common conditions presenting acutely to general physicians, long-term complications, treatment options and the long-term considerations for end-of-life care. Adult congenital heart disease patients are safest treated in a network with a multidisciplinary team approach. AU - Fitzsimmons, S. AU - Salmon, A. DB - Embase DO - 10.1016/j.mpmed.2014.08.006 IS - 11 KW - amiodarone anticoagulant agent beta adrenergic receptor blocking agent dipeptidyl carboxypeptidase inhibitor diuretic agent acute heart failure adult adulthood anticoagulant therapy aortic coarctation article atriopulmonary connection cardioversion congenital heart disease diuretic therapy Eisenmenger complex emergency care Fallot tetralogy Fontan procedure general practitioner great vessels transposition heart atrium arrhythmia heart atrium septum defect heart palpitation heart surgery heart ventricle arrhythmia heart ventricle septum defect holistic care human long term care lung blood flow palliative therapy prevalence priority journal quality of life randomized controlled trial (topic) terminal care LA - English M3 - Article N1 - L603807366 2015-04-21 2015-05-01 PY - 2014 SN - 1365-4357 1357-3039 SP - 656-659 ST - Congenital heart disease in adults T2 - Medicine (United Kingdom) TI - Congenital heart disease in adults UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L603807366 http://dx.doi.org/10.1016/j.mpmed.2014.08.006 VL - 42 ID - 973 ER - TY - JOUR AB - Background - Down's syndrome (DS) is the most common chromosomal abnormality due to a trisomy of chromosome 21 commonly associated with congenital heart defects (CHDs). This study aimed to evaluate the frequency and types of CHD patterns in Turkish children with DS. Method - The data relate to paediatric patients with DS who underwent cardiologic screening between 1994 and 2007 and were reviewed in our Paediatric Cardiology unit. Results - Four hundred and twenty-one out of the 1042 paediatric patients with DS studied over a 13-year period had associated CHD. Of these, 320 (77.6%) had a single cardiac lesion, while the remaining 92 patients (22.4%) had multiple defects. The most common single defect was an atrio-ventricular septal defect (AVSD) found in 141 patients (34.2%), followed by 69 patients (16.7%) showing secundum type atrial septal defect, and ventricular septal defect in 68 patients (16.5%). AVSDs were the leading type, isolated or combined with other cardiac anomalies with an overall occurrence of 19.8% of paediatric patients with DS, and 49.2% of paediatric patients with both DS and CHD. Conclusion - This is the first study concerning the frequency and type of CHD observed in Turkish children with DS. The high frequency of AVSD in Turkish children with DS implied that early screening for CHDs by echocardiography is crucial. The correction of AVSDs in paediatric patients with DS should be performed in the first 6 months of life to avoid irreversible haemodynamic consequences of the defect. AD - K. Nisli, Istanbul Medical Faculty, Paediatric Cardiology Division, Paediatrics Department, Millet cd./Çapa, Istanbul 34390 AU - Nisli, K. AU - Oner, N. AU - Candan, S. AU - Kayserili, H. AU - Tansel, T. AU - Tireli, E. AU - Karaman, B. AU - Omeroglu, R. E. AU - Dindar, A. AU - Aydogan, U. AU - Basaran, S. AU - Ertugrul, T. DB - Embase Medline DO - 10.2143/AC.63.5.2033225 IS - 5 KW - adolescent amniocentesis aortic valve stenosis article cardiovascular surgery child chorion villus sampling comorbidity congenital heart disease controlled study Doppler echocardiography Down syndrome Fallot tetralogy female fetus blood sampling great vessels transposition heart atrium septum defect heart right ventricle double outlet heart ventricle septum defect human hypoplastic left heart syndrome infant major clinical study male medical record review prenatal diagnosis pulmonary valve stenosis superior cava vein syndrome Turkey (republic) two dimensional echocardiography Vivid 3 LA - English M3 - Article N1 - L352668798 2008-11-25 PY - 2008 SN - 0001-5385 SP - 585-589 ST - Congenital heart disease in children with Down's syndrome: Turkish experience of 13 years T2 - Acta Cardiologica TI - Congenital heart disease in children with Down's syndrome: Turkish experience of 13 years UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L352668798 http://dx.doi.org/10.2143/AC.63.5.2033225 VL - 63 ID - 1196 ER - TY - JOUR AB - Background: Congenital heart diseases (CHD) comprise about one-third of all major birth defects. Children with delayed diagnosis and improper treatment are at a high risk of morbidity and mortality. Objective: To determine the prevalence of unrecognized congenital heart disease among elementary school students and study the types and frequency of congenital heart disease in Phitsanulok, Pichit, and Kampangpetch provinces of Thailand using trained nurses and other health care personnel. Methods: Between September 2008 and September 2010, 138,529 students from 1,243 elementary schools were screened for abnormal heart conditions by trained nurses or health care workers. The students who were suspected to have CHD were referred to a pediatric cardiologist for complete evaluation and identification of the lesions. Results: Five hundred forty eight students were suspected to have heart diseases and 102 students were diagnosed as CHD. The average prevalence of unrecognized CHD in Phitsanulok, Pichit, and Kampangpetch was 0.74 per 1,000. Simple acyanotic lesions (ventricular septal defect, atrial septal defect, and pulmonary stenosis) were common in this study (32.35%, 16.67%, and 11.76%, respectively). Conclusion: Qualified nurses or health care workers can be useful for identifying patients with CHD. The role of these personnel in the national heart screening program should be further clarified. AD - E. Cholkraisuwat, Department of Pediatrics, Faculty of Medicine, Naresuan University, Phitsanulok, 65000, Thailand AU - Cholkraisuwat, E. AU - Buddharaksa, Y. AU - Sayasathid, J. DB - Embase DO - 10.5372/1905-7415.0702.178 IS - 2 KW - adolescent aortic stenosis aortic regurgitation article cardiologist child collateral circulation congenital heart disease dextrocardia Ebstein anomaly Fallot tetralogy health care personnel health program heart atrium septum defect heart right ventricle double outlet heart surgery heart ventricle septum defect human hypertrophic obstructive cardiomyopathy major clinical study mitral valve regurgitation nurse patent ductus arteriosus preschool child prevalence primary school pulmonary artery stenosis pulmonary valve atresia pulmonary valve insufficiency pulmonary valve stenosis quality of life rural area school child Thailand tricuspid valve atresia tricuspid valve regurgitation ventricular noncompaction LA - English M3 - Article N1 - L369270605 2013-07-17 2013-07-19 http://abm.digitaljournals.org/index.php/abm/article/viewFile/1828/729 PY - 2013 SN - 1905-7415 1875-855X SP - 287-291 ST - Congenital heart disease in elementary school children in rural Thailand: The role of the trained noncardiologist T2 - Asian Biomedicine TI - Congenital heart disease in elementary school children in rural Thailand: The role of the trained noncardiologist UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L369270605 http://dx.doi.org/10.5372/1905-7415.0702.178 VL - 7 ID - 1007 ER - TY - JOUR AB - Congenital heart disease occurs in approximately 8/1000 live births. The most common lesion at birth is a ventricular septal defect but many are small and do not need surgery. Cyanotic heart disease includes Fallot's tetralogy and transposition of the great arteries, which are both amenable to correction in childhood. More complicated cyanotic lesions are treated by separation of the systemic venous flow by a cavopulmonary connection, often referred to as a Fontan circulation. Some genetic syndromes are associated with congenital heart disease (Marfan's, Noonan's, Williams'), as are chromosomal disorders such as Down's and Turner's. DiGeorge's syndrome (thymic aplasia, hypoparathyroidism and cono-truncal cardiac defect) and velocardiofacial syndrome (palatal abnormalities, heart defects and dysmorphic features) are associated with microdeletions within the q11 region of chromosome 22. AU - Bellsham-Revell, H. AU - Burch, M. DB - Embase DO - 10.1016/j.mpmed.2014.08.004 IS - 11 KW - aortic coarctation aortic stenosis article congenital heart disease Down syndrome Eisenmenger complex Fallot tetralogy gene deletion genetic disorder great vessels transposition heart atrium septum defect heart ventricle septum defect Holt Oram syndrome human lung vein drainage anomaly Marfan syndrome Noonan syndrome patent ductus arteriosus pregnancy termination prenatal diagnosis priority journal pulmonary valve stenosis spontaneous abortion stillbirth Turner syndrome LA - English M3 - Article N1 - L603807354 2015-04-21 2015-05-01 PY - 2014 SN - 1365-4357 1357-3039 SP - 650-655 ST - Congenital heart disease in infancy and childhood T2 - Medicine (United Kingdom) TI - Congenital heart disease in infancy and childhood UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L603807354 http://dx.doi.org/10.1016/j.mpmed.2014.08.004 VL - 42 ID - 972 ER - TY - JOUR AB - Objective To determine the birth prevalence of congenital heart defects (CHDs) across the spectrum of common defects in very/extremely premature infants and to compare mortality rates between premature infants with and without CHDs. Study design The Kids’ Inpatient Databases (2003-2012) were used to estimate the birth prevalence of CHDs (excluding patent ductus arteriosus) in very/extremely premature infants born between 25 and 32 weeks’ gestational age. Birth prevalence was compared with term infants for a subset of “severe” defects expected to be near universally diagnosed in the neonatal period. Weighted multivariable logistic regression was used to calculate aORs of mortality comparing very and extremely premature infants with vs without CHDs. Results We identified 249 011 very/extremely premature infants, including 28 806 with CHDs. The overall birth prevalence of CHDs was 116 per 1000 very/extremely premature births. Severe CHDs had significantly higher birth prevalence in very/extremely premature infants when compared with term infants (7.4 per 1000 very/premature births vs 1.5 per 1000 term births; P < .001). Very/extremely premature infants with severe CHDs had an overall 26.3% in-hospital mortality and a 7.5-fold increased adjusted odds of death compared with those without CHDs. Mortality varied widely by defect in very/extremely premature infants, ranging from 12% for interrupted aortic arch to 67% for truncus arteriosus. Conclusions Given the increased birth prevalence of severe CHDs in very/extremely premature infants, and significantly higher mortality, there is justification for intensive interventions aimed at decreasing the likelihood of premature delivery for patients where CHD is diagnosed in utero. AD - K.D. Hill, Department of Pediatrics, Division of Cardiology, Duke Clinical Research Institute, Duke University Medical Center, P.O. Box 17969, Durham, NC, United States AU - Chu, P. Y. AU - Li, J. S. AU - Kosinski, A. S. AU - Hornik, C. P. AU - Hill, K. D. DB - Embase Medline DO - 10.1016/j.jpeds.2016.10.033 KW - aortic arch aortic stenosis arterial trunk article childhood mortality congenital heart disease DiGeorge syndrome Down syndrome Ebstein anomaly Fallot tetralogy female genetic disorder gestational age heart atrium septum defect heart ventricle septum defect hospital mortality human infant lung vein drainage anomaly major clinical study male prematurity priority journal pulmonary valve atresia pulmonary valve stenosis term birth tricuspid valve atresia LA - English M3 - Article N1 - L613728999 2016-12-20 2017-02-21 PY - 2017 SN - 1097-6833 0022-3476 SP - 37-41 ST - Congenital Heart Disease in Premature Infants 25-32 Weeks’ Gestational Age T2 - Journal of Pediatrics TI - Congenital Heart Disease in Premature Infants 25-32 Weeks’ Gestational Age UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L613728999 http://dx.doi.org/10.1016/j.jpeds.2016.10.033 VL - 181 ID - 774 ER - TY - JOUR AB - PURPOSE OF REVIEW: As the specialties of pediatrics and pediatric cardiology continue to forge ahead with better diagnoses, medical care, and surgical results, an expanding population of patients with congenital heart disease (CHD) outgrows the pediatric age group, yet does not quite graduate to routine adult cardiology or general medicine. The adult with congenital heart disease (ACHD) faces medical, surgical, and psychosocial issues that are unique to this population and must be addressed as such. This review attempts to discuss and highlight some of the important advances and controversies brought up in the past year, in the care and management of these patients. RECENT FINDINGS: The past five to 10 years have seen dynamic interest in understanding sequelae of corrected, uncorrected, or palliated congenital heart disease. The search for the ideal surgery, optimal prosthesis, and a smooth transition to adult care continues and is reflected in the vast amount of academic work and publications in this field. Of particular interest, conduit reoperations and single ventricle pathway modifications are still an art and a science in evolution. SUMMARY: While all are agreed that there is a pressing need to focus on the delivery of care to the adult with congenital heart disease, this essentially requires a clearer understanding of late sequelae of CHD. The sheer heterogeneity of anatomy, age, surgery, and institutional management protocols can make it difficult to develop clear guidelines. This review attempts to give an up-to-date perspective on some of the new findings related to the more common lesions and problems faced in this group. AD - The Clinical Care Center for Congenital Heart Disease, Oregon Health & Science University, Portland, Oregon 97239-3098, USA. AN - 15367842 AU - Bhat, A. H. AU - Sahn, D. J. DA - Oct DB - PubMed DO - 10.1097/01.mop.0000140996.24408.1a DP - NLM ET - 2004/09/16 IS - 5 KW - Adult Aorta/surgery Bioprosthesis Death, Sudden, Cardiac/prevention & control Fontan Procedure Heart Defects, Congenital/*surgery Heart Septal Defects, Ventricular/surgery Humans Pulmonary Artery/surgery Reoperation/instrumentation/methods Tetralogy of Fallot/surgery Transplantation, Autologous Transposition of Great Vessels/surgery LA - eng N1 - Bhat, Aarti Hejmadi Sahn, David J Journal Article Review United States Curr Opin Pediatr. 2004 Oct;16(5):500-7. doi: 10.1097/01.mop.0000140996.24408.1a. PY - 2004 SN - 1040-8703 (Print) 1040-8703 SP - 500-7 ST - Congenital heart disease never goes away, even when it has been 'treated': the adult with congenital heart disease T2 - Curr Opin Pediatr TI - Congenital heart disease never goes away, even when it has been 'treated': the adult with congenital heart disease VL - 16 ID - 357 ER - TY - JOUR AB - The prevalence rate of cardiac malformations (CM) in Bohemia is 6.64/1000 live births and 21/1000 stillbirths. The most common were ventricular and atrial septal defects, pulmonary and aortic stenoses in live-born infants, and ventricular septal defect, hypoplastic left heart syndrome and transposition in stillborns. Seasonal and regional differences of the prevalence were found in the whole cohort and in individual forms of CM. The boys to girls ratio was 1.09:1 in live births and 1.3:1 stillbirths. Boys predominated in double outlet right ventricle, transposition and aortic stenosis and girls in patent ductus arteriosus, Ebstein's anomaly and truncus arteriosus. 35.3% of all CM, i.e., 2.36/1000 of all live births presented with life-threatening symptomatology or died. Of all children born with CM, 78.3% reached the age of 10. The 10-year survival rate was highest in pulmonary stenosis, atrial septal defect, aortic stenosis, ventricular septal defect and patent ductus arteriosus and lowest in hypoplastic left heart, truncus arteriosus and pulmonary atresia. Only 17.7% of suviving children are on medication. Physical activity is normal in 65.4%, slightly reduced in 28.4%, and reduced in 5.9% of long-term survivors. AD - M. Samanek, Kardiocentrum, Teaching Hospital Motol, V uvalu 84, 150 18 Praha 5, Czech Republic AU - Samanek, M. DB - Embase IS - 1 KW - article child congenital heart disease human infant major clinical study newborn quality of life LA - English M3 - Article N1 - L24092741 1994-04-10 PY - 1994 SN - 0010-8650 SP - 11-14 ST - Congenital heart diseases in Bohemia. Epidemiologic data T2 - Cor et Vasa TI - Congenital heart diseases in Bohemia. Epidemiologic data UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L24092741 VL - 36 ID - 1374 ER - TY - JOUR AB - Consanguineous marriage means a union occur between related individuals as second cousins or closer. Iraq as part Middle Eastern States show a high rate of consanguineous marriage due to tribal society. In Iraq, consanguineous marriage is a socially supported custom. To study the association of consanguinity as a risk factor for congenital heart diseases (CHDs). Patients with suggestive signs of CHD admitted Tikrit teaching hospital were subject to diagnostic investigations. Case data includes: name, age, gender, and cause of admission. Parents data includes: age, residence, degree of consanguinity, and history of family recurrent CHDs. Congenital heart diseases was slightly higher in females than males, and there is 40% of studied cases were from positive consanguinity families. This study shows that the least congenital heart disease age reported was neonatal period, and the highest in ages between 1-5 years. Consanguinity in CHD was present in 81 cases (40%) and absent in 123 cases (60%). Family history in CHD was positive in (38%). most common congenital abnormality associated with CHD was Down’s syndrome. AD - A.R. Sarhat, Tikrit Nursing College, Iraq AU - Abd, M. A. AU - Sulaiman, R. AU - Sarhat, A. R. DB - Embase DO - 10.5958/0976-5506.2019.01428.1 IS - 6 KW - age aortic stenosis aortopulmonary septal defect article child congenital heart disease consanguineous marriage cross-sectional study Down syndrome echocardiography Edwards syndrome family history female heart single ventricle human infant Iraq major clinical study male newborn observational study pulmonary valve atresia questionnaire risk factor school child LA - English M3 - Article N1 - L2002344968 2019-08-20 2019-08-21 PY - 2019 SN - 0976-5506 0976-0245 SP - 1070-1072 ST - Congenital heart diseases in consanguineous marriages in tikrit-Iraq T2 - Indian Journal of Public Health Research and Development TI - Congenital heart diseases in consanguineous marriages in tikrit-Iraq UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2002344968 http://dx.doi.org/10.5958/0976-5506.2019.01428.1 VL - 10 ID - 609 ER - TY - JOUR AD - Congenital Heart Surgery, Texas Children's Hospital, 6621 Fannin St, Houston, TX 77030, United States AU - Fraser, C. D. AU - Carberry, K. E. DB - Scopus IS - 6 M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2010 SP - 662-664 ST - Congenital heart surgery in the adult T2 - Texas Heart Institute Journal TI - Congenital heart surgery in the adult UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-79251483841&partnerID=40&md5=970a99d75ba5d28df0b70e4e6561e7b3 VL - 37 ID - 2142 ER - TY - JOUR AB - OBJECTIVE: We describe patient characteristics and postoperative morbidity and mortality rates for patients with Down syndrome undergoing congenital heart disease surgery. METHODS: This retrospective cohort study used the Society of Thoracic Surgeons Congenital Heart Surgery Database to compare patient characteristics and postoperative outcomes for patients (0-18 years) with or without Down syndrome who underwent surgery in 2000-2008. RESULTS: A total of 45 579 patients (4350 patients with Down syndrome and 41 229 without Down syndrome) were included (median age: 7 months [interquartile range [IQR]: 47 days to 4 years]; 56% male). Patients with Down syndrome were younger at surgery, with the exception of those undergoing tetralogy of Fallot repair or atrioventricular septal defect repair. Mortality rates for patients with or without Down syndrome did not differ significantly. Lengths of stay were prolonged for patients with Down syndrome undergoing atrial septal defect closure (median: 4 days [IQR: 3-5 days] vs 3 days [IQR: 2-4 days]; P < .0001), ventricular septal defect closure (median: 5 days [IQR: 4-8 days] vs 4 days [IQR: 3-6 days]; P < .0001), or tetralogy of Fallot repair (7 days [IQR: 5-10 days] vs 6 days [IQR: 5-9 days]; P < .001) and were associated with postoperative respiratory and infectious complications. Patients with Down syndrome undergoing ventricular septal defect closure had a higher rate of heart block requiring pacemaker placement (2.9% vs 0.8%; P < .0001). CONCLUSION: In this large, contemporary cohort, Down syndrome did not confer a significant mortality risk for the most common operations; however, postoperative morbidity remained common. Copyright © 2010 by the American Academy of Pediatrics. AD - S. K. Pasquali, University Medical Center, Department of Pediatrics, Division of Cardiology, PO Box 17969, Durham, NC 27715, United States AU - Fudge Jr, J. C. AU - Li, S. AU - Jaggers, J. AU - O'Brien, S. M. AU - Peterson, E. D. AU - Jacobs, J. P. AU - Welke, K. F. AU - Jacobs, M. L. AU - Li, J. S. AU - Pasquali, S. K. DB - Embase Medline DO - 10.1542/peds.2009-3245 IS - 2 KW - adolescent adult article artificial heart pacemaker cavopulmonary connection child clinical trial cohort analysis congenital heart disease controlled study data base disease association Down syndrome endocarditis Fallot tetralogy female Fontan procedure heart atrium septum defect heart block heart rate heart surgery heart ventricle septum defect human infant kidney failure length of stay major clinical study male mediastinitis medical society mitral valve repair morbidity multicenter study neurologic disease Norwood procedure outcome assessment postoperative complication postoperative infection postoperative period preschool child priority journal pulmonary hypertension respiratory tract infection retrospective study school child seizure sepsis surgical mortality systemic pulmonary shunt treatment outcome wound dehiscence LA - English M3 - Article N1 - L359335570 2010-08-19 2010-08-26 http://pediatrics.aappublications.org/cgi/reprint/126/2/315 PY - 2010 SN - 0031-4005 1098-4275 SP - 315-322 ST - Congenital heart surgery outcomes in down syndrome: Analysis of a national clinical database T2 - Pediatrics TI - Congenital heart surgery outcomes in down syndrome: Analysis of a national clinical database UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L359335570 http://dx.doi.org/10.1542/peds.2009-3245 VL - 126 ID - 1137 ER - TY - JOUR AB - A recent case of conjoined twins required multiple hospitalizations in the pediatric intensive care unit and led to a difficult situation confronting staff regarding the potential separation where surgery would result in the death of one twin. The hospital ethics committee was consulted. A systematic approach was utilized to examine medical standards, historical precedents, and various ethical and legal frameworks. The ethics committee believed that either proceeding with or forgoing attempted separation surgery would be ethically acceptable. We share our reasoning and lessons learned for others facing this situation in the future. AD - B.M. Cummings, Pediatric Intensive Care, MGH Pediatrics Ethics Committee, Department of Pediatrics, Massachusetts General Hospital, Boston, MA, United States AU - Cummings, B. M. AU - Paris, J. J. DB - Embase Medline DO - 10.1177/0885066618791953 IS - 1 KW - antecedent variable article bladder function case report child child hospitalization circulation clinical article clinical decision making conjoined twins cyanotic heart disease death dietary intake ethics female human intestine function medical assessment mobilization pediatric intensive care unit pelvis physical disease by anatomical structure preschool child priority journal pulmonary valve atresia quality of life sternum survival tricuspid valve atresia LA - English M3 - Article N1 - L623909677 2018-09-21 2018-12-19 PY - 2019 SN - 1525-1489 0885-0666 SP - 81-84 ST - Conjoined Twins Separation Leading to the Death of One Twin: An Expanded Ethical Analysis of Issues Facing the ICU Team T2 - Journal of Intensive Care Medicine TI - Conjoined Twins Separation Leading to the Death of One Twin: An Expanded Ethical Analysis of Issues Facing the ICU Team UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L623909677 http://dx.doi.org/10.1177/0885066618791953 VL - 34 ID - 638 ER - TY - JOUR AB - Coupling shape optimization to three-dimensional unsteady cardiovascular simulations poses several key challenges, including high computational cost, a need to handle constraints, and a need for automatic generation of parameterized vessel geometry. In this work we extend our previous framework for cardiovascular optimization to include constraints, pulsatile flow under both rest and exercise conditions, and multiple geometric parameters. Optimization is performed using a derivative-free algorithm called the surrogate management framework, with constraints enforced using a filter method. In this work, we examine a specific surgery called the Fontan, which is performed to treat single-ventricle heart patients. These patients typically undergo a series of three surgeries, the last of which connects the inferior vena cava to the pulmonary arteries. Our group and others have recently proposed and evaluated a new Y-graft modification of the Fontan operation that replaces the current tube shaped baffle. Preliminary simulations have shown that the Y-graft modification is a promising design that increases energy efficiency and improves flow distribution to the pulmonary arteries. In this work, we perform optimization on a model Y-graft design problem. This work represents the first use of formal design optimization methods for the Fontan surgery, and also demonstrates the applicability of the optimization framework on a pulsatile flow problem with multiple design parameters and constraints. The idealized Y-graft model was parameterized with six geometric parameters including graft diameter and anastomosis locations, and the optimization procedure, including model construction, meshing, and simulation, was executed automatically. Energy efficiency was chosen as the objective function. A constraint on the wall shear stress (WSS), a presumed correlate to thrombosis risk, was added to the problem using a filter method, which allowed for exploration of the trade-offs between WSS and energy efficiency. Optimization was performed at two exercise levels with effects of respiration incorporated, and differences in optimal solutions were examined. It was shown that optimal shapes differed between rest and exercise, as well as steady and pulsatile flow conditions, with wide-span branches and decreasing graft branch size favored with increasing exercise level. The optimization method was found to be robust for different polling strategies, and computationally efficient both with and without constraints. © 2010 Elsevier B.V. AD - Mechanical and Aerospace Engineering Department, University of California San Diego, United States Departments of Pediatrics and Bioengineering, Stanford University, United States AU - Yang, W. AU - Feinstein, J. A. AU - Marsden, A. L. DB - Scopus DO - 10.1016/j.cma.2010.03.012 IS - 33-36 KW - Cardiovascular surgery CFD Derivative-free optimization Fontan surgery Mesh adaptive direct search Pediatric cardiology Surrogate management framework M3 - Article N1 - Cited By :50 Export Date: 15 June 2020 PY - 2010 SP - 2135-2149 ST - Constrained optimization of an idealized Y-shaped baffle for the Fontan surgery at rest and exercise T2 - Computer Methods in Applied Mechanics and Engineering TI - Constrained optimization of an idealized Y-shaped baffle for the Fontan surgery at rest and exercise UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-77953616515&doi=10.1016%2fj.cma.2010.03.012&partnerID=40&md5=f00e665aaf105cb2834f3e1cea7b8c69 VL - 199 ID - 2150 ER - TY - JOUR AB - Ellis - van Creveld syndrome is an autosomal recessive disorder manifest by short-limb dwarfism, thoracic dystrophy, postaxial polydactyly, dysplastic nails and teeth, and an approximately 60% incidence of congenital malformations of the heart. Despite patients with Ellis - van Creveld syndrome being regarded as having a high surgical risk, few data are available regarding their outcomes following surgery for congenital malformations of the heart in the current era. Materials and methods: In this retrospective report, we summarise the clinical observations and outcomes of nine infants with Ellis - van Creveld syndrome who underwent surgery for congenital malformations of the heart between 2004 and 2009. Results: We identified 15 patients with Ellis - van Creveld syndrome during the study period; 11 (73%) had haemodynamically significant congenital malformations of the heart warranting surgery. In two of these patients, surgery was not performed. Of the nine patients who underwent surgery, all of whom were infants, eight (89%) had various forms of an atrioventricular septal defect and one patient (11%) had hypoplastic left heart syndrome (mitral and aortic atresia). Among the nine patients who underwent surgery, four (44%) died at a median of 102 days with a range of 25-149 days post-operatively, mostly from respiratory failure. Respiratory morbidity was seen in all surviving patients, of whom three underwent tracheostomy. Conclusions: Surgery for congenital malformations of the heart can be successful in infants with Ellis - van Creveld syndrome, but mortality is high and post-operative respiratory morbidity should be expected. © Copyright Cambridge University Press 2010. AD - M.J. O'Connor, Cardiac Center, Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104-4399, United States AU - O'Connor, M. J. AU - Rider, N. L. AU - Thomas Collins, Ii R. AU - Hanna, B. D. AU - Holmes Morton, D. AU - Strauss, K. A. DB - Embase Medline DO - 10.1017/S1047951110001587 IS - 2 KW - aorta valve atresia aortic valve disease article cause of death clinical article disease association Ellis van Creveld syndrome heart atrium septum defect heart surgery heart ventricle septum defect human hypoplastic left heart syndrome infant mitral valve atresia newborn morbidity outcome assessment postoperative period respiratory failure retrospective study surgical mortality survival time tracheostomy treatment indication LA - English M3 - Article N1 - L51149148 2010-11-18 2011-10-13 PY - 2011 SN - 1047-9511 1467-1107 SP - 145-152 ST - Contemporary management of congenital malformations of the heart in infants with Ellis - Van Creveld syndrome: A report of nine cases T2 - Cardiology in the Young TI - Contemporary management of congenital malformations of the heart in infants with Ellis - Van Creveld syndrome: A report of nine cases UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51149148 http://dx.doi.org/10.1017/S1047951110001587 VL - 21 ID - 1107 ER - TY - JOUR AB - Improvements in the care of children with cardiomyopathy, CHDs, and acquired heart disease have led to an increased number of children surviving with advanced heart failure. In addition, the advent of more durable mechanical circulatory support options in children has changed the outcome for many patients who otherwise would have succumbed while waiting for heart transplantation. As a result, more children with end-stage heart failure are being referred for heart transplantation, and there is increased demand for a limited donor organ supply. A review of important publications in the recent years related to paediatric heart failure, transplantation, and mechanical circulatory support show a trend towards pushing the limits of the current therapies to address the needs of this growing population. There have been a number of publications focussing on previously published risk factors perceived as barriers to successful heart transplantation, including elevated pulmonary vascular resistance, medication non-adherence, re-transplantation, transplantation of the failed Fontan patient, and transplantation in an infant or child bridged with mechanical circulatory support. This review will highlight some of these key articles from the last 3 years and describe recent advances in the understanding, diagnosis, and management of children with end-stage heart disease. AD - Department of Pediatric Cardiology, Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America Heart Institute, Children's Hospital Colorado, University of Colorado, Aurora, Colorado, United States of America AN - 115612947. Language: English. Entry Date: 20170212. Revision Date: 20170601. Publication Type: journal article AU - Kindel, Steven J. AU - Everitt, Melanie D. DB - ccm DO - 10.1017/S1047951116000184 DP - EBSCOhost IS - 5 KW - Heart Transplantation Myocardial Diseases -- Therapy Heart Assist Devices -- Adverse Effects Extracorporeal Membrane Oxygenation Heart Failure -- Therapy Risk Factors Child Pediatrics Waiting Lists Treatment Outcomes Medication Compliance Heart Failure -- Surgery Myocardial Diseases -- Surgery Questionnaires Scales N1 - review. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Instrumentation: Infant Characteristics Questionnaire (ICQ) (Bates et al); Perceived Stress Scale (PSS) (Cohen et al). NLM UID: 9200019. PMID: NLM26979140. PY - 2016 SN - 1047-9511 SP - 851-859 ST - A contemporary review of paediatric heart transplantation and mechanical circulatory support T2 - Cardiology in the Young TI - A contemporary review of paediatric heart transplantation and mechanical circulatory support UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=115612947&site=ehost-live&scope=site VL - 26 ID - 1581 ER - TY - JOUR AD - University Medical Center Groningen, Department of Pediatrics, University of Groningen, Groningen, Netherlands University of Montreal, Division of Neonatology and Clinical Ethics, Sainte-Justine Hospital, Montreal, QC, Canada AU - Verhagen, A. A. E. AU - Janvier, A. DB - Scopus DO - 10.1001/jamapediatrics.2013.3065 IS - 11 M3 - Short Survey N1 - Cited By :16 Export Date: 15 June 2020 PY - 2013 SP - 987-988 ST - The continuing importance of how neonates die T2 - JAMA Pediatrics TI - The continuing importance of how neonates die UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84887451132&doi=10.1001%2fjamapediatrics.2013.3065&partnerID=40&md5=6b294fa06392008adb3b82a7701e2d69 VL - 167 ID - 2071 ER - TY - JOUR AB - OBJECTIVE: Single-ventricle patients undergoing pediatric heart surgery are a high-risk group owing to reoxygenation injury during cardiopulmonary bypass (CPB). The present study investigated the effects of controlled reoxygenation CPB on biomarkers of organ damage, inflammation, stress, and long-term functional outcomes in cyanotic patients with either a single or double ventricle during open heart surgery. METHODS: Cyanotic patients with either a single (n = 32) or double (n = 47) ventricle undergoing surgical correction were randomized to receive CPB using either standard oxygen levels or controlled reoxygenation. The markers of cardiac injury, inflammation, stress, and cerebral and hepatic injury were measured preoperatively, at 10 and 30 minutes after starting CPB, and at 10 minutes and 4 and 24 hours after CPB. The data were analyzed using a mixed regression model. RESULTS: No difference was found in the pre- or intraoperative characteristics between the standard and controlled reoxygenation CPB groups for single- or double-ventricle patients. In the single-ventricle patients, controlled reoxygenation CPB significantly (P < .05) decreased the markers of organ damage, inflammation, stress, and oxidative stress. In contrast, the markers of inflammation and cardiac injury were not altered by controlled reoxygenation CPB in the double-ventricle patients. CONCLUSIONS: Controlled reoxygenation CPB decreased the markers of organ damage, stress, inflammation, and oxidative stress in single-ventricle patients undergoing cardiac surgery. AD - Bristol Royal Hospital for Children, Bristol, United Kingdom; Rush University Medical Center, Chicago, Ill. Electronic address: M.Caputo@bristol.ac.uk. Bristol Heart Institute, University of Bristol, Bristol, United Kingdom. Rush University Medical Center, Chicago, Ill. AN - 25052821 AU - Caputo, M. AU - Mokhtari, A. AU - Miceli, A. AU - Ghorbel, M. T. AU - Angelini, G. D. AU - Parry, A. J. AU - Suleiman, S. M. DA - Sep DB - PubMed DO - 10.1016/j.jtcvs.2014.06.001 DP - NLM ET - 2014/07/24 IS - 3 KW - Age Factors Biomarkers/blood Cardiac Surgical Procedures/*adverse effects Cardiopulmonary Bypass/*adverse effects Child Child, Preschool Dinoprost/analogs & derivatives/blood England Female Heart Defects, Congenital/blood/*surgery Heart Ventricles/abnormalities/metabolism/*surgery Humans Infant Inflammation/blood/etiology/*prevention & control Inflammation Mediators/*blood Male *Oxidative Stress Oxygen Inhalation Therapy/adverse effects/*methods Partial Pressure Time Factors Treatment Outcome Troponin/blood LA - eng N1 - 1097-685x Caputo, Massimo Mokhtari, Amir Miceli, Antonio Ghorbel, Mohamed T Angelini, Gianni D Parry, Andrew J Suleiman, Saadeh M British Heart Foundation/United Kingdom Journal Article Randomized Controlled Trial Research Support, Non-U.S. Gov't United States J Thorac Cardiovasc Surg. 2014 Sep;148(3):792-801.e8; discussion 800-1. doi: 10.1016/j.jtcvs.2014.06.001. Epub 2014 Jun 6. PY - 2014 SN - 0022-5223 SP - 792-801.e8; discussion 800-1 ST - Controlled reoxygenation during cardiopulmonary bypass decreases markers of organ damage, inflammation, and oxidative stress in single-ventricle patients undergoing pediatric heart surgery T2 - J Thorac Cardiovasc Surg TI - Controlled reoxygenation during cardiopulmonary bypass decreases markers of organ damage, inflammation, and oxidative stress in single-ventricle patients undergoing pediatric heart surgery VL - 148 ID - 236 ER - TY - JOUR AB - Many patients with a functional univentricular heart were treated in the 1970s and 1980s, using an atriopulmonary connection to create the Fontan circulation. Although this procedure, in many patients, was initially successful, and provided arterial saturations of oxygen close to normal, as these patients were followed over the years, in some cases they developed significant complications. One complication of the atriopulmonary connection is progressive right atrial dilation, which leads to atrial arrhythmias, such as atrial flutter or fibrillation. The combination of these two problems leads to low cardiac output, diminished quality of life, and poor categorization within the classification of the New York Heart Association. This, and other issues, has led most centres to abandon the atriopulmonary connection as a means of creating the Fontan circulation in favour of the lateral tunnel with cavopulmonary connections, or the extracardiac conduit. AD - C.L. Backer, Division of Cardiovascular-Thoracic Surgery, Children's Memorial Hospital, 2300 Children's Plaza, Chicago, IL 60614, United States AU - Backer, C. L. AU - Deal, B. J. AU - Mavroudis, C. AU - Franklin, W. H. AU - Stewart, R. D. DB - Embase DO - 10.1017/S1047951105002386 IS - SUPPL. 1 KW - antiarrhythmic agent oxygen adolescent arterial oxygen saturation article artificial heart pacemaker cardiopulmonary bypass child controlled study cryosurgery death disease classification follow up Fontan procedure graft rejection graft survival health care organization heart assist device heart atrium arrhythmia heart atrium enlargement atrial fibrillation heart atrium flutter heart output heart single ventricle heart transplantation hemodialysis human kidney failure kidney transplantation length of stay liver failure major clinical study outcome assessment peritoneal dialysis quality of life reentry tachycardia surgical equipment surgical technique treatment failure treatment indication treatment outcome United States AT 500 Surgifrost LA - English M3 - Article N1 - L44274716 2006-10-05 PY - 2006 SN - 1047-9511 1467-1107 SP - 85-91 ST - Conversion of the failed Fontan circulation T2 - Cardiology in the Young TI - Conversion of the failed Fontan circulation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L44274716 http://dx.doi.org/10.1017/S1047951105002386 VL - 16 ID - 1268 ER - TY - JOUR AB - Cor triloculare biatriatum or double inlet single ventricle is a congenital heart defect in which both atria are connected to a common or dominant ventricle. The present report presents an index case and describes the embryological basis and clinical aspects of this extremely rare anomaly. A three months old infant presented with extreme respiratory distress without cyanosis and repeated chest infections. The patient was diagnosed to be a case of single ventricle with both atria opening in the common ventricular chamber. The common ventricular chamber (single ventricle) was connected to a rudimentary outflow tract. The great arteries were in a position of d-transposition of great arteries. However, there was no pulmonary or aortic stenosis. A clear concept and awareness regarding this condition and its clinical manifestations is bound to facilitate timely intervention with improved success rates. AU - Agnihotri, G. AU - Mahajan, D. DB - Medline IS - 2-4 KW - case report congenital heart malformation congenital malformation echography heart ventricle human infant male neonatal respiratory distress syndrome respiratory tract infection LA - English M3 - Article N1 - L609319081 2016-04-04 PY - 2014 SP - 198-200 ST - Cor triloculare biatriatum--A case report T2 - Nepal Medical College journal : NMCJ TI - Cor triloculare biatriatum--A case report UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L609319081 VL - 16 ID - 905 ER - TY - JOUR AB - Objective: The aims of this study were to compare the level of posttraumatic stress disorder between adults with and without congenital heart disease, and to examine the correlates of posttraumatic stress disorder (e.g., sociodemographics). Design: Cross-sectional. Setting: Two university-affiliated heart hospitals in Tehran, Iran. Patients: A sample of 347 adults with congenital heart disease aged 18–64 years (52% women), and 353 adults without congenital heart disease matched by sex and age (±2 years) was recruited. Outcome Measures: The PTSD Scale: Self-report version was used to assess the diagnosis and severity of posttraumatic stress disorder. Hierarchical multivariate logistic regression analyses were performed to explore correlates of likely posttraumatic stress disorder diagnosis among each group of participants. Results: The posttraumatic stress disorder in the patients was comparable to those of the control group, except for increased arousal (P =.027) which was scored higher among the patients. Over 52% of adults with congenital heart disease met the criteria for a likely posttraumatic stress disorder diagnosis compared with 48% of adults without congenital heart disease. The regression analyses among patients revealed that elevated depressive symptoms (OR = 1.27) and a positive history of cardiac surgery (OR = 2.02) were significantly associated with posttraumatic stress disorder. The model could explain 29% of the variance in posttraumatic stress disorder. Conclusions: The high and comparable prevalence of posttraumatic stress disorder among patients and nonpatients highlight the significance of the context in which adults with congenital heart disease may face other/additional stressors than disease-related ones, an issue that clinicians need also take into account. Furthermore, the association of posttraumatic stress disorder with elevated depressive symptoms warrant a comprehensive psychological assessment and management of adults with congenital heart disease, in particular among those with a history of invasive procedures. © 2017 Wiley Periodicals, Inc. AD - Division of Public Health Science, Department of Health Sciences, Mid Sweden University, Sundsvall, Sweden Department of Research, Tehran Heart Center, Tehran University of Medical Sciences, Tehran, Iran AU - Eslami, B. DB - Scopus DO - 10.1111/chd.12452 IS - 3 KW - cardiac surgery depression developing country grown up with CHD mental health M3 - Article N1 - Cited By :6 Export Date: 15 June 2020 PY - 2017 SP - 357-363 ST - Correlates of posttraumatic stress disorder in adults with congenital heart disease T2 - Congenital Heart Disease TI - Correlates of posttraumatic stress disorder in adults with congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85013378437&doi=10.1111%2fchd.12452&partnerID=40&md5=b322ad71d075aedc1f329d9576a7d9ed VL - 12 ID - 1864 ER - TY - JOUR AB - Background: Plastic bronchitis is a dreaded complication of single ventricle physiology occurring following palliation via Fontan procedure. Medical management of plastic bronchitis often fails, requiring heart transplantation. Percutaneous lymphatic embolization is an emerging treatment for plastic bronchitis. Methods: To determine the cost-effectiveness of competing management strategies, a modified Markov model was constructed with patients transiting through treatments—medical management, lymphatic embolization, or heart transplantation from a hospital system perspective. Health state transitions were modeled using an institutional review board–approved retrospective review of the Children’s Hospital of Pennsylvania’s plastic bronchitis cohort. Medication pricing data were obtained from the National Inpatient Sample. Differences in costs and quality-adjusted life years (QALYs) over a five-year horizon for each group were determined. The incremental cost-effectiveness ratio was then calculated. Results: The mean cost of lymphatic embolization from procedure performance was US$340,941, US$385,841 for heart transplantation, and US$594,520 for medical management. The mean quality-adjusted survival of lymphatic embolization yielded an additional 0.66 QALYs (P <.03) relative to heart transplantation and 1.3 (P <.0001) relative to medical management. Orthotopic heart transplantation yielded an additional 0.66 QALYs (P =.06) when comparing heart transplantation to medical management. Compared to medical management, lymphatic embolization generated an incremental cost-effectiveness ratio of US$192,105. Similarly, compared to heart transplantation, lymphatic embolization yielded an incremental cost-effectiveness ratio of US$68,030. Conclusions: Of the available plastic bronchitis treatments, with a willingness to pay of US$150,000, lymphatic embolization produces an incremental cost-effectiveness ratio within the bounds considered to be cost-effective, potentially causing financial benefits to the health system. AD - M. Itkin, Center for Lymphatic Disorders, Perelman School of Medicine, University of Pennsylvania, United States AU - Benjamin, J. L. AU - Rychik, J. AU - Johnstone, J. A. AU - Nadolski, G. J. AU - Itkin, M. DB - Embase Medline DO - 10.1177/2150135119842866 IS - 4 KW - azathioprine basiliximab cyclosporine furosemide mycophenolate mofetil prednisolone prednisone prostacyclin rapamycin sildenafil tacrolimus thymocyte antibody tissue plasminogen activator article artificial embolization bronchitis bronchoscopy child circannual rhythm cohort analysis computer simulation controlled study cost effectiveness analysis cost utility analysis drug cost European Quality of Life 5 Dimensions questionnaire government health care utilization health status heart catheterization heart transplantation human ICD-10 ICD-9 immunosuppressive treatment institutional review machine learning modified markov model orthotopic heart transplantation percutaneous lymphatic embolization plastic bronchitis priority journal quality adjusted life year Rankin scale retrospective study LA - English M3 - Article N1 - L628614491 2019-07-25 2020-03-03 PY - 2019 SN - 2150-136X 2150-1351 SP - 407-413 ST - Cost-Effectiveness of Percutaneous Lymphatic Embolization for Management of Plastic Bronchitis T2 - World Journal for Pediatric and Congenital Heart Surgery TI - Cost-Effectiveness of Percutaneous Lymphatic Embolization for Management of Plastic Bronchitis UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L628614491 http://dx.doi.org/10.1177/2150135119842866 VL - 10 ID - 611 ER - TY - JOUR AB - Summary. The novel severe acute respiratory syndrome coronavirus 2 (SARS-COV 2) has rapidly spread worldwide with increasing hospitalization and mortality rate. Ongoing studies and accumulated data are detailing the features and the effects of the new coronavirus disease 19 (COVID 19) in the adult population, and cardiovascular involvement is emerging as the most significant and life-threatening complication, with an increased risk of morbidity and mortality in patients with underlying cardiovascular disease. At present, though the limited data on the effects of COVID 19 in pediatric patients, children seem to count for a little proportion of SARS-COV 2 infection, and present with less severe disease and effects However infants and toddlers are at risk of developing critical course. The disease has a range of clinical presentations in children, for which the potential need for further investigation of myocardial injury and cardiovascular issues should be kept in mind to avoid misdiagnosing severe clinical entities. Overlapping with Kawasaki disease is a concern, particularly the incomplete and atypical form. We aim to summarize the initial considerations and potential cardiovascular implications of COVID-19 for children and patients with congenital heart disease. (www.actabiomedica.it). © Mattioli 1885. AD - Pediatric Cardiology Unit, Parma General and University Hospital, Italy School of Specialization in Pediatrics, University of Parma, Italy General and Emergency Pediatrics Unit, Parma General and University Hospital, Italy Department of Medicine and Surgery, University of Parma, Italy AU - Bertoncelli, D. AU - Guidarini, M. AU - Della Greca, A. AU - Ratti, C. AU - Falcinella, F. AU - Iovane, B. AU - Luigi Dutto, M. AU - Caffarelli, C. AU - Tchana, B. DB - Scopus DO - 10.23750/abm.v91i2.9655 IS - 2 KW - Cardiovascular Children Congenital heart diseases COVID-19 Kawasaki disease Myocarditis M3 - Article N1 - Export Date: 15 June 2020 PY - 2020 SP - 177-183 ST - Covid19: Potential cardiovascular issues in pediatric patients T2 - Acta Biomedica TI - Covid19: Potential cardiovascular issues in pediatric patients UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85084943709&doi=10.23750%2fabm.v91i2.9655&partnerID=40&md5=34207f2281f0c97d190fed7928d6e918 VL - 91 ID - 1714 ER - TY - JOUR AB - Plastic bronchitis is a rare life-threatening complication of the Fontan operation. Transcatheter Fontan fenestration can ameliorate symptoms by decompressing elevated venous pressures. Transcatheter creation of a fenestration can be technically challenging in cases with complex venous anatomy. We report a case of a 5-year-old boy with heterotaxy, dextrocardia with unbalanced atrioventricular canal (AVC), atrial and visceral situs inversus, left-sided superior vena cava (SVC), and left-sided interrupted inferior vena cava (IVC) with azygos continuation. With few modifications to the equipment, a successful Fontan fenestration with stent implantation was performed via transjugular approach. At 2-year follow-up, his symptoms of plastic bronchitis improved significantly. AD - Department of Pediatrics, Division of Cardiology, Riley Hospital for Children, Indiana University School of Medicine, 705 Riley Hospital Drive RR127, Indianapolis, IN 46202, United States AU - Charlagorla, P. AU - Breinholt, J. P. DB - Scopus DO - 10.4103/0974-2069.173547 IS - 2 KW - Fenestration heterotaxy lateral tunnel Fontan plastic bronchitis transjugular approach M3 - Article N1 - Export Date: 15 June 2020 PY - 2016 SP - 179-182 ST - Creating a Fontan fenestration in a child with dextrocardia and interrupted inferior vena cava T2 - Annals of Pediatric Cardiology TI - Creating a Fontan fenestration in a child with dextrocardia and interrupted inferior vena cava UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84968867604&doi=10.4103%2f0974-2069.173547&partnerID=40&md5=a80c777bb2341a39dc6b3c7aa0061f47 VL - 9 ID - 1905 ER - TY - JOUR AB - OBJECTIVES: To determine the gross motor skills of school-aged children after the Fontan procedure and compare the locomotor and object control skills with normative data. Study design This study followed a cross-sectional design. SETTING: This study was based on hospital outpatient visit, with accelerometry conducted at home. Patients This study included 55 patients, including 22 girls in the age group of 6-10 years, 5.1 years after Fontan. Main outcome measures Test of Gross Motor Development - Version 2, daily activity by accelerometer, medical history review, child and parent perceptions of activity. RESULTS: Being involved in active team sports increased locomotor percentile score by 10.3 points (CI: 4.4, 16.1). Preference for weekend outdoor activities (6.9, CI: 2.0, 11.8), performing at least 30 minutes of moderate-to-vigorous physical activity daily (24.5, CI: 7.3, 41.8), and reporting that parents seldom criticise the child's physical activity (21.8, CI: 8.9, 34.8) were also associated with higher locomotor percentile scores (p < 0.01). Object control percentile scores were higher (p < 0.03) with involvement in formal instruction (5.9, CI: 1.1, 10.6) and being restricted to "activities within comfortable limits" (27.6, CI: 7.7, 47.5). Older chronological age (r = 0.28), a more complicated medical history (r = 0.36), and older age at Fontan (r = 0.28) were associated with greater skill delay (p < 0.04). CONCLUSIONS: Children after Fontan attain basic motor skills at a later age than their peers, and deficits continue for more complex skills as age increases, suggesting a need for longitudinal monitoring of gross motor skill development through the elementary school years. Future research might investigate whether a gross motor skill rehabilitation programme can provide these children with the motor skills needed to successfully participate in a physically active lifestyle with peers. AD - Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Ontario, Canada. plongmuir@cheo.on.ca AN - 22269013 AU - Longmuir, P. E. AU - Banks, L. AU - McCrindle, B. W. DA - Aug DB - PubMed DO - 10.1017/s104795111100206x DP - NLM ET - 2012/01/25 IS - 4 KW - Accelerometry Child *Child Development Child, Preschool Cross-Sectional Studies Developmental Disabilities/*physiopathology Female *Fontan Procedure Heart Defects, Congenital/*surgery Humans Locomotion Male Motor Activity *Motor Skills Sedentary Behavior LA - eng N1 - 1467-1107 Longmuir, Patricia E Banks, Laura McCrindle, Brian W Canadian Institutes of Health Research/Canada Journal Article Research Support, Non-U.S. Gov't England Cardiol Young. 2012 Aug;22(4):443-50. doi: 10.1017/S104795111100206X. Epub 2012 Jan 24. PY - 2012 SN - 1047-9511 SP - 443-50 ST - Cross-sectional study of motor development among children after the Fontan procedure T2 - Cardiol Young TI - Cross-sectional study of motor development among children after the Fontan procedure VL - 22 ID - 237 ER - TY - JOUR AB - Patients with Crouzon and acanthosis nigricans syndrome show craniofacial features similar to those observed in patients with classic Crouzon syndrome, in addition to acanthosis nigricans with peculiar characteristics. More severe physical manifestations, such as Chiari malformation, hydrocephalus, and atresia or stenosis of the choanas, which are unusual in individuals with classic Crouzon syndrome, are reported in these patients. The molecular abnormality associated with Crouzon syndrome with acanthosis nigricans (CAN) is a transition in the transmembrane domain of the FGFR3 gene that results in an Ala391Glu mutation. We describe two unrelated patients showing this mutation and compare their clinical features with those of other patients with CAN reported in the literature. In addition to craniosynostosis with crouzonoid facies and acanthosis nigricans (present in all patients), melanocytic nevi, choanal atresia or stenosis, hydrocephalus, Chiari malformations and oral abnormalities were observed in the majority of the 35 patients analyzed. Vertebral anomalies and conductive hearing loss were present with less frequency. Some characteristics considered typical of this condition (jaw cementomas, acanthomas and finger abnormalities) were absent in most of the patients. © 2007 The Authors Journal compilation © 2007 Blackwell Munksgaard. AD - P. Barros-Núñez, Sierra Mojada 800, 44340 Guadalajara, Jalisco, Mexico AU - Arnaud-López, L. AU - Fragoso, J. AU - Mantilla-Capacho, J. AU - Barros-Núñez, P. DB - Embase Medline DO - 10.1111/j.1399-0004.2007.00884.x IS - 5 KW - fibroblast growth factor receptor 3 membrane protein acanthoma acanthosis nigricans adolescent article brachycephaly case report Chiari Frommel syndrome clinical feature computer assisted tomography conduction deafness craniofacial synostosis Crouzon syndrome Crouzon syndrome with acanthosis nigricans differential diagnosis exophthalmos female gene mutation genetic screening human hydrocephalus hypertelorism hypochondroplasia hypopigmentation hypoplastic left heart syndrome kidney failure pigmented nevus membranous glomerulonephritis mental deficiency mouth malformation preschool child priority journal skeleton malformation thorax radiography vertebra malformation LA - English M3 - Article N1 - L47570693 2007-12-04 PY - 2007 SN - 0009-9163 1399-0004 SP - 405-410 ST - Crouzon with acanthosis nigricans. Further delineation of the syndrome T2 - Clinical Genetics TI - Crouzon with acanthosis nigricans. Further delineation of the syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L47570693 http://dx.doi.org/10.1111/j.1399-0004.2007.00884.x VL - 72 ID - 1224 ER - TY - JOUR AB - A 17-year-old patient with tricuspid atresia, palliatively corrected by a right Glenn and a left Potts anastomosis during his early childhood and a banding of the left pulmonary artery 6 years ago, developed a false aneurysm of the left pulmonary artery secondary to the erosion of the band fabric through the vascular wall with subsequent thrombosis, infection, and leakage. The diagnosis was confused by both chest roentgenography and angiography but was accurately made by postcontrast computed tomography (CT). We stress the importance and usefulness of CT in the diagnosis of a false aneurysm, not demonstrated by angiography. AD - Dep. Diagn. Radiol., Sch. Med., Univ. Alabama, Birmingham, AL 35233 AU - Shin, M. S. AU - Ceballos, R. AU - Bini, R. M. AU - Ho, K. J. DB - Embase Medline IS - 3 KW - adolescent case report computer analysis computer assisted tomography congenital disorder diagnosis false aneurysm great blood vessel heart human lung artery banding peripheral vascular system therapy tricuspid valve atresia LA - English M3 - Article N1 - L13058292 1983-08-10 PY - 1983 SN - 0363-8715 SP - 524-526 ST - CT Diagnosis of false aneurysm of the pulmonary artery not demonstrated by angiography: Case report T2 - Journal of Computer Assisted Tomography TI - CT Diagnosis of false aneurysm of the pulmonary artery not demonstrated by angiography: Case report UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L13058292 VL - 7 ID - 1398 ER - TY - JOUR AB - Over a decade ago, I co-authored a review in Cardiology in the Young regarding neurological outcomes following surgery for congenital cardiac disease. In that review, I placed much emphasis on the conduct of cardiopulmonary bypass, and its role in neurodevelopmental disabilities. Much has been learned in the intervening years regarding the multifactorial causes of abnormal school-age development, in particular, the role of prenatal, perioperative, socioeconomic, and genetic influences. In this update, I will highlight some of the recent advances in our understanding of the protean causes of neurological, behavioral, and developmental abnormalities in children and young adults with complex forms of congenital cardiac disease. In addition, I will summarize the current data on patients at particular high-risk for adverse neurodevelopmental outcomes, specifically those with a functionally univentricular heart who have had staged reconstruction with ultimate conversion to the Fontan circulation. © 2006 Cambridge University Press. AD - G. Wernovsky, Division of Pediatric Cardiology, The Cardiac Center, The Children's Hospital of Philadelphia, 34th Street/Civic Center Boulevard, Philadelphia, PA 19104, United States AU - Wernovsky, G. DB - Embase DO - 10.1017/S1047951105002398 IS - SUPPL. 1 KW - academic achievement adult article attention brain malformation cardiopulmonary bypass central nervous system disease child child behavior clinical study congenital heart disease developmental disorder encephalomalacia environmental factor experimental design fetus circulation Fontan procedure handwriting heart arrest heredity hospital patient human hypothermia intensive care intraoperative period laboratory test longitudinal study mental function microcephaly neurologic disease outpatient care postoperative period preoperative period prevalence quality of life reoperation risk assessment surgical risk treatment outcome LA - English M3 - Article N1 - L44375396 2006-10-10 PY - 2006 SN - 1047-9511 1467-1107 SP - 92-104 ST - Current insights regarding neurological and developmental abnormalities in children and young adults with complex congenital cardiac disease T2 - Cardiology in the Young TI - Current insights regarding neurological and developmental abnormalities in children and young adults with complex congenital cardiac disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L44375396 http://dx.doi.org/10.1017/S1047951105002398 VL - 16 ID - 1265 ER - TY - JOUR AB - Children with single ventricle anatomy are among the most complicated and challenging patients encountered in pediatric cardiology. Current management involves staged surgical procedures, beginning with neonatal palliation and followed by a bidirectional cavopulmonary anastomosis in infancy and culminating in the Fontan procedure. The Fontan procedure, despite separating the circulation, remains a palliative procedure with many long-term concerns. This report discusses the staged surgical management of patients with single ventricle anatomy and the nursing issues relevant to each stage. AD - Cardiovascular Program, Children's Hospital, Boston, MA 02115, USA. patricia.obrien@tch.harvard.edu AN - 11598866 AU - O'Brien, P. AU - Boisvert, J. T. DA - Oct DB - PubMed DO - 10.1053/jpdn.2001.26573 DP - NLM ET - 2001/10/13 IS - 5 KW - Child Child, Preschool Congenital Abnormalities/nursing/physiopathology/surgery Fontan Procedure/adverse effects/*methods/*nursing Heart Bypass, Right/adverse effects/*methods/nursing Heart Ventricles/*abnormalities Humans Infant Infant, Newborn Long-Term Care/methods Palliative Care/*methods Parents/education/psychology Pediatric Nursing/methods Perioperative Care/methods/nursing Pulmonary Circulation Social Support LA - eng N1 - O'Brien, P Boisvert, J T Journal Article Review United States J Pediatr Nurs. 2001 Oct;16(5):338-50. doi: 10.1053/jpdn.2001.26573. PY - 2001 SN - 0882-5963 (Print) 0882-5963 SP - 338-50 ST - Current management of infants and children with single ventricle anatomy T2 - J Pediatr Nurs TI - Current management of infants and children with single ventricle anatomy VL - 16 ID - 240 ER - TY - JOUR AB - BACKGROUND: Re-establishment of Fontan circulation by eliminating the drawbacks of classic Fontan modifications has been proposed recently to improve the functional class and quality of life of patients with failed Fontan circulation. METHODS: Five patients underwent extracardiac and lateral tunnel Fontan conversion due to failure of the Fontan circulation, after classic Fontan modifications. All of the patients were female and mean age was 10.2 +/- 2.2 years (range: 8-14 years). Previous Fontan modifications were atrio-pulmonary anastomosis in 3 and Kawashima operation in 2 patients. Time to re-operation for Fontan conversion was 6 +/- 1.5 years (range: 4-8 years). Indications for re-operation were right heart failure, right atrial dilatation, and intractable atrial arrhythmias in patients with previous atrio-pulmonary anastomosis, progressive hypoxia and exercise intolerance in patients with previous Kawashima operation due to pulmonary arteriovenous fistulas. Previous atrio-pulmonary anastomoses were converted to intra-atrial or lateral tunnel Fontan operation with modified right atrial Maze procedure. Extracardiac Fontan completion was carried out after previous Kawashima operations by redirection of hepatic veins to the lungs. RESULTS: There was no mortality and no major morbidity. All patients were discharged from the hospital in good condition and in sinus rhythm. No prolonged or recurrent effusions were observed. On follow-up, all patients were in sinus rhythm and had NYHA class I functional capacity. In two Kawashima patients, SpO(2) gradually increased from 60 % to 90 % six months after the operation. DISCUSSION: We suggest that Fontan conversion should be considered in patients with previous atrio-pulmonary anastomosis, when right atrial dilatation or intractable atrial arrhythmias with deteriorating functional status develops. Redirection of hepatic venous flow to lungs induces regression of pulmonary arteriovenous fistulas and improves arterial saturation in patients with previous Kawashima operation. AD - Acibadem Hospital Bakirkoy, Department of Cardiovascular Surgery, Bakirkoy, Istanbul, Turkey. AN - 16485184 AU - Erek, E. AU - Yalcinbas, Y. K. AU - Sarioglu, A. AU - Sarioglu, T. DA - Feb DB - PubMed DO - 10.1055/s-2005-865872 DP - NLM ET - 2006/02/18 IS - 1 KW - Adolescent Anastomosis, Surgical Arteriovenous Fistula/surgery Child Dilatation, Pathologic/etiology/surgery Exercise Tolerance Female Follow-Up Studies Fontan Procedure/*adverse effects/*methods Heart Atria/abnormalities/surgery Heart Failure/etiology/surgery Humans Hypoxia/etiology/surgery Length of Stay Pulmonary Veins/abnormalities/surgery Quality of Life Reoperation Tachycardia/etiology/surgery Time Factors Treatment Failure Vena Cava, Superior/surgery LA - eng N1 - Erek, E Yalcinbas, Y K Sarioglu, A Sarioglu, T Journal Article Germany Thorac Cardiovasc Surg. 2006 Feb;54(1):21-5. doi: 10.1055/s-2005-865872. PY - 2006 SN - 0171-6425 (Print) 0171-6425 SP - 21-5 ST - Current management of late failure after classic Fontan modifications: Fontan conversion T2 - Thorac Cardiovasc Surg TI - Current management of late failure after classic Fontan modifications: Fontan conversion VL - 54 ID - 336 ER - TY - JOUR AB - Background: Re-establishment of Fontan circulation by eliminating the drawbacks of classic Fontan modifications has been proposed recently to improve the functional class and quality of life of patients with failed Fontan circulation. Methods: Five patients underwent extracardiac and lateral tunnel Fontan conversion due to failure of the Fontan circulation, after classic Fontan modifications. All of the patients were female and mean age was 10.2 ± 2.2 years (range: 8-14 years). Previous Fontan modifications were atriopulmonary anastomosis in 3 and Kawashima operation in 2 patients. Time to re-operation for Fontan conversion was 6 ± 1.5 years (range: 4-8 years). Indications for re-operation were right heart failure, right atrial dilatation, and intractable atrial arrhythmias in patients with previous atriopulmonary anastomosis, progressive hypoxia and exercise intolerance in patients with previous Kawashima operation due to pulmonary arteriovenous fistulas. Previous atriopulmonary anastomoses were converted to intra-atrial or lateral tunnel Fontan operation with modified right atrial Maze procedure. Extracardiac Fontan completion was carried out after previous Kawashima operations by redirection of hepatic veins to the lungs. Results: There was no mortality and no major morbidity. All patients were discharged from the hospital in good condition and in sinus rhythm. No prolonged or recurrent effusions were observed. On follow-up, all patients were in sinus rhythm and had NYHA class I functional capacity. In two Kawashima patients, SpO2 gradually increased from 60% to 90% six months after the operation. Discussion: We suggest that Fontan conversion should be considered in patients with previous atriopulmonary anastomosis, when right atrial dilatation or intractable atrial arrhythmias with deteriorating functional status develops. Redirection of hepatic venous flow to lungs induces regression of pulmonary arteriovenous fistulas and improves arterial saturation in patients with previous Kawashima operation. © Georg Thieme Verlag KG. AD - T. Sarioglu, Department of Cardiovascular Surgery, Acibadem Hospital Bakirkoy, Halit Ziya Usakligil Cad. No.1, 34140 Bakirkoy, Istanbul, Turkey AU - Erek, E. AU - Yalcinbas, Y. K. AU - Sarioglu, A. AU - Sarioglu, T. DB - Embase Medline DO - 10.1055/s-2005-865872 IS - 1 KW - adolescent arterial oxygen tension arteriovenous fistula article clinical article deterioration exercise tolerance female Fontan procedure heart atrium arrhythmia heart atrium enlargement heart failure heart function heart muscle ischemia heart rhythm human kawashima operation liver vein lung blood flow morbidity mortality priority journal quality of life reoperation school child surgical technique treatment indication vein blood flow LA - English M3 - Article N1 - L43232139 2006-03-02 PY - 2006 SN - 0171-6425 SP - 21-25 ST - Current management of late failure after classic Fontan modifications: Fontan conversions T2 - Thoracic and Cardiovascular Surgeon TI - Current management of late failure after classic Fontan modifications: Fontan conversions UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L43232139 http://dx.doi.org/10.1055/s-2005-865872 VL - 54 ID - 1266 ER - TY - JOUR AB - Objective: Tremendous strides have been made in the outcomes for hypoplastic left heart syndrome and other functional single-ventricle malformations over the past 25 years. This progress relates primarily to improvements in survival for patients undergoing the Norwood procedure. Previous reports on risk factors have been on smaller groups of patients or collected over relatively long periods of time, during which management has evolved. We analyzed our current results for the Norwood procedure with attention to risk factors for poor outcome. Methods: A single-institution review of all patients undergoing a Norwood procedure for a single-ventricle malformation from May 1, 2001, through April 30, 2003, was performed. Patient demographics, anatomy, clinical condition, associated anomalies, operative details, and outcomes were recorded. Results: Of the 111 patients, there were 23 (21%) hospital deaths. Univariate analysis revealed noncardiac abnormalities (genetic or significant extracardiac diagnosis, P = .0018), gestational age (P = .03), diagnosis of unbalanced atrioventricular septal defect (P = .017), and weight of less than 2.5 kg (P = .0072) to be related to hospital death. On multivariate analysis, only weight of less than 2.5 kg and noncardiac abnormalities were found to be independent risk factors. Patients with either of these characteristics had a hospital survival of 52% (12/23), whereas those at standard risk had a survival of 86% (76/88). Conclusions: Although improvements in management might have lessened the effect of some of the traditionally reported risk factors related to variations in the cardiovascular anatomy, noncardiac abnormalities and low birth weight remain as a future challenge for the physician caring for the patient with single-ventricle physiology. Copyright © 2006 by The American Association for Thoracic Surgery. AD - R.G. Ohye, F7830 Mott Hospital, 1500 East Medical Center Dr, Ann Arbor, MI 48109-0223, United States AU - Stasik, C. N. AU - Goldberg, C. S. AU - Bove, E. L. AU - Devaney, E. J. AU - Ohye, R. G. DB - Embase Medline DO - 10.1016/j.jtcvs.2005.09.030 IS - 2 KW - Alagille syndrome bile duct atresia body weight brain artery aneurysm cerebrovascular accident controlled study corpus callosum agenesis Costello syndrome death demography female gestational age heart atrium septum defect heart single ventricle human kidney dysplasia kidney failure lung dysplasia major clinical study male McKusick Kaufman syndrome newborn Norwood procedure priority journal review risk factor treatment outcome Turner syndrome LA - English M3 - Article N1 - L43121565 2006-02-08 PY - 2006 SN - 0022-5223 SP - 412-417 ST - Current outcomes and risk factors for the Norwood procedure T2 - Journal of Thoracic and Cardiovascular Surgery TI - Current outcomes and risk factors for the Norwood procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L43121565 http://dx.doi.org/10.1016/j.jtcvs.2005.09.030 VL - 131 ID - 1270 ER - TY - JOUR AB - Pediatric heart transplantation is standard of care for children with end-stage heart failure. The diverse age range, diagnoses, and practice variations continue to challenge the development of evidence-based practices and new technologies. Outcomes in the most recent era are excellent, especially with the more widespread use of ventricular assist devices (VADs). Waitlist mortality remains high and knowledge of risk factors for death while waiting and following transplantation contributes to decision-making around transplant candidacy and timing of listing. The biggest gap impacting both waitlist and overall survival remains mechanical support options for infants and patients with single ventricle physiology. Though acute rejection has decreased progressively, both diagnosis and management of antibody-mediated rejection has become increasingly challenging and complex, as has the ability to understand the implication of anti-HLA antibodies detected both pre- and post-transplantation-including when and how to intervene. Trends in immunosuppression protocols include more use of induction therapy and steroid avoidance or withdrawal protocols. Common long-term morbidities include renal insufficiency, which can be mitigated with surveillance and renal-sparing strategies, and infections. Functional outcomes are excellent, but significant psychosocial challenges exist in relation to neurodevelopment, non-adherence, and transition from child-centered to adult-centered care. Cardiac allograft vasculopathy (CAV) remains a barrier to long-term survival, though it is more apparent that objective evidence of an impact on the allograft is important with regards to impact on outcomes. Retransplantation is rare in pediatric heart transplant recipients. Pediatric heart transplantation continues to evolve in order to address the challenges of the diverse group of patients that reach end-stage heart failure during childhood. AD - Labatt Family Heart Centre, Hospital for Sick Children, University of Toronto, Toronto, Canada. AN - 29492382 AU - Dipchand, A. I. C2 - PMC5827130 DA - Jan DB - PubMed DO - 10.21037/acs.2018.01.07 DP - NLM ET - 2018/03/02 IS - 1 KW - Heart transplant mechanical support outcomes sensitization waitlist LA - eng N1 - 2304-1021 Dipchand, Anne I Journal Article Review Ann Cardiothorac Surg. 2018 Jan;7(1):31-55. doi: 10.21037/acs.2018.01.07. PY - 2018 SN - 2225-319X (Print) 2225-319x SP - 31-55 ST - Current state of pediatric cardiac transplantation T2 - Ann Cardiothorac Surg TI - Current state of pediatric cardiac transplantation VL - 7 ID - 60 ER - TY - JOUR AB - Background: Current surgical outcomes of congenital heart surgery for patients with Down syndrome are unclear. Methods and Results: Of 29,087 operations between 2008 and 2012 registered in the Japan Congenital Cardiovascular Surgery Database (JCCVSD), 2,651 were carried out for patients with Down syndrome (9%). Of those, 5 major biventricular repair procedures [ventricular septal defect repair (n=752), atrioventricular septal defect repair (n=452), patent ductus arteriosus closure (n=184), atrial septal defect repair (n=167), tetralogy of Fallot (TOF) repair (n=108)], as well as 2 major single ventricular palliations [bidirectional Glenn (n=21) and Fontan operation (n=25)] were selected and their outcomes were compared. The 90-day and in-hospital mortality rates for all 5 major biventricular repair procedures and bidirectional Glenn were similarly low in patients with Down syndrome compared with patients without Down syndrome. On the other hand, mortality after Fontan operation in patients with Down syndrome was significantly higher than in patients without Down syndrome (42/1,558=2.7% vs. 3/25=12.0%, P=0.005). Conclusions: Although intensive management of pulmonary hypertension is essential, analysis of the JCCVSD revealed favorable early prognostic outcomes after 5 major biventricular procedures and bidirectional Glenn in patients with Down syndrome. Indication of the Fontan operation for patients with Down syndrome should be carefully decided. AD - T. Hoashi, The Japan Cardiovascular Surgery Database Organization, 5-7-1 Fujishirodai, Suita, Japan AU - Hoashi, T. AU - Hirahara, N. AU - Murakami, A. AU - Hirata, Y. AU - Ichikawa, H. AU - Kobayashi, J. AU - Takamoto, S. DB - Embase Medline DO - 10.1253/circj.CJ-17-0483 IS - 2 KW - article artificial ventilation atrioventricular septal defect catheterization child Down syndrome Fallot tetralogy female heart atrium septum defect heart surgery heart ventricle septum defect home oxygen therapy hospital discharge hospital mortality hospital readmission human length of stay lung vascular resistance major clinical study male multicenter study outcome assessment patent ductus arteriosus pH postoperative period pulmonary hypertension retrospective study surgical mortality LA - English M3 - Article N1 - L620378046 2018-02-01 2018-02-09 PY - 2018 SN - 1347-4820 1346-9843 SP - 403-408 ST - Current surgical outcomes of congenital heart surgery for patients with down syndrome in Japan T2 - Circulation Journal TI - Current surgical outcomes of congenital heart surgery for patients with down syndrome in Japan UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L620378046 http://dx.doi.org/10.1253/circj.CJ-17-0483 VL - 82 ID - 719 ER - TY - JOUR AB - Objective: Neonates with profound heart failure resulting from Ebstein anomaly have historically had poor outcomes. We report our institutional experience with the surgical management of Ebstein anomaly in severely symptomatic neonates. Methods: A retrospective review of all patients (n = 16) undergoing neonatal intervention for Ebstein anomaly between 1992 and 2005 has been carried out. The indications for operation were overt heart failure, cyanosis, and acidosis associated with tricuspid regurgitation, depressed right ventricular function, and severe cardiomegaly. The magnitude of cardiac enlargement was assessed by cardiothoracic ratio and Great Ormond Street ratio (area of right atrium + atrialized right ventricle/area of functional left atrium + left ventricle). The operative strategy was first to assess for the possibility of tricuspid valve repair with or without right ventricular outflow tract reconstruction. If this was not feasible, then right ventricular exclusion was performed by oversewing the tricuspid valve with a pericardial patch. A reduction atrioplasty was done and, depending on the extent of the atrialized portion of the right ventricle, plication was performed. A modified Blalock-Taussig shunt provided pulmonary blood flow. This univentricular approach (Starnes procedure) evolved to include a fenestration in the tricuspid valve patch to allow for right ventricular decompression. Analysis included overall and group-specific survival as well as the testing of perioperative clinical, morphologic, and surgical variables for correlation with mortality and morbidity. Results: Mean age and weight at operation were 8 ±10 days and 3.1 ± 0.4 kg. Tricuspid valve repair was undertaken in 3 patients with 1 requiring conversion to right ventricular exclusion 3 months after the initial operation. In those with right ventricular exclusion, the tricuspid valve patch was fenestrated in 10 and nonfenestrated in 3. One patient had heart transplant as the initial procedure. There were 5 hospital deaths (31%) and no late deaths among the survivors. Survival in the cohort with a fenestrated tricuspid valve patch was 80% (8/10) versus 33% (1/3) for the nonfenestrated group. This difference did not reach statistical significance, although the trend seems clinically important. There was no difference in the cardiothoracic ratio (0.82 fenestrated vs 0.84 nonfenestrated: P = .802) or the Great Ormond Street ratio (1.2 fenestrated vs 1.02 nonfenestrated: P = .477) between the two groups. Among the 9 survivors of right ventricular exclusion, 3 have had completion of their Fontan, and all 9 have undergone a bidirectional Glenn procedure. All operations including homograft placement in the right ventricular outflow tract, whether during repair or during right ventricular exclusion, ended in death. Conclusion: Right ventricular exclusion with a fenestrated tricuspid valve patch combined with right atrioplasty and right ventriculoplasty and a Blalock-Taussig shunt (Starnes procedure) has provided effective palliation for neonates presenting with critical Ebstein anomaly and a tricuspid valve that cannot be repaired. © 2006 The American Association for Thoracic Surgery. AD - B.L. Reemtsen, Children's Hospital Los Angeles, the Keck School of Medicine, Los Angeles, CA, United States AU - Reemtsen, B. L. AU - Fagan, B. T. AU - Wells, W. J. AU - Starnes, V. A. DB - Embase Medline DO - 10.1016/j.jtcvs.2006.08.044 IS - 6 KW - acidosis article Blalock Taussig shunt cardiomegaly clinical article controlled study correlation analysis cyanosis decompression surgery disease severity Ebstein anomaly female fenestration Fontan procedure Glenn shunt heart atrioplasty heart failure heart left atrium heart left ventricle heart right atrium heart right ventricle heart right ventricle failure heart right ventricle outflow tract heart right ventricle outflow tract obstruction heart surgery heart valve surgery heart ventriculoplasty human male newborn newborn surgery overall survival perioperative period postoperative period priority journal Starnes procedure surgical technique survival rate symptom treatment indication treatment outcome tricuspid valve regurgitation LA - English M3 - Article N1 - L44820240 2006-12-27 PY - 2006 SN - 0022-5223 SP - 1285-1289 ST - Current surgical therapy for Ebstein anomaly in neonates T2 - Journal of Thoracic and Cardiovascular Surgery TI - Current surgical therapy for Ebstein anomaly in neonates UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L44820240 http://dx.doi.org/10.1016/j.jtcvs.2006.08.044 VL - 132 ID - 1246 ER - TY - JOUR AB - Universally fatal only 4 decades ago, the progress in the 3-stage palliation of hypoplastic left heart syndrome and related single right ventricular lesions has drastically improved the outlook for these patients. Although the stage II operation (hemi-Fontan or bidirectional Glenn) and stage III Fontan procedure have evolved into relatively low-risk operations, the stage I Norwood procedure remains one of the highest-risk and costliest common operations performed in congenital heart surgery. Yet, despite this fact, experienced centers now report hospital survivals of >90% for the Norwood procedure. This traditional 3-stage surgical palliation has seen several innovations in the past decade aimed at improving outcomes, particularly for the Norwood procedure. One significant change is a renewed interest in the right ventricle-to-pulmonary artery shunt as the source of pulmonary blood flow, rather than the modified Blalock-Taussig shunt for the Norwood. The multi-institutional Single Ventricle Reconstruction trial randomly assigned 555 patients to one or the other shunt, and these subjects continue to be followed closely as they now approach 10 years postrandomization. In addition to modifications to the Norwood procedure, the hybrid procedure, a combined catheter-based and surgical approach, avoids the Norwood procedure in the newborn period entirely. The initial hybrid procedure is then followed by a comprehensive stage II, which combines components of both the Norwood and the traditional stage II, and later completion of the Fontan. Proponents of this approach hope to improve not only short-term survival, but also potentially longer-term outcomes, such as neurodevelopment, as well. Regardless of the approach, traditional surgical staged palliation or the hybrid procedure, survivals have vastly improved, and large numbers of these patients are surviving not only through their Fontan in early childhood, but also into adolescence and young adulthood. As this population grows, it becomes increasingly important to understand the longer-term outcomes of these Fontan patients, not only in terms of survival, but also in terms of the burden of disease, neurodevelopmental outcomes, psychosocial development, and quality of life. AD - From University of Michigan C. S. Mott Children's Hospital, Ann Arbor (R.G.O.); Pediatric Heart Center, Justus Liebig University Giessen, Germany (D.S.); and Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia (Y.D'U.). ohye@umich.edu. From University of Michigan C. S. Mott Children's Hospital, Ann Arbor (R.G.O.); Pediatric Heart Center, Justus Liebig University Giessen, Germany (D.S.); and Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia (Y.D'U.). AN - 27777296 AU - Ohye, R. G. AU - Schranz, D. AU - D'Udekem, Y. C2 - PMC5119545 C6 - NIHMS818228 DA - Oct 25 DB - PubMed DO - 10.1161/circulationaha.116.022816 DP - NLM ET - 2016/10/26 IS - 17 KW - Disease-Free Survival Female *Fontan Procedure *Heart Ventricles/physiopathology/surgery Humans *Hypoplastic Left Heart Syndrome/mortality/pathology/physiopathology/surgery Male Multicenter Studies as Topic Randomized Controlled Trials as Topic Survival Rate *congenital *heart defects *heart ventricles *hybrid imaging *hypoplastic left heart syndrome *surgery LA - eng N1 - 1524-4539 Ohye, Richard G Schranz, Dietmar D'Udekem, Yves U01 HL068269/HL/NHLBI NIH HHS/United States U01 HL068279/HL/NHLBI NIH HHS/United States U01 HL068290/HL/NHLBI NIH HHS/United States U01 HL068288/HL/NHLBI NIH HHS/United States U10 HL068270/HL/NHLBI NIH HHS/United States U01 HL068281/HL/NHLBI NIH HHS/United States U01 HL068270/HL/NHLBI NIH HHS/United States U10 HL109737/HL/NHLBI NIH HHS/United States U01 HL068292/HL/NHLBI NIH HHS/United States U01 HL085057/HL/NHLBI NIH HHS/United States U01 HL068285/HL/NHLBI NIH HHS/United States Journal Article Review Circulation. 2016 Oct 25;134(17):1265-1279. doi: 10.1161/CIRCULATIONAHA.116.022816. PY - 2016 SN - 0009-7322 (Print) 0009-7322 SP - 1265-1279 ST - Current Therapy for Hypoplastic Left Heart Syndrome and Related Single Ventricle Lesions T2 - Circulation TI - Current Therapy for Hypoplastic Left Heart Syndrome and Related Single Ventricle Lesions VL - 134 ID - 5 ER - TY - JOUR AB - In October 2017, a pediatric heart transplant summit was held in Seattle-the first of its kind internationally-which focused solely upon controversies in pediatric end-stage heart failure management and pediatric heart transplantation. We selected five of the most popular and contentious topics and asked the speakers to provide a position paper. Worldwide, the vast majority of programs perform only a handful of pediatric heart transplants a year. Because of this, these "orphan" areas of investigation provide an opportunity for us as a community to aggregate our collective knowledge, which may represent the only viable way that we might sort through these complex and controversial issues in the field. We hope this represents the first of many such conferences and that this initial selection of papers encourages us to begin this collaborative process. AD - 1 Cardiothoracic Surgery, Seattle Children's Hospital, University of Washington, Seattle, WA, USA. 2 Washington University School of Medicine, St Louis, MO, USA. 3 Children's Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, NY, USA. 4 Herma Heart Institute, Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI, USA. 5 Pediatrics, Seattle Children's Hospital, University of Washington, Seattle, WA, USA. 6 Clinical Psychology, Seattle Children's Hospital, University of Washington, Seattle, WA, USA. 7 Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA. 8 C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI, USA. AN - 30157743 AU - Chen, J. M. AU - Canter, C. E. AU - Hsu, D. T. AU - Kindel, S. J. AU - Law, Y. M. AU - McKeever, J. E. AU - Pahl, E. AU - Schumacher, K. R. DA - Sep DB - PubMed DO - 10.1177/2150135118782895 DP - NLM ET - 2018/08/31 IS - 5 KW - Child *Congresses as Topic Heart Defects, Congenital/*surgery Heart Transplantation/*methods Humans United States *Fontan *congenital heart disease (CHD) *heart *quality of life *transplantation LA - eng N1 - 2150-136x Chen, Jonathan M Canter, Charles E Hsu, Daphne T Kindel, Steven J Law, Yuk M McKeever, James E Pahl, Elfriede Schumacher, Kurt R Journal Article Review United States World J Pediatr Congenit Heart Surg. 2018 Sep;9(5):575-581. doi: 10.1177/2150135118782895. PY - 2018 SN - 2150-1351 SP - 575-581 ST - Current Topics and Controversies in Pediatric Heart Transplantation: Proceedings of the Pediatric Heart Transplantation Summit 2017 T2 - World J Pediatr Congenit Heart Surg TI - Current Topics and Controversies in Pediatric Heart Transplantation: Proceedings of the Pediatric Heart Transplantation Summit 2017 VL - 9 ID - 105 ER - TY - JOUR AB - In the past two decades, long-term survival of patients with cyanotic congenital heart disease has dramatically improved. After successful intracardiac repair, many patients can anticipate a normal lifestyle. Health care providers must increase their knowledge of the nature and effects of surgical intervention, the presence, type, and extent of the patient's postoperative residua and sequelae, and the patient's psychosocial adjustment. Prompt surgical intervention and comprehensive follow-up with early identification and treatment of problems will further enhance both survival of patients born with cyanotic heart disease and the quality of life they achieve in the future. AD - C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI AU - Uzark, K. DB - Embase Medline IS - 3 KW - child congenital cyanotic heart malformation congenital disorder cyanosis Ebstein anomaly Fallot tetralogy heart human lung blood flow peripheral vascular system respiratory system short survey therapy tricuspid valve atresia LA - English M3 - Article N1 - L14023000 1984-12-19 PY - 1984 SN - 0029-6465 SP - 503-519 ST - Cyanotic congenital heart disease with decreased pulmonary blood flow T2 - Nursing Clinics of North America TI - Cyanotic congenital heart disease with decreased pulmonary blood flow UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L14023000 VL - 19 ID - 1396 ER - TY - JOUR AB - Of the 'five T's' of cyanotic congenital heart disease-tetralogy of Fallot, TGA, TAPVC, truncus, and tricuspid valve abnormalities (tricuspid atresia, stenosis, and displacement)-the first and last are commonly associated with diminished PBF. The four features that comprise tetralogy of Fallot-right ventricular hypertrophy, VSD, overriding aorta, and subpulmonary stenosis-are all secondary to a single morphogenetic defect: failure of expansion of the subpulmonary conus. This also explains the variability in clinical presentation. When neonates need intervention, shunts are usually performed. Coronary arterial anatomy must be defined before repair, which is usually done after these infants are 3 months of age. Although children with repaired tetralogy of Fallot are not completely 'normal,' markedly increased longevity and improvement in quality of life can be achieved. When major associated defects are present, such as atrioventricular canal defect, diminutive pulmonary arteries or collateral vessels, or left heart lesions, the prognosis changes from excellent to merely good. Tetralogy of Fallot with absent pulmonary valve syndrome is physiologically different from other tetralogy of Fallot conditions and characterized primarily by airway obstruction from massive dilatation of the central and perihilar pulmonary arteries; repair with pulmonary artery reduction is necessary. Tricuspid valve abnormalities include atresia, hypoplasia (i.e., pulmonary atresia with intact ventricular septum), and displacement (i.e., Ebstein anomaly). The pathophysiology that dictates these children's clinical condition (and prognosis) relates to three factors: (1) status of the tricuspid valve, (2) presence and size of a VSD, and (3) TGA or normally related great arteries. Virtually all children with tricuspid valve abnormalities can be palliated; reparative options include repair using two-ventricle, one-ventricle, or 1- 1/2 ventricle repair. Children with critical pulmonary stenosis generally have a normal tricuspid valve and right ventricle. Balloon dilation is usually the only therapy necessary. AD - J.D. Waldman, Children's Hospital Heart Center, Univ. of New Mexico Hlth. Sci. Ctr., 2211 Lomas NE, Albuquerque, NM 87106, United States AU - Waldman, J. D. AU - Wernly, J. A. DB - Embase Medline DO - 10.1016/S0031-3955(05)70125-5 IS - 2 KW - prostaglandin E1 article cyanotic heart disease Ebstein anomaly echocardiography electrocardiogram Fallot tetralogy Fontan procedure heart catheterization interventricular septum human pulmonary valve atresia lung blood flow pulmonary valve stenosis right heart hypoplasia tricuspid valve atresia tricuspid valve stenosis vein anastomosis LA - English M3 - Article N1 - L29177708 1999-05-09 PY - 1999 SN - 0031-3955 SP - 385-404 ST - Cyanotic congenital heart disease with decreased pulmonary blood flow in children T2 - Pediatric Clinics of North America TI - Cyanotic congenital heart disease with decreased pulmonary blood flow in children UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L29177708 http://dx.doi.org/10.1016/S0031-3955(05)70125-5 VL - 46 ID - 1343 ER - TY - JOUR AB - Perturbed biomechanical stimuli are thought to be critical for the pathogenesis of a number of congenital heart defects, including Hypoplastic Left Heart Syndrome (HLHS). While embryonic cardiomyocytes experience biomechanical stretch every heart beat, their molecular responses to biomechanical stimuli during heart development are poorly understood. We hypothesized that biomechanical stimuli activate specific signaling pathways that impact proliferation, gene expression and myocyte contraction. The objective of this study was to expose embryonic mouse cardiomyocytes (EMCM) to cyclic stretch and examine key molecular and phenotypic responses. Analysis of RNA-Sequencing data demonstrated that gene ontology groups associated with myofibril and cardiac development were significantly modulated. Stretch increased EMCM proliferation, size, cardiac gene expression, and myofibril protein levels. Stretch also repressed several components belonging to the Transforming Growth Factor-β (Tgf-β) signaling pathway. EMCMs undergoing cyclic stretch had decreased Tgf-β expression, protein levels, and signaling. Furthermore, treatment of EMCMs with a Tgf-β inhibitor resulted in increased EMCM size. Functionally, Tgf-β signaling repressed EMCM proliferation and contractile function, as assayed via dynamic monolayer force microscopy (DMFM). Taken together, these data support the hypothesis that biomechanical stimuli play a vital role in normal cardiac development and for cardiac pathology, including HLHS. © 2014 Elsevier Ltd. AD - Department of Cardiology, University of California San Diego, 9500 Gilman Drive, La Jolla, CA 92093, United States Department of Pediatrics (Cardiology), University of California San Diego, United States Department of Mechanical and Aerospace Engineering, University of California San Diego, United States Biomedical Genomics Microarray Core Facility, University of California San Diego, United States Department of Medicine, University of California San Diego, 9500 Gilman Drive, La Jolla, CA 92093, United States Muscle Development and Regeneration Program, Sanford-Burnham Medical Research Institute, 10901 North Torrey Pines Road, La Jolla, CA 92037, United States Department of Pediatrics (Allergy), University of California San Diego, United States Rady Children's Hospital San Diego, United States Cell and Developmental Biology, University of California San Diego, United States School of Pharmacology Keck Graduate Institute, 9500 Gilman Drive, La Jolla, CA 92093, United States Department of Medicine, Medical University of South Carolina, 135 Cannon Street, Suite 303 MSC 835, Charleston, SC 29425, United States Institute for Engineering in Medicine, University of California San Diego, United States AU - Banerjee, I. AU - Carrion, K. AU - Serrano, R. AU - Dyo, J. AU - Sasik, R. AU - Lund, S. AU - Willems, E. AU - Aceves, S. AU - Meili, R. AU - Mercola, M. AU - Chen, J. AU - Zambon, A. AU - Hardiman, G. AU - Doherty, T. A. AU - Lange, S. AU - del Álamo, J. C. AU - Nigam, V. DB - Scopus DO - 10.1016/j.yjmcc.2014.11.003 KW - Cardiac development Cardiomyocytes Cell biology/structural biology Contractile function Contractility Gene regulation Hypoplastic Left Heart Syndrome Mechanical stretch Other myocardial biology Pediatric and congenital heart disease Physiologic and pathologic control of gene expression Subject Codes M3 - Article N1 - Cited By :22 Export Date: 15 June 2020 PY - 2015 SP - 133-144 ST - Cyclic stretch of embryonic cardiomyocytes increases proliferation, growth, and expression while repressing Tgf-β signaling T2 - Journal of Molecular and Cellular Cardiology TI - Cyclic stretch of embryonic cardiomyocytes increases proliferation, growth, and expression while repressing Tgf-β signaling UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84912018751&doi=10.1016%2fj.yjmcc.2014.11.003&partnerID=40&md5=93a962d6f6a7854b7c27e7efac07d36f VL - 79 ID - 1955 ER - TY - JOUR AB - We describe a 6-year-old male, diagnosed at birth with double outlet right ventricle (DORV), anterior aorta, multiple ventricular septal defects, pulmonary stenosis, microcephaly and mildly dysmorphic craniofacial findings. Chromosomal analysis showed a normal male karyotype but on subsequent array comparative genomic hybridization (array CGH) analysis a de novo 2.5 Mb loss in chromosome 13q at 13q33.3q34, together with an inherited gain at 4p12, were detected. The propositus underwent placement of a Blalock Taussig shunt and subsequently a Glenn and Fontan operation was performed. In this report we propose that COL4A1 and COL4A2 may be candidate genes for congenital heart disease (CHD) in individuals with a deletion in 13q within the 6Mb critical region for cardiac development proposed by Huang et al., [2012]. AD - Department of Pediatric Cardiology, Our Lady's Children's Hospital, Crumlin, Dublin, Ireland. AN - 25810372 AU - McMahon, C. J. AU - Breathnach, C. AU - Betts, D. R. AU - Sharkey, F. H. AU - Greally, M. T. DA - May DB - PubMed DO - 10.1002/ajmg.a.36978 DP - NLM ET - 2015/03/27 IS - 5 KW - Child *Chromosome Deletion Chromosomes, Human, Pair 13/genetics Collagen Type IV/genetics Comparative Genomic Hybridization Craniofacial Abnormalities/*genetics/physiopathology Developmental Disabilities/genetics/physiopathology Double Outlet Right Ventricle/*genetics/physiopathology Genetic Association Studies Heart Defects, Congenital/*genetics/physiopathology Humans Male Microcephaly/*genetics/physiopathology Serine Endopeptidases/genetics Col4a1 Col4a2 Corin Congenital heart defect (CHD) array comparative genomic hybridization (array CGH) chromosome 13q33.3q34 deletion double outlet right ventricle (DORV) dysmorphic craniofacial findings inherited paternal gain at 4p12 LA - eng N1 - 1552-4833 McMahon, Colin J Breathnach, Colm Betts, David R Sharkey, Freddie H Greally, Marie T Case Reports Journal Article United States Am J Med Genet A. 2015 May;167A(5):1134-41. doi: 10.1002/ajmg.a.36978. Epub 2015 Mar 21. PY - 2015 SN - 1552-4825 SP - 1134-41 ST - De Novo interstitial deletion 13q33.3q34 in a male patient with double outlet right ventricle, microcephaly, dysmorphic craniofacial findings, and motor and developmental delay T2 - Am J Med Genet A TI - De Novo interstitial deletion 13q33.3q34 in a male patient with double outlet right ventricle, microcephaly, dysmorphic craniofacial findings, and motor and developmental delay VL - 167a ID - 545 ER - TY - JOUR AD - L. Fontanilla, John A. Burns School of Medicine, USA. AU - Fontanilla Jr, L. DB - Medline IS - 3 KW - article decision making female human hypoplastic left heart syndrome medical ethics newborn patient advocacy professional-patient relationship religion Religious Approach right to die treatment refusal LA - English M3 - Article N1 - L127242022 1997-05-26 PY - 1997 SN - 0017-8594 SP - 59-60 ST - The death of an innocent T2 - Hawaii medical journal TI - The death of an innocent UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L127242022 VL - 56 ID - 1358 ER - TY - JOUR AB - OBJECTIVE: Children born with hypoplastic left heart syndrome (HLHS) may experience cardiac dysfunction after staged surgery or transplantation, which may worsen with age. We examined the hypothesis that exercise testing can address cardiovascular capacity and suggest interventions to improve quality of life. STUDY DESIGN: Children with HLHS > or = 8 years old performed treadmill or bicycle ergometric testing at 4 centers. Results were compared with norms for age and sex. RESULTS: Of the 42 participants, the mean age was 12.9 years (range, 8.5-17.0 years), 64% were boys, 20 had staged surgery, and 34 completed metabolic assessment. The percent of predicted maximal oxygen uptake (mVO2) was higher in younger children. Children aged 8 to 12 years achieved 70% of predicted mVO2; children aged 13 to 17 years achieved 60% of predicted mVO2 (P = .02). The percent of predicted peak heart rate trended higher in younger patients (83% versus 75%, P = .07). Electrocardiographic changes were more common in older children. In treadmill testing, patients who had a transplant had better exercise performance than patients who underwent staged surgery in percent of predicted exercise time (82% versus 54%, P < .0001) and peak rate-pressure product (241 x 10(3) versus 195 x 10(3), P = .02). The percent of predicted mVO2 did not differ between patients who had a transplant (66%) and patients who underwent staged surgery (61%, P = .25). CONCLUSION: Children with HLHS showed considerable age-related decline in exercise performance, regardless of surgical strategy. AD - Department of Pediatrics, Dartmouth Medical School, Hanover, NH 03756, USA. pcj@hitchcock.org AN - 18346505 AU - Jenkins, P. C. AU - Chinnock, R. E. AU - Jenkins, K. J. AU - Mahle, W. T. AU - Mulla, N. AU - Sharkey, A. M. AU - Flanagan, M. F. DA - Apr DB - PubMed DO - 10.1016/j.jpeds.2007.09.050 DP - NLM ET - 2008/03/19 IS - 4 KW - Adolescent Analysis of Variance Blood Pressure Case-Control Studies Child Electrocardiography Exercise Test *Exercise Tolerance Female *Fontan Procedure Heart Rate Heart Transplantation/*physiology Humans Hypoplastic Left Heart Syndrome/metabolism/*physiopathology/surgery Male Oxygen Consumption Quality of Life Reference Values LA - eng N1 - 1097-6833 Jenkins, Pamela C Chinnock, Richard E Jenkins, Kathy J Mahle, William T Mulla, Neda Sharkey, Angela M Flanagan, Michael F Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S. United States J Pediatr. 2008 Apr;152(4):507-12. doi: 10.1016/j.jpeds.2007.09.050. Epub 2007 Nov 19. PY - 2008 SN - 0022-3476 SP - 507-12 ST - Decreased exercise performance with age in children with hypoplastic left heart syndrome T2 - J Pediatr TI - Decreased exercise performance with age in children with hypoplastic left heart syndrome VL - 152 ID - 228 ER - TY - JOUR AB - Background The purpose of this study was to assess deep hypothermic circulatory arrest (DHCA) as a modifier of neurodevelopmental (ND) outcomes in preschool children after cardiac surgery in infancy for repair of congenital heart defects (CHD). Methods This is a planned analysis of infants enrolled in a prospective study of apolipoprotein E polymorphisms and ND outcome after cardiac surgery. The effect of DHCA was assessed in patients with single or biventricular CHD without aortic arch obstruction. Neurodevelopmental assessment at 4 years of age included cognition, language, attention, impulsivity, executive function, social competence, and visual-motor and fine-motor skills. Patient and procedural variables were evaluated in univariate and multivariate models. Results Neurodevelopmental testing was completed in 238 of 307 eligible patients (78%). Deep hypothermic circulatory arrest was used at the discretion of the surgeon at least once in 92 infants (38.6%) with a median cumulative duration of 36 minutes (range, 1 to 132 minutes). By univariate analysis, DHCA patients were more likely to have single-ventricle CHD (p = 0.013), lower socioeconomic status (p < 0.001), a higher incidence of preoperative ventilation (p < 0.001), and were younger and smaller at the first surgery (p < 0.001). By multivariate analysis, use of DHCA was not predictive of worse performance for any ND outcome. Conclusions In this cohort of children undergoing repair of CHD in infancy, patients who underwent DHCA had risk factors associated with worse ND outcomes. Despite these, use of DHCA for repair of single-ventricle and biventricular CHD without aortic arch obstruction was not predictive of worse performance for any ND domain tested at 4 years of age. © 2010 The Society of Thoracic Surgeons. AD - S. Fuller, Division of Cardiothoracic Surgery, Children's Hospital of Philadelphia, 34th and Civic Center Blvd, Philadelphia, PA 19104, United States AU - Fuller, S. AU - Rajagopalan, R. AU - Jarvik, G. P. AU - Gerdes, M. AU - Bernbaum, J. AU - Wernovsky, G. AU - Clancy, R. R. AU - Solot, C. AU - Nicolson, S. C. AU - Spray, T. L. AU - Gaynor, J. W. DB - Embase DO - 10.1016/j.athoracsur.2010.08.005 IS - 6 KW - apolipoprotein E article attention child clinical assessment cognition congenital heart malformation controlled study deep hypothermic circulatory arrest female genetic polymorphism groups by age heart surgery human impulsiveness infant language major clinical study male motor performance nervous system development outcome assessment preoperative evaluation preschool child priority journal prospective study risk factor school child social competence social status surgeon visuomotor coordination LA - English M3 - Article N1 - L360036290 2010-12-03 2010-12-09 PY - 2010 SN - 0003-4975 SP - 1985-1994 ST - Deep hypothermic circulatory arrest does not impair neurodevelopmental outcome in school-age children after infant cardiac surgery T2 - Annals of Thoracic Surgery TI - Deep hypothermic circulatory arrest does not impair neurodevelopmental outcome in school-age children after infant cardiac surgery UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L360036290 http://dx.doi.org/10.1016/j.athoracsur.2010.08.005 VL - 90 ID - 1125 ER - TY - JOUR AB - Background: This study aimed to assess cortical gray matter growth and maturation in neonates with congenital heart disease (CHD).Methods: Thirty-one (near) term neonates with severe CHD (8 univentricular heart malformation (UVH), 21 d-transposition of great arteries (d-TGA) and 2 aortic coarctation) underwent cerebral MRI before (postnatal-day 7) and after (postnatal-day 24) surgery. Eighteen controls with similar gestational age had one MRI (postnatal-day 23). Cortical gray matter volume (CGM), inner cortical surface (iCS), and median cortical thickness were extracted as measures of volumetric growth, and gyrification index (GI) as measure of maturation.Results: Over a median of 18 d, CGM increased by 21%, iCS by 17%, thickness and GI both by 9%. Decreased postoperative CGM and iCS were seen for CHD compared to controls (P values < 0.01), however with similar thickness and GI. UVH showed lower postoperative iCS, thickness (P values < 0.05) and GI (P value < 0.01) than d-TGA and controls. Infants requiring preoperative balloon-atrioseptostomy (BAS, 61%) had reduced postoperative CGM, iCS, and GI (P values < 0.05).Conclusion: Infants with severe CHD show reduced cortical volumes compared to controls with gyrification being delayed in UVH, but not in d-TGA. Infants requiring BAS show higher risk of impaired cortical volume and gyrification. AD - Department of Neonatology, Wilhelmina Children's Hospital, University Medical Centre Utrecht, The Netherlands Image Sciences Institute, University Medical Centre Utrecht,The Netherlands Department of Neurology, University Hospital Zurich, Switzerland Child Development Center, University Children´s Hospital Zurich, Switzerland Children's Research Center, University Children's Hospital, Zurich, Switzerland Department of Cardiology, University Children's Hospital, Zurich, Switzerland Diagnostic Imaging, University Children´s Hospital, Zurich, Switzerland Developmental Pediatrics, SPZ, Kantonsspital Winterthur, Switzerland AN - 119555972. Language: English. Entry Date: 20171129. Revision Date: 20181017. Publication Type: journal article AU - Claessens, Nathalie H. P. AU - Moeskops, Pim AU - Buchmann, Andreas AU - Latal, Beatrice AU - Knirsch, Walter AU - Scheer, Ianina AU - Išgum, Ivana AU - de Vries, Linda S. AU - Benders, Manon J. N. L. AU - von Rhein, Michael DB - ccm DO - 10.1038/pr.2016.145 DP - EBSCOhost IS - 5 KW - Heart Defects, Congenital -- Diagnosis Developmental Disabilities -- Diagnosis Cerebral Cortex -- Pathology Gray Matter -- Pathology Female Transposition of Great Arteries -- Complications Cerebral Cortex Heart Defects, Congenital -- Complications Developmental Disabilities -- Complications Time Factors Infant, Newborn Prospective Studies Magnetic Resonance Imaging Infant Aortic Coarctation -- Complications Cerebral Cortex -- Abnormalities Risk Factors Male Gray Matter Gray Matter -- Abnormalities Human N1 - research. Journal Subset: Biomedical; USA. NLM UID: 0100714. PMID: NLM27434120. PY - 2016 SN - 0031-3998 SP - 668-674 ST - Delayed cortical gray matter development in neonates with severe congenital heart disease T2 - Pediatric Research TI - Delayed cortical gray matter development in neonates with severe congenital heart disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=119555972&site=ehost-live&scope=site VL - 80 ID - 1520 ER - TY - JOUR AB - INTRODUCTION: We sought to evaluate the prevalence of delayed puberty and abnormal anthropometry and its association with quality of life (QoL) in young Fontan survivors. METHODS: This was a cross-sectional study at 11 Pediatric Heart Network centers. Demographic and clinical data, anthropomety, and Tanner stage were collected. Anthropometric measurements and pubertal stage were compared to US norms. QoL was assessed using Pediatric Quality of Life inventory (PedsQL). Mixed effects regression modeling adjusting for clustering by center was used to evaluate factors associated with abnormal anthropometry and delayed puberty and associations with QoL. RESULTS: Of the 299 subjects, 42% were female. The median enrollment age was 13.9 years, and the median age at Fontan was 3 years. Fontan survivors had a higher prevalence of short stature relative to normative data (20% vs 5%, P < .0001) and an increased prevalence of abnormal BMI (16% vs 10%, P < .0001) (low [43%] and high [57%]). Fontan subjects, both males (58%) and females (58%), had a delay of 1.5-2 years in ≥1 Tanner stage parameter compared to normal population. There was no association between delayed puberty and QoL. Abnormal anthropometry was associated with lower overall (62.3 ± 17.3 vs 72.5 ± 16.6; P < .001) and physical appearance scores (72.2 ± 27.4 vs 79.8 ± 21.5; P < .01). Lower exercise capacity was associated with abnormal anthropometry and >2 surgeries before Fontan was associated with delayed puberty. Lower family income (<$25 000) and hypoplastic left heart syndrome were associated with lower QoL. CONCLUSION: Compared to the normal population, Fontan survivors have high prevalence of short stature, abnormal BMI and delayed puberty. Abnormal anthropometry, but not delayed puberty, was associated with lower overall QoL and perceived physical appearance scores. Routine screening for abnormal anthropometry, especially in HLHS and in lower socioeconomic status families, should be considered to allow interventions, which might ameliorate the negative psychosocial impact. AD - University of Utah and Primary Children's Hospital, Salt Lake City, Utah, USA. The Hospital for Sick Children, Toronto, Ontario, Canada. Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA. Emory University School of Med, Atlanta, Georgia, USA. Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA. Texas Children's Hospital, Houston, Texas, USA. University of Michigan, Ann Arbor, Michigan, USA. Columbia University Medical Center, New York, New York, USA. Medical University of South Carolina, Charleston, South Carolina, USA. Duke University School of Medicine, Durham, North Carolina, USA. Riley Hospital for Children, Indianapolis, Indiana, USA. AN - 29521004 AU - Menon, S. C. AU - Al-Dulaimi, R. AU - McCrindle, B. W. AU - Goldberg, D. J. AU - Sachdeva, R. AU - Goldstein, B. H. AU - Seery, T. AU - Uzark, K. C. AU - Chelliah, A. AU - Butts, R. AU - Henderson, H. AU - Johnson, T. AU - Williams, R. V. DA - May DB - PubMed DO - 10.1111/chd.12597 DP - NLM ET - 2018/03/10 IS - 3 KW - Adolescent *Anthropometry Child Cross-Sectional Studies Female Fontan Procedure/*psychology *Health Status Humans Hypoplastic Left Heart Syndrome/psychology/*surgery Male Puberty, Delayed/complications/physiopathology/*psychology *Quality of Life Surveys and Questionnaires Survivors/*statistics & numerical data Fontan abnormal growth delayed puberty quality of life LA - eng N1 - 1747-0803 Menon, Shaji C Orcid: 0000-0002-0421-0203 Al-Dulaimi, Ragheed McCrindle, Brian W Goldberg, David J Sachdeva, Ritu Goldstein, Bryan H Orcid: 0000-0001-8508-9523 Seery, Thomas Uzark, Karen C Chelliah, Anjali Butts, Ryan Henderson, Heather Johnson, Tiffanie Williams, Richard V Journal Article Multicenter Study United States Congenit Heart Dis. 2018 May;13(3):463-469. doi: 10.1111/chd.12597. Epub 2018 Mar 9. PY - 2018 SN - 1747-079x SP - 463-469 ST - Delayed puberty and abnormal anthropometry and its associations with quality of life in young Fontan survivors: A multicenter cross-sectional study T2 - Congenit Heart Dis TI - Delayed puberty and abnormal anthropometry and its associations with quality of life in young Fontan survivors: A multicenter cross-sectional study VL - 13 ID - 67 ER - TY - JOUR AB - OBJECTIVES: This study was undertaken to compare conventional cardioplegic strategies with a new approach that uses a modified non-potassium maintenance solution between cardioplegia doses in stressed neonatal hearts. METHODS: Thirty-five neonatal piglets underwent 60 minutes of ventilator hypoxia (inspired oxygen fraction 8%-10%) followed by 20 minutes of ischemia on cardiopulmonary bypass. In 10 animals bypass was discontinued without further ischemia (stress control group). The other 25 received a warm blood cardioplegic induction and were separated into 5 groups. In 5 animals cardiopulmonary bypass was discontinued without further ischemia (cardioplegia control group); the remaining 20 underwent an additional 70 minutes of cold blood cardioplegic arrest. Five received only intermittent cardioplegia every 20 minutes, whereas 15 also received cold blood maintenance infusions between cardioplegic doses (integrated strategy). In 5 of these animals the blood was unmodified, whereas in 10 a modified non-potassium "cardioplegia-like" solution was delivered either antegradely (n = 5) or retrogradely (n = 5). Myocardial function was assessed by pressure-volume loops (expressed as percentage of control); vascular function was assessed by coronary vascular resistance. RESULTS: All piglets that underwent hypoxic ischemic stress alone (controls) died. Warm induction alone (cardioplegic controls) partially repaired the stress injury. Intermittent cardioplegia preserved the depressed systolic function (end-systolic elastance 40% vs 39%), increased diastolic stiffness (255% vs 239%), reduced adenosine triphosphate (10.6 vs 12.2 microg/g tissue), and elevated coronary vascular resistance at levels identical to warm induction alone; infusing unmodified blood between cardioplegia doses (standard integrated) improved results slightly. In contrast, infusion of a cold modified solution (antegrade or retrograde) between cardioplegia doses (modified integrated) completely restored systolic function (end-systolic elastance 100% and 97%, P <.001 vs intermittent and standard integrated), only minimally increased diastolic stiffness (159% and 156%, P <.001 vs intermittent and standard integrated), restored adenosine triphosphate (18.8 and 16.6 microg/g, P <.001 vs intermittent and standard integrated), and normalized coronary vascular resistance (P <.001 vs intermittent and standard integrated). This strategy was used in 72 consecutive hypoxic patients (21 arterial switch operations, retrograde; 51 Fontan procedures, antegrade) with a 2.8% mortality. CONCLUSIONS: Infusion of a cold modified solution between cardioplegic doses (modified integrated protection) significantly improved myocardial protection in the stressed neonatal heart, was effective delivered either antegradely or retrogradely, and was used successfully for hypoxic (stressed) pediatric patients. AD - Division of Cardiovascular Surgery, The Heart Institute for Children, Hope Children's Hospital, Oak Lawn, IL 60453, USA. AN - 11782765 AU - Kronon, M. T. AU - Allen, B. S. AU - Halldorsson, A. AU - Rahman, S. AU - Barth, M. J. AU - Ilbawi, M. DA - Jan DB - PubMed DP - NLM ET - 2002/01/10 IS - 1 KW - Adenosine Diphosphate/metabolism Adenosine Triphosphate/metabolism Animals Animals, Newborn/*physiology Antioxidants/metabolism Body Water/metabolism Cardioplegic Solutions/*administration & dosage Coronary Circulation Heart/*physiopathology Heart Arrest, Induced/methods Hypoxia/complications/*physiopathology Malondialdehyde/metabolism Myocardial Ischemia/complications/*physiopathology Myocardium/metabolism Oxygen Consumption Peroxidase/metabolism Swine Temperature Vascular Resistance Ventricular Function, Left Ventricular Pressure LA - eng N1 - Kronon, Michael T Allen, Bradley S Halldorsson, Ari Rahman, Shaikh Barth, Mary Jane Ilbawi, Michel Journal Article Research Support, Non-U.S. Gov't United States J Thorac Cardiovasc Surg. 2002 Jan;123(1):119-29. PY - 2002 SN - 0022-5223 (Print) 0022-5223 SP - 119-29 ST - Delivery of a non-potassium modified maintenance solution to enhance myocardial protection in stressed neonatal hearts: a new approach T2 - J Thorac Cardiovasc Surg TI - Delivery of a non-potassium modified maintenance solution to enhance myocardial protection in stressed neonatal hearts: a new approach VL - 123 ID - 483 ER - TY - JOUR AB - Objectives: This study was undertaken to compare conventional cardioplegic strategies with a new approach that uses a modified nonpotassium maintenance solution between cardioplegia doses in stressed neonatal hearts. Methods: Thirty-five neonatal piglets underwent 60 minutes of ventilator hypoxia (inspired oxygen fraction 8%-10%) followed by 20 minutes of ischemia on cardiopulmonary bypass. In 10 animals bypass was discontinued without further ischemia (stress control group). The other 25 received a warm blood cardioplegic induction and were separated into 5 groups. In 5 animals cardiopulmonary bypass was discontinued without further ischemia (cardioplegia control group); the remaining 20 underwent an additional 70 minutes of cold blood cardioplegic arrest. Five received only intermittent cardioplegia every 20 minutes, whereas 15 also received cold blood maintenance infusions between cardioplegic doses (integrated strategy). In 5 of these animals the blood was unmodified, whereas in 10 a modified nonpotassium "cardioplegia-like" solution was delivered either antegradely (n = 5) or retrogradely (n = 5). Myocardial function was assessed by pressure-volume loops (expressed as percentage of control); vascular function was assessed by coronary vascular resistance. Results: All piglets that underwent hypoxic ischemic stress alone (controls) died. Warm induction alone (cardioplegic controls) partially repaired the stress injury. Intermittent cardioplegia preserved the depressed systolic function (end-systolic elastance 40% vs 39%), increased diastolic stiffness (255% vs 239%), reduced adenosine triphosphate (10.6 vs 12.2 μg/g tissue), and elevated coronary vascular resistance at levels identical to warm induction alone; infusing unmodified blood between cardioplegia doses (standard integrated) improved results slightly. In contrast, infusion of a cold modified solution (antegrade or retrograde) between cardioplegia doses (modified integrated) completely restored systolic function (end-systolic elastance 100% and 97%, P < .001 vs intermittent and standard integrated), only minimally increased diastolic stiffness (159% and 156%, P < .001 vs intermittent and standard integrated), restored adenosine triphosphate (18.8 and 16.6 μg/g, P < .001 vs intermittent and standard integrated), and normalized coronary vascular resistance (P < .001 vs intermittent and standard integrated). This strategy was used in 72 consecutive hypoxic patients (21 arterial switch operations, retrograde; 51 Fontan procedures, antegrade) with a 2.8% mortality. Conclusions: Infusion of a cold modified solution between cardioplegic doses (modified integrated protection) significantly improved myocardial protection in the stressed neonatal heart, was effective delivered either antegradely or retrogradely, and was used successfully for hypoxic (stressed) pediatric patients. AD - B.S. Allen, Heart Institute for Children, Hope Children's Hospital, 4440 W 95th St, Oak Lawn, IL 60453, United States AU - Kronon, M. T. AU - Allen, B. S. AU - Halldorsson, A. AU - Rahman, S. AU - Barth, M. J. AU - Ilbawi, M. C2 - Research Medical(United States) DB - Embase Medline IS - 1 KW - aspartic acid cardioplegic agent citric acid glucose glutamic acid potassium chloride trometamol animal experiment animal model article cardioplegia cardiopulmonary bypass controlled study coronary vascular resistance heart function cardiac muscle heart performance heart protection newborn nonhuman priority journal pig LA - English M3 - Article N1 - L35086813 2002-10-14 PY - 2002 SN - 0022-5223 SP - 119-129 ST - Delivery of a nonpotassium modified maintenance solution to enhance myocardial protection in stressed neonatal hearts: A new approach T2 - Journal of Thoracic and Cardiovascular Surgery TI - Delivery of a nonpotassium modified maintenance solution to enhance myocardial protection in stressed neonatal hearts: A new approach UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L35086813 VL - 123 ID - 1316 ER - TY - JOUR AB - Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative disease and shows a wide range of early clinical manifestation. We report the case of Ms. A, a 50-year-old woman, who exhibited an atypical psychiatric presentation of CJD at the beginning of the disease, as a depressive disorder with psychotic features. We discuss this case, the clinical attitudes that can lead to misdiagnose such presentation of CJD with other psychiatric diseases, and propose to repeat complementary investigations when the diagnostic is evocated, even if these tests are often negative or nonspecific in the early stages of CJD. (PsycINFO Database Record (c) 2018 APA, all rights reserved) AD - Jardri, Renaud, Child and Adolescent Psychiatry Department, Michel Fontan Clinic, Lille University Hospital Centre, Rue Andre Verhaeghe, 59 037, Lille, France, cedex AN - 2006-11660-018 AU - Jardri, Renaud AU - DiPaola, Claire AU - Lajugie, Christine AU - Thomas, Pierre AU - Goeb, Jean-Louis DB - psyh DO - 10.1016/j.genhosppsych.2006.05.005 DP - EBSCOhost IS - 5 KW - Creutzfeldt-Jakob disease psychotic symptoms depressive disorder neurodegenerative disease early clinical manifestation Affective Disorders, Psychotic Aggression Brain Creutzfeldt-Jakob Syndrome Depressive Disorder, Major Diagnostic Errors Disease Progression Electroencephalography Fatal Outcome Female Humans Magnetic Resonance Imaging Middle Aged Neurocognitive Disorders Neurologic Examination Patient Admission Referral and Consultation Creutzfeldt Jakob Syndrome Major Depression Neurodegenerative Diseases Psychiatric Symptoms N1 - Child and Adolescent Psychiatry Department, Michel Fontan Clinic, Lille University Hospital Centre, Lille, France. Release Date: 20060918. Correction Date: 20180115. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Creutzfeldt Jakob Syndrome; Major Depression; Neurodegenerative Diseases; Psychiatric Symptoms. Classification: Neurological Disorders & Brain Damage (3297). Population: Human (10); Female (40). Age Group: Adulthood (18 yrs & older) (300); Middle Age (40-64 yrs) (360). Methodology: Clinical Case Study. References Available: Y. Page Count: 3. Issue Publication Date: Sep-Oct, 2006. PY - 2006 SN - 0163-8343 1873-7714 SP - 452-454 ST - Depressive disorder with psychotic symptoms as psychiatric presentation of sporadic Creutzfeldt-Jakob disease: A case report T2 - General Hospital Psychiatry TI - Depressive disorder with psychotic symptoms as psychiatric presentation of sporadic Creutzfeldt-Jakob disease: A case report UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2006-11660-018&site=ehost-live&scope=site ORCID: 0000-0003-4596-1502 r.jardri@free.fr VL - 28 ID - 1697 ER - TY - JOUR AB - Background: Clinical trials in children with congenital heart disease are often limited by the absence of the following: (1) a primary outcome that can be observed in a reasonable period; (2) information regarding health-related quality of life; (3) knowledge of the correlation between health status and ventricular function and exercise performance; (4) a sufficient number of children at a single institution to provide adequate statistical power; and (5) procedural and management differences between and within institutions. Methods: The NHLBI-funded Pediatric Heart Network designed a cross-sectional study of children aged 6 to 18 years, from 7 pediatric clinical centers, who had undergone a Fontan procedure as treatment for congenital heart disease. Health-related quality of life was measured by the Child Health Questionnaire and the Congenital Heart Adolescent and Teenager Questionnaire. Ventricular function was assessed by maximal exercise testing, echocardiography, cardiac magnetic resonance imaging, and B-type natriuretic peptide. The study was designed to detect a correlation of R ≥ 0.30 between health status scores and measures of ventricular function and performance in a subcohort with all study measures completed. Results: A total of 1078 children were screened by chart review; 644 (60%) were eligible. The consent rate was 85% and 546 children were enrolled. Acquisition of echocardiograms, B-type natriuretic peptide, and health status was ≥94%; completion rates were lower for maximal exercise testing (76%) and cardiac magnetic resonance imaging (41%). Conclusions: This large study provides unique information regarding the relationship between health status and clinical measures in post-Fontan patients that will facilitate the design of future randomized trials. © 2006 Mosby, Inc. All rights reserved. AD - L.A. Sleeper, New England Research Institutes, Watertown, MA, United States AU - Sleeper, L. A. AU - Anderson, P. AU - D, T. Hsu AU - Mahony, L. AU - McCrindle, B. W. AU - Roth, S. J. AU - Saul, J. P. AU - Williams, R. V. AU - Geva, T. AU - Colan, S. D. AU - Clark, B. J. DB - Embase Medline DO - 10.1016/j.ahj.2006.02.009 IS - 3 KW - brain natriuretic peptide adolescent adult article child congenital heart disease echocardiography electrocardiogram exercise test female fontan palliation health status heart surgery heart ventricle function human informed consent major clinical study male nuclear magnetic resonance imaging pediatric hospital priority journal quality of life questionnaire screening surgical approach LA - English M3 - Article N1 - L44233415 2006-08-27 PY - 2006 SN - 0002-8703 1097-6744 SP - 427-433 ST - Design of a large cross-sectional study to facilitate future clinical trials in children with the Fontan palliation T2 - American Heart Journal TI - Design of a large cross-sectional study to facilitate future clinical trials in children with the Fontan palliation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L44233415 http://dx.doi.org/10.1016/j.ahj.2006.02.009 VL - 152 ID - 1253 ER - TY - JOUR AB - BACKGROUND: Clinical trials in children with congenital heart disease are often limited by the absence of the following: (1) a primary outcome that can be observed in a reasonable period; (2) information regarding health-related quality of life; (3) knowledge of the correlation between health status and ventricular function and exercise performance; (4) a sufficient number of children at a single institution to provide adequate statistical power; and (5) procedural and management differences between and within institutions. METHODS: The NHLBI-funded Pediatric Heart Network designed a cross-sectional study of children aged 6 to 18 years, from 7 pediatric clinical centers, who had undergone a Fontan procedure as treatment for congenital heart disease. Health-related quality of life was measured by the Child Health Questionnaire and the Congenital Heart Adolescent and Teenager Questionnaire. Ventricular function was assessed by maximal exercise testing, echocardiography, cardiac magnetic resonance imaging, and B-type natriuretic peptide. The study was designed to detect a correlation of R > or = 0.30 between health status scores and measures of ventricular function and performance in a subcohort with all study measures completed. RESULTS: A total of 1078 children were screened by chart review; 644 (60%) were eligible. The consent rate was 85% and 546 children were enrolled. Acquisition of echocardiograms, B-type natriuretic peptide, and health status was > or = 94%; completion rates were lower for maximal exercise testing (76%) and cardiac magnetic resonance imaging (41%). CONCLUSIONS: This large study provides unique information regarding the relationship between health status and clinical measures in post-Fontan patients that will facilitate the design of future randomized trials. AD - New England Research Institutes, Watertown, MA 02472, USA. lsleeper@neriscience.com AN - 16923408 AU - Sleeper, L. A. AU - Anderson, P. AU - Hsu, D. T. AU - Mahony, L. AU - McCrindle, B. W. AU - Roth, S. J. AU - Saul, J. P. AU - Williams, R. V. AU - Geva, T. AU - Colan, S. D. AU - Clark, B. J. C2 - PMC4266479 C6 - NIHMS12163 DA - Sep DB - PubMed DO - 10.1016/j.ahj.2006.02.009 DP - NLM ET - 2006/08/23 IS - 3 KW - Adolescent Child Clinical Trials as Topic/*methods/trends *Cross-Sectional Studies Female Fontan Procedure/*statistics & numerical data/trends Heart Defects, Congenital/*epidemiology/surgery Humans Magnetic Resonance Imaging/methods/trends Male Multicenter Studies as Topic/methods/trends Palliative Care/*methods/trends Quality of Life Randomized Controlled Trials as Topic/methods/trends Surveys and Questionnaires LA - eng N1 - 1097-6744 Sleeper, Lynn A Anderson, Page Hsu, Daphne T Mahony, Lynn McCrindle, Brian W Roth, Stephen J Saul, J Phillip Williams, Richard V Geva, Tal Colan, Steven D Clark, Bernard J Pediatric Heart Network Investigators U01 HL068279-05/HL/NHLBI NIH HHS/United States U01 HL068285-05/HL/NHLBI NIH HHS/United States U01 HL068290-05/HL/NHLBI NIH HHS/United States U01 HL068281-05/HL/NHLBI NIH HHS/United States U01 HL068270-05/HL/NHLBI NIH HHS/United States U01 HL068288-05/HL/NHLBI NIH HHS/United States U01 HL068269/HL/NHLBI NIH HHS/United States U01 HL068279/HL/NHLBI NIH HHS/United States U01 HL68270/HL/NHLBI NIH HHS/United States U01 HL068290/HL/NHLBI NIH HHS/United States U01 HL068288/HL/NHLBI NIH HHS/United States U01 HL068281/HL/NHLBI NIH HHS/United States U01 HL068269-05/HL/NHLBI NIH HHS/United States U01 HL068270/HL/NHLBI NIH HHS/United States U01 HL068292-05/HL/NHLBI NIH HHS/United States U01 HL068292/HL/NHLBI NIH HHS/United States U01 HL068285/HL/NHLBI NIH HHS/United States Journal Article Research Support, N.I.H., Extramural Am Heart J. 2006 Sep;152(3):427-33. doi: 10.1016/j.ahj.2006.02.009. PY - 2006 SN - 0002-8703 (Print) 0002-8703 SP - 427-33 ST - Design of a large cross-sectional study to facilitate future clinical trials in children with the Fontan palliation T2 - Am Heart J TI - Design of a large cross-sectional study to facilitate future clinical trials in children with the Fontan palliation VL - 152 ID - 163 ER - TY - JOUR AB - Currently, no long-term mechanical bridge-to-transplant or bridge-to-recovery therapeutic alternative exists for failing single ventricles. A blood pump that would augment pressure in the cavopulmonary circulation is needed, and would lead to a reduction in elevated systemic venous pressure, and improve cardiac output. Thus, we are developing a collapsible, percutaneously inserted, axial flow blood pump to support the cavopulmonary circulation in adult patients with a failing single ventricle physiology. This collapsible axial flow pump is designed for percutaneous positioning. The outer protective cage will be designed with radially arranged filaments as touchdown surfaces to protect the vessel wall from the rotating components. This study examined the geometric characteristics of the protective cage of filaments and the impeller through the development and numerical analysis of 13 models. A blood damage analysis was also performed on selected geometric models to assess the probability of blood trauma. All models demonstrated an acceptable hydraulic performance by delivering 2-6 L/min at a rotational speed of 6000-10 000 rpm and generating pressure rise of 5-20 mm Hg. Expected trends in the hydraulic performance of the pump models were found. This study represents the initial first design phase of the impeller and protective cage of filaments. Validation of these flow and performance predictions will be completed in the next round of experimental testing with blood bag evaluation. © 2009, International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc. AD - Department of Mechanical Engineering, Virginia Commonwealth University, 401 W. Main Street, Richmond, VA 23284, United States AU - Throckmorton, A. L. AU - Kishore, R. A. DB - Scopus DO - 10.1111/j.1525-1594.2009.00779.x IS - 8 KW - Artificial right ventricle Blood pump Cavopulmonary assist device Fontan conversion Heart pump Intravascular blood pump Mechanical cavopulmonary assist Pediatric circulatory support Single ventricle physiology Ventricular assist device M3 - Article N1 - Cited By :21 Export Date: 15 June 2020 PY - 2009 SP - 611-621 ST - Design of a protective cage for an intravascular axial flow blood pump to mechanically assist the failing fontan T2 - Artificial Organs TI - Design of a protective cage for an intravascular axial flow blood pump to mechanically assist the failing fontan UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-68549118733&doi=10.1111%2fj.1525-1594.2009.00779.x&partnerID=40&md5=598b266d922f29d4eb01d100a8f8dd63 VL - 33 ID - 2175 ER - TY - JOUR AB - Limited treatment options for patients having dysfunctional single ventricle physiology motivate the necessity for alternative therapeutic options. To address this unmet need, we are developing a collapsible axial flow blood pump. This study investigated the impact of geometric simplicity to facilitate percutaneous placement and maintain optimal performance. Three new pump designs were numerically evaluated. A transient simulation explored the impact of respiration on blood flow conditions over the entire respiratory cycle. Prototype testing of the top performing pump design was completed. The top performing Rec design generated the highest pressure rise range of 2-38 mm Hg for flow rates of 1-4 L/min at 4000-7000 RPM, exceeding the performance of the other two configurations by more than 26%. The blood damage indices for the new pump designs were determined to be below 0.5% and predicted hemolysis levels remained low at less than 7 × 10(-5)  g/100 L. Prototype testing of the Rec design confirmed numerical predictions to within an average of approximately 22%. These findings demonstrate that the pumps are reasonably versatile in operational ability, meet pressure-flow requirements to support Fontan patients, and are expected to have low levels of blood trauma. AD - Department of Mechanical and Nuclear Engineering, School of Engineering, Virginia Commonwealth University, Richmond, VA, USA. AN - 25626578 AU - Kafagy, D. H. AU - Dwyer, T. W. AU - McKenna, K. L. AU - Mulles, J. P. AU - Chopski, S. G. AU - Moskowitz, W. B. AU - Throckmorton, A. L. DA - Jan DB - PubMed DO - 10.1111/aor.12443 DP - NLM ET - 2015/01/30 IS - 1 KW - Adolescent Adult Child Child, Preschool *Computer-Aided Design Equipment Failure Analysis Equipment Safety Female Fontan Procedure/adverse effects/*methods Heart Defects, Congenital/diagnosis/*surgery *Heart-Assist Devices Humans Male Models, Cardiovascular *Prosthesis Design Risk Assessment Blood pump Computational fluid dynamics Fontan physiology Intravascular blood pump Mechanical cavopulmonary assist Pediatric circulatory support Single ventricle physiology Total cavopulmonary connection LA - eng N1 - 1525-1594 Kafagy, Dhyaa H Dwyer, Thomas W McKenna, Kelli L Mulles, Jean P Chopski, Steven G Moskowitz, William B Throckmorton, Amy L Evaluation Study Journal Article Research Support, U.S. Gov't, Non-P.H.S. United States Artif Organs. 2015 Jan;39(1):34-42. doi: 10.1111/aor.12443. PY - 2015 SN - 0160-564x SP - 34-42 ST - Design of axial blood pumps for patients with dysfunctional fontan physiology: computational studies and performance testing T2 - Artif Organs TI - Design of axial blood pumps for patients with dysfunctional fontan physiology: computational studies and performance testing VL - 39 ID - 405 ER - TY - JOUR AB - Durable ventricular assist devices (VADs) have significantly improved survival to transplantation among children with advanced stages of heart failure. The fundamental goals of VAD therapy include decreasing mortality, minimizing adverse events, and improving quality of life. As the pediatric VAD experience has evolved with reduced device related complications and improved survival, VAD therapy is being considered not only as a bridge to transplantation (BTT) but also as a bridge to decision (BTD) and as destination therapy (DT). Data regarding pediatric DT VAD are limited to anecdotal or case reports of children being supported for long periods with VADs and by default being classified as DT VAD. This article reviews current trends in the use of DT VAD and adverse events in children vs adults on VAD, and provides a framework for patient selection with the use of a multidisciplinary approach including palliative care. The general approach to determining DT VAD candidacy should include: 1) a reasonable success that the patient will survive the peri- and postoperative state; and 2) a high likelihood that the patient will be able to be discharged out of hospital and have adequate caregiver support. Patients with muscular dystrophy and failing Fontan physiology are examples of pediatric populations for whom DT VAD may be considered and which require unique considerations. AD - Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA. Department of Paediatrics, Stollery Children's Hospital, Edmonton, Alberta, Canada. Pediatric Cardiology, Cincinnati Children's Medical Center, Cincinnati, Ohio, USA. Department of Paediatrics, Hospital for Sick Children, Toronto, Ontario, Canada. Department of Paediatrics, Hospital for Sick Children, Toronto, Ontario, Canada. Electronic address: aamir.jeewa@sickkids.ca. AN - 31924452 AU - Tunuguntla, H. AU - Conway, J. AU - Villa, C. AU - Rapoport, A. AU - Jeewa, A. DA - Feb DB - PubMed DO - 10.1016/j.cjca.2019.10.033 DP - NLM ET - 2020/01/12 IS - 2 LA - eng N1 - 1916-7075 Tunuguntla, Hari Conway, Jennifer Villa, Chet Rapoport, Adam Jeewa, Aamir Journal Article Review England Can J Cardiol. 2020 Feb;36(2):216-222. doi: 10.1016/j.cjca.2019.10.033. Epub 2019 Nov 4. PY - 2020 SN - 0828-282x SP - 216-222 ST - Destination-Therapy Ventricular Assist Device in Children: "The Future Is Now" T2 - Can J Cardiol TI - Destination-Therapy Ventricular Assist Device in Children: "The Future Is Now" VL - 36 ID - 175 ER - TY - JOUR AB - Survivors of repairs of complex congenital cardiac malformations in infancy have an increased risk of permanent abnormalities in motor, cognitive, expressive, and behavioral functioning. These functional deficits are expressions of complex interactions of environment, including prolonged hospitalization and conditioned child-parental behaviours, alterations of social environment, the effects of physical limitations, biological influences including genetic determinants, prenatal injury, and acquired reversible and irreversible neuronal injury.1,2 The magnitude of the problem is large, with incidence dependent upon the measures used for assessment. Overt postoperative neurologic signs have been recorded in up to one-tenth of postoperative infants and children, with double that rate found in those with abnormalities of the aortic arch.3 A decreased potential for development, based upon parent-sibling models, has been estimated to occur in one-third of survivors.4,5 Evidence of injury is provided by magnetic resonance imaging in up to one-third of patients preoperatively, and between half and nine-tenths postoperatively, although most of these early postoperative changes will disappear.5 Although recent changes in perioperative management are likely to reduce such neurologic injury, their significance remains high. © 2005 Cambridge University Press. AD - G.M. Hoffman, Department of Anesthesiology and Pediatrics, Medical College of Wisconsin, Milwaukee, WI 53226, United States AU - Hoffman, G. M. DB - Embase Medline DO - 10.1017/S1047951105001198 IS - SUPPL. 1 KW - protein S100B aortic arch anomaly article brain injury brain metabolism brain oxygen consumption computer assisted tomography congenital heart malformation disease severity echography electroencephalography heart surgery high risk patient human hypoplastic left heart syndrome incidence induced hypothermia intensive care intensive care unit near infrared spectroscopy neurologic disease nuclear magnetic resonance imaging pathophysiology patient safety pediatric surgery perioperative period postoperative period preoperative evaluation primary prevention risk factor risk reduction surgical risk LA - English M3 - Article N1 - L44604542 2006-11-13 PY - 2005 SN - 1047-9511 1467-1107 SP - 149-153 ST - Detection and prevention of neurologic injury in the intensive care unit T2 - Cardiology in the Young TI - Detection and prevention of neurologic injury in the intensive care unit UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L44604542 http://dx.doi.org/10.1017/S1047951105001198 VL - 15 ID - 1284 ER - TY - JOUR AB - Continuous hemofiltration (CH) is being used in increasing numbers of pediatric intensive care unit patients. Experience with 114 CH treatments in 98 critically ill children from March 1988 to March 1993 is presented in this study. Ages ranged from 1 day to 23 yr (mean +/- SE = 7.1 +/- 0.7 yr), and 54% of patients were male. Seventeen percent of all treatments were performed in neonates under 1 month of age. The most common primary diagnoses were sepsis and adult respiratory distress syndrome (11 patients each), liver transplantation and hypoplastic left heart syndrome (10 patients each), and hemolytic uremic syndrome (9 patients). The most frequent indications for CH were fluid overload and acute renal failure (42% each). Choices for CH included: continuous arteriovenous hemofiltration (CAVH, 50%), continuous arteriovenous hemodiafiltration (CAVH, 23%), continuous venovenous hemofiltration (CVVH, 18%), and continuous venovenous hemodiafiltration (CVVH-D, 9%). Choices for anticoagulation included: none (47%), regional (49%), and systemic (4%). Treatment duration ranged from 1 to 25 days (mean = 5.3 +/- 0.4 days). Mean filter life span for 363 filters was 0.94 +/- 0.1 filters/patient per day. Despite an overall survival rate of 43%, survival to discharge varied greatly (0 to 100%) among the 24 diagnostic groups: tumor lysis syndrome and systemic lupus erythematosus (3/3 patients each, 100%), hemolytic uremic syndrome (8/9 patients, 89%). This compares with: bone marrow transplantation (0/6 patients, 0%), hypoplastic left heart syndrome (2/10 patients, 20%), and leukemia (1/4 patients, 25%). Survival to hospital discharge was better in patients who did not receive pressors (P < 0.005) and in patients treated with combined ultrafiltration and dialysis (CAVH-D, CVVH-D) compared with ultrafiltration alone (CAVH, CVVH) (P < 0.005), but was not notably affected by patient age, sex, use of anticoagulation, filter life span, blood pump-assisted versus spontaneous CH, or duration of therapy. Filter life span was not affected by use of anticoagulation, but was remarkably longer in patients with arteriovenous versus venovenous CH (P < 0.004). It was concluded that: (1) empirical anticoagulation of patients treated with CH is not necessary; (2) children with a minority of underlying diseases and those requiring pressor support at initiation of CH appear to have relatively poor survival rates despite the technically effective use of CH; and (3) the addition of countercurrent dialysis to routine CH may enhance patient survival to hospital discharge. AD - Department of Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania, School of Medicine 19104, USA. AN - 8589315 AU - Smoyer, W. E. AU - McAdams, C. AU - Kaplan, B. S. AU - Sherbotie, J. R. DA - Nov DB - PubMed DP - NLM ET - 1995/11/01 IS - 5 KW - Adolescent Adult Child Child, Preschool *Critical Care Female *Hemofiltration/adverse effects/instrumentation Humans Infant Infant, Newborn Male Retrospective Studies Survival Rate LA - eng N1 - Smoyer, W E McAdams, C Kaplan, B S Sherbotie, J R Journal Article United States J Am Soc Nephrol. 1995 Nov;6(5):1401-9. PY - 1995 SN - 1046-6673 (Print) 1046-6673 SP - 1401-9 ST - Determinants of survival in pediatric continuous hemofiltration T2 - J Am Soc Nephrol TI - Determinants of survival in pediatric continuous hemofiltration VL - 6 ID - 448 ER - TY - JOUR AB - While clinical outcomes in pediatric cardiac disease have improved in recent years, marked institutional and individual cardiology practice variability exists. Quality improvement science has demonstrated that reducing process variation leads to more favorable outcomes, safer practices, cost savings, and improved operating efficiency. This report describes the process undertaken to develop the first collaborative quality improvement project of the Joint Council on Congenital Heart Disease. The project chosen aims to reduce mortality and improve the quality of life of infants with hypoplastic left heart syndrome during the interstage period between discharge from the Norwood procedure and admission for the bidirectional Glenn procedure. The objective of this special article is to inform the pediatric cardiology and cardiac surgery communities of the project to help ensure that the early work by the project pilot participants will spread to clinicians caring for children with cardiovascular disease. It is anticipated that this project will add to our understanding of care for this challenging group of children with hypoplastic left heart syndrome, identifying clinical care changes with the potential to lead to improvements in outcome. It will also introduce the field of pediatric cardiology to the science of collaborative quality improvement and assist in reducing clinical process variation and improving patient outcomes across centers. Finally, it will establish an ongoing network of pediatric cardiologists and their teams linked through a longitudinal data set and collaboration for improvement and research. AD - UNMC/CUMC Joint Division of Pediatric Cardiology, Children's Hospital & Medical Center, Omaha, Nebraska 68114, USA. jkugler@me.com AN - 19740186 AU - Kugler, J. D. AU - Beekman Iii, R. H. AU - Rosenthal, G. L. AU - Jenkins, K. J. AU - Klitzner, T. S. AU - Martin, G. R. AU - Neish, S. R. AU - Lannon, C. DA - Sep-Oct DB - PubMed DO - 10.1111/j.1747-0803.2009.00328.x DP - NLM ET - 2009/09/11 IS - 5 KW - Advisory Committees/*standards Cardiac Surgical Procedures/adverse effects/mortality/*standards Cardiology/*standards Continuity of Patient Care/standards Cooperative Behavior Health Services Research Humans Hypoplastic Left Heart Syndrome/mortality/*surgery Infant Infant, Newborn Interdisciplinary Communication Outcome and Process Assessment, Health Care/*standards Patient Care Team/standards Pediatrics/*standards Practice Guidelines as Topic Program Development Program Evaluation Quality Indicators, Health Care/*standards Quality of Life Treatment Outcome United States LA - eng N1 - 1747-0803 Kugler, John D Beekman Iii, Robert H Rosenthal, Geoffrey L Jenkins, Kathy J Klitzner, Thomas S Martin, Gerard R Neish, Steven R Lannon, Carole Journal Article Research Support, Non-U.S. Gov't United States Congenit Heart Dis. 2009 Sep-Oct;4(5):318-28. doi: 10.1111/j.1747-0803.2009.00328.x. PY - 2009 SN - 1747-079x SP - 318-28 ST - Development of a pediatric cardiology quality improvement collaborative: from inception to implementation. From the Joint Council on Congenital Heart Disease Quality Improvement Task Force T2 - Congenit Heart Dis TI - Development of a pediatric cardiology quality improvement collaborative: from inception to implementation. From the Joint Council on Congenital Heart Disease Quality Improvement Task Force VL - 4 ID - 313 ER - TY - JOUR AB - While clinical outcomes in pediatric cardiac disease have improved in recent years, marked institutional and individual cardiology practice variability exists. Quality improvement science has demonstrated that reducing process variation leads to more favorable outcomes, safer practices, cost savings, and improved operating efficiency. This report describes the process undertaken to develop the first collaborative quality improvement project of the Joint Council on Congenital Heart Disease. The project chosen aims to reduce mortality and improve the quality of life of infants with hypoplastic left heart syndrome during the interstage period between discharge from the Norwood procedure and admission for the bidirectional Glenn procedure. The objective of this special article is to inform the pediatric cardiology and cardiac surgery communities of the project to help ensure that the early work by the project pilot participants will spread to clinicians caring for children with cardiovascular disease. It is anticipated that this project will add to our understanding of care for this challenging group of children with hypoplastic left heart syndrome, identifying clinical care changes with the potential to lead to improvements in outcome. It will also introduce the field of pediatric cardiology to the science of collaborative quality improvement and assist in reducing clinical process variation and improving patient outcomes across centers. Finally, it will establish an ongoing network of pediatric cardiologists and their teams linked through a longitudinal data set and collaboration for improvement and research. © 2009 Copyright the Author Journal compilation © 2009 Wiley Periodicals, Inc. AD - J.D. Kugler, UNMC/CUMC Joint Division of Pediatric Cardiology, Children's Hospital and Medical Center, 8200 Dodge Street, Omaha, NE 68114, United States AU - Kugler, J. D. AU - Beekmani, Iii R. H. AU - Rosenthal, G. L. AU - Jenkins, K. J. AU - Klitzner, T. S. AU - Martin, G. R. AU - Neish, S. R. AU - Lannon, C. DB - Embase Medline DO - 10.1111/j.1747-0803.2009.00328.x IS - 5 KW - article cardiovascular disease child health care child nutrition clinical protocol consensus development data base disease surveillance funding health care quality health center health program hypoplastic left heart syndrome information dissemination medical informatics parental behavior participatory management patient monitoring pediatric cardiology priority journal prognosis registration total quality management treatment planning LA - English M3 - Article N1 - L355242831 2009-10-30 PY - 2009 SN - 1747-079X 1747-0803 SP - 318-328 ST - Development of a pediatric cardiology quality improvement collaborative: From inception to implementation. From the joint council on congenital heart disease quality Improvement task force T2 - Congenital Heart Disease TI - Development of a pediatric cardiology quality improvement collaborative: From inception to implementation. From the joint council on congenital heart disease quality Improvement task force UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L355242831 http://dx.doi.org/10.1111/j.1747-0803.2009.00328.x VL - 4 ID - 1174 ER - TY - JOUR AB - OBJECTIVE: Mortality after surgery for congenital heart disease (CHD) has decreased. Quality of life (QOL) assessment in survivors has become increasingly important. The purpose of this project was to create the Pediatric Cardiac Quality of Life Inventory (PCQLI). METHODS: Items were generated through nominal groups of patients, parents, and providers. The pilot PCQLI was completed by children (age 8-12), adolescents (age 13-18), and their parents at three cardiology clinics. Item reduction was performed through analysis of items, principal components, internal consistency (IC), and patterns of correlation. RESULTS: A total of 655 patient-parent pairs completed the pilot PCQLI. Principal components identified included: impact of disease (ID); psychosocial impact (PI); and emotional environment (EE). After item reduction ID and PI had excellent IC (ID = 0.88-0.91; PI = 0.78-0.85) and correlated highly with each other (0.81-0.90) and with the total score (TS) (ID = 0.95-0.96; PI = 0.87-0.93). EE was not correlated with ID, PI, or TS and was removed from the final forms. Two-ventricle CHD patients had a higher TS than single-ventricle CHD patients across all forms (P < 0.001). CONCLUSION: The PCQLI has patient and parent-proxy forms, has wide age range, and discriminates between CHD subgroups. The ID and PI subscales of the PCQLI have excellent IC and correlate well with each other and the TS. AD - Department of Pediatrics, Divisions of Cardiology and Critical Care Medicine, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH 45229, USA. bradley.marino@cchmc.org AN - 18347927 AU - Marino, B. S. AU - Shera, D. AU - Wernovsky, G. AU - Tomlinson, R. S. AU - Aguirre, A. AU - Gallagher, M. AU - Lee, A. AU - Cho, C. J. AU - Stern, W. AU - Davis, L. AU - Tong, E. AU - Teitel, D. AU - Mussatto, K. AU - Ghanayem, N. AU - Gleason, M. AU - Gaynor, J. W. AU - Wray, J. AU - Helfaer, M. A. AU - Shea, J. A. DA - May DB - PubMed DO - 10.1007/s11136-008-9323-8 DP - NLM ET - 2008/03/19 IS - 4 KW - *Adaptation, Psychological Adolescent Cardiac Care Facilities Child Child Welfare Databases as Topic Feasibility Studies Female Heart Diseases/*psychology Humans Male Pilot Projects Psychometrics *Quality of Life/psychology LA - eng N1 - Marino, Bradley S Shera, David Wernovsky, Gil Tomlinson, Ryan S Aguirre, Abigail Gallagher, Maureen Lee, Angela Cho, Catherine J Stern, Whitney Davis, Lauren Tong, Elizabeth Teitel, David Mussatto, Kathleen Ghanayem, Nancy Gleason, Marie Gaynor, J William Wray, Jo Helfaer, Mark A Shea, Judy A Journal Article Research Support, Non-U.S. Gov't Validation Study Netherlands Qual Life Res. 2008 May;17(4):613-26. doi: 10.1007/s11136-008-9323-8. PY - 2008 SN - 0962-9343 (Print) 0962-9343 SP - 613-26 ST - The development of the pediatric cardiac quality of life inventory: a quality of life measure for children and adolescents with heart disease T2 - Qual Life Res TI - The development of the pediatric cardiac quality of life inventory: a quality of life measure for children and adolescents with heart disease VL - 17 ID - 267 ER - TY - JOUR AB - Objective: Mortality after surgery for congenital heart disease (CHD) has decreased. Quality of life (QOL) assessment in survivors has become increasingly important. The purpose of this project was to create the Pediatric Cardiac Quality of Life Inventory (PCQLI).Methods: Items were generated through nominal groups of patients, parents, and providers. The pilot PCQLI was completed by children (age 8-12), adolescents (age 13-18), and their parents at three cardiology clinics. Item reduction was performed through analysis of items, principal components, internal consistency (IC), and patterns of correlation.Results: A total of 655 patient-parent pairs completed the pilot PCQLI. Principal components identified included: impact of disease (ID); psychosocial impact (PI); and emotional environment (EE). After item reduction ID and PI had excellent IC (ID = 0.88-0.91; PI = 0.78-0.85) and correlated highly with each other (0.81-0.90) and with the total score (TS) (ID = 0.95-0.96; PI = 0.87-0.93). EE was not correlated with ID, PI, or TS and was removed from the final forms. Two-ventricle CHD patients had a higher TS than single-ventricle CHD patients across all forms (P < 0.001).Conclusion: The PCQLI has patient and parent-proxy forms, has wide age range, and discriminates between CHD subgroups. The ID and PI subscales of the PCQLI have excellent IC and correlate well with each other and the TS. AD - Department of Pediatrics, Divisions of Cardiology and Critical Care Medicine, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH 45229, USA AN - 105685318. Language: English. Entry Date: 20081107. Revision Date: 20171109. Publication Type: journal article AU - Marino, B. S. AU - Shera, D. AU - Wernovsky, G. AU - Tomlinson, R. S. AU - Aguirre, A. AU - Gallagher, M. AU - Lee, A. AU - Cho, C. J. AU - Stern, W. AU - Davis, L. AU - Tong, E. AU - Teitel, D. AU - Mussatto, K. AU - Ghanayem, N. AU - Gleason, M. AU - Gaynor, J. W. AU - Wray, J. AU - Helfaer, M. A. AU - Shea, J. A. AU - Marino, Bradley S. DB - ccm DO - 10.1007/s11136-008-9323-8 DP - EBSCOhost IS - 4 KW - Adaptation, Psychological Heart Diseases -- Psychosocial Factors Quality of Life -- Psychosocial Factors Adolescence Child Welfare Child Databases Female Hospitals, Special Male Pilot Studies Psychometrics Human N1 - research. Journal Subset: Allied Health; Public Health; USA. Instrumentation: Quality of Life Inventory (QOLI). NLM UID: 9210257. PMID: NLM18347927. PY - 2008 SN - 0962-9343 SP - 613-626 ST - The development of the pediatric cardiac quality of life inventory: a quality of life measure for children and adolescents with heart disease T2 - Quality of Life Research TI - The development of the pediatric cardiac quality of life inventory: a quality of life measure for children and adolescents with heart disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105685318&site=ehost-live&scope=site VL - 17 ID - 1474 ER - TY - JOUR AB - OBJECTIVE: To describe the outcome of children treated for hypoplastic left heart syndrome (HLHS) with heart transplantation. STUDY DESIGN: We evaluated outcomes in 26 children treated for HLHS in a single center; 13 children were evaluated with the Bayley Scales of Infant Development, Child Behavior Checklist (CBCL), and Vineland Adaptive Behavior Scales (VABS), and 13 were seen after 36 months of age and were evaluated with the Wechsler Preschool and Primary Scale of Intelligence, CBCL, and VABS at 36 to 72 months or the Wechsler Intelligence Scale for Children-III, CBCL, and VABS for those older than 72 months of age. RESULTS: Bayley Scales of Infant Development results revealed a median Mental Developmental Index of 88 (range <50 to 102) and a Psychomotor Developmental Index of 86.5 (<50 to 113), both significantly lower than expected in the general population. Intelligence quotient results on either the Wechsler Preschool and Primary Scale of Intelligence or Wechsler Intelligence Scale for Children-III were also significantly lower than expected, with a mean verbal score of 90.5 +/- 12.4, performance score of 88.9 +/- 14.5, and full scale score of 88.5 +/- 13.0. On the Vineland scales, 39% scored >1 SD below the mean on measures of daily living scales, 22% on the socialization subscale, 48% on the communication subscale, and 52% on the adaptive behavior scale. CONCLUSIONS: In this small population of children treated for HLHS with heart transplantation, both cognitive deficits and adaptive/behavioral abnormalities are described. Early identification with appropriate referral for services could potentially enhance the outcomes for these children. AD - Section of Neonatology, Department of Pediatrics, University of Colorado School of Medicine, Denver, Colorado, USA. AN - 12520249 AU - Ikle, L. AU - Hale, K. AU - Fashaw, L. AU - Boucek, M. AU - Rosenberg, A. A. DA - Jan DB - PubMed DO - 10.1067/mpd.2003.mpd0340 DP - NLM ET - 2003/01/10 IS - 1 KW - Child *Child Behavior *Child Development *Cognition Developmental Disabilities/*etiology Female *Heart Transplantation Humans Hypoplastic Left Heart Syndrome/physiopathology/*psychology/*surgery Infant Infant, Newborn Intelligence Intelligence Tests Male Neuropsychological Tests Social Behavior Treatment Outcome LA - eng N1 - Ikle, Linda Hale, Kathy Fashaw, Lucy Boucek, Mark Rosenberg, Adam A Comment Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S. United States J Pediatr. 2003 Jan;142(1):20-5. doi: 10.1067/mpd.2003.mpd0340. PY - 2003 SN - 0022-3476 (Print) 0022-3476 SP - 20-5 ST - Developmental outcome of patients with hypoplastic left heart syndrome treated with heart transplantation T2 - J Pediatr TI - Developmental outcome of patients with hypoplastic left heart syndrome treated with heart transplantation VL - 142 ID - 211 ER - TY - JOUR AB - OBJECTIVE: The hemodynamic, respiratory, and sedative effects of dexmedetomidine (DEX) for pediatric patients post-Fontan surgery. DESIGN: Retrospective. SETTING: Single institutional intensive care unit. PARTICIPANTS: Fourteen patients undergoing Fontan-type surgery. RESULT: A retrospective review was conducted on 14 pediatric patients who had undergone a Fontan procedure for congenital heart disease. A vital component of postoperative management of these patients is to prevent an increase in pulmonary vascular resistance (PVR) that may lead to a serious reduction in cardiac output. DEX an alpha-2 adrenergic receptor agonist might offer an advantage over current sedation methods in preventing a rise in PVR. Nine patients received sedation with DEX and five patients in a control group were administered standard regimens of sedation and analgesia. The DEX group exhibited no evidence of an increased partial pressure of arterial carbon dioxide postoperatively as opposed to the control group. This lack of respiratory depression made the DEX group less likely to increase their PVR. However, the DEX group did experience a significant incidence of bradycardia that required the use of a cardiac pacemaker. CONCLUSIONS: The results of this retrospective review of the role of DEX in the management of the post-Fontan surgical pediatric patient indicate some potential advantages. AD - Intensive Care Unit, Osaka City General Hospital, Miyakojima-ku, Osaka, Japan. natsueteto@yahoo.co.jp AN - 19188869 AU - Tokuhira, N. AU - Atagi, K. AU - Shimaoka, H. AU - Ujiro, A. AU - Otsuka, Y. AU - Ramsay, M. DA - Mar DB - PubMed DO - 10.1097/PCC.0b013e31819a3a3e DP - NLM ET - 2009/02/04 IS - 2 KW - Adrenergic alpha-Agonists/*administration & dosage Child Child, Preschool Dexmedetomidine/*administration & dosage Female *Fontan Procedure Humans Hypnotics and Sedatives/*administration & dosage Infant Male *Pediatrics Retrospective Studies LA - eng N1 - Tokuhira, Natusko Atagi, Kazuaki Shimaoka, Hideki Ujiro, Atsushi Otsuka, Yasunori Ramsay, Michael Journal Article United States Pediatr Crit Care Med. 2009 Mar;10(2):207-12. doi: 10.1097/PCC.0b013e31819a3a3e. PY - 2009 SN - 1529-7535 (Print) 1529-7535 SP - 207-12 ST - Dexmedetomidine sedation for pediatric post-Fontan procedure patients T2 - Pediatr Crit Care Med TI - Dexmedetomidine sedation for pediatric post-Fontan procedure patients VL - 10 ID - 287 ER - TY - JOUR AB - A review largely based on the clinical and laboratory findings in 96 newborns who were also cardiac patients with cyanosis or respiratory distress in the first week and without major noncardiovascular anomalies or noncardiac disease. All patiente had cardiac catheterization or cardiac surgery or were autopsied at the Children's Hospital, Buffalo, during a 5-yr period. After a short discussion on the hemodynamic changes occurring during the first hours and days of life, the characteristics of the following defects are presented; complete transposition of the great vessels (22 cases), hypoplastic left heart syndrome (21), tetralogy of Fallot (12), multiple major defects (10), coarctation of the aorta with ventricular septal defect (6), single ventricle (4), pulmonary atresia (4), pulmonic stenosis (3), tricuspid atresia (3), tricuspid regurgitation (2), interruption of the aortic arch (2), systemic arteriovenous fistula (2), aortic stenosis (1), Ebstein's anomaly (1), asplenia complex (1), truncus arteriovenosus (1), pulmonary regurgitation (1) Other congenital defects causing symptoms in the first week of life and encountered in the study arc also presented; common atrioventricular canal, vascular rings, total anomalous pulmonary venous drainage with venous obstruction, endocardial fibroelastosis. The delayed appearance of severe cardiac manifestations in most cases with certain common congenital cardiac defects is discussed. The differential diagnosis is reviewed: some manifestations in normal infants, in polycythemia due to delayed clamping of the cord or fetal transfusion syndrome, effects of some drugs absorbed before delivery, heart diseases other than congenital malformations, various noncardiac causes of cyanosis, anemia. The diagnostic possibilities are discussed: the history and the physical examination, the electrocardiogram, X-ray and cardiac catheterization. Infants with persistent cyanosis, respiratory distress or a loud murmur should be referred in the first week of life to a specialized center where an immediate physical examination, a chest X-ray and ECG should be performed. If a serious cardiac anomaly is suspected consultation with the cardiac surgeon should be obtained and in most cases cardiac catheterization and selective angiocardiography performed. Patients with multiple severe associated noncardiac anomalies or moribund infants need not be studied. Patients with congenital heart disease but not in distress will require only careful observation. Vigorous medical measures are often necessary shortly before catheterization. The congenital anomalies are divided in 3 groups: (1) operation to be performed as soon as the diagnosis seems clear; (2) operation to be deferred as long as the patient is not losing ground; (3) a small group for which no surgery is available but a vigorous medical management is often needed. The performance of operation is a matter of judgment in concrete cases and depends on the excellence of the surgery available. AD - E.C. Lambert, Child. Hosp., Buffalo, NY, United States AU - Lambert, E. C. AU - Tingelstad, J. B. AU - Hohn, A. R. DB - Embase Classic Medline IS - 4 KW - anemia angiocardiography aorta aortic arch aortic stenosis arteriovenous fistula asplenia blood transfusion reaction buffalo cardiac patient catheterization congenital disorder congenital heart disease congenital malformation consultation cyanosis decision making diagnosis differential diagnosis disease management dying Ebstein anomaly electrocardiogram endocardial cushion defect endocardial fibroelastosis Fallot tetralogy great blood vessel heart catheterization heart disease heart single ventricle heart surgery heart ventricle septum defect hypoplastic left heart syndrome infant intrauterine blood transfusion laboratory pulmonary valve atresia lung vein drainage anomaly newborn patient pediatric hospital physical examination polycythemia pulmonary valve insufficiency respiratory distress stenosis surgeon surgery thorax radiography tricuspid valve atresia tricuspid valve regurgitation trunk vascular ring vein occlusion X ray LA - English M3 - Article N1 - L287039128 1966-12-01 PY - 1966 SN - 0031-3955 SP - 943-982 ST - Diagnosis and management of congenital heart disease in the first week of life T2 - Pediatric Clinics of North America TI - Diagnosis and management of congenital heart disease in the first week of life UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L287039128 VL - 13 ID - 1412 ER - TY - JOUR AB - Since the original description, the Fontan operation has been widely used for the palliation of children with single ventricle physiology. Although the Fontan operation revolutionized the survival rates of patients with single ventricle physiology, it carries an inevitable risk for long-term morbidity and mortality that impacts clinical outcomes and quality of life. This review will focus on the evaluation and treatment of the patient with the failing Fontan phenotype, with an emphasis on creating an individualized treatment plan. AD - Department of Pediatrics, Section of Pediatric and Adult Congenital Cardiology, Texas Children's Hospital/Baylor College of Medicine, Houston, TX, USA. Division of Pediatric Cardiology, Children's National Hospital, George Washington University, Washington, DC, USA. Division of Pediatric Cardiology, Children's National Hospital, George Washington University, Washington, DC, USA. anjohn@cnmc.org. Division of Pediatric Cardiology, Children's National Hospital, 111 Michigan Ave, NW, Washington, DC, 20010, USA. anjohn@cnmc.org. AN - 32185561 AU - Broda, C. R. AU - Downing, T. E. AU - John, A. S. DA - Jul DB - PubMed DO - 10.1007/s10741-020-09932-0 DP - NLM ET - 2020/03/19 IS - 4 KW - Congenital heart disease Fontan Heart failure Single ventricle LA - eng N1 - 1573-7322 Broda, Christopher R Downing, Tacy E John, Anitha S Orcid: 0000-0002-7355-6304 Journal Article Review United States Heart Fail Rev. 2020 Jul;25(4):633-646. doi: 10.1007/s10741-020-09932-0. PY - 2020 SN - 1382-4147 SP - 633-646 ST - Diagnosis and management of the adult patient with a failing Fontan circulation T2 - Heart Fail Rev TI - Diagnosis and management of the adult patient with a failing Fontan circulation VL - 25 ID - 400 ER - TY - JOUR AB - Background - The goal of this statement is to review available literature and to put forth a scientific statement on the current practice of fetal cardiac medicine, including the diagnosis and management of fetal cardiovascular disease. Methods and Results - A writing group appointed by the American Heart Association reviewed the available literature pertaining to topics relevant to fetal cardiac medicine, including the diagnosis of congenital heart disease and arrhythmias, assessment of cardiac function and the cardiovascular system, and available treatment options. The American College of Cardiology/ American Heart Association classification of recommendations and level of evidence for practice guidelines were applied to the current practice of fetal cardiac medicine. Recommendations relating to the specifics of fetal diagnosis, including the timing of referral for study, indications for referral, and experience suggested for performance and interpretation of studies, are presented. The components of a fetal echocardiogram are described in detail, including descriptions of the assessment of cardiac anatomy, cardiac function, and rhythm. Complementary modalities for fetal cardiac assessment are reviewed, including the use of advanced ultrasound techniques, fetal magnetic resonance imaging, and fetal magnetocardiography and electrocardiography for rhythm assessment. Models for parental counseling and a discussion of parental stress and depression assessments are reviewed. Available fetal therapies, including medical management for arrhythmias or heart failure and closed or open intervention for diseases affecting the cardiovascular system such as twin-twin transfusion syndrome, lung masses, and vascular tumors, are highlighted. Catheter-based intervention strategies to prevent the progression of disease in utero are also discussed. Recommendations for delivery planning strategies for fetuses with congenital heart disease including models based on classification of disease severity and delivery room treatment will be highlighted. Outcome assessment is reviewed to show the benefit of prenatal diagnosis and management as they affect outcome for babies with congenital heart disease. Conclusions - Fetal cardiac medicine has evolved considerably over the past 2 decades, predominantly in response to advances in imaging technology and innovations in therapies. The diagnosis of cardiac disease in the fetus is mostly made with ultrasound; however, new technologies, including 3- and 4-dimensional echocardiography, magnetic resonance imaging, and fetal electrocardiography and magnetocardiography, are available. Medical and interventional treatments for select diseases and strategies for delivery room care enable stabilization of high-risk fetuses and contribute to improved outcomes. This statement highlights what is currently known and recommended on the basis of evidence and experience in the rapidly advancing and highly specialized field of fetal cardiac care. © 2014 American Heart Association, Inc. AU - Donofrio, M. T. AU - Moon-Grady, A. J. AU - Hornberger, L. K. AU - Copel, J. A. AU - Sklansky, M. S. AU - Abuhamad, A. AU - Cuneo, B. F. AU - Huhta, J. C. AU - Jonas, R. A. AU - Krishnan, A. AU - Lacey, S. AU - Lee, W. AU - Michelfelder Sr, E. C. AU - Rempel, G. R. AU - Silverman, N. H. AU - Spray, T. L. AU - Strasburger, J. F. AU - Tworetzky, W. AU - Rychik, J. DB - Scopus DO - 10.1161/01.cir.0000437597.44550.5d IS - 21 KW - AHA scientific statements Cardiology pediatric Congenital Fetus Heart defects congenital M3 - Article N1 - Cited By :366 Export Date: 15 June 2020 PY - 2014 SP - 2183-2242 ST - Diagnosis and treatment of fetal cardiac disease: A scientific statement from the american heart association T2 - Circulation TI - Diagnosis and treatment of fetal cardiac disease: A scientific statement from the american heart association UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84901621369&doi=10.1161%2f01.cir.0000437597.44550.5d&partnerID=40&md5=8519115747284dd25a3a79c24dde5c13 VL - 129 ID - 1992 ER - TY - JOUR AB - OBJECTIVES: Diastolic dysfunction (DD), a key driver of long-term Fontan outcomes, may be concealed during standard haemodynamic evaluation. We sought to identify Fontan patients with occult DD using 'ventricular stress testing' with rapid volume expansion (RVE). METHODS: Cardiac catheterisation with RVE was performed routinely in Fontan patients between 11/2012 and 4/2015. Baseline and post-stress haemodynamic data were compared using t test, Mann-Whitney U test, χ(2) and Fisher's exact tests. A post-stress ventricular end diastolic pressure (EDP) threshold of 15 mm Hg defined occult DD. RESULTS: Forty-six Fontan patients (48% female, median age 14.1 (IQR 9.1 to 21.3) years) were included. The median Fontan duration was 10.8 (IQR 5.1 to 17.8) years and dominant left ventricular morphology was present in 63% of patients. Volume expansion increased mean Fontan pressure (15.2±2.5 vs 12.4±2.2 mm Hg, p<0.001), pulmonary capillary wedge pressure (11.3±2.6 vs 7.9±2 mm Hg, p<0.001) and EDP (12.7±3.3 vs 8.5±2.1 mm Hg, p<0.001). Sixteen patients (35%) had occult DD, demonstrating higher baseline EDP (10.3±1.9 vs 7.6±1.5 mm Hg, p<0.001) and greater increase in EDP (6.3±2.4 vs 3.1±1.4 mm Hg, p<0.001) compared with patients without DD. Higher baseline EDP, lower baseline cardiac index and longer duration of Fontan circulation were associated with higher post-stress EDP. There were no complications related to RVE. CONCLUSIONS: Ventricular stress testing by RVE is feasible, safe and identifies a subgroup of Fontan patients with occult DD. Higher baseline EDP and longer duration of Fontan circulation are associated with worse diastolic function. Future work is necessary to better understand the aetiology, associations and clinical implications of occult DD in Fontan survivors. AD - The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA. Department of Pediatrics (Cardiology), University of Arizona, Tucson, Arizona, USA. AN - 26917538 AU - Averin, K. AU - Hirsch, R. AU - Seckeler, M. D. AU - Whiteside, W. AU - Beekman, R. H., 3rd AU - Goldstein, B. H. DA - Jul 15 DB - PubMed DO - 10.1136/heartjnl-2015-309042 DP - NLM ET - 2016/02/27 IS - 14 KW - Adolescent Cardiac Catheterization/*methods Chi-Square Distribution Child Diastole Feasibility Studies Female Fontan Procedure/*adverse effects Heart Defects, Congenital/diagnosis/physiopathology/*surgery Humans Linear Models Logistic Models Male Predictive Value of Tests Retrospective Studies Time Factors Treatment Outcome Ventricular Dysfunction/*diagnosis/etiology/physiopathology *Ventricular Function Ventricular Pressure Young Adult LA - eng N1 - 1468-201x Averin, Konstantin Hirsch, Russel Seckeler, Michael D Whiteside, Wendy Beekman, Robert H 3rd Goldstein, Bryan H Journal Article England Heart. 2016 Jul 15;102(14):1109-14. doi: 10.1136/heartjnl-2015-309042. Epub 2016 Feb 25. PY - 2016 SN - 1355-6037 SP - 1109-14 ST - Diagnosis of occult diastolic dysfunction late after the Fontan procedure using a rapid volume expansion technique T2 - Heart TI - Diagnosis of occult diastolic dysfunction late after the Fontan procedure using a rapid volume expansion technique VL - 102 ID - 203 ER - TY - JOUR AB - BACKGROUND: Hypoplastic left heart syndrome (HLHS) is characterized by underdevelopment of the left ventricle (LV) and increased biomechanical stress on the right ventricle (RV) from single ventricle physiology. Despite the clinical significance, the signaling pathways active during RV remodeling and disease progression are not known. To address this, we examined differential changes in expression of genes associated with transforming growth factor-beta (TGF-beta)/bone morphogenetic protein (BMP) signaling in RV tissue isolated from HLHS patients relative to RV and LV tissue from control subjects. METHODS AND RESULTS: Quantitative real-time polymerase chain reaction was used to detect changes in expression of 84 genes involved in TGF-beta/BMP-mediated cardiac development, cell growth, and differentiation in RV tissue collected from 6 neonates with HLHS undergoing stage 1 Norwood procedure (age, 1-7 days; mean, 4 days) and RV and LV tissue obtained from 5 infants with noncardiac pathology (age range, 1-135 days: mean, 85 days) that served as controls. Analysis of gene expression profiles between control-LV and control-RV revealed significant depression of TGF-beta/BMP signaling in RV compared with LV. Of the 84 genes analyzed, 38 were differentially expressed between HLHS-RV and control-RV, whereas only 22 compared with control-LV. Significant changes were observed in: tissue remodeling genes including Activin receptor type IIA (ACVR2A) (+2.13) and Activin receptor-like kinase 1 (ACVRL1) (+2.22); and cell survival, growth, and differentiation genes including CDC25A (+2.18), p21 (-3.64), p15 (+2.15), BMP5 (+4.58), BMP3 (+2.16), GDF3 (+8.59), NODAL (+2.32), and BMP binding endothelial regulator (BMPER) (+4.58). The most significant changes common to HLHS-RV versus control-RV and control-LV sample groups is observed for Anti müllerian hormone receptor 2 (AMHR2) (+18.79 control-RV, +3.38 control-LV), and the BMP antagonist Inhibin alpha (INHA) (+11.47 control-RV, +5.73 control-LV). CONCLUSIONS: Although this descriptive study does not allow cause-effect inferences, our results suggest changes in cardiac development pathways and upregulation of genes associated with cell growth and differentiation in the neonatal RV of children with HLHS. These molecular profiles are more closely related to those observed in the normal LV rather than normal RV at similar maturational age. This work provides the basis for future mechanistic studies to elucidate the molecular mechanisms regulating RV remodeling in HLHS. AD - Division of Cardiothoracic Surgery, University of Miami Miller School of Medicine and Holtz Children's Hospital, Miami, FL, USA. mricci@med.miami.edu AN - 104914607. Language: English. Entry Date: 20110520. Revision Date: 20150711. Publication Type: Journal Article AU - Ricci, M. AU - Mohapatra, B. AU - Urbiztondo, A. AU - Birusingh, R. J. AU - Morgado, M. AU - Rodriguez, M. M. AU - Lincoln, J. AU - Vatta, M. DB - ccm DO - 10.1016/j.cardfail.2010.03.007 DP - EBSCOhost IS - 8 KW - Bone Morphogenetic Proteins -- Physiology Heart Ventricle -- Pathology Hypoplastic Left Heart Syndrome -- Metabolism Myocardium -- Metabolism Signal Transduction -- Physiology Growth Substances -- Physiology Prospective Studies Female Heart Ventricle -- Physiopathology Human Hypoplastic Left Heart Syndrome -- Pathology Hypoplastic Left Heart Syndrome -- Surgery Infant Infant, Newborn Male Myocardium -- Pathology N1 - research. Journal Subset: Biomedical; Double Blind Peer Reviewed; Peer Reviewed; USA. NLM UID: 9442138. PMID: NLM20670841. PY - 2010 SN - 1071-9164 SP - 628-634 ST - Differential changes in TGF-beta/BMP signaling pathway in the right ventricular myocardium of newborns with hypoplastic left heart syndrome T2 - Journal of Cardiac Failure TI - Differential changes in TGF-beta/BMP signaling pathway in the right ventricular myocardium of newborns with hypoplastic left heart syndrome UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104914607&site=ehost-live&scope=site VL - 16 ID - 1569 ER - TY - JOUR AB - BACKGROUND: Hypoplastic left heart syndrome (HLHS) is characterized by underdevelopment of the left ventricle (LV) and increased biomechanical stress on the right ventricle (RV) from single ventricle physiology. Despite the clinical significance, the signaling pathways active during RV remodeling and disease progression are not known. To address this, we examined differential changes in expression of genes associated with transforming growth factor-beta (TGF-beta)/bone morphogenetic protein (BMP) signaling in RV tissue isolated from HLHS patients relative to RV and LV tissue from control subjects. METHODS AND RESULTS: Quantitative real-time polymerase chain reaction was used to detect changes in expression of 84 genes involved in TGF-beta/BMP-mediated cardiac development, cell growth, and differentiation in RV tissue collected from 6 neonates with HLHS undergoing stage 1 Norwood procedure (age, 1-7 days; mean, 4 days) and RV and LV tissue obtained from 5 infants with noncardiac pathology (age range, 1-135 days: mean, 85 days) that served as controls. Analysis of gene expression profiles between control-LV and control-RV revealed significant depression of TGF-beta/BMP signaling in RV compared with LV. Of the 84 genes analyzed, 38 were differentially expressed between HLHS-RV and control-RV, whereas only 22 compared with control-LV. Significant changes were observed in: tissue remodeling genes including Activin receptor type IIA (ACVR2A) (+2.13) and Activin receptor-like kinase 1 (ACVRL1) (+2.22); and cell survival, growth, and differentiation genes including CDC25A (+2.18), p21 (-3.64), p15 (+2.15), BMP5 (+4.58), BMP3 (+2.16), GDF3 (+8.59), NODAL (+2.32), and BMP binding endothelial regulator (BMPER) (+4.58). The most significant changes common to HLHS-RV versus control-RV and control-LV sample groups is observed for Anti müllerian hormone receptor 2 (AMHR2) (+18.79 control-RV, +3.38 control-LV), and the BMP antagonist Inhibin alpha (INHA) (+11.47 control-RV, +5.73 control-LV). CONCLUSIONS: Although this descriptive study does not allow cause-effect inferences, our results suggest changes in cardiac development pathways and upregulation of genes associated with cell growth and differentiation in the neonatal RV of children with HLHS. These molecular profiles are more closely related to those observed in the normal LV rather than normal RV at similar maturational age. This work provides the basis for future mechanistic studies to elucidate the molecular mechanisms regulating RV remodeling in HLHS. AD - Division of Cardiothoracic Surgery, University of Miami Miller School of Medicine and Holtz Children's Hospital, Miami, FL, USA. mricci@med.miami.edu AN - 20670841 AU - Ricci, M. AU - Mohapatra, B. AU - Urbiztondo, A. AU - Birusingh, R. J. AU - Morgado, M. AU - Rodriguez, M. M. AU - Lincoln, J. AU - Vatta, M. DA - Aug DB - PubMed DO - 10.1016/j.cardfail.2010.03.007 DP - NLM ET - 2010/07/31 IS - 8 KW - Bone Morphogenetic Proteins/*physiology Cohort Studies Female Heart Ventricles/*pathology/physiopathology Humans Hypoplastic Left Heart Syndrome/*metabolism/pathology/surgery Infant Infant, Newborn Male Myocardium/*metabolism/pathology Signal Transduction/*physiology Transforming Growth Factor beta/*physiology LA - eng N1 - 1532-8414 Ricci, Marco Mohapatra, Bhagyalaxmi Urbiztondo, Arnel Birusingh, Rhea J Morgado, Micaela Rodriguez, Maria M Lincoln, Joy Vatta, Matteo Comparative Study Journal Article Research Support, Non-U.S. Gov't United States J Card Fail. 2010 Aug;16(8):628-34. doi: 10.1016/j.cardfail.2010.03.007. Epub 2010 May 4. PY - 2010 SN - 1071-9164 SP - 628-34 ST - Differential changes in TGF-β/BMP signaling pathway in the right ventricular myocardium of newborns with hypoplastic left heart syndrome T2 - J Card Fail TI - Differential changes in TGF-β/BMP signaling pathway in the right ventricular myocardium of newborns with hypoplastic left heart syndrome VL - 16 ID - 317 ER - TY - JOUR AB - PURPOSE: Progressive impairment of hemodynamics in patients with Fontan circulation is common, multifactorial, and associated with decreased quality of life and increased morbidity. We sought to assess hemodynamic differences between patients with preserved (preserved Fontans) and those with impaired circulation (impaired Fontans) after pulmonary vasodilation using oxygen and under forced breathing conditions. MATERIALS AND METHODS: Real-time phase-contrast cardiovascular magnetic resonance was performed using non-ECG triggered echo-planar imaging (temporal resolution=24 to 28 ms) in the ascending aorta (AAo) and superior vena cava (SVC)/inferior vena cava (IVC) on room air, after 100% oxygen inhalation (4 L/min; 10 min) and on forced breathing in 29 Fontan patients (17.2±7.3 y) and in 32 controls on room air (13.4±3.7 y). The simultaneously recorded patients' respiratory cycle was divided into 4 segments (expiration, end-expiration, inspiration, and end-inspiration) to generate respiratory-dependent stroke volumes (SVs). The imaging data were matched with physiological data and analyzed with home-made software. RESULTS: The mean SVi (AAo) was 46.1±11.1 mL/m in preserved Fontans versus 30.4±6.2 mL/m in impaired Fontans (P=0.002) and 51.1±6.9 mL/m in controls (P=0.107). The cutoff value for differentiation of Fontan groups was SVi (AAo, end-expiratory) of 32.1 mL/m. After hyperoxygenation, the mean SVi (AAo) increased to 48.7±12.7 mL/m in preserved Fontans (P=0.045) but remained unchanged in impaired Fontans (31.1±5.8 mL/m, P=0.665). Simultaneously, heart rates decreased from 75.2±15.9 to 70.8±16.4 bpm (preserved; P=0.000) but remained unchanged in impaired circulation (baseline: 84.1±9.8 bpm, P=0.612). Compared with physiological respiration, forced breathing increased the maximum respiratory-related cardiac index difference (ΔCImax) in preserved Fontans (SVC: 2.5-fold, P=0.000; and IVC: 1.8-fold, P=0.000) and to a lower extent in impaired Fontans (both veins, 1.5-fold; P(SVC)=0.011, P(IVC)=0.013). There was no impact on mean blood flow. CONCLUSIONS: Oxygen affected the pulmonary vascular system by vasodilation and increased SVi in preserved Fontans but had no effect on impaired Fontans. Forced breathing increased ΔCImax but did not change the mean blood flow by sole activation of the ventilatory pump. End-expiratory aortic SVi represents a valuable measure for classifying the severity of Fontan hemodynamics impairment. AD - *Institute for Radiology, Nuclear Medicine and Molecular Imaging †Department for Congenital Heart Defects, Heart and Diabetes Centre North Rhine Westphalia Ruhr University Bochum, Bad Oeynhausen ‡Department of Radiology, University of Bonn, Bonn §Department for Pediatric Cardiology and Pediatric Intensive Care, Ludwig Maximilians University, Medical Hospital of the University of Munich, Campus Grosshadern, Munich, Germany. AN - 28346330 AU - Körperich, H. AU - Müller, K. AU - Barth, P. AU - Gieseke, J. AU - Haas, N. AU - Schulze-Neick, I. AU - Burchert, W. AU - Kececioglu, D. AU - Laser, K. T. C2 - PMC5538303 identification No:W-BDO-019/2013. The authors declare no conflict of interest. DA - May DB - PubMed DO - 10.1097/rti.0000000000000261 DP - NLM ET - 2017/03/28 IS - 3 KW - Adolescent Adult Aorta/diagnostic imaging/*physiopathology Child Diagnosis, Differential Echo-Planar Imaging/methods Female *Fontan Procedure Heart Rate/physiology Hemodynamics/*physiology Humans Magnetic Resonance Imaging/*methods Male Prospective Studies Vena Cava, Inferior/diagnostic imaging/*physiopathology Vena Cava, Superior/diagnostic imaging/*physiopathology Young Adult LA - eng N1 - 1536-0237 Körperich, Hermann Müller, Katja Barth, Peter Gieseke, Jürgen Haas, Nikolaus Schulze-Neick, Ingram Burchert, Wolfgang Kececioglu, Deniz Laser, Kai T Journal Article J Thorac Imaging. 2017 May;32(3):159-168. doi: 10.1097/RTI.0000000000000261. PY - 2017 SN - 0883-5993 (Print) 0883-5993 SP - 159-168 ST - Differentiation of Impaired From Preserved Hemodynamics in Patients With Fontan Circulation Using Real-time Phase-velocity Cardiovascular Magnetic Resonance T2 - J Thorac Imaging TI - Differentiation of Impaired From Preserved Hemodynamics in Patients With Fontan Circulation Using Real-time Phase-velocity Cardiovascular Magnetic Resonance VL - 32 ID - 249 ER - TY - JOUR AB - Background-Interstage mortality (IM) remains significant after stage 1 palliation (S1P) for single-ventricle heart disease (SVD), with many deaths sudden and unexpected. We sought to determine whether digoxin use post-S1P is associated with reduced IM, utilizing the multicenter database of the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC). Methods and Results-From June 2008 to July 2013, 816 infants discharged after S1P from 50 surgical sites completed the interstage to stage II palliation, transplant, or IM. Arrhythmia during S1P hospitalization or discharge on antiarrhythmic medications were exclusions (n=270); 2 patients were lost to follow-up. Two analyses were performed: (1) propensity-score adjusted logistic regression with IM as outcome and (2) retrospective cohort analysis for patients discharged on digoxin versus not, matched for surgical site and other established IM risk factors. Of 544 study patients, 119 (21.9%) were discharged on digoxin. Logistic regression analysis with propensity score, site-size group, and digoxin use as predictor variables showed an increased risk of IM in those not discharged on digoxin (odds ratio, 8.6; lower confidence limit, 1.9; upper confidence limit, 38.3; P<0.01). The retrospective cohort analysis for 60 patients on digoxin (matched for site of care, type of S1P, post-S1P ECMO use, genetic syndrome, discharge feeding route, ventricular function, tricuspid regurgitation, and aortic arch gradient) showed 0% IM in the digoxin at discharge group and an estimated IM difference between the 2 groups of 9% (P=0.04). Conclusions-Among SVD infants in the NPCQIC database discharged post-S1P with no history of arrhythmia, use of digoxin at discharge was associated with reduced IM. AD - D.W. Brown, Department of Cardiology, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, United States AU - Brown, D. W. AU - Mangeot, C. AU - Anderson, J. B. AU - Peterson, L. E. AU - King, E. C. AU - Lihn, S. L. AU - Neish, S. R. AU - Fleishman, C. AU - Phelps, C. AU - Hanke, S. AU - Beekman, R. H. AU - Lannon, C. M. DB - Embase Medline DO - 10.1161/JAHA.115.002376 IS - 1 KW - digoxin aortic arch article cardiovascular mortality cohort analysis drug use extracorporeal oxygenation female genetic disorder heart arrhythmia heart disease heart ventricle function hospital discharge human infant major clinical study male palliative therapy priority journal retrospective study risk reduction single ventricle heart disease survival rate tricuspid valve regurgitation LA - English M3 - Article N1 - L613254259 2016-12-05 2019-12-23 PY - 2016 SN - 2047-9980 ST - Digoxin use is associated with reduced interstage mortality in patients with no history of arrhythmia after stage i palliation for single ventricle heart disease T2 - Journal of the American Heart Association TI - Digoxin use is associated with reduced interstage mortality in patients with no history of arrhythmia after stage i palliation for single ventricle heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L613254259 http://dx.doi.org/10.1161/JAHA.115.002376 VL - 5 ID - 838 ER - TY - JOUR AB - We report the use of a dilated right internal thoracic artery as a systemic-pulmonary shunt in a patient with a single ventricle who developed a systemic-pulmonary shunt stenosis following a modified Norwood's procedure. The systemic-to-pulmonary artery shunt was performed at 13 months of age, and the patient has stable oxygen saturations five years after the surgery. © 2016 Wiley Periodicals, Inc. AD - Department of Cardiovascular Surgery, Nara Hospital, Kinki University School of Medicine, Nara, Japan Department of Thoracic and Cardiovascular Surgery, Nara Medical University, Nara, Japan AU - Kaneda, K. AU - Yokoyama, S. AU - Nagato, H. AU - Nagasaka, S. AU - Nishiwaki, N. AU - Taniguchi, S. DB - Scopus DO - 10.1111/jocs.12816 IS - 10 M3 - Article N1 - Export Date: 15 June 2020 PY - 2016 SP - 639-641 ST - Dilated right internal thoracic artery as a systemic pulmonary shunt T2 - Journal of Cardiac Surgery TI - Dilated right internal thoracic artery as a systemic pulmonary shunt UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84989236159&doi=10.1111%2fjocs.12816&partnerID=40&md5=3ca8c22a57f48c2ea05af19172756715 VL - 31 ID - 1891 ER - TY - JOUR AB - Patients who have undergone Fontan's operation are known to have impaired cardiac output response to dynamic exercise. This may be due to either poor cardiac function or a limited ability to mobilize blood from capacitance vessels due to increased resting venous tone. We tested the latter hypothesis by determining venous vascular capacitance at rest and during orthostatic stress produced by lower body negative pressure (LBNP) in 6 subjects who had undergone the Fontan operation and 6 healthy age-, sex-, height-, and weight-matched controls. Resting blood volume was similar for Fontan and control subjects (79 +/- 6 vs 70 +/- 3 ml/kg body weight, respectively), while central venous pressure (CVP) was elevated in Fontan subjects (18.4 +/- 1.0 vs 3.5 +/- 0.9 mm Hg, p < 0.05). Forearm venous capacitance at a distending pressure of 40 mm Hg was less in Fontan subjects than in controls (2.6 +/- 0.1 vs 3.9 +/- 0.5 ml/100 ml), while resting plasma norepinephrine level was elevated in Fontan subjects (255 +/- 28 vs 144 +/- 9 pg/ml, p < 0.05). The increase in calf volume (1.6 +/- 0.2 vs 2.3 +/- 0.2 ml) and decrease in CVP (-5.0 +/- 0.5 vs -6.7 +/- 1.1 mm Hg) during -30 mm Hg LBNP were smaller for Fontan than control subjects (p < 0.05). Reduced forearm venous capacitance and diminished pooling of blood into capacitance vessels of the leg during orthostatic stress indicated higher venous tone in Fontan than control subjects.(ABSTRACT TRUNCATED AT 250 WORDS) AD - Department of Pediatrics, Yale University School of Medicine, Yale-New Haven Hospital, Connecticut, USA. AN - 7611151 AU - Kelley, J. R. AU - Mack, G. W. AU - Fahey, J. T. DA - Jul 15 DB - PubMed DO - 10.1016/s0002-9149(99)80049-6 DP - NLM ET - 1995/07/15 IS - 3 KW - Adolescent Adult Analysis of Variance Dye Dilution Technique Exercise Test/methods/statistics & numerical data *Fontan Procedure/statistics & numerical data Heart/*physiopathology Heart Ventricles/physiopathology Hemodynamics Humans Lower Body Negative Pressure/methods/statistics & numerical data Postoperative Period Rest/physiology Time Factors *Vascular Resistance LA - eng N1 - Kelley, J R Mack, G W Fahey, J T HL07272/HL/NHLBI NIH HHS/United States HL20634/HL/NHLBI NIH HHS/United States RR06022/RR/NCRR NIH HHS/United States Comparative Study Journal Article Research Support, U.S. Gov't, P.H.S. United States Am J Cardiol. 1995 Jul 15;76(3):158-63. doi: 10.1016/s0002-9149(99)80049-6. PY - 1995 SN - 0002-9149 (Print) 0002-9149 SP - 158-63 ST - Diminished venous vascular capacitance in patients with univentricular hearts after the Fontan operation T2 - Am J Cardiol TI - Diminished venous vascular capacitance in patients with univentricular hearts after the Fontan operation VL - 76 ID - 472 ER - TY - JOUR AB - Objective The objective is to quantify radiation dose in children undergoing cardiac catheterization and determine the impact of increased reporting transparency on total radiation exposure. Background: Cardiac catheterization (cath) can result in significant radiation exposure in children. There has been growing interest in quantifying and reducing radiation exposure in pediatric cath procedures. Our center underwent a slight change in practice recently that resulted in direct physician reporting of radiation dose following every case. Methods We reviewed cath procedures across three different eras in four cath categories: post-heart transplant annual cath, unilateral pulmonary artery (PA) stent placement, pre-Fontan cath, and pre-Glenn cath. The eras were defined as: Era 1, 1/2009 - 1/2011; Era 2, 1/2011 - 9/2013; and Era 3, 9/2013 - 5/2014. In Era 3, the physician performing the cath was responsible for reporting the radiation data. Results Across the three eras, there were significant decreases in cumulative air KERMA (mGy) among all four cath categories. From Era 2 to Era 3, the greatest decreases in radiation were noted, particularly in dose area product (cGy·cm2) in the transplant annual evaluation and pre-Glenn cases. In Era 1, 2 cases (1.2%) had a frame rate reduction, while in Era 2, 22 cases (12.0%), and in Era 3, 83 cases (21.6%) had frame rate changes (P < 0.01). Conclusions Increased physician awareness of radiation exposure is associated with a significant reduction in radiation dose across a variety of cath procedures. This is seen not only by the overall reduction in radiation across case types, but also as the frame rate was more frequently changed during individual cases, indicating an important change in physician behavior and practice. AD - G.T. Nicholson, Division of Pediatric Cardiology, Department of Pediatrics, Children's Healthcare of Atlanta, Emory University School of Medicine, 1405 Clifton Road, NE, Atlanta, GA, United States AU - Nicholson, G. T. AU - Gao, K. AU - In Kim, S. AU - Kim, D. W. AU - Vincent, R. N. AU - Balfour, V. AU - Petit, C. J. DB - Embase Medline DO - 10.1002/ccd.26098 IS - 5 KW - angioplasty article child child care child health cohort analysis Fontan procedure Glenn shunt heart catheterization heart transplantation human major clinical study medical practice physician attitude protocol compliance radiation dose radiation dose reduction radiation exposure radiation hazard retrospective study risk reduction voluntary reporting LA - English M3 - Article N1 - L605147481 2015-07-14 2015-11-06 PY - 2015 SN - 1522-726X 1522-1946 SP - 834-840 ST - Direct physician reporting is associated with reductions in radiation exposure in pediatric cardiac catheterizations T2 - Catheterization and Cardiovascular Interventions TI - Direct physician reporting is associated with reductions in radiation exposure in pediatric cardiac catheterizations UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L605147481 http://dx.doi.org/10.1002/ccd.26098 VL - 86 ID - 845 ER - TY - JOUR AB - Hypoplastic left heart syndrome (HLHS) is a devastating congenital cardiac defect. Currently, there are three management options for children with HLHS: the Norwood procedure, cardiac transplantation, and comfort care without surgery. In a recent publication, my colleagues and I discussed our findings that many physicians do not disclose all three management options with parents of affected neonates [Kon AA, Ackerson L, Lo B: How pediatricians counsel parents when no 'best-choice' treatment exists: lessons to be learned from the hypoplastic left heart syndrome. Arch Pediatr Adolesc Med 158:436-441, 2004]. When I have presented this research at conferences, many audience members have debated the pros and cons of offering nonsurgical care to parents. There is clearly a group of practitioners who feel very strongly that comfort care without surgery should never be presented to parents. I, however, disagree. I believe that all parents must be presented with all three management options. In the following essay, I will first provide background on HLHS, describe the two surgical options and discuss the current published data on outcomes, outline the four arguments generally put forth by those who feel that nonsurgical care should not be offered to parents, and finally, rebut each of these arguments and explain why all parents of children with HLHS must be told about comfort care without surgery as a reasonable choice. Copyright © 2005 by Elsevier Science (USA). AD - Pediatric Critical Care Medicine and the Program in Bioethics, University of California, Davis, 2516 Stockton Blvd, Sacramento, CA 95817 AN - 106525908. Language: English. Entry Date: 20051014. Revision Date: 20150820. Publication Type: Journal Article. Journal Subset: Core Nursing AU - Kon, A. A. DB - ccm DP - EBSCOhost IS - 2 KW - Hypoplastic Left Heart Syndrome -- Ethical Issues Hypoplastic Left Heart Syndrome -- Therapy Palliative Care -- Ethical Issues -- In Infancy and Childhood Anoxemia -- Etiology -- In Infancy and Childhood Child, Preschool Consent -- Ethical Issues Decision Making, Family Developmental Disabilities -- Etiology Heart Surgery -- Methods -- In Infancy and Childhood Heart Transplantation -- In Infancy and Childhood Hypoplastic Left Heart Syndrome -- Complications Hypoplastic Left Heart Syndrome -- Mortality Hypoplastic Left Heart Syndrome -- Physiopathology Hypoplastic Left Heart Syndrome -- Prognosis Hypoplastic Left Heart Syndrome -- Surgery Infant Infant, Newborn Parents -- Ethical Issues Parents -- Psychosocial Factors Postoperative Complications -- In Infancy and Childhood Professional-Family Relations -- Ethical Issues Survival -- In Infancy and Childhood Treatment Refusal -- Ethical Issues N1 - Nursing; USA. NLM UID: 101126037. PY - 2005 SN - 1527-3369 SP - 60-68 ST - Discussing nonsurgical care with parents of newborns with hypoplastic left heart syndrome T2 - Newborn & Infant Nursing Reviews TI - Discussing nonsurgical care with parents of newborns with hypoplastic left heart syndrome UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106525908&site=ehost-live&scope=site VL - 5 ID - 1612 ER - TY - JOUR DB - Embase DO - 10.1016/j.jtcvs.2009.04.062 IS - 1 KW - abortion algorithm amniocentesis article autopsy congenital heart malformation conservative treatment data base heart surgery heart transplantation hospital human hypoplastic left heart syndrome neonatology newborn newborn surgery Norwood procedure patient transport priority journal quality of life Streptococcus infection surgical mortality surgical risk survival treatment outcome LA - English M3 - Article N1 - L50699118 2009-11-12 2010-01-20 PY - 2010 SN - 0022-5223 SP - 126-127 ST - Discussion T2 - Journal of Thoracic and Cardiovascular Surgery TI - Discussion UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50699118 http://dx.doi.org/10.1016/j.jtcvs.2009.04.062 VL - 139 ID - 1158 ER - TY - JOUR AB - This study aimed to evaluate the time course of perioperative blood glucose levels of children undergoing cardiac surgery for congenital heart disease in relation to endogenous stress hormones, inflammatory mediators, and exogenous factors such as caloric intake and glucocorticoid use. The study prospectively included 49 children undergoing cardiac surgery. Blood glucose levels, hormonal alterations, and inflammatory responses were investigated before and at the end of surgery, then 12 and 24 h afterward. In general, blood glucose levels were highest at the end of surgery. Hyperglycemia, defined as a glucose level higher than 8.3 mmol/l (>150 mg/dl) was present in 52% of the children at the end of surgery. Spontaneous normalization of blood glucose occurred in 94% of the children within 24 h. During surgery, glucocorticoids were administered to 65% of the children, and this was the main factor associated with hyperglycemia at the end of surgery (determined by univariate analysis of variance). Hyperglycemia disappeared spontaneously without insulin therapy after 12-24 h for the majority of the children. Postoperative morbidity was low in the study group, so the presumed positive effects of glucocorticoids seemed to outweigh the adverse effects of iatrogenic hyperglycemia. © 2010 The Author(s). AD - J. J. Verhoeven, Intensive Care, Erasmus MC-Sophia Children's Hospital, P.O. Box 2060, Rotterdam 3000 CB, Netherlands AU - Verhoeven, J. J. AU - Hokken-Koelega, A. C. S. AU - Den Brinker, M. AU - Hop, W. C. J. AU - Van Thiel, R. J. AU - Bogers, A. J. J. C. AU - Helbing, W. A. AU - Joosten, K. F. M. DB - Embase Medline DO - 10.1007/s00246-010-9829-z IS - 2 KW - dexamethasone glucocorticoid glucose hydrocortisone insulin interleukin 10 interleukin 6 lactic acid methylprednisolone stress hormone adolescent adult article blood glucose monitoring caloric intake child child health clinical article clinical evaluation congenital heart disease controlled study coronary artery anomaly disease course drug effect drug use Fallot tetralogy female glucose blood level glucose homeostasis heart atrium septum defect heart left ventricle outflow tract obstruction heart right ventricle outflow tract obstruction heart single ventricle heart ventricle septum defect hormone synthesis human hyperglycemia iatrogenic disease infant insulin blood level male mitral valve regurgitation open heart surgery pediatric surgery postoperative complication preschool child school child LA - English M3 - Article N1 - L51154525 2010-11-20 2011-03-24 PY - 2011 SN - 0172-0643 SP - 131-138 ST - Disturbance of glucose homeostasis after pediatric cardiac surgery T2 - Pediatric Cardiology TI - Disturbance of glucose homeostasis after pediatric cardiac surgery UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51154525 http://dx.doi.org/10.1007/s00246-010-9829-z VL - 32 ID - 1116 ER - TY - JOUR AB - Introduction. Genetic abnormalities occur in approximately 20% of children with congenital heart disease. The purpose of this study was to evaluate the effect of genetic abnormalities on short-term outcomes following neonatal cardiac surgery. Methods. Retrospective review of all neonates (n = 609) undergoing cardiac surgery from January 2003 to December 2006. Genetic abnormalities were identified in 93 neonates (15%). Genetic abnormalities identified were 22q11.2 deletion (23), chromosomal abnormalities including various monosomies, trisomies, deletions, duplications, and inversions (17), dysmorphic undefined syndrome without recognized chromosomal abnormality (27), Down syndrome (9), laterality sequences (9), recognixed syndromes and genetic etiology including Mendelian (i.e. Alagille, CHARGE) (8). Results. Neonates with genetic abnormalities had lower birth weights and were older at time of surgery. There was no difference in operative variables, duration of mechanical ventilation or ICU length of stay between the two groups. There was an increase in total hospital length of stay and postoperative complications in the neonates with genetic abnormalities. Importantly, in hospital mortality was not different. Conclusion. Neonates with genetic abnormalities have a higher risk of postoperative complications and a longer hospital length of stay. However, there is no increase in hospital mortality. This information may aid in patient management decisions and parental counseling. Longer-term studies are needed for understanding the total impact of genetic abnormalities on neonates with congenital heart disease. © Journal compilation © 2009 Wiley Periodicals, Inc. AD - J.M. Simsic, Sibley Heart Center Cardiology, Atlanta, GA 30341, United States AU - Simsic, J. M. AU - Coleman, K. AU - Maher, K. O. AU - Cuadrado, A. AU - Kirshbom, P. M. DB - Embase Medline DO - 10.1111/j.1747-0803.2009.00281.x IS - 3 KW - aortic coarctation article artificial ventilation body dysmorphic disorder cardiopulmonary bypass chromosome 22q chromosome deletion chromosome duplication chromosome inversion congenital heart disease DiGeorge syndrome Down syndrome enteric feeding Fallot tetralogy feeding disorder genetic disorder heart arrhythmia heart surgery heart ventricle septum defect human hypoplastic left heart syndrome intensive care unit length of stay low birth weight monosomy newborn newborn morbidity newborn mortality parent counseling pericardial effusion postoperative complication priority journal pulmonary valve atresia reoperation risk factor trisomy LA - English M3 - Article N1 - L354689301 2009-06-23 PY - 2009 SN - 1747-079X 1747-0803 SP - 160-165 ST - Do neonates with genetic abnormalities have an increased morbidity and mortality following cardiac surgery? T2 - Congenital Heart Disease TI - Do neonates with genetic abnormalities have an increased morbidity and mortality following cardiac surgery? UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L354689301 http://dx.doi.org/10.1111/j.1747-0803.2009.00281.x VL - 4 ID - 1179 ER - TY - JOUR AB - Understanding (patho)physiological phenomena and mechanisms of failure in patients with Fontan circulation-a surgically established circulation for patients born with a functionally single ventricle-remains challenging due to the complex hemodynamics and high inter-patient variations in anatomy and function. In this work, we present a biomechanical model of the heart and circulation to augment the diagnostic evaluation of Fontan patients with early-stage heart failure. The proposed framework employs a reduced-order model of heart coupled with a simplified circulation including venous return, creating a closed-loop system. We deploy this framework to augment the information from data obtained during combined cardiac catheterization and magnetic resonance exams (XMR), performed at rest and during dobutamine stress in 9 children with Fontan circulation and 2 biventricular controls. We demonstrate that our modeling framework enables patient-specific investigation of myocardial stiffness, contractility at rest, contractile reserve during stress and changes in vascular resistance. Hereby, the model allows to identify key factors underlying the pathophysiological response to stress in these patients. In addition, the rapid personalization of the model to patient data and fast simulation of cardiac cycles make our framework directly applicable in a clinical workflow. We conclude that the proposed modeling framework is a valuable addition to the current clinical diagnostic XMR exam that helps to explain patient-specific stress hemodynamics and can identify potential mechanisms of failure in patients with Fontan circulation. AD - School of Biomedical Engineering & Imaging Sciences, St Thomas' Hospital, King's College London, London, England, United Kingdom. Department of Pediatrics, UT Southwestern Medical Center, Dallas, TX, United States of America. Inria Centre de Recherche Saclay-Ile-de-France, Palaiseau, France. LMS, Ecole Polytechnique, CNRS, Institut Polytechnique de Paris, Paris, France. AN - 32084180 AU - Ruijsink, B. AU - Zugaj, K. AU - Wong, J. AU - Pushparajah, K. AU - Hussain, T. AU - Moireau, P. AU - Razavi, R. AU - Chapelle, D. AU - Chabiniok, R. C2 - PMC7034893 DB - PubMed DO - 10.1371/journal.pone.0229015 DP - NLM ET - 2020/02/23 IS - 2 KW - Biomechanical Phenomena Dobutamine/*pharmacology Fontan Procedure/*methods Heart Hemodynamics/drug effects Humans Models, Cardiovascular Vascular Resistance/drug effects LA - eng N1 - 1932-6203 Ruijsink, Bram Orcid: 0000-0001-8313-5709 Zugaj, Konrad Wong, James Pushparajah, Kuberan Hussain, Tarique Moireau, Philippe Razavi, Reza Chapelle, Dominique Chabiniok, Radomír Orcid: 0000-0002-7527-2751 DH_/Department of Health/United Kingdom WT 203148/Z/16/Z/WT_/Wellcome Trust/United Kingdom Journal Article Research Support, Non-U.S. Gov't PLoS One. 2020 Feb 21;15(2):e0229015. doi: 10.1371/journal.pone.0229015. eCollection 2020. PY - 2020 SN - 1932-6203 SP - e0229015 ST - Dobutamine stress testing in patients with Fontan circulation augmented by biomechanical modeling T2 - PLoS One TI - Dobutamine stress testing in patients with Fontan circulation augmented by biomechanical modeling VL - 15 ID - 373 ER - TY - JOUR AB - Background Many patients with congenital heart disease do not meet current public health guidelines to participate in moderate-to-vigorous physical activity for ≥60 minutes per day. They are less fit than their healthy peers. We hypothesized that exercise training would increase cardiopulmonary fitness and daily physical activity in these patients. We therefore assessed effects of an exercise training program on cardiopulmonary fitness and daily physical activity in patients with corrected tetralogy of Fallot (ToF) or Fontan circulation. Methods In a multicenter prospective controlled trial, patients with ToF or Fontan circulation (age 10-25 years) were randomized, 56 patients to the exercise group and 37 to the control group. The exercise group participated in a 12-week standardized aerobic exercise training program. The control group continued lifestyle as usual. Cardiopulmonary exercise testing and activity measurements were performed before and after 12 weeks. Results Peak oxygen uptake increased in the exercise group by 5.0% (1.7 ± 4.2 mL/kg per minute; P =.011) but not in the control group (0.9 ± 5.2 mL/kg per minute; P = not significant). Workload increased significantly in the exercise group compared with the control group (6.9 ± 11.8 vs 0.8 ± 13.9 W; P =.047). Subgroup analysis showed a significant increase in pre-to-post peak oxygen uptake in the exercise group of ToF patients but not in the exercise group of Fontan patients. Percentage of measured time spent in moderate-to-vigorous activity at baseline was 13.6% ± 8.6%, which did not significantly change after training. Conclusions Aerobic exercise training improved cardiopulmonary fitness in patients with ToF but not in patients with Fontan circulation. Exercise training did not change daily physical activity. AD - W. Helbing, Department of Pediatrics, Division of Cardiology, Erasmus Medical Center-Sophia Children's Hospital, Sp-2429, PO Box 2060, Rotterdam, Netherlands AU - Duppen, N. AU - Etnel, J. R. AU - Spaans, L. AU - Takken, T. AU - Van Den Berg-Emons, R. J. AU - Boersma, E. AU - Schokking, M. AU - Dulfer, K. AU - Utens, E. M. AU - Helbing, W. AU - Hopman, M. T. DB - Embase Medline DO - 10.1016/j.ahj.2015.06.018 IS - 3 KW - NTR2731 adolescent adult aerobic exercise article cardiopulmonary exercise test cardiorespiratory fitness cardiovascular magnetic resonance child congenital heart disease controlled study Fallot tetralogy female Fontan circulation heart rehabilitation heart stroke volume heart work human lifestyle lung gas exchange major clinical study male multicenter study outcome assessment oxygen consumption patient compliance physical activity physical performance priority journal program efficacy program evaluation prospective study randomized controlled trial school child single blind procedure treatment duration whole body MRI young adult LA - English M3 - Article N1 - L605506314 2016-03-02 PY - 2015 SN - 1097-6744 0002-8703 SP - 606-614 ST - Does exercise training improve cardiopulmonary fitness and daily physical activity in children and young adults with corrected tetralogy of Fallot or Fontan circulation? A randomized controlled trial T2 - American Heart Journal TI - Does exercise training improve cardiopulmonary fitness and daily physical activity in children and young adults with corrected tetralogy of Fallot or Fontan circulation? A randomized controlled trial UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L605506314 http://dx.doi.org/10.1016/j.ahj.2015.06.018 VL - 170 ID - 852 ER - TY - JOUR AB - PURPOSE: It is important to identify those children with a Fontan circulation who are at risk for impaired health-related quality of life. We aimed to determine the predictive value of functional health status - medical history and present medical status - on both physical and psychosocial domains of health-related quality of life, as reported by patients themselves and their parents. METHODS: We carried out a prospective cross-sectional multi-centre study in Fontan patients aged between 8 and 15, who had undergone staged completion of total cavopulmonary connection according to a current technique before the age of 7 years. Functional health status was assessed as medical history - that is, age at Fontan, type of Fontan, ventricular dominance, and number of cardiac surgical procedures - and present medical status - assessed with magnetic resonance imaging, exercise testing, and rhythm assessment. Health-related quality of life was assessed with The TNO/AZL Child Questionnaire Child Form and Parent Form. RESULTS: In multivariate prediction models, several medical history variables, such as more operations post-Fontan completion, lower age at Fontan completion, and dominant right ventricle, and present medical status variables, such as smaller end-diastolic volume, a higher score for ventilatory efficiency, and the presence of sinus node dysfunction, predicted worse outcomes on several parent-reported and self-reported physical as well as psychosocial health-related quality of life domains. CONCLUSIONS: Medical history and worse present medical status not only predicted worse physical parent-reported and self-reported health-related quality of life but also worse psychosocial health-related quality of life and subjective cognitive functioning. These findings will help in identifying patients who are at risk for developing impaired health-related quality of life. AD - 1Department of Child and Adolescent Psychiatry/Psychology,Erasmus Medical Centre-Sophia Children's Hospital,Rotterdam,the Netherlands. 2Department of Paediatrics,Division of Cardiology,Erasmus Medical Centre - Sophia Children's Hospital,Rotterdam,the Netherlands. 3Department of Paediatrics,Division of Cardiology,Academic Medical Centre,Amsterdam,the Netherlands. 4Department of Paediatrics,Division of Cardiology,Radboud University Nijmegen Medical Centre,Nijmegen,the Netherlands. 6Department of Paediatrics,Division of Cardiology,University Medical Centre Utrecht - Wilhelmina Children's Hospital,Utrecht,the Netherlands. 7Department of Paediatrics,Division of Cardiology,Leiden University Medical Centre,Leiden,the Netherlands. 8Child Development and Exercise Centre,University Medical Centre Utrecht - Wilhelmina Children's Hospital,Utrecht,the Netherlands. AN - 25906441 AU - Dulfer, K. AU - Bossers, S. S. AU - Utens, E. M. AU - Duppen, N. AU - Kuipers, I. M. AU - Kapusta, L. AU - van Iperen, G. AU - Schokking, M. AU - ten Harkel, A. D. AU - Takken, T. AU - Helbing, W. A. DA - Mar DB - PubMed DO - 10.1017/s1047951115000426 DP - NLM ET - 2015/04/24 IS - 3 KW - Adolescent Child Cross-Sectional Studies Exercise Test Female *Fontan Procedure *Health Status Heart Defects, Congenital/*surgery Heart Ventricles/*physiopathology Humans Magnetic Resonance Imaging Male Multivariate Analysis Netherlands Parents Prospective Studies *Quality of Life Surveys and Questionnaires Treatment Outcome Fontan circulation congenital heart disease quality of life LA - eng N1 - 1467-1107 Dulfer, Karolijn Bossers, Sjoerd S M Utens, Elisabeth M W J Duppen, Nienke Kuipers, Irene M Kapusta, Livia van Iperen, Gabrielle Schokking, Michiel ten Harkel, Arend D J Takken, Tim Helbing, Willem A Journal Article Multicenter Study Research Support, Non-U.S. Gov't England Cardiol Young. 2016 Mar;26(3):459-68. doi: 10.1017/S1047951115000426. Epub 2015 Apr 23. PY - 2016 SN - 1047-9511 SP - 459-68 ST - Does functional health status predict health-related quality of life in children after Fontan operation? T2 - Cardiol Young TI - Does functional health status predict health-related quality of life in children after Fontan operation? VL - 26 ID - 102 ER - TY - JOUR AB - Purpose It is important to identify those children with a Fontan circulation who are at risk for impaired health-related quality of life. We aimed to determine the predictive value of functional health status - medical history and present medical status - on both physical and psychosocial domains of health-related quality of life, as reported by patients themselves and their parents. Methods We carried out a prospective cross-sectional multi-centre study in Fontan patients aged between 8 and 15, who had undergone staged completion of total cavopulmonary connection according to a current technique before the age of 7 years. Functional health status was assessed as medical history - that is, age at Fontan, type of Fontan, ventricular dominance, and number of cardiac surgical procedures - and present medical status - assessed with magnetic resonance imaging, exercise testing, and rhythm assessment. Health-related quality of life was assessed with The TNO/AZL Child Questionnaire Child Form and Parent Form. Results In multivariate prediction models, several medical history variables, such as more operations post-Fontan completion, lower age at Fontan completion, and dominant right ventricle, and present medical status variables, such as smaller end-diastolic volume, a higher score for ventilatory efficiency, and the presence of sinus node dysfunction, predicted worse outcomes on several parent-reported and self-reported physical as well as psychosocial health-related quality of life domains. Conclusions Medical history and worse present medical status not only predicted worse physical parent-reported and self-reported health-related quality of life but also worse psychosocial health-related quality of life and subjective cognitive functioning. These findings will help in identifying patients who are at risk for developing impaired health-related quality of life. AD - W.A. Helbing, Department of Pediatric Cardiology, Division of Cardiology, Erasmus Medical Centre, Sophia Children's Hospital, PO Box 2060, Rotterdam, Netherlands AU - Dulfer, K. AU - Bossers, S. S. M. AU - Utens, E. M. W. J. AU - Duppen, N. AU - Kuipers, I. M. AU - Kapusta, L. AU - Van Iperen, G. AU - Schokking, M. AU - Ten Harkel, A. D. J. AU - Takken, T. AU - Helbing, W. A. DB - Embase Medline DO - 10.1017/S1047951115000426 IS - 3 KW - adolescent article child clinical protocol cognition controlled study emotionality exercise test female Fontan procedure functional status health status heart rhythm human major clinical study male medical literature motor performance nuclear magnetic resonance imaging outcome assessment predictive value prospective study quality of life social status LA - English M3 - Article N1 - L603984528 2015-04-30 2016-06-27 PY - 2016 SN - 1467-1107 1047-9511 SP - 459-468 ST - Does functional health status predict health-related quality of life in children after Fontan operation? T2 - Cardiology in the Young TI - Does functional health status predict health-related quality of life in children after Fontan operation? UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L603984528 http://dx.doi.org/10.1017/S1047951115000426 VL - 26 ID - 815 ER - TY - JOUR AB - Plasma samples from pediatric cardiac patients undergoing cardiopulmonary bypass (CPB) procedures were used to identify and characterize patterns of changes in potential biomarkers related to tissue damage and inflammation. These included proteins associated with systemic inflammatory response syndrome. Potential biomarkers were identified using a dual-platform proteomics approach requiring ∼150 μL of plasma, which included two-dimensional difference gel electrophoresis (2D-DIGE) and a multiplexed immunoassay. Methods used in the dual approach measured levels of 129 proteins in plasma from pediatric CPB patients. Of these, 70 proteins changed significantly (p < 0.05) between time points, and 36 of these retained significance after the highly stringent Bonferroni correction [p < 0.001 for 2D-DIGE and p < 0.00056 for multianalyte profile (MAP) assays]. Many of the changing proteins were associated with tissue damage, inflammation, and oxidative stress. This study uses a novel approach that combines two discovery proteomics techniques to identify a pattern of potential biomarkers changing after CPB. This approach required only 150 μL of plasma per time point and provided quantitative information on 129 proteins. The changes in levels of expression of these proteins may provide insight into the understanding, treatment, and prevention of systemic inflammation, thereby helping to improve the outcomes of pediatric CPB patients. © 2010 International Pediatric Research Foundation, Inc. AD - A. Ündar, Departments of Pediatrics, Surgery and Bioengineering, Penn State Hershey College of Medicine, 500 University Drive, Hershey, PA 17033, United States AU - Umstead, T. M. AU - Lu, C. J. K. AU - Freeman, W. M. AU - Myers, J. L. AU - Clark, J. B. AU - Thomas, N. J. AU - Chinchilli, V. M. AU - Vrana, K. E. AU - Ündar, A. AU - Phelps, D. S. DB - Embase Medline DO - 10.1203/PDR.0b013e3181dceef5 IS - 6 KW - albumin alpha 1 antichymotrypsin alpha 1 antitrypsin alpha 2 macroglobulin alpha globulin angiotensinogen apolipoprotein A1 apolipoprotein A2 aryldialkylphosphatase biological marker blood clotting factor 13b complement component C3 complement component C6 complement factor I fetuin A fibrinogen haptoglobin hemopexin immunoglobulin A immunoglobulin G immunoglobulin M kininogen orosomucoid transthyretin serum amyloid P sex hormone binding globulin tetranectin thrombin transferrin article blood sampling cardiopulmonary bypass child clinical article controlled study endocardial cushion defect Fallot tetralogy female Fontan procedure heart atrium septum defect heart ventricle septum defect human immunoassay infant inflammation male matrix assisted laser desorption ionization time of flight mass spectrometry oxidative stress preschool child priority journal protein analysis protein expression proteomics quantitative analysis systemic inflammatory response syndrome tissue injury two dimensional gel electrophoresis LA - English M3 - Article N1 - L50875758 2010-04-20 2010-06-07 PY - 2010 SN - 0031-3998 1530-0447 SP - 641-649 ST - Dual-platform proteomics study of plasma biomarkers in pediatric patients undergoing cardiopulmonary bypass T2 - Pediatric Research TI - Dual-platform proteomics study of plasma biomarkers in pediatric patients undergoing cardiopulmonary bypass UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50875758 http://dx.doi.org/10.1203/PDR.0b013e3181dceef5 VL - 67 ID - 1143 ER - TY - JOUR AB - The implementation of simultaneous mechanical cavopulmonary assistance having blood pumps located in both of the vena cavae is investigated as an approach to treating patients with an ailing Fontan physiology. Identical intravascular blood pumps are employed to model the hemodynamic support of a patient-specific Fontan. Pressure flow characteristics, energy gain calculations, and blood damage analyses are assessed for each model. The performance of the dual-support scenario is compared to conditions of mechanical support in the inferior vena cava only and to a nonsupported cavopulmonary circuit. The blood pump in the superior vena cava generates pressures ranging from 1 to 22mmHg for flow rates of 1-4L/min at operating speeds of 1250-2500rpm. The blood pump in the inferior vena cava produces pressures at levels approximately 20% lower. The blood pumps positively augment the hydraulic energy in the total cavopulmonary connection circuit as a function of flow rate and rotational speed. Scalar stress levels and fluid residence times are at acceptable levels. Damage indices for the dual-support case, however, are elevated slightly above 3.5%. These results suggest that concurrent, mechanical assistance of the inferior vena cava and superior vena cava in Fontan patients has the potential to be beneficial, but additional studies are needed to further explore this approach. © 2013, Authors. Artificial Organs © 2013, International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc. AD - BioCirc Research Laboratory, Department of Mechanical and Nuclear Engineering, School of Engineering, Virginia Commonwealth University, Richmond, VA, United States Division of Pediatric Cardiology, Children's Hospital of Richmond and School of Medicine, Virginia Commonwealth University, Richmond, VA, United States AU - Throckmorton, A. L. AU - Lopez-Isaza, S. AU - Moskowitz, W. DB - Scopus DO - 10.1111/aor.12039 IS - 6 KW - Artificial right ventricle Blood pump Cavopulmonary assist device Computational fluid dynamics Fontan physiology Mechanical cavopulmonary assist Pediatric circulatory support Single ventricle physiology Total cavopulmonary connection M3 - Article N1 - Cited By :11 Export Date: 15 June 2020 PY - 2013 SP - 513-522 ST - Dual-pump support in the inferior and superior vena cavae of a patient-specific fontan physiology T2 - Artificial Organs TI - Dual-pump support in the inferior and superior vena cavae of a patient-specific fontan physiology UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84879107247&doi=10.1111%2faor.12039&partnerID=40&md5=b1940476502696860208074063f53c0f VL - 37 ID - 2046 ER - TY - JOUR AB - Airway abnormalities are frequently associated with congenital heart disease in infants and children. Respiratory distress in these children frequently requires prolonged respiratory support. Wide-detector dynamic pulmonary computed tomography angiography (DP-CTA) is a non-invasive technique that completely evaluates vascular and airway abnormalities during a single breathe. Our purpose was to evaluate the efficacy of DP-CTA to provide unique actionable information for patient care in newborns and infants with congenital heart disease and persistent respiratory distress. 23 infants with congenital heart disease and persistent respiratory distress underwent DP-CTA. All were intubated at the time of the examinations. The most common cardiac anomalies were tetralogy of Fallot (6) and hypoplastic left heart syndrome variants (5). The most common cardiac surgeries prior to DP-CTA were Norwood (4) and hybrid (3) procedures. The protocol for DP-CTA for intubated infants is four gantry rotations in 1.4 s after intravenous contrast injection. 3D and multiplanar reconstruction with cine loops were created for combined cardiopulmonary imaging. Tracheobronchomalacia was present in 17 children. Lobar bronchomalacia was identified in six children. Branch pulmonary artery stenosis was the most common vascular finding (10 children). Medical management was changed or a surgical procedure performed based on the information resulting from DP-CTA in 16 of the 23 patients (70 %). DP-CTA is non-invasive, fast and provides unique information for the management of infants with congenital heart disease and persistent respiratory distress. DP-CTA is uniquely suited for comprehensive and simultaneous evaluation of airway and vascular abnormalities in infants. AD - Arkansas Children's Hospital, 1 Children's Way, Slot #105, Little Rock, AR, 72202, USA, greenbergsbruce@uams.edu. AN - 24322888 AU - Greenberg, S. B. AU - Dyamenahalli, U. DA - Feb DB - PubMed DO - 10.1007/s10554-013-0344-1 DP - NLM ET - 2013/12/11 IS - 2 KW - *Abnormalities, Multiple Cardiac Surgical Procedures *Cineangiography Constriction, Pathologic Contrast Media/administration & dosage Heart Defects, Congenital/complications/diagnostic imaging/surgery Humans Infant Infant, Newborn Injections, Intravenous Intubation, Intratracheal Predictive Value of Tests Pulmonary Artery/abnormalities/*diagnostic imaging/surgery Respiration Respiration, Artificial Respiratory Distress Syndrome, Newborn/diagnostic imaging/etiology/therapy Retrospective Studies Tomography, X-Ray Computed/*methods Tracheobronchomalacia/complications/*diagnostic imaging/therapy Vascular Malformations/complications/*diagnostic imaging/therapy LA - eng N1 - 1875-8312 Greenberg, S Bruce Dyamenahalli, Umesh Journal Article United States Int J Cardiovasc Imaging. 2014 Feb;30(2):407-14. doi: 10.1007/s10554-013-0344-1. Epub 2013 Dec 10. PY - 2014 SN - 1569-5794 SP - 407-14 ST - Dynamic pulmonary computed tomography angiography: a new standard for evaluation of combined airway and vascular abnormalities in infants T2 - Int J Cardiovasc Imaging TI - Dynamic pulmonary computed tomography angiography: a new standard for evaluation of combined airway and vascular abnormalities in infants VL - 30 ID - 522 ER - TY - JOUR AB - The article presents abstracts on pediatric topics including the use of dexamethasone therapy for septic arthritis in children, the implementation of a neonatal abstinence syndrome weaning protocol and the trends in emergency department visits for unsupervised pediatric medication exposures. AN - 110231525. Language: English. Entry Date: 20151013. Revision Date: 20160610. Publication Type: Abstract DB - ccm DP - EBSCOhost IS - 4 KW - Arthritis, Infectious Alcoholism Drug Rehabilitation Programs Home Visits Neonatal Abstinence Syndrome Attention Deficit Hyperactivity Disorder Adverse Drug Event Language Disorders Autistic Disorder Eating Disorders Hypoplastic Left Heart Syndrome Catheters, Urinary Cytomegalovirus Infections Spina Bifida Genotype Sleep Heart Surgery Cerebral Palsy Intensive Care Units, Pediatric Family Centered Care Truth Disclosure Genomics Parents Consent (Research) Infant, Very Low Birth Weight Vascular Diseases Bronchiolitis Sudden Infant Death Chest Pain Pneumonia Colorectal Neoplasms Tracheoesophageal Fistula N1 - abstract. Journal Subset: Biomedical; Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. NLM UID: 0376422. PY - 2015 SN - 0031-4005 SP - 765-780 ST - e-Articles T2 - Pediatrics TI - e-Articles UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=110231525&site=ehost-live&scope=site VL - 136 ID - 1509 ER - TY - JOUR AB - BACKGROUND: Troponin-I (Tn-I) is a well-recognized early postoperative marker for myocardial damage in adults and children. The present prospective study was undertaken to investigate whether a postoperative Tn-I value higher than 35 microg/l is able to predict long-term outcome as it does in early postoperative course, after surgery for congenital heart defects (CHD). MATERIALS AND METHODS: Five hundred and twenty patients (median age 11 months; male 54.7%: 284 patients) undergoing congenital heart defect repair on cardiopulmonary by-pass were prospectively updated in our database including postoperative Tn-I values. Seventy of them (13.4%) (mean age 2.6+/-5.8 months) (70/520) experienced low output syndrome in the early postoperative period. According to the complexity of their malformations, we have arbitrarily divided these patients into two groups: group A included atrial and ventricular septal defects (13 patients), while group B included hypoplastic left heart syndrome, atrio-ventricular canal, transposition of great vessels, tetralogy of Fallot, double outlet right ventricle, truncus arteriosus, total anomalous venous return, and other combined diseases (57 patients). These patients are the object of our study. We reviewed clinical, laboratory, and echocardiographic data performed in the immediate postoperative course (within 24 h) and in the follow-up. RESULTS: In this study, 13 patients died (13/70 patients; 18.5%), 12 in group B and 1 in group A. In deceased patients, mean Tn-I value was 130+/-175 microg/l (CK-MB 570+/-280 microg/l). Conversely, survivors showed a lower mean Tn-I value (25.5+/-28.9 microg/l; CK-MB 76+/-86 microg/l). Overall, Tn-I peak value was higher than 35 microg/l in 19 patients (19/70; 27.2%); among these, 9 died (median Tn-I was 163+/-186 microg/l), whereas in survivors it was 73.4+/-37 microg/l (p=0.37). The remaining four patients who died had a median Tn-I value of 21 microg/l. When Tn-I exceeded 35 microg/l (>100 microg/l in two cases), at echocardiogram a severely depressed cardiac function was evident. Nevertheless, at long-term follow-up (12+/-6 months), the echocardiogram showed an enhanced cardiac performance with an ejection fraction of 70+/-8.5% in all; none of these patients presented with worsened ventricular function. CONCLUSION: Cardiac Tn-I is a specific and sensitive marker of myocardial injury after cardiac surgery and it may predict early in-hospital outcomes. However, by long-term echocardiographic analysis, cardiac Tn-I value looses its prognostic significance and therefore it is not a predictor of long-term ventricular dysfunction. AD - Cardiovascular Institute, School of Medicine of Brescia, Brescia, Italy. bottio@med.unibs.it AN - 16835014 AU - Bottio, T. AU - Vida, V. AU - Padalino, M. AU - Gerosa, G. AU - Stellin, G. DA - Aug DB - PubMed DO - 10.1016/j.ejcts.2006.05.001 DP - NLM ET - 2006/07/13 IS - 2 KW - Adolescent Adult Biomarkers/blood Cardiopulmonary Bypass Child Child, Preschool Epidemiologic Methods Female Heart Defects, Congenital/blood/diagnostic imaging/*surgery Humans Infant Infant, Newborn Male Middle Aged Postoperative Period Prognosis Stroke Volume Treatment Outcome Troponin I/*blood Ultrasonography LA - eng N1 - Bottio, Tomaso Vida, Vladimiro Padalino, Massimo Gerosa, Gino Stellin, Giovanni Evaluation Study Journal Article Research Support, Non-U.S. Gov't Germany Eur J Cardiothorac Surg. 2006 Aug;30(2):250-5. doi: 10.1016/j.ejcts.2006.05.001. Epub 2006 Jul 10. PY - 2006 SN - 1010-7940 (Print) 1010-7940 SP - 250-5 ST - Early and long-term prognostic value of Troponin-I after cardiac surgery in newborns and children T2 - Eur J Cardiothorac Surg TI - Early and long-term prognostic value of Troponin-I after cardiac surgery in newborns and children VL - 30 ID - 523 ER - TY - JOUR AB - OBJECTIVE: To assess intellect and adaptive behavior in children with hypoplastic left heart syndrome (HLHS) who had undergone at least two surgical stages of the Norwood procedure. METHODS: Fourteen children with HLHS >3 years of age participated in the study. The patients underwent intelligence quotient (IQ) testing, and their parents were interviewed regarding their children's adaptive behavior. Results were compared with those of 10 family controls. Outcomes were studied for possible correlation with perioperative variables. RESULTS: Among the HLHS patients, the median scores for full scale IQ and adaptive behavior were 88 and 91, respectively (normal = 100 +/- 15). One child met criteria for mental retardation. Family controls scored generally higher than did HLHS patients, but only differences in adaptive behavior were statistically significant. A negative correlation was found between stage I circulatory arrest time and full scale IQ. CONCLUSIONS: Children with HLHS most often function in the low-normal range of intelligence and adaptive behavior. A prolonged circulatory arrest time may result in decreased intellectual function. AD - Columbia-Presbyterian Medical Center, Department of Pediatrics, New York, NY, USA. AN - 9794947 AU - Kern, J. H. AU - Hinton, V. J. AU - Nereo, N. E. AU - Hayes, C. J. AU - Gersony, W. M. DA - Nov DB - PubMed DO - 10.1542/peds.102.5.1148 DP - NLM ET - 1998/10/31 IS - 5 KW - *Adaptation, Psychological Case-Control Studies Child *Child Behavior *Child Development Child, Preschool Female Humans Hypoplastic Left Heart Syndrome/psychology/*surgery *Intelligence Male Motor Skills Risk Factors LA - eng N1 - 1098-4275 Kern, J H Hinton, V J Nereo, N E Hayes, C J Gersony, W M Journal Article United States Pediatrics. 1998 Nov;102(5):1148-52. doi: 10.1542/peds.102.5.1148. PY - 1998 SN - 0031-4005 SP - 1148-52 ST - Early developmental outcome after the Norwood procedure for hypoplastic left heart syndrome T2 - Pediatrics TI - Early developmental outcome after the Norwood procedure for hypoplastic left heart syndrome VL - 102 ID - 435 ER - TY - JOUR AB - BACKGROUND: Survivors of the Norwood procedure may experience neurodevelopmental impairment. Clinical trials to improve outcomes have focused primarily on methods of vital organ support during cardiopulmonary bypass. METHODS AND RESULTS: In the Single Ventricle Reconstruction trial of the Norwood procedure with modified Blalock-Taussig shunt versus right-ventricle-to-pulmonary-artery shunt, 14-month neurodevelopmental outcome was assessed by use of the Psychomotor Development Index (PDI) and Mental Development Index (MDI) of the Bayley Scales of Infant Development-II. We used multivariable regression to identify risk factors for adverse outcome. Among 373 transplant-free survivors, 321 (86%) returned at age 14.3 ± 1.1 (mean ± SD) months. Mean PDI (74 ± 19) and MDI (89 ± 18) scores were lower than normative means (each P<0.001). Neither PDI nor MDI score was associated with type of Norwood shunt. Independent predictors of lower PDI score (R(2)=26%) were clinical center (P=0.003), birth weight <2.5 kg (P=0.023), longer Norwood hospitalization (P<0.001), and more complications between Norwood procedure discharge and age 12 months (P<0.001). Independent risk factors for lower MDI score (R(2)=34%) included center (P<0.001), birth weight <2.5 kg (P=0.04), genetic syndrome/anomalies (P=0.04), lower maternal education (P=0.04), longer mechanical ventilation after the Norwood procedure (P<0.001), and more complications after Norwood discharge to age 12 months (P<0.001). We found no significant relationship of PDI or MDI score to perfusion type, other aspects of vital organ support (eg, hematocrit, pH strategy), or cardiac anatomy. CONCLUSIONS: Neurodevelopmental impairment in Norwood survivors is more highly associated with innate patient factors and overall morbidity in the first year than with intraoperative management strategies. Improved outcomes are likely to require interventions that occur outside the operating room. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifier: NCT00115934. AD - Children's Hospital Boston & Harvard Medical School, MA, USA. jane.newburger@cardio.chboston.org AN - 22456475 AU - Newburger, J. W. AU - Sleeper, L. A. AU - Bellinger, D. C. AU - Goldberg, C. S. AU - Tabbutt, S. AU - Lu, M. AU - Mussatto, K. A. AU - Williams, I. A. AU - Gustafson, K. E. AU - Mital, S. AU - Pike, N. AU - Sood, E. AU - Mahle, W. T. AU - Cooper, D. S. AU - Dunbar-Masterson, C. AU - Krawczeski, C. D. AU - Lewis, A. AU - Menon, S. C. AU - Pemberton, V. L. AU - Ravishankar, C. AU - Atz, T. W. AU - Ohye, R. G. AU - Gaynor, J. W. C2 - PMC3341507 C6 - NIHMS368025 DA - May 1 DB - PubMed DO - 10.1161/circulationaha.111.064113 DP - NLM ET - 2012/03/30 IS - 17 KW - Abnormalities, Multiple/epidemiology *Blalock-Taussig Procedure Brain Damage, Chronic/epidemiology/etiology/prevention & control/rehabilitation Child Child, Preschool Developmental Disabilities/*epidemiology/etiology/rehabilitation Early Intervention, Educational Female Heart Ventricles/*surgery Humans Hypoplastic Left Heart Syndrome/complications/*surgery Hypoxia, Brain/etiology/psychology Infant Infant, Newborn Male Neuropsychological Tests *Norwood Procedures Postoperative Complications/*epidemiology/etiology/rehabilitation Prospective Studies Psychomotor Disorders/*epidemiology/etiology/rehabilitation Pulmonary Artery/*surgery Risk Factors Socioeconomic Factors Vascular Grafting/*methods LA - eng N1 - 1524-4539 Newburger, Jane W Sleeper, Lynn A Bellinger, David C Goldberg, Caren S Tabbutt, Sarah Lu, Minmin Mussatto, Kathleen A Williams, Ismee A Gustafson, Kathryn E Mital, Seema Pike, Nancy Sood, Erica Mahle, William T Cooper, David S Dunbar-Masterson, Carolyn Krawczeski, Catherine Dent Lewis, Alan Menon, Shaji C Pemberton, Victoria L Ravishankar, Chitra Atz, Teresa W Ohye, Richard G Gaynor, J William Pediatric Heart Network Investigators U01 HL068285-10/HL/NHLBI NIH HHS/United States HL068288/HL/NHLBI NIH HHS/United States K23 HD061601/HD/NICHD NIH HHS/United States HL068285/HL/NHLBI NIH HHS/United States U01 HL068269/HL/NHLBI NIH HHS/United States U10 HL109816/HL/NHLBI NIH HHS/United States U01 HL068279/HL/NHLBI NIH HHS/United States U01 HL068290/HL/NHLBI NIH HHS/United States HL068279/HL/NHLBI NIH HHS/United States U01 HL068288/HL/NHLBI NIH HHS/United States U10 HL068270/HL/NHLBI NIH HHS/United States U01 HL068281/HL/NHLBI NIH HHS/United States U01 HL068270/HL/NHLBI NIH HHS/United States HL085057/HL/NHLBI NIH HHS/United States UL1 TR000055/TR/NCATS NIH HHS/United States UL1 RR025758/RR/NCRR NIH HHS/United States UL1 RR 025758/RR/NCRR NIH HHS/United States HL068281/HL/NHLBI NIH HHS/United States U01 HL068292/HL/NHLBI NIH HHS/United States HL068269/HL/NHLBI NIH HHS/United States HL068270/HL/NHLBI NIH HHS/United States U01 HL085057/HL/NHLBI NIH HHS/United States HL068290/HL/NHLBI NIH HHS/United States U01 HL068285/HL/NHLBI NIH HHS/United States HL068292/HL/NHLBI NIH HHS/United States Journal Article Multicenter Study Randomized Controlled Trial Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't Circulation. 2012 May 1;125(17):2081-91. doi: 10.1161/CIRCULATIONAHA.111.064113. Epub 2012 Mar 28. PY - 2012 SN - 0009-7322 (Print) 0009-7322 SP - 2081-91 ST - Early developmental outcome in children with hypoplastic left heart syndrome and related anomalies: the single ventricle reconstruction trial T2 - Circulation TI - Early developmental outcome in children with hypoplastic left heart syndrome and related anomalies: the single ventricle reconstruction trial VL - 125 ID - 278 ER - TY - JOUR AB - Background: The concept of early extubation following congenital heart surgery is not new. Changes in health care have generated increasing interest in this technique. Some of the problems following surgery are related to the endotracheal tube and mechanical ventilation, and the interventions necessary to maintain them. Methods: To evaluate the impact of early extubation (within 6 h postoperatively) on children undergoing congenital heart surgery, retrospective data were obtained from records of 1000 consecutive patients. Results: Early extubation occurred in 80.2% of patients (73% in the operating room). Early extubation was not limited to simple anomalies; it was also possible for complex anomalies such as Fallot's tetralogy (including those patients with pulmonary atresia, absent pulmonary valve and complete atrioventricular septal defects; n = 106), where 91% of patients had early extubation, and Fontan operations (n = 17), where 88% did. There were no deaths related to early extubation. Preoperative intubation was a risk factor for postoperative ventilation. As expected, the patients requiring ventilation after surgery were younger, smaller and more critically ill than those that met the criteria for early extubation. Conclusions: A change in attitude combined with appropriate anaesthetic and surgical techniques permitted safe, early extubation in a large number of patients. Patient populations vary between institutions. Early extubation is not always possible, but for those patients in whom it is feasible, the benefits include simplified postoperative care and increased patient and family satisfaction. When combined with clinical practice guidelines, it can result in a significant reduction in the cost of patient care after cardiac surgery. AD - R.A. Neirotti, Pediatric Cardiac Surgery, DeVos Children's Hospital, 100 Michigan NE, Grand Rapids, MI 49503, United States AU - Neirotti, R. A. AU - Jones, D. AU - Hackbarth, R. AU - Fosse, G. P. DB - Embase DO - 10.1046/j.1444-2892.2002.00144.x IS - 3 KW - anesthesiological procedure article breathing congenital heart disease critical illness data analysis death extubation Fallot tetralogy family Fontan procedure health care cost heart septum defect heart surgery human infant intubation pulmonary valve atresia major clinical study medical record patient care patient satisfaction physician attitude postoperative care postoperative period practice guideline preoperative period preschool child priority journal pulmonary valve disease retrospective study risk factor surgical technique LA - English M3 - Article N1 - L36040765 2003-01-16 PY - 2002 SN - 1443-9506 SP - 157-161 ST - Early extubation in congenital heart surgery T2 - Heart Lung and Circulation TI - Early extubation in congenital heart surgery UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L36040765 http://dx.doi.org/10.1046/j.1444-2892.2002.00144.x VL - 11 ID - 1309 ER - TY - JOUR AB - Background The reported incidence of necrotising enterocolitis in neonates with complex CHD with ductus-dependent systemic circulation ranges from 6.8 to 13% despite surgical treatment; the overall mortality is between 25 and 97%. The incidence of gastrointestinal complications after hybrid palliation for neonates with ductus-dependent systemic circulation still has to be defined, but seems comparable with that following the Norwood procedure. Methods We reviewed the incidence of gastrointestinal complications in a series of 42 consecutive neonates with ductus-dependent systemic circulation, who received early hybrid palliation associated with a standardised feeding protocol. Results The median age and birth weight at the time of surgery were 3 days (with a range from 1 to 10 days) and 3.07 kg (with a range from 1.5 to 4.5 kg), respectively. The median ICU length of stay was 7 days (1-70 days), and the median hospital length of stay was 16 days (6-70 days). The median duration of mechanical ventilation was 3 days. Hospital mortality was 16% (7/42). In the postoperative period, 26% of patients were subjected to early extubation, and all of them received treatment with systemic vasodilatory agents. Feeding was started 6 hours after extubation according to a dedicated feeding protocol. After treatment, none of our patients experienced any grade of necrotising enterocolitis or major gastrointestinal adverse events. Conclusions Our experience indicates that the combination of an early hybrid approach, systemic vasodilator therapy, and dedicated feeding protocol adherence could reduce the incidence of gastrointestinal complications in this group of neonates. Fast weaning from ventilatory support, which represents a part of our treatment strategy, could be associated with low incidence of necrotising enterocolitis. AD - L. Manuri, Cardiovascular Department, Mediterranean Pediatric Cardiology Center, Bambino Gesù Children's Hospital, Contrada Sirina, Taormina, Italy AU - Manuri, L. AU - Morelli, S. AU - Agati, S. AU - Saitta, M. B. AU - Oreto, L. AU - Mandraffino, G. AU - Iannace, E. AU - Iorio, F. S. AU - Guccione, P. DB - Embase Medline DO - 10.1017/S1047951116000275 IS - 1 KW - amrinone dobutamine dopamine epinephrine milrinone noradrenalin prostaglandin algorithm article artificial ventilation birth weight clinical article disease severity drug dose reduction ductus dependent systemic circulation early hybrid approach enteric feeding extubation female hospital mortality human incidence length of stay male necrotizing enterocolitis neonatal intensive care unit newborn Norwood procedure palliative therapy patent ductus arteriosus postoperative period protocol compliance risk reduction standardization systemic circulation systemic therapy ventilator weaning LA - English M3 - Article N1 - L614784771 2017-03-17 2017-04-11 PY - 2017 SN - 1467-1107 1047-9511 SP - 154-160 ST - Early hybrid approach and enteral feeding algorithm could reduce the incidence of necrotising enterocolitis in neonates with ductus-dependent systemic circulation T2 - Cardiology in the Young TI - Early hybrid approach and enteral feeding algorithm could reduce the incidence of necrotising enterocolitis in neonates with ductus-dependent systemic circulation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L614784771 http://dx.doi.org/10.1017/S1047951116000275 VL - 27 ID - 779 ER - TY - JOUR AB - Children with single ventricle congenital heart defects (SVCHD) experience a significant risk of early mortality throughout their lifespan, particularly during their first year of life. Due to the intense care needed for these children and families, pediatric palliative care (PPC) team consults should be routine; however, medical staff are often reluctant to broach the idea of PPC to families. The involvement of PPC for many carries with it an association to end-of-life (EOL) care. Setting the standard of PPC involvement from the time of admission for the first palliative surgery led to increased family support, decreased days to consult, improved acceptance and communication. The purpose of this article is to describe a quality improvement project of early integration of PPC with families of children with SVCHD. Lessons learned will be presented, including the resources needed and the barriers encountered in assimilating PPC into the standard of care for all patients with SVCHD. The single ventricle (SV) and PPC teams collaborated to enhance the support given to SV families. Education was initiated with cardiology and PPC providers to understand the goal of consistent PPC consults beginning after birth for patients with SVCHD. Parents were educated during fetal consultation regarding the involvement of the PPC team. The SV team ensured compliance with the PPC initiative by identifying eligible patients and requesting consult orders from the primary providers. PPC consultation increased significantly over the 40 month study period to nearly 100% compliance for children with SVCHD who are undergoing pre-Fontan surgery. In addition, mean days to consult decreased dramatically during the study to a current average of 3 days into the patient's hospitalization; the data likely suggest that more PPC consults were routinely ordered versus urgently placed for unexpected complications. Data indicate that patients are being followed by the PPC team at an earlier age and stage in their SV journey which allows for more opportunity to provide meaningful support to these patients and families. The early involvement of the PPC team for children with SV physiology was operationally feasible and was accepted by families, thus allowing PPC providers to establish a therapeutic relationship early in the disease trajectory with the family. It allowed more continuity throughout the SV journey in a proactive fashion rather than a reactive manner. AD - Nationwide Children's Hospital, 700 Children's Dr, Columbus, OH, 43205, USA. joann.davis@nationwidechildrens.org. Nationwide Children's Hospital, 700 Children's Dr, Columbus, OH, 43205, USA. Nationwide Children's Hospital, The Ohio State School of Medicine, 700 Children's Dr, Columbus, OH, 43205, USA. AN - 31676955 AU - Davis, J. A. M. AU - Bass, A. AU - Humphrey, L. AU - Texter, K. AU - Garee, A. DA - Jan DB - PubMed DO - 10.1007/s00246-019-02231-y DP - NLM ET - 2019/11/05 IS - 1 KW - Female Humans Infant Infant, Newborn Male Palliative Care/*methods/psychology Parents/*psychology Patient Care Team/organization & administration *Professional-Family Relations Quality Improvement Referral and Consultation/statistics & numerical data Retrospective Studies Time Factors Univentricular Heart/mortality/*therapy Cardiac Collaboration End-of-life Palliative Pediatric Single ventricle LA - eng N1 - 1432-1971 Davis, Jo Ann M Orcid: 0000-0002-9609-8262 Bass, Alice Humphrey, Lisa Texter, Karen Garee, Amy Journal Article United States Pediatr Cardiol. 2020 Jan;41(1):114-122. doi: 10.1007/s00246-019-02231-y. Epub 2019 Nov 1. PY - 2020 SN - 0172-0643 SP - 114-122 ST - Early Integration of Palliative Care in Families of Children with Single Ventricle Congenital Heart Defects: A Quality Improvement Project to Enhance Family Support T2 - Pediatr Cardiol TI - Early Integration of Palliative Care in Families of Children with Single Ventricle Congenital Heart Defects: A Quality Improvement Project to Enhance Family Support VL - 41 ID - 178 ER - TY - JOUR AB - Objective: The critical importance of a secure mother-infant attachment relationship for long-term physical and mental health of the child is well established. Our study aim was to explore mothers' subjective experience of the mother-infant relationship after discharge from hospital following neonatal cardiac surgery. Design: Participants were 97 infants who underwent cardiac surgery before the age of 3 months and their mothers. Mothers completed Maternal Postnatal Attachment Scale (MPAS) and Edinburgh Postnatal Depression Scale (EPDS) questionnaires and were interviewed after the infant had been discharged home for 4 weeks. Interviews were analysed using inductive thematic analysis. Results: Mean sores on the MPAS were similar to community norms (84.5 (SD 7.2) vs 84.6 (SD 7), p=0.47). 66/91 mothers interviewed described impacts which encompassed four themes; enhanced emotional ties (n=34, 37%), 'bonding' difficulties (n=22, 23%), anxiety and worry (n=17, 19%), and caregiving behaviours (n=10, 11%). Mothers who described bonding difficulties had lower MPAS scores (mean 80.6 (SD 10) vs 85.7 (SD 5.7), p=0.0047), were more likely to have a prenatal diagnosis of the cardiac abnormality (OR 2.6, 95% CI 0.89 to 8.9) and higher EPDS score (9.1 (SD 5.3) vs 6.2 (SD 3.9), p=0.01). Higher EPDS scores were associated with lower MPAS scores (r= -0.44, p=0.0001). Conclusions: Most mothers report a positive relationship with their infant following cardiac surgery but almost a quarter have difficulties forming a strong emotional tie. Clinical care (including prenatal) of the infant with congenital heart disease requiring surgery should include screening, assessment and appropriate referral for early intervention if mothers are struggling to form a bond with their infant. AD - B. Jordan, UOM Department of Paediatrics, Royal Children's Hospital, Level 2 West, Flemington Road, Parkville, VIC 3052, Australia AU - Jordan, B. AU - Franich-Ray, C. AU - Albert, N. AU - Anderson, V. AU - Northam, E. AU - Cochrane, A. AU - Menahem, S. DB - Embase Medline DO - 10.1136/archdischild-2012-303488 IS - 7 KW - adult anxiety aortic arch interruption aortic coarctation article community Edinburgh Postnatal Depression Scale Fallot tetralogy female great vessels transposition heart surgery heart ventricle septum defect hospital discharge human hypoplastic left heart syndrome infancy infant lung vein drainage anomaly major clinical study male maternal postnatal attachment scale mother mother child relation assessment of humans newborn surgery Norwood procedure patent ductus arteriosus patient worry personal experience prenatal diagnosis priority journal pulmonary valve atresia qualitative analysis telephone interview thematic analysis tricuspid valve atresia LA - English M3 - Article N1 - L53061438 2014-03-24 2014-06-30 PY - 2014 SN - 1468-2044 0003-9888 SP - 641-645 ST - Early mother-infant relationships after cardiac surgery in infancy T2 - Archives of Disease in Childhood TI - Early mother-infant relationships after cardiac surgery in infancy UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L53061438 http://dx.doi.org/10.1136/archdischild-2012-303488 VL - 99 ID - 951 ER - TY - JOUR AB - The hybrid strategy is one approach to single ventricle palliation. In this study, we reported neurodevelopment at 12 months for two cohorts of children managed with the hybrid and clinical factors associated with neurodevelopment in the entire sample. We performed a retrospective study of children with single ventricle who had undergone a neonatal hybrid procedure. One group included infants with hypoplastic left heart syndrome (HLHS); another group included infants with non-HLHS single ventricle. Neurodevelopment was assessed with 12-month Bayley III. Parametric and non-parametric statistics were used for analysis. Nine infants with HLHS and 15 with non-HLHS were identified. Abnormal neurodevelopment was identified in 11 of 24 (46%), primarily motor (46%). Development did not differ between groups. In the whole sample, higher lactate levels were associated with lower cognitive scores (p = 0.04). Fewer mechanical ventilation days were associated with higher cognitive scores (p = 0.05) after Stage 1 and higher motor scores after Stage 2. Shorter ICU length of stay (p = 0.01), shorter hospital length of stay (p = 0.01), and fewer complications (p = 0.01) after stage 2 were associated with higher motor scores. Higher cognitive (p = 0.02) and language (p = 0.002) scores were associated with higher weight at 12 months. In the largest cohort of single ventricle children treated with neonatal hybrid palliation yet reported, significant neurodevelopmental impairment was identified. No differences in neurodevelopment were found between children with HLHS and those with non-HLHS variants. A multicenter trial is needed to test differences in neurodevelopment between hybrid and Norwood approaches. AD - The Heart Center, Nationwide Children's Hospital, 700 Children's Dr, Columbus, OH, 43205, USA. omar.khalid@nationwidechildrens.org. The Ohio State University College of Nursing, 1585 Neil Ave., Columbus, OH, 43210, USA. AN - 31471627 AU - Khalid, O. M. AU - Harrison, T. M. DA - Dec DB - PubMed DO - 10.1007/s00246-019-02191-3 DP - NLM ET - 2019/09/01 IS - 8 KW - Cardiac Surgical Procedures/*adverse effects/methods Case-Control Studies Developmental Disabilities/*etiology Female Humans Hypoplastic Left Heart Syndrome/*psychology/*surgery Infant Male Palliative Care/methods Retrospective Studies Treatment Outcome Congenital heart disease Hybrid procedure Hypoplastic left heart syndrome Neurodevelopmental outcome LA - eng N1 - 1432-1971 Khalid, O M Orcid: 0000-0001-5139-7333 Harrison, T M Journal Article United States Pediatr Cardiol. 2019 Dec;40(8):1591-1598. doi: 10.1007/s00246-019-02191-3. Epub 2019 Aug 30. PY - 2019 SN - 0172-0643 SP - 1591-1598 ST - Early Neurodevelopmental Outcomes in Children with Hypoplastic Left Heart Syndrome and Related Anomalies After Hybrid Procedure T2 - Pediatr Cardiol TI - Early Neurodevelopmental Outcomes in Children with Hypoplastic Left Heart Syndrome and Related Anomalies After Hybrid Procedure VL - 40 ID - 29 ER - TY - JOUR AB - OBJECTIVES: This study investigated the relationship between early postoperative serum C-reactive protein (CRP) levels, a marker of systemic inflammatory response, and 2-year neurodevelopment-associated outcomes among survivors after undergoing the Norwood procedure. METHODS: Among 53 neonates with hypoplastic left heart syndrome undergoing the Norwood procedure during 2003-2009, CRP was measured in 43 patients twice weekly within postoperative day 20. Two-year cognition, language, and motor scores were assessed with Bayley Scales of Infant and Toddler Development III in 26 patients (9 deaths, 2 lost, and 6 assessed with Bayley Scales of Infant Development II). Peak CRP levels of the 26 patients were recorded, with peak total and differential white blood cell counts (lowest lymphocytes) and glucose. Demographic data included age at surgery, socioeconomic status of the families, durations of cardiopulmonary bypass and aortic crossclamp, deep hypothermic circulatory arrest, and intensive care unit stay. RESULTS: The cognitive score was 91 ± 13, language score was 86 ± 13, and motor score was 85 ± 17. The peak CRP level was 79 ± 37 mg/L. Univariate regression showed that the cognitive score significantly and negatively correlated with peak CRP level (P = .004), and trended to a negative correlation with age at surgery (P = .097). The language score significantly and negatively correlated with peak CRP level (P < .0001) and age (P = .005). The motor score trended to a negative correlation with age (P = .08). Multivariate regression showed that both cognitive and language scores significantly and negatively correlated only with peak CRP level (P < .01 for both). CONCLUSIONS: The magnitude of systemic inflammatory response, among the perioperative factors examined, may be an important determinant for adverse 2-year cognition and language outcomes after the Norwood procedure. Confirmatory studies in larger populations, including those undergoing other types of cardiac surgeries, are warranted. AD - Department of Cardiovascular Diseases, Children's Hospital affiliated to Capital Institute of Pediatrics, Beijing, China. Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada; Pediatric Rehabilitation Outcomes Evaluation and Research Unit, Glenrose Rehabilitation Hospital, Edmonton, Alberta, Canada. Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada. School of Public Health, University of Alberta, Edmonton, Alberta, Canada. Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada; Clinical Physiology Research Center, Capital Institute of Pediatrics, Beijing, China. Electronic address: j1al1@yahoo.com. AN - 24060363 AU - Li, X. AU - Robertson, C. M. AU - Yu, X. AU - Cheypesh, A. AU - Dinu, I. A. AU - Li, J. DA - Jul DB - PubMed DO - 10.1016/j.jtcvs.2013.07.079 DP - NLM ET - 2013/09/26 IS - 1 KW - Age Factors Biomarkers/blood Blood Glucose/metabolism C-Reactive Protein/metabolism *Child Development Child Language Child, Preschool Cognition Developmental Disabilities/diagnosis/*etiology/physiopathology/psychology Humans Hypoplastic Left Heart Syndrome/diagnosis/*surgery Infant Infant Behavior Infant, Newborn Inflammation Mediators/blood Leukocyte Count Linear Models Logistic Models Motor Activity Multivariate Analysis Nervous System/*growth & development Neuropsychological Tests Norwood Procedures/*adverse effects Odds Ratio Retrospective Studies Risk Factors Systemic Inflammatory Response Syndrome/blood/diagnosis/*etiology Treatment Outcome LA - eng N1 - 1097-685x Li, Xiaohui Robertson, Charlene M T Yu, Xiaoyang Cheypesh, Andriy Dinu, Irina A Li, Jia Journal Article Research Support, Non-U.S. Gov't United States J Thorac Cardiovasc Surg. 2014 Jul;148(1):202-6. doi: 10.1016/j.jtcvs.2013.07.079. Epub 2013 Sep 20. PY - 2014 SN - 0022-5223 SP - 202-6 ST - Early postoperative systemic inflammatory response is an important determinant for adverse 2-year neurodevelopment-associated outcomes after the Norwood procedure T2 - J Thorac Cardiovasc Surg TI - Early postoperative systemic inflammatory response is an important determinant for adverse 2-year neurodevelopment-associated outcomes after the Norwood procedure VL - 148 ID - 388 ER - TY - JOUR AB - Objectives This study investigated the relationship between early postoperative serum C-reactive protein (CRP) levels, a marker of systemic inflammatory response, and 2-year neurodevelopment-associated outcomes among survivors after undergoing the Norwood procedure. Methods Among 53 neonates with hypoplastic left heart syndrome undergoing the Norwood procedure during 2003-2009, CRP was measured in 43 patients twice weekly within postoperative day 20. Two-year cognition, language, and motor scores were assessed with Bayley Scales of Infant and Toddler Development III in 26 patients (9 deaths, 2 lost, and 6 assessed with Bayley Scales of Infant Development II). Peak CRP levels of the 26 patients were recorded, with peak total and differential white blood cell counts (lowest lymphocytes) and glucose. Demographic data included age at surgery, socioeconomic status of the families, durations of cardiopulmonary bypass and aortic crossclamp, deep hypothermic circulatory arrest, and intensive care unit stay. Results The cognitive score was 91 ± 13, language score was 86 ± 13, and motor score was 85 ± 17. The peak CRP level was 79 ± 37 mg/L. Univariate regression showed that the cognitive score significantly and negatively correlated with peak CRP level (P =.004), and trended to a negative correlation with age at surgery (P =.097). The language score significantly and negatively correlated with peak CRP level (P <.0001) and age (P =.005). The motor score trended to a negative correlation with age (P =.08). Multivariate regression showed that both cognitive and language scores significantly and negatively correlated only with peak CRP level (P <.01 for both). Conclusions The magnitude of systemic inflammatory response, among the perioperative factors examined, may be an important determinant for adverse 2-year cognition and language outcomes after the Norwood procedure. Confirmatory studies in larger populations, including those undergoing other types of cardiac surgeries, are warranted. © 2014 by The American Association for Thoracic Surgery. AD - J. Li, Clinical Physiology Research Center, Capital Institute of Pediatrics, 2 Yabao Rd, Beijing, 100020, China AU - Li, X. AU - Robertson, C. M. T. AU - Yu, X. AU - Cheypesh, A. AU - Dinu, I. A. AU - Li, J. DB - Embase Medline DO - 10.1016/j.jtcvs.2013.07.079 IS - 1 KW - C reactive protein glucose article Bayley Scales of Infant Development cardiopulmonary bypass cognition deep hypothermic circulatory arrest human hypoplastic left heart syndrome intensive care unit language length of stay leukocyte leukocyte differential count major clinical study medical record review motor performance nervous system development newborn Norwood procedure operation duration postoperative inflammation priority journal protein blood level social status surgical mortality treatment outcome LA - English M3 - Article N1 - L52788112 2013-09-25 2014-07-02 PY - 2014 SN - 1097-685X 0022-5223 SP - 202-206 ST - Early postoperative systemic inflammatory response is an important determinant for adverse 2-year neurodevelopment-associated outcomes after the Norwood procedure T2 - Journal of Thoracic and Cardiovascular Surgery TI - Early postoperative systemic inflammatory response is an important determinant for adverse 2-year neurodevelopment-associated outcomes after the Norwood procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52788112 http://dx.doi.org/10.1016/j.jtcvs.2013.07.079 VL - 148 ID - 944 ER - TY - JOUR AB - We aimed to study the association between use of antihistamines in early pregnancy and congenital heart defects (CHD) in the offspring. Design: Two case–control studies. Setting: HAVEN study, Erasmus MC, University Medical Centre, Rotterdam, and Eurocat Northern Netherlands (NNL), University Medical Center Groningen, Groningen, the Netherlands. We studied 361 children with CHD and 410 controls without congenital malformations from the HAVEN study and replicated the analyses in 445 children with CHD and 530 controls from the Eurocat NNL registry. Information about antihistamine use in early pregnancy and potential confounders was obtained from questionnaires postpartum. We calculated the association between antihistamines and CHD risk by multivariable logistic regression analysis. Main outcome measures: Odds ratios (OR) with 95 % confidence intervals (CI). In the HAVEN study, 25 of 771 mothers used antihistamines that were associated with an increased CHD risk (OR 3.0, 95 % CI 1.2–7.3), particularly atrioventricular septal defects (AVSD) (OR 5.1, 95 % CI 1.3–20.5) and perimembranous ventricular septal defects (pVSD) (OR 5.1, 95 % CI 1.8–14.4). Mothers with severe nausea who did not use antihistamines had a reduced risk (OR 0.7, 95 % CI 0.5–0.98), whereas nauseous mothers using antihistamines showed an almost fivefold increased risk of pVSD (OR 4.8, 95 % CI 1.1–21.8). The association between antihistamines and AVSD was confirmed in the Eurocat cohort (OR 3.5, 95 % CI 1.4–8.7), but we could not replicate the association with overall CHD risk. We found a positive association between antihistamine use in early pregnancy and CHD risk, particularly AVSD, which seemed to be independent of nausea/vomiting. AD - R.P.M. Steegers-Theunissen, Department of Obstetrics and Gynaecology, Erasmus MC, University Medical Centre, Dr. Molewaterplein 40, Room Ee 2271a, Rotterdam, Netherlands AU - Smedts, H. P. M. AU - de Jonge, L. AU - Bandola, S. J. G. AU - Baardman, M. E. AU - Bakker, M. K. AU - Stricker, B. H. C. AU - Steegers-Theunissen, R. P. M. DB - Embase Medline DO - 10.1007/s10654-014-9925-0 IS - 9 KW - antihistaminic agent cetirizine levocetirizine meclozine meclozine plus pyridoxine adult age distribution aortic valve stenosis article atrioventricular septal defect case control study congenital heart malformation controlled study disease association disease severity Fallot tetralogy female first trimester pregnancy heart single ventricle heart ventricle septum defect heredity human hypoplastic left heart syndrome major clinical study male maternal age mitral valve atresia nausea nausea and vomiting outcome assessment perimembranous ventricular septal defect phenotype prenatal drug exposure pulmonary valve stenosis risk factor risk reduction tricuspid valve atresia LA - English M3 - Article N1 - L601206910 2015-01-20 2015-01-23 PY - 2014 SN - 1573-7284 0393-2990 SP - 653-661 ST - Early pregnancy exposure to antihistamines and risk of congenital heart defects: results of two case–control studies T2 - European Journal of Epidemiology TI - Early pregnancy exposure to antihistamines and risk of congenital heart defects: results of two case–control studies UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L601206910 http://dx.doi.org/10.1007/s10654-014-9925-0 VL - 29 ID - 971 ER - TY - JOUR AB - Background: Different types of conduits are used for extracardiac Fontan procedure. The use of autologous pericardial tube as an alternative conduit for connecting the inferior vena cava to the pulmonary artery is investigated. Methods: We performed 25 extracardiac Fontan procedures by using pericardial tube between June 2000 and October 2003. Fifteen patients were male. Mean age and weight were 7.6 ± 4.9 years (range 3 to 24 years) and 22.8 ± 10.4 kg (range 12-50 kg), respectively. Results: All patients survived after extracardiac Fontan procedure. Prolonged chest tube drainage (> 7 days) was required in 9 (36%) patients. Follow-up was complete and mean follow-up was 24.6 ± 11.5 months (1-39 months). One patient died 3 months postoperatively. Routine serial postoperative echocardiographic examinations in all patients and magnetic resonance angiography in 6 patients with suspect flow patterns in echocardiography did not reveal any problems with the Fontan circuit. Conclusions: Fresh autologous pericardial tube conduit is a suitable and safe alternative for extracardiac Fontan procedures. Major advantages are availability, no cost, easy handling and hemostasis, low risk of thrombosis and emboli, and growth potential. AD - T. Sarioglu, Department of Cardiovascular Surgery, Acibadem Hospital Bakirkoy, Halit Ziya Usakligil Cad. 1, 34140 Bakirkoy, Istanbul, Turkey AU - Yalcinbas, Y. K. AU - Erek, E. AU - Salihoglu, E. AU - Sarioglu, A. AU - Sarioglu, T. DB - Embase Medline DO - 10.1055/s-2004-830459 IS - 1 KW - adolescent adult article blood flow child clinical article controlled study echocardiography embolism female follow up Fontan procedure heart output hemostasis human magnetic resonance angiography male oxygen saturation postoperative complication postoperative period postoperative thrombosis priority journal risk reduction safety surgical technique survival rate treatment outcome tube LA - English M3 - Article N1 - L40288941 2005-03-10 PY - 2005 SN - 0171-6425 SP - 37-40 ST - Early results of extracardiac Fontan procedure with autologous pericardial tube conduit T2 - Thoracic and Cardiovascular Surgeon TI - Early results of extracardiac Fontan procedure with autologous pericardial tube conduit UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L40288941 http://dx.doi.org/10.1055/s-2004-830459 VL - 53 ID - 1282 ER - TY - JOUR AB - Motor skills and neurodevelopment in infants with hypoplastic left heart syndrome (HLHS) who have undergone Hybrid Stage I palliation is unknown. The purpose of this study is to assess early neurodevelopment in infants with HLHS after Hybrid Stage I palliation. Developmental assessment was performed in HLHS infants who underwent Hybrid Stage I palliation at 2 and 4 months of age using the Test of Infant Motor Performance, and at 6 months of age, prior to undergoing the second staged surgery, using the Bayley Scales of Infant and Toddler Development, 3rd edition (Bayley-III). Results were compared to healthy control subjects and norm-referenced data. The HLHS group scored between -1 and -2 standard deviations (SD) below the mean at 2 months of age (p = 0.002), and within -1 SD of the mean, at 4 months of age (p = 0.0019), on the TIMP. Compared to the control group, composite motor skills were significantly lower at 6 months of age on the Bayley-III in the HLHS group (p = 0.0489), however, not significant for cognitive (p = 0.29) or language (p = 0.68). Percentile rank motor scores were 17 ± 20 % in the HLHS group compared to 85 ± 12 % for the healthy age-matched control group. Infants with HLHS who undergo Hybrid Stage I palliation score lower on standardized motor skill tests compared to healthy age-matched controls and the norm-referenced population. This suggests that infants with HLHS have poorer motor skill performance than typically developing infants at 6 months of age. AD - The Heart Center, Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH, 43205, USA, Sharon.Cheatham@Nationwidechildrens.org. AN - 25380966 AU - Cheatham, S. L. AU - Carey, H. AU - Chisolm, J. L. AU - Heathcock, J. C. AU - Steward, D. DA - Mar DB - PubMed DO - 10.1007/s00246-014-1065-5 DP - NLM ET - 2014/11/09 IS - 3 KW - *Child Development Child Language Cognition Female Humans Hypoplastic Left Heart Syndrome/physiopathology/psychology/*surgery Infant Male *Motor Skills Neuropsychological Tests Palliative Care/*methods Time Factors Treatment Outcome LA - eng N1 - 1432-1971 Cheatham, Sharon L Carey, Helen Chisolm, Joanne L Heathcock, Jill C Steward, Deborah F31NR011253/NR/NINR NIH HHS/United States Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't United States Pediatr Cardiol. 2015 Mar;36(3):685-91. doi: 10.1007/s00246-014-1065-5. Epub 2014 Nov 8. PY - 2015 SN - 0172-0643 SP - 685-91 ST - Early results of neurodevelopment following hybrid stage I for hypoplastic left heart syndrome T2 - Pediatr Cardiol TI - Early results of neurodevelopment following hybrid stage I for hypoplastic left heart syndrome VL - 36 ID - 161 ER - TY - JOUR AB - OBJECTIVES: To investigate the long-term impact of early-term birth (37-38 weeks' gestation) relative to full-term birth (≥39 weeks' gestation) on neurodevelopmental and psychiatric outcomes in adolescents with single-ventricle congenital heart disease (CHD). STUDY DESIGN: This cross-sectional cohort study analyzed retrospective medical records from full term adolescents with single-ventricle CHD who underwent the Fontan procedure. Participants underwent neurodevelopmental and psychiatric evaluations, as well as structural brain magnetic resonance imaging. Early-term born adolescents were compared with full-term born adolescents using regression models with adjustments for family social status, birth weight, and genetic abnormality status. Medical and demographic risk factors were examined as well. RESULTS: Compared with the full-term group (n = 100), adolescents born early term (n = 33) scored significantly worse on daily-life executive functions, as measured by the Behavior Rating Inventory of Executive Function parent-report (mean scores: early term, 62.0 ± 10.9; full-term, 55.6 ± 12.2; P = .009) and self-report (P = .02) composites. Adolescents born early term were more likely than those born full term to have a lifetime attention-deficit/hyperactivity disorder (ADHD) diagnosis (early term, 55%; full term, 26%; P = .001). The early-term group also displayed greater psychiatric symptom severity, as indicated by the clinician-reported Brief Psychiatric Rating Scale (mean score: early term, 16.1 ± 8.6; full-term, 12.5 ± 8.2; P = .007). CONCLUSION: Early-term birth is associated with greater prevalence of executive dysfunction, ADHD diagnosis, and psychiatric problems in adolescents with single-ventricle CHD. Early-term birth should be included as a potential risk factor in the algorithm for closer developmental surveillance in CHD. AD - Department of Psychiatry, Boston Children's Hospital, Boston, MA; Department of Psychiatry, Harvard Medical School, Boston, MA. Department of Cardiology, Boston Children's Hospital, Boston, MA. Department of Cardiology, Boston Children's Hospital, Boston, MA; Department of Pediatrics, Harvard Medical School, Boston, MA; Department of Biostatistics, Harvard T.H. Chan School of Public Health, Boston, MA. Department of Neurology, Boston Children's Hospital, Boston, MA. Department of Cardiology, Boston Children's Hospital, Boston, MA; Department of Pediatrics, Harvard Medical School, Boston, MA. Department of Psychiatry, Boston Children's Hospital, Boston, MA; Department of Neurology, Boston Children's Hospital, Boston, MA; Department of Neurology, Harvard Medical School, Boston, MA. AN - 27692462 AU - Calderon, J. AU - Stopp, C. AU - Wypij, D. AU - DeMaso, D. R. AU - Rivkin, M. AU - Newburger, J. W. AU - Bellinger, D. C. DA - Dec DB - PubMed DO - 10.1016/j.jpeds.2016.08.084 DP - NLM ET - 2016/10/04 KW - Adolescent Attention Deficit Disorder with Hyperactivity/epidemiology Cohort Studies Cross-Sectional Studies Executive Function Female *Fontan Procedure Gestational Age Heart Defects, Congenital/*surgery Heart Ventricles/*abnormalities/*surgery Humans Male Mental Disorders/*epidemiology Neurodevelopmental Disorders/*epidemiology Term Birth *adhd *CHD neurodevelopment *adolescence *gestational age LA - eng N1 - 1097-6833 Calderon, Johanna Stopp, Christian Wypij, David DeMaso, David R Rivkin, Michael Newburger, Jane W Bellinger, David C U54 HD090255/HD/NICHD NIH HHS/United States Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't United States J Pediatr. 2016 Dec;179:96-103. doi: 10.1016/j.jpeds.2016.08.084. Epub 2016 Sep 28. PY - 2016 SN - 0022-3476 SP - 96-103 ST - Early-Term Birth in Single-Ventricle Congenital Heart Disease After the Fontan Procedure: Neurodevelopmental and Psychiatric Outcomes T2 - J Pediatr TI - Early-Term Birth in Single-Ventricle Congenital Heart Disease After the Fontan Procedure: Neurodevelopmental and Psychiatric Outcomes VL - 179 ID - 135 ER - TY - JOUR AB - Aortic-mitral valve discontinuity has previously been described in double outlet right ventricle, endocardial cushion defect, single ventricle, tetralogy of Fallot, and prolapse of the mitral valve. We are reporting two additional examples of aortic-mitral valve discontinuity including 15 cases of gross left ventricular dilation and a case of acute pneumococcal bacterial endocarditis with a large subannular erosion. While nonspecific, aortic-mitral valve discontinuity is a clinically important sign that should be sought with slow-M-mode scanning and strip chart recording. AN - 123181 AU - Strunk, B. L. AU - Guss, S. B. AU - Hicks, R. E. AU - Kotler, M. N. DA - Apr DB - PubMed DO - 10.1161/01.cir.51.4.594 DP - NLM ET - 1975/04/01 IS - 4 KW - Adolescent Adult Alcoholism/complications Aorta/physiopathology Aortic Valve Insufficiency/*physiopathology Cardiac Catheterization Cardiomegaly/*physiopathology Cardiomyopathies/*physiopathology Child Coronary Disease/*physiopathology *Echocardiography Humans Middle Aged Mitral Valve/*physiopathology Mitral Valve Insufficiency/*physiopathology LA - eng N1 - Strunk, B L Guss, S B Hicks, R E Kotler, M N Journal Article United States Circulation. 1975 Apr;51(4):594-8. doi: 10.1161/01.cir.51.4.594. PY - 1975 SN - 0009-7322 (Print) 0009-7322 SP - 594-8 ST - Echocardiographic recognition of the mitral valve-posterior aortic wall relationship T2 - Circulation TI - Echocardiographic recognition of the mitral valve-posterior aortic wall relationship VL - 51 ID - 515 ER - TY - JOUR AB - The purpose of this study was to measure reproducibility in strain measurements by echocardiography and cardiac magnetic resonance imaging (CMR) in functional single ventricles in the Fontan circulation. Fontan echocardiograms and CMRs between 1/2005 and 6/2013 were analyzed retrospectively. Patients were included if studies occurred <1 year apart and without interim catheter-based or surgical intervention. Global peak systolic circumferential (GCS) and longitudinal (GLS) strain, and strain rates (GCSR and GLSR) were measured by speckle or feature tracking (echocardiogram vs. CMR) using commercial software. 134 study pairs were identified; 74 with dominant left and 60 with dominant right ventricles. Agreement for strain and strain rate between echocardiogram and CMR was modest for circumferential measurements, concordance correlation coefficient (Rc) = 0.56 for GCS, 0.44 for GCSR; and low for longitudinal measurements, Rc = 0.34 for GLS, and 0.27 for GLSR. There was good inter-observer agreement (n = 30) for strain measurements by echocardiogram and CMR (echocardiogram GCS Rc = 0.84, echocardiogram GLS Rc = 0.79, CMR GCS Rc = 0.94, CMR GLS Rc = 0.75). Inter-observer agreement for strain rate was slightly lower (echocardiogram GCSR Rc = 0.79, echocardiogram GLSR Rc = 0.73, CMR GCSR Rc = 0.93, CMR GLSR Rc = 0.54). Coefficients for intra-observer agreement for these variables were similar to inter-observer agreement. In conclusion, intra-modality reproducibility for strain assessment in functional single ventricles in the Fontan circulation is high, measured both by echocardiography and CMR; inter-modality reproducibility, however, is modest. These findings caution against mixing values from multiple modalities in the long-term assessment of patient health in this population. AD - Department of Cardiology, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, 02115, USA. Department of Pediatrics, Harvard Medical School, Boston, MA, USA. Department of Cardiology, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, 02115, USA. rahul.rathod@childrens.harvard.edu. Department of Pediatrics, Harvard Medical School, Boston, MA, USA. rahul.rathod@childrens.harvard.edu. AN - 27091734 AU - Ghelani, S. J. AU - Harrild, D. M. AU - Gauvreau, K. AU - Geva, T. AU - Rathod, R. H. DA - Jul DB - PubMed DO - 10.1007/s10554-016-0882-4 DP - NLM ET - 2016/04/20 IS - 7 KW - Adolescent Boston Child *Echocardiography Female Fontan Procedure Heart Defects, Congenital/*diagnostic imaging/physiopathology/surgery Heart Ventricles/abnormalities/*diagnostic imaging/physiopathology/surgery Humans *Magnetic Resonance Imaging Male *Myocardial Contraction Observer Variation Predictive Value of Tests Reproducibility of Results Retrospective Studies Stress, Mechanical *Ventricular Function Young Adult *Congenital heart disease *Fontan *Single ventricle *Strain LA - eng N1 - 1875-8312 Ghelani, Sunil J Harrild, David M Gauvreau, Kimberlee Geva, Tal Rathod, Rahul H Comparative Study Journal Article United States Int J Cardiovasc Imaging. 2016 Jul;32(7):1113-20. doi: 10.1007/s10554-016-0882-4. Epub 2016 Apr 18. PY - 2016 SN - 1569-5794 SP - 1113-20 ST - Echocardiography and magnetic resonance imaging based strain analysis of functional single ventricles: a study of intra- and inter-modality reproducibility T2 - Int J Cardiovasc Imaging TI - Echocardiography and magnetic resonance imaging based strain analysis of functional single ventricles: a study of intra- and inter-modality reproducibility VL - 32 ID - 198 ER - TY - JOUR AB - Background: Extracorporeal membrane oxygenation (ECMO) is a temporary mechanical support system that handles the function of the heart and/or lungs in patients with a variety of cardiac and respiratory problems. Veno-arterial ECMO is most commonly used for patients recovering from heart surgery, suffering from cardiac shock, or awaiting a heart transplant. Case report: A 7 year old boy with a single ventricle physiology was admitted for a Fontan procedure with repair of the common valve. At the end of surgery, transesophagal echocardiography showed a severely depressed myocardial function, and weaning of bypass required a high inotropic support. During the following week, the heart never recovered, with an ejection fraction at 24%. Veno-Arterial ECMO was decided on postoperative day 9 with a 19 Fr venous cannula inserted through the right jugular vein into the intracardiac Fontan baffle, and a 15 Fr arterial cannula into the right carotid artery. On ECMO day 7, after failure to wean from ECMO, he was listed for an urgent heart transplant, with the suggestion of a transfer to Europe, for a shorter transplantation delay. The ECMO team from Hamad Hospital in Qatar accomplished the transfer successfully, on a humanitarian basis. The transfer was done on ECMO Day 10. The child was safely admitted to a hospital in Belgium, and listed again for an urgent heart transplant. Conclusion: With an experienced team, and the proper equipment, on-ECMO air transport of critical patients over thousands of kilometers is today safely feasible. AD - I. El Rassi, Children's Heart Center, American University of Beirut Medical Center, Cairo Street, Beirut, Lebanon AU - Assy, J. AU - Fawzi, I. AU - Arabi, M. AU - Bulbul, Z. AU - Bitar, F. AU - Majdalani, M. AU - Sharara, R. AU - El Rassi, I. DB - Embase DO - 10.1016/j.ejccm.2018.12.010 IS - 3 KW - arterial cannula left ventricular assist device epinephrine inotropic agent levosimendan milrinone noradrenalin aircraft ambulance article Belgium Blalock Taussig shunt case report child clinical article disease severity extracorporeal oxygenation Fontan procedure forward heart failure Glenn shunt heart catheterization heart transplantation heart valve replacement heart ventricle septum defect helicopter hospital admission human inotropism Lebanon low drug dose male patient history of surgery patient transport pediatric intensive care unit preoperative evaluation Qatar school child transesophageal echocardiography LA - English M3 - Article N1 - L2001380136 2018-12-19 2019-01-11 PY - 2018 SN - 2090-7303 SP - 151-153 ST - ECMO is in the air: Long distance air/ground transport of a child on extra corporeal membrane oxygenation T2 - Egyptian Journal of Critical Care Medicine TI - ECMO is in the air: Long distance air/ground transport of a child on extra corporeal membrane oxygenation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2001380136 http://dx.doi.org/10.1016/j.ejccm.2018.12.010 VL - 6 ID - 646 ER - TY - JOUR AD - CHU de Nantes, Pediatric Cardiology Unit, 38, boulevard Jean-Monnet, 44093 Nantes Cedex 1, France INSERM, UMR915, L'Institut du Thorax, 8, quai Moncousu, 44000 Nantes, France AU - Gournay, V. AU - Guérin, P. DB - Scopus DO - 10.1016/j.acvd.2013.06.003 IS - 8-9 M3 - Article N1 - Export Date: 15 June 2020 PY - 2013 SP - 460 ST - Editorial T2 - Archives of Cardiovascular Diseases TI - Editorial UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84903495301&doi=10.1016%2fj.acvd.2013.06.003&partnerID=40&md5=ed4682ed24413316483688e5509654b6 VL - 106 ID - 2068 ER - TY - JOUR AB - The Fontan operation is a common end point for children born with a single functional ventricle. Fontan patients typically experience physiological deterioration leading to transplant or death in their third or fourth decades of life. This deterioration is partially attributable to progressive increases in pulmonary vascular resistance (PVR) and as such endothelin receptor antagonists, which are known to decrease pulmonary vascular resistance, have been proposed as potentially beneficial in this population. We conducted a single-center, randomized, double-blind, placebo-controlled, crossover study of 12 weeks of ambrisentan therapy (10 mg per day) versus placebo to test the hypothesis that endothelin receptor antagonism will improve cardiopulmonary exercise test parameters and 36-item short form (SF-36) assessed quality of life in adult Fontan patients. Twenty-eight patients entered the trial, 19 patients completed the protocol. Ambrisentan therapy improved peak oxygen consumption by 1.7 ml/kg/min in patients who achieved a respiratory exchange ratio of >0.95 (p = 0.05) and decreased the slope of the ventilatory equivalent ratio for oxygen (-2.8, p = 0.019) in all completers. It did not change SF-36 physical function score compared with placebo (p = 0.28). Ambrisentan therapy resulted in a decrease in (-1.4 g/dl, p <0.001) with no change in liver or renal function. Therapy was generally well tolerated, with no greater rate of side effects than placebo. In conclusion, ambrisentan is well tolerated and improves exercise capacity in adult Fontan patients. AD - Department of Cardiology, Baylor University Hospital, Dallas, Texas. Electronic address: acedars97@gmail.com. Department of Cardiology, Washington University School of Medicine, St. Louis, Missouri. AN - 27063478 AU - Cedars, A. M. AU - Saef, J. AU - Peterson, L. R. AU - Coggan, A. R. AU - Novak, E. L. AU - Kemp, D. AU - Ludbrook, P. A. DA - May 1 DB - PubMed DO - 10.1016/j.amjcard.2016.02.024 DP - NLM ET - 2016/04/12 IS - 9 KW - Adolescent Adult Cross-Over Studies Double-Blind Method Exercise Tolerance/*physiology Female *Fontan Procedure Heart Defects, Congenital/drug therapy/*physiopathology/*surgery Humans Male Oxygen Consumption/physiology Phenylpropionates/*therapeutic use Pyridazines/*therapeutic use Quality of Life Treatment Outcome Young Adult LA - eng N1 - 1879-1913 Cedars, Ari M Saef, Joshua Peterson, Linda R Coggan, Andrew R Novak, Eric L Kemp, Debra Ludbrook, Philip A Journal Article Randomized Controlled Trial Research Support, Non-U.S. Gov't United States Am J Cardiol. 2016 May 1;117(9):1524-32. doi: 10.1016/j.amjcard.2016.02.024. Epub 2016 Feb 23. PY - 2016 SN - 0002-9149 SP - 1524-32 ST - Effect of Ambrisentan on Exercise Capacity in Adult Patients After the Fontan Procedure T2 - Am J Cardiol TI - Effect of Ambrisentan on Exercise Capacity in Adult Patients After the Fontan Procedure VL - 117 ID - 130 ER - TY - JOUR AB - OBJECTIVES: To investigate the effect of bosentan in patients with a failing Fontan circulation. DESIGN: A multicentric open label, non-controlled study. SETTING: 5 tertiary care centres for congenital cardiology. PATIENTS: We included 10 patients with a failing Fontan circulation. Their median age at inclusion was 12.12 years, with a range from 4.41 to 33,41 years. The median interval between the Fontan operation and inclusion was 7.84 years, with a range from 1.96 to 12,18 years. Participants received half the usual dose of bosentan for 4 weeks, and then the full dose for a further 12 weeks. MAIN MEASURES OF OUTCOMES: We assessed saturations of oxygen at rest and during exercise, using a 6 minutes walk test, at baseline, and during and after 16 weeks of treatment. At each visit, we assessed blood chemistry and hepatic function, and asked the patients to complete a questionnaire concerning quality of life. All medical events and possible side effects were recorded. RESULTS: Of the cohort, 1 patient withdrew. The changes in saturations of oxygen, exercise performance, and scores for the questionnaire did not reach statistical significance for the whole group. We noted, nonetheless, that saturations of oxygen and/or exercise capacity improved in 5 of the patients. This was further confirmed when those patients deteriorated again when the drug was discontinued. CONCLUSIONS: Our study failed to show significant improvement after 3 months of treatment with bosentan in a small group of patients with failing Fontan circulations. Some individuals, nonetheless, did improve. When planning larger trials, it would be better to identify those patients who might potentially benefit from the treatment prior to commencing the trial. AD - Department of Pediatric Cardiology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium. AN - 19519964 AU - Ovaert, C. AU - Thijs, D. AU - Dewolf, D. AU - Ottenkamp, J. AU - Dessy, H. AU - Moons, P. AU - Gewillig, M. AU - Mertens, L. DA - Aug DB - PubMed DO - 10.1017/s1047951109990023 DP - NLM ET - 2009/06/13 IS - 4 KW - Adolescent Adult Antihypertensive Agents/*therapeutic use Bosentan Child Child, Preschool Female Follow-Up Studies Fontan Procedure/*adverse effects Heart Defects, Congenital/physiopathology/*therapy Humans Hypertension, Pulmonary/*drug therapy/etiology/physiopathology Male Pilot Projects Pulmonary Circulation/drug effects/*physiology Retrospective Studies Sulfonamides/*therapeutic use Treatment Outcome Young Adult LA - eng N1 - 1467-1107 Ovaert, Caroline Thijs, Daisy Dewolf, Daniel Ottenkamp, Jaap Dessy, Hugues Moons, Philip Gewillig, Marc Mertens, Luc Clinical Trial Journal Article Multicenter Study Research Support, Non-U.S. Gov't England Cardiol Young. 2009 Aug;19(4):331-9. doi: 10.1017/S1047951109990023. Epub 2009 Jun 11. PY - 2009 SN - 1047-9511 SP - 331-9 ST - The effect of bosentan in patients with a failing Fontan circulation T2 - Cardiol Young TI - The effect of bosentan in patients with a failing Fontan circulation VL - 19 ID - 311 ER - TY - JOUR AB - BACKGROUND: Palliative treatment with the Fontan procedure has greatly improved survival for children with functionally univentricular heart. Since Fontan performed the first successful operation, the procedure has evolved and is now performed as Total Cavo-Pulmonary Connection (TCPC).An increasing prevalence and longer life expectancy of TCPC patients have raised new challenges. The survivors are often suffering complications such as arrhythmias, myocardial dysfunction, thromboembolic events, neuropsychological deficit, protein-losing enteropathy and reduced exercise capacity. Several causes for the reduced exercise capacity may be present e.g. impaired function of the single ventricle, valve dysfunction and chronotropic impairment, and perhaps also increased pulmonary vascular resistance. Thus, plasma endothelin-1 has been shown to correlate with increased pulmonary vascular resistance and the risk of failing Fontan circulation. This has raised the question of the role for pulmonary vasodilation therapy, especially endothelin receptor antagonist in the management of TCPC patients. METHODS/DESIGN: The TEMPO trial aims to investigate whether Bosentan, an endothelin receptor antagonist, can be administered safely and improve exercise capacity in TCPC patients. The trial design is randomized, double-blind and placebo-controlled. Bosentan/placebo is administered for 14 weeks with control visits every four weeks. The primary endpoint is change in maximal oxygen consumption as assessed on bicycle ergometer test. Secondary endpoints include changes in pulmonary blood flow during exercise test, pro brain natriuretic peptide and quality of life. DISCUSSION: We hypothesize that treatment with Bosentan, an endothelin receptor antagonist, can be administered safely and improve exercise capacity in TCPC patients. AD - Department of Cardiology, Copenhagen University Hospital, Rigshospitalet, Blegdamsvej 9, Copenhagen, Denmark. andershebert@gmail.com AN - 23663658 AU - Hebert, A. AU - Jensen, A. S. AU - Idorn, L. AU - Sørensen, K. E. AU - Søndergaard, L. C2 - PMC3658877 DA - May 11 DB - PubMed DO - 10.1186/1471-2261-13-36 DP - NLM ET - 2013/05/15 KW - Bosentan Child Child, Preschool Cyclic N-Oxides/*therapeutic use Double-Blind Method *Endothelin Receptor Antagonists Exercise Test/*methods Female *Fontan Procedure Heart Defects, Congenital/*drug therapy/epidemiology/surgery Humans Male Sulfonamides/*therapeutic use Treatment Outcome LA - eng N1 - 1471-2261 Hebert, Anders Jensen, Annette S Idorn, Lars Sørensen, Keld E Søndergaard, Lars Journal Article Multicenter Study Randomized Controlled Trial BMC Cardiovasc Disord. 2013 May 11;13:36. doi: 10.1186/1471-2261-13-36. PY - 2013 SN - 1471-2261 SP - 36 ST - The effect of bosentan on exercise capacity in Fontan patients; rationale and design for the TEMPO study T2 - BMC Cardiovasc Disord TI - The effect of bosentan on exercise capacity in Fontan patients; rationale and design for the TEMPO study VL - 13 ID - 230 ER - TY - JOUR AB - Objectives We sought to assess the effect of congenital heart disease requiring infant surgery with cardiopulmonary bypass on neurodevelopmental outcomes and growth at 4 years of age, while matching for gestational age, socioeconomic status, maternal gestational conditions, home environment, and parental intelligence by studying multiple-gestation births. Methods We performed within-family comparison of 14 multiple-gestation births in which 1 child had congenital heart disease requiring surgery with cardiopulmonary bypass at ≤6 months of age. Between 4 and 5 years of age, a comprehensive neurodevelopmental assessment was performed. Paired comparisons were conducted between siblings with and without heart defects using a series of nonparametric tests. Results On average, the children qualified as late preterm (mean gestational age 35.4 ± 2.6 weeks). At an average age of 4.8 ± 0.1 years, children with congenital heart disease weighed less than their siblings (median weight for age z score −0.4 vs 0.1, P =.02) and had worse performance for cognition (median full-scale IQ 99 vs 109, P =.02) and fine motor skills (median Wide Range Assessment of Visual Motor Ability, Fine Motor score 94.5 vs 107.5, P <.01). Conclusions After controlling for socioeconomic status, home environment, parental intelligence, and gestational factors by using multiple-gestation births, congenital heart disease requiring surgery with cardiopulmonary bypass at ≤6 months of age is associated with lower weight, cognitive abilities and fine motor skills at 4 years of age. AD - A.H. Schultz, Division of Cardiology, Seattle Children's Hospital, 4800 Sand Point Way NE, RC.2.820, Seattle, WA, United States AU - Schultz, A. H. AU - Ittenbach, R. F. AU - Gerdes, M. AU - Jarvik, G. P. AU - Wernovsky, G. AU - Bernbaum, J. AU - Solot, C. AU - Clancy, R. R. AU - Nicolson, S. C. AU - Spray, T. L. AU - McDonald-McGinn, D. AU - Zackai, E. AU - Gaynor, J. W. DB - Embase Medline DO - 10.1016/j.jtcvs.2017.02.022 IS - 1 KW - apolipoprotein E article birth weight cardiopulmonary bypass child child health care congenital heart disease deep hypothermic circulatory arrest executive function female follow up gestational age head circumference heart single ventricle home environment human intelligence quotient major clinical study male motor performance nerve cell differentiation outcome assessment pregnancy preschool child priority journal sibling single nucleotide polymorphism social competence social status surgical mortality two ventricle heart disease LA - English M3 - Article N1 - L614895107 2017-03-23 2017-06-30 PY - 2017 SN - 1097-685X 0022-5223 SP - 273-281.e2 ST - Effect of congenital heart disease on 4-year neurodevelopment within multiple-gestation births T2 - Journal of Thoracic and Cardiovascular Surgery TI - Effect of congenital heart disease on 4-year neurodevelopment within multiple-gestation births UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L614895107 http://dx.doi.org/10.1016/j.jtcvs.2017.02.022 VL - 154 ID - 743 ER - TY - JOUR AB - OBJECTIVE: The aim of this study was to evaluate the effects of a standardized exercise program on sports enjoyment and leisure-time spending in adolescents with congenital heart disease and to know what the moderating impact of their baseline health behavior and disease knowledge is. METHODS: Included were 93 patients, aged 10 to 25, with surgical repair for tetralogy of Fallot or with a Fontan circulation for single-ventricle physiology, of 5 participating centers of pediatric cardiology in The Netherlands. They were randomly allocated, stratified for age, gender, and type of congenital heart disease to a 12-week period with either: (1) three times per week standardized exercise training or (2) care as usual (randomization ratio 2:1). At baseline and after 12 weeks, participants completed Web-based questionnaires and were interviewed by phone. OUTCOME MEASURES: Primary analyses tested changes from baseline to follow-up in sports enjoyment and leisure-time spending in the exercise group vs. control group. Secondary analyses concerned the moderating influence of baseline health behavior and disease knowledge on changes from baseline to follow-up, and comparison with normative data. RESULTS: At follow-up, the exercise group reported a decrease in passive leisure-time spending (watching television and computer usage) compared with controls. Exercise training had no effect on sports enjoyment and active leisure-time spending. Disease knowledge had a moderating effect on improvement in sports enjoyment, whereas health behavior did not. Compared with normative data, patients obtained similar leisure time scores and lower frequencies as to drinking alcohol and smoking. CONCLUSIONS: Exercise training decreased passive, but not active, leisure-time spending. It did not influence sports enjoyment. AD - Department of Child and Adolescent Psychiatry/Psychology, Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands. AN - 24313906 AU - Dulfer, K. AU - Duppen, N. AU - Blom, N. A. AU - van Dijk, A. P. AU - Helbing, W. A. AU - Verhulst, F. C. AU - Utens, E. M. DA - Sep-Oct DB - PubMed DO - 10.1111/chd.12154 DP - NLM ET - 2013/12/10 IS - 5 KW - Adolescent *Adolescent Behavior Adult Age Factors Child *Child Behavior Exercise Therapy/*psychology Female Fontan Procedure *Health Behavior *Health Knowledge, Attitudes, Practice Heart Defects, Congenital/diagnosis/physiopathology/psychology/*therapy Humans Male Netherlands Pleasure Sports/*psychology Surveys and Questionnaires Time Factors Treatment Outcome Young Adult Adolescents Aerobic Exercise Behavior Children Congenital Heart Disease Randomized Controlled Trial LA - eng N1 - 1747-0803 Dulfer, Karolijn Duppen, Nienke Blom, Nico A van Dijk, Arie P J Helbing, Wim A Verhulst, Frank C Utens, Elisabeth M W J Journal Article Multicenter Study Randomized Controlled Trial Research Support, Non-U.S. Gov't United States Congenit Heart Dis. 2014 Sep-Oct;9(5):415-23. doi: 10.1111/chd.12154. Epub 2013 Dec 9. PY - 2014 SN - 1747-079x SP - 415-23 ST - Effect of exercise training on sports enjoyment and leisure-time spending in adolescents with complex congenital heart disease: the moderating effect of health behavior and disease knowledge T2 - Congenit Heart Dis TI - Effect of exercise training on sports enjoyment and leisure-time spending in adolescents with complex congenital heart disease: the moderating effect of health behavior and disease knowledge VL - 9 ID - 234 ER - TY - JOUR AB - Objective: The aim of this study was to evaluate the effects of a standardized exercise program on sports enjoyment and leisure-time spending in adolescents with congenital heart disease and to know what the moderating impact of their baseline health behavior and disease knowledge is. Methods: Included were 93 patients, aged 10 to 25, with surgical repair for tetralogy of Fallot or with a Fontan circulation for single-ventricle physiology, of 5 participating centers of pediatric cardiology in NLD. They were randomly allocated, stratified for age, gender, and type of congenital heart disease to a 12-week period with either: (1) three times per week standardized exercise training or (2) care as usual (randomization ratio 2:1). At baseline and after 12 weeks, participants completed Web-based questionnaires and were interviewed by phone. Outcome Measures: Primary analyses tested changes from baseline to follow-up in sports enjoyment and leisure-time spending in the exercise group vs. control group. Secondary analyses concerned the moderating influence of baseline health behavior and disease knowledge on changes from baseline to follow-up, and comparison with normative data. Results: At follow-up, the exercise group reported a decrease in passive leisure-time spending (watching television and computer usage) compared with controls. Exercise training had no effect on sports enjoyment and active leisure-time spending. Disease knowledge had a moderating effect on improvement in sports enjoyment, whereas health behavior did not. Compared with normative data, patients obtained similar leisure time scores and lower frequencies as to drinking alcohol and smoking. Conclusions: Exercise training decreased passive, but not active, leisure-time spending. It did not influence sports enjoyment. AD - E.M.W.J. Utens, Erasmus MC-Sophia Children's Hospital, Department of Child and Adolescent Psychiatry/Psychology, P.O. Box 2060, Rotterdam, Netherlands AU - Dulfer, K. AU - Duppen, N. AU - Blom, N. A. AU - van Dijk, A. P. J. AU - Helbing, W. A. AU - Verhulst, F. C. AU - Utens, E. M. W. J. DB - Embase Medline DO - 10.1111/chd.12154 IS - 5 KW - adolescent adult article child computer congenital heart disease controlled study drinking behavior exercise Fallot tetralogy female Fontan procedure health behavior human knowledge base leisure major clinical study male Netherlands priority journal randomized controlled trial (topic) smoking sport television viewing LA - English M3 - Article N1 - L52912928 2013-12-16 2015-05-07 PY - 2014 SN - 1747-0803 1747-079X SP - 415-423 ST - Effect of exercise training on sports enjoyment and leisure-time spending in adolescents with complex congenital heart disease: The moderating effect of health behavior and disease Knowledge T2 - Congenital Heart Disease TI - Effect of exercise training on sports enjoyment and leisure-time spending in adolescents with complex congenital heart disease: The moderating effect of health behavior and disease Knowledge UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52912928 http://dx.doi.org/10.1111/chd.12154 VL - 9 ID - 948 ER - TY - JOUR AB - OBJECTIVE: Exercise intolerance afflicts Fontan patients with total cavopulmonary connections (TCPCs) causing a reduction in quality of life. Optimising TCPC design is hypothesised to have a beneficial effect on exercise capacity. This study investigates relationships between TCPC geometries and exercise haemodynamics and performance. METHODS: This study included 47 patients who completed metabolic exercise stress test with cardiac magnetic resonance (CMR). Phase-contrast CMR images were acquired immediately following supine lower limb exercise. Both anatomies and exercise vessel flow rates at ventilatory anaerobic threshold (VAT) were extracted. The vascular modelling toolkits were used to analyse TCPC geometries. Computational simulations were performed to quantify TCPC indexed power loss (iPL) at VAT. RESULTS: A highly significant inverse correlation was found between the TCPC diameter index, which factors in the narrowing of TCPC vessels, with iPL at VAT (r=-0.723, p<0.001) but positive correlations with exercise performance variables, including minute oxygen consumption (VO(2)) at VAT (r=0.373, p=0.01), VO(2) at peak exercise (r=0.485, p=0.001) and work at VAT/weight (r=0.368, p=0.01). iPL at VAT was negatively correlated with VO(2) at VAT (r=-0.337, p=0.02), VO(2) at peak exercise (r=-0.394, p=0.007) and work at VAT/weight (r=-0.208, p=0.17). CONCLUSIONS: Eliminating vessel narrowing in TCPCs and reducing elevated iPL at VAT could enhance exercise tolerance for patients with TCPCs. These findings could help plan surgical or catheter-based strategies to improve patients' exercise capacity. AD - School of Chemical and Biomolecular Engineering, Georgia Institute of Technology, Atlanta, Georgia, USA. Wallace H. Coulter Department of Biomedical Engineering, Georgia Institute of Technology, Emory University, Atlanta, Georgia, USA. Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA. Westat Inc, Rockville, Maryland, USA. Division of Cardiology, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois, USA. AN - 28522658 AU - Tang, E. AU - Wei, Z. A. AU - Whitehead, K. K. AU - Khiabani, R. H. AU - Restrepo, M. AU - Mirabella, L. AU - Bethel, J. AU - Paridon, S. M. AU - Marino, B. S. AU - Fogel, M. A. AU - Yoganathan, A. P. DA - Nov DB - PubMed DO - 10.1136/heartjnl-2016-310855 DP - NLM ET - 2017/05/20 IS - 22 KW - Adolescent Anaerobic Threshold *Exercise Exercise Test *Exercise Tolerance Female Fontan Procedure/*adverse effects Heart Defects, Congenital/diagnosis/physiopathology/*surgery *Hemodynamics Humans Magnetic Resonance Imaging Male Models, Cardiovascular Oxygen Consumption Patient-Specific Modeling Quality of Life Retrospective Studies Time Factors Treatment Outcome Young Adult *congenital heart disease Fontan procedure exercise hemodynamics computational fluid dynamics LA - eng N1 - 1468-201x Tang, Elaine Wei, Zhenglun Alan Whitehead, Kevin K Khiabani, Reza H Restrepo, Maria Mirabella, Lucia Bethel, James Paridon, Stephen M Marino, Bradley S Fogel, Mark A Yoganathan, Ajit P Journal Article England Heart. 2017 Nov;103(22):1806-1812. doi: 10.1136/heartjnl-2016-310855. Epub 2017 May 18. PY - 2017 SN - 1355-6037 SP - 1806-1812 ST - Effect of Fontan geometry on exercise haemodynamics and its potential implications T2 - Heart TI - Effect of Fontan geometry on exercise haemodynamics and its potential implications VL - 103 ID - 285 ER - TY - JOUR AB - BACKGROUND: Exercise capacity following Fontan surgery is often depressed. An inability to reduce pulmonary vascular resistance appropriately during exercise may contribute to this phenomenon. The aim of this study was to determine whether administration of iloprost, a selective pulmonary vasodilator, would improve exercise function after Fontan procedure. METHODS: Double-blind, randomized, placebo controlled, crossover trial. Patients performed two cardiopulmonary exercise tests (CPX) separated by <1 month. A single nebulizer treatment (iloprost or placebo) was administered before each CPX. RESULTS: 18 patients aged 12-49 (median 17) years were recruited. Mild throat discomfort developed in 10/18 patients during iloprost administration; all but 1 were able to complete treatment. No symptoms developed during placebo treatments (p<0.001). Two additional patients did not complete CPX: one with atrial flutter; another with developmental issues that precluded adequate CPX. In the 15 remaining subjects oxygen pulse (a surrogate for forward stroke volume) at peak exercise was higher following iloprost (median increase 1.2 ml/beat; p<0.001). Peak VO2 also rose (median increase 1.3 ml/kg/min; p<0.04). Nine patients had peak VO2 <30 ml/kg/min; each of these patients had higher peak VO2 following iloprost. Only 3/6 patients with peak VO2 >30 ml/kg/min had higher peak VO2 following iloprost (p<0.04). CONCLUSIONS: Iloprost improves the peak oxygen pulse and peak VO2 of patients with Fontan physiology and appears to be particularly beneficial among patients with impaired exercise function. Treatment is associated with minor side effects. These findings support the concept of pulmonary vasodilator therapy in Fontan patients with limited functional capacity. AD - Department of Cardiology, Boston Children's Hospital, Boston, MA, United States. Electronic address: jonathan.rhodes@cardio.chboston.org. AN - 23545150 AU - Rhodes, J. AU - Ubeda-Tikkanen, A. AU - Clair, M. AU - Fernandes, S. M. AU - Graham, D. A. AU - Milliren, C. E. AU - Daly, K. P. AU - Mullen, M. P. AU - Landzberg, M. J. C2 - PMC4288936 C6 - NIHMS640272 DA - Oct 3 DB - PubMed DO - 10.1016/j.ijcard.2013.03.014 DP - NLM ET - 2013/04/03 IS - 3 KW - Administration, Inhalation Adolescent Adult Child Cross-Over Studies Double-Blind Method Exercise/*physiology Exercise Test Female *Fontan Procedure Humans Iloprost/*administration & dosage Male Middle Aged Prospective Studies Vasodilator Agents/*administration & dosage Young Adult Exercise testing Fontan procedure Heart defects, congenital Vasodilation from Actelion Corporation. 2. Michael J. Landzberg: Consultant/Advisory Board, Research Steering Committee, Actelion Corporation. LA - eng N1 - 1874-1754 Rhodes, Jonathan Ubeda-Tikkanen, Ana Clair, Mathieu Fernandes, Susan M Graham, Dionne A Milliren, Carly E Daly, Kevin P Mullen, Mary P Landzberg, Michael J K12 HD052896/HD/NICHD NIH HHS/United States UL1RR025758/RR/NCRR NIH HHS/United States T32 HL07572/HL/NHLBI NIH HHS/United States T32 HL007572/HL/NHLBI NIH HHS/United States K12HD052896-06/HD/NICHD NIH HHS/United States L40 HL110356/HL/NHLBI NIH HHS/United States UL1 RR025758/RR/NCRR NIH HHS/United States Journal Article Randomized Controlled Trial Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't Int J Cardiol. 2013 Oct 3;168(3):2435-40. doi: 10.1016/j.ijcard.2013.03.014. Epub 2013 Mar 29. PY - 2013 SN - 0167-5273 (Print) 0167-5273 SP - 2435-40 ST - Effect of inhaled iloprost on the exercise function of Fontan patients: a demonstration of concept T2 - Int J Cardiol TI - Effect of inhaled iloprost on the exercise function of Fontan patients: a demonstration of concept VL - 168 ID - 348 ER - TY - JOUR AB - Introduction: Pregestational and gestational diabetes mellitus (PGDM; GDM) are significant health concerns because they are associated with an increased rate of malformations and maternal health complications. Methods: We reviewed the data that help us to understand the effects of diabetes in pregnancy. Results: Diabetic embryopathy can affect any developing organ system, but cardiovascular and neural tube defects are among the most frequent anomalies. Other complications include preeclampsia, preterm delivery, fetal growth abnormalities, and perinatal mortality. Neurodevelopmental studies on offspring of mothers with diabetes demonstrated increased rate of Gross and Fine motor abnormalities, of Attention Deficit Hyperactivity Disorder, learning difficulties, and possibly also Autism Spectrum Disorder. The mechanisms underlying the effects of maternal hyperglycemia on the developing fetus may involve increased oxidative stress, hypoxia, apoptosis, and epigenetic changes. Evidence for epigenetic changes are the following: not all progeny are affected and not to the same extent; maternal diet may influence pregnancy outcomes; and maternal diabetes alters embryonic transcriptional profiles and increases the variation between transcriptomic profiles as a result of altered gene regulation. Research in animal models has revealed that maternal hyperglycemia is a teratogen, and has helped uncover potential therapeutic targets which, when blocked, can mitigate or ameliorate the negative effects of diabetes on the developing fetus. Conclusions: Tight metabolic control, surveillance, and labor management remain the cornerstone of care for pregnant women with diabetes, but advances in the field indicate that new treatments to protect the mother and baby are not far from becoming clinical realities. AD - A. Ornoy, Laboratory of Teratology, Department of Medical Neurobiology, Hebrew University, Hadassah Medical School, Jerusalem, Israel AU - Ornoy, A. AU - Reece, E. A. AU - Pavlinkova, G. AU - Kappen, C. AU - Miller, R. K. DB - Embase Medline DO - 10.1002/bdrc.21090 IS - 1 KW - adiponectin ghrelin glucose leptin acrania anencephalus apoptosis arrhinencephaly article attention deficit disorder autism brain malformation breast feeding cardiovascular disease cataract chromatin assembly and disassembly cleft lip cleft palate cognitive defect congenital malformation craniofacial malformation embryopathy epigenetics exencephaly Fallot tetralogy fetus growth follow up frontal nasal dysplasia gene expression great vessels transposition growth disorder heart left ventricle double outlet heart right ventricle double outlet hemifacial microsomia heredity holoprosencephaly human hyperglycemia hypoplastic left heart syndrome hypoxia intrauterine growth retardation learning disorder low birth weight macrosomia maternal diabetes mellitus meningomyelocele metabolic disorder microcephaly micrognathia microophthalmia microtia mitral valve atresia motor dysfunction neural tube defect neurologic disease nutrition oxidative stress perinatal mortality postnatal growth preeclampsia pregnancy complication pregnancy outcome premature labor prevention priority journal spinal dysraphism transcription regulation tricuspid valve atresia LA - English M3 - Article N1 - L603278857 2015-03-31 2015-04-08 PY - 2015 SN - 1542-9768 1542-975X SP - 53-72 ST - Effect of maternal diabetes on the embryo, fetus, and children: Congenital anomalies, genetic and epigenetic changes and developmental outcomes T2 - Birth Defects Research Part C - Embryo Today: Reviews TI - Effect of maternal diabetes on the embryo, fetus, and children: Congenital anomalies, genetic and epigenetic changes and developmental outcomes UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L603278857 http://dx.doi.org/10.1002/bdrc.21090 VL - 105 ID - 873 ER - TY - JOUR AB - Significant noncardiac and genetic abnormalities (NC and GA) are common in neonates with congenital heart defects. We sought to examine current-era effect of those abnormalities on early and late outcomes following cardiac surgery. The method from 2002-2012, 1538 neonates underwent repair (n = 860, 56%) or palliation (n = 678, 44%) of congenital heart defects. Regression models examined the effect of NC and GA on operative results, resource utilization, and late outcomes. Neonates with NC and GA (n = 312, 20%) had higher incidence of prematurity (21% vs 13%; P < 0.001) and weight ≤2.5 kg (24% vs 12%; P < 0.001) than neonates without NC and GA (n = 1226, 80%). Although the incidence of single ventricle was comparable (34% vs 31%; P = 0.37), neonates with NC and GA underwent more palliation (52% vs 42%; P = 0.001) and subsequently had higher percentage of STAT mortality categories (Society of Thoracic Surgeons (STS) and the European Association for Cardio-thoracic Surgery (EACTS) Congenital Heart Surgery Mortality Categories) 4 and 5 procedures (78% vs 66%; P < 0.001). Adjusted logistic regression models that included disparate patient and operative variables showed that the presence of NC and GA was associated with increased unplanned reoperation (odds ratio = 1.7; 95% CI: 1.1-2.7; P = 0.03) and hospital mortality (odds ratio = 2.2; 95% CI: 1.3-3.6; P = 0.002). Adjusted linear regression models showed significant association between NC and GA and increased postoperative mechanical ventilation duration, intensive care unit, and hospital stays (P < 0.001 each). Adjusted hazard analysis showed that the presence of NC and GA was associated with diminished late survival (hazard ratio = 2.4; 95% CI: 1.9-3.1; P < 0.001) and that was evident in all subgroups of patients (P < 0.001 each). Conclusion is neonates with NC and GA commonly have associated risk factors for morbidity and mortality such as prematurity and low weight. After adjusting for those factors, the presence of NC and GA continues to have significant association with increased unplanned reoperation, hospital mortality, and resource utilization after palliative and corrective cardiac surgery. Importantly, the hazard of death in those patients continues beyond the perioperative period for at least 1 year. Our findings show that the presence of NC and GA should be emphasized during parent counseling and decision making; and underscore the need to explore strategies to improve outcomes for this high-risk population that must address perioperative care, outpatient surveillance, and management. AD - B. Alsoufi, Division of Cardiothoracic Surgery, Childrens Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Georgia, United States AU - Alsoufi, B. AU - Gillespie, S. AU - Mahle, W. T. AU - Deshpande, S. AU - Kogon, B. AU - Maher, K. AU - Kanter, K. DB - Embase Medline DO - 10.1053/j.semtcvs.2015.10.016 IS - 1 KW - acrocephalosyndactyly Alagille syndrome article artificial ventilation body weight cardiopulmonary bypass cat cry syndrome CHILD syndrome cohort analysis controlled study Dandy Walker syndrome DiGeorge syndrome Down syndrome extracorporeal oxygenation female genetic disorder Goldenhar syndrome happy puppet syndrome hazard assessment health care utilization heart single ventricle heart surgery heterotaxy syndrome Holt Oram syndrome hospital mortality hospitalization human incidence intensive care unit Jacobsen syndrome karyotype 47,XYY Klinefelter syndrome Klippel Feil syndrome logistic regression analysis major clinical study male Mowat Wilson syndrome newborn Noonan syndrome palliative therapy phenylketonuria Pierre Robin syndrome prematurity reoperation sickle cell anemia survival syndrome CHARGE trisomy 13 trisomy 18 Turner syndrome VACTREL syndrome LA - English M3 - Article N1 - L607320512 2015-12-24 2016-12-15 PY - 2016 SN - 1532-9488 1043-0679 SP - 105-114 ST - The Effect of Noncardiac and Genetic Abnormalities on Outcomes Following Neonatal Congenital Heart Surgery T2 - Seminars in Thoracic and Cardiovascular Surgery TI - The Effect of Noncardiac and Genetic Abnormalities on Outcomes Following Neonatal Congenital Heart Surgery UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L607320512 http://dx.doi.org/10.1053/j.semtcvs.2015.10.016 VL - 28 ID - 826 ER - TY - JOUR AB - Incorporation of the right ventricle (RV) into the pulmonary circulation of patients with tricuspid atresia undergoing a Fontan procedure has been advocated. The consequences of this approach on the exercise function of these patients was studied by examining the effects of progressive and steady-state bicycle exercise tests performed by 11 patients with right atrial (RA)-RV Fontan anastomoses, seven patients with RA-pulmonary artery (PA) Fontan anastomoses, 13 patients after repair of tetralogy of Fallot, and 34 normal control patients. All patients were in New York Heart Association class I. The exercise function of the patients undergoing RA-RV and RA-PA Fontan procedures were similar. The achieved peak work loads 60% and 67% of control and peak oxygen consumptions 60% and 64% of control, respectively. Both groups also displayed excessive ventilation, elevated dead space/tidal volume ratios, and depressed cardiac output during steady-state exercise. In contrast, tetralogy of Fallot patients achieved peak work loads and oxygen consumptions 83% of control and maintained normal cardiac outputs and dead space/tidal volume ratios during steady-state exercise. These results suggest that the presence of an RV within the pulmonary circulation of the Fontan patient does not result in improved exercise function. This may be due to the development of obstructive gradients across the RA-RV conduits during exercise or to the RV's negative effect on left ventricular compliance. Moreover, in contrast with the postoperative tetralogy of Fallot patient, the hypoplastic RV of tricuspid atresia may not have sufficient myocardium to assume the active pumping function required by exercise. AD - Department of Pediatrics, Columbia University College of Physicians and Surgeons, New York, NY. AN - 2344677 AU - Rhodes, J. AU - Garofano, R. P. AU - Bowman, F. O., Jr. AU - Grant, G. P. AU - Bierman, F. Z. AU - Gersony, W. M. DA - Jun DB - PubMed DO - 10.1161/01.cir.81.6.1811 DP - NLM ET - 1990/06/01 IS - 6 KW - Adolescent Adult Anastomosis, Surgical Child Child, Preschool Echocardiography, Doppler Exercise Test Follow-Up Studies Heart/*physiopathology Heart Ventricles/*abnormalities/physiopathology/*surgery Hemodynamics Humans Oxygen Consumption Physical Exertion/*physiology Pulmonary Artery/physiopathology/surgery Respiration/*physiology Tetralogy of Fallot/physiopathology/*surgery Tricuspid Valve/*abnormalities/physiopathology/*surgery LA - eng N1 - Rhodes, J Garofano, R P Bowman, F O Jr Grant, G P Bierman, F Z Gersony, W M Journal Article United States Circulation. 1990 Jun;81(6):1811-7. doi: 10.1161/01.cir.81.6.1811. PY - 1990 SN - 0009-7322 (Print) 0009-7322 SP - 1811-7 ST - Effect of right ventricular anatomy on the cardiopulmonary response to exercise. Implications for the Fontan procedure T2 - Circulation TI - Effect of right ventricular anatomy on the cardiopulmonary response to exercise. Implications for the Fontan procedure VL - 81 ID - 504 ER - TY - JOUR AB - Sildenafil has been reported to improve exercise capacity in Fontan patients, but the physiologic mechanisms behind these findings are not completely understood. The objective of this study was to study the acute effect of sildenafil on pressure-volume loop (PVL) measures of ventricular function in Fontan patients. Patients after Fontan operation who were presenting for a clinically indicated catheterization were enrolled. Patients were randomized in a double-blinded fashion to receive placebo (n = 9) or sildenafil (n = 10) 30-90 min prior to catheterization. PVLs were recorded using microconductance catheters at baseline and after infusion of dobutamine (10 mcg/kg/min). The primary outcome was change in ventriculoarterial (VA) coupling. For the entire cohort, VA coupling trended toward improvement with dobutamine (1.4 ± 0.4 to 1.8 ± 0.9, p = 0.07). End-systolic elastance showed improvement (2.6 ± 0.9 to 3.8 ± 1.4 mmHg m(2)/ml, p < 0.01) with dobutamine infusion. The cohorts had similar VA coupling at baseline (p = 0.32), but the sildenafil cohort trended toward having less of an improvement in VA coupling with dobutamine stress (p = 0.06). There were no differences between PVL measures of systolic or diastolic function between treatment groups, both at baseline and after dobutamine infusion. Patients with Fontan circulation had improved contractility and trended toward improvement in VA coupling with dobutamine stress. Acute sildenafil administration was not associated with improved PVL measurements of ventricular function in this population. These results suggest that clinical improvements seen with administration of sildenafil in Fontan patients are not associated with an acute improvement in ventricular function. CLINICAL TRIAL REGISTRATION: www.clinicaltrials.gov ; Clinicaltrials.gov Identifier: NCT01815502. AD - Division of Cardiology, Department of Pediatrics, Medical University of South Carolina, 165 Ashley Avenue, MSC 915, Charleston, SC, 29425, USA. butts@musc.edu. Division of Cardiology, Department of Pediatrics, Medical University of South Carolina, 165 Ashley Avenue, MSC 915, Charleston, SC, 29425, USA. AN - 26409473 AU - Butts, R. J. AU - Chowdhury, S. M. AU - Baker, G. H. AU - Bandisode, V. AU - Savage, A. J. AU - Atz, A. M. C2 - PMC4833409 C6 - NIHMS775037 DA - Jan DB - PubMed DO - 10.1007/s00246-015-1262-x DP - NLM ET - 2015/09/28 IS - 1 KW - Adolescent Adrenergic beta-1 Receptor Agonists/administration & dosage Adult Blood Pressure/drug effects Cardiac Catheterization Child Child, Preschool Dobutamine/administration & dosage Double-Blind Method Female *Fontan Procedure/methods Humans Male Myocardial Contraction/*drug effects Phosphodiesterase 5 Inhibitors/adverse effects/*therapeutic use Sildenafil Citrate/adverse effects/*therapeutic use Ventricular Function/*drug effects Young Adult Fontan Pressure–volume loop Sildenafil Single-ventricle heart disease LA - eng N1 - 1432-1971 Butts, Ryan J Chowdhury, Shahryar M Baker, George H Bandisode, Varsha Savage, Andrew J Atz, Andrew M T32 HL007710/HL/NHLBI NIH HHS/United States UL1 TR000062/TR/NCATS NIH HHS/United States 5T32-HL07710-19/HL/NHLBI NIH HHS/United States UL1TR000062/TR/NCATS NIH HHS/United States Journal Article Randomized Controlled Trial Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't Pediatr Cardiol. 2016 Jan;37(1):184-91. doi: 10.1007/s00246-015-1262-x. Epub 2015 Sep 26. PY - 2016 SN - 0172-0643 (Print) 0172-0643 SP - 184-91 ST - Effect of Sildenafil on Pressure-Volume Loop Measures of Ventricular Function in Fontan Patients T2 - Pediatr Cardiol TI - Effect of Sildenafil on Pressure-Volume Loop Measures of Ventricular Function in Fontan Patients VL - 37 ID - 232 ER - TY - JOUR AB - Sildenafil has been reported to improve exercise capacity in Fontan patients, but the physiologic mechanisms behind these findings are not completely understood. The objective of this study was to study the acute effect of sildenafil on pressure–volume loop (PVL) measures of ventricular function in Fontan patients. Patients after Fontan operation who were presenting for a clinically indicated catheterization were enrolled. Patients were randomized in a double-blinded fashion to receive placebo (n = 9) or sildenafil (n = 10) 30–90 min prior to catheterization. PVLs were recorded using microconductance catheters at baseline and after infusion of dobutamine (10 mcg/kg/min). The primary outcome was change in ventriculoarterial (VA) coupling. For the entire cohort, VA coupling trended toward improvement with dobutamine (1.4 ± 0.4 to 1.8 ± 0.9, p = 0.07). End-systolic elastance showed improvement (2.6 ± 0.9 to 3.8 ± 1.4 mmHg m2/ml, p < 0.01) with dobutamine infusion. The cohorts had similar VA coupling at baseline (p = 0.32), but the sildenafil cohort trended toward having less of an improvement in VA coupling with dobutamine stress (p = 0.06). There were no differences between PVL measures of systolic or diastolic function between treatment groups, both at baseline and after dobutamine infusion. Patients with Fontan circulation had improved contractility and trended toward improvement in VA coupling with dobutamine stress. Acute sildenafil administration was not associated with improved PVL measurements of ventricular function in this population. These results suggest that clinical improvements seen with administration of sildenafil in Fontan patients are not associated with an acute improvement in ventricular function. Clinical Trial Registration: www.clinicaltrials.gov; Clinicaltrials.gov Identifier: NCT01815502. AD - R.J. Butts, Division of Cardiology, Department of Pediatrics, Medical University of South Carolina, 165 Ashley Avenue, MSC 915, Charleston, SC, United States AU - Butts, R. J. AU - Chowdhury, S. M. AU - Baker, G. H. AU - Bandisode, V. AU - Savage, A. J. AU - Atz, A. M. DB - Embase Medline DO - 10.1007/s00246-015-1262-x IS - 1 KW - NCT01815502 dobutamine placebo sildenafil article catheter catheterization child clinical article controlled study double blind procedure Fontan procedure heart muscle contractility heart ventricle function human measurement outcome assessment pressure volume curve pressure volume loop randomized controlled trial LA - English M3 - Article N1 - L606537831 2015-10-26 2016-02-22 PY - 2016 SN - 1432-1971 0172-0643 SP - 184-191 ST - Effect of Sildenafil on Pressure–Volume Loop Measures of Ventricular Function in Fontan Patients T2 - Pediatric Cardiology TI - Effect of Sildenafil on Pressure–Volume Loop Measures of Ventricular Function in Fontan Patients UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L606537831 http://dx.doi.org/10.1007/s00246-015-1262-x VL - 37 ID - 831 ER - TY - JOUR AB - Better postoperative management of patients who have undergone single ventricle (SV) Fontan procedure could potentially reduce long-term complications and improve the quality of life for patients. The present study determined the effect of tadalafil on myocardial and endothelial function and exercise performance after modified Fontan operation. Patients who had undergone SV modified Fontan operation were enrolled in this clinical trial. The demographic characteristics of the patients were recorded. Before administration of tadalafil and after the trial, ventricular function (MPI, EF, FS, E/A, VTI), exercise performance, and endothelial function were evaluated for sonographic and biochemical markers (FMD, IMT, ICAM, VCAM, NO) using echocardiography, exercise testing, vascular ultrasonography, and biochemical measurements, respectively. A single dose of tadalafil of 1 mg/kg was administered daily for 6 weeks, and the functional class of the patients before and after tadalafil was determined. A total of 15 patients completed this clinical trial. Tadalafil was shown to have a significant effect on myocardial function, exercise performance, and improvement in NYHA functional class (p < 0.05) of study population. It had no significant effect on the biochemical variables and endothelial function except for IMT (p > 0.05), which decreased significantly after tadalafil administration (p < 0.05). The findings indicate that tadalafil is a safe, well-tolerated agent for the use after modified Fontan operation to improve myocardial function and exercise performance and possibly reduce long-term morbidity and mortality of patients. More conclusive results could be obtained from further study with a larger sample size and long-term follow-up. AD - Isfahan University of Medical Sciences, Isfahan, Iran. Isfahan University of Medical Sciences, Isfahan, Iran. dr.alizolfi@gmail.com. AN - 26215768 AU - Sabri, M. R. AU - Zolfi-Gol, A. AU - Ahmadi, A. AU - Haghjooy-Javanmard, S. DA - Jan DB - PubMed DO - 10.1007/s00246-015-1238-x DP - NLM ET - 2015/07/29 IS - 1 KW - Adolescent Adult Child Echocardiography Endothelium/*drug effects Exercise/*physiology Exercise Test Female *Fontan Procedure Heart Ventricles/*drug effects/physiopathology Humans Male Myocardium Phosphodiesterase 5 Inhibitors/adverse effects/*therapeutic use Postoperative Period Tadalafil/adverse effects/*therapeutic use Young Adult Endothelial function Exercise Fontan procedure Single ventricle Tadalafil LA - eng N1 - 1432-1971 Sabri, Mohammad-Reza Zolfi-Gol, Ali Ahmadi, Alireza Haghjooy-Javanmard, Shaghayegh Clinical Trial Journal Article United States Pediatr Cardiol. 2016 Jan;37(1):55-61. doi: 10.1007/s00246-015-1238-x. Epub 2015 Jul 28. PY - 2016 SN - 0172-0643 SP - 55-61 ST - Effect of Tadalafil on Myocardial and Endothelial Function and Exercise Performance After Modified Fontan Operation T2 - Pediatr Cardiol TI - Effect of Tadalafil on Myocardial and Endothelial Function and Exercise Performance After Modified Fontan Operation VL - 37 ID - 147 ER - TY - JOUR AD - Dept. of Child and Adolescent Psychiatry/Psychology, Erasmus Medical Center - Sophia Children's Hospital, Rotterdam, The Netherlands. Dept. of Paediatrics, Division of Cardiology, Erasmus Medical Center - Sophia Children's Hospital, Rotterdam, The Netherlands. Dept. of Paediatric Cardiology, Leiden University Medical Center, Leiden, The Netherlands. Dept. of Cardiology, Thoraxcenter, Erasmus Medical Center, Rotterdam, The Netherlands. Dept. of Child and Adolescent Psychiatry/Psychology, Erasmus Medical Center - Sophia Children's Hospital, Rotterdam, The Netherlands. Electronic address: e.utens@erasmusmc.nl. AN - 24491873 AU - Dulfer, K. AU - Duppen, N. AU - Blom, N. A. AU - Van Domburg, R. T. AU - Helbing, W. A. AU - Verhulst, F. C. AU - Utens, E. M. DA - Apr 1 DB - PubMed DO - 10.1016/j.ijcard.2013.12.244 DP - NLM ET - 2014/02/05 IS - 3 KW - Adolescent Adolescent Behavior/*physiology Child Child Behavior/*physiology *Emotions Exercise/*physiology Exercise Therapy/*methods Female Follow-Up Studies Hemodynamics/*physiology Humans Male Retrospective Studies Tetralogy of Fallot/physiopathology/psychology/*therapy Treatment Outcome Adolescents Aerobic exercise Behavioral problems Congenital heart defects Emotional problems LA - eng N1 - 1874-1754 Dulfer, K Duppen, N Blom, N A Van Domburg, R T Helbing, W A Verhulst, F C Utens, E M W J Letter Randomized Controlled Trial Netherlands Int J Cardiol. 2014 Apr 1;172(3):e425-7. doi: 10.1016/j.ijcard.2013.12.244. Epub 2014 Jan 11. PY - 2014 SN - 0167-5273 SP - e425-7 ST - Effects of exercise training on behavioral and emotional problems in adolescents with tetralogy of Fallot or a Fontan circulation: a randomized controlled trial T2 - Int J Cardiol TI - Effects of exercise training on behavioral and emotional problems in adolescents with tetralogy of Fallot or a Fontan circulation: a randomized controlled trial VL - 172 ID - 459 ER - TY - JOUR AD - Erasmus MC, Sophia Children's Hospital, Department of Child and Adolescent Psychiatry/Psychology, P.O. Box 2060, 3000 CB Rotterdam, Netherlands Dept. of Paediatrics, Erasmus Medical Center, Sophia Children's Hospital, Rotterdam, Netherlands Dept. of Cardiology, Thoraxcenter, Erasmus Medical Center, Rotterdam, Netherlands Dept. of Paediatric Cardiology, Leiden University Medical Center, Leiden, Netherlands AU - Dulfer, K. AU - Duppen, N. AU - Blom, N. A. AU - Van Domburg, R. T. AU - Helbing, W. A. AU - Verhulst, F. C. AU - Utens, E. M. W. J. DB - Scopus DO - 10.1016/j.ijcard.2013.12.244 IS - 3 KW - Adolescents Aerobic exercise Behavioral problems Congenital heart defects Emotional problems M3 - Article N1 - Cited By :8 Export Date: 15 June 2020 PY - 2014 SP - e425-e427 ST - Effects of exercise training on behavioral and emotional problems in adolescents with tetralogy of Fallot or a Fontan circulation: A randomized controlled trial T2 - International Journal of Cardiology TI - Effects of exercise training on behavioral and emotional problems in adolescents with tetralogy of Fallot or a Fontan circulation: A randomized controlled trial UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84900641165&doi=10.1016%2fj.ijcard.2013.12.244&partnerID=40&md5=346e1005b7929e76b7cab537ddb46b2e VL - 172 ID - 1993 ER - TY - JOUR AB - The present investigation was aimed at assessing the effect of home-based training on cardiorespiratory responses in children surgically corrected for congenital heart impairment using dyspnea threshold (DT) as training intensity. A group of 23 children aged 15 ± 1.4 years who had undergone surgical correction for congenital heart disease (CHD group) and 12 healthy children used as a control group performed an exercise protocol during which aerobic capacity, ventilatory threshold (VT), and DT were assessed. Afterwards, the CHD group was divided into two subgroups: trained and untrained subjects. Training sessions over a period of 2 months were performed at an intensity corresponding to DT by the CHD trained group. Dyspnea was scored according to a visual analog scale during the warm up, at each exercise stage, and during the recovery period. DT was located on the oxygen uptake/dyspnea curve when a sudden increase in the dyspnea score occured, while VT was defined according to the methodology previously employed by Beaver and colleagues. The results showed that children who had undergone surgery for CHD had reduced aerobic capacity. No significant difference was observed between VT and DT in children corrected for CHD. These two thresholds were highly related. Home-based training at DT did not strongly improve aerobic capacity nor did it influence the relationship between VT and DT. Further studies should be conducted to confirm these preliminary findings, particularly when aerobic capacity is improved by training. © 2008 Copyright the Authors Journal Compilation © 2008 Wiley Periodicals, Inc. AD - S. Ahmaidi, Lab. de Recherches EA-3300 Adaptations Physiologiques a l'Exercice/Readaptation a l'Effort, Faculté des Sciences du Sport, Université de Picardie Jules Verne, F-80025 Amiens Cedex, France AU - Amiard, V. AU - Jullien, H. AU - Nassif, D. AU - Bach, V. AU - Maingourd, Y. AU - Ahmaidi, S. DB - Embase Medline DO - 10.1111/j.1747-0803.2008.00191.x IS - 3 KW - anticoagulant agent carbon dioxide cardiotonic agent dipeptidyl carboxypeptidase inhibitor diuretic agent immunosuppressive agent oxygen adolescent aerobic capacity article bicycle ergometry cardiopulmonary exercise test cardiopulmonary function cardiovascular surgery clinical article congenital heart disease controlled study dyspnea exercise Fallot tetralogy female functional training great vessels transposition heart atrium septum defect heart failure heart single ventricle home care human lung gas exchange lung minute volume lung ventilation male medical assessment methodology oxygen consumption priority journal pulmonary valve atresia pulmonary valve stenosis scoring system task performance treatment outcome visual analog scale LA - English M3 - Article N1 - L351843831 2008-06-27 PY - 2008 SN - 1747-079X 1747-0803 SP - 191-199 ST - Effects of home-based training at dyspnea threshold in children surgically repaired for congenital heart disease T2 - Congenital Heart Disease TI - Effects of home-based training at dyspnea threshold in children surgically repaired for congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L351843831 http://dx.doi.org/10.1111/j.1747-0803.2008.00191.x VL - 3 ID - 1212 ER - TY - JOUR AB - Infants with hypoplastic left heart syndrome often experience difficulty with oral feeding, which contributes to growth failure, morbidity, and mortality. In response to feeding difficulty, clinicians often change the bottle nipple, and thus milk flow rate. Slow-flow nipples have been found to reduce the stress of feeding in other fragile infants, but no research has evaluated the responses of infants with hypoplastic left heart syndrome to alterations in milk flow. The purpose of this study was to evaluate the physiological and behavioural responses of an infant with hypoplastic left heart syndrome to bottle feeding with either a slow-flow (Dr. Brown's Preemie) or a standard-flow (Dr. Brown's Level 2) nipple. A single infant was studied for three feedings: two slow-flow and one standard-flow. Oral feeding, whether with a slow-flow or a standard-flow nipple, was distressing for this infant. During slow-flow feeding, she experienced more coughing events, whereas during standard-flow she experienced more gagging. Disengagement and compelling disorganisation were most common during feeding 3, that is slow-flow, which occurred 2 days after surgical placement of a gastrostomy tube. Clinically significant changes in heart rate, oxygen saturation, and respiratory rate were seen during all feedings. Heart rate was higher during standard-flow and respiratory rate was higher during slow-flow. Further research is needed to examine the responses of infants with hypoplastic left heart syndrome to oral feeding and to identify strategies that will support these fragile infants as they learn to feed. Future research should evaluate an even slower-flow nipple along with additional supportive feeding strategies. AD - B.F. Pados, School of Nursing, University of North Carolina at Chapel Hill, Carrington Hall, CB#7460, Chapel Hill, NC, United States AU - Pados, B. F. AU - Thoyre, S. M. AU - Estrem, H. H. AU - Park, J. AU - Knafl, G. J. AU - Nix, B. DB - Embase Medline DO - 10.1017/S1047951116000251 IS - 1 KW - article bottle feeding breathing rate case report child behavior child nutrition conceptual framework coughing disorientation echocardiography feeding behavior female gastrointestinal symptom heart rate human hypoplastic left heart syndrome infant oxygen saturation stomach tube LA - English M3 - Article N1 - L609137299 2016-03-28 2017-04-04 PY - 2017 SN - 1467-1107 1047-9511 SP - 139-153 ST - Effects of milk flow on the physiological and behavioural responses to feeding in an infant with hypoplastic left heart syndrome T2 - Cardiology in the Young TI - Effects of milk flow on the physiological and behavioural responses to feeding in an infant with hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L609137299 http://dx.doi.org/10.1017/S1047951116000251 VL - 27 ID - 775 ER - TY - JOUR AB - Baffle fenestration is associated with a significantly better outcome in standard and high-risk patients undergoing completion of Fontan. We report the effects of subsequent transcatheter closure of fenestration on exercise capacity and oxygen saturation. Sixteen patients with a mean age of 10.3 years underwent Amplatzer septal occluder (ASO) device transcatheter closure of Fontan fenestration. All had a fenestrated Fontan operation 6 month to 8 years prior to the procedure. A stress test was performed before and after device closure of fenestration in 14 patients (2 patients did not tolerate stress test before the procedure). The fenestrations in all patients were successfully occluded with the use of the Amplatzer device occluder. No complications occurred during or after the procedure. O2 saturation increased from a mean 85.1 ± 7.89% to 94.5 ± 3.63% (p < 0.01) at rest and from 66.2 ± 12.86% to 87.2 ± 8.64% (p < 0.01) following exercise. Exercise duration has also increased from 8.22 ± 2.74 min to 10.29 ± 1.91 min (p < 0.05). Transcatheter closure of Fontan fenestration increases the duration of exercise capacity and increases O2 saturation at rest and after exercise. © 2007 Springer Science+Business Media, LLC. AD - T. S. Momenah, Prince Sultan Cardiac Center, P.O. Box 7897, Riyadh 1159, Saudi Arabia AU - Momenah, T. S. AU - Eltayb, H. AU - Oakley, R. E. AU - Qethamy, H. A. AU - Faraidi, Y. A. DB - Embase Medline DO - 10.1007/s00246-007-9154-3 IS - 3 KW - adolescent article artificial embolization child devices exercise exercise test exercise tolerance fenestration Fontan procedure heart catheterization heart right atrium human oxygen saturation surgical technique LA - English M3 - Article N1 - L50011790 2008-06-25 PY - 2008 SN - 0172-0643 SP - 585-588 ST - Effects of transcatheter closure of fontan fenestration on exercise tolerance T2 - Pediatric Cardiology TI - Effects of transcatheter closure of fontan fenestration on exercise tolerance UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50011790 http://dx.doi.org/10.1007/s00246-007-9154-3 VL - 29 ID - 1213 ER - TY - JOUR AB - Baffle fenestration is associated with a significantly better outcome in standard and high-risk patients undergoing completion of Fontan. We report the effects of subsequent transcatheter closure of fenestration on exercise capacity and oxygen saturation. Sixteen patients with a mean age of 10.3 years underwent Amplatzer septal occluder (ASO) device transcatheter closure of Fontan fenestration. All had a fenestrated Fontan operation 6 month to 8 years prior to the procedure. A stress test was performed before and after device closure of fenestration in 14 patients (2 patients did not tolerate stress test before the procedure). The fenestrations in all patients were successfully occluded with the use of the Amplatzer device occluder. No complications occurred during or after the procedure. O2 saturation increased from a mean 85.1 +/- 7.89% to 94.5 +/- 3.63% (p < 0.01) at rest and from 66.2 +/- 12.86% to 87.2 +/- 8.64% (p < 0.01) following exercise. Exercise duration has also increased from 8.22 +/- 2.74 min to 10.29 +/- 1.91 min (p < 0.05). Transcatheter closure of Fontan fenestration increases the duration of exercise capacity and increases O2 saturation at rest and after exercise. AD - Prince Sultan Cardiac Center, P.O. Box 7897, Riyadh, 1159, Kingdom of Saudi Arabia, kidecho@yahoo.com AN - 18071783 AU - Momenah, T. S. AU - Eltayb, H. AU - Oakley, R. E. AU - Qethamy, H. A. AU - Faraidi, Y. A. DA - May DB - PubMed DO - 10.1007/s00246-007-9154-3 DP - NLM ET - 2007/12/12 IS - 3 KW - Adolescent *Balloon Occlusion Cardiac Catheterization Child *Exercise Tolerance *Fontan Procedure Heart Septal Defects/surgery Humans LA - eng N1 - Momenah, Tarek S Eltayb, Haifa Oakley, Reida El Qethamy, Howeida Al Faraidi, Yahya Al Journal Article United States Pediatr Cardiol. 2008 May;29(3):585-8. doi: 10.1007/s00246-007-9154-3. Epub 2007 Dec 11. PY - 2008 SN - 0172-0643 (Print) 0172-0643 SP - 585-8 ST - Effects of transcatheter closure of Fontan fenestration on exercise tolerance. kidecho@yahoo.com T2 - Pediatr Cardiol TI - Effects of transcatheter closure of Fontan fenestration on exercise tolerance. kidecho@yahoo.com VL - 29 ID - 329 ER - TY - JOUR AB - AIMS: In univentricular hearts, selective lung vasodilators such as phosphodiesterase type 5 (PDE5) inhibitors would decrease pulmonary resistance and improve exercise tolerance. However, the level of evidence for the use of PDE5 inhibitors in patients with a single ventricle (SV) remains limited. We present the SV-INHIBITION study rationale, design, and methods. METHODS AND RESULTS: The SV-INHIBITION trial is a nationwide multicentre, randomized, double blind, placebo-controlled, Phase III study, aiming to evaluate the efficacy of sildenafil on the ventilatory efficiency during exercise, in teenagers and adult patients (>15 years old) with an SV. Patients with a mean pulmonary arterial pressure >15 mmHg and a trans-pulmonary gradient >5 mmHg, measured by cardiac catheterization, will be eligible. The primary outcome is the variation of the VE/VCO(2) slope, measured by a cardiopulmonary exercise test, between baseline and 6 months of treatment. A total of 50 patients are required to observe a decrease of 5 ± 5 points in the VE/VCO(2) slope, with a power of 90% and an alpha risk of 5%. The secondary outcomes are clinical outcomes, oxygen saturation, 6 min walk test, SV function, NT-proBNP, peak VO(2) , stroke volume, mean pulmonary arterial pressure, trans-pulmonary gradient, SF36 quality of life score, safety, and acceptability. CONCLUSIONS: The SV-INHIBITION study aims to answer the question whether PDE5 inhibitors should be prescribed in patients with an SV. This trial has been built focusing on the three levels of research defined by the World Health Organization: disability (exercise tolerance), deficit (SV function), and handicap (quality of life). AD - Paediatric and Congenital Cardiology Department, M3C Regional Reference CHD Centre, CHU Montpellier, Montpellier, France. PhyMedExp, CNRS, INSERM, University of Montpellier, CHU Montpellier, Montpellier, France. Department of Epidemiology and Biostatistics, CHU Montpellier, Montpellier, France. Paediatric Cardiology Department, AP-HP, Necker-Enfants malades, M3C National Reference Centre, Paris Descartes University, Sorbonne Paris Cite, Paris, France. Adult Congenital Heart Disease Unit, Department of Cardiology, M3C National Reference Centre, European Hospital Georges Pompidou, Paris, France. Paediatric and Congenital Medico-Surgical Cardiology Department, M3C Regional Reference Centre, AP-HM, La Timone University Hospital, Marseille, France. Laboratory of Medical Genetics, INSERM UMR 1251, Aix-Marseille University, Marseille, France. Paediatric and Adult Congenital Heart Disease Department, Hôpital Cardiologique du Haut-Lévêque, M3C National Reference Centre, CHU de Bordeaux, Bordeaux, France. Pediatric Cardiology Service, Royal Brompton and Harefield Hospital Trust, London, UK. Department of Pulmonology, PH National Reference Centre, APHP, Kremlin-Bicetre, Paris, France. Paris-Sud Faculty of Medicine, INSERM U999, Marie-Lannelongue Hospital, M3C National Reference Centre, Paris-Sud University, Paris-Saclay, Le Plessis-Robinson, Paris, France. Paediatric and Congenital Cardiology Department, M3C Regional Reference CHD Centre, CHU Nantes, Nantes, France. Paediatric and Congenital Cardiology Department, M3C Regional Reference CHD Centre, CHU Toulouse, Toulouse, France. AN - 32147955 AU - Amedro, P. AU - Gavotto, A. AU - Abassi, H. AU - Picot, M. C. AU - Matecki, S. AU - Malekzadeh-Milani, S. AU - Levy, M. AU - Ladouceur, M. AU - Ovaert, C. AU - Aldebert, P. AU - Thambo, J. B. AU - Fraisse, A. AU - Humbert, M. AU - Cohen, S. AU - Baruteau, A. E. AU - Karsenty, C. AU - Bonnet, D. AU - Hascoet, S. C2 - PMC7160497 DA - Apr DB - PubMed DO - 10.1002/ehf2.12630 DP - NLM ET - 2020/03/10 IS - 2 KW - Congenital heart defect Exercise capacity Pulmonary hypertension Pulmonary vasodilator Sildenafil Single ventricle LA - eng N1 - 2055-5822 Amedro, Pascal Gavotto, Arthur Abassi, Hamouda Picot, Marie-Christine Matecki, Stefan Malekzadeh-Milani, Sophie Levy, Marilyne Ladouceur, Magalie Ovaert, Caroline Aldebert, Philippe Thambo, Jean-Benoit Fraisse, Alain Humbert, Marc Cohen, Sarah Baruteau, Alban-Elouen Karsenty, Clement Bonnet, Damien Hascoet, Sebastien SV-INHIBITION study investigators DGOS-PHRC-N-2017/French Department of Health/ Journal Article ESC Heart Fail. 2020 Apr;7(2):747-756. doi: 10.1002/ehf2.12630. Epub 2020 Mar 9. PY - 2020 SN - 2055-5822 SP - 747-756 ST - Efficacy of phosphodiesterase type 5 inhibitors in univentricular congenital heart disease: the SV-INHIBITION study design T2 - ESC Heart Fail TI - Efficacy of phosphodiesterase type 5 inhibitors in univentricular congenital heart disease: the SV-INHIBITION study design VL - 7 ID - 396 ER - TY - JOUR AB - Aims: In univentricular hearts, selective lung vasodilators such as phosphodiesterase type 5 (PDE5) inhibitors would decrease pulmonary resistance and improve exercise tolerance. However, the level of evidence for the use of PDE5 inhibitors in patients with a single ventricle (SV) remains limited. We present the SV-INHIBITION study rationale, design, and methods. Methods and results: The SV-INHIBITION trial is a nationwide multicentre, randomized, double blind, placebo-controlled, Phase III study, aiming to evaluate the efficacy of sildenafil on the ventilatory efficiency during exercise, in teenagers and adult patients (>15 years old) with an SV. Patients with a mean pulmonary arterial pressure >15 mmHg and a trans-pulmonary gradient >5 mmHg, measured by cardiac catheterization, will be eligible. The primary outcome is the variation of the VE/VCO2 slope, measured by a cardiopulmonary exercise test, between baseline and 6 months of treatment. A total of 50 patients are required to observe a decrease of 5 ± 5 points in the VE/VCO2 slope, with a power of 90% and an alpha risk of 5%. The secondary outcomes are clinical outcomes, oxygen saturation, 6 min walk test, SV function, NT-proBNP, peak VO2, stroke volume, mean pulmonary arterial pressure, trans-pulmonary gradient, SF36 quality of life score, safety, and acceptability. Conclusions: The SV-INHIBITION study aims to answer the question whether PDE5 inhibitors should be prescribed in patients with an SV. This trial has been built focusing on the three levels of research defined by the World Health Organization: disability (exercise tolerance), deficit (SV function), and handicap (quality of life). © 2020 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology AD - Paediatric and Congenital Cardiology Department, M3C Regional Reference CHD Centre, CHU Montpellier, Montpellier, France PhyMedExp, CNRS, INSERM, University of Montpellier, CHU Montpellier, Montpellier, France Department of Epidemiology and Biostatistics, CHU Montpellier, Montpellier, France Paediatric Cardiology Department, AP-HP, Necker-Enfants malades, M3C National Reference Centre, Paris Descartes University, Sorbonne Paris Cite, Paris, France Adult Congenital Heart Disease Unit, Department of Cardiology, M3C National Reference Centre, European Hospital Georges Pompidou, Paris, France Paediatric and Congenital Medico-Surgical Cardiology Department, M3C Regional Reference Centre, AP-HM, La Timone University Hospital, Marseille, France Laboratory of Medical Genetics, INSERM UMR 1251, Aix-Marseille University, Marseille, France Paediatric and Adult Congenital Heart Disease Department, Hôpital Cardiologique du Haut-Lévêque, M3C National Reference Centre, CHU de Bordeaux, Bordeaux, France Pediatric Cardiology Service, Royal Brompton and Harefield Hospital Trust, London, United Kingdom Department of Pulmonology, PH National Reference Centre, APHP, Kremlin-Bicetre, Paris, France Paris-Sud Faculty of Medicine, INSERM U999, Marie-Lannelongue Hospital, M3C National Reference Centre, Paris-Sud University, Paris-Saclay, Le Plessis-Robinson, Paris, France Paediatric and Congenital Cardiology Department, M3C Regional Reference CHD Centre, CHU Nantes, Nantes, France Paediatric and Congenital Cardiology Department, M3C Regional Reference CHD Centre, CHU Toulouse, Toulouse, France AU - Amedro, P. AU - Gavotto, A. AU - Abassi, H. AU - Picot, M. C. AU - Matecki, S. AU - Malekzadeh-Milani, S. AU - Levy, M. AU - Ladouceur, M. AU - Ovaert, C. AU - Aldebert, P. AU - Thambo, J. B. AU - Fraisse, A. AU - Humbert, M. AU - Cohen, S. AU - Baruteau, A. E. AU - Karsenty, C. AU - Bonnet, D. AU - Hascoet, S. AU - investigators, Sv-Inhibition study DB - Scopus DO - 10.1002/ehf2.12630 IS - 2 KW - Congenital heart defect Exercise capacity Pulmonary hypertension Pulmonary vasodilator Sildenafil Single ventricle M3 - Article N1 - Export Date: 15 June 2020 PY - 2020 SP - 747-756 ST - Efficacy of phosphodiesterase type 5 inhibitors in univentricular congenital heart disease: the SV-INHIBITION study design T2 - ESC Heart Failure TI - Efficacy of phosphodiesterase type 5 inhibitors in univentricular congenital heart disease: the SV-INHIBITION study design UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85081559255&doi=10.1002%2fehf2.12630&partnerID=40&md5=27d3aa7e5fa84f117e1f6d2408b8fe38 VL - 7 ID - 1704 ER - TY - JOUR AB - The exercise tolerance of Fontan patients is poorer than that of healthy people. Some previous studies reported that exercise tolerance can be improved in this population by cardiac rehabilitation. This study aimed to determine the factors associated with peak oxygen uptake (VO2) and to subsequently clarify the correlation between participation in sports club activities and variables related to exercise tolerance. Cardiopulmonary exercise tests were performed by 115 Fontan patients aged between 6 and 20 years. The patients completed questionnaires on their daily physical activities including participation in extracurricular sports clubs in middle (junior high school) and high school. Peak VO2 had a significant negative correlation with age and a positive correlation with hemoglobin, stroke volume, and resting tidal volume in the entire study group. Additionally, the sports club participants who were middle and high school students had significantly higher peak VO2 and resting tidal volume. Exercise habits including participation in sports club activities may promote exercise tolerance by improving respiratory function in Fontan adolescents. AD - Y. Kodama, Department of Pediatric Cardiology, Fukuoka Children’s Hospital, 5-1-1 Kashiiteriha, Higashi-ku, Fukuoka, Japan AU - Kodama, Y. AU - Koga, K. AU - Kuraoka, A. AU - Ishikawa, Y. AU - Nakamura, M. AU - Sagawa, K. AU - Ishikawa, S. DB - Embase Medline DO - 10.1007/s00246-018-1900-1 IS - 7 KW - hemoglobin adolescent adult age article cardiopulmonary exercise test child controlled study correlation analysis exercise tolerance female Fontan procedure heart stroke volume hemoglobin blood level high school student human major clinical study male middle school student oxygen consumption patient participation physical activity preschool child questionnaire respiratory function school child sport tidal volume young adult LA - English M3 - Article N1 - L622242371 2018-05-28 2018-12-27 PY - 2018 SN - 1432-1971 0172-0643 SP - 1339-1345 ST - Efficacy of Sports Club Activities on Exercise Tolerance Among Japanese Middle and High School Children and Adolescents After Fontan Procedure T2 - Pediatric Cardiology TI - Efficacy of Sports Club Activities on Exercise Tolerance Among Japanese Middle and High School Children and Adolescents After Fontan Procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L622242371 http://dx.doi.org/10.1007/s00246-018-1900-1 VL - 39 ID - 661 ER - TY - JOUR AB - Objective: The utility of extracorporeal membrane oxygenation (ECMO) as an elective support modality for high-risk cardiac procedures is extensively described in adults, but its use in children is limited to isolated reports. The objective of this study was to analyze the outcomes of patients who underwent elective cannulation to ECMO for this purpose. Design: Single-center, retrospective chart review. Setting: Free-standing pediatric tertiary care center. Participants: Patients who underwent elective cannulation to ECMO for cardiorespiratory support during a high-risk cardiac catheterization procedure. Interventions: Elective ECMO cannulation for high-risk percutaneous cardiac interventions or electrophysiology procedures. Measurements and Main Results: Survival to discharge was 71.4% compared with 30% for patients who required extracorporeal cardiopulmonary resuscitation in the cardiac catheterization laboratory. The mean duration of cannulation was 137.43 hours (range 27-615 h, median 55 h). There were no major neurologic sequelae, but ECMO circuit thrombosis (57%) was relatively common. Conclusion: The use of elective ECMO support for high-risk pediatric cardiac catheterizations can be accomplished safely and may allow for an improved rate of survival with lower rates of severe adverse events compared with extracorporeal cardiopulmonary resuscitation as rescue therapy. © 2019 Elsevier Inc. AD - Department of Anesthesiology, Critical Care Medicine and Pain Medicine, Division of Cardiac Anesthesia, Boston Children's Hospital, Boston, MA, United States Department of Cardiology, Boston Children's Hospital, Boston, MA, United States AU - Zaleski, K. L. AU - Scholl, R. L. AU - Thiagarajan, R. R. AU - Porras, D. AU - Mah, D. AU - DiNardo, J. A. AU - Nasr, V. G. DB - Scopus DO - 10.1053/j.jvca.2019.01.008 IS - 7 KW - cardiac catheterization cardiopulmonary resuscitation extracorporeal membrane oxygenation pediatric retrospective study treatment outcome M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2019 SP - 1932-1938 ST - Elective Extracorporeal Membrane Oxygenation Support for High-Risk Pediatric Cardiac Catheterization T2 - Journal of Cardiothoracic and Vascular Anesthesia TI - Elective Extracorporeal Membrane Oxygenation Support for High-Risk Pediatric Cardiac Catheterization UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85062993252&doi=10.1053%2fj.jvca.2019.01.008&partnerID=40&md5=1dc357869615dd619f4bcd06b7ac4de4 VL - 33 ID - 1743 ER - TY - JOUR AB - Previous studies noted that Fontan patients have electrocardiographic ST depressions during exercise tests and on 24-hour ambulatory electrocardiographic (ECG) monitoring. This study investigated whether the ST depressions were correlated to clinical function (NYHA Functional Classification). Forty-five children in two groups were examined--15 patients with Fontan circulation and 30 with a structurally normal heart, matched for sex, height, and weight. Clinical and echocardiographic examinations were performed. The clinical state was defined as NYHA functional classes I-IV. All 45 children underwent 24-hour ECG and an analysis of ST changes was performed, with ST segment depression > 0.20 mV at ST 60 regarded as significant. Twelve of 15 patients had significant 24-hour ECG ST depressions; in 9 of 12 patients these occurred on more than one occasion. Comparing 24-hour ECG recordings from patients in NYHA I with patients in NYHA II or III showed a significant difference in depth (p = 0.0023), duration (p = 0.0016), and number of ST depressions (p = 0.0001). None of the matched healthy children showed ST depressions. Repeated ST analysis on 24-hour ECG shows that the ST depressions seem to be correlated to functional status and this may be a useful tool in clinical praxis. AD - Section of Pediatric Cardiology, Department of Clinical Sciences, Umeå Hospital, S-901 85 Umeå, Sweden. AN - 14627315 AU - Rydberg, A. AU - Rask, P. AU - Teien, D. E. AU - Hörnsten, R. DA - Sep-Oct DB - PubMed DO - 10.1007/s00246-002-0374-2 DP - NLM ET - 2003/11/25 IS - 5 KW - Adolescent Case-Control Studies Child Child, Preschool *Coronary Circulation *Electrocardiography, Ambulatory *Fontan Procedure Heart/*physiology Humans LA - eng N1 - Rydberg, A Rask, P Teien, D E Hörnsten, R Journal Article United States Pediatr Cardiol. 2003 Sep-Oct;24(5):468-72. doi: 10.1007/s00246-002-0374-2. PY - 2003 SN - 0172-0643 (Print) 0172-0643 SP - 468-72 ST - Electrocardiographic ST segment depression and clinical function in children with Fontan circulation T2 - Pediatr Cardiol TI - Electrocardiographic ST segment depression and clinical function in children with Fontan circulation VL - 24 ID - 356 ER - TY - JOUR AB - Despite successful operation, many patients palliated with a Fontan-type procedure continue to show effort limitation. We previously observed that these children showed electrocardiographic ST depression during exercise tests. The purpose of the study was to investigate whether electrocardiographic ST depression is a common finding in children with Fontan circulation. Forty-two children in two groups were examined: group A consisted of 14 patients who had all undergone a modified Fontan procedure, and group B consisted of 28 children with a structurally normal heart, matched for length, weight and gender to group A. Complete echocardiographic examinations were performed in all patients and controls. All 14 patients and all 28 healthy children underwent standard 24 h ambulatory electrocardiographic monitoring. All the recordings were analysed by a PC-based Holter system where an analysis of ST changes was performed. Seven patients and 14 matched healthy children were exercised on bicycle ergometers. Four patients and eight matched healthy children underwent exercise testing by walking/running a treadmill. Ten of 13 patients analysed had significant ST depressions on ambulatory electrocardiogram (> 0.20 mV). Three of the 10 patients with ST depression were on digoxin. Three patients showed depressions of the ST segment in the electrocardiogram during exercise, with a maximal depression of 0.20-0.35 mV. None of the 28 matched healthy children showed electrocardiographic ST depression on the ambulatory 24 h ECG. These findings indicate that ST depression in daily activity is a common finding in children with Fontan circulation. AD - A. Rydberg, Department of Paediatrics, Umea University, S-90185 Umea, Sweden AU - Rydberg, A. DB - Embase Medline DO - 10.1046/j.1365-2281.2000.00227.x IS - 1 KW - digoxin adolescent ambulatory monitoring article bicycle child clinical article congenital heart malformation controlled study electrocardiogram ergometer exercise test female Fontan procedure Holter monitoring human male normal human priority journal ST segment ST segment depression treadmill LA - English M3 - Article N1 - L30064041 2000-02-07 PY - 2000 SN - 0144-5979 SP - 69-78 ST - Electrocardiographic ST-segment depression in children with Fontan circulation T2 - Clinical Physiology TI - Electrocardiographic ST-segment depression in children with Fontan circulation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L30064041 http://dx.doi.org/10.1046/j.1365-2281.2000.00227.x VL - 20 ID - 1338 ER - TY - JOUR AB - Despite successful operation, many patients palliated with a Fontan-type procedure continue to show effort limitation. We previously observed that these children showed electrocardiographic ST depression during exercise tests. The purpose of the study was to investigate whether electrocardiographic ST depression is a common finding in children with Fontan circulation. Forty-two children in two groups were examined: group A consisted of 14 patients who had all undergone a modified Fontan procedure, and group B consisted of 28 children with a structurally normal heart, matched for length, weight and gender to group A. Complete echocardiographic examinations were performed in all patients and controls. All 14 patients and all 28 healthy children underwent standard 24 h ambulatory electrocardiographic monitoring. All the recordings were analysed by a PC-based Holter system where an analysis of ST changes was performed. Seven patients and 14 matched healthy children were exercised on bicycle ergometers. Four patients and eight matched healthy children underwent exercise testing by walking/running a treadmill. Ten of 13 patients analysed had significant ST depressions on ambulatory electrocardiogram (>0.20 mV). Three of the 10 patients with ST depression were on digoxin. Three patients showed depressions of the ST segment in the electrocardiogram during exercise, with a maximal depression of 0.20-0.35 mV. None of the 28 matched healthy children showed electrocardiographic ST depression on the ambulatory 24 h ECG. These findings indicate that ST depression in daily activity is a common finding in children with Fontan circulation. AD - Department of Paediatrics, Division of Paediatric Cardiology, University Hospital of Umeâ, Umeâ, Sweden. AN - 10651795 AU - Rydberg, A. AU - Teien, D. E. AU - Rask, P. AU - Hörnsten, R. DA - Jan DB - PubMed DO - 10.1046/j.1365-2281.2000.00227.x DP - NLM ET - 2000/01/29 IS - 1 KW - Adolescent Arteriovenous Fistula/diagnosis Cardiac Output Cardiotonic Agents/therapeutic use Child Child, Preschool Coronary Circulation Digoxin/therapeutic use Echocardiography *Electrocardiography, Ambulatory Exercise Test Female *Fontan Procedure Humans Male Oxygen Consumption Pulmonary Circulation Tricuspid Atresia/*diagnosis/drug therapy/surgery Ventricular Function, Left LA - eng N1 - Rydberg, A Teien, D E Rask, P Hörnsten, R Journal Article England Clin Physiol. 2000 Jan;20(1):69-78. doi: 10.1046/j.1365-2281.2000.00227.x. PY - 2000 SN - 0144-5979 (Print) 0144-5979 SP - 69-78 ST - Electrocardiographic ST-segment depression in children with fontan circulation T2 - Clin Physiol TI - Electrocardiographic ST-segment depression in children with fontan circulation VL - 20 ID - 368 ER - TY - JOUR AB - Myocardial ischemia in children is generally not suspected when they present to an emergency department with congestive heart failure. However, the opposite holds true for an adult patient. Elevated cardiac troponin is a specific biochemical marker for myocardial damage for both adults and children, however, they have not been routinely used in children at risk for myocardial damage. A case is presented of an infant with a complex congenital heart defect with congestive heart failure who was found to have an elevated cardiac troponin. Infants at risk for myocardial damage should have an evaluation of cardiac markers, as infants with elevated levels are prone to dysrhythmias or worsening cardiac function and thus warrant closer observations and therapy. AD - Department of Emergency Medicine, Virginia Cmw. Univ. Health System, Richmond, VA, United States Department of Emergency Medicine, Virginia Cmw. Univ. Health System, 401 North 12th Street, Richmond, VA 23298-0401, United States AU - Muñiz, A. E. DB - Scopus DO - 10.1097/01.pec.0000142952.04007.e7 IS - 10 KW - Troponin M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2004 SP - 674-676 ST - Elevated cardiac troponin I in a 9-week-old infant T2 - Pediatric Emergency Care TI - Elevated cardiac troponin I in a 9-week-old infant UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-6044236799&doi=10.1097%2f01.pec.0000142952.04007.e7&partnerID=40&md5=d80a62540e84dadd57c5e808ea02449f VL - 20 ID - 2249 ER - TY - JOUR AB - Ethical treatment dilemmas are not new to the NICU. With technologic advances over the past 20 years, NICU care has developed rapidly, and survival rates have improved for some of the tiniest and most critically ill infants. In guiding clinical practice, however, standards in evidenced-based medicine have often superseded standards in evidence-based ethics. Neonatal nurses attain a more in-depth understanding of the clinical significance of the four principles of bioethics: autonomy, nonmaleficence, beneficence, and justice. Case studies illustrate the principles discussed. AD - Clinical Instructor of Pediatrics and Coordinator, NICU Clinical Research and HRI Follow-Up, David Geffen School of Medicine at UCLA, Division of Neonatology and Developmental Biology, 10833 Le Conte Avenue, Room B2-375, MDCC Mailcode: 175217, Los Angeles, CA 90095-1752; ipurdy@mednet.ucla.edu AN - 106430592. Language: English. Entry Date: 20060428. Revision Date: 20150819. Publication Type: Journal Article AU - Purdy, I. B. AU - Wadhwani, R. T. DB - ccm DP - EBSCOhost IS - 1 KW - Autonomy Infant, Premature -- Ethical Issues Intensive Care, Neonatal -- Ethical Issues Neonatal Nursing -- Ethical Issues Parents -- Ethical Issues Abnormalities, Multiple -- Ethical Issues Asphyxia Neonatorum -- Ethical Issues Beneficence Education, Continuing (Credit) Ethics Committees Ethics, Nursing Female Health Resource Allocation -- Ethical Issues Hypoplastic Left Heart Syndrome -- Ethical Issues Infant, Newborn Intracranial Hemorrhage -- Ethical Issues Kohlberg's Theory of Moral Development Male Medical Futility -- Ethical Issues Patient Care Conferences Quality of Life -- Ethical Issues Respiratory Distress Syndrome -- Ethical Issues Social Justice Treatment Refusal -- Ethical Issues N1 - case study; CEU; exam questions; tables/charts. Journal Subset: Core Nursing; Double Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Nursing; Peer Reviewed; USA. NLM UID: 8503921. PMID: NLM16514866. PY - 2006 SN - 0730-0832 SP - 43-64 ST - Embracing bioethics in neonatal intensive care, part II: case histories in neonatal ethics T2 - Neonatal Network TI - Embracing bioethics in neonatal intensive care, part II: case histories in neonatal ethics UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106430592&site=ehost-live&scope=site VL - 25 ID - 1622 ER - TY - JOUR AB - Background: Subgaleal hematomas frequently occur in children after head trauma and extend over the cranial sutures. Although conservative treatment suffices in most cases, surgical removal of a subgaleal hematoma is indicated when the patient presents with anemia and headache associated with its progressive enlargement. Copyright: Case Description: We present the case of a 7-year-old boy who was medicated with warfarin and aspirin due to a hypoplastic left ventricle and fell from a rock wherein he hit his head in the frontal region. Although a computed tomography scan of the head revealed no intracranial lesion, an extracranial hematoma was found to extend over the cranial sutures, leading to the diagnosis of subgaleal hematoma. The hematoma continued to grow gradually despite the cessation of warfarin and aspirin therapy immediately after the head trauma. Since the patient's headache and anemia were progressing as the hematoma enlarged, removal of the hematoma was performed 3 days after admission. Endoscopic hematoma removal was planned to enable accurate coagulation of the sites of bleeding and removal of the maximal amount of hematoma through minimal incision. The hematoma was completely removed, and the patient's postoperative course was excellent with alleviation of both the anemia and the headache. No sign of hematoma recurrence could be detected during 2 years follow-up. Conclusion: An angled endoscope can allow visualization of the deep subgaleal space, and this technique enabled direct visualization of the bleeding sites and accurate coagulation to prevent recurrence of hematoma. Endoscopic techniques, such as minimally invasive techniques, can allow sufficient removal of subgaleal hematoma with minimal morbidity, especially in patients such as ours. AD - Y. Hayashi, Department of Neurosurgery, Kanazawa University, Ishikawa, 13-1, Takara-machi, Kanazawa, Japan AU - Hayashi, Y. AU - Kita, D. AU - Furuta, T. AU - Oishi, M. AU - Hamada, J. I. DB - Embase DO - 10.4103/2152-7806.134911 IS - Supplement KW - acetylsalicylic acid warfarin article brain hematoma brain surgery case report child clinical feature computer assisted tomography consciousness disorder conservative treatment cranial suture disease course head injury headache human hypoplastic left heart syndrome male postoperative period priority journal school child stupor subgaleal hematoma treatment response LA - English M3 - Article N1 - L600742477 2014-12-19 2015-12-15 PY - 2014 SN - 2152-7806 ST - Endoscopic removal of subgaleal hematoma in a 7-year-old patient treated with anticoagulant and antiplatelet agents T2 - Surgical Neurology International TI - Endoscopic removal of subgaleal hematoma in a 7-year-old patient treated with anticoagulant and antiplatelet agents UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L600742477 http://dx.doi.org/10.4103/2152-7806.134911 VL - 5 ID - 968 ER - TY - JOUR AB - Plastic bronchitis is a rare condition, characterized by the formation of thick mucus plugs that cause airway obstruction. It is seen in a variety of disorders, including congenital cyanotic heart disease following palliative surgery. We describe the case of a 5-year-old child with hypoplastic left-heart syndrome who had undergone a Fontan procedure and developed recurrent bronchial cast formation. These casts were treated aggressively with repeated rigid bronchoscopy and adjuvant medical therapy. We present a brief review of the pathogenesis, treatment options, and description of the challenges involved with endoscopic removal of bronchial casts. ©2011 American Medical Association. All rights reserved. AD - Department of Otolaryngology, University of Florida, PO Box 100264, Gainesville, FL 32610, United States University of Pittsburgh School of Medicine, Pittsburgh, PA, United States AU - Silva, R. C. AU - Simons, J. P. AU - Chi, D. H. AU - Yellon, R. F. AU - Alper, C. M. DB - Scopus DO - 10.1001/archoto.2011.43 IS - 4 M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2011 SP - 401-403 ST - Endoscopic treatment of plastic bronchitis T2 - Archives of Otolaryngology - Head and Neck Surgery TI - Endoscopic treatment of plastic bronchitis UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-79954513055&doi=10.1001%2farchoto.2011.43&partnerID=40&md5=a222c57c493b1fc5ca72f896b9a539e5 VL - 137 ID - 2129 ER - TY - JOUR AB - BACKGROUND: Fontan survivors demonstrate diminished vascular function and functional outcomes, but the relationships between these measures have not been established. METHODS AND RESULTS: We performed a cross-sectional study of 60 Fontan survivors (52% male) with a mean age of 13.9±4.1 years and mean Fontan duration of 9.9±4.2 years. Multimodality assessment of endothelial function (reactive hyperemia index and flow-mediated dilation) and arterial stiffness (augmentation index and baseline pulse amplitude) was performed with peripheral arterial tonometry and brachial flow-mediated dilation. Aerobic capacity was determined using cardiopulmonary exercise testing; mean peak and percentage of predicted oxygen consumption (VO(2)) were 27.8±7.6 mL/kg per minute and 71.0±21.2%, respectively. Quality of life and physical activity were assessed using the Pediatric Quality of Life Inventory (PedsQL) and the Physical Activity Questionnaire. Vascular measures served as predictor variables, whereas functional measures served as outcome variables. In all cases, worse vascular measures were associated with worse functional measures. Flow-mediated dilation-derived reactive hyperemia index (P<0.05) was positively associated with VO(2) at anaerobic threshold. Peripheral arterial tonometry-derived baseline pulse amplitude (P<0.05) was negatively associated with the ratio of minute ventilation to carbon dioxide at anaerobic threshold. Flow-mediated dilation-derived reactive hyperemia index and peripheral arterial tonometry-derived augmentation index (P<0.05) were positively and negatively associated, respectively, with peak VO(2). Maximum flow-mediated dilation (P<0.05) was positively associated with Physical Activity Questionnaire score. Peripheral arterial tonometry-derived augmentation index and baseline pulse amplitude (P<0.05) were negatively associated with parent-reported PedsQL total and physical heath summary scores. CONCLUSIONS: Increased arterial stiffness and decreased endothelial function are associated with lower aerobic capacity, physical activity, and quality of life in adolescent and young adult Fontan survivors. Understanding the cause-effect relationship between vascular function and functional outcomes is an important next step. AD - The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH bryan.goldstein@cchmc.org. The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH. Division of Cardiology, Northwestern University Feinberg School of Medicine and Lurie Children's Hospital, Chicago, IL. AN - 27664807 AU - Goldstein, B. H. AU - Urbina, E. M. AU - Khoury, P. R. AU - Gao, Z. AU - Amos, M. A. AU - Mays, W. A. AU - Redington, A. N. AU - Marino, B. S. C2 - PMC5079057 DA - Sep 24 DB - PubMed DO - 10.1161/jaha.116.004258 DP - NLM ET - 2016/09/25 IS - 9 KW - Fontan procedure arterial stiffness endothelial function exercise capacity quality of life LA - eng N1 - 2047-9980 Goldstein, Bryan H Urbina, Elaine M Khoury, Philip R Gao, Zhiqian Amos, Michelle A Mays, Wayne A Redington, Andrew N Marino, Bradley S UL1 TR001425/TR/NCATS NIH HHS/United States Journal Article J Am Heart Assoc. 2016 Sep 24;5(9):e004258. doi: 10.1161/JAHA.116.004258. PY - 2016 SN - 2047-9980 ST - Endothelial Function and Arterial Stiffness Relate to Functional Outcomes in Adolescent and Young Adult Fontan Survivors T2 - J Am Heart Assoc TI - Endothelial Function and Arterial Stiffness Relate to Functional Outcomes in Adolescent and Young Adult Fontan Survivors VL - 5 ID - 164 ER - TY - JOUR AB - There were three objectives of this study: to investigate possible specificity in the association between specific cardiac defects and chromosomal anomalies; to evaluate ways of categorizing cardiac defects into larger groups with epidemiological similarities that could indicate similarities in etiology or pathogenesis; and to analyze the relationship between specific cardiac defects and diabetes. We pooled data on infants (aged 1 year or younger) with congenital cardiovascular defects from three large birth defect registries in California, Sweden, and France. The registries in Sweden and France obtained data through reporting from various sources; in California, medical records were reviewed. For severe congenital heart defects, the percentage of infants with identified chromosomal anomalies varied between 0.9% for d-TGV to 68.4% for ECD. In general, specific cardiac conditions have different risk factors. For example, conotruncal defects have been traditionally grouped, but the data presented in this paper indicates more differences for risk factors for the components of conotruncal defects: tetralogy of Fallot, d-TGV, common truncus, and DORV. In general, we suggest the strategy of "splitting" rather than "lumping" when searching for specific genetic factors and/or teratogens. Adequate analysis thus requires large registries or collaboration among registries. The findings did not support constellations between mothers' diabetes and specific defects. AD - J.A. Harris, CA Birth Defects Monitoring Prog., California Dept. of Health Services, 1830 Embarcadero, Oakland, CA 94606, United States AU - Harris, J. A. AU - Francannet, C. AU - Pradat, P. AU - Robert, E. DB - Embase Medline DO - 10.1007/s00246-002-9402-5 IS - 3 KW - aortic coarctation aortic valve stenosis article congenital malformation cardiovascular malformation chromosome aberration diabetes mellitus disease association disease classification Down syndrome Edwards syndrome endocardial cushion defect Fallot tetralogy female France great vessels transposition heart atrium septum defect heart right ventricle double outlet heart single ventricle heart ventricle septum defect heredity human hypoplastic left heart syndrome infant Klinefelter syndrome pulmonary valve atresia lung vein drainage anomaly major clinical study male trisomy 13 pulmonary valve stenosis risk factor Sweden teratogenesis tricuspid valve atresia triploidy trisomy 18 Turner syndrome United States LA - English M3 - Article N1 - L36920007 2003-08-11 PY - 2003 SN - 0172-0643 SP - 222-235 ST - The epidemiology of cardiovascular defects, Part 2: A study based on data from three large registries of congenital malformations T2 - Pediatric Cardiology TI - The epidemiology of cardiovascular defects, Part 2: A study based on data from three large registries of congenital malformations UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L36920007 http://dx.doi.org/10.1007/s00246-002-9402-5 VL - 24 ID - 1305 ER - TY - JOUR AB - A 24-year-old parturient with single ventricle and moderate aortic stenosis was admitted due to preeclampsia and fetal distress at 31 weeks' gestation. Emergency Caesarean section was performed under lumbar epidural anesthesia and epidural analgesia was given for post-operative pain control. Mother and baby both survived. The anesthetic techniques and managements in other parturients with similar congenital cardiac anomalies are also reviewed and described. AD - T.C. Peng, Department of Anesthesiology, Chang-Gung Memorial Hospital, Taipei, R.O.C. AU - Peng, T. C. AU - Chuah, E. C. AU - Tan, P. P. DB - Medline IS - 1 KW - adult aortic valve stenosis article case report cesarean section congenital malformation epidural anesthesia female heart ventricle human male newborn obstetric anesthesia pathophysiology pregnancy pregnancy complication LA - English M3 - Article N1 - L127280852 1997-08-13 PY - 1997 SN - 0529-5769 SP - 39-44 ST - Epidural anesthesia for emergency caesarean section in a patient with single ventricle and aortic stenosis T2 - Acta anaesthesiologica Sinica TI - Epidural anesthesia for emergency caesarean section in a patient with single ventricle and aortic stenosis UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L127280852 VL - 35 ID - 1359 ER - TY - JOUR AB - Objectives: Neonates undergoing complex congenital heart surgery have a significant incidence of neurologic problems. Erythropoietin has antiapoptotic, antiexcitatory, and anti-inflammatory properties to prevent neuronal cell death in animal models, and improves neurodevelopmental outcomes in full-term neonates with hypoxic ischemic encephalopathy. We designed a prospective phase I/II trial of erythropoietin neuroprotection in neonatal cardiac surgery to assess safety and indicate efficacy. Methods: Neonates undergoing surgery for D-transposition of the great vessels, hypoplastic left heart syndrome, or aortic arch reconstruction were randomized to 3 perioperative doses of erythropoietin or placebo. Neurodevelopmental testing using the Bayley Scales of Infant and Toddler Development III was performed at age 12 months. Results: Fifty-nine patients received the study drug. Safety profile, including magnetic resonance imaging brain injury, clinical events, and death, was not different between groups. Three patients in each group died. Forty-two patients (22 in the erythropoietin group and 20 in the placebo group; 79% of survivors) returned for 12-month follow-up. In the group receiving erythropoietin, mean Cognitive Scale scores were 101.1 ± 13.6, Language Scale scores were 88.5 ± 12.8, and Motor Scale scores were 89.9 ± 12.3. In the group receiving placebo, Cognitive Scale scores were 106.3 ± 10.8 (P =.19), Language Scores were 92.4 ± 12.4 (P =.33), and Motor Scale scores were 92.6 ± 14.1 (P =.51). Conclusions: Safety profile for erythropoietin administration was not different than placebo. Neurodevelopmental outcomes were not different between groups; however, this pilot study was not powered to definitively address this outcome. Lessons learned suggest optimized study design features for a larger prospective trial to definitively address the utility of erythropoietin for neuroprotection in this population. © 2013 by The American Association for Thoracic Surgery. AD - D.B. Andropoulos, Baylor College of Medicine, 6621 Fannin, W 17417, Houston, TX 77030, United States AU - Andropoulos, D. B. AU - Brady, K. AU - Easley, R. B. AU - Dickerson, H. A. AU - Voigt, R. G. AU - Shekerdemian, L. S. AU - Meador, M. R. AU - Eisenman, C. A. AU - Hunter, J. V. AU - Turcich, M. AU - Rivera, C. AU - McKenzie, E. D. AU - Heinle, J. S. AU - Fraser Jr, C. D. DB - Embase Medline DO - 10.1016/j.jtcvs.2012.09.046 IS - 1 KW - NCT00513240 placebo recombinant erythropoietin article Bayley Scales of Infant Development clinical article cognition controlled study drug efficacy drug safety follow up heart surgery human language motor activity neuroprotection newborn newborn surgery nuclear magnetic resonance imaging perioperative period phase 1 clinical trial phase 2 clinical trial priority journal prospective study randomized controlled trial LA - English M3 - Article N1 - L52269198 2012-10-26 2013-07-01 PY - 2013 SN - 0022-5223 1097-685X SP - 124-131 ST - Erythropoietin neuroprotection in neonatal cardiac surgery: A phase I/II safety and efficacy trial T2 - Journal of Thoracic and Cardiovascular Surgery TI - Erythropoietin neuroprotection in neonatal cardiac surgery: A phase I/II safety and efficacy trial UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52269198 http://dx.doi.org/10.1016/j.jtcvs.2012.09.046 VL - 146 ID - 993 ER - TY - JOUR AB - The history of cardiac surgery began with procedures to treat congenital heart disease, from the Blalock-Taussig shunt in the 1940s to the first use of extra-corporeal circulation in the 1950s. The whole speciality of cardiothoracic surgery has grown from the work of these early pioneers and the wider applications of cardio-pulmonary bypass have led to surgery for coronary artery disease and degenerative valvular disease in adults. Surgery for congenital heart disease now accounts for 10-15% of all cardiac surgery and continues to be at the forefront of the speciality in terms of innovation and evolving approaches. The speciality has advanced in parallel with advances in neonatal medicine and paediatric intensive care that have enabled an increasingly more complex range of conditions to become treatable. Outcomes have steadily improved, although this remains one of the most high-risk areas of modern surgery. Major congenital heart disease is a very serious condition and only 15% of children survived infancy prior to the surgical era. Now over 85% of children survive into adulthood and, for the first time, there are now more adults alive with congenital heart disease than there are children. Caring for these patients has led to the development of 'adult congenital heart disease' as an emerging specialist area. Surgery has become increasingly specialized and technically demanding with a trend to repair lesions at an earlier age and to aim for complete anatomical repair in infancy rather than a series of staged procedures. Over two-thirds of all procedures are now carried out before the age of 1 year. AD - D.J. Barron, Birmingham Children's Hospital, United Kingdom AU - Barron, D. J. AU - Jones, T. J. AU - Mussa, S. DB - Embase DO - 10.1016/j.mpsur.2011.10.003 IS - 1 KW - immunosuppressive agent adulthood aortic coarctation aortic stenosis article atrioventricular septal defect Blalock Taussig shunt cardiac graft rejection cardiopulmonary bypass child congenital heart disease coronary artery disease extracorporeal circulation Fallot tetralogy great vessels transposition heart atrium septum defect heart catheterization heart failure heart right ventricle double outlet heart septum defect heart surgery heart transplantation heart ventricle septum defect high risk patient human hypoplastic left heart syndrome immunosuppressive treatment incidence infancy intensive care interventional cardiovascular procedure lung circulation medical specialist open heart surgery patent ductus arteriosus pediatric surgery pediatrics priority journal pulmonary valve stenosis quality of life surgical anatomy surgical approach surgical technique survival rate thorax surgery treatment planning tricuspid valve atresia LA - English M3 - Article N1 - L363142954 2011-12-30 2012-01-03 PY - 2012 SN - 0263-9319 1878-1764 SP - 39-44 ST - Essentials of paediatric cardiac surgery T2 - Surgery TI - Essentials of paediatric cardiac surgery UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L363142954 http://dx.doi.org/10.1016/j.mpsur.2011.10.003 VL - 30 ID - 1070 ER - TY - JOUR AB - Hypoplastic left heart syndrome was once considered a lethal congenital heart defect; yet, the prognosis is improving with advances in health care. Many ethical dilemmas still surround this diagnosis and treatment. This article explores the ethical side of hypoplastic left heart syndrome care. Copyright 2002, Elsevier Science (USA). All rights reserved. AD - Division of Neonatology, Schneider Children's Hospital at North Shore, Manhasset, NY AN - 106698974. Language: English. Entry Date: 20040206. Revision Date: 20150820. Publication Type: Journal Article AU - Cavaliere, T. A. DB - ccm DP - EBSCOhost IS - 2 KW - Hypoplastic Left Heart Syndrome -- Ethical Issues Hypoplastic Left Heart Syndrome -- Therapy Autonomy Beneficence Consent Decision Making, Family -- Ethical Issues Heart Transplantation -- Ethical Issues Hypoplastic Left Heart Syndrome -- Surgery Infant Infant, Newborn Neonatal Nursing -- Ethical Issues Palliative Care -- Ethical Issues -- In Infancy and Childhood Quality of Life -- In Infancy and Childhood Social Justice Survival -- In Infancy and Childhood N1 - case study. Commentary: Kenner C, Lott JW, Strodtbeck F, Theorell C. Postscript. (NEWBORN INFANT NURS REV) 2002 Jun; 2 (2): 139-142. Journal Subset: Core Nursing; Nursing; USA. NLM UID: 101126037. PY - 2002 SN - 1527-3369 SP - 75-82 ST - Ethical considerations in the management of hypoplastic left heart syndrome T2 - Newborn & Infant Nursing Reviews TI - Ethical considerations in the management of hypoplastic left heart syndrome UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106698974&site=ehost-live&scope=site VL - 2 ID - 1606 ER - TY - JOUR AB - It is unclear how parents make life-and-death decisions on behalf of their children. To address this issue we interviewed 20 mothers whose children were born with hypoplastic left heart syndrome (HLHS), 10 whose child survived the operations and 10 mothers who chose comfort care. Semistructured interviews lasting from 1.5 to 3.5 h took place on median 4.6 (range 2 .5-6) years after birth. The mothers who chose comfort care had more years of education (p = 0.03), reported a better childhood environment (p= 0.05) and had more often been employed in the healthcare services. The two groups described the information received in similar terms. The main motive for choosing surgery was that this was perceived to be the only acceptable choice. The motive for choosing non-treatment was to prevent the child from suffering. Lack of knowledge about long-term outcome, a state of shock and short time to deliberate limit the parents' possibilities for making autonomous choices. Autonomous decisions may be neither possible nor desirable for all parents. The care perspective, which highlights the parents' preferences, resources and values, should represent ethical guidelines for care providers willing to decide on behalf of the child. AD - I.H. Vandvik, Div. of Child/Adolescent Psychiat., National Hospital of Norway, NO-0027 Oslo, Norway AU - Vandvik, I. H. AU - Forde, R. DB - Embase Medline IS - 9 KW - article child care child parent relation clinical article controlled study decision making education environment ethics female health service heart surgery human hypoplastic left heart syndrome informed consent maternal care priority journal survival LA - English M3 - Article N1 - L30774980 2000-10-30 PY - 2000 SN - 0803-5253 SP - 1129-1133 ST - Ethical issues in parental decision-making. An interview study of mothers of children with hypoplastic left heart syndrome T2 - Acta Paediatrica, International Journal of Paediatrics TI - Ethical issues in parental decision-making. An interview study of mothers of children with hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L30774980 VL - 89 ID - 1331 ER - TY - JOUR AB - There is a strong need for well-designed clinical trials in congenital heart disease. Research in children, including children with congenital heart disease, must adhere to the highest ethical standards at all times. © The Author(s) 2012. AD - R. E. Shaddy, Division of Pediatric Cardiology, The Children's Hospital of Philadelphia, 8NW 90, 8th Floor, Northwest Tower, 34th St. and Civic Cen, Philadelphia, PA 19104, United States AU - Shaddy, R. E. DB - Embase Medline DO - 10.1177/2150135112454551 IS - 1 KW - placebo sildenafil tadalafil article beneficence child health care clinical trial (topic) congenital heart disease exercise Fontan procedure human justice medical ethics medical research patient autonomy phase 1 clinical trial (topic) phase 2 clinical trial (topic) phase 3 clinical trial (topic) practice guideline priority journal randomized controlled trial (topic) total quality management LA - English M3 - Article N1 - L368408850 2013-03-05 2020-04-07 PY - 2013 SN - 2150-136X 2150-1351 SP - 50-52 ST - Ethics and Clinical Trials in Congenital Heart Disease T2 - World Journal for Pediatric and Congenital Hearth Surgery TI - Ethics and Clinical Trials in Congenital Heart Disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L368408850 http://dx.doi.org/10.1177/2150135112454551 VL - 4 ID - 1026 ER - TY - JOUR AB - Aim: This study aims to show that palliative care can be offered to babies born with lethal malformations according to the Islamic Code of Ethics, which takes into account the well-being of the mother and her fetus. Methods: For our study, a lethal congenital malformation is defined as a condition that invariably leads to death in utero or the newborn period regardless of attempted supportive care. The cases were identified during weekly prenatal meetings, which discuss abnormal fetal ultrasound findings and genetic testing to provide plans for their management. In addition, cases that were not diagnosed antenatally but diagnosed at birth were also included. The management plans for those cases with lethal malformations were discussed before birth, and the families were counseled, and their consent was taken for nonmonitoring of the fetus during pregnancy and noninterference with the newborn baby except for palliative care. Families who requested full support during pregnancy and delivery were given that option. Results: Over a period of 16 years from 2001 to 2016, a total number of 92705 babies were born in our hospital. Antenatal ultrasounds were performed by the feto-maternal physician team. There were 1563 abnormal fetal ultrasounds (1.7%) with significant various organs malformations. Of these abnormal ultrasounds, 384 were diagnosed as possible lethal congenital malformations (24.6%). The remaining abnormal fetal ultrasounds were compatible with life and were managed according to the standard clinical practices. Fetuses diagnosed with lethal malformations were discussed at length and parents were informed about the lethal outcome and the plan of nonmonitoring of the mother at delivery and avoidance of cesarean section except for maternal indication and eventual palliative care for the live newborn. Parents' consent was obtained, and the decision and the plan of the management are documented clearly in the mother's medical record file. With this management plan, we were able to offer palliative care to the cases of lethal congenital malformations who eventually expired within a short time. Conclusion: Within the guidance of the Islamic Jurisprudence (Figh), it is possible to manage cases of congenital malformations, which are considered incompatible with life palliatively and avoid unnecessary cesarean sections in the mother. This practice has resulted in better utilization of the beds in our busy neonatal intensive care unit. AD - O. Abdelbasit, Department of Pediatrics, Division of Neonatology, Security Forces Hospital, Riyadh, Saudi Arabia AU - Abdelbasit, O. AU - Seidahmed, M. AU - Alhussein, K. AU - Miqdad, A. AU - Mohsen, A. AU - Khalil, M. AU - Al-Mardawi, E. AU - Bahyan, R. DB - Embase DO - 10.4103/jcn.JCN_39_19 IS - 4 KW - anencephalus article bone dysplasia cesarean section chondrodysplasia chromosome aberration congenital diaphragm hernia congenital hydrocephalus congenital malformation disease severity family counseling family history female fetus echography fetus hydrops genetic screening hemophagocytic syndrome human hydramnios hypoplastic left heart syndrome inborn error of metabolism kidney agenesis kidney polycystic disease lactic acidemia lethality live birth lung hypoplasia major clinical study Meckel syndrome medical ethics neonatal intensive care unit newborn newborn care newborn death newborn period observational study oligohydramnios overall survival palliative therapy parental consent pregnancy prenatal period priority journal prospective study survival time thanatophoric dwarfism trisomy 13 trisomy 18 trisomy 7 Walker Warburg syndrome LA - English M3 - Article N1 - L629649966 2019-10-25 2019-11-01 PY - 2019 SN - 2249-4847 SP - 216-221 ST - The ethics of lethal congenital malformations. Is palliative therapy possible? T2 - Journal of Clinical Neonatology TI - The ethics of lethal congenital malformations. Is palliative therapy possible? UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L629649966 http://dx.doi.org/10.4103/jcn.JCN_39_19 VL - 8 ID - 595 ER - TY - JOUR AU - Spike, J. P. DB - Medline DO - 10.1080/15265161.2017.1314707 IS - 7 KW - case report child parent relation contraindication cost of illness education ethics heart surgery human hypoplastic left heart syndrome male newborn palliative nursing prognosis psychology quality of life risk assessment LA - English M3 - Article N1 - L625031223 2018-11-22 2019-07-05 PY - 2017 SN - 1536-0075 SP - 65-66 ST - The Ethics of Treatment for Hypoplastic Left Heart Syndrome (HLHS) T2 - The American journal of bioethics : AJOB TI - The Ethics of Treatment for Hypoplastic Left Heart Syndrome (HLHS) UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L625031223 http://dx.doi.org/10.1080/15265161.2017.1314707 VL - 17 ID - 747 ER - TY - JOUR AD - D. Biarent, Paediatric Intensive Care, Hôpital Universitaire des Enfants, 15 av JJ Crocq, Brussels, Belgium AU - Biarent, D. AU - Bingham, R. AU - Eich, C. AU - López-Herce, J. AU - Maconochie, I. AU - Rodríguez-Núñez, A. AU - Rajka, T. AU - Zideman, D. DB - Embase Medline DO - 10.1016/j.resuscitation.2010.08.012 IS - 10 KW - NCT00878644 NCT00880087 adenosine epinephrine alpha adrenergic receptor blocking agent amiodarone antipyretic agent atropine bicarbonate calcium dobutamine dopamine glucose lidocaine magnesium naloxone nitric oxide noradrenalin oxygen procainamide prostacyclin terlipressin vasopressin absence of side effects airway airway obstruction article assisted ventilation automated external defibrillator blood glucose monitoring bradycardia breathing calcium channel blocking agent overdose capnometry cardiac channelopathy cardiopulmonary arrest cardiopulmonary hemodynamics child parent relation child safety childhood disease childhood mortality clinical trial compression therapy consciousness continuous infusion cricoid cartilage decompression defibrillation defibrillator disease course drug dose titration drug efficacy drug half life drug overdose drug safety drug substitution electric potential electrocardiography monitoring emergency treatment end tidal carbon dioxide tension endotracheal intubation endotracheal tube extracorporeal circulation family relation fever foreign body heart arrest heart arrhythmia heart single ventricle heart ventricle fibrillation heart ventricle tachycardia human hyperkalemia hypermagnesemia hypocalcemia hypomagnesemia intensive care international cooperation laryngeal mask lung ventilation perfusion ratio medical device metabolic acidosis mouth myocardial disease oxygenation patient safety pediatric advanced life support practice guideline priority journal prognosis pulmonary hypertension pulse oximetry respiratory failure resuscitation single drug dose supraventricular tachycardia systemic vascular resistance tachycardia procedures temperature measurement thorax titrimetry tricyclic antidepressant agent overdose unconsciousness unspecified side effect vascular access LA - English M3 - Article N1 - L51113409 2010-10-20 2010-12-08 PY - 2010 SN - 0300-9572 SP - 1364-1388 ST - European Resuscitation Council Guidelines for Resuscitation 2010 Section 6. Paediatric life support T2 - Resuscitation TI - European Resuscitation Council Guidelines for Resuscitation 2010 Section 6. Paediatric life support UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51113409 http://dx.doi.org/10.1016/j.resuscitation.2010.08.012 VL - 81 ID - 1135 ER - TY - JOUR AD - Department of Pediatrics, Baylor College of Medicine, Houston, Texas. Psychology Service, Texas Children's Hospital, Houston, Texas; and. Department of Pediatrics, Baylor College of Medicine, Houston, Texas; mrlanti2@texaschildrens.org. Pediatric Cardiology, Texas Children's Heart Center, Houston, Texas. AN - 31628207 AU - Cutitta, K. AU - Lantin-Hermoso, M. R. DA - Nov DB - PubMed DO - 10.1542/peds.2019-1664 DP - NLM ET - 2019/10/20 IS - 5 KW - Child Humans *Hypoplastic Left Heart Syndrome Quality of Life Surveys and Questionnaires conflicts of interest to disclose. LA - eng N1 - 1098-4275 Cutitta, Katherine Lantin-Hermoso, M Regina Comment Journal Article United States Pediatrics. 2019 Nov;144(5):e20191664. doi: 10.1542/peds.2019-1664. Epub 2019 Oct 18. PY - 2019 SN - 0031-4005 ST - Evaluating Quality of Life in Young School-Aged Children With Hypoplastic Left Heart Syndrome T2 - Pediatrics TI - Evaluating Quality of Life in Young School-Aged Children With Hypoplastic Left Heart Syndrome VL - 144 ID - 151 ER - TY - JOUR AB - Home international normalized ratio (INR) monitors are being increasingly used for monitoring the adequacy of anticoagulation in children on long-term warfarin. Their safety and effectiveness in the home setting has not been fully established. The purpose of this study was to explore the safety and effectiveness of home INR monitoring service in managing anticoagulation in children with congenital heart disease. At the Pediatric Cardiology Regional Referral Center, we studied records of 41 children on warfarin and assessed the control of anticoagulation and frequency of adverse reactions over a 1-year period. User satisfaction was assessed by means of telephone interviews. The study found that the therapeutic INR range was maintained for over 57% of the total study period. There were no reported instances of major or minor hemorrhages. On six occasions, subtherapeutic INR levels necessitated hospitalization for heparinization. Mitral valve replacement was significantly associated with poor control (p < 0.05). User satisfaction was high for all aspects of the service evaluated. The hospital-assisted home INR monitoring service is a safe and effective means of managing anticoagulation in children. In addition, it ensures good compliance and a high level of user satisfaction. © 2009 Springer Science+Business Media, LLC. AD - D. Bhat, Department of Pediatrics, MetroHealth Medical Center, Cleveland, OH, United States AU - Bhat, D. AU - Upponi, A. AU - Rakecha, A. AU - Thomson, J. DB - Embase Medline DO - 10.1007/s00246-009-9535-x IS - 1 KW - warfarin adolescent adult anticoagulant therapy aortic valve replacement article bleeding child clinical article controlled study drug indication female Fontan procedure heparinization home monitoring hospitalization human international normalized ratio interview male medical record review mitral valve replacement patient safety patient satisfaction preschool child school child United Kingdom LA - English M3 - Article N1 - L50662278 2010-02-10 2010-02-22 PY - 2010 SN - 0172-0643 SP - 18-21 ST - Evaluating safety, effectiveness, and user satisfaction of home international normalized ratio monitoring service: Experience from a tertiary pediatric cardiology unit in the United Kingdom T2 - Pediatric Cardiology TI - Evaluating safety, effectiveness, and user satisfaction of home international normalized ratio monitoring service: Experience from a tertiary pediatric cardiology unit in the United Kingdom UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50662278 http://dx.doi.org/10.1007/s00246-009-9535-x VL - 31 ID - 1157 ER - TY - JOUR AB - It has been 50 years since Francis Fontan pioneered the operation that today bears his name. Initially designed for patients with tricuspid atresia, this procedure is now offered for a vast array of congenital cardiac lesions when a circulation with 2 ventricles cannot be achieved. As a result of technical advances and improvements in patient selection and perioperative management, survival has steadily increased, and it is estimated that patients operated on today may hope for a 30-year survival of >80%. Up to 70 000 patients may be alive worldwide today with Fontan circulation, and this population is expected to double in the next 20 years. In the absence of a subpulmonary ventricle, Fontan circulation is characterized by chronically elevated systemic venous pressures and decreased cardiac output. The addition of this acquired abnormal circulation to innate abnormalities associated with single-ventricle congenital heart disease exposes these patients to a variety of complications. Circulatory failure, ventricular dysfunction, atrioventricular valve regurgitation, arrhythmia, protein-losing enteropathy, and plastic bronchitis are potential complications of the Fontan circulation. Abnormalities in body composition, bone structure, and growth have been detected. Liver fibrosis and renal dysfunction are common and may progress over time. Cognitive, neuropsychological, and behavioral deficits are highly prevalent. As a testimony to the success of the current strategy of care, the proportion of adults with Fontan circulation is increasing. Healthcare providers are ill-prepared to tackle these challenges, as well as specific needs such as contraception and pregnancy in female patients. The role of therapies such as cardiovascular drugs to prevent and treat complications, heart transplantation, and mechanical circulatory support remains undetermined. There is a clear need for consensus on how best to follow up patients with Fontan circulation and to treat their complications. This American Heart Association statement summarizes the current state of knowledge on the Fontan circulation and its consequences. A proposed surveillance testing toolkit provides recommendations for a range of acceptable approaches to follow-up care for the patient with Fontan circulation. Gaps in knowledge and areas for future focus of investigation are highlighted, with the objective of laying the groundwork for creating a normal quality and duration of life for these unique individuals. AN - 31256636 AU - Rychik, J. AU - Atz, A. M. AU - Celermajer, D. S. AU - Deal, B. J. AU - Gatzoulis, M. A. AU - Gewillig, M. H. AU - Hsia, T. Y. AU - Hsu, D. T. AU - Kovacs, A. H. AU - McCrindle, B. W. AU - Newburger, J. W. AU - Pike, N. A. AU - Rodefeld, M. AU - Rosenthal, D. N. AU - Schumacher, K. R. AU - Marino, B. S. AU - Stout, K. AU - Veldtman, G. AU - Younoszai, A. K. AU - d'Udekem, Y. DA - Jul 1 DB - PubMed DO - 10.1161/cir.0000000000000696 DP - NLM ET - 2019/07/02 KW - AHA Scientific Statements Fontan procedure congenital heart defects quality of life LA - eng N1 - 1524-4539 Rychik, Jack Atz, Andrew M Celermajer, David S Deal, Barbara J Gatzoulis, Michael A Gewillig, Marc H Hsia, Tain-Yen Hsu, Daphne T Kovacs, Adrienne H McCrindle, Brian W Newburger, Jane W Pike, Nancy A Rodefeld, Mark Rosenthal, David N Schumacher, Kurt R Marino, Bradley S Stout, Karen Veldtman, Gruschen Younoszai, Adel K d'Udekem, Yves American Heart Association Council on Cardiovascular Disease in the Young and Council on Cardiovascular and Stroke Nursing Journal Article United States Circulation. 2019 Jul 1:CIR0000000000000696. doi: 10.1161/CIR.0000000000000696. PY - 2019 SN - 0009-7322 SP - Cir0000000000000696 ST - Evaluation and Management of the Child and Adult With Fontan Circulation: A Scientific Statement From the American Heart Association T2 - Circulation TI - Evaluation and Management of the Child and Adult With Fontan Circulation: A Scientific Statement From the American Heart Association ID - 2 ER - TY - JOUR AB - INTRODUCTION: Liver fibrosis and cirrhosis are late complications in Fontan palliation. Liver biopsy is the gold standard. The goal of this study is to correlate transjugular liver biopsy (TJLB) in the setting of Fontan palliation with noninvasive testing and hemodynamics. METHODS: Between August 2014 and July 2017, 49 Fontan patients underwent TJLB. All the patients had hemodynamic evaluation, 28 patients had MRE (magnetic resonance elastography) and 40 patients had cardiopulmonary exercise test. Histologic liver fibrosis was quantitated using traditional histologic scoring systems and a modified Ishak congestive hepatic fibrosis score. RESULTS: Median age 17.8 years, median time since Fontan 15.2 years. Primary diagnosis and Fontan type were variables, but predominantly LV morphology (30/49), lateral tunnel Fontan (29/49), originally fenestrated (37/49), and 11/49 had a pacemaker. Histologic fibrosis correlated with MRE (R = 0.62, P ≤ .001). Histologic fibrosis and MRE correlated with Fontan pressure (R = 0.38, P = .008 & R = 0.59, P ≤ .001). Morphology of the single ventricle did not correlate with liver fibrosis. The presence of a fenestration resulted in a higher cardiac index (P = .026) but did not resulted in lower liver fibrosis (P = .64). CONCLUSION: Noninvasive tests, such as MRE, may be suitable for longitudinal follow-up in patients with single ventricle physiology. Our data suggest that there is reasonable correlation of MRE liver stiffness with biopsy scoring systems and Fontan pressures. We demonstrated the feasibility of TJLB in the setting of Fontan palliation and demonstrated its correlation with noninvasive measures particularly MRE. We recommend selective use of TJLB when MRE score is >5 KPa or when there are other clinical signs of cirrhosis. AD - Division of Pediatric Cardiology, University of California San Diego and Rady Children's Hospital, USA. Division of Pediatric ICU, University of California San Diego and Rady Children's Hospital, USA. Division of Gastroenterology, University of California San Diego, USA. Division of Pediatric Pathology, University of California San Diego and Rady Children's Hospital, USA. Division of Pathology, University of California San Diego, USA. Department of Radiology, Rady Children's Hospital, USA. Department of medicine, Division of digestive diseases & transplant, University of Emory, USA. Department of Pathology, Sharp Memorial hospital, USA. Department of Interventional Radiology, Sharp Memorial hospital, USA. University of California, Los Angeles, USA. University of Zagazig, Faculty of Medicine, Egypt. Division of Cardiology, University of California San Diego, USA. AN - 31038848 AU - Silva-Sepulveda, J. A. AU - Fonseca, Y. AU - Vodkin, I. AU - Vaughn, G. AU - Newbury, R. AU - Vavinskaya, V. AU - Dwek, J. AU - Perry, J. C. AU - Reshamwala, P. AU - Baehling, C. AU - Lyon, J. AU - Davis, C. AU - Lee, J. W. AU - El-Sabrout, H. AU - Shahbah, D. AU - Alshawabkeh, L. AU - Moore, J. W. AU - El-Said, H. DA - Jul DB - PubMed DO - 10.1111/chd.12770 DP - NLM ET - 2019/05/01 IS - 4 KW - Adolescent Adult Biopsy/*methods Child Child, Preschool Feasibility Studies Female Follow-Up Studies Fontan Procedure/*adverse effects Heart Defects, Congenital/diagnosis/physiopathology/*surgery Heart Ventricles/*diagnostic imaging/physiopathology Hemodynamics/*physiology Humans Jugular Veins Liver/*pathology Liver Cirrhosis/*diagnosis/etiology/physiopathology Magnetic Resonance Imaging/*methods Male Retrospective Studies Young Adult cirrhosis exercise stress test fontan liver biopsy magnetic resonance elastography transient elastography LA - eng N1 - 1747-0803 Silva-Sepulveda, Jose A Fonseca, Yudy Vodkin, Irine Vaughn, Gabrielle Newbury, Robert Vavinskaya, Vera Dwek, Jerry Perry, James C Reshamwala, Preeti Baehling, Cynthia Lyon, James Davis, Christopher Lee, Jesse W Orcid: 0000-0001-8335-5357 El-Sabrout, Hannah Shahbah, Doaa Alshawabkeh, Laith Moore, John W El-Said, Howaida Orcid: 0000-0002-3447-7398 Journal Article United States Congenit Heart Dis. 2019 Jul;14(4):600-608. doi: 10.1111/chd.12770. Epub 2019 Apr 30. PY - 2019 SN - 1747-079x SP - 600-608 ST - Evaluation of Fontan liver disease: Correlation of transjugular liver biopsy with magnetic resonance and hemodynamics T2 - Congenit Heart Dis TI - Evaluation of Fontan liver disease: Correlation of transjugular liver biopsy with magnetic resonance and hemodynamics VL - 14 ID - 27 ER - TY - JOUR AB - Background: There are a limited number of reports on the technical and clinical feasibility of prospective electrocardiogram (ECG)-gated multi-detector computed tomography (MDCT) in infants with congenital heart disease (CHD). Objective: To evaluate image quality and radiation dose at weight-based low-dose prospectively gated 256-slice MDCT angiography in infants with CHD. Materials and methods: From November 2009 to February 2010, 64 consecutive infants with CHD referred for pre-operative or post-operative CT were included. All were scanned on a 256-slice MDCT system utilizing a low-dose protocol (80 kVp and 60-120 mAs depending on weight: 60 mAs for ≤3 kg, 80 mAs for 3.1-6 kg, 100 mAs for 6.1-10 kg, 120 mAs for 10.1-15 kg). Results: No serious adverse events were recorded. A total of 174 cardiac deformities, confirmed by surgery or heart catheterization, were studied. The sensitivity of MDCT for cardiac deformities was 97.1%; specificity, 99.4%; accuracy, 95.9%. The mean heart rate during scan was 136.7 ± 14.9/min (range, 91-160) with a corresponding heart rate variability of 2.8 ± 2.2/min (range, 0-8). Mean scan length was 115.3 ± 11.7 mm (range, 93.6-143.3). Mean volume CT dose index, mean dose-length product and effective dose were 2.1 ± 0.4 mGy (range, 1.5-2.8), 24.7 ± 5.9 mGy•cm (range, 14.7-35.8) and 1.6 ± 0.3 mSv (range, 1.1-2.5), respectively. Diagnostic-quality images were achieved in all cases. Satisfactory diagnostic quality for visualization of all/proximal/distal coronary artery segments was achieved in 88.4/98.8/80.0% of the scans. Conclusion: Low-dose prospectively gated axial 256-slice CT angiography is a valuable tool in the routine clinical evaluation of infants with CHD, providing a comprehensive three-dimensional evaluation of the cardiac anatomy, including the coronary arteries. © 2011 Springer-Verlag. AD - C.-H. Liang, Department of Radiology, Guangdong Academy of Medical Sciences, Guangdong General Hospital, 106 Zhongshan Er Road, Guangzhou 510080, China AU - Huang, M. P. AU - Liang, C. H. AU - Zhao, Z. J. AU - Liu, H. AU - Li, J. L. AU - Zhang, J. E. AU - Cui, Y. H. AU - Yang, L. AU - Liu, Q. S. AU - Ivanc, T. B. AU - Vembar, M. C1 - ultravist 300(Bayer,Germany) C2 - Bayer(Germany) DB - Embase Medline DO - 10.1007/s00247-011-2079-2 IS - 7 KW - iopromide accuracy aortic arch interruption aortic coarctation aortopulmonary septal defect article computed tomographic angiography congenital heart disease coronary artery electrocardiogram Fallot tetralogy female great vessels transposition heart atrium septum defect heart catheterization heart rate heart rate variability heart right ventricle double outlet heart single ventricle heart ventricle septum defect human image quality infant lung vein drainage anomaly major clinical study male multidetector computed tomography newborn patent ductus arteriosus priority journal pulmonary valve atresia radiation dose sensitivity and specificity vascular ring Williams Beuren syndrome ultravist 300 LA - English M3 - Article N1 - L51401515 2011-05-04 2011-07-14 PY - 2011 SN - 0301-0449 1432-1998 SP - 858-866 ST - Evaluation of image quality and radiation dose at prospective ECG-triggered axial 256-slice multi-detector CT in infants with congenital heart disease T2 - Pediatric Radiology TI - Evaluation of image quality and radiation dose at prospective ECG-triggered axial 256-slice multi-detector CT in infants with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51401515 http://dx.doi.org/10.1007/s00247-011-2079-2 VL - 41 ID - 1101 ER - TY - JOUR AB - BACKGROUND: Thromboembolic complications have been noted after the Fontan operation. However, the prevalence of silent events among an adult contemporary population is not known. Noninvasive screening by any method including computed tomography (CT) has been technically limited to date. OBJECTIVES: The objective of this study was to evaluate a novel dual-energy CT (DECT) protocol in determining the prevalence of "silent" intracardiac thrombus and thrombus in the Fontan and pulmonary circulations among adults after the Fontan operation. METHODS: All post-Fontan patients attending the Pacific Adult Congenital Heart Clinic were approached for study participation. Those agreeable underwent a full clinical assessment, cardiopulmonary stress testing, transthoracic echocardiogram, and DECT low kilovoltage imaging protocol. RESULTS: Twenty-three patients were included in the study (30 ± 10 years, 26% women). Three (13%) patients had evidence of silent thrombi detected on DECT. All three of these patients had an extracardiac conduit and mural thrombus was found within the conduit. Older age at the time of the Fontan operation was associated with the presence of thrombus (21 ± 14 vs. 11 ± 6 years, P =.05). CONCLUSIONS: Thirteen percent of adult patients post-Fontan procedure have clinically silent thrombi. These were all found among patients with an extracardiac conduit traditionally thought to be at low risk for thromboembolism. Given the significant risk of thromboembolic complications, large randomized prospective studies looking at anticoagulation therapy in all Fontan patients are urgently needed. In the meanwhile, given the important rate of silent thrombi, a systematic robust screening protocol that includes noninvasive low radiation methods such as DECT methods should be considered. AD - Division of Cardiology, St. Paul's Hospital, University of British Columbia, Vancouver, BC, Canada. jasmine.grewal@vch.ca AN - 22897869 AU - Grewal, J. AU - Al Hussein, M. AU - Feldstein, J. AU - Kiess, M. AU - Ellis, J. AU - Human, D. AU - Leipsic, J. DA - Jan-Feb DB - PubMed DO - 10.1111/j.1747-0803.2012.00699.x DP - NLM ET - 2012/08/18 IS - 1 KW - Adult Age Factors Exercise Test Female Fontan Procedure/*adverse effects Heart Defects, Congenital/surgery Heart Diseases/*diagnostic imaging Humans Image Processing, Computer-Assisted Male Postoperative Complications/diagnostic imaging Prospective Studies Risk Assessment Thrombosis/*diagnostic imaging Tomography, X-Ray Computed/*methods Young Adult LA - eng N1 - 1747-0803 Grewal, Jasmine Al Hussein, Mosaad Feldstein, Jaimee Kiess, Marla Ellis, Jennifer Human, Derek Leipsic, Jonathon Journal Article United States Congenit Heart Dis. 2013 Jan-Feb;8(1):40-7. doi: 10.1111/j.1747-0803.2012.00699.x. Epub 2012 Aug 16. PY - 2013 SN - 1747-079x SP - 40-7 ST - Evaluation of silent thrombus after the Fontan operation T2 - Congenit Heart Dis TI - Evaluation of silent thrombus after the Fontan operation VL - 8 ID - 258 ER - TY - JOUR AB - To evaluate for evidence of systemic glucocorticoid absorption in cases of Fontan-associated protein-losing enteropathy (PLE) treated with enteral budesonide, we reviewed the charts of 27 patients with Fontan-associated PLE followed at Children’s Hospital Colorado from 2005 to 2018. Cases were excluded for lack of budesonide thserapy or a treatment duration of less than 6 months. Charts were examined by two endocrinologists for review of prior biochemical endocrine evaluations, alterations in linear growth, and physical exam findings consistent with steroid excess. Twelve patients met inclusion criteria. Eight had prior documented cortisol screening. Three patients were tested while on treatment with a median fasting AM cortisol of 0.9 mcg/dL; two of these had a concomitantly measured ACTH, both below the detectable limit. Five patients were tested while weaning or having discontinued budesonide, with a median fasting AM cortisol of 9.1 mcg/dL. Eleven patients had decreases in height velocity associated with starting budesonide. Six patients had documentation of cushingoid features by an endocrinologist. In this cohort of children treated with budesonide for PLE following Fontan, clinical signs of systemic glucocorticoid absorption were frequent. Cortisol secretion was suppressed while on therapy, with adrenal recovery noted once budesonide was discontinued. Growth failure and cushingoid features were common findings. While these findings should be confirmed in larger cohorts, we recommend that the evaluation for systemic absorption of exogenous steroids be considered in patients treated with long-term enteral budesonide given the potential risk for adrenal crisis in times of physiologic stressors. AD - R.O. Roberts, Section of Endocrinology, Department of Pediatrics, Children’s Hospital Colorado, University of Colorado Anschutz Medical Campus, 13123 E 16th Ave, Box 265, Aurora, CO, United States AU - Roberts, R. O. AU - Di Maria, M. V. AU - Brigham, D. AU - Hsu, S. DB - Embase Medline DO - 10.1007/s00246-019-02248-3 IS - 2 KW - budesonide corticosteroid corticotropin derivative corticotropin releasing factor glucocorticoid hydrocortisone prednisolone spironolactone adrenal cortex insufficiency adrenal suppression article child clinical article cohort analysis controlled study Cushingoid syndrome drug absorption drug metabolism endocrinologist female growth disorder human incidence male physical examination pleiotropy protein losing gastroenteropathy quality of life retrospective study screening test treatment duration LA - English M3 - Article N1 - L2003573566 2019-11-19 2020-04-07 PY - 2020 SN - 1432-1971 0172-0643 SP - 241-250 ST - Evidence of Systemic Absorption of Enteral Budesonide in Patients with Fontan-Associated Protein-Losing Enteropathy T2 - Pediatric Cardiology TI - Evidence of Systemic Absorption of Enteral Budesonide in Patients with Fontan-Associated Protein-Losing Enteropathy UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2003573566 http://dx.doi.org/10.1007/s00246-019-02248-3 VL - 41 ID - 566 ER - TY - JOUR AB - Since initial descriptions of staged palliation for hypoplastic left heart syndrome (HLHS) in the 1980's, much has been learned about the pathophysiology of the single ventricle circulation. New therapies that leverage systems biology and clinical derivatives have been developed. While in-hospital mortality and morbidity for babies with HLHS have continued to improve, there remains a long (and daresay winding) road ahead to achieve ideal outcomes. Important variation in even these abbreviated in-hospital metrics persists among institutions and currently utilized prediction models explain only a small amount of this variation. Moreover, long-term survival and neurodevelopmental health for patients with HLHS are infrequently reported and remain suboptimal despite improved in-hospital outcomes. This focused review will describe the evolution of national outcomes for HLHS over time and the potential factors motivating improved time-related mortality. Emerging modifiable risk-factors that hold promise in terms of moving the needle for long-term success, including social determinants of health and the delineation of genetic profiles, will be discussed. Specifically, this review will integrate contemporary data based on the first murine HLHS models that suggest a genetically elicited modular phenotype with environmental factors known to impact the initial durability of surgical therapies. A comprehensive approach to the management of HLHS, which leverages both proactive transplantation and hybrid palliation, in addition to traditional Norwood palliation, will be emphasized to extend and match management to the complete spectrum of patient risk-profiles. Finally, we will explore the critical role that national collaboratives and quality reporting initiatives have played in improving outcomes and shifting the focus to more meaningful long-term survival and neurodevelopment. AD - Division of Pediatric Cardiac Surgery, Cleveland Clinic Heart Vascular Institute, Cleveland, OH, USA AN - 142575931. Language: English. Entry Date: 20200413. Revision Date: 20200413. Publication Type: Article AU - Karamlou, Tara AU - Najm, Hani K. DB - ccm DO - 10.21037/jtd.2019.10.75 DP - EBSCOhost IS - 3 KW - Hypoplastic Left Heart Syndrome -- Therapy Patient Care Plans -- In Infancy and Childhood Outcomes (Health Care) Quality Improvement Hypoplastic Left Heart Syndrome -- Physiopathology Hospital Mortality Morbidity Hypoplastic Left Heart Syndrome -- Risk Factors Mental Disorders Diagnosed in Childhood -- Prevention and Control Survival Time Factors Social Determinants of Health Hypoplastic Left Heart Syndrome -- Familial and Genetic Environment Hypoplastic Left Heart Syndrome -- Surgery Heart Transplantation Child N1 - pictorial; review. Journal Subset: Asia; Biomedical; Peer Reviewed. Special Interest: Pediatric Care. NLM UID: 101533916. PY - 2020 SN - 2072-1439 SP - 1174-1183 ST - Evolution of care pathways for babies with hypoplastic left heart syndrome: integrating mechanistic and clinical process investigation, standardization, and collaborative study T2 - Journal of Thoracic Disease TI - Evolution of care pathways for babies with hypoplastic left heart syndrome: integrating mechanistic and clinical process investigation, standardization, and collaborative study UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=142575931&site=ehost-live&scope=site VL - 12 ID - 1436 ER - TY - JOUR AB - Background. Surgical approaches to single ventricle variants include staged, fenestrated, and completed Fontan operations. This study compares outcomes with these modifications of the Fontan operation at a single center. Methods. Preoperative risk factors and operative results were analyzed by multivariate techniques in 129 patients undergoing modified Fontan operations since March 1988. Results. Overall early and late mortality was 5.41% and 0.8%, respectively. Before 1993, completed Fontan operation using right atrial to pulmonary artery anastomoSis without fenestration was performed in the majority of patients (44 of 58; 76%). During this period, 10 of 17 patients at high risk had completed Fontan with three takedowns. In 1994, the staged hemi-Fontan and modified Fontan with a lateral tunnel anastomosis and with or without small fenestration (2.5 to 4 mm) were introduced. The majority of patients at high risk during this period underwent hemi-Fontan followed by fenestrated Fontan with no takedowns. Late atrial dysrhythmias occurred in 6 patients (4.7%), generally with larger fenestrations or right atrial to pulmonary anastomose. Three patients (2.3%) had a stroke, 2 with large (≥ 4 mm) fenestrations. Of 38 fenestrations, 32 (84%) closed spontaneously by 1 year. No protein-losing enteropathy occurred. Most patients (118 of 121) were in New York Heart Association class I/II 4.5 years postoperatively. By multivariate analysis, only Down's syndrome (p < 0.001) predicted early mortality, whereas both Down's syndrome and a systemic right ventricle decreased late survival (p < 0.006). Conclusions. Proper selection of patients modifications of the Fontan procedure resulted in excellent early and late survival with a low incidence of atrial dysrhythmia and stroke. Midterm functional outcomes were excellent. (C) 2000 by The Society of Thoracic Surgeons. AD - K. Bando, Department of Cardiovascular Surgery, National Cardiovascular Center, 5-7-1, Fujishirodai, Suita, Osaka 565-8565, Japan AU - Bando, K. AU - Turrentine, M. W. AU - Park, H. J. AU - Sharp, T. G. AU - Scavo, V. AU - Brown, J. W. DB - Embase Medline DO - 10.1016/S0003-4975(00)01316-3 IS - 6 KW - adolescent adult article child cyanotic heart disease Down syndrome Fontan procedure heart atrium arrhythmia heart single ventricle human infant major clinical study patient selection preoperative evaluation priority journal risk factor sternotomy cerebrovascular accident surgical approach surgical technique survival rate thromboembolism LA - English M3 - Article N1 - L30445536 2000-07-25 PY - 2000 SN - 0003-4975 SP - 1873-1879 ST - Evolution of the Fontan procedure in a single center T2 - Annals of Thoracic Surgery TI - Evolution of the Fontan procedure in a single center UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L30445536 http://dx.doi.org/10.1016/S0003-4975(00)01316-3 VL - 69 ID - 1336 ER - TY - JOUR AB - The principles of Fontan conversion with arrhythmia surgery are to restore the cardiac anatomy by converting the original atriopulmonary connection to a total cavopulmonary artery extracardiac connection and treat the underlying atrial arrhythmias. Successful outcomes of this procedure are dependent on a thorough understanding of several factors: the patient's fundamental diagnosis of single-ventricle anatomy, the resultant cardiac configuration from the original atriopulmonary Fontan connection, right atrial dilatation that leads to atrial flutter or fibrillation, and associated congenital cardiac anomalies. The purpose of this article is to present some of the more challenging anatomic and electrophysiologic problems we have encountered with Fontan conversion and arrhythmia surgery and the innovative solutions we have used to treat them. The cases reviewed herein include: takedown of a Bjork-Fontan modification, right ventricular hypertension and tricuspid regurgitation after atriopulmonary Fontan for pulmonary atresia and intact ventricular septum, takedown of atrioventricular valve isolation patch for right-sided maze procedure, resultant hemodynamic considerations leading to intraoperative pulmonary vein stenosis after Fontan conversion, unwanted inferior vena cava retraction during the extracardiac connection, right atrial cannulation in the presence of a right atrial clot, distended left superior vena cava causing left pulmonary vein stenosis, dropped atrial septum, and the modified right-sided maze procedure for various single-ventricle pathology. Since 1994 we have performed Fontan conversion with arrhythmia surgery on 109 patients with a 0.9% mortality rate. We attribute our program's success in no small measure to the strong collaborative efforts of the cardiothoracic surgery and cardiology teams. © 2007 Elsevier Inc. All rights reserved. AD - Divisions of Cardiovascular-Thoracic Surgery and Pediatric Cardiology, The Children's Memorial Hospital, Northwestern University Medical School, Chicago, IL, United States AU - Mavroudis, C. AU - Backer, C. L. AU - Deal, B. J. AU - Stewart, R. D. AU - Franklin, W. H. AU - Tsao, S. AU - Ward, K. DB - Scopus DO - 10.1053/j.pcsu.2007.01.018 IS - 1 KW - arrhythmia Fontan maze procedure single ventricle M3 - Article N1 - Cited By :29 Export Date: 15 June 2020 PY - 2007 SP - 136-145 ST - Evolving Anatomic and Electrophysiologic Considerations Associated With Fontan Conversion T2 - Pediatric Cardiac Surgery Annual TI - Evolving Anatomic and Electrophysiologic Considerations Associated With Fontan Conversion UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-34047275772&doi=10.1053%2fj.pcsu.2007.01.018&partnerID=40&md5=bfc3d3b7d064948a2007e0e703885cb3 VL - 10 ID - 2212 ER - TY - JOUR AD - McGill Adult Unit for Congenital Heart Disease Excellence (MAUDE Unit), McGill University Health Centre, Montreal, H4A 3J1, Canada Inserm-UMRS 1138 Team 22, Cordeliers Research Centre, Paris Descartes University, Paris, 75006, France AU - Cohen, S. AU - Marelli, A. DB - Scopus DO - 10.1016/j.acvd.2016.05.001 IS - 10 KW - Congenital heart disease Heart failure Heart transplantation M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2016 SP - 511-513 ST - Évolution et perspectives de la transplantation cardiaque aux différents âges de la vie : une population grandissante d'adultes avec cardiopathie congénitale T2 - Archives of Cardiovascular Diseases TI - Evolving heart transplantation across the lifespan: A growing population of adults with congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84994157024&doi=10.1016%2fj.acvd.2016.05.001&partnerID=40&md5=e9f0ae3f5c57ded8cc4491e0a050558d VL - 109 ID - 1889 ER - TY - JOUR AB - OBJECTIVES: To assess the general health and activity levels of 4- and 5-year-old children after intervention for congenital cardiac disease. METHODS: Health behaviour outcomes were assessed in 91 children who had surgery or catheter intervention for congenital cardiac disease. The children were classified into four groups according to severity. The main parameters of classification were the presence of residual symptoms, frequency of visits to general practitioner or the Accident and Emergency Department, and ability to participate in physical activity according to a calculated "activity score". RESULTS: Children had very few residual symptoms after "corrective surgery". Those with complex congenital cardiac disease post-Fontan-type repair still had symptoms on average 18.2 days per month. Surprisingly, the complex group had fewer days "sick" from non-cardiac causes and had fewer visits to general practitioner or Accident and Emergency Departments. Regression analysis indicates that three variables had significant relevance to the general practitioner or Accident and Emergency visits: complex congenital cardiac disease, fewer visits; Townsend score - more deprivation - more visits; and maternal worry - higher maternal worry score - more visits. Regression analysis indicates that lower activity score is significantly related to complex cardiac disease and higher maternal worry score. CONCLUSIONS: The majority of this group of 4- and 5-year-old children had few residual symptoms and had good exercise tolerance. Maternal worry is a significant factor in influencing both activity levels and frequency of unscheduled health service demands - general practitioner or Accident and Emergency visits. AD - Department of Paediatric Cardiology, The Royal Belfast Hospital for Sick children, Northern Ireland, United Kingdom. frank.casey@belfasttrust.hscni.net AN - 20519053 AU - Casey, F. A. AU - Stewart, M. AU - McCusker, C. G. AU - Morrison, M. L. AU - Molloy, B. AU - Doherty, N. AU - Craig, B. G. AU - Sands, A. J. AU - Rooney, N. AU - Mulholland, H. C. DA - Oct DB - PubMed DO - 10.1017/s1047951110000673 DP - NLM ET - 2010/06/04 IS - 5 KW - Child, Preschool Exercise Tolerance/*physiology Female Follow-Up Studies *Health Behavior Heart Defects, Congenital/physiopathology/*psychology Humans Male Motor Activity/*physiology Preoperative Care Prognosis LA - eng N1 - 1467-1107 Casey, Frank A Stewart, M McCusker, C G Morrison, M L Molloy, B Doherty, N Craig, B G Sands, A J Rooney, N Mulholland, H C Comparative Study Journal Article Research Support, Non-U.S. Gov't England Cardiol Young. 2010 Oct;20(5):532-7. doi: 10.1017/S1047951110000673. Epub 2010 Jun 2. PY - 2010 SN - 1047-9511 SP - 532-7 ST - Examination of the physical and psychosocial determinants of health behaviour in 4-5-year-old children with congenital cardiac disease T2 - Cardiol Young TI - Examination of the physical and psychosocial determinants of health behaviour in 4-5-year-old children with congenital cardiac disease VL - 20 ID - 269 ER - TY - JOUR AB - OBJECTIVES: To assess the general health and activity levels of 4- and 5-year-old children after intervention for congenital cardiac disease. METHODS: Health behaviour outcomes were assessed in 91 children who had surgery or catheter intervention for congenital cardiac disease. The children were classified into four groups according to severity. The main parameters of classification were the presence of residual symptoms, frequency of visits to general practitioner or the Accident and Emergency Department, and ability to participate in physical activity according to a calculated 'activity score'. RESULTS: Children had very few residual symptoms after 'corrective surgery'. Those with complex congenital cardiac disease post-Fontan-type repair still had symptoms on average 18.2 days per month. Surprisingly, the complex group had fewer days 'sick' from non-cardiac causes and had fewer visits to general practitioner or Accident and Emergency Departments. Regression analysis indicates that three variables had significant relevance to the general practitioner or Accident and Emergency visits: complex congenital cardiac disease, fewer visits; Townsend score - more deprivation - more visits; and maternal worry - higher maternal worry score - more visits. Regression analysis indicates that lower activity score is significantly related to complex cardiac disease and higher maternal worry score. CONCLUSIONS: The majority of this group of 4- and 5-year-old children had few residual symptoms and had good exercise tolerance. Maternal worry is a significant factor in influencing both activity levels and frequency of unscheduled health service demands - general practitioner or Accident and Emergency visits. AD - 1Department of Paediatric Cardiology, The Royal Belfast Hospital for Sick children, Belfast, Northern Ireland, United Kingdom. AN - 104919503. Language: English. Entry Date: 20110204. Revision Date: 20160531. Publication Type: Journal Article AU - Casey, F. A. AU - Stewart, M. AU - McCusker, C. G. AU - Morrison, M. L. AU - Molloy, B. AU - Doherty, N. AU - Craig, B. G. AU - Sands, A. J. AU - Rooney, N. AU - Mulholland, H. C. DB - ccm DO - 10.1017/S1047951110000673 DP - EBSCOhost IS - 1 KW - Exercise Tolerance -- Physiology Health Behavior Heart Defects, Congenital -- Psychosocial Factors Motor Activity -- Physiology Child, Preschool Female Prospective Studies Heart Defects, Congenital -- Physiopathology Human Male Preoperative Care Prognosis N1 - research. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Special Interest: Pediatric Care. NLM UID: 9200019. PMID: NLM20519053. PY - 2011 SN - 1047-9511 SP - 532-537 ST - Examination of the physical and psychosocial determinants of health behaviour in 4-5-year-old children with congenital cardiac disease T2 - Cardiology in the Young TI - Examination of the physical and psychosocial determinants of health behaviour in 4-5-year-old children with congenital cardiac disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104919503&site=ehost-live&scope=site VL - 21 ID - 1579 ER - TY - JOUR AB - Background: Although interstage mortality for infants with hypoplastic left heart syndrome has declined within the National Pediatric Cardiology Quality Improvement Collaborative, variation across centres persists. It remains unclear whether centres with lower interstage mortality have lower-risk patients or whether differences in care may explain this variation. We examined previously established risk factors across National Pediatric Cardiology Quality Improvement Collaborative centres with lower and higher interstage mortality rates.Methods: Lower-mortality centres were defined as those with >25 consecutive interstage survivors. Higher-mortality centres were defined as those with cumulative interstage mortality rates >10%, which is a collaborative historic baseline rate. Baseline risk factors and perioperative characteristics were compared.Results: Seven lower-mortality centres were identified (n=331 patients) and had an interstage mortality rate of 2.7%, as compared with 13.3% in the four higher-mortality centres (n=173 patients, p<0.0001). Of all baseline risk factors examined, the only factor that differed between the lower- and higher-mortality centres was postnatal diagnosis (18.4 versus 31.8%, p=0.001). In multivariable analysis, there remained a significant mortality difference between the two groups of centres after adjusting for this variable: adjusted mortality rate was 2.8% in lower-mortality centres compared with 12.6% in higher-mortality centres, p=0.003. Secondary analyses identified multiple differences between groups in perioperative practices and other variables.Conclusions: Variation in interstage mortality rates between these two groups of centres does not appear to be explained by differences in baseline risk factors. Further study is necessary to evaluate variation in care practices to identify targets for improvement efforts. AD - Department of Pediatrics and Communicable Diseases, Congenital Heart Center, C.S. Mott Children’s Hospital, Ann Arbor, MI, USA Department of Cardiology, Boston Children’s Hospital, Boston, MA, USA Department of Surgery, Division of Cardiothoracic Surgery, St. Louis Children’s Hospital, St. Louis, MO, USA Department of Cardiac Surgery, University of Michigan Medical School, Ann Arbor, MI, USA AN - 130887320. Language: English. Entry Date: 20181111. Revision Date: 20190801. Publication Type: journal article AU - Bates, Katherine E. AU - Yu, Sunkyung AU - Lowery, Ray AU - Pasquali, Sara K. AU - Brown, David W. AU - Manning, Peter B. AU - Uzark, Karen DB - ccm DO - 10.1017/S1047951118000926 DP - EBSCOhost IS - 8 KW - Quality Improvement -- Administration Critical Care -- Standards Perioperative Care -- Standards Hypoplastic Left Heart Syndrome -- Mortality Cardiology Female Infant Infant, Newborn United States Cooperative Behavior Data Collection Treatment Outcomes Multivariate Analysis Male Risk Factors Hypoplastic Left Heart Syndrome -- Surgery Logistic Regression Human Validation Studies Comparative Studies Evaluation Research Multicenter Studies N1 - research. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. NLM UID: 9200019. PMID: NLM29925455. PY - 2018 SN - 1047-9511 SP - 1031-1036 ST - Examining variation in interstage mortality rates across the National Pediatric Cardiology Quality Improvement Collaborative: do lower-mortality centres have lower-risk patients? T2 - Cardiology in the Young TI - Examining variation in interstage mortality rates across the National Pediatric Cardiology Quality Improvement Collaborative: do lower-mortality centres have lower-risk patients? UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=130887320&site=ehost-live&scope=site VL - 28 ID - 1460 ER - TY - JOUR AB - OBJECTIVE: To test a model to predict psychosocial quality of life (QOL) in children with congenital heart disease (CHD) via executive dysfunction. STUDY DESIGN: Parents of 91 children with CHD requiring surgery in the first year of life completed questionnaires by mail or as part of their cardiology clinic visit. Latent class analysis identified 2 groups of patients with different likelihoods of executive dysfunction. Select medical and demographic characteristics and executive dysfunction group membership were evaluated as predictors of QOL using structural equation modeling. RESULTS: In children with CHD, aortic obstruction, male sex, and premature birth predicted worse executive function, explaining 59% of the variance. Structural equation modeling results indicated that executive dysfunction plays an important mediating role, through which CHD with aortic obstruction, male sex, and premature birth indirectly affect psychosocial QOL. Neurologic abnormalities and single-ventricle CHD did not significantly predict executive dysfunction or QOL. CONCLUSIONS: Executive dysfunction is a strong predictor of psychosocial QOL at school age. Select medical and demographic risk factors did not directly predict QOL at school age in CHD; however, aortic obstruction, premature birth, and male sex impacted QOL indirectly by contributing to executive dysfunction. These findings suggest important risk factors for executive dysfunction that can be monitored, allowing for provision of early supports for executive skills development in an effort to improve long term psychosocial QOL in at-risk children with CHD. AD - Division of Neuropsychology, Children's National Health System, Washington, DC; Department of Psychiatry and Behavioral Sciences at The George Washington University School of Medicine, Washington, DC; Department of Pediatrics at The George Washington University School of Medicine, Washington, DC. Electronic address: jsanz@childrensnational.org. Biostatistics, Children's National Health System, Washington, DC; Research Professor of Epidemiology and Biostatistics, George Washington University, Washington, DC. Division of Neuropsychology, Children's National Health System, Washington, DC; Department of Psychiatry and Behavioral Sciences at The George Washington University School of Medicine, Washington, DC; Department of Pediatrics at The George Washington University School of Medicine, Washington, DC. Division of Neuropsychology, Children's National Health System, Washington, DC. Biostatistics, Children's National Health System, Washington, DC. Department of Pediatrics at The George Washington University School of Medicine, Washington, DC; Division of Cardiology, Children's National Health System, Washington, DC. AN - 30243535 AU - Sanz, J. H. AU - Wang, J. AU - Berl, M. M. AU - Armour, A. C. AU - Cheng, Y. I. AU - Donofrio, M. T. DA - Nov DB - PubMed DO - 10.1016/j.jpeds.2018.07.018 DP - NLM ET - 2018/09/24 KW - Adolescent Arterial Occlusive Diseases/physiopathology Child Executive Function/*physiology Female Heart Defects, Congenital/classification/*physiopathology/*psychology Humans Male Models, Statistical Parents/psychology Pregnancy Premature Birth *Quality of Life Sex Factors Stress, Psychological Surveys and Questionnaires *congenital heart defects *executive function *neurodevelopmental outcome LA - eng N1 - 1097-6833 Sanz, Jacqueline H Wang, Jichuan Berl, Madison M Armour, Anna C Cheng, Yao I Donofrio, Mary T U54 HD090257/HD/NICHD NIH HHS/United States Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't United States J Pediatr. 2018 Nov;202:63-69. doi: 10.1016/j.jpeds.2018.07.018. Epub 2018 Sep 19. PY - 2018 SN - 0022-3476 SP - 63-69 ST - Executive Function and Psychosocial Quality of Life in School Age Children with Congenital Heart Disease T2 - J Pediatr TI - Executive Function and Psychosocial Quality of Life in School Age Children with Congenital Heart Disease VL - 202 ID - 15 ER - TY - JOUR AB - Children and adolescents with critical cyanotic congenital heart disease (CHD) are at risk for deficits in aspects of executive function (EF). The primary aim of this investigation was to compare EF outcomes in three groups of children/adolescents with severe CHD and controls (ages 10-19 years). Participants included 463 children/adolescents with CHD [dextro-transposition of the great arteries (TGA), n=139; tetralogy of Fallot (TOF), n=68; and, single-ventricle anatomy requiring Fontan procedure (SVF), n=145] and 111 controls, who underwent laboratory and informant-based evaluation of EF skills. Rates of EF impairment on D-KEFS measures were nearly twice as high for CHD groups (75-81%) than controls (43%). Distinct EF profiles were documented between CHD groups on D-KEFS tasks. Deficits in flexibility/problem-solving and verbally mediated EF skills were documented in all three CHD groups; visuo-spatially mediated EF abilities were impaired in TOF and SVF groups, but preserved in TGA. Parent, teacher, and self-report ratings on the BRIEF highlighted unique patterns of metacognitive and self-regulatory concerns across informants. CHD poses a serious threat to EF development. Greater severity of CHD is associated with worse EF outcomes. With increased understanding of the cognitive and self-regulatory vulnerabilities experienced by children and adolescents with CHD, it may be possible to identify risks early and provide individualized supports to promote optimal neurodevelopment. AD - 1Department of Psychiatry,Boston Children's Hospital,Harvard Medical School,Boston,Massachusetts. 2Department of Cardiology,Boston Children's Hospital,Harvard Medical School,Boston,Massachusetts. AN - 25487044 AU - Cassidy, A. R. AU - White, M. T. AU - DeMaso, D. R. AU - Newburger, J. W. AU - Bellinger, D. C. C2 - PMC4762262 C6 - NIHMS757331 DA - Jan DB - PubMed DO - 10.1017/s1355617714001027 DP - NLM ET - 2014/12/10 IS - 1 KW - Adolescent Analysis of Variance Child Cognition Disorders/diagnosis/*etiology Executive Function/*physiology Female Heart Defects, Congenital/*complications Humans Male Neuropsychological Tests Photic Stimulation Space Perception Surveys and Questionnaires Young Adult BRIEF: Behavior Rating Inventory of Executive Function. CHD: congenital heart disease Congenital heart defects D-KEFS: Delis-Kaplan Executive Function System Executive function Fontan procedure HLHS: hypoplastic left heart syndrome SVF: single-ventricle cardiac conditions requiring Fontan procedure Single-ventricle TGA: dextro-transposition of the great arteries TOF: tetralogy of Fallot Tetralogy of Fallot Transposition of great vessels LA - eng N1 - 1469-7661 Cassidy, Adam R White, Matthew T DeMaso, David R Newburger, Jane W Bellinger, David C R01 HL077681/HL/NHLBI NIH HHS/United States HL77681/HL/NHLBI NIH HHS/United States M01 RR002172/RR/NCRR NIH HHS/United States RR02172/RR/NCRR NIH HHS/United States P50 HL074734/HL/NHLBI NIH HHS/United States HL74734/HL/NHLBI NIH HHS/United States HL096825/HL/NHLBI NIH HHS/United States R01 HL096825/HL/NHLBI NIH HHS/United States Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't J Int Neuropsychol Soc. 2015 Jan;21(1):34-49. doi: 10.1017/S1355617714001027. Epub 2014 Dec 9. PY - 2015 SN - 1355-6177 (Print) 1355-6177 SP - 34-49 ST - Executive Function in Children and Adolescents with Critical Cyanotic Congenital Heart Disease T2 - J Int Neuropsychol Soc TI - Executive Function in Children and Adolescents with Critical Cyanotic Congenital Heart Disease VL - 21 ID - 119 ER - TY - JOUR AB - OBJECTIVE: To investigate the presence and severity of real-world impairments in executive functioning-responsible for children's regulatory skills (metacognition, behavioral regulation)-and its potential impact on school performance among pediatric survivors of complex congenital heart disease (CHD). STUDY DESIGN: Survivors of complex CHD aged 8-16 years (n = 143) and their parents/guardians from a regional CHD survivor registry participated (81% participation rate). Parents completed proxy measures of executive functioning, school competency, and school-related quality of life (QOL). Patients also completed a measure of school QOL and underwent IQ testing. Patients were categorized into 2 groups based on heart lesion complexity: 2-ventricle or single-ventricle. RESULTS: Survivors of complex CHD performed significantly worse than norms for executive functioning, IQ, school competency, and school QOL. Metacognition was more severely affected than behavioral regulation, and metacognitive deficits were more often present in older children. Even after taking into account demographic factors, disease severity, and IQ, metacognition uniquely and strongly predicted poorer school performance. In exploratory analyses, patients with single-ventricle lesions were rated as having lower school competency and school QOL, and patients with 2-ventricle lesions were rated as having poorer behavioral regulation. CONCLUSIONS: Survivors of complex CHD experience greater executive functioning difficulties than healthy peers, with metacognition particularly impacted and particularly relevant for day-to-day school performance. Especially in older children, clinicians should watch for metacognitive deficits, such as problems with organization, planning, self-monitoring, and follow-through on tasks. AD - Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH; Division of Behavioral Medicine and Clinical Psychology, Cincinnati Children's Hospital Medical Center (CCHMC), Cincinnati, OH. Electronic address: melissa.gerstle@cchmc.org. Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH; Division of Behavioral Medicine and Clinical Psychology, Cincinnati Children's Hospital Medical Center (CCHMC), Cincinnati, OH. Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH; Department of Sociology, McMicken College of Arts and Science, University of Cincinnati, Cincinnati, OH. Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, IL; Divisions of Cardiology and Critical Care Medicine, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL. AN - 26875011 AU - Gerstle, M. AU - Beebe, D. W. AU - Drotar, D. AU - Cassedy, A. AU - Marino, B. S. C2 - PMC4884495 C6 - NIHMS751433 DA - Jun DB - PubMed DO - 10.1016/j.jpeds.2016.01.028 DP - NLM ET - 2016/02/15 KW - Adolescent Child Cross-Sectional Studies *Educational Status Executive Function/*physiology Female Heart Defects, Congenital/*physiopathology Humans Intelligence Tests Male Quality of Life Survivors *cardiac *child *cognitive *neuropsychology *scholastic LA - eng N1 - 1097-6833 Gerstle, Melissa Beebe, Dean W Drotar, Dennis Cassedy, Amy Marino, Bradley S UL1 RR026314/RR/NCRR NIH HHS/United States UL1 TR001425/TR/NCATS NIH HHS/United States Journal Article Research Support, N.I.H., Extramural J Pediatr. 2016 Jun;173:154-9. doi: 10.1016/j.jpeds.2016.01.028. Epub 2016 Feb 11. PY - 2016 SN - 0022-3476 (Print) 0022-3476 SP - 154-9 ST - Executive Functioning and School Performance among Pediatric Survivors of Complex Congenital Heart Disease T2 - J Pediatr TI - Executive Functioning and School Performance among Pediatric Survivors of Complex Congenital Heart Disease VL - 173 ID - 84 ER - TY - JOUR AB - This study sought to determine whether exercise capacity, self-efficacy, and gross motor skills are associated with moderate-to-vigorous physical activity (MVPA) levels in children, and if these associations differ by congenital heart disease (CHD) type. Medical history was abstracted from chart review. We assessed MVPA levels (via accelerometry), percent-predicted peak oxygen consumption (VO2; cardiopulmonary exercise test), gross motor skill percentiles (test of gross motor development version-2), and self-efficacy [children’s self-perceptions of adequacy and predilection for physical activity scale (CSAPPA scale)]. CHD patients (n = 137, range 4–12 years) included children with a repaired atrial septal defect (n = 31, mean ± standard deviation MVPA = 454 ± 246 min/week), transposition of the great arteries after the arterial switch operation (n = 34, MVPA = 423 ± 196 min/week), tetralogy of Fallot after primary repair (n = 37, MVPA = 389 ± 211 min/week), or single ventricle after the Fontan procedure (n = 35, MVPA = 405 ± 256 min/week). MVPA did not differ significantly between CHD groups (p = 0.68). Higher MVPA was associated with a higher percent-predicted VO2 (EST[95% CI] = 16.9[−0.2, 34] MVPA min/week per 10% increase in percent-predicted VO2,p = 0.05) and higher self-efficacy (EST[95% CI] = 5.2[1.0, 9.3] MVPA min/week per 1-unit increase in CSAPPA score, p = 0.02), after adjustment for age, sex, and testing seasonality, with no association with CHD type. Higher MVPA was not associated with gross motor skill percentile (p = 0.92). There were no significant interactions between CHD type and percent-predicted VO2, self-efficacy scores, and gross motor skill percentiles regarding their association with MVPA (p > 0.05 for all). Greater MVPA was associated with higher exercise capacity and self-efficacy, but not gross motor skills. © 2017, Springer Science+Business Media New York. AD - Labatt Family Heart Centre, The Hospital for Sick Children, 555 University Avenue, Toronto, ON M5G 1X8, Canada Children’s Hospital of Eastern Ontario Research Institute, Ottawa, ON, Canada Department of Pediatrics, University of Toronto, Toronto, ON, Canada AU - Banks, L. AU - Rosenthal, S. AU - Manlhiot, C. AU - Fan, C. P. S. AU - McKillop, A. AU - Longmuir, P. E. AU - McCrindle, B. W. DB - Scopus DO - 10.1007/s00246-017-1645-2 IS - 6 KW - Accelerometry Gross motor skill development Peak oxygen consumption Self-efficacy M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2017 SP - 1206-1214 ST - Exercise Capacity and Self-Efficacy are Associated with Moderate-to-Vigorous Intensity Physical Activity in Children with Congenital Heart Disease T2 - Pediatric Cardiology TI - Exercise Capacity and Self-Efficacy are Associated with Moderate-to-Vigorous Intensity Physical Activity in Children with Congenital Heart Disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85020693578&doi=10.1007%2fs00246-017-1645-2&partnerID=40&md5=9e2fab7b88707ab0fb48b1113a07f66b VL - 38 ID - 1846 ER - TY - JOUR AB - This study sought to determine whether exercise capacity, self-efficacy, and gross motor skills are associated with moderate-to-vigorous physical activity (MVPA) levels in children, and if these associations differ by congenital heart disease (CHD) type. Medical history was abstracted from chart review. We assessed MVPA levels (via accelerometry), percent-predicted peak oxygen consumption ([Formula: see text] cardiopulmonary exercise test), gross motor skill percentiles (test of gross motor development version-2), and self-efficacy [children's self-perceptions of adequacy and predilection for physical activity scale (CSAPPA scale)]. CHD patients (n = 137, range 4-12 years) included children with a repaired atrial septal defect (n = 31, mean ± standard deviation MVPA = 454 ± 246 min/week), transposition of the great arteries after the arterial switch operation (n = 34, MVPA = 423 ± 196 min/week), tetralogy of Fallot after primary repair (n = 37, MVPA = 389 ± 211 min/week), or single ventricle after the Fontan procedure (n = 35, MVPA = 405 ± 256 min/week). MVPA did not differ significantly between CHD groups (p = 0.68). Higher MVPA was associated with a higher percent-predicted [Formula: see text] (EST[95% CI] = 16.9[-0.2, 34] MVPA min/week per 10% increase in percent-predicted [Formula: see text] p = 0.05) and higher self-efficacy (EST[95% CI] = 5.2[1.0, 9.3] MVPA min/week per 1-unit increase in CSAPPA score, p = 0.02), after adjustment for age, sex, and testing seasonality, with no association with CHD type. Higher MVPA was not associated with gross motor skill percentile (p = 0.92). There were no significant interactions between CHD type and percent-predicted [Formula: see text] self-efficacy scores, and gross motor skill percentiles regarding their association with MVPA (p > 0.05 for all). Greater MVPA was associated with higher exercise capacity and self-efficacy, but not gross motor skills. AD - Labatt Family Heart Centre, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada. Children's Hospital of Eastern Ontario Research Institute, Ottawa, ON, Canada. Labatt Family Heart Centre, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada. brian.mccrindle@sickkids.ca. Department of Pediatrics, University of Toronto, Toronto, ON, Canada. brian.mccrindle@sickkids.ca. AN - 28608149 AU - Banks, L. AU - Rosenthal, S. AU - Manlhiot, C. AU - Fan, C. S. AU - McKillop, A. AU - Longmuir, P. E. AU - McCrindle, B. W. DA - Aug DB - PubMed DO - 10.1007/s00246-017-1645-2 DP - NLM ET - 2017/06/14 IS - 6 KW - Accelerometry Child Child, Preschool Exercise/*physiology/*psychology Exercise Test *Exercise Tolerance Female Heart Defects, Congenital/*physiopathology/*psychology Humans Male Motor Skills *Self Efficacy Surveys and Questionnaires Task Performance and Analysis Gross motor skill development Peak oxygen consumption Self-efficacy LA - eng N1 - 1432-1971 Banks, Laura Rosenthal, Shelly Manlhiot, Cedric Fan, Chun-Po Steve McKillop, Adam Longmuir, Patricia E McCrindle, Brian W Journal Article United States Pediatr Cardiol. 2017 Aug;38(6):1206-1214. doi: 10.1007/s00246-017-1645-2. Epub 2017 Jun 12. PY - 2017 SN - 0172-0643 SP - 1206-1214 ST - Exercise Capacity and Self-Efficacy are Associated with Moderate-to-Vigorous Intensity Physical Activity in Children with Congenital Heart Disease T2 - Pediatr Cardiol TI - Exercise Capacity and Self-Efficacy are Associated with Moderate-to-Vigorous Intensity Physical Activity in Children with Congenital Heart Disease VL - 38 ID - 303 ER - TY - JOUR AB - OBJECTIVE: Elevated energy loss in the total cavopulmonary connection (TCPC) is hypothesised to have a detrimental effect on clinical outcomes in single-ventricle physiology, which may be magnified with exercise. This study investigates the relationship between TCPC haemodynamic energy dissipation and exercise performance in single-ventricle patients. METHODS: Thirty consecutive Fontan patients with TCPC and standard metabolic exercise testing were included. Specific anatomies and flow rates at rest and exercise were obtained from cardiac MR (CMR) and phase-encoded velocity mapping. Exercise CMR images were acquired immediately following supine lower limb exercise using a CMR-compatible cycle ergometer. Computational fluid dynamics simulations were performed to determine power loss of the TCPC anatomies using in vivo anatomies and measured flows. RESULTS: A significant negative linear correlation was observed between indexed power loss at exercise and (a) minute oxygen consumption (r=-0.60, p<0.0005) and (b) work (r=-0.62, p<0.0005) at anaerobic threshold. As cardiac output increased during exercise, indexed power loss increased in an exponential fashion (y=0.9671x(3.0263), p<0.0001). CONCLUSIONS: This is the first study to demonstrate the relationship between power loss and exercise performance with the TCPC being one of the few modifiable factors to allow for improved quality of life. These results suggest that aerobic exercise tolerance in Fontan patients may, in part, be a consequence of TCPC power loss. AD - Wallace H. Coulter Department of Biomedical Engineering, Georgia Institute of Technology & Emory University, Atlanta, Georgia, USA. Division of Cardiology and Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA. Westat, Rockville, Maryland, USA. AN - 25184826 AU - Khiabani, R. H. AU - Whitehead, K. K. AU - Han, D. AU - Restrepo, M. AU - Tang, E. AU - Bethel, J. AU - Paridon, S. M. AU - Fogel, M. A. AU - Yoganathan, A. P. DA - Jan DB - PubMed DO - 10.1136/heartjnl-2014-306337 DP - NLM ET - 2014/09/04 IS - 2 KW - Adolescent Adult Blood Flow Velocity Cardiac Output Disease Management Exercise Test/methods Exercise Therapy/methods *Exercise Tolerance Female *Fontan Procedure/methods/psychology/rehabilitation *Heart Defects, Congenital/diagnosis/metabolism/physiopathology/surgery Heart Ventricles/*abnormalities Hemodynamics Humans Male Patient-Specific Modeling Pennsylvania *Physical Exertion *Postoperative Complications/diagnosis/physiopathology/prevention & control *Quality of Life Congenital heart disease LA - eng N1 - 1468-201x Khiabani, Reza H Whitehead, Kevin K Han, David Restrepo, Maria Tang, Elaine Bethel, James Paridon, Stephen M Fogel, Mark A Yoganathan, Ajit P HL089647/HL/NHLBI NIH HHS/United States HL098252/HL/NHLBI NIH HHS/United States HL67622/HL/NHLBI NIH HHS/United States Journal Article Research Support, N.I.H., Extramural England Heart. 2015 Jan;101(2):139-43. doi: 10.1136/heartjnl-2014-306337. Epub 2014 Sep 2. PY - 2015 SN - 1355-6037 SP - 139-43 ST - Exercise capacity in single-ventricle patients after Fontan correlates with haemodynamic energy loss in TCPC T2 - Heart TI - Exercise capacity in single-ventricle patients after Fontan correlates with haemodynamic energy loss in TCPC VL - 101 ID - 235 ER - TY - JOUR AB - AIMS: Patients with congenital heart disease usually show diminished exercise capacity and quality of life. However, there is only little information about daily activity, a marker for lifestyle, exercise capacity, and the prevention of arteriosclerosis. This study investigated exercise capacity, quality of life, daily activity, and their interaction with univentricular heart physiology after total cavopulmonary connection (TCPC). METHODS AND RESULTS: Fifty-seven patients (18 females, 39 males, age 8-52 years) after TCPC (lateral tunnel 28, extra-cardiac conduit 29) who underwent surgery during 1994-2001 were examined in our institution. They performed a symptom-limited cardiopulmonary exercise test. Those patients 14 years of age and older filled in the health-related quality-of-life questionnaire SF-36, and those who were 8-13 years of age, the CF-87. Daily activity parameters were obtained by using a triaxial accelerometer over the next three consecutive days. Exercise capacity was severely reduced after TCPC (25.0 mL/min/kg corresponding to 59.7% of age- and sex-related reference values). Daily activity was within the recommendations of the United Kingdom Expert Consensus Group (> or =60 min, > or =3 metabolic equivalent, > or =5 days/week) in 72% of the investigated patients. It was reduced in older patients (Spearman r = - 0.506, P < 0.001) and patients with a lower peak oxygen uptake (Spearman r = 0.432, P = 0.001). In children <14 years, mental health was related to daily activity. CONCLUSION: Despite their diminished exercise capacity, patients after TCPC show a fairly normal activity pattern. However, their activity depends not only on age, but also on exercise capacity, which, in contrast to healthy people, decreases already from early adolescence on. AD - Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Lazarettstr. 36, D-80636 München, Germany. muellerjan@dhm.mhn.de AN - 19692392 AU - Müller, J. AU - Christov, F. AU - Schreiber, C. AU - Hess, J. AU - Hager, A. DA - Dec DB - PubMed DO - 10.1093/eurheartj/ehp305 DP - NLM ET - 2009/08/21 IS - 23 KW - *Activities of Daily Living Adolescent Adult Child Epidemiologic Methods Exercise Test *Exercise Therapy Exercise Tolerance/physiology Female Heart/*physiology Heart Bypass, Right Heart Defects, Congenital/*rehabilitation/surgery Heart Ventricles/abnormalities Humans Male Middle Aged Postoperative Care *Quality of Life Young Adult LA - eng N1 - 1522-9645 Müller, Jan Christov, Florian Schreiber, Christian Hess, John Hager, Alfred Journal Article Research Support, Non-U.S. Gov't England Eur Heart J. 2009 Dec;30(23):2915-20. doi: 10.1093/eurheartj/ehp305. Epub 2009 Aug 18. PY - 2009 SN - 0195-668x SP - 2915-20 ST - Exercise capacity, quality of life, and daily activity in the long-term follow-up of patients with univentricular heart and total cavopulmonary connection T2 - Eur Heart J TI - Exercise capacity, quality of life, and daily activity in the long-term follow-up of patients with univentricular heart and total cavopulmonary connection VL - 30 ID - 251 ER - TY - JOUR AB - Single ventricle physiology and palliation via the Fontan operation lead to a series of cardiovascular changes. In addition, organs such as the kidneys and liver have been shown to experience insults and subsequent injury. This has led to routine surveillance of patients. We present findings from a small cohort of patients that was deeply phenotyped to illustrate the need for comprehensive evaluation. A cohort of four Fontan patients with fairly high cardiovascular function was recruited 5-10 years post-Fontan. Patients underwent a rigorous clinical work-up after which a research MRI scan was performed during which (I) data were obtained during exercise to evaluate changes in stroke volume during supine exercise and (II) magnetic resonance angiograms with phase-contrast images were obtained for computational modeling of flows through the Fontan circulation at rest. Clinical measures were consistent with a fairly homogeneous high function cohort (peak oxygen consumption >20 mL/kg/min, robust response to exercise, peak ventilatory efficiency below levels associated with heart failure, MR-derived ejection fraction >50%). Liver evaluation did not reveal clear signs of cirrhosis or extensive fibrosis. However, we observed considerable variability (27-162%) in the increase in stroke index with exercise [100%±64% increase, 53.9±17.4 mL/beat m² (rest), 101.1±20.7 mL/beat m², (exercise)]. Computational flow modeling at rest in two patients also showed marked differences in flow distribution and shear stress. We report marked differences in both changes in stroke index during an exercise MRI protocol as well as computational flow patterns at rest suggesting different compensation strategies may be associated with high functioning Fontan patients. The observed heterogeneity illustrates the need for deep phenotyping to capture patient-specific adaptive mechanisms. AD - Department of Bioengineering, UC San Diego, La Jolla CA, USA Department of Radiology, UC San Diego, La Jolla CA, USA Department of Pediatrics, Stanford University, Palo Alto, CA, USA Department of Pediatric Cardiology, Rady Children's Hospital, San Diego, CA, USA Department of Medicine, UC San Diego, La Jolla, CA, USA Department of Pathology, UC San Diego, La Jolla, CA, USA Department of Bioengineering, Stanford University, Palo Alto, CA, USA Department of Society and Genetics, UC Los Angeles, Los Angeles, CA, USA AN - 142575983. Language: English. Entry Date: 20200413. Revision Date: 20200413. Publication Type: Article AU - Contijoch, Francisco AU - Bochao, Li AU - Weiguang, Yang AU - Silva-Sepulveda, Jose A. AU - Vodkin, Irine AU - Printz, Beth AU - Vavinskaya, Vera AU - Hegde, Sanjeet AU - Marsden, Alison AU - El-Sabrout, Hannah AU - Alshawabkeh, Laith AU - Moore, John W. AU - El-Said, Howaida DB - ccm DO - 10.21037/jtd.2019.09.59 DP - EBSCOhost IS - 3 KW - Magnetic Resonance Imaging Exercise Cardiovascular System Physiology Human Magnetic Resonance Angiography Oxygen Consumption Heart Failure Liver -- Physiology Phenotype Descriptive Statistics Blood Vessels -- Physiology T-Tests Male Female Blood Circulation Adolescence Young Adult Ventricular Ejection Fraction N1 - pictorial; research; tables/charts. Journal Subset: Asia; Biomedical; Peer Reviewed. NLM UID: 101533916. PY - 2020 SN - 2072-1439 SP - 1204-1212 ST - Exercise MRI highlights heterogeneity in cardiovascular mechanics among patients with Fontan circulation: proposed protocol for routine evaluation T2 - Journal of Thoracic Disease TI - Exercise MRI highlights heterogeneity in cardiovascular mechanics among patients with Fontan circulation: proposed protocol for routine evaluation UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=142575983&site=ehost-live&scope=site VL - 12 ID - 1429 ER - TY - JOUR AB - Background : The optimal timing, need for primary/staged procedure in patients undergoing univentricular palliation, is debatable. Aims : We performed this study to assess the exercise performance of patients undergoing various forms of univentricular palliation. Setting and Design : This was a retrospective, prospective comparative study conducted at a multispecialty tertiary referral center. Patients and Methods : Between January 2012 and June 2015, 117 patients undergoing either bidirectional Glenn (BDG) (n = 43) or Fontan (total cavopulmonary connection [TCPC]) (n = 74) underwent exercise testing. Statistical Analysis : Comparisons between subgroups for continuous data were made with Student's ttest if normally distributed and Wilcoxon ranksum test otherwise. Tests between subgroups for qualitative data were made with Pearson's Chisquare test. Results : Patients who underwent BDG with open antegrade pulmonary blood flow (APBF) had higher saturations (oxygen saturation [SpO2]) compared to those without it (87.5 ± 5.0% vs. 81.1 ± 4.8%; P = 0.0001). However, we found no differences in exercise parameters of patients undergoing BDG with or without APBF. Extracardiac TCPC (n = 42) patients demonstrated better exercise capacity (15.0 ± 7.7 vs. 11.2 ± 6.2 min; P = 0.02) and increased SpO2 on exercise (87.0 ± 8.0% vs. 83.4 ± 7.6%; P ≤ 0.05) compared to lateral tunnel TCPC (n = 32). Fenestrated TCPC (n = 30) patients had higher exercise capacity reflected by higher metabolic equivalents (METs) consumption (6.4 ± 2.3 vs. 5.2 ± 2.0 METs, P = 0.02), fewer pleural effusions (7.0 ± 3.2 vs. 9.2 ± 6.2 days, P ≤ 0.05), and lower hospital stay (9.5 ± 4.0 vs. 12.7 ± 7.7 days, P = 0.04) compared to nonfenestrated TCPC (n = 44) patients. Conclusions : We observed no differences in exercise parameters of patients undergoing BDG with or without APBF. Extracardiac TCPC patients had better exercise capacity but longer postoperative hospital stay and pleural effusions than patients with lateral tunnel Fontan. Fenestrated TCPC patients seemed to fare better than nonfenestrated ones. Patients undergoing TCPC had better exercise capacity than patients undergoing BDG alone. AD - S. Talwar, Department of Cardiothoracic and Vascular Surgery, Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India AU - Talwar, S. AU - Kumar, M. AU - Sreenivas, V. AU - Gupta, V. AU - Choudhary, S. AU - Airan, B. DB - Embase DO - 10.4103/apc.APC_43_17 IS - 1 KW - anxiety article cavopulmonary connection child chronotropism cohort analysis comparative study electrocardiography exercise female Fontan procedure heart rate heart single ventricle hospitalization human lung blood flow major clinical study male metabolic equivalent outpatient department oxygen saturation pleura effusion prospective study retrospective study tertiary care center LA - English M3 - Article N1 - L620388440 2018-02-02 2018-02-07 PY - 2018 SN - 0974-5149 0974-2069 SP - 40-47 ST - Exercise performance after univentricular palliation T2 - Annals of Pediatric Cardiology TI - Exercise performance after univentricular palliation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L620388440 http://dx.doi.org/10.4103/apc.APC_43_17 VL - 11 ID - 720 ER - TY - JOUR AB - It is well documented that children with a Fontan circulation have a reduced exercise capacity. One of the modalities to improve exercise capacity might be exercise training. We performed a systematic literature review on the effects of exercise training in patients with a Fontan circulation. Six published studies were included that reported on the effects of exercise training in 40 patients. All studies had a small sample size and/or did not include a control group.Based on the six published studies we can conclude that children who have undergone a Fontan operation and who are in a stable haemodynamic condition can safely participate in an exercise training programme and that exercise training results in an improved exercise capacity. However, more research is needed to establish the optimal exercise mode, dose-response relation, and the effects of exercise training on cardiac function, peripheral muscle function, physical activity, and health-related quality of life. (Neth Heart J 2007;15:142-7.). AD - Department of Paediatric Physical Therapy & Exercise Physiology, Wilhelmina Children’s Hospital, University Medical Centre, Utrecht, the Netherlands. AN - 17612674 AU - Takken, T. AU - Hulzebos, H. J. AU - Blank, A. C. AU - Tacken, M. H. AU - Helders, P. J. AU - Strengers, J. L. C2 - PMC1847768 DB - PubMed DO - 10.1007/bf03085970 DP - NLM ET - 2007/07/07 IS - 4 LA - eng N1 - Takken, T Hulzebos, H J Blank, A C Tacken, M H P Helders, P J M Strengers, J L M Journal Article Neth Heart J. 2007;15(4):142-7. doi: 10.1007/BF03085970. PY - 2007 SN - 1568-5888 (Print) 1568-5888 SP - 142-7 ST - Exercise prescription for patients with a Fontan circulation: current evidence and future directions T2 - Neth Heart J TI - Exercise prescription for patients with a Fontan circulation: current evidence and future directions VL - 15 ID - 324 ER - TY - JOUR AB - The object of our study was to analyze the results of bidirectional cavopulmonary anastomosis (BCPA) and modified Fontan operations (MFO) in patients with a functionally single ventricle and heterotaxy syndrome and to reveal risk factors for these surgical interventions. During 1983-2010, 681 patients underwent BCPA or MFO. Thirty-nine had heterotaxy syndrome. The median follow-up period after BCPA and MFO was nine and 1.5 years, respectively. Risk factors for lethal outcomes were determined by logistic regression analysis. Hospital mortality after BCPA and MFO was 7.9% and 12.5%, respectively and did not significantly differ from patients without heterotaxy. The most frequent hospital complications were heart failure, pleural effusions, and arrhythmias. Late mortality after BCPA and MFO was 8.7% and did not significantly differ from patients without heterotaxy. Late deaths were caused by congestive heart failure or pulmonary thromboembolism. The main non-lethal complication was arrhythmia. Patients have significantly improved their functional class at follow-up. The independent risk factor for lethal outcomes after BCPA and MFO was preoperative regurgitation at atrioventricular valves (P=0.012). BCPA and MFO in patients with a functionally single ventricle and heterotaxy syndrome allow to significantly improves their quality of life. Preoperative regurgitation at atrioventricular valves worsens surgical results. AD - Department of Congenital Heart Defects, Bakoulev Center for Cardiovascular Surgery, Moscow, Russia. AN - 21233261 AU - Yurlov, I. A. AU - Podzolkov, V. P. AU - Zelenikin, M. M. AU - Kovalev, D. V. AU - Babaev, G. K. AU - Putiato, N. A. AU - Zaets, S. B. DA - Apr DB - PubMed DO - 10.1510/icvts.2010.253567 DP - NLM ET - 2011/01/15 IS - 4 KW - *Abnormalities, Multiple Adolescent Adult Analysis of Variance Chi-Square Distribution Child Child, Preschool Dextrocardia/complications/mortality/physiopathology *Fontan Procedure/adverse effects/mortality Genetic Diseases, X-Linked/complications/mortality/physiopathology Heart Defects, Congenital/complications/mortality/physiopathology/*surgery Heart Ventricles/abnormalities/physiopathology/*surgery Heterotaxy Syndrome Hospital Mortality Humans Infant Kaplan-Meier Estimate Logistic Models Odds Ratio Recovery of Function Retrospective Studies Risk Assessment Risk Factors Russia Situs Inversus/complications/mortality/physiopathology Time Factors Treatment Outcome Young Adult LA - eng N1 - 1569-9285 Yurlov, Ivan A Podzolkov, Vladimir P Zelenikin, Mikhail M Kovalev, Dmitry V Babaev, Guvandg K Putiato, Neele A Zaets, Sergey B Comparative Study Journal Article England Interact Cardiovasc Thorac Surg. 2011 Apr;12(4):563-8. doi: 10.1510/icvts.2010.253567. Epub 2011 Jan 13. PY - 2011 SN - 1569-9285 SP - 563-8 ST - Experience with bidirectional cavopulmonary anastomosis and modified Fontan operation in patients with single ventricle and concomitant visceral heterotaxy T2 - Interact Cardiovasc Thorac Surg TI - Experience with bidirectional cavopulmonary anastomosis and modified Fontan operation in patients with single ventricle and concomitant visceral heterotaxy VL - 12 ID - 254 ER - TY - JOUR AB - Objective: To explore the need for information and what information was actually received following prenatal diagnosis of a congenital heart defect, in a country where termination of pregnancy beyond 22weeks of gestation is not easily possible because of legal constraints. Methods: Twenty-six Swedish-speaking pregnant women (n=14) and partners (n=12) were consecutively recruited for semi-structured telephone interviews following the prenatal diagnosis of a congenital heart defect. Data were analyzed using content analysis. Results: Although high satisfaction with the specialist information was described, the information was considered overwhelming and complex. Objective, honest, and detailed information about multiple subjects were needed, delivered repeatedly, and supplemented by written information/illustrations. Eighteen respondents had used the Internet to search for information and identified issues involving searching difficulties, low quality, and that it was too complex, insufficient, or unspecific. Those who terminated their pregnancy criticized that there was a lack of information about termination of pregnancy, both from health professionals and online sources, resulting in unanswered questions and unpreparedness. Conclusion: Individuals faced with a prenatal diagnosis of a congenital heart defect need individualized and repeated information. These needs are not all adequately met, as individuals are satisfied with the specialist consultation but left with unanswered questions regarding pregnancy termination. What's Already Known About This Topic? The introduction of ultrasound screening during the second trimester of pregnancy has increased the detection rate of major congenital heart defects. Information matched to individual needs is crucial for women to cope with the situation following a prenatal diagnosis. What Does This Study Add? There is a need for information on multiple subjects following the prenatal diagnosis of congenital heart defect, including termination of pregnancy. Although information is available on the Internet on congenital heart defects, it is not easily searchable and its quality may be less than desired. Apparently, there is insufficient information about termination of pregnancy following the prenatal diagnosis of congenital heart defect. © 2016 John Wiley & Sons, Ltd. AD - Department of Public Health and Caring Sciences, Uppsala University, Uppsala, Sweden Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden Department of Women's and Children's Health, Uppsala University, Uppsala, Sweden AU - Carlsson, T. AU - Bergman, G. AU - Wadensten, B. AU - Mattsson, E. DB - Scopus DO - 10.1002/pd.4815 IS - 6 M3 - Article N1 - Cited By :16 Export Date: 15 June 2020 PY - 2016 SP - 515-522 ST - Experiences of informational needs and received information following a prenatal diagnosis of congenital heart defect T2 - Prenatal Diagnosis TI - Experiences of informational needs and received information following a prenatal diagnosis of congenital heart defect UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84971525024&doi=10.1002%2fpd.4815&partnerID=40&md5=ab7d8e8bbcc7e27676044eebd31293db VL - 36 ID - 1904 ER - TY - JOUR AB - A viscous impeller pump (VIP) based on the Von Karman viscous pump is specifically designed to provide cavopulmonary assist in a univentricular Fontan circulation. The technology will make it possible to biventricularize the univentricular Fontan circulation. Ideally, it will reduce the number of surgeries required for Fontan conversion from three to one early in life, while simultaneously improving physiological conditions. Later in life, it will provide a currently unavailable means of chronic support for adolescent and adult patients with failing Fontan circulations. Computational fluid dynamics simulations demonstrate that the VIP can satisfactorily augment cavopulmonary blood flow in an idealized total cavopulmonary connection (TCPC). When the VIP is deployed at the TCPC intersection as a static device, it stabilizes the four-way flow pattern and is not obstructive to the flow. Experimental studies are carried out to assess performance, hemodynamic characteristics, and flow structures of the VIP in an idealized TCPC model. Stereoscopic particle image velocimetry is applied using index-matched blood analog. Results show excellent performance of the VIP without cavitation and with reduction of the energy losses. The non-rotating VIP smoothes and accelerates flow, and decreases stresses and turbulence in the TCPC. The rotating VIP generates the desired low-pressure Fontan flow augmentation (0-10 mmHg) while maintaining acceptable stress thresholds. © 2013 Springer-Verlag Berlin Heidelberg. AD - School of Mechanical Engineering, Purdue University, West Lafayette, IN 47907, United States Department of Bioengineering, University of Louisville, Louisville, KY 40292, United States Department of Surgery, Indiana University, School of Medicine, Indianapolis, IN 46202, United States AU - Kerlo, A. E. M. AU - Delorme, Y. T. AU - Xu, D. AU - Frankel, S. H. AU - Giridharan, G. A. AU - Rodefeld, M. D. AU - Chen, J. C7 - 1581 DB - Scopus DO - 10.1007/s00348-013-1581-8 IS - 8 M3 - Article N1 - Cited By :12 Export Date: 15 June 2020 PY - 2013 ST - Experimental characterization of powered Fontan hemodynamics in an idealized total cavopulmonary connection model T2 - Experiments in Fluids TI - Experimental characterization of powered Fontan hemodynamics in an idealized total cavopulmonary connection model UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84880448243&doi=10.1007%2fs00348-013-1581-8&partnerID=40&md5=326f0986b7cfe7f6295e19b28ddde365 VL - 54 ID - 2042 ER - TY - JOUR AB - Introduction. The Ability Index was developed to classify patients with congenital heart disease into four functional classes. Functional class is typically determined by the cardiologist, based on data from a clinical interview. The validity of the Ability Index as assessed by the patient has never been scrutinized. Objective. We tested the agreement between cardiologists and patients in assessing functional status using the Ability Index and compared the accuracy of the two assessments in explaining patient-reported outcomes (PRO). Methods. The Ability Index Scale was completed for 57 patients, independently by cardiologists and patients. Cohen's Kappa coefficient with quadratic weighting (Kw) was calculated. The area under the receiver operating characteristic (ROC) curve (AUC) (=C-index) was used to test the accuracy of the Ability Index in explaining PRO, as assessed by the cardiologist or the patient. Results. Agreement was observed in 61.4% of the patients. The Kw was 0.55, showing a moderate agreement; and the R2 was 0.29, displaying a limited shared variance. The AUC for cardiologists' assessments of the Ability Index in explaining PRO was consistently lower than the AUC for patients' assessments. The appraisal of the patients was more accurate. Discussion. When cardiologists and patients are assessing functional status using the Ability Index, two different constructs are measured. These assessments cannot be interchanged, but should be used complementarily. Because the assessment of the patients regarding the Ability Index is more precise in terms of explaining PRO, it could be valuable as a simple crude marker to identify patients at risk for poor functional and psychosocial outcomes. © 2012 Wiley Periodicals, Inc. AD - The Heart Center, Rigshospitalet, Copenhagen University Hospital, Denmark University College Metropol, Copenhagen, Denmark The Heart Center, Aarhus University Hospital, Skejby, Denmark Catholic University of Leuven, Center for Health Services and Nursing Research, Leuven, Belgium AU - Overgaard, D. AU - Schrader, A. M. AU - Lisby, K. H. AU - King, C. AU - Christensen, R. F. AU - Jensen, H. F. AU - Moons, P. DB - Scopus DO - 10.1111/j.1747-0803.2012.00675.x IS - 6 KW - Ability index Congenital heart disease Functional status Patient-Reported Outcomes (PRO) Single Ventricle Physiology (SVP) M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2012 SP - 559-564 ST - Explanatory Value of the Ability Index as Assessed by Cardiologists and Patients with Congenital Heart Disease T2 - Congenital Heart Disease TI - Explanatory Value of the Ability Index as Assessed by Cardiologists and Patients with Congenital Heart Disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84870336683&doi=10.1111%2fj.1747-0803.2012.00675.x&partnerID=40&md5=1df6ebd1b709bc384c247ecfff558137 VL - 7 ID - 2082 ER - TY - JOUR AB - Background: In general, the existing evidence points to a role for maternal sleep in pregnancy complications and fetal growth, however, little has been focused on birth defects. We aimed to explore the association between periconceptional poor sleep and the risk of congenital heart disease (CHD), and to examine if daytime napping could to some extent change the association. Methods: A case–control study was conducted in Shanghai Children's Medical Center, in which, a total of 524 cases (262 simple CHD vs. 262 severe CHD), along with 262 controls. Results: In the multivariable logistic analysis, poor sleep could increase the risk of both simple CHD (OR = 2.486, 95% CI = 1.619–3.818) and severe CHD (OR = 1.950, 95% CI = 1.269–2.997), while routine daytime nap could decrease risk of simple CHD (OR = 0.634, 95% CI = 0.435–0.923). In the stratified analysis, the concurrence with routine daytime nap could weaken the risk of simple CHD caused by poor sleep (OR = 3.183, 95% CI: 1.830–5.537 decreased to OR = 2.236, 95% CI: 1.200–4.165). The examinations were repeated in ventricular septal defect and tetralogy of Fallot, and the established associations can be verified. Moreover, all these findings were also similarly observed in both propensity-score-adjusted and propensity-score-matched analyses. Conclusions: Poor maternal sleep around periconceptional period seems to be an independent risk factor for CHD. The concurrence with daytime nap could to some extent reduce the risk in simple CHD. The results individually and collectively put forward the importance of maternal sleep in embryonic heart development. © 2019 The Authors. Birth Defects Research published by Wiley Periodicals, Inc. AD - School of Public Health, Shanghai Jiao Tong University School of Medicine, Shanghai, China Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China Shanghai Jiao Tong University School of Medicine, Shanghai, China AU - Zhao, A. AU - Zhao, K. AU - Xia, Y. AU - Yin, Y. AU - Zhu, J. AU - Hong, H. AU - Li, S. DB - Scopus DO - 10.1002/bdr2.1536 IS - 13 KW - case–control study congenital heart disease daytime nap maternal sleep pregnancy M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2019 SP - 920-931 ST - Exploring associations of maternal sleep during periconceptional period with congenital heart disease in offspring T2 - Birth Defects Research TI - Exploring associations of maternal sleep during periconceptional period with congenital heart disease in offspring UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85067466911&doi=10.1002%2fbdr2.1536&partnerID=40&md5=6090a8454df5f372c7e4fd085fd57b74 VL - 111 ID - 1777 ER - TY - JOUR AB - Background Examining seasonal patterns of birth defects may help to identify environmental risk factors. Because the teratogenic window for most birth defects is during gestational weeks 3 to 8, investigating exposures closer to the timing of conception is important. However, studies are usually based on month of birth, which is not the biologically relevant exposure period and does not account for differences in gestational length. We aimed to determine whether the occurrence of birth defects varied by month of conception using the population-based New York State Congenital Malformations Registry (CMR). Methods: We merged live birth certificates (n = 2,044,091) with CMR records for mothers residing in New York State, excluding New York City, for the years 1992 through 2006. We categorized birth defects according to the National Birth Defects Prevention Network guidelines and performed Cochran-Armitage trend, Hewitt-Rogerson, and Walter-Elwood tests on month of conception and chi-square tests on season of conception. We graphed seasonal distributions and seasonality test results. We performed stratified analyses by maternal and infant characteristics. Results: Of 42 groups examined in the 15-year period, 24 (57%) had at least one statistically significant test result, suggesting a trend or seasonal variation: Cochran-Armitage (18), Hewitt-Rogerson (17), Walter-Elwood (4), and chi-square (5). Ventricular septal defect showed the most consistent results: Cochran-Armitage (p = 0.0006), Hewitt-Rogerson (December to May; p = 0.0130), Walter-Elwood (March 14; p = 0.0027), and chi-square (winter; p = 0.0046). Congenital cataract, pulmonary valve atresia/stenosis, coarctation of aorta, biliary atresia, and renal agenesis or hypoplasia had at least three significant tests. Discussion: These results may help to generate hypotheses about environmental factors that vary by season for further studies. © 2012 Wiley Periodicals, Inc. AD - A.R. Caton, Department of Epidemiology and Biostatistics, University at Albany, School of Public Health, One University Place 131, Rensselaer, NY 12144-3456, United States AU - Caton, A. R. DB - Embase Medline DO - 10.1002/bdra.23006 IS - 6 KW - amnion band syndrome anencephalus anophthalmia anus atresia aortic coarctation aortic valve stenosis article bile duct atresia birth certificate congenital malformation chi square test choana atresia cleft lip cleft palate Cochran Armitage trend congenital cataract congenital diaphragm hernia congenital hip dislocation congenital hydrocephalus congenital malformations registry disease registry Down syndrome Ebstein anomaly encephalocele endocardial cushion defect epispadias esophagus atresia Fallot tetralogy female fetal alcohol syndrome gastroschisis great vessels transposition heart atrium septum defect heart ventricle septum defect Hewitt Rogerson test Hirschsprung disease human hypoplastic left heart syndrome hypospadias infant intestine stenosis kidney agenesis kidney hypoplasia limb reduction defect live birth major clinical study male microcephaly microphthalmia microtia obstructive uropathy omphalocele patent ductus arteriosus practice guideline priority journal pulmonary valve atresia pulmonary valve stenosis pylorus stenosis seasonal variation spinal dysraphism statistical analysis summer tracheoesophageal fistula tricuspid valve atresia trisomy 13 trisomy 18 Walter Elwood test winter LA - English M3 - Article N1 - L364997173 2012-06-21 2012-06-26 PY - 2012 SN - 1542-0752 1542-0760 SP - 424-437 ST - Exploring the seasonality of birth defects in the New York State Congenital Malformations Registry T2 - Birth Defects Research Part A - Clinical and Molecular Teratology TI - Exploring the seasonality of birth defects in the New York State Congenital Malformations Registry UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L364997173 http://dx.doi.org/10.1002/bdra.23006 VL - 94 ID - 1055 ER - TY - JOUR AB - We report the results of follow-up of the complete cohort of Norwegian children born in the period from 1987 through 1998 in whom there was the intention to treat surgically hypoplasia of the left heart using the Norwood sequence of operations. Of the 54 children, 21 are alive. Of these, 15 have been extensively studied, while the medical state of all the remaining survivors is known from reports from other hospitals. Of the survivors, the majority have reasonably acceptable cardiac and haemodynamic function, but significant neurological and neuropsychological morbidity is identified within the group as a whole, which requires special attention from qualified personnel of various kinds. © 2007 Cambridge University Press. AD - P.S. Hagemo, Department of Pediatric Cardiology, Section for Neurology, Rikshospitalet, N-0027 Oslo, Norway AU - Hagemo, P. S. AU - Skarbø, A. B. AU - Rasmussen, M. AU - Fredriksen, P. M. AU - Schage, S. DB - Embase Medline DO - 10.1017/S1047951106001284 IS - 1 KW - lamotrigine valproic acid article artificial heart pacemaker child child death clinical article cohort analysis controlled study follow up Fontan procedure heart function heart transplantation hemodynamics human hypoplastic left heart syndrome mental deficiency morbidity Norway Norwood procedure seizure stent survivor LA - English M3 - Article N1 - L46322010 2007-03-21 PY - 2007 SN - 1047-9511 1467-1107 SP - 51-55 ST - An extensive long term follow-up of a cohort of patients with hypoplasia of the left heart T2 - Cardiology in the Young TI - An extensive long term follow-up of a cohort of patients with hypoplasia of the left heart UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L46322010 http://dx.doi.org/10.1017/S1047951106001284 VL - 17 ID - 1241 ER - TY - JOUR AB - Background: Limited therapeutic options are available for Fontan patients with dysfunctional or failing single ventricle physiology. This study describes the evaluation of an alternative, non-invasive, at-home therapeutic compression treatment for Fontan patients. Our hypothesis is that routinely administered, externally applied compression treatments to the lower extremities will augment systemic venous return, improve ventricular preload, and thus enhance cardiac output in Fontan patients. Methods: To initially evaluate this hypothesis, we employed the NormaTec pneumatic compression device (PCD) in a pilot clinical study (n=2). This device is composed of inflatable trouser compartments that facilitate circumferentially and uniformly applied pressure to a patient’s lower extremities. Following an initial health screening, test subjects were pre-evaluated with a modified-Bruce treadmill exercise stress test, and baseline data on cardiorespiratory health was collected. After training, test subjects conducted 6 days of external compression therapy at-home. Subjects were then re-evaluated with a final treadmill stress test and data acquisition of new cardiorespiratory parameters. Results: Both subjects demonstrated improvement in exercise duration time, peak oxygen volume, and ventilator threshold, as compared to the baseline evaluation. Conclusions: These findings are promising and provide the foundation for future studies that will focus on increasing study participation (sample size) to better assess the clinical benefit of compression therapy for Fontan patients. AD - A.L. Throckmorton, School of Biomedical Engineering, Science and Health Systems, Drexel University, Bossone Research Enterprise Center, 3141 Chestnut Street, Rm. 718, Philadelphia, PA, United States AU - Hernandez, J. AU - Chopski, S. G. AU - Lee, S. AU - Moskowitz, W. B. AU - Throckmorton, A. L. DB - Embase DO - 10.21037/tp.2017.08.01 IS - 1 KW - intermittent pneumatic compression device NormaTec adolescent adult aerobic capacity article cardiorespiratory fitness cardiorespiratory parameters cardiovascular parameters clinical article clinical evaluation clinical study compression therapy controlled study exercise exercise duration time exercise intensity externally applied compression therapy female Fontan procedure heart function home care human inflatable trouser compartment information processing lower limb male oxygen consumption oxygen uptake efficiency slope peak oxygen volume peak respiratory exchange rate pilot study pressure reevaluation respiratory tract parameters training treadmill exercise treatment outcome ventilator threshold ventilatory threshold LA - English M3 - Article N1 - L620825262 2018-02-28 2018-03-09 PY - 2018 SN - 2224-4344 2224-4336 SP - 14-22 ST - Externally applied compression therapy for Fontan patients T2 - Translational Pediatrics TI - Externally applied compression therapy for Fontan patients UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L620825262 http://dx.doi.org/10.21037/tp.2017.08.01 VL - 7 ID - 722 ER - TY - JOUR AB - OBJECTIVE: The extracardiac Fontan procedure, as compared with classic atriopulmonary connections, may have the potential for optimizing ventricular and pulmonary vascular function by maximizing the laminar flow principle, by the avoidance of intra-atrial suture lines and cardiac manipulation, and by minimizing cardiopulmonary bypass time. In this study the clinical results of this procedure are assessed. METHODS: From January 1990 until January 1997, 45 patients (33 males and 12 females) with a median age of 4.0 years (range 2.7-38 years) underwent an extracardiac Fontan procedure for univentricular physiology. The underlying diagnoses included tricuspid atresia (n=19), double-inlet left ventricle (n=11), and complex anomalies (n=15). Forty patients (89%) were in sinus rhythm. The median ventricular ejection fraction was 60%. In 37 patients (82%) the procedure was staged. RESULTS: Median cardiopulmonary bypass time was 72 min, with a decrease to a median time of 24 min in the last ten patients. Aortic cross-clamping was avoided in 33 patients (73%). The intraoperative Fontan pressure and transpulmonary gradient were low: 13.6+/-3.2 and 8.5+/-3.9 mmHg, respectively. Transient supraventricular tachyarrhythmias were observed in six patients (13%). There was no early or late mortality. At a median follow-up of 64 months (range 26-105 months), 39 patients (87%) were in NYHA class I, four (9%) were in NYHA class II, and two (4%) were in class III. Forty patients (89%) remained in sinus rhythm. The median ventricular ejection fraction was 59%. The median arterial oxygen saturation raised from 82% preoperatively to 97%. Functional class (P=0.02), maintenance of sinus rhythm (P=0.04), and preservation of ventricular function (P=0.05) was superior in patients who were appropriately staged. None of the patients had atrial thrombus, chronic pleural effusions, or protein losing enteropathy. CONCLUSIONS: In the majority of patients, the extracardiac Fontan procedure, when performed as a staged procedure, provides excellent early and midterm results in terms of quality of life, maintenance of sinus rhythm, and preservation of ventricular function. AD - Children's Hospital, Tampa, FL, USA. AN - 10856854 AU - Haas, G. S. AU - Hess, H. AU - Black, M. AU - Onnasch, J. AU - Mohr, F. W. AU - van Son, J. A. DA - Jun DB - PubMed DO - 10.1016/s1010-7940(00)00433-4 DP - NLM ET - 2000/06/17 IS - 6 KW - Adolescent Adult Child Child, Preschool Female Follow-Up Studies Fontan Procedure/*methods Heart Defects, Congenital/diagnosis/*surgery Heart Ventricles/abnormalities/*surgery Humans Male Pulmonary Valve Stenosis/diagnosis/surgery Retrospective Studies Sensitivity and Specificity Survival Rate Treatment Outcome Tricuspid Atresia/diagnosis/surgery Ventricular Dysfunction, Left/diagnosis/surgery LA - eng N1 - Haas, G S Hess, H Black, M Onnasch, J Mohr, F W van Son, J A Journal Article Germany Eur J Cardiothorac Surg. 2000 Jun;17(6):648-54. doi: 10.1016/s1010-7940(00)00433-4. PY - 2000 SN - 1010-7940 (Print) 1010-7940 SP - 648-54 ST - Extracardiac conduit fontan procedure: early and intermediate results T2 - Eur J Cardiothorac Surg TI - Extracardiac conduit fontan procedure: early and intermediate results VL - 17 ID - 378 ER - TY - JOUR AB - Objective: The extracardiac Fontan procedure, as compared with classic atriopulmonary connections, may have the potential for optimizing ventricular and pulmonary vascular function by maximizing the laminar flow principle, by the avoidance of intra-atrial suture lines and cardiac manipulation, and by minimizing cardiopulmonary bypass time. In this study the clinical results of this procedure are assessed. Methods: From January 1990 until January 1997, 45 patients (33 males and 12 females) with a median age of 4.0 years (range 2.7-38 years) underwent an extracardiac Fontan procedure for univentricular physiology. The underlying diagnoses included tricuspid atresia (n=19), double-inlet left ventricle (n=11), and complex anomalies (n=15). Forty patients (89%) were in sinus rhythm. The median ventricular ejection fraction was 60%. In 37 patients (82%) the procedure was staged. Results: Median cardiopulmonary bypass time was 72 min, with a decrease to a median time of 24 min in the last ten patients. Aortic cross-clamping was avoided in 33 patients (73%). The intraoperative Fontan pressure and transpulmonary gradient were low: 13.6±3.2 and 8.5±3.9 mmHg, respectively. Transient supraventricular tachyarrhythmias were observed in six patients (13%). There was no early or late mortality. At a median follow-up of 64 months (range 26-105 months), 39 patients (87%) were in NYHA class I, four (9%) were in NYHA class II, and two (4%) were in class III. Forty patients (89%) remained in sinus rhythm. The median ventricular ejection fraction was 59%. The median arterial oxygen saturation raised from 82% preoperatively to 97%. Functional class (P=0.02), maintenance of sinus rhythm (P=0.04), and preservation of ventricular function (P=0.05) was superior in patients who were appropriately staged. None of the patients had atrial thrombus, chronic pleural effusions, or protein losing enteropathy. Conclusions: In the majority of patients, the extracardiac Fontan procedure, when performed as a staged procedure, provides excellent early and midterm results in terms of quality of life, maintenance of sinus rhythm, and preservation of ventricular function. Copyright (C) 2000 Elsevier Science B.V. AD - J.A.M. Van Son, Herzzentrum, University of Leipzig, Russenstrasse 19, D-04289 Leipzig, Germany AU - Haas, G. S. AU - Hess, H. AU - Black, M. AU - Onnasch, J. AU - Mohr, F. W. AU - Van Son, J. A. M. DB - Embase Medline DO - 10.1016/S1010-7940(00)00433-4 IS - 6 KW - adolescent adult anastomosis article cannulation cardiopulmonary bypass child clinical article clinical trial comparative study controlled clinical trial controlled study female Fontan procedure heart single ventricle heart ventricle ejection Holter monitoring human male priority journal supraventricular tachycardia LA - English M3 - Article N1 - L30364940 2000-06-30 PY - 2000 SN - 1010-7940 SP - 648-654 ST - Extracardiac conduit Fontan procedure: Early and intermediate results T2 - European Journal of Cardio-thoracic Surgery TI - Extracardiac conduit Fontan procedure: Early and intermediate results UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L30364940 http://dx.doi.org/10.1016/S1010-7940(00)00433-4 VL - 17 ID - 1335 ER - TY - JOUR AB - OBJECTIVES: The clinical outcome of prenatally diagnosed congenital heart defects (CHD) continues to be affected significantly by associated extracardiac and chromosomal abnormalities. We sought to: determine the frequency and type of major extracardiac abnormalities (with impact on quality of life) and chromosomal abnormalities associated with fetal CHD; and compare the extracardiac abnormalities detected prenatally to the postnatal and autopsy findings in affected fetuses, to find the incidence of extracardiac abnormalities missed on prenatal ultrasound. METHODS: We reviewed the computerized database of the Division of Cardiology of the Hospital for Sick Children in Toronto to identify all cases of major CHD detected prenatally from 1990 to 2002. Medical records, fetal echocardiograms and ultrasound, cytogenetic and autopsy reports were reviewed. The types of CHD detected were grouped into categories and the frequencies of major extracardiac and chromosomal abnormalities in these categories were noted. Prenatal ultrasound findings were compared with those at autopsy or postnatal examination. RESULTS: Of 491 fetuses with major structural CHD, complete data were obtained for 382. Of these, there were 141 (36.9%) with major extracardiac abnormalities at autopsy or postnatal exam, of which 46 had chromosomal abnormalities and 95 did not. In the absence of chromosomal abnormalities, the organ systems most affected were urogenital (12.2%) and gastrointestinal (11.6%). CHDs with the highest incidence of extracardiac abnormalities (>25%) included: heterotaxy, single left ventricle and tricuspid atresia, hypoplastic left heart syndrome and tetralogy of Fallot. Ninety-four of 334 (28.1%) fetuses tested had chromosomal abnormalities. The most common chromosomal abnormalities were trisomies 21 (43.6%), 18 (19.1%) and 13 (9.6%), monosomy X (7.4%) and 22q11.2 deletion (7.4%). Of 289 extracardiac abnormalities from the complete series, 134 (46.4%) were not identified prenatally. Of the missed extracardiac abnormalities, 65 were considered not detectable at prenatal ultrasound, so 23.9% (69/289) of detectable extracardiac abnormalities were missed prenatally. CONCLUSIONS: Major extracardiac and chromosomal abnormalities are common in fetuses with major fetal CHD. Many important associated extracardiac abnormalities may be missed prenatally, which should be taken into consideration in the prenatal counseling for fetal CHD. AD - Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. AN - 19350566 AU - Song, M. S. AU - Hu, A. AU - Dyamenahalli, U. AU - Chitayat, D. AU - Winsor, E. J. AU - Ryan, G. AU - Smallhorn, J. AU - Barrett, J. AU - Yoo, S. J. AU - Hornberger, L. K. DA - May DB - PubMed DO - 10.1002/uog.6309 DP - NLM ET - 2009/04/08 IS - 5 KW - Abnormalities, Multiple/*diagnosis/genetics/pathology Autopsy Chromosome Aberrations/*embryology Female *Fetal Heart/diagnostic imaging/pathology Genetic Counseling Gestational Age Heart Defects, Congenital/*diagnosis/*genetics/pathology Humans Incidence Pregnancy Pregnancy Outcome Prenatal Diagnosis/standards Retrospective Studies Ultrasonography, Prenatal LA - eng N1 - 1469-0705 Song, M S Hu, A Dyamenahalli, U Chitayat, D Winsor, E J T Ryan, G Smallhorn, J Barrett, J Yoo, S-J Hornberger, L K Comparative Study Journal Article England Ultrasound Obstet Gynecol. 2009 May;33(5):552-9. doi: 10.1002/uog.6309. PY - 2009 SN - 0960-7692 SP - 552-9 ST - Extracardiac lesions and chromosomal abnormalities associated with major fetal heart defects: comparison of intrauterine, postnatal and postmortem diagnoses T2 - Ultrasound Obstet Gynecol TI - Extracardiac lesions and chromosomal abnormalities associated with major fetal heart defects: comparison of intrauterine, postnatal and postmortem diagnoses VL - 33 ID - 363 ER - TY - JOUR AB - Objectives: The clinical outcome of prenatally diagnosed congenital heart defects (CHD) continues to be affected significantly by associated extracardiac and chromosomal abnormalities. We sought to: determine the frequency and type of major extracardiac abnormalities (with impact on quality of life) and chromosomal abnormalities associated with fetal CHD; and compare the extracardiac abnormalities detected prenatally to the postnatal and autopsy findings in affected fetuses, to find the incidence of extracardiac abnormalities missed on prenatal ultrasound. Methods: We reviewed the computerized database of the Division of Cardiology of the Hospital for Sick Children in Toronto to identify all cases of major CHD detected prenatally from 1990 to 2002. Medical records, fetal echocardiograms and ultrasound, cytogenetic and autopsy reports were reviewed. The types of CHD detected were grouped into categories and the frequencies of major extracardiac and chromosomal abnormalities in these categories were noted. Prenatal ultrasound findings were compared with those at autopsy or postnatal examination. Results: Of 491 fetuses with major structural CHD, complete data were obtained for 382. Of these, there were 141 (36.9%) with major extracardiac abnormalities at autopsy or postnatal exam, of which 46 had chromosomal abnormalities and 95 did not. In the absence of chromosomal abnormalities, the organ systems most affected were urogenital (12.2%) and gastrointestinal (11.6%). CHDs with the highest incidence of extracardiac abnormalities (>25%) included: heterotaxy, single left ventricle and tricuspid atresia, hypoplastic left heart syndrome and tetralogy of Fallot. Ninety-four of 334 (28.1%) fetuses tested had chromosomal abnormalities. The most common chromosomal abnormalities were trisomies 21 (43.6%), 18 (19.1%) and 13 (9.6%), monosomy X (7.4%) and 22q11.2 deletion (7.4%). Of 289 extracardiac abnormalities from the complete series, 134 (46.4%) were not identified prenatally. Of the missed extracardiac abnormalities, 65 were considered not detectable at prenatal ultrasound, so 23.9% (69/289) of detectable extracardiac abnormalities were missed prenatally. Conclusions: Major extracardiac and chromosomal abnormalities are common in fetuses with major fetal CHD. Many important associated extracardiac abnormalities may be missed prenatally, which should be taken into consideration in the prenatal counseling for fetal CHD. AD - Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada AN - 105220441. Language: English. Entry Date: 20100514. Revision Date: 20170411. Publication Type: journal article AU - Song, M. S. AU - Hu, A. AU - Dyhamenahali, U. AU - Chitayat, D. AU - Winsor, E. J. AU - Ryan, G. AU - Smallhorn, J. AU - Barrett, J. AU - Yoo, S. J. AU - Hornberger, L. K. AU - Song, M. S. AU - Hu, A. AU - Dyamenahalli, U. AU - Dyhamenahali, U. AU - Chitayat, D. AU - Winsor, E. J. T. AU - Ryan, G. AU - Smallhorn, J. AU - Barrett, J. AU - Yoo, S. J. DB - ccm DO - 10.1002/uog.6309 DP - EBSCOhost IS - 5 KW - Abnormalities, Multiple -- Diagnosis Chromosome Disorders -- Embryology Fetal Heart -- Pathology Fetal Heart -- Ultrasonography Heart Defects, Congenital -- Diagnosis Heart Defects, Congenital Abnormalities, Multiple Abnormalities, Multiple -- Pathology Autopsy Female Genetic Counseling Gestational Age Heart Defects, Congenital -- Pathology Human Incidence Pregnancy Pregnancy Outcomes Prenatal Diagnosis -- Standards Retrospective Design Ultrasonography, Prenatal N1 - research. Journal Subset: Biomedical; Europe; Peer Reviewed; UK & Ireland. NLM UID: 9108340. PMID: NLM19350566. PY - 2009 SN - 0960-7692 SP - 552-559 ST - Extracardiac lesions and chromosomal abnormalities associated with major fetal heart defects: comparison of intrauterine, postnatal and postmortem diagnoses T2 - Ultrasound in Obstetrics & Gynecology TI - Extracardiac lesions and chromosomal abnormalities associated with major fetal heart defects: comparison of intrauterine, postnatal and postmortem diagnoses UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105220441&site=ehost-live&scope=site VL - 33 ID - 1616 ER - TY - JOUR AB - Objectives The clinical outcome of prenatally diagnosed congenital heart defects (CHD) continues to be affected significantly by associated extracardiac and chromosomal abnormalities. We sought to: determine the frequency and type of major extracardiac abnormalities (with impact on quality of life) and chromosomal abnormalities associated with fetal CHD; and compare the extracardiac abnormalities detected prenatally to the postnatal and autopsy findings in affected fetuses, to find the incidence of extracardiac abnormalitiesmissed on prenatal ultrasound. Methods We reviewed the computerized database of the Division of Cardiology of the Hospital for Sick Children in Toronto to identify all cases of major CHD detected prenatally from 1990 to 2002. Medical records, fetal echocardiograms and ultrasound, cytogenetic and autopsy reports were reviewed. The types of CHD detected were grouped into categories and the frequencies of major extracardiac and chromosomal abnormalities in these categories were noted. Prenatal ultrasound findings were compared with those at autopsy or postnatal examination. Results Of 491 fetuses with major structural CHD, complete data were obtained for 382. Of these, there were 141 (36.9%) with major extracardiac abnormalities at autopsy or postnatal exam, of which 46 had chromosomal abnormalities and 95 did not. In the absence of chromosomal abnormalities, the organ systems most affected were urogenital (12.2%) and gastrointestinal (11.6%). CHDs with the highest incidence of extracardiac abnormalities (>25%) included: heterotaxy, single left ventricle and tricuspid atresia, hypoplastic left heart syndrome and tetralogy of Fallot. Ninety-four of 334 (28.1%) fetuses tested had chromosomal abnormalities. The most common chromosomal abnormalities were trisomies 21 (43.6%), 18 (19.1%) and 13 (9.6%), monosomy X (7.4%) and 22q11.2 deletion (7.4%). Of 289 extracardiac abnormalities from the complete series, 134 (46.4%) were not identified prenatally. Of the missed extracardiac abnormalities, 65 were considered not detectable at prenatal ultrasound, so 23.9% (69/289) of detectable extracardiac abnormalities were missed prenatally. Conclusions Major extracardiac and chromosomal abnormalities are common in fetuses with major fetal CHD. Many important associated extracardiac abnormalities may be missed prenatally, which should be taken into consideration in the prenatal counseling for fetal CHD. © 2009 ISUOG. AD - L. K. Hornberger, Divisions of Cardiology, Pediatric Cardiology, University of Alberta, WMC 4C2.23, 8440 112th Street, Edmonton, AB T6G 2B7, Canada AU - Song, M. S. AU - Hu, A. AU - Dyhamenahali, U. AU - Chitayat, D. AU - Winsor, E. J. T. AU - Ryan, G. AU - Smallhorn, J. AU - Barrett, J. AU - Yoo, S. J. AU - Hornberger, L. K. DB - Embase Medline DO - 10.1002/uog.6309 IS - 5 KW - aortic coarctation aortic stenosis aortopulmonary septal defect arterial trunk article atrioventricular septal defect autopsy cardiology chromosome 22q chromosome aberration chromosome deletion comparative study congenital heart disease congenital heart malformation controlled study counseling cytogenetics data base echocardiography Fallot tetralogy fetus fetus echography gastrointestinal tract great vessels transposition heart disease heart left ventricle failure heart right ventricle double outlet heterotaxy syndrome hospital human hypoplastic left heart syndrome lung vein drainage anomaly major clinical study medical record review monosomy X morbidity perinatal period prenatal diagnosis prenatal period priority journal pulmonary valve atresia pulmonary valve stenosis quality of life tricuspid valve atresia tricuspid valve dysplasia trisomy 13 trisomy 18 trisomy 21 ultrasound United States urogenital system uterus vascular ring LA - English M3 - Article N1 - L354658891 2009-06-10 http://www3.interscience.wiley.com/cgi-bin/fulltext/122302965/PDFSTART PY - 2009 SN - 0960-7692 1469-0705 SP - 552-559 ST - Extracardiac lesions and chromosomal abnormalities associated with major fetal heart defects: Comparison of intrauterine, postnatal and postmortem diagnoses T2 - Ultrasound in Obstetrics and Gynecology TI - Extracardiac lesions and chromosomal abnormalities associated with major fetal heart defects: Comparison of intrauterine, postnatal and postmortem diagnoses UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L354658891 http://dx.doi.org/10.1002/uog.6309 VL - 33 ID - 1183 ER - TY - JOUR AB - OBJECTIVE: Although extracorporeal membrane oxygenation (ECMO) is an acceptable strategy for children with refractory cardiac dysfunction after cardiac surgery, its role after stage I reconstruction for hypoplastic left heart syndrome and its variants is controversial. Our objective is to describe the outcome of "nonelective" ECMO after stage I reconstruction. DESIGN: Retrospective case series. SETTING: Pediatric cardiac intensive care unit. PATIENTS: Infants placed on ECMO after stage I reconstruction from January 1998 to May 2005. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Of the 382 infants who underwent stage I reconstruction during the study period, 36 (9.4%) required ECMO in the postoperative period. There were 22 infants with hypoplastic left heart syndrome. Indications for ECMO included inability to separate from cardiopulmonary bypass in 14 and cardiac arrest in 22. Fourteen infants (38.8%) survived to hospital discharge. Nonsurvivors had longer cardiopulmonary bypass time (150.1 +/- 70.0 mins vs. 103.9 +/- 30.0 mins, p =. 01). 9/14 infants (64%) supported with ECMO> than 24 hrs after stage I reconstruction survived while only 5/22 infants (22%) requiring ECMO< 24 hrs of stage I reconstruction survived (p =. 02). Of note, all five infants diagnosed with an acute shunt thrombosis were early survivors. Mean duration of ECMO was 50.1 +/- 12.5 hrs for survivors and 125.2 +/- 25.0 for nonsurvivors (p =. 01). 7/14 early survivors are alive at a median follow-up of 20 months (2-78 months). CONCLUSIONS: In our experience, ECMO after stage I reconstruction can be life saving in about a third of infants with otherwise fatal conditions. It is particularly useful in potentially reversible conditions such as acute shunt thrombosis and transient depression of ventricular function. AD - Division of Cardiology, The Children's Hospital of Philadelphia, PA 19104-4399, USA. Ravishankar@email.chop.edu AN - 16738497 AU - Ravishankar, C. AU - Dominguez, T. E. AU - Kreutzer, J. AU - Wernovsky, G. AU - Marino, B. S. AU - Godinez, R. AU - Priestley, M. A. AU - Gruber, P. J. AU - Gaynor, W. J. AU - Nicolson, S. C. AU - Spray, T. L. AU - Tabbutt, S. DA - Jul DB - PubMed DO - 10.1097/01.Pcc.0000227109.82323.Ce DP - NLM ET - 2006/06/02 IS - 4 KW - *Extracorporeal Membrane Oxygenation/adverse effects Female Heart Arrest/therapy Humans Hypoplastic Left Heart Syndrome/*surgery Infant Infant, Newborn Logistic Models Male Multivariate Analysis *Postoperative Care Retrospective Studies Risk Factors Survival Analysis Treatment Outcome LA - eng N1 - Ravishankar, Chitra Dominguez, Troy E Kreutzer, Jacqueline Wernovsky, Gil Marino, Bradley S Godinez, Rodolfo Priestley, Margaret A Gruber, Peter J Gaynor, William J Nicolson, Susan C Spray, Thomas L Tabbutt, Sarah Journal Article United States Pediatr Crit Care Med. 2006 Jul;7(4):319-23. doi: 10.1097/01.PCC.0000227109.82323.CE. PY - 2006 SN - 1529-7535 (Print) 1529-7535 SP - 319-23 ST - Extracorporeal membrane oxygenation after stage I reconstruction for hypoplastic left heart syndrome T2 - Pediatr Crit Care Med TI - Extracorporeal membrane oxygenation after stage I reconstruction for hypoplastic left heart syndrome VL - 7 ID - 327 ER - TY - JOUR AB - Extracorporeal membrane oxygenation (ECMO) has become a valuable adjunct in caring for infants and children with heart disease. Since the initial reports of ECMO support for cardiac failure in children, the number of centers providing cardiac support and the number of cases of cardiac ECMO have steadily increased. The International Registry for Extracorporeal Life Support Organization has reported survival statistics for cardiac cases in neonates, children, and adults ranging from 33% to 43%. These numbers are similar to the survival from recent reports by Morris (39%) and Chaturvedi (49%). Survival is influenced by ability to be weaned from bypass in the operating room and by residual structural disease and multi-organ system failure but not by cardiac arrest and single ventricle physiology. To improve results in the future, we need to focus on better predicting the need for support and avoiding multi-organ system failure before initiating ECMO. Rapid deployment of ECMO may further improve results for patients who deteriorate suddenly in the intensive care unit. © 2005 Elsevier Inc. All rights reserved. AD - Department of Cardiac Surgery, Children's National Medical Center, 111 Michigan Avenue, NW, Washington, DC 20010, United States Department of Pediatrics, George Washington Univ. Sch. of Med., Children's National Medical Center, 111 Michigan Avenue, NW, Washington, DC 20010, United States Children's National Heart Institute, Children's National Medical Center, 111 Michigan Avenue, NW, Washington, DC 20010, United States AU - Di Russo, G. B. AU - Martin, G. R. DB - Scopus DO - 10.1053/j.pcsu.2005.01.021 IS - 1 KW - Congenital heart disease Extracorporeal membrane oxygenation (ECMO) Survival M3 - Article N1 - Cited By :11 Export Date: 15 June 2020 PY - 2005 SP - 34-40 ST - Extracorporeal membrane oxygenation for cardiac disease: No longer a mistaken diagnosis T2 - Pediatric Cardiac Surgery Annual TI - Extracorporeal membrane oxygenation for cardiac disease: No longer a mistaken diagnosis UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-16344371281&doi=10.1053%2fj.pcsu.2005.01.021&partnerID=40&md5=28e47e7f0eea0c3a6df78ce5ac6a20ad VL - 8 ID - 2239 ER - TY - JOUR AB - Background/objective: We examined the relationship between extreme winter temperatures and birth defects to determine whether pregnant women might be vulnerable to the weather extremes expected with climate change. Methods: In this population-based, case-control study, we linked the New York State Congenital Malformations Registry to birth certificates (1992-2006). Cases were defined as live births with birth defects, and controls were selected from a 10% random sample of live births. We assigned meteorological data based on maternal birth residence and summarized universal apparent temperature across gestational weeks 3-8 (embryogenesis). We defined an extreme cold day as a day with mean temperature below the 10th percentile of the regional winter temperature distribution and a cold spell as 3 consecutive extreme cold days. We averaged temperature for each week of the first trimester to identify susceptible periods. We estimated adjusted odds ratios (ORs) and 95% confidence intervals (CIs) with multivariable logistic regression for 30 birth defects groups. Results: Among 13,044 cases and 59,884 controls with at least 1 week of embryogenesis in winter, coarctation of the aorta was associated with a 1. °C decrease in mean universal apparent temperature (OR 1.06, 95% CI 1.02-1.11), cold spell (OR 1.61, 95% CI 1.11-2.34), and number of extreme cold days. We observed reduced odds of hypoplastic left heart syndrome and dislocated hip for some cold indicators. Conclusions: Most birth defects were not associated with cold indicators; however, we found positive associations between cold indicators and coarctation of the aorta in the biologically-relevant developmental window which warrants replication. AD - S. Lin, New York State Department of Health, Bureau of Environmental and Occupational Epidemiology, Empire State Plaza, Corning Tower, Albany, NY, United States AU - Van Zutphen, A. R. AU - Hsu, W. H. AU - Lin, S. DB - Embase Medline DO - 10.1016/j.envres.2013.11.006 KW - ozone adult aortic coarctation aortic valve stenosis article choana atresia cleft lip cleft palate climate change cold congenital cataract congenital hip dislocation congenital malformation controlled study diaphragm hernia embryo development endocardial cushion defect Fallot tetralogy female first trimester pregnancy gastroschisis gestational age heart atrium septum defect heart ventricle septum defect hip dislocation Hirschsprung disease human hydrocephalus hypoplastic left heart syndrome hypospadias intestine atresia kidney agenesis low birth weight major clinical study maternal age maternal smoking microcephaly omphalocele patent ductus arteriosus population based case control study pregnant woman premature labor priority journal pulmonary valve atresia pylorus stenosis smoking spinal dysraphism tracheoesophageal fistula vapor pressure winter young adult LA - English M3 - Article N1 - L605998923 2015-09-24 2015-09-28 PY - 2014 SN - 1096-0953 0013-9351 SP - 1-8 ST - Extreme winter temperature and birth defects: A population-based case-control study T2 - Environmental Research TI - Extreme winter temperature and birth defects: A population-based case-control study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L605998923 http://dx.doi.org/10.1016/j.envres.2013.11.006 VL - 128 ID - 977 ER - TY - JOUR AB - The purpose of the study was to conceptualize the needs of parents of young children with hypoplastic left heart syndrome (HLHS) to provide a theoretical framework to inform the development of future parent interventions. Participants were parents and grandparents (n = 53) of 15 young children who had undergone the Sano surgical approach for HLHS. Analysis of recorded and transcribed single interviews with each participant was done as directed by interpretive description methodology. A model of five facets of parenting was conceptualized. These included survival parenting, "hands-off" parenting, expert parenting, uncertain parenting, and supported parenting. The facets of parenting delineated through this study provide a theoretical framework that can be used to guide the development and evaluation of interventions for parents of children with complex congenital heart disease and potentially other life-threatening conditions. Each facet constitutes a critical component for educational or psychosocial intervention for parents. AD - Faculty of Nursing, University of Alberta, Edmonton, AB, Canada. AN - 22548161 AU - Rempel, G. R. AU - Rogers, L. G. AU - Ravindran, V. AU - Magill-Evans, J. C2 - PMC3324165 DB - PubMed DO - 10.1155/2012/714178 DP - NLM ET - 2012/05/02 LA - eng N1 - 2090-1437 Rempel, Gwen R Rogers, Laura G Ravindran, Vinitha Magill-Evans, Joyce Journal Article Nurs Res Pract. 2012;2012:714178. doi: 10.1155/2012/714178. Epub 2012 Apr 2. PY - 2012 SN - 2090-1429 (Print) 2090-1429 SP - 714178 ST - Facets of parenting a child with hypoplastic left heart syndrome T2 - Nurs Res Pract TI - Facets of parenting a child with hypoplastic left heart syndrome VL - 2012 ID - 193 ER - TY - JOUR AB - OBJECTIVE: To measure neurodevelopment at 3 years of age in children with single right-ventricle anomalies and to assess its relationship to Norwood shunt type, neurodevelopment at 14 months of age, and patient and medical factors. STUDY DESIGN: All subjects in the Single Ventricle Reconstruction Trial who were alive without cardiac transplant were eligible for inclusion. The Ages and Stages Questionnaire (ASQ, n = 203) and other measures of behavior and quality of life were completed at age 3 years. Medical history, including measures of growth, feeding, and complications, was assessed through annual review of the records and phone interviews. The Bayley Scales of Infant Development, Second Edition (BSID-II) scores from age 14 months were also evaluated as predictors. RESULTS: Scores on each ASQ domain were significantly lower than normal (P < .001). ASQ domain scores at 3 years of age varied nonlinearly with 14-month BSID-II. More complications, abnormal growth, and evidence of feeding, vision, or hearing problems were independently associated with lower ASQ scores, although models explained <30% of variation. Type of shunt was not associated with any ASQ domain score or with behavior or quality-of-life measures. CONCLUSION: Children with single right-ventricle anomalies have impaired neurodevelopment at 3 years of age. Lower ASQ scores are associated with medical morbidity, and lower BSID-II scores but not with shunt type. Because only a modest percentage of variation in 3-year neurodevelopmental outcome could be predicted from early measures, however, all children with single right-ventricle anomalies should be followed longitudinally to improve recognition of delays. AD - University of Michigan Medical School and CS Mott Children's Hospital, Ann Arbor, MI. Electronic address: cgoldber@umich.edu. New England Research Institute, Watertown, MA. Children's Healthcare of Atlanta and Emory University School of Medicine, Atlanta, GA. Childrens Hospital of Philadelphia, Philadelphia, PA. Columbia University and Morgan Stanley Children's Hospital, New York, NY. Children's Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee, WI. University of Michigan Medical School and CS Mott Children's Hospital, Ann Arbor, MI. Medical University of South Carolina, Charleston, SC. University of Utah Primary Children's Medical Center, Salt Lake City, UT. All Children's Hospital, Johns Hopkins Medicine, St Petersburg, FL. Cincinnati Children's Hospital Medical Center, Cincinnati, OH. Children's Hospital of Los Angeles, Los Angeles, CA. National Heart, Lung, and Blood Institute, Bethesda, MD. Hospital for Sick Children, Toronto, Ontario, Canada. Alfred I. duPont Hospital for Children, Wilmington, DE. Duke University Medical Center, Durham, NC. Boston Children's Hospital, Boston, MA. AN - 24952712 AU - Goldberg, C. S. AU - Lu, M. AU - Sleeper, L. A. AU - Mahle, W. T. AU - Gaynor, J. W. AU - Williams, I. A. AU - Mussatto, K. A. AU - Ohye, R. G. AU - Graham, E. M. AU - Frank, D. U. AU - Jacobs, J. P. AU - Krawczeski, C. AU - Lambert, L. AU - Lewis, A. AU - Pemberton, V. L. AU - Sananes, R. AU - Sood, E. AU - Wechsler, S. B. AU - Bellinger, D. C. AU - Newburger, J. W. C2 - PMC4356168 C6 - NIHMS607212 DA - Sep DB - PubMed DO - 10.1016/j.jpeds.2014.05.019 DP - NLM ET - 2014/06/24 IS - 3 KW - Child, Preschool Developmental Disabilities/*etiology Female Heart Defects, Congenital/*complications/*surgery Heart Ventricles/*abnormalities/*surgery Humans Infant Male Nervous System/*growth & development Prospective Studies LA - eng N1 - 1097-6833 Goldberg, Caren S Lu, Minmin Sleeper, Lynn A Mahle, William T Gaynor, J William Williams, Ismee A Mussatto, Kathleen A Ohye, Richard G Graham, Eric M Frank, Deborah U Jacobs, Jeffrey P Krawczeski, Catherine Lambert, Linda Lewis, Alan Pemberton, Victoria L Sananes, Renee Sood, Erica Wechsler, Stephanie B Bellinger, David C Newburger, Jane W Pediatric Heart Network Investigators HL068288/HL/NHLBI NIH HHS/United States HL068285/HL/NHLBI NIH HHS/United States HL109737/HL/NHLBI NIH HHS/United States U10 HL109781/HL/NHLBI NIH HHS/United States U01 HL068269/HL/NHLBI NIH HHS/United States U10 HL109816/HL/NHLBI NIH HHS/United States U01 HL068279/HL/NHLBI NIH HHS/United States U10 HL109743/HL/NHLBI NIH HHS/United States U01 HL068290/HL/NHLBI NIH HHS/United States HL068279/HL/NHLBI NIH HHS/United States U01 HL068288/HL/NHLBI NIH HHS/United States HL109781/HL/NHLBI NIH HHS/United States U10 HL068270/HL/NHLBI NIH HHS/United States U01 HL068281/HL/NHLBI NIH HHS/United States U01 HL068270/HL/NHLBI NIH HHS/United States HL085057/HL/NHLBI NIH HHS/United States U10 HL109737/HL/NHLBI NIH HHS/United States HL068281/HL/NHLBI NIH HHS/United States U01 HL068292/HL/NHLBI NIH HHS/United States HL068269/HL/NHLBI NIH HHS/United States HL068270/HL/NHLBI NIH HHS/United States U01 HL085057/HL/NHLBI NIH HHS/United States U10 HL109778/HL/NHLBI NIH HHS/United States HL068290/HL/NHLBI NIH HHS/United States U10 HL109673/HL/NHLBI NIH HHS/United States U01 HL068285/HL/NHLBI NIH HHS/United States HL068292/HL/NHLBI NIH HHS/United States Journal Article Randomized Controlled Trial Research Support, N.I.H., Extramural J Pediatr. 2014 Sep;165(3):490-496.e8. doi: 10.1016/j.jpeds.2014.05.019. Epub 2014 Jun 19. PY - 2014 SN - 0022-3476 (Print) 0022-3476 SP - 490-496.e8 ST - Factors associated with neurodevelopment for children with single ventricle lesions T2 - J Pediatr TI - Factors associated with neurodevelopment for children with single ventricle lesions VL - 165 ID - 207 ER - TY - JOUR AB - Background: Children with complex heart defects are sedentary, with activity level unrelated to exercise capacity. We sought to identify factors associated with physical activity level for children who have the Fontan procedure. Methods: We used a cross-sectional study, 64 children (25 female, 5-11 years) after Fontan. Measurements were weekly minutes of moderate-to-vigorous physical activity, cardiac status, resting/exercise cardiopulmonary capacity, gross motor skill, health-related endurance/strength/ body composition, and parent/child activity perceptions. Results: Participants performed 361 ± 137 minutes per week of moderate-to-vigorous physical activity. Increased activity related to antithrombotic medication use (86 min/wk), lower resting heart rate (3 min/wk), higher weekday outdoor time (0.7 minutes per outside minute), lower family income (13 minutes per $10,000), and higher parent rating of child's activity relative to peers (36 min/wk). Factors related to decreased activity were winter season (-84 min/wk), history of arrhythmia (-96 min/wk), and greater child confidence in own ability to be active (-113 min/wk). Conclusions: Physical activity after the Fontan procedure is primarily associated with factors unrelated to cardiac status. Interventions that impact these modifiable factors would be expected to enable these children to achieve the recommended activity levels associated with optimal health. © 2011 Mosby, Inc. AD - B. W. McCrindle, Labatt Family Heart Centre, Hospital for Sick Children, 555 University Avenue, Toronto, ON M5G 1X8, Canada AU - Longmuir, P. E. AU - Russell, J. L. AU - Corey, M. AU - Faulkner, G. AU - McCrindle, B. W. DB - Embase Medline DO - 10.1016/j.ahj.2010.11.019 IS - 2 KW - antithrombocytic agent article body composition child cross-sectional study endurance exercise family female Fontan procedure health status heart arrhythmia heart function heart rate human income major clinical study male motor performance parent peer group perception physical activity preschool child priority journal rating scale school child strength winter LA - English M3 - Article N1 - L361244223 2011-02-17 2011-02-22 PY - 2011 SN - 0002-8703 1097-6744 SP - 411-417 ST - Factors associated with the physical activity level of children who have the Fontan procedure T2 - American Heart Journal TI - Factors associated with the physical activity level of children who have the Fontan procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L361244223 http://dx.doi.org/10.1016/j.ahj.2010.11.019 VL - 161 ID - 1115 ER - TY - JOUR AB - Background: Noncompaction of the ventricular myocardium is a rare congenital cardiomyopathy characterized by numerous excessively prominent trabeculations and deep intertrabecular recesses. Noncompaction of the ventricular myocardium is most often an isolated cardiac malformation presenting as a sporadic disease. Associated cardiac anomalies are present in some patients. We report a family with three adult males from consecutive generations having a biventricular form of noncompaction of the myocardium. Two of the patients have an associated Ebstein's malformation of the tricuspid valve. Methods: Clinical evaluation and follow-up, electrocardiography, echocardiography, heart catheterization, coronary angiography, contrast cineventriculography, and magnetic resonance imaging. Results and conclusions: The association of noncompaction of the ventricular myocardium and Ebstein's malformation has not been reported so far. We believe that both defects were caused by a developmental arrest of the right ventricular myocardium. Echocardiography is the diagnostic modality of choice in patients and in the male relatives, irrespective of their clinical status. Thromboembolic events, cardiac rhythm disorders and heart failure mandate treatment. Anticoagulation treatment and implantation of cardioverter-defibrillator pacemaker have to be strongly considered in these patients. © 2004 Elsevier Ireland Ltd. All rights reserved. AD - M. Šinkovec, Department of Cardiology, University Medical Centre Ljubljana, Zaloška 7, 1000 Ljubljana, Slovenia AU - Šinkovec, M. AU - Koželj, M. AU - Podnar, T. C1 - aspirin DB - Embase Medline DO - 10.1016/j.ijcard.2004.05.033 IS - 2 KW - acetylsalicylic acid amiodarone bisoprolol contrast medium dipeptidyl carboxypeptidase inhibitor furosemide glipizide heparin metformin spironolactone warfarin adolescent adult angiocardiography anticoagulation aphasia article artificial heart pacemaker biventricular myocardial noncompaction cardiomegaly cardiomyopathy case report cerebrovascular accident color Doppler flowmetry defibrillator diabetes mellitus disease association drug hypersensitivity drug safety echocardiography electrocardiography familial disease family follow up health status heart arrest heart atrium flutter heart catheterization heart cineventriculography heart development heart left bundle branch block heart left ventricle hypertrophy heart ventricle tachycardia hemiparesis human lung congestion male medical assessment middle cerebral artery occlusion mitral valve regurgitation nuclear magnetic resonance imaging priority journal pulmonary hypertension relative supraventricular tachycardia thorax injury thorax radiography thromboembolism traffic accident tricuspid valve atresia tricuspid valve regurgitation aspirin LA - English M3 - Article N1 - L40854046 2005-08-04 PY - 2005 SN - 0167-5273 SP - 297-302 ST - Familial biventricular myocardial noncompaction associated with Ebstein's malformation T2 - International Journal of Cardiology TI - Familial biventricular myocardial noncompaction associated with Ebstein's malformation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L40854046 http://dx.doi.org/10.1016/j.ijcard.2004.05.033 VL - 102 ID - 1274 ER - TY - JOUR AB - Providing support for a family whose infant is expected to die requires planning and coordinated intervention by members of the health care team. From diagnosis until the death of the infant, the family's choices and needs must be integrated into an acceptable plan of care. The role of the health care team in this process includes four principal phases: planning for the baby's birth, preparing the siblings, caring for the baby, and supporting the family both at the time of the death and afterward. Specific strategies used by physicians, nurses, and child life specialists can encourage the family's full participation in the birth and death of the infant. A description of one family's experiences highlights implications for family-centered practice. AD - Children's Hospital, Milwaukee, WI, USA. AN - 9165935 AU - Pearson, L. DA - Mar-Apr DB - PubMed DP - NLM ET - 1997/03/01 IS - 2 KW - *Death Family/*psychology Health Services Needs and Demand Humans Hypoplastic Left Heart Syndrome/*nursing Infant, Newborn Neonatal Nursing/*methods Patient Care Planning *Patient-Centered Care Social Support Terminal Care LA - eng N1 - Pearson, L Case Reports Journal Article Review United States Pediatr Nurs. 1997 Mar-Apr;23(2):178-82. PY - 1997 SN - 0097-9805 (Print) 0097-9805 SP - 178-82 ST - Family-centered care and the anticipated death of a newborn T2 - Pediatr Nurs TI - Family-centered care and the anticipated death of a newborn VL - 23 ID - 480 ER - TY - JOUR AB - Background Exercise stress echocardiography (ESE) can unmask ventricular dysfunction in asymptomatic patients with congenital heart disease (CHD), but its acquisition and interpretation is often challenging, and the method has not been validated in CHD. This study aimed to evaluate the feasibility of ESE using Doppler imaging and to assess myocardial response to exercise in patients with biventricular (BiV) and univentricular (UniV) circulation after CHD repair. Methods In this single-center prospective study, we recruited 55 participants (17 females), median age 14 years (8-22 years). Our analysis categorized participants in these three groups: with structurally normal hearts as controls (n = 21), with BiV circulation (n = 20) and with UniV circulation (n = 14). We acquired ESE images of the systemic ventricle including pulsed-wave flow and spectral tissue Doppler imaging (TDI) of lateral free wall before and immediately after standard, symptom-limited exercise tests on an electronically braked cycle ergometer. Results During ESE we obtained inflow E-wave and TDI systolic (S′) and early diastolic (E′) velocities in 93% to 100% of participants at rest and in 90% to 100% of participants post exercise. Feasibility to obtain Doppler imaging parameter was the same across study groups. The myocardial response to exercise was increase in heart rate (HR), S′ and inflow E-wave velocity in all participants. Patients with BiV circulation had preserved ventricular function at rest. While patients with UniV circulation had low S′, E′, and E-wave velocities at rest in comparison to controls and to BiV group (all P < .001), both patients with BiV and UniV circulation showed significant increases in HR, S′ velocity and inflow E-wave velocity post exercise, with magnitudes of these increases higher in controls than in the BiV and UniV group. The S′ and E′ velocities were strongly associated with lower percent predicted peak oxygen consumption VO2 (rs = 0.614 and rs = 0.64, respectively, both P < .001). Conclusion ESE with Doppler imaging is a practical noninvasive diagnostic method and sufficiently robust for the assessment of morphologic LV/systemic ventricles under exercise in patients after biventricular and univentricular CHD repair. Although patients with BiV and UniV circulation had both preserved myocardial response to exercise, the magnitude of this response was the lowest in patients with UniV circulation. AD - B.S. Hasan, Department of Pediatric and Child Health, The Aga Khan University, Stadium Rd., Karachi, Pakistan AU - Hasan, B. S. AU - Lunze, F. I. AU - Alvi, N. AU - Shafer, K. M. AU - Rhodes, J. DB - Embase Medline DO - 10.1016/j.ahj.2017.02.029 KW - adolescent adult anaerobic threshold article bicuspid aortic valve cardiac muscle child clinical article cohort analysis congenital heart disease cross-sectional study exercise exercise tolerance Fallot tetralogy female heart atrioventricular valve heart cycle heart function heart left ventricle ejection fraction heart left ventricle function heart muscle oxygen consumption heart rate heart surgery human hypoplastic left heart syndrome left ventricular diastolic dysfunction male mitral valve priority journal prospective study pulsed Doppler ultrasonography stress echocardiography tissue Doppler imaging LA - English M3 - Article N1 - L614843197 2017-03-23 2017-03-31 PY - 2017 SN - 1097-6744 0002-8703 SP - 1-10 ST - Feasibility of exercise stress echocardiography and myocardial response in patients with repaired congenital heart disease T2 - American Heart Journal TI - Feasibility of exercise stress echocardiography and myocardial response in patients with repaired congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L614843197 http://dx.doi.org/10.1016/j.ahj.2017.02.029 VL - 188 ID - 753 ER - TY - JOUR AB - Objective: To assess the feasibility of initiating enteral nutrition support with first 24 h of congenital heart repairs in neonates and Infants and its impact on outcomes following surgery. Design: It is a prospective randomized control single blind study. Setting: It is a single centre prospective study carried out in a tertiary care centre at Pediatric cardiac intensive care unit. Patients: All patients with the cyanotic congenital heart disease with increase pulmonary blood flow, weighing less than 5 kg and undergoing congenital heart repair during the study period were included in the study. Patients with single ventricle status, those undergoing palliative procedures (PA band), open chest, requiring ECMO before leaving operating room, having any other contraindication for starting enteral feeding or those who refuse for consent were excluded from study. Interventions: The patients were randomized into two groups. Group 1 received trophic feeds (10–20 ml/kg/day) starting 4–6 h after surgery while feeds children in group 2 were kept NPO and received feeds after 48 h after surgery. Measurements and main results: 15 children enrolled in both the groups. Both pre-operative and intraoperative variables were comparable in both the groups. There was no complication (vomiting, diarrhea, NEC, bowel necrosis) noted in the children who received feeds after surgery. Mean duration of mechanical ventilation in the feeds group was 58.2 ± 4.71 h, which was less then significantly less than those in the NPO group (P value 0.05). Similarly, duration of ICU stay was only 179.04 ± 41.28 h in feeds group as compared to 228.72 ± 85.44 h in the NPO group. Conclusions: Neonates and Infants tolerate feeds immediately following congenital heart repairs. Moreover, feeds appear to decrease duration of mechanical ventilation and duration of ICU stay. © 2018 European Society for Clinical Nutrition and Metabolism AD - Pediatric Cardiac Sciences, Sir Ganga Ram Hospital, New Delhi, 110060, India AU - Kalra, R. AU - Vohra, R. AU - Negi, M. AU - Joshi, R. AU - Aggarwal, N. AU - Aggarwal, M. AU - Joshi, R. DB - Scopus DO - 10.1016/j.clnesp.2018.03.127 KW - Congenital heart surgery CPB Enteral nutrition Infants M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2018 SP - 100-102 ST - Feasibility of initiating early enteral nutrition after congenital heart surgery in neonates and infants T2 - Clinical Nutrition ESPEN TI - Feasibility of initiating early enteral nutrition after congenital heart surgery in neonates and infants UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85045224050&doi=10.1016%2fj.clnesp.2018.03.127&partnerID=40&md5=71618b6f7b47e517bc24a84e9c20d380 VL - 25 ID - 1804 ER - TY - JOUR AB - Objective: The extracardiac conduit Fontan procedure has led to improved outcomes. We performed the procedure in patients weighing less than 10 kg and evaluated its feasibility. Methods: Since January 1999, 72 patients weighing less than 20 kg underwent extracardiac conduit Fontan procedure with polytetrafluoroethylene conduits. The patients were divided into 2 groups: 36 patients weighing less than 10 kg in group S and 36 weighing more than 10 kg in group L. Mean weight, median age, and median follow-up period in groups S and L were 8.5 ± 1.1 and 14.0 ± 3.0 kg, 18.9 and 42.0 months, and 29.2 (1.7-79.7) and 42.1 (2.8-94.2) months, respectively. Postoperatively, most patients received peritoneal drainage catheters. We reviewed data precatheterization and postcatheterization and postoperative course. Results: Conduit sizes in groups S and L were 17.0 ± 1.3 and 17.9 ± 1.9 mm, respectively (P = .03). Five patients required fenestrations. There were 2 hospital deaths, 1 in each group, and 2 late deaths in group S. The postoperative course was identical in both groups, except for median length of stay in the intensive care unit and peritoneal drainage volume. Group S versus L: ventilator support, 11 versus 7 hours; pleural drainage, 9 days each; pleural drainage greater than 14 days, 6 versus 5 cases; peritoneal drainage, 8 versus 7 days; intensive care unit stay, 7 versus 4 days (P = .01), peritoneal drainage volume, 26.1 versus 14.1 mL · kg · d-1 (P = .0007). Conclusions: The early outcome of the extracardiac conduit Fontan procedure was satisfactory in patients weighing less than 10 kg. However, the required size of the conduit remains debatable. © 2008 The American Association for Thoracic Surgery. AD - K. Sakamoto, Department of Cardiovascular Surgery, Shizuoka Children's Hospital, Shizuoka, Japan AU - Ikai, A. AU - Fujimoto, Y. AU - Hirose, K. AU - Ota, N. AU - Tosaka, Y. AU - Nakata, T. AU - Ide, Y. AU - Sakamoto, K. DB - Embase Medline DO - 10.1016/j.jtcvs.2007.12.013 IS - 5 KW - isoprenaline politef abdominal drainage article artificial ventilation body weight controlled study feasibility study fenestration Fontan procedure human intensive care unit length of stay major clinical study patient satisfaction peritoneal catheter pleura fluid postoperative care preschool child priority journal sternotomy surgical mortality surgical technique treatment outcome LA - English M3 - Article N1 - L351609044 2008-06-03 PY - 2008 SN - 0022-5223 SP - 1145-1152 ST - Feasibility of the extracardiac conduit Fontan procedure in patients weighing less than 10 kilograms T2 - Journal of Thoracic and Cardiovascular Surgery TI - Feasibility of the extracardiac conduit Fontan procedure in patients weighing less than 10 kilograms UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L351609044 http://dx.doi.org/10.1016/j.jtcvs.2007.12.013 VL - 135 ID - 1211 ER - TY - JOUR AB - BACKGROUND: Nationally representative data on the prevalence of certain birth defects are largely unavailable. We evaluated the feasibility of using data from the National Hospital Discharge Survey (NHDS) to describe the prevalence of selected birth defects. METHODS: All live births recorded in the NHDS during 1999-2001 were included. The prevalence for selected birth defects was calculated using weighted ratio estimators. Prevalence ratios comparing the NHDS estimates to published national estimates from the National Birth Defects Prevention Network (NBDPN) were calculated. RESULTS: With the exception of common truncus, the NHDS prevalence for the selected defects was consistently lower than the NBDPN estimates. The prevalence ratios ranged from 0.38 for trisomy 18 and anopthalmia/micropthalmia to 1.16 for common truncus. The NHDS prevalence estimates for spina bifida without anencephaly (PR 0.89, 95% CI: 0.57-1.22) and gastroschisis/omphalocele (PR 0.94, 95% CF: 0.48-1.40) most closely approximated the NBDPN estimates. CONCLUSIONS: NHDS data underestimate the prevalence of most birth defects. Additional research is needed to determine whether NHDS estimates may be useful for evaluating trends in certain conditions. Surveillance systems employing active case-finding continue to provide more accurate estimates of birth defects prevalence. © 2006 Wiley-Liss, Inc. AD - S.L. Boulet, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, MS-E87, 1600 Clifton Rd., Atlanta, GA 30333, United States AU - Boulet, S. L. AU - Correa-Villaseñor, A. AU - Hsia, J. AU - Atrash, H. DB - Embase Medline DO - 10.1002/bdra.20291 IS - 11 KW - accuracy anencephalus anophthalmia anus atresia article congenital malformation cleft lip cleft lip palate cleft palate diaphragm hernia Down syndrome endocardial cushion defect esophagus atresia Fallot tetralogy feasibility study gastroschisis great vessels transposition hospital discharge human hypoplastic left heart syndrome infant intestine atresia intestine stenosis limb reduction defect microphthalmia newborn omphalocele prevalence priority journal spinal dysraphism tracheoesophageal fistula trisomy 13 trisomy 18 LA - English M3 - Article N1 - L44777831 2006-12-05 PY - 2006 SN - 1542-0752 1542-0760 SP - 757-761 ST - Feasibility of using the national hospital discharge survey to estimate the prevalence of selected birth defects T2 - Birth Defects Research Part A - Clinical and Molecular Teratology TI - Feasibility of using the national hospital discharge survey to estimate the prevalence of selected birth defects UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L44777831 http://dx.doi.org/10.1002/bdra.20291 VL - 76 ID - 1251 ER - TY - JOUR AB - Objective: An important area concerning morbidity among infants with congenital heart defects (CHD) is related to feeding problems. Our objectives were to characterize the evolution of feeding milestones related to transition to per oral feeding among infants with CHD, and to identify associated variables impacting the feeding abilities. Specifically, we differentiated the feeding characteristics in neonates with acyanotic vs cyanotic CHD. Study Design: Feeding progress was tracked during the first hospitalization in a retrospective chart review study involving 76 infants (29 acyanotic, 47 cyanotic CHD). The ages at which the following milestones attained were recorded: first feeds, maximum gavage feeds, first nipple feeds and maximum nipple feeds, in addition to the length of hospital stay. Effects of perinatal factors, duration of respiratory support, vasopressor and narcotic use and use of cardiopulmonary bypass on the feeding milestones were also evaluated. ANOVA, t-test, and stepwise linear regression analysis were applied as appropriate. Data stated as mean±s.e.m., or %; P<0.05 was considered significant. Result: Prenatal and birth characteristics were similar (P=NS) between the neonates with acyanotic and cyanotic CHD. Cyanotic CHD required three times prolonged use of ventilation, narcotics and vasopressor use (all P<0.05, compared to the acyanotic group). In the acyanotic group, prolonged respiratory support correlated linearly with time to attain maximal gavage feeds and nippling (both, R2=0.8). In the cyanotic group, delayed initiation of gavage feeds and prolonged respiratory support both correlated linearly with time to attain maximal gavage feeds and nippling (both, R2=0.8). Age at first gavage feed correlated with maximum gavage feeds among neonates with cyanotic CHD, and first nipple feed correlated with maximum nipple feeds among all groups (P<0.01). Use of cardiopulmonary bypass in cyanotic CHD delayed the feeding milestones and prolonged the length of stay (both, P<0.05 vs non-bypass group); similar findings were not seen in the acyanotic group. Conclusion: In contrast to neonates with acyanotic CHD, cyanotic CHD group had significant delays with (a) feeding readiness, (b) successful gastric feeding, (c) oromotor readiness and (d) successful oromotor skills. Co-morbid factors that may directly influence the delay in feeding milestones include the (a) duration of respiratory support and (b) use of cardiopulmonary bypass. Delays in achieving maximum gavage and maximum nippling may suggest foregut dysmotility and oropharyngeal dysphagia. AD - S.R. Jadcherla, Division of Neonatology, Pediatric Gastroenterology and Nutrition, Department of Pediatrics, The Ohio State University College of Medicine, Columbus, OH, United States AU - Jadcherla, S. R. AU - Vijayapal, A. S. AU - Leuthner, S. DB - Embase Medline DO - 10.1038/jp.2008.136 IS - 2 KW - hypertensive factor narcotic agent aortic coarctation aortic stenosis aortoplasty article assisted ventilation atrioventricular block Blalock Taussig shunt bottle feeding cardiopulmonary bypass congenital heart disease controlled study cyanosis drug use dysphagia Fallot tetralogy feeding behavior great vessels transposition heart atrium septum defect hospitalization human hypoplastic left heart syndrome infant feeding infant nutrition intestinal dysmotility length of stay lung artery banding lung vein drainage anomaly major clinical study medical record review motor performance newborn newborn morbidity Norwood procedure treatment duration LA - English M3 - Article N1 - L50284506 2009-02-18 PY - 2009 SN - 0743-8346 1476-5543 SP - 112-118 ST - Feeding abilities in neonates with congenital heart disease: A retrospective study T2 - Journal of Perinatology TI - Feeding abilities in neonates with congenital heart disease: A retrospective study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50284506 http://dx.doi.org/10.1038/jp.2008.136 VL - 29 ID - 1189 ER - TY - JOUR AB - The objective of this study was to identify the incidence of feeding difficulties in infants with hypoplastic left heart syndrome (HLHS) and d-transposition of the great arteries (d-TGA). Congenital heart disease is a risk factor for growth failure. The etiologies include poor caloric intake, inability to utilize calories effectively, and increased metabolic demands. The goals of our study were to (1) identify feeding difficulties in infants with HLHS and d-TGA and (2) assess their growth in the first year of life. We performed a chart review of 27 consecutive infants with HLHS and 26 with d-TGA. Descriptive statistics were generated for demographic and clinical variables within each group and are presented as means ± standard deviations. HLHS and d-TGA groups were compared on time to achieving nutritional goals using the log rank test, on complication rate using the chi-square test, and on weight using the t-test. A significance level of 0.05 was used for all tests. Birth weight was similar for both the HLHS and d-TGA groups (3.19 ± 0.69 vs 3.35 ± 0.65 kg, respectively; p = 0.38). Infants with HLHS weighed less than those with d-TGA at l month (3.29 ± 0.58 vs 3.70 ± 0.60 kg, respectively; p = 0.021), 6 months (6.27 ± 1.06 vs 7.31 ± 1.02 kg, p = 0.003), and 12 months of age (8.40 ± 1.11 vs 9.49 ± 1.01 kg, p = 0.006). Time to achieving full caloric intake (at least 100 kcal/kg/day) for the HLHS group (24 ± 11.9 days) was significantly longer than for the d-TGA group (12.0 ± 11.2 days, p < 0.001). In addition, infants with HLHS had a higher incidence of feeding-related complications that those with d-TGA (48 vs 4%, respectively; p = 0.001). Compared to the d-TGA group, infants with HLHS weighed less at follow-up, took longer to reach nutritional goals, and had a much higher incidence of feeding-related complications. © 2007 Springer Science+Business Media, LLC. AD - D. Davis, Division of Pediatrics, Children's Hospital, Cleveland Clinic, 9500 Euclid Avenue, S20, Cleveland, OH 44195, United States AU - Davis, D. AU - Davis, S. AU - Cotman, K. AU - Worley, S. AU - Londrico, D. AU - Kenny, D. AU - Harrison, A. M. DB - Embase Medline DO - 10.1007/s00246-007-9027-9 IS - 2 KW - article birth weight caloric intake clinical assessment clinical feature controlled study feeding disorder great vessels transposition growth retardation heart surgery human hypoplastic left heart syndrome infant major clinical study occupational therapy postoperative period respiratory distress speech therapy vocal cord paralysis body weight gain LA - English M3 - Article N1 - L351414849 2008-04-14 PY - 2008 SN - 0172-0643 SP - 328-333 ST - Feeding difficulties and growth delay in children with hypoplastic left heart syndrome versus d-transposition of the great arteries T2 - Pediatric Cardiology TI - Feeding difficulties and growth delay in children with hypoplastic left heart syndrome versus d-transposition of the great arteries UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L351414849 http://dx.doi.org/10.1007/s00246-007-9027-9 VL - 29 ID - 1217 ER - TY - JOUR AB - OBJECTIVE: To determine the prevalence of feeding dysfunction in children with single ventricle defects and identify associated risk factors. STUDY DESIGN: Patients aged 2-6 years with single ventricle physiology presenting for routine cardiology follow-up at the Children's Hospital of Wisconsin were prospectively identified. Parents of the patients completed 2 validated instruments for assessment of feeding dysfunction. Chart review was performed to retrospectively obtain demographic and diagnostic data. RESULTS: Instruments were completed for 56 patients; median age was 39 months. Overall, 28 (50%) patients had some form of feeding dysfunction. Compared with a normal reference population, patients with single ventricle had statistically significant differences in dysfunctional food manipulation (P < .001), mealtime aggression (P = .002), choking/gagging/vomiting (P < .001), resistance to eating (P < .001), and parental aversion to mealtime (P < .001). Weight and height for age z-scores were significantly lower in subjects with feeding dysfunction (-0.84 vs -0.33; P < .05 and -1.46 vs -0.56; P = .001, respectively). Multivariable analysis identified current gastrostomy tube use (P = .02) and a single parent household (P = .01) as risk factors for feeding dysfunction. CONCLUSION: Feeding dysfunction is common in children with single ventricle defects, occurring in 50% of our cohort. Feeding dysfunction is associated with worse growth measures. Current gastrostomy tube use and a single parent household were identified as independent risk factors for feeding dysfunction. AD - Division of Cardiology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI. Electronic address: GHill@chw.org. Division of Gastroenterology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI. Division of Quantitative Health Sciences, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI. Division of Cardiology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI. Division of Cardiology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI; Division of Adult Cardiovascular Medicine, Department of Internal Medicine, Medical College of Wisconsin, Milwaukee, WI. AN - 24161218 AU - Hill, G. D. AU - Silverman, A. H. AU - Noel, R. J. AU - Simpson, P. M. AU - Slicker, J. AU - Scott, A. E. AU - Bartz, P. J. C2 - PMC3946833 C6 - NIHMS526279 DA - Feb DB - PubMed DO - 10.1016/j.jpeds.2013.09.030 DP - NLM ET - 2013/10/29 IS - 2 KW - Body Weight Child Child, Preschool Enteral Nutrition/*methods Feeding Behavior/*physiology Feeding and Eating Disorders/epidemiology/*etiology/physiopathology Female Follow-Up Studies Heart Defects, Congenital/*surgery Heart Ventricles/*abnormalities/surgery Humans Male Palliative Care/*methods Postoperative Period Prevalence Retrospective Studies Surveys and Questionnaires Treatment Outcome Wisconsin/epidemiology Ayce About Your Child's Eating G Gastrostomy Haz Height for age z-score Mbq Mealtime Behavior Questionnaire S1p S2p Stage 1 palliation Stage 2 palliation Waz Weight for age z-score LA - eng N1 - 1097-6833 Hill, Garick D Silverman, Alan H Noel, Richard J Simpson, Pippa M Slicker, Julie Scott, Ann E Bartz, Peter J UL1 RR031973/RR/NCRR NIH HHS/United States UL1 TR000055/TR/NCATS NIH HHS/United States UL1RR031973/RR/NCRR NIH HHS/United States Comparative Study Journal Article Randomized Controlled Trial Research Support, N.I.H., Extramural J Pediatr. 2014 Feb;164(2):243-6.e1. doi: 10.1016/j.jpeds.2013.09.030. Epub 2013 Oct 22. PY - 2014 SN - 0022-3476 (Print) 0022-3476 SP - 243-6.e1 ST - Feeding dysfunction in children with single ventricle following staged palliation T2 - J Pediatr TI - Feeding dysfunction in children with single ventricle following staged palliation VL - 164 ID - 241 ER - TY - JOUR AB - PURPOSE: To determine whether caregivers of children with single ventricle heart defects identified as having feeding disorder will report more frequent feeding dysfunction, or maladaptive mealtime behavior and/or interactions, when compared with reference populations. METHODS: As part of routine evaluation, parents of children evaluated at the Feeding, Swallowing, and Nutrition Center at the Children's Hospital of Wisconsin completed previously validated questionnaires to assess feeding dysfunction and parental stress. Parental responses for single ventricle patients were compared with all other children evaluated with a feeding disorder. RESULTS: Questionnaires were completed in eight patients with single ventricle heart defects. The mean age was 36 ± 23 months, with five females (63%). Mean weight-for-age z-score was -1.4 ± 0.9. Compared with noncardiac feeding clinic children, there was more reported child resistance to eating (83 ± 15% vs. 44 ± 2%; P = .05). Single ventricle parents were more likely to report distress (50 ± 18% vs. 21 ± 2%; P = .04) and a difficult child (63 ± 17% vs. 31 ± 2%; P = .05). There was also more defensive responding among parents of single ventricle children (63 ± 17% vs. 29 ± 2%; P = .04). CONCLUSION: Single ventricle patients evaluated for disordered feeding more frequently showed resistance to eating and parental distress than noncardiac feeding clinic patients. Parents of these children underestimated the degree of feeding difficulty by defensive responding and had more parental distress. These findings suggest that feeding dysfunction can contribute to longer-term feeding and growth problems in single ventricle patients with feeding disorder. AD - Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, Milwaukee, Wis, USA. AN - 23647967 AU - Hill, G. AU - Silverman, A. AU - Noel, R. AU - Bartz, P. J. C2 - PMC4422377 C6 - NIHMS682255 DA - Jan-Feb DB - PubMed DO - 10.1111/chd.12071 DP - NLM ET - 2013/05/08 IS - 1 KW - Age Factors Caregivers/psychology Case-Control Studies Child, Preschool *Eating *Feeding Behavior Feeding and Eating Disorders of Childhood/*complications/diagnosis/psychology Female Heart Defects, Congenital/*complications/diagnosis Heart Ventricles/*abnormalities Humans Infant *Infant Behavior Infant, Newborn Male Nutritional Status Parents/psychology Risk Factors Stress, Psychological/diagnosis/etiology Surveys and Questionnaires Weight Gain Wisconsin Feeding Growth Nutrition Single Ventricle interests or relationships to industry, relevant to the subject matter or materials discussed in the manuscript. LA - eng N1 - 1747-0803 Hill, Garick Silverman, Alan Noel, Richard Bartz, Peter J UL1 TR000055/TR/NCATS NIH HHS/United States Journal Article Congenit Heart Dis. 2014 Jan-Feb;9(1):26-9. doi: 10.1111/chd.12071. Epub 2013 May 7. PY - 2014 SN - 1747-079X (Print) 1747-079x SP - 26-9 ST - Feeding dysfunction in single ventricle patients with feeding disorder T2 - Congenit Heart Dis TI - Feeding dysfunction in single ventricle patients with feeding disorder VL - 9 ID - 223 ER - TY - JOUR AB - BACKGROUND. The fenestrated Fontan operation has been applied to high-risk patients with univentricular hearts, resulting in improved survival. The purpose of this study was to determine the hemodynamic factors responsible for these improved results. METHODS AND RESULTS. We performed the fenestrated Fontan operation in 17 high-risk patients with univentricular hearts (median age, 3 years; age range, 1.2-25 years). High-risk characteristics were depressed ventricular function and/or hypertrophy (n = 12), atrioventricular valve insufficiency (n = 5), pulmonary artery distortion (n = 6), elevated pulmonary vascular resistance (> 2 units/m2) (n = 4), previously failed Fontan operation (n = 2), or associated Wolff-Parkinson-White syndrome (n = 1). Intraoperative hemodynamic measurements (n = 8) included cardiac index (by aortic flow probe), peripheral arterial O2 saturations, and left and right atrial pressures during inflow occlusion, followed by volume loading with open versus closed fenestration. Mean baffle fenestration was 3.5 mm (range, 2.7-5.0 mm). Multiple regression analysis (cardiac index versus atrial pressure) revealed cardiac index was greater with open than with closed fenestration (p < 0.001) during volume loading. Oxygen delivery (cardiac index multiplied by oxygen content) was also greater with open than with closed fenestration (p < 0.001). Survival was 100% with a mean follow-up of 10.4 months; pleural drainage was high in two patients. Subsequent transcatheter fenestration closure resulted in increased O2 saturation (87 +/- 1% to 96 +/- 0.3%, p < 0.05). CONCLUSIONS. The fenestrated Fontan operation improves survival in high-risk patients by increasing cardiac index and maintaining oxygen delivery, despite mild arterial O2 desaturation. Subsequent transcatheter fenestration closure can be performed after hemodynamic assessment. AD - Division of Cardiovascular-Thoracic Surgery, Children's Memorial Hospital, Chicago, IL 60614. AN - 1424040 AU - Mavroudis, C. AU - Zales, V. R. AU - Backer, C. L. AU - Muster, A. J. AU - Latson, L. A. DA - Nov DB - PubMed DP - NLM ET - 1992/11/01 IS - 5 Suppl KW - Cardiac Catheterization/methods Cardiac Output/physiology Child, Preschool Female Heart Atria/*surgery Heart Defects, Congenital/mortality/physiopathology/*surgery Hemodynamics/*physiology Humans Intraoperative Care Male Myocardium/metabolism Oxygen Consumption/physiology Pulmonary Artery/*surgery Regression Analysis Risk Factors Time Factors LA - eng N1 - Mavroudis, C Zales, V R Backer, C L Muster, A J Latson, L A Journal Article United States Circulation. 1992 Nov;86(5 Suppl):II85-92. PY - 1992 SN - 0009-7322 (Print) 0009-7322 SP - Ii85-92 ST - Fenestrated Fontan with delayed catheter closure. Effects of volume loading and baffle fenestration on cardiac index and oxygen delivery T2 - Circulation TI - Fenestrated Fontan with delayed catheter closure. Effects of volume loading and baffle fenestration on cardiac index and oxygen delivery VL - 86 ID - 366 ER - TY - JOUR AB - BACKGROUND: Fenestration in the Fontan circulation potentially liberates patients from factors leading to cardiovascular remodelling, through stable haemodynamics with attenuated venous congestion. We hypothesised that a fenestrated Fontan procedure would possess chronic haemodynamic advantages beyond the preload preservation. METHODS: We enrolled 35 patients with fenestrated Fontan with a constructed pressure-volume relationship under dobutamine (DOB) infusion and/or transient fenestration occlusion (TFO). Despite the use of antiplatelets and anticoagulants, natural closure of fenestration was confirmed in 11 patients. Cardiovascular properties in patients with patent fenestration (P-F) were compared with those in patients with naturally closed fenestration (NC-F). To further delineate the roles of fenestration, paired analysis in patients with P-F was performed under DOB or rapid atrial pacing with/without TFO. RESULTS: As compared with P-F, patients with NC-F had a higher heart rate (HR), smaller ventricular end-diastolic area, better ejection fraction and higher central venous pressure, with higher pulmonary resistance. While this was similarly observed after DOB infusion, DOB markedly augmented diastolic and systolic ventricular stiffness in patients with NC-F compared with patients with P-F. As a mirror image of the relationship between patients with P-F and NC-F, TFO markedly reduced preload, suppressed cardiac output, and augmented afterload and diastolic stiffness. Importantly, rapid atrial pacing compromised these haemodynamic advantages of fenestration. CONCLUSIONS: As compared with patients with NC-F, patients with P-F had robust haemodynamics with secured preload reserve, reduced afterload and a suppressed beta-adrenergic response, along with a lower HR at baseline, although these advantages had been overshadowed, or worsened, by an increased HR. AD - Department of Pediatrics and Pediatric Cardiology, Kitasato University School of Medicine, Sagamihara, Japan. Department of Pediatric Cardiology, Saitama Medical University, Kawagoe, Japan. Department of Pediatrics, Hokkaido Ryoiku-en, Asahikawa, Japan. AN - 30826770 AU - Saiki, H. AU - Kuwata, S. AU - Iwamoto, Y. AU - Ishido, H. AU - Taketazu, M. AU - Masutani, S. AU - Nishida, T. AU - Senzaki, H. DA - Aug DB - PubMed DO - 10.1136/heartjnl-2018-314183 DP - NLM ET - 2019/03/04 IS - 16 KW - Adrenergic beta-1 Receptor Agonists Child Child, Preschool Dobutamine Echocardiography Echocardiography, Stress Female Fontan Procedure/*methods Heart Defects, Congenital/physiopathology/*surgery Heart Failure/prevention & control Heart Rate/*physiology Hemodynamics/*physiology Humans Hyperemia Male Myocardial Contraction/*physiology *Fontan physiology *congenital heart disease *congenital heart surgery *heart failure LA - eng N1 - 1468-201x Saiki, Hirofumi Kuwata, Seiko Iwamoto, Yoichi Ishido, Hirotaka Taketazu, Mio Masutani, Satoshi Nishida, Takashi Senzaki, Hideaki Orcid: 0000-0002-8289-0953 Journal Article Research Support, Non-U.S. Gov't England Heart. 2019 Aug;105(16):1266-1272. doi: 10.1136/heartjnl-2018-314183. Epub 2019 Mar 2. PY - 2019 SN - 1355-6037 SP - 1266-1272 ST - Fenestration in the Fontan circulation as a strategy for chronic cardioprotection T2 - Heart TI - Fenestration in the Fontan circulation as a strategy for chronic cardioprotection VL - 105 ID - 35 ER - TY - JOUR AB - AIM: The fetal or post-natal diagnosis of major congenital cardiac abnormality has important medical and psychological consequences. METHODS: We reviewed infants who underwent cardiac surgery in the first year of life at the Heart Centre for Children, The Children's Hospital at Westmead during 2009. The aims of this study were to: (i) examine the key features of cardiac diagnosis and clinical outcome, and (ii) consider how these data can inform priorities for the delivery of clinical services. RESULTS: Over the 12-month study period, a first cardiac surgical procedure was performed on 195 infants, with 85 infants (44%) diagnosed in the antenatal period. Of the total sample, a subset of 90 babies (46%) underwent their first procedure in the neonatal period, with 62% having had a fetal diagnosis. Major intracardiac lesions including truncus arteriosus (100%), single ventricular lesions (83%), pulmonary atresia with ventricular septal defect (78%) and transposition of the great arteries (53%) were diagnosed prior to birth. Improved haemodynamic stability at initial presentation was found in those with a fetal diagnosis. The overall mortality rate for all patients was 6.1% at 12 months, with a higher mortality in infants with single ventricle. CONCLUSIONS: The contemporary paradigm of care for infants with major congenital heart disease requires a multidisciplinary approach to care, with improvements in clinician-clinician and clinician-family communication, and psychological support and education for families. Changes in the allocation of resources are required to meet this model of best practice. AD - Heart Centre for Children, The Children's Hospital at Westmead, Westmead, Australia. garys@chw.edu.au AN - 21449901 AU - Sholler, G. F. AU - Kasparian, N. A. AU - Pye, V. E. AU - Cole, A. D. AU - Winlaw, D. S. DA - Oct DB - PubMed DO - 10.1111/j.1440-1754.2011.02039.x DP - NLM ET - 2011/04/01 IS - 10 KW - Family/*psychology Female Gestational Age Heart Defects, Congenital/*diagnosis/mortality/surgery Humans Infant Mortality Infant, Newborn Medical Audit New South Wales Outcome Assessment, Health Care *Postnatal Care *Prenatal Diagnosis LA - eng N1 - 1440-1754 Sholler, Gary F Kasparian, Nadine A Pye, Victoria E Cole, Andrew D Winlaw, David S Journal Article Research Support, Non-U.S. Gov't Australia J Paediatr Child Health. 2011 Oct;47(10):717-22. doi: 10.1111/j.1440-1754.2011.02039.x. Epub 2011 Mar 30. PY - 2011 SN - 1034-4810 SP - 717-22 ST - Fetal and post-natal diagnosis of major congenital heart disease: implications for medical and psychological care in the current era T2 - J Paediatr Child Health TI - Fetal and post-natal diagnosis of major congenital heart disease: implications for medical and psychological care in the current era VL - 47 ID - 275 ER - TY - JOUR AB - A prospective observational study at the newly established Fetal Medicine Unit, Hamad Hospital, Qatar, evaluated the impact of the service on the detection rate of critical congenital heart defects, patterns of referral and subsequent yield for structural congenital heart disease in a population with a significant proportion of high risk factors. Of 391 pregnant females examined between January 2003 and December 2004, 58 (14.8%) had fetal cardiac abnormalities of which 23 (5.8% of total referrals) had major structural malformations of the heart. Cases of fetal congenital heart disease had further evaluation using real time three-dimensional echocardiography (RT3DE) which is new equipment in the paediatric cardiology department. All cases with cardiac defects whether minor or major had follow up fetal echocardiography. Neonatal echocardiography confirmed the diagnosis in all cases with major defects (100% specificity). False positive cases that were found to be normal post natal were 1% of the total cases referred (12% of cases with congenital malformation). False negative cases were 1% and all had a small ventricular septal defect (VSD) except for one Down's syndrome with a very large VSD. Three patients needed urgent Caesarean section (CS) deliveries, one with complete heart block (HB) and two with supraventricular tachycardia (SVT). One patient traveled abroad as the fetus had left isomerism and major cardiac defects and complete HB. Fifteen newborns had to receive prostaglandin based on thefetal diagnosis before being seen by paediatric cardiologists. There was no termination of pregnancy due to major cardiac defects even in cases of HLHS. The preliminary results of this clinic are very satisfactory and have affected favorably the outcome of the new-borns with congenital heart defects. It is hoped that the results of this study will encourage more referrals to the FMU. AD - B. Ahmed, Obstetrics and Gynecology Department, Women's Hospital, Hamad Medical Corporation, Doha, Qatar AU - Ahmed, B. AU - El Sisi, A. AU - Khenyab, N. AU - Saleh, N. AU - Al Mansoori, Z. AU - Gendi, S. AU - El Said, H. AU - Numan, M. DB - Embase IS - 1 KW - antiarrhythmic agent prostaglandin adult article artificial heart pacemaker cesarean section complete heart block congenital heart disease congenital heart malformation diagnostic accuracy disease predisposition Down syndrome early diagnosis Ebstein anomaly echocardiography false negative result false positive result female fetus fetus echography fetus hydrops follow up great vessels transposition health care delivery health care quality heart right ventricle double outlet heart single ventricle heart surgery heart ventricle septum defect high risk population hospital department human hypoplastic left heart syndrome pulmonary valve atresia major clinical study newborn newborn disease observational study outcome assessment patient referral pregnancy termination prenatal diagnosis prospective study Qatar rhabdomyoma risk factor sensitivity and specificity supraventricular tachycardia three dimensional echocardiography travel LA - English M3 - Article N1 - L44349558 2006-10-01 PY - 2006 SN - 0253-8253 SP - 31-33 ST - Fetal echocardiography service in Qatar: Establishment, challenges and outcome T2 - Qatar Medical Journal TI - Fetal echocardiography service in Qatar: Establishment, challenges and outcome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L44349558 VL - 15 ID - 1271 ER - TY - JOUR AB - We evaluated differences in growth between fetuses with and without congenital heart disease (CHD) and tested associations between growth and early childhood neurodevelopment (ND). In this prospective cohort study, fetuses with hypoplastic left heart syndrome (HLHS), transposition of the great arteries (TGA), and tetralogy of Fallot (TOF) and controls had biparietal diameter (BPD), head (HC) and abdominal circumference (AC), femur length (FL), and estimated fetal weight (EFW) recorded serially during pregnancy at 18-26 weeks GA (F1), at 27-33 weeks GA (F2), and at 34-40 weeks GA (F3). CHD subjects underwent Bayley Scales of Infant Development-III ND testing at 18 months. Differences between CHD fetuses and controls were assessed using t tests and generalized linear modeling. Correlations between biometry and ND informed regression modeling. We enrolled 41 controls and 68 fetuses with CHD (N = 24 HLHS, N = 21 TGA, N = 23 TOF), 46 of whom had ND scores available. At 18-26 weeks, CHD fetuses were smaller than controls in all biometric parameters. Differences in growth rates were observed for HC, BPD, and AC, but not for FL or EFW. Cognitive score correlated with HC/AC at F2 (r = -0.33, P = 0.04) and mean HC/AC across gestation (r = -0.35, P = 0.03). Language correlated with FL/BPD at F2 (r = 0.34, P = 0.04). In stepwise linear regression, mean HC/AC predicted Cognition (B = -102, P = 0.026, R (2) = 0.13) and FL/BPD at F2 predicted Language score (B = 127, P = 0.03, R (2) = 0.12). Differences in growth between CHD fetuses and controls can be measured early in pregnancy. In CHD fetuses, larger abdominal relative to head circumference is associated with better 18-month neurodevelopment. AD - Division of Pediatric Cardiology, Department of Pediatrics, Morgan Stanley Children's Hospital of New York-Presbyterian, Columbia University Medical Center, 3959 Broadway, New York, NY, 10032, USA, iib6@columbia.edu. AN - 25753684 AU - Williams, I. A. AU - Fifer, W. P. AU - Andrews, H. C2 - PMC5289064 C6 - NIHMS845160 DA - Aug DB - PubMed DO - 10.1007/s00246-015-1132-6 DP - NLM ET - 2015/03/11 IS - 6 KW - Abdomen/growth & development Anthropometry *Cognition Cohort Studies Female *Fetal Development Gestational Age Head/growth & development Heart Defects, Congenital/*diagnosis/diagnostic imaging/physiopathology/*psychology Humans Hypoplastic Left Heart Syndrome/diagnosis/psychology Infant Infant, Newborn *Language Linear Models Male Pregnancy Prospective Studies Tetralogy of Fallot/diagnosis/psychology Transposition of Great Vessels/diagnosis/psychology *Ultrasonography, Prenatal LA - eng N1 - 1432-1971 Williams, Ismée A Fifer, William P Andrews, Howard K23 HD061601/HD/NICHD NIH HHS/United States UL1 TR000040/TR/NCATS NIH HHS/United States 1K23HD061601/HD/NICHD NIH HHS/United States Journal Article Research Support, N.I.H., Extramural Pediatr Cardiol. 2015 Aug;36(6):1135-44. doi: 10.1007/s00246-015-1132-6. Epub 2015 Mar 10. PY - 2015 SN - 0172-0643 (Print) 0172-0643 SP - 1135-44 ST - Fetal Growth and Neurodevelopmental Outcome in Congenital Heart Disease T2 - Pediatr Cardiol TI - Fetal Growth and Neurodevelopmental Outcome in Congenital Heart Disease VL - 36 ID - 264 ER - TY - JOUR AB - Although the majority of newly born babies will establish normal respiratory and circulatory function without help, 1-2% might run into difficulties because of a disturbance in the normal adaptive processes required for a smooth transition from intrauterine to extrauterine life. An understanding of the normal and abnormal perinatal physiology is important to appreciate the practical differences in the approach to caring for such babies, and also for avoiding actions that might be detrimental in the longer term. © 2006 Elsevier Ltd. All rights reserved. AD - S.K. Sinha, The James Cook University Hospital, University of Durham, Marton Road, Middlesborough, TS4 3BW, United Kingdom AU - Sinha, S. K. AU - Donn, S. M. DB - Embase Medline DO - 10.1016/j.siny.2006.01.008 IS - 3 KW - analgesic agent anesthetic agent corticosteroid magnesium sulfate nitric oxide prostacyclin sedative agent sildenafil surfactant tolazoline vasodilator agent anemia aortic stenosis article artificial ventilation baby birth birth injury central nervous system malformation circulation congenital diaphragm hernia cyanotic heart disease cystic adenomatoid malformation extracorporeal oxygenation fetal hemorrhage fetus hypoxia heart right left shunt human hypercapnia hyperviscosity syndrome hypoplastic left heart syndrome hypotension hypoventilation hypoxemia infection life lung hypoplasia maladjustment meconium aspiration metabolism nervous system injury neuromuscular disease newborn care oxygen therapy perinatal period pneumonia prenatal period pulmonary hypertension respiration depression respiratory acidosis respiratory distress respiratory distress syndrome respiratory function resuscitation sepsis thermoregulation tricuspid valve atresia LA - English M3 - Article N1 - L43626241 2006-05-31 PY - 2006 SN - 1744-165X SP - 166-173 ST - Fetal-to-neonatal maladaptation T2 - Seminars in Fetal and Neonatal Medicine TI - Fetal-to-neonatal maladaptation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L43626241 http://dx.doi.org/10.1016/j.siny.2006.01.008 VL - 11 ID - 1260 ER - TY - JOUR AB - We are developing an intravascular axial flow blood pump to support adolescent and adult Fontan patients. To protect the blood vessel, this pump has an outer cage with radially arranged filaments and a newly designed spindle at the pump outlet. The outlet spindle is included to limit the axial movement of the rotor and to house bearings that support the rotor. This study evaluates the impact of the outlet spindle on pump performance using computational fluid dynamics (CFD) and experimental testing of a prototype configuration. We measured the pressure-flow performance of the prototype with a protective cage using a blood analog fluid. The pump with the cage filaments and spindle generated 1 to 16mmHg of pressure rise for flow rates of 1 to 4L/min at 4000 to 7000rpm. The difference between the CFD predictions and experimental results was found to be approximately 9.8%. Scalar stress levels remained below 570Pa with exposure times on the order of 1.5s. These results are acceptable and support the continued development of this cavopulmonary assist device with an outlet spindle to reinforce the protective cage filament design. AD - BioCirc Research Laboratory, Department of Mechanical Engineering, Virginia Commonwealth University, Richmond, VA 23284, USA. althrock@vcu.edu AN - 21092047 AU - Throckmorton, A. L. AU - Kapadia, J. Y. AU - Wittenschlaeger, T. M. AU - Medina, T. J. AU - Hoang, H. Q. AU - Bhavsar, S. S. DA - Nov DB - PubMed DO - 10.1111/j.1525-1594.2010.01139.x DP - NLM ET - 2010/11/26 IS - 11 KW - Adolescent Adult Blood Flow Velocity Blood Pressure Computer Simulation Fontan Procedure/*instrumentation *Heart-Assist Devices *Hemodynamics Humans Materials Testing Models, Cardiovascular Numerical Analysis, Computer-Assisted Prosthesis Design Regression Analysis Stress, Mechanical LA - eng N1 - 1525-1594 Throckmorton, Amy L Kapadia, Jugal Y Wittenschlaeger, Thomas M Medina, Tanisha J Hoang, Hien Q Bhavsar, Sonya S Evaluation Study Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, Non-P.H.S. United States Artif Organs. 2010 Nov;34(11):1039-44. doi: 10.1111/j.1525-1594.2010.01139.x. PY - 2010 SN - 0160-564x SP - 1039-44 ST - Filament support spindle for an intravascular cavopulmonary assist device T2 - Artif Organs TI - Filament support spindle for an intravascular cavopulmonary assist device VL - 34 ID - 479 ER - TY - JOUR AB - Objectives: To describe the first Lebanese fetal echocardiography experience for prenatal diagnosis of congenital heart diseases (CHD), showcase successes, and hurdles. Methods: This was a retrospective study from January 2014 to December 2017. A total of 350 fetal echocardiograms for 299 fetuses were performed at the Children's Heart Center at the American University of Beirut, the only fetal center in Lebanon. Data were collected regarding diagnosis, reasons for referral, and timing of referral. Results: The mean gestational age at presentation was 25.3 weeks (standard deviation 4.9 weeks). The primary reasons for referral were abnormal anomaly scan (81 27%), history of previous child with CHD (48 16%), and pre-existing maternal congenital heart disease (15 5%). A total of 144 fetal echocardiograms were normal and 155 patients were diagnosed prenatally with CHD giving a detection rate of 44%. The most identified cardiac lesions were ventricular septal defects (31, 20%), atrial septal defects (15, 9.7%). Significant CHD defined as major abnormalities which would impact pregnancy and future quality of life of the baby were identified in 78 fetuses, with a detection rate of 22%. Conclusion: High rates of detection are mainly due to low rates of referral when indicated and possibly parental anxiety regarding CHD diagnosis. AD - M. Arabi, Director, Fetal Heart Program – Children's Heart Center, Department of Pediatrics and Adolescent Medicine, American University of Beirut Medical Center, Riad El-Solh, Beirut, Lebanon AU - Charafeddine, F. AU - Hachem, A. AU - Kibbi, N. AU - Abutaqa, M. AU - Bitar, F. AU - Bulbul, Z. AU - El-Rassi, I. AU - Arabi, M. DB - Embase DO - 10.1016/j.jsha.2019.04.001 IS - 3 KW - arterial trunk article congenital heart disease Ebstein anomaly Fallot tetralogy fetus fetus electrocardiography gestational age great vessels transposition heart atrium septum defect heart center heart left right shunt heart outflow tract obstruction heart right ventricle double outlet heart ventricle septum defect human hypoplastic left heart syndrome Lebanon major clinical study patient referral prenatal diagnosis priority journal quality of life retrospective study LA - English M3 - Article N1 - L2001909886 2019-05-14 2019-05-15 PY - 2019 SN - 2212-5043 1016-7315 SP - 125-129 ST - The first Fetal Echocardiography experience for Prenatal diagnosis of Congenital Heart Disease in Lebanon: Successes and challenges T2 - Journal of the Saudi Heart Association TI - The first Fetal Echocardiography experience for Prenatal diagnosis of Congenital Heart Disease in Lebanon: Successes and challenges UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2001909886 http://dx.doi.org/10.1016/j.jsha.2019.04.001 VL - 31 ID - 608 ER - TY - JOUR AB - Background: The treatment of advanced heart failure (HF) in children and infants poses a serious management problem. Heart failure in that patient group is usually of congenital aetiology. The treatment schedules for paediatric patients are in most cases adapted from the guidelines for treatment of adults. Up to 2009, the treatment of that extremely difficult group of patients was limited to pharmacological therapy and occasional heart transplantations. Constantly increasing problems with recruiting donors, especially for the paediatric group, contribute to the fact that mechanical support with the use of ventricular assist devices is for many children the only chance of surviving the period of waiting for a heart donor. Aim: The aim of the study was to analyse the outcomes of circulatory support in Poland and to assess the advisability of this method for treatment of children with severe HF. Methods: This treatment of paediatric patients is currently used in three Polish centres. From December 28, 2009 to August 1, 2015, 27 implantations of BerlinHeart EXCOR® mechanical circulatory support system were performed in children aged from one month to 16 years (10 patients below one year of age; 37%). Left ventricular assist devices were implanted to 21 patients, whereas the remaining children received biventricular support. The most common reason for using this method was HF developed in the course of cardiomyopathy. In one case, HF after Fontan operation was the indication. Results: The duration of the circulatory support period ranged from six to 1215 days. It was followed by successful heart transplantations in 10 (37%) patients, in five (18.1%) it resulted in regeneration of the heart, enabling explantation of the device, whereas three children are still waiting for transplantations. Nine (33%) children died during the therapy because of thromboembolic complications. Conclusions: As follows from our data, circulatory support utilising the BerlinHeart EXCOR® system is an effective and promising method used as a bridge to cardiac transplantation, or for regeneration of the myocardium in paediatric patients. In the group of the youngest and the most difficult patients, the method requires close cooperation of the medical and nursing personnel. AD - S. Pawlak, Department of Cardiac Surgery, Heart Transplantation and Mechanical Circulatory Support for Children, Silesian Centre for Heart Diseases, ul. M. Skłodowskiej-Curie 9, Zabrze, Poland AU - Pawlak, S. AU - Przybylski, R. AU - Skalski, J. AU - Śliwka, J. AU - Kansy, A. AU - Grzybowski, A. AU - Wierzyk, A. AU - Białkowski, J. AU - Maruszewski, B. AU - Zembala, M. DB - Embase Medline DO - 10.5603/KP.a2017.0201 IS - 1 KW - BerlinHeart EXCOR biventricular assist device heart assist device left ventricular assist device LEVITRONIX ventricular assist device acetylsalicylic acid alteplase antivitamin K dipyridamole heparin inotropic agent low molecular weight heparin prostacyclin activated partial thromboplastin time adolescent article cardiomyopathy cardiopulmonary insufficiency child childhood mortality clinical article clinical effectiveness cooperation device removal device therapy disease severity enteric feeding extracorporeal oxygenation female Fontan procedure heart failure heart transplantation human infant international normalized ratio lung embolism male medical staff nursing staff Poland Polish citizen retrospective study rotary pump support survival rate thromboembolism thrombosis treatment duration treatment indication LA - English M3 - Article N1 - L620405034 2018-02-05 2019-06-13 PY - 2018 SN - 1897-4279 0022-9032 SP - 83-90 ST - First Polish analysis of the treatment of advanced heart failure in children with the use of BerlinHeart EXCOR® mechanical circulatory support T2 - Kardiologia Polska TI - First Polish analysis of the treatment of advanced heart failure in children with the use of BerlinHeart EXCOR® mechanical circulatory support UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L620405034 http://dx.doi.org/10.5603/KP.a2017.0201 VL - 76 ID - 721 ER - TY - JOUR AB - Objective: To evaluate the performance of first trimester biochemical markers, pregnancy-associated plasma protein-A (PAPP-A), free beta human chorionic gonadotropin (fβ-hCG), and nuchal translucency (NT) in detection of severe congenital heart defects (CHDs). Methods: During the study period from 1 January 2008 to 31 December 2011, biochemical markers and NT were measured in 31,144 women as part of voluntary first trimester screening program for Down’s syndrome in Northern Finland. Data for 71 severe CHD cases and 762 controls were obtained from the hospital records and from the National Medical Birth Register, which records the birth of all liveborn and stillborn infants, and from the National Register of Congenital Malformations that receives information about all the CHD cases diagnosed in Finland. Results: Both PAPP-A and fβ-hCG multiple of median (MoM) values were decreased in all severe CHDs: 0.71 and 0.69 in ventricular septal defects (VSDs), 0.58 and 0.88 in tetralogy of Fallot cases (TOFs), 0.82 and 0.89 in hypoplastic left heart syndromes (HLHSs), and 0.88 and 0.96 in multiple defects, respectively. NT was increased in all study groups except of VSD group. ROC AUC was 0.72 for VSD when combining prior risk with PAPP-A and fβ-hCG. Adding NT did not improve the detection rate. With normal NT but decreased (<0.5 MoM) PAPP-A and fβ-hCG odds ratios for VSD and HLHS were 19.5 and 25.6, respectively. Conclusions: Maternal serum biochemistry improves the detection of CHDs compared to NT measurement only. In cases with normal NT measurement but low concentrations of both PAPP-A and fβ-hCG, an alert for possible CHD, especially VSD, could be given with thorough examination of fetal heart in later ultrasound scans. AD - J. Nevalainen, Department of Obstetrics and Gynecology, 90100 OYS, Oulu University Hospital, Oulu, Finland AU - Alanen, J. AU - Korpimaki, T. AU - Kouru, H. AU - Sairanen, M. AU - Leskinen, M. AU - Gissler, M. AU - Ryynanen, M. AU - Nevalainen, J. DB - Embase Medline DO - 10.1080/14767058.2018.1462324 IS - 19 KW - chorionic gonadotropin beta subunit pregnancy associated plasma protein A adult article case control study congenital heart malformation controlled study Down syndrome embryo Fallot tetralogy female fetus fetus echography fetus heart Finland first trimester pregnancy heart ventricle septum defect human hypoplastic left heart syndrome live birth major clinical study maternal serum newborn nuchal translucency measurement pregnancy outcome prenatal screening priority journal prospective study receiver operating characteristic stillbirth LA - English M3 - Article N1 - L621796343 2018-04-30 2019-07-19 PY - 2019 SN - 1476-4954 1476-7058 SP - 3272-3277 ST - First trimester combined screening biochemistry in detection of congenital heart defects T2 - Journal of Maternal-Fetal and Neonatal Medicine TI - First trimester combined screening biochemistry in detection of congenital heart defects UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L621796343 http://dx.doi.org/10.1080/14767058.2018.1462324 VL - 32 ID - 592 ER - TY - JOUR AB - Purpose: Bupropion is a drug uniquely used both to treat depression and as an aid to smoking cessation. We investigated previously reported associations between first-trimester exposure to bupropion and cardiac defects. Methods: Using data gathered since 2003 by the Slone Epidemiology Center's Case-control Birth Defects Study, we classified subjects with cardiac defects into subgroups. Exposure categories included first-trimester bupropion alone or in combination with other antidepressants, first-trimester antidepressants other than bupropion, and no exposure to any antidepressant at any time from 2months prior to pregnancy through delivery. We calculated odds ratios and 95% confidence intervals, controlling for confounding using logistic regression. Results: There were 8611 non-malformed infants and 7913 infants with cardiac defects. Eight cardiac subgroups had sufficient subjects (two or more exposed cases) for analysis. The adjusted odds ratio (aOR) for first-trimester bupropion use in relation to ventricular septal defect (VSD) was slightly elevated (1.6, 95% confidence interval 1.0-2.8); for exposure to bupropion alone, the aOR was 2.5 (95% confidence interval 1.3-5.0). Risks were not materially elevated for bupropion in relation to the other seven cardiac subgroups. Conclusions: We did not confirm previously reported associations for left-sided defects overall but had too few exposed cases to evaluate specific defects in this category. We did observe an elevated risk of VSD following first-trimester bupropion use, particularly when used without other antidepressants. This pattern for bupropion alone was observed in all our risk comparisons and was not explained by higher doses or gestational timing. AD - C. Louik, Slone Epidemiology Center, Boston University, 1010 Commonwealth Ave., Boston, MA, United States AU - Louik, C. AU - Kerr, S. AU - Mitchell, A. A. DB - Embase Medline DO - 10.1002/pds.3661 IS - 10 KW - amfebutamone antidepressant agent adult aortic coarctation article cardiovascular risk congenital heart malformation controlled study disease association drug indication drug safety female first trimester pregnancy heart left ventricle outflow tract obstruction heart ventricle septum defect high risk infant human hypoplastic left heart syndrome infant major clinical study pregnancy outcome prenatal drug exposure risk assessment LA - English M3 - Article N1 - L53193487 2014-06-17 2017-07-20 PY - 2014 SN - 1099-1557 1053-8569 SP - 1066-1075 ST - First-trimester exposure to bupropion and risk of cardiac malformations T2 - Pharmacoepidemiology and Drug Safety TI - First-trimester exposure to bupropion and risk of cardiac malformations UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L53193487 http://dx.doi.org/10.1002/pds.3661 VL - 23 ID - 909 ER - TY - JOUR AB - Aim: We investigated the prevalence of Down syndrome in a nationwide birth cohort, focusing on congenital heart defects (CHDs), their associations with extracardiac malformations (ECM) and survival. Methods: National registers were used to identify Norwegian births (1994–2009) and deaths (1994–2014) and updated with hospital diagnoses. We estimated birth defect frequencies in Down syndrome and the general population, the association between CHDs and ECM and hazard ratios for death from different combinations of CHDs and ECM. Results: Down syndrome was found in 1672 of 953 450 births (17.6 per 10 000). Of the 1251 live births (13.3 per 10 000), 58% had CHD and 9% ECM. CHDs were associated with oesophageal atresia (p = 0.02) and Hirschsprung's disease (p = 0.03) but with no other malformations. The five-year survival for Down syndrome increased from 91.8% (1994–1999) to 95.8% (2000–2009) (p = 0.006), and overall survival was 92.0% with CHD and 97.4% without. Compared with Down syndrome children without CHD or ECM, the five-year mortality was similar for those with nonsevere CHDs, without or with ECM, but 4–7 times higher in those with severe CHDs without ECM and 13–28 times higher in those with severe CHDs and ECM. Conclusion: Down syndrome childhood survival improved, but mortality remained high with severe CHDs and extracardiac defects. AD - K. Brodwall, Department of Global Public Health and Primary Care, Faculty of Medicine, University of Bergen, Bergen, Norway AU - Brodwall, K. AU - Greve, G. AU - Leirgul, E. AU - Klungsøyr, K. AU - Holmstrøm, H. AU - Vollset, S. E. AU - Øyen, N. DB - Embase Medline DO - 10.1111/apa.14223 IS - 5 KW - aortic valve stenosis article atrioventricular septal defect cardiovascular mortality child childhood mortality congenital heart malformation congenital malformation controlled study disease association disease severity Down syndrome esophagus atresia extracardiac malformation Fallot tetralogy heart left ventricle outflow tract obstruction heart right ventricle double outlet Hirschsprung disease human hypoplastic left heart syndrome ICD-10 ICD-9 live birth lung vein drainage anomaly major clinical study maternal age overall survival prevalence priority journal stillbirth LA - English M3 - Article N1 - L620605507 2018-02-13 2018-04-25 PY - 2018 SN - 1651-2227 0803-5253 SP - 845-853 ST - The five-year survival of children with Down syndrome in Norway 1994–2009 differed by associated congenital heart defects and extracardiac malformations T2 - Acta Paediatrica, International Journal of Paediatrics TI - The five-year survival of children with Down syndrome in Norway 1994–2009 differed by associated congenital heart defects and extracardiac malformations UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L620605507 http://dx.doi.org/10.1111/apa.14223 VL - 107 ID - 688 ER - TY - JOUR AB - Aim: To determine survival of infants with major congenital anomalies (CA) and assess the effect of co-existing anomalies and gestational age. Methods: All liveborn infants with major CA born in New South Wales (NSW), Australia, 2004–2009 were identified from the NSW Register of Congenital Conditions. Deaths were identified via record linkage to death registrations and five-year survival was estimated using Kaplan–Meier methods. Results: There were 8521 liveborn infants with CA of whom 617 (7.2%) died within the first five years of life. Half of deaths occurred in the first week of life. The overall five-year survival rate was 92.8% (95%CI: 92.2–93.3) and 83.2% (95%CI: 79.0–87.4) for syndromes, 83.4% (95%CI: 80.9–85.9) for multiple, 85.1% (95%CI: 82.6–87.5) for chromosomal, 95.3% (95%CI: 94.8–95.8) for isolated and 96.2% (95%CI: 94.3–98.1) for non-Q chapter anomalies. Five-year survival for chromosomal, syndromes and sub-groups was higher for isolated compared with multiple anomalies ranging from 77.5% to 98.9% and 68.6% to 89.5%, respectively. Survival was lower for preterm (79.4%; 95%CI: 77.5-81.4) than for term infants (95.8%; 95%CI: 95.3–96.3). Conclusion: Nine in ten infants with major CA survive up to five years, although there is variability in survival across CA groups. Survival of infants with major congenital anomalies has improved in recent years. AD - F.J. Schneuer, Child Population and Translational Health Research, The Children's Hospital at Westmead Clinical School, The University of Sydney, Sydney, NSW, Australia AU - Schneuer, F. J. AU - Bell, J. C. AU - Shand, A. W. AU - Walker, K. AU - Badawi, N. AU - Nassar, N. DB - Embase Medline DO - 10.1111/apa.14833 IS - 11 KW - anencephalus anus atresia aortic coarctation aortic valve stenosis arthrogryposis article atrioventricular septal defect beta thalassemia bile duct atresia bladder exstrophy breast malformation childhood mortality choana atresia cleft lip cleft lip palate cleft palate clubfoot comorbidity congenital hypothyroidism congenital malformation controlled study corpus callosum agenesis craniofacial synostosis cystic fibrosis diaphragm hernia Down syndrome duodenum atresia duodenum stenosis Edwards syndrome encephalocele epispadias esophagus atresia esophagus fistula Fallot tetralogy fetus hydrops gastroschisis gestational age great vessels transposition heart atrium septum defect heart ventricle septum defect Hirschsprung disease holoprosencephaly human hydrocephalus hydronephrosis hypoplastic left heart syndrome hypospadias kidney agenesis kidney malformation kidney polycystic disease Klinefelter syndrome limb reduction defect lung adenoma lung sequestration lung vein drainage anomaly major clinical study malrotation syndrome microcephaly mortality rate multiple malformation syndrome neural tube defect New South Wales newborn omphalocele optic nerve hypoplasia patent ductus arteriosus phenylketonuria polydactyly Potter syndrome prematurity priority journal prune belly syndrome pseudohermaphroditism pulmonary valve stenosis small intestine disease spinal dysraphism spine malformation survival survival rate syndactyly term birth thorax malformation trisomy 13 Turner syndrome urethra malformation LA - English M3 - Article N1 - L628179265 2019-06-25 2019-11-07 PY - 2019 SN - 1651-2227 0803-5253 SP - 2008-2018 ST - Five-year survival of infants with major congenital anomalies: a registry based study T2 - Acta Paediatrica, International Journal of Paediatrics TI - Five-year survival of infants with major congenital anomalies: a registry based study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L628179265 http://dx.doi.org/10.1111/apa.14833 VL - 108 ID - 591 ER - TY - JOUR AB - Children born with single ventricle heart defects typically undergo a staged surgical procedure culminating in a total cavopulmonary connection (TCPC) or Fontan surgery. The goal of this work was to perform physiologic, patient-specific hemodynamic simulations of two post-operative TCPC patients by using fluid-structure interaction (FSI) simulations. Data from two patients are presented, and post-op anatomy is reconstructed from MRI data. Respiration rate, heart rate, and venous pressures are obtained from catheterization data, and inflow rates are obtained from phase contrast MRI data and are used together with a respiratory model. Lumped parameter (Windkessel) boundary conditions are used at the outlets. We perform FSI simulations by using an arbitrary Lagrangian-Eulerian finite element framework to account for motion of the blood vessel walls in the TCPC. This study is the first to introduce variable elastic properties for the different areas of the TCPC, including a Gore-Tex conduit. Quantities such as wall shear stresses and pressures at critical locations are extracted from the simulation and are compared with pressure tracings from clinical data as well as with rigid wall simulations. Hepatic flow distribution and energy efficiency are also calculated and compared for all cases. There is little effect of FSI on pressure tracings, hepatic flow distribution, and time-averaged energy efficiency. However, the effect of FSI on wall shear stress, instantaneous energy efficiency, and wall motion is significant and should be considered in future work, particularly for accurate prediction of thrombus formation. AD - University of California, San Diego, 9500 Gilman Drive, Mail Code 0411, La Jolla, CA, 92093-0411, USA. AN - 25099455 AU - Long, C. C. AU - Hsu, M. C. AU - Bazilevs, Y. AU - Feinstein, J. A. AU - Marsden, A. L. DA - May DB - PubMed DO - 10.1002/cnm.1485 DP - NLM ET - 2012/05/01 IS - 5 KW - Biomechanical Phenomena/*physiology Child Child, Preschool *Computer Simulation Female Finite Element Analysis *Fontan Procedure *Heart Defects, Congenital/pathology/physiopathology/surgery Hemodynamics/*physiology Humans Male *Models, Cardiovascular Pressure Stress, Mechanical Fontan procedure congenital heart disease finite element method fluid-structure interaction LA - eng N1 - 2040-7947 Long, C C Hsu, M-C Bazilevs, Y Feinstein, J A Marsden, A L Journal Article Research Support, Non-U.S. Gov't England Int J Numer Method Biomed Eng. 2012 May;28(5):513-27. doi: 10.1002/cnm.1485. Epub 2012 Jan 17. PY - 2012 SN - 2040-7939 SP - 513-27 ST - Fluid-structure interaction simulations of the Fontan procedure using variable wall properties T2 - Int J Numer Method Biomed Eng TI - Fluid-structure interaction simulations of the Fontan procedure using variable wall properties VL - 28 ID - 332 ER - TY - JOUR AB - Presents a collection of abstracts from the Journal of Pediatrics. The main theme of the abstracts is treatment and prevalence of different disorders in pediatrics. The first article is a study which provides pivotal evidence in support of simplifying Pneumocystis pneumonia (PCP) prophylaxis standard of care across specialties and countries. Next article discusses significant differences in the perception of understanding of parents, with healthcare providers. In next article, author report an examination of emergency department records from 3 years in the very busy Children’s Medical Center Dallas trauma center. These data may become useful in designing preventive strategies, especially educational programs for teenage parents. Next article report a retrospective study of children born preterm or at early term with regards to risk of asthma. Next article presents rate of readmission of children with asthma. Next article provides some insights into the epidemiology of accidental injuries in children. Next article presents the prevalence of feeding dysfunction in children with single ventricle and to identify associated risk factors. Next article discusses the functional hearing deficit in children. Next article discusses convulsive status epilepticus among children. (PsycINFO Database Record (c) 2016 APA, all rights reserved) AN - 2014-02638-011 AU - Long, Sarah S. DB - psyh DO - 10.1016/j.jpeds.2013.11.054 DP - EBSCOhost IS - 2 KW - speech perceptions FM listening systems autism pediatrics status epilepticus Autism Spectrum Disorders Risk Factors Speech Perception N1 - Risk Factors. Minor Descriptor: Pediatrics; Speech Perception; Status Epilepticus. Classification: Developmental Disorders & Autism (3250). Population: Human (10). Page Count: 3. Issue Publication Date: Feb, 2014. Copyright Statement: Mosby, Inc. 2014. PY - 2014 SN - 0022-3476 SP - 223-225 ST - FM-listening systems improve speech perception in some children with autism T2 - The Journal of Pediatrics TI - FM-listening systems improve speech perception in some children with autism UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2014-02638-011&site=ehost-live&scope=site VL - 164 ID - 1678 ER - TY - JOUR AB - The update course in foetal cardiology held by the Fetal Working Group of the Association for European Paediatric and Congenital Cardiology in Istanbul in May 2012 included a session on foetal cardiac therapy. In the introductory overview to this symposium, we critically examine the level of evidence supporting or refuting proposed foetal cardiac therapies including transplacental treatment of foetal tachyarrhythmias, steroid treatment in foetal atrioventricular block, and foetal aortic valvuloplasty. In summary, the evidence for the ef ficiency and safety of currently available foetal cardiac therapies is low, with no therapy based on a randomised controlled trial. Transplacental treatment of foetal tachycardia is generally accepted as effective and safe, based on extensive and widespread clinical experience; however, there is no consensus on which drugs are the most effective in different electrophysiological situations. Randomised studies may be able to resolve this, but this is complicated because tachyarrhythmias are relatively rare conditions, the foetus is not accessible for direct treatment, and it is the healthy mother who accepts treatment she does not need on behalf of her foetus. The indications for steroid treatment in foetal atrioventricular block and for foetal valvuloplasty are even more controversial. Although randomised trials would be desirable, the practical issues of recruiting sufficient sample sizes and controlling for variation in practice across multiple sites is not to be underestimated. Multicentre registries, analysed free of bias, may be an alternative way to improve the evidence base of foetal cardiac therapy. AD - M. Mellander, Department of Paediatric Cardiology, Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Göteborg, Sweden AU - Mellander, M. AU - Gardiner, H. DB - Embase Medline DO - 10.1017/S1047951114001231 KW - antiarrhythmic agent digoxin flecainide fluorinated steroid placebo sotalol steroid aortic valve stenosis article atrioventricular block cardiomyopathy drug efficacy drug response electrophysiology fetal aortic valve stenosis fetal atrioventricular block fetal balloon valvuloplasty fetal heart block fetal tachycardia fetal therapy fetus disease heart block hepatitis human hypoplastic left heart syndrome myocarditis outcome assessment prenatal care prognosis pulmonary atresia intact ventricular septum pulmonary valve stenosis quality of life safety sinus rhythm tachycardia treatment indication valvuloplasty LA - English M3 - Article N1 - L600654348 2014-12-09 2014-12-16 PY - 2014 SN - 1467-1107 1047-9511 SP - 36-40 ST - Foetal therapy, what works? An overview T2 - Cardiology in the Young TI - Foetal therapy, what works? An overview UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L600654348 http://dx.doi.org/10.1017/S1047951114001231 VL - 24 ID - 967 ER - TY - JOUR AB - Introduction: Subclinical hypothyroidism or mild thyroid failure is a common problem in patients without known thyroid disease. Methods: Demographic and analytical data were collected in 309, of which 181 were Male and 128 were feMale, congenital heart disease (CHD) patients. CHD patients with thyroid-stimulating hormone above 5.5 mIU/L were also followed up from an analytical point of view to determine changes in serum glucose, cholesterol, N-terminal pro b-type natriuretic peptide, and C-reactive protein concentrations. Results: Of the CHD patients, 35 (11.3%) showed thyroid-stimulating hormone concentration above 5.5 mIU/L. Of them, 27 were followed up during 2.4±1.2 years - 10 were under thyroid hormone replacement treatment, and 17 were not. Of the 27 patients (25.9%), 7 with subclinical hypothyroidism had positive anti-thyroid peroxidase, and 3 of them (42.8%) with positive anti-thyroid peroxidase had Down syndrome. Down syndrome and hypoxaemic CHD patients showed higher thyroid-stimulating hormone concentrations than the rest of the congenital patients (p<0.001). No significant differences were observed in serum thyroxine, creatinine, uric acid, lipids, C-reactive protein, or N-terminal pro b-type natriuretic peptide concentrations before and after the follow-up in those CHD patients with thyroid-stimulating hormone above 5.5 mIU/L whether or not they received levothyroxine therapy. Conclusions: CHD patients with subclinical hypothyroidism showed no significant changes in serum thyroxine, cholesterol, C-reactive protein, or N-terminal pro b-type natriuretic peptide concentrations whether or not they were treated with thyroid hormone replacement therapy. AD - E. Martínez-Quintana, Cardiology Service, Complejo Hospitalario, Universitario Insular-Materno Infantil, Avenida Martima del Sur s/n. 35016, Las Palmas de Gran Canaria, Spain AU - Martínez-Quintana, E. AU - Rodríguez-González, F. DB - Embase Medline DO - 10.1017/S1047951114001711 IS - 6 KW - amino terminal pro brain natriuretic peptide C reactive protein cholesterol creatinine glucose high density lipoprotein cholesterol levothyroxine low density lipoprotein cholesterol thyroid peroxidase antibody thyrotropin uric acid adolescent adult aged aortic coarctation aortic stenosis article atrioventricular septal defect bicuspid aortic valve cholesterol blood level congenital heart disease creatinine blood level Down syndrome Fallot tetralogy female follow up glucose blood level great vessels transposition heart atrium septum defect heart right ventricle double outlet heart single ventricle heart ventricle septum defect hormone substitution human major clinical study male protein blood level pulmonary valve atresia pulmonary valve stenosis sex difference subclinical hypothyroidism thyrotropin blood level uric acid blood level very elderly LA - English M3 - Article N1 - L600356521 2014-11-13 2015-11-06 PY - 2015 SN - 1467-1107 1047-9511 SP - 1111-1118 ST - Follow-up of congenital heart disease patients with subclinical hypothyroidism T2 - Cardiology in the Young TI - Follow-up of congenital heart disease patients with subclinical hypothyroidism UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L600356521 http://dx.doi.org/10.1017/S1047951114001711 VL - 25 ID - 913 ER - TY - JOUR AB - The primary extracardiac inferior cavopulmonary connection is an unusual novel palliation for single-ventricle physiology, which we first performed in the setting of unfavourable upper-body systemic venous anatomy for a standard bi-directional Glenn, and in lieu of leaving our patient with shunt-dependent physiology. After an initial 16-month satisfactory follow-up, increasing cyanosis led to the discovery of a veno-venous collateral that was coiled, but, more importantly, to impressive growth of a previously diminutive superior caval vein, which allowed us to perform completion Fontan with a good outcome. Performing the single-ventricle staging in a reverse manner, first from below with a primary inferior cavopulmonary connection, followed by Fontan completion from above with a standard superior caval vein bi-directional Glenn, is also possible when deemed necessary. AD - A. Dodge-Khatami, Pediatric and Congenital Heart Surgery, Children's Heart Center, University of Mississippi Medical Center, 2500 North State Street, Jackson, MS, United States AU - Dodge-Khatami, J. AU - Aggarwal, A. AU - Taylor, M. B. AU - Maposa, D. AU - Salazar, J. D. AU - Dodge-Khatami, A. DB - Embase Medline DO - 10.1017/S1047951116001839 IS - 5 KW - acetylsalicylic acid anastomosis article cardiac conduit case report cavopulmonary connection child cyanosis follow up Fontan procedure Glenn shunt heart catheterization hospitalization human intensive care unit lung artery pressure lung blood flow outcome assessment oxygen consumption oxygen saturation patient satisfaction postoperative care preschool child pulmonary valve stenosis pulse oximetry reoperation sternotomy superior cava vein weaning body weight gain LA - English M3 - Article N1 - L612988398 2016-11-07 2017-06-12 PY - 2017 SN - 1467-1107 1047-9511 SP - 925-928 ST - Fontan completion in reverse order out of necessity: Secondary Glenn after primary extracardiac inferior cavopulmonary artery connection T2 - Cardiology in the Young TI - Fontan completion in reverse order out of necessity: Secondary Glenn after primary extracardiac inferior cavopulmonary artery connection UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L612988398 http://dx.doi.org/10.1017/S1047951116001839 VL - 27 ID - 740 ER - TY - JOUR AB - BACKGROUND: In patients with a modified Fontan connection, particularly the right atrial to right ventricular connection, the pulmonary ventricle may enlarge with time. METHODS: Between January 1990 and December 2006, 10 patients (median age, 24 years) underwent Fontan conversion to a one and one half ventricle repair. Tricuspid atresia was the most common diagnosis (n=7). The right atrial to right ventricular connection was present in 8 patients; 3 patients had a prior bidirectional cavopulmonary anastomosis. Preoperative median right atrial pressure was 14 mm Hg (range, 12 to 20 mm Hg). Indications for surgery were exercise intolerance, arrhythmias, and conduit obstruction. Echocardiography showed moderate right ventricular hypoplasia, with right atrial to right ventricular regurgitation. The conversion included closure of septal defects, tricuspid valve replacement (n=8), bidirectional cavopulmonary anastomosis (n=7), valved right ventricular outflow tract reconstruction (n=2), and arrhythmia surgery (n=6). RESULTS: There was no early mortality. There was one reoperation for residual ventricular septal defect. Prolonged chest tube drainage occurred in 2 patients. Postoperative right atrial pressure ranged from 8 to 14 mm Hg (median, 10 mm Hg; p=0.02). Median follow-up was 8 years. There was no late mortality. Nine patients are in New York Heart Association class I or II, and 1 patient has biventricular failure and was awaiting transplantation. There were two late reoperations, 1 for thrombosis of the mechanical tricuspid valve prosthesis and 1 for obstruction of the valved right ventricular outflow tract conduit. Three patients had recurrent atrial arrhythmias requiring cardioversion. CONCLUSIONS: Fontan conversion to one and one half ventricle repair is feasible in selected patients with a failing Fontan circulation. Operation can be performed with low early mortality. Arrhythmia surgery should be performed routinely. Quality of life is excellent. AD - Division of Cardiovascular Surgery, Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA. AN - 22727323 AU - Sharma, V. AU - Burkhart, H. M. AU - Cetta, F. AU - Hagler, D. J. AU - Phillips, S. D. AU - Dearani, J. A. DA - Oct DB - PubMed DO - 10.1016/j.athoracsur.2012.04.060 DP - NLM ET - 2012/06/26 IS - 4 KW - Adolescent Adult Echocardiography Female Follow-Up Studies Fontan Procedure/*methods Heart Defects, Congenital/diagnostic imaging/*surgery Heart Ventricles/abnormalities/diagnostic imaging/*surgery Humans Male Retrospective Studies Time Factors Treatment Outcome Young Adult LA - eng N1 - 1552-6259 Sharma, Vikas Burkhart, Harold M Cetta, Frank Hagler, Donald J Phillips, Sabrina D Dearani, Joseph A Comparative Study Journal Article Netherlands Ann Thorac Surg. 2012 Oct;94(4):1269-74; discussion 1274. doi: 10.1016/j.athoracsur.2012.04.060. Epub 2012 Jun 21. PY - 2012 SN - 0003-4975 SP - 1269-74; discussion 1274 ST - Fontan conversion to one and one half ventricle repair T2 - Ann Thorac Surg TI - Fontan conversion to one and one half ventricle repair VL - 94 ID - 219 ER - TY - JOUR AB - OBJECTIVES: The aim of this study was to prospectively examine the effects of elective Fontan fenestration closure upon exercise capacity and ventilatory abnormalities. BACKGROUND: For patients undergoing Fontan procedures as palliation of single ventricle physiology, the addition of a fenestration to the procedure mitigates perioperative morbidity. Although some fenestrations may close spontaneously, many remain patent and subject patients to arterial hypoxemia and risk for paradoxical emboli. For these reasons fenestration closure is routinely performed post-operatively in the cardiac catheterization laboratory. Although closure of Fontan fenestrations typically results in an immediate improvement in systemic arterial oxygen saturation, it is also associated with an acute decrease in cardiac index and systemic O(2) delivery. The sum result of these physiologic changes upon exercise capacity has not been examined. METHODS: Twenty consecutive patients, age 5 to 46 (median 10) years, underwent pre- and post-fenestration closure exercise testing with expiratory gas analysis. RESULTS: Before fenestration closure, peak oxygen consumption (VO(2)) was depressed and there was systemic desaturation at rest that worsened with exercise. The ventilatory response to exercise was also abnormal, characterized by elevation of the minute ventilation (VE)/CO(2) elimination slope (VE/VCO(2)), a low end-tidal CO(2), and high end-tidal O(2) at the ventilatory anaerobic threshold. Although arterial saturation improved significantly after fenestration closure, there was no change in peak VO(2) (70.9 +/- 18.6% to 74.0 +/- 18.6%, p = NS), heart rate, or O(2) pulse at peak exercise. In contrast, ventilatory abnormalities (VE/VCO(2)) improved considerably (44.4 +/- 10.9 to 33.3 +/- 5.5, p 2 SD below mean. The χ(2) test compared groups. Predictors of GTF were analyzed using multiple logistic regression analysis, results expressed as OR with 95% CI. RESULTS: Of the survivors, 67/334 (20%) had GTF any time before the 21-month assessment. Developmental delays in children with GTF were cognitive in 16 (24%), motor in 18 (27%), language in 24 (36%) vs without GTF in 7 (3%), 8 (3%), and 32 (12%), respectively (P < .001). Gastrostomy group had almost 8 times the number of children delayed on the general adaptive composite score. Independent OR for GTF are presence of a chromosomal abnormality, OR 4.6 (95% CI 1.8, 12.0) (P = .002), single ventricle anatomy, OR 3.4 (95% CI 1.7, 6.8) (P < .001), total postoperative days of open sternum, OR 1.15 (95% CI 1.1, 1.3) (P = .031), and total number of hospital days at CCS, OR 1.03 (95% CI 1.1, 1.04) (P = .002). CONCLUSIONS: GTF identifies CCS survivors at risk for delay, who would benefit from early developmental intervention. The described mostly nonmodifiable predictors may guide counseling of these children's families. AD - Division of Developmental Pediatrics, Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada. Pediatric Rehabilitation, Glenrose Rehabilitation Hospital, Edmonton, Alberta, Canada. Division of Cardiac Surgery, Department of Surgery, University of Alberta, Edmonton, Alberta, Canada. Division of General Surgery, University of Alberta, Edmonton, Alberta, Canada. Department of Pediatrics and Child Health, University of Manitoba, Winnipeg, Canada. Division of Developmental Pediatrics, Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada; Pediatric Rehabilitation, Glenrose Rehabilitation Hospital, Edmonton, Alberta, Canada. Electronic address: Charlene.Robertson@albertahealthservices.ca. AN - 26651431 AU - Ricci, M. F. AU - Alton, G. Y. AU - Ross, D. B. AU - Dicken, B. J. AU - Moddemann, D. M. AU - Robertson, C. M. DA - Feb DB - PubMed DO - 10.1016/j.jpeds.2015.10.087 DP - NLM ET - 2015/12/15 KW - *Cardiac Surgical Procedures Developmental Disabilities/*prevention & control *Early Medical Intervention *Enteral Nutrition Female *Gastrostomy Humans Infant, Newborn Male *Postoperative Care Prospective Studies LA - eng N1 - 1097-6833 Ricci, M Florencia Alton, Gwen Y Ross, David B Dicken, Bryan J Moddemann, Diane M Robertson, Charlene M T Western Canadian Complex Pediatric Therapies Follow-up Group Comparative Study Journal Article Research Support, Non-U.S. Gov't United States J Pediatr. 2016 Feb;169:160-5.e1. doi: 10.1016/j.jpeds.2015.10.087. Epub 2015 Dec 2. PY - 2016 SN - 0022-3476 SP - 160-5.e1 ST - Gastrostomy Tube Feeding after Neonatal Complex Cardiac Surgery Identifies the Need for Early Developmental Intervention T2 - J Pediatr TI - Gastrostomy Tube Feeding after Neonatal Complex Cardiac Surgery Identifies the Need for Early Developmental Intervention VL - 169 ID - 518 ER - TY - JOUR AB - Objective To compare the proportion of developmental delay in early complex cardiac surgery (CCS) survivors with and without gastrostomy tube feeding (GTF). To explore acute care predictors of GTF that might help improve care in CCS survivors. Study group This comparison study of 2 groups within an inception cohort included 334 CCS survivors after cardiopulmonary bypass at ≤6 weeks of age (2005-2012) who did not require extracorporeal membrane oxygenation or heart transplantation. Children were assessed at 21 ± 3 months with the Bayley Scales of Infant and Toddler Development-Third Edition and the Adaptive Behavior Assessment System-Second Edition: general adaptive composite score. Delay was determined by scores >2 SD below mean. The χ2 test compared groups. Predictors of GTF were analyzed using multiple logistic regression analysis, results expressed as OR with 95% CI. Results Of the survivors, 67/334 (20%) had GTF any time before the 21-month assessment. Developmental delays in children with GTF were cognitive in 16 (24%), motor in 18 (27%), language in 24 (36%) vs without GTF in 7 (3%), 8 (3%), and 32 (12%), respectively (P <.001). Gastrostomy group had almost 8 times the number of children delayed on the general adaptive composite score. Independent OR for GTF are presence of a chromosomal abnormality, OR 4.6 (95% CI 1.8, 12.0) (P =.002), single ventricle anatomy, OR 3.4 (95% CI 1.7, 6.8) (P <.001), total postoperative days of open sternum, OR 1.15 (95% CI 1.1, 1.3) (P =.031), and total number of hospital days at CCS, OR 1.03 (95% CI 1.1, 1.04) (P =.002). Conclusions GTF identifies CCS survivors at risk for delay, who would benefit from early developmental intervention. The described mostly nonmodifiable predictors may guide counseling of these children's families. AD - C.M.T. Robertson, Pediatric Rehabilitation, Glenrose Rehabilitation Hospital, 10230 111 Ave, Edmonton, AB, Canada AU - Ricci, M. F. AU - Alton, G. Y. AU - Ross, D. B. AU - Dicken, B. J. AU - Moddemann, D. M. AU - Robertson, C. M. T. AU - Rebeyka, I. M. AU - Garcia Guerra, G. AU - Atallah, J. AU - Dinu, I. A. AU - Blakley, P. AU - Bodani, J. AU - Synnes, A. DB - Embase Medline DO - 10.1016/j.jpeds.2015.10.087 KW - anatomy article Bayley Scales of Infant Development cardiopulmonary bypass child chromosome aberration cognitive defect developmental disorder early intervention enteric feeding extracorporeal oxygenation female heart single ventricle heart surgery heart transplantation human intestine malrotation language delay length of stay lung vein drainage anomaly major clinical study male motor dysfunction postoperative period prenatal diagnosis priority journal stomach tube survivor LA - English M3 - Article N1 - L607199625 2015-12-15 2016-03-18 PY - 2016 SN - 1097-6833 0022-3476 SP - 160-165e1 ST - Gastrostomy Tube Feeding after Neonatal Complex Cardiac Surgery Identifies the Need for Early Developmental Intervention T2 - Journal of Pediatrics TI - Gastrostomy Tube Feeding after Neonatal Complex Cardiac Surgery Identifies the Need for Early Developmental Intervention UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L607199625 http://dx.doi.org/10.1016/j.jpeds.2015.10.087 VL - 169 ID - 825 ER - TY - JOUR AB - Among the writing craft there exists a motto: "If you steal from one author, it is plagiarism; if you steal from many, it is research.". In the realm of psychology probably lies the explanation of a type of conduct one finds so consistently present in the evolution of some particular surgical technic. So consistently and even persistently does one find it that it cannot by any chance be attributed to mere coincidence. To the non-psychologist, but one having some experience in the detached scrutiny of the ways of human conduct there is only one explanation. A terrifically frustrated ego exists which drives eternally for expression, for a labeling or attachment of self by way of name to something-in medical and surgical parlance-some step in treatment or procedure in surgical technic, that the individual hopes may go down into posterity, thereby perhaps justifying one's having passed through this cosmos called world. This seems to be a part of the makeup of so many men in the field of medicine. How else can one explain the making of an incision a half inch or so to this side or that side and calling it a "new method"; or reversing a suture line, starting at the top or vice versa at the bottom, or the accidental happening upon some procedure, which some less fortunate colleague used years before, and then rushing into print with a grand flourish, "a new method.". One sees this type of thing so much in a search or study of the medical literature of the past that one becomes stonily cynical and rather hard boiled in approach to anything labeled "a new." However, as a matter of historical accuracy it behooves some of us to scan with a degree of honesty, yet critically current contemporary claims to priority in these so-called "new methods" of doing this or that. Medical achievement has been gradual and step by step. This is as it should be. One person here makes an observation and records it. Fortunate indeed that it should be this way. In another place some one tries it, and possibly finds it wanting and strives to improve upon it, adding a little something. Somebody else points out some deficiencies and tries to solve them. Thus by an evolution of ideas final perfection may be reached or approached. Medical historians in later years scan these contributions and give priority where it is due because there exists material evidence and corroboration in the printed word-in the original publication-in which the ideas were set forth for the use of the medical world at large. This is particularly well illustrated in the historical evolution of the procedure of gastrostomy. We know and have proof, documented proof, that a military surgeon named Egeberg, in statements before a medical society in Christiania made the following remarks: "I cannot see why the indications should not be just as great to open the intestinal canal to put nourishment in as to open it to take a foreign body out.". We surmise that Egeberg may have been influenced by reading of the experiences of another military surgeon, Wm. Beaumont with "old fistulous Alexis," illustrating to him that a man could live in health with a permanent opening in his stomach. We surmise this because we are aware that Dr. Beaumont's experience was much publicized in Europe and in addition because there exists a certain co-incidence as to dates and time. From here on we find that process of evolutionary step by step improvement upon the original. A French surgeon, Sédillot in 1846, lifts a cone-shaped segment of the anterior wall of the stomach through an abdominal incision and behold you have the first completed operation of gastrostomy. But for reasons mentioned in the body of this article the patients died. Therefore, although the idea is a good one it must be made to work better. Along in 1888, in 1890 and in 1893, three men, Girard, Von Hacker and Jaboulay proposed that instead of bringing the cone of stomach wall directly to the skin through the abdominal wall, it should be brought through the subcutaneous tissues in such manner that the rectus abdominis muscle shall act n a sphincteric manner as to stop leakage around the attachment. Although this was a minor improvement it did not solve the problem of spillage, skin excoriation, etcetra. So we find more names attached to contributions, one Sabanieff, one Hartmann, and a Frank, in the years from 1890 to 1893. These suggested various and sundry complicated passages of a cone or anterior gastric wall through and between the subcutaneous tissues and muscles. One suggested bringing the cone through the skin between the costal cartilages hoping to utilize the latter for sphincteric action. It is important to realize and emphasize that these men all were using a cone of tissue in view of what is coming later. In 1894 and 1902, two men suggested twisting this cone of tissue so that when fastened to the abdominal wall, the torsion of the tissues would obstruct leakage and spillage. These men were Ullmann and Souligoux.28. We return to France and the French school of surgery for the first milestone in evolution oj the operation, and give credit where credit is due. In 1893, Pénières proposed constructing a valve from gastric mucosa to combat leakage, and proceeded to perform the operation on dogs in his laboratory in Toulouse; whereupon another Frenchman, Fontan, not, however, without giving due credit to his countryman, proceeds to perform the operation on humans, differing in technic from his confrere in that he made his valve using all the layers of stomach wall instead of mucosa alone as did Pénières. The operation now consisted of a canal instead of a cone with a valve arrangement to prevent leakage. That was the operation as proposed by Fontan and Pénières, a completed operation, the principles originated and laid down by them and the basis upon which any subsequent minor changes were laid. To these men should go the credit for the development of the procedure. The operation to many was still one which could be improved upon. Here again crops up that curious conduct on the part of the surgical mind. Where one man would propose placing a catheter through a stab wound into the stomach, then plicating the serosa over it by means of one or two purse-string sutures, one would rush into print with "a new method." The "new" consisted of placing first the stab wound a half inch or so higher or lower than the first proposer or instead of one or two layers of purse-string sutures advocating three or four, as providing greater strength to the finished canal, therefore, greater safety. Then along came the men who courageously departed a bit farther and who may have been honest and upright in their thinking and advocacy of their ideas. These were the men who advocated the substitution of other hollow viscera as tubes between the stomach and skin. Such was the isolated pedicled loop of jejunum as advocated by Roux and Lexer and the isolated segment or pedicled loop of transverse colon advocated by Vuillet in 1911, and Kelling in the same year. Somehow one gets an impression that Roux must have been in his seventh heaven playing with loops of intestines, attaching here and there, the more complicated, the more enthusiastic and delighted he was, much as a child with an erector set, but he used gut, instead of blocks or metal. It is a laborious piece of work to read of the different anastamoses he made and proposed. Then along came the men who proposed the construction of tubed pedicled flaps from the stomach wall. Most prominent of these were Dépage and Janeway and both names were used connectedly to describe a technic and procedure originated by Dépage in 1901; but the story goes that Janeway was using and had originated a similar operation, entirely unaware of Dépage's work, so somewhere the idea got abroad that he should be given credit with Dépage and the operation has since been spoken of as the Dépage-Janeway technic. It is curious that Dépage's article describing his procedure appeared in 1901, and Janeway's in 1913, a considerable discrepancy in time. Here again that curious pattern of conduct: Dépage made his tubed flap with the base at the lesser curvature. Janeway made his with the base at the greater curvature, and insisted that his was the better method because it had a better blood supply, at any rate it was a "new method." Watsudjii combined Von Hacker's and Fontan's operations giving them credit while Spivack combined Fontan's and Dépage's operations and described it an "original"-"new method" without allocating proper credits. Then came the tubed flap made by partial severance of the greater curvature of the stomach with the base at cardia and another with base at the fundus. These were: (i) the operation worked out in the laboratory by Alexis Carrell with Beck and called the Carrell-Beck procedure, and (2) the method of Jianu used successfully by Dr. George Pack. It is quite possible that a more detailed and thorough perusal of available literature on the subject of gastrostomy might have brought to light even more material than is quoted here. 29,30 However, over fifty different technics or plans of operation have been published by various authors and the prédominent part of these had "A New Method" as part of their title. However, in the past thirty-five years nothing new has been added to our technical knowledge concerning gastrostomy and no surgeon of recent times, or contemporary to the day is warranted lifting the work of the men who actually made the contribution and applying bis own name to it. Sufficient documentary proof exists in published articles with names and dates to make any such conduct too easy to refute and make a lie. © 1946. AD - sSan Francisco, CA, United States AU - Cunha, F. DB - Scopus DO - 10.1016/0002-9610(46)90402-3 IS - 4 M3 - Article N1 - Cited By :51 Export Date: 15 June 2020 PY - 1946 SP - 610-634 ST - Gastrostomy. Its inception and evolution T2 - The American Journal of Surgery TI - Gastrostomy. Its inception and evolution UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0000315079&doi=10.1016%2f0002-9610%2846%2990402-3&partnerID=40&md5=5988620669a03464ca2b0c4f20d0037d VL - 72 ID - 2339 ER - TY - JOUR AB - Background: We examined the effect of genetic syndromes and extracardiac (GS/EC) anomalies on single-ventricle (SV) palliation with focus on hospital and interstage death and progression toward subsequent palliation stages. Methods: First-stage palliation was performed in 530 neonates with SV: Norwood in 284 (53%), shunt in 173 (33%), and band in 73 (14%). Outcomes were compared between those with GS/EC anomalies (121 [23%]) and without GS/EC anomalies (409 [77%]). Regression analyses were adjusted for other risk factors (age, sex, prematurity, weight, SV anomaly, and first-stage palliation operation). Results: GS/EC anomalies varied among SV defects (range, 3% for double-inlet left ventricle to 100% for atrial isomerism). Patients with GS/EC anomalies required significantly longer durations of mechanical ventilation and intensive care unit and hospital stay. Although patients had comparable rates of extracorporeal membrane oxygenation (13% vs 11%, p = 0.552) and unplanned reoperation (16% vs 11%, p = 0.189), hospital mortality was higher in patients with GS/EC anomalies (24% vs 12%, p = 0.0008). After discharge, patients with GS/EC anomalies had higher interstage death, with lower progression to Glenn (60% vs 77%, p = 0.002) and lower 10-year survival (56% vs 76%, p < 0.001). After adjustment for other risk factors, GS/EC anomalies significantly affected survival in almost all subgroups of patients. Conclusions: The presence of GS/EC anomalies varies among SV anomalies and is associated with additional risk factors such as prematurity and low weight. After adjusting for other risk factors, GS/EC anomalies are associated with prolonged recovery after first-stage palliation and increased hospital and interstage death, with subsequently fewer patients progressing toward Glenn shunt. The increased death risk in those patients is highest in the first 6 months and persists for 2 to 3 years after first-stage palliation, suggesting the need for more vigilant monitoring and outpatient care in these high-risk patients. AD - B. Alsoufi, University of Louisville, 201 Abraham Flexner Way, Louisville, KY, United States AU - Alsoufi, B. AU - McCracken, C. AU - Oster, M. AU - Shashidharan, S. AU - Kanter, K. DB - Embase Medline DO - 10.1016/j.athoracsur.2018.04.043 IS - 4 KW - anus atresia article artificial ventilation Blalock Taussig shunt child chromosome aberration chromosome analysis chromosome deletion 22q11 comparative study congenital heart disease congenital ichthyosiform erythroderma coronary care unit Dandy Walker syndrome disease association disease course Down syndrome extracardiac anomaly extracorporeal oxygenation female follow up genetic anomaly genetic disorder heart single ventricle heart transplantation heterotaxy syndrome high risk patient Hirschsprung disease hospital mortality hospitalization human hypoplastic left heart syndrome in situ hybridization intensive care unit intestine atresia karyotype Klinefelter syndrome limb defect major clinical study male medical record monosomy newborn newborn screening Norwood procedure omphalocele outcome assessment outpatient care palliative therapy patient monitoring priority journal reoperation risk assessment risk factor survival syndrome CHARGE tracheoesophageal fistula Turner syndrome LA - English M3 - Article N1 - L2001056799 2018-09-03 2019-09-20 PY - 2018 SN - 1552-6259 0003-4975 SP - 1204-1212 ST - Genetic and Extracardiac Anomalies Are Associated With Inferior Single Ventricle Palliation Outcomes T2 - Annals of Thoracic Surgery TI - Genetic and Extracardiac Anomalies Are Associated With Inferior Single Ventricle Palliation Outcomes UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2001056799 http://dx.doi.org/10.1016/j.athoracsur.2018.04.043 VL - 106 ID - 660 ER - TY - JOUR AB - Clinical genetic testing for specific isolated congenital heart defects (CHD) is becoming standard of care in pediatric cardiology practice. Both genetic knowledge and attitudes toward genetic testing are associated with an increased utilization of genetic testing, but these factors have not been evaluated in parents of children with CHD. We mailed a survey to measure the demographics, genetic knowledge, and attitudes towards genetic testing of parents of children with CHD who previously consented to participate in a separate research study of the genetic etiology of left ventricular outflow tract malformations (LVOT). Of the 378 eligible families, 190 (50%) returned surveys with both parents completing surveys in 97 (51%) families, resulting in 287 participants. Genetic knowledge was assessed on an adapted measure on which the mean percent correct was 73.8%. Educational attainment and household income were directly and significantly associated with genetic knowledge (P<0.001). Attitudes about the health effects of genetic testing were favorable with at least 57% agreeing that genetic testing would be used for managing health care and finding cures for disease. Conversely, a minority of participants found it likely that genetic testing would be used for insurance (up to 39.9%), employment (15.8%), or racial/social discrimination (up to 11.2%). Parents of younger children were less likely to endorse employment or racial/social discrimination. Genetic knowledge was not correlated with specific attitudes. Among parents of children with CHD, genetic knowledge was directly associated with household income and education, but additional research is necessary to determine what factors influence attitudes towards genetic testing. AD - S.M. Fitzgerald-Butt, The Research Institute, WB4157, Nationwide Children's Hospital, 700 Children's Dr., Columbus, OH, United States AU - Fitzgerald-Butt, S. M. AU - Klima, J. AU - Kelleher, K. AU - Chisolm, D. AU - McBride, K. L. DB - Embase Medline DO - 10.1002/ajmg.a.36763 IS - 12 KW - adolescent adult aortic coarctation article attitude to health bicuspid aortic valve child child health care congenital heart malformation educational status employment female genetic knowledge genetic screening genetics health care survey health insurance heart left ventricle outflow tract obstruction human hypoplastic left heart syndrome income infant knowledge major clinical study male parental attitude preschool child priority journal racism school child social discrimination social status young adult LA - English M3 - Article N1 - L600504833 2015-01-27 PY - 2014 SN - 1552-4833 1552-4825 SP - 3069-3075 ST - Genetic knowledge and attitudes of parents of children with congenital heart defects T2 - American Journal of Medical Genetics, Part A TI - Genetic knowledge and attitudes of parents of children with congenital heart defects UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L600504833 http://dx.doi.org/10.1002/ajmg.a.36763 VL - 164 ID - 903 ER - TY - JOUR AB - On March 16, 2012, the Ethics of the Heart 2012: Ethical and Policy Challenges in Pediatric and Adult Congenital Heart Disease Conference took place in Philadelphia, Pennsylvania. The first session focused on the ethics surrounding genetic testing in patients with congenital heart disease. Summarized here is the introductory presentation given by Dr Elizabeth Goldmuntz entitled “The Role of Genetic Testing in Congenital Heart Disease,” followed by a case presentation given by Dr Lisa D’Alessandro. The case and the panel discussion that ensued highlight several ethical principles and challenges in this unique patient population. AD - K.Y. Lin, 34th St and Civic Center Blvd, Philadelphia, PA, United States AU - Lin, K. Y. AU - D’Alessandro, L. C. A. AU - Goldmuntz, E. DB - Embase Medline DO - 10.1177/2150135112459523 IS - 1 KW - act article chromosome deletion 22q11 congenital heart disease ethics fluorescence in situ hybridization gastroesophageal reflux gene mutation genetic screening genetic variability genotype health insurance hospitalization human hypoplastic left heart syndrome intensive care unit multiplex ligation dependent probe amplification nuclear magnetic resonance imaging priority journal recurrent disease survival velocardiofacial syndrome LA - English M3 - Article N1 - L604281697 2015-05-13 2020-03-03 PY - 2013 SN - 2150-136X 2150-1351 SP - 53-57 ST - Genetic Testing in Congenital Heart Disease: Ethical Considerations T2 - World Journal for Pediatric and Congenital Heart Surgery TI - Genetic Testing in Congenital Heart Disease: Ethical Considerations UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L604281697 http://dx.doi.org/10.1177/2150135112459523 VL - 4 ID - 1030 ER - TY - JOUR AB - BACKGROUND: Preterm delivery and low birth weight (LBW) are generally associated with worse outcomes in hypoplastic left heart syndrome (HLHS), but an individual preterm or small neonate may do well. We sought to explore the interactions between gestational age, birth weight, and birth weight for gestational age with intermediate outcomes in HLHS. METHODS: We analyzed survival, growth, neurodevelopment, length of stay, and complications to age 6 years in subjects with HLHS from the Single Ventricle Reconstruction trial. Univariate and multivariable survival and regression analyses examined the effects and interactions of LBW (,2500 g), weight for gestational age, and gestational age category. RESULTS: Early-term delivery (n = 234) was more common than term (n = 219) delivery. Small for gestational age (SGA) was present in 41% of subjects, but only 14% had LBW. Preterm, compared with term, delivery was associated with an increased risk of death or transplant at age 6 years (all: hazard ratio = 2.58, confidence interval = 1.43–4.67; Norwood survivors: hazard ratio = 1.96, confidence interval = 1.10–3.49) independent of LBW and weight for gestational age. Preterm delivery, early-term delivery, LBW, and SGA were each associated with lower weight at 6 years. Neurodevelopmental outcomes were worst in the LBW cohort. CONCLUSIONS: Preterm delivery in HLHS was associated with worse survival, even beyond Norwood hospitalization. LBW, SGA, and early-term delivery were associated with worse growth but not survival. LBW was associated with worse neurodevelopment, despite similar length of stay and complications. These data suggest that preterm birth and LBW (although often concomitant) are not equivalent, impacting clinical outcomes through mechanisms independent of perioperative course complexity. AD - T.A. Miller, Department of Pediatrics, University of Utah, 81 N Mario Capecchi Dr, Salt Lake City, UT, United States AU - Miller, T. A. AU - Ghanayem, N. S. AU - Newburger, J. W. AU - McCrindle, B. W. AU - Hu, C. AU - DeWitt, A. G. AU - Cnota, J. F. AU - Tractenberg, F. L. AU - Pemberton, V. L. AU - Wolf, M. J. AU - Votava-Smith, J. K. AU - Fifer, C. G. AU - Lambert, L. M. AU - Shah, A. AU - Graham, E. M. AU - Pizarro, C. AU - Jacobs, J. P. AU - Miller, S. G. AU - LuAnn Minich, L. DB - Embase Medline DO - 10.1542/peds.2018-2577 IS - 5 KW - article birth weight Blalock Taussig shunt child child growth child hospitalization cohort analysis controlled study death descriptive research female gestational age human hypoplastic left heart syndrome infant length of stay low birth weight major clinical study male nerve cell differentiation Norwood procedure obstetric delivery postoperative period premature labor priority journal quality of life right ventricle to pulmonary artery conduit small for date infant survival treatment outcome LA - English M3 - Article N1 - L2002035064 2019-06-05 2019-11-21 PY - 2019 SN - 1098-4275 0031-4005 ST - Gestational age, birth weight, and outcomes six years after the norwood procedure T2 - Pediatrics TI - Gestational age, birth weight, and outcomes six years after the norwood procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2002035064 http://dx.doi.org/10.1542/peds.2018-2577 VL - 143 ID - 616 ER - TY - JOUR AB - BACKGROUND: Atrial tachyarrhythmia is a common cause of morbidity and mortality in patients with univentricular physiology undergoing the Fontan operation. We examined cardiac autonomic nervous activity, a predictor of arrhythmia and sudden death in other cardiovascular disease, in patients late after the Fontan operation, employing heart rate variability (HRV) and baroreflex sensitivity. METHODS AND RESULTS: We measured HRV and baroreflex sensitivity in 22 consecutive patients (8 male, age 26+/-9 years) who had undergone the Fontan operation 13+/-6 years previously, and 22 age- and sex-matched healthy controls. Fontan patients had significantly lower HRV (P<0.0001). Baroreflex sensitivity was measured by the alpha-index method (square root of ratio of RR interval spectral power to systolic blood pressure (SBP) spectral power, in the LF and the HF band) and was also significantly depressed in the Fontan group (P<0.0001 for both). Both low frequency (LF) and high frequency (HF) components of HRV were reduced in the Fontan patients (P<0.0001), but there was interindividual variation so that the LF/(LF+HF) ratio may be high, normal, or low, and decreased with increasing right atrial dimensions (r=-0.62, P=0.006). Patients with a history of sustained atrial arrhythmia had a stronger baroreflex than those without (P=0.005). CONCLUSIONS: Autonomic nervous control of the heart is markedly deranged in patients late after the Fontan operation, with reduced HRV and baroreflex sensitivity. A relative suppression of the sympathetic-compared with the parasympathetic-system was observed in patients with marked right atrial dilation within the Fontan group. Furthermore, stronger baroreflexes were seen in Fontan patients in association with a higher incidence of sustained atrial tachyarrhythmia, implying that sinus node dysfunction is unlikely to be the dominant mechanism. Additional studies are clearly required to examine the prognostic importance of impaired BRS and HRV in these patients. AD - Royal Brompton Adult Congenital Heart Programme, and Department of Clinical Cardiology, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College School of Medicine, London, UK. AN - 12970229 AU - Davos, C. H. AU - Francis, D. P. AU - Leenarts, M. F. AU - Yap, S. C. AU - Li, W. AU - Davlouros, P. A. AU - Wensel, R. AU - Coats, A. J. AU - Piepoli, M. AU - Sreeram, N. AU - Gatzoulis, M. A. DA - Sep 9 DB - PubMed DO - 10.1161/01.cir.0000087946.47069.cb DP - NLM ET - 2003/09/13 KW - Adolescent Adult Autonomic Nervous System/*physiopathology Baroreflex Child Female *Fontan Procedure Heart/*innervation Heart Defects, Congenital/complications/diagnosis/*physiopathology/*surgery Heart Rate Humans Male Prospective Studies Sympathetic Nervous System/physiopathology Tachycardia/etiology Vagus Nerve/physiopathology LA - eng N1 - 1524-4539 Davos, Constantinos H Francis, Darrel P Leenarts, Marjolein F E Yap, Sing-Chien Li, Wei Davlouros, Periklis A Wensel, Roland Coats, Andrew J S Piepoli, Massimo Sreeram, Narayanswami Gatzoulis, Michael A Journal Article Research Support, Non-U.S. Gov't United States Circulation. 2003 Sep 9;108 Suppl 1:II180-5. doi: 10.1161/01.cir.0000087946.47069.cb. PY - 2003 SN - 0009-7322 SP - Ii180-5 ST - Global impairment of cardiac autonomic nervous activity late after the Fontan operation T2 - Circulation TI - Global impairment of cardiac autonomic nervous activity late after the Fontan operation VL - 108 Suppl 1 ID - 399 ER - TY - JOUR AB - BACKGROUND: Previous congenital heart disease estimates came from few data sources, were geographically narrow, and did not evaluate congenital heart disease throughout the life course. Completed as part of the Global Burden of Diseases, Injuries, and Risk Factors Study 2017, this study aimed to provide comprehensive estimates of congenital heart disease mortality, prevalence, and disability by age for 195 countries and territories from 1990 to 2017. METHODS: Mortality estimates were generated for aggregate congenital heart disease and non-fatal estimates for five subcategories (single ventricle and single ventricle pathway congenital heart anomalies; severe congenital heart anomalies excluding single ventricle heart defects; critical malformations of great vessels, congenital valvular heart disease, and patent ductus arteriosus; ventricular septal defect and atrial septal defect; and other congenital heart anomalies), for 1990 through to 2017. All available global data were systematically analysed to generate congenital heart disease mortality estimates (using Cause of Death Ensemble modelling) and prevalence estimates (DisMod-MR 2·1). Systematic literature reviews of all types of congenital anomalies to capture information on prevalence, associated mortality, and long-term health outcomes on congenital heart disease informed subsequent disability estimates. FINDINGS: Congenital heart disease caused 261 247 deaths (95% uncertainty interval 216 567-308 159) globally in 2017, a 34·5% decline from 1990, with 180 624 deaths (146 825-214 178) being among infants (aged <1 years). Congenital heart disease mortality rates declined with increasing Socio-demographic Index (SDI); most deaths occurred in countries in the low and low-middle SDI quintiles. The prevalence rates of congenital heart disease at birth changed little temporally or by SDI, resulting in 11 998 283 (10 958 658-13 123 888) people living with congenital heart disease globally, an 18·7% increase from 1990 to 2017, and causing a total of 589 479 (287 200-973 359) years lived with disability. INTERPRETATION: Congenital heart disease is a large, rapidly emerging global problem in child health. Without the ability to substantially alter the prevalence of congenital heart disease, interventions and resources must be used to improve survival and quality of life. Our findings highlight the large global inequities in congenital heart disease and can serve as a starting point for policy changes to improve screening, treatment, and data collection. FUNDING: Bill & Melinda Gates Foundation. AN - 31978374 DA - Mar DB - PubMed DO - 10.1016/s2352-4642(19)30402-x DP - NLM ET - 2020/01/25 IS - 3 LA - eng N1 - 2352-4650 GBD 2017 Congenital Heart Disease Collaborators K23 HL140133/HL/NHLBI NIH HHS/United States Journal Article England Lancet Child Adolesc Health. 2020 Mar;4(3):185-200. doi: 10.1016/S2352-4642(19)30402-X. Epub 2020 Jan 21. PY - 2020 SN - 2352-4642 SP - 185-200 ST - Global, regional, and national burden of congenital heart disease, 1990-2017: a systematic analysis for the Global Burden of Disease Study 2017 T2 - Lancet Child Adolesc Health TI - Global, regional, and national burden of congenital heart disease, 1990-2017: a systematic analysis for the Global Burden of Disease Study 2017 VL - 4 ID - 543 ER - TY - JOUR AB - Background: Previous congenital heart disease estimates came from few data sources, were geographically narrow, and did not evaluate congenital heart disease throughout the life course. Completed as part of the Global Burden of Diseases, Injuries, and Risk Factors Study 2017, this study aimed to provide comprehensive estimates of congenital heart disease mortality, prevalence, and disability by age for 195 countries and territories from 1990 to 2017. Methods: Mortality estimates were generated for aggregate congenital heart disease and non-fatal estimates for five subcategories (single ventricle and single ventricle pathway congenital heart anomalies; severe congenital heart anomalies excluding single ventricle heart defects; critical malformations of great vessels, congenital valvular heart disease, and patent ductus arteriosus; ventricular septal defect and atrial septal defect; and other congenital heart anomalies), for 1990 through to 2017. All available global data were systematically analysed to generate congenital heart disease mortality estimates (using Cause of Death Ensemble modelling) and prevalence estimates (DisMod-MR 2·1). Systematic literature reviews of all types of congenital anomalies to capture information on prevalence, associated mortality, and long-term health outcomes on congenital heart disease informed subsequent disability estimates. Findings: Congenital heart disease caused 261 247 deaths (95% uncertainty interval 216 567–308 159) globally in 2017, a 34·5% decline from 1990, with 180 624 deaths (146 825–214 178) being among infants (aged <1 years). Congenital heart disease mortality rates declined with increasing Socio-demographic Index (SDI); most deaths occurred in countries in the low and low-middle SDI quintiles. The prevalence rates of congenital heart disease at birth changed little temporally or by SDI, resulting in 11 998 283 (10 958 658–13 123 888) people living with congenital heart disease globally, an 18·7% increase from 1990 to 2017, and causing a total of 589 479 (287 200–973 359) years lived with disability. Interpretation: Congenital heart disease is a large, rapidly emerging global problem in child health. Without the ability to substantially alter the prevalence of congenital heart disease, interventions and resources must be used to improve survival and quality of life. Our findings highlight the large global inequities in congenital heart disease and can serve as a starting point for policy changes to improve screening, treatment, and data collection. Funding: Bill & Melinda Gates Foundation. AD - C.A. Sable, Department of Cardiology, Children's National Health System, Washington, DC, United States AU - Zimmerman, M. S. AU - Smith, A. G. C. AU - Sable, C. A. AU - Echko, M. M. AU - Wilner, L. B. AU - Olsen, H. E. AU - Atalay, H. T. AU - Awasthi, A. AU - Bhutta, Z. A. AU - Boucher, J. L. AU - Castro, F. AU - Cortesi, P. A. AU - Dubey, M. AU - Fischer, F. AU - Hamidi, S. AU - Hay, S. I. AU - Hoang, C. L. AU - Hugo-Hamman, C. AU - Jenkins, K. J. AU - Kar, A. AU - Khalil, I. A. AU - Kumar, R. K. AU - Kwan, G. F. AU - Mengistu, D. T. AU - Mokdad, A. H. AU - Naghavi, M. AU - Negesa, L. AU - Negoi, I. AU - Negoi, R. I. AU - Nguyen, C. T. AU - Nguyen, H. L. T. AU - Nguyen, L. H. AU - Nguyen, S. H. AU - Nguyen, T. H. AU - Nixon, M. R. AU - Noubiap, J. J. AU - Patel, S. AU - Peprah, E. K. AU - Reiner, R. C. AU - Roth, G. A. AU - Temsah, M. H. AU - Tovani-Palone, M. R. AU - Towbin, J. A. AU - Tran, B. X. AU - Tran, T. T. AU - Truong, N. T. AU - Vos, T. AU - Vosoughi, K. AU - Weintraub, R. G. AU - Weldegwergs, K. G. AU - Zaidi, Z. AU - Zheleva, B. AU - Zuhlke, L. AU - Murray, C. J. L. AU - Martin, G. R. AU - Kassebaum, N. J. DB - Embase Medline DO - 10.1016/S2352-4642(19)30402-X IS - 3 KW - article brain disease cardiovascular mortality congenital heart disease disability geography global disease burden great vessels transposition heart atrium septum defect heart single ventricle heart ventricle septum defect human infant mortality lower respiratory tract infection mortality rate patent ductus arteriosus premature labor prevalence rheumatic heart disease socio demographic index sudden infant death syndrome systematic review LA - English M3 - Article N1 - L2004979667 2020-02-26 2020-03-03 PY - 2020 SN - 2352-4642 SP - 185-200 ST - Global, regional, and national burden of congenital heart disease, 1990–2017: a systematic analysis for the Global Burden of Disease Study 2017 T2 - The Lancet Child and Adolescent Health TI - Global, regional, and national burden of congenital heart disease, 1990–2017: a systematic analysis for the Global Burden of Disease Study 2017 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2004979667 http://dx.doi.org/10.1016/S2352-4642(19)30402-X VL - 4 ID - 562 ER - TY - JOUR AB - AIM: This paper is a report of a study of the process of grandparent involvement with siblings of preschool children with hypoplastic left heart syndrome. BACKGROUND: An increasing number of grandparents are involved in parental or near-parental roles with their grandchildren. Most research concerns grandparent involvement due to parental issues (e.g. teenage pregnancy, mental illness, addiction). Some research addresses grandparent involvement when their grandchild is ill. Grandparents''double concern' for both their adult children and their ill grandchildren is reported in the literature. In this paper, we describe a third concern for grandparents: the sibling(s) of their sick grandchild. METHOD: Individual interviews were conducted in 2007 with 15 grandparents of six preschool children with complex congenital heart disease. Open and selective coding, categorization, and theoretical memoing were used to analyse the data. FINDINGS: 'Stepping in as needed' and 'safeguarding relationships' were identified as two core categories related to grandparenting siblings of children with heart disease. Grandparents stepped into a parent role with toddler and preschool-aged siblings by attending to their daily care routines, recreational and play times, and relational needs while parents were occupied with their sick and hospitalized infants. Grandparents' concerted efforts to sustain parent-child and child-sibling relationships were also striking. CONCLUSION: Our findings extend the concept of 'double concern' to 'triple concern', and direct a research and practice focus towards the unexplored roles and needs of grandparents and siblings in families whose young children have life-threatening illnesses. AD - Faculty of Nursing, University of Alberta, Edmonton, Alberta, Canada. AN - 21039778 AU - Ravindran, V. P. AU - Rempel, G. R. DA - Jan DB - PubMed DO - 10.1111/j.1365-2648.2010.05482.x DP - NLM ET - 2010/11/03 IS - 1 KW - Adolescent Adult Aged Child Child Care/organization & administration Child, Preschool *Family Characteristics *Family Health Female Home Nursing/psychology Humans Hypoplastic Left Heart Syndrome/*psychology/therapy Infant *Intergenerational Relations Male Middle Aged Parenting/psychology Pregnancy Qualitative Research *Role *Siblings LA - eng N1 - 1365-2648 Ravindran, Vinitha Paul Rempel, Gwen R Canadian Institutes of Health Research/Canada Journal Article Research Support, Non-U.S. Gov't England J Adv Nurs. 2011 Jan;67(1):169-75. doi: 10.1111/j.1365-2648.2010.05482.x. Epub 2010 Oct 8. PY - 2011 SN - 0309-2402 SP - 169-75 ST - Grandparents and siblings of children with congenital heart disease T2 - J Adv Nurs TI - Grandparents and siblings of children with congenital heart disease VL - 67 ID - 199 ER - TY - JOUR AB - This paper reports on a study that examined the grief and coping of 29 parents whose child has hypoplastic left heart syndrome using the Dual Process Model. The study employed a secondary thematic analysis of interviews at key times of treatment and recovery for the child. After the diagnosis, parents experienced intense loss (LO), but focused upon restoration-orientated tasks (RO) to support their child. Over time, most parents employed a healthy oscillation between LO coping and RO coping, with waves of grief and with some grieving suppressed. There are some specific grief and coping and gender patterns employed by parents. AD - a Vic , Australia. AN - 29338622 AU - Cantwell-Bartl, A. DA - Oct DB - PubMed DO - 10.1080/07481187.2017.1407380 DP - NLM ET - 2018/01/18 IS - 9 KW - *Adaptation, Psychological/classification Adult Child *Disabled Children Female *Grief Humans Hypoplastic Left Heart Syndrome/*nursing Male *Models, Psychological Parents/*psychology LA - eng N1 - 1091-7683 Cantwell-Bartl, Annie Journal Article United States Death Stud. 2018 Oct;42(9):569-578. doi: 10.1080/07481187.2017.1407380. Epub 2018 Apr 9. PY - 2018 SN - 0748-1187 SP - 569-578 ST - Grief and coping of parents whose child has a constant life-threatening disability, hypoplastic left heart syndrome with reference to the Dual-Process Model T2 - Death Stud TI - Grief and coping of parents whose child has a constant life-threatening disability, hypoplastic left heart syndrome with reference to the Dual-Process Model VL - 42 ID - 65 ER - TY - JOUR AU - Paul, E. A. AU - Kohlberg, E. M. AU - Orfali, K. DB - Medline DO - 10.1080/15265161.2017.1314051 IS - 7 KW - child parent relation counseling decision making education ethics human hypoplastic left heart syndrome informed consent medical ethics newborn parental consent patient comfort psychology risk assessment treatment withdrawal LA - English M3 - Article N1 - L625031350 2018-11-22 2019-07-05 PY - 2017 SN - 1536-0075 SP - 67-68 ST - Growing Discomfort With Comfort Care for Hypoplastic Left Heart Syndrome: Why We Should Still Defer to Parental Wishes T2 - The American journal of bioethics : AJOB TI - Growing Discomfort With Comfort Care for Hypoplastic Left Heart Syndrome: Why We Should Still Defer to Parental Wishes UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L625031350 http://dx.doi.org/10.1080/15265161.2017.1314051 VL - 17 ID - 748 ER - TY - JOUR AB - OBJECTIVE: To assess the variability in asymmetric growth and its association with neurodevelopment in infants with single ventricle (SV). STUDY DESIGN: We analyzed weight-for-age z-score minus head circumference-for-age z-score (HCAZ), relative head growth (cm/kg), along with individual growth variables in subjects prospectively enrolled in the Infant Single Ventricle Trial. Associations between growth indices and scores on the Psychomotor Developmental Index (PDI) and Mental Developmental Index (MDI) of the Bayley Scales of Infant Development-II (BSID-II) at 14 months were assessed. RESULTS: Of the 230 subjects enrolled in the Infant Single Ventricle trial, complete growth data and BSID-II scores were available in 168 (73%). Across the cohort, indices of asymmetric growth varied widely at enrollment and before superior cavopulmonary connection (SCPC) surgery. BSID-II scores were not associated with these asymmetry indices. In bivariate analyses, greater pre-SCPC HCAZ correlated with higher MDI (r = 0.21; P = .006) and PDI (r = 0.38; P < .001) and a greater HCAZ increase from enrollment to pre-SCPC with higher PDI (r = 0.15; P = .049). In multivariable modeling, pre-SCPC HCAZ was an independent predictor of PDI (P = .03), but not MDI. CONCLUSION: In infants with SV, growth asymmetry was not associated with neurodevelopment at 14 months, but pre-SCPC HCAZ was associated with PDI. Asymmetric growth, important in other high-risk infants, is not a brain-sparing adaptation in infants with SV. TRIAL REGISTRATION: Clinicaltrials.gov: NCT00113087. AD - Department of Pediatrics, University of Utah, Salt Lake City, UT. Electronic address: thomas.a.miller@hsc.utah.edu. New England Research Institute, Watertown, MA. Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA. National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD. Department of Cardiology, Boston Children's Hospital, and Department of Pediatrics, Harvard Medical School, Boston, MA. Department of Pediatrics, Children's Hospital of Wisconsin, Milwaukee, WI. Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH. Department of Surgery, University of Utah, Salt Lake City, UT. Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada. Department of Pediatrics, Columbia University Medical Center, New York, NY. Department of Pediatrics, Children's Hospital at Montefiore/Albert Einstein College of Medicine, New York, NY. Department of Pediatrics, Duke University Medical Center, Durham, NC. Department of Pediatrics, Medical University of South Carolina, Charleston, SC. Department of Pediatrics, University of Utah, Salt Lake City, UT. AN - 26490132 AU - Miller, T. A. AU - Zak, V. AU - Shrader, P. AU - Ravishankar, C. AU - Pemberton, V. L. AU - Newburger, J. W. AU - Shillingford, A. J. AU - Dagincourt, N. AU - Cnota, J. F. AU - Lambert, L. M. AU - Sananes, R. AU - Richmond, M. E. AU - Hsu, D. T. AU - Miller, S. G. AU - Zyblewski, S. C. AU - Williams, R. V. C2 - PMC4698012 C6 - NIHMS723668 DA - Jan DB - PubMed DO - 10.1016/j.jpeds.2015.09.041 DP - NLM ET - 2015/10/23 KW - Angiotensin-Converting Enzyme Inhibitors/therapeutic use Cardiovascular Abnormalities *Cephalometry Double-Blind Method Enalapril/therapeutic use Female Growth Disorders/*etiology Heart Defects, Congenital/*complications/drug therapy Heart Ventricles/*abnormalities Humans Infant Infant, Newborn Male Neurodevelopmental Disorders/*etiology Prospective Studies LA - eng N1 - 1097-6833 Miller, Thomas A Zak, Victor Shrader, Peter Ravishankar, Chitra Pemberton, Victoria L Newburger, Jane W Shillingford, Amanda J Dagincourt, Nicholas Cnota, James F Lambert, Linda M Sananes, Renee Richmond, Marc E Hsu, Daphne T Miller, Stephen G Zyblewski, Sinai C Williams, Richard V Pediatric Heart Network Investigators HL068288/HL/NHLBI NIH HHS/United States HL068285/HL/NHLBI NIH HHS/United States HL109737/HL/NHLBI NIH HHS/United States U10 HL109781/HL/NHLBI NIH HHS/United States U01 HL068269/HL/NHLBI NIH HHS/United States U01 HL068279/HL/NHLBI NIH HHS/United States U10 HL109743/HL/NHLBI NIH HHS/United States U01 HL068290/HL/NHLBI NIH HHS/United States HL068279/HL/NHLBI NIH HHS/United States U01 HL068288/HL/NHLBI NIH HHS/United States HL109781/HL/NHLBI NIH HHS/United States U10 HL068270/HL/NHLBI NIH HHS/United States U01 HL068281/HL/NHLBI NIH HHS/United States U01 HL068270/HL/NHLBI NIH HHS/United States HL085057/HL/NHLBI NIH HHS/United States U54 HD090255/HD/NICHD NIH HHS/United States U10 HL109737/HL/NHLBI NIH HHS/United States HL068281/HL/NHLBI NIH HHS/United States U01 HL068292/HL/NHLBI NIH HHS/United States HL068269/HL/NHLBI NIH HHS/United States HL068270/HL/NHLBI NIH HHS/United States U01 HL085057/HL/NHLBI NIH HHS/United States HL068290/HL/NHLBI NIH HHS/United States U10 HL109673/HL/NHLBI NIH HHS/United States U01 HL068285/HL/NHLBI NIH HHS/United States HL068292/HL/NHLBI NIH HHS/United States Journal Article Multicenter Study Randomized Controlled Trial Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S. J Pediatr. 2016 Jan;168:220-5.e1. doi: 10.1016/j.jpeds.2015.09.041. Epub 2015 Oct 17. PY - 2016 SN - 0022-3476 (Print) 0022-3476 SP - 220-5.e1 ST - Growth Asymmetry, Head Circumference, and Neurodevelopmental Outcomes in Infants with Single Ventricles T2 - J Pediatr TI - Growth Asymmetry, Head Circumference, and Neurodevelopmental Outcomes in Infants with Single Ventricles VL - 168 ID - 138 ER - TY - JOUR AB - BACKGROUND: In patients after the Fontan procedure, assessment of ventricular function is difficult and amino-terminal pro-B-type natriuretic peptide levels failed to be directly related to echocardiographic measures of systolic ventricular function. The aim of the study was to evaluate growth differentiation factor 15 (GDF-15), a marker of various stress pathways in the heart and extracardiac tissues. METHODS: Plasma GDF-15 levels were measured in 38 consecutive patients after the Fontan procedure and compared to clinical, echocardiographic, and laboratory data; liver tissue stiffness; and venous hepatic flow velocities. RESULTS: Mean GDF-15 levels were 987.2±440.5 pg/mL in patients with an ejection fraction (EF)<50% as compared to 520.2±143.1 pg/mL in those with an EF≥50% (P<.001). Growth differentiation factor 15 levels were significantly related to the EF of the single ventricle (r=-0.66, P<.001), New York Heart Association functional class (r=0.43, P=.008), and γGT levels (r=0.50, P=.002) but weakly to liver tissue stiffness. According to receiver operating characteristic curve analysis, an EF<50% was best predicted by GDF-15 levels (area under the curve [AUC] 0.90, P<.001), peak venous hepatic flow at deep inspiration (AUC 0.89, P=.002), and age at Fontan operation (AUC 0.86, P=.001). Growth differentiation factor 15 and age at Fontan operation proved to be independent predictors in the multivariate analysis. The optimal cutoff of GDF-15 for the prediction of an EF<50% was calculated to be 613 pg/mL with a sensitivity of 90.0% and specificity of 85.7%. CONCLUSIONS: Growth differentiation factor 15 might be helpful in detecting early abnormal function of the Fontan circuit in patients with univentricular hearts. In patients with GDF-15 levels exceeding 613 pg/mL, further cardiac evaluation should be considered because impaired systolic function of the single ventricle may be present. AD - Department of Pediatric Cardiology, Saarland University Hospital, Homburg/Saar, Germany. tanja.raedle-hurst@uks.eu AN - 21146665 AU - Raedle-Hurst, T. M. AU - Koenigstein, K. AU - Gruenhage, F. AU - Raedle, J. AU - Herrmann, E. AU - Abdul-Khaliq, H. DA - Dec DB - PubMed DO - 10.1016/j.ahj.2010.08.033 DP - NLM ET - 2010/12/15 IS - 6 KW - Adolescent Adult Biomarkers/blood Blood Flow Velocity Child Child, Preschool Disease Progression Echocardiography, Doppler, Color Female Follow-Up Studies *Fontan Procedure Growth Differentiation Factor 15/*blood Heart Defects, Congenital/*blood/physiopathology/surgery Heart Ventricles/*abnormalities/diagnostic imaging/physiopathology Hepatic Veins/physiopathology Humans Male Postoperative Period Severity of Illness Index Systole Ventricular Dysfunction, Left/*blood/diagnostic imaging/physiopathology Ventricular Function, Left/physiology Young Adult LA - eng N1 - 1097-6744 Raedle-Hurst, Tanja M Koenigstein, Karsten Gruenhage, Frank Raedle, Jochen Herrmann, Eva Abdul-Khaliq, Hashim Comparative Study Journal Article United States Am Heart J. 2010 Dec;160(6):1105-12. doi: 10.1016/j.ahj.2010.08.033. PY - 2010 SN - 0002-8703 SP - 1105-12 ST - Growth differentiation factor 15--an early marker of abnormal function of the Fontan circuit in patients with univentricular hearts T2 - Am Heart J TI - Growth differentiation factor 15--an early marker of abnormal function of the Fontan circuit in patients with univentricular hearts VL - 160 ID - 346 ER - TY - JOUR AB - Background: In patients after the Fontan procedure, assessment of ventricular function is difficult and amino-terminal pro-B-type natriuretic peptide levels failed to be directly related to echocardiographic measures of systolic ventricular function. The aim of the study was to evaluate growth differentiation factor 15 (GDF-15), a marker of various stress pathways in the heart and extracardiac tissues. Methods: Plasma GDF-15 levels were measured in 38 consecutive patients after the Fontan procedure and compared to clinical, echocardiographic, and laboratory data; liver tissue stiffness; and venous hepatic flow velocities. Results: Mean GDF-15 levels were 987.2±440.5 pg/mL in patients with an ejection fraction (EF)<50% as compared to 520.2±143.1 pg/mL in those with an EF≥50% (P<.001). Growth differentiation factor 15 levels were significantly related to the EF of the single ventricle (r=-0.66, P<.001), New York Heart Association functional class (r=0.43, P=.008), and γGT levels (r=0.50, P=.002) but weakly to liver tissue stiffness. According to receiver operating characteristic curve analysis, an EF<50% was best predicted by GDF-15 levels (area under the curve [AUC] 0.90, P<.001), peak venous hepatic flow at deep inspiration (AUC 0.89, P=.002), and age at Fontan operation (AUC 0.86, P=.001). Growth differentiation factor 15 and age at Fontan operation proved to be independent predictors in the multivariate analysis. The optimal cutoff of GDF-15 for the prediction of an EF<50% was calculated to be 613 pg/mL with a sensitivity of 90.0% and specificity of 85.7%. Conclusions: Growth differentiation factor 15 might be helpful in detecting early abnormal function of the Fontan circuit in patients with univentricular hearts. In patients with GDF-15 levels exceeding 613 pg/mL, further cardiac evaluation should be considered because impaired systolic function of the single ventricle may be present. AD - Department of Pediatric Cardiology, Saarland University Hospital, Homburg/Saar, Germany Department of Pediatric Cardiology, Saarland University Hospital, Homburg/Saar, Germany. AN - 104966545. Language: English. Entry Date: 20110218. Revision Date: 20170411. Publication Type: journal article AU - Raedle-Hurst, T. M. AU - Koenigstein, K. AU - Gruenhage, F. AU - Raedle, J. AU - Herrmann, E. AU - Abdul-Khaliq, H. AU - Raedle-Hurst, Tanja M. AU - Koenigstein, Karsten AU - Gruenhage, Frank AU - Raedle, Jochen AU - Herrmann, Eva AU - Abdul-Khaliq, Hashim DB - ccm DO - 10.1016/j.ahj.2010.08.033 DP - EBSCOhost IS - 6 KW - Cardiopulmonary Bypass Cytokines -- Blood Heart Defects, Congenital -- Blood Heart Ventricle -- Abnormalities Ventricular Dysfunction, Left -- Blood Adolescence Adult Biological Markers -- Blood Blood Flow Velocity Child Child, Preschool Disease Progression Echocardiography, Doppler, Color Female Prospective Studies Heart Defects, Congenital -- Physiopathology Heart Defects, Congenital -- Surgery Heart Ventricle -- Physiopathology Heart Ventricle -- Ultrasonography Hepatic Veins -- Physiopathology Human Male Postoperative Period Severity of Illness Indices Systole Ventricular Dysfunction, Left -- Physiopathology Ventricular Dysfunction, Left -- Ultrasonography Cardiovascular System Physiology Young Adult N1 - research. Journal Subset: Biomedical; USA. NLM UID: 0370465. PMID: NLM21146665. PY - 2010 SN - 0002-8703 SP - 1105-1112 ST - Growth differentiation factor 15--an early marker of abnormal function of the Fontan circuit in patients with univentricular hearts T2 - American Heart Journal TI - Growth differentiation factor 15--an early marker of abnormal function of the Fontan circuit in patients with univentricular hearts UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104966545&site=ehost-live&scope=site VL - 160 ID - 1578 ER - TY - JOUR AB - Background: In patients after the Fontan procedure, assessment of ventricular function is difficult and amino-terminal pro-B-type natriuretic peptide levels failed to be directly related to echocardiographic measures of systolic ventricular function. The aim of the study was to evaluate growth differentiation factor 15 (GDF-15), a marker of various stress pathways in the heart and extracardiac tissues. Methods: Plasma GDF-15 levels were measured in 38 consecutive patients after the Fontan procedure and compared to clinical, echocardiographic, and laboratory data; liver tissue stiffness; and venous hepatic flow velocities. Results: Mean GDF-15 levels were 987.2 ± 440.5 pg/mL in patients with an ejection fraction (EF) <50% as compared to 520.2 ± 143.1 pg/mL in those with an EF ≥50% (P < .001). Growth differentiation factor 15 levels were significantly related to the EF of the single ventricle (r = -0.66, P < .001), New York Heart Association functional class (r = 0.43, P = .008), and γGT levels (r = 0.50, P = .002) but weakly to liver tissue stiffness. According to receiver operating characteristic curve analysis, an EF <50% was best predicted by GDF-15 levels (area under the curve [AUC] 0.90, P < .001), peak venous hepatic flow at deep inspiration (AUC 0.89, P = .002), and age at Fontan operation (AUC 0.86, P = .001). Growth differentiation factor 15 and age at Fontan operation proved to be independent predictors in the multivariate analysis. The optimal cutoff of GDF-15 for the prediction of an EF <50% was calculated to be 613 pg/mL with a sensitivity of 90.0% and specificity of 85.7%. Conclusions: Growth differentiation factor 15 might be helpful in detecting early abnormal function of the Fontan circuit in patients with univentricular hearts. In patients with GDF-15 levels exceeding 613 pg/mL, further cardiac evaluation should be considered because impaired systolic function of the single ventricle may be present. © 2010 Mosby, Inc. AD - T. M. Raedle-Hurst, Department of Pediatric Cardiology, Saarland University Hospital, Kirrberger Str., D-66421 Homburg/Saar, Germany AU - Raedle-Hurst, T. M. AU - Koenigstein, K. AU - Gruenhage, F. AU - Raedle, J. AU - Herrmann, E. AU - Abdul-Khaliq, H. DB - Embase Medline DO - 10.1016/j.ahj.2010.08.033 IS - 6 KW - amino terminal pro brain natriuretic peptide biological marker gamma glutamyltransferase growth differentiation factor 15 adolescent adult article blood flow velocity child clinical article correlation analysis elastography female Fontan procedure functional assessment gamma glutamyl transferase blood level groups by age heart ejection fraction heart single ventricle heart ventricle function human liver blood flow liver fibrosis male predictor variable preschool child priority journal protein blood level receiver operating characteristic school child sensitivity and specificity two dimensional echocardiography vein blood flow LA - English M3 - Article N1 - L360154216 2010-12-22 2010-12-30 PY - 2010 SN - 0002-8703 1097-6744 SP - 1105-1112 ST - Growth differentiation factor 15-an early marker of abnormal function of the Fontan circuit in patients with univentricular hearts T2 - American Heart Journal TI - Growth differentiation factor 15-an early marker of abnormal function of the Fontan circuit in patients with univentricular hearts UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L360154216 http://dx.doi.org/10.1016/j.ahj.2010.08.033 VL - 160 ID - 1129 ER - TY - JOUR AB - Tricuspid atresia has been regarded as one of the rare congenital cardiac anomalies, but it must be considered in the differential diagnosis of every infant with cyanotic heart disease. The physiological consequences of the anomaly are manifold. Arterial desaturation is caused by complete mixing of systemic venous and pulmonary venous blood in the left atrium. The degree of unsaturation and cardiac handicap depends on the pulmonary flow and the size of the atrial scptal opening. If the atrial sept al defect is small it causes raised right atrial pressure, and this in turn results in raised systemic venous pressure and severe congestive failure. In such cased enlargement of the atrial septal defect should decrease central venous pressure and thus relieve the congestive heart failure. The authors report here the application of the technique of atrial septostomy in a small infant with tricuspid atresia and transposition of the great vessels with an extremely small intra-atrial communication. (18,1,7*). AD - S.P. Singh, Unit. Birmingham Hosps, Birmingham AU - Singh, S. P. DB - Embase Classic KW - catheter central venous pressure congestive heart failure cyanotic heart disease differential diagnosis disability fatty acid desaturation great blood vessel heart atrium septum defect heart disease heart left atrium heart right atrium pressure hemodynamics infant interpersonal communication tricuspid valve atresia venous blood venous pressure LA - English M3 - Article N1 - L288039511 1968-12-01 PY - 1968 SN - 0007-1447 SP - 225-226 ST - Haemodynamic effects of balloon septostomy in tricuspid atresia T2 - British medical Journal (1857) TI - Haemodynamic effects of balloon septostomy in tricuspid atresia UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L288039511 ID - 1408 ER - TY - JOUR AB - BACKGROUND: Five decades after its introduction, the Glenn shunt remains an integral step for patients undergoing single-ventricle palliation. We performed a longitudinal follow-up of the original cohort of patients who underwent Glenn shunt. METHODS: We performed a retrospective study of the original cohort of patients who underwent Glenn shunt at Yale between 1958 and 1988. Electronic medical records and chart review up to current era were used to collect data. RESULTS: Ninety-one patients underwent a Glenn shunt at an average age of 6.6 ± 2.5 years, of which 89 were classic Glenn shunts. Median overall survival was 43 years (range, 2 to 56; 95% confidence interval [CI], 39.5 to 46.5) while median survival from the Glenn shunt was 31.4 years (range, 0 to 45; 95% CI, 23.9 to 38.9). Forty-six patients died, 7 in the early postoperative period and 39 late deaths. Twenty-six patients were lost to follow-up. Nineteen patients remain alive with active clinical follow-up, 6 of whom still live with their classic Glenn shunt without conversion to bidirectional Glenn. Twenty-six patients (31%) developed pulmonary arteriovenous fistula with 11 patients (42%) requiring coil embolization. No patient developed thrombosis of the Glenn shunt. There were a total of 28 patients who developed arrhythmias, mostly in the tricuspid atresia group (n = 16), with the majority being atrial tachyarrhythmias (48%). Sixteen patients required permanent pacemaker placement for sinus node dysfunction. CONCLUSIONS: The Glenn shunt continues to provide excellent staged palliation in single-ventricle patients and a bridge to two-ventricle repair. Arrhythmias and pulmonary arteriovenous fistulas were common among single-ventricle cohort. Quality-of-life evaluation of the surviving patients would be an important outcome measure for future investigation. AD - Department of Pediatrics, Section of Pediatric Cardiology, Yale University School of Medicine, New Haven, Connecticut. Department of Surgery, Section of Pediatric Cardiothoracic Surgery, Yale University School of Medicine, New Haven, Connecticut. Department of Surgery, Section of Pediatric Cardiothoracic Surgery, Yale University School of Medicine, New Haven, Connecticut. Electronic address: mohsen.karimi@yale.edu. AN - 26603022 AU - Zahr, R. A. AU - Kirshbom, P. M. AU - Kopf, G. S. AU - Sainathan, S. AU - Steele, M. M. AU - Elder, R. W. AU - Karimi, M. DA - Jan DB - PubMed DO - 10.1016/j.athoracsur.2015.08.018 DP - NLM ET - 2015/11/26 IS - 1 KW - Child Female Follow-Up Studies Fontan Procedure/*methods *Forecasting Heart Defects, Congenital/*surgery Heart Ventricles/abnormalities/*surgery Humans Male Palliative Care/methods Pulmonary Artery/*surgery Retrospective Studies Treatment Outcome LA - eng N1 - 1552-6259 Zahr, Riad Abou Kirshbom, Paul M Kopf, Gary S Sainathan, Sandeep Steele, Margaret M Elder, Robert W Karimi, Mohsen Journal Article Netherlands Ann Thorac Surg. 2016 Jan;101(1):177-82. doi: 10.1016/j.athoracsur.2015.08.018. Epub 2015 Oct 23. PY - 2016 SN - 0003-4975 SP - 177-82 ST - Half a Century's Experience With the Superior Cavopulmonary (Classic Glenn) Shunt T2 - Ann Thorac Surg TI - Half a Century's Experience With the Superior Cavopulmonary (Classic Glenn) Shunt VL - 101 ID - 260 ER - TY - JOUR AB - Hallucinations in children and adolescents are now known to occur on a continuum from healthy to psychopathology-related phenomena. Although hallucinations in young populations are mostly transient, they can cause substantial distress. Despite hallucinations being widely investigated, research so far has had limited implications for clinical practice. The present article has 3 main aims: (1) to review research findings since 2014 (when the last major review of the area was published); (2) to present assessment tools validated to measure hallucinations in children and adolescents; and (3) to discuss therapeutic strategies and clinical issues. We conclude by presenting a tailored care model for clinicians and outline future challenges for research. (PsycInfo Database Record (c) 2020 APA, all rights reserved) AD - Maijer, Kim, University Medical Center Utrecht, Psychiatry department, HP A00.241, Heidelberglaan 100, 3485CX, Utrecht, Netherlands AN - 2019-11814-003 AU - Maijer, Kim AU - Hayward, Mark AU - Fernyhough, Charles AU - Calkins, Monica E. AU - Debbané, Martin AU - Jardri, Renaud AU - Kelleher, Ian AU - Raballo, Andrea AU - Rammou, Aikaterini AU - Scott, James G. AU - Shinn, Ann K. AU - Steenhuis, Laura A. AU - Wolf, Daniel H. AU - Bartels-Velthuis, Agna A. DB - psyh DO - 10.1093/schbul/sby119 DP - EBSCOhost IS - Suppl 1 KW - youth psychotic experiences assessment intervention (mental) health care Adolescent Psychopathology Child Psychopathology Hallucinations Psychological Assessment Psychosis Community Mental Health Services Psychotherapy N1 - Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht, Netherlands. Other Publishers: National Institute of Mental Health. Release Date: 20200427. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Grant Information: Fernyhough, Charles. Major Descriptor: Adolescent Psychopathology; Child Psychopathology; Hallucinations; Psychological Assessment; Psychosis. Minor Descriptor: Community Mental Health Services; Psychotherapy. Classification: Schizophrenia & Psychotic States (3213). Population: Human (10); Male (30); Female (40). Age Group: Childhood (birth-12 yrs) (100); Adolescence (13-17 yrs) (200). Methodology: Literature Review. Page Count: 19. Issue Publication Date: Jan, 2019. Copyright Statement: Published by Oxford University Press on behalf of the Maryland Psychiatric Research Center. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited. The Author(s). 2018. Sponsor: Wellcome Trust. Grant: WT108720. Recipients: Fernyhough, Charles Sponsor: Australian National Health and Medical Research Council, Australia. Grant: APP1105807. Other Details: Practitioner Fellowship. Recipients: Scott, James G. Sponsor: Norwegian University of Science and Technology, Norway. Grant: 70440154. Other Details: Onsager Fellowship Program in Psychopathology and Development. Recipients: Raballo, Andrea PY - 2019 SN - 0586-7614 1745-1701 SP - S5-S23 ST - Hallucinations in children and adolescents: An updated review and practical recommendations for clinicians T2 - Schizophrenia Bulletin TI - Hallucinations in children and adolescents: An updated review and practical recommendations for clinicians UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2019-11814-003&site=ehost-live&scope=site ORCID: 0000-0002-7451-2119 ORCID: 0000-0003-3709-4111 ORCID: 0000-0003-4596-1502 ORCID: 0000-0002-4677-8753 ORCID: 0000-0001-6567-7723 k.maijer@umcutrecht.nl VL - 45 ID - 1661 ER - TY - JOUR AB - BACKGROUND: When performed by cardiologists, hand-held echocardiography (HHE) can assess ventricular systolic function and valve disease in adults, but its accuracy and utility in congenital heart disease is unknown. In hypoplastic left heart syndrome (HLHS), the echocardiographic detection of depressed right ventricular (RV) systolic function and higher grade tricuspid regurgitation (TR) can identify patients who are at increased risk of morbidity and mortality and who may benefit from additional imaging or medical therapies. METHODS: Children with HLHS after Stage I or II surgical palliation (Norwood or Glenn procedures) were prospectively enrolled. Subjects underwent HHE by a pediatric cardiologist on the same day as standard echocardiography (SE). Using 4-point scales, bedside HHE assessment of RV systolic function and TR were compared with blinded assessment of offline SE images. Concordance correlation coefficient (CCC) was used to evaluate agreement. RESULTS: Thirty-two HHEs were performed on 15 subjects (Stage I: n = 17 and Stage II: n = 15). Median subject age was 3.4 months (14 days-4.2 years). Median weight was 5.9 kg (2.6-15.4 kg). Bedside HHE assessment of RV systolic function and TR severity had substantial agreement with SE (CCC = 0.80, CCC = 0.74, respectively; P < .001). HHE sensitivity and specificity for any grade of depressed RV systolic function were 100% and 92%, respectively, and were 94% and 88% for moderate or greater TR, respectively. Average HHE scan time was 238 seconds. CONCLUSIONS: HHE offers a rapid, bedside tool for pediatric cardiologists to detect RV systolic dysfunction and hemodynamically significant TR in HLHS. AD - Section of Pediatric Cardiology, Department of Pediatrics, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas. Newborn Center, Texas Children's Hospital, Houston, Texas. AN - 30973683 AU - Riley, A. F. AU - Ocampo, E. C. AU - Hagan, J. AU - Lantin-Hermoso, M. R. DA - Sep DB - PubMed DO - 10.1111/chd.12774 DP - NLM ET - 2019/04/12 IS - 5 KW - Cardiac Surgical Procedures/methods Child, Preschool Echocardiography/*methods Female Follow-Up Studies Humans Hypoplastic Left Heart Syndrome/*diagnosis/physiopathology/surgery Infant Infant, Newborn Male Prospective Studies Reproducibility of Results Systole Ventricular Function, Right/*physiology congenital heart disease focused cardiac ultrasound hypoplastic left heart syndrome LA - eng N1 - 1747-0803 Riley, Alan F Orcid: 0000-0003-0114-6928 Ocampo, Elena C Hagan, Joseph Lantin-Hermoso, M Regina Journal Article United States Congenit Heart Dis. 2019 Sep;14(5):706-712. doi: 10.1111/chd.12774. Epub 2019 Apr 11. PY - 2019 SN - 1747-079x SP - 706-712 ST - Hand-held echocardiography in children with hypoplastic left heart syndrome T2 - Congenit Heart Dis TI - Hand-held echocardiography in children with hypoplastic left heart syndrome VL - 14 ID - 62 ER - TY - JOUR AB - Infants with single ventricle require staged cardiac surgery, with stage I typically performed shortly after birth, stage II at 4 to 6 months of age, and stage III at 3 to 5 years of age. There is a high risk of interstage mortality and morbidity after infants are discharged from the hospital between stages I and II. Traditional home monitoring requires caregivers to record measurements of weight and oxygen saturation into a binder and requires families to assume a surveillance role. We have developed a tablet PC-based solution that provides secure and nearly instantaneous transfer of patient information to a cloud-based server, with the capacity for instant alerts to be sent to the caregiver team. The cloud-based IT infrastructure lends itself well to being able to be scaled to multiple sites while maintaining strict control over the privacy of each site. All transmitted data are transferred to the electronic medical record daily. The system conforms to recently released Food and Drug Administration regulation that pertains to mobile health technologies and devices. Since this platform was developed in March 2014, 30 patients have been monitored. There have been no interstage deaths. The experience of care providers has been unanimously positive. The addition of video has added to the use of the monitoring program. Of 30 families, 23 expressed a preference for the tablet PC over the notebook, 3 had no preference, and 4 preferred the notebook to the tablet PC. AD - From the Ward Family Heart Center, Children's Mercy Kansas City, MO (G.S., L.E., J.A., D.T., M.B., L.R., R.S.); Health Services and Outcomes Research, Children's Mercy Kansas City, MO (K.G., D.W., K.R., A.B.-E.); and Saint Luke's Mid America Heart Institute, Kansas City, MO (J.S.). gsshirali@cmh.edu. From the Ward Family Heart Center, Children's Mercy Kansas City, MO (G.S., L.E., J.A., D.T., M.B., L.R., R.S.); Health Services and Outcomes Research, Children's Mercy Kansas City, MO (K.G., D.W., K.R., A.B.-E.); and Saint Luke's Mid America Heart Institute, Kansas City, MO (J.S.). AN - 27166202 AU - Shirali, G. AU - Erickson, L. AU - Apperson, J. AU - Goggin, K. AU - Williams, D. AU - Reid, K. AU - Bradley-Ewing, A. AU - Tucker, D. AU - Bingler, M. AU - Spertus, J. AU - Rabbitt, L. AU - Stroup, R. DA - May DB - PubMed DO - 10.1161/circoutcomes.115.002452 DP - NLM ET - 2016/05/12 IS - 3 KW - Attitude to Computers *Cardiac Surgical Procedures/adverse effects/mortality Cardiology Service, Hospital/*organization & administration Caregivers/psychology Child, Preschool Cloud Computing Computers, Handheld Delivery of Health Care, Integrated/*organization & administration Diffusion of Innovation Health Knowledge, Attitudes, Practice Heart Defects, Congenital/diagnosis/mortality/*surgery Heart Ventricles/*surgery Humans Infant Models, Organizational Patient Care Team/*organization & administration Predictive Value of Tests Process Assessment, Health Care/*organization & administration Program Evaluation Remote Sensing Technology Telemedicine/*organization & administration Time Factors Treatment Outcome *caregivers *congenital *mortality *survival *telemedicine LA - eng N1 - 1941-7705 Shirali, Girish Erickson, Lori Apperson, Jonathan Goggin, Kathy Williams, David Reid, Kimberly Bradley-Ewing, Andrea Tucker, Dawn Bingler, Michael Spertus, John Rabbitt, Leslie Stroup, Richard Journal Article United States Circ Cardiovasc Qual Outcomes. 2016 May;9(3):303-11. doi: 10.1161/CIRCOUTCOMES.115.002452. Epub 2016 May 10. PY - 2016 SN - 1941-7713 SP - 303-11 ST - Harnessing Teams and Technology to Improve Outcomes in Infants With Single Ventricle T2 - Circ Cardiovasc Qual Outcomes TI - Harnessing Teams and Technology to Improve Outcomes in Infants With Single Ventricle VL - 9 ID - 540 ER - TY - JOUR AB - Objectives: To identify and predict neo-aortic arch obstruction (NAAO) in children after Norwood/Sano operation (NO) for hypoplastic left heart syndrome (HLHS). Background: NAAO is associated with morbidity and mortality after NO for HLHS and no objective measure has predicted the initial occurrence of NAAO. Computational flow models of aortic coarctation demonstrate increased wall shear stress (WSS) in vessels proximal to the coarctation segment, which we believe also occurs with NAAO. These vessels respond by increasing their luminal diameter to maintain normal WSS. We hypothesized that the relative increase in diameters of head and neck vessels to the isthmus, as measured by angiography, would identify hemodynamically significant NAAO and predict future NAAO. Methods: Retrospective review of patients with HLHS and at least one catheterization with aortic angiography after NO. Diameters of head and neck vessels were totaled and divided by the isthmus diameter to give a head and neck index (HNI), which was compared to coarctation index (CI) for identifying and predicting future NAAO. Results: Forty-four patients were identified, 17 with and 27 without NAAO. Receiver operator characteristic analysis using a value for CI ≤0.5 showed a sensitivity of 47% and specificity of 89%. For HNI, a value >2.65 gave a sensitivity of 77% and specificity of 93%. Three patients who developed NAAO after their initial catheterization had CI >0.5, but abnormally high HNI >2.65. Conclusions: HNI is a more robust indicator of hemodynamically significant NAAO than CI and may predict its future occurrence after NO for HLHS. AD - M.D. Seckeler, Cincinnati Children's Hospital Medical Center, Division of Cardiology, 3333 Burnet Ave., Cincinnati, OH 45299, United States AU - Seckeler, M. D. AU - Raucci, F. J. AU - Saunders, C. AU - Gangemi, J. J. AU - Peeler, B. B. AU - Anitha Jayakumar, K. DB - Embase Medline IS - 2 KW - angiography aortic arch aortic coarctation aortic occlusion article blood vessel diameter clinical article head human hypoplastic left heart syndrome medical record review neck retrospective study sensitivity and specificity shear stress LA - English M3 - Article N1 - L368815788 2013-05-08 2013-05-13 PY - 2013 SN - 1042-3931 1557-2501 SP - 73-75 ST - Head and neck vessel size by angiography predicts neo-aortic arch obstruction after norwood/sano operation for hypoplastic left heart syndrome T2 - Journal of Invasive Cardiology TI - Head and neck vessel size by angiography predicts neo-aortic arch obstruction after norwood/sano operation for hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L368815788 VL - 25 ID - 1020 ER - TY - JOUR AB - OBJECTIVES: To identify and predict neo-aortic arch obstruction (NAAO) in children after Norwood/Sano operation (NO) for hypoplastic left heart syndrome (HLHS). BACKGROUND: NAAO is associated with morbidity and mortality after NO for HLHS and no objective measure has predicted the initial occurrence of NAAO. Computational flow models of aortic coarctation demonstrate increased wall shear stress (WSS) in vessels proximal to the coarctation segment, which we believe also occurs with NAAO. These vessels respond by increasing their luminal diameter to maintain normal WSS. We hypothesized that the relative increase in diameters of head and neck vessels to the isthmus, as measured by angiography, would identify hemodynamically significant NAAO and predict future NAAO. METHODS: Retrospective review of patients with HLHS and at least one catheterization with aortic angiography after NO. Diameters of head and neck vessels were totaled and divided by the isthmus diameter to give a head and neck index (HNI), which was compared to coarctation index (CI) for identifying and predicting future NAAO. RESULTS: Forty-four patients were identified, 17 with and 27 without NAAO. Receiver operator characteristic analysis using a value for CI ≤0.5 showed a sensitivity of 47% and specificity of 89%. For HNI, a value >2.65 gave a sensitivity of 77% and specificity of 93%. Three patients who developed NAAO after their initial catheterization had CI >0.5, but abnormally high HNI >2.65. CONCLUSIONS: HNI is a more robust indicator of hemodynamically significant NAAO than CI and may predict its future occurrence after NO for HLHS. AD - Cincinnati Children's Hospital Medical Center, Division of Cardiology, 3333 Burnet Ave, Cincinnati, OH 45299, USA. seckeler@hotmail.com AN - 23388224 AU - Seckeler, M. D. AU - Raucci, F. J. AU - Saunders, C. AU - Gangemi, J. J. AU - Peeler, B. B. AU - Jayakumar, K. A. DA - Feb DB - PubMed DP - NLM ET - 2013/02/08 IS - 2 KW - Aorta, Thoracic/*diagnostic imaging/surgery Aortography/*methods Female Head/*blood supply Humans Hypoplastic Left Heart Syndrome/*diagnostic imaging/surgery Infant Male Neck/*blood supply Norwood Procedures/*methods Prognosis LA - eng N1 - 1557-2501 Seckeler, Michael D Raucci, Frank J Saunders, Christine Gangemi, James J Peeler, Benjamin B Jayakumar, K Anitha Comparative Study Journal Article United States J Invasive Cardiol. 2013 Feb;25(2):73-5. PY - 2013 SN - 1042-3931 SP - 73-5 ST - Head and neck vessel size by angiography predicts neo-aortic arch obstruction after Norwood/Sano operation for hypoplastic left heart syndrome T2 - J Invasive Cardiol TI - Head and neck vessel size by angiography predicts neo-aortic arch obstruction after Norwood/Sano operation for hypoplastic left heart syndrome VL - 25 ID - 294 ER - TY - JOUR AB - BACKGROUND: Health care professionals (HCPs) may be involved in counselling women after an antenatal diagnosis of various fetal anomalies. Many pregnant women consider termination of pregnancy (TOP) after antenatal diagnosis of various fetal anomalies. Little is known, however, about the attitudes of HCPs regarding TOP for specific antenatal diagnoses. OBJECTIVE: To determine the attitudes and opinions of HCPs in maternal and child health regarding TOP for fetal anomalies of varying severity. METHODS: An anonymous questionnaire was distributed to four groups of HCPs: obstetric residents; paediatric residents; delivery room nurses; and neonatal intensive care nurses. Respondents were asked about TOP if they or their spouse were to receive an antenatal diagnosis for five prenatally diagnosed conditions: trisomy 21; trisomy 18; cleft lip and palate; Turner syndrome; and hypoplastic left heart syndrome. RESULTS: Two hundred eighty HCPs answered the questionnaire (90% response rate). Ten per cent of respondents would not consider TOP under any of the circumstances described. Among those who would consider TOP, they were most likely to do so for trisomy 18 and least likely for cleft lip and palate, and fairly evenly divided among the remaining three conditions (hypoplastic left heart syndrome [65%], trisomy 21 [56%] and Turner syndrome [37%]). Paediatric residents were less likely to choose TOP than other groups and obstetrics residents were most likely. CONCLUSIONS: Attitudes of HCPs toward TOP vary according to prenatally identified condition and professional group. More rigorous analysis should be performed regarding the process of counselling and the impact of HCPs beliefs on parental decisions. ©2012 Pulsus Group Inc. All rights reserved. AD - A. Janvier, Department of Pediatrics and Clinical Ethics, University of Montreal, Sainte-Justine Hospital, 3175 Chemin Côte-Sainte-Catherine, Montreal, QC H3T 1C5, Canada AU - Janvier, A. AU - Couture, E. AU - Deschenes, M. AU - Nadeau, S. AU - Barrington, K. AU - Lantos, J. DB - Embase DO - 10.1093/pch/17.8.e86 IS - 8 KW - adult article child health cleft lip palate disease severity female fetus fetus malformation health personnel attitude human hypoplastic left heart syndrome male maternal welfare medical decision making nurse attitude parent counseling parenthood pediatrician physician attitude pregnancy termination prenatal diagnosis questionnaire religion resident trisomy 18 trisomy 21 Turner syndrome LA - English M3 - Article N1 - L365799960 2012-10-16 2012-10-20 http://www.pulsus.com/journals/Login.jsp?sCurrPg=Log%20in&theAction= PY - 2012 SN - 1205-7088 SP - e86-e88 ST - Health care professionals' attitudes about pregnancy termination for different fetal anomalies T2 - Paediatrics and Child Health (Canada) TI - Health care professionals' attitudes about pregnancy termination for different fetal anomalies UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L365799960 http://dx.doi.org/10.1093/pch/17.8.e86 VL - 17 ID - 1038 ER - TY - JOUR AB - After corrective surgery for congenital heart defects (CHD) many patients suffer from residual defects, some with reduced cardiorespiratory capacity and possible impairment of their health related quality of life (HRQoL). The aim of our study is to evaluate, how children after surgery for CHD rate their HRQoL. METHOD: A standardised questionnaire deve-loped for self-rating in children between 8 and 18 years and dealing with 7 different domains concerning the HRQoL (friends, family, physical functioning, cognition, body image, emotional function and autonomy) was independently answered from patients and their parents during an outpatient visit at their pediatric cardiologist throughout Germany. RESULTS: 173 patients (40% female, 60% male, mean age 11.6 years) were interviewed, 167 questionnaires could be evaluated. The mean time interval after surgery was 9.8±3.4 years. Patient had had surgery for complete different types of CHD (ventricular septal defects n=50, Tetralogy of Fallot n=51, univentricular heart n=26, transposition of great arteries n=40). The results were compared with those of an age-matched control group (n=169). Patients with CHD reported a better HRQoL than the controls for all items (p<0.01). There was no significant gender specific difference. After puberty, the rating for most items, except of "body image", had become very similar compared to controls. Pa-rents assessed their children significantly worse in 3 domains (friends, body image and emotion; p<0.01). CONCLUSION: Children with congenital heart defects are able to develop coping structures, that enable them to live a normal life from their individual point of view. Integration in psychosocial structures seems to be rather normal when compared to healthy controls. Many patients considered their HRQoL as even better. AD - Abteilung Paediatrische Kardiologie/Pulmonologie, Helios-Klinikum Wuppertal, Wuppertal. Pediatric Oncology, University Muenster, Muenster, Germany. Kinderkardiologische Praxis, Uhlandstrasse, Duesseldorf, Germany. Pediatric Cardiology, University Duesseldorf, Duesseldorf, Germany. AN - 25062110 AU - Heusch, A. AU - Calaminus, G. AU - Kahl, J. AU - Schmidt, K. DA - Sep DB - PubMed DO - 10.1055/s-0034-1382019 DP - NLM ET - 2014/07/26 IS - 5 KW - Adaptation, Psychological Adolescent Child Female Follow-Up Studies *Health Status Heart Defects, Congenital/*psychology/*surgery Humans Interview, Psychological Male Matched-Pair Analysis Parents/psychology Personal Autonomy Postoperative Complications/*psychology Quality of Life/*psychology Social Adjustment Surveys and Questionnaires LA - eng N1 - 1439-3824 Heusch, A Calaminus, G Kahl, J Schmidt, K Journal Article Germany Klin Padiatr. 2014 Sep;226(5):281-6. doi: 10.1055/s-0034-1382019. Epub 2014 Jul 25. PY - 2014 SN - 0300-8630 SP - 281-6 ST - Health related quality of life after corrective surgery for congenital heart disease T2 - Klin Padiatr TI - Health related quality of life after corrective surgery for congenital heart disease VL - 226 ID - 162 ER - TY - JOUR AB - INTRODUCTION: Advances in diagnostic methods, surgical techniques and postoperative intensive care have significantly increased the survival rates for children with congenital heart disease. In this context, long term sequelae acquired greater relevance. Health-related quality of life (HRQOL) is a concept that helps to measure the impact of chronic diseases. The purpose of the current study was to describe HRQOL in children with congenital heart disease that undergo cardiac surgery during their first year of life. METHODS: A cross-sectional observational study was conducted between August 2017 and December 2018 at a University General Hospital. PedsQL 4.0 Generic Core Scales were used. Children with congenital heart disease between 2 and 4 years old who had cardiac surgery during their first year of life and healthy children were included. Scores were compared with T-test or Wilcoxon according to the observed distribution. p value < 0.05 was considered significant. RESULTS: A total of 31 children with congenital heart disease (26 % with a single ventricle) and 62 healthy children were enrolled. The first surgery was in the neonatal period in 61.3 %. Our study showed no statistical differences (p = 0.10) between HRQOL Total Scale Score of children with congenital heart disease compared to healthy children. However, lower scores were observed with statistically significant differences in social (p = 0.0092) and school (p = 0.0001) scales. CONCLUSIONS: Our cohort of children diagnosed with congenital heart disease has a global quality of life comparable with healthy children except in social and school functioning scales. AD - Servicio de Clínica Pediátrica, Departamento de Pediatría, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires. melina.saavedra@hiba.org.ar. Servicio de Cardiología Pediátrica, Departamento de Pediatría, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires. Servicio de Clínica Pediátrica, Departamento de Pediatría, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires. AN - 32470251 AU - Saavedra, M. J. AU - Eymann, A. AU - Pérez, L. AU - Busaniche, J. AU - Nápoli, N. AU - Marantz, P. AU - Llera, J. DA - Jun DB - PubMed DO - 10.5546/aap.2020.eng.166 DP - NLM ET - 2020/05/30 IS - 3 KW - cardiac surgery chronic disease congenital heart disease health related quality of life LA - eng spa N1 - 1668-3501 Saavedra, Melina J Eymann, Alfredo Pérez, Lucía Busaniche, Julio Nápoli, Natalia Marantz, Pablo Llera, Julián Journal Article Argentina Arch Argent Pediatr. 2020 Jun;118(3):166-172. doi: 10.5546/aap.2020.eng.166. OP - Calidad de vida relacionada con la salud en niños con cardiopatía congénita operados durante el primer año de vida. PY - 2020 SN - 0325-0075 SP - 166-172 ST - Health related quality of life in children with congenital heart disease that undergo cardiac surgery during their first year of life T2 - Arch Argent Pediatr TI - Health related quality of life in children with congenital heart disease that undergo cardiac surgery during their first year of life VL - 118 ID - 525 ER - TY - CHAP AB - BACKGROUND: While early outcomes of paediatric cardiac surgery have improved, less attention has been given to later outcomes including post-discharge mortality and emergency readmissions. OBJECTIVES: Our objectives were to use a mixed-methods approach to build an evidenced-based guideline for postdischarge management of infants undergoing interventions for congenital heart disease (CHD). METHODS: Systematic reviews of the literature – databases used: MEDLINE (1980 to 1 February 2013), EMBASE (1980 to 1 February 2013), Cumulative Index to Nursing and Allied Health Literature (CINAHL; 1981 to 1 February 2013), The Cochrane Library (1999 to 1 February 2013), Web of Knowledge (1980 to 1 February 2013) and PsycINFO (1980 to 1 February 2013). Analysis of audit data from the National Congenital Heart Disease Audit and Paediatric Intensive Care Audit Network databases pertaining to records of infants undergoing interventions for CHD between 1 January 2005 and 31 December 2010. Qualitative analyses of online discussion posted by 73 parents, interviews with 10 helpline staff based at user groups, interviews with 20 families whose infant either died after discharge or was readmitted urgently to intensive care, and interviews with 25 professionals from tertiary care and 13 professionals from primary and secondary care. Iterative multidisciplinary review and discussion of evidence incorporating the views of parents on suggestions for improvement. RESULTS: Despite a wide search strategy, the studies identified for inclusion in reviews related only to patients with complex CHD, for whom adverse outcome was linked to non-white ethnicity, lower socioeconomic status, comorbidity, age, complexity and feeding difficulties. There was evidence to suggest that home monitoring programmes (HMPs) are beneficial. Of 7976 included infants, 333 (4.2%) died postoperatively, leaving 7634 infants, of whom 246 (3.2%) experienced outcome 1 (postdischarge death) and 514 (6.7%) experienced outcome 2 (postdischarge death plus emergency intensive care readmissions). Multiple logistic regression models for risk of outcomes 1 and 2 had areas under the receiver operator curve of 0.78 [95% confidence interval (CI) 0.75 to 0.82] and 0.78 (95% CI 0.75 to 0.80), respectively. Six patient groups were identified using classification and regression tree analysis to stratify by outcome 2 (range 3–24%), which were defined in terms of neurodevelopmental conditions, high-risk cardiac diagnosis (hypoplastic left heart, single ventricle or pulmonary atresia), congenital anomalies and length of stay (LOS) > 1 month. Deficiencies and national variability were noted for predischarge training and information, the process of discharge to non-specialist services including documentation, paediatric cardiology follow-up including HMP, psychosocial support post discharge and the processes for accessing help when an infant becomes unwell. CONCLUSIONS: National standardisation may improve discharge documents, training and guidance on ‘what is normal’ and ‘signs and symptoms to look for’, including how to respond. Infants with high-risk cardiac diagnoses, neurodevelopmental conditions or LOS > 1 month may benefit from discharge via their local hospital. HMP is suggested for infants with hypoplastic left heart, single ventricle or pulmonary atresia. Discussion of postdischarge deaths for infant CHD should occur at a network-based multidisciplinary meeting. Audit is required of outcomes for this stage of the patient journey. FUTURE WORK: Further research may determine the optimal protocol for HMPs, evaluate the use of traffic light tools for monitoring infants post discharge and develop the analytical steps and processes required for audit of postdischarge metrics. STUDY REGISTRATION: This study is registered as PROSPERO CRD42013003483 and CRD42013003484. FUNDING: The National Institute for Health Research Health Services and Delivery Research programme. The National Congenital Heart Diseases Audit (NCHDA) and Paediatric Intensive Care Audit Network (PICANet) are funded by the National Clinical Audit and Patient Outcomes Programme, administered by the Healthcare Quality Improvement Partnership (HQIP). PICAnet is also funded by Welsh Health Specialised Services Committee; NHS Lothian/National Service Division NHS Scotland, the Royal Belfast Hospital for Sick Children, National Office of Clinical Audit Ireland, and HCA International. The study was supported by the National Institute for Health Research Biomedical Research Centre at Great Ormond Street Hospital for Children NHS Foundation Trust and University College London. Sonya Crowe was supported by the Health Foundation, an independent charity working to continuously improve the quality of health care in the UK. This study identified risk factors for adverse postdischarge outcomes in infants undergoing cardiac surgery and areas where improvement is needed in the quality of care of these infants, including specifically: additional monitoring for higher risk babies, standardised discharge documents, provision of training and guidance on ‘what is normal’ and ‘signs and symptoms to look for’, including how to respond to these. eng AD - Cardiac Unit, Great Ormond Street Hospital NHS Foundation Trust, London, UK Population Policy and Practice Programme, University College London Institute of Child Health, London, UK Clinical Operational Research Unit, University College London, London, UK Cardiac Surgery Department, Birmingham Children’s Hospital NHS Foundation Trust, Birmingham, UK National Institute for Cardiovascular Outcomes Research, University College London, London, UK Faculty of Medicine and Health, University of Leeds, Leeds, UK Paediatric Cardiac Unit, Royal Brompton and Harefield Hospitals NHS Trust, London, UK Department of Paediatrics, Northampton General Hospital NHS Trust, Northampton, UK Primary Care Department, Queen Mary University of London, London, UK AN - 27252995 AU - Brown, K. L. AU - Wray, J. AU - Knowles, R. L. AU - Crowe, S. AU - Tregay, J. AU - Ridout, D. AU - Barron, D. J. AU - Cunningham, D. AU - Parslow, R. AU - Franklin, R. AU - Barnes, N. AU - Hull, S. AU - Bull, C. CY - Southampton (UK) DB - PubMed DO - 10.3310/hsdr04190 LA - eng N1 - Brown, Katherine L Wray, Jo Knowles, Rachel L Crowe, Sonya Tregay, Jenifer Ridout, Deborah Barron, David J Cunningham, David Parslow, Roger Franklin, Rodney Barnes, Nick Hull, Sally Bull, Catherine Review Book NBK363028 [bookaccession] PB - NIHR Journals Library Copyright © Queen’s Printer and Controller of HMSO 2016. This work was produced by Brown et al. under the terms of a commissioning contract issued by the Secretary of State for Health. This issue may be freely reproduced for the purposes of private research and study and extracts (or indeed, the full report) may be included in professional journals provided that suitable acknowledgement is made and the reproduction is not associated with any form of advertising. Applications for commercial reproduction should be addressed to: NIHR Journals Library, National Institute for Health Research, Evaluation, Trials and Studies Coordinating Centre, Alpha House, University of Southampton Science Park, Southampton SO16 7NS, UK. PY - 2016 ST - Health Services and Delivery Research T2 - Infant deaths in the UK community following successful cardiac surgery: building the evidence base for optimal surveillance, a mixed-methods study TI - Health Services and Delivery Research ID - 531 ER - TY - JOUR AB - Aim To determine whether, in children with congenital heart disease (CHD), disease severity is associated with health-related quality of life (HRQOL) and impact on the family. Methods Cross-sectional, single-centre study comparing HRQOL outcomes of age and sex matched children with hypoplasia of the left ventricle (HLV) (n = 31) and tetralogy of Fallot (n = 29) was performed in Queensland, Australia. HRQOL was assessed using generic and disease-specific components of the Paediatric Quality of Life Inventory Measurement Model (PedsQL). Intra-diagnostic age group comparisons of HRQOL were examined. Impact of CHD on families and parental HRQOL was assessed using the PedsQL Family Impact Scale. Results Child and parent-proxy reporting indicate children with HLV have significantly lower overall HRQOL than children with tetralogy of Fallot across generic domains of HRQOL (P < 0.0001), with significantly lower scores in physical (P < 0.0001) and psychosocial (P < 0.0001) health domains. No significant difference in child reporting across domains of the Cardiac Module is evident. Parent-proxy reporting indicates significantly lower scores on the symptom scales for children with HLV (P < 0.001), with greater cognitive problems (P < 0.02) and perceived treatment anxiety (P < 0.01). No significant differences in HRQOL were identified between age groups. HLV has a greater overall family impact, with significantly lower parental HRQOL (P = 0.0001) and family functioning (P < 0.0001) summary scores. Conclusions The more severe condition of HLV is associated with poorer HRQOL in some domains and has greater impact on parental HRQOL and family functioning. © 2013 The Authors. Journal of Paediatrics and Child Health. AD - K.J. Eagleson, Mater Health Services, Raymond Terrace, South Brisbane, QLD 4101, Australia AU - Eagleson, K. J. AU - Justo, R. N. AU - Ware, R. S. AU - Johnson, S. G. AU - Boyle, F. M. DB - Embase Medline DO - 10.1111/jpc.12296 IS - 10 KW - adolescent adult article Australia child child parent relation disease severity Fallot tetralogy family functioning female human hypoplastic left heart syndrome major clinical study male parent patient participation PedsQL Family Impact Scale preschool child priority journal prognosis quality of life rating scale school child LA - English M3 - Article N1 - L52658853 2013-07-04 2013-10-30 PY - 2013 SN - 1440-1754 1034-4810 SP - 856-864 ST - Health-related quality of life and congenital heart disease in Australia T2 - Journal of Paediatrics and Child Health TI - Health-related quality of life and congenital heart disease in Australia UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52658853 http://dx.doi.org/10.1111/jpc.12296 VL - 49 ID - 1029 ER - TY - JOUR AB - Data from neurological and radiological research show an abnormal neurological development in patients treated for hypoplastic left heart syndrome. Thus, the aim of this study was to survey the quality of life scores in comparison with healthy children and children with other heart diseases (mild, moderate, and severe heart defects, heart defects in total). Children with hypoplastic left heart syndrome (aged 6.3-16.9 years) under compulsory education requirements, who were treated at the Children's Heart Center Linz between 1997 and 2009 (n = 74), were surveyed. Totally, 41 children and 44 parents were examined prospectively by psychologists according to Pediatric Quality of Life Inventory, a health-related quality of life measurement. The results of the self-assessments of health-related quality of life on a scale of 1-100 showed a wide range, from a minimum of 5.00 (social functioning) to a maximum of 100 (physical health-related summary scores, emotional functioning, school functioning), with a total score of 98.44. The parents' assessments (proxy) were quite similar, showing a range from 10 (social functioning) up to 100. Adolescent hypoplastic left heart syndrome patients rated themselves on the same level as healthy youths and youths with different heart diseases. The results show that patients with hypoplastic left heart syndrome aged 6-16 years can be successfully supported and assisted in their psychosocial development even if they show low varying physical and psychosocial parameters. The finding that adolescent hypoplastic left heart syndrome patients estimated themselves similar to healthy individuals suggests that they learnt to cope with a severe heart defect. AD - Children's Heart Center Linz, Kepler University Hospital, 4020Linz, Austria. Department of Inclusive Education, University of Education of Upper Austria, 4020Linz, Austria. AN - 32216849 AU - Oberhuber, R. D. AU - Huemer, S. AU - Mair, R. AU - Sames-Dolzer, E. AU - Kreuzer, M. AU - Tulzer, G. DA - Apr DB - PubMed DO - 10.1017/s1047951120000554 DP - NLM ET - 2020/03/29 IS - 4 KW - Adolescence children health-related quality of life hypoplastic left heart syndrome long-term follow-up LA - eng N1 - 1467-1107 Oberhuber, Raphael D Orcid: 0000-0001-5640-5128 Huemer, Sonja Mair, Rudolf Sames-Dolzer, Eva Kreuzer, Michaela Tulzer, Gerald Journal Article England Cardiol Young. 2020 Apr;30(4):539-548. doi: 10.1017/S1047951120000554. Epub 2020 Mar 27. PY - 2020 SN - 1047-9511 SP - 539-548 ST - Health-related quality of life for children and adolescents in school age with hypoplastic left heart syndrome: a single-centre study T2 - Cardiol Young TI - Health-related quality of life for children and adolescents in school age with hypoplastic left heart syndrome: a single-centre study VL - 30 ID - 344 ER - TY - JOUR AB - The primary objective of this study was to ascertain the long-term health-related quality of life (HRQOL) of adult patients who underwent a childhood Fontan operation for palliation of univentricular cardiac anomalies. The secondary objective was to compare the long-term HRQOL of Fontan survivors to that of pediatric heart transplant recipients. This cross-sectional study examined adult survivors (>19 years) who underwent a Fontan operation during childhood (Fontan group) or a pediatric heart transplant (HT group) between 1988 and 2011 (23-year span). HRQOL was assessed using the short form 36 survey. The study group consisted of 49 Fontan group patients and 13 HT group patients who responded to the survey. HRQOL scores of the Fontan group were similar to those of an age-controlled healthy US population in social and mental functioning, energy or vitality, and overall mental component score (P ≥ 0.2). However, Fontan scores in physical functioning, bodily pain, general health, and overall by physical component were significantly lower than those of the age-controlled US population (P < 0.05). No differences were identified between Fontan and HT patients. This favorable life-satisfaction period (average 18 years) should be considered when informing patients and families of expectations with the Fontan pathway vs certain higher-risk procedures. AD - Department of Surgery, University of Alabama at Birmingham, Birmingham, AL. Department of Surgery, University of Alabama at Birmingham, Birmingham, AL. Electronic address: jkirklin@uab.edu. AN - 26708372 AU - Kukreja, M. AU - Bryant, A. S. AU - Cleveland, D. C. AU - Dabal, R. AU - Hingorani, N. AU - Kirklin, J. K. DA - Autumn DB - PubMed DO - 10.1053/j.semtcvs.2015.08.007 DP - NLM ET - 2015/12/29 IS - 3 KW - Adolescent Adult Alabama/epidemiology Child Child, Preschool Cross-Sectional Studies Female Follow-Up Studies *Fontan Procedure *Health Status Heart Defects, Congenital/mortality/*surgery Humans Male Postoperative Period *Quality of Life Retrospective Studies Surveys and Questionnaires Survival Rate/trends Time Factors Young Adult Fontan procedures health-related quality of life single-ventricle conditions LA - eng N1 - 1532-9488 Kukreja, Manisha Bryant, Ayesha S Cleveland, David C Dabal, Robert Hingorani, Neha Kirklin, James K Comparative Study Journal Article United States Semin Thorac Cardiovasc Surg. 2015 Autumn;27(3):299-306. doi: 10.1053/j.semtcvs.2015.08.007. Epub 2015 Sep 9. PY - 2015 SN - 1043-0679 SP - 299-306 ST - Health-Related Quality of Life in Adult Survivors After the Fontan Operation T2 - Semin Thorac Cardiovasc Surg TI - Health-Related Quality of Life in Adult Survivors After the Fontan Operation VL - 27 ID - 74 ER - TY - JOUR AB - BACKGROUND: As survival of children with CHD needing surgery has improved significantly, the need for follow-up in terms of health-related quality of life has become increasingly important. In this study, we sought to describe health-related quality of life in children with CHD in relation to cardiac surgery. METHODS: A retrospective Swedish National Registry for Congenital Heart Disease survey measured using DISABKIDS chronic generic measure-short version included 337 children (age 9-17 years; 39% girls). The majority (n=319, 95%) of children had a biventricular heart, whereas the remaining had a univentricular heart. Cardiac surgery was performed in 197 (58%) children. Health-related quality of life was expressed as total score (100 highest) and given as medians and 10-90th percentiles. RESULTS: The overall total score was 95 (88-100). Children with a biventricular heart who had undergone three or more surgeries (n=31; 9%) had the lowest total score of 81 (61-97; p<0.001). Children with two or more surgeries and those with univentricular heart were classified in NYHA II more frequently than children with one or no cardiac surgery (p=0.005 and <0.001, respectively). Children with three or more surgeries and those with univentricular heart needed more help at school (p<0.001). Compared with children with other chronic diseases, children with CHD had a high total score except for children with three or more surgeries who had comparable total scores with children with other chronic diseases. CONCLUSION: Children with three or more cardiac surgeries and those with a univentricular heart appear to have lower health-related quality of life, cognitive ability, and NYHA classification. AD - 1Department of Cardiology,Pediatric Heart Center,Skåne University Hospital,Lund,Sweden. 2Department of Care and Science, Faculty of Health and Society,Malmö University,Malmö,Sweden. 3Research and Development Centre,Medical Statistics and Epidemiology,Skåne University Hospital,Lund,Sweden. AN - 27225489 AU - Svensson, B. AU - Idvall, E. AU - Nilsson, F. AU - Liuba, P. DA - Mar DB - PubMed DO - 10.1017/s1047951116000585 DP - NLM ET - 2016/05/27 IS - 2 KW - Adolescent *Cardiac Surgical Procedures Child Female *Health Status Heart Defects, Congenital/epidemiology/psychology/*surgery Humans Incidence Male *Quality of Life *Registries Retrospective Studies Risk Factors Surveys and Questionnaires Sweden/epidemiology Chd Health-related quality of life children registry study LA - eng N1 - 1467-1107 Svensson, Birgitta Idvall, Ewa Nilsson, Fredrik Liuba, Petru Journal Article England Cardiol Young. 2017 Mar;27(2):333-343. doi: 10.1017/S1047951116000585. Epub 2016 May 26. PY - 2017 SN - 1047-9511 SP - 333-343 ST - Health-related quality of life in children with surgery for CHD: a study from the Swedish National Registry for Congenital Heart Disease T2 - Cardiol Young TI - Health-related quality of life in children with surgery for CHD: a study from the Swedish National Registry for Congenital Heart Disease VL - 27 ID - 85 ER - TY - JOUR AB - Background People with a Fontan circulation experience a range of physical, psychosocial and neurodevelopmental challenges alongside, or caused by, their cardiac condition, with significant consequences for health-related quality of life (HRQOL). We meta-analyzed HRQOL outcomes reported by people with a Fontan circulation or their proxies and evaluated predictors of poorer HRQOL. Methods and Results Six electronic databases were searched for peer-reviewed, English-language articles published before March 2019. Standardized mean differences (SMD) were calculated using fixed and random-effects models. Fifty articles reporting on 29 unique studies capturing HRQOL outcomes for 2793 people with a Fontan circulation and 1437 parent-proxies were analyzed. HRQOL was lower in individuals with a Fontan circulation compared with healthy referents or normative samples (SMD, -0.92; 95% CI, -1.36 to -0.48; P<0.001). Lower scores were reported across all HRQOL domains, with the largest differences found for physical (SMD, -0.90; 95% CI, -1.13 to -0.67; P<0.001) and school/work functioning (SMD, -0.71; 95% CI, -0.90 to -0.52; P<0.001). Meta-regression analyses found no significant predictors of self-reported physical functioning, but older age at Fontan operation was associated with poorer emotional functioning (β=-0.124; P=0.004), and diagnosis of hypoplastic left heart was associated with poorer social functioning (β=-0.007; P=0.048). Sensitivity analyses showed use of the PedsQL Core Module was associated with lower HRQOL scores compared with the Short-Form Health Survey-36. Conclusions HRQOL outcomes for people with a Fontan circulation are lower than the general population. Optimal care acknowledges the lifelong impact of the Fontan circulation on HRQOL and offers targeted strategies to improve outcomes for this growing population. AD - Heart Centre for Children The Children's Hospital at Westmead Sydney New South Wales Australia. Discipline of Paediatrics School of Women's and Children's Health University of New South Wales Medicine The University of New South Wales Sydney New South Wales Australia. Heart Research Group Murdoch Children's Research Institute Melbourne Victoria Australia. Department of Cardiac Surgery The Royal Children's Hospital Melbourne Victoria Australia. Sydney Medical School The University of Sydney New South Wales Australia. Department of Pediatrics University of Cincinnati College of Medicine Cincinnati Ohio United States. Cincinnati Adult Congenital Heart Disease Program Heart Institute Cincinnati Children's Hospital Cincinnati Ohio United States. Department of Cardiology Boston Children's Hospital Boston Massachusetts United States. Harvard Medical School Boston Massachusetts United States. Pain Management Research Institute Royal North Shore Hospital Sydney New South Wales Australia. School of Psychology The University of Sydney New South Wales Australia. Department of Cardiology Royal Prince Alfred Hospital Sydney New South Wales Australia. Cincinnati Children's Center for Heart Disease and the Developing Mind Heart Institute and Division of Behavioral Medicine & Clinical Psychology Cincinnati Children's Hospital Cincinnati Ohio United States. AN - 32172648 AU - Marshall, K. H. AU - D'Udekem, Y. AU - Sholler, G. F. AU - Opotowsky, A. R. AU - Costa, D. S. J. AU - Sharpe, L. AU - Celermajer, D. S. AU - Winlaw, D. S. AU - Newburger, J. W. AU - Kasparian, N. A. DA - Mar 17 DB - PubMed DO - 10.1161/jaha.119.014172 DP - NLM ET - 2020/03/17 IS - 6 KW - Fontan circulation chronic illness congenital heart disease health‐related quality of life mental health psychological stress LA - eng N1 - 2047-9980 Marshall, Kate H D'Udekem, Yves Sholler, Gary F Opotowsky, Alexander R Costa, Daniel S J Sharpe, Louise Celermajer, David S Winlaw, David S Newburger, Jane W Kasparian, Nadine A Journal Article England J Am Heart Assoc. 2020 Mar 17;9(6):e014172. doi: 10.1161/JAHA.119.014172. Epub 2020 Mar 16. PY - 2020 SN - 2047-9980 SP - e014172 ST - Health-Related Quality of Life in Children, Adolescents, and Adults With a Fontan Circulation: A Meta-Analysis T2 - J Am Heart Assoc TI - Health-Related Quality of Life in Children, Adolescents, and Adults With a Fontan Circulation: A Meta-Analysis VL - 9 ID - 93 ER - TY - JOUR AB - The aim of the study was to investigate the impact of a number of surgical interventions for a various congenital cardiac defects (CHDs) on self-reported HRQoL.Methods: Patients who had received corrective surgery of several congenital heart defects (surgical VSD closure, Fallot, TGA after atrial or arterial switch or Fontan-type circulation for univentricular AV-connection) were interviewed in the office of their home peadiatric cardiologist. HRQoL in children along 7 dimensions was assessed using a standardised questionnaire (PEDQoL); information on the medical case history of each respondent was also collected.Statistics: HRQoL was assessed in the questionnaire by asking about the frequency (never, rarely, often, always) of specific negative experiences; more frequent experiences indicate a lower quality of life. Frequency expressions were transformed into numerical values (25, 50, 75, 100%), and mean values for HRQoL were calculated for each patient and for each domain. Differences in HRQoL among patients with different types of interventions were analysed using the Mann-Whitney Test or the Kruskal-Wallis Test as appropriate; p values <0.05 were considered to indicate significant differences, while p values <0.1 were considered to indicate notable trends.Results: Patients: 169 patients (60% male, 40% female) were part of the study. The mean age was 11.6 years; 50 patients had surgical VSD closure, 52 surgeries for Tetralogy of Fallot (22 transannular patch, 18 no transannular patch, 12 inaccurate description), 40 had complete transposition of the great arteries (28 atrial switch, 12 arterial switch), 22 had a Fontan-type procedure for univentricular AV-connection. HRQoL differed little among patients with different CHDs for the items "relation to friends," "interactions in the affected families", and "own body image". For other items, notable differences emerged: patients with univentricular hearts rated their physical capacity worse and showed a tendency towards negative ratings in other domains. On the other hand, patients after Fallot or TGA correction tended to rate their HRQoL in several domains as relatively high. Focusing on the mode of surgery for Fallot repair, respectively, TGA correction the only significant difference was found for "physical capacity" in TGA (atrial vs. arterial repair). Mustard patients tended to rate most items worse. Physical capacity was rated worst by patients with a Fontan circulation. Repeated surgery led to lower ratings for all domains except "physical capacity" and "body image".Conclusions: Different surgical techniques for CHD do not affect children's and adolescents' self-reported HRQoL to the extent that one would expect. This observation is in line with observations in groups of children with different chronic diseases. Specialised psychosocial support is necessary in order to maintain this positive self-evaluation and to ensure patients are able to lead autonomous personal and professional lives. AD - Wuppertal Germany Düsseldorf Germany Bonn Germany , Düsseldorf, Germany , Wuppertal, Germany AN - 125840214. Language: English. Entry Date: 20180720. Revision Date: 20190515. Publication Type: journal article AU - Heusch, A. AU - Kahl, H. AU - Hensel, K. AU - Calaminus, G. AU - Kahl, H. J. AU - Hensel, K. O. DB - ccm DO - 10.1007/s11136-017-1653-y DP - EBSCOhost IS - 11 KW - Heart Defects, Congenital -- Surgery Heart Defects, Congenital -- Psychosocial Factors Female Heart Defects, Congenital -- Pathology Child Male Questionnaires Human N1 - research. Journal Subset: Allied Health; Public Health; USA. Instrumentation: Pediatric Quality of Life Inventory (PEDSQL). NLM UID: 9210257. PMID: NLM28730300. PY - 2017 SN - 0962-9343 SP - 3111-3117 ST - Health-related quality of life in paediatric patients with congenital heart defects: association with the type of heart defect and the surgical technique T2 - Quality of Life Research TI - Health-related quality of life in paediatric patients with congenital heart defects: association with the type of heart defect and the surgical technique UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=125840214&site=ehost-live&scope=site VL - 26 ID - 1438 ER - TY - JOUR AB - The aim of the study was to investigate the impact of a number of surgical interventions for a various congenital cardiac defects (CHDs) on self-reported HRQoL. METHODS: Patients who had received corrective surgery of several congenital heart defects (surgical VSD closure, Fallot, TGA after atrial or arterial switch or Fontan-type circulation for univentricular AV-connection) were interviewed in the office of their home peadiatric cardiologist. HRQoL in children along 7 dimensions was assessed using a standardised questionnaire (PEDQoL); information on the medical case history of each respondent was also collected. STATISTICS: HRQoL was assessed in the questionnaire by asking about the frequency (never, rarely, often, always) of specific negative experiences; more frequent experiences indicate a lower quality of life. Frequency expressions were transformed into numerical values (25, 50, 75, 100%), and mean values for HRQoL were calculated for each patient and for each domain. Differences in HRQoL among patients with different types of interventions were analysed using the Mann-Whitney Test or the Kruskal-Wallis Test as appropriate; p values <0.05 were considered to indicate significant differences, while p values <0.1 were considered to indicate notable trends. RESULTS: Patients: 169 patients (60% male, 40% female) were part of the study. The mean age was 11.6 years; 50 patients had surgical VSD closure, 52 surgeries for Tetralogy of Fallot (22 transannular patch, 18 no transannular patch, 12 inaccurate description), 40 had complete transposition of the great arteries (28 atrial switch, 12 arterial switch), 22 had a Fontan-type procedure for univentricular AV-connection. HRQoL differed little among patients with different CHDs for the items "relation to friends," "interactions in the affected families", and "own body image". For other items, notable differences emerged: patients with univentricular hearts rated their physical capacity worse and showed a tendency towards negative ratings in other domains. On the other hand, patients after Fallot or TGA correction tended to rate their HRQoL in several domains as relatively high. Focusing on the mode of surgery for Fallot repair, respectively, TGA correction the only significant difference was found for "physical capacity" in TGA (atrial vs. arterial repair). Mustard patients tended to rate most items worse. Physical capacity was rated worst by patients with a Fontan circulation. Repeated surgery led to lower ratings for all domains except "physical capacity" and "body image". CONCLUSIONS: Different surgical techniques for CHD do not affect children's and adolescents' self-reported HRQoL to the extent that one would expect. This observation is in line with observations in groups of children with different chronic diseases. Specialised psychosocial support is necessary in order to maintain this positive self-evaluation and to ensure patients are able to lead autonomous personal and professional lives. AD -, Wuppertal, Germany. andreas.heusch@helios-kliniken.de. , Düsseldorf, Germany. , Wuppertal, Germany. , Bonn, Germany. AN - 28730300 AU - Heusch, A. AU - Kahl, H. J. AU - Hensel, K. O. AU - Calaminus, G. DA - Nov DB - PubMed DO - 10.1007/s11136-017-1653-y DP - NLM ET - 2017/07/22 IS - 11 KW - Child Female Heart Defects, Congenital/pathology/*psychology/*surgery Humans Male Sickness Impact Profile Surveys and Questionnaires *Congenital heart defect *Heart surgery *Quality of life LA - eng N1 - 1573-2649 Heusch, A Orcid: 0000-0003-2805-1670 Kahl, H J Hensel, K O Calaminus, G Journal Article Netherlands Qual Life Res. 2017 Nov;26(11):3111-3117. doi: 10.1007/s11136-017-1653-y. Epub 2017 Jul 20. PY - 2017 SN - 0962-9343 SP - 3111-3117 ST - Health-related quality of life in paediatric patients with congenital heart defects: association with the type of heart defect and the surgical technique T2 - Qual Life Res TI - Health-related quality of life in paediatric patients with congenital heart defects: association with the type of heart defect and the surgical technique VL - 26 ID - 141 ER - TY - JOUR AB - BACKGROUND: Little is known about health-related quality of life in young children undergoing staged palliation for single-ventricle CHD. The aim of this study was to assess the impact of CHD on daily life in pre-schoolers with single-ventricle CHD and to identify determinants of health-related quality of life. METHOD: Prospective two-centre cohort study assessing health-related quality of life using the Preschool Paediatric Cardiac Quality of Life Inventory in 46 children at a mean age of 38 months and 3 weeks. Children with genetic anomalies were excluded. Scores were compared with reference data of children with biventricular CHD. Multiple linear regression analysis was used to identify determinants of health-related quality of life. RESULTS: Health-related quality of life in pre-schoolers with single-ventricle CHD was comparable to children with biventricular CHD. Preterm birth and perioperative variables were significant predictors of low health-related quality of life. Notably, pre-Fontan brain MRI findings and neurodevelopmental status were not associated with health-related quality of life. Overall, perioperative variables explained 24% of the variability of the total health-related quality of life score.InterpretationDespite substantial health-related burden, pre-schoolers with single-ventricle CHD showed good health-related quality of life. Less-modifiable treatment-related risk factors and preterm birth had the highest impact on health-related quality of life. Long-term follow-up assessment of self-reported health-related quality of life is needed to identify patients with poorer health-related quality of life and to initiate supportive care. AD - Child Research Centre, University Children's Hospital, Zurich, Switzerland. Child Development Centre, University Children's Hospital, Zurich, Switzerland. Paediatric Cardiology, Paediatric Heart Centre, University Children's Hospital, Zurich, Switzerland. Diagnostic Imaging and MR-research Centre, University Children's Hospital, Zurich, Switzerland. Child Development Centre, SPZ Frankfurt Mitte, Frankfurt/Main, Germany. Paediatric Neurology, University Hospital Giessen, Germany. Paediatric Heart Centre, University Hospital Giessen, Justus-Liebig-University, Giessen, Germany. Department of Psychosomatics and Psychiatry, University Children's Hospital Zurich, Zurich, Switzerland. Division of Child and Adolescent Health Psychology, Department of Psychology, University of Zurich, Zurich, Switzerland. AN - 30599815 AU - Heye, K. N. AU - Knirsch, W. AU - Scheer, I. AU - Beck, I. AU - Wetterling, K. AU - Hahn, A. AU - Hofmann, K. AU - Latal, B. AU - Reich, B. AU - Landolt, M. A. DA - Feb DB - PubMed DO - 10.1017/s1047951118001993 DP - NLM ET - 2019/01/03 IS - 2 KW - Child, Preschool Female Follow-Up Studies *Health Status Heart Defects, Congenital/*psychology Heart Ventricles/*abnormalities Humans Infant Male Prospective Studies *Quality of Life Risk Factors Surveys and Questionnaires Chd Health-related quality of life single-ventricle LA - eng N1 - 1467-1107 Heye, Kristina N Orcid: 0000-0003-3578-9200 Knirsch, Walter Scheer, Ianina Beck, Ingrid Wetterling, Kristina Hahn, Andreas Hofmann, Karoline Latal, Beatrice Reich, Bettina Landolt, Markus A Journal Article England Cardiol Young. 2019 Feb;29(2):162-168. doi: 10.1017/S1047951118001993. Epub 2019 Jan 2. PY - 2019 SN - 1047-9511 SP - 162-168 ST - Health-related quality of life in pre-school age children with single-ventricle CHD T2 - Cardiol Young TI - Health-related quality of life in pre-school age children with single-ventricle CHD VL - 29 ID - 40 ER - TY - JOUR AB - Background: Little is known about health-related quality of life in young children undergoing staged palliation for single-ventricle CHD. The aim of this study was to assess the impact of CHD on daily life in pre-schoolers with single-ventricle CHD and to identify determinants of health-related quality of life.Method: Prospective two-centre cohort study assessing health-related quality of life using the Preschool Paediatric Cardiac Quality of Life Inventory in 46 children at a mean age of 38 months and 3 weeks. Children with genetic anomalies were excluded. Scores were compared with reference data of children with biventricular CHD. Multiple linear regression analysis was used to identify determinants of health-related quality of life.Results: Health-related quality of life in pre-schoolers with single-ventricle CHD was comparable to children with biventricular CHD. Preterm birth and perioperative variables were significant predictors of low health-related quality of life. Notably, pre-Fontan brain MRI findings and neurodevelopmental status were not associated with health-related quality of life. Overall, perioperative variables explained 24% of the variability of the total health-related quality of life score.InterpretationDespite substantial health-related burden, pre-schoolers with single-ventricle CHD showed good health-related quality of life. Less-modifiable treatment-related risk factors and preterm birth had the highest impact on health-related quality of life. Long-term follow-up assessment of self-reported health-related quality of life is needed to identify patients with poorer health-related quality of life and to initiate supportive care. AD - Child Research Centre, University Children's Hospital, Zurich, Switzerland Child Development Centre, University Children's Hospital, Zurich, Switzerland Paediatric Cardiology, Paediatric Heart Centre, University Children's Hospital, Zurich, Switzerland Diagnostic Imaging and MR-research Centre, University Children's Hospital, Zurich, Switzerland Department of Psychosomatics and Psychiatry, University Children's Hospital Zurich, Zurich, Switzerland Division of Child and Adolescent Health Psychology, Department of Psychology, University of Zurich, Zurich, Switzerland Department of Cell Biology, New York University Medical Center, New York, New York, U.S.A AN - 134823712. Language: English. Entry Date: 20190531. Revision Date: 20200203. Publication Type: journal article AU - Latal, Beatrice AU - Heye, Kristina N. AU - Knirsch, Walter AU - Scheer, Ianina AU - Landolt, Markus A. AU - Beck, Ingrid AU - Wetterling, Kristina AU - Hahn, Andreas AU - Hofmann, Karoline AU - Reich, Bettina DB - ccm DO - 10.1017/S1047951118001993 DP - EBSCOhost IS - 2 KW - Heart Ventricle -- Abnormalities Heart Defects, Congenital -- Psychosocial Factors Health Status Quality of Life Female Infant Prospective Studies Male Risk Factors Child, Preschool Scales Human N1 - research; tables/charts. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. NLM UID: 9200019. PMID: NLM30599815. PY - 2019 SN - 1047-9511 SP - 162-168 ST - Health-related quality of life in pre-school age children with single-ventricle CHD T2 - Cardiology in the Young TI - Health-related quality of life in pre-school age children with single-ventricle CHD UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=134823712&site=ehost-live&scope=site VL - 29 ID - 1440 ER - TY - JOUR AB - OBJECTIVES: To compare health-related quality of life (HRQOL) in a group of pediatric patients with congenital heart disease (CHD) and healthy controls and patients with other chronic diseases, and to compare HRQOL among patients with CHD of various severity categories with one another, with controls, and with patients with other chronic diseases. STUDY DESIGN: In this cross-sectional survey, t tests were used to compare patient and proxy-reported Pediatric Quality of Life Inventory 4.0 Generic Core Scales (PedsQL) scores (including total, physical health, and psychosocial health summary scores) in children (aged 8-12 years) and adolescents (aged 13-18 years) between controls and (1) a composite CHD population; and (2) patients in each of 3 CHD severity categories: mild (no intervention), biventricle (BV; postintervention), and single ventricle (SV; postpalliation). PedsQL scores among CHD severity categories were compared by ANOVA. PedsQL scores were also compared in the CHD population and children with other chronic diseases without age stratification using t tests. RESULTS: There were 1138 (children, n = 625; adolescents, n = 513) and 771 (children, n = 528; adolescents, n = 243) patient and/or proxy reporters in the CHD and healthy control groups, respectively. Total, physical health, and psychosocial health summary scores were lower in the composite CHD, BV, and SV groups compared with controls (P < .0001). There were significant differences among disease severity categories for all scores (P < .01). The composite CHD, BV, and SV groups had similar PedsQL scores as end-stage renal disease, asthma, and obesity populations. CONCLUSION: Children and adolescents with BV and SV CHD have significantly lower HRQOL than healthy controls and similar HRQOL as patients with other chronic pediatric diseases. Interventions targeting both physical and psychosocial domains are needed to improve HRQOL in this high-risk population. AD - Division of Cardiology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH. Electronic address: katelyn.mellion@rockets.utoledo.edu. Division of Cardiology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH. Department of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH. Division of Behavioral and Clinical Psychology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH. Division of Cardiology, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA. Division of Cardiology, Department of Pediatrics, Children's Hospital Boston, Boston, MA. Division of Cardiology, Department of Pediatrics, University of Texas Southwestern Medical Center Dallas, Dallas, TX. Division of Cardiology, Department of Pediatrics, Children's Hospital of Wisconsin, Milwaukee, WI. Department of Cardiology, Phoenix Children's Hospital, Phoenix, AZ. Department of Psychology and Neuroscience, Baylor University, Waco, TX. Division of Cardiology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH; Division of Critical Care Medicine, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH. AN - 24412135 AU - Mellion, K. AU - Uzark, K. AU - Cassedy, A. AU - Drotar, D. AU - Wernovsky, G. AU - Newburger, J. W. AU - Mahony, L. AU - Mussatto, K. AU - Cohen, M. AU - Limbers, C. AU - Marino, B. S. DA - Apr DB - PubMed DO - 10.1016/j.jpeds.2013.11.066 DP - NLM ET - 2014/01/15 IS - 4 KW - Adolescent Child Chronic Disease Cross-Sectional Studies Female *Heart Defects, Congenital/diagnosis Heart Diseases/*congenital/diagnosis Humans Male *Quality of Life Retrospective Studies LA - eng N1 - 1097-6833 Mellion, Katelyn Uzark, Karen Cassedy, Amy Drotar, Dennis Wernovsky, Gil Newburger, Jane W Mahony, Lynn Mussatto, Kathy Cohen, Mitchell Limbers, Christine Marino, Bradley S Pediatric Cardiac Quality of Life Inventory Testing Study Consortium K23 HD048637/HD/NICHD NIH HHS/United States 5-K23-HD048637/HD/NICHD NIH HHS/United States Comparative Study Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't United States J Pediatr. 2014 Apr;164(4):781-788.e1. doi: 10.1016/j.jpeds.2013.11.066. Epub 2014 Jan 10. PY - 2014 SN - 0022-3476 SP - 781-788.e1 ST - Health-related quality of life outcomes in children and adolescents with congenital heart disease T2 - J Pediatr TI - Health-related quality of life outcomes in children and adolescents with congenital heart disease VL - 164 ID - 122 ER - TY - JOUR AB - The advancements in surgical technique and perioperative care have significantly improved the survival of children with single ventricle (SV) congenital heart disease (CHD) over the past decade. The population who have undergone the Fontan operation are growing into adulthood and facing many unique challenges. Past research has focused on functional and neurodevelopmental outcomes with inferences made to health-related quality of life (HRQOL). With the population who have undergone the Fontan operation surviving into adulthood, little research has been directed toward the self-report of HRQOL in adolescents and young adults after surgical palliation. Questions still remain on how these patients will transition into adulthood and whether they will live normal productive lives. This article reviews the literature related to HRQOL in the SV subgroup of CHD. In addition, an overview of newly developed disease-specific HRQOL instruments is presented as well as limitations and future research in HRQOL of the SV Fontan population. AD - Division of Cardiothoracic Surgery, Childrens Hospital Los Angeles, CA 90027, USA. AN - 17234472 AU - Pike, N. A. AU - Evangelista, L. S. AU - Doering, L. V. AU - Koniak-Griffin, D. AU - Lewis, A. B. AU - Child, J. S. DA - Jan-Feb DB - PubMed DO - 10.1016/j.hrtlng.2006.06.002 DP - NLM ET - 2007/01/20 IS - 1 KW - *Biomedical Research Fontan Procedure/*psychology Heart Defects, Congenital/psychology/*surgery Humans *Quality of Life LA - eng N1 - Pike, Nancy A Evangelista, Lorraine S Doering, Lynn V Koniak-Griffin, Deborah Lewis, Alan B Child, John S Journal Article Review United States Heart Lung. 2007 Jan-Feb;36(1):3-15. doi: 10.1016/j.hrtlng.2006.06.002. PY - 2007 SN - 0147-9563 (Print) 0147-9563 SP - 3-15 ST - Health-related quality of life: a closer look at related research in patients who have undergone the Fontan operation over the last decade T2 - Heart Lung TI - Health-related quality of life: a closer look at related research in patients who have undergone the Fontan operation over the last decade VL - 36 ID - 233 ER - TY - JOUR AB - Background: As the population of patients with a Fontan palliation grows so does, the number of patients with cardiac failure necessitating orthotopic heart transplant (OHT) and combined heart–liver transplant (CHLT). There is recent evidence that current era cardiac transplant in Fontan patients has improved outcomes, but most studies have a preponderance of pediatrics patients in their cohorts. We examine our institutional experience with adult OHT and CHLT transplantation for failed Fontan physiology. Methods and Results: Retrospective analysis of patients at the Ahmanson/UCLA Adult Congenital Heart Disease Center who underwent OHT or CHLT for failing Fontan physiology from January 1, 2002 to May 31, 2017. We identified 20 patients with single-ventricle physiology and Fontan palliation who underwent OHT or CHLT. The median age was 29.5 years (range 19-44). Five patients underwent CHLT because of biopsy proven hepatic cirrhosis. The median length of hospital stay was 23 days (range 8-76) post-OHT and 51 days (range 26-77) post-CHLT. During a median follow-up of 56 months (range 2-178), there was one mortality occurring at 34 months post-OHT due to coronary vasculopathy. Most frequent early postoperative complications included bleeding and infection (55% and 20%, respectively) and surgical reintervention for bleeding complications (n = 8, 40%). One CHLT patient experienced clinically significant hepatic rejection requiring admission and steroid treatment. Conclusions: Despite inherent risks and complexities of OHT or CHLT in patients with a failed Fontan, transplant is a reasonable therapy. Peri- and postoperative complications are common and may require surgical reintervention. Continued observation of practices and unifying themes may help improve patient selection, pre- and postoperative treatment and ultimately outcomes. © 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd AD - Ahmanson/UCLA Adult Congenital Heart Disease Center, Los Angeles, CA, United States Division of Pediatric Cardiology, UCLA Children's Heart Center, Los Angeles, CA, United States Ahmanson/UCLA Cardiomyopathy Center, Los Angeles, CA, United States UCLA Department of Surgery, Cardiothoracic Surgery, Los Angeles, CA, United States UCLA Department of Surgery, Pfleger Liver Institute, Los Angeles, CA, United States UCLA Department of Medicine, Pfleger Liver Institute, Los Angeles, United States UCLA Department of Pediatric Gastroenterology, Los Angeles, United States David Geffen School of Medicine, University of California, Los Angeles, CA, United States AU - Reardon, L. C. AU - DePasquale, E. C. AU - Tarabay, J. AU - Cruz, D. AU - Laks, H. AU - Biniwale, R. M. AU - Busuttil, R. W. AU - Kaldas, F. M. AU - Saab, S. AU - Venick, R. S. AU - Lin, J. P. AU - Nsair, A. AU - Deng, M. C. AU - Ardehali, A. AU - Caderias, M. AU - Iygengar, A. AU - Aboulhosn, J. A. C7 - e13329 DB - Scopus DO - 10.1111/ctr.13329 IS - 8 KW - fontan procedure liver outcomes research transplantation M3 - Article N1 - Cited By :8 Export Date: 15 June 2020 PY - 2018 ST - Heart and heart–liver transplantation in adults with failing Fontan physiology T2 - Clinical Transplantation TI - Heart and heart–liver transplantation in adults with failing Fontan physiology UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85050451585&doi=10.1111%2fctr.13329&partnerID=40&md5=8f328a2c68902e67eea8ca7db5e2dc52 VL - 32 ID - 1792 ER - TY - JOUR AB - Case 1: a 5-yr-old boy. At 9 mth. permanent cyanosis set in; on cardiovascular examination physical signs, roentgenographic, electrovectorcardiographic and catheterization findings substantiated the diagnosis of tetralogy of Fallot. The boy died soon after an attempt to repair the intracardiac defects. The diagnosis of Fallot's tetralogy was verified by necropsy; there was also some thickening of the wall of the left ventricle. Case 2: an 18-mth-old boy; he showed only slight systemic hypertension on catheterization and a midsystolic murmur maximal at the 2nd left intercostal space. Electrovectorcardiography suggested left ventricular hypertrophy The child is still alive. Case 3: a 36-hr-old boy with a complicated heart disease; cyanosis developed at 3 weeks on crying and exertion. The boy died at the age of 3 mth. At necropsy complete transposition of the great vessels, a single (left) ventricle n diminutive subaortic chamber, atrial and ventricular eeptal defects, anomalous drainage of the i. v. c. into the azygous system and of pulmonary veins into the right atrium, absent gallbladder, extrahepatic biliary atresia as well as other abnormalities were found. Not one among these 3 cases had retinitis pigmentosa. A review of the literature is given. Up to now, there have been published only 6 documented cases of cardiac malformation among 330 patients with the syndrome. Acquired cardiovascular disease was connected with renal disease in 6 cases. There are still other, less well-documented, congenital or acquired cardiac lesions reported in the literature. There are also reports on systemic hypertension without renal disease, as in case 2 described above. It is supposed that there were more cases of heart disease among the published patients with the LMBB syndrome but that they had not been diagnosed. Cases with cardiovascular disease have apparently no peculiar clinical feature separating them from other patients with the syndrome without heart lesions. 43 references. AD - T.G. McLoughlin, Dept. of Ped, Coll. of Med., Univ. of Florida, Gainesville, FL, United States AU - McLoughlin, T. G. AU - Krovetz, L. J. AU - Schiebler, G. L. DB - Embase Classic Medline DO - 10.1016/S0022-3476(64)80403-0 IS - 3 KW - autopsy Bardet Biedl syndrome bile duct atresia boy cardiovascular disease catheterization child clinical feature congenital malformation crying cyanosis diagnosis examination exercise Fallot tetralogy gallbladder great blood vessel heart heart disease heart injury heart left ventricle heart left ventricle hypertrophy heart right atrium heart single ventricle hypertension intracardiac drug administration kidney disease patient pulmonary vein retinitis pigmentosa LA - English M3 - Article N1 - L281313658 1964-12-01 PY - 1964 SN - 0022-3476 SP - 388-399 ST - Heart disease in the laurence-moon-biedl-bardet syndrome. a review and a report of 3 brothers T2 - Journal of Pediatrics TI - Heart disease in the laurence-moon-biedl-bardet syndrome. a review and a report of 3 brothers UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L281313658 http://dx.doi.org/10.1016/S0022-3476(64)80403-0 VL - 65 ID - 1417 ER - TY - JOUR AB - There are significant challenges involved in the perinatal and postnatal care of an infant with hypoplastic left heart syndrome (HLHS) and the infant's family. In the blink of an eye, the perfect child is lost, and a fragile infant is about to join the family. This case study and discussion is an overview of HLHS, a family's desire to make the birth of their infant normal, and how that desire initiated a change in philosophy and practice in our neonatal intensive care unit. Keywords: hypoplastic left heart syndrome, HLHS, family centered care, parent-infant bonding, breastfeeding. © 2012 Springer Publishing Company. AD - M.E. Parker, Neonatal Intensive Care Nursery, Duke University Medical Center, 2301 Erwin Road, Box 100505, Durham, NC 27710, United States AU - Parker, M. E. AU - Bradshaw, W. T. AU - Smith, H. R. DB - Medline DO - 10.1891/0730-0832.31.5.305 IS - 5 KW - adult article case report female human hypoplastic left heart syndrome methodology newborn nursing nursing object relation palliative therapy pregnancy prenatal diagnosis LA - English M3 - Article N1 - L366236877 2013-02-07 PY - 2012 SN - 0730-0832 1539-2880 SP - 305-309 ST - Heart for bonding: A new protocol of care for hypoplastic left heart syndrome T2 - Neonatal Network TI - Heart for bonding: A new protocol of care for hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L366236877 http://dx.doi.org/10.1891/0730-0832.31.5.305 VL - 31 ID - 1043 ER - TY - JOUR AB - PURPOSE: To compare early changes in autonomic nervous system (ANS) tone between newborns with complex congenital heart disease (CHD) and newborns without CHD. METHODS: We performed a case-control study of heart rate variability (HRV) in newborns with complex CHD [transposition of the great arteries (TGA) or hypoplastic left heart syndrome (HLHS)] and low-risk control newborns without CHD. Cases with CHD were admitted following birth to a pediatric cardiac intensive care unit and had archived continuous ECG data. Control infants were prospectively enrolled at birth. ECG data in cases and controls were analyzed for HRV in the time and frequency domains at 24 h of age. We analyzed the following HRV metrics: alpha short (α(s)), alpha long (α(L)), root mean square short and long (RMS(s) and RMS(L)), low-frequency (LF) power, normalized LF (nLF), high-frequency (HF) power, and normalized HF (nHF). We used ANOVA to compare HRV metrics between groups and to control for medication exposures. RESULTS: HRV data from 57 infants with CHD (TGA, n = 33 and HLHS, n = 24) and from 29 controls were analyzed. The HRV metrics α(S), RMS(L), LF, and nLF were significantly lower in infants with CHD than in the controls. Due to the effect of normalization, nHF was higher in CHD infants (P < 0.0001), although absolute HF was lower (P = 0.0461). After adjusting for medications, α(S) and nLF remained lower and nHF higher in newborns with CHD (P  < 0.0005). CONCLUSIONS: Infants with complex CHD have depressed autonomic balance in the early postnatal period, which may complicate the fetal-neonatal transition. AD - Division of Fetal and Transitional Medicine, Children's National Health System, Washington, DC, USA. sbmulkey@childrensnational.org. Department of Pediatrics, The George Washington University School of Medicine and Health Sciences, Washington, DC, USA. sbmulkey@childrensnational.org. Department of Neurology, The George Washington University School of Medicine and Health Sciences, Washington, DC, USA. sbmulkey@childrensnational.org. Division of Fetal and Transitional Medicine, Children's National Health System, Washington, DC, USA. Biostatistics and Study Methodology, The George Washington University School of Medicine and Health Sciences, Washington, DC, USA. Department of Pediatrics, Inova Children's Hospital, Fairfax, VA, USA. Fairfax Neonatal Associates, Fairfax, VA, USA. Department of Obstetrics and Gynecology, Inova Fairfax Hospital, Fairfax, VA, USA. Department of Cardiology, Children's National Health System, Washington, DC, USA. Department of Pediatrics, The George Washington University School of Medicine and Health Sciences, Washington, DC, USA. Department of Neurology, The George Washington University School of Medicine and Health Sciences, Washington, DC, USA. AN - 31240423 AU - Mulkey, S. B. AU - Govindan, R. AU - Metzler, M. AU - Swisher, C. B. AU - Hitchings, L. AU - Wang, Y. AU - Baker, R. AU - Larry Maxwell, G. AU - Krishnan, A. AU - du Plessis, A. J. C2 - PMC6930356 C6 - NIHMS1532821 DA - Apr DB - PubMed DO - 10.1007/s10286-019-00616-w DP - NLM ET - 2019/06/27 IS - 2 KW - Autonomic nervous system Congenital heart disease Heart rate variability Newborn LA - eng N1 - 1619-1560 Mulkey, Sarah B Orcid: 0000-0002-8084-526x Govindan, Rathinaswamy Metzler, Marina Swisher, Christopher B Hitchings, Laura Wang, Yunfei Baker, Robin Larry Maxwell, G Krishnan, Anita du Plessis, Adre J UL1TR001876/TR/NCATS NIH HHS/United States KL2TR001877/TR/NCATS NIH HHS/United States KL2 TR001877/TR/NCATS NIH HHS/United States UL1 TR001876/TR/NCATS NIH HHS/United States Journal Article Clin Auton Res. 2020 Apr;30(2):165-172. doi: 10.1007/s10286-019-00616-w. Epub 2019 Jun 25. PY - 2020 SN - 0959-9851 (Print) 0959-9851 SP - 165-172 ST - Heart rate variability is depressed in the early transitional period for newborns with complex congenital heart disease T2 - Clin Auton Res TI - Heart rate variability is depressed in the early transitional period for newborns with complex congenital heart disease VL - 30 ID - 394 ER - TY - JOUR AB - Background Cardiac transplantation in adult patients with congenital heart disease poses numerous challenges. The optimal operative and postoperative management strategies remain unclear. The purpose of our study was to (1) characterize the adult patient with a congenital heart condition undergoing transplantation, the operation, and the postoperative course; (2) report the survival after heart transplantation at our center; and (3) discuss issues surrounding the unique setting we provide for the operative and postoperative care of this complex patient cohort. Methods We performed a retrospective cohort study of 12 consecutive adult patients with a prior history of congenital heart disease who underwent heart transplantation at a single, large, academic center between September 1, 2005, and September 1, 2013. The operations were performed by a surgeon specializing in congenital heart disease in an adult hospital. Postoperative care was provided jointly by that surgeon and the adult cardiac transplantation team. Results At operation, the median age and weight were 41 years (range, 16 to 72 years) and 65 kg (range, 45 to 104 kg), and 100% of patients had undergone previous operations. The median donor ischemic time was 197 minutes (range, 137 to 282 minutes). The median cardiopulmonary bypass time was 210 minutes (range, 175 to 457 minutes), and the median total operating time was 582 minutes (range, 389 to 968 minutes). Three patients required mechanical support to be weaned from cardiopulmonary bypass. Postoperatively, 3 patients required the addition of mechanical support in the intensive care unit, and 3 patients required tracheostomy for prolonged ventilation. The majority of patients had a complicated postoperative course (66%). The median number of noncardiac consultants required to help care for these patients was four (range, two to 12). The mortality was 0%. Conclusions Cardiac transplantation in adults with congenital cardiac disease is challenging, is fraught with adverse events, and requires meticulous care and teamwork for success. A surgeon specializing in congenital heart conditions may be best to handle the operative challenges, and an adult hospital with access to certain technology and consultant services may be best to handle the postoperative challenges in this difficult patient population. © 2015 The Society of Thoracic Surgeons. AD - Department of Cardiothoracic Surgery and Cardiology, Emory University School of Medicine, 1405 Clifton Rd, Atlanta, GA 30322, United States AU - Mori, M. AU - Vega, D. AU - Book, W. AU - Kogon, B. E. DB - Scopus DO - 10.1016/j.athoracsur.2015.03.001 IS - 6 M3 - Article N1 - Cited By :16 Export Date: 15 June 2020 PY - 2015 SP - 2173-2178 ST - Heart transplantation in adults with congenital heart disease: 100% survival with operations performed by a surgeon specializing in congenital heart disease in an adult hospital T2 - Annals of Thoracic Surgery TI - Heart transplantation in adults with congenital heart disease: 100% survival with operations performed by a surgeon specializing in congenital heart disease in an adult hospital UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84930924266&doi=10.1016%2fj.athoracsur.2015.03.001&partnerID=40&md5=870703a2ef07960b0c13f76fa5d4db85 VL - 99 ID - 1977 ER - TY - JOUR AB - Heart transplantation is an accepted therapeutic modality for end-stage congenital heart disease for both biventricular and univentricular anomalies. Many transplant centers have pushed the limits of transplantation to include patients with high pulmonary vascular resistance, high panel reactive antibodies, positive cross-matches, and ABOincompatibility. Excellent results have been possible, particularly with the development of improved diagnostic and therapeutic algorithms to prevent and treat rejection, infection, and post-transplant lymphoproliferative disease. Late graft failure and chronic rejection remain vexing problems. The vast majority of patients with biventricular congenital heart disease have undergone prior cardiac surgical procedures. Indications for transplantation in this subgroup are primarily progressive refractory heart failure following prior cardiac surgical reconstructive procedures. Contraindications to transplantation mimic those for other forms of end-stage heart disease. A determination of pulmonary vascular resistance is important in listing patients with biventricular congenital heart disease for heart transplantation. Modifications in the implant technique are necessary and vary depending on underlying recipient anatomy. Risk factors for perioperative outcomes in patients with biventricular congenital heart disease include the need for reoperation, the degree of anatomic reconstruction necessary during the implant procedure, and the degree of antibody sensitization, in addition to a number of other recipient and donor factors. Postoperative outcomes and survival are very good but remain inferior to those with cardiomyopathy in most series. In conclusion, patients with end-stage biventricular congenital heart disease represent a complex group of patients for heart transplantation, and require careful evaluation and management to ensure optimal outcomes. © 2011 Bentham Science Publishers. AD - Section of Pediatric Cardiothoracic Surgery, St. Louis Children's Hospital, Washington University School of Medicine, St. Louis, MO, United States AU - Mora, B. N. AU - Huddleston, C. B. DB - Scopus DO - 10.2174/157340311797484196 IS - 2 KW - Biventricular Cardiac transplantation Congenital heart disease Failing ventricle Heart failure Heart transplant Orthotopic heart transplantation Pediatric cardiac surgery Perioperative and long-term outcomes M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 2011 SP - 92-101 ST - Heart transplantation in biventricular congenital heart disease: Indications, techniques, and outcomes T2 - Current Cardiology Reviews TI - Heart transplantation in biventricular congenital heart disease: Indications, techniques, and outcomes UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-80054109185&doi=10.2174%2f157340311797484196&partnerID=40&md5=4c8441e31e41ef5260a5c7735797df5f VL - 7 ID - 2127 ER - TY - JOUR AB - Since 1988, 82 heart transplants have been performed in 80 infants and children. Diagnoses pretransplant were: hypoplastic left heart syndrome (HLHS) (n = 43); cardiomyopathy (n = 19); endocardial fibroelastosis (n = 6); and other complex congenital heart diseases (n = 12). Age at transplantation was < 1 yr in 61 patients. Overall survival rate was 79% at 1 yr and 73% at 5 and 10 yr. To date, 20 patients have died after transplantation. Causes of death were: rejection (eight patients); right ventricular failure (four patients); transplant coronary artery disease (TCAD) (two patients); and other causes (six patients). In the majority of patients somatic growth is not impaired, and renal function is reduced (but stable) in all patients. Two patients developed post-transplant lymphoproliferative disease, which was treated successfully. Major long-term morbidity is neurologic deficit - severe in three patients and minor in six. TCAD was present or suspected in six surviving patients. We conclude that heart transplantation in infants and children can be performed with good early and late results. Quality of life is excellent in most patients. TCAD, however, will become an increasing problem in the long term. AD - Department of Pediatric Cardiology, University Giessen, Giessen, Germany. Juergen.Bauer@paediat.med.uni-giessen.de AN - 11737772 AU - Bauer, J. AU - Thul, J. AU - Krämer, U. AU - Hagel, K. J. AU - Akintürk, H. AU - Valeske, K. AU - Schindler, E. AU - Bohle, R. M. AU - Schranz, D. DA - Dec DB - PubMed DO - 10.1034/j.1399-3046.2001.00023.x DP - NLM ET - 2001/12/12 IS - 6 KW - Child Follow-Up Studies Glomerular Filtration Rate Heart Diseases/surgery *Heart Transplantation/adverse effects/mortality/physiology Humans Immunosuppressive Agents/therapeutic use Infant Quality of Life Survival Analysis Treatment Outcome LA - eng N1 - Bauer, J Thul, J Krämer, U Hagel, K J Akintürk, H Valeske, K Schindler, E Bohle, R M Schranz, D Journal Article Denmark Pediatr Transplant. 2001 Dec;5(6):457-62. doi: 10.1034/j.1399-3046.2001.00023.x. PY - 2001 SN - 1397-3142 (Print) 1397-3142 SP - 457-62 ST - Heart transplantation in children and infants: short-term outcome and long-term follow-up T2 - Pediatr Transplant TI - Heart transplantation in children and infants: short-term outcome and long-term follow-up VL - 5 ID - 403 ER - TY - JOUR AB - BACKGROUND: Following the Norwood procedure for hypoplastic left heart syndrome (HLHS), pulmonary artery distortion and hypoplasia are common and may negatively impact late outcome. The hemi-Fontan procedure (HFP) augments the central pulmonary arteries and establishes a connection between the right atrial/superior vena cava junction and the pulmonary arteries, while excluding the inferior vena cava. METHODS: The hospital records of all 114 patients undergoing a HFP for HLHS between August 1993 and April 1998 were reviewed to assess patient, procedural, and morphologic determinations of outcome. The results of cardiac catheterization, Doppler/echocardiography, 12 lead electrocardiograms, hospital and subsequent course, as well as suitability and outcome for the Fontan procedure were analyzed. RESULTS: Mean age was 5.4 months (range 1.5 to 15 months). Right ventricular function was normal in 95 patients, moderately depressed in 14, and severely depressed in five. Tricuspid regurgitation was absent or mild in 91 patients, moderate in 13, and severe in 10. Concomitant procedures included left superior vena cava to pulmonary artery anastomosis (12), tricuspid valve repair (10), pulmonary artery stent placement (3), coarctation repair (2), and aortic pseudoaneurysm repair (1). Hospital survival was 112/114, 98% (95% confidence interval [CI]: 95% to 100%). There were two late deaths, one noncardiac. Sinus rhythm is present in 105 patients (92%, 95% CI: 87% to 97%). To date, 79 of these patients have undergone the Fontan procedure with 74 survivors (94%, 95% CI: 89% to 99%). CONCLUSIONS: The HFP may be performed with excellent results for HLHS. It effectively augments the central pulmonary arteries while preserving sinus rhythm in the majority. In addition, the HFP facilitates the subsequent Fontan procedure and has significantly improved the overall outcome. AD - Department of Surgery, University of Michigan School of Medicine, Ann Arbor, USA. AN - 10543507 AU - Douglas, W. I. AU - Goldberg, C. S. AU - Mosca, R. S. AU - Law, I. H. AU - Bove, E. L. DA - Oct DB - PubMed DO - 10.1016/s0003-4975(99)00915-7 DP - NLM ET - 1999/10/30 IS - 4 KW - Angiography Cardiac Catheterization Child, Preschool Echocardiography, Doppler Electrocardiography Female Fontan Procedure/*methods Hospital Mortality Humans Hypoplastic Left Heart Syndrome/mortality/*surgery Infant Male Postoperative Complications/diagnosis/mortality Pulmonary Artery/surgery Risk Factors Suture Techniques Treatment Outcome Vena Cava, Superior/surgery LA - eng N1 - Douglas, W I Goldberg, C S Mosca, R S Law, I H Bove, E L Journal Article Netherlands Ann Thorac Surg. 1999 Oct;68(4):1361-7; discussion 1368. doi: 10.1016/s0003-4975(99)00915-7. PY - 1999 SN - 0003-4975 (Print) 0003-4975 SP - 1361-7; discussion 1368 ST - Hemi-Fontan procedure for hypoplastic left heart syndrome: outcome and suitability for Fontan T2 - Ann Thorac Surg TI - Hemi-Fontan procedure for hypoplastic left heart syndrome: outcome and suitability for Fontan VL - 68 ID - 282 ER - TY - JOUR AB - BACKGROUND: Exercise intolerance is frequent among Fontan patients and an important determinant for quality of life. This study investigated the hemodynamic causes of impaired exercise capacity in Fontan patients with particular focus on the influence of stroke volume index (SVI) and heart rate (HR). METHODS AND RESULTS: In 38 Fontan patients, peak oxygen consumption (VO2), SVI and HR were recorded during incremental load exercise test and compared with 19 age and gender matched controls. SVI (ml/m(2)) was lower in patients than controls during warm-up (28[26-31] vs. 35[30-39], p=0.0093), at submaximal (40[37-43] vs. 55[51-59], p<0.0001) and at maximal exercise (38[35-40] vs. 54[51-58], p<0.0001). Similarly, HR (% of expected maximum) was lower in patients at warm-up (45[43-48]% vs. 64[57-64]%, p<0.0001), submaximal (71[68-75]% vs 85[82-88]%, p<0.0001) and maximal exercise (84[80-88]% vs. 97[95-99]%, p<0.0001). Furthermore, SVI dropped 14% (from 44[41-48] to 38[35-40] ml/m(2)) in Fontan patients from the peak plateau to maximal exercise vs. 5% (from 57[53-61] to 54[51-58] ml/m(2)) in controls, p<0.0001. The low SVI and HR explained 67% and 20% of the difference in peak VO2 between Fontan patients and controls respectively. CONCLUSION: SVI decreased significantly in Fontan patients near the end of maximal effort exercise. The low SVI at maximal exercise was the most important hemodynamic factor limiting exercise capacity in Fontan patients, whereas chronotropic impairment had a smaller impact. The low SVI and HR at maximal exercise accounted for the difference in peak VO₂ between Fontan patients and controls in this study. CLINICAL TRIAL REGISTRATION: http://www.cvk.sum.dk/CVK/Home/English.aspx (protocol nr: H-3-2010-045). AD - Department of Cardiology, Copenhagen University Hospital, Rigshospitalet, Denmark. Electronic address: andershebert@gmail.com. Department of Cardiology, Copenhagen University Hospital, Rigshospitalet, Denmark. Institute of Sports Medicine, Department of Orthopedic Surgery, Bispebjerg Hospital and Center for Healthy Ageing, Faculty of Health and Medical Sciences, University of Copenhagen, Denmark. Department of Cardiology, Aarhus University Hospital, Skejby, Aarhus, Denmark. Department of Cardiology, University Hospital of Lund, Sweden. Department of Pediatric Cardiology, University Hospital of Lund, Sweden. AN - 25023794 AU - Hebert, A. AU - Jensen, A. S. AU - Mikkelsen, U. R. AU - Idorn, L. AU - Sørensen, K. E. AU - Thilen, U. AU - Hanseus, K. AU - Søndergaard, L. DA - Aug 20 DB - PubMed DO - 10.1016/j.ijcard.2014.06.015 DP - NLM ET - 2014/07/16 IS - 3 KW - Adolescent Adult Case-Control Studies Child Exercise Test/methods/*trends Exercise Tolerance/*physiology Female Fontan Procedure/*trends Heart Rate/physiology Hemodynamics/*physiology Humans Male Oxygen Consumption/*physiology Stroke Volume/physiology Young Adult Cardio-pulmonary exercise test Chronotropic impairment Fontan Stroke volume LA - eng N1 - 1874-1754 Hebert, Anders Jensen, Annette S Mikkelsen, Ulla R Idorn, Lars Sørensen, Keld E Thilen, Ulf Hanseus, Katarina Søndergaard, Lars Clinical Trial Journal Article Multicenter Study Research Support, Non-U.S. Gov't Netherlands Int J Cardiol. 2014 Aug 20;175(3):478-83. doi: 10.1016/j.ijcard.2014.06.015. Epub 2014 Jun 28. PY - 2014 SN - 0167-5273 SP - 478-83 ST - Hemodynamic causes of exercise intolerance in Fontan patients T2 - Int J Cardiol TI - Hemodynamic causes of exercise intolerance in Fontan patients VL - 175 ID - 243 ER - TY - JOUR AB - Numerical analysis of cardiovascular flow is one of several methods of use for the quantitative evaluation of patient-specific treatments. However, due to the complexity of vascular geometry and flow conditions, the cardiovascular flow simulation continues to be a challenging project. As the flow at the peak of systolic heart beat displayed full turbulence, the congenital heart treatments, Norwood and TCPC procedure, were investigated through the use of computational hemodynamic technology, in the present study. On the other hand, at diastolic period, the flow fell to an almost sedentary state. This indicated that the cardiovascular flow experienced a strange transition of flow from systolic peak to diastole. Thus, in order to accurately simulate this transitional flow, a very small time step was applied in the k - ε turbulent model calculation. Energy losses (EL), local pressure and wall shear stress were analyzed to estimate the result of clinical treatments. It was found that the value of EL, including the influence of respiration, was 1.5 times higher than the value of EL, disregarding respiratory influences. These results indicated that the hemodynamic outcomes of TCPC treatment are noticeably influenced by respiration. The effect of respiration plays an important role in estimating the results of TCPC treatment and thus should be included as one of the important conditions of computational hemodynamic analysis. AD - Y. Qian, Australian School of Advanced Medicine, Macquarie University, NSW 2109, Australia. AU - Qian, Y. AU - Liu, J. L. AU - Liu, J. F. DB - Medline KW - article biological model computer assisted surgery computer simulation coronary artery blood flow Fontan procedure hemodynamics human hypoplastic left heart syndrome infant newborn Norwood procedure pathology pathophysiology statistics three-dimensional imaging LA - English M3 - Article N1 - L369758167 2013-09-13 PY - 2012 SN - 1557-170X SP - 661-664 ST - Hemodynamic simulation for surgical treatment of congenital heart disease T2 - Conference proceedings : ... Annual International Conference of the IEEE Engineering in Medicine and Biology Society. IEEE Engineering in Medicine and Biology Society. Conference TI - Hemodynamic simulation for surgical treatment of congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L369758167 VL - 2012 ID - 1034 ER - TY - JOUR AB - Patients who undergo a Fontan procedure experience some degree of liver disease. Hemodynamic changes such as central venous hypertension, depressed dynamic cardiac output, and late ventricular dysfunction combined with long-standing hypoxia preceding the Fontan procedure all are recognized risk factors for hepatic injury. The histopathologic changes associated with cardiac hepatopathy include chronic passive congestion, centrilobular necrosis, and cardiac cirrhosis. However, hepatic adenoma and hepatic adenomatosis (HA) are not well-known pathologies during the course of cardiac hepatopathy. This study focused on a 7-year-old girl with chronic hepatic changes and HA who had undergone a Fontan procedure. Hepatic adenomatosis was diagnosed on the basis of magnetic resonance imaging (MRI) and MRI-guided liver biopsy. To the best of the authors' knowledge, this case involved the youngest patient with hepatocellular adenomatosis documented in the literature. It was a unique case because the patient experienced HA after a Fontan procedure. AD - Department of Pediatric Cardiology, Faculty of Medicine, Kocaeli University, Kocaeli, Turkey. babaogluk@yahoo.com AN - 20204345 AU - Babaoglu, K. AU - Binnetoglu, F. K. AU - Aydoğan, A. AU - Altun, G. AU - Gürbüz, Y. AU - Inan, N. AU - Corapçioğlu, F. DA - Aug DB - PubMed DO - 10.1007/s00246-010-9685-x DP - NLM ET - 2010/03/06 IS - 6 KW - Adenoma, Liver Cell/*complications/diagnosis Biopsy Child Diagnosis, Differential Echo-Planar Imaging Female Fontan Procedure/*methods Heart Defects, Congenital/complications/*surgery Humans Liver Neoplasms/*complications/diagnosis Time Factors LA - eng N1 - 1432-1971 Babaoglu, Kadir Binnetoglu, Fatih Köksal Aydoğan, Ayşen Altun, Gürkan Gürbüz, Yeşim Inan, Nagihan Corapçioğlu, Funda Case Reports Journal Article United States Pediatr Cardiol. 2010 Aug;31(6):861-4. doi: 10.1007/s00246-010-9685-x. Epub 2010 Mar 4. PY - 2010 SN - 0172-0643 SP - 861-4 ST - Hepatic adenomatosis in a 7-year-old child treated earlier with a Fontan procedure T2 - Pediatr Cardiol TI - Hepatic adenomatosis in a 7-year-old child treated earlier with a Fontan procedure VL - 31 ID - 408 ER - TY - JOUR AB - OBJECTIVES: A novel Y-shaped baffle has been proposed for the Fontan operation with promising initial results. However, previous studies have relied either on idealized models or a single patient-specific model. The objective of this study is to comprehensively compare the hemodynamic performance and hepatic blood flow distribution of the Y-graft Fontan baffle with 2 current designs using multiple patient-specific models. METHODS: Y-shaped and tube-shaped grafts were virtually implanted into 5 patient-specific Glenn models forming 3 types of Fontan geometries: Y-graft, T-junction, and offset. Unsteady flow simulations were performed at rest and at varying exercise conditions. The hepatic flow distribution between the right and left lungs was carefully quantified using a particle tracking method. Other physiologically relevant parameters such as energy dissipation, superior vena cava pressure, and wall shear stress were evaluated. RESULTS: The Fontan geometry significantly influences the hepatic flow distribution. The Y-graft design improves the hepatic flow distribution effectively in 4 of 5 patients, whereas the T-junction and offset designs may skew as much as 97% of hepatic flow to 1 lung in 2 cases. Sensitivity studies show that changes in pulmonary flow split can affect the hepatic flow distribution dramatically but that some Y-graft and T-junction designs are relatively less sensitive than offset designs. The Y-graft design offers moderate improvements over the traditional designs in power loss and superior vena cava pressure in all patients. CONCLUSIONS: The Y-graft Fontan design achieves overall superior hemodynamic performance compared with traditional designs. However, the results emphasize that no one-size-fits-all solution is available that will universally benefit all patients and that designs should be customized for individual patients before clinical application. AD - Department of Mechanical and Aerospace Engineering, University of California San Diego, 9500 Gilman Dr, La Jolla, CA 92093-0411, USA. AN - 21962841 AU - Yang, W. AU - Vignon-Clementel, I. E. AU - Troianowski, G. AU - Reddy, V. M. AU - Feinstein, J. A. AU - Marsden, A. L. DA - May DB - PubMed DO - 10.1016/j.jtcvs.2011.06.042 DP - NLM ET - 2011/10/04 IS - 5 KW - Biomechanical Phenomena Child, Preschool *Computer Simulation Equipment Design Fontan Procedure/*instrumentation Heart Defects, Congenital/diagnosis/physiopathology/*surgery *Hemodynamics Humans *Hydrodynamics *Liver Circulation Magnetic Resonance Imaging Materials Testing *Models, Cardiovascular Pulmonary Circulation Regional Blood Flow Stress, Mechanical Vena Cava, Inferior/physiopathology Venous Pressure LA - eng N1 - 1097-685x Yang, Weiguang Vignon-Clementel, Irene E Troianowski, Guillaume Reddy, V Mohan Feinstein, Jeffrey A Marsden, Alison L Comparative Study Evaluation Study Journal Article Research Support, Non-U.S. Gov't United States J Thorac Cardiovasc Surg. 2012 May;143(5):1086-97. doi: 10.1016/j.jtcvs.2011.06.042. Epub 2011 Sep 29. PY - 2012 SN - 0022-5223 SP - 1086-97 ST - Hepatic blood flow distribution and performance in conventional and novel Y-graft Fontan geometries: a case series computational fluid dynamics study T2 - J Thorac Cardiovasc Surg TI - Hepatic blood flow distribution and performance in conventional and novel Y-graft Fontan geometries: a case series computational fluid dynamics study VL - 143 ID - 411 ER - TY - JOUR AB - Introduction: Wolf–Hirschhorn syndrome (WHS) is a contiguous gene syndrome involving deletions of the chromosome 4p16 region associated with growth failure, characteristic craniofacial abnormalities, cardiac defects, and seizures. Case Report: This report describes a six-month-old girl with WHS with growth failure and typical craniofacial features who died of complex congenital heart disease. Genetic studies revealed a 9.8 Mb chromosome 4p-terminal deletion. At autopsy, the liver was grossly unremarkable. Routine sampling and histologic examination revealed two hepatocellular nodular lesions with expanded cell plates and mild cytologic atypia. Immunohistochemical staining revealed these nodules were positive for glutamine synthetase and glypican 3, with increased Ki-67 signaling and diffuse CD34 expression in sinusoidal endothelium. These findings are consistent with hepatoblastoma or hepatocellular carcinoma. Conclusions: A possible association between WHS and hepatic malignancy may be an important consideration in the care and management of WHS patients. AD - P.G. Gallagher, Department of Pathology, Yale University School of Medicine, 333 Cedar Street, P.O. Box 208064, New Haven, CT, United States AU - Rutter, S. AU - Morotti, R. A. AU - Peterec, S. AU - Gallagher, P. G. DB - Embase Medline DO - 10.1080/15513815.2017.1293201 IS - 3 KW - CD34 antigen glutamate ammonia ligase glypican 3 Ki 67 antigen prostaglandin amnion fluid analysis anterior fontanel article ascending aorta auricle autopsy bicuspid aortic valve birth weight body height case report chromosome 4p chromosome deletion 4 chronic lung disease clinical feature drug withdrawal female fetus karyotyping follicle cyst follow up forehead gestational age growth disorder head circumference heart arrest heart atrium septum defect heart left ventricle hypertrophy hepatoblastoma human human tissue hyperbilirubinemia hypercalcemia hyperparathyroidism hypertelorism immunohistochemistry infant intrauterine growth retardation liver cell carcinoma liver histology low set ear lung blood flow lung congestion lung edema Meckel diverticulum micrognathia palpebral fissure priority journal pulmonary valve atresia signal transduction tricuspid valve atresia ventilator associated pneumonia Wolf Hirschhorn syndrome LA - English M3 - Article N1 - L614711252 2017-03-13 2017-06-14 PY - 2017 SN - 1551-3823 1551-3815 SP - 256-262 ST - Hepatic Malignancy in an Infant with Wolf–Hirschhorn Syndrome T2 - Fetal and Pediatric Pathology TI - Hepatic Malignancy in an Infant with Wolf–Hirschhorn Syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L614711252 http://dx.doi.org/10.1080/15513815.2017.1293201 VL - 36 ID - 755 ER - TY - JOUR AB - BACKGROUND: As patients began to survive for longer periods following modified Fontan operations (conventional atrio-pulmonary connection), the late morbidity after this procedure became increasingly apparent. The purpose of the present study was to evaluate late sequelae of modified Fontan operations in long-term survivors (n=14) at our institute. METHODS AND RESULTS: The cohort consisted of patients who underwent a modified Fontan operation between 1981 and 1990. Thus, all patients were examined at least 10 years postoperatively in this study. Early mortality, within 30 days of the operation, was 17.6% (three of 17 patients died from low output syndrome). Excluding these early deaths, the cumulative survival rate at 5 and 10 years was 100 and 79%, respectively. Arrhythmias including atrial fibrillation or flutter were the main late causes of morbidity. The arrhythmia-free rate at 5 and 10 years was 77 and 50%, respectively. Although the quality of life was considered good because all patients (n=11) who survived for 10 years or more were in class I or II according to the New York Heart Association classification, most of them in fact suffered from potentially life-threatening arrhythmias. CONCLUSIONS: Meticulous attention to and utilization of established treatment strategies for arrhythmias including anti-arrhythmics, anticoagulants, catheter ablation or re-operation converting the circulation to the total cavopulmonary connection must be considered in long-term survivors following the modified Fontan operation. The fact that no one knows when the thrombogenic arrhythmias occur suggests anticoagulants should be initiated in the early postoperative period. AD - Department of Thoracic and Cardiovascular Surgery, Sapporo Medical University School of Medicine, South-1, West-16, Cho-ku, Sapporo 060-8556, Japan. satoru-sugimoto@hokkaido.med.or.jp AN - 11259811 AU - Sugimoto, S. AU - Takagi, N. AU - Hachiro, Y. AU - Abe, T. DA - Mar DB - PubMed DO - 10.1016/s0167-5273(00)00460-5 DP - NLM ET - 2001/03/22 IS - 1 KW - Adolescent Adult Anticoagulants/*administration & dosage Arrhythmias, Cardiac/*etiology Child Child, Preschool Female Fontan Procedure/*adverse effects/methods/mortality Humans Male Quality of Life Time Factors LA - eng N1 - Sugimoto, S Takagi, N Hachiro, Y Abe, T Journal Article Netherlands Int J Cardiol. 2001 Mar;78(1):33-9. doi: 10.1016/s0167-5273(00)00460-5. PY - 2001 SN - 0167-5273 (Print) 0167-5273 SP - 33-9 ST - High frequency of arrhythmias after Fontan operation indicates earlier anticoagulant therapy T2 - Int J Cardiol TI - High frequency of arrhythmias after Fontan operation indicates earlier anticoagulant therapy VL - 78 ID - 386 ER - TY - JOUR AB - INTRODUCTION: Experience with high-frequency oscillatory ventilation (HFOV) after congenital cardiac surgery is limited despite evidence about reduction in pulmonary vascular resistance after the Fontan procedure. HFOV is recommended in adults and children with acute respiratory distress syndrome. The aim of the present study was to assess associations between commencement of HFOV on the day of surgery and length of mechanical ventilation, length of Intensive Care Unit (ICU) stay and mortality in neonates and infants with respiratory distress following cardiac surgery. METHODS: A logistic regression model was used to develop a propensity score, which accounted for the probability of being switched from conventional mechanical ventilation (CMV) to HFOV on the day of surgery. It included baseline characteristics, type of procedure and postoperative variables, and was used to match each patient with HFOV with a control patient, in whom CMV was used exclusively. Length of mechanical ventilation, ICU stay and mortality rates were compared in the matched set. RESULTS: Overall, 3,549 neonates and infants underwent cardiac surgery from January 2001 through June 2010, 120 patients were switched to HFOV and matched with 120 controls. After adjustment for the delay to sternal closure, duration of renal replacement therapy, occurrence of pulmonary hypertension and year of surgery, the probability of successful weaning over time and the probability of ICU delivery over time were significantly higher in patients with HFOV, adjusted hazard ratios and 95% confidence intervals: 1.63, 1.17 to 2.26 (P = 0.004). and 1.65, 95% confidence intervals: 1.20 to 2.28 (P = 0.002) respectively. No association was found with mortality. CONCLUSIONS: When commenced on the day of surgery in neonates and infants with respiratory distress following cardiac surgery, HFOV was associated with shorter lengths of mechanical ventilation and ICU stay than CMV. AD - Anesthesiolgy and Critical Care Department, Necker-Enfants Malades Hospital, Assistance Publique-Hôpitaux de Paris, 149 rue de Sèvres, 75015 Paris, France. mirela.bojan@nck.aphp.fr AN - 22035562 AU - Bojan, M. AU - Gioanni, S. AU - Mauriat, P. AU - Pouard, P. C2 - PMC3334810 DB - PubMed DO - 10.1186/cc10521 DP - NLM ET - 2011/11/01 IS - 5 KW - Heart Defects, Congenital/*surgery *High-Frequency Ventilation/statistics & numerical data Hospital Mortality Humans Infant Infant, Newborn Intensive Care Units/statistics & numerical data Length of Stay/statistics & numerical data Perioperative Care/*methods Postoperative Complications/mortality Propensity Score Respiratory Distress Syndrome, Newborn/mortality Retrospective Studies Time Factors Treatment Outcome LA - eng N1 - 1466-609x Bojan, Mirela Gioanni, Simone Mauriat, Philippe Pouard, Philippe Journal Article Crit Care. 2011;15(5):R259. doi: 10.1186/cc10521. Epub 2011 Oct 28. PY - 2011 SN - 1364-8535 (Print) 1364-8535 SP - R259 ST - High-frequency oscillatory ventilation and short-term outcome in neonates and infants undergoing cardiac surgery: a propensity score analysis T2 - Crit Care TI - High-frequency oscillatory ventilation and short-term outcome in neonates and infants undergoing cardiac surgery: a propensity score analysis VL - 15 ID - 537 ER - TY - JOUR AB - Background: Of the children with Down syndrome 40-50% have cardiac defects and the majority of these cardiac defects are amenable to biventricular repair. The outcome of single ventricle palliation is improving; nonetheless, there are limited data on Down syndrome patients with associated high-risk factors undergoing single ventricle palliation. Our aim was to study the outcomes of children with Down syndrome and high-risk factors on the single ventricle palliation pathway. Methods: A retrospective study on all patients with Down syndrome on the single ventricle palliation pathway from 2005 until 2011 was conducted. Operative, clinical, echocardiographic, haemodynamic data, and follow-up data were reviewed. Results: A total of 310 patients underwent at least one single ventricle surgical intervention. Of those, eight patients had Down syndrome, five of which had associated risk factors - low birth weight, high pulmonary vascular resistance, pulmonary vein stenosis, significant atrioventricular valve regurgitation, and extracardiac anomalies. Mortality in the high-risk group was 80% (4/5), compared with 33% (1/3) in the non-high-risk patients. Overall, after a median follow-up period of 138 days (8-576 days), only 37.5% (3/8) of patients were alive. Conclusion: Despite many improvements in the care of single ventricle patients, the fate of those with Down syndrome and associated high-risk factors remains poor. Further multicentre longer-term studies are needed to validate and quantify the cumulative effects of negative prognostic factors in this complex group of patients. AD - A. Harahsheh, Department of Pediatrics, George Washington University, School of Medicine, Washington, DC, United States AU - Ooi, Y. K. AU - Sinha, P. AU - Gierdalski, M. AU - Harahsheh, A. DB - Embase Medline DO - 10.1017/S1047951114000419 IS - 3 KW - article cardiovascular mortality cardiovascular risk child Down syndrome echocardiography follow up heart disease heart hemodynamics heart surgery high risk patient human low birth weight lung vascular resistance major clinical study mitral valve regurgitation pulmonary vein obstruction retrospective study single ventricle palliation LA - English M3 - Article N1 - L53072665 2014-04-04 2015-02-25 PY - 2015 SN - 1467-1107 1047-9511 SP - 539-543 ST - High-risk single ventricle palliation in children with Down syndrome: Single institution experience T2 - Cardiology in the Young TI - High-risk single ventricle palliation in children with Down syndrome: Single institution experience UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L53072665 http://dx.doi.org/10.1017/S1047951114000419 VL - 25 ID - 866 ER - TY - JOUR AB - Adults with congenital heart disease (CHD) are a rapidly increasing population and their impact on healthcare resources is not fully understood. The purpose of this study was to describe the costs of hospitalizations for non-cardiac disease for adults with CHD. We conducted a retrospective review of hospital discharge data from the University HealthSystem Consortium Clinical Data Base/Resource Manager from January 2011 through December 2013. Patients were ≥ 18 years old at admission with any ICD-9 code for moderate or high severity CHD; cardiac surgical admissions were excluded. The comparison group consisted of patients ≥ 18 years old with no ICD-9 codes for any severity CHD. There were 9,169,700 non-CHD, 28,224 moderate CHD, and 3045 high severity CHD hospital admissions. Total length of stay was longer for acute kidney injury, depressive disorder, esophageal reflux, and obstructive sleep apnea for any severity CHD; ICU admission rates were higher for all diagnoses with any severity CHD. Mean observed direct costs were higher for all diagnoses for moderate CHD and all diagnoses except dehydration, type 2 diabetes, obesity, and obstructive sleep apnea for high severity CHD. This review identified significantly increased hospitalization costs for adults with moderate and high severity CHD who are admitted for non-cardiac medical conditions not associated with concomitant cardiac surgical procedures. Admissions with CHD diagnoses had higher ICU admission rates, longer lengths of stay, and higher mortality for most non-cardiac admission diagnoses. These data will add to our understanding of the economic impact of adults with CHD. © 2017, Springer Science+Business Media, LLC, part of Springer Nature. AD - Department of Pediatrics (Cardiology), University of Arizona, 1501 N. Campbell Ave, PO Box 245073, Tucson, AZ 85724, United States Department of Pediatrics, University of Arizona, Tucson, AZ, United States University of Arizona College of Medicine, Tucson, AZ, United States Arizona Pediatric Cardiology, Phoenix, AZ, United States Sarver Heart Center, University of Arizona, Tucson, AZ, United States AU - Seckeler, M. D. AU - Thomas, I. D. AU - Andrews, J. AU - Meziab, O. AU - Moe, T. AU - Heller, E. AU - Klewer, S. E. DB - Scopus DO - 10.1007/s00246-017-1770-y IS - 3 KW - Administrative dataset Cost comparison Fontan procedure Heart defects, congenital Hospitalizations Tetralogy of fallot Transposition of the great arteries M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2018 SP - 437-444 ST - Higher Cost of Hospitalizations for Non-cardiac Diagnoses in Adults with Congenital Heart Disease T2 - Pediatric Cardiology TI - Higher Cost of Hospitalizations for Non-cardiac Diagnoses in Adults with Congenital Heart Disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85033706518&doi=10.1007%2fs00246-017-1770-y&partnerID=40&md5=4312081d3532c01fe1cb81a99bbb2893 VL - 39 ID - 1819 ER - TY - JOUR AB - OBJECTIVES: US regulations allow local institutional review boards to approve greater than minimal risk, no-benefit research when the research enrolls children with the condition under study but not when it enrolls healthy children. We aim to describe the opinions of parents regarding higher-hazard, no-benefit research METHODS: Quantitative and qualitative interviews with parents of children without heart disease or chronic medical conditions (no heart disease [noHD], n = 30), children with fully correctable heart disease (FCHD, n = 30), and children with life-altering heart disease (LAHD, n = 30). RESULTS: Parents of children with heart disease endorse higherhazard, no-benefit heart disease research more strongly than noHD parents. Eight of 30 noHD parents, 19 of 30 FCHD parents, and 26 of 30 LAHD parents reported willingness to enroll their children in a heart disease research study involving an otherwise unnecessary chest radiograph (P , .01). There was no difference among groups in willingness to enroll their children in a similar study focused on childhood cancer. Twenty-two of 30 FCHD and 30 of 30 LAHD parents reported that parents have a responsibility to enroll their children in medical research to help future children with heart disease. Twentyone of 30 noHD parents, 29 of 30 FCHD parents, and 30 of 30 LAHD parents feel able to evaluate the risks of medical research (P = .01). CONCLUSIONS: Parental opinions regarding higher-hazard, no-benefit research align with federal regulations. Parental willingness to enroll their children in higher-hazard, no-benefit research is driven in part by a sense of obligation to a community of families affected by childhood heart disease. Pediatrics 2013;132:e1302-e1309. Copyright © 2013 by the American Academy of Pediatrics. AD - M.C. Morris, Department of Pediatrics, Children's Hospital of New York, 3959 Broadway, 10-24, New York, NY 10032, United States AU - Sachdeva, T. AU - Morris, M. C. DB - Embase Medline DO - 10.1542/peds.2013-1700 IS - 5 KW - aortic coarctation aortic disease appendicitis article bronchiolitis cardiomyopathy cellulitis child childhood cancer controlled study Fallot tetralogy female fever funnel chest great vessels transposition heart arrhythmia heart atrium septum defect heart single ventricle heart ventricle septum defect higher hazard no benefit research human hypertension infant injury interview male medical research mitral valve stenosis parental attitude parental behavior pneumonia preschool child priority journal public opinion pulmonary hypertension pyelonephritis qualitative research quantitative study school child thorax radiography tonsillectomy LA - English M3 - Article N1 - L370188631 2013-12-03 2013-12-05 PY - 2013 SN - 1098-4275 0031-4005 SP - e1302-e1309 ST - Higher-hazard, no benefit research involving children: Parental perspectives T2 - Pediatrics TI - Higher-hazard, no benefit research involving children: Parental perspectives UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L370188631 http://dx.doi.org/10.1542/peds.2013-1700 VL - 132 ID - 983 ER - TY - JOUR AB - The distribution of lipid in the fetal adrenal cortex is reported to correlate with the duration of hypoxia and degree of fetal stress. The original studies were based on Oil Red O staining, requiring frozen tissue that is often not available. To investigate the reliability of these observations, the distribution of lipid in the fetal adrenal cortex was studied in hydrops fetalis (HF) of different etiologies, using immunostaining for adipophilin on formalin-fixed material. Twenty cases of HF due to hemoglobin (Hb) Bart were compared to 34 cases of HF due to other causes. In the fetal zone, lipid distribution was more diffuse in Hb Bart HF compared to other causes of HF, including those due to anemia, supporting the concept that increased lipid in the fetal zone is associated with severity of hypoxia. A more diffuse distribution of lipid correlated with adrenal cytomegaly (P < 0.01) and extramedullary hematopoiesis (P < 0.01) but not Hb level (P = 0.68) nor compact cell change (P = 0.7) or cystic degeneration (P = 0.07) in the definitive zone. A greater degree of cystic degeneration correlated with lower gestational age, rather than the specific etiology of HF. Thus, cystic degeneration is more a reflection of the onset of fetal stress than severity. The combined histologic changes in the fetal and definitive zones of the adrenal gland provide complementary information about fetal status in HF. Immnunostaining for adipophilin circumvents the need for frozen tissue for assessing lipid content by Oil Red O staining, facilitating studies based on archival material. © 2014 Society for Pediatric Pathology. AD - M. Taweevisit, Department of Pathology, Faculty of Medicine, Chulalongkorn University, 1873 King Rama IV Street, Pathumwan, Bangkok, 10330, Thailand AU - Taweevisit, M. AU - Atikankul, T. AU - Thorner, P. S. DB - Embase Medline DO - 10.2350/13-07-1354-OA.1 IS - 3 KW - adipophilin formaldehyde hemoglobin hemoglobin H lipid adrenal cytomegaly adrenal disease adrenal gland anemia aortic coarctation article congenital heart disease controlled study cystic degeneration cystic lymphangioma cytomegaly degeneration disease severity Ebstein anomaly erythropoiesis extramedullary hematopoiesis Fallot tetralogy female fetus fetus distress fetus hydrops gestational age histology human hypoplastic left heart syndrome hypoxia immunohistochemistry lung hemorrhage lung malformation major clinical study male myelopoiesis priority journal tricuspid valve stenosis twin twin transfusion syndrome LA - English M3 - Article N1 - L373644572 2014-08-07 2014-08-15 PY - 2014 SN - 1615-5742 1093-5266 SP - 190-197 ST - Histologic changes in the adrenal gland reflect fetal distress in hydrops fetalis T2 - Pediatric and Developmental Pathology TI - Histologic changes in the adrenal gland reflect fetal distress in hydrops fetalis UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L373644572 http://dx.doi.org/10.2350/13-07-1354-OA.1 VL - 17 ID - 936 ER - TY - JOUR AB - Objective Fontan circulations have obligatory venous hypertension, depressed cardiac output and abnormal arterial elastance. Ventriculovascular coupling is known to be abnormal, but the underlying mechanisms are poorly defined. We aim to describe the histopathological features of vascular remodelling encountered in the central arteries and veins in the Fontan circulation as a possible underlying pathological representation of abnormal ventriculovascular coupling. Methods Postmortemvasculature (inferior vena cava (IVC), superior vena cava (SVC), pulmonary artery (PA), pulmonary vein (PV) and aorta) of 13 patients with a Fontan circulation (mean age 29.9 years, range 9.0-59.8 years) and 2 biventricular controls (ages 17.9 and 30.2 years) was examined. Results IVC and SVC: Eccentric and variable intimal fibromuscular proliferation occurred in 11 Fontan subjects. There was variable loss of medial smooth muscle bundles with reciprocal replacement with dense collagenous tissue. PA: Similar intimal fibromuscular proliferation was seen; however, these intimal changes were accompanied by medial thinning rather than expansion, medial myxoid degeneration and elastic alteration. PV: The PVs demonstrated intimal fibroproliferation and disorganisation of the muscular media. Aorta: The aortic lamina intima was thickened, with associated fibromuscular proliferation and elasticisation. There was also moderate lymphocytic inflammation in the aortic wall. Conclusions Vascular architectural remodelling is common in Fontan patients. The central veins demonstrate profound changes of eccentric intimal expansion and smooth muscle replacement with collagen. The pulmonary demonstrated abnormal intimal proliferation, and aortic remodelling was characterised by intima lamina thickening and a moderate degree of aortic wall inflammation. AD - G.R. Veldtman, Adolescent and Adult Congenital Heart Disease Program, Cincinnati Children's Hospital Medical Centre, Cincinnati, OH, United States AU - Hays, B. S. AU - Baker, M. AU - Laib, A. AU - Tan, W. AU - Udholm, S. AU - Goldstein, B. H. AU - Sanders, S. P. AU - Opotowsky, A. R. AU - Veldtman, G. R. DB - Embase Medline DO - 10.1136/heartjnl-2017-311838 IS - 4 KW - adult article child circulation clinical article controlled study descending aorta double inlet left ventricle female great vessels transposition heart right ventricle double outlet histopathology human human cell human tissue hypoplastic left heart syndrome immunohistochemistry inferior cava vein male morphometry priority journal pulmonary artery pulmonary vein superior cava vein tricuspid valve atresia LA - English M3 - Article N1 - L623100849 2018-07-27 2018-07-31 PY - 2018 SN - 1468-201X 1355-6037 ST - Histopathological abnormalities in the central arteries and veins of Fontan subjects T2 - Heart TI - Histopathological abnormalities in the central arteries and veins of Fontan subjects UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L623100849 http://dx.doi.org/10.1136/heartjnl-2017-311838 VL - 104 ID - 709 ER - TY - JOUR AB - Background: HLHS (hypoplastic left heart syndrome) is a serious congenital heart defect. In neonates with congenital heart disease HLHS accounts for nearly 25% of neonatal deaths. The etiology of HLHS is unknown. At present we expect to have those cases diagnosed in the second trimester of pregnancy. Aim: The main aim was to find the rate and outcome of the congenital heart defect - left heart hypoplasia (HLHS). Material and methods: All cases of this heart defect found in the High Risk Pregnancy Clinic in 2001-2007 were presented. Twice the congenital malformation was diagnosed after delivery and once during pregnancy - in the second trimester. All babies were delivered between 38 and 39 weeks of pregnancy, one by the spontaneous normal delivery, one by elective caesarean section and one by emergency caesarean section because of fetal distress during the delivery. All of the newborns were transported to the Pediatric Cardio surgery Unit of M.U. and operated in first weeks after delivery. Results: In two cases the operation was complicated and children died due to the cardiac arrest. One of the babies died before surgical correction. Two of the women, whose babies died after the operation gave birth to healthy newborns with no cardiac abnormalities. Conclusion: The HLHS is the most serious heart congenital malformation diagnosed often after the delivery because of lack of sufficient diagnostic system in healthy women. The prognosis for the children is bad, but the chance of giving birth to healthy children in the future is good. © 2008 Polskie Towarzystwo Ginekologiczne. AD - High Risk Pregnancy Clinic Medical University Lodz, 1-st Department of Obstetrics and Gynecology, Wileńska 37, Lodz, Poland Unit of Patophysiology of Genital Tract, Medical University Lodz, 1-st Department of Obstetrics and Gynecology, Lodz, Poland Instytut Połoznictwa i Ginekologii, ul. Wileńska 37, 94-029 Łódz, Poland AU - Karowicz-Bilińska, A. AU - Berner-Trabska, M. AU - Kazimierak, W. AU - Brzozowska, M. AU - Kowalska-Koprek, U. AU - Pasiński, J. AU - Kuś, E. DB - Scopus IS - 6 KW - Congenital heart defect Hypoplastic left heart syndrome Neonate M3 - Article N1 - Export Date: 15 June 2020 PY - 2008 SP - 422-425 ST - HLHS - Congenital heart defect in high risk pregnancy unit in 2001-2007 T2 - Ginekologia Polska TI - HLHS - Congenital heart defect in high risk pregnancy unit in 2001-2007 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-48849098771&partnerID=40&md5=c8ce0a37eff752ae6f425ccbfd5fb086 VL - 79 ID - 2200 ER - TY - JOUR AB - BACKGROUND: HLHS (hypoplastic left heart syndrome) is a serious congenital heart defect. In neonates with congenital heart disease HLHS accounts for nearly 25% of neonatal deaths. The etiology of HLHS is unknown. At present we expect to have those cases diagnosed in the second trimester of pregnancy. AIM: The main aim was to find the rate and outcome of the congenital heart defect--left heart hypoplasia (HLHS). MATERIAL AND METHODS: All cases of this heart defect found in the High Risk Pregnancy Clinic in 2001-2007 were presented. Twice the congenital malformation was diagnosed after delivery and once during pregnancy--in the second trimester. All babies were delivered between 38 and 39 weeks of pregnancy, one by the spontaneous normal delivery, one by elective caesarean section and one by emergency caesarean section because of fetal distress during the delivery. All of the newborns were transported to the Pediatric Cardio surgery Unit of M.U. and operated in first weeks after delivery. RESULTS: In two cases the operation was complicated and children died due to the cardiac arrest. One of the babies died before surgical correction. Two of the women, whose babies died after the operation gave birth to healthy newborns with no cardiac abnormalities. CONCLUSION: The HLHS is the most serious heart congenital malformation diagnosed often after the delivery because of lack of sufficient diagnostic system in healthy women. The prognosis for the children is bad, but the chance of giving birth to healthy children in the future is good. AD - High Risk Pregnancy Clinic Medical University Lodz, 1-st Department of Obstetrics and Gynecology Wileńska 37. agakar@interia.pl AN - 18652130 AU - Karowicz-Bilińska, A. AU - Berner-Trabska, M. AU - Kazimierak, W. AU - Brzozowska, M. AU - Kowalska-Koprek, U. AU - Pasiński, J. AU - Kuś, E. DA - Jun DB - PubMed DP - NLM ET - 2008/07/26 IS - 6 KW - Adult Diagnosis, Differential Female Humans Hypoplastic Left Heart Syndrome/*diagnosis/*surgery Poland/epidemiology Pregnancy Pregnancy Outcome Pregnancy Trimester, Second *Pregnancy, High-Risk Prenatal Care/*methods Prenatal Diagnosis/*methods Prognosis Retrospective Studies LA - eng N1 - Karowicz-Bilińska, Agata Berner-Trabska, Marlena Kazimierak, Wojciech Brzozowska, Maria Kowalska-Koprek, Urszula Pasiński, Jacek Kuś, Ewa Journal Article Poland Ginekol Pol. 2008 Jun;79(6):422-5. PY - 2008 SN - 0017-0011 (Print) 0017-0011 SP - 422-5 ST - HLHS--congenital heart defect in High Risk Pregnancy Unit in 2001-2007 T2 - Ginekol Pol TI - HLHS--congenital heart defect in High Risk Pregnancy Unit in 2001-2007 VL - 79 ID - 478 ER - TY - JOUR AB - BACKGROUND: Exercise training has been shown to increase exercise capacity in survivors of Fontan surgery. The geographic distribution of the Fontan population has been a barrier to hospital-based exercise training programmes. The objective of this study was to establish whether a home exercise training programme could achieve similar improvements to a hospital programme. METHODS: Adolescents with a Fontan circulation aged 12-19 years were prospectively recruited in a hospital or home exercise training programme. Patients underwent cardiopulmonary exercise testing and completed the Paediatric Quality of Life Inventory at initial assessment and after completion of an 8-week programme. Both groups performed two 1-hour training sessions per week. Patients in the home training programme had their first session in the hospital, and then progressed independently with one phone consult per week and one home visit by a physiotherapist. RESULTS: In total, 17 patients, with a mean age of 15±3 years, completed the training programme (six hospital). Characteristics and baseline performance of patients were similar in both groups. Oxygen consumption at anaerobic threshold increased from 19.3±3.8 to 21.6±6.0 ml/kg/minute (p=0.02) and peak oxygen pulse increased from 8.8±2.5 to 9.5±2.7 ml/beat (p=0.049). Total quality of life scale improved from 68 to 74% (p=0.01) and psychosocial health improved from 67 to 74% (p=0.02). No patient experienced training-related complications. CONCLUSIONS: Exercise training is beneficial and most likely safe after Fontan, resulting in improved exercise capacity and self-reported quality of life. Home exercise training programmes are probably as effective as hospital programmes. Home exercise training programmes should be integrated in the follow-up care of patients undergoing Fontan surgery. AD - 1Cardiac Surgery Department,Royal Children's Hospital,Melbourne,Vic,Australia. 2Murdoch Childrens Research Institute,Melbourne,Vic,Australia. 5Department of Physiotherapy,The Royal Children's Hospital,Melbourne,Vic,Australia. AN - 30149816 AU - Sutherland, N. AU - Jones, B. AU - Westcamp Aguero, S. AU - Melchiori, T. AU - du Plessis, K. AU - Konstantinov, I. E. AU - Cheung, M. M. H. AU - d'Udekem, Y. DA - Nov DB - PubMed DO - 10.1017/s1047951118001166 DP - NLM ET - 2018/08/29 IS - 11 KW - Adolescent Child Exercise Therapy/*methods Exercise Tolerance/physiology Female *Fontan Procedure Heart Defects, Congenital/physiopathology/rehabilitation/*surgery *Home Care Services Humans *Inpatients Male Postoperative Care/*methods *Quality of Life Surveys and Questionnaires Treatment Outcome Young Adult Chd Fontan procedure exercise therapy rehabilitation resistance training LA - eng N1 - 1467-1107 Sutherland, Nigel Orcid: 0000-0002-4859-7630 Jones, Bryn Westcamp Aguero, Sofia Melchiori, Tristan du Plessis, Karin Konstantinov, Igor E Cheung, Michael M H d'Udekem, Yves Journal Article Multicenter Study England Cardiol Young. 2018 Nov;28(11):1299-1305. doi: 10.1017/S1047951118001166. Epub 2018 Aug 28. PY - 2018 SN - 1047-9511 SP - 1299-1305 ST - Home- and hospital-based exercise training programme after Fontan surgery T2 - Cardiol Young TI - Home- and hospital-based exercise training programme after Fontan surgery VL - 28 ID - 17 ER - TY - JOUR AB - Background: Exercise training has been shown to increase exercise capacity in survivors of Fontan surgery. The geographic distribution of the Fontan population has been a barrier to hospital-based exercise training programmes. The objective of this study was to establish whether a home exercise training programme could achieve similar improvements to a hospital programme.Methods: Adolescents with a Fontan circulation aged 12-19 years were prospectively recruited in a hospital or home exercise training programme. Patients underwent cardiopulmonary exercise testing and completed the Paediatric Quality of Life Inventory at initial assessment and after completion of an 8-week programme. Both groups performed two 1-hour training sessions per week. Patients in the home training programme had their first session in the hospital, and then progressed independently with one phone consult per week and one home visit by a physiotherapist.Results: In total, 17 patients, with a mean age of 15±3 years, completed the training programme (six hospital). Characteristics and baseline performance of patients were similar in both groups. Oxygen consumption at anaerobic threshold increased from 19.3±3.8 to 21.6±6.0 ml/kg/minute (p=0.02) and peak oxygen pulse increased from 8.8±2.5 to 9.5±2.7 ml/beat (p=0.049). Total quality of life scale improved from 68 to 74% (p=0.01) and psychosocial health improved from 67 to 74% (p=0.02). No patient experienced training-related complications.Conclusions: Exercise training is beneficial and most likely safe after Fontan, resulting in improved exercise capacity and self-reported quality of life. Home exercise training programmes are probably as effective as hospital programmes. Home exercise training programmes should be integrated in the follow-up care of patients undergoing Fontan surgery. AD - Cardiac Surgery Department, Royal Children's Hospital, Melbourne, Vic, Australia Murdoch Childrens Research Institute, Melbourne, Vic, Australia Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Melbourne, Vic, Australia Department of Cardiology, The Royal Children's Hospital, Melbourne, Vic, Australia Department of Physiotherapy, The Royal Children's Hospital, Melbourne, Vic, Australia AN - 132530627. Language: English. Entry Date: 20181124. Revision Date: 20191101. Publication Type: Article AU - Sutherland, Nigel AU - Konstantinov, Igor E. AU - d'Udekem, Yves AU - du Plessis, Karin AU - Jones, Bryn AU - Cheung, Michael M. H. AU - Westcamp Aguero, Sofia AU - Melchiori, Tristan DB - ccm DO - 10.1017/S1047951118001166 DP - EBSCOhost IS - 11 KW - Home Health Care Postoperative Care -- Methods Cardiopulmonary Bypass Inpatients Therapeutic Exercise -- Methods Quality of Life Heart Defects, Congenital -- Surgery Male Female Treatment Outcomes Child Exercise Tolerance -- Physiology Young Adult Heart Defects, Congenital -- Physiopathology Human Heart Defects, Congenital -- Rehabilitation Adolescence Validation Studies Comparative Studies Evaluation Research Multicenter Studies Scales Questionnaires N1 - research. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Instrumentation: Pediatric Quality of Life Inventory (PEDSQL). NLM UID: 9200019. PMID: NLM30149816. PY - 2018 SN - 1047-9511 SP - 1299-1305 ST - Home- and hospital-based exercise training programme after Fontan surgery T2 - Cardiology in the Young TI - Home- and hospital-based exercise training programme after Fontan surgery UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=132530627&site=ehost-live&scope=site VL - 28 ID - 1448 ER - TY - JOUR AB - This randomized trial compared physical activity enhancing exercise prescription and education programs in 61 children (36 male) with single-ventricle physiology after Fontan. After Fontan, children are less active than recommended for optimal health. They are often geographically dispersed and unable to attend weekday programs. Participants, 5.9-11.7 years of age who were status 5.3 years post-Fontan, received 12-month, parent-delivered home programs to enhance physical activity, motor skill, fitness, and activity attitudes. Daily moderate-to-vigorous physical activity (MVPA) was measured at baseline and again at 6, 12, and 24 months. Secondary outcomes were gross motor skill, fitness, and activity attitudes. Gross motor skill (p =.01) was significantly greater at the end of the 2-year study period for both intervention groups combined. MVPA at 2 years was significantly greater (p =.03) than the predicted decrease with age. Spring season (85 ± 25 min), male sex (69 ± 21 min), greater baseline activity (0.3 ± 0.1 min/baseline minute), and better gross motor skill (1.1 ± 0.4 min/percentile) increased weekly MVPA in a multivariable repeated-measures regression model adjusted for intervention, maturation during the 2-year study, sex, season, and baseline activity. Benefits were not influenced by type of rehabilitation, compliance, or rural/urban location. Home-based, pediatric physical activity rehabilitation enhances physical activity, gross motor skill, exercise capacity, and physical fitness among preadolescent children after Fontan regardless of rural/urban location. Prescribed education and exercise programs are similarly effective for providing the important health benefits of daily physical activity. Enhanced gross motor skill is associated with increased MVPA despite exercise capacity limitations after Fontan. Rehabilitation attenuates the expected decrease in MVPA with age. © 2013 Springer Science+Business Media New York. AD - P.E. Longmuir, Healthy Active Living and Obesity Research Unit, Children's Hospital of Eastern Ontario, 401 Smyth Road, R1#219, Ottawa, ON K1H 8L1, Canada AU - Longmuir, P. E. AU - Tyrrell, P. N. AU - Corey, M. AU - Faulkner, G. AU - Russell, J. L. AU - McCrindle, B. W. DB - Embase Medline DO - 10.1007/s00246-012-0618-8 IS - 5 KW - adolescent article child controlled study education program exercise female fitness Fontan procedure gender heart rehabilitation heart single ventricle home care human major clinical study male motor performance multivariate analysis outcome assessment patient compliance physical activity preschool child randomized controlled trial regression analysis rural area school child spring urban area LA - English M3 - Article N1 - L52412877 2013-01-30 2013-06-05 PY - 2013 SN - 0172-0643 1432-1971 SP - 1130-1151 ST - Home-based rehabilitation enhances daily physical activity and motor skill in children who have undergone the fontan procedure T2 - Pediatric Cardiology TI - Home-based rehabilitation enhances daily physical activity and motor skill in children who have undergone the fontan procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52412877 http://dx.doi.org/10.1007/s00246-012-0618-8 VL - 34 ID - 996 ER - TY - JOUR AB - Single ventricle congenital heart disease (SV CHD) has transformed from a nearly universally fatal condition to a chronic illness. As the number of adults living with SV CHD continues to increase, there needs to be an understanding of health care resource utilization (HCRU), particularly for noncardiac conditions, for this patient population. We performed a retrospective database review of the University HealthSystem Consortium Clinical Database/Resource Manager for adult patients with SV CHD hospitalized for noncardiac conditions from January 2011 to November 2014. Patients with SV CHD were identified using International Classification of Disease (ICD)-9 codes associated with SV CHD (hypoplastic left heart, tricuspid atresia, and SV) and stratified into 2 groups by age (18 to 29 years and 30 to 40 years). Direct cost, length of stay (LOS), intensive care unit (ICU) admission rate and mortality data were compared with age-matched patients without CHD. There were 2,083,651 non-CHD and 590 SV CHD admissions in Group 1 and 2,131,046 non-CHD and 297 SV CHD admissions in Group 2. There was no difference in LOS in Group 1, but there were higher costs for several diagnoses. LOS and costs were higher for several diagnoses in Group 2. ICU admission rate and in-hospital mortality were higher for several diagnoses for patients with SV CHD in both groups. In conclusion, adults with SV CHD admitted for noncardiac diagnoses have higher HCRU (longer LOS and higher ICU admission rates) compared with similarly aged patients without CHD. These findings stress the importance of good primary care in this population with complex, chronic cardiac disease to prevent hospitalizations and higher HCRU. AD - Department of Pediatrics (Cardiology), University of Arizona, Tucson, Arizona. Electronic address: mseckeler@peds.arizona.edu. Arizona Pediatric Cardiology, Phoenix Children's Hospital, Phoenix, Arizona. Department of Pediatrics, University of Arizona, Tucson, Arizona. University of Arizona College of Medicine, Tucson, Arizona. Department of Pediatrics (Cardiology), University of Arizona, Tucson, Arizona. Sarver Heart Center, University of Arizona, Tucson, Arizona. AN - 26455384 AU - Seckeler, M. D. AU - Moe, T. G. AU - Thomas, I. D. AU - Meziab, O. AU - Andrews, J. AU - Heller, E. AU - Klewer, S. E. DA - Dec 1 DB - PubMed DO - 10.1016/j.amjcard.2015.09.008 DP - NLM ET - 2015/10/13 IS - 11 KW - Adolescent Adult Comorbidity Heart Defects, Congenital/*epidemiology/mortality Heart Ventricles/*abnormalities Hospital Costs Hospitalization/economics/*statistics & numerical data Humans Intensive Care Units/statistics & numerical data Length of Stay Retrospective Studies *Survivors Young Adult LA - eng N1 - 1879-1913 Seckeler, Michael D Moe, Tabitha G Thomas, Ian D Meziab, Omar Andrews, Jennifer Heller, Elissa Klewer, Scott E Journal Article United States Am J Cardiol. 2015 Dec 1;116(11):1756-61. doi: 10.1016/j.amjcard.2015.09.008. Epub 2015 Sep 10. PY - 2015 SN - 0002-9149 SP - 1756-61 ST - Hospital Resource Utilization for Common Noncardiac Diagnoses in Adult Survivors of Single Cardiac Ventricle T2 - Am J Cardiol TI - Hospital Resource Utilization for Common Noncardiac Diagnoses in Adult Survivors of Single Cardiac Ventricle VL - 116 ID - 310 ER - TY - JOUR AB - OBJECTIVE: To determine incidence and clinical characteristics of hospital-associated venous thromboembolism (VTE) in pediatric patients. STUDY DESIGN: A retrospective analysis of patients with hospital-associated VTE at the Johns Hopkins Hospital from 1994 to 2009 was performed. Clinical characteristics of patients aged 21 years and younger who developed VTE symptoms after 2 days of hospitalization or <90 days after hospital discharge were examined. International Classification of Diseases, Ninth Revision codes were used to categorize patients with complex chronic medical conditions and trauma. RESULTS: There were 270 episodes of hospital-associated VTE in 90,485 admissions (rate 30 per 10,000 admissions). Young adults (18-21 years) and adolescents (14-17 years) had significantly increased rates of VTE compared with children (2-9 years) (incidence rate ratio [IRR] 7.7, 95% CI 5.1-12.0; IRR 4.3, 95% CI 2.7-6.8, respectively). A central venous catheter (CVC) was present in 50% of patients, and a surgical procedure was performed in 45% of patients before VTE diagnosis. For patients without a CVC, trauma was the most common admitting diagnosis. CVC-related VTE was diagnosed most frequently in infants (<1 year old) and in patients with malignancy. Renal and cardiac diseases were associated with the highest rates of VTE (51 and 48 per 10,000, respectively). Rates were significantly higher among those with ≥ 4 medical conditions compared with those with 1 medical condition (IRR 4.0, 95% CI 1.4-8.9). CONCLUSION: Older age and multiple medical conditions were associated with increased rates of hospital-associated VTE. These data can contribute to the design of future clinical trials to prevent hospital-associated VTE in high-risk children. AD - Division of Pediatric Hematology, Johns Hopkins University, Baltimore, MD. Electronic address: ctakemot@jhmi.edu. Division of Pediatric Hematology, Johns Hopkins University, Baltimore, MD. Division of Quality and Safety, Department of Pediatrics, Johns Hopkins University, Baltimore, MD. Division of Pediatric Hematology, Johns Hopkins University, Baltimore, MD; Pediatric Thrombosis Program, All Children's Hospital-Johns Hopkins Medicine, St. Petersburg, FL. Division of Pediatric Hematology, Johns Hopkins University, Baltimore, MD; Division of Adult Hematology, The Johns Hopkins Hospital, Baltimore, MD. Division of Adult Hematology, The Johns Hopkins Hospital, Baltimore, MD. AN - 24332452 AU - Takemoto, C. M. AU - Sohi, S. AU - Desai, K. AU - Bharaj, R. AU - Khanna, A. AU - McFarland, S. AU - Klaus, S. AU - Irshad, A. AU - Goldenberg, N. A. AU - Strouse, J. J. AU - Streiff, M. B. DA - Feb DB - PubMed DO - 10.1016/j.jpeds.2013.10.025 DP - NLM ET - 2013/12/18 IS - 2 KW - Adolescent Child Child, Preschool Female *Hospitalization Humans Incidence Infant Kidney Diseases/*complications/epidemiology Male Neoplasms/*complications/epidemiology Prognosis Retrospective Studies United States/epidemiology Venous Thromboembolism/*epidemiology/etiology Wounds and Injuries/*complications/epidemiology Young Adult Ccc Cvc Central venous catheter Complex chronic condition Dvt Deep venous thrombosis Hlhs Hypoplastic left heart syndrome Icd-9 Irr Incidence rate ratio International Classification of Diseases, Ninth Revision Pe Pulmonary embolism Vte Venous thromboembolism LA - eng N1 - 1097-6833 Takemoto, Clifford M Sohi, Sajeet Desai, Kruti Bharaj, Raman Khanna, Anuj McFarland, Susan Klaus, Sybil Irshad, Alia Goldenberg, Neil A Strouse, John J Streiff, Michael B Comparative Study Journal Article United States J Pediatr. 2014 Feb;164(2):332-8. doi: 10.1016/j.jpeds.2013.10.025. Epub 2013 Dec 12. PY - 2014 SN - 0022-3476 SP - 332-8 ST - Hospital-associated venous thromboembolism in children: incidence and clinical characteristics T2 - J Pediatr TI - Hospital-associated venous thromboembolism in children: incidence and clinical characteristics VL - 164 ID - 292 ER - TY - JOUR AB - BACKGROUND: Poor long-term outcomes are expected after Fontan surgery, but these perspectives have been tainted by the poorly functioning Fontans suffering from arrhythmias. No predictions of outcome can be quoted to the increasing number of Fontan patients free from arrhythmic complications. The parameters determining improved exercise capacity and quality of life in this subgroup are yet unknown. METHODS: Fontan survivors from our institution and living in Victoria were invited to participate in the study if they were more than 10 years of age, and free of arrhythmias. A mean of 17 +/- 4 years after Fontan, 36 patients, 23 with a classical atriopulmonary connection (AP) and 13 with a lateral tunnel (LT) underwent transthoracic echocardiography, cycloergometer exercise study, neurohumoral screening, and assessment of quality of life. RESULTS: The only factor predicting worse exercise capacity was the type of Fontan performed; patients with LT having better exercise capacity than those with AP (percentage of predicted anaerobic threshold: 88 +/- 14% vs 72 +/- 14%, p < 0.005; percentage of predicted VO(2)max: 62 +/- 8% vs 54 +/- 7%, p < 0.005). Endothelin-1 levels were elevated in all patients (2.9 pmol/L, 2.5 to 3.7). Responses from the quality of life measures placed our Fontan cohort mainly within the normal population range. None of the preoperative and postoperative variables adversely affected patients' quality of life. CONCLUSIONS: The anaerobic threshold of arrhythmia-free Fontan patients operated with the lateral tunnel technique was relatively preserved. Despite restricted exercise capacity, Fontan patients, provided that they are free of arrhythmias, have a normal quality of life reflected in their reports of psychiatric symptoms and family relationships. AD - Department of Cardiac Surgery, Royal Children's Hospital, Parkville, Victoria, Australia. yves.dudekem@rch.org.au AN - 19932269 AU - d'Udekem, Y. AU - Cheung, M. M. AU - Setyapranata, S. AU - Iyengar, A. J. AU - Kelly, P. AU - Buckland, N. AU - Grigg, L. E. AU - Weintraub, R. G. AU - Vance, A. AU - Brizard, C. P. AU - Penny, D. J. DA - Dec DB - PubMed DO - 10.1016/j.athoracsur.2009.07.079 DP - NLM ET - 2009/11/26 IS - 6 KW - Adolescent Arrhythmias, Cardiac/*epidemiology Child Child, Preschool Disease-Free Survival Exercise Tolerance/*physiology Female Follow-Up Studies *Fontan Procedure Heart Defects, Congenital/mortality/*surgery Humans Incidence Male Postoperative Period *Quality of Life Retrospective Studies Time Factors Treatment Outcome Victoria/epidemiology Young Adult LA - eng N1 - 1552-6259 d'Udekem, Yves Cheung, Michael M H Setyapranata, Stella Iyengar, Ajay J Kelly, Patricia Buckland, Naomi Grigg, Leeanne E Weintraub, Robert G Vance, Alasdair Brizard, Christian P Penny, Dan J Comparative Study Journal Article Netherlands Ann Thorac Surg. 2009 Dec;88(6):1961-9. doi: 10.1016/j.athoracsur.2009.07.079. PY - 2009 SN - 0003-4975 SP - 1961-9 ST - How good is a good Fontan? Quality of life and exercise capacity of Fontans without arrhythmias T2 - Ann Thorac Surg TI - How good is a good Fontan? Quality of life and exercise capacity of Fontans without arrhythmias VL - 88 ID - 216 ER - TY - JOUR AB - Background: Poor long-term outcomes are expected after Fontan surgery, but these perspectives have been tainted by the poorly functioning Fontans suffering from arrhythmias. No predictions of outcome can be quoted to the increasing number of Fontan patients free from arrhythmic complications. The parameters determining improved exercise capacity and quality of life in this subgroup are yet unknown. Methods: Fontan survivors from our institution and living in Victoria were invited to participate in the study if they were more than 10 years of age, and free of arrhythmias. A mean of 17 ± 4 years after Fontan, 36 patients, 23 with a classical atriopulmonary connection (AP) and 13 with a lateral tunnel (LT) underwent transthoracic echocardiography, cycloergometer exercise study, neurohumoral screening, and assessment of quality of life. Results: The only factor predicting worse exercise capacity was the type of Fontan performed; patients with LT having better exercise capacity than those with AP (percentage of predicted anaerobic threshold: 88 ± 14% vs 72 ± 14%, p < 0.005; percentage of predicted Vo2max: 62 ± 8% vs 54 ± 7%, p < 0.005). Endothelin-1 levels were elevated in all patients (2.9 pmol/L, 2.5 to 3.7). Responses from the quality of life measures placed our Fontan cohort mainly within the normal population range. None of the preoperative and postoperative variables adversely affected patients' quality of life. Conclusions: The anaerobic threshold of arrhythmia-free Fontan patients operated with the lateral tunnel technique was relatively preserved. Despite restricted exercise capacity, Fontan patients, provided that they are free of arrhythmias, have a normal quality of life reflected in their reports of psychiatric symptoms and family relationships. © 2009 The Society of Thoracic Surgeons. AD - Y. d'Udekem, Department of Cardiac Surgery, Royal Children's Hospital, Department of Pediatrics of the University of Melbourne, Parkville, Vic., Australia AU - d'Udekem, Y. AU - Cheung, M. M. H. AU - Setyapranata, S. AU - Iyengar, A. J. AU - Kelly, P. AU - Buckland, N. AU - Grigg, L. E. AU - Weintraub, R. G. AU - Vance, A. AU - Brizard, C. P. AU - Penny, D. J. DB - Embase Medline DO - 10.1016/j.athoracsur.2009.07.079 IS - 6 KW - endothelin 1 adolescent anaerobic threshold article atriopulmonary connection child clinical article cohort analysis controlled study ergometer exercise female Fontan procedure heart arrhythmia human male population prediction preoperative evaluation preschool child priority journal quality of life school child screening survivor transthoracic echocardiography LA - English M3 - Article N1 - L355778746 2010-01-27 PY - 2009 SN - 0003-4975 SP - 1961-1969 ST - How Good Is a Good Fontan? Quality of Life and Exercise Capacity of Fontans Without Arrhythmias T2 - Annals of Thoracic Surgery TI - How Good Is a Good Fontan? Quality of Life and Exercise Capacity of Fontans Without Arrhythmias UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L355778746 http://dx.doi.org/10.1016/j.athoracsur.2009.07.079 VL - 88 ID - 1163 ER - TY - JOUR AB - BACKGROUND: Little is known about adults living with a Fontan circulation's concerns outside the scope of their clinical outcomes. We examined adults with a Fontan circulations' greatest concerns, as well as their concerns around anti-coagulation, pregnancy and finances. METHODS: Adults with a Fontan circulation in the Australian and New Zealand Fontan Registry were invited to complete an anonymous online survey, of which 57 participated. A qualitative method approach using thematic analyses was used. RESULTS: The greatest concerns for adults living with a Fontan circulation were fear of death/uncertainty around life expectancy which for many individuals colored their concerns around physical health, pregnancy and having children, quality of life and finances. Improving information about outcomes to patients with a Fontan circulation might alleviate uncertainties about their future. CONCLUSIONS: Fear of death is the primary concern of adults with a Fontan circulation. It may require improved communication and targeted psychological interventions. Physical exercise incorporated as part of their lifestyle should be encouraged to alleviate physical concerns and also improve psychological well-being. AD - Murdoch Children's Research Institute, Melbourne, Victoria, Australia. Murdoch Children's Research Institute, Melbourne, Victoria, Australia; Australian and New Zealand Fontan Advisory Committee, Australia and New Zealand. Australian and New Zealand Fontan Advisory Committee, Australia and New Zealand. Murdoch Children's Research Institute, Melbourne, Victoria, Australia; Australian and New Zealand Fontan Advisory Committee, Australia and New Zealand; Cardiac Surgery Department, Royal Children's Hospital, Melbourne, Victoria, Australia; Department of Pediatrics, Faculty of Medicine, The University of Melbourne, Melbourne, Victoria, Australia. Electronic address: yves.dudekem@rch.org.au. AN - 29622455 AU - du Plessis, K. AU - Peters, R. AU - King, I. AU - Robertson, K. AU - Mackley, J. AU - Maree, R. AU - Stanley, T. AU - Pickford, L. AU - Rose, B. AU - Orchard, M. AU - Stewart, H. AU - d'Udekem, Y. DA - Jun 1 DB - PubMed DO - 10.1016/j.ijcard.2018.01.098 DP - NLM ET - 2018/04/07 KW - Adolescent Adult Australia/epidemiology Fear/*psychology Female Fontan Procedure/*psychology/*trends Humans Male Middle Aged New Zealand/epidemiology Registries *Self Report Time Factors Young Adult *Adult congenital *Congenital heart disease *Fontan *Qualitative research LA - eng N1 - 1874-1754 du Plessis, Karin Peters, Rebecca King, Ingrid Robertson, Kirsty Mackley, Jonathan Maree, Rachel Stanley, Tracy Pickford, Louise Rose, Brian Orchard, Matthew Stewart, Helen d'Udekem, Yves Journal Article Netherlands Int J Cardiol. 2018 Jun 1;260:54-59. doi: 10.1016/j.ijcard.2018.01.098. PY - 2018 SN - 0167-5273 SP - 54-59 ST - "How long will I continue to be normal?" Adults with a Fontan circulation's greatest concerns T2 - Int J Cardiol TI - "How long will I continue to be normal?" Adults with a Fontan circulation's greatest concerns VL - 260 ID - 125 ER - TY - JOUR AB - An online survey for parents of children with congenital heart disease (CHD) was developed to study the perceptions and experiences of parents when receiving the diagnosis. The survey was distributed to online support groups. A total of 841 responses from parents of children with CHD in the United States were received over a 4-week period in 2010. The authors hypothesized that the counseling and demeanor of the pediatric cardiologist (PC) may be important factors in determining whether parents of children with CHD seek second opinions, and that the terminology used in counseling may be variably interpreted. Of the 841 respondents, 349 (41 %) received the diagnosis prenatally. A minority of the respondents received: support group information (14 %), Internet resources (21 %), success rates at other hospitals (16 %), or maximum ages of survivors (29 %). Among 26 % of the parents who reported seeking a second opinion from another PC, the majority (71 %) chose the second PC for long-term follow-up care. Those receiving a prenatal diagnosis were more likely to seek a second opinion than those receiving the diagnosis postnatally (32 vs 22 %; p < 0.01). Parents' perception of the PC's compassion and empathy was inversely related to the likelihood of seeking a second opinion. Parents were more likely to seek a second opinion when they were not optimistic about their child's life expectancy, felt pressured by the PC to terminate the pregnancy, were told that their child's death was "somewhat" or "very" likely, or were told the child's CHD was "rare" (all p < 0.01). Two thirds (66 %) of the respondents were told that their child's condition was "rare." The majority of these (77 %) reported that the term was used by the PC. "Rare" was interpreted as "occurring in less than a million births" by 25 %, and as "few or no other people alive with this defect" by 27 %. Parental interpretation of "rare" was unrelated to their levels of education. As reported by the respondents, 13 % felt pressured to terminate the pregnancy by the PC. Those with hypoplastic left heart syndrome were more likely to report feeling pressure to terminate the pregnancy by the PC (21 vs 9 %; p < 0.001) or the perinatologist (23 vs 14 %; p = 0.026). The approach to counseling and the demeanor of the PC have important implications for parents' perceptions of their child's chance of survival. The information given at diagnosis, the manner in which it is presented, and the parents' understanding and interpretation of that information are critical factors in shaping parents' perceptions and management decisions. AD - California Heart Connection, Irvine, CA, USA, DebraHiltonKamm@gmail.com. AN - 23925415 AU - Hilton-Kamm, D. AU - Sklansky, M. AU - Chang, R. K. DA - Feb DB - PubMed DO - 10.1007/s00246-013-0765-6 DP - NLM ET - 2013/08/09 IS - 2 KW - Adolescent Adult Child Child, Preschool Counseling/*methods Female Heart Defects, Congenital/*diagnosis Humans Infant *Internet Male Parents/*psychology Pregnancy Prenatal Diagnosis Retrospective Studies *Surveys and Questionnaires Truth Disclosure/*ethics United States Young Adult LA - eng N1 - 1432-1971 Hilton-Kamm, Debra Sklansky, Mark Chang, Ruey-Kang Journal Article United States Pediatr Cardiol. 2014 Feb;35(2):239-52. doi: 10.1007/s00246-013-0765-6. Epub 2013 Aug 8. PY - 2014 SN - 0172-0643 SP - 239-52 ST - How not to tell parents about their child's new diagnosis of congenital heart disease: an Internet survey of 841 parents T2 - Pediatr Cardiol TI - How not to tell parents about their child's new diagnosis of congenital heart disease: an Internet survey of 841 parents VL - 35 ID - 277 ER - TY - JOUR AB - OBJECTIVE: We describe a hybrid approach to the treatment of aortic obstruction after stage 1 palliation (S1P) of hypoplastic left heart syndrome. BACKGROUND: Recurrent aortic obstruction is a common problem after S1P of hypoplastic left heart syndrome. Even mild aortic obstruction is poorly tolerated so early and definitive therapy is desirable. Although stent implantation is an effective treatment for aortic obstruction in older children and adults, technical issues due to small vessels and concerns regarding future potential for expansion have generally precluded the use of stents in this setting. METHODS: Six patients underwent hybrid aortic reconstruction (HAR) in the operating room or catheterization laboratory, with the interventional cardiologist and cardiac surgeon working in cooperation. RESULTS: Patients had a mean weight of 5.8 kg (2.9-7.7) and a mean age of 5.6 months (0.5-12.9) at the time of HAR. Five patients had undergone prior balloon angioplasty at a mean age of 2.8 months (2.1-3.5), and five had moderately depressed single ventricular function prior to HAR. The balloons used had a diameter of 7-10 mm and introducer sheath size ranged from 6 to 10 F. There were no immediate or late procedure related complications. Stent redilation was performed in 5 patients for relief of recurrent obstruction or to keep pace with somatic growth. At a median follow up of 4.8 years (0.2-7.9), there were 3 patients progressing well after Fontan palliation and 3 deaths. CONCLUSIONS: HAR allows for placement of stents that can ultimately reach adult size in small infants who have recurrent aortic obstruction after balloon angioplasty following S1P. Advantages include freedom from delivery sheath constraints when determining stent type/size, facilitation of precise stent position, and avoidance of vascular damage or hemodynamic compromise during the procedure. Longer follow-up and larger experience are required to determine if this therapy will provide a long-term solution to this difficult problem. AD - University of Nebraska/Creighton University Joint Division of Pediatric Cardiology, Children's Hospital and Medical Center, Omaha, Nebraska, USA. AN - 21456031 AU - Kutty, S. AU - Burke, R. P. AU - Hannan, R. L. AU - Zahn, E. M. DA - Jul 1 DB - PubMed DO - 10.1002/ccd.22964 DP - NLM ET - 2011/04/02 IS - 1 KW - *Angioplasty, Balloon/adverse effects/instrumentation/mortality Aortic Diseases/etiology/mortality/physiopathology/*therapy Arterial Occlusive Diseases/etiology/mortality/physiopathology/*therapy Florida Fontan Procedure/*adverse effects/mortality Humans Hypoplastic Left Heart Syndrome/mortality/physiopathology/*surgery Infant Kaplan-Meier Estimate Norwood Procedures/*adverse effects/mortality Palliative Care Prosthesis Design Recurrence Stents Time Factors Treatment Outcome LA - eng N1 - 1522-726x Kutty, Shelby Burke, Redmond P Hannan, Robert L Zahn, Evan M Journal Article United States Catheter Cardiovasc Interv. 2011 Jul 1;78(1):93-100. doi: 10.1002/ccd.22964. Epub 2011 Mar 31. PY - 2011 SN - 1522-1946 SP - 93-100 ST - Hybrid aortic reconstruction for treatment of recurrent aortic obstruction after stage 1 single ventricle palliation: medium term outcomes and results of redilation T2 - Catheter Cardiovasc Interv TI - Hybrid aortic reconstruction for treatment of recurrent aortic obstruction after stage 1 single ventricle palliation: medium term outcomes and results of redilation VL - 78 ID - 279 ER - TY - JOUR AB - Objective The objective of a hybrid approach to staged palliation of single-ventricle anomalies is designed to minimize the trauma of the first stage. However, the second stage is a complex procedure that may negate the advantages of the first stage. We sought to devise a "hybrid" approach to the second stage when aortic outflow is expected to remain unobstructed. Methods The procedure involves a simple incision into the main pulmonary artery, dilation/stenting of the ductal continuation, formation of a stented baffle between the branch pulmonary arteries' orifices, and a bidirectional Glenn connection. It avoids dissection of the distal arch and ductal continuation and obviates the need for a Damus-Kaye-Stansel connection. We carried out this procedure in 2 patients, one with unbalanced atrioventricular canal and the other with mitral atresia. Results Both patients underwent an uncomplicated operative procedure. Both patients were successfully weaned from the ventilator, with no clinically evident neurologic injury. The first patient died of complications related to thrombosis of the left pulmonary artery before initiation of anticoagulation. The second patient is alive and well 1 year postoperation with no obstruction to either systemic or pulmonary flow and no baffle leak and good right ventricle function. Conclusions This hybrid comprehensive stage II operation appears feasible and technically simpler than the conventional comprehensive stage II procedure. It is applicable to a subset of single-ventricle cases in which aortic outflow is anticipated to remain unobstructed. We recommend early postoperative anticoagulation to avoid early left pulmonary artery thrombosis. AD - W.M. Decampli, Division of Cardiothoracic Surgery, Heart Center at Arnold Palmer Hospital for Children, 92 W Miller St, Orlando, FL, United States AU - Decampli, W. M. AU - Fleishman, C. E. AU - Nykanen, D. G. DB - Embase Medline DO - 10.1016/j.jtcvs.2014.11.081 IS - 4 KW - acetylsalicylic acid enoxaparin heparin tissue plasminogen activator anastomosis anticoagulant therapy aortic arch aortic root article ascending aorta atrioventricular canal balloon catheter balloon dilatation bare metal stent cardiac patient cardiopulmonary bypass case report computed tomographic angiography descending aorta Down syndrome echocardiography extracorporeal oxygenation femoral artery heart catheterization heart right ventricle function heart single ventricle heart surgery heart ventricle enddiastolic pressure human incision laparoscopic video camera lung infection mitral valve atresia newborn newborn disease newborn surgery peripheral artery stent priority journal pulmonary artery radial artery sternotomy superior cava vein surgical approach systemic circulation Opta Pro balloon Palmaz Genesis 2510B PG 1910B LA - English M3 - Article N1 - L601455505 2015-01-23 2015-05-11 PY - 2015 SN - 1097-685X 0022-5223 SP - 1095-1100 ST - Hybrid approach to the comprehensive stage II operation in a subset of single-ventricle variants T2 - Journal of Thoracic and Cardiovascular Surgery TI - Hybrid approach to the comprehensive stage II operation in a subset of single-ventricle variants UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L601455505 http://dx.doi.org/10.1016/j.jtcvs.2014.11.081 VL - 149 ID - 870 ER - TY - JOUR AB - Objectives: Open heart surgery for interrupted aortic arch in the neonatal period is still a high-risk procedure related in part to patient factors such as low birth weight, other morphologic anomalies, and, especially, small aortic valve size. Recently, we performed hybrid palliation with bilateral pulmonary artery banding and ductal stenting as the first-stage palliation for such cases. In this study, the outcomes of this procedure were examined. Methods: Six cases of interrupted aortic arch with a small aortic valve underwent the hybrid procedure in the neonatal period in our institute from 2010 to 2015 (mean age: 6.8 days, mean body weight: 3.2 kg, mean z score of the aortic valve annulus: −8.3). Their postoperative clinical courses and results of the second-stage surgery were evaluated. Results: No mortality or severe morbidity was seen in association with initial hybrid palliation. Five of six patients were discharged from the hospital; the one exception had a significant urinary tract anomaly. None needed an additional catheter intervention or surgical procedure postoperatively. All surviving patients underwent second-stage surgery; three had biventricular repair by the conventional method or Damus-Kaye-Stansel anastomosis with the Rastelli procedure and the other three proceeded toward staged Fontan reconstruction. Growth of the aortic valve was seen in four patients, and increased indexed left ventricle volume was recognized in one after the palliation. Conclusion: Hybrid palliation could be useful not only to avoid high-risk neonatal surgery but also to allow for eventual selection of the second-stage surgery based on the observations of potential interval development of left ventricular structures. AD - Y. Uno, 4-19-4 Egota Nakano-ku, Tokyo, Japan AU - Uno, Y. AU - Masuoka, A. AU - Hotoda, K. AU - Katogi, T. AU - Suzuki, T. DB - Embase Medline DO - 10.1177/2150135117690125 IS - 3 KW - anxiety aortic arch surgery article bariatric surgery echocardiography female follow up heart ventricle septum defect hospital human male newborn priority journal respiratory tract infection retrospective study scoring system subvalvular aortic stenosis thorax radiography LA - English M3 - Article N1 - L616313860 2017-05-25 2020-03-03 PY - 2017 SN - 2150-136X 2150-1351 SP - 332-336 ST - Hybrid Palliation for Interrupted Aortic Arch With Small Aortic Valve T2 - World Journal for Pediatric and Congenital Heart Surgery TI - Hybrid Palliation for Interrupted Aortic Arch With Small Aortic Valve UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L616313860 http://dx.doi.org/10.1177/2150135117690125 VL - 8 ID - 758 ER - TY - JOUR AD - S.-H. Kim, Department of Cardiology, Shizuoka Children’s Hospital, Shizuoka, Japan AU - Kim, S. H. DB - Embase DO - 10.1007/s11748-013-0344-z IS - 8 KW - nitrogen article cardiologist cardiovascular surgery congenital heart disease echocardiography Fontan procedure interatrial septum heart catheterization heart left atrium human hybrid hypoplastic left heart syndrome intact heart atrium septum interventional cardiovascular procedure lung artery banding newborn Norwood procedure pediatric cardiology procedures teamwork LA - English M3 - Article N1 - L52906358 2013-12-11 2015-08-25 PY - 2015 SN - 1863-6713 1863-6705 SP - 476-477 ST - Hybrid procedure and collaborative approach for hypoplastic left heart syndrome T2 - General Thoracic and Cardiovascular Surgery TI - Hybrid procedure and collaborative approach for hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52906358 http://dx.doi.org/10.1007/s11748-013-0344-z VL - 63 ID - 855 ER - TY - JOUR AB - Background: Minimally invasive operations and percutaneous interventions are well-accepted options in the treatment of congenital heart defects. However, percutaneous interventions may be associated with an increased risk due to limited vascular access or a very tortuous catheter course. In these cases, combining operative and interventional approaches with direct puncture of the heart or the great vessels may facilitate implantation of even large devices. Furthermore, in some situations, cardiopulmonary bypass or circulatory arrest can be omitted when doing a hybrid procedure. Patients: Between January 2000 and April 2007 17 patients were operated in a hybrid fashion. Age ranged from 14 days to 45 years. Operative procedures consisted of implantation of an atrial septal defect occluder via direct puncture of the right atrium (n = 4), closure of a ventricular septal defect via direct puncture of the right ventricle (n = 1), implantation of isthmus stents via the ascending aorta (n = 5), redilation of an isthmus stent (n = 1), redilation of a ductal stent (n = 1), angioplasty of a pulmonary artery stenosis (n = 1), interventional occlusion of an intrahepatic porto-caval shunt (n = 1), stent implantation into the right pulmonary artery (n = 1) and into the right ventricular outflow tract (n = 1) under direct vision as well as atrioseptoplasty combined with a bilateral pulmonary artery banding in one newborn with a single ventricle and very low birth weight (n = 1). Results: The planned intervention could be performed in all cases under the assistance of intraoperative fluoroscopy, transesophageal or epicardial echocardiography, or under direct vision. In all cases, the primary hemodynamic objectives were achieved. Conclusion: In selected patients, the combination of a surgical procedure and a percutaneous intervention may help to reduce both operative and interventional risks. This concept may enable new treatment options, especially in patients with complex congenital heart defects or complex vascular situations. © 2008 European Association for Cardio-Thoracic Surgery. AD - C. Schmitz, Department of Cardiac Surgery, University of Munich, Munich, Germany AU - Schmitz, C. AU - Esmailzadeh, B. AU - Herberg, U. AU - Lang, N. AU - Sodian, R. AU - Kozlik-Feldmann, R. AU - Welz, A. AU - Breuer, J. DB - Embase Medline DO - 10.1016/j.ejcts.2008.06.028 IS - 4 KW - heparin adolescent adult angiography angioplasty aortic coarctation article ascending aorta Blalock Taussig shunt cardiovascular surgery child clinical article congenital heart malformation echocardiography endoprosthesis Fallot tetralogy fluoroscopy Glenn shunt heart atrium septum defect heart right atrium heart right ventricle heart right ventricle outflow tract heart single ventricle heart ventricle septum defect hemodynamics human hybrid congenital cardiovascular surgery implantation infant low birth weight lung artery banding newborn newborn surgery patent foramen ovale portocaval shunt priority journal pulmonary artery stenosis retrospective study risk reduction stent surgical technique transesophageal echocardiography treatment indication treatment outcome Amplatzer CP-stent 8Z16 LA - English M3 - Article N1 - L50227390 2008-11-03 PY - 2008 SN - 1010-7940 SP - 718-725 ST - Hybrid procedures can reduce the risk of congenital cardiovascular surgery T2 - European Journal of Cardio-thoracic Surgery TI - Hybrid procedures can reduce the risk of congenital cardiovascular surgery UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50227390 http://dx.doi.org/10.1016/j.ejcts.2008.06.028 VL - 34 ID - 1197 ER - TY - JOUR AB - Hybrid pediatric cardiac surgery is an emerging field that combines skills and techniques used by pediatric cardiac surgeons and interventional pediatric cardiologists. This article describes the emerging indications and techniques in hybrid pediatric cardiac surgery and discusses potential future applications. It focuses on peratrial and perventricular septal defect closure, intraoperative stenting, hybrid stage I palliation for hypoplastic left heart syndrome, and percutaneous valve implantation. © 2005 Elsevier Inc. All rights reserved. AD - E.A. Bacha, Dept. Congen./Pediat. Cardiac Surg., Univ. of Chicago Children's Hospital, 5841 S. Maryland Ave., Chicago, IL 60637, United States AU - Bacha, E. A. AU - Hijazi, Z. M. DB - Embase DO - 10.1053/j.pcsu.2005.01.001 IS - 1 KW - artery compression article heart atrium septum defect heart surgery heart valve replacement heart ventricle septum defect human hypoplastic left heart syndrome intraoperative period mediastinitis palliative therapy pediatric surgery pleura effusion postoperative complication skill stent surgeon surgical technique treatment indication LA - English M3 - Article N1 - L40468457 2005-04-20 PY - 2005 SN - 1092-9126 SP - 78-85 ST - Hybrid procedures in pediatric cardiac surgery T2 - Pediatric Cardiac Surgery Annual TI - Hybrid procedures in pediatric cardiac surgery UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L40468457 http://dx.doi.org/10.1053/j.pcsu.2005.01.001 VL - 8 ID - 1279 ER - TY - JOUR AB - Introduction: Procedural cooperation between cardiac surgeon and interventional cardiologist to facilitate interventions such as device delivery or angioplasty (hybrid procedure) has become increasingly common in the management of patients with congenital heart disease. Design: Data were prospectively collected using a multicenter registry (C3PO). Between February 2007 and December 2008, seven institutions submitted data regarding 7019 cardiac catheterization procedures. Procedural data and adverse events (AEs) of 128 hybrid procedures were evaluated. Results: There was significant variability in the number of hybrid procedures per center, ranging from one to 89 with a median of eight. A total of 60% of interventional (vs. strictly diagnostic) hybrid procedures were performed by one center. The median weight was 3.7 kg (0.7-86 kg). Single-ventricle circulation was present in 60% of the procedures. Hybrid procedures included: patent ductus arteriosus (PDA) stent placement (n = 55), vascular rehabilitation (n = 25), ventricular septal defect (VSD) device closure (n = 7), valvotomy (n = 3), and diagnostic hybrid procedures (n = 38). Sixteen AEs occurred in 15/128 (12%) procedures. These included minor or trivial AEs (n = 9), moderate AEs (n = 5), major AEs (n = 1), and catastrophic AEs (n = 1). The type of AE documented included arrhythmias (n = 6), hypoxia or hypotension (n = 3), vessel or cardiac trauma (n = 2), and other events (n = 5). Of documented AEs, 9/16 (56%) were classified as not preventable, 6/16 (38%) as possibly preventable, and 1/16 (6%) as preventable. The incidence of AE related to PDA stent placement with surgical exposure (5/50, 10%) was significantly lower when compared with PDA stent placement performed percutaneously (4/5, 80%, P= .002). Conclusion: Hybrid procedures appear to have a low incidence of associated major AEs. PDA stent placement performed as a palliation of hypoplastic left heart syndrome (HLHS) or complex single/two ventricle patients may have a lower incidence of AEs if performed using a direct approach with surgical exposure rather than a percutaneous approach. Accurate definitions of these innovative procedures are required to facilitate prospective data collection. © 2010 Copyright the Authors. Journal Compilation © 2010 Wiley Periodicals, Inc. AD - Department of Cardiology, Nationwide Children's Hospital, Columbus, OH, United States Department of Cardiology, Cincinnati Children's Hospital, Cincinnati, OH, United States Department of Cardiology, Children's Hospital Boston, Boston, MA, United States Department of Cardiology, Children's Hospital of Pittsburgh Pittsburgh, Pa, United States AU - Holzer, R. AU - Marshall, A. AU - Kreutzer, J. AU - Hirsch, R. AU - Chisolm, J. AU - Hill, S. AU - Galantowicz, M. AU - Phillips, A. AU - Cheatham, J. AU - Bergerson, L. DB - Scopus DO - 10.1111/j.1747-0803.2010.00416.x IS - 3 KW - Adverse events Cardiac catheterization Hybrid procedures M3 - Article N1 - Cited By :25 Export Date: 15 June 2020 PY - 2010 SP - 233-242 ST - Hybrid procedures: Adverse events and procedural characteristics - results of a multi-institutional registry T2 - Congenital Heart Disease TI - Hybrid procedures: Adverse events and procedural characteristics - results of a multi-institutional registry UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-77954111647&doi=10.1111%2fj.1747-0803.2010.00416.x&partnerID=40&md5=b895d4909996729a2a2ba74dfc700a05 VL - 5 ID - 2153 ER - TY - JOUR AB - INTRODUCTION: Procedural cooperation between cardiac surgeon and interventional cardiologist to facilitate interventions such as device delivery or angioplasty (hybrid procedure) has become increasingly common in the management of patients with congenital heart disease. DESIGN: Data were prospectively collected using a multicenter registry (C3PO). Between February 2007 and December 2008, seven institutions submitted data regarding 7019 cardiac catheterization procedures. Procedural data and adverse events (AEs) of 128 hybrid procedures were evaluated. RESULTS: There was significant variability in the number of hybrid procedures per center, ranging from one to 89 with a median of eight. A total of 60% of interventional (vs. strictly diagnostic) hybrid procedures were performed by one center. The median weight was 3.7 kg (0.7-86 kg). Single-ventricle circulation was present in 60% of the procedures. Hybrid procedures included: patent ductus arteriosus (PDA) stent placement (n = 55), vascular rehabilitation (n = 25), ventricular septal defect (VSD) device closure (n = 7), valvotomy (n = 3), and diagnostic hybrid procedures (n = 38). Sixteen AEs occurred in 15/128 (12%) procedures. These included minor or trivial AEs (n = 9), moderate AEs (n = 5), major AEs (n = 1), and catastrophic AEs (n = 1). The type of AE documented included arrhythmias (n = 6), hypoxia or hypotension (n = 3), vessel or cardiac trauma (n = 2), and other events (n = 5). Of documented AEs, 9/16 (56%) were classified as not preventable, 6/16 (38%) as possibly preventable, and 1/16 (6%) as preventable. The incidence of AE related to PDA stent placement with surgical exposure (5/50, 10%) was significantly lower when compared with PDA stent placement performed percutaneously (4/5, 80%, P= .002). CONCLUSION: Hybrid procedures appear to have a low incidence of associated major AEs. PDA stent placement performed as a palliation of hypoplastic left heart syndrome (HLHS) or complex single/two ventricle patients may have a lower incidence of AEs if performed using a direct approach with surgical exposure rather than a percutaneous approach. Accurate definitions of these innovative procedures are required to facilitate prospective data collection. AD - Department of Cardiology, Nationwide Children's Hospital, Columbus, Ohio 43205, USA. ralf.holzer@nationwidechildrens.org AN - 20576042 AU - Holzer, R. AU - Marshall, A. AU - Kreutzer, J. AU - Hirsch, R. AU - Chisolm, J. AU - Hill, S. AU - Galantowicz, M. AU - Phillips, A. AU - Cheatham, J. AU - Bergerson, L. DA - May-Jun DB - PubMed DO - 10.1111/j.1747-0803.2010.00416.x DP - NLM ET - 2010/06/26 IS - 3 KW - Cardiac Catheterization/*adverse effects/instrumentation/statistics & numerical data Cardiac Surgical Procedures/*adverse effects/instrumentation/statistics & numerical data Child Child, Preschool Cooperative Behavior Heart Defects, Congenital/*diagnosis/surgery/*therapy Humans Infant Infant, Newborn Palliative Care Patient Care Team Patient Selection Predictive Value of Tests Prospective Studies Registries Risk Assessment Risk Factors Stents Treatment Outcome United States LA - eng N1 - 1747-0803 Holzer, Ralf Marshall, Audrey Kreutzer, Jackie Hirsch, Russel Chisolm, Joanne Hill, Sharon Galantowicz, Mark Phillips, Alistair Cheatham, John Bergerson, Lisa Journal Article Multicenter Study Research Support, Non-U.S. Gov't United States Congenit Heart Dis. 2010 May-Jun;5(3):233-42. doi: 10.1111/j.1747-0803.2010.00416.x. PY - 2010 SN - 1747-079x SP - 233-42 ST - Hybrid procedures: adverse events and procedural characteristics--results of a multi-institutional registry T2 - Congenit Heart Dis TI - Hybrid procedures: adverse events and procedural characteristics--results of a multi-institutional registry VL - 5 ID - 529 ER - TY - JOUR AB - Tricuspid regurgitation in the context of hypoplastic left heart syndrome (HLHS) carries a significant burden of both mortality and morbidity. We report successful hybrid trans-atrial implantation of an Edwards Sapien XT valve into a dysfunctional bioprosthetic valve in the tricuspid position of a 9-year-old patient with HLHS. There was an immediate fall in the Fontan pressures combined with significant clinical improvement and the valve continues to function well nearly 3 years post implant. To the best of our knowledge, this is the first described implantation of a transcatheter valve in this specific position and anatomy. AD - A.B. Ho, Department of Paediatric Cardiology, Southampton General Hospital, Southampton, United Kingdom AU - Ho, A. B. AU - Kaarne, M. AU - Mullen, M. AU - Hayes, N. DB - Embase Medline DO - 10.1002/ccd.27910 IS - 3 KW - Amplatzer sizing balloon angiographic catheter CarboMedics prosthetic heart valve Carpentier Edwards bioprosthesis Carpentier-Edwards Perimount Magna Ease catheter sheath guide wire pacemaker electrode percutaneous aortic valve sizing balloon catheter Terumo HLA antibody warfarin angiography article case report cavopulmonary connection child clinical article complete heart block exercise tolerance heart transplantation human hypoplastic left heart syndrome jugular vein thrombosis loss of appetite male New York Heart Association class Norwood procedure operation duration preschool child prosthetic valve dysfunction prosthetic valve thrombosis school attendance school child transesophageal echocardiography transthoracic echocardiography tricuspid valve regurgitation tricuspid valve repair tricuspid valve replacement Carbomedics valve Edwards Sapien XT Judkins LA - English M3 - Article N1 - L624892404 2018-11-19 2019-03-05 PY - 2019 SN - 1522-726X 1522-1946 SP - 481-483 ST - Hybrid transcatheter tricuspid valve-in-valve placement of an Edwards Sapien XT valve in palliated hypoplastic left heart syndrome T2 - Catheterization and Cardiovascular Interventions TI - Hybrid transcatheter tricuspid valve-in-valve placement of an Edwards Sapien XT valve in palliated hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L624892404 http://dx.doi.org/10.1002/ccd.27910 VL - 93 ID - 627 ER - TY - JOUR AB - OBJECTIVE: Hyperglycaemia has been associated with worse outcome following traumatic brain injury and cardiac surgery in adults. We have previously reported no relationship between early postoperative hyperglycaemia and worse neurodevelopmental outcome at 1 year following biventricular repair of congenital heart disease. It is not known if postoperative hyperglycaemia results in worse neurodevelopmental outcome after infant cardiac surgery for single-ventricle lesions. METHODS: Secondary analysis of postoperative glucose levels in infants <6 months of age undergoing Stage I palliation for various forms of single ventricle with arch obstruction. The patients were enrolled in a prospective study of genetic polymorphisms and neurodevelopmental outcomes assessed at 1 year of age with the Bayley Scales of Infant Development-II yielding two indices: mental developmental index (MDI) and psychomotor developmental index (PDI). RESULTS: Stage I palliation was performed on 162 infants with 13 hospital and 15 late deaths (17.3% 1-year mortality). Neurodevelopmental evaluation was performed in 89 of 134 (66.4%) survivors. Glucose levels at admission to the cardiac intensive care unit and during the first 48 postoperative hours were available for 85 of 89 (96%) patients. Mean admission glucose value was 274+/-91 mg dl(-1); the maximum was 291+/-90 mg dl(-1), with 69 of 85 (81%) patients having at least one glucose value >200 mg dl(-1). Only two patients had a value <50 mg dl(-1). Mean MDI and PDI scores were 88+/-16 and 71+/-18, respectively. There were no statistically significant correlations between initial, mean, minimum or maximum glucose measurements and MDI or PDI scores. Only delayed sternal closure resulted in a statistically significant relationship between initial, minimum and maximum glucose values within the context of a multivariate analysis of variance model. CONCLUSIONS: Hyperglycaemia following Stage I palliation in the neonatal period was not associated with lower MDI or PDI scores at 1 year of age. AD - The Children's Hospital of Philadelphia, The University of Pennsylvania, Philadelphia, PA, USA. ballweg@email.chop.edu AN - 19699107 AU - Ballweg, J. A. AU - Ittenbach, R. F. AU - Bernbaum, J. AU - Gerdes, M. AU - Dominguez, T. E. AU - Zackai, E. H. AU - Clancy, R. R. AU - Gaynor, J. W. C2 - PMC2840384 C6 - NIHMS181839 DA - Oct DB - PubMed DO - 10.1016/j.ejcts.2009.04.021 DP - NLM ET - 2009/08/25 IS - 4 KW - Blood Glucose/metabolism Child Development Developmental Disabilities/*etiology Heart Defects, Congenital/*surgery Heart Ventricles/abnormalities/surgery Humans Hyperglycemia/blood/*psychology Infant, Newborn Neuropsychological Tests Palliative Care/methods Postoperative Complications/*psychology Prognosis Prospective Studies Psychomotor Performance Treatment Outcome Ventricular Outflow Obstruction/surgery LA - eng N1 - 1873-734x Ballweg, Jean A Ittenbach, Richard F Bernbaum, Judy Gerdes, Marsha Dominguez, Troy E Zackai, Elaine H Clancy, Robert R Gaynor, James William R01 HL071834/HL/NHLBI NIH HHS/United States R01 HL071834-01A1/HL/NHLBI NIH HHS/United States Journal Article Research Support, Non-U.S. Gov't Eur J Cardiothorac Surg. 2009 Oct;36(4):688-93. doi: 10.1016/j.ejcts.2009.04.021. Epub 2009 Aug 20. PY - 2009 SN - 1010-7940 (Print) 1010-7940 SP - 688-93 ST - Hyperglycaemia after Stage I palliation does not adversely affect neurodevelopmental outcome at 1 year of age in patients with single-ventricle physiology T2 - Eur J Cardiothorac Surg TI - Hyperglycaemia after Stage I palliation does not adversely affect neurodevelopmental outcome at 1 year of age in patients with single-ventricle physiology VL - 36 ID - 534 ER - TY - JOUR AB - Background-Restrictive atrial septal defect (ASD) (including intact atrial septum [LAS]) has been reported to be a risk factor that negatively impacts survival in hypoplastic left heart syndrome (HLHS). Although lymphangiectasia and "arterialization" of the veins of the lung in HLHS with restrictive ASD have been reported, they cannot fully explain the high mortality. We have introduced a new method of evaluating the development of the pulmonary vasculature in histological sections and used it to assess patients' lungs. We tested the hypothesis that the small pulmonary arteries (SPA), which are pulmonary arteries in a histological section whose radii are ≈25 μm to 250 μm, in HLHS with restrictive ASD are hypoplastic, but that the alveoli are not, to elucidate the mechanism underlying the poor outcome of these patients. Methods and Results-Fourteen HLHS patients between 1 day and 40 days of age were studied. In 8 cases, the ASD was restrictive [R(+) group], and in the other 6 cases it was not [R(-) group]. Specimens from 12 autopsies of cases with no congenital heart or pulmonary disease were examined as a control group (C group). As a novel histological parameter, we assessed the size of SPA in relation to the size of accompanying bronchioles to identify SPA underdevelopment. To evaluate the development of alveoli and interstitial tissue, radial alveolar counts (RAC), which reflect alveolar maturity and complexity, were also performed. Statistical comparisons between groups were made by analysis of covariance with age as a covariant factor. When the radius of the accompanying bronchiole was 100 μm, the radius of the SPA was 34.0±10.8 μm in the R(+) group, and significantly lower than the 46.6±8.5 μm in R(-) group (P=0.0022) and 70.5±8.4 μm in the C group (P<0.0001). The RAC was in 3.5±0.9 in the R(+) group, 3.4±0.6 in the R(-) group, and 3.7±0.9 in the C group (no significant differences between groups). Conclusion-The SPA in HLHS with restrictive ASD were underdeveloped compared with the SPA in HLHS with nonrestrictive ASD and the controls, but their alveoli were not hypoplastic. Based on these results, it is speculated that SPA hypoplasia may be responsible for the poor outcome of HLHS with restrictive ASD. AD - K. Maeda, Department of Cardiothoracic Surgery, Graduate School of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan AU - Maeda, K. AU - Yamaki, S. AU - Kado, H. AU - Asou, T. AU - Murakami, A. AU - Takamoto, S. DB - Embase Medline DO - 10.1161/01.CIR.0000138223.74524.4e IS - 11 SUPPL. KW - analysis of variance artery dysplasia article autopsy bronchiole clinical article congenital heart disease controlled study correlation analysis female heart atrium septum defect human human tissue hypoplastic left heart syndrome infant intermethod comparison interstitium lung artery hypoplasia lung disease male medical assessment newborn outcomes research pathogenesis patient satisfaction postoperative complication priority journal restrictive cardiomyopathy statistical significance surgical technique LA - English M3 - Article N1 - L39263713 2004-10-11 PY - 2004 SN - 0009-7322 SP - II139-II146 ST - Hypoplasia of the small pulmonary arteries in hypoplastic left heart syndrome with restrictive atrial septal defect T2 - Circulation TI - Hypoplasia of the small pulmonary arteries in hypoplastic left heart syndrome with restrictive atrial septal defect UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L39263713 http://dx.doi.org/10.1161/01.CIR.0000138223.74524.4e VL - 110 ID - 1290 ER - TY - JOUR AB - Hypoplastic left heart syndrome (HLHS) represents a severe spectrum of congenital cardiac malformations, which lead to systemic blood supply being dependent on patency of the arterial duct after birth. Antenatal diagnosis, advances in postnatal care and modifications of surgical palliation strategies have led to improved outcomes with the majority of patients born in the current era being expected to live into adulthood. This review discusses the surgical treatment strategies for HLHS and the wider implications these may have on the wellbeing of patients and families. The ‘Fontan’ circulation constitutes a common endpoint for HLHS patients who often experience physiological circulatory compromise rather than pump failure of the univentricular heart. Heart transplantation has been performed in HLHS, but donor shortage in countries such as the United Kingdom make this a viable option only for a few patients. Protein losing enteropathy and plastic bronchitis may be signs of physiological failure in ‘Fontan’ patients and require specialist input. Family-centred care should enable paediatricians to build relationship outside specialised cardiac centres and optimise healthcare experiences and outcomes through multidisciplinary care systems for these vulnerable patients. AD - Henning Clausen MD MRCPCH FRACP is Paediatric Cardiology Registrar in the Department of Paediatric Cardiology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK. Conflict of interest: none AN - 99916050. Language: English. Entry Date: 20141215. Revision Date: 20190322. Publication Type: Article AU - Clausen, Henning DB - ccm DO - 10.1016/j.paed.2014.07.006 DP - EBSCOhost IS - 1 KW - Hypoplastic Left Heart Syndrome -- Diagnosis Hypoplastic Left Heart Syndrome -- Surgery Heart Surgery -- Methods -- In Infancy and Childhood Prenatal Diagnosis Child Infant Infant, Newborn Blood Circulation Heart Transplantation Family Centered Care Multidisciplinary Care Team N1 - review. Journal Subset: Biomedical; Europe; UK & Ireland. Special Interest: Perioperative Care. NLM UID: 101309435. PY - 2015 SN - 1751-7222 SP - 18-22 ST - Hypoplastic left heart syndrome T2 - Paediatrics & Child Health TI - Hypoplastic left heart syndrome UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=99916050&site=ehost-live&scope=site VL - 25 ID - 1611 ER - TY - JOUR AB - Sixteen years ago, hypoplastic left heart syndrome (HLHS) was a fatal diagnosis. Following birth and closing of the ductus arteriosus, HLHS infants developed metabolic acidosis, decreased systemic perfusion, circulatory collapse, and death. Currently, HLHS can be detected in fetuses in the first trimester of pregnancy. Today's families may choose from four HLHS treatment options, which include (a) medical termination of pregnancy (MTP), (b) supportive care, (c) Norwood (3 Staged) surgical procedure, and/or (d) cardiac transplantation. Presently, pharmacologic therapy with prostaglandin E1 stabilizes HLHS infants until the initiation of a treatment modality. As with many new treatment technologies, the availability of four options forces parents to make difficult decisions. Therefore, parents of HLHS infants require ongoing emotional support, information, and continual family teaching. Nurses caring for these critically ill infants must possess specific skills and knowledge. AN - 12964663 AU - Claxon-McKinney, B. DA - May-Jun DB - PubMed DP - NLM ET - 2003/09/11 IS - 3 KW - Abortion, Therapeutic Acidosis/etiology Choice Behavior Fontan Procedure Heart Transplantation Humans Hypoplastic Left Heart Syndrome/complications/*diagnosis/mortality/*therapy Infant Palliative Care Parents/psychology Patient Selection Pediatric Nursing/methods Perioperative Care/nursing Risk Factors Shock, Cardiogenic/etiology Survival Rate Treatment Outcome Ultrasonography, Prenatal LA - eng N1 - Claxon-McKinney, B Journal Article Review United States Pediatr Nurs. 2001 May-Jun;27(3):245-8, 251-2, 286. PY - 2001 SN - 0097-9805 (Print) 0097-9805 SP - 245-8, 251-2, 286 ST - Hypoplastic left heart syndrome T2 - Pediatr Nurs TI - Hypoplastic left heart syndrome VL - 27 ID - 209 ER - TY - JOUR AB - Hypoplastic left heart syndrome (HLHS) refers to the abnormal development of the left-sided cardiac structures, resulting in obstruction to blood flow from the left ventricular outflow tract. In addition, the syndrome includes underdevelopment of the left ventricle, aorta, and aortic arch, as well as mitral atresia or stenosis. HLHS has been reported to occur in approximately 0.016 to 0.036% of all live births. Newborn infants with the condition generally are born at full term and initially appear healthy. As the arterial duct closes, the systemic perfusion becomes decreased, resulting in hypoxemia, acidosis, and shock. Usually, no heart murmur, or a non-specific heart murmur, may be detected. The second heart sound is loud and single because of aortic atresia. Often the liver is enlarged secondary to congestive heart failure. The embryologic cause of the disease, as in the case of most congenital cardiac defects, is not fully known. The most useful diagnostic modality is the echocardiogram. The syndrome can be diagnosed by fetal echocardiography between 18 and 22 weeks of gestation. Differential diagnosis includes other left-sided obstructive lesions where the systemic circulation is dependent on ductal flow (critical aortic stenosis, coarctation of the aorta, interrupted aortic arch). Children with the syndrome require surgery as neonates, as they have duct-dependent systemic circulation. Currently, there are two major modalities, primary cardiac transplantation or a series of staged functionally univentricular palliations. The treatment chosen is dependent on the preference of the institution, its experience, and also preference. Although survival following initial surgical intervention has improved significantly over the last 20 years, significant mortality and morbidity are present for both surgical strategies. As a result pediatric cardiologists continue to be challenged by discussions with families regarding initial decision relative to treatment, and long-term prognosis as information on long-term survival and quality of life for those born with the syndrome is limited. AD - Cardiovascular Program, Children's Hospital Boston, USA. Jean.Connor@CARDIO.CHBOSTON.ORG AN - 17498282 AU - Connor, J. A. AU - Thiagarajan, R. C2 - PMC1877799 DA - May 11 DB - PubMed DO - 10.1186/1750-1172-2-23 DP - NLM ET - 2007/05/15 KW - Cardiac Surgical Procedures/methods Child, Preschool Diagnosis, Differential Female Heart Defects, Congenital/diagnosis Humans Hypoplastic Left Heart Syndrome/*diagnosis/genetics/*surgery Infant Infant, Newborn Male Palliative Care/methods Pregnancy Prenatal Diagnosis/methods Treatment Outcome LA - eng N1 - 1750-1172 Connor, Jean Anne Thiagarajan, Ravi Journal Article Review Orphanet J Rare Dis. 2007 May 11;2:23. doi: 10.1186/1750-1172-2-23. PY - 2007 SN - 1750-1172 SP - 23 ST - Hypoplastic left heart syndrome T2 - Orphanet J Rare Dis TI - Hypoplastic left heart syndrome VL - 2 ID - 108 ER - TY - JOUR AD - Birmingham Children's Hospital NHS Trust, Birmingham, UK. oliver.stumper@bch.nhs.uk AN - 20237013 AU - Stumper, O. DA - Mar DB - PubMed DO - 10.1136/hrt.2008.159889 DP - NLM ET - 2010/03/20 IS - 1013 KW - Child Child, Preschool Counseling Developmental Disabilities/etiology Female Humans Hypoplastic Left Heart Syndrome/diagnostic imaging/psychology/*surgery Infant Pregnancy Prognosis Ultrasonography, Prenatal/methods LA - eng N1 - 1469-0756 Stumper, Oliver Journal Article Review England Postgrad Med J. 2010 Mar;86(1013):183-8. doi: 10.1136/hrt.2008.159889. PY - 2010 SN - 0032-5473 SP - 183-8 ST - Hypoplastic left heart syndrome T2 - Postgrad Med J TI - Hypoplastic left heart syndrome VL - 86 ID - 165 ER - TY - JOUR AD - Birmingham Children's Hospital NHS Trust, Birmingham, UK Birmingham Children's Hospital NHS Trust, Birmingham, UK. oliver.stumper@bch.nhs.uk. AN - 105147210. Language: English. Entry Date: 20100514. Revision Date: 20170411. Publication Type: journal article AU - Stumper, O. AU - Stumper, Oliver DB - ccm DO - 10.1136/hrt.2008.159889 DP - EBSCOhost IS - 1013 KW - Hypoplastic Left Heart Syndrome -- Surgery Child Child, Preschool Counseling Developmental Disabilities -- Etiology Female Hypoplastic Left Heart Syndrome -- Psychosocial Factors Hypoplastic Left Heart Syndrome -- Ultrasonography Infant Pregnancy Prognosis Ultrasonography, Prenatal -- Methods N1 - review. Journal Subset: Biomedical; Europe; Peer Reviewed; UK & Ireland. NLM UID: 0234135. PMID: NLM20237013. PY - 2010 SN - 0032-5473 SP - 183-188 ST - Hypoplastic left heart syndrome T2 - Postgraduate Medical Journal TI - Hypoplastic left heart syndrome UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105147210&site=ehost-live&scope=site VL - 86 ID - 1647 ER - TY - JOUR AB - Background: Hypoplastic left heart syndrome (HLHS) is the third most common cause of critical congenital heart disease in newborns, and one of the most challenging forms to treat. Secondary pulmonary lymphangiectasia has been recognized in association with HLHS, an appearance described on fetal MRI as the "nutmeg lung."Objective: To investigate the association of fetal nutmeg lung with HLHS survival.Materials and Methods: A retrospective search of the fetal MRI database was performed. The nutmeg lung pattern was defined as T2 heterogeneous signal with tubular structures radiating peripherally from the hila. Postnatal echocardiograms and charts were reviewed.Results: Forty-four fetal MR studies met inclusion criteria, of which 4 patients (9%) had the nutmeg lung pattern and 3 of whom also had restrictive lesions. Mortality in this nutmeg lung group was 100% by 5 months of age. Of the 40 patients without nutmeg lung, mortality/orthotopic heart transplant (OHT) was 35%. Of these 40 patients without nutmeg lung, 5 had restriction on echo, 3 of whom died/had OHT before 5 months of age (60% of patients with restriction and non-nutmeg lung). There was a significantly higher incidence of restrictive lesions (P = 0.02) and mortality/OHT (P = 0.02) in patients with nutmeg lung compared to those without.Conclusion: The nutmeg lung MR appearance in HLHS fetuses is associated with increased mortality/OHT (100% in the first 5 months of life compared to 35% with HLHS alone). Not all patients with restrictive lesions develop nutmeg lung, and outcome is not as poor when restriction is present in isolation. Dedicated evaluation for nutmeg lung pattern on fetal MR studies may be useful to guide prognostication and aid clinicians in counseling parents of fetuses with HLHS. AD - Radiology Department, The Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard Philadelphia 10104 USA Cardiac Center and Fetal Heart Program, The Children's Hospital of Philadelphia, Philadelphia USA Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia USA Pathology Department, The Children's Hospital of Philadelphia, Philadelphia USA Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA, USA Pathology Department, The Children's Hospital of Philadelphia, Philadelphia, PA, USA Radiology Department, The Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA, 10104, USA AN - 114120710. Language: English. Entry Date: 20160803. Revision Date: 20180713. Publication Type: journal article AU - Saul, David AU - Degenhardt, Karl AU - Iyoob, Suzanne AU - Surrey, Lea AU - Johnson, Ann AU - Johnson, Mark AU - Rychik, Jack AU - Victoria, Teresa AU - Iyoob, Suzanne D. AU - Surrey, Lea F. AU - Johnson, Ann M. AU - Johnson, Mark P. DB - ccm DO - 10.1007/s00247-015-3514-6 DP - EBSCOhost IS - 4 KW - Hypoplastic Left Heart Syndrome -- Mortality Prenatal Diagnosis -- Statistics and Numerical Data Hypoplastic Left Heart Syndrome Lymphatic Diseases Lung Diseases Child, Preschool Causal Attribution Comorbidity Lung Risk Factors Survival Pennsylvania Infant, Newborn Infant Prognosis Lymphatic Diseases -- Mortality Female Lung -- Embryology Male Lung Diseases -- Mortality Hypoplastic Left Heart Syndrome -- Embryology Magnetic Resonance Imaging -- Statistics and Numerical Data Human N1 - diagnostic images; pictorial; research; tables/charts. Journal Subset: Biomedical; Continental Europe; Europe; Peer Reviewed. NLM UID: 0365332. PMID: NLM26691156. PY - 2016 SN - 0301-0449 SP - 483-489 ST - Hypoplastic left heart syndrome and the nutmeg lung pattern in utero: a cause and effect relationship or prognostic indicator? T2 - Pediatric Radiology TI - Hypoplastic left heart syndrome and the nutmeg lung pattern in utero: a cause and effect relationship or prognostic indicator? UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=114120710&site=ehost-live&scope=site VL - 46 ID - 1458 ER - TY - JOUR AB - OBJECTIVES: To analyze the main prenatal characteristics of hypoplastic left heart syndrome (HLHS), its association with extracardiac anomalies including increased nuchal translucency (NT) and the outcome of affected patients. METHODS: We searched our database for classical forms of HLHS (aortic atresia, mitral and aortic atresia and critical aortic stenosis evolved to a severely hypoplastic left ventricle) prenatally diagnosed between 1998 and 2006. Data on 101 fetuses were retrieved and analyzed. RESULTS: The main reason for referral was suspected heart defect on a routine ultrasound scan (82%). The mean gestational age at diagnosis was 21 weeks. Most cases were detected at < or = 22 weeks (72%), the upper limit for termination of pregnancy (TOP) in our country (Spain). An intact atrial septum was diagnosed in 11 of the 58 fetuses (19%) in which pulmonary vein blood flow was assessed, and this diagnosis was proved to be correct in the six liveborn babies. Most fetuses (68%) had an isolated HLHS. Fourteen fetuses (14%) were chromosomally abnormal and all had associated extracardiac defects. NT was above the 95th centile in 21 of the 74 cases (28%) in which this measurement was available. 79% (58/73) of the cases in which HLHS was detected at < or = 22 weeks were terminated, and no differences in the rate of TOP were found through the study period. Among the 43 continuing pregnancies, seven fetuses died in utero and there were 36 live births; in 12 cases the parents opted for compassionate care and 24 chose to have the infant surgically treated. In the cohort of intention-to-treat cases, the overall survival rate was 36% (9/25). This rate improved from 18% (2/11) in the period 1998-2002 to 50% (7/14) in 2003-2006. There were no survivors in cases with intact atrial septum or when there were associated defects. At follow-up, 2/9 survivors suffered from significant neurological morbidity. CONCLUSIONS: Fetal echocardiography allows an accurate diagnosis of HLHS, which is made in most instances in the first half of pregnancy. Despite the advantage offered by the prenatal detection of HLHS, which provides the opportunity to plan perinatal management, our up-to-date results show that the outlook for these fetuses is still poor, and highlight the importance of presenting these figures when counseling parents with affected fetuses. AD - Fetal Medicine Unit, Department of Obstetrics and Gynaecology, Hospital Universitario 12 de Octubre, Madrid, Spain. agalindo.hdoc@salud.madrid.org AN - 19367583 AU - Galindo, A. AU - Nieto, O. AU - Villagrá, S. AU - Grañeras, A. AU - Herraiz, I. AU - Mendoza, A. DA - May DB - PubMed DO - 10.1002/uog.6355 DP - NLM ET - 2009/04/16 IS - 5 KW - Abnormalities, Multiple/*diagnostic imaging/genetics/mortality Abortion, Induced/psychology/statistics & numerical data Adolescent Adult Decision Making Female Fetal Heart/diagnostic imaging Genetic Counseling/psychology Gestational Age Humans Hypoplastic Left Heart Syndrome/*diagnostic imaging/mortality/surgery Infant, Newborn Karyotyping Nuchal Translucency Measurement/methods Palliative Care Pregnancy Pregnancy Outcome Prenatal Diagnosis Survival Rate Young Adult LA - eng N1 - 1469-0705 Galindo, A Nieto, O Villagrá, S Grañeras, A Herraiz, I Mendoza, A Journal Article England Ultrasound Obstet Gynecol. 2009 May;33(5):560-6. doi: 10.1002/uog.6355. PY - 2009 SN - 0960-7692 SP - 560-6 ST - Hypoplastic left heart syndrome diagnosed in fetal life: associated findings, pregnancy outcome and results of palliative surgery T2 - Ultrasound Obstet Gynecol TI - Hypoplastic left heart syndrome diagnosed in fetal life: associated findings, pregnancy outcome and results of palliative surgery VL - 33 ID - 256 ER - TY - JOUR AB - The association of cardiac defects with Kabuki syndrome has been well described. The majority of these defects are isolated shunt lesions, conotruncal abnormalities, or various forms of arch obstruction. This report describes a series of three patients with hypoplastic left heart syndrome and Kabuki syndrome. The series illustrates the full spectrum of left-sided obstructive lesions and expands the phenotype of cardiac defects associated with Kabuki syndrome. © 2009 Springer Science+Business Media, LLC. AD - G. C. Kung, Division of Cardiology, USC Keck School of Medicine, Children's Hospital Los Angeles, 4650 Sunset Boulevard, Los Angeles, CA 90027, United States AU - Kung, G. C. AU - Chang, P. M. AU - Sklansky, M. S. AU - Randolph, L. M. DB - Embase Medline DO - 10.1007/s00246-009-9554-7 IS - 1 KW - article case report child enteric feeding female follow up Fontan procedure gastroesophageal reflux Glenn shunt human hypoplastic left heart syndrome infant Kabuki makeup syndrome male microcephaly nasogastric tube Norwood procedure preschool child pulmonary hypertension stomach tube surgical approach LA - English M3 - Article N1 - L50727418 2010-02-10 2010-02-22 PY - 2010 SN - 0172-0643 SP - 138-141 ST - Hypoplastic left heart syndrome in patients with Kabuki syndrome T2 - Pediatric Cardiology TI - Hypoplastic left heart syndrome in patients with Kabuki syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50727418 http://dx.doi.org/10.1007/s00246-009-9554-7 VL - 31 ID - 1159 ER - TY - JOUR AB - There is evidence to suggest that patients undergoing a Norwood for non-HLHS anatomy may have lower mortality than classic HLHS, but differences in neurodevelopmental outcome have not been assessed. Our objective was to compare survival and neurodevelopmental outcome during the same surgical era in a large, well-described cohort. All subjects who underwent a Norwood-Sano operation between 2005 and 2014 were included. Follow-up clinical, neurological, and developmental data were obtained from the Western Canadian Complex Pediatric Therapies Follow-up Program database. Developmental outcomes were assessed at 2 years of age using the Bayley Scales of Infant and Toddler Development (Bayley-III). Survival was assessed using Kaplan-Meier analysis. Baseline characteristics, survival, and neurodevelopmental outcomes were compared between those with HLHS and those with non-HLHS anatomy (non-HLHS). The study comprised 126 infants (75 male), 87 of whom had HLHS. Five-year survival was the same for subjects with HLHS and those with non-HLHS (HLHS 71.8%, non-HLHS 76.9%; p = 0.592). Ninety-three patients underwent neurodevelopmental assessment including Bayley-III scores. The overall mean cognitive composite score was 91.5 (SD 14.6), language score was 86.6 (SD 16.7) and overall mean motor composite score was 85.8 (SD 14.5); being lower than the American normative population mean score of 100 (SD 15) for each (p-value for each comparison, <0.0001). None of the cognitive, language, or motor scores differed between those with HLHS and non-HLHS (all p > 0.05). In the generalized linear models, dominant right ventricle anatomy (present in 117 (93%) of patients) was predictive of lower language and motor scores. Comparative analysis of the HLHS and non-HLHS groups undergoing single ventricle palliation including a Norwood-Sano, during the same era, showed comparable 2-year survival and neurodevelopmental outcomes. AD - Department of Surgery, University of Alberta, Edmonton, Canada. billiejean@ualberta.ca. Department of Surgery, University of Alberta, Edmonton, Canada. Department of Pediatrics, University of Alberta, Edmonton, Canada. Pediatric Rehabilitation, Glenrose Rehabilitation Hospital, Edmonton, Canada. Stollery Children's Hospital, University of Alberta, Edmonton, Canada. Royal University Hospital, University of Saskatchewan, Saskatoon, Canada. AN - 28341901 AU - Martin, B. J. AU - De Villiers Jonker, I. AU - Joffe, A. R. AU - Bond, G. Y. AU - Acton, B. V. AU - Ross, D. B. AU - Robertson, C. M. T. AU - Rebeyka, I. M. AU - Atallah, J. DA - Jun DB - PubMed DO - 10.1007/s00246-017-1598-5 DP - NLM ET - 2017/03/28 IS - 5 KW - Female Humans Hypoplastic Left Heart Syndrome/*surgery Infant Infant, Newborn Male Neurodevelopmental Disorders/*etiology Norwood Procedures/adverse effects/*mortality Treatment Outcome Chd Hypoplastic left heart syndrome Neurocognitive deficits Norwood operation Outcomes LA - eng N1 - 1432-1971 Martin, Billie-Jean Orcid: 0000-0002-5019-8209 De Villiers Jonker, I Joffe, Ari R Bond, Gwen Y Acton, Bryan V Ross, David B Robertson, Charlene M T Rebeyka, Ivan M Atallah, Joseph Comparative Study Journal Article United States Pediatr Cardiol. 2017 Jun;38(5):922-931. doi: 10.1007/s00246-017-1598-5. Epub 2017 Mar 24. PY - 2017 SN - 0172-0643 SP - 922-931 ST - Hypoplastic Left Heart Syndrome is not Associated with Worse Clinical or Neurodevelopmental Outcomes Than Other Cardiac Pathologies After the Norwood-Sano Operation T2 - Pediatr Cardiol TI - Hypoplastic Left Heart Syndrome is not Associated with Worse Clinical or Neurodevelopmental Outcomes Than Other Cardiac Pathologies After the Norwood-Sano Operation VL - 38 ID - 116 ER - TY - JOUR AD - Department of Pediatrics, Yale University School of Medicine, 333 Cedar St, PO Box 208064, New Haven, CT 06520-8064, USA. mark.mercurio@yale.edu AN - 105786637. Language: English. Entry Date: 20080808. Revision Date: 20150711. Publication Type: Journal Article. Journal Subset: Biomedical AU - Mercurio, M. R. AU - Peterec, S. M. AU - Weeks, B. DB - ccm DP - EBSCOhost IS - 1 KW - Consent -- Ethical Issues Hypoplastic Left Heart Syndrome -- Therapy Infant, Premature Palliative Care -- Ethical Issues Treatment Refusal -- Ethical Issues Ethics Hypoplastic Left Heart Syndrome -- Mortality Hypoplastic Left Heart Syndrome -- Surgery Infant, Newborn Resuscitation Social Justice Terminal Care N1 - Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. NLM UID: 0376422. PMID: NLM18596004. PY - 2008 SN - 0031-4005 SP - 186-189 ST - Hypoplastic left heart syndrome, extreme prematurity, comfort care only, and the principle of justice T2 - Pediatrics TI - Hypoplastic left heart syndrome, extreme prematurity, comfort care only, and the principle of justice UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105786637&site=ehost-live&scope=site VL - 122 ID - 1619 ER - TY - JOUR AB - Therapy for the hypoplastic left heart syndrome (HLHS) is still under debate. We report about our experience in the treatment of this cardiac malformation using heart transplantation and Norwood palliation. From 1988 to 1997 a total number of 30 infants with hypoplastic left heart underwent heart transplantation. Mean age at transplantation was 66 days. Mean waiting time was 53 days. Donor-recipient weight ratio ranged from 0.6 to 3.9. There were 6 early hospital deaths and one late death until now. Causes of death were rejection (3), right heart failure (2), infection (1) and multi system organ failure (1). Overall survival was 77%. Increasing experience improved results substantially. From 1988 to 1993 (n=12) survival rate was 58%, whereas from 1994 to 1997 (n=18) survival rate increased to 88%. Quality of life is excellent in the 23 surviving infants. Only one infant shows severe morbidity (neurological defect). The Norwood procedure has been performed in 18 patients since 1993. Five patients died after the first stage. Up until now 10 infants have undergone the second stage and one infant the third stage procedure. None of them have died. Survival rate is 72% for the whole group. Taking into account that 12 infants died on the waiting list for transplanatation (28%), we have comparable results in terms of the survival rate for transplantation and the Norwood procedure in infants with HLHS since 1994. However quality of life and physical development are far better in infants after transplantation according to our experience. Therefore we prefer, whenever possible heart transplantation in the treatment of hypoplastic left heart syndrome. Certainly not all infants with this malformation can undergo transplantation because of the lack of donor organs. Criteria for the decision regarding the operative strategy in our opinion are function of the right ventricle and tricuspid valve, size of the interaterial communication and parental request. AD - F.W. Hehrlein, Department of Cardiovascular Surgery, Justus-Liebig-University, D-35385 Giessen, Germany. AU - Hehrlein, F. W. AU - Yamamoto, T. AU - Orime, Y. AU - Bauer, J. DB - Medline IS - 3 KW - angiocardiography article comparative study donor follow up graft rejection heart surgery heart transplantation hospital admission human hypoplastic left heart syndrome infant methodology mortality newborn palliative therapy postoperative complication radiography retrospective study risk factor survival rate LA - English M3 - Article N1 - L128278812 1998-08-16 PY - 1998 SN - 1341-1098 SP - 125-132 ST - Hypoplastic left heart syndrome: "Which is the best operative strategy?" T2 - Annals of thoracic and cardiovascular surgery : official journal of the Association of Thoracic and Cardiovascular Surgeons of Asia TI - Hypoplastic left heart syndrome: "Which is the best operative strategy?" UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L128278812 VL - 4 ID - 1350 ER - TY - JOUR AD - Department of Medicine, University of Chicago, Chicago, IL 60637, USA. Lross@uchicago.edu AN - 19559290 AU - Ross, L. F. AU - Frader, J. DA - Jul DB - PubMed DO - 10.1016/j.jpeds.2009.03.008 DP - NLM ET - 2009/06/30 IS - 1 KW - Attitude of Health Personnel Counseling *Decision Making Humans Hypoplastic Left Heart Syndrome/*therapy Infant Outcome Assessment, Health Care Palliative Care/ethics Parents/*psychology Professional-Family Relations LA - eng N1 - 1097-6833 Ross, Lainie Friedman Frader, Joel Journal Article United States J Pediatr. 2009 Jul;155(1):12-5. doi: 10.1016/j.jpeds.2009.03.008. PY - 2009 SN - 0022-3476 SP - 12-5 ST - Hypoplastic left heart syndrome: a paradigm case for examining conscientious objection in pediatric practice T2 - J Pediatr TI - Hypoplastic left heart syndrome: a paradigm case for examining conscientious objection in pediatric practice VL - 155 ID - 469 ER - TY - JOUR AB - Caring for infants with HLHS and their parents is challenging. Social policies of the 1980s such as 'Baby Doe', 'Baby Fae', and diagnosis-related groups raise additional ethical and economic or financial questions. The nurse must diligently monitor tissue perfusion in the infant and the parents' coping with loss, while struggling with difficult ethical/legal issues. AD - College of Nursing, University of Arizona, Tucson, AZ AU - Clochesy, J. M. AU - Whittaker, A. A. AU - Murdaugh, C. L. DB - Embase Medline IS - 1 KW - case report congenital disorder diagnosis heart human hypoplastic left heart syndrome newborn priority journal survey LA - English M3 - Article N1 - L16160055 1986-05-20 PY - 1986 SN - 0147-9563 SP - 23-28 ST - Hypoplastic left heart syndrome: A review and the report of two cases T2 - Heart and Lung: Journal of Acute and Critical Care TI - Hypoplastic left heart syndrome: A review and the report of two cases UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L16160055 VL - 15 ID - 1395 ER - TY - JOUR AD - J. Huhta, Department of Newborn Pediatrics, Pennsylvania Hospital, Philadelphia 19107-9956. AU - Huhta, J. DB - Medline IS - 3 KW - article congenital heart malformation congenital malformation decision making health care delivery heart ventricle human newborn prenatal care syndrome treatment refusal LA - English M3 - Article N1 - L22975060 1992-12-08 PY - 1992 SN - 0743-8346 SP - 306-307 ST - Hypoplastic left heart syndrome: access to medical care T2 - Journal of perinatology : official journal of the California Perinatal Association TI - Hypoplastic left heart syndrome: access to medical care UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L22975060 VL - 12 ID - 1384 ER - TY - JOUR AB - Hypoplastic left heart syndrome is one of the most complex congenital heart diseases and requires several cardiac surgeries for survival. The diagnosis is usually established prenatally or shortly after birth. Each stage of surgery poses a unique hemodynamic situation that requires deeper understanding to manage common pediatric problems such as dehydration and respiratory infections. Careful multidisciplinary involvement in the care of these complex patients is improving their outcome; however, morbidity and mortality are still substantial. In this review, we focus on the hemodynamic aspects of various surgical stages that a primary care provider should know to manage these challenging patients. AD - Wanek Family Program for Hypoplastic Left Heart Syndrome. Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine. Department of Cardiovascular Medicine. Department of Cardiovascular Surgery, Mayo Clinic, Rochester, MN. AN - 31263042 AU - Javed, R. AU - Cetta, F. AU - Said, S. M. AU - Olson, T. M. AU - O'Leary, P. W. AU - Qureshi, M. Y. DA - Jul DB - PubMed DO - 10.1542/pir.2018-0005 DP - NLM ET - 2019/07/03 IS - 7 KW - Cardiac Surgical Procedures/*methods Dehydration/etiology Developmental Disabilities/etiology Fontan Procedure Humans *Hypoplastic Left Heart Syndrome/complications/diagnosis/physiopathology/surgery Infant Palliative Care/methods Primary Health Care Respiratory Tract Infections/etiology LA - eng N1 - 1526-3347 Javed, Rabia Cetta, Frank Said, Sameh M Olson, Timothy M O'Leary, Patrick W Qureshi, Muhammad Yasir Journal Article Review United States Pediatr Rev. 2019 Jul;40(7):344-353. doi: 10.1542/pir.2018-0005. PY - 2019 SN - 0191-9601 SP - 344-353 ST - Hypoplastic Left Heart Syndrome: An Overview for Primary Care Providers T2 - Pediatr Rev TI - Hypoplastic Left Heart Syndrome: An Overview for Primary Care Providers VL - 40 ID - 18 ER - TY - JOUR AB - Hypoplastic left heart syndrome is the most common lethal cardiac defect in neonates. Options for treatment include cardiac transplantation and surgical palliation. When cardiac transplantation is chosen as the preferred option, a considerable delay may occur until a suitable donor is available. During this time, anesthetic care may be required for various surgical procedures. Associated anomalies seen in these infants and the anesthetic implications imposed by the abnormal cardiac anatomy are discussed. AD - J.D. Tobias, Division Pediatric Anesthesiology, Department of Pediatrics, Vanderbilt University, Nashville, TN 37232, United States AU - Testa, L. AU - Tobias, J. D. AU - Kavanaugh-McHugh, A. DB - Embase Medline DO - 10.1016/0952-8180(94)90092-2 IS - 6 KW - atropine etomidate fentanyl prostaglandin E1 acrocephalosyndactyly anesthetic recovery article artificial ventilation case report congenital heart malformation Ellis van Creveld syndrome female heart transplantation human human cell human tissue hypoplastic left heart syndrome infant Noonan syndrome palliative therapy priority journal syndrome CHARGE LA - English M3 - Article N1 - L25003419 1995-01-09 PY - 1994 SN - 0952-8180 SP - 500-504 ST - Hypoplastic left heart syndrome: Anesthetic care prior to transplantation or surgical palliation T2 - Journal of Clinical Anesthesia TI - Hypoplastic left heart syndrome: Anesthetic care prior to transplantation or surgical palliation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L25003419 http://dx.doi.org/10.1016/0952-8180(94)90092-2 VL - 6 ID - 1371 ER - TY - JOUR AB - OBJECTIVE: To determine the attitudes of pediatric residents and nurses towards fetal/neonatal management of hypoplastic left heart syndrome (HLHS), and their basis. METHODS: Pediatric residents and nurses from three cardiac centers completed a survey with hypothetical scenarios in which their own fetuses or newborns had HLHS. While Institution A performs many HLHS surgeries, Institution C performs very few. RESULTS: A total of 43% of residents and 50% of nurses would terminate an affected pregnancy. More experience (4 to 7 years, p = 0.04; >7 years, p = 0.05) and employment at institution C (p = 0.04) predicted termination. Expected better quality of life (QOL) (p = 0.02) and five-year survival >50% (p = 0.06) predicted not terminating. Postnatally, 48% of residents and 68% of nurses would choose, or seriously consider, comfort care. Marriage (p = 0.04) and more experience (4 to 7 years, p = 0.04; >7 years, p = 0.02) predicted choosing comfort care. Asian/Pacific Islander descent (p = 0.01) and expected 5-year survival >50% (p = 0.02) predicted choosing surgery. CONCLUSIONS: Approximately one-half of the pediatric residents and nurses surveyed would choose termination of pregnancy or seriously consider declining neonatal surgery, if their own fetus or infant had HLHS. These attitudes reflect perceptions of long-term QOL and survival. These attitudes may be of interest to caregivers who care for HLHS patients. AD - Division of Pediatric Cardiology, Mattel Children's Hospital at the University of California Los Angeles (UCLA) Medical Center, 10833 Le Conte Avenue, MDCC, B2-427 Los Angeles, CA 90095, USA. prenella@mednet.ucla.edu AN - 17729373 AU - Renella, P. AU - Chang, R. K. AU - Ferry, D. A. AU - Bart, R. D. AU - Sklansky, M. S. DA - Nov DB - PubMed DO - 10.1002/pd.1835 DP - NLM ET - 2007/08/31 IS - 11 KW - Abortion, Eugenic/psychology *Attitude of Health Personnel Cross-Sectional Studies Decision Making Female Humans Hypoplastic Left Heart Syndrome/diagnosis/*psychology/*therapy Infant Care/*psychology Infant, Newborn *Internship and Residency Male *Nurses/psychology Parents/psychology Perception Pregnancy *Prenatal Care Socioeconomic Factors Surveys and Questionnaires LA - eng N1 - Renella, P Chang, R-K R Ferry, D A Bart, R D Sklansky, M S Journal Article England Prenat Diagn. 2007 Nov;27(11):1045-55. doi: 10.1002/pd.1835. PY - 2007 SN - 0197-3851 (Print) 0197-3851 SP - 1045-55 ST - Hypoplastic left heart syndrome: attitudes among pediatric residents and nurses towards fetal and neonatal management T2 - Prenat Diagn TI - Hypoplastic left heart syndrome: attitudes among pediatric residents and nurses towards fetal and neonatal management VL - 27 ID - 220 ER - TY - JOUR AB - Objective: To determine the attitudes of pediatric residents and nurses towards fetal/neonatal management of hypoplastic left heart syndrome (HLHS), and their basis. Methods: Pediatric residents and nurses from three cardiac centers completed a survey with hypothetical scenarios in which their own fetuses or newborns had HLHS. While Institution A performs many HLHS surgeries, Institution C performs very few. Results: A total of 43% of residents and 50% of nurses would terminate an affected pregnancy. More experience (4 to 7 years, p = 0.04; >7 years, p = 0.05) and employment at institution C (p = 0.04) predicted termination. Expected better quality of life (QOL) (p = 0.02) and five-year survival >50% (p = 0.06) predicted not terminating. Postnatally, 48% of residents and 68% of nurses would choose, or seriously consider, comfort care. Marriage (p = 0.04) and more experience (4 to 7 years, p = 0.04; >7 years, p = 0.02) predicted choosing comfort care. Asian/Pacific Islander descent (p = 0.01) and expected 5-year survival >50% (p = 0.02) predicted choosing surgery. Conclusions: Approximately one-half of the pediatric residents and nurses surveyed would choose termination of pregnancy or seriously consider declining neonatal surgery, if their own fetus or infant had HLHS. These attitudes reflect perceptions of long-term QOL and survival. These attitudes may be of interest to caregivers who care for HLHS patients. Copyright © 2007 John Wiley & Sons, Ltd. AD - P. Renella, Mattel Children's Hospital, University of California at Los Angeles (UCLA) Medical Center, MDCC, 10833 Le Conte Avenue, Los Angeles, CA 90095, United States AU - Renella, P. AU - Chang, R. K. R. AU - Ferry, D. A. AU - Bart, R. D. AU - Sklansky, M. S. DB - Embase Medline DO - 10.1002/pd.1835 IS - 11 KW - adult article Asian controlled study coronary care unit experience fetus health survey heart surgery human hypoplastic left heart syndrome newborn Pacific Islander pediatric nurse practitioner pediatrician pediatrics pregnancy termination priority journal quality of life residency education survival treatment indication LA - English M3 - Article N1 - L350114304 2007-11-26 PY - 2007 SN - 0197-3851 1097-0223 SP - 1045-1055 ST - Hypoplastic left heart syndrome: Attitudes among pediatric residents and nurses towards fetal and neonatal management T2 - Prenatal Diagnosis TI - Hypoplastic left heart syndrome: Attitudes among pediatric residents and nurses towards fetal and neonatal management UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L350114304 http://dx.doi.org/10.1002/pd.1835 VL - 27 ID - 1223 ER - TY - JOUR AB - In the recent era, no congenital heart defect has undergone a more dramatic change in diagnostic approach, management, and outcomes than hypoplastic left heart syndrome (HLHS). During this time, survival to the age of 5 years (including Fontan) has ranged from 50% to 69%, but current expectations are that 70% of newborns born today with HLHS may reach adulthood. Although the 3-stage treatment approach to HLHS is now well founded, there is significant variation among centers. In this white paper, we present the current state of the art in our understanding and treatment of HLHS during the stages of care: 1) pre-Stage I: fetal and neonatal assessment and management; 2) Stage I: perioperative care, interstage monitoring, and management strategies; 3) Stage II: surgeries; 4) Stage III: Fontan surgery; and 5) long-term follow-up. Issues surrounding the genetics of HLHS, developmental outcomes, and quality of life are addressed in addition to the many other considerations for caring for this group of complex patients. AN - 104623681. Language: English. Entry Date: 20120323. Revision Date: 20200308. Publication Type: journal article AU - Feinstein, J. A. AU - Benson, D. W. AU - Dubin, A. M. AU - Cohen, M. S. AU - Maxey, D. M. AU - Mahle, W. T. AU - Pahl, E. AU - Villafañe, J. AU - Bhatt, A. B. AU - Peng, L. F. AU - Johnson, B. A. AU - Marsden, A. L. AU - Daniels, C. J. AU - Rudd, N. A. AU - Caldarone, C. A. AU - Mussatto, K. A. AU - Morales, D. L. AU - Ivy, D. D. AU - Gaynor, J. W. AU - Tweddell, J. S. DB - ccm DO - 10.1016/j.jacc.2011.09.022 DP - EBSCOhost IS - 1 KW - Cardiopulmonary Bypass -- Methods Hypoplastic Left Heart Syndrome -- Diagnosis Hypoplastic Left Heart Syndrome -- Surgery Prenatal Diagnosis -- Methods Heart Surgery -- Methods Heart Surgery -- Mortality Child Development Echocardiography, Doppler -- Methods Female Cardiopulmonary Bypass -- Mortality Hypoplastic Left Heart Syndrome -- Mortality Infant, Newborn Male Monitoring, Physiologic -- Methods Perioperative Care -- Methods Pregnancy Prognosis Risk Assessment Survival Analysis Treatment Outcomes Ultrasonography, Doppler -- Methods N1 - research. Supplement Title: Jan2012 Supplement. Journal Subset: Biomedical; Peer Reviewed; USA. Grant Information: UL1 TR000154/TR/NCATS NIH HHS/United States. NLM UID: 8301365. PMID: NLM22192720. PY - 2012 SN - 0735-1097 SP - S1-S42 ST - Hypoplastic left heart syndrome: current considerations and expectations T2 - Journal of the American College of Cardiology (JACC) TI - Hypoplastic left heart syndrome: current considerations and expectations UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104623681&site=ehost-live&scope=site VL - 59 ID - 1638 ER - TY - JOUR AB - In the recent era, no congenital heart defect has undergone a more dramatic change in diagnostic approach, management, and outcomes than hypoplastic left heart syndrome (HLHS). During this time, survival to the age of 5 years (including Fontan) has ranged from 50% to 69%, but current expectations are that 70% of newborns born today with HLHS may reach adulthood. Although the 3-stage treatment approach to HLHS is now well founded, there is significant variation among centers. In this white paper, we present the current state of the art in our understanding and treatment of HLHS during the stages of care: 1) pre-Stage I: fetal and neonatal assessment and management; 2) Stage I: perioperative care, interstage monitoring, and management strategies; 3) Stage II: surgeries; 4) Stage III: Fontan surgery; and 5) long-term follow-up. Issues surrounding the genetics of HLHS, developmental outcomes, and quality of life are addressed in addition to the many other considerations for caring for this group of complex patients. AD - Department of Pediatrics, Stanford University School of Medicine, Lucile Salter Packard Children's Hospital, Palo Alto, California 94304, USA. jeff.feinstein@stanford.edu AN - 22192720 AU - Feinstein, J. A. AU - Benson, D. W. AU - Dubin, A. M. AU - Cohen, M. S. AU - Maxey, D. M. AU - Mahle, W. T. AU - Pahl, E. AU - Villafañe, J. AU - Bhatt, A. B. AU - Peng, L. F. AU - Johnson, B. A. AU - Marsden, A. L. AU - Daniels, C. J. AU - Rudd, N. A. AU - Caldarone, C. A. AU - Mussatto, K. A. AU - Morales, D. L. AU - Ivy, D. D. AU - Gaynor, J. W. AU - Tweddell, J. S. AU - Deal, B. J. AU - Furck, A. K. AU - Rosenthal, G. L. AU - Ohye, R. G. AU - Ghanayem, N. S. AU - Cheatham, J. P. AU - Tworetzky, W. AU - Martin, G. R. C2 - PMC6110391 C6 - NIHMS983456 DA - Jan 3 DB - PubMed DO - 10.1016/j.jacc.2011.09.022 DP - NLM ET - 2012/01/04 IS - 1 Suppl KW - Cardiac Surgical Procedures/methods/mortality Child Development/physiology Echocardiography, Doppler/methods Female Fontan Procedure/*methods/mortality Humans Hypoplastic Left Heart Syndrome/*diagnosis/mortality/*surgery Infant, Newborn Male Monitoring, Physiologic/methods Perioperative Care/methods Pregnancy Prenatal Diagnosis/*methods Prognosis Risk Assessment Survival Analysis Treatment Outcome Ultrasonography, Doppler/methods LA - eng N1 - 1558-3597 Feinstein, Jeffrey A Benson, D Woodrow Dubin, Anne M Cohen, Meryl S Maxey, Dawn M Mahle, William T Pahl, Elfriede Villafañe, Juan Bhatt, Ami B Peng, Lynn F Johnson, Beth Ann Marsden, Alison L Daniels, Curt J Rudd, Nancy A Caldarone, Christopher A Mussatto, Kathleen A Morales, David L Ivy, D Dunbar Gaynor, J William Tweddell, James S Deal, Barbara J Furck, Anke K Rosenthal, Geoffrey L Ohye, Richard G Ghanayem, Nancy S Cheatham, John P Tworetzky, Wayne Martin, Gerard R UL1 TR000154/TR/NCATS NIH HHS/United States Journal Article Review J Am Coll Cardiol. 2012 Jan 3;59(1 Suppl):S1-42. doi: 10.1016/j.jacc.2011.09.022. PY - 2012 SN - 0735-1097 (Print) 0735-1097 SP - S1-42 ST - Hypoplastic left heart syndrome: current considerations and expectations T2 - J Am Coll Cardiol TI - Hypoplastic left heart syndrome: current considerations and expectations VL - 59 ID - 3 ER - TY - JOUR AB - Hypoplastic left heart syndrome (HLHS) accounts for 2% to 3% of all congenital heart disease but is responsible for 25% to 40% of all neonatal cardiac deaths. Although the exact genetic origins of HLHS have not been clearly defined, various genetic and chromosomal associations have been identified. Advancements in fetal echocardiography have resulted in accurate diagnosis of congenital heart disease. On the basis of physical examination findings, fetuses may be candidates for prenatal intervention. In general, after prenatal diagnosis of HLHS, parents are faced with 2 choices: termination or continuation of pregnancy. If pregnancy is continued to delivery, patients may choose comfort care, surgical palliation with the Fontan procedure, or transplantation. A once lethal congenital anomaly, HLHS has undergone a marked evolution in management and prognosis during the last several decades.With advancements in prenatal diagnosis, neonatal management, and surgical palliation, patient survival has drastically improved: at an experienced center, current survival rates are very high after the Norwood procedure, with high rates of overall freedom from death or transplantation through 20 years. With survival becoming more promising, the issues that now take precedence are neurodevelopmental outcomes, Fontan procedure complications, and quality of life. Although much progress has been made in caring for this patient population, HLHS remains a high-risk condition that requires lifelong medical follow-up and has significant long-term morbidity, affecting overall quality of life for patients and their families. © 2015 by the American Academy of Pediatrics. All rights reserved. AD - British Columbia Children’s Hospital, Vancouver, BC, Canada Lucile Packard Children’s Hospital at Stanford University Medical Center, Palo Alto, CA, United States AU - Moodley, S. AU - Tacy, T. A. DB - Scopus DO - 10.1542/neo.16-2-e109 IS - 2 M3 - Article N1 - Export Date: 15 June 2020 PY - 2015 SP - e109-e119 ST - Hypoplastic left heart syndrome: Diagnosis, care and management from fetal life and beyond T2 - NeoReviews TI - Hypoplastic left heart syndrome: Diagnosis, care and management from fetal life and beyond UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84922329193&doi=10.1542%2fneo.16-2-e109&partnerID=40&md5=559aec5877e2542812b0122994261205 VL - 16 ID - 1958 ER - TY - JOUR AB - Background: Mortality rate for heart transplantation for patients with hypoplastic left heart syndrome (HLHS) has improved, but there is a considerable wait until a suitable donor is available. Thus it is important to examine the duration of survival and risk factors for early death in patients with HLHS who did not undergo surgical intervention. Methods and Results: Twenty-six consecutive patients were studied retrospectively. Duration of survival and the 14 following variables were investigated: date of birth, body weight at birth, cardiothoracic ratio, ascending aorta diameter, interatrial communication size, coarctation of the aorta, tricuspid regurgitation, anatomic subtype (patency) of mitral and aortic valve, arterial blood gas findings (pH, PaO2, SaO2, PaCO2, base excess), and ST depression in the electrocardiogram. Twenty patients survived <60 days (group A) and 6 patients survived beyond 60 days (group B). The duration of survival (mean [SD]) was 60 (151) days overall (1 patient is currently alive at 783 days). The long-term survivors (beyond 60 days) increased significantly after 1991 (P < .05). Coarctation of the aorta was a significant risk of early death (<60 days) (P < .05). Interatrial communication size was significantly smaller in group B than in group A (P < .05). The mean pH and base excess were significantly lower in group A than in group B. The other 9 variables showed no significant difference between the 2 groups. Conclusions: There was a significant correlation of long-term survival with stabilized ductal blood flow without coarctation of the aorta, adequate restriction of interatrial communication without severe hypoxemia, and no metabolic acidosis. AD - K. Hoshino, Department of Pediatric Cardiology, Saitama Children's Medical Center, 2100 Magome, Iwatsuki City, Saitama 339, Japan AU - Hoshino, K. AU - Ogawa, K. AU - Hishitani, T. AU - Kitazawa, R. AU - Uehara, R. DB - Embase Medline DO - 10.1016/S0002-8703(99)70503-X IS - 3 KW - alkalosis aortic coarctation arterial gas article blood pH clinical article controlled study female human hypoplastic left heart syndrome infant male mortality newborn priority journal risk factor ST segment depression survival time LA - English M3 - Article N1 - L29124124 1999-04-13 PY - 1999 SN - 0002-8703 SP - 535-542 ST - Hypoplastic left heart syndrome: Duration of survival without surgical intervention T2 - American Heart Journal TI - Hypoplastic left heart syndrome: Duration of survival without surgical intervention UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L29124124 http://dx.doi.org/10.1016/S0002-8703(99)70503-X VL - 137 ID - 1344 ER - TY - JOUR AB - From February, 1983, to November, 1984, 11 infants with hypoplastic left heart syndrome were managed medically prior to operative intervention. Only 2 of the 11 infants appeared to be in mild distress at birth. Despite relatively normal Apgar scores, 9 of the infants were seen in shock. All patients were managed medically with fluid restriction, diuretics, and the institution of prostaglandin E1. There was marked improvement in arterial pH from a mean value of 7.13 prior to prostaglandin to 7.42 following prostaglandin infusion. There was also a dramatic decrease in the serum creatinine level from a mean pretreatment level of 1.5 mg/dl to 0.7 mg/dl following infusion. Ten of the 11 infants were operated on at a mean age of 4 days with a Norwood or conduit procedure. Three infants who died at 3, 13, and 24 days of age had sustained myocardial infarctions prior to operation. Three patients also had multiple infarcts of the liver. Three patients are alive 23, 22, and 6 months after operation, and are growing and developing normally. The ability to maintain the infant with hypoplastic left heart syndrome in a stable hemodynamic condition has made surgical palliation a realistic option for this otherwise fatal anomaly. Nevertheless, preoperative infarctions of the heart and viscera continue to play a major role in mortality. AD - Department of Cardiology, Cleveland Clinic Foundation, Cleveland, OH 44106 AU - Moodie, D. S. AU - Gill, C. C. AU - Sterba, R. DB - Embase Medline DO - 10.1016/S0003-4975(10)62741-5 IS - 3 KW - creatinine prostaglandin prostaglandin E1 arterial pH autopsy blood and hemopoietic system clinical article drug dose drug efficacy drug therapy heart heart infarction histology human hypoplastic left heart syndrome infant intravenous drug administration liver liver infarction newborn priority journal serum surgery therapy LA - English M3 - Article N1 - L16013828 1986-11-19 PY - 1986 SN - 0003-4975 SP - 307-311 ST - The hypoplastic left heart syndrome: Evidence of preoperative myocardial and hepatic infarction in spite of prostaglandin therapy T2 - Annals of Thoracic Surgery TI - The hypoplastic left heart syndrome: Evidence of preoperative myocardial and hepatic infarction in spite of prostaglandin therapy UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L16013828 http://dx.doi.org/10.1016/S0003-4975(10)62741-5 VL - 42 ID - 1391 ER - TY - JOUR AB - Parent's choices among therapeutic options for their infants born with hypoplastic left heart syndrome are difficult and controversial. Currently, management options include surgical reconstruction, cardiac transplantation, and comfort measures only. We retrospectively reviewed medical records of 47 patients (1989-1999) to create a database of clinical features of infants who received either an operation or comfort care only. Eleven families were interviewed by means of a structured questionnaire pertaining to their experience and reasons for their choice. Of the 47, 20 were prenatally diagnosed and nine of these (45%) aborted. The remaining 38 of the 47 were liveborns. Of the 38, 20/38 (53%) chose comfort care only. The other 18 chose operation. Although 17 were able to survive until first stage repair, only 8/17 (47%) survived beyond five months. At the time of last contact (ages one to 4.5 years), 5/17 (29%) remained alive. Over the nine years an increasing proportion of parents chose operative reconstruction; 8/11 (73%) for 1996-99 vs 10/27 (37%) for 1989-1995. Interviewed families who chose comfort care were more likely to believe the rate of survival following operation was poor, quality of life was diminished, and seemed concerned that their infant would suffer. Influence by optimistic physicians at surgical centers seemed important for an operative choice. Most suggested that provision of written materials, professional family counseling, and support groups of hypoplastic left heart syndrome families are or would be helpful. AD - C. Corrow, University of Connecticut School of Medicine, Farmington, USA. AU - Corrow, C. AU - Lapuk, S. AU - Mazzarella, K. AU - Sable, A. AU - Leopold, H. AU - Eisenfeld, L. DB - Medline IS - 4 KW - adult article decision making female human hypoplastic left heart syndrome induced abortion infant male newborn palliative therapy parent pregnancy psychological aspect questionnaire retrospective study LA - English M3 - Article N1 - L33481126 2001-05-22 PY - 2001 SN - 0010-6178 SP - 195-203 ST - Hypoplastic left heart syndrome: factors influencing therapeutic choice T2 - Connecticut medicine TI - Hypoplastic left heart syndrome: factors influencing therapeutic choice UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L33481126 VL - 65 ID - 1324 ER - TY - JOUR AD - Division of Cardiovascular and Thoracic Surgery, Department of Surgery, Duke University School of Medicine, Durham, NC. Electronic address: robert.jaquiss@duke.edu. AN - 26454519 AU - Jaquiss, R. D. DA - Dec DB - PubMed DO - 10.1016/j.jtcvs.2015.08.107 DP - NLM ET - 2015/10/12 IS - 6 KW - Child Humans Hypoplastic Left Heart Syndrome/mortality/*surgery *Quality of Life LA - eng N1 - 1097-685x Jaquiss, Robert D B Editorial United States J Thorac Cardiovasc Surg. 2015 Dec;150(6):1401-3. doi: 10.1016/j.jtcvs.2015.08.107. Epub 2015 Sep 5. PY - 2015 SN - 0022-5223 SP - 1401-3 ST - Hypoplastic left heart syndrome: How to improve late survival and quality of life T2 - J Thorac Cardiovasc Surg TI - Hypoplastic left heart syndrome: How to improve late survival and quality of life VL - 150 ID - 229 ER - TY - JOUR AB - Of the 12 children who represent the complete cohort of Norwegian patients surviving the complete palliative operative programme for hypoplastic left heart syndrome, 10 were studied by a pediatric cardiologist, a pediatric neurologist, a neuropsychologist and a child psychiatrist. We found significant morbidity. Hemodynamic function was relatively uncompromised in eight of the patients, but one had significant cyanosis due to use of an operative technique subsequently abandoned and one had obstruction of the flow of blood from the left to the right atrium. Five children had disturbances of rhythm, with one requiring insertion of a pacemaker. One child had epilepsy. Delayed motor development, most often to mild degree, was found in seven children, while six had attention problems. Mental retardation was found in two children and autistic traits in one. Despite these various problems, the parents were seemingly satisfied with their own lives, and the quality of life of their children. © Greenwich Medical Media Ltd. AD - Department of Pediatric Cardiology, Rikshospitalet, N-0027 Oslo, Norway Department of Child Neurology and Rehabilitation, Rikshospitalet, N-0027 Oslo, Norway Department of Child and Adolescent Psychiatry, Rikshospitalet, N-0027 Oslo, Norway AU - Hagemo, P. S. AU - Rasmussen, M. AU - Bryhn, G. AU - Vandvik, I. H. DB - Scopus IS - 3 KW - Follow-up Hypoplastic left heart syndrome Norwood palliation M3 - Article N1 - Cited By :14 Export Date: 15 June 2020 PY - 1997 SP - 248-253 ST - Hypoplastic left heart syndrome: Multiprofessional follow-up in the mid-term following palliative procedures T2 - Cardiology in the Young TI - Hypoplastic left heart syndrome: Multiprofessional follow-up in the mid-term following palliative procedures UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0001284414&partnerID=40&md5=e01353f3a5bb9c757c474cfffd8f17d0 VL - 7 ID - 2292 ER - TY - JOUR AB - Prior to 1980, the diagnosis of hypoplastic left heart syndrome (HLHS) was almost uniformly lethal. Over the past 25 years, the development of operative options, including staged surgical palliation and infant heart transplant, have resulted in major improvements in survival and quality-of-life outcomes. Throughout this period, the optimal treatment strategy for children with HLHS has continued to be controversial. Current advances include fetal diagnosis, medical management, catheter intervention and operative techniques, and hold great promise for further improvements. However, as new techniques continue to evolve, controversies will continue to arise. This article will explore the treatment strategies for children with HLHS and review current controversies surrounding this complex congenital cardiac disease. AD - Department of Pediatrics and Communicable Diseases, Division of Pediatric Cardiology and the Congenital Heart Center, C. S. Mott Children's Hospital, University of Michigan Medical Center, Ann Arbor, MI 48109-0204, USA. Cgoldber@umich.edu AN - 15001118 AU - Goldberg, C. S. AU - Gomez, C. A. DA - Dec DB - PubMed DO - 10.1016/s1084-2756(03)00116-7 DP - NLM ET - 2004/03/06 IS - 6 KW - *Cardiac Surgical Procedures/methods Humans Hypoplastic Left Heart Syndrome/*diagnosis/embryology/*therapy *Infant Welfare Infant, Newborn Intensive Care Units, Pediatric Palliative Care/methods Prognosis Quality of Life Risk Factors Ultrasonography, Prenatal LA - eng N1 - Goldberg, Caren S Gomez, Carlen A Journal Article Review Netherlands Semin Neonatol. 2003 Dec;8(6):461-8. doi: 10.1016/S1084-2756(03)00116-7. PY - 2003 SN - 1084-2756 (Print) 1084-2756 SP - 461-8 ST - Hypoplastic left heart syndrome: new developments and current controversies T2 - Semin Neonatol TI - Hypoplastic left heart syndrome: new developments and current controversies VL - 8 ID - 319 ER - TY - JOUR AD - E.A. Hedenkamp AU - Hedenkamp, E. A. DB - Medline IS - 3 KW - article congenital heart malformation congenital malformation heart heart ventricle hemodynamics human human relation newborn nursing pathophysiology syndrome LA - English M3 - Article N1 - L17822546 1987-12-08 PY - 1987 SN - 0889-7204 SP - 80-85 ST - Hypoplastic left heart syndrome: options for the infant and the family T2 - Progress in cardiovascular nursing TI - Hypoplastic left heart syndrome: options for the infant and the family UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L17822546 VL - 2 ID - 1390 ER - TY - JOUR AB - Hypoplastic left heart syndrome is a life threatening congenital cardiac anomaly. After a child has been diagnosed with hypoplastic left heart syndrome, parents must make life or death decisions within days of birth. Healthcare providers must provide appropriate education so that parents are able to make informed, timely decisions. Information regarding the diagnosis, treatment options, and parental decision making process for initial decision making for hypoplastic left heart syndrome are provided to guide nurses who work with these families. The challenging decision making process that parents must go through after diagnosis of hypoplastic left heart syndrome will be described. AD - Purdue University, West Lafayette, IN, USA. teletoebbe@aol.com AN - 23246301 AU - Toebbe, S. AU - Yehle, K. AU - Kirkpatrick, J. AU - Coddington, J. DA - Jul-Aug DB - PubMed DO - 10.1016/j.pedn.2012.11.005 DP - NLM ET - 2012/12/19 IS - 4 KW - *Cause of Death Counseling/methods Critical Illness/therapy *Decision Making Female Grief Humans Hypoplastic Left Heart Syndrome/*diagnosis/mortality/therapy Infant, Newborn Life Support Care/methods/*psychology Male Nurse's Role Parents/education/*psychology Risk Assessment Withholding Treatment LA - eng N1 - 1532-8449 Toebbe, Sarah Yehle, Karen Kirkpatrick, Jane Coddington, Jennifer Journal Article Review United States J Pediatr Nurs. 2013 Jul-Aug;28(4):383-92. doi: 10.1016/j.pedn.2012.11.005. Epub 2012 Dec 11. PY - 2013 SN - 0882-5963 SP - 383-92 ST - Hypoplastic left heart syndrome: parent support for early decision making T2 - J Pediatr Nurs TI - Hypoplastic left heart syndrome: parent support for early decision making VL - 28 ID - 174 ER - TY - JOUR AU - Erkonen, G. E. AU - Hanfland, R. A. DB - Medline DO - 10.1080/15265161.2017.1314053 IS - 7 KW - child parent relation contraindication decision making education ethics heart surgery human hypoplastic left heart syndrome newborn palliative therapy parental consent patient education psychology quality of life LA - English M3 - Article N1 - L625031372 2018-11-22 2019-07-05 PY - 2017 SN - 1536-0075 SP - 71-72 ST - Hypoplastic Left Heart Syndrome: The Case Against Palliative Surgery T2 - The American journal of bioethics : AJOB TI - Hypoplastic Left Heart Syndrome: The Case Against Palliative Surgery UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L625031372 http://dx.doi.org/10.1080/15265161.2017.1314053 VL - 17 ID - 749 ER - TY - JOUR AB - OBJECTIVE: To examine the survival, developmental status, quality of life, and direct medical costs of children with hypoplastic left heart syndrome who have undergone stage I, II, and III reconstructive surgery. METHODS: A total of 106 children underwent staged repair for classic hypoplastic left heart syndrome between February 1990 and March 1999 (stage I: 106; stage II: 49; stage III: 25; 4 converted to heart transplantation). Survival was analyzed by the Kaplan-Meier method. In a cross-sectional study, parents assessed quality of life by completing the Infant/Toddler Child Health Questionnaire or Child Health Questionnaire Parent Format-28; they assessed developmental progress by completing the Ages and Stages Questionnaire. The ratio-of-costs-to-charges method was used to derive hospital costs, and payments were used to capture physician time and wholesale pricing for outpatient medications. RESULTS: Institutional 1-year and 5-year actuarial survivals were 58% and 54%. Birth weight, the need for preoperative inotropic drugs, and surgical experience were predictors of survival. Norwood I patients achieved fewer developmental benchmarks than those who survived to subsequent stages. Child Health Questionnaire Parent Format-28 mean summary scores for physical and psychosocial health were 48.5 +/- 6.3 and 42.8 +/- 9.9. The median inpatient costs for stage I, II, and III repairs were $51,000, $33,892, and $52,183, respectively. Monthly outpatient and readmission costs were less than 10% of total costs. CONCLUSION: A prospective, large-scale study of the comprehensive outcomes of staged repair and transplantation is needed. This study will need to address the longer-term developmental and quality-of-life outcomes, as well as the long-term cost effectiveness of these procedures. AD - International Center for Health Outcomes and Innovation Research, Department of Surgery, Columbia University, College of Physicians and Surgeons, New York Presbyterian Hospital, New York, NY, USA. AN - 10733760 AU - Williams, D. L. AU - Gelijns, A. C. AU - Moskowitz, A. J. AU - Weinberg, A. D. AU - Ng, J. H. AU - Crawford, E. AU - Hayes, C. J. AU - Quaegebeur, J. M. DA - Apr DB - PubMed DO - 10.1016/s0022-5223(00)70007-9 DP - NLM ET - 2000/03/25 IS - 4 Pt 1 KW - Child Development Female Health Care Costs Humans Hypoplastic Left Heart Syndrome/economics/*mortality/*surgery Infant Infant, Newborn Male Quality of Life Surveys and Questionnaires Survival Rate LA - eng N1 - Williams, D L Gelijns, A C Moskowitz, A J Weinberg, A D Ng, J H Crawford, E Hayes, C J Quaegebeur, J M Journal Article United States J Thorac Cardiovasc Surg. 2000 Apr;119(4 Pt 1):720-31. doi: 10.1016/S0022-5223(00)70007-9. PY - 2000 SN - 0022-5223 (Print) 0022-5223 SP - 720-31 ST - Hypoplastic left heart syndrome: valuing the survival T2 - J Thorac Cardiovasc Surg TI - Hypoplastic left heart syndrome: valuing the survival VL - 119 ID - 168 ER - TY - JOUR AN - 9161330 AU - Thwaites, R. C2 - PMC2126658 DA - May 10 DB - PubMed DP - NLM ET - 1997/05/10 IS - 7091 KW - Child Child, Preschool Humans Hypoplastic Left Heart Syndrome/*surgery Infant *Quality of Life LA - eng N1 - 1468-5833 Thwaites, R Comment Letter BMJ. 1997 May 10;314(7091):1414. PY - 1997 SN - 0959-8138 (Print) 0959-8138 SP - 1414 ST - Hypoplastic left heart syndrome. Quality of life is also important T2 - Bmj TI - Hypoplastic left heart syndrome. Quality of life is also important VL - 314 ID - 475 ER - TY - JOUR AB - In our current practice, either the Norwood operation or heart transplantation must be considered reasonable options for neonates born with hypoplastic left-heart syndrome. Clearly some children with this otherwise lethal defect have been helped by both approaches. Is it yet, in 1993, appropriate to delete compassionate care from our group of options? Despite all the advances of the past decade, we are not at the point where we can declare hypoplastic left-heart syndrome "cured" even for the survivors of the Norwood operation or heart transplantation. Compassionate care (including withdrawal of prostaglandin E1) is still appropriate for those families who feel that the chance of success and the quality of life are not great enough to justify the challenges the infants and their families must face. Clearly as technical modifications are made in the Norwood operation and as advances are made in transplantation biology, the number of families who choose compassionate care will decrease. AD - Division of Pediatric Cardiology, Case Western Reserve University School of Medicine, Rainbow Babies and Childrens Hospital, Cleveland, Ohio. AN - 8458162 AU - Zahka, K. G. AU - Spector, M. AU - Hanisch, D. DA - Mar DB - PubMed DP - NLM ET - 1993/03/01 IS - 1 KW - Heart Defects, Congenital/genetics/physiopathology/*surgery *Heart Transplantation Humans Infant, Newborn Preoperative Care Syndrome LA - eng N1 - Zahka, K G Spector, M Hanisch, D Journal Article Review United States Clin Perinatol. 1993 Mar;20(1):145-54. PY - 1993 SN - 0095-5108 (Print) 0095-5108 SP - 145-54 ST - Hypoplastic left-heart syndrome Norwood operation, transplantation, or compassionate care T2 - Clin Perinatol TI - Hypoplastic left-heart syndrome Norwood operation, transplantation, or compassionate care VL - 20 ID - 109 ER - TY - JOUR AD - J. Gehrmann, Child. Hosp. Dept. Pediat. Cardiol., Westfaelische Wilhelms- Universitaet, Albert-Schweitzer-Strasse 33, 48149 Muenster, Germany AU - Gehrmann, J. AU - Krasemann, T. AU - Kehl, H. G. AU - Vogt, J. DB - Embase Medline DO - 10.1378/chest.120.4.1368 IS - 4 KW - aorta aortic valve article atresia clinical feature death disease course disease severity heart left atrium heart left ventricle human hypoplastic left heart syndrome mitral valve newborn patent ductus arteriosus pathophysiology priority journal sucking symptomatology LA - English M3 - Article N1 - L32960114 2001-10-24 PY - 2001 SN - 0012-3692 SP - 1368-1371 ST - Hypoplastic left-heart syndrome: The first description of the pathophysiology in 1851; translation of a publication by Dr. Bardeleben from Giessen, Germany T2 - Chest TI - Hypoplastic left-heart syndrome: The first description of the pathophysiology in 1851; translation of a publication by Dr. Bardeleben from Giessen, Germany UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L32960114 http://dx.doi.org/10.1378/chest.120.4.1368 VL - 120 ID - 1320 ER - TY - JOUR AU - Chiakpo, E. DB - Medline DO - 10.22454/FamMed.2019.301407 IS - 6 KW - antidiabetic agent metformin female fertility general practice human hypoplastic left heart syndrome newborn obstetric delivery ovary polycystic disease palliative therapy perinatal death pregnancy psychology LA - English M3 - Article N1 - L628259951 2019-07-01 2020-01-14 PY - 2019 SN - 1938-3800 SP - 525-526 ST - "I'm Sticking With You, Doc" T2 - Family medicine TI - "I'm Sticking With You, Doc" UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L628259951 http://dx.doi.org/10.22454/FamMed.2019.301407 VL - 51 ID - 615 ER - TY - JOUR AB - Objectives To investigate the spectrum, etiology, and management of traumatic aortopulmonary (AP) communications after transcatheter interventions on the pulmonary circulation. Background An iatrogenic AP communication is an unusual complication after balloon pulmonary artery (PA) angioplasty or stenting, or transcatheter pulmonary valve replacement (TPVR). However, with the increasing application of transcatheter therapies for postoperative PA stenosis and right ventricular outflow tract (RVOT) dysfunction, including percutaneous pulmonary valve replacement, consideration of the etiology, diagnosis, and management of this problem is important for interventional cardiologists performing such procedures. Methods and Results We present three new cases, as well as gross anatomy and histopathology data, related to AP communications after PA interventions. We also review the literature relevant to this topic. Including these new cases, there have been 18 reported cases of iatrogenic AP communication after transcatheter interventions on the PAs or RVOT, primarily patients with transposition of the great arteries who underwent PA angioplasty after an arterial switch operation, or after TPVR in patients who had undergone a Ross procedure. The likely cause of such defects is PA trauma plus distortion of the neo-aortic anastomosis resulting from angioplasty or stenting of the RVOT or central PAs, with subsequent dissection through the extravascular connective tissue and into the closely adjacent vessel through the devitalized tissue at the anastomosis. Conclusions Cardiologists performing PA or RVOT interventions should be aware of the possibility of a traumatic AP communication and consider this diagnosis when confronted with suggestive signs and symptoms. AD - D.B. McElhinney, Lucille Packard Children's Hospital Stanford, 780 Welch Road, Palo Alto, CA, United States AU - Torres, A. AU - Sanders, S. P. AU - Vincent, J. A. AU - El-Said, H. G. AU - Leahy, R. A. AU - Padera, R. F. AU - McElhinney, D. B. DB - Embase Medline DO - 10.1002/ccd.25897 IS - 3 KW - adult angioplasty arterial switch operation artery catheterization article ascending aorta cardiac conduit case report child stent graft Doppler echocardiography echocardiography Fontan procedure great vessels transposition heart left ventricle outflow tract obstruction heart murmur heart right ventricle outflow tract heart surgery heart valve prosthesis human hypotension iatrogenic aortopulmonary communication lung artery pressure lung circulation lung edema male pulmonary angioplasty pulmonary artery pulmonary artery stenosis pulmonary valve replacement Ross Konno procedure Ross procedure transcatheter pulmonary valve replacement vascular disease vascular stent young adult LA - English M3 - Article N1 - L605658121 2015-08-24 2015-08-31 PY - 2015 SN - 1522-726X 1522-1946 SP - 438-452 ST - Iatrogenic aortopulmonary communications after transcatheter interventions on the right ventricular outflow tract or pulmonary artery: Pathophysiologic, diagnostic, and management considerations T2 - Catheterization and Cardiovascular Interventions TI - Iatrogenic aortopulmonary communications after transcatheter interventions on the right ventricular outflow tract or pulmonary artery: Pathophysiologic, diagnostic, and management considerations UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L605658121 http://dx.doi.org/10.1002/ccd.25897 VL - 86 ID - 853 ER - TY - JOUR AB - Little data exist to identify pediatric patients who have developed transplant coronary artery vasculopathy (CAV). Transplant patients do not exhibit the usual signs of coronary disease, making diagnosis more difficult. The aim of this study is to assess the use of myocardial perfusion imaging to identify CAV in transplant patients and to derive an incidence of occurrence. We studied pediatric cardiac transplant recipients who have undergone myocardial perfusion imaging on a routine basis. All patients from September 1999 through November 2004 with nuclear perfusion scans were included. Twenty patients age 7-24 years (mean: 12.7), 11 male and 9 female, were studied by SPECT technetium 99M tetrofosmin imaging. Sixteen of the 20 patients were studied based on a newly instituted protocol for surveillance. Transplant was 1-14 years (mean: 7.9) earlier. Patients were also studied by stress echocardiography. Six of 20 patients (30%) had a positive perfusion scan. Ages ranged from 8 to 18 years (mean: 12). Time from transplant to positive scans ranged from 1 to 9 years (mean: 5.6). One patient showed the same perfusion defect as 2 years earlier. Five patients with positive nuclear perfusion scans showed the abnormality on the initial study; one had a previous negative study 6 months earlier. Four patients who demonstrated ischemia with exercise showed resolution at rest; the other two had no resting study. Five of these six patients with abnormal perfusion scans had negative stress echocardiograms. Only one patient identified with coronary involvement reported symptoms (exertional dyspnea). Hypertension and rejection episodes were similar in all patients and in those with positive nuclear scans. Of the six patients with positive nuclear perfusion scans, two demonstrated coronary disease at cardiac catheterization. Two patients with coronary disease at catheterization had normal nuclear perfusion scans; one of two had a normal stress echo. When three imaging modalities were used, the incidence of CAV was 30%. Symptoms in pediatric patients with CAV are seldom reported. Unfortunately, coronary arteriopathy occurs frequently and might be found as early as 1 year posttransplant. Six of 20 patients had abnormal perfusion; only 1 had any other noninvasive marker. Importantly, not all patients with CAV were identified by perfusion imaging, stress echocardiography, or coronary injection alone. Therefore, transplant patients need continued evaluation by multiple modalities for detection of developing coronary lesions. AD - Department of Pediatrics, Indiana University School of Medicine, 702, Barnhill Drive, Riley Research Rm. 127, Indianapolis, IN 46202, USA. jamaiers@gmail.com AN - 17891435 AU - Maiers, J. AU - Hurwitz, R. DA - Jan DB - PubMed DO - 10.1007/s00246-007-9038-6 DP - NLM ET - 2007/09/25 IS - 1 KW - Adolescent Adult Cardiomyopathy, Dilated/surgery Child Coronary Angiography Coronary Artery Disease/*diagnosis/etiology *Diagnostic Imaging Echocardiography, Stress Female Heart Defects, Congenital/*surgery Heart Transplantation/*adverse effects Humans Hypoplastic Left Heart Syndrome/surgery Male Retrospective Studies Tomography, Emission-Computed, Single-Photon LA - eng N1 - Maiers, Jacqueline Hurwitz, Roger Journal Article United States Pediatr Cardiol. 2008 Jan;29(1):19-23. doi: 10.1007/s00246-007-9038-6. Epub 2007 Sep 21. PY - 2008 SN - 0172-0643 (Print) 0172-0643 SP - 19-23 ST - Identification of coronary artery disease in the pediatric cardiac transplant patient T2 - Pediatr Cardiol TI - Identification of coronary artery disease in the pediatric cardiac transplant patient VL - 29 ID - 423 ER - TY - JOUR AB - Objective: To examine whether second trimester biomarkers could be used to identify screen positive pregnancies at increased risk for congenital heart defects (CHDs) and measure the effect of using different biomarker cut points on the detection of CHDs and on the performance of predictive models. Methods: Included were 19,402 pregnancies without chromosomal defects, which were screen positive for Down syndrome or other birth defects based on maternal serum measurements of alpha-fetoprotein (AFP), human chorionic gonadotrophin (hCG), and unconjugated estriol (uE3). Logistic regression models were built that compared biomarkers for CHD cases compared to controls. Results: CHD cases were more likely to be screen positive for trisomy-18, to have a nuchal fold (NF) ≥5 mm, and/or to have an hCG multiple of the median (MoM) ≥95th percentile in models that considered screen positive grouping. In models that did not consider screen positive grouping, cases were more likely to have a NF ≥5 mm, an AFP MoM ≤10th percentile, an hCG MoM ≤25th percentile, and/or an hCG MoM ≥75th percentile. Conclusion: Along with NF, second trimester maternal serum biomarkers may be useful indicators for fetal and newborn evaluation for CHDs in screen positive pregnancies without identified chromosomal defects. Copyright © 2009 John Wiley & Sons, Ltd. AD - L. L. Jelliffe-Pawlowski, Genetic Disease Screening Program, California Department of Public Health, 850 Marina Bay Parkway, Richmond, CA 94804, United States AU - Jelliffe-Pawlowski, L. L. AU - Walton-Haynes, L. AU - Currier, R. J. DB - Embase Medline DO - 10.1002/pd.2239 IS - 6 KW - alpha fetoprotein chorionic gonadotropin estriol adult aortic coarctation article congenital malformation congenital heart malformation controlled study Down syndrome endocardial cushion defect Fallot tetralogy female fetus fetus echography genetic counseling heart atrium septum defect heart ventricle septum defect high risk pregnancy human hypoplastic left heart syndrome maternal serum neural tube defect newborn nuchal translucency measurement patent ductus arteriosus pregnancy outcome prenatal care prenatal screening priority journal pulmonary valve disease second trimester pregnancy tricuspid valve atresia tricuspid valve stenosis trisomy 18 LA - English M3 - Article N1 - L354725483 2009-07-06 http://www3.interscience.wiley.com/cgi-bin/fulltext/122233313/PDFSTART PY - 2009 SN - 0197-3851 1097-0223 SP - 570-577 ST - Identification of second trimester screen positive pregnancies at increased risk for congenital heart defects T2 - Prenatal Diagnosis TI - Identification of second trimester screen positive pregnancies at increased risk for congenital heart defects UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L354725483 http://dx.doi.org/10.1002/pd.2239 VL - 29 ID - 1180 ER - TY - JOUR AB - OBJECTIVE: To comprehensively characterize the immunologic characteristics of patients with protein-losing enteropathy (PLE) post-Fontan and compare them with patients without PLE post-Fontan. STUDY DESIGN: Patients with PLE post-Fontan and age-matched controls post-Fontan were prospectively studied with laboratory markers of immune function. Infectious history was obtained by interview and chart review. The groups' demographics, cardiac history, immune characteristics, and infection history were compared using appropriate 2-group statistics. RESULTS: A total of 16 patients enrolled (8 patients with PLE and 8 controls). All patients with PLE had lymphopenia compared with 25% of controls (P = .01). All patients with PLE had markedly depressed CD4 T cell counts (median 58 cells/μL) compared with controls (median 450 cells/μL, P = .0002); CD4% was also low in the PLE group (12.3%) and normal in control (36.9%, P = .004). Both groups had mildly depressed CD8 T cells and normal to slightly elevated natural killer and B-cell subsets. A majority of patients with PLE (62.5%) had negative titers to measles, mumps, and rubella vaccination, compared with no control Fontan with a negative titer (P = .03). Despite profoundly low CD4 counts, the frequency of infection was not different between groups with no reported opportunistic infections. CONCLUSIONS: Patients with Fontan-associated PLE have extensive quantitative immune abnormalities, particularly CD4 deficiency. These immune abnormalities are similar to those found in non-Fontan patients with PLE caused by intestinal lymphangiectasia. AD - University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, Ann Arbor, MI. Electronic address: hmagdo@med.umich.edu. University of Michigan Pediatric Infectious Disease, Ann Arbor, MI. University of Michigan Allergy and Immunology, Ann Arbor, MI. University of Michigan College of Pharmacy, Ann Arbor, MI. University of Michigan, Michigan Congenital Heart Outcomes Research and Discovery Ann Arbor, MI. University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, Ann Arbor, MI. AN - 26009017 AU - Magdo, H. S. AU - Stillwell, T. L. AU - Greenhawt, M. J. AU - Stringer, K. A. AU - Yu, S. AU - Fifer, C. G. AU - Russell, M. W. AU - Schumacher, K. R. DA - Aug DB - PubMed DO - 10.1016/j.jpeds.2015.04.061 DP - NLM ET - 2015/05/27 IS - 2 KW - CD4 Lymphocyte Count *CD4-Positive T-Lymphocytes *CD8-Positive T-Lymphocytes Case-Control Studies Child Child, Preschool Female Fontan Procedure/*adverse effects Heart Defects, Congenital/blood/immunology/*surgery Humans Immunoglobulin Isotypes/blood Infant Lymphopenia/*epidemiology Male Prospective Studies Protein-Losing Enteropathies/blood/*immunology LA - eng N1 - 1097-6833 Magdo, H Sonali Stillwell, Terri L Greenhawt, Matthew J Stringer, Kathleen A Yu, Sunkyung Fifer, Carlen G Russell, Mark W Schumacher, Kurt R Journal Article Research Support, Non-U.S. Gov't United States J Pediatr. 2015 Aug;167(2):331-7. doi: 10.1016/j.jpeds.2015.04.061. Epub 2015 May 23. PY - 2015 SN - 0022-3476 SP - 331-7 ST - Immune Abnormalities in Fontan Protein-Losing Enteropathy: A Case-Control Study T2 - J Pediatr TI - Immune Abnormalities in Fontan Protein-Losing Enteropathy: A Case-Control Study VL - 167 ID - 304 ER - TY - JOUR AB - We evaluated the effect of an interdisciplinary single-ventricle task force (SVTF) that utilizes a family-driven, telemedicine home monitoring program on clinical outcomes of stage II admissions and its acceptance by parents and cardiologists. Study population was divided into two cohorts, one with Norwood surgery dates before the SVTF (pre-SVTF) and one interventional (post-SVTF). Post-SVTF data also included surveys of parents and cardiologists on the efficacy of the SVTF. Comparative and multivariate statistical testing was performed. Compared to the pre-SVTF group, the post-SVTF group had lower complications after stage II (18.4 vs. 34.1 %, p = 0.02), higher weight-for-age z scores at stage II (−1.5 ± 0.97 vs. −1.58 ± 1.34, p = 0.02) and were less likely to have a stage II weight-for-age z score below −2 (26.5 vs. 31.7 %, p = 0.03). A multivariate regression analysis showed providing a written red-flag action plan to parents at discharge was independently associated with higher weight at stage II (β = 0.42, p = 0.04) and higher weight-for-age z score (β = 0.48, p = 0.02). Parents’ satisfaction with SVTF (α = 0.97) was 4.34 ± 0.62; (95 % CI 4.01–4.67) and cardiologists’ acceptance (α = 0.93) was 4.1 ± 0.7 (95 % CI 3.79–4.42). Development of SVTF was associated with a reduction in complications post-stage II and improved weight status at stage II. A written red-flag action plan provided to parents at the time of Norwood discharge was associated with higher weight status at stage II. Parents and cardiologists expressed satisfaction with the utility of SVTF and encouraged expansion to cover all children with congenital heart disease. AD - A.S. Harahsheh, Department of Pediatrics, Division of Cardiology, Children’s National Health System, The George Washington University School of Medicine, 111 Michigan Ave, N.W., Washington, DC, United States AU - Harahsheh, A. S. AU - Hom, L. A. AU - Clauss, S. B. AU - Cross, R. R. AU - Curtis, A. R. AU - Steury, R. D. AU - Mitchell, S. J. AU - Martin, G. R. DB - Embase Medline DO - 10.1007/s00246-016-1366-y IS - 5 KW - acute kidney failure adult article assisted circulation atrioventricular block cardiologist central line infection child child care chylothorax cohort analysis comparative study congenital heart disease diaphragm paralysis endocarditis female heart arrest home monitoring human intervention study major clinical study male mediastinitis necrotizing enterocolitis neurologic disease Norwood procedure outcome assessment parent patient satisfaction pericardial effusion phrenic nerve injury pleura effusion pneumonia pneumothorax postoperative complication postoperative hemorrhage pulmonary hypertension pulmonary vein obstruction recurrent laryngeal nerve injury reoperation respiratory failure seizure septicemia sternal dehiscence telemedicine tracheitis urinary tract infection vein occlusion vocal cord paralysis wound infection LA - English M3 - Article N1 - L609598507 2016-04-11 2016-07-11 PY - 2016 SN - 1432-1971 0172-0643 SP - 899-912 ST - The Impact of a Designated Cardiology Team Involving Telemedicine Home Monitoring on the Care of Children with Single-Ventricle Physiology After Norwood Palliation T2 - Pediatric Cardiology TI - The Impact of a Designated Cardiology Team Involving Telemedicine Home Monitoring on the Care of Children with Single-Ventricle Physiology After Norwood Palliation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L609598507 http://dx.doi.org/10.1007/s00246-016-1366-y VL - 37 ID - 804 ER - TY - JOUR AB - INTRODUCTION: Although the Fontan palliation is now being applied to younger patients, the influence of patients' age at the time of surgery on mid- and long-term results remains unclear. It has been our policy to perform Fontan operations when patients are around 5 years old in order to fit a larger conduit and minimize the risk of later obstruction. We sought to investigate whether age at Fontan completion affects subsequent clinical status in patients followed up for more than 3 years. METHODS: We carried out a cross-sectional study of Fontan patients who underwent total cavopulmonary connection between 1997 and 2009 and were followed up for 3 years with detailed chart review, ECG, echocardiogram, and functional status questionnaire. RESULTS: Of the total of 58 patients there were 56 long-term survivors. Of these, 41 had undergone surgery 3 years prior to the time of the study. We were able to contact 37 patients, who comprised our study cohort (mean follow up 6.3, range 3.0-11.0 years). Mean age at operation was 7.4 years (median 5.5, range 3.0-29.5 years). At latest follow up, 97% were in NYHA class I or II. In terms of somatic development, 21% (8/37) for weight and 19% (7/37) for height were below the 25th percentile for age-matched controls. Excluding one patient with developmental delay due to an underlying syndrome, there were 5 patients (13.5%) with mild learning disabilities and one with moderate delays. No additional surgeries had been performed. Tissue Doppler imaging measurements of systolic and diastolic velocities of the lateral annulus of the dominant ventricle revealed abnormalities of both systolic function and diastolic compliance, although qualitatively the systolic function of the single ventricle was considered adequate in all patients. CONCLUSIONS: Although earlier Fontan palliation is considered beneficial in the long term from the viewpoint of exercise capacity and hemodynamics, even late Fontan completion provides acceptable mid-term results in terms of the patients' functional status. AD - Dept. of Pediatric Cardiology, MITERA Children's Hospital, Athens, Greece. renalytrivi@yahoo.com AN - 21478121 AU - Lytrivi, I. D. AU - Sfyridis, P. G. AU - Papagiannis, J. AU - Kirvassilis, G. AU - Zavaropoulos, P. AU - Sarris, G. E. DA - Mar-Apr DB - PubMed DP - NLM ET - 2011/04/12 IS - 2 KW - Adolescent Adult *Age Factors Child *Child Development Child, Preschool Cross-Sectional Studies Female Follow-Up Studies *Fontan Procedure Humans Male Treatment Outcome Tricuspid Atresia/diagnosis/*psychology/*surgery Young Adult LA - eng N1 - 2241-5955 Lytrivi, Irene D Sfyridis, Panagiotis G Papagiannis, John Kirvassilis, George Zavaropoulos, Prodromos Sarris, George E Journal Article Netherlands Hellenic J Cardiol. 2011 Mar-Apr;52(2):118-22. PY - 2011 SN - 1109-9666 SP - 118-22 ST - Impact of age at fontan completion on functional status at mid-term follow up T2 - Hellenic J Cardiol TI - Impact of age at fontan completion on functional status at mid-term follow up VL - 52 ID - 338 ER - TY - JOUR AB - AIMS: An endothelin-1 receptor blocker, shown to be effective in patients with pulmonary arterial hypertension, might decrease pulmonary vascular resistance to increase cardiac filling and consequently improve exercise capacity in Fontan patients. METHODS AND RESULTS: This was a prospective, multicentre randomized open label trial in Fontan patients. One group received bosentan for 6 months. The other group did not receive study medication for the first 3 months, followed by bosentan for 6 months. The primary endpoint was exercise capacity, and secondary endpoints were NT-proBNP level, cardiac output, SF-36 (Short Form-36) quality of life (QoL), and NYHA class. Forty-two adults (median age 29 (range 18-56) years, 52% male, 88% NYHA class I-II) from five tertiary referral centres participated in the study. Ten patients were on diuretics. Ten patients were not motivated to finish the study. Analysis of all 32 patients who finished the study at 6 months of treatment showed that mean peak V'O2 (24 vs. 25 mL/kg/min), median SQUASH score (6614 vs. 6390), median NT-proBNP (314 vs. 274 ng/L), and mental QoL (50 vs. 51) remained unchanged as compared with baseline (P = NS, for all). After treatment, NYHA class had improved in 6 (19%), was unchanged in 24 (75%), and declined in 2 (6%) patients. Subgroup analysis on age, ventricular morphology, type of Fontan circulation, or baseline NT-proBNP level did not reveal efficacy of bosentan. Six transient adverse effects were reported. CONCLUSION: An increased NT-proBNP level was present in the majority of Fontan patients. Six months of bosentan treatment was not beneficial. Trial registration NTR1557. AD - Department of Cardiology, Academic Medical Centre, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands. AN - 23361871 AU - Schuuring, M. J. AU - Vis, J. C. AU - van Dijk, A. P. AU - van Melle, J. P. AU - Vliegen, H. W. AU - Pieper, P. G. AU - Sieswerda, G. T. AU - de Bruin-Bon, R. H. AU - Mulder, B. J. AU - Bouma, B. J. DA - Jun DB - PubMed DO - 10.1093/eurjhf/hft017 DP - NLM ET - 2013/01/31 IS - 6 KW - Adolescent Adult Antihypertensive Agents/adverse effects/*therapeutic use Bosentan Cardiac Output/physiology Echocardiography Exercise/*physiology Exercise Test Female Follow-Up Studies *Fontan Procedure Heart Defects, Congenital/*drug therapy/*physiopathology/surgery Humans Male Middle Aged Natriuretic Peptide, Brain/blood Peptide Fragments/blood Postoperative Period Prospective Studies Quality of Life Severity of Illness Index Sulfonamides/adverse effects/*therapeutic use Treatment Outcome Young Adult Exercise capacity Fontan LA - eng N1 - 1879-0844 Schuuring, Mark J Vis, Jeroen C van Dijk, Arie P J van Melle, Joost P Vliegen, Hubert W Pieper, Petronella G Sieswerda, Gertjan T de Bruin-Bon, Rianne H A C M Mulder, Barbara J M Bouma, Berto J Journal Article Multicenter Study Randomized Controlled Trial Research Support, Non-U.S. Gov't England Eur J Heart Fail. 2013 Jun;15(6):690-8. doi: 10.1093/eurjhf/hft017. Epub 2013 Jan 29. PY - 2013 SN - 1388-9842 SP - 690-8 ST - Impact of bosentan on exercise capacity in adults after the Fontan procedure: a randomized controlled trial T2 - Eur J Heart Fail TI - Impact of bosentan on exercise capacity in adults after the Fontan procedure: a randomized controlled trial VL - 15 ID - 316 ER - TY - JOUR AD - Department of Cardiology, Academic Medical Centre, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands. AN - 104074429. Language: English. Entry Date: 20140131. Revision Date: 20150710. Publication Type: Journal Article AU - Schuuring, Mark J. AU - Vis, Jeroen C. AU - van Dijk, Arie P. J. AU - van Melle, Joost P. AU - Vliegen, Hubert W. AU - Pieper, Petronella G. AU - Sieswerda, Gertjan T. AU - de Bruin-Bon, Rianne H. A. C. M. AU - Mulder, Barbara J. M. AU - Bouma, Berto J. DB - ccm DO - eurjhf/hft017 DP - EBSCOhost IS - 6 KW - Antihypertensive Agents -- Therapeutic Use Exercise Physiology Cardiopulmonary Bypass Heart Defects, Congenital -- Drug Therapy Heart Defects, Congenital -- Physiopathology Sulfonamides -- Therapeutic Use Adolescence Adult Antihypertensive Agents -- Adverse Effects Cardiac Output -- Physiology Echocardiography Exercise Test Female Prospective Studies Heart Defects, Congenital -- Surgery Human Male Middle Age Natriuretic Peptide, Brain -- Blood Peptides -- Blood Postoperative Period Quality of Life Severity of Illness Indices Sulfonamides -- Adverse Effects Treatment Outcomes Young Adult N1 - research; randomized controlled trial. Journal Subset: Biomedical; Blind Peer Reviewed; Continental Europe; Europe; Expert Peer Reviewed; Peer Reviewed. NLM UID: 100887595. PMID: NLM23361871. PY - 2013 SN - 1388-9842 SP - 690-698 ST - Impact of bosentan on exercise capacity in adults after the Fontan procedure: a randomized controlled trial T2 - European Journal of Heart Failure TI - Impact of bosentan on exercise capacity in adults after the Fontan procedure: a randomized controlled trial UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104074429&site=ehost-live&scope=site VL - 15 ID - 1570 ER - TY - JOUR AB - OBJECTIVES: The exercise capacity of children with congenital heart disease (CHD) is often depressed. This depression is thought to be attributable to (1) residual hemodynamic defects and (2) deconditioning secondary to physical inactivity. We hypothesized that this latter component would be ameliorated by a formal cardiac rehabilitation program designed specifically for children. The objective of this study was to characterize the effect of a cardiac rehabilitation program on the exercise performance of children with CHD and to define the physiologic mechanisms that might account for any improvements that are observed. METHODS: Nineteen patients with CHD who were referred for exercise testing and found to have a peak oxygen consumption (VO2) and/or peak work rate <80% of predicted were enrolled in the study. Sixteen patients (11 Fontan patients, 5 with other CHD) completed the program and had postrehabilitation exercise tests, results of which were compared with the prerehabilitation studies. RESULTS: Improvements were found in 15 of 16 patients. Peak VO2 rose from 26.4 +/- 9.1 to 30.7 +/- 9.2 mL/kg per min; peak work rate from 93 +/- 32 to 106 +/- 34 W, and the ventilatory anaerobic threshold from 14.2 +/- 4.8 to 17.4 +/- 4.5 mL/kg per min. The peak heart rate and peak respiratory exchange ratio did not change, suggesting that the improvements were not attributable merely to an increased effort. In contrast, the peak oxygen pulse rose significantly, from 7.6 +/- 2.8 to 9.7 +/- 4.1 mL/beat, an improvement that can be attributed only to an increase in stroke volume and/or oxygen extraction at peak exercise. No patient experienced rehabilitation-related complications. CONCLUSION: Cardiac rehabilitation can improve the exercise performance of children with CHD. This improvement is mediated by an increase in stroke volume and/or oxygen extraction during exercise. Routine use of formal cardiac rehabilitation may greatly reduce the morbidity of complex CHD. AD - Department of Pediatric Cardiology, Children's Hospital, Boston, Massachusetts, USA. jonathan.rhodes@cardio.chboston.org AN - 16322156 AU - Rhodes, J. AU - Curran, T. J. AU - Camil, L. AU - Rabideau, N. AU - Fulton, D. R. AU - Gauthier, N. S. AU - Gauvreau, K. AU - Jenkins, K. J. DA - Dec DB - PubMed DO - 10.1542/peds.2004-2697 DP - NLM ET - 2005/12/03 IS - 6 KW - Adolescent Child Exercise/*physiology Exercise Test Female Heart Defects, Congenital/*physiopathology/*rehabilitation/surgery Humans Male Pilot Projects LA - eng N1 - 1098-4275 Rhodes, Jonathan Curran, Tracy J Camil, Laurel Rabideau, Nicole Fulton, David R Gauthier, Naomi S Gauvreau, Kimberlee Jenkins, Kathy J 5U01 HL068285/HL/NHLBI NIH HHS/United States Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't United States Pediatrics. 2005 Dec;116(6):1339-45. doi: 10.1542/peds.2004-2697. PY - 2005 SN - 0031-4005 SP - 1339-45 ST - Impact of cardiac rehabilitation on the exercise function of children with serious congenital heart disease T2 - Pediatrics TI - Impact of cardiac rehabilitation on the exercise function of children with serious congenital heart disease VL - 116 ID - 508 ER - TY - JOUR AB - BACKGROUND AND AIM: Chylothorax is the accumulation of chyle in the pleural cavity, which usually develops after disruption of the thoracic duct along its intra-thoracic route. In the majority of cases, this rupture is secondary to trauma (including cardio thoracic surgeries). Chylothorax is a potentially serious complication after cardiovascular surgeries that require early diagnosis and adequate management. This study aims to determine the risk factors and the impact of chylothorax on the early postoperative course after pediatric cardiac surgery. METHODS: A retrospective study of all cases complicated with chylothorax after pediatric cardiac surgery was conducted at King Abdulaziz Cardiac Center between January 2007 and December 2009. RESULTS: There were 1135 cases operated on during the study period. Of these, 57 cases (5%) were complicated by chylothorax in the postoperative period. Thirty patients (54%) were males, while 27 (47%) were females. Ages ranged from 4 to 2759 days. The most common surgeries complicated by chylothorax were the single ventricle repair surgeries (15 cases, 27%); arch repairs (10 cases, 18%); ventricular septal defect repairs (10 cases, 18%); atrioventricular septal defect repairs (7 cases, 12%); arterial switch repair (6 cases, 11%), and others (8 cases, 14%). The intensive care unit (ICU) and the length of hospital stays were significantly longer in the chylothorax group. Additionally, some early postoperative parameters such as incidence of sepsis, ventilation time, and inotropes duration and number were higher in the chylothorax group. CONCLUSION: Chylothorax after pediatric cardiac surgery is not a rare complication. It occurs more commonly with single ventricle repair and aortic arch repair surgeries, and has a significant impact on the postoperative course and post operative morbidity. AD - King Abdulaziz Medical City, King Saud University for Health Sciences, Department of Cardiac Sciences, National Guard Hospital Health Affairs, Riyadh. AN - 24719538 AU - Ismail, S. R. AU - Kabbani, M. S. AU - Najm, H. K. AU - Shaath, G. A. AU - Jijeh, A. M. AU - Hijazi, O. M. C2 - PMC3978859 DA - Apr DB - PubMed DO - 10.1016/j.jsha.2014.01.001 DP - NLM ET - 2014/04/11 IS - 2 KW - Cardiac surgery Chylothorax Pediatric Post operative LA - eng N1 - 2212-5043 Ismail, Sameh R Kabbani, Mohamed S Najm, Hani K Shaath, Ghassan A Jijeh, Abdulraouf M Z Hijazi, Omar M Journal Article J Saudi Heart Assoc. 2014 Apr;26(2):87-92. doi: 10.1016/j.jsha.2014.01.001. Epub 2014 Jan 13. PY - 2014 SN - 1016-7315 (Print) 1016-7315 SP - 87-92 ST - Impact of chylothorax on the early post operative outcome after pediatric cardiovascular surgery T2 - J Saudi Heart Assoc TI - Impact of chylothorax on the early post operative outcome after pediatric cardiovascular surgery VL - 26 ID - 383 ER - TY - JOUR AB - Background and aim: Chylothorax is the accumulation of chyle in the pleural cavity, which usually develops after disruption of the thoracic duct along its intra-thoracic route. In the majority of cases, this rupture is secondary to trauma (including cardio thoracic surgeries). Chylothorax is a potentially serious complication after cardiovascular surgeries that require early diagnosis and adequate management. This study aims to determine the risk factors and the impact of chylothorax on the early postoperative course after pediatric cardiac surgery. Methods: A retrospective study of all cases complicated with chylothorax after pediatric cardiac surgery was conducted at King Abdulaziz Cardiac Center between January 2007 and December 2009. Results: There were 1135 cases operated on during the study period. Of these, 57 cases (5%) were complicated by chylothorax in the postoperative period. Thirty patients (54%) were males, while 27 (47%) were females. Ages ranged from 4 to 2759. days. The most common surgeries complicated by chylothorax were the single ventricle repair surgeries (15 cases, 27%); arch repairs (10 cases, 18%); ventricular septal defect repairs (10 cases, 18%); atrioventricular septal defect repairs (7 cases, 12%); arterial switch repair (6 cases, 11%), and others (8 cases, 14%). The intensive care unit (ICU) and the length of hospital stays were significantly longer in the chylothorax group. Additionally, some early postoperative parameters such as incidence of sepsis, ventilation time, and inotropes duration and number were higher in the chylothorax group. Conclusion: Chylothorax after pediatric cardiac surgery is not a rare complication. It occurs more commonly with single ventricle repair and aortic arch repair surgeries, and has a significant impact on the postoperative course and post operative morbidity. © 2014 King Saud University. AD - S.R. Ismail, Department of Cardiac Sciences (MC: 1420), King Abdulaziz Medical City, P.O. Box 22490, Riyadh 11426, Saudi Arabia AU - Ismail, S. R. AU - Kabbani, M. S. AU - Najm, H. K. AU - Shaath, G. A. AU - Jijeh, A. M. Z. AU - Hijazi, O. M. DB - Embase DO - 10.1016/j.jsha.2014.01.001 IS - 2 KW - aortic arch surgery article atrioventricular septal defect cardiovascular risk cardiovascular surgery chylothorax controlled study female heart single ventricle heart ventricle septum defect hospitalization human incidence infant intensive care unit major clinical study male pediatrics postoperative complication postoperative period priority journal retrospective study sepsis treatment outcome LA - English M3 - Article N1 - L52996747 2014-02-18 2014-03-27 PY - 2014 SN - 2212-5043 1016-7315 SP - 87-92 ST - Impact of chylothorax on the early post operative outcome after pediatric cardiovascular surgery T2 - Journal of the Saudi Heart Association TI - Impact of chylothorax on the early post operative outcome after pediatric cardiovascular surgery UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52996747 http://dx.doi.org/10.1016/j.jsha.2014.01.001 VL - 26 ID - 949 ER - TY - JOUR AB - The objective of this analysis was to update trends in LOS and costs by survivorship and ECMO use among neonates with hypoplastic left heart syndrome (HLHS) undergoing stage 1 palliation surgery using 2016 data from the Healthcare Cost and Utilization Project Kids’ Inpatient Database. We identified neonates ≤ 28 days old with HLHS undergoing Stage 1 surgery, defined as a Norwood procedure with modified Blalock–Taussig (BT) shunt, Sano modification, or both. Multivariable regression with year random effects was used to compare LOS and costs by hospital region, case volume, survivorship, and ECMO vs. no ECMO. An E-value analysis, an approach for conducting sensitivity analysis for unmeasured confounding, was performed to determine if unmeasured confounding contributed to the observed effects. Significant differences in total costs, LOS, and mortality were noted by hospital region, ECMO use, and sub-analyses of case volume. However, other than ECMO use and mortality, the maximum E-value confidence interval bound was 1.71, suggesting that these differences would disappear with an unmeasured confounder 1.71 times more associated with both the outcome and exposure (e.g., socioeconomic factors, environment, etc.) Our findings confirm previous literature demonstrating significant resource utilization among Norwood patients, particularly those undergoing ECMO use. Based on our E-value analysis, differences by hospital region and case volume can be explained by moderate unobserved confounding, rather than a reflection of the quality of care provided. Future analyses on surgical quality must account for unobserved factors to provide meaningful information for quality improvement. AD - C.L. Gong, Division of Neonatology, Department of Pediatrics, Fetal & Neonatal Institute, Children’s Hospital Los Angeles, 4650 Sunset Boulevard, MS #31, Los Angeles, CA, United States AU - Gong, C. L. AU - Song, A. Y. AU - Horak, R. AU - Friedlich, P. S. AU - Lakshmanan, A. AU - Pruetz, J. D. AU - Yieh, L. AU - Ram Kumar, S. AU - Williams, R. G. DB - Embase Medline DO - 10.1007/s00246-020-02348-5 KW - article Blalock Taussig shunt child controlled study health care cost health services research hospital patient human hypoplastic left heart syndrome length of stay mortality newborn Norwood procedure sensitivity analysis socioeconomics survivorship total quality management LA - English M3 - Article in Press N1 - L2004783819 2020-05-08 PY - 2020 SN - 1432-1971 0172-0643 ST - Impact of Confounding on Cost, Survival, and Length-of-Stay Outcomes for Neonates with Hypoplastic Left Heart Syndrome Undergoing Stage 1 Palliation Surgery T2 - Pediatric Cardiology TI - Impact of Confounding on Cost, Survival, and Length-of-Stay Outcomes for Neonates with Hypoplastic Left Heart Syndrome Undergoing Stage 1 Palliation Surgery UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2004783819 http://dx.doi.org/10.1007/s00246-020-02348-5 ID - 572 ER - TY - JOUR AB - Background The importance of registries for collaborative quality improvement has been overlooked in low/middle-income countries (LMIC). Aga Khan University Hospital (AKUH) in Pakistan joined the Congenital Cardiac Catheterization Project on Outcomes-Quality Improvement (C3PO-QI) in March 2017 with the goal of leveraging international collaboration to improve patient care and institutional standards. Methods The C3PO-QI key driver-based approach was used, with certain modifications, for process re-engineering in AKUH's congenital cardiac catheterisation laboratory (CCL) to reduce radiation exposure during cardiac catheterisation procedures (the primary outcome of C3PO- QI). Educating staff and standardising procedural documentation were the principal goals of the process re-engineering. Data survey was used to assess staff knowledge, attitude and practice before and after the initiative. Additionally, case demographics and outcomes were compared between AKUH and C3PO-QI centres. Results There was an increase in appropriate recording of radiation surrogates (0%-100%, p=0.00) and in the percentage of cases that met the established benchmark of 'Ideal documentation' (35% vs 95%, p=0.001). There was also an increase in self-reported staff interest during the case (25% vs 75%, p=0.001). AKUH versus C3PO-QI data showed similar demographic characteristics. There was a slight over-representation of diagnostic cases (42% vs 32%) as compared with interventional (58% vs 68%) at AKUH. Furthermore, interventional procedures were predominately PDA and ASD device closures (n=19 and 15, respectively). The frequency of adverse events were the same between AKUH and collaborative sites. Conclusion Collaborative efforts between developed and LMIC CCL are significant in advancing system-level processes. AD - B. Hasan, Department of Pediatrics and Child Health, Aga Khan University Hospital, Karachi, Pakistan AU - Ali, F. AU - Qasim Mehdi, M. AU - Akhtar, S. AU - Aslam, N. AU - Abbas, R. AU - Shah, I. AU - Abidi, J. AU - Arthur, S. AU - Nizar, Z. AU - Goodmann, A. AU - Bergersen, L. AU - Hasan, B. DB - Embase DO - 10.1136/heartasia-2018-011105 IS - 1 KW - cardiovascular stent patent ductus arteriosus stent adolescent adverse event aortic coarctation aortic stenosis aortic valve repair article atrioventricular septal defect child clinical laboratory clinical outcome clinical practice comparative study congenital heart disease cross-sectional study demography descriptive research female fluoroscopy great vessels transposition health personnel attitude heart arrhythmia heart atrium septum defect heart catheterization heart hemodynamics heart single ventricle heart ventricle septum defect hematoma human interventional cardiovascular procedure low income country major clinical study male medical documentation medical staff middle income country patent ductus arteriosus preschool child priority journal professional knowledge pulmonary valve atresia pulmonary valve stenosis pulmonary valvuloplasty radiation dose distribution radiation exposure radiation hazard radiation protection sedation self report staff training standardization stent thrombosis teaching hospital total quality management LA - English M3 - Article N1 - L625911240 2019-01-18 2019-01-24 PY - 2019 SN - 1759-1104 ST - Impact of Congenital Cardiac Catheterization Project on Outcomes-Quality Improvement (C3PO-QI) in LMICs T2 - Heart Asia TI - Impact of Congenital Cardiac Catheterization Project on Outcomes-Quality Improvement (C3PO-QI) in LMICs UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L625911240 http://dx.doi.org/10.1136/heartasia-2018-011105 VL - 11 ID - 640 ER - TY - JOUR AB - OBJECTIVES: We sought to determine if early ventricular volume unloading improves aerobic capacity in patients with single ventricle Fontan physiology. BACKGROUND: Surgical strategies for patients with single ventricle include intermediate staging or early Fontan completion to reduce the adverse affects of prolonged ventricular volume load. The impact of this strategy on exercise performance has not been evaluated. METHODS: Retrospectively, we reviewed the exercise stress test results of all preadolescents with single ventricle Fontan physiology. "Volume unloading" was considered to have occurred at the time of bidirectional cavopulmonary anastomosis or at the time of Fontan surgery in those patients who did not undergo intermediate staging. Potential predictors of aerobic capacity were analyzed using multivariate regression. RESULTS: The patients (n = 46) achieved a mean percentage predicted of maximal oxygen consumption (VO2max) of 76.1% +/- 21.1%. The mean age at the time of volume unloading was 2.7 +/- 2.4 years, and the mean age at testing was 8.7 +/- 2 years. Intermediate staging was performed in 16 of 46 patients (35%). In multivariate analysis, younger age at volume unloading was associated with increased aerobic capacity (p = 0.003). Other variables were not predictive. The subgroup of patients who underwent volume unloading before two years of age achieved a mean percentage predicted VO2max of 88.6% +/- 24.1%. CONCLUSIONS: Preadolescents with single ventricle who undergo volume unloading surgery at an early age demonstrate superior aerobic capacity compared with those whose surgery is delayed until a later age. AD - Cardiovascular Exercise Physiology Laboratory, Division of Cardiology, The Children's Hospital of Philadelphia, Pennsylvania, USA. AN - 10551717 AU - Mahle, W. T. AU - Wernovsky, G. AU - Bridges, N. D. AU - Linton, A. B. AU - Paridon, S. M. DA - Nov 1 DB - PubMed DO - 10.1016/s0735-1097(99)00392-7 DP - NLM ET - 1999/11/07 IS - 5 KW - Age Factors Child Exercise Test Female *Fontan Procedure Heart Defects, Congenital/*physiopathology/*surgery Humans Hypoplastic Left Heart Syndrome/physiopathology/*surgery Male *Oxygen Consumption Respiratory Function Tests Retrospective Studies Stroke Volume Tricuspid Atresia/physiopathology/surgery LA - eng N1 - Mahle, W T Wernovsky, G Bridges, N D Linton, A B Paridon, S M Journal Article United States J Am Coll Cardiol. 1999 Nov 1;34(5):1637-43. doi: 10.1016/s0735-1097(99)00392-7. PY - 1999 SN - 0735-1097 (Print) 0735-1097 SP - 1637-43 ST - Impact of early ventricular unloading on exercise performance in preadolescents with single ventricle Fontan physiology T2 - J Am Coll Cardiol TI - Impact of early ventricular unloading on exercise performance in preadolescents with single ventricle Fontan physiology VL - 34 ID - 419 ER - TY - JOUR AB - Extracorporeal membrane oxygenation (ECMO) is widely used for circulatory support in pediatric cardiac patients with low cardiac output and hypoxemia. We retrospectively evaluated the efficacy of ECMO support for respiratory and heart failure in infants and children. From April 2002 to February 2011, 14 patients aged 19 days to 20 years old (average 44 months), with body weight 2.6 kg to 71 kg (median 14.1 kg), underwent ECMO support for failing cardiac function, hypoxemia, and low cardiac output syndrome. In 12 patients, ECMO was introduced after operation for congenital heart disease (four with complete repair including Fontan circulation, and eight with palliative repair). In one patient, ECMO was introduced after partial pulmonary resection for congenital cystic adenomatoid malformation because of respiratory failure. ECMO was introduced in a patient with severe heart failure caused by fulminant myocarditis. Patients' demographics, duration of extracorporeal membrane oxygenation, additional support, and outcomes were analyzed. Ten patients (71%) were successfully weaned from ECMO, and eight patients (57%) were discharged from the hospital. The mean duration of ECMO support was 332 h (range 11-2030 h). Although management of the ECMO circuit, including anticoagulation (activated clotting time: 150-250), was conducted following the institutional practice guidelines, it was difficult to control the bleeding. Seven patients required renal replacement therapy during ECMO support using peritoneal dialysis or continuous hemodiafiltration. Five patients had additional operative procedures: systemic-pulmonary shunt in two, bidirectional Glenn takedown with right modified Blalock-Taussig shunt, total cavopulmonary connection takedown, and redo ECMO in one patient each. The patient who had the longest ECMO support for respiratory failure due to acute respiratory distress syndrome after lung surgery was successfully weaned from ECMO because high-frequency oscillation (HFO) improved respiratory function. ECMO for heart and respiratory failure in infants and children is effective and allows time for recovery of cardiac dysfunction and acute hypoxic insult. The long-term ECMO support for over 2000 h was very rare, but it was possible to wean this patient from ECMO using HFO. AD - Department of Clinical Engineering, Hirosaki University School of Medicine and Hospital, Hirosaki, Aomori, Japan. AN - 22097977 AU - Goto, T. AU - Suzuki, Y. AU - Suzuki, Y. AU - Osanai, A. AU - Aoki, K. AU - Yamazaki, A. AU - Daitoku, K. AU - Fukuda, I. DA - Nov DB - PubMed DO - 10.1111/j.1525-1594.2011.01374.x DP - NLM ET - 2011/11/22 IS - 11 KW - Adult Child Child, Preschool *Extracorporeal Membrane Oxygenation/instrumentation/methods Heart Failure/*therapy Humans Infant Respiratory Distress Syndrome, Adult/therapy Respiratory Insufficiency/*therapy Retrospective Studies Survival Analysis Treatment Outcome Young Adult LA - eng N1 - 1525-1594 Goto, Takeshi Suzuki, Yasuyuki Suzuki, Yuta Osanai, Ai Aoki, Kaori Yamazaki, Akio Daitoku, Kazuyuki Fukuda, Ikuo Journal Article United States Artif Organs. 2011 Nov;35(11):1002-9. doi: 10.1111/j.1525-1594.2011.01374.x. PY - 2011 SN - 0160-564x SP - 1002-9 ST - The impact of extracorporeal membrane oxygenation on survival in pediatric patients with respiratory and heart failure: review of our experience T2 - Artif Organs TI - The impact of extracorporeal membrane oxygenation on survival in pediatric patients with respiratory and heart failure: review of our experience VL - 35 ID - 284 ER - TY - JOUR AB - OBJECTIVE: In the long-term period after Fontan operation, atrial arrhythmia was one of the important factors to decide the postoperative quality of life. We reviewed the impact of Fontan conversion with arrhythmia surgery and pacemaker therapy. METHODS: Thirty-eight patients underwent Fontan conversion using extracardiac conduit from 1992, and 22 patients with atrial arrhythmia underwent maze procedure simultaneously using cryoablation or radiofrequency ablation and epicardial DDD pacemaker implantation and 16 patients had regular 'sinus' rhythm before Fontan conversion. Mean follow-up period was 52 months. Pre- and postoperative clinical course were analyzed. Average weight, age at Fontan conversion, and years after first Fontan operation were 49.0 kg, 25.8 years old, 14.7 years, respectively. Nineteen percent of patients were in New York Heart Association class I (NYHA I), and 74% of patients were in NYHA II, and 7% were in NYHA III, respectively. RESULTS: Except three early deaths, actual survival rate at 1 year and 5 years were 80% and 64%, respectively. In survivors, 80% of the patients obtained regular heart rhythm including artificial pacemaker rhythm, although only 43% of the patients had regular 'sinus' rhythm before the Fontan conversion. Postoperative average cardiothoracic ratio and oxygen saturation (SpO(2)) were 50% and 94%, and 74% of patients were in NYHA I and 26% were in NYHA II, respectively, after Fontan conversion. CONCLUSIONS: Mid-term results of Fontan conversion with arrhythmia surgery and pacemaker therapy were acceptable. Restoration of regular rhythm might improve the postoperative NYHA status and the activity of the daily life. AD - Department of Cardiovascular Surgery, Tokyo Women's Medical University, Tokyo, Japan. shiramat@hij.twmu.ac.jp AN - 21354811 AU - Hiramatsu, T. AU - Iwata, Y. AU - Matsumura, G. AU - Konuma, T. AU - Yamazaki, K. DA - Oct DB - PubMed DO - 10.1016/j.ejcts.2011.01.022 DP - NLM ET - 2011/03/01 IS - 4 KW - Adult Arrhythmias, Cardiac/etiology/*surgery Catheter Ablation/methods Cryosurgery/methods Follow-Up Studies Fontan Procedure/adverse effects/*methods Humans *Pacemaker, Artificial Quality of Life Reoperation/methods Survival Analysis Treatment Outcome Young Adult LA - eng N1 - 1873-734x Hiramatsu, Takeshi Iwata, Yusuke Matsumura, Goki Konuma, Takeshi Yamazaki, Kenji Evaluation Study Journal Article Germany Eur J Cardiothorac Surg. 2011 Oct;40(4):1007-10. doi: 10.1016/j.ejcts.2011.01.022. Epub 2011 Feb 26. PY - 2011 SN - 1010-7940 SP - 1007-10 ST - Impact of Fontan conversion with arrhythmia surgery and pacemaker therapy T2 - Eur J Cardiothorac Surg TI - Impact of Fontan conversion with arrhythmia surgery and pacemaker therapy VL - 40 ID - 306 ER - TY - JOUR AB - Left ventricular dimensions and contractility were determined by echocardiography in 33 patients with tricuspid atresia in 1985 and again in 1988. Eight patients remained palliated throughout the 3-year period; neither the left ventricular end-diastolic diameter (153 ± 15% of normal vs. 157 ± 19%, p = NS) nor a load-independent index of contractility (rate-corrected velocity of shortening [VCFc]/end-systolic meridional stress [ESSM]) changed. Eleven patients underwent a Fontan operation during the study and were reevaluated at least 6 months after surgery; left ventricular dimension decreased (130 ± 15% vs. 114 ± 19%, p < 0.001), and the contractility index VCFc/ESSM improved (p < 0.05). Fourteen patients had undergone a Fontan operation 0.9-9.5 years (mean, 4.2 years) before initial examination in 1985. Over the 3-year period, left ventricular dimensions did not change (121 ± 17% vs. 118 ± 11%, p = NS), but the contractility index showed significant improvement (p < 0.01). Eight additional patients were studied just before and after a Fontan operation to examine the early effects of surgery. Left ventricular dimensions decreased from 130 ± 14% to 100 ± 13% by 10 days p < 0.001) with no further change at 2 months. An inappropriate degree of ventricular hypertrophy was observed in only the early postoperative period. Successful Fontan repair resutls in rapid reduction of left ventricular size, followed by regression of hypertrophy to a normal mass-to-volume ratio. Operating at more favorable dimensions and loading conditions results in an early increase in left ventricular contractility, which further improves in the medium term follow-up. AD - R.K.H. Wyse, Dept. of Paediatric Cardiology, Hospital for Sick Children, Great Ormond Street, London WC1 3JH, United Kingdom AU - Gewillig, M. H. AU - Lundstrom, U. R. AU - Deanfield, J. E. AU - Bull, C. AU - Franklin, R. C. AU - Graham Jr, T. P. AU - Wyse, R. K. DB - Embase Medline IS - 1 KW - adolescent article child clinical article diagnosis echocardiography female heart left ventricle performance human male priority journal renard fontaine syndrome school child tricuspid valve atresia LA - English M3 - Article N1 - L20031775 1990-03-02 PY - 1990 SN - 0009-7322 SP - 118-127 ST - Impact of Fontan operation on left ventricular size and contractility in tricuspid atresia T2 - Circulation TI - Impact of Fontan operation on left ventricular size and contractility in tricuspid atresia UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L20031775 VL - 81 ID - 1387 ER - TY - JOUR AB - BACKGROUND: Children and young adults with single-ventricle physiology have abnormal exercise capacity after the Fontan operation. A medication capable of decreasing pulmonary vascular resistance should allow improved cardiac filling and improved exercise capacity. METHODS AND RESULTS: This study was a double-blind, placebo-controlled, crossover trial conducted in children and young adults after Fontan. Subjects were randomized to receive placebo or sildenafil (20 mg three times daily) for 6 weeks. After a 6-week washout, subjects crossed over for an additional 6 weeks. Each subject underwent an exercise stress test at the start and finish of each phase. After taking sildenafil, subjects had a significantly decreased respiratory rate and decreased minute ventilation at peak exercise. At the anaerobic threshold, subjects had significantly decreased ventilatory equivalents of carbon dioxide. There was no change in oxygen consumption during peak exercise, although there was a suggestion of improved oxygen consumption at the anaerobic threshold. Improvement at the anaerobic threshold was limited to the subgroup with single left or mixed ventricular morphology and to the subgroup with baseline serum brain natriuretic peptide levels ≥100 pg/mL. CONCLUSIONS: In this cohort, sildenafil significantly improved ventilatory efficiency during peak and submaximal exercise. There was also a suggestion of improved oxygen consumption at the anaerobic threshold in 2 subgroups. These findings suggest that sildenafil may be an important agent for improving exercise performance in children and young adults with single-ventricle physiology after the Fontan operation. Clinical Trial Registration- URL: http://clinicaltrials.gov. Unique identifier: NCT00507819. AD - Division of Cardiology, The Children's Hospital of Philadelphia, 34th and Civic Center Blvd, Philadelphia, PA 19104, USA. AN - 21382896 AU - Goldberg, D. J. AU - French, B. AU - McBride, M. G. AU - Marino, B. S. AU - Mirarchi, N. AU - Hanna, B. D. AU - Wernovsky, G. AU - Paridon, S. M. AU - Rychik, J. C2 - PMC3073351 C6 - NIHMS276840 DA - Mar 22 DB - PubMed DO - 10.1161/circulationaha.110.981746 DP - NLM ET - 2011/03/09 IS - 11 KW - Administration, Oral Adolescent Adult Child Cross-Over Studies Double-Blind Method *Exercise Female *Fontan Procedure Heart Defects, Congenital/physiopathology/*surgery Humans Male Natriuretic Peptide, Brain/blood Oxygen Consumption/drug effects Phosphodiesterase 5 Inhibitors/*therapeutic use Piperazines/adverse effects/*therapeutic use Purines/adverse effects/therapeutic use Sildenafil Citrate Sulfones/adverse effects/*therapeutic use Young Adult LA - eng N1 - 1524-4539 Goldberg, David J French, Benjamin McBride, Michael G Marino, Bradley S Mirarchi, Nicole Hanna, Brian D Wernovsky, Gil Paridon, Stephen M Rychik, Jack T32 HL007915/HL/NHLBI NIH HHS/United States T32 HL007915-10/HL/NHLBI NIH HHS/United States HL07915/HL/NHLBI NIH HHS/United States Journal Article Randomized Controlled Trial Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't Circulation. 2011 Mar 22;123(11):1185-93. doi: 10.1161/CIRCULATIONAHA.110.981746. Epub 2011 Mar 7. PY - 2011 SN - 0009-7322 (Print) 0009-7322 SP - 1185-93 ST - Impact of oral sildenafil on exercise performance in children and young adults after the fontan operation: a randomized, double-blind, placebo-controlled, crossover trial T2 - Circulation TI - Impact of oral sildenafil on exercise performance in children and young adults after the fontan operation: a randomized, double-blind, placebo-controlled, crossover trial VL - 123 ID - 261 ER - TY - JOUR AB - Objectives: This study examines university graduation rates among individuals with congenital heart disease (CHD) in comparison to their healthy peers. The effect of disease severity, type of surgery, and timing of surgery on graduation rate was also evaluated. Study design: One hundred forty-five male patients with CHD at military age were enrolled in the study between the dates of January 2005 and May 2007. Severity of disease was operationalised in term of initial diagnosis (According to classification of 32th ACC Bethesta Conference Task Force 1). University graduation rates of among two groups of CHD patients (mild disease (group 1) or moderate to severe disease (group 2)) are compared to each other and to healthy peers. Results: Patients with CHD have reduced rates of participation in higher education compared with healthy individuals (13.1% vs 20.7%, p=0.01). Furthermore, this negative effect on education participation rate is independent of the severity of disease (group 1, 16.4%, p=0.01; group 2, 9.7%, p<0.001). Although the university graduation rate was relatively higher in patients with mild disease severity, no significant difference was found between the two patient groups (p=0.23). Having an operation does not effect graduation rate (p=0.58), however greater age at the time of operation increases the likelihood of graduation (p=0.02). Conclusion: Being born with CHD significantly reduces the chance of completing higher education. This negative impact on university graduation rate is independent of the severity of the disease. No negative effects of disease related surgery or subsequent corrective surgery on education were observed. Patients who were operated on later in life were more likely to complete university education. Mean operation age of this group corresponds to the typical age during the last year of elementary school in Turkey. © 2012 Turkish Society of Cardiology. AD - Department of Cardiology, Semmelweis University Heart Center, Budapest, Hungary Department of Cardiology, Kasimpaşa Military Hospital, Istanbul, Turkey AU - Özcan, E. E. AU - Küçük, A. DB - Scopus DO - 10.5543/tkda.2012.21549 IS - 3 KW - Adult Cardiology/education Cardiovascular/ etiology Congenital Heart defects Quality of life M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2012 SP - 229-234 ST - Impact of severity of congenital heart diseases on university graduation rate among male patients T2 - Turk Kardiyoloji Dernegi Arsivi TI - Impact of severity of congenital heart diseases on university graduation rate among male patients UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84864196695&doi=10.5543%2ftkda.2012.21549&partnerID=40&md5=2f48f624753441c86d8765de47bdff19 VL - 40 ID - 2098 ER - TY - JOUR AB - BACKGROUND: Surgical complexity and related morbidities may affect long-term patient quality of life (QOL). Aristotle Basic Complexity (ABC) score and Risk Adjustment in Congenital Heart Surgery (RACHS-1) category stratify the complexity of pediatric cardiac operations. The purpose of this study was to examine the relationship between surgical complexity and QOL and to investigate other demographic and clinical variables that might explain variation in QOL in pediatric cardiac surgical survivors. METHODS AND RESULTS: Pediatric Cardiac Quality of Life (PCQLI) study participants who had undergone cardiac surgery were included. The PCQLI database provided sample characteristics and QOL scores. Surgical complexity was defined by the highest ABC raw score or RACHS-1 category. Relationships among surgical complexity and demographic, clinical, and QOL variables were assessed using ordinary least squares regression. A total of 1416 patient-parent pairs were included. Although higher ABC scores and RACHS-1 categories were associated with lower QOL scores (P<0.005), correlation with QOL scores was poor to fair (r=-0.10 to -0.29) for all groups. Ordinary least squares regression showed weak association with R(2)=0.06 to R(2)=0.28. After accounting for single-ventricle anatomy, number of doctor visits, and time since last hospitalization, surgical complexity scores added no additional explanation to the variance in QOL scores. CONCLUSIONS: ABC scores and RACHS-1 categories are useful tools for morbidity and mortality predictions prior to cardiac surgery and quality of care initiatives but are minimally helpful in predicting a child's or adolescent's long-term QOL scores. Further studies are warranted to determine other predictors of QOL variation. AD - Division of Cardiology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH. Cardiorespiratory Department, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK Department of Pediatric Cardiology, Royal Brompton and Harefield NHS Foundation Trust, London, UK. Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH. Division of Thoracic and Cardiovascular Surgery, The Congenital Heart Institute of Florida, All Children's Hospital and Children's Hospital of Tampa, University of South Florida College of Medicine, Cardiac Surgical Associates, St. Petersburg and Tampa, FL. Department of Cardiology, Children's Hospital Boston, Harvard Medical School, Boston, MA Department of Pediatrics, Harvard Medical School, Boston, MA. Cardiorespiratory Department, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK. Department of Pediatric Cardiology, Royal Brompton and Harefield NHS Foundation Trust, London, UK. Division of Cardiology, Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX. Division of Cardiology, Department of Pediatrics, The Children's Hospital of Wisconsin, Milwaukee, WI. Division of Cardiology, Department of Pediatrics, The Children's Hospital of Philadelphia, PA Division of Critical Care Medicine, Department of Anesthesiology and Critical Care, The Children's Hospital of Philadelphia, PA. Division of Behavioral Medicine and Clinical Psychology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH. Division of Cardiology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH Division of Critical Care Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH bradley.marino@northwestern.edu. AN - 27451455 AU - O'Connor, A. M. AU - Wray, J. AU - Tomlinson, R. S. AU - Cassedy, A. AU - Jacobs, J. P. AU - Jenkins, K. J. AU - Brown, K. L. AU - Franklin, R. C. AU - Mahony, L. AU - Mussatto, K. AU - Newburger, J. W. AU - Wernovsky, G. AU - Ittenbach, R. F. AU - Drotar, D. AU - Marino, B. S. C2 - PMC5015349 DA - Jul 22 DB - PubMed DO - 10.1161/jaha.114.001234 DP - NLM ET - 2016/07/28 IS - 7 KW - Adolescent *Cardiac Surgical Procedures Child Databases, Factual Female *Health Status Heart Defects, Congenital/*surgery Humans Male *Quality of Life Survivors Therapeutics United Kingdom United States *Aristotle Basic Complexity *Risk Adjustment in Congenital Heart Surgery *congenital heart disease surgery LA - eng N1 - 2047-9980 O'Connor, Amy M Wray, Jo Tomlinson, Ryan S Cassedy, Amy Jacobs, Jeffrey P Jenkins, Kathy J Brown, Kate L Franklin, Rodney C G Mahony, Lynn Mussatto, Kathleen Newburger, Jane W Wernovsky, Gil Ittenbach, Richard F Drotar, Dennis Marino, Bradley S Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't J Am Heart Assoc. 2016 Jul 22;5(7):e001234. doi: 10.1161/JAHA.114.001234. PY - 2016 SN - 2047-9980 ST - Impact of Surgical Complexity on Health-Related Quality of Life in Congenital Heart Disease Surgical Survivors T2 - J Am Heart Assoc TI - Impact of Surgical Complexity on Health-Related Quality of Life in Congenital Heart Disease Surgical Survivors VL - 5 ID - 136 ER - TY - JOUR AB - Background: Surgical complexity and related morbidities may affect long-term patient quality of life (QOL). Aristotle Basic Complexity (ABC) score and Risk Adjustment in Congenital Heart Surgery (RACHS-1) category stratify the complexity of pediatric cardiac operations. The purpose of this study was to examine the relationship between surgical complexity and QOL and to investigate other demographic and clinical variables that might explain variation in QOL in pediatric cardiac surgical survivors.Methods and Results: Pediatric Cardiac Quality of Life (PCQLI) study participants who had undergone cardiac surgery were included. The PCQLI database provided sample characteristics and QOL scores. Surgical complexity was defined by the highest ABC raw score or RACHS-1 category. Relationships among surgical complexity and demographic, clinical, and QOL variables were assessed using ordinary least squares regression. A total of 1416 patient-parent pairs were included. Although higher ABC scores and RACHS-1 categories were associated with lower QOL scores (P<0.005), correlation with QOL scores was poor to fair (r=-0.10 to -0.29) for all groups. Ordinary least squares regression showed weak association with R(2)=0.06 to R(2)=0.28. After accounting for single-ventricle anatomy, number of doctor visits, and time since last hospitalization, surgical complexity scores added no additional explanation to the variance in QOL scores.Conclusions: ABC scores and RACHS-1 categories are useful tools for morbidity and mortality predictions prior to cardiac surgery and quality of care initiatives but are minimally helpful in predicting a child's or adolescent's long-term QOL scores. Further studies are warranted to determine other predictors of QOL variation. AD - Division of Cardiology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati OH Cardiorespiratory Department, Great Ormond Street Hospital for Children NHS Foundation Trust, London UK Department of Pediatric Cardiology, Royal Brompton and Harefield NHS Foundation Trust, London UK Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati OH Division of Thoracic and Cardiovascular Surgery, The Congenital Heart Institute of Florida, All Children's Hospital and Children's Hospital of Tampa, University of South Florida College of Medicine, Cardiac Surgical Associates, St. Petersburg and Tampa FL Department of Cardiology, Children's Hospital Boston, Harvard Medical School, Boston MA Department of Pediatrics, Harvard Medical School, Boston MA Division of Cardiology, Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas TX Division of Cardiology, Department of Pediatrics, The Children's Hospital of Wisconsin, Milwaukee WI Division of Cardiology, Department of Pediatrics, The Children's Hospital of Philadelphia, PA Division of Critical Care Medicine, Department of Anesthesiology and Critical Care, The Children's Hospital of Philadelphia, PA Division of Behavioral Medicine and Clinical Psychology, Cincinnati Children's Hospital Medical Center, Cincinnati OH Division of Critical Care Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati OH AN - 117155989. Language: English. Entry Date: 20180108. Revision Date: 20190515. Publication Type: journal article AU - O'Connor, Amy M. AU - Wray, Jo AU - Tomlinson, Ryan S. AU - Cassedy, Amy AU - Jacobs, Jeffrey P. AU - Jenkins, Kathy J. AU - Brown, Kate L. AU - Franklin, Rodney C. G. AU - Mahony, Lynn AU - Mussatto, Kathleen AU - Newburger, Jane W. AU - Wernovsky, Gil AU - Ittenbach, Richard F. AU - Drotar, Dennis AU - Marino, Bradley S. DB - ccm DO - 10.1161/JAHA.114.001234 DP - EBSCOhost IS - 7 KW - Heart Surgery Quality of Life Health Status Heart Defects, Congenital -- Surgery Male Therapeutics Child Survivors Female Resource Databases Adolescence United States Human N1 - research; tables/charts. Journal Subset: Biomedical; Europe; UK & Ireland. NLM UID: 101580524. PMID: NLM27451455. PY - 2016 SN - 2047-9980 SP - 1-N.PAG ST - Impact of Surgical Complexity on Health-Related Quality of Life in Congenital Heart Disease Surgical Survivors T2 - Journal of the American Heart Association TI - Impact of Surgical Complexity on Health-Related Quality of Life in Congenital Heart Disease Surgical Survivors UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=117155989&site=ehost-live&scope=site VL - 5 ID - 1478 ER - TY - JOUR AB - Objectives: There is no consensus or theoretical explanation regarding the optimal location for the fenestration during the Fontan operation. We investigated the impact of the location of the fenestration on Fontan haemodynamics using a three-dimensional Fontan model in various physiological conditions.Methods: A three-dimensional Fontan model was constructed on the basis of CT images, and a 4-mm-diameter fenestration was located between the extracardiac Fontan conduit and the right atrium at three positions: superior, middle, and inferior part of the conduit. Haemodynamics in the Fontan route were analysed using a three-dimensional computational fluid dynamic model in realistic physiological conditions, which were predicted using a lumped parameter model of the cardiovascular system. The respiratory effect of the caval flow was taken into account. The flow rate through the fenestration, the effect of lowering the central venous pressure, and wall shear stress in the Fontan circuit were evaluated under central venous pressures of 10, 15, and 20 mmHg. The pulse power index and pulsatile energy loss index were calculated as energy loss indices.Results: Under all central venous pressures, the middle-part fenestration demonstrated the most significant effect on enhancing the flow rate through the fenestration while lowering the central venous pressure. The middle-part fenestration produced the highest time-averaged wall shear stress, pressure pulse index, and pulsatile energy loss index.Conclusions: Despite slightly elevated energy loss, the middle-part fenestration most significantly increased cardiac output and lowered central venous pressure under respiration in the Fontan circulation. AD - Department of Cardiovascular Surgery, Kitasato University School of Medicine, Sagamihara, Japan Department of Cardiac Surgery, The Royal Children’s Hospital, Melbourne, Australia Department of Paediatrics, University of Melbourne, Melbourne, Australia Graduate School of Engineering, Chiba University, Chiba, Japan Shanghai Jiao Tong University and Chiba University International Cooperative Research Centre (SJTU-CUICRC), Shanghai Jiao Tong University, Shanghai, China Department of Cell Biology, New York University Medical Center, New York, New York, U.S.A AN - 124503263. Language: English. Entry Date: 20180520. Revision Date: 20190515. Publication Type: journal article AU - Miyaji, Kagami AU - Sughimoto, Koichi AU - Brizard, Christian P. AU - Asakura, Yuta AU - Fujiwara, Takashi AU - Liu, Hao AU - Liang, Fuyou DB - ccm DO - 10.1017/S1047951117000099 DP - EBSCOhost IS - 7 KW - Heart Defects, Congenital Cardiopulmonary Bypass -- Methods Heart Defects, Congenital -- Surgery Heart Defects, Congenital -- Physiopathology Cardiac Output Tomography, X-Ray Computed Child Imaging, Three-Dimensional Models, Biological Human N1 - pictorial; research; tables/charts. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. NLM UID: 9200019. PMID: NLM28376950. PY - 2017 SN - 1047-9511 SP - 1289-1294 ST - Impact of the location of the fenestration on Fontan circulation haemodynamics: a three-dimensional, computational model study T2 - Cardiology in the Young TI - Impact of the location of the fenestration on Fontan circulation haemodynamics: a three-dimensional, computational model study UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=124503263&site=ehost-live&scope=site VL - 27 ID - 1482 ER - TY - JOUR AB - OBJECTIVES: There is no consensus or theoretical explanation regarding the optimal location for the fenestration during the Fontan operation. We investigated the impact of the location of the fenestration on Fontan haemodynamics using a three-dimensional Fontan model in various physiological conditions. METHODS: A three-dimensional Fontan model was constructed on the basis of CT images, and a 4-mm-diameter fenestration was located between the extracardiac Fontan conduit and the right atrium at three positions: superior, middle, and inferior part of the conduit. Haemodynamics in the Fontan route were analysed using a three-dimensional computational fluid dynamic model in realistic physiological conditions, which were predicted using a lumped parameter model of the cardiovascular system. The respiratory effect of the caval flow was taken into account. The flow rate through the fenestration, the effect of lowering the central venous pressure, and wall shear stress in the Fontan circuit were evaluated under central venous pressures of 10, 15, and 20 mmHg. The pulse power index and pulsatile energy loss index were calculated as energy loss indices. RESULTS: Under all central venous pressures, the middle-part fenestration demonstrated the most significant effect on enhancing the flow rate through the fenestration while lowering the central venous pressure. The middle-part fenestration produced the highest time-averaged wall shear stress, pressure pulse index, and pulsatile energy loss index. CONCLUSIONS: Despite slightly elevated energy loss, the middle-part fenestration most significantly increased cardiac output and lowered central venous pressure under respiration in the Fontan circulation. AD - 1Department of Cardiovascular Surgery,Kitasato University School of Medicine,Sagamihara,Japan. 3Graduate School of Engineering,Chiba University,Chiba,Japan. 2Department of Cardiac Surgery,The Royal Children's Hospital,Melbourne,Australia. 5School of Naval Architecture, Ocean and Civil Engineering,Shanghai Jiao Tong University,Shanghai,China. AN - 28376950 AU - Sughimoto, K. AU - Asakura, Y. AU - Brizard, C. P. AU - Liang, F. AU - Fujiwara, T. AU - Miyaji, K. AU - Liu, H. DA - Sep DB - PubMed DO - 10.1017/s1047951117000099 DP - NLM ET - 2017/04/06 IS - 7 KW - Cardiac Output Child Fontan Procedure/*methods Heart Defects, Congenital/*diagnostic imaging/*physiopathology/*surgery Humans Imaging, Three-Dimensional Models, Cardiovascular Tomography, X-Ray Computed Fontan computer fluid dynamics fenestration haemodynamics LA - eng N1 - 1467-1107 Sughimoto, Koichi Asakura, Yuta Brizard, Christian P Liang, Fuyou Fujiwara, Takashi Miyaji, Kagami Liu, Hao Journal Article England Cardiol Young. 2017 Sep;27(7):1289-1294. doi: 10.1017/S1047951117000099. Epub 2017 Apr 5. PY - 2017 SN - 1047-9511 SP - 1289-1294 ST - Impact of the location of the fenestration on Fontan circulation haemodynamics: a three-dimensional, computational model study T2 - Cardiol Young TI - Impact of the location of the fenestration on Fontan circulation haemodynamics: a three-dimensional, computational model study VL - 27 ID - 127 ER - TY - JOUR AB - BACKGROUND: Right ventricular (RV) morphology has been associated with adverse clinical outcomes in Fontan patients. The impact of RV versus left ventricular morphology on ventricular stress and strain in single ventricles is not well known. METHODS AND RESULTS: Cardiac magnetic resonance examinations in 193 patients with the Fontan circulation were retrospectively analyzed. Ventricular mass, volume, global circumferential and longitudinal strain, and global average end-systolic fiber stress were calculated using previously published methods. Compared with left ventricular morphology, RV morphology (48%) was associated with higher ventricular end-diastolic volume (110 mL/BSA(1.3) versus 84 mL/BSA(1.3), P<0.001), lower mass-to-volume ratio (0.46 versus 0.57, P<0.001), higher global average end-systolic fiber stress (23 kPa versus 20 kPa, P=0.002), worse global circumferential strain (-21% versus -24%, P<0.001), and higher prevalence of greater than or equal to moderate atrioventricular valve regurgitation (25% versus 6%, P<0.001). Ejection fraction and global longitudinal strain were similar between the groups. Death or listing for heart transplantation occurred in 24 (12%) with a median follow-up of 6.2 years. On univariate analysis, RV morphology, ventricular dilatation, and worse global circumferential strain were associated with this composite outcome. CONCLUSIONS: In comparison to Fontan patients with a dominant left ventricle, those with a dominant RV have higher fiber stress, a higher rate of ventricular dilatation, lower circumferential fiber shortening, and similar longitudinal shortening. RV morphology, ventricular dilation, and worse circumferential strain are associated with death or heart transplantation. The difference in myofiber architecture may contribute to suboptimal adaptation of the RV as a systemic ventricle. AD - Department of Cardiology, Boston Children's Hospital, MA. Department of Pediatrics, Harvard Medical School, Boston, MA. sunil.ghelani@cardio.chboston.org. Department of Cardiology, Boston Children's Hospital, MA. Department of Pediatrics, Harvard Medical School, Boston, MA. AN - 29970379 AU - Ghelani, S. J. AU - Colan, S. D. AU - Azcue, N. AU - Keenan, E. M. AU - Harrild, D. M. AU - Powell, A. J. AU - Geva, T. AU - Rathod, R. H. DA - Jul DB - PubMed DO - 10.1161/circimaging.117.006738 DP - NLM ET - 2018/07/05 IS - 7 KW - Adolescent Cardiac Catheterization Child Exercise Test Female *Fontan Procedure/adverse effects/mortality Heart Defects, Congenital/diagnostic imaging/mortality/physiopathology/*surgery Heart Transplantation Heart Ventricles/diagnostic imaging/physiopathology/*surgery Humans Magnetic Resonance Imaging Male *Myocardial Contraction Retrospective Studies Risk Factors Time Factors Treatment Outcome *Ventricular Function, Left *Ventricular Function, Right *Ventricular Remodeling Young Adult *blood pressure *dilatation *heart transplantation *prevalence LA - eng N1 - 1942-0080 Ghelani, Sunil J Colan, Steven D Azcue, Nina Keenan, Ellen M Harrild, David M Powell, Andrew J Geva, Tal Rathod, Rahul H Comparative Study Journal Article Research Support, Non-U.S. Gov't United States Circ Cardiovasc Imaging. 2018 Jul;11(7):e006738. doi: 10.1161/CIRCIMAGING.117.006738. PY - 2018 SN - 1941-9651 SP - e006738 ST - Impact of Ventricular Morphology on Fiber Stress and Strain in Fontan Patients T2 - Circ Cardiovasc Imaging TI - Impact of Ventricular Morphology on Fiber Stress and Strain in Fontan Patients VL - 11 ID - 53 ER - TY - JOUR AB - The IMproving Pediatric and Adult Congenital Treatments (IMPACT) Registry and the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) are two efforts initiated to improve outcomes in the congenital heart disease field. The IMPACT Registry is focused on evaluating the use, risks, adverse events (AEs), and outcomes associated with diagnostic and common interventional catheterization procedures in all children and adults with congenital heart disease. Utilizing a modular approach, the common procedures include diagnostic cardiac catheterization, atrial septal defect device closure, patent ductus arteriosus device closure, pulmonary valvuloplasty, aortic valvuloplasty, balloon and stent angioplasty of coarctation of the aorta, pulmonary artery balloon stent angioplasty, transcatheter pulmonary valve replacement, and electrophysiology procedures including radiofrequency ablation. To date, important observations on the common procedures have been made and a risk stratification methodology has been created to allow comparisons between centers in AEs and quality improvement activity. The registry is open to international participation. The NPC-QIC was developed to reduce mortality and improve the quality of life of infants with Hypoplastic Left Heart Syndrome (HLHS) during the interstage period between discharge from the Norwood operation and admission for the bidirectional Glenn procedure. Mortality in the interstage has been reduced by 44%. The IMPACT Registry and the NPC-QIC have demonstrated value to the congenital heart disease community. The IMPACT Registry, however, has not yet demonstrated an impact on patient outcomes. The NPC-QIC, which combines both a registry with a learning collaborative with specific aims, key drivers, and change strategies, has made more significant gains with reductions in variation, growth failures, and mortality. AD - 1 Division of Cardiology, Children's National Heart Institute and the George Washington University School of Medicine, Washington, DC, USA. 2 Heart Institute at Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA. 3 Division of Pediatric Cardiology, Children's Healthcare of Atlanta-Emory University, Atlanta, GA, USA. AN - 30799721 AU - Martin, G. R. AU - Anderson, J. B. AU - Vincent, R. N. DA - Jan DB - PubMed DO - 10.1177/2150135118815059 DP - NLM ET - 2019/02/26 IS - 1 KW - Cardiology/*standards Child Heart Defects, Congenital/*surgery Humans Norwood Procedures/*standards Quality Improvement/*organization & administration *Registries *Societies, Medical *cardiac catheterization/intervention *congenital heart disease *databases *outcomes LA - eng N1 - 2150-136x Martin, Gerard R Anderson, Jeffrey B Vincent, Robert N Journal Article Review United States World J Pediatr Congenit Heart Surg. 2019 Jan;10(1):72-80. doi: 10.1177/2150135118815059. PY - 2019 SN - 2150-1351 SP - 72-80 ST - IMPACT Registry and National Pediatric Cardiology Quality Improvement Collaborative: Contributions to Quality in Congenital Heart Disease T2 - World J Pediatr Congenit Heart Surg TI - IMPACT Registry and National Pediatric Cardiology Quality Improvement Collaborative: Contributions to Quality in Congenital Heart Disease VL - 10 ID - 39 ER - TY - JOUR AB - Thymus, as a primary site of appropriate adaptive immunity formation, is an essential organ in face of a self-tolerance as well as a potential menace from impairment of body integrity. Due to vital selection processes during differentiation and maturation of T lymphocytes, control over cell survival and programmed cell death must be orchestrated in detail. Indeed, thymus is highly sensitive to wide spectrum of stressors that initiate acute structural changes. Hypoxia, one of the most common complications in congenital heart defects (CHDs) patients, provokes stress-induced thymus involution. Disrupted embryolonic development of thymus in association with congenital heart defects, may negatively affect physiological immune mechanisms. We propose that detailed analysis of thymic morphology could critically contribute to unveil the pathophysiology of diseases associated with disrupted adaptive immunity in children with heterogeneous congenital heart diseases. AD - V. Mestanova, Department of Histology and Embryology, Jessenius Faculty of Medicine in Martin, Comenius University in Bratislava, Malá Hora 4, Martin, Slovakia AU - Mestanova, V. AU - Varga, I. AU - Adamkov, M. DB - Embase Medline DO - 10.1016/j.mehy.2020.109599 KW - cytokeratin AE1 cytokeratin AE3 Ki 67 antigen protein bcl 2 protein p53 survivin adaptive immunity aorta anomaly apoptosis article CD4+ T lymphocyte CD8+ T lymphocyte cell maturation cell survival congenital aortic atresia disease severity epithelium cell Fallot tetralogy G2 phase cell cycle checkpoint great vessels transposition heart right ventricle double outlet heart ventricle septum defect human hypoplastic left heart syndrome hypoxia immunohistochemistry macrophage medullary thymic epithelial cell pathophysiology protein expression stress thymocyte thymus ventricular septal defect type 1 ventricular septal defect type 4 LA - English M3 - Article N1 - L2004866873 2020-02-10 2020-02-18 PY - 2020 SN - 1532-2777 0306-9877 ST - Impaired histomorphology might provoke cell cycle regulators alteration in thymus of children with various congenital heart defects T2 - Medical Hypotheses TI - Impaired histomorphology might provoke cell cycle regulators alteration in thymus of children with various congenital heart defects UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2004866873 http://dx.doi.org/10.1016/j.mehy.2020.109599 VL - 138 ID - 551 ER - TY - JOUR AB - BACKGROUND: Impaired pulmonary function is an independent predictor of mortality in adult congenital heart disease (CHD), but has been scarcely studied in the paediatric CHD population. AIMS: To compare the pulmonary function of children with CHD to healthy controls, and evaluate its association with clinical outcomes, exercise capacity, and quality of life. METHODS: Cross-sectional multicentre study among 834 children (555 CHD and 279 control subjects) who underwent a complete spirometry and a cardiopulmonary exercise test (CPET). The 5th centile (Z-score = -1.64) was used to define the lower limit of normal. The association of clinical and CPET variables with spirometry was studied using a multivariate analysis. Children and their parents filled in the Kidscreen health-related quality of life questionnaire. RESULTS: Forced vital capacity (FVC) and forced expiratory volume in 1 s (FEV1) Z-scores values were lower in children with CHD than controls (-0.4 ± 1.5 vs. 0.4 ± 1.3, P < 0.001 and -0.5 ± 1.4 vs. 0.4 ± 1.2, P < 0.001, respectively), without any obstructive airway disorder. Restrictive pattern was more frequent in CHD patients than in controls (20% vs. 4%, P < 0.0001). FVC Z-scores were predominantly impaired in complex CHD, such as heterotaxy (-1.1 ± 0.6), single ventricle (-1.0 ± 0.2), and complex anomalies of the ventricular outflow tracts (-0.9 ± 0.1). In multivariate analysis, FVC was associated with age, body mass index, peak oxygen uptake, genetic anomalies, the number of cardiac surgery and cardiac catheter procedures. FVC and FEV1 correlated with self and proxy-related quality of life scores. CONCLUSION: These results suggest that pulmonary function should be monitored early in life, from childhood, in the CHD population. TRIAL REGISTRATION NUMBER: NCT01202916, post-results. AD - Paediatric and Congenital Cardiology Department, M3C Regional Reference Centre, Montpellier University Hospital, Montpellier, France; Center for Studies and Research on Health Services and Quality of Life, Aix-Marseille University, Marseille, France. Paediatric and Congenital Cardiology Department, M3C Regional Reference Centre, Montpellier University Hospital, Montpellier, France. Epidemiology Department, Montpellier University Hospital, Clinical Investigation Centre, INSERM-CIC 1411, University of Montpellier, Montpellier, France. PhyMedExp, University of Montpellier, INSERM, CNRS, France. Paediatric and Congenital Cardiology Department, M3C Regional Reference Centre, Montpellier University Hospital, Montpellier, France; Paediatric Cardiology and Rehabilitation Unit, St-Pierre Institute, Palavas-Les-Flots, France. Paediatric and Congenital Cardiology Department, St-Luc University Hospital, Brussels, Belgium. Center for Studies and Research on Health Services and Quality of Life, Aix-Marseille University, Marseille, France. Paediatric and Congenital Cardiology Department, M3C Regional Reference Centre, Montpellier University Hospital, Montpellier, France; Center for Studies and Research on Health Services and Quality of Life, Aix-Marseille University, Marseille, France; PhyMedExp, University of Montpellier, INSERM, CNRS, France. Electronic address: p-amedro@chu-montpellier.fr. AN - 30857849 AU - Abassi, H. AU - Gavotto, A. AU - Picot, M. C. AU - Bertet, H. AU - Matecki, S. AU - Guillaumont, S. AU - Moniotte, S. AU - Auquier, P. AU - Moreau, J. AU - Amedro, P. DA - Jun 15 DB - PubMed DO - 10.1016/j.ijcard.2019.02.069 DP - NLM ET - 2019/03/13 KW - Adolescent Child Child, Preschool Cross-Sectional Studies Exercise Test/*methods Exercise Tolerance/*physiology Female Forced Expiratory Volume/*physiology Heart Defects, Congenital/diagnosis/*physiopathology/psychology Humans Lung/*physiopathology Male Oxygen Consumption *Quality of Life Retrospective Studies Vital Capacity/*physiology *Children *Congenital heart disease *Lung *Pulmonary function *Spirometry LA - eng N1 - 1874-1754 Abassi, Hamouda Gavotto, Arthur Picot, Marie Christine Bertet, Helena Matecki, Stefan Guillaumont, Sophie Moniotte, Stephane Auquier, Pascal Moreau, Johan Amedro, Pascal Journal Article Multicenter Study Netherlands Int J Cardiol. 2019 Jun 15;285:86-92. doi: 10.1016/j.ijcard.2019.02.069. Epub 2019 Mar 1. PY - 2019 SN - 0167-5273 SP - 86-92 ST - Impaired pulmonary function and its association with clinical outcomes, exercise capacity and quality of life in children with congenital heart disease T2 - Int J Cardiol TI - Impaired pulmonary function and its association with clinical outcomes, exercise capacity and quality of life in children with congenital heart disease VL - 285 ID - 28 ER - TY - JOUR AB - Aims: To assess the feasibility of interatrial stenting for left atrial decompression in infants with hypoplastic left heart syndrome treated by a "hybrid-approach", with bilateral surgical banding of the pulmonary arteries and percutaneous stenting of the arterial duct. Patients and methods: We stented the atrial septum in 5 infants aged from 21 to 77 days, making the intervention as an elective procedure in 4, but as a rescue procedure in the fifth patient, who had a restrictive foramen. The stents, comprising 2 Jo-stents of 17 millimetres hand-crimped on a balloon catheter with dimensions of 10 by 20 millimetres, and 3 premounted Genesis stents with dimensions of 10 by 19 millimetres, were placed using a 6 French long or short sheath by femoral venous access. The stents were expanded under fluoroscopic guidance to create a slightly diabolo-shaped form that fitted the septum. Results: The percutaneous interventions were successfully performed in all cases, producing significant improvement in clinical condition after placement. The saturations of oxygen increased from an average of 64% plus or minus 18% to 88% plus or minus 7%, (p < 0.05). During a mean follow up of 2.5 months, without any anticoagulant therapy, there were no complications related to the stenting. Surgical removal of the stents was uneventful during reconstruction of the aortic arch and creation of a bidirectional cavopulmonary connection in 4 patients, and during cardiac transplantation in one. Conclusion: In the context of the hybrid approach, definitive decompression of the left atrium can be achieved by stenting the atrial septum in infants with hypoplastic left heart syndrome. Placement of the stents is safe and effective, with insertion in the form of a diabolo reducing the risk of dislocation, as well as embolisation of the stent. © 2007 Cambridge University Press. AD - D. Schranz, Paediatric Heart Centre, Justus-Liebig University, Feulgenstraße 12, 35385 Giessen, Germany AU - Rupp, S. AU - Michel-Behnke, I. AU - Valeske, K. AU - Akintürk, H. AU - Schranz, D. DB - Embase Medline DO - 10.1017/S104795110700090X IS - 5 KW - heparin oxygen aortic arch artery catheterization artery ligation artery reconstruction article balloon catheter conscious sedation coronary stent female fluoroscopy interatrial septum heart left atrium pressure heart transplantation human hypoplastic left heart syndrome infant intracardiac thrombosis male newborn Norwood procedure operation duration oxygen saturation percutaneous coronary intervention pulmonary artery risk reduction surgical technique Genesis Jo-stent LA - English M3 - Article N1 - L350065551 2007-12-05 PY - 2007 SN - 1047-9511 1467-1107 SP - 535-540 ST - Implantation of stents to ensure an adequate interatrial communication in patients with hypoplastic left heart syndrome T2 - Cardiology in the Young TI - Implantation of stents to ensure an adequate interatrial communication in patients with hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L350065551 http://dx.doi.org/10.1017/S104795110700090X VL - 17 ID - 1226 ER - TY - JOUR AB - Objective: To evaluate whether a nationwide prenatal anomaly screening programme improves detection rates of univentricular heart (UVH) and transposition of great arteries (TGA), and whether maternal risk factors for severe fetal heart disease affect prenatal detection. Design: Population-based cohort study. Setting: Nationwide data from Finnish registries 2004–14. Population: A total of 642 456 parturients and 3449 terminated pregnancies due to severe fetal anomaly. Methods: Prenatal detection rates were calculated in three time periods (prescreening, transition and screening phase). The effect of maternal risk factors (obesity, in vitro fertilisation, pregestational diabetes and smoking) was evaluated. Main outcome measures: Change in detection rates and impact of maternal risk factors on screening programme efficacy. Results: In total, 483 cases of UVH and 184 of TGA were detected. The prenatal detection rate of UVH increased from 50.4% to 82.8% and of TGA from 12.3% to 41.0% (P < 0.0001). Maternal risk factors did not affect prenatal detection rate, but detection rate differed substantially by region. Conclusions: A nationwide screening programme improved overall UVH and TGA detection rates, but regional differences were observed. Obesity or other maternal risk factors did not affect the screening programme efficacy. The establishment of structured guidelines and recommendations is essential when implementing the screening programme. In addition, a prospective screening register is highly recommended to ensure high quality of screening. Tweetable abstract: Implementation of a nationwide prenatal anomaly screening improved detection rates of UVH and TGA. AD - J. Hautala, Department of Obstetrics and Gynaecology, Women's Hospital, Helsinki University Hospital and University of Helsinki, Helsinki, Finland AU - Hautala, J. AU - Gissler, M. AU - Ritvanen, A. AU - Tekay, A. AU - Pitkänen-Argillander, O. AU - Stefanovic, V. AU - Sarkola, T. AU - Helle, E. AU - Pihkala, J. AU - Pätilä, T. AU - Mattila, I. P. AU - Jokinen, E. AU - Räsänen, J. AU - Ojala, T. DB - Embase Medline DO - 10.1111/1471-0528.15589 IS - 7 KW - adult article cohort analysis congenital heart malformation controlled study female fetus outcome fetus risk great vessels transposition heart single ventricle high risk infant human in vitro fertilization major clinical study maternal obesity maternal smoking pregnancy diabetes mellitus pregnancy termination prenatal diagnosis prenatal screening priority journal program evaluation risk assessment risk factor LA - English M3 - Article N1 - L626132790 2019-02-01 2019-06-14 PY - 2019 SN - 1471-0528 1470-0328 SP - 864-873 ST - The implementation of a nationwide anomaly screening programme improves prenatal detection of major cardiac defects: an 11-year national population-based cohort study T2 - BJOG: An International Journal of Obstetrics and Gynaecology TI - The implementation of a nationwide anomaly screening programme improves prenatal detection of major cardiac defects: an 11-year national population-based cohort study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L626132790 http://dx.doi.org/10.1111/1471-0528.15589 VL - 126 ID - 613 ER - TY - JOUR AB - Objective: To investigate etiology, outcome and complications related to neonatal peritoneal dialysis (PD). Methods: Neonates treated with PD in our neonatal intensive care unit during 20072010 were analyzed retrospectively. Results: Among 4036 hospitalized neonates; 20 neonates (0.5%) who underwent 21 cycles of PD [7 preterm, 13 term; 13 female, 7 male] were included. The mean birth weight was 2930.2 ± 720.6 g (11204570), mean gestational age was 37.5 ± 3.5 weeks (2741). The etiologic disorders included inborn errors of metabolism (propionic acidemia, methylmalonic acidemia, citrullinemia, glutaric aciduria type2, maple syrup urine disease, 10), or acute renal failure secondary to perinatal asphyxia (4), sepsis (2), prematurity (2), hypoplastic left heart syndrome (1), kernicterus (1). The complications included peritonitis (2), early leakage (4), hemorrhage (1), catheter removal (3) and occlusion (2). The mortality rate was 50%. The gestational ages and birth weights of surviving neonates were higher (p < 0.05). Among surviving neonates, chronic renal failure (1), severe (4) and moderate neuromotor impairment (2) developed within 443 months. Conclusion: PD, although invasive, is an effective therapy in neonates. The complexity and invasiveness of the procedure is probably responsible for high rate of complications and mortality. If appropriate catheter selection and technique in the placement should be done, PD might improve outcome. © 2012 Informa UK, Ltd. AD - S. Unal, Department of Neonatology, Ankara Children's Health Hematology, Oncology Research Hospital, Ankara, Turkey AU - Unal, S. AU - Bilgin, L. AU - Gunduz, M. AU - Uncu, N. AU - Azili, M. N. AU - Tiryaki, T. DB - Embase Medline DO - 10.3109/14767058.2012.665105 IS - 10 KW - acute kidney failure article birth weight bleeding catheter occlusion catheter removal child hospitalization chronic kidney failure citrullinemia clinical article clinical practice disease severity female gestational age human hypoplastic left heart syndrome inborn error of metabolism kernicterus male maple syrup urine disease methylmalonic acidemia motor dysfunction multiple acyl CoA dehydrogenase deficiency newborn newborn care newborn intensive care newborn morbidity newborn mortality outcome assessment perinatal asphyxia peritoneal dialysis peritonitis prematurity priority journal propionic acidemia retrospective study sepsis LA - English M3 - Article N1 - L365664345 2012-09-27 2012-10-01 PY - 2012 SN - 1476-7058 1476-4954 SP - 2111-2114 ST - The implementation of neonatal peritoneal dialysis in a clinical setting T2 - Journal of Maternal-Fetal and Neonatal Medicine TI - The implementation of neonatal peritoneal dialysis in a clinical setting UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L365664345 http://dx.doi.org/10.3109/14767058.2012.665105 VL - 25 ID - 1037 ER - TY - JOUR AB - Although different guidelines on adult congenital heart disease (ACHD) care advocate for lifetime cardiac follow-up, a critical appraisal of the guideline implementation is lacking. We investigated the implementation of the American College of Cardiology/American Heart Association 2008 guidelines for ACHD follow-up by investigating the type of health care professional, care setting, and frequency of outpatient visits in young adults with CHD. Furthermore, correlates for care in line with the recommendations or untraceability were investigated. A cross-sectional observational study was conducted, including 306 patients with CHD who had a documented outpatient visit at pediatric cardiology before age 18 years. In all, 210 patients (68.6%) were in cardiac follow-up; 20 (6.5%) withdrew from follow-up and 76 (24.9%) were untraceable. Overall, 198 patients were followed up in tertiary care, 1/4 (n = 52) of which were seen at a formalized ACHD care program and 3/4 (n = 146) remained at pediatric cardiology. Of those followed in formalized ACHD and pediatric cardiology care, the recommended frequency was implemented in 94.2% and 89%, respectively (p = 0.412). No predictors for the implementation of the guidelines were identified. Risk factors for becoming untraceable were none or lower number of heart surgeries, health insurance issues, and nonwhite ethnicity. In conclusion, a significant number of adults continue to be cared for by pediatric cardiologists, indicating that transfer to adult-oriented care was not standard practice. Frequency of follow-up for most patients was in line with the ACC/AHA 2008 guidelines. A considerable proportion of young adults were untraceable in the system, which makes them vulnerable for discontinuation of care. AD - P. Moons, Department of Public Health and Primary Care, KU Leuven, University of Leuven, Leuven, Belgium AU - Goossens, E. AU - Fernandes, S. M. AU - Landzberg, M. J. AU - Moons, P. DB - Embase Medline DO - 10.1016/j.amjcard.2015.04.041 IS - 3 KW - adult adult disease aortic coarctation aortic valve disease article cardiac patient cardiologist congenital heart disease cross-sectional study Ebstein anomaly endocardial cushion defect ethnicity Fallot tetralogy female follow up frequency great vessels transposition health care planning health insurance health program heart atrium septum defect heart catheterization heart single ventricle heart surgery heart ventricle septum defect human hypoplastic left heart syndrome major clinical study male medical record review medical society mitral valve disease observational study pediatric cardiology practice guideline priority journal pulmonary valve atresia pulmonary valve disease pulmonary vein malformation risk factor self report tertiary health care tricuspid valve atresia young adult LA - English M3 - Article N1 - L605039181 2015-07-06 2015-07-28 PY - 2015 SN - 1879-1913 0002-9149 SP - 452-457 ST - Implementation of the American College of Cardiology/American Heart Association 2008 Guidelines for the Management of Adults with Congenital Heart Disease T2 - American Journal of Cardiology TI - Implementation of the American College of Cardiology/American Heart Association 2008 Guidelines for the Management of Adults with Congenital Heart Disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L605039181 http://dx.doi.org/10.1016/j.amjcard.2015.04.041 VL - 116 ID - 888 ER - TY - JOUR AB - Objectives Parental understanding of their children's heart disease is inadequate, which may contribute to poor health outcomes. The purpose of this study was to determine what parental knowledge is important in the care of children with heart disease from the perspective of parents, nurses, and physicians. Methods Focus groups were formed with parents of children with single ventricle congenital heart disease (CHD), biventricular CHD, and heart transplantation, and with nurses and physicians who provide care for these children. A nominal group technique was used to identify and prioritise important parental knowledge items and themes. The voting data for each theme were reported by participant type - parent, nurse, and physician - and patient diagnosis - single ventricle CHD, biventricular CHD, and heart transplantation. Results The following three themes were identified as important by all groups: recognition of and response to clinical deterioration, medications, and prognosis and plan. Additional themes that were unique to specific groups included the following: medical team members and interactions (parents), tests and labs (parents), neurodevelopmental outcomes and interventions (physicians), lifelong disease requiring lifelong follow-up (physicians and nurses), and diagnosis, physiology, and interventions (single ventricle and biventricular CHD). Conclusions Parents, nurses, and physicians have both common and unique views regarding what parents should know to effectively care for their children with single ventricle CHD, biventricular CHD, or heart transplantation. Specific targeted parental education that incorporates these findings should be provided to each group. Further development of questionnaires regarding parental knowledge with appropriate content validity is warranted. AD - J. Daily, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH, United States AU - Daily, J. AU - Fitzgerald, M. AU - Downing, K. AU - King, E. AU - Del Rey, J. G. AU - Ittenbach, R. AU - Marino, B. DB - Embase DO - 10.1017/S1047951114002625 IS - 1 KW - adult article child child health care clinical article clinical assessment congenital heart disease demography ethnicity exercise female follow up health care planning heart transplantation human knowledge male nurse attitude outcome assessment parental attitude physician attitude prognosis resource allocation LA - English M3 - Article N1 - L601889101 2015-02-04 2016-01-11 PY - 2014 SN - 1467-1107 1047-9511 SP - 61-69 ST - Important knowledge for parents of children with heart disease: Parent, nurse, and physician views T2 - Cardiology in the Young TI - Important knowledge for parents of children with heart disease: Parent, nurse, and physician views UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L601889101 http://dx.doi.org/10.1017/S1047951114002625 VL - 26 ID - 901 ER - TY - JOUR AB - Objectives: Two innovative surgical approaches addressing systemic venous anomalies in single-ventricle patients are evaluated. Methods: Between 2003 and 2007, 7 patients underwent a unifocal bilateral bidirectional cavopulmonary anastomosis, and 5 patients underwent a hepatoazygos venous connection associated with a previous (n = 4) or concomitant (n = 1) Kawashima operation. Computational fluid dynamics simulations allowed investigation of 2 sets of comparative models: (1) bifocal versus unifocal bilateral bidirectional cavopulmonary anastomosis and (2) classic hepatic vein-pulmonary artery channel versus hepatoazygos direct anastomosis for Fontan completion after or combined with the Kawashima operation. Results: There was 1 hospital death in the unifocal bilateral bidirectional cavopulmonary anastomosis group. At a mean follow-up of 15.6 ± 7.40 months after a unifocal bilateral bidirectional cavopulmonary anastomosis and of 38.7 ± 13.2 months after direct hepatoazygos venous connection, respectively, all 11 survivors are in New York Heart Association class I with functional anastomoses. Computational assessment of bifocal bilateral bidirectional cavopulmonary anastomosis demonstrated weak perfusion between caval veins against symmetric and steady bilateral flow fields in the unifocal arrangement. In the classic post-Kawashima Fontan completion model, the hepatic venous flow to the pulmonary artery was held back by means of preponderant opposite flow, whereas in the direct hepatoazygos venous connection model, the hepatic venous flow merged smoothly into the azygos vein. Power-loss calculation showed no significant difference between bifocal and unifocal bilateral bidirectional cavopulmonary anastomosis topology, whereas the hepatoazygos connection clearly had better energy preservation than the classical connection. Conclusions: This limited clinical and computational fluid dynamics assessment suggests the efficacy of this new rationale to reduce the additional thrombotic risks produced by systemic venous anomalies in single-ventricle patients. © 2009 The American Association for Thoracic Surgery. AD - A. Amodeo, Cardiac Surgery Service, the Medical-Surgical Department of Paediatric Cardiology, Bambino Gesù Paediatric Hospital, Rome, Italy AU - Amodeo, A. AU - Grigioni, M. AU - Filippelli, S. AU - Gagliardi, M. G. AU - Del Gaudio, C. AU - Morbiducci, U. AU - D'Avenio, G. AU - Brancaccio, G. AU - Di Donato, R. M. DB - Embase Medline DO - 10.1016/j.jtcvs.2009.04.039 IS - 5 KW - article cava vein cavopulmonary connection child clinical article follow up Fontan procedure heart single ventricle human infant mathematical computing perfusion priority journal pulmonary artery risk reduction simulation surgical approach surgical technique thrombosis vein malformation LA - English M3 - Article N1 - L50575425 2010-01-13 PY - 2009 SN - 0022-5223 SP - 1154-1159 ST - Improved management of systemic venous anomalies in a single ventricle: New rationale T2 - Journal of Thoracic and Cardiovascular Surgery TI - Improved management of systemic venous anomalies in a single ventricle: New rationale UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50575425 http://dx.doi.org/10.1016/j.jtcvs.2009.04.039 VL - 138 ID - 1172 ER - TY - JOUR AB - OBJECTIVES: We performed a prospective longitudinal study of the neuroanatomic and developmental changes in infants with transposition of the great arteries (TGA) or single-ventricle (SV) physiology to identify variables in anatomic development of the brain associated with functional impairment. METHODS: Thirty-three infants with congenital heart defects, 23 with SV and 10 with TGA, were studied at around 1 year old (time 1) and 3 years old (time 2) by magnetic resonance imaging of the brain. Neurodevelomental assessment was performed at the same time. RESULTS: The whole and frontal lobe volumes were significantly reduced in both groups at time 1 compared with normal control subjects (P < .01). However, by time 2 whole and frontal brain volumes were normal in the TGA group but remained significantly smaller (P < .01) in the SV group. In agreement with these findings, the mental development index (MDI) was lower (P < .05) at time 1 in both groups but improved to normal levels at time 2 in the TGA group. In the SV group, both MDI and the psychomotor development index (PDI) were significantly decreased at both time 1 and time 2 (P < .01). These patients continued to experience hypoxia, and multivariate analysis revealed that functional oxygen saturation was significantly associated with PDI. Further, the PDI score correlated with whole and regional brain volumes (P < .05). CONCLUSIONS: Neuroanatomic and developmental outcomes improve progressively in infants with TGA, unlike those with SV physiology. Impaired cerebral circulation and hypoxia may have significant effects on brain growth and development in infants with critical congenital heart disease. AD - Department of Pediatrics, University of Toyama, Toyama, Japan. AN - 21963327 AU - Ibuki, K. AU - Watanabe, K. AU - Yoshimura, N. AU - Kakimoto, T. AU - Matsui, M. AU - Yoshida, T. AU - Origasa, H. AU - Ichida, F. DA - May DB - PubMed DO - 10.1016/j.jtcvs.2011.08.042 DP - NLM ET - 2011/10/04 IS - 5 KW - Age Factors Brain/*growth & development/pathology/physiopathology *Cardiac Surgical Procedures Case-Control Studies *Child Development Child, Preschool Developmental Disabilities/*etiology/pathology/physiopathology Female Heart Ventricles/abnormalities/physiopathology/*surgery Humans Hypoxia/*etiology/pathology/physiopathology Imaging, Three-Dimensional Infant Japan Longitudinal Studies Magnetic Resonance Imaging Male Neuropsychological Tests Organ Size Prospective Studies Psychomotor Performance Risk Assessment Risk Factors Transposition of Great Vessels/complications/pathology/physiopathology/*surgery Treatment Outcome LA - eng N1 - 1097-685x Ibuki, Keijiro Watanabe, Kazuhiro Yoshimura, Naoki Kakimoto, Tachiyo Matsui, Mie Yoshida, Taketoshi Origasa, Hideki Ichida, Fukiko Journal Article Research Support, Non-U.S. Gov't United States J Thorac Cardiovasc Surg. 2012 May;143(5):1077-85. doi: 10.1016/j.jtcvs.2011.08.042. Epub 2011 Oct 2. PY - 2012 SN - 0022-5223 SP - 1077-85 ST - The improvement of hypoxia correlates with neuroanatomic and developmental outcomes: comparison of midterm outcomes in infants with transposition of the great arteries or single-ventricle physiology T2 - J Thorac Cardiovasc Surg TI - The improvement of hypoxia correlates with neuroanatomic and developmental outcomes: comparison of midterm outcomes in infants with transposition of the great arteries or single-ventricle physiology VL - 143 ID - 142 ER - TY - JOUR AB - Objective: We hypothesize that neurodevelopmental surveillance of targeted patients with congenital heart disease during the admission for their cardiac surgery would improve neurodevelopmental assessment and outpatient follow-up rates. Design: All patients under 12 months of age who were operated on between October 2013 and October 2014 and were considered at risk for neurodevelopmental delay in accordance with the 2012 American Heart Association Scientific Statement were included. A protocol was implemented to increase surveillance of targeted patients during the hospitalization for their cardiac surgery. A historical control cohort was used from a 6-month period that preceded initiation of the program from July 2012 to December 2012. Univariate analysis assessed the effects of patient demographics, anatomy, postoperative course, and distance from clinic on inpatient screening and follow-up to evaluate areas for future improvement. Results: Neurodevelopmental surveillance in the post-protocol period increased from 21% to 82% (P<.001) as did compliance rates for outpatient follow-up from 38% to 52% (P<.001). Patients receiving consultation were younger (median 1.2 months range 0.3-3.1 vs. 4.0 range 1.2-5.5, P=.002), had a longer intensive care unit duration (median 8 days range 4-13 vs. 4 range 3-8, P=.044), and a longer total hospital duration (median 14 days range 8-25 vs. 8 range 6-16, P=.023). The presence of single ventricle anatomy was associated with a lower follow-up rate at 29% than those with biventricular hearts at 64% (P=.009). Distance from the clinic did not have an effect on follow-up (P=.39). Conclusion: The protocol described increased neurodevelopmental surveillance of high risk patients. Individuals that were younger and in the hospital longer were more likely to be successfully seen and comply with outpatient follow-up than those not receiving inpatient risk assessment. Patients with single ventricle anatomy may benefit from a modified follow-up schedule to improve compliance rates. Travel distance has no effect on likelihood of outpatient cardiac neurodevelopmental follow-up. AD - M. Michael, Newark Beth Israel Medical Center, 201 Lyons Ave, Suite L5, Newark, NJ, United States AU - Michael, M. AU - Scharf, R. AU - Letzkus, L. AU - Vergales, J. DB - Embase Medline DO - 10.1111/chd.12333 IS - 2 KW - age distribution article biventricular heart congenital heart disease congenital heart malformation consultation developmental disorder developmental screening disease surveillance female follow up health care utilization heart single ventricle human infant intensive care unit length of stay major clinical study male mental disease neurodevelopmental delay priority journal travel LA - English M3 - Article N1 - L608560273 2016-03-01 2016-04-26 PY - 2016 SN - 1747-0803 1747-079X SP - 183-188 ST - Improving Neurodevelopmental Surveillance and Follow-up in Infants with Congenital Heart Disease T2 - Congenital Heart Disease TI - Improving Neurodevelopmental Surveillance and Follow-up in Infants with Congenital Heart Disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L608560273 http://dx.doi.org/10.1111/chd.12333 VL - 11 ID - 817 ER - TY - JOUR AB - There are many reasons to consider using the Hybrid Stage 1 procedure as the initial palliation for hypoplastic left heart syndrome. It allows all options for treatment, including a delayed traditional approach, as well as a platform to evolve our overall approach to this challenging heart defect. © 2013 Elsevier Inc. AD - M. Galantowicz, The Heart Center, Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH 43205, United States AU - Galantowicz, M. DB - Embase Medline DO - 10.1053/j.pcsu.2013.01.005 IS - 1 KW - article borderline left heart structure cohort analysis cost evidence based practice extracorporeal oxygenation Fontan procedure heart catheterization heart surgery heart transplantation heart ventricle function hemodynamics hospitalization human hybrid stage 1 hypoplastic left heart syndrome intermethod comparison lung artery banding Norwood procedure outcome assessment overall survival palliative therapy pediatrics quality of life right ventricle to pulmonary artery conduit surgical mortality treatment indication LA - English M3 - Article N1 - L368659789 2013-04-12 2013-04-23 PY - 2013 SN - 1092-9126 1876-4665 SP - 62-64 ST - In favor of the hybrid stage 1 as the initial palliation for hypoplastic left heart syndrome T2 - Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual TI - In favor of the hybrid stage 1 as the initial palliation for hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L368659789 http://dx.doi.org/10.1053/j.pcsu.2013.01.005 VL - 16 ID - 1004 ER - TY - JOUR AU - Long, S. S. DB - Embase DO - 10.1016/j.jpeds.2008.07.023 IS - 3 KW - diphtheria pertussis tetanus vaccine minocycline article attention deficit disorder autoimmunity brain disease certification child health care cystic fibrosis developmental psychology Down syndrome esophagus atresia esophagus fistula human hypoplastic left heart syndrome necrotizing enterocolitis newborn screening Norovirus pediatrics prematurity priority journal smoking LA - English M3 - Article N1 - L352154714 2008-10-01 PY - 2008 SN - 0022-3476 SP - A1 ST - In-training examination and the general pediatric certification examination: Are there lessons? T2 - Journal of Pediatrics TI - In-training examination and the general pediatric certification examination: Are there lessons? UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L352154714 http://dx.doi.org/10.1016/j.jpeds.2008.07.023 VL - 153 ID - 1200 ER - TY - JOUR AB - OBJECTIVE: Diaphragmatic paralysis (DP) caused by phrenic nerve injury is potentially life-threatening in infants. Phrenic nerve injury due to thoracic surgery is the most common cause of DP in children. We retrospectively analyzed incidence, surgical details, management and follow-up of our patients with DP after cardiac surgery to develop an algorithm for the management and follow-up. METHODS: Retrospective analysis of 43 patients with DP after cardiac surgery performed between 1996 and 2000. RESULTS: Median age at cardiac surgery was 1 month (range 3 days to 9 years). Incidence of DP was 5.4%. A trend towards higher incidences of DP were observed after arterial switch operation (10.8%, P=0.18), Fontan procedure (17.6%, P=0.056) and Blalock-Taussig Shunt (12.8%, P=0.10). Median time from cardiac surgery to surgical plication was 21 days (range 7-210 days). Transthoracic diaphragmatic plication was performed in 29/43 patients, no plication was done in 14/43 patients. Patients in whom diaphragmatic plication was required were younger (median age 2 months, range 21 days to 53 months versus 17.5 months, range 4 days to 110 months; P<0.001). Indications for plication were failure to wean from ventilator (n=22), respiratory distress (n=4), cavopulmonary anastomosis (n=2), and failure to thrive (n=1). All these symptoms resolved after diaphragmatic plication, however, 8/29 patients with plication and 2/14 without plication died. Cause of death was not related to diaphragmatic plication in any patient. Position of plicated diaphragm was normal in 18/21 surviving patients 1 month after plication. In 2/12 surviving patients without plication hemidiaphragm showed a normal position 1 year after surgery. The rate of pulmonary infections was not significantly different during 12-60 months follow-up. CONCLUSIONS: DP is an occasional complication of cardiac surgery. High incidences of DP were seen after arterial switch operation, Fontan procedure and Blalock-Taussig shunt (BT). Respiratory insufficiency requires diaphragmatic plication in most infants with DP whereas older children may tolerate DP. Transthoracic diaphragmatic plication is an effective treatment of DP and achieves relief of respiratory insufficiency in most patients. Spontaneous recovery from postsurgical DP is rare. AD - Department of Neonatology and Pediatric Intensive Care, University Children's Hospital Zurich, Steinwiesstrasse 75, CH-8032 Zurich, Switzerland. AN - 15621471 AU - Joho-Arreola, A. L. AU - Bauersfeld, U. AU - Stauffer, U. G. AU - Baenziger, O. AU - Bernet, V. DA - Jan DB - PubMed DO - 10.1016/j.ejcts.2004.10.002 DP - NLM ET - 2004/12/29 IS - 1 KW - Algorithms Cardiac Surgical Procedures/*adverse effects/methods Child, Preschool Heart Diseases/*congenital/surgery Humans Infant Infant, Newborn Phrenic Nerve/*injuries Respiration, Artificial/methods Respiratory Paralysis/diagnosis/etiology/*surgery Retrospective Studies Thoracic Surgical Procedures/methods Time Factors Treatment Outcome LA - eng N1 - Joho-Arreola, Ana L Bauersfeld, Urs Stauffer, Urs G Baenziger, Oskar Bernet, Vera Journal Article Germany Eur J Cardiothorac Surg. 2005 Jan;27(1):53-7. doi: 10.1016/j.ejcts.2004.10.002. PY - 2005 SN - 1010-7940 (Print) 1010-7940 SP - 53-7 ST - Incidence and treatment of diaphragmatic paralysis after cardiac surgery in children T2 - Eur J Cardiothorac Surg TI - Incidence and treatment of diaphragmatic paralysis after cardiac surgery in children VL - 27 ID - 520 ER - TY - JOUR AB - The incidence of congenital heart disease (CHD) in the Western industrialized world has varied from a low value of about 3 to 5 per 1000 live births to about 12 per 1000 live births. Most of the lower incidence figures were obtained before there were sufficiently well trained pediatric cardiologists and before the success of cardiac surgery put a premium on early and correct diagnosis of CHD. The advent of echocardiography with Doppler color flow measurements has made it possible to diagnose lesions that are asymptomatic, minor, and even without murmurs. Given these differences, there does not appear to have been a significant increase in the incidence of CHD over the last 20-30 years. The incidence of CHD in underdeveloped countries is not known, but the distribution of different lesions is fairly similar to those in developed countries except perhaps for fewer with aortic stenosis and coarctation of the aorta. AD - J.I.E. Hoffman, Department of Pediatrics, Cardiovascular Research Institute, University of California, San Francisco, CA 94143, United States AU - Hoffman, J. I. E. DB - Embase Medline IS - 3 KW - aortic coarctation aortic valve stenosis aortopulmonary septal defect article child congenital heart disease diagnostic accuracy Down syndrome ethnic difference Fallot tetralogy great vessels transposition heart atrium septum defect heart right ventricle double outlet heart single ventricle heart ventricle septum defect human hypoplastic left heart syndrome incidence infant lung vein drainage anomaly major clinical study patent ductus arteriosus preschool child pulmonary valve stenosis trisomy 21 LA - English M3 - Article N1 - L25151803 1995-05-29 PY - 1995 SN - 0172-0643 SP - 103-113 ST - Incidence of congenital heart disease: I. Postnatal incidence T2 - Pediatric Cardiology TI - Incidence of congenital heart disease: I. Postnatal incidence UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L25151803 VL - 16 ID - 1368 ER - TY - JOUR AB - An increase in N-terminus pro-B-type natriuretic peptide (NT-proBNP) during exercise is related to myocardial ischemia, myocardial dysfunction, and inflammatory stress. Its value for patients with a univentricular heart after total cavopulmonary connection (TCPC) is unknown. For 66 patients with TCPC, a cardiopulmonary exercise test was performed. Venous NT-proBNP samples were drawn with the patient at rest and then 2-3 min after peak exercise. The median NT-proBNP during rest was 82 ng/l (range, 11-2,554 ng/l), with 22 patients above the upper reference limit. A higher NT-proBNP during rest was related to a worse peak oxygen uptake (peak [Formula: see text]), a higher ventilatory equivalent (VE/VCO(2) slope), and a need for diuretics administration. A small but significant increase in NT-proBNP during exercise at 6 ng/l (range, 0-314 ng/l) was related mainly to its resting value. The relative increase was solely related to a higher body mass and not to any of the investigated functional parameters. Usually, NT-proBNP during rest is not elevated in TCPC patients. If so, it is a valuable predictor of cardiac function. During exercise, only a minor increase in NT-proBNP occurs. Its extent is not related to any of the investigated functional parameters. Maybe the filling restriction from the lungs prevents atrial and ventricular overload as well as BNP secretion in TCPC patients. AD - Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Lazarettstrasse 36, 80636 Munich, Germany. a-hager@web.de AN - 22374379 AU - Hager, A. AU - Christov, F. AU - Hess, J. DA - Jun DB - PubMed DO - 10.1007/s00246-012-0210-2 DP - NLM ET - 2012/03/01 IS - 5 KW - Adolescent Adult Biomarkers/blood Child Exercise Test Female Heart Defects, Congenital/*blood/*physiopathology/surgery Humans Male Middle Aged Natriuretic Peptide, Brain/*blood Oximetry Peptide Fragments/*blood Predictive Value of Tests LA - eng N1 - 1432-1971 Hager, Alfred Christov, Florian Hess, John Journal Article Research Support, Non-U.S. Gov't United States Pediatr Cardiol. 2012 Jun;33(5):764-9. doi: 10.1007/s00246-012-0210-2. Epub 2012 Feb 29. PY - 2012 SN - 0172-0643 SP - 764-9 ST - Increase in N-terminus-pro-B-type natriuretic peptide during exercise of patients with univentricular heart after a total cavopulmonary connection T2 - Pediatr Cardiol TI - Increase in N-terminus-pro-B-type natriuretic peptide during exercise of patients with univentricular heart after a total cavopulmonary connection VL - 33 ID - 331 ER - TY - JOUR AB - Objective: We sought to examine the relationship between extracorporeal membrane oxygenation center case volume and survival in pediatric patients requiring extracorporeal membrane oxygenation support. Methods: Pediatric patients (≤20 years) undergoing extracorporeal membrane oxygenation cannulation were identified using the Healthcare Cost and Utilization Project Kids' Inpatient Database for 2000 to 2009. Annual hospital extracorporeal membrane oxygenation volume tertiles were <15 patients/year (low volume), 15 to 30 patients/year (medium volume), and >30 patients/year (high volume). Cases of extracorporeal membrane oxygenation were segregated by indication into cardiac and noncardiac groups. Cases of cardiac extracorporeal membrane oxygenation were mapped to Risk Adjustment for Congenital Heart Surgery categories to adjust for case complexity. Weighted multivariable logistic and linear regression models identified determinants of in-hospital mortality. Results: Overall, 3867 cases of extracorporeal membrane oxygenation were identified, yielding a national estimate of 6333 ± 495 cases. Extracorporeal membrane oxygenation was used with nearly equivalent prevalence across volume tertiles for all Risk Adjustment for Congenital Heart Surgery categories, suggesting that patient selection for extracorporeal membrane oxygenation was fairly uniform. A higher annual extracorporeal membrane oxygenation volume tertile was associated with reduced in-hospital mortality (P = .01) within nearly all Risk Adjustment for Congenital Heart Surgery categories. After adjustment for Risk Adjustment for Congenital Heart Surgery category and other patient variables, lower extracorporeal membrane oxygenation volume remained an important determinant of in-hospital death (odds ratio, 1.75; 95% confidence interval, 1.03-2.94; P = .03). Conclusions: Higher extracorporeal membrane oxygenation case volume is associated with improved hospital survival in pediatric cardiac extracorporeal membrane oxygenation patients. The results of this study may support the paradigm of regionalized centers of excellence for managing pediatric cardiac extracorporeal membrane oxygenation patients. Copyright © 2013 by The American Association for Thoracic Surgery. AD - D.M. McMullan, Section of Pediatric Cardiothoracic Surgery, University of Washington School of Medicine, Seattle Children's Hospital, 4800 Sand Point Way NE, Seattle, WA 98105, United States AU - Karamlou, T. AU - Vafaeezadeh, M. AU - Parrish, A. M. AU - Cohen, G. A. AU - Welke, K. F. AU - Permut, L. AU - McMullan, D. M. DB - Embase Medline DO - 10.1016/j.jtcvs.2012.11.037 IS - 2 KW - oxygen article atrioventricular septal defect cannulation child data base extracorporeal oxygenation female hospital patient human hypoplastic left heart syndrome major clinical study male mortality oxygen supply pediatrics preschool child prevalence priority journal risk reduction survival workload LA - English M3 - Article N1 - L52352373 2012-12-18 2013-01-29 PY - 2013 SN - 0022-5223 1097-685X SP - 470-475 ST - Increased extracorporeal membrane oxygenation center case volume is associated with improved extracorporeal membrane oxygenation survival among pediatric patients T2 - Journal of Thoracic and Cardiovascular Surgery TI - Increased extracorporeal membrane oxygenation center case volume is associated with improved extracorporeal membrane oxygenation survival among pediatric patients UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52352373 http://dx.doi.org/10.1016/j.jtcvs.2012.11.037 VL - 145 ID - 1023 ER - TY - JOUR AB - OBJECTIVES: Despite improved survival in children with hypoplastic left heart syndrome (HLHS), significant concern persists regarding their neurodevelopmental (ND) outcomes. Previous studies have identified patient factors, such as prematurity and genetic syndromes, to be associated with worse ND outcomes. However, no consistent relationships have been identified among modifiable management factors, including cardiopulmonary bypass strategies, and ND outcomes after cardiac surgery in infancy. Studies in immature animals, including primates, have demonstrated neurodegeneration and apoptosis in the brain after certain levels and extended durations of anesthetic exposure. Retrospective human studies have also suggested relationships between adverse ND effects and anesthetic exposure. METHODS: Cumulative minimum alveolar concentration hours (MAC-hrs) of exposure to volatile anesthetic agents (VAA) (desflurane, halothane, isoflurane, and sevoflurane) were collected from an anesthetic database and medical record review for 96 patients with HLHS or variants. ND testing was performed between ages 4 and 5 years, including full-scale IQ, verbal IQ, performance IQ, and processing speed. Four generalized linear modes were hypothesized a priori and tested using a Gaussian (normal) distribution with an identity link. RESULTS: Cumulative VAA exposure ranged from 0 to 35.3 MAC-hrs (median 7.5 hours). Using specified covariates identified previously as significant predictors of ND outcomes, statistically significant relationships were identified between total MAC-hrs exposure and worse full-scale IQ and verbal IQ scores (P's < .05) alone and after adjusting for relevant covariates. CONCLUSIONS: Increased cumulative MAC-hrs exposure to VAA is associated with worse ND outcomes in certain domains in children with HLHS and variants. AD - Department of Anesthesia and Critical Care Medicine, The Children's Hospital of Philadelphia, Perelman School of Medicine at The University of Pennsylvania, Philadelphia, Pa. Division of Pediatric Cardiothoracic Surgery, The Children's Hospital of Philadelphia, Perelman School of Medicine at The University of Pennsylvania, Philadelphia, Pa. Electronic address: gaynor@email.chop.edu. Division of Pediatric Cardiothoracic Surgery, The Children's Hospital of Philadelphia, Perelman School of Medicine at The University of Pennsylvania, Philadelphia, Pa. Division of Biostatistics and Epidemiology, Cincinnati Children's Medical Center, University of Cincinnati School of Medicine, Cincinnati, Ohio. Department of Pediatrics, The Children's Hospital of Philadelphia, Perelman School of Medicine at The University of Pennsylvania, Philadelphia, Pa. Center for Biomedical Informatics, The Children's Hospital of Philadelphia, Perelman School of Medicine at The University of Pennsylvania, Philadelphia, Pa. AN - 27183886 AU - Diaz, L. K. AU - Gaynor, J. W. AU - Koh, S. J. AU - Ittenbach, R. F. AU - Gerdes, M. AU - Bernbaum, J. C. AU - Zackai, E. H. AU - Clancy, R. R. AU - Rehman, M. A. AU - Pennington, J. W. AU - Burnham, N. AU - Spray, T. L. AU - Nicolson, S. C. C2 - PMC5662941 C6 - NIHMS787203 DA - Aug DB - PubMed DO - 10.1016/j.jtcvs.2016.03.095 DP - NLM ET - 2016/05/18 IS - 2 KW - Age Factors Anesthesia, Inhalation/*adverse effects Anesthetics, Inhalation/administration & dosage/*adverse effects *Cardiac Surgical Procedures/adverse effects Child Behavior/*drug effects Child Development/*drug effects Child, Preschool Databases, Factual Developmental Disabilities/*chemically induced/diagnosis/physiopathology/psychology Dose-Response Relationship, Drug Executive Function Female Humans Hypoplastic Left Heart Syndrome/diagnostic imaging/physiopathology/*surgery Intelligence Linear Models Male Medical Records Nervous System/*drug effects/growth & development Neuropsychological Tests Retrospective Studies Risk Factors Verbal Behavior *anesthesia *congenital heart surgery *neurodevelopment *neurotoxicity LA - eng N1 - 1097-685x Diaz, Laura K Gaynor, J William Koh, Shannon J Ittenbach, Richard F Gerdes, Marsha Bernbaum, Judy C Zackai, Elaine H Clancy, Robert R Rehman, Mohamed A Pennington, Jeffrey W Burnham, Nancy Spray, Thomas L Nicolson, Susan C R01 HL071834/HL/NHLBI NIH HHS/United States Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't J Thorac Cardiovasc Surg. 2016 Aug;152(2):482-9. doi: 10.1016/j.jtcvs.2016.03.095. Epub 2016 Apr 18. PY - 2016 SN - 0022-5223 (Print) 0022-5223 SP - 482-9 ST - Increasing cumulative exposure to volatile anesthetic agents is associated with poorer neurodevelopmental outcomes in children with hypoplastic left heart syndrome T2 - J Thorac Cardiovasc Surg TI - Increasing cumulative exposure to volatile anesthetic agents is associated with poorer neurodevelopmental outcomes in children with hypoplastic left heart syndrome VL - 152 ID - 185 ER - TY - JOUR AB - Adults with congenital heart disease (CHD) are an increasing population requiring cardiac operations. To date, the perioperative risk factors for this group have not been identified. This study aimed to identify clinical, morphologic, and hemodynamic risk factors for an adverse outcome. This study retrospectively analyzed a cohort of 500 patients (ages[16 years) who underwent 543 operations between January 2004 and December 2008 at a single center. The composite end point of an adverse outcome was in-hospital death, a prolonged intensive care exceeding 4 days, or both. The composite end point was reached by 253 of the patients (50.6%). Of the 500 patients, 13 (2.6%) died within 30 days after the operation. After logistic regression analysis, the following eight items remained significant: male gender (P = 0.003; odds ratio [OR] 1.8; 95% confidence interval [CI] 1.2-2.6), cyanosis (P > 0.006; OR 3.7; 95% CI 1.5-9.4), functional class exceeding 2 (P = 0.004; OR 2.2; 95% CI 1.3-3.7), chromosomal abnormalities (P = 0.004; OR 3.3; 95% CI 1.4-7.7), impaired renal function (P = 0.019; OR 3.8; 95% CI 1.2-11.5), systemic right ventricle (RV) in a biventricular circulation (P = 0.027; OR 3.3; 95% CI 1.1-9.5), enlargement of the systemic ventricle (P = 0.011; OR 1.7; 95% CI 1.1-2.6), and operation with extracorporeal circulation (P = 0.002; OR 4.3; 95% CI 1.7-11.4). Early mortality in the current adult CHD population is low. Morbidity, however, is significant and influenced by the patients' conditions (male gender, chromosomal abnormalities), history (cyanosis, New York Hospital Association [NYHA] class), and underlying morphology (systemic RV). This information for a large cohort of patients could help progress toward more adequate counseling for adults with a congenital heart defect. © Springer Science+Business Media, LLC 2011. AD - M.O. Vogt, Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Lazarettstrasse 36, 80636 Munich, Germany AU - Vogt, M. O. AU - Hörer, J. AU - Grünewald, S. AU - Otto, D. AU - Kaemmerer, H. AU - Schreiber, C. AU - Hess, J. DB - Embase Medline DO - 10.1007/s00246-011-0093-7 IS - 1 KW - liver enzyme adolescent adult adverse outcome aged article assisted circulation body mass chromosome aberration cohort analysis congenital heart disease cyanosis diabetes mellitus DiGeorge syndrome disease classification Down syndrome endocarditis enzyme blood level female functional status glomerulus filtration rate heart arrhythmia heart hemodynamics heart right ventricle heart single ventricle heart surgery human hypertension hyperthyroidism hyponatremia hypothyroidism intubation kidney dysfunction major clinical study male Marfan syndrome neurologic disease outcome assessment pericardial effusion pleura effusion polycythemia postoperative complication postoperative hemorrhage postoperative infection reoperation retrospective study sex difference surgical mortality surgical risk tracheotomy LA - English M3 - Article N1 - L51610511 2011-09-09 2012-07-17 PY - 2012 SN - 0172-0643 1432-1971 SP - 75-82 ST - Independent risk factors for cardiac operations in adults with congenital heart disease: A retrospective study of 543 operations for 500 patients T2 - Pediatric Cardiology TI - Independent risk factors for cardiac operations in adults with congenital heart disease: A retrospective study of 543 operations for 500 patients UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51610511 http://dx.doi.org/10.1007/s00246-011-0093-7 VL - 33 ID - 1072 ER - TY - JOUR AB - A number of guidelines are available for the management of congenital heart diseases (CHD) from infancy to adult life. However, these guidelines are for patients living in high-income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for CHD, as often these patients present late in the course of the disease and may have coexisting morbidities and malnutrition. Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on August 10 and 11, 2018, at the All India Institute of Medical Sciences. The meeting was supported by Children's HeartLink, a nongovernmental organization based in Minnesota, USA. The aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common CHD; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for CHD; and (iii) indications for use of pacemakers in children. Evidence-based recommendations are provided for indications and timing of intervention in common CHD, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus, and others), obstructive lesions (pulmonary stenosis, aortic stenosis, and coarctation of aorta), and cyanotic CHD (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein's anomaly, and others). In addition, protocols for follow-up of postsurgical patients are also described, disease wise. Guidelines are also given on indications for implantation of permanent pacemakers in children. AD - A. Saxena, Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India AU - Saxena, A. AU - Relan, J. AU - Agarwal, R. AU - Awasthy, N. AU - Azad, S. AU - Chakrabarty, M. AU - Dagar, K. AU - Devagourou, V. AU - Dharan, B. AU - Gupta, S. AU - Iyer, K. AU - Jayranganath, M. AU - Joshi, R. AU - B, J. Kannan AU - Katewa, A. AU - Kohli, V. AU - Kothari, S. AU - Krishnamoorthy, K. AU - Kulkarni, S. AU - Kumar, R. M. AU - Kumar, R. K. AU - Maheshwari, S. AU - Manohar, K. AU - Marwah, A. AU - Mishra, S. AU - Mohanty, S. AU - Murthy, K. S. AU - Rao, K. N. AU - Suresh, P. V. AU - Radhakrishnan, S. AU - Rajashekar, P. AU - Ramakrishnan, S. AU - Rao, N. AU - Rao, S. AU - Chinnaswamy Reddy, H. AU - Sharma, R. AU - Shivaprakash, K. AU - Subramanyan, R. AU - Kumar, R. AU - Talwar, S. AU - Tomar, M. AU - Verma, S. AU - Vijaykumar, R. DB - Embase DO - 10.4103/apc.APC_32_19 IS - 3 KW - aortic stent artificial heart pacemaker atrial septal occluder echocardiograph endocardial pacemaker electrode epicardial pacemaker vascular stent acetylsalicylic acid anticoagulant therapy antiplatelet activity aortic coarctation aortic regurgitation aortic stenosis aortopulmonary septal defect article atrioventricular septal defect balloon dilatation clinical assessment congenital heart disease consensus development cyanosis Down syndrome Ebstein anomaly echocardiography electrocardiogram Fallot tetralogy follow up great vessels transposition heart atrium enlargement heart atrium septum defect heart catheterization heart electrophysiology heart right ventricle double outlet heart right ventricle function heart single ventricle heart valve replacement heart ventricle septum defect human lung artery pressure lung vein drainage anomaly mitral valve regurgitation mouth hygiene pacemaker implantation patent ductus arteriosus practice guideline prematurity pulmonary hypertension pulmonary valve atresia pulmonary valve replacement pulmonary valve stenosis pulmonary vascular obstructive disease thorax radiography transesophageal echocardiography transthoracic echocardiography LA - English M3 - Article N1 - L629056928 2019-08-30 2019-09-04 PY - 2019 SN - 0974-5149 0974-2069 SP - 254-286 ST - Indian guidelines for indications and timing of intervention for common congenital heart diseases: Revised and updated consensus statement of the Working group on management of congenital heart diseases T2 - Annals of Pediatric Cardiology TI - Indian guidelines for indications and timing of intervention for common congenital heart diseases: Revised and updated consensus statement of the Working group on management of congenital heart diseases UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L629056928 http://dx.doi.org/10.4103/apc.APC_32_19 VL - 12 ID - 600 ER - TY - JOUR AB - Background: Serious congenital heart disease frequently requires major congenital heart surgery. It causes much distress for parents, which may not always be recognized and treated appropriately. Patients and Methods: As part of a larger study, 26 mothers of two-month-old infants subjected to recent cardiac surgery were interviewed in depth. Each mother was invited to describe her own and what she perceived were her infant's experiences and to comment on the interview process. A systematic content analysis of the interviews was performed using qualitative research methodology. Results: Almost all participants described acute stress symptoms relating to the diagnosis and the infant's surgery. In addition, most mothers reported that the interview helped them to think about and integrate what had happened to them and their infant, suggesting a probable therapeutic value to the interview. Conclusions: A suitably qualified and experienced mental health professional, assisting the mother to tell her story about the diagnosis and her infant's cardiac surgery, may provide a valuable, brief, and very cost-effective therapeutic intervention for these mothers and infants. It has the potential to alleviate maternal distress, with associated gains for the developing mother-infant relationship, reducing infant morbidity, and enhancing the quality of life for both infant and mother. © The Author(s) 2013. AD - M. Samuel, MonashHeart, Monash Medical Centre, 246 Clayton Road, Clayton, VIC 3168, Australia AU - Re, J. AU - Dean, S. AU - Menahem, S. DB - Embase Medline DO - 10.1177/2150135113481480 IS - 3 KW - acute stress altruism anticipatory anxiety article child care congenital heart disease content analysis coping behavior cost effectiveness analysis female heart surgery heart ventricle septum defect helplessness human hypoplastic left heart syndrome infant infant mortality intensive care unit interview maternal stress mental dissociation morbidity mother child relation personal experience posttraumatic stress disorder priority journal qualitative analysis qualitative research quality of life self report separation anxiety social participation treatment outcome LA - English M3 - Article N1 - L369397993 2013-08-03 2020-04-07 PY - 2013 SN - 2150-136X 2150-1351 SP - 278-285 ST - Infant cardiac surgery: Mothers tell their story: A therapeutic experience T2 - World Journal for Pediatric and Congenital Hearth Surgery TI - Infant cardiac surgery: Mothers tell their story: A therapeutic experience UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L369397993 http://dx.doi.org/10.1177/2150135113481480 VL - 4 ID - 1028 ER - TY - JOUR AB - BACKGROUND: Many uncertainties regarding the fate of children undergoing heart transplantation as infants were present when we and others embarked on this program. Although no truly long-term results are available, a significant cohort of children has now reached preteen and early teenage status. We reviewed our group of infants transplanted more than 10 years ago to assess survival and quality of life as they approach their teenage years. METHODS: We retrospectively reviewed the medical records of all infant (younger than 6 months of age) heart transplant recipients, transplanted between 1988 and 1995, to ascertain survival statistics, incidence of complications, and current health status. RESULTS: A total of 42 patients were identified. The majority of these underwent transplantation for hypoplastic left heart syndrome. Eleven patients have died, 4 early and 7 late. The actual survival at 10 years is 76%. Twenty-seven of the 31 long-term survivors attend regular school; 4 are in special education classes owing to developmental delay. Five patients take medication for attention-deficit disorder. Malignancies have been discovered in 5, and 1 died secondary to this. Six patients have significant renal insufficiency, 1 of whom has undergone renal transplantation. One patient has undergone retransplantation for coronary artery disease. One patient required reoperation for supravalvar aortic stenosis. Other general medical problems that are being treated include sleep apnea (n = 1), hypertension (n = 5), and recurrent pneumonias (n = 1). CONCLUSIONS: Although these children require ongoing medical attention, including daily medications and regular follow-up visits, most have a satisfactory quality of life and behave much like normal children. AD - Department of Surgery, Washington University School of Medicine, St. Louis Children's Hospital, St. Louis, Missouri, USA. gandhis@wustl.edu AN - 17184654 AU - Gandhi, S. K. AU - Canter, C. E. AU - Kulikowska, A. AU - Huddleston, C. B. DA - Jan DB - PubMed DO - 10.1016/j.athoracsur.2006.08.016 DP - NLM ET - 2006/12/23 IS - 1 KW - Child Coronary Disease/etiology Follow-Up Studies Graft Rejection *Heart Transplantation/adverse effects/mortality/psychology Humans Infant Infant, Newborn Kidney/physiopathology Postoperative Complications/etiology Quality of Life Reoperation Retrospective Studies LA - eng N1 - 1552-6259 Gandhi, Sanjiv K Canter, Charles E Kulikowska, Agnieszka Huddleston, Charles B Journal Article Netherlands Ann Thorac Surg. 2007 Jan;83(1):169-71; discussion 172. doi: 10.1016/j.athoracsur.2006.08.016. PY - 2007 SN - 0003-4975 SP - 169-71; discussion 172 ST - Infant heart transplantation ten years later--where are they now? T2 - Ann Thorac Surg TI - Infant heart transplantation ten years later--where are they now? VL - 83 ID - 416 ER - TY - JOUR AB - Background: Many uncertainties regarding the fate of children undergoing heart transplantation as infants were present when we and others embarked on this program. Although no truly long-term results are available, a significant cohort of children has now reached preteen and early teenage status. We reviewed our group of infants transplanted more than 10 years ago to assess survival and quality of life as they approach their teenage years. Methods: We retrospectively reviewed the medical records of all infant (younger than 6 months of age) heart transplant recipients, transplanted between 1988 and 1995, to ascertain survival statistics, incidence of complications, and current health status. Results: A total of 42 patients were identified. The majority of these underwent transplantation for hypoplastic left heart syndrome. Eleven patients have died, 4 early and 7 late. The actual survival at 10 years is 76%. Twenty-seven of the 31 long-term survivors attend regular school; 4 are in special education classes owing to developmental delay. Five patients take medication for attention-deficit disorder. Malignancies have been discovered in 5, and 1 died secondary to this. Six patients have significant renal insufficiency, 1 of whom has undergone renal transplantation. One patient has undergone retransplantation for coronary artery disease. One patient required reoperation for supravalvar aortic stenosis. Other general medical problems that are being treated include sleep apnea (n = 1), hypertension (n = 5), and recurrent pneumonias (n = 1). Conclusions: Although these children require ongoing medical attention, including daily medications and regular follow-up visits, most have a satisfactory quality of life and behave much like normal children. © 2007 The Society of Thoracic Surgeons. AD - S.K. Gandhi, Department of Surgery, Washington University School of Medicine, St. Louis Children's Hospital, St. Louis, MO, United States AU - Gandhi, S. K. AU - Canter, C. E. AU - Kulikowska, A. AU - Huddleston, C. B. DB - Embase Medline DO - 10.1016/j.athoracsur.2006.08.016 IS - 1 KW - aortic stenosis article attention deficit disorder cause of death clinical article cohort analysis coronary artery disease follow up health status heart transplantation human hypertension hypoplastic left heart syndrome incidence infant kidney failure kidney transplantation pneumonia priority journal quality of life recipient recurrent disease reoperation retransplantation retrospective study school sleep disordered breathing special education survival systematic review LA - English M3 - Article N1 - L44917891 2007-01-24 PY - 2007 SN - 0003-4975 SP - 169-172 ST - Infant Heart Transplantation Ten Years Later-Where Are They Now? T2 - Annals of Thoracic Surgery TI - Infant Heart Transplantation Ten Years Later-Where Are They Now? UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L44917891 http://dx.doi.org/10.1016/j.athoracsur.2006.08.016 VL - 83 ID - 1242 ER - TY - JOUR AB - Heart transplantation in infancy has become the fastest growing area in pediatric heart transplantation. Infant heart transplantation presents a profoundly complex decision-making situation for parents. There is a relationship between physician beliefs and their manner of presenting heart transplantation options to parents. The purpose of this study was (1) to determine the beliefs of physicians about neonatal heart transplantation, (2) to define their perceptions about their role in counseling parents about heart transplantation, and (3) to evaluate the differences of beliefs by physician specialties (pediatric cardiologists, neonatologists, and pediatricians) and practice settings (university, private, health maintenance organization). A questionnaire was designed to survey 117 physicians on their beliefs about heart transplantation. Findings revealed significant differences related to beliefs: (1) infant heart transplantation is still experimental (pediatric cardiologists, 56%; neonatologists, 85%; pediatricians, 63%; p < 0.05), (2) neonatal heart transplantation is acceptable for fatal heart disease (pediatric cardiologists, 86%; neonatologists, 73%; pediatricians, 63%; p < 0.05), (3) newborn heart transplantation should be presented negatively to protect parents from potential negative consequences on their quality of life (pediatric cardiologists, 14%; neonatologists, 33%; pediatricians, 48%; p < 0.05); (university, 25%; private, 18%; health maintenance organization, 61%; p < 0.05), and (4) infants with hypoplastic left heart syndrome should not be treated (university, 28%; private, 23%; health maintenance organization, 55%; p < 0.05). Though it is critical to impart information to parents with a fair representation of infant heart transplantation, we conclude that many physicians surveyed had a knowledge deficit or bias about the current state of the art of infant heart transplantation. AD - Division of Pediatric Cardiology, Children's Hospital, Oakland, CA 94609. AN - 8167129 AU - Higgins, S. S. AU - Paul, S. M. AU - Hardy, C. E. AU - Ternullo-Retta, C. AU - Affonso, D. DA - Jan-Feb DB - PubMed DP - NLM ET - 1994/01/01 IS - 1 Pt 1 KW - Academic Medical Centers *Attitude of Health Personnel Cardiology Counseling Female Health Maintenance Organizations Heart Defects, Congenital/surgery *Heart Transplantation Humans *Infant, Newborn Male Middle Aged Neonatology Parents Patient Acceptance of Health Care Patient Participation Pediatrics *Physicians Private Practice Professional-Family Relations LA - eng N1 - Higgins, S S Paul, S M Hardy, C E Ternullo-Retta, C Affonso, D Journal Article Research Support, Non-U.S. Gov't United States J Heart Lung Transplant. 1994 Jan-Feb;13(1 Pt 1):59-65. PY - 1994 SN - 1053-2498 (Print) 1053-2498 SP - 59-65 ST - Infant heart transplantation: a survey of physician beliefs T2 - J Heart Lung Transplant TI - Infant heart transplantation: a survey of physician beliefs VL - 13 ID - 485 ER - TY - JOUR AB - We present a case in which a fetal diagnosis of complex congenital heart disease and trisomy 18 led to a series of decisions for an infant who was critically ill. The parents wanted everything done. The surgeons believed that surgery would be futile. The parents publicized the case on social media, which led to publicity and pressure on the hospital. The case reveals the intersection of parental values, clinical judgments, ethics consultation, insurance company decisions about reimbursement, and social media publicity. Together, these factors complicate the already delicate ethical deliberations and decisions. AD - Center for Bioethics and Social Sciences in Medicine, University of Michigan, Ann Arbor, Michigan Division of Neonatal-Perinatal Medicine, Department of Pediatrics, University of Michigan, Ann Arbor, Michigan Division of Professional Education, Department of Learning Health Sciences, University of Michigan, Ann Arbor, Michigan Department of Surgery, University of Michigan, Ann Arbor, Michigan Children's Mercy Bioethics Center, Children's Mercy Hospital, Kansas City, Missouri AN - 136246644. Language: English. Entry Date: 20190508. Revision Date: 20190510. Publication Type: Article AU - Kukora, Stephanie AU - Firn, Janice AU - Laventhal, Naomi AU - Vercler, Christian AU - Moore, Bryanna AU - Lantos, John D. DB - ccm DO - 10.1542/peds.2018-3779 DP - EBSCOhost IS - 5 KW - Trisomy 18 -- Diagnosis -- In Infancy and Childhood Hypoplastic Left Heart Syndrome -- Diagnosis Infant Parental Attitudes Physician Attitudes Decision Making, Clinical Ethics, Medical Insurance, Health Insurance, Health, Reimbursement Social Media N1 - case study. Journal Subset: Biomedical; Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. NLM UID: 0376422. PY - 2019 SN - 0031-4005 SP - 1-6 ST - Infant With Trisomy 18 and Hypoplastic Left Heart Syndrome T2 - Pediatrics TI - Infant With Trisomy 18 and Hypoplastic Left Heart Syndrome UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=136246644&site=ehost-live&scope=site VL - 143 ID - 1447 ER - TY - JOUR AU - Daniels, S. R. DB - Embase DO - 10.1016/j.jpeds.2011.06.018 IS - 2 KW - article cardiologist child hospitalization complex congenital heart disease congenital heart disease consultation cooperation emergency ward health care facility health care utilization heart single ventricle human infant infant disease intensivist medical education outcome assessment patient care pediatrician primary medical care priority journal simulation LA - English M3 - Article N1 - L619858946 2017-12-25 2018-01-02 PY - 2011 SN - 1097-6833 0022-3476 SP - A2-A3 ST - Infants with complex congenital heart disease in the Emergency Department T2 - Journal of Pediatrics TI - Infants with complex congenital heart disease in the Emergency Department UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L619858946 http://dx.doi.org/10.1016/j.jpeds.2011.06.018 VL - 159 ID - 1099 ER - TY - JOUR AD - Department of Pediatrics, Wayne State University, in affiliation with Children's Hospital of Michigan, Detroit, MI. AN - 104660143. Language: English. Entry Date: 20111028. Revision Date: 20150711. Publication Type: Journal Article AU - Cashen, K. AU - Gupta, P. AU - Lieh-Lai, M. AU - Mastropietro, C. DB - ccm DP - EBSCOhost IS - 2 KW - Attitude of Health Personnel Heart Surgery -- Utilization Emergency Service -- Utilization Heart Ventricle -- Abnormalities Hypoplastic Left Heart Syndrome -- Epidemiology Physicians -- Utilization Cross Sectional Studies Female Gestational Age Heart Catheterization Heart Ventricle -- Surgery Hypoplastic Left Heart Syndrome -- Diagnosis Hypoplastic Left Heart Syndrome -- Surgery Incidence Infant, Newborn Length of Stay Male Michigan Prognosis Retrospective Design Severity of Illness Indices N1 - research. Journal Subset: Biomedical; Peer Reviewed; USA. Special Interest: Pediatric Care. NLM UID: 0375410. PMID: NLM21392789. PY - 2011 SN - 0022-3476 SP - 273-277.e1 ST - Infants with single ventricle physiology in the emergency department: are physicians prepared? T2 - Journal of Pediatrics TI - Infants with single ventricle physiology in the emergency department: are physicians prepared? UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104660143&site=ehost-live&scope=site VL - 159 ID - 1646 ER - TY - JOUR AB - Background: Single ventricle, a rare congenital cardiac defect, often occurs as part of a complex group of cardiovascular abnormalities. Little is known of its epidemiologic associations. Methods: Using data from the Baltimore-Washington Infant Study [BWIS], (1981-89), a population based case-control study of cardiovascular malformations, infants with single ventricle were evaluated with respect to infant and family characteristics and maternal and paternal exposures. The cases were analyzed according to presence/absence of abnormal cardio-visceral situs. Controls were 3,572 infants without heart defects randomly selected from the regional cohort of live births. Odds ratios and 95% confidence intervals were used as measures of association. Results: Single ventricle occurred in 1.25% of infants with congenital cardiovascular defects in the BWIS. Fifty-five infants had single ventricle. In 48 families (87.3%) the parents were interviewed. Thirty-three infants had normal situs and 15 had abnormal situs. Paternal alcohol consumption (OR = 2.0, 95% Cl 1.1-3.9) and paternal cigarette smoking (OR = 2.4, 95% Cl 1.1-5.1) were associated with all cases of single ventricle. These associations were even stronger in the subset of infants with abnormal situs. Maternal history of a previous induced abortion was also associated with infants born with abnormal situs (OR = 3.2, 95% Cl 1.1-11.5). Paternal marijuana use was associated with cases of single ventricle in normal situs (OR = 2.2, 95% Cl 1.0-5.2). Conclusions: Potential risk factors included paternal smoking and alcohol consumption, highlighting the need for future studies to consider environmental factors in the pathogenesis of this cardiac defect. © 2002 Wiley-Liss, Inc. AD - C.A. Loffredo, Lombardi Cancer Center, Georgetown University, 3800 Reservoir Road, Washington, DC 20007, United States AU - Steinberger, E. K. AU - Ferencz, C. AU - Loffredo, C. A. DB - Embase Medline DO - 10.1002/tera.10017 IS - 3 KW - alcohol consumption article cannabis addiction cardiovascular malformation cigarette smoking congenital heart disease controlled study environmental exposure female heart single ventricle human induced abortion major clinical study male newborn pathogenesis priority journal risk factor LA - English M3 - Article N1 - L34177397 2002-03-12 PY - 2002 SN - 0040-3709 SP - 106-115 ST - Infants with single ventricle: A population-based epidemiological study T2 - Teratology TI - Infants with single ventricle: A population-based epidemiological study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L34177397 http://dx.doi.org/10.1002/tera.10017 VL - 65 ID - 1315 ER - TY - JOUR AB - Background In 2007, the American Heart Association modified the infective endocarditis prophylaxis guidelines by limiting the use of antibiotics in patients with cardiac conditions associated with the highest risk of adverse outcomes after infective endocarditis. Our objective was to evaluate current practice for infective endocarditis prophylaxis among paediatric cardiologists. Methods A web-based survey focussing on current practice, describing the use of antibiotics for infective endocarditis prophylaxis in various congenital and acquired heart diseases, was distributed via e-mail to paediatric cardiologists. The survey was kept anonymous and was distributed twice. Results Data from 253 participants were analysed. Most paediatric cardiologists discontinued infective endocarditis prophylaxis in patients with simple lesions such as small ventricular septal defect, patent ductus arteriosus, and bicuspid aortic valve without stenosis or regurgitation; however, significant disagreement persists in prescribing infective endocarditis prophylaxis in certain conditions such as rheumatic heart disease, Fontan palliation without fenestration, and the Ross procedure. Use of antibiotic prophylaxis in certain selected conditions for which infective endocarditis prophylaxis has been indicated as per the current guidelines varies from 44 to 83%. Only 44% follow the current guidelines exclusively, and 34% regularly discuss the importance of oral hygiene with their patients at risk for infective endocarditis. Conclusion Significant heterogeneity still persists in recommending infective endocarditis prophylaxis for several cardiac lesions among paediatric cardiologists. More than half of the participants (56%) do not follow the current guidelines exclusively in their practice. Counselling for optimal oral health in patients at risk for infective endocarditis needs to be optimised in the current practice. AD - N.C. Shah, Department of Pediatrics, Penn State Hershey Medical Center, Penn State College of Medicine, 500 University Drive, Hershey, PA, United States AU - Naik, R. J. AU - Patel, N. R. AU - Wang, M. AU - Shah, N. C. DB - Embase Medline DO - 10.1017/S1047951115002176 IS - 6 KW - antibiotic prophylaxis aortic regurgitation article bacterial endocarditis bicuspid aortic valve cardiologist controlled study doctor patient relationship Fontan procedure health survey heart ventricle septum defect human patent ductus arteriosus patient care patient counseling practice guideline rheumatic heart disease risk factor Ross procedure LA - English M3 - Article N1 - L607445669 2016-01-05 2016-07-14 PY - 2016 SN - 1467-1107 1047-9511 SP - 1176-1182 ST - Infective endocarditis prophylaxis: Current practice trend among paediatric cardiologists: Are we following the 2007 guidelines? T2 - Cardiology in the Young TI - Infective endocarditis prophylaxis: Current practice trend among paediatric cardiologists: Are we following the 2007 guidelines? UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L607445669 http://dx.doi.org/10.1017/S1047951115002176 VL - 26 ID - 798 ER - TY - JOUR AB - OBJECTIVE: The goal was to determine the effect of birth hospital pediatric cardiac specialty center status and neonatal level of care on 90-day mortality for infants with ductal-dependent congenital heart disease. METHODS: A population-based, retrospective, cohort study was conducted in Washington State in 1987-2006. All infants born in Washington with gestational ages of ≥32 weeks and birth weights of ≥1500 g who were admitted to a Washington hospital for the care of a congenital cardiac anomaly likely to be ductal dependent were included. Subjects were required to receive a surgical or interventional cardiac procedure within 30 days after birth. Birth certificate data were linked with death certificate data and hospital administrative records. The exposures of interest were birth hospital pediatric cardiac specialty center status and neonatal level of care. The primary outcome was death within 90 days. RESULTS: A total of 823 infants met the inclusion criteria, 285 born at specialty centers and 538 at other centers. After adjustment for cardiac diagnoses, other congenital anomalies, birth year, maternal income quartile, and definitive-care hospital, there was no significant difference in 90-day mortality for infants born at specialty centers versus other centers (odds ratio: 1.05 [95% confidence interval: 0.65-1.68]). CONCLUSION: For infants with ductal-dependent congenital heart disease, there was no difference in 90-day mortality for infants born at specialty centers versus other centers in the state of Washington. Copyright © 2010 by the American Academy of Pediatrics. AD - T. D. Bennett, University of Utah, School of Medicine, Pediatric Critical Care, PO Box 581289, Salt Lake City, UT 84158-1289, United States AU - Bennett, T. D. AU - Klein, M. B. AU - Sorensen, M. D. AU - De Roos, A. J. AU - Rivara, F. P. DB - Embase Medline DO - 10.1542/peds.2009-2829 IS - 6 KW - aortic arch interruption aorta atresia aortic coarctation aortic valve stenosis article birth weight cohort analysis congenital heart disease ductus arteriosus esophagus malformation female gestational age great vessels transposition human hypoplastic left heart syndrome infant infant mortality intestine malformation limb malformation major clinical study male newborn care outcome assessment priority journal pulmonary valve atresia pulmonary valve stenosis retrospective study situs inversus tricuspid valve atresia trisomy 13 trisomy 18 trisomy 21 Turner syndrome urinary tract malformation velocardiofacial syndrome LA - English M3 - Article N1 - L360075967 2010-12-09 2010-12-15 http://pediatrics.aappublications.org/cgi/reprint/126/6/1156 PY - 2010 SN - 0031-4005 1098-4275 SP - 1156-1164 ST - Influence of birth hospital on outcomes of ductal-dependent cardiac lesions T2 - Pediatrics TI - Influence of birth hospital on outcomes of ductal-dependent cardiac lesions UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L360075967 http://dx.doi.org/10.1542/peds.2009-2829 VL - 126 ID - 1126 ER - TY - JOUR AB - Objective: The present study was designed to examine psychological characteristics of adolescents and adults with operated congenital heart disease (ACHD). Particularly it was to be examined whether cardiological parameters may be associated with subjectively perceived impairments and measures of psychological distress. Patients: A total of 361 men (209) and women (152) between 14 and 45years underwent medical checkups and an interview on psychological and sociological issues. Setting: The medical part consisted of a complete cardiological examination including the classification of residual symptoms according to the New York Heart Association (NYHA), and spiroergometry. The Brief Symptom Inventory was used for depicting current psychological and somatic symptoms. These were assessed on 9 subscales: somatization, obsession-compulsion, interpersonal sensitivity, depression, anxiety, hostility, phobic anxiety, paranoid ideation, and psychoticism. Results: The analyses revealed statistically significant associations between the degree of NYHA class and psychological symptoms. These findings could not be reproduced for physical fitness as measured by peak oxygen consumption. No gender differences emerged. Conclusions: Our results suggest that psychological measures of ACHD are not directly dependent on their physical fitness or on the severity of residual symptoms. Instead, patients'subjective appraisal of their disease severity and the conviction to what degree one can depend on the operated heart may be important determinants of psychological states. © 2006 the Authors; Journal compilation © 2006 Blackwell Publishing, Inc. AD - K. Norozi, Department of Paediatric Cardiology and Intensive Care Medicine, Medical School Hannover, OE 6730 Carl-Neuberg-Str. 1, 30623 Hannover, Germany AU - Norozi, K. AU - Zoege, M. AU - Buchhorn, R. AU - Wessel, A. AU - Geyer, S. DB - Embase Medline DO - 10.1111/j.1747-0803.2006.00048.x IS - 6 KW - oxygen adolescent adult anxiety disorder aortic coarctation aortic valve disease article Brief Symptom Inventory cardiovascular parameters cardiovascular system examination congenital heart disease controlled study depression disease association disease classification disease severity distress syndrome ergometry exercise test Fallot tetralogy female fitness Fontan procedure great vessels transposition heart left right shunt heart single ventricle heart surgery hostility human human relation interview major clinical study male medical examination mental health obsessive compulsive disorder oxygen consumption paranoia patient attitude phobia priority journal psychological aspect psychosis psychosomatics pulmonary valve disease rating scale sex difference social psychology somatization spirometry statistical significance surgical technique symptom LA - English M3 - Article N1 - L44756292 2006-12-06 PY - 2006 SN - 1747-079X 1747-0803 SP - 282-288 ST - The influence of congenital heart disease on psychological conditions in adolescents and adults after corrective surgery T2 - Congenital Heart Disease TI - The influence of congenital heart disease on psychological conditions in adolescents and adults after corrective surgery UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L44756292 http://dx.doi.org/10.1111/j.1747-0803.2006.00048.x VL - 1 ID - 1248 ER - TY - JOUR AB - The total cavopulmonary connection (TCPC) is a palliative cardiothoracic surgical procedure used in patients with one functioning ventricle that excludes the heart from the systemic venous to pulmonary artery pathway. Blood in the superior and inferior vena cavae (SVC, IVC) is diverted directly to the pulmonary arteries. Since only one ventricle is left in the circulation, minimizing pressure drop by optimizing connection geometry becomes crucial. Although there have been numerical and in-vitro studies documenting the effect of connection geometry on overall pressure drop, there is little published data examining the effect of SVC-IVC flow rate ratio on detailed fluid mechanical structures within the various connection geometries. We present here results from a numerical study of the TCPC connection, configured with various connections and SVC:IVC flow ratios. The role of major flow parameters: shear stress, secondary flow, recirculation regions, flow stagnation regions, and flow separation, was examined. Results show a complex interplay among connection geometry, flow rate ratio and the types and effects of the various flow parameters described above. Significant changes in flow structures affected local distribution of pressure, which in turn changed overall pressure drop. Likewise, changes in local flow structure also produced changes in maximum shear stress values; this may have consequences for platelet activation and thrombus formation in the clinical situation. This study sheds light on the local flow structures created by the various connections andflow configurations and as such, provides an additional step toward understanding the detailed fluid mechanical behavior of the more complex physiological configurations seen clinically. AD - Department of Mechanical Engineering, University of Colorado Boulder 80309-0427, USA. AN - 12188203 AU - Khunatorn, Y. AU - Mahalingam, S. AU - DeGroff, C. G. AU - Shandas, R. DA - Aug DB - PubMed DO - 10.1115/1.1487880 DP - NLM ET - 2002/08/22 IS - 4 KW - Algorithms Blood Flow Velocity Child, Preschool Computer Simulation *Fontan Procedure *Hemodynamics Humans *Models, Cardiovascular Pressure Pulmonary Artery/*physiopathology/surgery Reproducibility of Results Sensitivity and Specificity Stress, Mechanical Vena Cava, Inferior/*physiopathology/surgery Vena Cava, Superior/*physiopathology/surgery LA - eng N1 - Khunatorn, Yottana Mahalingam, Shankar DeGroff, Curt G Shandas, Robin HL67393/HL/NHLBI NIH HHS/United States Comparative Study Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S. United States J Biomech Eng. 2002 Aug;124(4):364-77. doi: 10.1115/1.1487880. PY - 2002 SN - 0148-0731 (Print) 0148-0731 SP - 364-77 ST - Influence of connection geometry and SVC-IVC flow rate ratio on flow structures within the total cavopulmonary connection: a numerical study T2 - J Biomech Eng TI - Influence of connection geometry and SVC-IVC flow rate ratio on flow structures within the total cavopulmonary connection: a numerical study VL - 124 ID - 409 ER - TY - JOUR AB - Background The desirable distance, defined as offset, between the central line of the superior vena cava (SVC) and the intra-atrial conduit after an intra-atrial conduit (IAC) Fontan's procedure remained unclear. We compared the hemodynamic features using virtual surgery with different offset designs in our study. Methods Three-dimensional models of IAC Fontan's procedure were reconstructed according to the magnetic resonance imagings (MRIs) of three patients, then four models for each patient with different offsets equaling 100, 67, 33, and 0% of the diameter of the IVC were reconstructed. Computational fluid dynamics (CFD) were performed in each model to predict the best hemodynamic features, including streamlines of blood flow, wall shear stress (WSS), energy loss (EL), and the hepatic flow distribution (HFD) ratio. Results Comprehensive evaluation of WSS, EL, and HFD revealed than an offset of 33% presents the best hemodynamic performance among the three patients modeled. In patient A, an offset of 33% resulted in the best HFD (left pulmonary artery/right pulmonary artery [LPA/RPA] = 35/65%). In patient B, the best trade-off between HFD (35/65%), and WSS was achieved with an offset of 33%. In patient C, EL peaked at an offset of 0% and significantly dropped at an offset of 33% with a desirable HFD (60/40%). Conclusions We verified that the offset distance influences hemodynamic performance in IAC Fontan's procedure. Considering several hemodynamic parameters, the best trade-offs between hemorheology, pulmonary perfusion, and energy efficiency were achieved at an offset of 33%. This distance should be taken into consideration and optimized during the surgical planning for the IAC Fontan's procedure. AD - H. Zhang, Department of Pediatric Cardio-Thoracic Surgery, Shanghai Children's Medical Center, Dongfang Road 1678, Shanghai, China AU - Hu, J. AU - Wang, Q. AU - Tong, Z. AU - Shen, J. AU - Liu, J. AU - Sun, Y. AU - Zhang, H. DB - Embase Medline DO - 10.1055/s-0038-1677490 IS - 1 KW - politef implant adolescent article blood flow clinical article clinical outcome computational fluid dynamics computer aided design congenital heart disease female Fontan procedure heart atrium septum defect heart cycle heart right ventricle double outlet heart single ventricle hemodynamic parameters hemodynamics human image reconstruction intra atrial conduit Fontan procedure left pulmonary artery liver blood flow lung blood vessel lung resistance male nuclear magnetic resonance imaging priority journal pulmonary artery pulmonary valve stenosis right pulmonary artery shear stress superior cava vein viscosity LA - English M3 - Article N1 - L630771065 2020-02-12 2020-02-26 PY - 2020 SN - 1439-1902 0171-6425 SP - 38-44 ST - Influence of Offset on Hemodynamics of Intra-atrial Conduit Fontan's Procedure and Its Clinical Implications T2 - Thoracic and Cardiovascular Surgeon TI - Influence of Offset on Hemodynamics of Intra-atrial Conduit Fontan's Procedure and Its Clinical Implications UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L630771065 http://dx.doi.org/10.1055/s-0038-1677490 VL - 68 ID - 578 ER - TY - JOUR AB - OBJECTIVE: The study objective was to evaluate various types of Norwood arch reconstruction methods and to show the factors that affect the cardiac workload of the single ventricle. The Norwood procedure is one of the most challenging congenital heart surgeries. Several aortic arch reconstruction techniques have been reported to avoid recoarctation, ensure coronary perfusion, and improve long-term outcomes. Inside the arch, complicated turbulent flow is generated; however, little is known about the cause of the disadvantageous inefficient flow and the surgical techniques to avoid it. METHODS: We created patient-specific computational hemodynamic models of 9 patients who underwent different types of arch reconstruction methods. Four patients had aortic atresia, and 5 patients had aortic stenosis. Flow profiles were defined by echocardiography data corrected with body surface area. Turbulent pulsatile flow was analyzed with the finite volume method. Flow energy loss was calculated to estimate cardiac workload, and wall shear stress was calculated to estimate vessel wall stiffness increase. RESULTS: Recoarctation and acute arch angles increased wall shear stress and energy loss. In the patients with aortic atresia, a longitudinal incision toward the descending aorta was effective in creating a smooth arch angle. In the patients with aortic stenosis, arch repair with the Damus-Kaye-Stansel procedure in a single anastomotic site was effective in creating sufficient anastomosis space and a smooth arch angle. CONCLUSIONS: Creation of a large anastomotic space and a smooth aortic arch angle reduced wall shear stress and energy loss, and should improve long-term cardiac performance after the Norwood procedure. AD - Department of the Cardiovascular Surgery, Kitasato University School of Medicine, Kanagawa, Japan. AN - 21907359 AU - Itatani, K. AU - Miyaji, K. AU - Qian, Y. AU - Liu, J. L. AU - Miyakoshi, T. AU - Murakami, A. AU - Ono, M. AU - Umezu, M. DA - Jul DB - PubMed DO - 10.1016/j.jtcvs.2011.08.013 DP - NLM ET - 2011/09/13 IS - 1 KW - Aorta, Thoracic/abnormalities/physiopathology/*surgery Aortic Coarctation/physiopathology/*surgery Aortic Valve Stenosis/congenital/physiopathology/*surgery Child, Preschool Female Heart Ventricles/physiopathology/*surgery Humans Infant Male *Norwood Procedures Pulsatile Flow Treatment Outcome LA - eng N1 - 1097-685x Itatani, Keiichi Miyaji, Kagami Qian, Yi Liu, Jin Long Miyakoshi, Tomoyuki Murakami, Arata Ono, Minoru Umezu, Mitsuo Journal Article United States J Thorac Cardiovasc Surg. 2012 Jul;144(1):130-8. doi: 10.1016/j.jtcvs.2011.08.013. Epub 2011 Sep 9. PY - 2012 SN - 0022-5223 SP - 130-8 ST - Influence of surgical arch reconstruction methods on single ventricle workload in the Norwood procedure T2 - J Thorac Cardiovasc Surg TI - Influence of surgical arch reconstruction methods on single ventricle workload in the Norwood procedure VL - 144 ID - 526 ER - TY - JOUR AB - In utero diagnosis of complex progressive cardiac disease such as hypoplastic left heart syndrome presents a novel opportunity for antepartum, intrapartum, and neonatal management. The clinical possibilities and potential for differing outcomes challenge the mother-foetus dyad with regard to informed consent. Previous studies reveal that rates of termination of pregnancy for foetuses with hypoplastic left heart syndrome vary widely in the United States and Europe, leading us to surmise that informed consent may be practised differently. The purpose of this paper is to review the ethical considerations and physician responsibilities of informed consent as they relate to prenatal and postnatal patients with hypoplastic left heart syndrome. Special consideration is paid to the informed consent process as practised by the obstetrician, perinatologist, paediatric cardiologist, and paediatric cardiac surgeon as it relates to termination of pregnancy, comfort care, and surgical palliation. We will argue that informed consent as it relates to hypoplastic left heart syndrome is far from standardised and that there exists a state of bioethical equipoise concerning the extent and limits of its application in the current clinical setting. AD - Department of Pediatric and Adult Congenital Heart Surgery, Center for Ethics, Humanities and Spiritual Care, Cleveland Clinic Children's Hospital, 9500Euclid Avenue/M41-02, Cleveland, Ohio 44195, USA. mavrouc@ccf.org AN - 22152540 AU - Mavroudis, C. AU - Mavroudis, C. D. AU - Farrell, R. M. AU - Jacobs, M. L. AU - Jacobs, J. P. AU - Kodish, E. D. DA - Dec DB - PubMed DO - 10.1017/s1047951111001715 DP - NLM ET - 2011/12/14 KW - *Bioethical Issues Cardiac Surgical Procedures Delivery of Health Care/*ethics Female Humans *Hypoplastic Left Heart Syndrome/diagnosis/psychology/surgery Infant, Newborn Informed Consent/*ethics Pregnancy Pregnancy Outcome Prenatal Diagnosis/*ethics LA - eng N1 - 1467-1107 Mavroudis, Constantine Mavroudis, Constantine D Farrell, Ruth M Jacobs, Marshall L Jacobs, Jeffrey P Kodish, Eric D Journal Article Review England Cardiol Young. 2011 Dec;21 Suppl 2:133-40. doi: 10.1017/S1047951111001715. PY - 2011 SN - 1047-9511 SP - 133-40 ST - Informed consent, bioethical equipoise, and hypoplastic left heart syndrome T2 - Cardiol Young TI - Informed consent, bioethical equipoise, and hypoplastic left heart syndrome VL - 21 Suppl 2 ID - 153 ER - TY - JOUR AB - The Fontan operation, an efficient palliative surgery, is performed for patients with single-ventricle pathologies. The total cavopulmonary connection is a preferred Fontan procedure in which the superior and inferior vena cava are connected to the left and right pulmonary artery. The overall goal of this work is to develop an artificial right ventricle that can be introduced into the inferior vena cava, which would act to reverse the deleterious hemodynamics in post-Fontan patients. We present the initial design and computational analysis of a micro-axial pump, designed with the particular hemodynamics of Fontan physiology in mind. Preliminary in vitro data on a prototype pump are also presented. Computational studies showed that the new design can deliver a variety of advantageous operating conditions, including decreased venous pressure through proximal suction, increased pressure rise across the pump, increased pulmonary flows, and minimal changes in superior vena cava pressures. In vitro studies on a scaled prototype showed trends similar to those seen computationally. We conclude that a micro-axial flow pump can be designed to operate efficiently within the low-pressure, low-flow environment of cavopulmonary flows. The results provide encouragement to pursue this design to for in vitro studies and animal studies. AD - Department of Mechanical Engineering, University of Colorado, Boulder, Colorado, USA. AN - 17117059 AU - Wang, R. AU - Lacour-Gayet, F. G. AU - Lanning, C. J. AU - Rech, B. A. AU - Kilfoil, P. J. AU - Hertzberg, J. AU - Shandas, R. DA - Nov-Dec DB - PubMed DO - 10.1097/01.mat.0000249038.69048.3c DP - NLM ET - 2006/11/23 IS - 6 KW - Animals Blood Pressure Child *Fontan Procedure *Heart Bypass, Right Heart Defects, Congenital/*surgery *Heart-Assist Devices Hemolysis Humans In Vitro Techniques Models, Cardiovascular Prosthesis Design Pulmonary Wedge Pressure Stress, Mechanical Vena Cava, Inferior/physiology/surgery LA - eng N1 - 1538-943x Wang, Rui Lacour-Gayet, Francois G Lanning, Craig J Rech, Bryan A Kilfoil, Peter J Hertzberg, Jean Shandas, Robin Journal Article United States ASAIO J. 2006 Nov-Dec;52(6):682-92. doi: 10.1097/01.mat.0000249038.69048.3c. PY - 2006 SN - 1058-2916 SP - 682-92 ST - Initial experience with the development and numerical and in vitro studies of a novel low-pressure artificial right ventricle for pediatric Fontan patients T2 - Asaio j TI - Initial experience with the development and numerical and in vitro studies of a novel low-pressure artificial right ventricle for pediatric Fontan patients VL - 52 ID - 334 ER - TY - JOUR AB - Background: Children with single ventricle (SV) heart disease who undergo Fontan operation are at risk for developing multiorgan dysfunction. Although survival has improved, significant comorbidities involving multiple organ systems may develop, requiring evaluation and management by many subspecialists. Using data from an internal survey, we documented high care variability for our Fontan population. We then developed a multidisciplinary clinic, designed and implemented a clinical care pathway to decrease variability of patient assessment. Methods: After creating a multidisciplinary team and a clinical care pathway, we initiated a multidisciplinary clinic (MDC) where patients could see multiple subspecialists during a single encounter. We then monitored our effectiveness by retrospective chart review to determine care pathway adherence (process measure) and incidence of new diagnoses of end-organ injury (outcome measure) as well interventions implemented. Adherence was analyzed using statistical process control (SPC) charts. Results: Eighty-six patients were seen in the MDC from January 2016 to September 2017. The proportion of patients with appropriate testing increased, related to strong care pathway adherence. A significant amount of novel pathology was diagnosed in all evaluated organ systems, both Fontan-associated comorbidities and general pediatric diagnoses. Subsequent interventions included cardiac catheterization n = 21 (31%) with more than half of these patients undergoing intervention n = 17 (20%), and liver biopsy n = 9 (10%). Additionally, 58 patients (67%) were referred to a neuropsychologist based on perceived clinical need, with n = 34 (40%) undergoing a neuropsychological evaluation. Conclusions: Children who have undergone Fontan palliation are at risk for developing cardiac and noncardiac comorbidities. Use and adherence to an institutional care pathway resulted in the diagnosis of significant novel pathology and subsequently provided opportunity for intervention. AD - M.V. Di Maria, Heart Institute, Section of Pediatric Cardiology, Department of Pediatrics, University of Colorado School of Medicine, Children’s Hospital Colorado, Aurora, CO, United States AU - Di Maria, M. V. AU - Barrett, C. AU - Rafferty, C. AU - Wolfe, K. AU - Kelly, S. L. AU - Liptzin, D. R. AU - Brigham, D. AU - Younoszai, A. DB - Embase Medline DO - 10.1111/chd.12769 IS - 4 KW - adolescent adult article child clinical pathway comorbidity female Fontan procedure heart catheterization heart single ventricle human liver biopsy major clinical study male multidisciplinary team neuropsychological test outcome assessment patient assessment priority journal protocol compliance survivor young adult LA - English M3 - Article N1 - L627129026 2019-04-12 2019-09-02 PY - 2019 SN - 1747-0803 1747-079X SP - 590-599 ST - Initiating a Fontan multidisciplinary clinic: Decreasing care variability, improving surveillance, and subsequent treatment of Fontan survivors T2 - Congenital Heart Disease TI - Initiating a Fontan multidisciplinary clinic: Decreasing care variability, improving surveillance, and subsequent treatment of Fontan survivors UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L627129026 http://dx.doi.org/10.1111/chd.12769 VL - 14 ID - 641 ER - TY - JOUR AB - Hypoplastic left heart syndrome (HLHS) with intact atrial septum (HLHS-IAS) carries a high risk of mortality and affects about 6% of all patients with HLHS. Fetal interventions, postnatal transcatheter interventions, and postnatal surgical resection have all been used, but the mortality risk continues to be high in this subgroup of patients. We describe a novel, sequential approach to manage HLHS-IAS and progressive fetal hydrops. A 28-year-old, gravida 4 para 2 mother was referred to Mayo Clinic for fetal HLHS. Fetal echocardiography at 28 weeks of gestation demonstrated HLHS-IAS with progressive fetal hydrops. The atrial septum was thick and muscular with no interatrial communication. Ultrasound-guided fetal atrial septostomy was performed with successful creation of a small atrial communication. However, fetal echocardiogram at 33 weeks of gestation showed recurrence of a pleural effusion and restriction of the atrial septum. We proceeded with an Ex utero Intrapartum Treatment (EXIT) delivery and open atrial septectomy. This was performed successfully, and the infant was stabilized in the intensive care unit. The infant required venoarterial extracorporeal membrane oxygenator support on day of life 1. The patient later developed hemorrhagic complications, leading to his demise on day of life 9. This is the first reported case of an EXIT procedure and open atrial septectomy performed without cardiopulmonary bypass for an open-heart operation and provides a promising alternative strategy for the management of HLHS-IAS in select cases. © 2018 Mayo Foundation for Medical Education and Research AD - Department of Cardiovascular Surgery, Mayo Clinic College of Medicine, Rochester, MN, United States Division of Pediatric Cardiology, Mayo Clinic College of Medicine, Rochester, MN, United States Division of Obstetrics and Maternal Fetal Medicine, Mayo Clinic College of Medicine, Rochester, MN, United States Division of Pediatric Otorhinolaryngology, Mayo Clinic College of Medicine, Rochester, MN, United States Division of Pediatric Anesthesiology, Mayo Clinic College of Medicine, Rochester, MN, United States Division of Cardiovascular Anesthesia, Mayo Clinic College of Medicine, Rochester, MN, United States Department of Anesthesiology and Perioperative Medicine, Mayo Clinic College of Medicine, Rochester, MN, United States Department of Pediatric and Adolescent Medicine, Mayo Clinic College of Medicine, Rochester, MN, United States Division of Neonatal Medicine, Mayo Clinic College of Medicine, Rochester, MN, United States AU - Said, S. M. AU - Qureshi, M. Y. AU - Taggart, N. W. AU - Anderson, H. N. AU - O'Leary, P. W. AU - Cetta, F. AU - Alrahmani, L. AU - Cofer, S. A. AU - Segura, L. G. AU - Pike, R. B. AU - Sharpe, E. E. AU - Derleth, D. P. AU - Nemergut, M. E. AU - Van Dorn, C. S. AU - Gleich, S. J. AU - Rose, C. H. AU - Collura, C. A. AU - Ruano, R. DB - Scopus DO - 10.1016/j.mayocp.2018.08.004 IS - 2 M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2019 SP - 356-361 ST - Innovative 2-Step Management Strategy Utilizing EXIT Procedure for a Fetus With Hypoplastic Left Heart Syndrome and Intact Atrial Septum T2 - Mayo Clinic Proceedings TI - Innovative 2-Step Management Strategy Utilizing EXIT Procedure for a Fetus With Hypoplastic Left Heart Syndrome and Intact Atrial Septum UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85060728494&doi=10.1016%2fj.mayocp.2018.08.004&partnerID=40&md5=ac6b35ed8f648c4c343bfe4e9cd0f470 VL - 94 ID - 1766 ER - TY - JOUR AB - Mechanical circulatory support for infants with single ventricle physiology remains challenging. Utilization of a ventricular assist device (VAD) has potential advantages over extracorporeal circulatory membrane oxygenation. As such, VAD utilization in single ventricle patients with refractory heart failure continues to be explored. Herein, we describe a novel VAD strategy to support an infant with Glenn physiology who presented in cardiogenic shock related to myocardial depression of unknown etiology. This VAD configuration supported the systemic circulation independent of the Glenn circulation. Seven days of VAD support resulted in recovery of myocardial and end-organ function leading to VAD removal. The patient remains alive and free from transplantation 16 months post VAD explantation. AD - U. Boston, Division of Pediatric Cardiothoracic Surgery, Department of Surgery, University of Tennessee Health Science Center, Le Bonheur Children's Hospital, Memphis, TN, United States AU - Boston, U. AU - Sun, J. X. AU - Kumar, T. K. S. AU - Knott-Craig, C. DB - Embase Medline DO - 10.1097/MAT.0000000000000836 IS - 3 KW - ventricular assist device milrinone article cardiogenic shock depression device removal explant flow rate heart ejection fraction heart function heart ventricle contractility heart ventricle function human implantation infant myocardial disease physiology priority journal systemic circulation transplantation weaning LA - English M3 - Article N1 - L627402062 2019-05-14 PY - 2019 SN - 1538-943X 1058-2916 SP - e30-e32 ST - An innovative ventricular assist device strategy as a bridge-to-recovery in an infant with glenn physiology T2 - ASAIO Journal TI - An innovative ventricular assist device strategy as a bridge-to-recovery in an infant with glenn physiology UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L627402062 http://dx.doi.org/10.1097/MAT.0000000000000836 VL - 65 ID - 625 ER - TY - JOUR AB - Purpose: This study assessed the underexplored factors associated with significant improvement in mothers’ mental health during postpartum inpatient psychiatric care. Methods: This study analyzed clinical improvement in a prospective cohort of 869 women jointly admitted with their infant to 13 psychiatric Mother-Baby Units (MBUs) in France between 2001 and 2007. Predictive variables tested were: maternal mental illness (ICD-10), sociodemographic characteristics, mental illness and childhood abuse history, acute or chronic disorder, pregnancy and birth data, characteristics and mental health of the mother’s partner, and MBU characteristics. Results: Two thirds of the women improved significantly by discharge. Admission for 25% was for a first acute episode very early after childbirth. Independent factors associated with marked improvement at discharge were bipolar or depressive disorder, a first acute episode or relapse of such an episode. Schizophrenia, a personality disorder, and poor social integration (as measured by occupational status) were all related to poor clinical outcomes. Discussion: Most women improved significantly while under care in MBUs. Our results emphasize the importance of the type of disease but also its chronicity and the social integration when providing postpartum psychiatric care. (PsycInfo Database Record (c) 2020 APA, all rights reserved) AD - Glangeaud-Freudenthal, N.M.-C., Inserm, UMRS 953, Epidemiological Research Unit on Perinatal Health and Women’s and Children’s Health, 16, avenue Paul-Vaillant-Couturier, 94807, Villejuif, France, cedex AN - 2011-10849-003 AU - Glangeaud-Freudenthal, N. M. C. AU - Sutter, A. L. AU - Thieulin, A. C. AU - Dagens-Lafont, V. AU - Zimmermann, M. A. AU - Debourg, A. AU - Massari, B. AU - Cazas, O. AU - Cammas, R. AU - Rainelli, C. AU - Poinso, F. AU - Maron, M. AU - Nezelof, S. AU - Ancel, P. Y. AU - Khoshnood, B. DB - psyh DO - 10.1016/j.eurpsy.2010.03.006 DP - EBSCOhost IS - 4 KW - postpartum psychiatric care inpatients maternal mental health mothers Adolescent Adult Female Humans Infant Infant, Newborn International Classification of Diseases Male Mental Disorders Mental Health Middle Aged Patient-Centered Care Postnatal Care Postpartum Period Pregnancy Prospective Studies Surveys and Questionnaires Treatment Outcome Young Adult Hospitalized Patients Mental Health Services Postpartum Depression Psychiatric Evaluation N1 - Inserm, UMRS 953, Epidemiological Research Unit on Perinatal Health and Women’s and Children’s Health, Villejuif, France. Other Publishers: Cambridge University Press. Release Date: 20110801. Correction Date: 20200416. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Hospitalized Patients; Mental Health Services; Mothers; Postpartum Depression; Psychiatric Evaluation. Classification: Inpatient & Hospital Services (3379). Population: Human (10); Female (40); Inpatient (50). Location: France. Age Group: Childhood (birth-12 yrs) (100); Infancy (2-23 mo) (140); Adolescence (13-17 yrs) (200); Adulthood (18 yrs & older) (300); Young Adulthood (18-29 yrs) (320); Thirties (30-39 yrs) (340); Middle Age (40-64 yrs) (360). Tests & Measures: Marce Clinical Checklist-French Version. Methodology: Empirical Study; Longitudinal Study; Prospective Study; Quantitative Study. References Available: Y. Page Count: 9. Issue Publication Date: May, 2011. Publication History: First Posted Date: Jun 12, 2010; Accepted Date: Mar 11, 2010; Revised Date: Mar 3, 2010; First Submitted Date: Nov 14, 2009. Copyright Statement: All rights reserved. Elsevier Masson SAS. 2010. PY - 2011 SN - 0924-9338 1778-3585 SP - 215-223 ST - Inpatient mother-and-child postpartum psychiatric care: Factors associated with improvement in maternal mental health T2 - European Psychiatry TI - Inpatient mother-and-child postpartum psychiatric care: Factors associated with improvement in maternal mental health UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2011-10849-003&site=ehost-live&scope=site nine.glangeaud@inserm.fr VL - 26 ID - 1688 ER - TY - JOUR AB - BACKGROUND: Patients with a Fontan circulation have reduced exercise capacity and respiratory muscle strength. Inspiratory muscle training (IMT) improves exercise capacity and quality of life in adults with heart failure. We assessed whether 6 weeks of a home-based program of IMT improves inspiratory muscle strength and the ventilatory efficiency of exercise in adolescent patients with a Fontan circulation. METHODS AND RESULTS: Twenty-three adolescent participants (aged 16±2 years) with a Fontan circulation underwent 6 weeks of IMT for 30 minutes daily. Respiratory muscle strength (maximal inspiratory pressure and expiratory pressure), lung function, and exercise capacity (cardiopulmonary exercise testing) were assessed. Fourteen of 23 participants also underwent exercise cardiac magnetic resonance imaging to examine the effects of IMT on cardiac output and systemic and pulmonary blood flow. Six weeks of IMT improved maximal inspiratory pressure by 36±24 cm H(2)O (61±46%) with no change in maximal expiratory pressure. Ventilatory efficiency of exercise improved after 6 weeks of IMT (from 34.2±7.8 to 32.2±5.6, P=0.04). In those who underwent exercise cardiac magnetic resonance imaging, IMT increased resting cardiac output (from 4.2±1.2 to 4.5±1.0 L/min, P=0.03) and ejection fraction (from 50.1±4.3 to 52.8±6.1%, P=0.03). CONCLUSIONS: Six weeks of IMT is associated with improved inspiratory muscle strength, ventilatory efficiency of exercise, and resting cardiac output in young Fontan patients. IMT may be a simple beneficial addition to the current management of Fontan patients, potentially reducing exercise intolerance and long-term morbidity and mortality. AD - The Children's Hospital at Westmead, Sydney, NSW, Australia klaohachai@gmail.com. The University of Sydney, Sydney, NSW, Australia. The Children's Hospital at Westmead, Sydney, NSW, Australia. The Royal Children's Hospital, Melbourne, Vic., Australia. Murdoch Children's Research Institute, Melbourne, Vic., Australia. The Royal Prince Alfred Hospital, Sydney, NSW, Australia. AN - 28862962 AU - Laohachai, K. AU - Winlaw, D. AU - Selvadurai, H. AU - Gnanappa, G. K. AU - d'Udekem, Y. AU - Celermajer, D. AU - Ayer, J. C2 - PMC5586429 DA - Aug 21 DB - PubMed DO - 10.1161/jaha.117.005750 DP - NLM ET - 2017/09/02 IS - 8 KW - Adolescent Breathing Exercises/*methods *Cardiac Output Child Exercise Test *Exercise Tolerance Female *Fontan Procedure/adverse effects Health Status Heart Defects, Congenital/physiopathology/*surgery Humans *Inhalation Lung/*physiopathology Magnetic Resonance Imaging Male *Muscle Contraction *Muscle Strength Recovery of Function Respiratory Muscles/*physiopathology Spirometry Time Factors Treatment Outcome Young Adult Fontan procedure exercise exercise capacity inspiratory muscle strength LA - eng N1 - 2047-9980 Laohachai, Karina Winlaw, David Selvadurai, Hiran Gnanappa, Ganesh Kumar d'Udekem, Yves Celermajer, David Ayer, Julian Journal Article J Am Heart Assoc. 2017 Aug 21;6(8):e005750. doi: 10.1161/JAHA.117.005750. PY - 2017 SN - 2047-9980 ST - Inspiratory Muscle Training Is Associated With Improved Inspiratory Muscle Strength, Resting Cardiac Output, and the Ventilatory Efficiency of Exercise in Patients With a Fontan Circulation T2 - J Am Heart Assoc TI - Inspiratory Muscle Training Is Associated With Improved Inspiratory Muscle Strength, Resting Cardiac Output, and the Ventilatory Efficiency of Exercise in Patients With a Fontan Circulation VL - 6 ID - 38 ER - TY - JOUR AB - Because of improved survival among children born with heart disease, more adults than children are now living with congenital heart defects, providing a new challenge for ongoing evaluation and care. At Children's Memorial Hospital in Chicago, we have gained extensive expertise in the long-term outcome of patients with repaired single ventricle anatomy, particularly with regard to arrhythmias and impaired hemodynamics, and have developed an integrated approach to their care. This article will summarize (a) single ventricle physiology, (b) evolution of the Fontan operation and the long-term multi-system sequelae, (c) treatment options for patients with prior Fontan surgery, focusing on Fontan conversion with arrhythmia surgery, and (d) unique management of adult patients with prior Fontan surgery. For the foreseeable future, pediatric nurse practitioners will have an important role, including coordination of care and providing continuity in the care of adults with prior Fontan surgery. AD - Pediatric Nurse Practitioner in Electrophysiology/Cardiology, Children's Memorial Hospital, Chicago, IL AN - 105651416. Language: English. Entry Date: 20080926. Revision Date: 20200518. Publication Type: Journal Article AU - Dechert, B. E. AU - Deal, B. J. DB - ccm DO - 10.1016/j.pedhc.2007.08.016 DP - EBSCOhost IS - 4 KW - Arrhythmia -- Therapy Heart Defects, Congenital -- Surgery Heart Surgery Pediatric Nursing Adult Antiarrhythmia Agents Exercise Tolerance Heart Defects, Congenital -- Physiopathology Hemodynamics Quality of Life N1 - pictorial. Journal Subset: Core Nursing; Nursing; Peer Reviewed; USA. NLM UID: 8709735. PMID: NLM18590870. PY - 2008 SN - 0891-5245 SP - 246-253 ST - An integrated approach to the care of adult patients with prior atriopulmonary fontan surgery T2 - Journal of Pediatric Healthcare TI - An integrated approach to the care of adult patients with prior atriopulmonary fontan surgery UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105651416&site=ehost-live&scope=site VL - 22 ID - 1574 ER - TY - JOUR AB - Orthotopic cardiac transplantation has become established for selected infants with severe forms of congenital heart disease. This study reviews the combined experience and intermediate term results of infants undergoing orthotopic cardiac transplantation from Children's Memorial Hospital, Chicago, and Kosair Children's Hospital, Louisville. From June 1986 through December 1989, 20 orthotopic cardiac transplantations were performed in 19 patients. Sixteen patients had variants of hypoplastic left heart syndrome. One infant had anomalous origin of the left coronary artery with severe ischemic cardiomyopathy. Two infants had aortic stenosis with endocardial fibroelastosis, and one had extracorporeal membrane oxygenation as a bridge to transplantation. Immunosuppression included cyclosporine, azathioprine (Imuran), and corticosteroids with an effort to wean the patients from steroids by 6 months to 2 years. Three early deaths resulted--from technical errors in two patients and from hyperacute rejection in one patient at 3 days. Four late deaths have occurred. Two patients died at 2 and 13 months of acute rejection. One patient died at 15 months of acute rejection after retransplantation. One patient died at 7 months of respiratory syncytial viral pneumonia. The remaining 12 patients are surviving 5 to 47 months (means 20 months) after orthotopic cardiac transplantation. Rejection surveillance in the first 6 months is by clinical signs supplemented by echocardiography, electrocardiography, and cell cycle analysis; endomyocardial biopsy is used after 6 months of age. For the cumulative series, 24 episodes of suspected rejection have been treated during 277 at-risk patient months with intravenous methylprednisolone (Solu-Medrol) (n = 18) and monoclonal antibody (OKT3) (n = 6), for an incidence of 1.04 episodes of rejection per patient per year. Serious posttransplantation infections including endocarditis, catheter sepsis, meningitis, and colonic perforation were successfully treated in four patients. Subjectively, their quality of life is excellent as shown by normal growth and developmental milestones and a low hospital readmission rate (1.4 episodes per patient per year). These encouraging intermediate term results warrant continued application of infant orthotopic cardiac transplantation for severe forms of congenital heart disease. AD - Division of Cardiovascular-Thoracic Surgery, Children's Memorial Hospital, Chicago, IL 60614. AN - 1902539 AU - Backer, C. L. AU - Zales, V. R. AU - Harrison, H. L. AU - Idriss, F. S. AU - Benson, D. W., Jr. AU - Mavroudis, C. DA - May DB - PubMed DP - NLM ET - 1991/05/11 IS - 5 KW - Antibodies, Monoclonal/therapeutic use Aorta/*abnormalities Female Graft Rejection/drug effects Heart Defects, Congenital/*surgery Heart Transplantation/*methods/mortality Heart Ventricles/abnormalities/surgery Humans Infant Infant, Newborn Male Methylprednisolone/therapeutic use Muromonab-CD3 Survival Rate Syndrome LA - eng N1 - Backer, C L Zales, V R Harrison, H L Idriss, F S Benson, D W Jr Mavroudis, C Journal Article United States J Thorac Cardiovasc Surg. 1991 May;101(5):826-32. PY - 1991 SN - 0022-5223 (Print) 0022-5223 SP - 826-32 ST - Intermediate term results of infant orthotopic cardiac transplantation from two centers T2 - J Thorac Cardiovasc Surg TI - Intermediate term results of infant orthotopic cardiac transplantation from two centers VL - 101 ID - 547 ER - TY - JOUR AB - Orthotopic cardiac transplantation has become established for selected infants with severe forms of congenital heart disease. This study reviews the combined experience and intermediate term results of infants undergoing orthotopic cardiac transplantation from Children's Memorial Hospital, Chicago, and Kosair Children's Hospital, Louisville. From June 1986 through December 1989, 20 orthotopic cardiac transplantations were performed in 19 patients. Sixteen patients had variants of hypoplastic left heart syndrome. One infant had anomalous origin of the left coronary artery with severe ischemic cardiomyopathy. Two infants had aortic stenosis with endocardial fibroelastosis, and one had extracorporeal membrane oxygenation as a bridge to transplantation. Immunosuppression included cyclosporine, azathioprine (Imuran), and corticosteroids with an effort to wean the patients from steroids by 6 months to 2 years. Three early deaths resulted - from technical errors in two patients and from hyperacute rejection in one patient at 3 days. Four late deaths have occurred. Two patients died at 2 and 13 months of acute rejection. One patient died at 15 months of acute rejection after retransplantation. One patient died at 7 months of respiratory syncytial viral pneumonia. The remaining 12 patients are surviving 5 to 47 months (mean 20 months) after orthotopic cardiac transplantation. Rejection surveillance in the first 6 months is by clinical signs supplemented by echocardiography, electrocardiography, and cell cycle analysis; endomyocardial biopsy is used after 6 months of age. For the cumulative series, 24 episodes of suspected rejection have been treated during 277 at-risk patient months with intravenous methylprednisolone (Solu-Medrol) (n = 18) and monoclonal antibody (OKT3) (n = 6), for an incidence of 1.04 episodes of rejection per patient per year. Serious posttransplantation infections including endocarditis, catheter sepsis, meningitis, and colonic perforation were successfully treated in four patients. Subjectively, their quality of life is excellent as shown by normal growth and development milestones and a low hospital readmission rate (1.4 episodes per patient per year). These encouraging intermediate term results warrant continued application of infant orthotopic cardiac transplantation for severe forms of congenital heart disease. AD - C. Mavroudis, Division of Cardiovascular, Thoracic Surgery, Children's Memorial Hospital, Box 22, 2300 Children's Plaza, Chicago, IL 60614, United States AU - Backer, C. L. AU - Zales, V. R. AU - Harrison, H. L. AU - Idriss, F. S. AU - Benson Jr, D. W. AU - Mavroudis, C. DB - Embase Medline IS - 5 KW - article clinical article congenital heart disease graft rejection heart transplantation human infant mortality priority journal LA - English M3 - Article N1 - L21247386 1991-09-12 PY - 1991 SN - 0022-5223 SP - 826-832 ST - Intermediate term results of infant orthotopic cardiac transplantation from two centers T2 - Journal of Thoracic and Cardiovascular Surgery TI - Intermediate term results of infant orthotopic cardiac transplantation from two centers UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L21247386 VL - 101 ID - 1386 ER - TY - JOUR AD - Freeman Hospital, Newcastle Upon Tyne, United Kingdom Hospital for Sick Children, University of Toronto, Toronto, ON, Canada Stanford University, Stanford, CA, United States Columbia University Medical Center, New York, NY, United States Great Ormond Street Hospital, London, United Kingdom Joe DiMaggio Children's Hospital, Hollywood, FL, United States Primary Children's Medical Center, Salt Lake City, UT, United States C.S. Mott Children's Hospital, Ann Arbor, MI, United States Children's Hospital, Denver, CO, United States Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Ann and Robert H Lurie Children's Hospital, Chicago, IL, United States Children's Hospital of Philadelphia, Philadelphia, PA, United States Royal Melbourne Children's Hospital, Melbourne, VIC, Australia AU - Kirk, R. AU - Dipchand, A. I. AU - Rosenthal, D. N. AU - Addonizio, L. AU - Burch, M. AU - Chrisant, M. AU - Dubin, A. AU - Everitt, M. AU - Gajarski, R. AU - Mertens, L. AU - Miyamoto, S. AU - Morales, D. AU - Pahl, E. AU - Shaddy, R. AU - Towbin, J. AU - Weintraub, R. DB - Scopus DO - 10.1016/j.healun.2014.06.002 IS - 9 M3 - Article N1 - Cited By :100 Export Date: 15 June 2020 PY - 2014 SP - 888-909 ST - The International Society for Heart and Lung Transplantation Guidelines for the management of pediatric heart failure: Executive summary T2 - Journal of Heart and Lung Transplantation TI - The International Society for Heart and Lung Transplantation Guidelines for the management of pediatric heart failure: Executive summary UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84921842891&doi=10.1016%2fj.healun.2014.06.002&partnerID=40&md5=ea7a6ad0a22467b48d2d2c0a91b0b9ef VL - 33 ID - 2011 ER - TY - JOUR AB - BACKGROUND: The long-term impact of altered hemodynamics after stage II in children with hypoplastic left heart syndrome (HLHS) and hypoplastic left heart complex (HLHC) on cerebral growth and neurodevelopmental outcome is unknown. We aimed to investigate whether elevated central venous and atrial filling pressures before the Fontan procedure may be associated with smaller brain volumes and poorer neurodevelopmental outcome after the initial hybrid procedure. METHODS: In a two-center cohort study semiautomated segmentation of cerebral magnetic resonance imaging scans was conducted in 25 children with HLHS/HLHC (25 hybrid) before the Fontan procedure (27.6 ± 4.3 months) and in 8 healthy control subjects (29.7 ± 9.5 months). Study patients were evaluated with the Bayley Scales of Infant and Toddler Development III (Bayley-III) and a neurologic examination. Hemodynamic measures after stage II were assessed with cardiac catheterization at 2 years of age before Fontan completion. Children with known genetic comorbidities were excluded. RESULTS: In HLHS/HLHC patients higher atrial filling pressures (6 ± 3 mm Hg; range, 2-14) were correlated with reduced brain volumes and lower language composite score, whereas higher Glenn pressures (10 ± 3 mm Hg; range, 6-16) were related to higher cerebrospinal fluid, reduced brain volumes, and lower cognitive, language, and motoric composite scores in the Bayley-III. Compared with control subjects white matter volumes were reduced and cerebrospinal fluid volumes increased in study patients. CONCLUSIONS: These data suggest that altered cardiovascular hemodynamics after stage II influence brain growth and neurodevelopmental outcome in infants with HLHS/HLHC. AD - Pediatric Heart Center, University Hospital Giessen, Justus-Liebig-University, Giessen, Germany. Electronic address: bettina.reich@paediat.med.uni-giessen.de. Child Development Center, University Children's Hospital, Zurich, Switzerland; Division of Pediatric Cardiology, Pediatric Heart Center, University Children's Hospital, Zurich, Switzerland. Department of Diagnostic Imaging, MR-Center, University Children's Hospital, Zurich, Switzerland. Child Development Center, University Children's Hospital, Zurich, Switzerland. Child Development Center, SPZ Frankfurt Mitte, Frankfurt/Main, Germany. Department of Pediatric Neurology, University Hospital Giessen, Justus-Liebig-University, Giessen, Germany. Pediatric Heart Center, University Hospital Giessen, Justus-Liebig-University, Giessen, Germany. Division of Pediatric Cardiology, Pediatric Heart Center, University Children's Hospital, Zurich, Switzerland. Department of Surgery, Pediatric Cardiovascular Surgery, Pediatric Heart Center, University Children's Hospital, Zurich, Switzerland. AN - 30639363 AU - Reich, B. AU - Heye, K. N. AU - O'Gorman Tuura, R. AU - Beck, I. AU - Wetterling, K. AU - Hahn, A. AU - Aktintürk, H. AU - Schranz, D. AU - Jux, C. AU - Kretschmar, O. AU - Hübler, M. AU - Latal, B. AU - Knirsch, W. DA - Jun DB - PubMed DO - 10.1016/j.athoracsur.2018.12.012 DP - NLM ET - 2019/01/15 IS - 6 KW - Brain/growth & development/*pathology Child, Preschool Cohort Studies Female Hemodynamics/*physiology Humans Hypoplastic Left Heart Syndrome/*complications/*physiopathology Infant Infant, Newborn Male Neurodevelopmental Disorders/*etiology Organ Size LA - eng N1 - 1552-6259 Reich, Bettina Heye, Kristina N O'Gorman Tuura, Ruth Beck, Ingrid Wetterling, Kristina Hahn, Andreas Aktintürk, Hakan Schranz, Dietmar Jux, Christian Kretschmar, Oliver Hübler, Michael Latal, Beatrice Knirsch, Walter Journal Article Multicenter Study Research Support, Non-U.S. Gov't Netherlands Ann Thorac Surg. 2019 Jun;107(6):1838-1844. doi: 10.1016/j.athoracsur.2018.12.012. Epub 2019 Jan 9. PY - 2019 SN - 0003-4975 SP - 1838-1844 ST - Interrelationship Between Hemodynamics, Brain Volumes, and Outcome in Hypoplastic Left Heart Syndrome T2 - Ann Thorac Surg TI - Interrelationship Between Hemodynamics, Brain Volumes, and Outcome in Hypoplastic Left Heart Syndrome VL - 107 ID - 19 ER - TY - JOUR AB - Children born with hypoplastic left heart syndrome are at high risk for serious morbidity, growth failure, and mortality during the interstage period, which is the time from discharge home after first-stage hypoplastic left heart syndrome palliation until the second-stage surgical intervention. The single-ventricle circulatory physiology is complex, fragile, and potentially unstable. Multicenter initiatives have been successfully implemented to improve outcomes and optimize growth and survival during the interstage period. A crucial focus of care is the comprehensive family training in the use of home surveillance monitoring of oxygen saturation, enteral intake, weight, and the early recognition of "red flag" symptoms indicating potential cardiopulmonary or nutritional decompensation. Beginning with admission to the intensive care unit of the newborn with hypoplastic left heart syndrome, nurses provide critical care and education to prepare the family for interstage home care. This article presents detailed nursing guidelines for educating families on the home care of their medically fragile infant with single-ventricle circulation. AU - Nieves, J. A. AU - Uzark, K. AU - Rudd, N. A. AU - Strawn, J. AU - Schmelzer, A. AU - Dobrolet, N. DB - Medline DO - 10.4037/ccn2017763 IS - 2 KW - adult ambulatory monitoring caregiver education family female home care human hypoplastic left heart syndrome infant intensive care male middle aged newborn nurse attitude nursing organization and management practice guideline procedures retrospective study LA - English M3 - Article N1 - L619020156 2017-11-03 PY - 2017 SN - 1940-8250 SP - 72-88 ST - Interstage Home Monitoring After Newborn First-Stage Palliation for Hypoplastic Left Heart Syndrome: Family Education Strategies T2 - Critical care nurse TI - Interstage Home Monitoring After Newborn First-Stage Palliation for Hypoplastic Left Heart Syndrome: Family Education Strategies UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L619020156 http://dx.doi.org/10.4037/ccn2017763 VL - 37 ID - 763 ER - TY - JOUR AB - Thrombosis is a well-known and life-threatening complication of prosthetic mechanical valves. Therapy typically requires medical thrombolysis or surgical thrombectomy/valve replacement. We report the case of a thrombosed atrioventricular valve in a young boy with complex congenital heart disease, which was successfully treated with direct intra-atrial infusion of recombinant tissue plasminogen activator after failed attempts at systemic therapy. We present this treatment as an alternative to conventional medical therapy. © 2005 Wiley-Liss, Inc. AD - E.S. Siwik, RBC 380C, Pediatric Cardiology, 11100 Euclid Avenue, Cleveland, OH 44106, United States AU - Seltzer, S. M. AU - Reed, M. D. AU - Siwik, E. S. DB - Embase Medline DO - 10.1002/ccd.20557 IS - 1 KW - acetylsalicylic acid alteplase heparin politef tissue plasminogen activator warfarin anticoagulation artery graft article asplenia bleeding cardioversion case report fibrinolytic therapy fluoroscopy Fontan procedure great vessels transposition heart atrioventricular valve heart single ventricle heart valve prosthesis heart valve regurgitation hematoma hemodynamics human lung artery pressure lung vein drainage anomaly male mitral valve atresia patient compliance prothrombin time pulmonary valve stenosis school child thrombectomy thrombosis transesophageal echocardiography LA - English M3 - Article N1 - L43062619 2006-01-25 PY - 2006 SN - 1522-1946 SP - 139-141 ST - Intra-atrial tissue plasminogen activator infusion for prosthetic valve thrombosis T2 - Catheterization and Cardiovascular Interventions TI - Intra-atrial tissue plasminogen activator infusion for prosthetic valve thrombosis UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L43062619 http://dx.doi.org/10.1002/ccd.20557 VL - 67 ID - 1269 ER - TY - JOUR AB - RATIONALE: Patients with single ventricle physiology are at high risk of mortality resulting from ventricular dysfunction. The preliminary results of the phase 1 trial showed that cardiosphere-derived cells (CDCs) may be effective against congenital heart failure. OBJECTIVE: To determine whether intracoronary delivery of autologous CDCs improves cardiac function in patients with single ventricle physiology. METHODS AND RESULTS: We conducted a phase 2 randomized controlled study to assign in a 1:1 ratio 41 patients who had single ventricle physiology undergoing stage 2 or 3 palliation to receive intracoronary infusion of CDCs 4 to 9 weeks after surgery or staged reconstruction alone (study A). The primary outcome measure was to assess improvement in cardiac function at 3-month follow-up. Four months after palliation, controls had an alternative option to receive late CDC infusion on request (study B). Secondary outcomes included ventricular function, heart failure status, somatic growth, and health-related quality of life after a 12-month observation. At 3 months, the absolute changes in ventricular function were significantly greater in the CDC-treated group than in the controls (+6.4% [SD, 5.5] versus +1.3% [SD, 3.7]; P=0.003). In study B, a late CDC infusion in 17 controls increased the ventricular function at 3 months compared with that at baseline (38.8% [SD, 7.7] versus 34.8% [SD, 7.4]; P<0.0001). At 1 year, overall CDC infusion was associated with improved ventricular function (41.4% [SD, 6.6] versus 35.0% [SD, 8.2]; P<0.0001) and volumes (P<0.001), somatic growth (P<0.0001) with increased trophic factors production, such as insulin-like growth factor-1 and hepatocyte growth factor, and quality of life, along with a reduced heart failure status (P<0.0001) and cardiac fibrosis (P=0.014) relative to baseline. CONCLUSIONS: Intracoronary infusion of CDCs after staged palliation favorably affected cardiac function by reverse remodeling in patients with single ventricle physiology. This impact may improve heart failure status, somatic growth, and quality of life in patients and reduce parenting stress for their families. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifier: NCT01829750. AD - From the Departments of Cardiovascular Surgery (S.I., D.O., T.G., N.H., J.K., Y. Kuroko, Y. Kotani, S.A., S.K., S. Sano), Pediatrics (S.O., T.E., M.K., Y. Kurita, K.H., Y.F., K.B.), Anesthesiology and Resuscitology (T.I.), and Radiology (S. Sato), Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Japan; and Regenerative Medicine, Center for Innovative Clinical Medicine, Okayama University Hospital, Japan (H.O.). From the Departments of Cardiovascular Surgery (S.I., D.O., T.G., N.H., J.K., Y. Kuroko, Y. Kotani, S.A., S.K., S. Sano), Pediatrics (S.O., T.E., M.K., Y. Kurita, K.H., Y.F., K.B.), Anesthesiology and Resuscitology (T.I.), and Radiology (S. Sato), Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Japan; and Regenerative Medicine, Center for Innovative Clinical Medicine, Okayama University Hospital, Japan (H.O.). hidemasa@okayama-u.ac.jp. AN - 28052915 AU - Ishigami, S. AU - Ohtsuki, S. AU - Eitoku, T. AU - Ousaka, D. AU - Kondo, M. AU - Kurita, Y. AU - Hirai, K. AU - Fukushima, Y. AU - Baba, K. AU - Goto, T. AU - Horio, N. AU - Kobayashi, J. AU - Kuroko, Y. AU - Kotani, Y. AU - Arai, S. AU - Iwasaki, T. AU - Sato, S. AU - Kasahara, S. AU - Sano, S. AU - Oh, H. DA - Mar 31 DB - PubMed DO - 10.1161/circresaha.116.310253 DP - NLM ET - 2017/01/06 IS - 7 KW - Child, Preschool Coronary Vessels Female Humans Infant Infusions, Intra-Arterial/adverse effects/methods Male Myoblasts/cytology/*transplantation Myocytes, Cardiac/cytology/*transplantation Stem Cell Transplantation/*adverse effects/methods Ventricular Dysfunction/*therapy *cell therapy *heart disease *heart failure *hypoplastic left heart syndrome *stem cell LA - eng N1 - 1524-4571 Ishigami, Shuta Ohtsuki, Shinichi Eitoku, Takahiro Ousaka, Daiki Kondo, Maiko Kurita, Yoshihiko Hirai, Kenta Fukushima, Yosuke Baba, Kenji Goto, Takuya Horio, Naohiro Kobayashi, Junko Kuroko, Yosuke Kotani, Yasuhiro Arai, Sadahiko Iwasaki, Tatsuo Sato, Shuhei Kasahara, Shingo Sano, Shunji Oh, Hidemasa Clinical Trial, Phase II Journal Article Randomized Controlled Trial United States Circ Res. 2017 Mar 31;120(7):1162-1173. doi: 10.1161/CIRCRESAHA.116.310253. Epub 2017 Jan 4. PY - 2017 SN - 0009-7330 SP - 1162-1173 ST - Intracoronary Cardiac Progenitor Cells in Single Ventricle Physiology: The PERSEUS (Cardiac Progenitor Cell Infusion to Treat Univentricular Heart Disease) Randomized Phase 2 Trial T2 - Circ Res TI - Intracoronary Cardiac Progenitor Cells in Single Ventricle Physiology: The PERSEUS (Cardiac Progenitor Cell Infusion to Treat Univentricular Heart Disease) Randomized Phase 2 Trial VL - 120 ID - 44 ER - TY - JOUR AB - BACKGROUND: Inhalation of sulfur mustard (SM) and SM analog, 2-chloroethyl ethyl sulfide (CEES), cause fibrinous cast formation that occludes the conducting airways, similar to children with Fontan physiology-induced plastic bronchitis. These airway casts cause significant mortality and morbidity, including hypoxemia and respiratory distress. Our hypothesis was that intratracheal heparin, a highly cost effective and easily preserved rescue therapy, could reverse morbidity and mortality induced by bronchial cast formation. METHODS: Sprague-Dawley rats were exposed to 7.5% CEES via nose-only aerosol inhalation to produce extensive cast formation and mortality. The rats were distributed into three groups: non-treated, phosphate-buffered saline (PBS)-treated, and heparin-treated groups. Morbidity was assessed with oxygen saturations and clinical distress. Blood and bronchoalveolar lavage fluid (BALF) were obtained for analysis, and lungs were fixed for airway microdissection to quantify the extent of airway cast formation. RESULTS: Heparin, given intratracheally, improved survival (100%) when compared to non-treated (75%) and PBS-treated (90%) controls. Heparin-treated rats also had improved oxygen saturations, clinical distress and airway cast scores. Heparin-treated rats had increased thrombin clotting times, factor Xa inhibition and activated partial thromboplastin times, indicating systemic absorption of heparin. There were also increased red blood cells (RBCs) in the BALF in 2/6 heparin-treated rats compared to PBS-treated control rats. CONCLUSIONS: Intratracheal heparin 1 hr after CEES inhalation improved survival, oxygenation, airway obstruction, and clinical distress. There was systemic absorption of heparin in rats treated intratracheally. Some rats had increased RBCs in BALF, suggesting a potential for intrapulmonary bleeding if used chronically after SM inhalation. AD - Department of Pediatrics, University of Colorado Health Sciences Center, Aurora, Colorado. AN - 24692161 AU - Houin, P. R. AU - Veress, L. A. AU - Rancourt, R. C. AU - Hendry-Hofer, T. B. AU - Loader, J. E. AU - Rioux, J. S. AU - Garlick, R. B. AU - White, C. W. C2 - PMC4182164 C6 - NIHMS601609 DA - Feb DB - PubMed DO - 10.1002/ppul.23043 DP - NLM ET - 2014/04/03 IS - 2 KW - Animals Blood Coagulation Tests Bronchitis/*drug therapy Bronchoalveolar Lavage Fluid/cytology Chemical Warfare Agents/*toxicity Drug Administration Routes Erythrocytes/metabolism Fibrinolytic Agents/*administration & dosage Heparin/*administration & dosage Models, Animal Mustard Gas/*analogs & derivatives/toxicity Oxygen/blood Rats, Sprague-Dawley Trachea Cees heparin inhalation injury plastic bronchitis pulmonology sulfur mustard LA - eng N1 - 1099-0496 Houin, Paul R Veress, Livia A Rancourt, Raymond C Hendry-Hofer, Tara B Loader, Joan E Rioux, Jacqueline S Garlick, Rhonda B White, Carl W U54 ES015678/ES/NIEHS NIH HHS/United States Journal Article Pediatr Pulmonol. 2015 Feb;50(2):118-26. doi: 10.1002/ppul.23043. Epub 2014 Apr 1. PY - 2015 SN - 8755-6863 (Print) 1099-0496 SP - 118-26 ST - Intratracheal heparin improves plastic bronchitis due to sulfur mustard analog T2 - Pediatr Pulmonol TI - Intratracheal heparin improves plastic bronchitis due to sulfur mustard analog VL - 50 ID - 536 ER - TY - JOUR AB - To provide a viable bridge-to-transplant, bridge-to-recovery, or bridge-to-surgical reconstruction for patients with failing Fontan physiology, we are developing a collapsible, percutaneously inserted, magnetically levitated axial flow blood pump to support the cavopulmonary circulation in adolescent and adult patients. This unique blood pump will augment pressure and thus flow in the inferior vena cava through the lungs and ameliorate the poor hemodynamics associated with the univentricular circulation. Computational fluid dynamics analyses were performed to create the design of the impeller, the protective cage of filaments, and the set of diffuser blades for our axial flow blood pump. These analyses included the generation of pressure-flow characteristics, scalar stress estimations, and blood damage indexes. A quasi-steady analysis of the diffuser rotation was also completed and indicated an optimal diffuser rotational orientation of approximately 12 degrees. The numerical predictions of the pump performance demonstrated a pressure generation of 2-25 mm Hg for 1-7 L/min over 3000-8000 rpm. Scalar stress values were less than 200 Pa, and fluid residence times were found to be within acceptable ranges being less than 0.25 s. The maximum blood damage index was calculated to be 0.068%. These results support the continued design and development of this cavopulmonary assist device, building upon previous numerical work and experimental prototype testing. AD - Department of Mechanical Engineering, Virginia Commonwealth University, Richmond, VA 23284, USA. AN - 20021471 AU - Bhavsar, S. S. AU - Kapadia, J. Y. AU - Chopski, S. G. AU - Throckmorton, A. L. DA - Nov DB - PubMed DO - 10.1111/j.1525-1594.2009.00940.x DP - NLM ET - 2009/12/22 IS - 11 KW - Adolescent Adult Fontan Procedure/*instrumentation/methods *Heart-Assist Devices Hemodynamics Hemolysis Humans Prosthesis Design Young Adult LA - eng N1 - 1525-1594 Bhavsar, Sonya S Kapadia, Jugal Y Chopski, Steven G Throckmorton, Amy L Evaluation Study Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, Non-P.H.S. United States Artif Organs. 2009 Nov;33(11):977-87. doi: 10.1111/j.1525-1594.2009.00940.x. PY - 2009 SN - 0160-564x SP - 977-87 ST - Intravascular mechanical cavopulmonary assistance for patients with failing Fontan physiology T2 - Artif Organs TI - Intravascular mechanical cavopulmonary assistance for patients with failing Fontan physiology VL - 33 ID - 471 ER - TY - JOUR AB - Transesophageal echocardiography (TEE) has an important role during congenital heart surgery. TEE in small infants is associated with complications, including an inability to pass the TEE probe, esophageal trauma, airway compression, aortic compression, and interference with ventilation. Recently, a monoplane intravascular ultrasound catheter (IVUC) has been developed for intracardiac echocardiography. The efficacy of IVUC for transesophageal use has been shown in numerous animal studies and several human studies, but there have been few reports involving small infants using an IVUC probe. We examined 15 pediatric patients undergoing congenital cardiac surgery using an 8-Fr AcuNav IVUC probe. We checked the cardiac anatomy, cardiac function pre-operatively and de-aeration before weaning from CPB; the surgical repairs were evaluated post-operatively. Although the IVUC probe has limitations associated with the monoplane, we found the IVUC probe to be useful in small infants and safer than the TEE. Copyright © Korean Society of Anesthesiologists, 2010. AD - S.-H. Baek, Department of Anesthesiology and Pain Medicine, School of Medicine, Pusan National University, Beomeo-ri, Mulgeum-eup, Yangsan 626-770, South Korea AU - Kim, H. J. AU - Kim, J. Y. AU - Baek, S. H. AU - Kim, H. K. DB - Embase DO - 10.4097/kjae.2010.58.5.480 IS - 5 KW - fentanyl midazolam sevoflurane anesthesia induction anesthesiological procedure aortic arch interruption aortic coarctation arterial pressure article artificial ventilation blood pressure monitoring capnometry central venous pressure clinical article echocardiography electrocardiography Fallot tetralogy great vessels transposition heart atrium septum defect heart function heart right ventricle outflow tract heart surgery heart ventricle septum defect human hypoplastic left heart syndrome infant intravascular catheter intravascular ultrasound invasive procedure lung artery pressure lung blood flow maintenance therapy mechanical probe newborn Norwood procedure oximetry patient monitoring patient safety pediatric surgery postoperative period preoperative evaluation pressure gradient pulmonary artery pulmonary valve pulmonary valve disease pulse oximetry temperature measurement transesophageal echocardiography AcuNav LA - English M3 - Article N1 - L358977844 2010-06-17 2010-06-30 http://ekja.org/Synapse/Data/PDFData/0011KJAE/kjae-58-480.pdf PY - 2010 SN - 2005-6419 2005-7563 SP - 480-484 ST - Intravascular ultrasound catheter for transesophageal echocardiography in congenital heart surgery - A case report T2 - Korean Journal of Anesthesiology TI - Intravascular ultrasound catheter for transesophageal echocardiography in congenital heart surgery - A case report UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L358977844 http://dx.doi.org/10.4097/kjae.2010.58.5.480 VL - 58 ID - 1145 ER - TY - JOUR AB - Congenital heart disease is the most common birth defect in the United States, with an estimated frequency of approximately 12ĝ€"14 of 1000 live births per year. Neonates with congenital heart disease often need palliative or corrective surgery requiring cardiopulmonary bypass during the first weeks of life. The neonate undergoing cardiopulmonary bypass surgery experiences a more profound metabolic response to stress than that seen in older children and adults undergoing surgery. However, compared with older children and adults, the neonate has less metabolic reserves and is extremely vulnerable to the negative metabolic impact induced by stress, which can lead to suboptimal wound healing and growth failure. There are complications associated with the metabolic derangements of neonatal surgery requiring cardiopulmonary bypass, including but not limited to acute renal failure, chylothorax, and neurological dysfunction. This article discusses the importance of nutrition and metabolic support for the neonate undergoing cardiopulmonary bypass and the immediate postoperative nutrition needs of such a patient. Also, this article uses a case study to examine the feeding methodology used at one particular institution after neonatal cardiac surgery. The purpose of the case study is to provide an illustration of the many factors and obstacles that clinicians often face in the provision and timing of nutrition support. © 2009 American Society for Parenteral and Enteral Nutrition. AD - J. L. Owens, Medical College of Wisconsin, Pediatrics, Critical Care, 9000 W. Wisconsin Ave, Milwaukee, WI 53226 AU - Owens, J. L. AU - Musa, N. DB - Embase Medline DO - 10.1177/0884533609332086 IS - 2 KW - amino acid carbohydrate fat lipid protein acute kidney failure article body composition body weight cardiopulmonary bypass child nutrition chylothorax congenital heart disease energy expenditure enteric feeding food composition growth disorder human hypoplastic left heart syndrome intravenous feeding metabolic stress neurologic disease newborn newborn surgery nutritional support parenteral nutrition postoperative care wound healing impairment LA - English M3 - Article N1 - L354424883 2009-04-21 PY - 2009 SN - 0884-5336 SP - 242-249 ST - Invited Review: Nutrition support after neonatal cardiac surgery T2 - Nutrition in Clinical Practice TI - Invited Review: Nutrition support after neonatal cardiac surgery UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L354424883 http://dx.doi.org/10.1177/0884533609332086 VL - 24 ID - 1185 ER - TY - JOUR AB - Research that is closely connected with the population it is studying and in which the translational value to healthcare is high is a laudable goal, but it is not often achieved. The Australian and New Zealand Fontan Registry has developed a model for involving patients and parents of children with a Fontan circulation in its research. The model involves consumer participation in the overarching Steering Committee, and has set in place multiple channels of communication allowing the early dissemination of research findings before peer-reviewing, and consumer feedback at all levels of the research. Our focus was not only to provide information but also to give a voice to this community and include them as researchers. These communication channels are a part of a larger network involving the practitioner community, support groups, funding agencies, and health authorities. This close connection with the target research population has multiple benefits: safeguarding the project; controlling and adjusting both the messages conveyed and the investigations; building a community; raising new ideas for research; increasing our research participation rate; increasing the weight of our endeavours; and, above all, increasing our own satisfaction in our research undertakings. In conclusion, the interactions with patients and their families within Australia and New Zealand provide one potential model for the involvement of patients and parents that may result in research that is more relevant, focussed, and practically applicable in a healthcare setting. AD - Cardiac Surgery Department, Royal Children’s Hospital, Melbourne, Victoria, Australia Department of Pediatrics, Faculty of Medicine, The University of Melbourne, Melbourne, Victoria, Australia Murdoch Children’s Research Institute, Melbourne, Victoria, Australia Royal Children’s Hospital, Melbourne, Victoria, Australia Australian and New Zealand Fontan Registry Steering Committee Patient Representative, Victoria, Australia 1Cardiac Surgery Department,Royal Children's Hospital,Melbourne,Victoria,Australia AN - 128340403. Language: English. Entry Date: 20180524. Revision Date: 20190502. Publication Type: journal article AU - d’Udekem, Yves AU - Forsdick, Victoria AU - du Plessis, Karin AU - d'Udekem, Yves DB - ccm DO - 10.1017/S1047951117001494 DP - EBSCOhost IS - 4 KW - Heart Defects, Congenital -- Epidemiology Research, Medical Data Collection Decision Making Patient Satisfaction New Zealand Communication Australia Morbidity Child N1 - review. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. NLM UID: 9200019. PMID: NLM28814350. PY - 2018 SN - 1047-9511 SP - 517-521 ST - Involvement of patients and parents in research undertaken by the Australian and New Zealand Fontan Registry T2 - Cardiology in the Young TI - Involvement of patients and parents in research undertaken by the Australian and New Zealand Fontan Registry UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=128340403&site=ehost-live&scope=site VL - 28 ID - 1538 ER - TY - JOUR AB - BACKGROUND: Developing technology affords children with complex congenitally malformed hearts a chance for survival. Parents gratefully pursue life-saving options on behalf of their children, despite the risks to the life of their child, and uncertainty about outcomes. Little is known about how mothers and fathers experience parenting a child whose new state as a survivor may include less than optimal developmental sequels. METHOD: Our study involved multiple interactive interviews with 9 mothers and 7 fathers of infants and preschool children with hypoplastic left heart syndrome who had survived the Norwood surgical approach. Qualitative methodology included grounded theory methods of simultaneous collection and analysis of data, and we used open and selective coding of transcribed interviews. RESULTS: Parents used normalization in the context of uncertainty regarding the ongoing survival of their child. Parents described their underweight children as being on their own growth curve, and viewed their developmental progress, however delayed, as reason for celebration, as they had been prepared for their child to die. CONCLUSION: There is growing evidence that children with congenitally malformed hearts who require surgical intervention during the first year of life may experience developmental delay. The use of normalization by their parents may be effective in decreasing their worry regarding the uncertain future faced by their child, but may negatively affect the developmental progress of the child if they do not seek resources to assist development. Advice from paediatric specialists for parents to view their children as normal needs to be balanced with assistance for parents to access services to support optimal growth and development of their child. AD - Faculty of Nursing, University of Alberta, Edmonton, Alberta, Canada. gwen.rempel@ualberta.ca AN - 19272201 AU - Rempel, G. R. AU - Harrison, M. J. AU - Williamson, D. L. DA - Apr DB - PubMed DO - 10.1017/s1047951109003485 DP - NLM ET - 2009/03/11 IS - 2 KW - Adult Alberta/epidemiology Cardiac Surgical Procedures/*methods Child Development Child, Preschool Female Follow-Up Studies Humans Hypoplastic Left Heart Syndrome/mortality/*surgery Infant Informed Consent/*ethics/psychology Male Middle Aged *Parent-Child Relations Parents/*education Quality of Life Retrospective Studies Surveys and Questionnaires Survival Rate/trends Young Adult LA - eng N1 - 1467-1107 Rempel, Gwen R Harrison, Margaret J Williamson, Deanna L Comparative Study Journal Article Research Support, Non-U.S. Gov't England Cardiol Young. 2009 Apr;19(2):135-44. doi: 10.1017/S1047951109003485. Epub 2009 Mar 10. PY - 2009 SN - 1047-9511 SP - 135-44 ST - Is "treat your child normally" helpful advice for parents of survivors of treatment of hypoplastic left heart syndrome? T2 - Cardiol Young TI - Is "treat your child normally" helpful advice for parents of survivors of treatment of hypoplastic left heart syndrome? VL - 19 ID - 250 ER - TY - JOUR AB - OBJECTIVES: To determine whether a cardiac diagnosis is a predictor of neurodevelopmental outcomes after infant cardiac surgery. METHODS: Infants with ventricular septal defect (VSD), tetralogy of Fallot (TOF), transposition of the great arteries (TGA), and hypoplastic left heart syndrome (HLHS) in a study of apolipoprotein E (APOE) polymorphisms, and neurodevelopmental outcome underwent neurodevelopmental and genetic evaluation at 4 years of age. The domains tested included cognition, language, speech, memory, executive function, visual-motor, fine motor, and reading and math skills. RESULTS: Testing was completed in 178 patients with normal genetic evaluations: VSD (n = 26), TOF (n = 44), TGA (n = 41), and HLHS (n = 67). No differences were found in gestational age, ethnicity, APOE genotype, socioeconomic status, or maternal education among groups. Patient age at the first surgery was significantly lower for patients with TGA and HLHS compared with those with TOF and VSD. The postoperative length of stay was significantly longer for HLHS than all other groups and for TGA compared with TOF and VSD. HLHS correlated significantly with the use of deep hypothermic circulatory arrest and multiple operations. The mean scores for each domain were within normal limits for all groups. Compared with the other patients, those with HLHS had significantly lower scores for cognition, fine motor skills, executive function, and math skills. No significant differences were found among the TGA, TOF or VSD patients for any domain. Significant impairments in at least 1 domain were identified in 8% (2/25) of patients with VSD, 20% (8/41) with TOF, 17% (7/41) with TGA, and 18% (12/65) with HLHS. After correction for the demographic, preoperative, and operative variables, no significant differences were found among the groups for any domain. CONCLUSIONS: The mean scores for the neurodevelopmental outcomes domains tested were in the normal range for preschool children with no recognized genetic syndromes after surgery for VSD, TOF, TGA, and HLHS. In each diagnostic group, the number of children with impairments in at least 1 domain increased compared with the general population. Unadjusted neurodevelopmental outcomes for HLHS were lower for cognition, fine motor skills, executive function, and math skills compared with the other patients. After correction for the demographic, preoperative, and operative variables, no significant differences were found among the groups for any domain. The specific cardiac diagnosis determines a large portion of the variation in these covariates. Therefore, although HLHS did predict for poorer outcomes in some domains, it did not add predictive power to the other factors considered. AD - Division of Cardiothoracic Surgery, Children's Hospital of Philadelphia, Philadelphia, Pa 19104, USA. gaynor@email.chop.edu AN - 20951391 AU - Gaynor, J. W. AU - Gerdes, M. AU - Nord, A. S. AU - Bernbaum, J. AU - Zackai, E. AU - Wernovsky, G. AU - Clancy, R. R. AU - Heagerty, P. J. AU - Solot, C. B. AU - McDonald-McGinn, D. AU - Jarvik, G. P. C2 - PMC3278908 C6 - NIHMS353908 DA - Dec DB - PubMed DO - 10.1016/j.jtcvs.2010.07.069 DP - NLM ET - 2010/10/19 IS - 6 KW - Apolipoprotein E2/genetics *Child Development Child, Preschool Cognition Disorders/etiology/physiopathology Executive Function/physiology Female Heart Defects, Congenital/*diagnosis/*surgery Humans Hypothermia, Induced Infant Length of Stay/statistics & numerical data Male Motor Skills Nervous System Diseases/*etiology/physiopathology Neuropsychological Tests Predictive Value of Tests Prospective Studies Risk Factors Treatment Outcome LA - eng N1 - 1097-685x Gaynor, J William Gerdes, Marsha Nord, Alex S Bernbaum, Judy Zackai, Elaine Wernovsky, Gil Clancy, Robert R Heagerty, Patrick J Solot, Cynthia B McDonald-McGinn, Donna Jarvik, Gail P R01 HL071834/HL/NHLBI NIH HHS/United States R01 HL071834-01A1/HL/NHLBI NIH HHS/United States Journal Article J Thorac Cardiovasc Surg. 2010 Dec;140(6):1230-7. doi: 10.1016/j.jtcvs.2010.07.069. Epub 2010 Oct 15. PY - 2010 SN - 0022-5223 (Print) 0022-5223 SP - 1230-7 ST - Is cardiac diagnosis a predictor of neurodevelopmental outcome after cardiac surgery in infancy? T2 - J Thorac Cardiovasc Surg TI - Is cardiac diagnosis a predictor of neurodevelopmental outcome after cardiac surgery in infancy? VL - 140 ID - 533 ER - TY - JOUR AB - Background: Despite advances in pediatric cardiac surgery, perioperative myocardial injury can be the major determinant of postoperative dysfunction after cardiac surgery. This study investigated the pathology-related differences in 29 infants with congenital heart disease that led to death. The infants were treated at the University Hospital of Ribeirão Preto, Brazil. Methods: The patients were divided into four groups: Group 1, 16 infants who underwent operations for congenital heart disease on cardiopulmonary bypass; Group 2, four infants who underwent off-cardiopulmonary bypass operations for congenital heart disease; Group 3, nine infants who died from congenital heart disease prior to surgical treatment; and Group 4 (control group), five infants with no congenital heart disease and who died from other causes. The myocardial injuries and oxidative stress mechanisms were assessed by histopathology and immunohistochemistry and were quantified by morphometrical analyses. Results: Contraction band necrosis and dystrophic calcification were found primarily in infants of Group 1. Coagulation necrosis and healing were prominent in Group 2, while infants without repair (Group 3) showed mainly colliquative myocytolysis. Apoptotic cells were more prominent in the operative groups. The control group showed no significant myocardial lesions. Lipid peroxidation was the principal mechanism of oxidative stress accounting for the myocardial lesions. Conclusion: The diversity of the lesions observed in these hearts seemed to indicate a large spectrum of cell damage due to inadequate myocardial perfusion, especially when these infants underwent surgery. Oxidative mechanisms could be a common mediator in the pathogenesis of myocardial injuries, mediated by peroxidation of the membrane phospholipids and resulting in changes in the permeability of the cell membrane, cell death, and intracellular calcium overload. Furthermore, an immature and often hypertrophied myocardium may promote unfavorable conditions, leading to heart failure and a lethal outcome. © 2011 Elsevier Inc. All rights reserved. AD - S. G. Ramos, Department of Pathology, Faculty of Medicine of Ribeirão Preto, University of São Paulo, Av. Bandeirantes 3900, 14049-900 Ribeirão Preto, SP, Brazil AU - Oliveira, M. S. AU - Floriano, E. M. AU - Mazin, S. C. AU - Martinez, E. Z. AU - Vicente, W. V. A. AU - Peres, L. C. AU - Rossi, M. A. AU - Ramos, S. G. DB - Embase Medline DO - 10.1016/j.carpath.2010.01.012 IS - 1 KW - calcium membrane phospholipid anencephalus aortic arch interruption aortic coarctation aortic occlusion apoptosis article bicuspid aortic valve brain edema Brazil calcinosis calcium cell level cardiopulmonary bypass cell damage cell death cell membrane permeability child death clinical article congenital heart malformation controlled study cytolysis dextrocardia disease association encephalitis Fallot tetralogy female great vessels transposition heart atrium septum defect cardiac muscle cell heart muscle ischemia heart muscle perfusion heart right ventricle double outlet heart single ventricle heart surgery heart ventricle septum defect histopathology human human tissue hypoplastic left heart syndrome immunohistochemistry infant lipid peroxidation lung hypoplasia male mitral valve atresia morphometry newborn oxidative stress patent ductus arteriosus patent foramen ovale persistent left superior vena cava priority journal protein calorie malnutrition pulmonary valve atresia pulmonary valve stenosis sex difference wound healing LA - English M3 - Article N1 - L50808003 2010-03-05 2011-02-04 PY - 2011 SN - 1054-8807 SP - e43-e52 ST - Ischemic myocardial injuries after cardiac malformation repair in infants may be associated with oxidative stress mechanisms T2 - Cardiovascular Pathology TI - Ischemic myocardial injuries after cardiac malformation repair in infants may be associated with oxidative stress mechanisms UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50808003 http://dx.doi.org/10.1016/j.carpath.2010.01.012 VL - 20 ID - 1123 ER - TY - JOUR AB - Background: There are few specialist paediatric Chronic Fatigue Syndrome (CFS/ME) services in the UK. Therefore, the distance some families have to travel to reach these services can be a barrier to accessing evidence-based treatment. Videoconferencing technology such as Skype provides a means of delivering sessions remotely. This study aimed to explore the views of children and young people, their parents, and healthcare professionals of treatment delivered by videoconferencing in a specialist paediatric CFS/ME team. Method: To explore the experiences of the participants, a qualitative design was selected. Twelve young people (age 9–18), and 6 parents were interviewed about their experience of treatment sessions delivered via videoconferencing within a specialist CFS/ME service. A focus group explored the views of healthcare professionals (N = 9) from the service. Thematic analysis was used. Results: Three themes were identified from the data: “Challenges and concerns” “Benefits” and “Treatment provision”. Challenges and concerns that participants identified were; difficulties experienced with technology; a sense of a part of communication being lost with virtual connections; privacy issues with communicating online and feeling anxious on a screen. Participants felt that benefits of videoconferencing were; improving access to the chronic fatigue service; convenience and flexibility of treatment provision; a sense of being more open online and being in the comfort of their own home. In terms of treatment provision participants talked about videoconferencing as a part of a hierarchy of communication; the function of videoconferencing within the context of the chronic fatigue service; additional preparation needed to utilise videoconferencing and an assumption that videoconferencing is “part of young people's lives”. Conclusions: Although the experience of sessions provided by videoconferencing was different to sessions attended in person, participants tended to be positive about videoconferencing as an alternative means of accessing treatment, despite some barriers. Videoconferencing could be an additional option within an individualised care plan, but should not be an alternative to face to face support. AD - A. Haig-Ferguson, Royal United Hospital, Bath, United Kingdom AU - Haig-Ferguson, A. AU - Loades, M. AU - Whittle, C. AU - Read, R. AU - Higson-Sweeney, N. AU - Beasant, L. AU - Starbuck, J. AU - Crawley, E. DB - Embase DO - 10.1016/j.invent.2018.12.003 KW - adolescent adult article atriopulmonary connection behavior change child chronic fatigue syndrome clinical article clinical assessment clinical evaluation emotionality evaluation and follow up evidence based emergency medicine female geographic distribution health care delivery health care personnel health care planning health service human interview male mental health occupational therapy pediatrics physiotherapy priority journal privacy psychology qualitative analysis research school child social hierarchy technology teleconsultation thematic analysis training videoconferencing young adult LA - English M3 - Article N1 - L2001416921 2019-01-01 2019-01-11 PY - 2019 SN - 2214-7829 SP - 43-51 ST - “It's not one size fits all”; the use of videoconferencing for delivering therapy in a Specialist Paediatric Chronic Fatigue Service T2 - Internet Interventions TI - “It's not one size fits all”; the use of videoconferencing for delivering therapy in a Specialist Paediatric Chronic Fatigue Service UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2001416921 http://dx.doi.org/10.1016/j.invent.2018.12.003 VL - 15 ID - 623 ER - TY - JOUR AB - BACKGROUND: The purpose of this study was to assess deep hypothermic circulatory arrest (DHCA) as a modifier of neurodevelopmental (ND) outcomes in preschool children after cardiac surgery in infancy for repair of congenital heart defects (CHD). METHODS: This is a planned analysis of infants enrolled in a prospective study of apolipoprotein E polymorphisms and ND outcome after cardiac surgery. The effect of DHCA was assessed in patients with single or biventricular CHD without aortic arch obstruction. Neurodevelopmental assessment at 4 years of age included cognition, language, attention, impulsivity, executive function, social competence, and visual-motor and fine-motor skills. Patient and procedural variables were evaluated in univariate and multivariate models. RESULTS: Neurodevelopmental testing was completed in 238 of 307 eligible patients (78%). Deep hypothermic circulatory arrest was used at the discretion of the surgeon at least once in 92 infants (38.6%) with a median cumulative duration of 36 minutes (range, 1 to 132 minutes). By univariate analysis, DHCA patients were more likely to have single-ventricle CHD (p = 0.013), lower socioeconomic status (p < 0.001), a higher incidence of preoperative ventilation (p < 0.001), and were younger and smaller at the first surgery (p < 0.001). By multivariate analysis, use of DHCA was not predictive of worse performance for any ND outcome. CONCLUSIONS: In this cohort of children undergoing repair of CHD in infancy, patients who underwent DHCA had risk factors associated with worse ND outcomes. Despite these, use of DHCA for repair of single-ventricle and biventricular CHD without aortic arch obstruction was not predictive of worse performance for any ND domain tested at 4 years of age. AD - Division of Cardiothoracic Surgery, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA. fullers@email.chop.edu AN - 21095350 AU - Fuller, S. AU - Rajagopalan, R. AU - Jarvik, G. P. AU - Gerdes, M. AU - Bernbaum, J. AU - Wernovsky, G. AU - Clancy, R. R. AU - Solot, C. AU - Nicolson, S. C. AU - Spray, T. L. AU - Gaynor, J. W. C2 - PMC3297076 C6 - NIHMS353875 DA - Dec DB - PubMed DO - 10.1016/j.athoracsur.2010.08.005 DP - NLM ET - 2010/11/26 IS - 6 KW - Child Child, Preschool Circulatory Arrest, Deep Hypothermia Induced/*adverse effects Cognition/*physiology Developmental Disabilities/diagnosis/epidemiology/*etiology Female Follow-Up Studies Heart Defects, Congenital/*surgery Humans Incidence Infant Infant, Newborn Male Motor Activity/*physiology Postoperative Period Prognosis Prospective Studies Risk Factors Time Factors United States/epidemiology LA - eng N1 - 1552-6259 Fuller, Stephanie Rajagopalan, Ramakrishnan Jarvik, Gail P Gerdes, Marsha Bernbaum, Judy Wernovsky, Gil Clancy, Robert R Solot, Cynthia Nicolson, Susan C Spray, Thomas L Gaynor, J William R01 HL071834/HL/NHLBI NIH HHS/United States R01 HL071834-01A1/HL/NHLBI NIH HHS/United States Comparative Study Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't Ann Thorac Surg. 2010 Dec;90(6):1985-94; discussion 1994-5. doi: 10.1016/j.athoracsur.2010.08.005. PY - 2010 SN - 0003-4975 (Print) 0003-4975 SP - 1985-94; discussion 1994-5 ST - J. Maxwell Chamberlain Memorial Paper for congenital heart surgery. Deep hypothermic circulatory arrest does not impair neurodevelopmental outcome in school-age children after infant cardiac surgery T2 - Ann Thorac Surg TI - J. Maxwell Chamberlain Memorial Paper for congenital heart surgery. Deep hypothermic circulatory arrest does not impair neurodevelopmental outcome in school-age children after infant cardiac surgery VL - 90 ID - 393 ER - TY - JOUR AB - These 62 patients with the Kabuki make-up syndrome (KMS) were collected in a collaborative study among 33 institutions and analyzed clinically, cytogenetically, and epidemiologically to delineate the phenotypic spectrum of KMS and to learn about its cause. Among various manifestations observed, most patients had the following five cardinal manifestations: 1) a peculiar face (100%) characterized by eversion of the lower lateral eyelid; arched eyebrows, with sparse or dispersed lateral one-third; a depressed nasal tip; and prominent ears; 2) skeletal anomalies (92%), including brachydactyly V and a deformed spinal column, with or without sagittal cleft vertebrae; 3) dermatoglyphic abnormalities (93%), including increased digital ulnar loop and hypothenar loop patterns, absence of the digital triradius c and/or d, and presence of fingertip pads; 4) mild to moderate mental retardation (92%); and 5) postnatal growth deficiency (83%). Thus the core of the phenotypic spectrum of KMS is rather narrow and clearly defined. Many other inconsistent anomalies were observed. Important among them were early breast development in infant girls (23%), and congenital heart defects (31%), such as a single ventricle with a common atrium, ventricular septal defect, atrial septal defect, tetralogy of Fallot, coarctation of aorta, patent ductus arteriosus, aneurysm of aorta, transposition of great vessels, and right bundle branch block. Of the 62 KMS patients, 58 were Japanese, an indication that the syndrome is fairly common in Japan. It was estimated that its prevalence in Japanese newborn infants is 1/32,000. All the KMS cases in this study were sporadic, the sex ratio was even, there was no correlation with birth order, the consanguinity rate among the parents was not high, and no incriminated agent was found that was taken by the mothers during early pregnancy. Three of the 62 patients had a Y chromosome abnormality involving a possible common breakpoint (Yp11.2). This could indicate another possibility, i.e., that the KMS gene is on Yp11.2 and that the disease is pseudoautosomal dominant. These findings are compatible with an autosomal dominant disorder in which every patient represents a fresh mutation. The mutation rate was calculated at 15.6 X 10(6). AD - Department of Human Genetics, Nagasaki University School of Medicine, Japan. AN - 3067577 AU - Niikawa, N. AU - Kuroki, Y. AU - Kajii, T. AU - Matsuura, N. AU - Ishikiriyama, S. AU - Tonoki, H. AU - Ishikawa, N. AU - Yamada, Y. AU - Fujita, M. AU - Umemoto, H. AU - et al. DA - Nov DB - PubMed DO - 10.1002/ajmg.1320310312 DP - NLM ET - 1988/11/01 IS - 3 KW - *Abnormalities, Multiple Adolescent Adult Bone and Bones/*abnormalities Child Child, Preschool *Dermatoglyphics Facial Bones/abnormalities Female Growth Disorders/*genetics Humans Infant Intellectual Disability/*genetics Japan Karyotyping Male *Phenotype Syndrome LA - eng N1 - Niikawa, N Kuroki, Y Kajii, T Matsuura, N Ishikiriyama, S Tonoki, H Ishikawa, N Yamada, Y Fujita, M Umemoto, H Journal Article Review United States Am J Med Genet. 1988 Nov;31(3):565-89. doi: 10.1002/ajmg.1320310312. PY - 1988 SN - 0148-7299 (Print) 0148-7299 SP - 565-89 ST - Kabuki make-up (Niikawa-Kuroki) syndrome: a study of 62 patients T2 - Am J Med Genet TI - Kabuki make-up (Niikawa-Kuroki) syndrome: a study of 62 patients VL - 31 ID - 460 ER - TY - JOUR AB - Kabuki syndrome (MIM 147920) is a well-described, multiple congenital anomaly syndrome characterized by growth and developmental delay, cardiac, renal, and vertebral anomalies, as well as persistent fetal finger pads and distinct facial features. Facies are characterized by long palpebral fissures with eversion of lateral third of the lower eyelid, resembling the “Kabuki make-up” theatre genre after which the syndrome is named. Kabuki syndrome is estimated to affect 1/32,000 births, with 55–80% of patients showing nonsense or frameshift mutations in the KMT2D (MLL2) gene, which encodes a histone transferase located on chromosome 12q. Additionally, owing to the heterogeneous nature of Kabuki syndrome, a smaller number of diagnosed patients have been identified with mutations or deletions in KDM6A (a component of the same transcriptional complex as KMT2D) with no mutations in KMT2D, or as those diagnosed with Kabuki syndrome and without alterations in either KMT2D or KDM6A. Diagnosis of the syndrome in newborns and infants is difficult, as the facial features are not as evident as in toddler- or childhood. There are no known “tell-tale” signs of Kabuki syndrome prenatally, and there are no reports of common, specific findings in fetuses that might suggest the diagnosis. We present here two infants who presented with prenatal hydrops/ascites, who were subsequently diagnosed with Kabuki syndrome. Although relatively non-specific, we suggest that Kabuki syndrome be added to the list of genetic syndromes that are suspected in cases of prenatal hydrops, review the molecular etiology of Kabuki syndrome, and broaden the phenotype of this well-described disorder. © 2016 Wiley Periodicals, Inc. AD - S.A. Schrier Vergano, Division of Medical Genetics and Metabolism, Children's Hospital of The King's Daughters, Norfolk, VA, United States AU - Long, A. AU - Sinkovskaya, E. S. AU - Edmondson, A. C. AU - Zackai, E. AU - Schrier Vergano, S. A. DB - Embase Medline DO - 10.1002/ajmg.a.37956 IS - 12 KW - amino acid substitution amniocentesis article ascites case report cesarean section child female fetal biophysical profile fetus hydrops gene genetic association genetic variability gestational age human hypoplastic left heart syndrome Kabuki makeup syndrome KDM6A gene KMT2D gene lymphangiography male microarray analysis missense mutation molecular pathology newborn nuchal translucency measurement nuclear magnetic resonance imaging perinatal period phenotype physical examination pleura effusion premature fetus membrane rupture prenatal diagnosis preschool child priority journal single nucleotide polymorphism thoracocentesis LA - English M3 - Article N1 - L612907044 2016-10-28 2016-12-20 PY - 2016 SN - 1552-4833 1552-4825 SP - 3333-3337 ST - Kabuki syndrome as a cause of non-immune fetal hydrops/ascites T2 - American Journal of Medical Genetics, Part A TI - Kabuki syndrome as a cause of non-immune fetal hydrops/ascites UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L612907044 http://dx.doi.org/10.1002/ajmg.a.37956 VL - 170 ID - 785 ER - TY - JOUR AB - Music therapy for hospitalised newborn infants is an emerging clinical field. While a clear picture is being built in the literature about effective methods for direct work with infants, it is more difficult to inform the clinical reality, of providing services in a familv-centred practice model Beginning with a single case study, the authors engaged in length), discussion about the broader issues of providing effective clinical services to the families of hospitalised infants. The authors wrote their own narratives about working with families, using their practice wisdom as music therapists and their personal experiences of hospitalisation with family. These narratives' were combined and framed into categories. After a rest period of several months, these categories were revisited and repetitious material was deleted and overlapping material was' collapsed under major themes'. Finally the authors sought validation of the content from a colleague with 15 years experience working with families in hospital. The themes include.' The necessary character of the music therapist; music therapy is a triadic relationship: endurance -- the long journey; parents experience joy during music therapy; music therapy acknowledges the 'whole' developing child; the contingent relationship; a whole life. AD - Senior Music Therapist -- Neonate and Infant Program, Royal Children's Hospital, Melbourne AN - 105696855. Language: English. Entry Date: 20081121. Revision Date: 20150711. Publication Type: Journal Article AU - Shoemark, H. AU - Dearn, T. DB - ccm DP - EBSCOhost KW - Family Centered Care Intensive Care, Neonatal Music Therapy -- In Infancy and Childhood Female Hypoplastic Left Heart Syndrome Infant, Newborn Inpatients Intensive Care Units, Neonatal Narratives Parents -- Psychosocial Factors Support, Psychosocial N1 - case study; review. Commentary: Hanson-Abromeit D. Family centred music therapy: a commentary on Shoemark and Dearn's 2008 article. (AUST J MUSIC THER) 2008; 19: 25-26. Journal Subset: Alternative/Complementary Therapies; Australia & New Zealand; Blind Peer Reviewed; Double Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed. NLM UID: 9425712. PY - 2008 SN - 1036-9457 SP - 3-24 ST - Keeping parents at the centre of family centred music therapy with hospitalised infants T2 - Australian Journal of Music Therapy TI - Keeping parents at the centre of family centred music therapy with hospitalised infants UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105696855&site=ehost-live&scope=site VL - 19 ID - 1629 ER - TY - JOUR AB - National management guidelines recommend that patients with moderate and complex congenital heart disease (CHD) receive life-long cardiac care (LLCC), guided in adulthood by an adult congenital heart disease (ACHD) specialist. However, the percentage of adult CHD patients who receive such care is quite low. Inadequate knowledge regarding LLCC may contribute to care interruption. We, therefore, sought to determine the knowledge of adolescents and young adults regarding LLCC. In this multi-center study, we administered a survey to patients 13–20 years of age with surgically repaired congenital heart disease. We assessed the understanding of both their need for LLCC and awareness of the type of recommended care providers. A total of 290/302 (96%) patients approached in the outpatient clinic setting (10 centers) agreed to study participation; mean age was 16.3 ± 2.3 years; patients were 62% male. While the need for LLCC was recognized by 78% of subjects, only 37% understood that an ACHD specialist in adulthood should guide this care. Only 37% of respondents stated that their current cardiology team had spoken to them about LLCC, but 90% wished to learn more. A substantial number of adolescents and young adults with moderate and complex CHD lack adequate knowledge about LLCC, but most have a desire to learn more about the type of care they will require in adulthood. Transition education/assessment ensuring successful transfer to adult-oriented care for this population should emphasize the importance of LLCC. AD - S.M. Fernandes, Stanford University School of Medicine, 150 Governor’s Lane, T242, Stanford, CA, United States AU - Fernandes, S. M. AU - Verstappen, A. AU - Clair, M. AU - Rummell, M. AU - Barber, D. AU - Ackerman, K. AU - Dummer, K. AU - Mares, J. C. AU - Cannobio, M. M. AU - Reardon, L. C. AU - Long, J. AU - Crumb, S. AU - Bhatt, A. AU - Takahashi, M. AU - Khairy, P. AU - Williams, R. AU - Landzberg, M. J. AU - Moe, T. AU - Pearson, D. DB - Embase Medline DO - 10.1007/s00246-019-02154-8 IS - 7 KW - adolescent adult aortic coarctation arterial switch operation article attitude to illness awareness cardiologist Caucasian congenital heart disease controlled study cross-sectional study descriptive research Fallot tetralogy female Fontan procedure general adult cardiologist general practitioner great vessels transposition heart surgery Hispanic human major clinical study male multicenter study pediatric cardiologist sex difference terminal care young adult LA - English M3 - Article N1 - L2002354978 2019-08-26 2019-10-31 PY - 2019 SN - 1432-1971 0172-0643 SP - 1439-1444 ST - Knowledge of Life-Long Cardiac Care by Adolescents and Young Adults with Congenital Heart Disease T2 - Pediatric Cardiology TI - Knowledge of Life-Long Cardiac Care by Adolescents and Young Adults with Congenital Heart Disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2002354978 http://dx.doi.org/10.1007/s00246-019-02154-8 VL - 40 ID - 594 ER - TY - JOUR AB - BACKGROUND: Patients after the Fontan procedure are at risk for suboptimal functional health status, and associations with laboratory measures are important for planning interventions and outcome measures for clinical trials. METHODS AND RESULTS: Parents completed the generic Child Health Questionnaire for 511 Fontan Cross-Sectional Study patients 6 to 18 years of age (61% male). Associations of Child Health Questionnaire Physical and Psychosocial Functioning Summary Scores (FSS) with standardized measurements from prospective exercise testing, echocardiography, magnetic resonance imaging, and measurement of brain natriuretic peptide were determined by regression analyses. For exercise variables for maximal effort patients only, the final model showed that higher Physical FSS was associated only with higher maximum work rate, accounting for 9% of variation in Physical FSS. For echocardiography, lower Tei index (particularly for patients with extracardiac lateral tunnel connections), lower indexed end-systolic volume, and the absence of atrioventricular valve regurgitation for patients having Fontan procedure at age <2 years were associated with higher Physical FSS, accounting for 14% of variation in Physical FSS. For magnetic resonance imaging, ratio of lower mass to end-diastolic volume and midquartiles of indexed end-systolic volume (nonlinear) were associated with higher Physical FSS, accounting for 11% of variation. Lower brain natriuretic peptide was significantly but weakly associated with higher Physical FSS (1% of variation). Significant associations for Psychosocial FSS with laboratory measures were fewer and weaker than for Physical FSS. CONCLUSIONS: In relatively healthy Fontan patients, laboratory measures account for a small proportion of the variation in functional health status and therefore may not be optimal surrogate end points for trials of therapeutic interventions. AD - Hospital for Sick Children, 555 University Ave, Toronto, Ontario, Canada M5G 1X8. brian.mccrindle@sickkids.ca AN - 20026781 AU - McCrindle, B. W. AU - Zak, V. AU - Sleeper, L. A. AU - Paridon, S. M. AU - Colan, S. D. AU - Geva, T. AU - Mahony, L. AU - Li, J. S. AU - Breitbart, R. E. AU - Margossian, R. AU - Williams, R. V. AU - Gersony, W. M. AU - Atz, A. M. C2 - PMC2810546 C6 - NIHMS166828 DA - Jan 5 DB - PubMed DO - 10.1161/circulationaha.109.869396 DP - NLM ET - 2009/12/23 IS - 1 KW - Adolescent Cardiac Volume Child Cross-Sectional Studies Echocardiography Exercise Test Exercise Tolerance/*physiology Female *Fontan Procedure *Health Status Heart Defects, Congenital/*surgery Humans Magnetic Resonance Imaging Male Natriuretic Peptide, Brain/blood Postoperative Complications/diagnostic imaging/pathology/*physiopathology Surveys and Questionnaires Ventricular Dysfunction/diagnostic imaging/pathology/*physiopathology LA - eng N1 - 1524-4539 McCrindle, Brian W Zak, Victor Sleeper, Lynn A Paridon, Stephen M Colan, Steven D Geva, Tal Mahony, Lynn Li, Jennifer S Breitbart, Roger E Margossian, Renee Williams, Richard V Gersony, Welton M Atz, Andrew M Pediatric Heart Network Investigators U01 HL068288-06/HL/NHLBI NIH HHS/United States HL068288/HL/NHLBI NIH HHS/United States HL068285/HL/NHLBI NIH HHS/United States U01 HL068269/HL/NHLBI NIH HHS/United States U01 HL068279/HL/NHLBI NIH HHS/United States U01 HL068290/HL/NHLBI NIH HHS/United States HL068279/HL/NHLBI NIH HHS/United States U01 HL068288/HL/NHLBI NIH HHS/United States U10 HL068270/HL/NHLBI NIH HHS/United States U01 HL068281/HL/NHLBI NIH HHS/United States U01 HL068270/HL/NHLBI NIH HHS/United States HL068281/HL/NHLBI NIH HHS/United States U01 HL068292/HL/NHLBI NIH HHS/United States HL068269/HL/NHLBI NIH HHS/United States HL068270/HL/NHLBI NIH HHS/United States U10 HL109778/HL/NHLBI NIH HHS/United States U01 HL068288-01/HL/NHLBI NIH HHS/United States HL068290/HL/NHLBI NIH HHS/United States U01 HL068285/HL/NHLBI NIH HHS/United States HL068292/HL/NHLBI NIH HHS/United States Journal Article Research Support, N.I.H., Extramural Circulation. 2010 Jan 5;121(1):34-42. doi: 10.1161/CIRCULATIONAHA.109.869396. Epub 2009 Dec 21. PY - 2010 SN - 0009-7322 (Print) 0009-7322 SP - 34-42 ST - Laboratory measures of exercise capacity and ventricular characteristics and function are weakly associated with functional health status after Fontan procedure T2 - Circulation TI - Laboratory measures of exercise capacity and ventricular characteristics and function are weakly associated with functional health status after Fontan procedure VL - 121 ID - 280 ER - TY - JOUR AB - Children born with univentricular heart disease typically must undergo three open heart surgeries within the first 2-3 years of life to eventually establish the Fontan circulation. In that case the single working ventricle pumps oxygenated blood to the body and blood returns to the lungs flowing passively through the Total Cavopulmonary Connection (TCPC) rather than being actively pumped by a subpulmonary ventricle. The TCPC is a direct surgical connection between the superior and inferior vena cava and the left and right pulmonary arteries. We have postulated that a mechanical pump inserted into this circulation providing a 3-5 mmHg pressure augmentation will reestablish bi-ventricular physiology serving as a bridge-to-recovery, bridge-to-transplant or destination therapy as a "biventricular Fontan" circulation. The Viscous Impeller Pump (VIP) has been proposed by our group as such an assist device. It is situated in the center of the 4-way TCPC intersection and spins pulling blood from the vena cavae and pushing it into the pulmonary arteries. We hypothesized that Large Eddy Simulation (LES) using high-order numerical methods are needed to capture unsteady powered and unpowered Fontan hemodynamics. Inclusion of a mechanical pump into the CFD further complicates matters due to the need to account for rotating machinery. In this study, we focus on predictions from an in-house high-order LES code (WenoHemoTM) for unpowered and VIP-powered idealized TCPC hemodynamics with quantitative comparisons to Stereoscopic Particle Imaging Velocimetry (SPIV) measurements. Results are presented for both instantaneous flow structures and statistical data. Simulations show good qualitative and quantitative agreement with measured data. © 2012 Elsevier Ltd. AD - School of Mechanical Engineering, Purdue University, Lafayette, IN, United States Department of Surgery, Indiana University School of Medicine, Indianapolis, IN, United States AU - Delorme, Y. AU - Anupindi, K. AU - Kerlo, A. E. AU - Shetty, D. AU - Rodefeld, M. AU - Chen, J. AU - Frankel, S. DB - Scopus DO - 10.1016/j.jbiomech.2012.10.045 IS - 2 KW - Fontan circulation High order large eddy simulation Viscous impeller pump M3 - Article N1 - Cited By :20 Export Date: 15 June 2020 PY - 2013 SP - 408-422 ST - Large eddy simulation of powered Fontan hemodynamics T2 - Journal of Biomechanics TI - Large eddy simulation of powered Fontan hemodynamics UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84872609056&doi=10.1016%2fj.jbiomech.2012.10.045&partnerID=40&md5=7ea77125bfefae4c9e791e5c18a25d89 VL - 46 ID - 2065 ER - TY - JOUR AB - Congenital teratoma is a rare malformation, and few papers have been published about it. We present a large teratoma that arose from the hard palate in a neonate. The obstructive mass caused maternal polyhydramnios and was identified prenatally by ultrasonography. The mother went into labour at 35 week's gestation at home. The child was in respiratory distress as a result of airway obstruction, and a tracheostomy was done when she was 4 hours old. She also had major cardiac abnormalities. The palatal mass was removed successfully at 4 weeks of age. The typical components of a teratoma were identified including immature neural glial tissue. © 2008 The British Association of Oral and Maxillofacial Surgeons. AD - R.E. Benson, Oral and Maxillofacial Surgery, Leeds General Infirmary, United Kingdom AU - Benson, R. E. AU - Fabbroni, G. AU - Russell, J. L. DB - Embase Medline DO - 10.1016/j.bjoms.2007.12.015 IS - 1 KW - airway obstruction airway pressure article cardiovascular malformation case report computer assisted tomography female fetus echography gastrotomy gestational age glia hard palate heart single ventricle histopathology human human tissue hydramnios infant lung ventilation mouth tumor newborn nuclear magnetic resonance imaging patent ductus arteriosus physical examination postoperative care respiratory distress spontaneous placental delivery teratoma tracheostomy LA - English M3 - Article N1 - L50196538 2009-02-04 PY - 2009 SN - 0266-4356 SP - 46-49 ST - A large teratoma of the hard palate: a case report T2 - British Journal of Oral and Maxillofacial Surgery TI - A large teratoma of the hard palate: a case report UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50196538 http://dx.doi.org/10.1016/j.bjoms.2007.12.015 VL - 47 ID - 1192 ER - TY - JOUR AB - A child diagnosed with transposition of great arteries, multiple (Swiss cheese) ventricular septal defects, and a small right ventricle underwent pulmonary artery banding and patent ductus arteriosus ligation at the age of six months. At the age of three years bidirectional cavopulmonary shunt was performed as a first stage for univentricular repair. However, the patient was lost follow-up for four years, following which further evaluation showed that the right ventricle was reasonably adequate to support pulmonary circulation if the ventricular septal defects (VSDs) were closed using percutaneous techniques. Four VSDs were then closed using Amplatzer(R) devices (AGA Medical Corporation, Plymouth, MN, USA). At the age of eight years she underwent complex biventricular repair in the form of arterial switch, closure of atrial septal defect, take down of Glenn shunt, and reanastomosis of the distal end of the superior vena cava to the distal superior vena cava stump on the right atrium. One year later the patient is alive and well. In conclusion; biventricular repair may be considered before completion of Fontan whenever cardiac anatomy allows. AN - 105566427. Language: English. Entry Date: 20090109. Revision Date: 20150711. Publication Type: Journal Article AU - Qethamy, H. O. A. AU - Oakley, R. M. E. AU - Tageldin, M. M. AU - Abdulhamed, J. M. AU - Faraidi, Y. A. DB - ccm DP - EBSCOhost IS - 6 KW - Heart Diseases -- Familial and Genetic Heart Septal Defects -- Surgery Shunts, Surgical Surgery, Operative -- Methods Transposition of Great Arteries -- Surgery Angiography Echocardiography Equipment and Supplies Female Quality of Life Saudi Arabia N1 - case study; diagnostic images. Journal Subset: Biomedical; Peer Reviewed; USA. NLM UID: 8908809. PMID: NLM19016999. PY - 2008 SN - 0886-0440 SP - 719-721 ST - Late complex biventricular repair after bidirectional cavopulmonary shunt T2 - Journal of Cardiac Surgery TI - Late complex biventricular repair after bidirectional cavopulmonary shunt UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105566427&site=ehost-live&scope=site VL - 23 ID - 1628 ER - TY - JOUR AD - J. Rychik, Division of Cardiology, Cardiac Center, Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA, United States AU - Rychik, J. AU - Goldberg, D. J. DB - Embase Medline DO - 10.1161/CIRCULATIONAHA.114.005341 IS - 17 KW - acetylsalicylic acid alanine aminotransferase albumin alpha 1 antitrypsin aspartate aminotransferase budesonide plasma protein sildenafil abdominal radiography abdominal swelling adolescent alanine aminotransferase blood level albumin blood level article ascites aspartate aminotransferase blood level body height body weight cardiovascular magnetic resonance case report central venous pressure chronic vein insufficiency drug withdrawal echocardiography exercise feces analysis Fontan procedure forward heart failure fulminant hepatic failure gastrointestinal biopsy gastrointestinal endoscopy Glenn shunt heart catheterization heart output heart right ventricle pressure human hypoplastic left heart syndrome ileum inflammation liver biopsy liver fibrosis low drug dose lung artery pressure lung vascular resistance lymphangiectasis male physical activity priority journal protein blood level protein losing gastroenteropathy quality of life platelet count thromboembolism tricuspid valve regurgitation venous return LA - English M3 - Article N1 - L603859496 2015-04-23 2015-05-06 PY - 2014 SN - 1524-4539 0009-7322 SP - 1525-1528 ST - Late consequences of the fontan operation T2 - Circulation TI - Late consequences of the fontan operation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L603859496 http://dx.doi.org/10.1161/CIRCULATIONAHA.114.005341 VL - 130 ID - 975 ER - TY - JOUR AB - PURPOSE OF REVIEW: It has been more than 4 decades since the development of Fontan operation. With the contemporary surgical strategies to treat the patients with a single ventricle physiology, the medium-term survival of the patients following Fontan operation is excellent. Nonetheless, the Fontan circulation with a pumpless pulmonary circulation has fundamental physiologic limitations, which are associated with late Fontan failure and deaths. There has been an exponential growth of adolescence and adults living with a Fontan circulation, which poses significant challenges in future. RECENT FINDINGS: In this review, we discuss challenges and potential opportunities to treat the failing Fontan circulation. The specific topics include medical therapy, imaging, and therapeutic interventions for lymphatic abnormalities, transplantation, and mechanical support. The article also summarizes quality of life among the patients with the Fontan circulation. SUMMARY: Surgical techniques, developing novel diagnostic and therapeutic tools, and increasing our understanding of the failing Fontan physiology is essential to improve the overall long-term outcome of this entity. AD - Division of Cardiovascular Surgery, Labatt Family Heart Centre, The Hospital for Sick Children. Department of Surgery, University of Toronto, Toronto, Ontario, Canada. AN - 30575650 AU - Luo, S. AU - Honjo, O. DA - Mar DB - PubMed DO - 10.1097/hco.0000000000000603 DP - NLM ET - 2018/12/24 IS - 2 KW - Adolescent Adult *Fontan Procedure/mortality *Heart Defects, Congenital/surgery Humans Pulmonary Circulation Quality of Life Time Factors LA - eng N1 - 1531-7080 Luo, Shuhua Honjo, Osami Journal Article Review United States Curr Opin Cardiol. 2019 Mar;34(2):156-163. doi: 10.1097/HCO.0000000000000603. PY - 2019 SN - 0268-4705 SP - 156-163 ST - Late deaths after Fontan procedure: the next frontier T2 - Curr Opin Cardiol TI - Late deaths after Fontan procedure: the next frontier VL - 34 ID - 86 ER - TY - JOUR AB - BACKGROUND: Pulmonary ventricle (PV) to pulmonary artery (PA) conduits have made possible the correction of many complex congenital cardiac anomalies. METHODS: Between April 1964 and January 2001, 1270 patients underwent operation with conduit placement from the PV to PA. The present study evaluates late outcome of 1095 patients (612 males, 483 females) having an operation before July 1992. Mean age was 9.6 +/- 8.2 years old. Diagnoses included pulmonary atresia/tetralogy of Fallot (459), transposition of the great arteries (TGA) (232), truncus arteriosus (193), double outlet right ventricle (DORV) (121), corrected TGA (49), septated univentricular heart (36), and other (5). A porcine-valved Dacron conduit was used in 730, homograft in 239, and non-valved conduit in 126. RESULTS: Early mortality decreased from 23.5% prior to 1980 to 3.7% for the most recent decade. Mean follow-up was 10.9 years (maximum, 29 years). Actuarial survival for early survivors at 10 and 20 years was 77.0% +/- 1.5% and 59.5% +/- 2.6%. On univariate analysis, clinical and hemodynamic factors associated with late mortality were male gender, older age at operation, higher post-repair PV/systemic ventricle (SV) pressure ratio, higher distal PA pressure, and longer bypass time (p < or = 0.01 for all). On multivariate analysis, independent risk factors for late mortality were male gender, older age at operation, diagnosis of TGA, corrected TGA, truncus, or univentricular heart, and PV/SV pressure ratio > or = 0.72 (p < or = 0.03 for all). Freedom from reoperation for conduit failure at 10 and 20 years was 55.5% +/- 2.0% and 31.9% +/- 2.7%. On multivariate analysis, independent risk factors for conduit failure were homograft conduit, diagnosis of TGA, younger age at operation, and smaller conduit size (p < or = 0.007 for all). Reoperation for one conduit replacement was performed in 306 patients, two conduit replacements in 55 patients, three in 6 patients, and four in 3 patients. Overall early mortality for conduit replacement in this series was 4.9%; it was 1.7% for patients operated on from 1989 through 1992. At follow-up, 84% of survivors were in NYHA class I or II. CONCLUSIONS: Operations that include conduit placement and replacement can be performed with low early mortality. Younger age at operation was associated with improved late survival. The diagnosis of TGA was associated with increased risk for conduit failure, and the durability of the homograft, in this series, was inferior to the porcine-valved Dacron conduit. Quality of life was excellent for most patients despite the need for reoperation. AD - Division of Cardiovascular Surgery, Mayo Clinic and Mayo Foundation, Rochester, Minnesota 55905, USA. jdearani@mayo.edu AN - 12607647 AU - Dearani, J. A. AU - Danielson, G. K. AU - Puga, F. J. AU - Schaff, H. V. AU - Warnes, C. W. AU - Driscoll, D. J. AU - Schleck, C. D. AU - Ilstrup, D. M. DA - Feb DB - PubMed DO - 10.1016/s0003-4975(02)04547-2 DP - NLM ET - 2003/02/28 IS - 2 KW - Adolescent Adult Blood Vessel Prosthesis *Blood Vessel Prosthesis Implantation Cardiac Surgical Procedures Cardiopulmonary Bypass Child Child, Preschool Female Follow-Up Studies Heart Defects, Congenital/mortality/*surgery Humans Infant Infant, Newborn Male Middle Aged Prosthesis Design Risk Factors Survival Analysis LA - eng N1 - Dearani, Joseph A Danielson, Gordon K Puga, Francisco J Schaff, Hartzell V Warnes, Carole W Driscoll, David J Schleck, Cathy D Ilstrup, Duane M Journal Article Netherlands Ann Thorac Surg. 2003 Feb;75(2):399-410; discussion 410-1. doi: 10.1016/s0003-4975(02)04547-2. PY - 2003 SN - 0003-4975 (Print) 0003-4975 SP - 399-410; discussion 410-1 ST - Late follow-up of 1095 patients undergoing operation for complex congenital heart disease utilizing pulmonary ventricle to pulmonary artery conduits T2 - Ann Thorac Surg TI - Late follow-up of 1095 patients undergoing operation for complex congenital heart disease utilizing pulmonary ventricle to pulmonary artery conduits VL - 75 ID - 445 ER - TY - JOUR AB - Background. Pulmonary ventricle (PV) to pulmonary artery (PA) conduits have made possible the correction of many complex congenital cardiac anomalies. Methods. Between April 1964 and January 2001, 1270 patients underwent operation with conduit placement from the PV to PA. The present study evaluates late outcome of 1095 patients (612 males, 483 females) having an operation before July 1992. Mean age was 9.6 ± 8.2 years old. Diagnoses included pulmonary atresia/tetralogy of Fallot (459), transposition of the great arteries (TGA) (232), truncus arteriosus (193), double outlet right ventricle (DORV) (121), corrected TGA (49), septated univentricular heart (36), and other (5). A porcine-valved Dacron conduit was used in 730, homograft in 239, and non-valved conduit in 126. Results. Early mortality decreased from 23.5% prior to 1980 to 3.7% for the most recent decade. Mean follow-up was 10.9 years (maximum, 29 years). Actuarial survival for early survivors at 10 and 20 years was 77.0% ± 1.5% and 59.5% ± 2.6%. On univariate analysis, clinical and hemodynamic factors associated with late mortality were male gender, older age at operation, higher post-repair PV/systemic ventricle (SV) pressure ratio, higher distal PA pressure, and longer bypass time (p ≤ 0.01 for all). On multivariate analysis, independent risk factors for late mortality were male gender, older age at operation, diagnosis of TGA, corrected TGA, truncus, or univentricular heart, and PV/SV pressure ratio ≥ 0.72 (p ≤ 0.03 for all). Freedom from reoperation for conduit failure at 10 and 20 years was 55.5% ± 2.0% and 31.9% ≤ 2.7%. On multivariate analysis, independent risk factors for conduit failure were homograft conduit, diagnosis of TGA, younger age at operation, and smaller conduit size (p ≤ 0.007 for all). Reoperation for one conduit replacement was performed in 306 patients, two conduit replacements in 55 patients, three in 6 patients, and four in 3 patients. Overall early mortality for conduit replacement in this series was 4.9%; it was 1.7% for patients operated on from 1989 through 1992. At follow-up, 84% of survivors were in NYHA class I or II. Conclusions. Operations that include conduit placement and replacement can be performed with low early mortality. Younger age at operation was associated with improved late survival. The diagnosis of TGA was associated with increased risk for conduit failure, and the durability of the homograft, in this series, was inferior to the porcine-valved Dacron conduit. Quality of life was excellent for most patients despite the need for reoperation. © 2003 by The Society of Thoracic Surgeons. AD - J.A. Dearani, Mayo Clinic, 200 First St, SW, Rochester, MN 55905, United States AU - Dearani, J. A. AU - Danielson, G. K. AU - Puga, F. J. AU - Schaff, H. V. AU - Warnes, C. W. AU - Driscoll, D. J. AU - Schleck, C. D. AU - Ilstrup, D. M. AU - Clarke, D. R. AU - Brown, J. W. DB - Embase Medline DO - 10.1016/S0003-4975(02)04547-2 IS - 2 KW - adolescent adult aortopulmonary septal defect article Blalock Taussig shunt cardiovascular surgery child clinical article congenital heart disease Fallot tetralogy female follow up great vessels transposition heart right ventricle double outlet human infant lung artery pulmonary valve atresia male priority journal survival rate treatment outcome LA - English M3 - Article N1 - L36184480 2003-02-20 PY - 2003 SN - 0003-4975 SP - 399-411 ST - Late follow-up of 1095 patients undergoing operation for complex congenital heart disease utilizing pulmonary ventricle to pulmonary artery conduits T2 - Annals of Thoracic Surgery TI - Late follow-up of 1095 patients undergoing operation for complex congenital heart disease utilizing pulmonary ventricle to pulmonary artery conduits UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L36184480 http://dx.doi.org/10.1016/S0003-4975(02)04547-2 VL - 75 ID - 1307 ER - TY - JOUR AB - We report on a series of 12 patients with complex congenital heart disease who had reached adult life after surgical palliation in early childhood and who were systematically followed by clinical visits. Patients were born between 1953 and 1979 and were followed up into 2007. All patients had complex lesions with single ventricle physiology and were palliated either by banding of the pulmonary artery or by creation of systemic-pulmonary shunts. Single operations were performed in 4 cases, and 8 patients had 1 or more further palliative interventions. Late corrective surgery and heart transplantation were performed in 1 patient each. Eight survivors reached a mean age of 36 years at follow-up (range 28-48), whereas 4 patients died at a mean age of 32 years (range 22-53). All patients were in New York Heart Association classes II and III. Complications during follow-up were bacterial endocarditis (3), cerebrovascular accidents (3), arrhythmias (3), need for pacemakers (2). Deaths occurred perioperatively at transplant (1), or were caused by sudden death (2), and hypoxia related to fall in blood pressure (1). Successful pregnancies occurred in 3 patients with healthy (small) babies delivered by cesarean section in 2 of them, and vaginal delivery in 1. The course of life in these patients shows not only a wide spectrum with good quality of life in some of them but also a large number of potentially severe complications. Survival beyond 50 years is rare. AD - University Hospital, Division of Cardiology, Basel, Switzerland. andreas.hoffmann@unibas.ch AN - 18380768 AU - Hoffmann, A. AU - Wyler, F. AU - Günthard, J. AU - Grädel, E. DA - Mar-Apr DB - PubMed DO - 10.1111/j.1747-0803.2007.00166.x DP - NLM ET - 2008/04/03 IS - 2 KW - Adult Cardiovascular Surgical Procedures/*mortality Cause of Death Female Follow-Up Studies Heart Defects, Congenital/*mortality/*surgery Humans Life Expectancy Male Middle Aged *Palliative Care Postoperative Complications LA - eng N1 - 1747-0803 Hoffmann, Andreas Wyler, Felix Günthard, Joelle Grädel, Erich Journal Article United States Congenit Heart Dis. 2008 Mar-Apr;3(2):155-8. doi: 10.1111/j.1747-0803.2007.00166.x. PY - 2008 SN - 1747-079x SP - 155-8 ST - Late follow-up of patients who underwent palliation for complex congenital heart disease in childhood T2 - Congenit Heart Dis TI - Late follow-up of patients who underwent palliation for complex congenital heart disease in childhood VL - 3 ID - 541 ER - TY - JOUR AB - Optimizing late outcomes should be the end result of improvements in medical and surgical care for congenital heart disease (CHD). In addition to mortality, significant morbidities after surgery for CHD need to be considered. These include the need for reintervention, cardiovascular complications, exercise limitations, neurocognitive morbidities, effects on pregnancy, difficulty obtaining insurance, need for chronic medications, and impaired functional status and quality of life. Long-term outcome studies are difficult to perform, and their interpretation is complicated by intervening changes in management. Specific discussion of long-term follow-up of tetralogy of Fallot, D-transposition of the great arteries, and hypoplastic left heart syndrome illustrates the myriad management changes over the last three decades, the challenges in predicting outcomes for recent patients, and the need for ongoing initiation of long-term follow-up studies. © 2005 Elsevier Inc. All rights reserved. AD - A.H. Schultz, Division of Cardiology, Children's Hospital of Philadelphia, 34th and Civic Center Blvd., Philadelphia, PA 18104, United States AU - Schultz, A. H. AU - Wernovsky, G. DB - Embase DO - 10.1053/j.pcsu.2005.01.015 IS - 1 KW - article cardiovascular disease child cognitive defect congenital heart disease endocarditis exercise Fallot tetralogy follow up great vessels transposition health insurance health status heart arrhythmia heart failure heart surgery heart transplantation heart ventricle failure human hypoplasia medical care morbidity prediction pregnancy quality of life reoperation surgical mortality treatment outcome LA - English M3 - Article N1 - L40468465 2005-04-20 PY - 2005 SN - 1092-9126 SP - 145-156 ST - Late outcomes in patients with surgically treated congenital heart disease T2 - Pediatric Cardiac Surgery Annual TI - Late outcomes in patients with surgically treated congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L40468465 http://dx.doi.org/10.1053/j.pcsu.2005.01.015 VL - 8 ID - 1280 ER - TY - JOUR AB - Left ventricular function was evaluated by radionuclide angiocardiography in patients with tricuspid atresia before and after the Fontan operation. Ejection fraction was 0.54 +/- 0.13 in 29 patients without the Fontan procedure and 0.56 +/- 0.14 in 14 postoperative patients. Group means were not statistically different, but both were less than normal values for the institution (p less than 0.001). Function did not correlate significantly with age or aortic oxygen saturation. Serial studies demonstrated little change in eight of nine patients examined over 1 to 3 years preoperatively. From the preoperative group of nine patients with abnormal ejection fraction, three underwent surgery, with two survivors. Although 5 of 14 patients had abnormal systolic function after the Fontan operation, only 1 was symptomatic. Depressed response to isometric exercise was found in two patients with a normal ejection fraction at rest. Group systolic function after Fontan surgery did not correlate significantly with age at surgery but all patients were operated on at more than 5 years of age. Of seven patients studied both preoperatively and postoperatively two had an increase and three a decrease in ejection fraction. The trend in these seven studied serially after surgery was toward early improvement in ejection fraction. This study shows variability in left ventricular performance in patients with tricuspid atresia before and after surgery. Individuals undergoing the Fontan procedure after 5 years of age do not necessarily have an increase in ejection fraction over preoperative values, and may even have a decrease. Serial improvement over early postoperative results may be expected, without significant deterioration, during the next 1 to 3 years. AN - 3760364 AU - Hurwitz, R. A. AU - Caldwell, R. L. AU - Girod, D. A. AU - Wellman, H. DA - Oct DB - PubMed DO - 10.1016/s0735-1097(86)80435-1 DP - NLM ET - 1986/10/01 IS - 4 KW - Adolescent Adult Child Child, Preschool Humans Infant Isometric Contraction *Myocardial Contraction Radionuclide Angiography *Stroke Volume Tricuspid Valve/*abnormalities/surgery LA - eng N1 - Hurwitz, R A Caldwell, R L Girod, D A Wellman, H Journal Article United States J Am Coll Cardiol. 1986 Oct;8(4):916-21. doi: 10.1016/s0735-1097(86)80435-1. PY - 1986 SN - 0735-1097 (Print) 0735-1097 SP - 916-21 ST - Left ventricular function in tricuspid atresia: a radionuclide study T2 - J Am Coll Cardiol TI - Left ventricular function in tricuspid atresia: a radionuclide study VL - 8 ID - 477 ER - TY - JOUR AB - BACKGROUND: Within the spectrum of congenital heart disease referred to as hypoplastic left heart syndrome (HLHS), there is variation in the morphology and function of the left ventricle which could influence outcomes after stage I Norwood palliation. OBJECTIVE: To determine if left ventricular (LV) morphology is associated with outcome after stage I Norwood palliation for HLHS. METHODS: Echocardiograms were reviewed from 108 patients who had undergone Norwood palliation at our institution over the past 11 years. Total cardiac diameter, thickness of the interventricular septum (IVS), LV area and LV myocardial area were calculated. Competing risk analysis was performed for survival to a stage II operation and to determine potential predictors. RESULTS: From the Norwood operation up to stage II operation, mortality was predicted by IVS thickness, while the absence of right ventricular (RV) dysfunction was predictive of survival to stage II operation. For the complete pathway, from Norwood to the Fontan operation, mortality was predicted by IVS, a lower RV fractional area change and the presence of significant tricuspid regurgitation. Cardiac transplantation during this period was predicted by a lower RV fractional area change (p = 0.02) and a larger LV area in diastole. CONCLUSIONS: These results indicate that LV hypertrophy and decreased RV function adversely effect survival after the Norwood operation. They suggest that LV morphology, especially septal hypertrophy, can influence outcomes in HLHS and should be considered when evaluating treatment options. AD - Division of Cardiac Critical Care Medicine, Hospital for Sick Children, 555 University Ave, Toronto, ON M5G 1X8, Canada. AN - 105384416. Language: English. Entry Date: 20090828. Revision Date: 20150711. Publication Type: Journal Article AU - Walsh, M. A. AU - McCrindle, B. W. AU - Dipchand, A. AU - Manlhiot, C. AU - Hickey, E. AU - Caldarone, C. A. AU - Van Arsdell, G. S. AU - Schwartz, S. M. DB - ccm DO - 10.1136/hrt.2008.156612 DP - EBSCOhost IS - 15 KW - Hypoplastic Left Heart Syndrome -- Mortality Hypoplastic Left Heart Syndrome -- Surgery Female Heart Transplantation -- Mortality Heart Transplantation -- Statistics and Numerical Data Heart Ventricle -- Pathology Hypoplastic Left Heart Syndrome -- Pathology Infant Infant, Newborn Male Quality of Life Retrospective Design Risk Assessment Risk Factors Survival Analysis Human N1 - research. Journal Subset: Biomedical; Blind Peer Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. NLM UID: 9602087. PMID: NLM19457871. PY - 2009 SN - 1355-6037 SP - 1238-1244 ST - Left ventricular morphology influences mortality after the Norwood operation T2 - Heart TI - Left ventricular morphology influences mortality after the Norwood operation UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105384416&site=ehost-live&scope=site VL - 95 ID - 1613 ER - TY - JOUR AB - Fetal aortic valvuloplasty (FAV) has shown promise in averting the progression of fetal aortic stenosis to hypoplastic left-heart syndrome. Altered loading conditions due to valvar disease, intrinsic endomyocardial abnormalities, and procedures that alter endomyocardial mechanics may place patients with biventricular circulation (BiV) after FAV at risk of abnormal LV remodeling and function. Using the most recent echo data on BiV patients after technically successful FAV (n = 34), we evaluated LV remodeling pattern, risk factors for pathologic LV remodeling, and the association between LV remodeling pattern and LV function. Median age at follow-up was 4.7 years (range 1.0-12.5). Cardiac interventions were common. At latest follow-up, no patient had hypoplastic LV. Nineteen patients (55 %) had dilated LV, and five (16 %) patients had severely dilated LV. LV remodeling patterns were as follows: 12 (35 %) normal ventricle, 11 (32 %) mixed hypertrophy, 8 (24 %) eccentric hypertrophy or remodeling, and 3 (9 %) concentric hypertrophy. Univariate factors associated with pathologic LV remodeling were long-standing AR, ≥2 cardiac interventions, EFE resection, and aortic or mitral regurgitation ≥ moderate at most recent follow-up. In multivariate analysis, only long-standing AR fraction remained associated with pathologic remodeling. Pathologic LV remodeling was associated with depressed ejection fraction, lower septal E´, and higher E/E´. Pathologic LV remodeling, primarily eccentric or mixed hypertrophy, is common in BiV patients after FAV and is related to LV loading conditions imposed by valvar disease. Pathologic remodeling is associated with both systolic and diastolic dysfunction in this population. AD - Department of Cardiology, Children's Hospital Boston, Boston, MA, 02115, USA. Kevin.Friedman@cardio.chboston.org. Department of Pediatrics, Harvard Medical School, Boston, MA, USA. Kevin.Friedman@cardio.chboston.org. Department of Cardiology, Children's Hospital Boston, Boston, MA, 02115, USA. Department of Pediatrics, Harvard Medical School, Boston, MA, USA. AN - 25972285 AU - Friedman, K. G. AU - Freud, L. AU - Escobar-Diaz, M. AU - Banka, P. AU - Emani, S. AU - Tworetzky, W. C2 - PMC7001762 C6 - NIHMS1065503 DA - Oct DB - PubMed DO - 10.1007/s00246-015-1193-6 DP - NLM ET - 2015/05/15 IS - 7 KW - Aortic Valve/diagnostic imaging Aortic Valve Stenosis/*diagnostic imaging Cardiac Surgical Procedures/*adverse effects Child Child, Preschool Cohort Studies Diastole/physiology Echocardiography, Doppler Female Heart Ventricles/physiopathology Humans Hypoplastic Left Heart Syndrome/*surgery Infant Kaplan-Meier Estimate Male Postoperative Complications/*diagnostic imaging Ventricular Dysfunction, Left/*diagnostic imaging *Ventricular Remodeling Aortic stenosis Diastolic dysfunction Fetal cardiology Left ventricular remodeling LA - eng N1 - 1432-1971 Friedman, Kevin G Freud, Lindsay Escobar-Diaz, Maria Banka, Puja Emani, Sitaram Tworetzky, Wayne T32 HL007572/HL/NHLBI NIH HHS/United States Journal Article Pediatr Cardiol. 2015 Oct;36(7):1502-9. doi: 10.1007/s00246-015-1193-6. Epub 2015 May 15. PY - 2015 SN - 0172-0643 (Print) 0172-0643 SP - 1502-9 ST - Left Ventricular Remodeling and Function in Children with Biventricular Circulation After Fetal Aortic Valvuloplasty T2 - Pediatr Cardiol TI - Left Ventricular Remodeling and Function in Children with Biventricular Circulation After Fetal Aortic Valvuloplasty VL - 36 ID - 204 ER - TY - JOUR AB - Left ventricular wall stress and contractile function were determined by echocardiographic methods in 23 patients with tricuspid atresia after palliation only (group 1), in 19 patients after Fontan repair (group 2), and in 24 age-matched normal subjects. End-diastolic dimension was increased above normal in both groups with tricuspid atresia (group 1 [mean +/- SEM] 141 +/- 4%, group 2 129 +/- 5% of normal; both p less than .001) but were not different from each other. Left ventricular end-diastolic volume and wall mass also were increased above normal in both groups (group 1, 126 +/- 9 ml/m2 and 194 +/- 19 g/m2, respectively, p less than .001 and p less than .004; group 2, 80 +/- 7 ml/m2 and 128 +/- 10 g/m2, p less than .02 and p less than .001), and group 2 patients showed significantly lower values than group 1 patients (p less than .001 and p less than .004). Meridional end-systolic stress was increased above normal in both groups with tricuspid atresia but was not different between groups (normal, 43 +/- 3 g/cm2; group 1, 56 +/- 54 g/cm2, p less than .02; group 2, 59 +/- 7 g/cm2, p less than .001). Contractile function estimated by rate-corrected circumferential fiber shortening velocity was abnormal in nine of 23 (39%) group 1 patients and in five of 19 (26%) group 2 patients (percentages not different from each other by nonparametric testing). Contractile function was depressed in one of 11 group 1 patients under 5 years old and in eight of 12 over 5.3 years old.(ABSTRACT TRUNCATED AT 250 WORDS) AN - 2427252 AU - Graham, T. P., Jr. AU - Franklin, R. C. AU - Wyse, R. K. AU - Gooch, V. AU - Deanfield, J. E. DA - Sep DB - PubMed DP - NLM ET - 1986/09/01 IS - 3 Pt 2 KW - Adolescent Adult Child Child, Preschool Echocardiography Humans Infant *Myocardial Contraction *Palliative Care Reference Values Tricuspid Valve/*abnormalities/surgery LA - eng N1 - Graham, T P Jr Franklin, R C Wyse, R K Gooch, V Deanfield, J E Comparative Study Journal Article Research Support, Non-U.S. Gov't United States Circulation. 1986 Sep;74(3 Pt 2):I61-9. PY - 1986 SN - 0009-7322 (Print) 0009-7322 SP - I61-9 ST - Left ventricular wall stress and contractile function in childhood: normal values and comparison of Fontan repair versus palliation only in patients with tricuspid atresia T2 - Circulation TI - Left ventricular wall stress and contractile function in childhood: normal values and comparison of Fontan repair versus palliation only in patients with tricuspid atresia VL - 74 ID - 426 ER - TY - JOUR AB - A description of the history and definition of the pulmonary vascular bed are given. In the adult pulmonary vascular tree are 3 types of vessels: elastic arteries, muscular arteries and arterioles. The presence of muscles suggests a vasoconstrictive function. It is a low-resistance system. Pulmonary arteries are located close to the bronchial tree, veins are as far away as possible. A functional classification of congenital heart disease is given: (1) communication between ventricles or the systemic and pulmonary artery; (2) pulmonary venous drainage into the right atrium; (3) pulmonary venous obstruction. (I) Ventricular septal defect: (A) Without pulmonary stenosis. There are 3 factors influencing the haemodynamics: (1) the relation of the defect to the aortic valve (if the pulmonary vascular resistance is high the Eisenmenger syndrome appears); (2) the size of the defect: in cases of large defects (about the size of the aortic valve) the pulmonary vascular changes are extensive; (3) pulmonary vascular changes: in acquired ventricular septal defects the pulmonary arteries are not prepared to meet the sudden demand. There is no 'protective' resistance. Newborn children with ventricular septal defects may die of left ventricular failure as a result of large left-to-right-shunts. Later in life the L-R-shunt decreases and may be superseded by a R-L-shunt. Three functional types of vascular changes in the lung may occur: (a) high resistance-high reserve: the vessels show thick medial layers in the arterioles and defined elastic layers; (b) high resistance-low reserve: in the large muscular arteries there are obliterative intimai lesions; by dye dilution curves it could be shown that there is a R-L-shunt from the right ventricle but none from the pulmonary artery; (c) a transitional form between a and b: only a few small arteries are occluded ; the others show an increase in wall-thickness. The lumina are normal. The patients of group a would show a fall in the pulmonary artery pressure after surgical closure of the defect. In the groups b and c the pulmonary artery pressure would rise or remain the same. Here the defect functions as an escape valve. A test for the state of the pulmonary arteries is described: the ratio of total pulmonary resistance to total systemic resistance when breathing air and when breathing pure oxygen. If the pulmonary bed is still flexible the total pulmonary vascular resistance falls when the inspired gas has a higher oxygen content than air. (In group a the ratio was always smaller than 0.4:1, in group b always greater than 0.6:1.) Group a represents a persistence of the foetal type of pulmonary vessels, groups b and c are complications of long-standing pulmonary hypertension. Therefore older patients are in groups b and c. (B) Ventricular septal defect with pulmonary stenosis: (1) if pulmonary stenosis is mild a hypertrophy of the media of arteries and arterioles can be found; (2) if the pulmonary stenosis is moderate pulmonary arteries are normal; (3) if the pulmonary stenosis is severe. (II) Functional single ventricle: the situation is about the same as in I. The shape of the pulmonary arteries depends on the degree of pulmonary stenosis and the shunt-direction on the degree of pulmonary resistance. (III) Patent duct us arteriosus: later in life 2 groups of patients are found: (a) the ductus is thin and obstructive and functions like a valve; (b) the ductus is short and wide; this type may be compared with a large ventricular septal defect. (IV) Atrial septal defects in adults: type 1: low pulmonary resistance and normal pulmonary artery pressure; type 2: pulmonary resistance elevated and pulmonary artery pressure elevated (4th decade of life). (aa) Pulmonary artery pressure is greater than the aortic pressure (about 40-80 mm. Hg). Moderate pulmonary hypertension: the histological picture: either obstructions in the larger arteries and the smaller ones are free or the opposite can be found. (bb) The pulmonary pressure is equal to the aortic pressure - severe pulmonary hypertension. The wo atria function as a common chamber: since the right ventricle has a thinner wall than the left more blood will flow into the right. The excessive blood comes from the left atrium and - due to its anatomical location - from the right pulmonary vein. A small R-L-shunt results from a blood stream from the inferior vena cava which is directed toward the anterior edge of the atrial septal defect. When the pulmonary pressure goes up later in life the right ventricle hypertrophies and becomes less distensible -the R-L-shunt dominates. Atrial septal defects in infants and children: in newborns no shunt may be found. During foetal life the pulmonary vascular resistance is high and leads to a R-L-shunt. After birth the pulmonary resistance decreases and the L-R-shunt will be more evident. (V) Obstructions to pulmonary venous flow: these can be found in cases of acquired mitral stenosis, congenital stenosis of the pulmonary veins (cor triatriatum), endocardial sclerosis, chronic left ventricular failure due to mitral insufficiency, coarctation of the aorta and aortic stenosis. Histology: medial hypertrophy of pulmonary arteries and arterioles (more severe in congenital than in acquired lesions) and prominence of the elastic membranes. The occurrence of pulmonary oedema may be due to the temporary failure of a vasoconstrictive phenomenon at the pulmonary arteriolar level. An increased volume in the pulmonary capillaries is associated with an increased pressure at this level if the venous obstruction remains the same; oedema results. AD - J.E. Edwards, Mayo Clin., Mayo Found., Univ. of Minnesota, Minneapolis, MN, United States AU - Edwards, J. E. DB - Embase Classic IS - 2 KW - oxygen adult aorta aortic pressure aortic stenosis aortic valve arteriole artery blood blood flow breathing child classification congenital heart disease cor pulmonale cor triatriatum dye dilution curve edema Eisenmenger complex endocardium gas heart atrium heart atrium septum defect heart left atrium heart left right shunt heart left ventricle failure heart right atrium heart right ventricle heart single ventricle heart ventricle septum defect hemodynamics histology hypertrophy infant inferior cava vein interpersonal communication lung lung artery pressure lung blood vessel lung capillary lung edema lung pressure lung resistance lung vascular resistance pulmonary vein obstruction membrane mitral valve regurgitation mitral valve stenosis muscle newborn obstruction patent pathology patient pulmonary artery pulmonary hypertension pulmonary valve stenosis pulmonary vein sclerosis stenosis thickness tracheobronchial tree tree vein vein blood flow vein occlusion LA - English M3 - Article N1 - L280813178 1957-12-01 PY - 1957 SN - 0009-7322 SP - 164-196 ST - The Lewis A. Conner memorial lecture. Functional pathology of the pulmonary vascular tree in congenital cardiac disease T2 - Circulation TI - The Lewis A. Conner memorial lecture. Functional pathology of the pulmonary vascular tree in congenital cardiac disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L280813178 VL - 15 ID - 1424 ER - TY - JOUR AB - BACKGROUND: Patients who underwent Fontan operation have some degree of liver disease. We aimed to assess the long-term liver and cardiac function after Fontan operation. METHODS: Patients enrolled underwent physical examination, biochemical tests (aspartate aminotransferase, alanine aminotransferase, gamma glutamyl transpeptidase, bilirubin, international normalized ratio, coagulation factor V, protein profile, fecal alpha-1-antitrypsin), echocardiogram, and liver ultrasonography. A liver disease score was adopted to compare the degree of liver involvement with hemodynamic features. RESULTS: The study enrolled 34 patients, median age 14.7 years (range, 4.1 to 26.7), 26 with a residual left ventricle, 8 with a residual right ventricle, affected by tricuspid atresia (17), pulmonary atresia (4), hypoplastic left heart syndrome (5), double-outlet right ventricle (2), single left ventricle (2), and miscellaneous (4), with median follow-up of 11.5 years (range, 1.7 to 23.3). We found hepatomegaly in 18 of 34 (53%), splenomegaly in 3 of 33 (9%), abnormal transaminases in 10 of 33 (30%), elevated gamma GT in 19 of 31 (61%), elevated bilirubin in 10 of 31 (32%), coagulopathy in 17 of 29 (58%), and protein-losing enteropathy in 4 of 21 (19%). Median heart rate z-score was -1.72. Hepatic dysfunction was strictly correlated to low cardiac index (r(2) = 0.34, p = 0.008) and to a lesser extent to reduced heart rate (r(2) = 0.18, p = 0.07). CONCLUSIONS: In children who underwent Fontan operation, hepatic dysfunction is correlated with low cardiac index and reduced heart rate. Maintaining or reestablishing a normal cardiac index might prevent or reduce liver disease in the long-term. AD - Department of Pediatric Hepatology, University of Padova, Padova, Italy. AN - 18573420 AU - Camposilvan, S. AU - Milanesi, O. AU - Stellin, G. AU - Pettenazzo, A. AU - Zancan, L. AU - D'Antiga, L. DA - Jul DB - PubMed DO - 10.1016/j.athoracsur.2008.03.077 DP - NLM ET - 2008/06/25 IS - 1 KW - Adolescent Adult Age Distribution Child Child, Preschool Cohort Studies Evaluation Studies as Topic Female Follow-Up Studies Fontan Procedure/*adverse effects/methods Heart Defects, Congenital/diagnosis/*surgery Heart Function Tests Humans Incidence Liver Function Tests Male Postoperative Complications/*diagnosis/epidemiology Probability Prognosis Quality of Life Risk Assessment Sex Distribution Time Factors LA - eng N1 - 1552-6259 Camposilvan, Sonia Milanesi, Ornella Stellin, Giovanni Pettenazzo, Andrea Zancan, Lucia D'Antiga, Lorenzo Journal Article Netherlands Ann Thorac Surg. 2008 Jul;86(1):177-82. doi: 10.1016/j.athoracsur.2008.03.077. PY - 2008 SN - 0003-4975 SP - 177-82 ST - Liver and cardiac function in the long term after Fontan operation T2 - Ann Thorac Surg TI - Liver and cardiac function in the long term after Fontan operation VL - 86 ID - 341 ER - TY - JOUR AB - OBJECTIVES: This study sought to determine whether survival in this cohort of patients was adversely affected by increased residential altitude. BACKGROUND: The success of the Fontan procedure depends in large part on low pulmonary vascular resistance (PVR). Factors that increase PVR, including an increase in residential altitude, may adversely affect long-term outcome. Higher altitude has been shown to affect functional well-being in patients with a Fontan circulation. METHODS: Databases from a tertiary cardiac care center in the Intermountain West (elevation 5,000 feet) were analyzed for patients born with single-ventricle anatomy who would now be of adult age. Complete data were then collected on all identified patients who subsequently underwent the Fontan operation. Correlates of, and time to, adverse outcome, defined as death, cardiac transplantation, or clinical decompensation requiring a move to sea level, were determined. RESULTS: Of 149 patients with single-ventricle anatomy, 103 underwent the Fontan procedure, with 70 surviving to adulthood at moderate altitude. Adverse outcome occurred in 55, with death in 24 (23%), cardiac transplantation in 18 (17%), and clinical decompensation requiring move to sea level in 13 (13%). There was no relationship between type, age at, or era of Fontan procedure and long-term outcome. Correlates of long-term, transplant-free survival at moderate altitude included lower residential altitude (4,296 vs. 4,637 feet, p < 0.001), and lower pulmonary artery pressures before the Fontan procedure (13 vs. 15 mm Hg, p = 0.01), and after (14 vs. 18 mm Hg, p = 0.01). CONCLUSIONS: Long-term outcome after the Fontan procedure is adversely impacted by higher residential altitude. AD - Division of Cardiology, Department of Pediatrics, Primary Children's Medical Center, University of Utah, Salt Lake City, Utah 84113, USA. AN - 23414794 AU - Johnson, J. T. AU - Lindsay, I. AU - Day, R. W. AU - Van Dorn, C. S. AU - Hoffman, J. AU - Everitt, M. D. AU - Yetman, A. T. DA - Mar 26 DB - PubMed DO - 10.1016/j.jacc.2013.01.008 DP - NLM ET - 2013/02/19 IS - 12 KW - Adolescent Adult Altitude Sickness/*mortality/physiopathology Cause of Death Cohort Studies Disease Progression Female Fontan Procedure/*mortality Heart Transplantation/mortality Hemodynamics/physiology Humans Male Oxygen/blood Population Dynamics Postoperative Complications/*mortality/physiopathology Risk Factors Statistics as Topic Survival Rate Vascular Resistance/physiology Young Adult LA - eng N1 - 1558-3597 Johnson, Joy T Lindsay, Ian Day, Ronald W Van Dorn, Charlotte S Hoffman, James Everitt, Melanie D Yetman, Anji T Journal Article United States J Am Coll Cardiol. 2013 Mar 26;61(12):1283-9. doi: 10.1016/j.jacc.2013.01.008. Epub 2013 Feb 13. PY - 2013 SN - 0735-1097 SP - 1283-9 ST - Living at altitude adversely affects survival among patients with a Fontan procedure T2 - J Am Coll Cardiol TI - Living at altitude adversely affects survival among patients with a Fontan procedure VL - 61 ID - 265 ER - TY - JOUR AN - 11410548 AU - Treasure, T. C2 - PMC1729794 DA - Jul DB - PubMed DO - 10.1136/heart.86.1.5 DP - NLM ET - 2001/06/19 IS - 1 KW - Adolescent Adult Heart Transplantation Heart Ventricles/*abnormalities/surgery Humans Middle Aged *Quality of Life Surveys and Questionnaires LA - eng N1 - 1468-201x Treasure, T Comment Editorial Heart. 2001 Jul;86(1):5-6. doi: 10.1136/heart.86.1.5. PY - 2001 SN - 1355-6037 (Print) 1355-6037 SP - 5-6 ST - Living with a univentricular heart T2 - Heart TI - Living with a univentricular heart VL - 86 ID - 495 ER - TY - GEN AB - The Quality of Life instruments are probably most valuable when there is a treatment which has as many downsides as advantages, where the health gain is not immediately obvious, and there are two groups to compare. Long term treatment of hypertension is probably a reasonable example. The health gain is reduction in heart failure and stroke in years to come. The price is pill taking with side effects which might include a very wide range of manifestations including lack of energy, worsening asthma, cough, nightmares, impotence, urinary frequency, postural hypotension and others depending on which drugs are on trial. AD - Gardiothoracic Unit, Guy's and St Thomas' Hospital, St Thomas Street London SE1 9RT UK. AU - Treasure, Tom AU - Treasure, T. DB - ccm DO - 10.1136/heart.86.1.5 DP - EBSCOhost J2 - Heart KW - Quality of Life Heart Ventricle -- Abnormalities Heart Transplantation Adolescence Heart Ventricle -- Surgery Middle Age Adult N1 - Accession Number: 12940827. Language: English. Entry Date: 20020118. Revision Date: 20190515. Publication Type: commentary; editorial. Journal Subset: Biomedical; Blind Peer Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Instrumentation: Wide Range Achievement Test (WRAT). NLM UID: 9602087. PMID: NLM11410548. PB - BMJ Publishing Group PY - 2001 SN - 1355-6037 SP - 5-6 ST - Living with a univentricular heart TI - Living with a univentricular heart UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=12940827&site=ehost-live&scope=site VL - 86 ID - 1652 ER - TY - JOUR AB - A child with complex congenital heart disease in 2008 is very likely to survive a series of surgical and medical interventions, and confront an array of medical and psychosocial stressors that are presently poorly understood. As approaches to medical problems change, careful assessment of those results is essential, and the initial work of the multicentre Pediatric Heart Network is a huge step in the right direction, setting the stage for proper controlled trials of therapies. Major complications, notably ventricular failure, rhythm problems and thromboembolism, will affect nearly one-quarter of survivors, necessitating further interventions. Appropriate educational and psychosocial support for these children and their families is the next challenge for all of us in the field of paediatrics. How ironic would it be to have invested so much in early survival, only to allow the child to fail in life itself. Publisher: Abstract available from the publisher. fre AD - BC Children's Hospital and BC Women's Hospital & Health Centre, Vancouver, British Columbia. AN - 20190896 AU - Human, D. G. C2 - PMC2690545 DA - Mar DB - PubMed DO - 10.1093/pch/14.3.161 DP - NLM ET - 2010/03/02 IS - 3 KW - Congenital heart disease Fontan circulation Quality of life LA - eng N1 - 1918-1485 Human, Derek G Journal Article Paediatr Child Health. 2009 Mar;14(3):161-82. doi: 10.1093/pch/14.3.161. PY - 2009 SN - 1205-7088 (Print) 1205-7088 SP - 161-82 ST - Living with complex congenital heart disease T2 - Paediatr Child Health TI - Living with complex congenital heart disease VL - 14 ID - 436 ER - TY - JOUR AB - BACKGROUND AND RESEARCH OBJECTIVE: Approximately 3% of children with congenital heart disease born in Denmark have single ventricle physiology (SVP). In previous decades, these children did not survive into adulthood. However, because of new surgical techniques and improved medical care, they now have a 90% survival rate. Several studies have described the somatic status of SVP patients using clinical parameters; however, only a few studies have researched the life perspectives and coping skills in this patient group. The aim of this study was to investigate how young adults with an SVP diagnosis are coping with adulthood and the emotional experiences of daily life. SUBJECTS AND METHODS: Semistructured, qualitative interviews were held with 11 SVP respondents, selected by physical and psychological parameters identified in an earlier quantitative study. Data from the interviews were analyzed by a research group using a phenomenological methodology. RESULTS AND CONCLUSIONS: The goal for SVP patients is to gain control over their disease to live normal lives. Patients require special support from their core network to overcome physical and psychological challenges. Respondents underscored the need for friends and resource persons outside the family to help lift them out of their role as disabled cardiac patients and provide them with "normal" life experiences. AD - Heart Centre, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark. dorthe@overgaard.mail.dk AN - 22635059 AU - Overgaard, D. AU - King, C. AU - Christensen, R. F. AU - Schrader, A. M. AU - Adamsen, L. DA - Mar-Apr DB - PubMed DO - 10.1097/JCN.0b013e3182498677 DP - NLM ET - 2012/05/29 IS - 2 KW - Activities of Daily Living *Adaptation, Psychological Adolescent Adult Denmark Female Fontan Procedure/*rehabilitation Heart Ventricles/*abnormalities Humans Male Middle Aged *Quality of Life Survivors/psychology Young Adult LA - eng N1 - 1550-5049 Overgaard, Dorthe King, Catriona Christensen, Rie F Schrader, Anne-Marie Adamsen, Lis Journal Article United States J Cardiovasc Nurs. 2013 Mar-Apr;28(2):187-96. doi: 10.1097/JCN.0b013e3182498677. PY - 2013 SN - 0889-4655 SP - 187-96 ST - Living with half a heart--experiences of young adults with single ventricle physiology: a qualitative study T2 - J Cardiovasc Nurs TI - Living with half a heart--experiences of young adults with single ventricle physiology: a qualitative study VL - 28 ID - 181 ER - TY - JOUR AB - BACKGROUND AND RESEARCH OBJECTIVE:: Approximately 3% of children with congenital heart disease born in Denmark have single ventricle physiology (SVP). In previous decades, these children did not survive into adulthood. However, because of new surgical techniques and improved medical care, they now have a 90% survival rate. Several studies have described the somatic status of SVP patients using clinical parameters; however, only a few studies have researched the life perspectives and coping skills in this patient group. The aim of this study was to investigate how young adults with an SVP diagnosis are coping with adulthood and the emotional experiences of daily life. SUBJECTS AND METHODS:: Semistructured, qualitative interviews were held with 11 SVP respondents, selected by physical and psychological parameters identified in an earlier quantitative study. Data from the interviews were analyzed by a research group using a phenomenological methodology. RESULTS AND CONCLUSIONS:: The goal for SVP patients is to gain control over their disease to live normal lives. Patients require special support from their core network to overcome physical and psychological challenges. Respondents underscored the need for friends and resource persons outside the family to help lift them out of their role as disabled cardiac patients and provide them with "normal" life experiences. Copyright © 2013 Lippincott Williams & Wilkins. AD - Heart Centre, Copenhagen University Hospital, Rigshospitalet, and University Hospitals Centre for Nursing and Care Research (UCSF), Rigshospitalet, Copenhagen, Denmark Nurse at the Grown-up CHD, Heart Centre, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark Research Assistant, Heart Centre, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark University College, Metropol, Copenhagen, Denmark Institute of Public Health, Copenhagen University, University Hospitals Centre for Nursing and Care Research (UCSF), Rigshospitalet, Copenhagen, Denmark AU - Overgaard, D. AU - King, C. AU - Christensen, R. F. AU - Schrader, A. M. AU - Adamsen, L. DB - Scopus DO - 10.1097/JCN.0b013e3182498677 IS - 2 KW - congenital heart disease lived experiences network normalization single ventricle physiology M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 2013 SP - 187-196 ST - Living with half a heart-experiences of young adults with single ventricle physiology: A qualitative study T2 - Journal of Cardiovascular Nursing TI - Living with half a heart-experiences of young adults with single ventricle physiology: A qualitative study UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84873998526&doi=10.1097%2fJCN.0b013e3182498677&partnerID=40&md5=8706d216ef225168dad83f3fe57aa1f8 VL - 28 ID - 2059 ER - TY - JOUR AB - Approximately 3% of children with congenital heart disease born in Denmark have single ventricle physiology (SVP). In previous decades, these children did not survive into adulthood. However, because of new surgical techniques and improved medical care, they now have a 90% survival rate. Several studies have described the somatic status of SVP patients using clinical parameters; however, only a few studies have researched the life perspectives and coping skills in this patient group. The aim of this study was to investigate how young adults with an SVP diagnosis are coping with adulthood and the emotional experiences of daily life.Semistructured, qualitative interviews were held with 11 SVP respondents, selected by physical and psychological parameters identified in an earlier quantitative study. Data from the interviews were analyzed by a research group using a phenomenological methodology.The goal for SVP patients is to gain control over their disease to live normal lives. Patients require special support from their core network to overcome physical and psychological challenges. Respondents underscored the need for friends and resource persons outside the family to help lift them out of their role as disabled cardiac patients and provide them with “normal” life experiences. AD - Dorthe Overgaard, PhD, RN Researcher, the Heart Centre, Copenhagen University Hospital, Rigshospitalet, and University Hospitals Centre for Nursing and Care Research (UCSF), Rigshospitalet, Copenhagen, Denmark. Catriona King, RN Nurse at the Grown-up CHD, the Heart Centre, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark. Rie F. Christensen, MSc Research Assistant, the Heart Centre, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark. Anne-Marie Schrader, MPH, RN Lecturer, University College, Metropol, Copenhagen, Denmark. Lis Adamsen, PhD, RN Professor, Institute of Public Health, Copenhagen University, and University Hospitals Centre for Nursing and Care Research (UCSF), Rigshospitalet, Copenhagen, Denmark. AN - 112059346. Language: English. Entry Date: 20180427. Revision Date: 20190328. Publication Type: Article AU - Overgaard, Dorthe AU - King, Catriona AU - Christensen, Rie F. AU - Schrader, Anne-Marie AU - Adamsen, Lis DB - ccm DO - 10.1097/JCN.0b013e3182498677 DP - EBSCOhost IS - 2 KW - Heart Ventricle -- Pathology Heart Defects, Congenital -- Psychosocial Factors -- In Adolescence Adaptation, Psychological Quality of Life Human Male Female Adolescence Young Adult Adult Middle Age Qualitative Studies Semi-Structured Interview N1 - research; tables/charts. Journal Subset: Core Nursing; Nursing; Peer Reviewed; USA. NLM UID: 8703516. PY - 2013 SN - 0889-4655 SP - 187-196 ST - Living With Half a Heart—Experiences of Young Adults With Single Ventricle Physiology T2 - Journal of Cardiovascular Nursing TI - Living With Half a Heart—Experiences of Young Adults With Single Ventricle Physiology UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=112059346&site=ehost-live&scope=site VL - 28 ID - 1545 ER - TY - JOUR AD - Division of Pediatric Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA. Wernovsky@email.chop.edu AN - 15244152 AU - Wernovsky, G. AU - Chrisant, M. R. DA - Feb DB - PubMed DO - 10.1017/s1047951104006444 DP - NLM ET - 2004/07/13 KW - Cardiac Surgical Procedures/*methods/mortality Central Nervous System/pathology Child Follow-Up Studies Heart Defects, Congenital/mortality/*surgery *Heart Transplantation Humans Prognosis Quality of Life Reconstructive Surgical Procedures/*methods/mortality Survival Rate Thrombosis/complications Time Factors Treatment Outcome LA - eng N1 - Wernovsky, Gil Chrisant, Maryanne R K Journal Article Review England Cardiol Young. 2004 Feb;14 Suppl 1:115-26. doi: 10.1017/s1047951104006444. PY - 2004 SN - 1047-9511 (Print) 1047-9511 SP - 115-26 ST - Long-term follow-up after staged reconstruction or transplantation for patients with functionally univentricular heart T2 - Cardiol Young TI - Long-term follow-up after staged reconstruction or transplantation for patients with functionally univentricular heart VL - 14 Suppl 1 ID - 507 ER - TY - JOUR AB - Objective: The objective of this study was to evaluate health-related quality of life in long-term survivors of mechanical circulatory support after acute cardiopulmonary failure. Design: Prospective follow-up study. Setting: Single-institutional in a center for congenital heart disease and pediatric cardiology. Patients: Fifty patients who underwent 58 mechanical circulatory support therapies in our institution from 2001 to 2012. Median age was 2 (0-213) months, and median supporting time was 5 (1-234) days. Indication groups: 1) extracorporeal life support in low cardiac output: 30 cases (52%); 2) extracorporeal cardiopulmonary resuscitation: 13 cases (22%); 3) extracorporeal membrane oxygenation in acute respiratory distress syndrome: four cases (7%); and 4) ventricular assist devices: 11 cases (19%). Interventions: Health-related quality of life was measured using standardized questionnaires according to the age group and completed by either parent proxies in children under 7 years old or the survivors themselves. Measurements and Main Results: Fifty percentage of the patients were discharged home, and 22 long-term survivors (44%) were studied prospectively for health-related quality of life. Median follow-up period was 4.5 (0.3-11.3) years. Median age at follow-up was 5 (0.6-29) years old. Nineteen long-term survivors filled in the health-related quality of life questionnaires and were classified into three age groups: 0-4 years (n = 7): median health-related quality of life score, 69 (59-86) points; 4-12 years (n = 7): median health-related quality of life score, 50 (48-85) points; older than 12 years (n = 5): median health-related quality of life score, 90 (80-100) points. Conclusion: Long-term survivors' health-related quality of life as reported by their parents is lower than that of healthy children. However, the self-assessed health-related quality of life of the patients older than 12 years in our group is comparable to a healthy control population. AU - Fleck, T. P. K. AU - Dangel, G. AU - Bächle, F. AU - Benk, C. AU - Grohmann, J. AU - Kroll, J. AU - Siepe, M. AU - Höhn, R. AU - Kirschner, J. AU - Beyersdorf, F. AU - Stiller, B. DB - Embase Medline DO - 10.1097/PCC.0000000000001019 IS - 2 KW - antithrombin III epinephrine fibrinogen heparin noradrenalin adult respiratory distress syndrome article assisted circulation atrioventricular septal defect blood pump cardiopulmonary insufficiency child child development congestive cardiomyopathy extracorporeal oxygenation female follow up forward heart failure great vessels transposition heart arrest heart right ventricle double outlet human hypoplastic left heart syndrome infant intellectual impairment left ventricular assist device long term survival major clinical study male membrane oxygenator motor development pediatric cardiology priority journal prospective study quality of life questionnaire Short Form 36 speech disorder survivor ventricular assist device Deltastream DP2 Heart Excor Heart-mate II Paracorporeal VAD LA - English M3 - Article N1 - L613274594 2016-11-23 2017-02-14 PY - 2017 SN - 1947-3893 1529-7535 SP - 176-182 ST - Long-Term Follow-Up on Health-Related Quality of Life after Mechanical Circulatory Support in Children T2 - Pediatric Critical Care Medicine TI - Long-Term Follow-Up on Health-Related Quality of Life after Mechanical Circulatory Support in Children UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L613274594 http://dx.doi.org/10.1097/PCC.0000000000001019 VL - 18 ID - 773 ER - TY - JOUR AB - The Fontan procedure is used to treat various serious congenital heart defects. Although many people who have had the procedure live productively into adulthood, as they age, they face several health issues due to the physiology of the Fontan circulation.This article reviews the 4 types of Fontan procedures and the changes caused by the surgery, including single-ventricle physiology, non- pulsatile pulmonary perfusion, systemic venous hypertension, and intracardiac scarring, as well as their sequelae. Key nursing assessment items and possible treatment strategies are reviewed. Additional topics, including pregnancy in patients who have undergone the procedure, infective endocarditis prophylaxis, and health-related quality of life, are briefly discussed. Options for Fontan failure, including Fontan conversion or transplantation, are presented. Potential future solutions are outlined. AD - Nurse Practitioner-Congenital Heart Program, Advanced Health Sciences Pavilion, A3400-03 Cedars-Sinai Medical Center, Los Angeles, CA AN - 107964765. Language: English. Entry Date: 20130819. Revision Date: 20150818. Publication Type: Journal Article AU - McRae, Marion E. DB - ccm DO - 10.1097/NCI.0b013e31829744c7 DP - EBSCOhost IS - 3 KW - Heart Defects, Congenital -- Surgery Treatment Complications, Delayed Heart Surgery -- Methods -- In Infancy and Childhood Education, Continuing (Credit) Heart Failure Aortic Valve Insufficiency Arteriovenous Malformations Esophageal and Gastric Varices Hypertension, Portal Liver Diseases Venous Insufficiency Pericardial Effusion Physical Therapy Assessment After Care Atrial Fibrillation Tachycardia, Ventricular Pregnancy Female Contraception Depression Blood Circulation Electrocardiography Cicatrix Treatment Failure Heart Assist Devices Heart Transplantation Infant Child Child, Preschool N1 - CEU; exam questions; pictorial; tables/charts; tracings. Journal Subset: Core Nursing; Double Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Nursing; Peer Reviewed; USA. Special Interest: Advanced Nursing Practice; Critical Care. NLM UID: 101269322. PMID: NLM23880750. PY - 2013 SN - 1559-7768 SP - 264-284 ST - Long-term Issues After the Fontan Procedure T2 - AACN Advanced Critical Care TI - Long-term Issues After the Fontan Procedure UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=107964765&site=ehost-live&scope=site VL - 24 ID - 1533 ER - TY - JOUR AB - The first successful Fontan operation was performed in 1971, and this first cohort of Fontan patients is reaching adulthood with unclear outcome of this palliative procedure. We studied the mortality, morbidity, and quality of life in our adult Fontan patients. We examined all patients (n = 36) who underwent a Fontan procedure and were being seen in an adult outpatient clinic by using electrocardiography, exercise testing, and echocardiography. Quality of life was assessed by the Short Form 36 questionnaire. The mean follow-up period was 15 years (range 0 to 23). Of the initial 36 patients, 10 died (28%) at a mean of 10 years (range 0 to 21) after the Fontan operation and 1 patient underwent cardiac transplantation. Reoperations were performed in 21 patients (58%), and the most common reason was revision of the Fontan connection. Sustained supraventricular tachycardia was observed in 20 patients (56%) with an increased incidence of arrhythmias with longer follow-up. Thromboembolic events were detected in 9 patients (25%), 5 of whom had adequate anticoagulant levels at the time of event. The thromboembolic event was fatal for 3 patients. A total of 195 hospital admissions (mean 3.8 +/- 2.7, range 1 to 13) was recorded. Quality-of-life assessment showed physical functioning, mental health, and general health perception to be significantly lower for Fontan patients than for the normal Dutch population. Thus, we found high mortality and very high morbidity in adult patients after the Fontan operation. In particular, reoperations, arrhythmias, and thromboembolic events compromised quality of life. AD - Department of Cardiology, Thoraxcenter, Erasmus MC, Rotterdam, The Netherlands. AN - 15110207 AU - van den Bosch, A. E. AU - Roos-Hesselink, J. W. AU - Van Domburg, R. AU - Bogers, A. J. AU - Simoons, M. L. AU - Meijboom, F. J. DA - May 1 DB - PubMed DO - 10.1016/j.amjcard.2004.01.041 DP - NLM ET - 2004/04/28 IS - 9 KW - Adolescent Adult Anticoagulants/therapeutic use Child Child, Preschool Cross-Sectional Studies Echocardiography Exercise Tolerance/physiology Female Follow-Up Studies *Fontan Procedure Heart Atria/abnormalities/surgery Heart Defects, Congenital/mortality/physiopathology/therapy Heart Ventricles/abnormalities/surgery Humans Male Morbidity Netherlands/epidemiology Postoperative Complications/etiology/mortality/physiopathology *Quality of Life Time Time Factors Treatment Outcome Tricuspid Atresia/mortality/physiopathology/therapy LA - eng N1 - van den Bosch, Annemien E Roos-Hesselink, Jolien W Van Domburg, Ron Bogers, Ad J J C Simoons, Maarten L Meijboom, Folkert J Comparative Study Evaluation Study Journal Article United States Am J Cardiol. 2004 May 1;93(9):1141-5. doi: 10.1016/j.amjcard.2004.01.041. PY - 2004 SN - 0002-9149 (Print) 0002-9149 SP - 1141-5 ST - Long-term outcome and quality of life in adult patients after the Fontan operation T2 - Am J Cardiol TI - Long-term outcome and quality of life in adult patients after the Fontan operation VL - 93 ID - 166 ER - TY - JOUR AB - The first successful Fontan operation was performed in 1971, and this first cohort of Fontan patients is reaching adulthood with unclear outcome of this palliative procedure. We studied the mortality, morbidity, and quality of life in our adult Fontan patients. We examined all patients (n = 36) who underwent a Fontan procedure and were being seen in an adult outpatient clinic by using electrocardiography, exercise testing, and echocardiography. Quality of life was assessed by the Short Form 36 questionnaire. The mean follow-up period was 15 years (range 0 to 23). Of the initial 36 patients, 10 died (28%) at a mean of 10 years (range 0 to 21) after the Fontan operation and 1 patient underwent cardiac transplantation. Reoperations were performed in 21 patients (58%), and the most common reason was revision of the Fontan connection. Sustained supraventricular tachycardia was observed in 20 patients (56%) with an increased incidence of arrhythmias with longer follow-up. Thromboembolic events were detected in 9 patients (25%), 5 of whom had adequate anticoagulant levels at the time of event. The thromboembolic event was fatal for 3 patients. A total of 195 hospital admissions (mean 3.8 ± 2.7, range 1 to 13) was recorded. Quality-of-life assessment showed physical functioning, mental health, and general health perception to be significantly lower for Fontan patients than for the normal Dutch population. Thus, we found high mortality and very high morbidity in adult patients after the Fontan operation. In particular, reoperations, arrhythmias, and thromboembolic events compromised quality of life. © 2004 by Excerpta Medica, Inc. AD - J.W. Roos-Hesselink, Thoraxcenter Ba 308, Erasmus MC, Dr Molewaterplein 40, 3015 GD Rotterdam, Netherlands AU - Van Den Bosch, A. E. AU - Roos-Hesselink, J. W. AU - Van Domburg, R. AU - Bogers, A. J. J. C. AU - Simoons, M. L. AU - Meijboom, F. J. DB - Embase Medline DO - 10.1016/j.amjcard.2004.01.041 IS - 9 KW - adolescent adult article child clinical article electrocardiography follow up Fontan procedure heart arrhythmia heart transplantation hospital admission human medical assessment medical record mental health morbidity mortality outpatient priority journal quality of life reoperation supraventricular tachycardia thrombosis LA - English M3 - Article N1 - L38521044 2004-05-06 PY - 2004 SN - 0002-9149 SP - 1141-1145 ST - Long-term outcome and quality of life in adult patients after the Fontan operation T2 - American Journal of Cardiology TI - Long-term outcome and quality of life in adult patients after the Fontan operation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L38521044 http://dx.doi.org/10.1016/j.amjcard.2004.01.041 VL - 93 ID - 1295 ER - TY - JOUR AB - Introduction: Hypoplastic left heart syndrome (HLHS), one of the most serious congenital heart defects, can be surgically paliated using 3 subsequent stages. Long-term results of this approach are reviewed in this study. Methods: Fifty-two consecutive patients (pts.) operated for HLHS from 1999 to 2012 were evaluated retrospectively. Results: Norwood stage I operation was performed at the median age of 6.5 days with a total mortality of 19%. Significant risk factor for death was lower weight at surgery (Cox proportional risk per 1 g increase = 0.997, CI 0.995-0.990, P < 0.001). Between stages I and II, 15 catheter/surgical reintervention had to be carried out in 13 pts. (aortic arch narrowing in 10/13). Forty-two pts. underwent stage II operation at the median age of 6.8 months with a total mortality of 4.8% and 18 subsequent reinterventions in 13 patients. Finally, 26 patients aged median 3.9 years underwent stage III operation (total cavopulmonary connection) with a total mortality of 8%. The probability of survival at 1/5/10 years of age was 77/77/71%. At long-term follow up (median 7.8 years) 37 of the 38 surviving patients are in NYHA functional class I or II. Conclusions: Despite a highly centralized care, surgical treatment of HLHS is still associated with a significant mortality and morbidity. Long-term survivals, however, have an accept- able functional status during childhood corresponding to other groups of patients after surgical palliation for functionally single ventricle. AD - P. Vojtovič, Children's Heart Centre, University Hospital Motol, V Úvalu 84, Prague 5-Motol, Czech Republic AU - Vojtovič, P. AU - Tláskal, T. AU - Gebauer, R. AU - Reich, O. AU - Chaloupecký, V. AU - Tomek, V. AU - Krupičková, S. AU - Matějka, T. AU - Hecht, P. AU - Janoušek, J. DB - Embase DO - 10.1016/j.crvasa.2014.07.006 IS - 6 KW - antiarrhythmic agent anticonvulsive agent inotropic agent adolescent adrenal cortex insufficiency annuloplasty ring anticonvulsant therapy aortopulmonary shunt article autism Blalock Taussig shunt body weight cardiac resynchronization therapy cause of death cavopulmonary connection child chylothorax complete heart block controlled study Damus Kaye Stansel procedure dyslexia enteropathy female fenestration follow up functional status heart failure heart left ventricle failure heart muscle ischemia heart right bundle branch block heart surgery hospitalization human hypoplastic left heart syndrome junctional ectopic tachycardia long term care long term survival major clinical study male mediastinitis metabolic disorder morbidity New York Heart Association class Norwood procedure cardiac rhythm management device pacemaker implantation palliative therapy pediatric surgery percutaneous transluminal angioplasty postoperative complication pulmonary artery stenosis reoperation retrospective study right ventricle to pulmonary artery conduit risk factor second degree atrioventricular block sepsis special education staged palliation staging surgical mortality surgical risk survival time tricuspid annuloplasty tricuspid valve regurgitation tricuspid valve repair tricuspid valve replacement LA - English M3 - Article N1 - L600599218 2014-12-03 2014-12-05 PY - 2014 SN - 1803-7712 0010-8650 SP - e449-e455 ST - Long-term results of children operated for hypoplastic left heart syndrome in Children's Heart Centre T2 - Cor et Vasa TI - Long-term results of children operated for hypoplastic left heart syndrome in Children's Heart Centre UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L600599218 http://dx.doi.org/10.1016/j.crvasa.2014.07.006 VL - 56 ID - 966 ER - TY - JOUR AB - Background. We set out to examine the long-term results of relief of subaortic stenosis by enlargement of ventricular septal defect in patients with univentricular atrioventricular connection to a dominant left ventricle and discordant ventriculoarterial connections. Methods. Twenty-four patients underwent enlargement of ventricular septal defect between 1985 and 1998 at a median age of 3.2 years (range, 3 weeks to 14 years). Ten patients were younger than 1 year of age. Eighteen had undergone previous banding of the pulmonary trunk, 9 of whom also required repair of coarctation of the aorta. The median subaortic gradient before enlargement was 46 mm Hg. Twenty-three patients had a patch to enlarge the rudimentary right ventricle. Results. Five patients (21%) died in the early postoperative period. The overall survival at 1 and 3 years was 73%, and at 5 and 10 years was 68% and 60%, respectively. Complete heart block requiring insertion of a pacemaker occurred in 2 patients (8%). A Fontan operation was performed in 10 patients, 5 underwent a bidirectional Glenn procedure, and 2 required Cardiac transplantation. Follow-up was complete in all survivors at a median time of 6.7 years (range, 8 months to 13 years). From the earlier part of the series, 3 patients experienced aortic insufficiency and 2 had recurrent obstruction. Factors adversely affecting survival were age younger than 1 year at operation and presence of obstruction within the aortic arch. Conclusions. Our experience shows that, in patients with univentricular atrioventricular connection to a dominant left ventricle and subaortic stenosis, enlargement of the ventricular septal defect provides satisfactory relief of obstruction except in those younger than 1 year of age, and those who have associated obstruction in the aortic arch. © 2001 by The Society of Thoracic Surgeons. AD - M. Jahangiri, Department of Cardiac Surgery, Gt. Ormond St. Hospital for Children, Great Ormond St, London WC1N 3JH, United Kingdom AU - Jahangiri, M. AU - Shinebourne, E. A. AU - Ross, D. B. AU - Anderson, R. H. AU - Lincoln, C. DB - Embase Medline DO - 10.1016/S0003-4975(00)02544-3 IS - 3 KW - aortic arch subvalvular aortic stenosis article atrioventricular dissociation child clinical article Fontan procedure heart block heart ventricle septum defect human cardiac rhythm management device patient satisfaction postoperative period priority journal survival treatment outcome LA - English M3 - Article N1 - L32210399 2001-04-27 PY - 2001 SN - 0003-4975 SP - 907-910 ST - Long-term results of relief of subaortic stenosis in univentricular atrioventricular connection with discordant ventriculoarterial connections T2 - Annals of Thoracic Surgery TI - Long-term results of relief of subaortic stenosis in univentricular atrioventricular connection with discordant ventriculoarterial connections UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L32210399 http://dx.doi.org/10.1016/S0003-4975(00)02544-3 VL - 71 ID - 1323 ER - TY - JOUR AB - Modifications of the Fontan operation can afford excellent palliation for many patients with a variety of forms of single ventricle. In properly selected patients who are good candidates, early and late survival can be as high as 95-97%. However, mortality is considerably higher for patients with risk factors for poor outcome. For high-risk patients, inclusion of a fenestration may reduce morbidity and mortality. Important long-term undesirable events include the ongoing risk of death, arrhythmias, protein-losing enteropathy, and cardiac failure. The key to excellent long-term outcome is proper selection of patients for the operation. AD - Division of Pediatric Cardiology, Mayo Clinic, 200 1st Street S.W., Rochester, MN 55905, USA. driscoll.david@mayo.edu AN - 17768650 AU - Driscoll, D. J. DA - Nov-Dec DB - PubMed DO - 10.1007/s00246-007-9003-4 DP - NLM ET - 2007/09/05 IS - 6 KW - Adolescent Arrhythmias, Cardiac/*etiology Child Child, Preschool Fontan Procedure/*adverse effects/*methods Humans Infant Protein-Losing Enteropathies/*etiology Quality of Life Risk Factors Survival Analysis Thromboembolism/*etiology Treatment Outcome LA - eng N1 - Driscoll, D J Journal Article Review United States Pediatr Cardiol. 2007 Nov-Dec;28(6):438-42. doi: 10.1007/s00246-007-9003-4. PY - 2007 SN - 0172-0643 (Print) 0172-0643 SP - 438-42 ST - Long-term results of the Fontan operation T2 - Pediatr Cardiol TI - Long-term results of the Fontan operation VL - 28 ID - 323 ER - TY - JOUR AB - Pulmonary arterial (PA) flow is abnormal after the Fontan operation and is marked by a lack of pulsatility. We assessed the effects of this abnormal flow on the size and function of the PA's in Fontan patients in long-term serial follow-up. Twenty-three Fontan patients with serial follow-up were included. Median age was 11.1 (9.5-16.0) years at baseline and 15.5 (12.5-22.7) years at follow-up. Median follow-up duration was 4.4 (4.0-5.8) years. Flow and size of the left pulmonary artery were determined using phase-contrast MRI. From this wall shear stress (WSS), distensibility and pulsatility were determined. A group of healthy peers was included for reference. Flow and pulsatility were significantly lower in patients than in controls (p < 0.001). Mean area was comparable in patients and controls, but distensibility was significantly higher in controls (p < 0.001). Mean and peak WSS were significantly lower in Fontan patients (p < 0.001). Between baseline and follow-up, there was a significant increase in normalized flow (15.1 (14.3-19.1) to 18.7 (14.0-22.6) ml/s/m(2), p = 0.023). Area, pulsatility, distensibility and WSS did not change, but there was a trend toward a lower mean WSS (p = 0.068). Multivariable regression analysis showed that flow, area and age were important predictors for WSS. WSS in Fontan patients is decreased compared to healthy controls and tends to decrease further with age. Pulsatility and distensibility are significantly lower compared to healthy controls. Pulmonary artery size, however, is not significantly different from healthy controls and long-term growth after Fontan operation is proportionate to body size. AD - Division of Cardiology, Department of Pediatrics, Erasmus Medical Centre, Sophia Children's Hospital, Sp-2429, PO Box 2060, 3000 CB, Rotterdam, The Netherlands. Department of Radiology, Erasmus Medical Centre, Rotterdam, The Netherlands. Division of Cardiology, Department of Biomedical Engineering, Erasmus Medical Centre, Rotterdam, The Netherlands. Department of Pediatric Cardiology, Radboud University Medical Centre, Nijmegen, The Netherlands. Department of Pediatrics, Pediatric Cardiology Unit, Tel-Aviv Sourasky Medical Centre, Tel Aviv, Israel. Department of Radiology, Amsterdam Academic Medical Center, Amsterdam, The Netherlands. Department of Radiology, Radboud University Medical Centre, Nijmegen, The Netherlands. Division of Cardiology, Department of Pediatrics, Erasmus Medical Centre, Sophia Children's Hospital, Sp-2429, PO Box 2060, 3000 CB, Rotterdam, The Netherlands. w.a.helbing@erasmusmc.nl. Department of Radiology, Erasmus Medical Centre, Rotterdam, The Netherlands. w.a.helbing@erasmusmc.nl. AN - 26757738 AU - Bossers, S. S. AU - Cibis, M. AU - Kapusta, L. AU - Potters, W. V. AU - Snoeren, M. M. AU - Wentzel, J. J. AU - Moelker, A. AU - Helbing, W. A. C2 - PMC4826404 DA - Apr DB - PubMed DO - 10.1007/s00246-015-1326-y DP - NLM ET - 2016/01/14 IS - 4 KW - Adolescent *Blood Flow Velocity Case-Control Studies Child Cross-Sectional Studies Female Follow-Up Studies Fontan Procedure/*adverse effects Humans Linear Models Magnetic Resonance Imaging Male Multivariate Analysis Netherlands Pulmonary Artery/*diagnostic imaging/physiopathology *Pulsatile Flow Young Adult Cardiac magnetic resonance imaging Fontan Pulmonary arteries Shear stress Tcpc LA - eng N1 - 1432-1971 Bossers, Sjoerd S M Cibis, Merih Kapusta, Livia Potters, Wouter V Snoeren, Miranda M Wentzel, Jolanda J Moelker, Adriaan Helbing, Willem A Journal Article Multicenter Study Research Support, Non-U.S. Gov't Pediatr Cardiol. 2016 Apr;37(4):637-45. doi: 10.1007/s00246-015-1326-y. Epub 2016 Jan 12. PY - 2016 SN - 0172-0643 (Print) 0172-0643 SP - 637-45 ST - Long-Term Serial Follow-Up of Pulmonary Artery Size and Wall Shear Stress in Fontan Patients T2 - Pediatr Cardiol TI - Long-Term Serial Follow-Up of Pulmonary Artery Size and Wall Shear Stress in Fontan Patients VL - 37 ID - 214 ER - TY - JOUR AB - Pulmonary arterial (PA) flow is abnormal after the Fontan operation and is marked by a lack of pulsatility. We assessed the effects of this abnormal flow on the size and function of the PA’s in Fontan patients in long-term serial follow-up. Twenty-three Fontan patients with serial follow-up were included. Median age was 11.1 (9.5–16.0) years at baseline and 15.5 (12.5–22.7) years at follow-up. Median follow-up duration was 4.4 (4.0–5.8) years. Flow and size of the left pulmonary artery were determined using phase-contrast MRI. From this wall shear stress (WSS), distensibility and pulsatility were determined. A group of healthy peers was included for reference. Flow and pulsatility were significantly lower in patients than in controls (p < 0.001). Mean area was comparable in patients and controls, but distensibility was significantly higher in controls (p < 0.001). Mean and peak WSS were significantly lower in Fontan patients (p < 0.001). Between baseline and follow-up, there was a significant increase in normalized flow (15.1 (14.3–19.1) to 18.7 (14.0–22.6) ml/s/m2, p = 0.023). Area, pulsatility, distensibility and WSS did not change, but there was a trend toward a lower mean WSS (p = 0.068). Multivariable regression analysis showed that flow, area and age were important predictors for WSS. WSS in Fontan patients is decreased compared to healthy controls and tends to decrease further with age. Pulsatility and distensibility are significantly lower compared to healthy controls. Pulmonary artery size, however, is not significantly different from healthy controls and long-term growth after Fontan operation is proportionate to body size. AD - W.A. Helbing, Division of Cardiology, Department of Pediatrics, Erasmus Medical Centre, Sophia Children’s Hospital, Sp-2429, PO Box 2060, Rotterdam, Netherlands AU - Bossers, S. S. M. AU - Cibis, M. AU - Kapusta, L. AU - Potters, W. V. AU - Snoeren, M. M. AU - Wentzel, J. J. AU - Moelker, A. AU - Helbing, W. A. DB - Embase Medline DO - 10.1007/s00246-015-1326-y IS - 4 KW - adolescent artery blood flow artery compliance artery diameter article atriopulmonary connection cardiovascular magnetic resonance cavopulmonary connection child clinical article controlled study cross-sectional study female follow up Fontan procedure heart right ventricle double outlet human hypoplastic left heart syndrome long term care male multicenter study (topic) pulmonary artery pulse wave school child shear stress tricuspid valve atresia LA - English M3 - Article N1 - L607781155 2016-01-26 2016-05-05 PY - 2016 SN - 1432-1971 0172-0643 SP - 637-645 ST - Long-Term Serial Follow-Up of Pulmonary Artery Size and Wall Shear Stress in Fontan Patients T2 - Pediatric Cardiology TI - Long-Term Serial Follow-Up of Pulmonary Artery Size and Wall Shear Stress in Fontan Patients UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L607781155 http://dx.doi.org/10.1007/s00246-015-1326-y VL - 37 ID - 812 ER - TY - JOUR AB - Objective: To report the successful treatment of plastic bronchitis with aerosolized tissue plasminogen activator. Design: Case report. Patients: A 4-yr-old boy with congenital heart disease, who developed plastic bronchitis 33 months after a Fontan operation Interventions: Long-term treatment with aerosolized tissue plasminogen activator. Measurements and Main Results: We describe the case of a boy who developed recurrent episodes of life-threatening airway obstruction secondary to plastic bronchitis. Following the failure of multiple therapeutic interventions, his condition improved significantly with aerosolized tissue plasminogen activator. Several attempts to wean him off this treatment resulted in clinical deterioration. He has remained on long-term aerosolized tissue plasminogen activator. Conclusion: Treatment of plastic bronchitis with aerosolized tissue plasminogen activator may benefit patients in whom other therapies have failed. Copyright © 2005 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies. AD - Pediatric Critical Care, Advocate Hope Children's Hospital, Oak Lawn, IL, United States Pediatric Cardiology, Advocate Hope Children's Hospital, Oak Lawn, IL, United States Pediatric Pulmonology, Advocate Hope Children's Hospital, Oak Lawn, IL, United States Division of Critical Care, Department of Pediatrics, Hope Children's Hospital, 4440 W. 95th Street, Oak Lawn, IL 60453, United States AU - Wakeham, M. K. AU - Van Bergen, A. H. AU - Torero, L. E. AU - Akhter, J. DB - Scopus DO - 10.1097/01.PCC.0000149320.06424.1D IS - 1 KW - Airway obstruction Bronchial casts Congenital heart disease Fontan operation Plastic bronchitis Tissue plasminogen activator M3 - Article N1 - Cited By :40 Export Date: 15 June 2020 PY - 2005 SP - 76-78 ST - Long-term treatment of plastic bronchitis with aerosolized tissue plasminogen activator in a Fontan patient T2 - Pediatric Critical Care Medicine TI - Long-term treatment of plastic bronchitis with aerosolized tissue plasminogen activator in a Fontan patient UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-21744458795&doi=10.1097%2f01.PCC.0000149320.06424.1D&partnerID=40&md5=1a962f390ee01d1f4e20ac4d0fc5a194 VL - 6 ID - 2244 ER - TY - JOUR AB - Background-We sought to characterize growth between birth and age 3 years in infants with hypoplastic left heart syndrome who underwent the Norwood procedure. Methods and Results-We performed a secondary analysis using the Single Ventricle Reconstruction Trial database after excluding patients < 37 weeks gestation (N=498). We determined length-for-age z score (LAZ) and weight-for-age z score (WAZ) at birth and age 3 years and change in WAZ over 4 clinically relevant time periods. We identified correlates of change in WAZ and LAZ using multivariable linear regression with bootstrapping. Mean WAZ and LAZ were below average relative to the general population at birth (P < 0.001, P=0.05, respectively) and age 3 years (P < 0.001 each). The largest decrease in WAZ occurred between birth and Norwood discharge; the greatest gain occurred between stage II and 14 months. At age 3 years, WAZ and LAZ were < -2 in 6% and 18%, respectively. Factors associated with change in WAZ differed among time periods. Shunt type was associated with change in WAZ only in the Norwood discharge to stage II period; subjects with a Blalock-Taussig shunt had a greater decline in WAZ than those with a right ventricle-pulmonary artery shunt (P=0.002). Conclusions-WAZ changed over time and the predictors of change in WAZ varied among time periods. By age 3 years, subjects remained small and three times as many children were short as were underweight (> 2 SD below normal). Failure to find consistent risk factors supports the strategy of tailoring nutritional therapies to patient- and stage-specific targets. © 2014 The Authors. AD - Department of Surgery, University of Utah, Salt Lake City, UT, United States Department of Pediatrics, University of Utah, Salt Lake City, UT, United States New England Research Institutes, Watertown, MA, United States The Children's Hospital of Philadelphia, Philadelphia, PA, United States The Children's Hospital of Wisconsin, Milwaukee, WI, United States Nemours/A.I. DuPont Hospital for Children, Wilmington, DE, United States Children's Hospital Boston and Harvard Medical School, Boston, MA, United States The Hospital for Sick Children, Toronto, ON, Canada Columbia University Medical Center, New York, NY, United States Duke University Medical Center, Durham, NC, United States University of Michigan Medical School, Ann Arbor, MI, United States University of Cincinnati, Cincinnati, OH, United States National Institutes of Health, Bethesda, MD, United States University of California Los Angeles, Los Angeles, CA, United States Medical University of South Carolina, Charleston, SC, United States AU - Burch, P. T. AU - Gerstenberger, E. AU - Ravishankar, C. AU - Hehir, D. A. AU - Davies, R. R. AU - Colan, S. D. AU - Sleeper, L. A. AU - Newburger, J. W. AU - Clabby, M. L. AU - Williams, I. A. AU - Li, J. S. AU - Uzark, K. AU - Cooper, D. S. AU - Lambert, L. M. AU - Pemberton, V. L. AU - Pike, N. A. AU - Anderson, J. B. AU - Dunbar-Masterson, C. AU - Khaikin, S. AU - Zyblewski, S. C. AU - Minich, L. L. AU - Pediatric Heart Network, Investigators C7 - 000079 DB - Scopus DO - 10.1161/JAHA.114.000079 IS - 3 KW - Growth Hypoplastic left heart syndrome Pediatrics Risk factors M3 - Article N1 - Cited By :27 Export Date: 15 June 2020 PY - 2014 ST - Longitudinal assessment of growth in hypoplastic left heart syndrome: Results from the Single Ventricle Reconstruction Trial T2 - Journal of the American Heart Association TI - Longitudinal assessment of growth in hypoplastic left heart syndrome: Results from the Single Ventricle Reconstruction Trial UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84923904901&doi=10.1161%2fJAHA.114.000079&partnerID=40&md5=11a62db55ca5ba129b9264411d0bc2dd VL - 3 ID - 2006 ER - TY - JOUR AB - Aim: To examine the association of the severity of congenital heart defects (CHDs) with internalizing problems in 18-month-olds and to explore the extent to which the internalizing problems are influenced by maternal distress and emotional reactivity in the child at age 6 months. Methods: We linked prospective data from the Norwegian Mother and Child Cohort Study, conducted by the Norwegian Institute of Public Health, with a nationwide CHD registry and identified 198 18-month-olds with CHDs in a cohort of 47 692 toddlers. Maternal reports on the children's emotional reactivity at age 6 months, the children's internalizing problems (anxiety, sleep problems, emotional reactivity) at age 18 months and maternal distress were assessed by questionnaires. Results: We found an association at age 18 months between the severity of the CHD and anxiety but not sleep problems or emotional reactivity. Children with severe but not with mild or moderate CHDs were twice as likely to experience the symptoms of anxiety compared with controls. These symptoms are not merely sequelae of earlier psychological reactions or concurrent maternal distress. Conclusion: Should these findings be replicated, future studies ought to investigate the mechanisms leading to elevated anxiety in toddlers with CHDs. In addition, clinical interventions should address the child's anxiety as well as the interaction between the parents and the child. © 2010 Foundation Acta Pædiatrica. AD - K. Stene-Larsen, Department of Psychosomatics and Health Behavior, Division of Mental Health, Norwegian Institute of Public Health, PO Box 4404 Nydalen, 0403 Oslo, Norway AU - Stene-Larsen, K. AU - Brandlistuen, R. E. AU - Holmstrøm, H. AU - Landolt, M. A. AU - Eskedal, L. T. AU - Engdahl, B. AU - Vollrath, M. E. DB - Embase Medline DO - 10.1111/j.1651-2227.2010.02015.x IS - 2 KW - anxiety disorder aortic coarctation aortic stenosis article asthma birth weight brain malformation child chylothorax cleft lip cleft palate congenital heart malformation controlled study diabetes mellitus disease severity distress syndrome emotion esophagus atresia Fallot tetralogy female great vessels transposition heart atrium septum defect heart right ventricle double outlet heart single ventricle heart ventricle septum defect hemangioma human hydrocephalus hypoplastic left heart syndrome infant intestine malformation limb malformation lung vein drainage anomaly male maternal welfare micrognathia mood neoplasm Norway patent ductus arteriosus patent foramen ovale prematurity preschool child priority journal pulmonary subvalvular stenosis pulmonary valve atresia sleep disorder spherocytosis temperament LA - English M3 - Article N1 - L361096503 2011-01-20 2011-02-08 PY - 2011 SN - 0803-5253 1651-2227 SP - 236-241 ST - Longitudinal findings from a Norwegian case-cohort study on internalizing problems in children with congenital heart defects T2 - Acta Paediatrica, International Journal of Paediatrics TI - Longitudinal findings from a Norwegian case-cohort study on internalizing problems in children with congenital heart defects UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L361096503 http://dx.doi.org/10.1111/j.1651-2227.2010.02015.x VL - 100 ID - 1112 ER - TY - JOUR AB - BACKGROUND: Multicenter longitudinal objective data for survival into adulthood of patients who have undergone Fontan procedures are lacking. OBJECTIVES: This study sought to describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time. METHODS: Exercise testing, echocardiography, B-type natriuretic peptide, functional health assessment, and medical history abstraction were repeated 9.4 ± 0.4 years after the Fontan Cross-Sectional Study (Fontan 1) and compared with previous values. Cox regression analysis explored risk factors for interim death or cardiac transplantation. RESULTS: From the original cohort of 546 subjects, 466 were contacted again, and 373 (80%) were enrolled at 21.2 ± 3.5 years of age. Among subjects with paired testing, the percent predicted maximum oxygen uptake decreased (69 ± 14% vs. 61 ± 16%; p < 0.001; n = 95), ejection fraction decreased (58 ± 11% vs. 55 ± 10%; p < 0.001; n = 259), and B-type natriuretic peptide increased (median [interquartile range] 13 [7 to 25] pg/mol vs. 18 [9 to 36] pg/mol; p < 0.001; n = 340). At latest follow-up, a lower Pediatric Quality of Life Inventory physical summary score was associated with poorer exercise performance (R(2) adjusted = 0.20; p < 0.001; n = 274). Cumulative complications since the Fontan procedure included additional cardiac surgery (32%), catheter intervention (62%), arrhythmia treatment (32%), thrombosis (12%), and protein-losing enteropathy (8%). Since Fontan 1, 54 subjects (10%) have received a heart transplant (n = 23) or died without transplantation (n = 31). The interval risk of death or/cardiac transplantation was associated with poorer ventricular performance and functional health status assessed at Fontan 1, but it was not associated with ventricular morphology, the subject's age, or the type of Fontan connection. CONCLUSIONS: Interim transplant-free survival over 12 years in this Fontan cohort was 90% and was independent of ventricular morphology. Exercise performance decreased and was associated with worse functional health status. Future interventions might focus on preserving exercise capacity. (Relationship Between Functional Health Status and Ventricular Performance After Fontan-Pediatric Heart Network; NCT00132782). AD - Medical University of South Carolina, Charleston, South Carolina. Electronic address: atzam@musc.edu. New England Research Institutes, Watertown, Massachusetts. University of Texas Southwestern Medical Center, Dallas, Texas. University of Michigan, Ann Arbor, Michigan. Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. University of Utah, Salt Lake City, Utah. Boston Children's Hospital, Boston, Massachusetts. National Heart, Lung, and Blood Institute, Bethesda, Maryland. Duke University Medical Center, Durham, North Carolina. Columbia University Medical Center, New York, New York. Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada. AN - 28571639 AU - Atz, A. M. AU - Zak, V. AU - Mahony, L. AU - Uzark, K. AU - D'Agincourt, N. AU - Goldberg, D. J. AU - Williams, R. V. AU - Breitbart, R. E. AU - Colan, S. D. AU - Burns, K. M. AU - Margossian, R. AU - Henderson, H. T. AU - Korsin, R. AU - Marino, B. S. AU - Daniels, K. AU - McCrindle, B. W. C2 - PMC5604334 C6 - NIHMS867092 DA - Jun 6 DB - PubMed DO - 10.1016/j.jacc.2017.03.582 DP - NLM ET - 2017/06/03 IS - 22 KW - Adolescent Child Cross-Sectional Studies Echocardiography Female Follow-Up Studies Fontan Procedure/methods *Forecasting *Health Status Heart Defects, Congenital/diagnosis/mortality/*surgery Heart Ventricles/*abnormalities/diagnostic imaging Humans Male Ontario/epidemiology *Quality of Life Retrospective Studies Risk Factors Survival Rate/trends Treatment Outcome United States/epidemiology Young Adult Fontan procedure adult congenital heart disease exercise functional health status single ventricle LA - eng N1 - 1558-3597 Atz, Andrew M Zak, Victor Mahony, Lynn Uzark, Karen D'agincourt, Nicholas Goldberg, David J Williams, Richard V Breitbart, Roger E Colan, Steven D Burns, Kristin M Margossian, Renee Henderson, Heather T Korsin, Rosalind Marino, Bradley S Daniels, Kaitlyn McCrindle, Brian W Pediatric Heart Network Investigators U10 HL109818/HL/NHLBI NIH HHS/United States UG1 HL135680/HL/NHLBI NIH HHS/United States UG1 HL135646/HL/NHLBI NIH HHS/United States UG1 HL135685/HL/NHLBI NIH HHS/United States U01 HL068269/HL/NHLBI NIH HHS/United States U10 HL109816/HL/NHLBI NIH HHS/United States U01 HL068279/HL/NHLBI NIH HHS/United States UG1 HL135689/HL/NHLBI NIH HHS/United States U10 HL109743/HL/NHLBI NIH HHS/United States U01 HL068290/HL/NHLBI NIH HHS/United States U24 HL135691/HL/NHLBI NIH HHS/United States U01 HL068288/HL/NHLBI NIH HHS/United States U10 HL068270/HL/NHLBI NIH HHS/United States U01 HL068281/HL/NHLBI NIH HHS/United States U01 HL068270/HL/NHLBI NIH HHS/United States UL1 TR001117/TR/NCATS NIH HHS/United States U10 HL109777/HL/NHLBI NIH HHS/United States U01 HL068292/HL/NHLBI NIH HHS/United States U10 HL109778/HL/NHLBI NIH HHS/United States U01 HL068285/HL/NHLBI NIH HHS/United States Journal Article Multicenter Study J Am Coll Cardiol. 2017 Jun 6;69(22):2735-2744. doi: 10.1016/j.jacc.2017.03.582. PY - 2017 SN - 0735-1097 (Print) 0735-1097 SP - 2735-2744 ST - Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure T2 - J Am Coll Cardiol TI - Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure VL - 69 ID - 9 ER - TY - JOUR AB - Background Multicenter longitudinal objective data for survival into adulthood of patients who have undergone Fontan procedures are lacking. Objectives This study sought to describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time. Methods Exercise testing, echocardiography, B-type natriuretic peptide, functional health assessment, and medical history abstraction were repeated 9.4 ± 0.4 years after the Fontan Cross-Sectional Study (Fontan 1) and compared with previous values. Cox regression analysis explored risk factors for interim death or cardiac transplantation. Results From the original cohort of 546 subjects, 466 were contacted again, and 373 (80%) were enrolled at 21.2 ± 3.5 years of age. Among subjects with paired testing, the percent predicted maximum oxygen uptake decreased (69 ± 14% vs. 61 ± 16%; p < 0.001; n = 95), ejection fraction decreased (58 ± 11% vs. 55 ± 10%; p < 0.001; n = 259), and B-type natriuretic peptide increased (median [interquartile range] 13 [7 to 25] pg/mol vs. 18 [9 to 36] pg/mol; p < 0.001; n = 340). At latest follow-up, a lower Pediatric Quality of Life Inventory physical summary score was associated with poorer exercise performance (R2 adjusted = 0.20; p < 0.001; n = 274). Cumulative complications since the Fontan procedure included additional cardiac surgery (32%), catheter intervention (62%), arrhythmia treatment (32%), thrombosis (12%), and protein-losing enteropathy (8%). Since Fontan 1, 54 subjects (10%) have received a heart transplant (n = 23) or died without transplantation (n = 31). The interval risk of death or/cardiac transplantation was associated with poorer ventricular performance and functional health status assessed at Fontan 1, but it was not associated with ventricular morphology, the subject's age, or the type of Fontan connection. Conclusions Interim transplant-free survival over 12 years in this Fontan cohort was 90% and was independent of ventricular morphology. Exercise performance decreased and was associated with worse functional health status. Future interventions might focus on preserving exercise capacity. (Relationship Between Functional Health Status and Ventricular Performance After Fontan—Pediatric Heart Network; NCT00132782) © 2017 American College of Cardiology Foundation AD - Medical University of South Carolina, Charleston, South Carolina, United States New England Research Institutes, Watertown, Massachusetts, United States University of Texas Southwestern Medical Center, Dallas, Texas, United States University of Michigan, Ann Arbor, Michigan, United States Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States University of Utah, Salt Lake City, Utah, United States Boston Children's Hospital, Boston, Massachusetts, United States National Heart, Lung, and Blood Institute, Bethesda, Maryland, United States Duke University Medical Center, Durham, North Carolina, United States Columbia University Medical Center, New York, New York, United States Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, United States University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada AU - Atz, A. M. AU - Zak, V. AU - Mahony, L. AU - Uzark, K. AU - D'Agincourt, N. AU - Goldberg, D. J. AU - Williams, R. V. AU - Breitbart, R. E. AU - Colan, S. D. AU - Burns, K. M. AU - Margossian, R. AU - Henderson, H. T. AU - Korsin, R. AU - Marino, B. S. AU - Daniels, K. AU - McCrindle, B. W. AU - Pediatric Heart Network, Investigators DB - Scopus DO - 10.1016/j.jacc.2017.03.582 IS - 22 KW - adult congenital heart disease exercise Fontan procedure functional health status single ventricle M3 - Article N1 - Cited By :57 Export Date: 15 June 2020 PY - 2017 SP - 2735-2744 ST - Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure T2 - Journal of the American College of Cardiology TI - Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85019735251&doi=10.1016%2fj.jacc.2017.03.582&partnerID=40&md5=372262e9e2fa0c0a2d6fecf272803f7b VL - 69 ID - 1857 ER - TY - JOUR AB - Background: Multicenter longitudinal objective data for survival into adulthood of patients who have undergone Fontan procedures are lacking.Objectives: This study sought to describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time.Methods: Exercise testing, echocardiography, B-type natriuretic peptide, functional health assessment, and medical history abstraction were repeated 9.4 ± 0.4 years after the Fontan Cross-Sectional Study (Fontan 1) and compared with previous values. Cox regression analysis explored risk factors for interim death or cardiac transplantation.Results: From the original cohort of 546 subjects, 466 were contacted again, and 373 (80%) were enrolled at 21.2 ± 3.5 years of age. Among subjects with paired testing, the percent predicted maximum oxygen uptake decreased (69 ± 14% vs. 61 ± 16%; p < 0.001; n = 95), ejection fraction decreased (58 ± 11% vs. 55 ± 10%; p < 0.001; n = 259), and B-type natriuretic peptide increased (median [interquartile range] 13 [7 to 25] pg/mol vs. 18 [9 to 36] pg/mol; p < 0.001; n = 340). At latest follow-up, a lower Pediatric Quality of Life Inventory physical summary score was associated with poorer exercise performance (R2 adjusted = 0.20; p < 0.001; n = 274). Cumulative complications since the Fontan procedure included additional cardiac surgery (32%), catheter intervention (62%), arrhythmia treatment (32%), thrombosis (12%), and protein-losing enteropathy (8%). Since Fontan 1, 54 subjects (10%) have received a heart transplant (n = 23) or died without transplantation (n = 31). The interval risk of death or/cardiac transplantation was associated with poorer ventricular performance and functional health status assessed at Fontan 1, but it was not associated with ventricular morphology, the subject's age, or the type of Fontan connection.Conclusions: Interim transplant-free survival over 12 years in this Fontan cohort was 90% and was independent of ventricular morphology. Exercise performance decreased and was associated with worse functional health status. Future interventions might focus on preserving exercise capacity. (Relationship Between Functional Health Status and Ventricular Performance After Fontan-Pediatric Heart Network; NCT00132782). AD - Medical University of South Carolina, Charleston, South Carolina New England Research Institutes, Watertown, Massachusetts University of Texas Southwestern Medical Center, Dallas, Texas University of Michigan, Ann Arbor, Michigan Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania University of Utah, Salt Lake City, Utah Boston Children’s Hospital, Boston, Massachusetts National Heart, Lung, and Blood Institute, Bethesda, Maryland Duke University Medical Center, Durham, North Carolina Columbia University Medical Center, New York, New York Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada AN - 123130533. Corporate Author: Pediatric Heart Network Investigators. Language: English. Entry Date: 20170817. Revision Date: 20200222. Publication Type: journal article AU - Atz, Andrew M. AU - Zak, Victor AU - Mahony, Lynn AU - Uzark, Karen AU - D’agincourt, Nicholas AU - Goldberg, David J. AU - Williams, Richard V. AU - Breitbart, Roger E. AU - Colan, Steven D. AU - Burns, Kristin M. AU - Margossian, Renee AU - Henderson, Heather T. AU - Korsin, Rosalind AU - Marino, Bradley S. AU - Daniels, Kaitlyn AU - McCrindle, Brian W. AU - D'Agincourt, Nicholas DB - ccm DO - 10.1016/j.jacc.2017.03.582 DP - EBSCOhost IS - 22 KW - Heart Ventricle -- Abnormalities Quality of Life Heart Defects, Congenital -- Surgery Forecasting Health Status Ontario Child Survival -- Trends Heart Defects, Congenital -- Mortality Treatment Outcomes Retrospective Design Adolescence Male Human Cardiopulmonary Bypass -- Methods Risk Factors Young Adult Female United States Heart Defects, Congenital -- Diagnosis Cross Sectional Studies Prospective Studies Echocardiography Heart Ventricle Validation Studies Comparative Studies Evaluation Research Multicenter Studies Funding Source Questionnaires N1 - research. Journal Subset: Biomedical; Peer Reviewed; USA. Instrumentation: Pediatric Quality of Life Inventory physical summary. Grant Information: UG1 HL135689/HL/NHLBI NIH HHS/United States. NLM UID: 8301365. PMID: NLM28571639. PY - 2017 SN - 0735-1097 SP - 2735-2744 ST - Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure T2 - Journal of the American College of Cardiology (JACC) TI - Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=123130533&site=ehost-live&scope=site VL - 69 ID - 1471 ER - TY - JOUR AB - BACKGROUND: Growth abnormalities in single-ventricle survivors may reduce quality of life (QoL) and exercise capacity. METHODS: This multicenter, longitudinal analysis evaluated changes in height and body mass index (BMI) compared to population norms and their relationship to mortality, ventricular morphology, QoL, and exercise capacity in the Pediatric Heart Network Fontan studies. RESULTS: Fontan 1 (F1) included 546 participants (12 ± 3.4 years); Fontan 2 (F2), 427 (19 ± 3.4 years); and Fontan 3 (F3), 362 (21 ± 3.5 years), with ~60% male at each time point. Height z-score was -0.67 ± -1.27, -0.60 ± 1.34, and- 0.43 ± 1.14 at F1-F3, lower compared to norms at all time points (P ≤ .001). BMI z-score was similar to population norms. Compared to survivors, participants who died had lower height z-score (P ≤ .001). Participants with dominant right ventricle (n = 112) had lower height z-score (P ≤ .004) compared to dominant left (n = 186) or mixed (n = 64) ventricular morphologies. Higher height z-score was associated with higher Pediatric Quality of Life Inventory for the total score (slope = 2.82 ± 0.52; P ≤ .001). Increase in height z-score (F1 to F3) was associated with increased oxygen consumption (slope = 2.61 ± 1.08; P = .02), whereas, for participants >20 years old, an increase in BMI (F1 to F3) was associated with a decrease in oxygen consumption (slope = -1.25 ± 0.33; P ≤ .001). CONCLUSIONS: Fontan survivors, especially those with right ventricular morphology, are shorter when compared to the normal population but have similar BMI. Shorter stature was associated with worse survival. An increase in height z-score over the course of the study was associated with better QoL and exercise capacity; an increase in BMI was associated with worse exercise capacity. AD - University of Utah/Primary Children's Hospital, Salt Lake City, UT. Electronic address: linda.lambert@hsc.utah.edu. The Hospital for Sick Children, Toronto, Ontario, Canada. National Heart, Lung, and Blood Institute, NIH, Bethesda, MD. New England Research Institutes, Watertown, MA. Medical University of South Carolina, Charleston, SC. Children's Hospital of Philadelphia, Philadelphia, PA. Duke University Hospital, Durham, NC. Boston Children's Hospital, Boston, MA. University of Michigan/CS Mott Children's Hospital, Ann Arbor, MI. University of Utah/Primary Children's Hospital, Salt Lake City, UT. AN - 32428726 AU - Lambert, L. M. AU - McCrindle, B. W. AU - Pemberton, V. L. AU - Hollenbeck-Pringle, D. AU - Atz, A. M. AU - Ravishankar, C. AU - Campbell, M. J. AU - Dunbar-Masterson, C. AU - Uzark, K. AU - Rolland, M. AU - Trachtenberg, F. L. AU - Menon, S. C. C2 - PMC7293556 C6 - NIHMS1581927 DA - Jun DB - PubMed DO - 10.1016/j.ahj.2020.03.022 DP - NLM ET - 2020/05/20 LA - eng N1 - 1097-6744 Lambert, Linda M McCrindle, Brian W Pemberton, Victoria L Hollenbeck-Pringle, Danielle Atz, Andrew M Ravishankar, Chitra Campbell, M Jay Dunbar-Masterson, Carolyn Uzark, Karen Rolland, Martha Trachtenberg, Felicia L Menon, Shaji C Pediatric Heart Network Investigators U10 HL109818/HL/NHLBI NIH HHS/United States U10 HL109741/HL/NHLBI NIH HHS/United States U10 HL109781/HL/NHLBI NIH HHS/United States U10 HL109743/HL/NHLBI NIH HHS/United States U01 HL068290/HL/NHLBI NIH HHS/United States U01 HL068270/HL/NHLBI NIH HHS/United States U10 HL109737/HL/NHLBI NIH HHS/United States U10 HL109777/HL/NHLBI NIH HHS/United States U10 HL109778/HL/NHLBI NIH HHS/United States U10 HL109673/HL/NHLBI NIH HHS/United States Journal Article Am Heart J. 2020 Jun;224:192-200. doi: 10.1016/j.ahj.2020.03.022. Epub 2020 Apr 4. PY - 2020 SN - 0002-8703 (Print) 0002-8703 SP - 192-200 ST - Longitudinal study of anthropometry in Fontan survivors: Pediatric Heart Network Fontan study T2 - Am Heart J TI - Longitudinal study of anthropometry in Fontan survivors: Pediatric Heart Network Fontan study VL - 224 ID - 318 ER - TY - JOUR AB - Background: Growth abnormalities in single-ventricle survivors may reduce quality of life (QoL) and exercise capacity. Methods: This multicenter, longitudinal analysis evaluated changes in height and body mass index (BMI) compared to population norms and their relationship to mortality, ventricular morphology, QoL, and exercise capacity in the Pediatric Heart Network Fontan studies. Results: Fontan 1 (F1) included 546 participants (12 ± 3.4 years); Fontan 2 (F2), 427 (19 ± 3.4 years); and Fontan 3 (F3), 362 (21 ± 3.5 years), with ~60% male at each time point. Height z-score was −0.67 ± −1.27, −0.60 ± 1.34, and− 0.43 ± 1.14 at F1-F3, lower compared to norms at all time points (P ≤ .001). BMI z-score was similar to population norms. Compared to survivors, participants who died had lower height z-score (P ≤ .001). Participants with dominant right ventricle (n = 112) had lower height z-score (P ≤ .004) compared to dominant left (n = 186) or mixed (n = 64) ventricular morphologies. Higher height z-score was associated with higher Pediatric Quality of Life Inventory for the total score (slope = 2.82 ± 0.52; P ≤ .001). Increase in height z-score (F1 to F3) was associated with increased oxygen consumption (slope = 2.61 ± 1.08; P = .02), whereas, for participants >20 years old, an increase in BMI (F1 to F3) was associated with a decrease in oxygen consumption (slope = −1.25 ± 0.33; P ≤ .001). Conclusions: Fontan survivors, especially those with right ventricular morphology, are shorter when compared to the normal population but have similar BMI. Shorter stature was associated with worse survival. An increase in height z-score over the course of the study was associated with better QoL and exercise capacity; an increase in BMI was associated with worse exercise capacity. © 2020 Elsevier Inc. AD - University of Utah/Primary Children's Hospital, Salt Lake City, UT, United States The Hospital for Sick Children, Toronto, Ontario, Canada National Heart, Lung, and Blood Institute, NIH, Bethesda, MD, United States New England Research Institutes, Watertown MA Medical University of South Carolina, Charleston, SC, United States Children's Hospital of Philadelphia, Philadelphia, PA, United States Duke University Hospital, Durham, NC, United States Boston Children's Hospital, Boston, MA, United States University of Michigan/CS Mott Children's Hospital, Ann Arbor, MI, United States AU - Lambert, L. M. AU - McCrindle, B. W. AU - Pemberton, V. L. AU - Hollenbeck-Pringle, D. AU - Atz, A. M. AU - Ravishankar, C. AU - Campbell, M. J. AU - Dunbar-Masterson, C. AU - Uzark, K. AU - Rolland, M. AU - Trachtenberg, F. L. AU - Menon, S. C. AU - For the Pediatric Heart Network, Investigators DB - Scopus DO - 10.1016/j.ahj.2020.03.022 M3 - Article N1 - Export Date: 15 June 2020 PY - 2020 SP - 192-200 ST - Longitudinal study of anthropometry in Fontan survivors: Pediatric Heart Network Fontan study T2 - American Heart Journal TI - Longitudinal study of anthropometry in Fontan survivors: Pediatric Heart Network Fontan study UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85084644366&doi=10.1016%2fj.ahj.2020.03.022&partnerID=40&md5=35bbc9a6de9ec3dffe033d6e955a63a6 VL - 224 ID - 1698 ER - TY - JOUR AB - Purpose: This study aims to identify the prevalence of Congenital abdominal wall defects (AWD), hospital outcomes, and related congenital abnormalities in Taiwan by using the National Health Insurance Research Database (NHIRD). Materials and methods: From 1998 through 2013, all pediatric patients with AWD were collected via ICD-9-CM diagnostic code 756.7x (“congenital anomalies of abdominal wall”) or procedure codes (54.71, 54.72 for Gastroschisis repair (GS-repair); 54.63, 53.41, 53.49 for other abdominal wall repair (O-AWD)) recoded in NHIRD. We used public national birth data for calculating the prevalence. We used CDC/BPA coding rubrics to identify accompanying congenital abnormalities. Results: A total 594 babies with AWD, including 179 patients in GS-repair group and 326 patients in O-AWD-repair group, are identified with 113.5 months median followed-up. The overall prevalence for AWD was 1.65 in 10,000 births and decreased over time. The prevalences of GS-repaired and O-AWD-repaired are 0.50 and 0.90 in 10,000 births. The 1-year-mortality rates of GS-repair and O-AWD-repair are 6.15% and 7.23%. 24.58% GS-repair patients and 30.06% O-AWD-repair patients have congenital abnormalities. 8.38% GS-repair patients and 6.44% O-AWD-repair patients received the following groin hernia-repair procedures. Conclusion: Our results showed that the prevalence of AWD was low and decreased over time in Taiwan. Level of evidence: Level II: Prognosis Study, Retrospective study. AD - C.-H. Lee, Department of General Surgery, Buddhist Dalin Tzu Chi Hospital, Chia-Yi, Taiwan AU - Chen, M. C. AU - Chen, J. H. AU - Chen, Y. AU - Tsai, Y. H. AU - Lee, C. H. DB - Embase Medline DO - 10.1016/j.jpedsurg.2018.11.011 IS - 9 KW - abdominal wall defect anotia anus atresia aortic coarctation aortic valve stenosis article bile duct atresia bladder exstrophy cleft lip with or without cleft palate congenital cataract congenital hip dislocation diaphragm hernia Down syndrome encephalocele endocardial cushion defect epispadias esophagus atresia esophagus stenosis Fallot tetralogy female follow up gastroschisis great vessels transposition heart atrium septum defect heart ventricle septum defect Hirschsprung disease human hypospadias ICD-9-CM intestine atresia kidney agenesis major clinical study male microtia mortality rate national health insurance newborn obstructive genitourinary defect patent ductus arteriosus pediatric patient prevalence priority journal pulmonary valve atresia pulmonary valve stenosis pylorus stenosis renal dysgenesis spinal dysraphism Taiwan tracheoesophageal fistula tricuspid valve atresia tricuspid valve stenosis trisomy 13 trisomy 18 urogenital tract disease LA - English M3 - Article N1 - L2001522621 2019-02-06 2019-09-06 PY - 2019 SN - 1531-5037 0022-3468 SP - 1958-1964 ST - Low and decreased prevalence of congenital abdominal wall defect in Taiwan T2 - Journal of Pediatric Surgery TI - Low and decreased prevalence of congenital abdominal wall defect in Taiwan UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2001522621 http://dx.doi.org/10.1016/j.jpedsurg.2018.11.011 VL - 54 ID - 596 ER - TY - JOUR AB - Plastic bronchitis (PB) is an uncommon, potentially fatal disease, marked by endobronchial cast formation causing variable degrees of respiratory distress. Primary and secondary pulmonary lymphatic abnormalities have been identified among the underlying mechanisms of cast formation. We present a case of PB where lymphoscintigraphy demonstrated the underlying lymphatic defect. A 6-year-old Hispanic male with congenital heart disease (CHD; post-Fontan) presented with recurrent pneumonia, respiratory distress. Bronchoscopy showed inflamed hypervascular mucosa and thick mucus plugs; no casts were seen. Later, PB was diagnosed after the patient expectorated a bronchial cast. Cast analysis showed lymphocytic aggregates with mucin and fibrin. Lymphoscintigraphy revealed abnormal lymphatic collaterals and retrograde trace reflux into the superior mediastinum, a picture consistent with thoracic duct lymph leakage into the tracheobronchial tree. The pathogenesis of PB is not fully understood, especially in patients with CHD. Chyle in bronchial casts suggests abnormal lymphatic flow. Reports of lymph flow abnormalities, especially endobronchial lymph leakage in CHD are limited. Lymphoscintigraphy in our case demonstrated clear evidence of retrograde lymph reflux and leakage into the bronchial tree. The case presented suggests that in some patients following Fontan surgery, high intrathoracic lymphatic pressure and retrograde lymph flow may contribute to recurrent cast formation. Finding the underlying lymphatic abnormality helps in specific case management. Lymphoscintigraphy is a safer and easier method than lymphangiography. Surgical lymphatic-venous shunting may be possible in select cases. AD - Department of Pediatrics, University of Arizona, Tucson, Arizona 85724, USA. dezmigna@yahoo.com AN - 22949350 AU - Ezmigna, D. R. AU - Morgan, W. J. AU - Witte, M. H. AU - Brown, M. A. DA - May DB - PubMed DO - 10.1002/ppul.22673 DP - NLM ET - 2012/09/06 IS - 5 KW - Bronchitis/*diagnostic imaging/epidemiology/pathology/physiopathology Child Comorbidity Heart Defects, Congenital/epidemiology/surgery Humans Lymphatic System/pathology/physiopathology *Lymphoscintigraphy Male LA - eng N1 - 1099-0496 Ezmigna, Dima R Morgan, Wayne J Witte, Marlys H Brown, Mark A Case Reports Journal Article United States Pediatr Pulmonol. 2013 May;48(5):515-8. doi: 10.1002/ppul.22673. Epub 2012 Sep 4. PY - 2013 SN - 1099-0496 SP - 515-8 ST - Lymphoscintigraphy in plastic bronchitis, a pediatric case report T2 - Pediatr Pulmonol TI - Lymphoscintigraphy in plastic bronchitis, a pediatric case report VL - 48 ID - 501 ER - TY - JOUR AB - AIMS: Exercise limitation is common post-Fontan. Hybrid X-ray and magnetic resonance imaging (XMR) catheterization allows haemodynamic assessment by means of measurement of ventricular volumes and flow in major vessels with simultaneous invasive pressures. We aim to assess haemodynamic response to stress in patients with hypoplastic left heart syndrome (HLHS) post-Fontan. METHODS AND RESULTS: Prospective study of 13 symptomatic children (NHYA 2) with HLHS post-Fontan using XMR catheterization. Three conditions were applied: baseline (Stage 1), dobutamine at 10 µg/kg/min (Stage 2), and dobutamine at 20 µg/kg/min (Stage 3). Seven consecutive patients received inhaled nitric oxide (iNO) at peak stress. Control MRI data were from normal healthy adults. In the HLHS patients, baseline mean pulmonary vascular resistance (PVR) was 1.51 ± 0.59 WU m(2) and aortopulmonary collateral flow was 17.7 ± 13.6% of systemic cardiac output. Mean right ventricular end-diastolic pressure was 6.7 ± 2.5 mmHg which did not rise with stress. Cardiac index (CI) increased at Stage 2 in HLHS (40%) and controls (61%) but continued to increase at Stage 3 only in controls (19%) but not in HLHS. The blunted rise in CI in HLHS was due to a continuing fall in end-diastolic volume throughout stress, with no significant change in PVR or CI at peak stress in response to iNO. CONCLUSION: Cardiac output post-Fontan in HLHS at peak stress is blunted due to a limitation in preload which is not responsive to inhaled pulmonary vasodilators in the setting of normal PVR. AD - Division of Imaging Sciences, King's College London BHF Centre, NIHR Biomedical Research Centre at Guy's and St Thomas' NHS Foundation Trust, London, UK Department of Congenital Heart Disease, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK. Division of Imaging Sciences, King's College London BHF Centre, NIHR Biomedical Research Centre at Guy's and St Thomas' NHS Foundation Trust, London, UK. Department of Congenital Heart Disease, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK. University of Nebraska/Creighton University Joint Division of Pediatric Cardiology, Omaha, NE, USA. Division of Imaging Sciences, King's College London BHF Centre, NIHR Biomedical Research Centre at Guy's and St Thomas' NHS Foundation Trust, London, UK Department of Congenital Heart Disease, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK Paediatric Cardiovascular Sciences, Rayne Institute, King's College London and Evelina London Children's Hospital, Westminster Bridge Road, London SE1 7EH, UK reza.razavi@kcl.ac.uk. AN - 26188193 AU - Pushparajah, K. AU - Wong, J. K. AU - Bellsham-Revell, H. R. AU - Hussain, T. AU - Valverde, I. AU - Bell, A. AU - Tzifa, A. AU - Greil, G. AU - Simpson, J. M. AU - Kutty, S. AU - Razavi, R. C2 - PMC4871233 DA - Jun DB - PubMed DO - 10.1093/ehjci/jev178 DP - NLM ET - 2015/07/19 IS - 6 KW - Age Factors Analysis of Variance Cardiac Catheterization/methods Case-Control Studies Child Child, Preschool *Echocardiography, Stress Female Fontan Procedure/adverse effects/*methods Hemodynamics/*physiology Humans Hypoplastic Left Heart Syndrome/*diagnostic imaging/*surgery Magnetic Resonance Imaging Male Monitoring, Physiologic/methods Prognosis Risk Assessment Sex Factors Vascular Resistance/*physiology Fontan Mri dobutamine hypoplastic left heart syndrome pulmonary vascular resistance LA - eng N1 - 2047-2412 Pushparajah, Kuberan Wong, James K Bellsham-Revell, Hannah R Hussain, Tarique Valverde, Israel Bell, Aaron Tzifa, Aphrodite Greil, Gerald Simpson, John M Kutty, Shelby Razavi, Reza MR/J006742/1/Medical Research Council/United Kingdom Journal Article Eur Heart J Cardiovasc Imaging. 2016 Jun;17(6):644-51. doi: 10.1093/ehjci/jev178. Epub 2015 Jul 18. PY - 2016 SN - 2047-2404 (Print) 2047-2404 SP - 644-51 ST - Magnetic resonance imaging catheter stress haemodynamics post-Fontan in hypoplastic left heart syndrome T2 - Eur Heart J Cardiovasc Imaging TI - Magnetic resonance imaging catheter stress haemodynamics post-Fontan in hypoplastic left heart syndrome VL - 17 ID - 117 ER - TY - JOUR AB - Background: Patterns of heart diseases differ between the high income countries and low income countries.1, 2 Acquired heart diseases like RHD, cardiomyopathies, pericarditis are still a public health burden while congenital heart diseases still carry a poor outcome among children3. The absence of early diagnosis, surgical intervention and inadequate access to medication and the existing burden of communicable diseases increases the strain on a staggering economy of most sub Saharan countries. Aim: To assess the clinical and echocardiographic pattern of cardiac disease in children in Kordofan state and to develop a road map plan of their further management. Methods: An analytical cross sectional hospital based study. The study included the children of age groups 1 day up to 18 years with diagnosed or strongly suspected congenital or acquired cardiac disease who have their follow up in the pediatric sections of El-Obied hospitals in the western central part of the Sudan. Every child in the study group was assessed clinically with focused history taken and relevant physical examination. Chest x-ray and proper echocardiography was done for every child in the study group. Management plan was put by the pediatric cardiologist for all the children in the study group. Eighty children were enrolled in this study. Results: 80 children studied, 55% were males and females represent 45%. Most of the children with cardiac disease in the study were from the age group 1-5 years (27.5%) followed by infants (26.2%).after that, 5-10 years, more than 10 years and less than one month. Nine patients (11.25%) were severely affected, 26 (32.5%) were moderately affected and the growth of 45 (56.25%) was normal. Clinical evaluation of the study group revealed that, 19 children (24%) presented with respiratory symptoms and /or respiratory distress. A cardiac murmur was heard in most of the patients in this series, systolic murmurs heard in 55 patients (69%) of which 21 were pansystolic (26%) and 8 were ejection systolic murmurs (10%). Hepatomegaly was found in 25 patients (31%) and Hepatosplenomegaly in 4 patients (5%). Down syndrome was found in 5 patients (6%), William syndrome in one patient. Congenital HD found in 56 patients (70%) and acquired HD in 24 (30%), 19 patients (79% of the acquired heart disease and 23.7% of the total study group was diagnosed as having rheumatic heart disease and cardiomyopathy was found in 5 patients (20.8% of acquired heart disease). Conclusion: Rheumatic cardiac disease is preventable through the proper and complete antibiotic treatment of streptococcal pharyngitis and tonsillitis and it is vital to apply and strict to the recommendations of the national program of prevention of rheumatic fever and rheumatic heart disease. AD - A.E.M. Mustafa, Department of Child Health, College of Medicine, King Khalid University, Saudi Arabia AU - Mustafa, A. E. M. AU - Tahir Ali, N. M. AU - Elrahim, S. Y. A. AU - Bharti, R. K. R. AU - Chaudhary, S. DB - Embase IS - 4 KW - arterial trunk article bacterial endocarditis cardiologist cardiomegaly cardiomyopathy child clinical feature dextrocardia echocardiography Fallot tetralogy female follow up heart disease heart left right shunt heart murmur heart single ventricle hepatomegaly hepatosplenomegaly human infant major clinical study male newborn physical examination pulmonary valve atresia pulmonary valve stenosis respiratory distress rheumatic fever rheumatic heart disease Sudan systolic heart murmur thorax radiography tricuspid valve atresia LA - English M3 - Article N1 - L2001674060 2019-03-14 2019-03-20 PY - 2018 SN - 1996-7195 SP - 1760-1765 ST - The major clinical presentations and echocardiographic features of structural cardiac disease among children in Kordofan, Sudan T2 - Pakistan Journal of Medical and Health Sciences TI - The major clinical presentations and echocardiographic features of structural cardiac disease among children in Kordofan, Sudan UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2001674060 VL - 12 ID - 711 ER - TY - JOUR AB - Previous studies have shown that over 40% of babies with Down syndrome have a major cardiac anomaly and are more likely to have other major congenital anomalies. Since 2000, many countries in Europe have introduced national antenatal screening programs for Down syndrome. This study aimed to determine if the introduction of these screening programs and the subsequent termination of prenatally detected pregnancies were associated with any decline in the prevalence of additional anomalies in babies born with Down syndrome. The study sample consisted of 7,044 live births and fetal deaths with Down syndrome registered in 28 European population-based congenital anomaly registries covering seven million births during 2000-2010. Overall, 43.6% (95% CI: 42.4-44.7%) of births with Down syndrome had a cardiac anomaly and 15.0% (14.2-15.8%) had a non-cardiac anomaly. Female babies with Down syndrome were significantly more likely to have a cardiac anomaly compared to male babies (47.6% compared with 40.4%, P<0.001) and significantly less likely to have a non-cardiac anomaly (12.9% compared with 16.7%, P<0.001). The prevalence of cardiac and non-cardiac congenital anomalies in babies with Down syndrome has remained constant, suggesting that population screening for Down syndrome and subsequent terminations has not influenced the prevalence of specific congenital anomalies in these babies. AD - J.K. Morris, Centre for Environmental and Preventive Medicine, Wolfson Institute of Preventive Medicine, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, Charterhouse Square, London, United Kingdom AU - Morris, J. K. AU - Garne, E. AU - Wellesley, D. AU - Addor, M. C. AU - Arriola, L. AU - Barisic, I. AU - Beres, J. AU - Bianchi, F. AU - Budd, J. AU - Dias, C. M. AU - Gatt, M. AU - Klungsoyr, K. AU - Khoshnood, B. AU - Latos-Bielenska, A. AU - Mullaney, C. AU - Nelen, V. AU - Neville, A. J. AU - O'Mahony, M. AU - Queisser-Luft, A. AU - Randrianaivo, H. AU - Rankin, J. AU - Rissmann, A. AU - Rounding, C. AU - Sipek, A. AU - Stoianova, S. AU - Tucker, D. AU - de Walle, H. AU - Yevtushok, L. AU - Loane, M. AU - Dolk, H. DB - Embase Medline DO - 10.1002/ajmg.a.36780 IS - 12 KW - abdominal wall defect annular pancreas anophthalmia anus atresia aortic coarctation aortic valve stenosis aortic valve atresia article atrioventricular septal defect bladder exstrophy choana atresia cleft lip cleft lip palate cleft palate congenital cataract congenital glaucoma congenital heart malformation congenital malformation congenital skin disease controlled study craniofacial synostosis diaphragm hernia digestive system malformation Down syndrome duodenum atresia duodenum stenosis ear malformation Ebstein anomaly epispadias esophagus atresia European eye malformation face malformation Fallot tetralogy female fetus death gender genital malformation great vessels transposition heart atrium septum defect heart single ventricle heart ventricle septum defect Hirschsprung disease human hydrocephalus hydronephrosis hypoplastic right heart hypospadias intestine atresia intestine stenosis kidney agenesis kidney dysplasia limb malformation limb reduction defect live birth major clinical study male microcephaly mouth malformation neck malformation nervous system malformation neural tube defect newborn omphalocele patent ductus arteriosus perinatal mortality polydactyly population research Potter syndrome prenatal screening prevalence priority journal prune belly syndrome pulmonary valve atresia pulmonary valve stenosis rectal stenosis register respiratory tract malformation syndactyly talipes equinovarus terminations of pregnancy for fetal anomaly tracheoesophageal fistula trend study tricuspid valve atresia tricuspid valve stenosis urinary tract malformation LA - English M3 - Article N1 - L600504661 2015-01-27 PY - 2014 SN - 1552-4833 1552-4825 SP - 2979-2986 ST - Major congenital anomalies in babies born with Down syndrome: A EUROCAT population-based registry study T2 - American Journal of Medical Genetics, Part A TI - Major congenital anomalies in babies born with Down syndrome: A EUROCAT population-based registry study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L600504661 http://dx.doi.org/10.1002/ajmg.a.36780 VL - 164 ID - 902 ER - TY - JOUR AB - Background: Extracardiac malformations may be present in patients with congenital heart disease (CHD), bringing greater risk of comorbidity and mortality. Objective: Verify frequency and types of abdominal abnormalities detected in children with and without CHD through abdominal ultrasound (AUS), compare the patients in relation to their dysmorphic/cytogenetic findings and perform an estimative of the cost-effectiveness of the screening through AUS. Methods: We conducted a cross-sectional study with a control cohort. The cases consisted of patients with CHD admitted for the first time in a pediatric intensive care unit; the controls consisted of children without CHD who underwent AUS at the hospital shortly thereafter a case. All patients with CHD underwent AUS, high-resolution karyotype and fluorescence in situ hybridization (FISH) for microdeletion 22q11.2. Results: AUS identified clinically significant abnormalities in 12.2% of the cases and 5.2% of controls (p= 0.009), with a power of significance of 76.6%. Most malformations with clinical significance were renal anomalies (10.4% in cases and 4.9% in controls; p= 0.034). In Brazil, the cost of an AUS examination for the Unified Health System is US$ 21. Since clinically significant abnormalities were observed in one in every 8.2 CHD patients, the cost to identify an affected child was calculated as approximately US$ 176. Conclusion: Patients with CHD present a significant frequency of abdominal abnormalities detected by AUS, an inexpensive and noninvasive diagnostic method with good sensitivity. The cost of screening for these defects is considerably lower than the cost to treat the complications of late diagnoses of abdominal malformations such as renal disease. AD - P. R. G. Zen, Rua Sarmento Leite, 245, 403, Centro, Postal Code 90050-170, Porto Alegre, RS, Brazil AU - Rosa, R. C. M. AU - Fabiano Machado Rosa, R. AU - Flores, J. A. M. AU - Golendziner, E. AU - De Oliveira, C. A. V. AU - Varella-Garcia, M. AU - Paskulin, G. A. AU - Zen, P. R. G. DB - Embase Medline DO - 10.1590/S0066-782X2012005000111 IS - 6 KW - aortic coarctation article atrioventricular septal defect child chromosome analysis chromosome deletion 22q11 cohort analysis congenital heart disease controlled study cost effectiveness analysis cross-sectional study cyanotic heart disease Down syndrome echography Fallot tetralogy female fluorescence in situ hybridization heart atrium septum defect heart right ventricle double outlet heart ventricle septum defect hospitalization human hypoplastic left heart syndrome infant karyotyping kidney malformation lung vein drainage anomaly major clinical study male newborn patent ductus arteriosus pulmonary valve atresia LA - English M3 - Article N1 - L368203096 2013-02-05 2013-02-12 http://www.scielo.br/pdf/abc/v99n6/en_aop10712.pdf PY - 2012 SN - 0066-782X 1678-4170 SP - 1092-1099 ST - Malformations detected by abdominal ultrasound in children with congenital heart disease T2 - Arquivos Brasileiros de Cardiologia TI - Malformations detected by abdominal ultrasound in children with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L368203096 http://dx.doi.org/10.1590/S0066-782X2012005000111 VL - 99 ID - 1044 ER - TY - JOUR AB - Background: We reviewed our entire programmatic operative experience with children with hypoplastic left heart syndrome (HLHS) and related malformations. Methods: As of October 1, 2007, 239 patients with HLHS and related malformations underwent surgical treatment at the Congenital Heart Institute of Florida. This manuscript focuses on the 199 initially treated with Norwood stage 1. Results: One hundred and ninety-nine patients were initially treated with Norwood stage 1. Univariate analysis demonstrated the following significant predictors of mortality: right ventricular dominance (p = 0.0023), mechanical circulatory support before stage 1 (p = 0.0192), and significant noncardiac abnormality or syndrome, including Down syndrome, Turner syndrome, heterotaxy, asplenia, polysplenia, biliary atresia, or other chromosomal abnormality (p < 0.0001). Multivariable logistic regression analysis revealed the presence of a significant noncardiac abnormality or syndrome or prematurity less than 35 weeks or mechanical circulatory support before stage 1 to be a significant predictor of mortality (p < 0.0001). Over the 14 years of this patient series, survival for the 157 "low-risk" patients managed with Norwood staged palliation (those patients without significant noncardiac abnormality or syndrome or prematurity less than 35 weeks or mechanical circulatory support prior to Stage 1) was 86%, 80%, and 69% at 30 days after Stage 1, hospital discharge after Stage 1, and 1 year of after Stage 1, respectively. Conclusions: Several treatment options are available for HLHS and related malformations. The appropriate treatment strategy must be matched to the individual patient, taking into consideration anatomic variables as well as other patient-specific characteristics. The majority of patients with HLHS and related malformations can undergo successful staged palliation with risk that varies according to several documented anatomic and patient-specific variables. © 2008 The Society of Thoracic Surgeons. AD - J.P. Jacobs, The Congenital Heart Institute of Florida (CHIF), All Children's Hospital/Children's Hospital of Tampa, University of South Florida College of Medicine, Saint Petersburg and Tampa, FL, United States AU - Jacobs, J. P. AU - O'Brien, S. M. AU - Chai, P. J. AU - Morell, V. O. AU - Lindberg, H. L. AU - Quintessenza, J. A. DB - Embase Medline DO - 10.1016/j.athoracsur.2008.01.057 IS - 5 KW - accessory spleen article asplenia assisted circulation bile duct atresia chromosome aberration congenital heart malformation Down syndrome heart surgery heterotaxy syndrome human hypoplastic left heart syndrome priority journal Turner syndrome univariate analysis LA - English M3 - Article N1 - L351608184 2008-06-03 PY - 2008 SN - 0003-4975 SP - 1691-1697 ST - Management of 239 Patients with Hypoplastic Left Heart Syndrome and Related Malformations from 1993 to 2007 T2 - Annals of Thoracic Surgery TI - Management of 239 Patients with Hypoplastic Left Heart Syndrome and Related Malformations from 1993 to 2007 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L351608184 http://dx.doi.org/10.1016/j.athoracsur.2008.01.057 VL - 85 ID - 1210 ER - TY - JOUR AB - The combination of hypoplastic left heart syndrome and a right-sided aortic arch is extremely rare and lethal. To the best of our knowledge, no patient with this combination has previously been reported as surviving initial palliation. The anatomic variant is associated with abnormalities in the arteries branching from the aortic arch, making it difficult to construct a reliable source of flow of blood to the lungs. We present here a patient with this combination who survived an initial Damus-Kay-Stansel procedure combined with placement of a conduit from the right ventricle to the pulmonary arteries, and who has subsequently undergone a successful bidirectional cavopulmonary anastomosis. We believe that the conduit placed from the right ventricle provides the most reliable source of flow of blood to the lungs at the time of initial palliation in this usual combination of cardiac lesions. © 2006 Cambridge University Press. AD - S.C. Park, Division of Cardiology, Children's Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh, PA 15213, United States AU - Tatum, T. H. AU - Morell, V. O. AU - Park, S. C. DB - Embase Medline DO - 10.1017/S1047951106000448 IS - 5 KW - politef anastomosis aorta anomaly aortic arch anomaly aortic surgery article artificial ventilation atresia case report cava vein cocaine dependence echocardiography endocardial fibroelastosis endotracheal intubation heart catheterization heart right ventricle horseshoe kidney human hydramnios hypoplastic left heart syndrome lung blood flow male mitral valve stenosis newborn nuclear magnetic resonance imaging politef implant pregnancy diabetes mellitus prenatal care pulmonary artery respiratory distress surgical technique LA - English M3 - Article N1 - L44596627 2006-11-09 PY - 2006 SN - 1047-9511 1467-1107 SP - 504-506 ST - The management of hypoplastic left heart syndrome with aright aortic arch T2 - Cardiology in the Young TI - The management of hypoplastic left heart syndrome with aright aortic arch UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L44596627 http://dx.doi.org/10.1017/S1047951106000448 VL - 16 ID - 1252 ER - TY - JOUR AB - Plastic bronchitis is a rare complication of a variety of respiratory diseases and congenital heart disease surgery, particularly Fontan procedure. Bronchial casts with rubber-like consistency develop acutely and may cause severe life-threatening respiratory distress. The management of plastic bronchitis is yet not well defined. Early intermittent, self-administered nebulization of tissue plasminogen activator was found to be effective in preventing deterioration of acute respiratory symptoms in a patient with primary ciliary dyskinesia and recurrent cast formation. Further investigation into new therapeutic strategies for this devastating disease is advocated. AD - Department of Mother and Child Health, Salesi Children's Hospital, Azienda Ospedaliero-Universitaria Ospedali Riuniti, Ancona, Italy. debenedictis@ospedaliriuniti.marche.it. AN - 24524376 AU - Colaneri, M. AU - Quarti, A. AU - Pozzi, M. AU - Gasparini, S. AU - Carloni, I. AU - de Benedictis, F. M. C2 - PMC3974171 DA - Feb 13 DB - PubMed DO - 10.1186/1824-7288-40-18 DP - NLM ET - 2014/02/15 IS - 1 KW - Administration, Inhalation Adolescent Bronchitis/diagnosis/*drug therapy Bronchoscopy Diagnosis, Differential Follow-Up Studies Humans Male Microscopy, Electron Nebulizers and Vaporizers Respiratory Mucosa/ultrastructure Tissue Plasminogen Activator/*administration & dosage LA - eng N1 - 1824-7288 Colaneri, Massimo Quarti, Andrea Pozzi, Marco Gasparini, Stefano Carloni, Ines de Benedictis, Fernando Maria Case Reports Journal Article Ital J Pediatr. 2014 Feb 13;40(1):18. doi: 10.1186/1824-7288-40-18. PY - 2014 SN - 1720-8424 SP - 18 ST - Management of plastic bronchitis with nebulized tissue plasminogen activator: another brick in the wall T2 - Ital J Pediatr TI - Management of plastic bronchitis with nebulized tissue plasminogen activator: another brick in the wall VL - 40 ID - 486 ER - TY - JOUR AB - Today, most female children born with congenital heart disease will reach childbearing age. For many women with complex congenital heart disease, carrying a pregnancy carries a moderate to high risk for both the mother and her fetus. Many such women, however, do not have access to adult congenital heart disease tertiary centers with experienced reproductive programs. Therefore, it is important that all practitioners who will be managing these women have current information not only on preconception counseling and diagnostic evaluation to determine maternal and fetal risk but also on how to manage them once they are pregnant and when to refer them to a regional center with expertise in pregnancy management. AU - Canobbio, M. M. AU - Warnes, C. A. AU - Aboulhosn, J. AU - Connolly, H. M. AU - Khanna, A. AU - Koos, B. J. AU - Mital, S. AU - Rose, C. AU - Silversides, C. AU - Stout, K. DB - Embase Medline DO - 10.1161/CIR.0000000000000458 IS - 8 KW - adrenergic receptor stimulating agent antiarrhythmic agent anticoagulant agent antihypertensive agent brain natriuretic peptide diuretic agent inotropic agent purine nucleoside thiazide diuretic agent ablation therapy anticoagulant therapy arterial switch operation article atrial fibrillation blood volume cardiovascular surgery cardioversion clinical practice colloid computer assisted tomography congenital heart disease contraception cyanosis echocardiography electrocardiogram exercise test female fetus risk first trimester pregnancy genetic counseling great vessels transposition heart arrhythmia heart atrium flutter heart block heart catheterization heart failure heart left ventricle outflow tract obstruction heart output heart rhythm heart single ventricle heart ventricle tachycardia high risk pregnancy human implantable cardioverter defibrillator intrapartum care lifestyle lung artery pressure mechanical heart valve nuclear magnetic resonance imaging obstetric procedure oncotic pressure cardiac rhythm management device paroxysmal atrial fibrillation pregnancy prenatal care prenatal period priority journal puerperium pulmonary hypertension resuscitation second trimester pregnancy third trimester pregnancy thorax radiography LA - English M3 - Article N1 - L614243887 2017-02-03 2017-03-24 PY - 2017 SN - 1524-4539 0009-7322 SP - e50-e87 ST - Management of Pregnancy in Patients with Complex Congenital Heart Disease: A Scientific Statement for Healthcare Professionals from the American Heart Association T2 - Circulation TI - Management of Pregnancy in Patients with Complex Congenital Heart Disease: A Scientific Statement for Healthcare Professionals from the American Heart Association UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L614243887 http://dx.doi.org/10.1161/CIR.0000000000000458 VL - 135 ID - 771 ER - TY - JOUR AB - Congenital heart diseases are the most common birth defects in humans, affecting approximately 0.8% of all live births. In the past, many of the more severe defects resulted in profound disability and death during childhood, and adult survival was exceptional. The past 4 decades have seen dramatic improvements in the survival and quality of life of patients with the more severe defects. As a result of these improvements, the challenges of caring for adults with congenital heart disease are only now being realized. Most women with congenital heart disease are now expected to reach childbearing age and maternal cardiac disease is the major cause of maternal morbidity and mortality. As such, appropriate pre-pregnancy counseling and management during pregnancy are fundamental components of the care of these patients. This article describes the circulatory changes that occur during normal pregnancy and delivery, addresses the risks posed during pregnancy by specific congenital lesions, and reviews the current data on pregnancy outcomes in patients with individual congenital defects. AD - Division of Cardiology, Department of Medicine, University of California, San Francisco, CA 94143-0214, USA. harrisi@medicine.ucsf.edu AN - 21295672 AU - Harris, I. S. C2 - PMC3052938 C6 - NIHMS229795 DA - Jan-Feb DB - PubMed DO - 10.1016/j.pcad.2010.08.001 DP - NLM ET - 2011/02/08 IS - 4 KW - Adaptation, Physiological Adult Blood Volume Cardiac Output Counseling Female Fontan Procedure Heart Defects, Congenital/mortality/physiopathology/*therapy Heart Septal Defects/therapy Heart Valve Diseases/therapy Hemodynamics Humans Labor, Obstetric/physiology Loeys-Dietz Syndrome/therapy Marfan Syndrome/complications/therapy Pregnancy Pregnancy Complications, Cardiovascular/mortality/physiopathology/*therapy Pregnancy Outcome Pregnancy Trimester, First/physiology Pregnancy Trimester, Second/physiology Pregnancy Trimester, Third/physiology Survivors Ultrasonography, Prenatal Vascular Resistance LA - eng N1 - 1873-1740 Harris, Ian S K08 HL089330/HL/NHLBI NIH HHS/United States K08 HL089330-04/HL/NHLBI NIH HHS/United States Journal Article Review Prog Cardiovasc Dis. 2011 Jan-Feb;53(4):305-11. doi: 10.1016/j.pcad.2010.08.001. PY - 2011 SN - 0033-0620 (Print) 0033-0620 SP - 305-11 ST - Management of pregnancy in patients with congenital heart disease T2 - Prog Cardiovasc Dis TI - Management of pregnancy in patients with congenital heart disease VL - 53 ID - 390 ER - TY - JOUR AB - BACKGROUND AND OBJECTIVES: When innovative, not yet fully proven therapies are introduced, physicians may have neither experience nor sufficient data in the medical literature to assist in their decision to discuss them with and/or recommend them to patients. Little is known about how physicians deal with this uncertainty. Moreover, when multiple physicians caring for a single patient have reached different conclusions regarding this new therapy, the potential for disagreement exists that could give rise to ethical issues as well as cause confusion to the patient. To explore these topics, we investigated the attitudes of specialists to therapies for two life-threatening diseases: hypoplastic left heart syndrome (HLHS) and short bowel syndrome. METHODS: A forced choice questionnaire was distributed to the heads of neonatology, pediatric cardiology, and pediatric gastroenterology training programs asking about their outcome impressions and treatment recommendations and about the local availability of treatments. In addition, responses from specialists from the same institution were linked in a confidential manner to evaluate the frequency of disagreement within the same institution. Responses were analyzed using chi(2) and Wilcoxon matched pair analysis as appropriate. RESULTS: The overall rate of response was 79%. In institutions that had both neonatology and pediatric gastroenterology training programs, there was a 59% response rate compared with a 73% response rate from institutions that had both neonatology and cardiology programs. Significant differences were noted among specialists as to who would be involved in discussions of therapeutic options with patients in both HLHS and short bowel syndrome. Differences also were noted in the willingness of specialists to discuss and recommend therapies, in the perceived survival and quality of life by various specialists after transplant and palliative surgery, and in the local availability of various options. The neonatologists and gastroenterologists at the same institution disagreed on responses in 34% of the questions with only 1 of the 25 pairs in full agreement. In contrast, the neonatologists and pediatric cardiologists at the same institution disagreed in only 14% of the questions with 7 of the 28 pairs in full agreement. CONCLUSIONS: Substantial disagreement among specialists about new interventions was found. There seem to be fewer differences among specialists when dealing with the more mature therapy, HLHS. Two major ethical issues arise. First, there seems to be no accepted professional standard to which individuals can appeal when determining whether to discuss or recommend new, not-yet-fully-proven technologies. Second, there is the potential for much patient confusion when counseling physicians recommend different options. Colleagues as individuals and specialists as groups should talk to each other before individual discussions with families to ensure that there is a clear understanding of differing beliefs. AD - Department of Pediatrics, Baylor College of Medicine, Houston, TX 77030, USA. timothyc@bcm.tmc.edu AN - 10506273 AU - Cooper, T. R. AU - Garcia-Prats, J. A. AU - Brody, B. A. DA - Oct DB - PubMed DO - 10.1542/peds.104.4.e48 DP - NLM ET - 1999/10/03 IS - 4 KW - *Attitude of Health Personnel Cardiology Counseling *Decision Making Gastroenterology Heart Transplantation Humans Hypoplastic Left Heart Syndrome/surgery/*therapy Infant, Newborn *Interprofessional Relations Neonatology Palliative Care Parenteral Nutrition, Total Prognosis Quality of Life Short Bowel Syndrome/surgery/*therapy Surveys and Questionnaires LA - eng N1 - 1098-4275 Cooper, T R Garcia-Prats, J A Brody, B A Comparative Study Journal Article United States Pediatrics. 1999 Oct;104(4):e48. doi: 10.1542/peds.104.4.e48. PY - 1999 SN - 0031-4005 SP - e48 ST - Managing disagreements in the management of short bowel and hypoplastic left heart syndrome T2 - Pediatrics TI - Managing disagreements in the management of short bowel and hypoplastic left heart syndrome VL - 104 ID - 385 ER - TY - JOUR AB - BACKGROUND: The objective of the present study is to review the literature regarding the management of fertile patients under lithium treatment for bipolar disorder and to report the experience of our Teratology Information Service over the past thirteen years in managing women treated with lithium during preconception, pregnancy and breastfeeding. METHODS: This research focuses on a selective review of the literature and a retrospective survey has been carried out on fertile women under lithium treatment who called our service at A. Gemelli University Hospital in Rome from May 2002 to December 2015. RESULTS: A total of 140 women under lithium treatment called our TIS. A complete follow-up has been performed on 34 patients: 29 called during pregnancy and 5 called during preconception. None of the patients called during breastfeeding, while half of the patients were taking concomitant drugs during pregnancy. One major cardiac malformation (hypoplastic left heart syndrome) has been reported. No minor malformations have been detected. Twenty-one patients delivered a living child, with one premature neonate. Two patients underwent voluntary interruption of pregnancy and six patients had early spontaneous abortion. In one patient, intrauterine growth retardation occurred, but with no adverse neonatal outcomes. Four neonates experienced transient respiratory distress at birth. Two children developed mild to severe language delay, but normal motor development. CONCLUSIONS: Lithium treatment in fertile women is a very delicate topic, where risks and benefits of discontinuing therapy when women plan to become pregnant should be accurately evaluated. Thorough peri-conceptional counselling is crucial for the outcome of pregnancy and for maternal health status during preconception, gestation and breastfeeding. AD - Department of Women's and Children's Health, Sacro Cuore Catholic University, Agostino Gemelli University Hospital Foundation, Rome, Italy - caterina.neri@policlinicogemelli.it. Department of Women's and Children's Health, Sacro Cuore Catholic University, Agostino Gemelli University Hospital Foundation, Rome, Italy. AN - 29845825 AU - Neri, C. AU - De Luca, C. AU - D'Oria, L. AU - Licameli, A. AU - Nucci, M. AU - Pellegrino, M. AU - Caruso, A. AU - De Santis, M. DA - Jun DB - PubMed DO - 10.23736/s0026-4784.18.04190-4 DP - NLM ET - 2018/05/31 IS - 3 KW - Adult Antimanic Agents/adverse effects/*therapeutic use Bipolar Disorder/*drug therapy Breast Feeding Cohort Studies Counseling/methods Female Follow-Up Studies Humans Information Services/organization & administration Italy Lithium Compounds/adverse effects/*therapeutic use Middle Aged Preconception Care Pregnancy Pregnancy Complications/drug therapy/*epidemiology Retrospective Studies Teratology/organization & administration Young Adult LA - eng N1 - 1827-1650 Neri, Caterina De Luca, Carmen D'oria, Luisa Licameli, Angelo Nucci, Marta Pellegrino, Marcella Caruso, Alessandro De Santis, Marco Journal Article Review Italy Minerva Ginecol. 2018 Jun;70(3):261-267. doi: 10.23736/S0026-4784.18.04190-4. PY - 2018 SN - 0026-4784 SP - 261-267 ST - Managing fertile women under lithium treatment: the challenge of a Teratology Information Service T2 - Minerva Ginecol TI - Managing fertile women under lithium treatment: the challenge of a Teratology Information Service VL - 70 ID - 227 ER - TY - JOUR AD - Harefield Hospital, Harefield, Middlesex UB9 6JH England, United Kingdom AU - Coe, J. Y. AU - Radley-Smithr, R. AU - Yacoub, M. DB - Scopus DO - 10.1016/S0022-3476(82)80469-1 IS - 3 M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 1982 SP - 496-497 ST - Managment of tricuspid atresia with orally administered prostaglandin E2 T2 - The Journal of Pediatrics TI - Managment of tricuspid atresia with orally administered prostaglandin E2 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0020077047&doi=10.1016%2fS0022-3476%2882%2980469-1&partnerID=40&md5=ae2a959d19091ba2dbd04f4916f9df13 VL - 100 ID - 2333 ER - TY - JOUR AB - This cohort study examines the possible association between maternal alcohol intake, including binge drinking, during pregnancy, and the subsequent risk of having a child with an isolated congenital heart defect and, more specifically, with the isolated form of ventricular septal defect (VSD) or of an atrial septal defect (ASD). Methods: Participants were 80,346 pregnant women who were enrolled into the Danish National Birth Cohort in 1996-2002 and gave birth to a live-born singleton without any chromosome anomalies. Twice during pregnancy these women were asked about their intake of alcohol. Few (if any) women with an excessive/abusive intake of alcohol were enrolled into the Danish National Birth Cohort. Results: Through linkage with the National Hospital Discharge Registry, we identified 477 infants with a diagnosis of isolated congenital heart defect registered at any time during their first 31/2-years of life; they included 198 infants with a VSD and 145 with an ASD. Neither the number of episodes of binge drinking nor binge drinking during three different developmental periods was associated with VSD or ASD. Women drinking 1/2-11/2, 2, and 3+ drinks of alcohol per week had adjusted prevalence ratios of delivering an infant with a VSD of 1.22 (95% CI = 0.90-1.66); 1.38 (95% CI = 0.83-2.28); and 1.10 (95% CI = 0.54-2.23), respectively. The test for trend was 0.29. Conclusions: Prenatal exposure to low-to-moderate levels of alcohol on a weekly basis or occasional binge drinking during the early part of pregnancy was not statistical significantly associated with the prevalence of isolated VSD and ASD in offspring. © 2011 Wiley-Liss, Inc. AD - K. Strandberg-Larsen, Section of Social Medicine, Department of Public Health, University of Copenhagen, Øster Farimagsgade 5, Postboks 2099, DK-1014 Copenhagen K, Denmark AU - Strandberg-Larsen, K. AU - Skov-Ettrup, L. S. AU - Grønbæk, M. AU - Andersen, A. M. N. AU - Olsen, J. AU - Tolstrup, J. DB - Embase Medline DO - 10.1002/bdra.20818 IS - 7 KW - adult alcohol abuse alcohol consumption aortic coarctation aorta valve atresia aortic valve disease aortic valve stenosis arterial trunk article atrioventricular septal defect child chromosome aberration cohort analysis congenital heart malformation drinking behavior Ebstein anomaly Fallot tetralogy female fetus risk great vessels transposition heart atrium septum defect heart single ventricle heart ventricle septum defect hospital discharge human hypoplastic left heart syndrome hypoplastic right heart lung vein drainage anomaly major clinical study maternal behavior pregnant woman prenatal exposure preschool child priority journal tricuspid valve atresia tricuspid valve stenosis LA - English M3 - Article N1 - L362095377 2011-07-15 PY - 2011 SN - 1542-0752 1542-0760 SP - 616-622 ST - Maternal alcohol drinking pattern during pregnancy and the risk for an offspring with an isolated congenital heart defect and in particular a ventricular septal defect or an atrial septal defect T2 - Birth Defects Research Part A - Clinical and Molecular Teratology TI - Maternal alcohol drinking pattern during pregnancy and the risk for an offspring with an isolated congenital heart defect and in particular a ventricular septal defect or an atrial septal defect UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L362095377 http://dx.doi.org/10.1002/bdra.20818 VL - 91 ID - 1100 ER - TY - JOUR AB - BACKGROUND: Little is known about the association between maternal nativity and congenital malformations among Hispanics living in the United States. METHODS: We conducted a cross-sectional study to investigate the association between maternal nativity and various congenital malformations among singleton live-births born to Hispanic women in New York from 1993 to 2001. Birth certificates, used to identify maternal birthplace, were linked with congenital malformation registry files to obtain birth defects outcome. We examined how the risk of birth defects varied by maternal birthplace by estimating the adjusted odds ratios (aORs) using logistic regression. RESULTS: A foreign maternal birth showed statistically negative associations with overall congenital malformations (aOR, 0.70; 95% CI, 0.68-0.73), cardiovascular defects (aOR, 0.85; 95% CI, 0.77-0.93), central nervous system defects (aOR, 0.76; 95% CI, 0.63-0.91), and multiple defects (aOR, 0.80; 95% CI, 0.74-0.86). Specifically, foreign-born Hispanic women were statistically at reduced risk to deliver live babies with cleft palate (aOR, 0.56; 95% CI, 0.40-0.80), atresia and stenosis of rectum or anus (aOR, 0.58; 95% CI, 0.35-0.97), and craniosynostosis (aOR, 0.71; 95% CI, 0.51-0.99). Hispanic mothers born in Puerto Rico had a similar risk of delivering children with birth defects compared to U.S.-born Hispanic mothers. In contrast, Hispanic mothers born in Mexico, or Cuba and Central and South America were at reduced risk of delivering infants with overall congenital malformations (aOR, 0.64; 95% CI, 0.60-0.67) and (aOR, 0.65; 95% CI, 0.63-0.68), respectively. CONCLUSIONS: Foreign-born Hispanic mothers had a slightly lower risk to deliver live-born singleton infants with major congenital malformations than did U.S. born Hispanic mothers. © 2006 Wiley-Liss, Inc. AD - M. Zhu, State University of New York at Albany, School of Public Health, Department of Epidemiology and Biostatistics, One University Place, Rensselaer, NY 12144-3456, United States AU - Zhu, M. AU - Druschel, C. AU - Lin, S. DB - Embase Medline DO - 10.1002/bdra.20270 IS - 6 KW - illicit drug anus atresia aortic coarctation article birth certificate congenital malformation birthplace cardiovascular malformation central nervous system malformation child cigarette smoking cleft lip cleft palate colon stenosis craniofacial synostosis Cuba drinking behavior Fallot tetralogy great vessels transposition Hispanic human hypoplastic left heart syndrome infant limb malformation logistic regression analysis major clinical study Mexico multiple malformation syndrome nullipara prenatal care priority journal Puerto Rico register socioeconomics South and Central America spinal dysraphism spontaneous abortion tracheoesophageal fistula United States LA - English M3 - Article N1 - L44320498 2006-09-17 PY - 2006 SN - 1542-0752 1542-0760 SP - 467-473 ST - Maternal birthplace and major congenital malformations among New York Hispanics T2 - Birth Defects Research Part A - Clinical and Molecular Teratology TI - Maternal birthplace and major congenital malformations among New York Hispanics UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L44320498 http://dx.doi.org/10.1002/bdra.20270 VL - 76 ID - 1262 ER - TY - JOUR AB - The aim of this work is to describe the experience of being a mother by women who together with their children stay on the ward after cardiac surgical correction of congenital heart defects. The research material consisted of the narratives of mothers whose children were born with a heart defect and surgically treated. Four women aged 21-30 years were participants of this study. The age of the subjects' children ranged from 5 weeks to 1 year and three months. The heart defects with which the children were born were hypoplastic left heart syndrome (HLHS ) or atrioventricular canal defect. The research was carried out using the narrative interview. Qualitative analysis was carried out according to the rules of thematic analysis. The results of the research confirm that cardiac treatment can have a significant impact on maternal care patterns and the mother-infant bonding process. Intermittent contact with an infant can lead to loss of control over what happens to the child and the loss of a sense of competence to care for him. Medical staff face the challenge of supporting the parents of hospitalized children in the process of building relationships with a sick infant. The actions of doctors and nurses to enable parents to care for a baby can help foster their sense of competence and responsibility. AD - Zakład Psychologii Lekarskiej, Katedra Psychiatrii Uniwersytet Jagielloński, Collegium Medicum, ul. Kopernika 21a, 31-501 Kraków, Poland, tel. 602-469-928, e-mail: karo.sikora@uj.edu.pl. AN - 25874781 AU - Sikora, K. AU - Janusz, B. DA - Oct-Dec DB - PubMed DP - NLM ET - 2014/01/01 IS - 4 KW - Adaptation, Psychological Adult *Anecdotes as Topic Cardiac Surgical Procedures Female Heart Defects, Congenital/*psychology/*surgery Humans Infant Care/*psychology Infant, Newborn *Mother-Child Relations Mothers/*psychology Narration *Object Attachment Qualitative Research Young Adult LA - eng N1 - Sikora, Karolina Janusz, Bernadetta Journal Article Poland Dev Period Med. 2014 Oct-Dec;18(4):439-46. PY - 2014 SN - 1428-345X (Print) 1428-345x SP - 439-46 ST - Maternal bond with cardiosurgically treated infant. Qualitative analysis of mothers' narratives T2 - Dev Period Med TI - Maternal bond with cardiosurgically treated infant. Qualitative analysis of mothers' narratives VL - 18 ID - 320 ER - TY - JOUR AB - Background: Butalbital is a barbiturate contained in combination products with caffeine and an analgesic prescribed for the treatment of migraine and tension-type headaches. Controversy exists as to whether butalbital should continue to be prescribed in the United States because of the potential for abuse, overuse headache, and withdrawal syndromes. Butalbital crosses the placenta but there is limited information about potential teratogenicity. Objective: To evaluate associations between butalbital and a wide range of specific birth defects. Methods: The National Birth Defects Prevention Study is an ongoing, case-control study of nonsyndromic, major birth defects conducted in 10 states. The detailed case classification and large number of cases in the National Birth Defects Prevention Study allowed us to examine the association between maternal self-reported butalbital use and specific birth defects. We conducted an analysis of 8373 unaffected controls and 21,090 case infants with estimated dates of delivery between 1997 and 2007; included were birth defects with 250 or more cases. An exploratory analysis examined groups with 100 to 249 cases. Results: Seventy-three case mothers and 15 control mothers reported periconceptional butalbital use. Of 30 specific defect groups evaluated, adjusted odds ratios for maternal periconceptional butalbital use were statistically significant for 3 congenital heart defects: tetralogy of Fallot (adjusted odds ratio = 3.04; 95% confidence interval = 1.07-8.62), pulmonary valve stenosis (adjusted odds ratio = 5.73; 95% confidence interval = 2.25-14.62), and secundum-type atrial septal defect (adjusted odds ratio = 3.06; 95% confidence interval = 1.07-8.79). In the exploratory analysis, an elevated odds ratio was detected for 1 congenital heart defect, single ventricle. Conclusions: We observed relationships between maternal periconceptional butalbital use and certain congenital heart defects. These associations have not been reported before, and some may be spurious. Butalbital use was rare and despite the large size of the National Birth Defects Prevention Study, the number of exposed case and control infants was small. However, if confirmed in additional studies, our findings will be useful in weighing the risks and benefits of butalbital for the treatment of migraine and tension-type headaches. © Published 2013. This article is a U.S. Government work and is in the public domain in the USA. AD - M.L. Browne, Congenital Malformations Registry, New York State Department of Health, Corning Tower, Empire State Plaza, Albany, NY 12237, United States AU - Browne, M. L. AU - Van Zutphen, A. R. AU - Botto, L. D. AU - Louik, C. AU - Richardson, S. AU - Druschel, C. M. C1 - aspirin DB - Embase Medline DO - 10.1111/head.12203 IS - 1 KW - acetylsalicylic acid butalbital caffeine codeine paracetamol triptan derivative adolescent adult anus atresia article congenital malformation case control study child cleft lip cleft palate congenital heart malformation controlled study obstetric delivery disease association drug use environmental exposure exploratory research female great vessels transposition heart atrium septum defect heart septum defect heart single ventricle human hypoplastic left heart syndrome hypospadias major clinical study male mother pregnancy priority journal pulmonary valve stenosis school child self report young adult aspirin LA - English M3 - Article N1 - L52766825 2013-09-11 2014-01-24 PY - 2014 SN - 0017-8748 1526-4610 SP - 54-66 ST - Maternal butalbital use and selected defects in the national birth defects prevention study T2 - Headache TI - Maternal butalbital use and selected defects in the national birth defects prevention study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52766825 http://dx.doi.org/10.1111/head.12203 VL - 54 ID - 943 ER - TY - JOUR AB - Objective: To estimate risks of major congenital anomaly (MCA) among children of mothers prescribed antidepressants during early pregnancy or diagnosed with depression but without antidepressant prescriptions. Design: Population-based cohort study. Setting: Linked UK maternal-child primary care records. Population: A total of 349 127 singletons liveborn between 1990 and 2009. Methods: Odds ratios adjusted for maternal sociodemographics and comorbidities (aORs) were calculated for MCAs, comparing women with first-trimester selective serotonin reuptake inhibitors (SSRIs) or tricyclic antidepressants (TCAs) and women with diagnosed but unmedicated depression, or women without diagnosed depression. Main: outcome measures Fourteen system-specific MCA groups classified according to the European Surveillance of Congenital Anomalies and five specific heart anomaly groups. Results: Absolute risks of MCA were 2.7% (95% confidence interval, 95% CI, 2.6-2.8%) in children of mothers without diagnosed depression, 2.8% (95% CI 2.5-3.2%) in children of mothers with unmedicated depression, and 2.7% (95% CI 2.2-3.2%) and 3.1% (95% CI 2.2-4.1%) in children of mothers with SSRIs or TCAs, respectively. Compared with women without depression, MCA overall was not associated with unmedicated depression (aOR 1.07, 95% CI 0.96-1.18), SSRIs (aOR 1.01, 95% CI 0.88-1.17), or TCAs (aOR 1.09, 95% CI 0.87-1.38). Paroxetine was associated with increased heart anomalies (absolute risk 1.4% in the exposed group compared with 0.8% in women without depression; aOR 1.78, 95% CI 1.09-2.88), which decreased marginally when compared with women with diagnosed but unmedicated depression (aOR 1.67, 95% CI 1.00-2.80). Conclusions: Overall MCA risk did not increase with maternal depression or with antidepressant prescriptions. Paroxetine was associated with increases of heart anomalies, although this could represent a chance finding from a large number of comparisons undertaken. AD - L.J. Tata, Division of Epidemiology and Public Health, University of Nottingham, Nottingham City Hospital, Clinical Sciences Building, Nottingham, United Kingdom AU - Ban, L. AU - Gibson, J. E. AU - West, J. AU - Fiaschi, L. AU - Sokal, R. AU - Smeeth, L. AU - Doyle, P. AU - Hubbard, R. B. AU - Tata, L. J. DB - Embase Medline DO - 10.1111/1471-0528.12682 IS - 12 KW - citalopram escitalopram fluoxetine fluvoxamine paroxetine serotonin uptake inhibitor sertraline tricyclic antidepressant agent adolescent adult aortic coarctation arterial trunk article atrioventricular septal defect cardiovascular malformation chromosome aberration cohort analysis comorbidity conception congenital heart malformation congenital malformation controlled study digestive system malformation drug safety ear malformation Ebstein anomaly environmental exposure eye malformation face malformation Fallot tetralogy female first trimester pregnancy genital malformation head and neck malformation heart atrium septum defect heart single ventricle heart ventricle septum defect human limb malformation lung vein drainage anomaly major clinical study major congenital anomaly musculoskeletal system malformation neck malformation nervous system malformation patent ductus arteriosus prescription progeny postnatal depression respiratory tract malformation risk assessment tricuspid valve atresia tricuspid valve stenosis urinary tract malformation LA - English M3 - Article N1 - L53051361 2014-03-17 2014-12-15 PY - 2014 SN - 1471-0528 1470-0328 SP - 1471-1481 ST - Maternal depression, antidepressant prescriptions, and congenital anomaly risk in offspring: A population-based cohort study T2 - BJOG: An International Journal of Obstetrics and Gynaecology TI - Maternal depression, antidepressant prescriptions, and congenital anomaly risk in offspring: A population-based cohort study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L53051361 http://dx.doi.org/10.1111/1471-0528.12682 VL - 121 ID - 907 ER - TY - JOUR AB - Objective To identify maternal dietary patterns related to biomarkers of methylation and to investigate associations between these dietary patterns and the risk of congenital heart defects (CHDs) in the offspring. Design Case-control study. Setting Western part of the Netherlands, 2003-08. Population One hundred and seventy-nine mothers of children with CHD and 231 mothers of children without a congenital malformation. Methods Food intake was obtained by food frequency questionnaires. The reduced rank regression method was used to identify dietary patterns related to the biomarker concentrations of methylation in blood. Main outcome measures Dietary patterns, vitamin B and homocysteine concentrations, biomarkers of methylation (S-adenosylmethionine [SAM] and S-adenosylhomocysteine [SAH]) and the risk of CHD estimated by odds ratios and 95% confidence intervals. Results The one-carbon-poor dietary pattern, comprising a high intake of snacks, sugar-rich products and beverages, was associated with SAH (β = 0.92, P < 0.001). The one-carbon-rich dietary pattern with high fish and seafood intake was associated with SAM (β = 0.44, P < 0.001) and inversely with SAH (β = -0.08, P < 0.001). Strong adherence to this dietary pattern resulted in higher serum (P < 0.05) and red blood cell (P < 0.01) folate and a reduced risk of CHD in offspring: odds ratio, 0.3 (95% confidence interval, 0.2-0.6). Conclusions The one-carbon-rich dietary pattern, characterised by the high intake of fish and seafood, is associated with a reduced risk of CHD. This finding warrants further investigation in a randomised intervention trial. © 2011 The Authors BJOG An International Journal of Obstetrics and Gynaecology © 2011 RCOG. AD - R.P.M. Steegers-Theunissen, Department of Obstetrics and Gynaecology, Erasmus MC, University Medical Centre, PO Box 2040, 3000 CA Rotterdam, Netherlands AU - Obermann-Borst, S. A. AU - Vujkovic, M. AU - De Vries, J. H. AU - Wildhagen, M. F. AU - Looman, C. W. AU - De Jonge, R. AU - Steegers, E. A. P. AU - Steegers-Theunissen, R. P. M. DB - Embase Medline DO - 10.1111/j.1471-0528.2011.02984.x IS - 10 KW - biological marker folic acid homocysteine s adenosylhomocysteine ademetionine sugar vitamin B group adult alcohol consumption aortic coarctation aortic valve stenosis article beverage caloric intake cardiovascular risk congenital heart malformation controlled study disease association educational status erythrocyte Fallot tetralogy fast food female fish food frequency questionnaire food intake great vessels transposition heart ventricle septum defect human human experiment hypoplastic left heart syndrome maternal dietary pattern methylation Netherlands normal human priority journal progeny pulmonary valve stenosis risk assessment sea food smoking LA - English M3 - Article N1 - L51434929 2011-05-23 2011-08-26 PY - 2011 SN - 1470-0328 1471-0528 SP - 1205-1215 ST - A maternal dietary pattern characterised by fish and seafood in association with the risk of congenital heart defects in the offspring T2 - BJOG: An International Journal of Obstetrics and Gynaecology TI - A maternal dietary pattern characterised by fish and seafood in association with the risk of congenital heart defects in the offspring UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51434929 http://dx.doi.org/10.1111/j.1471-0528.2011.02984.x VL - 118 ID - 1094 ER - TY - JOUR AB - Background: Mothers and infants are exposed to multiple stresses when cardiac surgery is required for the infant. This study reviewed infant responsiveness using a standardized objective observational measure of social withdrawal and explored its association with measures of maternal distress. Methods: Mother–infant pairs involving infants surviving early cardiac surgery were assessed when the infant was aged two months. Infant social withdrawal was measured using the Alarm Distress Baby Scale. Maternal distress was assessed using self-report measures for maternal depression (Edinburgh Postnatal Depression Scale), anxiety (Spielberger State-Trait Anxiety Scale), and parenting stress (Parenting Stress Index–Short Form). Potential associations between infant social withdrawal and maternal distress were evaluated. Results: High levels of maternal distress and infant social withdrawal were identified relative to community norms with a positive association. Such an association was not found between infant social withdrawal and the cardiac abnormality and surgery performed. Conclusion: The vulnerability of infants requiring cardiac surgery may be better understood when factors beyond their medical condition are considered. The findings suggested an association between maternal distress and infant social withdrawal, which may be consistent with mothers’ distress placing infants subjected to cardiac surgery at substantially increased risk of social withdrawal. However, it is unclear to what extent infant withdrawal may trigger maternal distress and what the interactive effects are. Further research is warranted. Trialing a mother–infant support program may be helpful in alleviating distress and improving the well-being and outcomes for these families. AD - S. Menahem, Monash Medical Centre, Monash Health 246 Clayton Road, Clayton, VIC, Australia AU - Re, J. M. AU - Dean, S. AU - Mullaert, J. AU - Guedeney, A. AU - Menahem, S. DB - Embase Medline DO - 10.1177/2150135118788788 IS - 6 KW - adult anxiety aortic coarctation article Bayley Scales of Infant Development clinical article congenital heart disease depression Edinburgh Postnatal Depression Scale facial expression female heart atrium septum defect heart surgery heart ventricle septum defect human hypoplastic left heart syndrome infant Likert scale male maternal stress mother child relation New York Heart Association class parental stress Parenting Stress Index Short Form postnatal depression predictive value priority journal psychosocial withdrawal social adaptation State Trait Anxiety Inventory stress assessment treatment outcome vocalization LA - English M3 - Article N1 - L624459561 2018-10-25 2020-03-03 PY - 2018 SN - 2150-136X 2150-1351 SP - 624-637 ST - Maternal Distress and Infant Social Withdrawal (ADBB) Following Infant Cardiac Surgery for Congenital Heart Disease T2 - World Journal for Pediatric and Congenital Heart Surgery TI - Maternal Distress and Infant Social Withdrawal (ADBB) Following Infant Cardiac Surgery for Congenital Heart Disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L624459561 http://dx.doi.org/10.1177/2150135118788788 VL - 9 ID - 658 ER - TY - JOUR AB - OBJECTIVE: To investigate whether the association between the maternal methylation status as reflected by low S-adenosylmethionine and high S-adenosylhomocysteine, is detrimental for cardiogenesis and congenital heart disease (CHD) in the offspring. METHODS: As part of a case-control study in the western part of the Netherlands, we evaluated 231 mothers of children with CHD and 315 control mothers of nonmalformed children. The total case group was analyzed and stratified into isolated (n=180) and nonisolated CHDs (n=51). The latter subgroup was further subdivided into Nonsyndromic (n=20), Down Syndrome (n=19), and Other Syndromes (n=12). A multivariable general linear model was used to test for differences between the case groups and controls. All analyses were adjusted for current B vitamin supplement use. RESULTS: Plasma total homocysteine was significantly different between the total case group (median, range 10.3, 4.0-43.8, P=.026) and the nonisolated cases (11.1, 5.5-43.8, P=.006) compared with the controls (10.0, 5.3-42.0). The subgroup of Down Syndrome presented significantly higher total homocysteine and S-adenosylhomocysteine levels and a lower S-adenosylmethionine/S-adenosylhomocysteine ratio than controls. CONCLUSION: Maternal hyperhomocysteinemia, and not hypomethylation, is a risk factor for having a child with CHD. Maternal hypomethylation, however, seems to be associated with offspring having CHD and Down syndrome. © 2008 by The American College of Obstetricians and Gynecologists. AD - R. P. M. Steegers-Theunissen, Department of Obstetrics and Gynecology, Division of Obstetrics and Prenatal Medicine, University Medical Centre, Dr. Molewaterplein 50, 3015 GJ Rotterdam, Netherlands AU - Steegers-Theunissen, R. P. M. AU - Van Driel, L. M. J. W. AU - De Jonge, R. AU - Helbing, W. A. AU - Van Zelst, B. D. AU - Ottenkamp, J. AU - Steegers, E. A. P. DB - Embase Medline DO - 10.1097/AOG.0b013e31817dd058 IS - 2 PART 1 KW - biological marker homocysteine s adenosylhomocysteine ademetionine vitamin B group adult aortic coarctation aortic valve stenosis article cardiovascular risk case control study child congenital heart disease control group controlled study Down syndrome evaluation study Fallot tetralogy female great vessels transposition heart atrium septum defect heart ventricle septum defect human hyperhomocysteinemia hypoplastic left heart syndrome major clinical study methylation mother Netherlands priority journal progeny pulmonary valve stenosis risk factor stratification vitamin supplementation LA - English M3 - Article N1 - L354789946 2009-07-29 PY - 2008 SN - 0029-7844 SP - 277-283 ST - Maternal global methylation status and risk of congenital heart diseases T2 - Obstetrics and Gynecology TI - Maternal global methylation status and risk of congenital heart diseases UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L354789946 http://dx.doi.org/10.1097/AOG.0b013e31817dd058 VL - 112 ID - 1201 ER - TY - JOUR AB - Objective To establish possible aetiological factors contributing to congenital heart defects (CHD) overall and separately for different types of CHD, as causes are unknown for the vast majority of patients. Design To estimate a possible association with maternal diseases and related drug treatments as exposures in the mothers of cases with right-sided obstructive defects of the heart (RSODH). Setting A large population-based Hungarian Case- Control Surveillance of Congenital Abnormalities data set. Patients Newborn infants with four types of RSODH based on autopsy or surgical records. Interventions Comparison of 200 live-born cases with RSODH including 72 (36.0%) with pulmonary valve stenosis, 13 (6.5%) with tricuspid atresia/stenosis, 7 (3.5%) with Ebstein's anomaly and 108 (54.0%) with pulmonary atresia, with 304 matched controls and 38 151 population controls without any defects. Main outcome measures Risk of any RSODH and risk of each type of RSODH. Results High blood pressure, particularly chronic hypertension with nifedipine treatment, was associated with a risk for RSODH (OR 7.03, 95% CI 3.13 to 13.84). High doses of folic acid reduced the birth prevalence of pulmonary atresia (OR 0.29, 95% CI 0.16 to 0.53). Conclusions The multifactorial threshold model provides the best explanation for the origins of RSODH. Genetic predisposition may be triggered by maternal hypertension with nifedipine treatment, while the risk for pulmonary atresia is reduced by high doses of folic acid in early pregnancy. AD - A.E. Czeizel, Foundation for the Community Control of Hereditary Diseases, Törökvész lejto 32, Budapest, Hungary AU - Csáky-Szunyogh, M. AU - Vereczkey, A. AU - Gerencsér, B. AU - Czeizel, A. E. C1 - cordaflex(Egis) DB - Embase DO - 10.1136/heartasia-2013-010331 IS - 1 KW - folic acid nifedipine allergic rhinitis article autopsy congenital disorder congenital heart malformation constipation controlled study diabetes mellitus drug exposure drug megadose dyspepsia Ebstein anomaly female hemorrhoid human hypertension hypotension live birth major clinical study maternal hypertension migraine mother newborn panic population based case control study pulmonary valve atresia pulmonary valve stenosis right sided obstructive defects of the heart risk assessment skeleton tricuspid valve atresia tricuspid valve stenosis varicosis cordaflex LA - English M3 - Article N1 - L600215386 2014-10-29 2014-11-04 PY - 2014 SN - 1759-1104 SP - 3-7 ST - Maternal hypertension with nifedipine treatment associated with a higher risk for right-sided obstructive defects of the heart: A population-based case - Control study T2 - Heart Asia TI - Maternal hypertension with nifedipine treatment associated with a higher risk for right-sided obstructive defects of the heart: A population-based case - Control study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L600215386 http://dx.doi.org/10.1136/heartasia-2013-010331 VL - 6 ID - 962 ER - TY - JOUR AB - Seventeen newborns in a general hospital had a successful atrial septostomy when indicated. Mostly done at the bedside under echocardiographical guidance, the successful introduction of this procedure enabled the infants to remain with mother to facilitate feeding and bonding prior to elective transfer to a children's hospital for corrective surgery. BACKGROUND: Atrial septostomy is a well-recognised intervention in the newborn to facilitate atrial mixing in transposition of the great arteries (TGA) or to decompress an atrium where the connecting AV valve is absent or stenosed, e.g. tricuspid atresia (TA). AIMS: To review the outcome of this procedure in a general hospital with appropriate neonatal and cardiological facilities. METHODS: Retrospective review over an 11-year period. RESULTS: Seventeen inborn infants had successful atrial septostomies, 11 with TGA and 6 with TA. All done under general anaesthesia, 15 were performed in the newborn nurseries, under echocardiographic guidance, and 2 in the catheter laboratory. No complications occurred. Eleven had a prenatal diagnosis made. All infants were able to be subsequently nursed by their mothers, affording prime time facilitating feeding and bonding. They were electively transferred to a children's hospital for corrective surgery. CONCLUSIONS: Atrial septostomy can be safely performed in a general hospital with appropriate neonatal and cardiological expertise. Such intervention allows for elective transfer of the infant for corrective surgery, allowing the infant and mother to be initially cared for at the one hospital, thereby facilitating maternal contact, feeding and bonding, doing away with the added stress of emergency transfer. AD - Paediatric Cardiology Unit, Monash University, Melbourne, Victoria, Australia. AN - 12614946 AU - See, P. L. AU - David, E. AU - Anderson-Weller, K. AU - Fong, L. V. AU - Menahem, S. DA - Feb DB - PubMed DO - 10.1016/s0378-3782(02)00071-3 DP - NLM ET - 2003/03/05 IS - 1 KW - Cardiac Catheterization Catheterization/*methods *Cyanosis/etiology/psychology/therapy Echocardiography Female *Heart Septum/diagnostic imaging Hospitals, General Humans Infant, Newborn *Mother-Child Relations Object Attachment Retrospective Studies Transposition of Great Vessels/complications/diagnostic imaging/*therapy Tricuspid Atresia/complications/diagnostic imaging/*therapy LA - eng N1 - See, Pei Lee David, Elizabeth Anderson-Weller, Karen Fong, Lance V Menahem, Samuel Journal Article Ireland Early Hum Dev. 2003 Feb;71(1):9-17. doi: 10.1016/s0378-3782(02)00071-3. PY - 2003 SN - 0378-3782 (Print) 0378-3782 SP - 9-17 ST - Maternal infant bonding enhanced after atrial septostomy in cyanotic neonates in a general hospital T2 - Early Hum Dev TI - Maternal infant bonding enhanced after atrial septostomy in cyanotic neonates in a general hospital VL - 71 ID - 509 ER - TY - JOUR AB - Tinker SC, Reefhuis J, Dellinger AM, Jamieson DJ, the National Birth Defects Prevention Study. Maternal injuries during the periconceptional period and the risk of birth defects, National Birth Defects Prevention Study, 1997-2005. Paediatric and Perinatal Epidemiology 2011; 25: 487-496. Maternal injuries during pregnancy are common (â̂7% prevalence). However, few studies have examined the association between maternal injuries and birth defects. The National Birth Defects Prevention Study is a population-based case-control study of birth defects in 10 US states. Cases were ascertained through surveillance; controls were randomly selected from infants delivered without major birth defects in the study regions. Mothers completed a telephone interview on exposures before and during pregnancy, including injuries. We assessed associations between periconceptional (month before until the end of the third month of pregnancy) maternal injuries and birth defects. We used logistic regression to estimate adjusted odds ratios (AORs) and 95% confidence intervals (CI). Periconceptional injuries were associated with interrupted aortic arch type B [AOR = 5.2, 95% CI 1.2, 23.2]; atrioventricular septal defect [AOR = 2.2, 95% CI 1.1, 4.4]; pulmonary atresia [AOR = 3.2, 95% CI 1.6, 6.4]; tricuspid atresia [AOR = 2.8, 95% CI 1.2, 6.7]; hypoplastic left heart syndrome [AOR = 2.0, 95% CI 1.1, 3.4]; anorectal atresia/stenosis [AOR = 1.7, 95% CI 1.0, 2.7]; longitudinal limb deficiency [AOR = 2.1, 95% CI 1.1, 3.9]; and gastroschisis [AOR = 1.8, 95% CI 1.2, 2.8]. Associations with longitudinal limb deficiency, gastroschisis and hypoplastic left heart syndrome were stronger for intentional injuries. Our results suggest maternal injury during the periconceptional period, particularly those inflicted intentionally, may be associated with select birth defects. This analysis was hypothesis-generating, with many associations tested. Further research is warranted. © 2011 Blackwell Publishing Ltd. AD - S.C. Tinker, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention (CDC), Mail-Stop E-86, 1600 Clifton Road NE, Atlanta, GA 30333, United States AU - Tinker, S. C. AU - Reefhuis, J. AU - Dellinger, A. M. AU - Jamieson, D. J. DB - Embase Medline DO - 10.1111/j.1365-3016.2011.01215.x IS - 5 KW - adult amnion band syndrome anencephalus anophthalmia anterior eye chamber disease anus atresia aortic arch interruption aortic coarctation aortic stenosis article automutilation bile duct atresia cholestasis congenital malformation bladder exstrophy cleft lip palate congenital cataract controlled study craniofacial synostosis diaphragm hernia disease association encephalocele esophagus atresia esophagus stenosis Fallot tetralogy female gastroschisis glaucoma great vessels transposition heart atrium septum defect heart right ventricle double outlet heart ventricle septum defect holoprosencephaly human hydrocephalus hypoplastic left heart syndrome hypospadias interview intestine atresia intestine stenosis kidney agenesis limb defect lung vein drainage anomaly major clinical study maternal disease microphthalmia microtia omphalocele pregnancy pulmonary valve atresia pulmonary valve stenosis spinal dysraphism statistical analysis telephone tricuspid valve atresia LA - English M3 - Article N1 - L51539322 2011-07-28 2011-08-19 PY - 2011 SN - 0269-5022 1365-3016 SP - 487-496 ST - Maternal injuries during the periconceptional period and the risk of birth defects, National Birth Defects Prevention Study, 1997-2005 T2 - Paediatric and Perinatal Epidemiology TI - Maternal injuries during the periconceptional period and the risk of birth defects, National Birth Defects Prevention Study, 1997-2005 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51539322 http://dx.doi.org/10.1111/j.1365-3016.2011.01215.x VL - 25 ID - 1095 ER - TY - JOUR AB - BACKGROUND: The objective of this study was to assess, in a large data set from Swedish Medical Health Registries, whether maternal obesity and maternal morbid obesity were associated with an increased risk for various structural birth defects. METHODS: The study population consisted of 1,049,582 infants born in Sweden from January 1, 1995, through December 31, 2007, with known maternal weight and height data. Women were grouped in six categories of body mass index (BMI) according to World Health Organization classification. Infants with congenital birth defects were identified from three sources: the Swedish Medical Birth Registry, the Register of Birth Defects, and the National Patient Register. Maternal age, parity, smoking, and year of birth were thought to be potential confounders and were included as covariates in the adjusted odds ratio analyses. RESULTS: Ten percent of the study population was obese. Morbid obesity (BMI ≥ 40) occurred in 0.7%. The prevalence of congenital malformations was 4.7%, and the prevalence of relatively severe malformations was 3.2%. Maternal prepregnancy morbid obesity was associated with neural tube defects OR 4.08 (95% CI 1.87-7.75), cardiac defects OR 1.49 (95% CI 1.24-1.80), and orofacial clefts OR 1.90 (95% CI 1.27-2.86). Maternal obesity (BMI ≥ 30) significantly increased the risk of hydrocephaly, anal atresia, hypospadias, cystic kidney, pes equinovarus, omphalocele, and diaphragmatic hernia. CONCLUSION: The risk for a morbidly obese pregnant woman to have an infant with a congenital birth defect is small, but for society the association is important in the light of the ongoing obesity epidemic. © 2009 Wiley-Liss, Inc. AD - M. I. Blomberg, Division of Obstetrics and Gynaecology, Department of Clinical and Experimental Medicine, University of Linköping, SE-581 85 Linköping, Sweden AU - Blomberg, M. I. AU - Källén, B. DB - Embase Medline DO - 10.1002/bdra.20620 IS - 1 KW - abdominal wall defect anus atresia article congenital malformation body height body mass body weight choana atresia cigarette smoking cleft face congenital heart malformation controlled study craniofacial synostosis diaphragm hernia disease severity ear malformation esophagus atresia Fallot tetralogy female great vessels transposition high risk pregnancy human hydrocephalus hypoplastic left heart syndrome hypospadias infant intestine atresia kidney agenesis kidney polycystic disease limb reduction defect major clinical study maternal age maternal disease maternal obesity maternal smoking microcephaly microphthalmia morbid obesity mouth malformation neural tube defect obesity omphalocele orofacial cleft parity clubfoot priority journal progeny risk factor Sweden LA - English M3 - Article N1 - L358221303 2010-02-11 2010-03-02 http://www3.interscience.wiley.com/cgi-bin/fulltext/122578467/PDFSTART PY - 2010 SN - 1542-0752 1542-0760 SP - 35-40 ST - Maternal obesity and morbid obesity: The risk for birth defects in the offspring T2 - Birth Defects Research Part A - Clinical and Molecular Teratology TI - Maternal obesity and morbid obesity: The risk for birth defects in the offspring UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L358221303 http://dx.doi.org/10.1002/bdra.20620 VL - 88 ID - 1153 ER - TY - JOUR AB - OBJECTIVE: To investigate the existence of racial/ethnic disparity in mortality risk among children with individual congenital heart defects and identify any other risk factors. STUDY DESIGN: The study cohort, comprising children born between 1983 and 2006 with a selected congenital heart defect, was matched to death records to ascertain vital status. The birth and maternal risk factors were obtained from birth certificates. RESULTS: After adjusting for covariates using a multivariate regression model, the risk of mortality was significantly higher in children of non-Hispanic black mothers with transposition of the great arteries (hazard ratio (HR), 1.31; 95% CI, 1.07-1.60), tetralogy of Fallot (HR, 1.34; 95% CI, 1.06-1.69), and coarctation of the aorta (HR, 1.40; 95% CI, 1.10-1.79), compared with children of non-Hispanic white mothers. Time trends analysis examining the mortality risk by survival age and birth period found a significant decrease in 5-year mortality risk from 1983 to 2003 births, with a nearly 50% reduction for hypoplastic left heart syndrome and coarctation of the aorta across 3 maternal racial/ethnic groups examined. CONCLUSION: Our findings may help identify at-risk populations and mortality risk factors and thereby contribute to improved survival and quality of life for these children across the lifespan. AD - Congenital Malformations Registry, Bureau of Environmental and Occupational Epidemiology, Center for Environmental Health, New York State Department of Health, Albany, NY; School of Public Health, State University of New York, Albany, NY. Electronic address: wxy01@health.state.ny.us. AN - 23932315 AU - Wang, Y. AU - Liu, G. AU - Druschel, C. M. AU - Kirby, R. S. DA - Nov DB - PubMed DO - 10.1016/j.jpeds.2013.06.084 DP - NLM ET - 2013/08/13 IS - 5 KW - Adolescent Adult African Americans Aortic Coarctation/ethnology Child Child, Preschool Cohort Studies Disease Susceptibility European Continental Ancestry Group Female Heart Defects, Congenital/*ethnology/mortality Hispanic Americans Humans Infant Infant, Newborn Male *Mothers Multivariate Analysis Proportional Hazards Models Risk Factors Transposition of Great Vessels/ethnology Treatment Outcome Young Adult Ca Chd Cmr Coarctation of the aorta Congenital Malformations Registry Congenital heart defect Hlhs Hr Hazard ratio Hypoplastic left heart syndrome Icd-9-cm International Classification of Diseases, Ninth Revision, Clinical Modification Nh Non-Hispanic Tga Tof Tetralogy of Fallot Transposition of the great arteries Vsd Ventricular septal defect LA - eng N1 - 1097-6833 Wang, Ying Liu, Gang Druschel, Charlotte M Kirby, Russell S Journal Article United States J Pediatr. 2013 Nov;163(5):1437-42.e1-2. doi: 10.1016/j.jpeds.2013.06.084. Epub 2013 Aug 6. PY - 2013 SN - 0022-3476 SP - 1437-42.e1-2 ST - Maternal race/ethnicity and survival experience of children with congenital heart disease T2 - J Pediatr TI - Maternal race/ethnicity and survival experience of children with congenital heart disease VL - 163 ID - 190 ER - TY - JOUR AB - Background: Birth defects are responsible for a large proportion of disability and infant mortality. Exposure to a variety of pesticides have been linked to increased risk of birth defects. Methods: We conducted a case-control study to estimate the associations between a residence-based metric of agricultural pesticide exposure and birth defects. We linked singleton live birth records for 2003 to 2005 from the North Carolina (NC) State Center for Health Statistics to data from the NC Birth Defects Monitoring Program. Included women had residence at delivery inside NC and infants with gestational ages from 20 to 44 weeks (n=304,906). Pesticide exposure was assigned using a previously constructed metric, estimating total chemical exposure (pounds of active ingredient) based on crops within 500 meters of maternal residence, specific dates of pregnancy, and chemical application dates based on the planting/harvesting dates of each crop. Logistic regression was used to estimate odds ratios (ORs) and 95% confidence intervals for four categories of exposure (<10th, 10-50th, 50-90th, and >90th percentiles) compared with unexposed. Models were adjusted for maternal race, age at delivery, education, marital status, and smoking status. Results: We observed elevated ORs for congenital heart defects and certain structural defects affecting the gastrointestinal, genitourinary and musculoskeletal systems (e.g., OR [95% confidence interval] [highest exposure vs. unexposed] for tracheal esophageal fistula/esophageal atresia=1.98 [0.69, 5.66], and OR for atrial septal defects: 1.70 [1.34, 2.14]). Conclusion: Our results provide some evidence of associations between residential exposure to agricultural pesticides and several birth defects phenotypes. AD - K.M. Rappazzo, Oak Ridge Institute for Science and Education at the U.S. Environmental Protection Agency, National Center for Environmental Assessment, U.S. Environmental Protection Agency, Research Triangle Park, NC, United States AU - Rappazzo, K. M. AU - Warren, J. L. AU - Meyer, R. E. AU - Herring, A. H. AU - Sanders, A. P. AU - Brownstein, N. C. AU - Luben, T. J. DB - Embase Medline DO - 10.1002/bdra.23479 IS - 4 KW - pesticide age amnion band syndrome anencephalus anophthalmia anorectal stenosis anotia aortic coarctation aortic valve stenosis arm malformation article atrioventricular septal defect bile duct atresia case control study central nervous system malformation choana atresia cleft lip cleft palate cohort analysis congenital cataract congenital diaphragm hernia congenital malformation controlled study disease association Ebstein anomaly educational status encephalocele environmental exposure epispadias esophagus atresia Fallot tetralogy female gastroschisis great vessels transposition harvesting heart atrium septum defect heart ventricle septum defect Hirschsprung disease human hydrocephalus hypertrophic pylorus stenosis hypoplastic left heart syndrome hypospadias infant kidney agenesis leg malformation live birth logistic regression analysis major clinical study male marriage microcephaly microphthalmia microtia obstructive genitourinary defect omphalocele patent ductus arteriosus pregnancy priority journal pulmonary valve atresia race residential area smoking spinal dysraphism tracheal esophageal fistula tricuspid valve atresia trisomy 13 trisomy 18 trisomy 21 United States LA - English M3 - Article N1 - L609031304 2016-03-21 2016-05-02 PY - 2016 SN - 1542-0760 1542-0752 SP - 240-249 ST - Maternal residential exposure to agricultural pesticides and birth defects in a 2003 to 2005 North Carolina birth cohort T2 - Birth Defects Research Part A - Clinical and Molecular Teratology TI - Maternal residential exposure to agricultural pesticides and birth defects in a 2003 to 2005 North Carolina birth cohort UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L609031304 http://dx.doi.org/10.1002/bdra.23479 VL - 106 ID - 813 ER - TY - JOUR AB - OBJECTIVES.In a population-based case-control study, we investigated the association between congenital heart defects and maternal smoking. METHODS.The National Birth Defects Prevention Study enrolled 3067 infants with nonsyndromic congenital heart defects and their parents and 3947 infants without birth defects and their parents. Affected infants had ≥1 of the following defects: conotruncal, septal, anomalous pulmonary venous return, atrioventricular septal defects, and left-sided or right-sided obstructive heart defects. Mothers of case and control infants were asked if they smoked during the periconceptional period, defined as 1 month before pregnancy through the first trimester. Maternal home and workplace exposure to tobacco smoke during the same period was also determined. Logistic regression was used to compute odds ratios and 95% confidence intervals while controlling for potential confounders. RESULTS. Case infants were more likely to be premature and have lower birth weight than control infants. Women who smoked anytime during the month before pregnancy to the end of the first trimester were more likely to have infants with septal heart defects than women who did not smoke during this time period. This association was stronger for mothers who reported heavier smoking during this period. This relation was independent of potential confounding factors, including prenatal vitamin use, alcohol intake, maternal age, and race or ethnicity. Women who smoked ≥25 cigarettes per day were more likely than nonsmoking mothers to have infants with right-sided obstructive defects. There was no increased risk of congenital heart defects with maternal exposure to environmental tobacco smoke. CONCLUSIONS. Maternal smoking during pregnancy was associated with septal and right- sided obstructive defects. Additional investigation into the timing of tobacco exposure and genetic susceptibilities that could modify this risk will provide a more precise evidence base on which to build clinical and public health primary prevention strategies. © 2008 by the American Academy ofPediatrics. AD - C. A. Hobbs, Department of Pediatrics, College of Medicine, University of Arkansas for Medical Sciences, 1120 Marshall St., Little Rock, AR 72202 AU - Malik, S. AU - Cleves, M. A. AU - Honein, M. A. AU - Romitti, P. A. AU - Botto, L. D. AU - Yang, S. AU - Hobbs, C. A. DB - Embase Medline DO - 10.1542/peds.2007-1519 IS - 4 KW - tobacco smoke adult alcohol consumption aortic arch interruption aortic coarctation article comparative study congenital heart malformation controlled study disease association ethnicity Fallot tetralogy female first trimester pregnancy great vessels transposition heart atrium septum defect heart ventricle septum defect home environment human hypoplastic left heart syndrome infant low birth weight major clinical study male maternal age maternal care maternal smoking patent ductus arteriosus population based case control study prematurity prenatal exposure priority journal pulmonary valve stenosis pulmonary vein malformation tricuspid valve atresia venous return vitamin supplementation workplace LA - English M3 - Article N1 - L354416788 2009-04-28 http://pediatrics.aappublications.org/cgi/reprint/121/4/e810 PY - 2008 SN - 0031-4005 1098-4275 SP - e810-e816 ST - Maternal smoking and congenital heart defects T2 - Pediatrics TI - Maternal smoking and congenital heart defects UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L354416788 http://dx.doi.org/10.1542/peds.2007-1519 VL - 121 ID - 1216 ER - TY - JOUR AB - Study question Is oral contraceptive use around the time of pregnancy onset associated with an increased risk of major birth defects? Methods In a prospective observational cohort study, data on oral contraceptive use and major birth defects were collected among 880 694 live births from Danish registries between 1997 and 2011. We conservatively assumed that oral contraceptive exposure lasted up to the most recently filled prescription. The main outcome measure was the number of major birth defects throughout one year follow-up (defined according to the European Surveillance of Congenital Anomalies classification). Logistic regression estimated prevalence odds ratios of any major birth defect as well as categories of birth defect subgroups. Study answer and limitations Prevalence of major birth defects (per 1000 births) was consistent across each oral contraceptive exposure group (25.1, never users; 25.0, use >3 months before pregnancy onset (reference group); 24.9, use 0-3 months before pregnancy onset (that is, recent use); 24.8, use after pregnancy onset). No increase in prevalence of major birth defects was seen with oral contraceptive exposure among women with recent use before pregnancy (prevalence odds ratio 0.98 (95% confidence interval 0.93 to 1.03)) or use after pregnancy onset (0.95 (0.84 to 1.08)), compared with the reference group. There was also no increase in prevalence of any birth defect subgroup (for example, limb defects). It is unknown whether women took oral contraceptives up to the date of their most recently filled prescription. Also, the rarity of birth defects made disaggregation of the results difficult. Residual confounding was possible, and the analysis lacked information on folate, one of the proposed mechanisms. What this study adds Oral contraceptive exposure just before or during pregnancy does not appear to be associated with an increased risk of major birth defects. Funding, competing interests, data sharing BMC was funded by the Harvard T H Chan School of Public Health's Maternal Health Task Force and Department of Epidemiology Rose Traveling Fellowship; training grant T32HD060454 in reproductive, perinatal, and paediatric epidemiology and award F32HD084000 from the Eunice Kennedy Shriver National Institute of Child Health and Human Development; and grant T32CA09001 from the National Cancer Institute. The authors have no competing interests or additional data to share. AD - B.M. Charlton, Department of Epidemiology, Harvard T H Chan School of Public Health, Boston, MA, United States AU - Charlton, B. M. AU - Mølgaard-Nielsen, D. AU - Svanström, H. AU - Wohlfahrt, J. AU - Pasternak, B. AU - Melbye, M. DB - Embase Medline DO - 10.1136/bmj.h6712 KW - oral contraceptive agent abdominal wall defect adult article cohort analysis congenital heart malformation congenital malformation controlled study Denmark digestive system malformation disease association ear malformation eye malformation face malformation female follow up gastroschisis genital malformation human hypoplastic left heart syndrome induced abortion infant limb malformation live birth major clinical study neck malformation nervous system malformation observational study oral contraceptive use pregnancy prescription prevalence priority journal propensity score prospective study register respiratory tract malformation stillbirth urinary tract malformation LA - English M3 - Article N1 - L608676669 2016-03-09 2016-03-15 PY - 2016 SN - 1756-1833 0959-8146 ST - Maternal use of oral contraceptives and risk of birth defects in Denmark: Prospective, nationwide cohort study T2 - BMJ (Online) TI - Maternal use of oral contraceptives and risk of birth defects in Denmark: Prospective, nationwide cohort study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L608676669 http://dx.doi.org/10.1136/bmj.h6712 VL - 352 ID - 829 ER - TY - JOUR AB - Women with pre-gestational diabetes have a higher risk of producing children with congenital heart defects (CHDs), caused predominantly by hyperglycemia-induced oxidative stress. In this study, we evaluated if exercise during pregnancy could mitigate oxidative stress and reduce the incidence of CHDs in the offspring of diabetic mice. Female mice were treated with streptozotocin to induce pre-gestational diabetes, then mated with healthy males to produce offspring. They were also given access to running wheels 1 week before mating and allowed to exercise voluntarily until E18.5. Heart morphology, gene expression, and oxidative stress were assessed in foetal hearts. Maternal voluntary exercise results in a significantly lower incidence of CHDs from 59.5% to 25%. Additionally, diabetes-induced defects in coronary artery and capillary morphogenesis were also lower with exercise. Myocardial cell proliferation and epithelial-mesenchymal transition at E12.5 was significantly lower with pre-gestational diabetes which was mitigated with maternal exercise. Cardiac gene expression of Notch1, Snail1, Gata4 and Cyclin D1 was significantly higher in the embryos of diabetic mice that exercised compared to the non-exercised group. Furthermore, maternal exercise produced lower reactive oxygen species (ROS) and oxidative stress in the foetal heart. In conclusion, maternal exercise mitigates ROS and oxidative damage in the foetal heart, and results in a lower incidence of CHDs in the offspring of pre-gestational diabetes. Exercise may be an effective intervention to compliment clinical management and further minimize CHD risk in mothers with diabetes. AD - Q. Feng, Department of Physiology and Pharmacology, Schulich School of Medicine and Dentistry, University of Western Ontario, Children's Health Research Institute, London, ON, Canada AU - Saiyin, T. AU - Engineer, A. AU - Greco, E. R. AU - Kim, M. Y. AU - Lu, X. AU - Jones, D. L. AU - Feng, Q. DB - Embase Medline DO - 10.1111/jcmm.14439 IS - 8 KW - bone morphogenetic protein cyclin D1 endothelial nitric oxide synthase glucose hypoxia inducible factor 1alpha Notch1 receptor reactive oxygen metabolite RNA 28S superoxide dismutase transcription factor GATA 4 transcription factor Snail animal experiment animal model animal tissue article atrioventricular septal defect autism cell proliferation congenital blood vessel malformation congenital heart malformation controlled study densitometry embryo epithelial mesenchymal transition exercise exercise intensity female fetus gene expression glucose intake heart right ventricle double outlet heart ventricle septum defect histology hypoplastic left heart syndrome image reconstruction lipid peroxidation male mouse nonhuman oxidative stress pregnancy pregnancy diabetes mellitus pregnancy outcome priority journal progeny protein expression real time polymerase chain reaction reverse transcription polymerase chain reaction streptozotocin-induced diabetes mellitus three-dimensional imaging upregulation Western blotting LA - English M3 - Article N1 - L628693702 2019-08-02 2019-08-07 PY - 2019 SN - 1582-1838 SP - 5553-5565 ST - Maternal voluntary exercise mitigates oxidative stress and incidence of congenital heart defects in pre-gestational diabetes T2 - Journal of Cellular and Molecular Medicine TI - Maternal voluntary exercise mitigates oxidative stress and incidence of congenital heart defects in pre-gestational diabetes UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L628693702 http://dx.doi.org/10.1111/jcmm.14439 VL - 23 ID - 606 ER - TY - JOUR AU - Devereaux, M. AU - Kon, A. A. DB - Medline DO - 10.1080/15265161.2017.1314047 IS - 7 KW - child parent relation education ethics home care human hypoplastic left heart syndrome infant newborn palliative therapy parental consent patient education prognosis psychology treatment withdrawal LA - English M3 - Article N1 - L625030961 2018-11-22 2019-07-05 PY - 2017 SN - 1536-0075 SP - 72-74 ST - May We Take Our Baby With Hypoplastic Left Heart Syndrome Home? T2 - The American journal of bioethics : AJOB TI - May We Take Our Baby With Hypoplastic Left Heart Syndrome Home? UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L625030961 http://dx.doi.org/10.1080/15265161.2017.1314047 VL - 17 ID - 746 ER - TY - JOUR AB - Background: Exercise capacity in grown-ups with congenital heart disease (GUCH) is mostly reported by peak oxygen consumption (peak VO2). Our aim was to evaluate the maximal character of exercise tests, and to investigate submaximal measures of exercise capacity. Methods: Adults with Coarctation of the Aorta (COA, n = 155), Tetralogy of Fallot (TOF, n = 98), dextro-Transposition of the Great Arteries (dTGA, n = 68) and Univentricular Heart (UVH, n = 10), and 122 healthy adults performed cardiopulmonary exercise testing until exhaustion. Gas exchange was measured breath by breath. The maximal performance of the test was evaluated by respiratory exchange ratio (RER), ventilatory equivalent for oxygen and Borg scale. Oxygen uptake efficiency slope (OUES), VE/VCO2 slope and VO2/WR slope were calculated and ventilatory anaerobic threshold (VAT) was defined. Correlations of these measures with peak VO2 were calculated. Results: GUCH showed significantly lower peak VO2 than controls (p < 0.001), declining from 80% in COA, 74% in TOF, 64% in dTGA, to 55% in UVH. Compared to suggested criteria, mean peak RER and median Borg scale indicated a maximal effort in GUCH, however these results were significantly lower than controls (p < 0.05). OUES, VO2/WR slope and VAT were significantly lower in patients compared to controls. OUES (r = 0.853) and VAT (r = 0.840) correlated best with peak VO2; VO2/WR slope (r = 0.551) and VE/VCO2 slope (r = -0.421) correlated to a lesser degree (p < 0.001). Conclusion: The investigated GUCH show reduced exercise tolerance compared to controls, related to the underlying heart defect. Different expressions of exercise tolerance clearly reveal the same differences in exercise capacity across groups of GUCH. © 2010 Elsevier Ireland Ltd. AD - L. Vanhees, Department of Rehabilitation Sciences, Tervuursevest 101 - bus 01501, B-3001 Heverlee, Belgium AU - Buys, R. AU - Cornelissen, V. AU - De Bruaene, A. V. AU - Stevens, A. AU - Coeckelberghs, E. AU - Onkelinx, S. AU - Thomaes, T. AU - Delecluse, C. AU - Budts, W. AU - Vanhees, L. DB - Embase Medline DO - 10.1016/j.ijcard.2010.08.030 IS - 1 KW - accuracy adolescent adult anaerobic threshold article capnometry carbon dioxide volume cardiopulmonary exercise test cardiovascular parameters congenital heart disease controlled study disease association exercise intensity exercise tolerance female heart function human intermethod comparison lung gas exchange major clinical study male oxygen uptake efficiency slope priority journal task performance ventilatory efficiency slope ventilatory equivalent for oxygen LA - English M3 - Article N1 - L51068863 2010-09-21 2011-12-06 PY - 2011 SN - 0167-5273 1874-1754 SP - 26-30 ST - Measures of exercise capacity in adults with congenital heart disease T2 - International Journal of Cardiology TI - Measures of exercise capacity in adults with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51068863 http://dx.doi.org/10.1016/j.ijcard.2010.08.030 VL - 153 ID - 1083 ER - TY - JOUR AB - We are developing a collapsible, percutaneously inserted, axial flow blood pump to support the cavopulmonary circulation in infants with a failing single ventricle physiology. An initial design of the impeller for this axial flow blood pump was performed using computational fluid dynamics analysis, including pressure-flow characteristics, scalar stress estimations, blood damage indices, and fluid force predictions. A plastic prototype was constructed for hydraulic performance testing, and these experimental results were compared with the numerical predictions. The numerical predictions and experimental findings of the pump performance demonstrated a pressure generation of 2-16 mm Hg for 50-750 ml/min over 5,500-7,500 RPM with deviation found at lower rotational speeds. The axial fluid forces remained below 0.1 N, and the radial fluid forces were determined to be virtually zero due to the centered impeller case. The scalar stress levels remained below 250 Pa for all operating conditions. Blood damage analysis yielded a mean residence time of the released particles, which was found to be less than 0.4 seconds for both flow rates that were examined, and a maximum residence time was determined to be less than 0.8 seconds. We are in the process of designing a cage with hydrodynamically shaped filament blades to act as a diffuser and optimizing the impeller blade shape to reduce the flow vorticity at the pump outlet. This blood pump will improve the clinical treatment of patients with failing Fontan physiology and provide a unique catheter-based therapeutic approach as a bridge to recovery or transplantation. AD - Department of Mechanical Engineering, Virginia Commonwealth University, Richmond, Virginia 23284 - USA Department of Mechanical Engineering, Virginia Commonwealth University, Richmond, Virginia - USA. AN - 105619623. Language: English. Entry Date: 20090306. Revision Date: 20191030. Publication Type: journal article AU - Throckmorton, A. L. AU - Kapadia, J. AU - Madduri, D. AU - Throckmorton, A. L. AU - Kapadia, J. AU - Madduri, D. DB - ccm DP - EBSCOhost IS - 11 KW - Heart Assist Devices -- Adverse Effects Heart Catheterization -- Equipment and Supplies Heart Defects, Congenital -- Therapy Hemodynamics Pulmonary Circulation Cardiopulmonary Bypass Computer Simulation Computing Methodologies Heart Defects, Congenital -- Physiopathology Infant Infant, Newborn Materials Testing Models, Biological Prosthesis Design Prosthesis Failure Stress, Mechanical N1 - research. Journal Subset: Biomedical; Blind Peer Reviewed; Continental Europe; Double Blind Peer Reviewed; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed. NLM UID: 7802649. PMID: NLM19089799. PY - 2008 SN - 0391-3988 SP - 970-982 ST - Mechanical axial flow blood pump to support cavopulmonary circulation T2 - International Journal of Artificial Organs TI - Mechanical axial flow blood pump to support cavopulmonary circulation UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105619623&site=ehost-live&scope=site VL - 31 ID - 1617 ER - TY - JOUR AB - This study investigated the performance of a magnetically levitated, intravascular axial flow blood pump for mechanical circulatory support of the thousands of Fontan patients in desperate need of a therapeutic alternative. Four models of the extracardiac, total cavopulmonary connection (TCPC) Fontan configuration were evaluated to formulate numerical predictions: an idealized TCPC, a patient-specific TCPC per magnetic resonance imaging data, and each of these two models having a blood pump in the inferior vena cava (IVC). A lumped parameter model of the Fontan physiology was used to specify boundary conditions. Pressure-flow characteristics, energy gain calculations, scalar stress levels, and blood damage estimations were executed for each model. Suction limitation experiments using the Sylgard elastomer tubing were also conducted. The pump produced pressures of 1-16mmHg for 2000-6000rpm and flow rates of 0.5-4.5L/min. The pump inlet or IVC pressure was found to decrease at higher rotational speeds. Maximum scalar stress estimations were 3Pa for the nonpump models and 290Pa for the pump-supported cases. The blood residence times for the pump-supported cases were shorter (0.9s) as compared with the nonsupported configurations (2.5s). However, the blood damage indices were higher (1.5%) for the anatomic model with pump support. The pump successfully augmented pressure in the TCPC junction and increased the hydraulic energy of the TCPC as a function of flow rate and rotational speed. The suction experiments revealed minimal deformation (<3%) at 9000rpm. The findings of this study support the continued design and development of this blood pump. © 2011, Copyright the Authors. Artificial Organs © 2011, International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc. AD - Department of Mechanical Engineering, School of Engineering, Virginia Commonwealth University, United States Department of Radiology, School of Medicine, Virginia Commonwealth University, United States Division of Pediatric Cardiology, Children's Hospital of Richmond and School of Medicine, Virginia Commonwealth University, Richmond, VA, United States Department of Mechanical Engineering, School of Engineering, Behrend College, The Pennsylvania State University, Erie, PA, United States AU - Throckmorton, A. L. AU - Carr, J. P. AU - Tahir, S. A. AU - Tate, R. AU - Downs, E. A. AU - Bhavsar, S. S. AU - Wu, Y. AU - Grizzard, J. D. AU - Moskowitz, W. B. DB - Scopus DO - 10.1111/j.1525-1594.2011.01339.x IS - 11 KW - Artificial right ventricle Blood pump Cavopulmonary assist device Computational fluid dynamics Fontan physiology Mechanical cavopulmonary assist Pediatric circulatory support Single ventricle physiology Total cavopulmonary connection M3 - Article N1 - Cited By :22 Export Date: 15 June 2020 PY - 2011 SP - 1036-1047 ST - Mechanical Cavopulmonary Assistance of a Patient-Specific Fontan Physiology: Numerical Simulations, Lumped Parameter Modeling, and Suction Experiments T2 - Artificial Organs TI - Mechanical Cavopulmonary Assistance of a Patient-Specific Fontan Physiology: Numerical Simulations, Lumped Parameter Modeling, and Suction Experiments UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-81855198940&doi=10.1111%2fj.1525-1594.2011.01339.x&partnerID=40&md5=b59ce798ce110ab88d3ff4441b612fa2 VL - 35 ID - 2113 ER - TY - JOUR AB - It is now recognized that a majority of single ventricle patients, those with functionally univentricular hearts, who have survived palliative cavopulmonary connection will experience circulatory failure and end-organ dysfunction due to intrinsic inadequacies of a circulation supported by a single ventricle. Thus, there are an increasing number of patients with functional single ventricles presenting with failing circulations that may benefit from mechanical circulatory support (MCS). The paucity of experience with MCS in this population, even at high volume cardiac centers, contributes to limited available data to guide MCS device selection and management. Thus, a registry of MCS in this population would be beneficial to the field. A conference was convened in January 2012 of pediatric and adult cardiologists, pediatric cardiac intensivists, congenital heart surgeons, and adult cardiothoracic surgeons to discuss the current state of MCS, ventricular assist device, and total artificial heart therapy for patients who have undergone cavopulmonary connection, either superior cavopulmonary connection or total cavopulmonary connection. Specifically, individual experience and challenges with VAD therapy in this population was reviewed and creation of a multiinstitutional registry of MCS/ventricular assist device in this population was proposed. This document reflects the consensus from the meeting and provides a descriptive overview of the registry referred to as Mechanical Support as Failure Intervention in Patients with Cavopulmonary Shunts. © 2013 Wiley Periodicals, Inc. AD - J.W. Rossano, Department of Pediatrics, The Cardiac Center, The Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, United States AU - Rossano, J. W. AU - Woods, R. K. AU - Berger, S. AU - Gaynor, J. W. AU - Ghanayem, N. AU - Morales, D. L. S. AU - Ravishankar, C. AU - Mitchell, M. E. AU - Shah, T. K. AU - Mahr, C. AU - Tweddell, J. S. AU - Adachi, I. AU - Zangwill, S. AU - Wearden, P. D. AU - Icenogle, T. B. AU - Jaquiss, R. D. AU - Rychik, J. DB - Embase Medline DO - 10.1111/chd.12053 IS - 3 KW - article artificial heart assisted circulation biventricular assist device cavopulmonary connection clinical effectiveness congenital heart disease heart transplantation human ischemia left ventricular assist device mechanical circulatory support pediatrician priority journal quality of life right ventricular assist device surgeon thorax surgery ventricular assist device LA - English M3 - Article N1 - L369072243 2013-06-14 2013-06-24 PY - 2013 SN - 1747-079X 1747-0803 SP - 182-186 ST - Mechanical support as failure intervention in patients with cavopulmonary shunts (MFICS): Rationale and aims of a new registry of mechanical circulatory support in single ventricle patients T2 - Congenital Heart Disease TI - Mechanical support as failure intervention in patients with cavopulmonary shunts (MFICS): Rationale and aims of a new registry of mechanical circulatory support in single ventricle patients UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L369072243 http://dx.doi.org/10.1111/chd.12053 VL - 8 ID - 1002 ER - TY - JOUR AB - In children with systemic atrioventricular valve disease (SAVVD), reconstructive surgery is the primary goal. However, in cases with severely dysplastic valves or failed repair, valve replacement is the only option. The purpose of this study was to assess the early and late outcome following mechanical valve replacement in SAVVD. Between 1989 and 2003, 31 children underwent mechanical valve replacement (19 St. Jude Medical, 12 Carbomedics) in SAVVD (27 mitral, 3 tricuspid in corrected transposition of the great arteries, 1 common in an univentricular heart) at our institution. The ages ranged from 3 months to 15 years (mean 4 years) and body weight varied between 4.2 and 57 kg (mean 13.3 kg). The size of prostheses ranged between 16 and 31 mm (mean 23.9 mm). The main indication for valve replacement was severe insufficiency of left atrioventricular valve (84%); 84% of the patients had had a previous cardiac operation. The overall hospital mortality was 6.5% The mean follow up was 7.7 years (range 2-13 years). Ninety percent of children represent sinus rhythm, 87% are in NYHA class I. All patients were placed on a regimen of Phenprocoumon aiming to maintain the international normalized ratio (INR) between 3.0 and 4.0. Since 1994, INR self-management of oral anticoagulation was performed either by the patient or his or her parents. There was no anticoagulation-related complication in this patient group. Mechanical valve replacement in left atrioventricular valve disease carries a low operative risk across the spectrum of pediatric age despite previous operations in most cases. Long-term survival and quality of life are good in nearly all cases. The rate of anticoagulation-related complications is very low, especially when INR self management is performed. AD - Clinic for Thoracic and Cardiovascular Surgery, Heart Center North Rhine-Westphalia, Ruhr-University of Bochum, Bad Oeynhausen, Germany. AN - 16966859 AU - Reiss, N. AU - Blanz, U. AU - Breymann, T. AU - Kind, K. AU - Bairaktaris, A. AU - Körfer, R. DA - Sep-Oct DB - PubMed DO - 10.1097/01.mat.0000242598.30744.24 DP - NLM ET - 2006/09/13 IS - 5 KW - Adolescent Anticoagulants/therapeutic use Child Child, Preschool Female Heart Valve Diseases/surgery *Heart Valve Prosthesis Humans Infant Male Mitral Valve/abnormalities/*surgery Phenprocoumon/therapeutic use Retrospective Studies Treatment Outcome LA - eng N1 - Reiss, Nils Blanz, U Breymann, T Kind, K Bairaktaris, A Körfer, R Journal Article United States ASAIO J. 2006 Sep-Oct;52(5):559-61. doi: 10.1097/01.mat.0000242598.30744.24. PY - 2006 SN - 1058-2916 (Print) 1058-2916 SP - 559-61 ST - Mechanical valve replacement of the systemic atrioventricular valve in children T2 - Asaio j TI - Mechanical valve replacement of the systemic atrioventricular valve in children VL - 52 ID - 201 ER - TY - JOUR AB - A girl received an ABO-incompatible heart transplantation (ABOiHTx) at the age of 3.5 years for failed univentricular palliation with protein-losing enteropathy (PLE). She was born with a hypoplastic left heart syndrome and underwent multistage palliation to a Fontan circulation at 2 years of age. After the Fontan surgery, she developed PLE, necessitating a Fontan revision, followed by a Fontan takedown and eventually HTx, which was performed with a blood group B heart into an O recipient. Right ventricular (RV) failure secondary to increased pulmonary vascular resistance (PVR) evolved immediately after HTx. A temporary right ventricular assist device (RVAD) was implanted and later switched to a pneumatic pulsatile RVAD. With the adaption of PVR on the RVAD, the PLE resolved and the RVAD was explanted. In the following 12 months, she developed multiple relapses of PLE which eventually resolved after exchange of the calcineurin inhibitor. © 2014 Steunstichting ESOT. AD - S. Seitz, Department of Pediatrics, Stollery Childrens Hospital, University of Alberta, Edmonton, AB, Canada AU - Seitz, S. AU - Buchholz, H. AU - Rebeyka, I. AU - Ross, D. AU - West, L. AU - Urschel, S. DB - Embase Medline DO - 10.1111/tri.12294 IS - 7 KW - albumin budesonide cyclosporine diuretic agent mycophenolate mofetil politef rituximab sildenafil spironolactone steroid tacrolimus thymocyte antibody albumin blood level article blood group ABO incompatibility blood group B cardiac graft rejection case report child drug substitution drug withdrawal female Fontan procedure heart catheterization heart right ventricle failure heart transplantation human hypoplastic left heart syndrome left ventricular assist device lung vascular resistance mitral valve atresia Norwood procedure politef implant preschool child priority journal protein losing gastroenteropathy quality of life right ventricular assist device tricuspid valve regurgitation Centrimag Excor Gore-Tex LA - English M3 - Article N1 - L53081262 2014-04-04 2014-07-02 PY - 2014 SN - 1432-2277 0934-0874 SP - e54-e57 ST - Mechanical ventricular assist device as a bridge to recovery post-ABO-incompatible heart transplantation for failed Fontan circulation T2 - Transplant International TI - Mechanical ventricular assist device as a bridge to recovery post-ABO-incompatible heart transplantation for failed Fontan circulation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L53081262 http://dx.doi.org/10.1111/tri.12294 VL - 27 ID - 954 ER - TY - JOUR AB - Chronic heart failure (CHF) in children occurs mostly as a result of systolic dysfunction of the systemic ventricle or of congenital defects leading to large left-to-right shunts and pulmonary overcirculation. The ensuing symptoms and signs are similar in both cases, and include respiratory distress, poor feeding and growth, and hepatic congestion. Grading the severity of the symptoms accurately and reproducibly is important for studying CHF and the response to therapy. The Ross classification for young children and the New York Heart Association classification for older children are frequently utilized for such grading. The standard therapy for CHF in children consists of diuretics, to reduce cardiac preload and improve symptoms, and the maximization of nutritional support. The role of digoxin in treating CHF in children is controversial, especially regarding those children with pulmonary overcirculation where the function of the systemic ventricle is usually well preserved. As the importance of neurohormonal changes in the pathogenesis of worsening CHF is elucidated, newer medications aimed at counteracting such changes are becoming more important in the medical therapy of CHF in children. ACE inhibitors improve function and survival in adults with CHF, and they probably do the same in children with systemic ventricular dysfunction. It is less clear how effective they are in pulmonary overcirculation, but patients with high flow and low pulmonary resistance are most likely to benefit. In infants receiving treatment with ACE inhibitors, it is necessary to monitor for renal insufficiency or renal failure. beta-Adrenoceptor blockade has also been established as an effective therapy for adults with CHF with beneficial effects on survival and left ventricular function. While data for the pediatric population are limited, early studies suggest that beta-adrenoceptor antagonists (beta-blockers) may work well in infants and children with CHF. Caution must be used by starting treatment with very low dosages of beta-blockers and gradually increasing to the desired goals with close monitoring of blood pressure and heart rate. It is clear that larger multicenter trials are crucial to our ability to provide the most appropriate treatment for children with CHF. The demand for effective medical treatment will increase as more patients with palliated single ventricles survive surgery and then develop CHF from dysfunction of a hypertrophic and dilated single ventricle. AD - Division of Pediatric Cardiology, Children's Hospital of Michigan, Wayne State University School of Medicine, Detroit, Michigan 48201, USA. rross@dmc.org AN - 14728050 AU - Ross, R. D. DB - PubMed DO - 10.2165/00129784-200101010-00004 DP - NLM ET - 2004/01/20 IS - 1 KW - Adrenergic beta-Antagonists/pharmacology/therapeutic use Angiotensin-Converting Enzyme Inhibitors/pharmacology/therapeutic use Cardiotonic Agents/therapeutic use Child Clinical Trials as Topic Digoxin/therapeutic use Diuretics/therapeutic use Heart Failure/etiology/physiopathology/*therapy Humans Infant Neurotransmitter Agents/blood Nutrition Therapy LA - eng N1 - Ross, R D Journal Article New Zealand Am J Cardiovasc Drugs. 2001;1(1):37-44. doi: 10.2165/00129784-200101010-00004. PY - 2001 SN - 1175-3277 (Print) 1175-3277 SP - 37-44 ST - Medical management of chronic heart failure in children T2 - Am J Cardiovasc Drugs TI - Medical management of chronic heart failure in children VL - 1 ID - 511 ER - TY - JOUR AB - Megalencephaly, polymicrogyria, polydactyly and hydrocephalus (MPPH) syndrome: a new case with occipital encephalocele and cleft palate: The megalencephaly, polymicrogyria, polydactyly, and hydrocephalus (MPPH) syndrome is quite rarely seen. The four main findings in this syndrome may be accompanied by severe psychomotor retardation, blindness, hypotonia, convulsions, and facial dysmorphism. In this paper, we present a female newborn at 39 weeks gestational age bom to parents who are first degree cousins. Beside the facial dysmorphism and four main features of the MPPH syndrome, the findings on the physical examination of the patient were, hypertonicity, occipital encephalocele, cleft palate, and multiple polyps in the tongue. The presence of occipital encephalocele, cleft palate, and polyps in the tongue in this patient was not reported previously in the literature. AD - N. Demir, Yuzuncu Yil University, School of Medicine, Department of Pediatrics, Division of Neonatology, Hospital of the Yuzuncu Yil University, Van, Turkey AU - Demir, N. AU - Peker, E. AU - Gülşen, I. AU - Kaba, S. AU - Tuncer, O. DB - Embase Medline IS - 4 KW - phenobarbital anticonvulsant therapy article blindness brain ventricle dilatation brain ventricle peritoneum shunt case report chromosome cleft palate clinical feature computer assisted tomography congenital disorder convulsion disseminated intravascular clotting electroencephalography encephalocele epilepsy face dysmorphia female fetus echography first-degree relative gestational age heart single ventricle human intubation karyotype 46,XY lateral brain ventricle MPPH syndrome muscle hypotonia neonatal respiratory distress syndrome newborn newborn intensive care nuclear magnetic resonance imaging occipital encephalocele parent perinatal period physical examination psychomotor retardation sepsis shunt infection tongue disease tongue polyp LA - English M3 - Article N1 - L609404873 2016-04-07 2016-04-21 PY - 2015 SN - 1015-8146 SP - 381-385 ST - Megalencephaly, polymicrogyria, polydactyly and hydrocephalus (MPPH) syndrome: A new case with occipital encephalocele and cleft palate T2 - Genetic Counseling TI - Megalencephaly, polymicrogyria, polydactyly and hydrocephalus (MPPH) syndrome: A new case with occipital encephalocele and cleft palate UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L609404873 VL - 26 ID - 897 ER - TY - JOUR AD - Heart Centre for Children, The Sydney Children's Hospitals Network, Sydney, Australia Discipline of Paediatrics, School of Women's and Children's Health, UNSW Medicine, The University of New South Wales, Sydney, Australia Heart Research Group, Murdoch Children's Research Institute, Melbourne, Australia Department of Cardiac Surgery, The Royal Children's Hospital, Melbourne, Australia Sydney Medical School, The University of Sydney, Sydney, Australia Pain Management Research Institute, Royal North Shore Hospital, Sydney, Australia Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia Department of Cardiology, Boston Children's Hospital, Boston, United States Harvard Medical School, Boston, United States AN - 137704817. Language: English. Entry Date: In Process. Revision Date: 20190729. Publication Type: Article. Supplement Title: 2019 Supplement 4. Journal Subset: Biomedical AU - Marshall, K. AU - d'Udekem, Y. AU - Sholler, G. AU - Costa, D. AU - Celermajer, D. AU - Winlaw, D. AU - Kasparian, N. DB - ccm DO - 10.1016/j.hlc.2019.06.506 DP - EBSCOhost N1 - Continental Europe; Europe. NLM UID: 100963739. PY - 2019 SN - 1443-9506 SP - S351-S351 ST - Mental Health of Parents of Children, Adolescents and Adults with a Fontan Circulation: Findings from the Australian and New Zealand Fontan Registry T2 - Heart, Lung & Circulation TI - Mental Health of Parents of Children, Adolescents and Adults with a Fontan Circulation: Findings from the Australian and New Zealand Fontan Registry UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=137704817&site=ehost-live&scope=site VL - 28 ID - 1488 ER - TY - JOUR AB - A metabolic response to surgery has recently been confirmed in children. It is characterized by hyperglycaemia, suppression of the insulin response and increased catecholamine secretion during surgery. The pattern of hormonal and metabolic derangements is similar to that of the adult response, but changes are more marked and reverse more quickly in the postoperative period. The hormonal changes show considerable variation in the neonate. Premature neonates do not exhibit an insulin response to hyperglycaemia and this is also less predictable in the full-term neonate than in older children. The relationship of the severity of surgery to the degree of hyperglycaemia is less clear than in the adult, partly because of the wide variation in clinical conditions of measurement. A scoring system has been devised to assess the severity of surgery as minor, moderate or severe so that data can be interpreted in a more standardized manner. Observations during cardiac surgery with cardiopulmonary bypass in neonates confirm that severe surgery is associated with exaggerated and prolonged increases in plasma glucose and catecholamine concentrations and insulin suppression. The stress response to surgery can be obtunded by the addition of volatile anaesthetic agents or opiates to general anaesthetic techniques using oxygen, nitrous oxide and muscle relaxants. Local anaesthetic and regional blockade also modify the responses in conscious and unconscious patients. A possible relationship between pain perception and the stress response to surgery may have implications for changes in perioperative management. The possible benefits of inhibiting these stress responses during surgery in children need to be determined by further morbidity and mortality studies. Anaesthesia and surgery initiate metabolic and hormonal responses which result in a clearly defined clinical sequence of reactions in adults (Moore and Ball, 1952; Moore, 1953; Allison et al, 1969; Wright et al 1974; Lacoumenta et al, 1986; Simpson et al, 1987; Diltoer and Camu, 1988; see Chapter 2). Substrate mobilization produces an initial catabolic phase with oliguria, nitrogen loss, sodium retention and potassium excretion, to a degree which is proportional to the severity of the surgical procedure. During normal convalescence this is reversed within 1-2 days. This is followed by an anabolic phase of water diuresis, marked sodium excretion and decreased nitrogen and potassium losses, which restores positive (Anand et al, 1987 a, b). Similarly, Hansen and Hickey (1986) are finding that the mortality of neonates undergoing correction of hypoplastic left heart syndrome, using a high dose fentanyl and pancuronium technique, is improving. Further morbidity and mortality studies are needed to substantiate the merits of the abolition of the stress response to surgery in children. © 1989 Baillière Tindall. All rights reserved. AU - Bevan, J. C. DB - Scopus DO - 10.1016/S0950-3501(89)80005-4 IS - 2 M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 1989 SP - 349-364 ST - Metabolic response to surgery in children T2 - Bailliere's Clinical Anaesthesiology TI - Metabolic response to surgery in children UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0024853783&doi=10.1016%2fS0950-3501%2889%2980005-4&partnerID=40&md5=ea00150384f2eef07426ae6fe591513b VL - 3 ID - 2321 ER - TY - JOUR AB - Objective: A prenatal diagnosis of chromosome X short arm deletions may present a challenge in prenatal genetic counseling. We present clinical and molecular data of carriers of Xp distal deletions. Methods: We assessed prenatal and postnatal phenotypes of individuals from three families with large Xp distal deletions and from a fourth family with a small Xp distal deletion. The work-up included karyotyping, chromosomal microarray analysis, and assessment of the X inactivation pattern. Results: Five out of eight women with large deletions had a short stature (<3rd percentile). Subjects from one family had developmental and emotional problems. All female carriers with small deletions had markedly short stature, whereas the men had mildly short stature. Chromosomal microarray analysis revealed 11.7-19.3Mb deletions in three families and a small ~1Mb deletion in the fourth. The pseudoautosomal region 1 of the X chromosome was deleted in two families with large deletions. X inactivation was skewed in all tested cases with large deletions. Conclusion: Xp distal deletions are mainly associated with short stature. Skewing of the abnormal X chromosome may attenuate the phenotype in cases with large deletions. We suggest that prenatal evaluation in such cases should include sonographic follow-up and assessment of the X inactivation pattern. © 2014 John Wiley & Sons, Ltd. AD - R. Sukenik-Halevy, Genetic Institute and Prenatal Genetic Diagnosis Unit, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel AU - Sukenik-Halevy, R. AU - Reches, A. AU - Bar-Shira, A. AU - Simchoni, S. AU - Goldstein, M. AU - Orr-Ortreger, A. AU - Yaron, Y. AU - Ben-Shachar, S. DB - Embase Medline DO - 10.1002/pd.4354 IS - 6 KW - adult amniocentesis article atrioventricular canal child chromosome deletion chromosome Xp clinical article failure to thrive female fetus echography genetic counseling great vessels transposition haplotype heart single ventricle human in situ hybridization karyotyping microarray analysis nuchal translucency measurement phenotype pregnancy termination prenatal diagnosis priority journal psychomotor disorder short stature X chromosome inactivation LA - English M3 - Article N1 - L53075897 2014-04-04 2014-06-19 PY - 2014 SN - 1097-0223 0197-3851 SP - 592-597 ST - Microscopic chromosome Xp distal deletions - a challenging issue in prenatal genetic counseling T2 - Prenatal Diagnosis TI - Microscopic chromosome Xp distal deletions - a challenging issue in prenatal genetic counseling UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L53075897 http://dx.doi.org/10.1002/pd.4354 VL - 34 ID - 953 ER - TY - JOUR AB - The aim of this study was to determine the results and mid-term outcome of a modified Senning technique using autologous tissue for total cavopulmonary connection. The study involved 31 children, 8 with tricuspid atresia and 23 with complex congenital heart disease. In this operation, a flap of autologous atrial free wall tissue was used to tunnel inferior vena caval blood to the pulmonary arteries. An additional Damus-Kay-Stansel operation was required in 9 patients with subaortic obstruction. RESULTS: the early mortality rate was 16% (5 out of 31 patients) and there were four late deaths. COMPLICATIONS: Pleural effusions were encountered in 17 patients, of whom 4 had a concomitant pericardial effusion. Diaphragmatic paralysis was diagnosed in five patients, one of whom underwent surgical plication. Median hospital stay was 26 days. The 1- to 5-year actuarial survival was 68.6%. Follow-up ranged from 10 months to 7.1 years, mean 3.2 years. A serious atrial arrhythmia was diagnosed in one patient and another one died, possibly from rhythm disorders. Exercise tolerance and quality of life has improved in all but one of the survivors. Although follow-up is short, we have thus far witnessed a low incidence of hemodynamic and rhythm disturbances with this modification of the cavopulmonary connection. AD - Children's Heart Centre, Wilhelmina Children's Hospital, University of Utrecht, The Netherlands. AN - 7546805 AU - Benatar, A. AU - Tanke, R. AU - Roef, M. AU - Meyboom, E. J. AU - Van de Wal, H. J. DB - PubMed DO - 10.1016/s1010-7940(05)80190-3 DP - NLM ET - 1995/01/01 IS - 6 KW - Actuarial Analysis Adolescent Anastomosis, Surgical Arrhythmias, Cardiac/etiology Child Child, Preschool Female Follow-Up Studies Fontan Procedure/methods Heart Atria/surgery Heart Defects, Congenital/surgery Humans Infant Length of Stay Male Pericardial Effusion/etiology Pleural Effusion/etiology Postoperative Complications Pulmonary Artery/*surgery Respiratory Paralysis/etiology Surgical Flaps Survival Rate Tricuspid Valve/abnormalities/surgery Vena Cava, Inferior/*surgery LA - eng N1 - Benatar, A Tanke, R Roef, M Meyboom, E J Van de Wal, H J Journal Article Germany Eur J Cardiothorac Surg. 1995;9(6):320-4. doi: 10.1016/s1010-7940(05)80190-3. PY - 1995 SN - 1010-7940 (Print) 1010-7940 SP - 320-4 ST - Mid-term results of the modified Senning operation for cavopulmonary connection with autologous tissue T2 - Eur J Cardiothorac Surg TI - Mid-term results of the modified Senning operation for cavopulmonary connection with autologous tissue VL - 9 ID - 444 ER - TY - JOUR AB - Objective. - Neuropathologic evaluation was performed on an infant with fetal alcohol effects. Design. - Coronal brain sections and representative tissue blocks stained with hematoxylin-eosin, silver stain, and immunocytochemical stains for hypothalamic and pituitary hormones were evaluated for neuropathologic abnormalities. Patient. - A 2.5-month-old American Indian girl who had been exposed to first-trimester maternal binge alcohol abuse died after persistent problems of growth failure, sodium imbalance, aberrant temperature regulation, respiratory distress, and seizures. Results. - Autopsy revealed severe microcephaly, hypertelorism, midfacial hypoplasia, a high-arched palate, shortened palpebral fissures, and a small brain. The frontal lobes were fused anteriorly; olfactory bulbs and tracts were absent; and optic nerves were hypoplastic. An enlarged and bulbous hypothalamus obscured the pituitary gland. The thalamus and caudate nuclei were fused across the midline. Posteriorly, the single ventricle split to form rudimentary lateral horns. The anterior corpus callosum, septum pellucidum, fimbria, and fornices could not be identified. The anterior commissure and supraoptic nuclei were microscopically present. Many Purkinje cells were horizontally positioned, with abnormal dendritic structure. The posterior pituitary lobe was absent, and the infundibulum was flanked by a hypoplastic adenohypophysis and a large subarachnoid heterotopia. Immunocytochemical studies identified only vasopressin and neurophysin in the hypothalamus and only growth hormone and prolactin in the pituitary gland. Conclusion. - To our knowledge, an association between fetal alcohol effects and a complex cerebral anomaly with features of incomplete holoprosencephaly and septo-optic dysplasia has not previously been reported and suggests a possible common pathogenesis needing further study. AD - C.L. Coulter, Department of Neurology, Creighton University Medical Center, 601 N 30th St, Omaha, NE 68131, United States AU - Coulter, C. L. AU - Leech, R. W. AU - Schaefer, G. B. AU - Scheithauer, B. W. AU - Brumback, R. A. DB - Embase Medline IS - 7 KW - article brain malformation case report clinical feature endocrine disease female fetal alcohol syndrome high arched palate holoprosencephaly human hypertelorism hypothalamus hypophysis system infant microcephaly optic nerve disease priority journal syndrome LA - English M3 - Article N1 - L23203587 1993-07-28 PY - 1993 SN - 0003-9942 SP - 771-775 ST - Midline cerebral dysgenesis, dysfunction of the hypothalamic-pituitary axis, and fetal alcohol effects T2 - Archives of Neurology TI - Midline cerebral dysgenesis, dysfunction of the hypothalamic-pituitary axis, and fetal alcohol effects UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L23203587 VL - 50 ID - 1378 ER - TY - JOUR AB - Objective To determine whether prenatal diagnosis lowers the risk of preoperative brain injury by assessing differences in the incidence of preoperative brain injury across centers. Study design From 2 prospective cohorts of newborns with complex congenital heart disease studied by preoperative cerebral magnetic resonance imaging, one cohort from the University Medical Center Utrecht (UMCU) and a combined cohort from the University of California San Francisco (UCSF) and University of British Columbia (UBC), patients with aortic arch obstruction were selected and their imaging and clinical course reviewed. Results Birth characteristics were comparable between UMCU (n = 33) and UCSF/UBC (n = 54). Patients had a hypoplastic aortic arch with either coarctation/interruption or hypoplastic left heart syndrome. In subjects with prenatal diagnosis, there was a significant difference in the prevalence of white matter injury (WMI) between centers (11 of 22 [50%] at UMCU vs 4 of 30 [13%] at UCSF/UBC; P <.01). Prenatal diagnosis was protective for WMI at UCSF/UBC (13% prenatal diagnoses vs 50% postnatal diagnoses; P <.01), but not at UMCU (50% vs 46%, respectively; P >.99). Differences in clinical practice between prenatally diagnosed subjects at UMCU vs UCSF/UBC included older age at surgery, less time spent in the intensive care unit, greater use of diuretics, less use of total parenteral nutrition (P <.01), and a greater incidence of infections (P =.01). In patients diagnosed postnatally, the prevalence of WMI was similar in the 2 centers (46% at UMCU vs 50% at UCSF/UBC; P >.99). Stroke prevalence was similar in the 2 centers regardless of prenatal diagnosis (prenatal diagnosis: 4.5% at Utrecht vs 6.7% at UCSF/UBC, P =.75; postnatal diagnosis: 9.1% vs 13%, respectively, P >.99). Conclusion Prenatal diagnosis can be protective for WMI, but this protection may be dependent on specific clinical management practices that differ across centers. AD - L.S. De Vries, Department of Neonatology, University Medical Center Utrecht, Lundlaan 6, Utrecht, Netherlands AU - Algra, S. O. AU - Haas, F. AU - Poskitt, K. J. AU - Groenendaal, F. AU - Schouten, A. N. J. AU - Jansen, N. J. G. AU - Azakie, A. AU - Gandhi, S. AU - Campbell, A. AU - Miller, S. P. AU - McQuillen, P. S. AU - De Vries, L. S. DB - Embase Medline DO - 10.1016/j.jpeds.2014.08.066 IS - 6 KW - antibiotic agent diuretic agent inotropic agent prostaglandin E2 aortic arch anomaly aortic arch interruption aortic coarctation aortic arch obstruction Apgar score article artificial ventilation bloodstream infection brain injury cerebrovascular accident cesarean section clinical practice clinical study coagulase negative Staphylococcus congenital heart disease diffusion weighted imaging disease course echo planar imaging female human hypoplastic left heart syndrome incidence infant intensive care unit major clinical study male nuclear magnetic resonance imaging pneumonia prenatal diagnosis preoperative complication prevalence prospective study risk factor risk reduction Staphylococcus infection total parenteral nutrition white matter injury LA - English M3 - Article N1 - L600341395 2014-11-11 2014-12-15 PY - 2014 SN - 1097-6833 0022-3476 SP - 1116-1122.e3 ST - Minimizing the risk of preoperative brain injury in neonates with aortic arch obstruction T2 - Journal of Pediatrics TI - Minimizing the risk of preoperative brain injury in neonates with aortic arch obstruction UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L600341395 http://dx.doi.org/10.1016/j.jpeds.2014.08.066 VL - 165 ID - 963 ER - TY - JOUR AB - OBJECTIVE: In patients with congenital heart disease quality of life is only marginally associated with exercise capacity. The aim of this study was to determine the prevalence of depression and its impact on quality of life and exercise capacity. PATIENTS AND METHODS: From November 2007 to October 2009 a total of 767 patients (352 female, 14-67 years) with various congenital heart diseases (37 palliated/native cyanotic, 57 Fontan, 74 Transposition of the Great Arteries (TGA) after atrial switch, 50 other TGA, 136 Fallot, 38 Ebstein, 47 Pulmonic stenosis/regurgitation, 68 aortic coarctation, 103 aortic stenosis, 90 isolated shunts, 67 other) completed the health-related quality of life questionnaire SF-36 and the German translation of the "Center for Epidemiologic Studies Depression Scale" (CES-D) to assess depressive symptoms. Afterwards a cardiopulmonary exercise test was performed. RESULTS: Only 66 patients (8.6%) showed depressive symptoms fulfilling the CES-D definition for depression. The total prevalence of depression was lower than in the general population (Wilcoxon test, p<0.001) and did not differ substantially in between the diagnostic subgroups (Kruskal-Wallis test, p=0.195). CES-D score was correlated to all of the nine dimensions of quality of life (r=-0.170 to r=-0.740, p<0.001) and less pronounced to exercise capacity (r=-0.164, p<0.001). Correlation of peak oxygen uptake to quality of life was weaker than the CES-D scores in all subscales of life quality. CONCLUSIONS: Patients with congenital heart disease are rarely depressive. However, even minor depressive symptoms have a stronger impact on quality of life than limited exercise capacity as seen in many patients. AD - Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Lazarettstr. 36, D-80636 München, Germany. muellerjan@dhm.mhn.de AN - 20926144 AU - Müller, J. AU - Hess, J. AU - Hager, A. DA - Feb 9 DB - PubMed DO - 10.1016/j.ijcard.2010.09.029 DP - NLM ET - 2010/10/12 IS - 3 KW - Adolescent Adult Aged Depression/diagnosis/*epidemiology/*etiology *Exercise Tolerance Female Heart Diseases/*complications/*congenital Humans Male Middle Aged Prevalence Prospective Studies *Quality of Life Young Adult LA - eng N1 - 1874-1754 Müller, Jan Hess, John Hager, Alfred Journal Article Netherlands Int J Cardiol. 2012 Feb 9;154(3):265-9. doi: 10.1016/j.ijcard.2010.09.029. Epub 2010 Oct 5. PY - 2012 SN - 0167-5273 SP - 265-9 ST - Minor symptoms of depression in patients with congenital heart disease have a larger impact on quality of life than limited exercise capacity T2 - Int J Cardiol TI - Minor symptoms of depression in patients with congenital heart disease have a larger impact on quality of life than limited exercise capacity VL - 154 ID - 245 ER - TY - JOUR AB - Mitral atresia is a rare congenital heart defect when compared to atresia of other valves and it is often associated with other cardiac malformation such as patent ductus arteriosus, coarctation of the aorta, transposition of the great arteries, pulmonary stenosis, shunt defects, cor triatriatum, chamber, and valvular anomalies. There have been few reports of mitral atresia, therefore, the case of a 5-month-old male child with complaint of difficulty with breathing and echocardiographic diagnosis of atretic mitral valves, atrial septal defect of 15 mms, ventricular septal defect (VSD) of 10 mms and hypoplastic left ventricle is reported. AD - Department of Paediatrics, Aminu Kano Teaching Hospital, Bayero University, Kano, Nigeria AU - Aliyu, I. AU - Gambo, S. AU - Igoche, P. D. DB - Scopus DO - 10.4103/2211-4122.166078 IS - 3 KW - Atrial septal defect mitral atresia ventricular hypoplasia ventricular septal defect M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2015 SP - 77-79 ST - Mitral atresia with hypoplastic left ventricle and multiple shunt lesions T2 - Journal of Cardiovascular Echography TI - Mitral atresia with hypoplastic left ventricle and multiple shunt lesions UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84944069874&doi=10.4103%2f2211-4122.166078&partnerID=40&md5=007a2510138847d9e69fe109070ab390 VL - 25 ID - 1970 ER - TY - JOUR AB - BACKGROUND: Uncommonly, adults with functionally univentricular hearts are becoming candidates for a Fontan procedure. The purpose of this study was to evaluate the course of patients undergoing the modified Fontan procedure with an extracardiac conduit in recent years. METHODS: Between January 2003 and December 2013, 32 adult patients (17 female and 15 male) underwent total cavopulmonary connection (TCPC) with extracardiac conduit. The median age at procedure was 24.5 years (interquartile range [IQR] 20 to 33 years). The diagnoses included double-inlet left ventricle (DILV) in 10 patients (31.2%), tricuspid atresia in 8 patients (25%), double-outlet right ventricle in 4 patients (12.5%), heterotaxia in 4 patients (12.5%), and mitral atresia in 2 patients (6.2%). Seventy-eight percent of patients had undergone at least one prior palliative procedure; the most common procedures were Blalock-Taussig shunt (16 patients), superior cavopulmonary shunt (12 patients), and pulmonary artery banding (6 patients). All patients underwent cardiac catheterization preoperatively. Aortic cross-clamping was necessary in 15 patients for intracardiac procedures. Fenestration was required in 9 patients (28%). Four concomitant intraoperative cryoablation procedures were performed. RESULTS: There was no hospital mortality. One patient (3.1%) died 6 months after undergoing TCPC. Morbidities included prolonged pleural effusion lasting more than 7 days in 20 patients (62.5%), atrial arrhythmias in 4 patients (12.5%), and permanent pacemakers in 3 patients (9.3%). The median follow-up time was 33 months (interquartile range [IQR], 10.5 to 50 months). Actuarial survival was 91.83% (95% confidence limits, 71.07 to 97.89) at 1 year and 5 years. Ninety-two percent of patients were in New York Heart Association class I or II at follow-up. The median postoperative oxygen saturation was 95% (IQR, 93% to 95.5%). Cardiac arrhythmia occurred in 4 patients. Systolic ventricular function improved during follow-up for all patients except 1 patient, who underwent cardiac transplantation 7 months after the TCPC. CONCLUSIONS: The modified Fontan procedure with use of an extracardiac conduit can be performed in adults with encouraging early and midterm results. The majority of late survivors had improved quality of life. The incidence of late death, reoperation, arrhythmias, and thromboembolic events was low during follow-up. AD - Department of Congenital Heart Disease, Marie Lannelongue Hospital/M3C, Paris-Sud University, Le Plessis-Robinson, France. Electronic address: m.ly@ccml.fr. Department of Congenital Heart Disease, Marie Lannelongue Hospital/M3C, Paris-Sud University, Le Plessis-Robinson, France. Faculty of Medicine, Paris-Sud University, Orsay, France. AN - 25443023 AU - Ly, M. AU - Roubertie, F. AU - Kasdi, R. AU - Chatti, S. AU - Vergnat, M. AU - Luu, D. AU - Le Bret, E. AU - Roussin, R. AU - Capderou, A. AU - Belli, E. DA - Dec DB - PubMed DO - 10.1016/j.athoracsur.2014.07.043 DP - NLM ET - 2014/12/03 IS - 6 KW - Adult Cardiopulmonary Bypass/methods Female Follow-Up Studies Fontan Procedure/*methods Heart Bypass, Right/methods Heart Defects, Congenital/*surgery Humans Male Retrospective Studies Treatment Outcome Young Adult LA - eng N1 - 1552-6259 Ly, Mohamedou Roubertie, François Kasdi, Reda Chatti, Sana Vergnat, Mathieu Luu, David Le Bret, Emmanuel Roussin, Régine Capderou, André Belli, Emre Journal Article Netherlands Ann Thorac Surg. 2014 Dec;98(6):2181-6. doi: 10.1016/j.athoracsur.2014.07.043. Epub 2014 Oct 28. PY - 2014 SN - 0003-4975 SP - 2181-6 ST - The modified Fontan procedure with use of extracardiac conduit in adults: analysis of 32 consecutive patients T2 - Ann Thorac Surg TI - The modified Fontan procedure with use of extracardiac conduit in adults: analysis of 32 consecutive patients VL - 98 ID - 263 ER - TY - JOUR AB - OBJECTIVE: The modified Fontan procedure, usually performed in children, is used for the treatment of anomalies with a single functional ventricle. We reviewed our experience with the modified Fontan procedure performed in the adult patient. METHODS: Between October 1973 and May 2001, the modified Fontan procedure was performed on 132 adult patients (74 men, 58 women). Median age was 23 years (range, 18 to 53 years). Diagnoses included tricuspid atresia in 34 patients (26%), double-inlet left ventricle in 48 (36%), and complex lesions in 50 (38%). The majority of patients (89%) had at least one prior palliative procedure; the most common procedures were Blalock-Taussig shunt in 85 patients and Glenn anastomosis in 31. RESULTS: Operations included an atriopulmonary connection in 74 patients, lateral tunnel in 27, intra-atrial conduit in 14, right atrium-to-right ventricle in 9, extra-cardiac conduit in 3, and other in 5. Overall early mortality was 8.3%. Mortality was 6.5% for operations performed after 1980. This is comparable to the mortality of the modified Fontan procedure performed in children during the same time interval at our institution. All 7 of the early deaths since 1980 occurred in the complex lesion group. Morbidity included prolonged pleural effusion in 36 patients, atrial arrhythmias in 25, reoperation for bleeding in 13, permanent pacemaker in 8, and stroke in 2. Mean follow-up was 9.1 years with a maximum of 21.2 years. Actuarial survival for early survivors was 89% (84,95), 75% (67,84), and 68% (58,79) at 5, 10, and 15 years, respectively. Freedom from late reoperation was 89% (83,95), 85% (78,93), and 80% (70,91) at 5, 10 and 15 years, respectively. The majority (90%) of present survivors were New York Heart Association class I or II at follow-up. CONCLUSIONS: In properly selected adult patients with functional single ventricle, the modified Fontan procedure can be performed with early mortality similar to younger patients. Early mortality is more likely with complex lesions. The majority of late survivors have a good quality of life. AD - Division of Cardiovascular Surgery, Section of Pediatric Cardiology, Division of Cardiovascular Diseases, Mayo Clinic and Mayo Foundation, Rochester, MN 55905, USA. AN - 12830041 AU - Burkhart, H. M. AU - Dearani, J. A. AU - Mair, D. D. AU - Warnes, C. A. AU - Rowland, C. C. AU - Schaff, H. V. AU - Puga, F. J. AU - Danielson, G. K. DA - Jun DB - PubMed DO - 10.1016/s0022-5223(03)00117-x DP - NLM ET - 2003/06/28 IS - 6 KW - Adolescent Adult Double Outlet Right Ventricle/surgery Female Fontan Procedure/*methods/mortality Heart Defects, Congenital/*surgery Humans Male Middle Aged Treatment Outcome Tricuspid Atresia/surgery LA - eng N1 - Burkhart, Harold M Dearani, Joseph A Mair, Douglas D Warnes, Carole A Rowland, Charles C Schaff, Hartzell V Puga, Francisco J Danielson, Gordon K Journal Article United States J Thorac Cardiovasc Surg. 2003 Jun;125(6):1252-9. doi: 10.1016/s0022-5223(03)00117-x. PY - 2003 SN - 0022-5223 (Print) 0022-5223 SP - 1252-9 ST - The modified Fontan procedure: early and late results in 132 adult patients T2 - J Thorac Cardiovasc Surg TI - The modified Fontan procedure: early and late results in 132 adult patients VL - 125 ID - 16 ER - TY - JOUR AB - Objective: The modified Fontan procedure, usually performed in children, is used for the treatment of anomalies with a single functional ventricle. We reviewed our experience with the modified Fontan procedure performed in the adult patient. Methods: Between October 1973 and May 2001, the modified Fontan procedure was performed on 132 adult patients (74 men, 58 women). Median age was 23 years (range, 18 to 53 years). Diagnoses included tricuspid atresia in 34 patients (26%), double-inlet left ventricle in 48 (36%), and complex lesions in 50 (38%). The majority of patients (89%) had at least one prior palliative procedure; the most common procedures were Blalock-Taussig shunt in 85 patients and Glenn anastomosis in 31. Results: Operations included an atriopulmonary connection in 74 patients, lateral tunnel in 27, intra-atrial conduit in 14, right atrium-to-right ventricle in 9, extracardiac conduit in 3, and other in 5. Overall early mortality was 8.3%. Mortality was 6.5% for operations performed after 1980. This is comparable to the mortality of the modified Fontan procedure performed in children during the same time interval at our institution. All 7 of the early deaths since 1980 occurred in the complex lesion group. Morbidity included prolonged pleural effusion in 36 patients, atrial arrhythmias in 25, reoperation for bleeding in 13, permanent pacemaker in 8, and stroke in 2. Mean follow-up was 9.1 years with a maximum of 21.2 years. Actuarial survival for early survivors was 89% (84,95), 75% (67,84), and 68% (58,79) at 5, 10, and 15 years, respectively. Freedom from late reoperation was 89% (83,95), 85% (78,93), and 80% (70,91) at 5, 10 and 15 years, respectively. The majority (90%) of present survivors were New York Heart Association class I or II at follow-up. Conclusions: In properly selected adult patients with functional single ventricle, the modified Fontan procedure can be performed with early mortality similar to younger patients. Early mortality is more likely with complex lesions. The majority of late survivors have a good quality of life. AD - J.A. Dearani, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, United States AU - Burkhart, H. M. AU - Dearani, J. A. AU - Mair, D. D. AU - Warnes, C. A. AU - Rowland, C. C. AU - Schaff, H. V. AU - Puga, F. J. AU - Danielson, G. K. AU - Spray, T. AU - Drinkwater, D. DB - Embase Medline DO - 10.1016/S0022-5223(03)00117-X IS - 6 KW - adult article artificial heart pacemaker Blalock Taussig shunt bleeding cardiopulmonary bypass female follow up Fontan procedure Glenn shunt heart atrium arrhythmia heart single ventricle human major clinical study male mortality palliative therapy pleura effusion priority journal quality of life reoperation cerebrovascular accident surgical technique survival tricuspid valve atresia LA - English M3 - Article N1 - L36759222 2003-07-09 PY - 2003 SN - 0022-5223 SP - 1252-1259 ST - The modified Fontan procedure: Early and late results in 132 adult patients T2 - Journal of Thoracic and Cardiovascular Surgery TI - The modified Fontan procedure: Early and late results in 132 adult patients UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L36759222 http://dx.doi.org/10.1016/S0022-5223(03)00117-X VL - 125 ID - 1303 ER - TY - JOUR AB - Bartonella vinsonii subsp. berkhoffii, Bartonella henselae, or DNA of both organisms was amplified and sequenced from blood, enrichment blood cultures, or autopsy tissues from four family members. Historical and microbiological results support perinatal transmission of Bartonella species in this family. It is of clinical relevance that Bartonella spp. may adversely influence human reproductive performance. Copyright © 2010, American Society for Microbiology. All Rights Reserved. AD - E. B. Breitschwerdt, Intracellular Pathogens Research Laboratory, College of Veterinary Medicine, North Carolina State University, Raleigh, NC, United States AU - Breitschwerdt, E. B. AU - Maggi, R. G. AU - Farmer, P. AU - Mascarelli, P. E. DB - Embase Medline DO - 10.1128/JCM.00326-10 IS - 6 KW - azithromycin ciprofloxacin DNA doxycycline fluconazole nitazoxanide nystatin adult amnesia arthralgia article autopsy balance disorder Bartonella Bartonella berkhoffii Bartonella henselae Bartonella vinsonii bladder dysfunction Blastocystis hominis blastocystosis blood culture candidiasis case report cesarean section child chlamydial pneumonia cognitive defect confusion dyspnea ear infection fatigue female female infertility in vitro fertilization food allergy gene amplification gene sequence genital pain headache human human tissue hypoplastic left heart syndrome insomnia interstitial cystitis irritability irritable colon male myalgia Mycoplasma pneumonia night sweat nucleotide sequence perinatal infection priority journal pruritus restlessness school child sinusitis tachycardia verruca vulgaris vertical transmission LA - English M3 - Article N1 - L358912831 2010-06-10 2010-06-17 http://jcm.asm.org/cgi/reprint/48/6/2289 PY - 2010 SN - 0095-1137 SP - 2289-2293 ST - Molecular evidence of perinatal transmission of Bartonella vinsonii subsp. berkhoffii and Bartonella henselae to a child T2 - Journal of Clinical Microbiology TI - Molecular evidence of perinatal transmission of Bartonella vinsonii subsp. berkhoffii and Bartonella henselae to a child UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L358912831 http://dx.doi.org/10.1128/JCM.00326-10 VL - 48 ID - 1142 ER - TY - JOUR AB - OBJECTIVES: Near-infrared spectroscopy (NIRS) offers continuous non-invasive monitoring of regional tissue oxygenation. We evaluated NIRS monitoring during the postoperative course after superior cavopulmonary anastomosis in patients with hypoplastic left heart syndrome and anatomically related malformations. METHODS: Cerebral (cSO. 2) and somatic (sSO. 2) tissue oxygenations were recorded for 48 h and compared with routine measures of intensive care monitoring. Changes in parameters in the case of postoperative complications were evaluated. RESULTS: Data were obtained from 32 patients. Median age at operation was 2.9 (1.5-10.0) months and weight was 5.3 ± 1.0 kg. Postoperative complications occurred in 7 patients (pulmonary artery thrombus n = 4, pneumothorax n = 1, cardiopulmonary resuscitation n = 1 and low-cardiac output n = 1). cSO2 was 44 ± 14% at the end of the operation and reached its minimum of 40 ± 11% 2 h later (P = 0.018). Overall, cSO2 was depressed early after surgery and increased from a mean of 42 ± 11% during the first 4 postoperative hours to 57 ± 8% in the last 4 h of the study period (P < 0.001). The sSO2 decreased from 77 ± 11% during the early postoperative course to 68 ± 9% within the later course (P < 0.001). The cSO2 correlated with the arterial partial pressure of oxygen (pO2, r = 0.364, P < 0.001), with the arterial oxygen saturation (SaO2, r = 0.547, P < 0.001) and with the central venous oxygen saturation providing the strongest correlation (SvO2, r = 0.686, P < 0.001). Analysis of agreement between cSO2 and SvO2 measurements revealed a mean bias of 0.97 with limits of agreement between 19.8 and -17.9%. Inclusion of both cSO2 and sSO2 into a linear regression model slightly improved the prediction of SvO2 from NIRS values (r = 0.706, P < 0.001). The mean values of cSO2, sSO2, SaO2 and SvO2 during the early postoperative period were lower in patients with complications (cSO2: 45 ± 9 vs 29 ± 5%, P < 0.001; sSO2: 80 ± 11 vs 70 ± 6%, P = 0.004; SaO2: 76 ± 8 vs 66 ± 6%, P = 0.004; SvO2: 48 ± 14 vs 32 ± 6%, P < 0.001). CONCLUSIONS: NIRS technology allows inferring the global oxygenation from continuous non-invasive measurements of regional tissue oxygenation. The cSO. 2 is lowered in the early postoperative course. Lower cSO. 2 values in the early postoperative course may be predictive of postoperative complications. © The Author 2012. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. AD - J.H. Hansen, Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig Holstein, Campus Kiel,Arnold-Heller Strasse 3, Haus 9, 24105 Kiel, Germany AU - Hansen, J. H. AU - Schlangen, J. AU - Armbrust, S. AU - Jung, O. AU - Scheewe, J. AU - Kramer, H. H. DB - Embase Medline DO - 10.1093/ejcts/ezs581 IS - 2 KW - arterial oxygen saturation arterial oxygen tension artery thrombosis article cavopulmonary connection central venous pressure clinical article congenital heart malformation correlation coefficient heart hemodynamics heart output human hypoplastic left heart syndrome infant intensive care unit near infrared spectroscopy non invasive measurement oxygen saturation pneumothorax postoperative complication postoperative period priority journal pulmonary artery resuscitation tissue oxygenation LA - English M3 - Article N1 - L368170103 2013-01-30 2013-02-12 PY - 2013 SN - 1010-7940 1873-734X SP - e37-e43 ST - Monitoring of regional tissue oxygenation with near-infrared spectroscopy during the early postoperative course after superior cavopulmonary anastomosis T2 - European Journal of Cardio-thoracic Surgery TI - Monitoring of regional tissue oxygenation with near-infrared spectroscopy during the early postoperative course after superior cavopulmonary anastomosis UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L368170103 http://dx.doi.org/10.1093/ejcts/ezs581 VL - 43 ID - 1018 ER - TY - JOUR AB - This review from Guy's Hospital covers the largest group of patients yet treated in Great Britain, and should be read in the original by those interested in the subject. Broadly speaking findings corresponded closely with those reported by Blalock and Taussig. Ages ranged between 2 1/2 and 27 years. All patients were severely incapacitated and many had suddenly started to deteriorate when operation was undertaken. Cyanosis dated from early infancy, and in only five cases did it first become apparent after the age of 15 months; its severity corresponded roughly with the degree of polycythaemia. Squatting when exhausted was seen in 41 cases and panting was as frequent. Intelligence was average, appetite was poor, and the children were thin and pigeon-chested; they were of normal size. Cardiac enlargement was exceptional. The pulmonary second sound was more often normal, or slightly accentuated, than soft. Murmurs, if audible, were systolic and never harsh. The finding of a large heart, a drum-like pulmonary second sound, or a harsh murmur, suggests a mistaken diagnosis. X-ray studies confirmed that the heart was small and the 'coeur en sabot' configuration was observed in less than half the cases. Slight fullness of the pulmonary conus was relatively common, but vascular shadowing in the lungs was slight and hilar puisation absent. Angiocardiograms were useful for delincating the systemic vessels and pulmonary arteries for the benefit of the surgeon. The electrocardiograms showed large pointed P waves, especially in lead II, and right ventricular preponderance with absent Q1. Absence of preponderance was interpreted as being due to a single ventricle, and left preponderance as due to tricuspid atresia. In this hospital Blalock's operation is performed two or three times a week regularly. The posterolateral approach, on the left side, with complete resection of the fourth rib is preferred to Blalock's original method. The right side is used only when the pulmonary artery appears to be small or absent, so that clamping on the left might be fatal. Pott's operation has been used when the aortic branches have been small or high, since to use innominate or carotid arteries for Blalock's operation is considered unjustifiable in view of the dangers of cerebral damage. Postoperative pleural effusion rarely proved troublesome. Cerebral thrombosis occurred on three occasions; cardiac failure was not observed. Seven patients died after operation, three operations were abandoned without anastomosis, three patients did not improve, four were slightly improved, and in 33 results were 'perfect'. The heart size often increased during the first six weeks after operation, ability for physical exertion became almost normal, and the need for minor orthopaedic procedures was frequently disclosed. AD - Ch. Baker, Guy's Hosp., London AU - Baker, Ch AU - Brock, R. C. AU - Campbell, M. AU - Suzman, S. DB - Embase Classic Medline IS - 2 KW - BCG vaccine anastomosis appetite Blalock Taussig shunt brain damage cardiomegaly carotid artery child cyanosis diagnosis electrocardiogram exercise heart heart failure heart single ventricle heart size hospital infancy intelligence locus ceruleus lung occlusive cerebrovascular disease P wave patient Columbidae pleura effusion polycythemia pulmonary artery rib speech surgeon surgery tricuspid valve atresia United Kingdom X ray LA - English M3 - Article N1 - L280452762 1949-12-01 PY - 1949 SP - 170-198 ST - Morbus coeruleus. A study of 50 cases after the Blalock-Taussig operation T2 - British Heart Journal TI - Morbus coeruleus. A study of 50 cases after the Blalock-Taussig operation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L280452762 VL - 11 ID - 1428 ER - TY - JOUR AB - SETTING: Fontan-associated liver disease universally affects adults with single ventricle heart disease. Chronic kidney disease is also highly prevalent in adult Fontan patients. In this study, we evaluate the relationship of Fontan hemodynamics invasively and noninvasively with extra-cardiac dysfunction as measured by MELD and MELD-XI. OBJECTIVE: We hypothesize that invasive and noninvasive measures of Fontan circuit congestion and ventricular dysfunction are associated with increased MELD and MELD-XI scores. DESIGN: Single-center data from adults with Fontan palliation who had ongoing care, including cardiac catheterization, were retrospectively collected. Hemodynamic data from cardiac catheterization and echocardiographic assessment of ventricular and atrioventricular valve function were tested for association with serum creatinine, MELD, and MELD-XI. Linear regression was used to perform multivariable analysis in the echocardiogram cohort. RESULTS: Fifty-seven patients had congruent lab and catheterization data for analysis. Sixty-three and sixty-nine patients had congruent lab and echocardiogram data for MELD and MELD-XI, respectively. Of the hemodynamic variables analyzed, only decreased systemic oxygen saturation had significant correlation with elevated MELD and MELD-XI (P = .045). Patients with moderately or severely reduced ejection fraction by echocardiogram had significantly higher MELD and MELD-XI scores compared to those with normal or mildly depressed systolic ventricular function (P = .008 and P < .001 for MELD and MELD-XI, respectively). Significant differences in creatinine were also found among the ventricular dysfunction groups (P = .02). CONCLUSIONS: In adults following Fontan palliation, systolic ventricular dysfunction and decreased oxygen saturation were associated with hepatic and renal dysfunction as assessed by elevated serum creatinine, MELD, and MELD-XI scores. AD - Departments of Internal Medicine and Pediatrics, Vanderbilt University Medical Center, Nashville, Tennessee. Vanderbilt Heart and Vascular Institute, Vanderbilt University Medical Center, Nashville, Tennessee. Department of Biostatistics, Vanderbilt University Medical Center, Nashville, Tennessee. Department of Internal Medicine, Division of Gastroenterology, Hepatology and Nutrition, Vanderbilt University Medical Center, Nashville, Tennessee. Departments of Pediatrics and Internal Medicine, Division of Cardiology, Indiana University School of Medicine, Riley Hospital for Children, Indianapolis, Indiana. AN - 31282062 AU - Byrne, R. D. AU - Weingarten, A. J. AU - Clark, D. E. AU - Huang, S. AU - Perri, R. E. AU - Scanga, A. E. AU - Menachem, J. N. AU - Markham, L. W. AU - Frischhertz, B. P. DA - Sep DB - PubMed DO - 10.1111/chd.12820 DP - NLM ET - 2019/07/10 IS - 5 KW - Adolescent Adult Cardiac Catheterization Child Child, Preschool Echocardiography Female Fontan Procedure/*adverse effects Heart Defects, Congenital/diagnosis/*surgery Heart Ventricles/abnormalities/diagnostic imaging/*surgery Humans Incidence Kidney Diseases/epidemiology/*etiology Liver Diseases/epidemiology/*etiology Male *Postoperative Complications Retrospective Studies United States/epidemiology Ventricular Dysfunction, Left/*complications/diagnosis/physiopathology Ventricular Function, Left/physiology Young Adult Fontan Meld extra-cardiac liver renal LA - eng N1 - 1747-0803 Byrne, Ryan D Orcid: 0000-0003-1242-7020 Weingarten, Angela J Clark, Daniel E Huang, Shi Perri, Roman E Scanga, Andrew E Menachem, Jonathan N Markham, Larry W Frischhertz, Benjamin P Journal Article United States Congenit Heart Dis. 2019 Sep;14(5):765-771. doi: 10.1111/chd.12820. Epub 2019 Jul 7. PY - 2019 SN - 1747-079x SP - 765-771 ST - More than the heart: Hepatic, renal, and cardiac dysfunction in adult Fontan patients T2 - Congenit Heart Dis TI - More than the heart: Hepatic, renal, and cardiac dysfunction in adult Fontan patients VL - 14 ID - 23 ER - TY - JOUR AB - A total cavopulmonary connection (Fontan surgery) is rarely performed in a child with trisomy 21 (Down syndrome) for a univentricular heart, and the outcomes after surgery are not well defined, but the incidence of mortality has been reported to be higher. To determine the mortality rate and contributing factors after Fontan surgery in children with Down syndrome, mortality data after Fontan surgery from the Pediatric Cardiac Care Consortium Registry were evaluated. Among Fontan procedures (n = 2,853), all patients with Down syndrome (n = 17) were selected, of whom 13 had hemodynamic data available. Thirteen children without chromosomal aberrations were then selected as a control group, matched 1 to 1 for gender, age, weight, lesion, and type of Fontan procedure. The following variables were evaluated: pulmonary artery pressure and vascular resistance, weight, hemoglobin, degree of atrioventricular regurgitation, previous Glenn operation, fenestration, and length of stay in the hospital. In children with Down syndrome, mortality after the Glenn operation was 28%. Mortality after the Fontan operation was increased significantly (p = 0.001) in children with Down syndrome (35%) compared with those without Down syndrome (10%). Between patients with Down syndrome and controls, there were no significant differences in the perioperative parameters evaluated. Almost all mortality was in the early postoperative period in children with Down syndrome. The relative risk ratio of mortality was 2.5 (95% confidence interval 0.63 to 10). In conclusion, Down syndrome was found to be an independent parameter associated with a significantly higher risk for mortality in the early postoperative period after Fontan surgery. © 2010 Elsevier Inc. All rights reserved. AD - M. Gupta-Malhotra, Division of Pediatric Cardiology, Children's Memorial Hermann Hospital, University of Texas, Houston, TX, United States AU - Gupta-Malhotra, M. AU - Larson, V. E. V. AU - Rosengart, R. M. AU - Guo, H. AU - Moller, J. H. DB - Embase Medline DO - 10.1016/j.amjcard.2009.11.043 IS - 6 KW - hemoglobin article body weight cavopulmonary connection child chromosome aberration clinical article controlled study Down syndrome female Fontan procedure Glenn shunt hemodynamics human incidence length of stay lung artery pressure male perioperative period postoperative period preschool child priority journal risk factor school child surgical mortality tricuspid valve regurgitation vascular resistance LA - English M3 - Article N1 - L358381774 2010-03-10 2010-03-23 PY - 2010 SN - 0002-9149 SP - 865-868 ST - Mortality After Total Cavopulmonary Connection in Children With the Down Syndrome T2 - American Journal of Cardiology TI - Mortality After Total Cavopulmonary Connection in Children With the Down Syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L358381774 http://dx.doi.org/10.1016/j.amjcard.2009.11.043 VL - 105 ID - 1147 ER - TY - JOUR AB - OBJECTIVES: Neonates with hypoplastic left heart syndrome (HLHS) are at risk of high mortality and neurodevelopmental morbidity. As an alternative to Norwood-type stage I palliation, the hybrid procedure has been developed. It consists of bilateral pulmonary artery banding, catheter-based stenting of the arterial duct and balloon atrioseptostomy and delays open-heart surgery. Thus, it may be associated with a better outcome. The aim of this study was to determine the mortality and neurodevelopmental outcome in patients with HLHS and other univentricular heart (UVH) defects treated with hybrid or Norwood procedures. METHODS: Thirty-one children (18 males) with HLHS and other UVH defects undergoing Norwood or hybrid procedure between 2004 and 2008 were consecutively enrolled. Mortality and neurodevelopmental outcome at 1 year of age were determined. RESULTS: One-year mortality was 36% (31% in the hybrid vs. 39% in the Norwood group, P=0.71). Predictors of mortality were lower birth weight (P=0.02), older age at first procedure (P=0.02) and smaller size of ascending aorta (P=0.05). Overall, median psychomotor development index (PDI) and mental development index (MDI) of the Bayley Scales of Infant Development II were lower than the norm of 100 [PDI 57 (49-99), P<0.001; MDI 91 (65-109), P=0.002]. No effect of surgical treatment on neurodevelopmental outcome was found. Predictors of impaired motor outcome were length of hospital stay (LOHS) (P=0.01), lower body weight at second procedure (P=0.004) and female sex (P=0.01). Predictors of impaired cognitive outcome were longer mechanical ventilation time (P=0.03), intensive care unit stay (P=0.04) and LOHS (P<0.001), respectively. CONCLUSIONS: Mortality at 1 year of age is comparable between patients undergoing hybrid and Norwood procedures. Early neurodevelopmental outcome is significantly impaired in patients with both HLHS and other UVH defects. Multicentre randomized studies are needed to determine the long-term neurodevelopmental outcome of children treated with the hybrid procedure. AD - Division of Paediatric Cardiology, University Children's Hospital Zurich, Zurich, Switzerland. walter.knirsch@kispi.uzh.ch AN - 22290896 AU - Knirsch, W. AU - Liamlahi, R. AU - Hug, M. I. AU - Hoop, R. AU - von Rhein, M. AU - Prêtre, R. AU - Kretschmar, O. AU - Latal, B. DA - Jul DB - PubMed DO - 10.1093/ejcts/ezr286 DP - NLM ET - 2012/02/01 IS - 1 KW - Cardiac Surgical Procedures/*methods Cardiopulmonary Bypass Developmental Disabilities/*etiology Female Follow-Up Studies Heart Ventricles/abnormalities/surgery Humans Hypoplastic Left Heart Syndrome/complications/mortality/*surgery Infant Infant, Newborn Intellectual Disability/*etiology Male Neuropsychological Tests *Norwood Procedures Prospective Studies Psychomotor Disorders/*etiology Quality of Life Risk Factors Survival Rate Treatment Outcome LA - eng N1 - 1873-734x Knirsch, Walter Liamlahi, Rabia Hug, Maja I Hoop, Ricarda von Rhein, Michael Prêtre, René Kretschmar, Oliver Latal, Beatrice Clinical Trial Comparative Study Journal Article Research Support, Non-U.S. Gov't Germany Eur J Cardiothorac Surg. 2012 Jul;42(1):33-9. doi: 10.1093/ejcts/ezr286. Epub 2012 Jan 18. PY - 2012 SN - 1010-7940 SP - 33-9 ST - Mortality and neurodevelopmental outcome at 1 year of age comparing hybrid and Norwood procedures T2 - Eur J Cardiothorac Surg TI - Mortality and neurodevelopmental outcome at 1 year of age comparing hybrid and Norwood procedures VL - 42 ID - 221 ER - TY - JOUR AB - Background: Merging United Network for Organ Sharing (UNOS) and Pediatric Health Information Systems databases has enabled a more granular analysis of pediatric heart transplant outcomes and resource utilization. We evaluated whether transplant indication at time of transplantation was associated with mortality, resource utilization, and inpatient costs during the first year after transplantation.Methods and Results: We analyzed transplant outcomes and resource utilization from 2004 to 2015. Patients were categorized as congenital (CHD), myocarditis, or cardiomyopathy based on UNOS-defined primary indication. CHD complexity subgroup analyses (single-ventricle, complex, and simple biventricular CHD) were also performed. Of 2251 transplants (49% CHD, 5% myocarditis, 46% cardiomyopathy), CHD recipients were younger (2 [IQR 0-10], 6 [IQR 0-12], and 7 [IQR 1-14] years, respectively; P < .001) and less likely to have a ventricular assist device (VAD) at transplantation (3%, 27%, and 13%, respectively; P < .001). Patients with single-ventricle CHD had the longest time on the waitlist and were least likely to receive a VAD before transplantation. After adjusting for patient-level factors, transplant recipients with single-ventricle CHD had the greatest mortality during transplantation admission and within 1 year (odds ratio [OR] 11.8 [95% confidence interval (CI) 5.9-23.6] and OR 6.0 [95% CI 3.6-10.2], respectively, vs cardiomyopathy). Mortality was similar between patients with myocarditis and cardiomyopathy. Post-transplantation length of stay (LOS) was longer in transplant recipients with CHD than myocarditis or cardiomyopathy (25 [interquartile range [IQR] 15-45] vs 21 [IQR 12-35] vs 16 [IQR 12-25] days; P < .001), related in part to longer duration of intensive care unit-level care (ICU LOS 8 [IQR 4-20] vs 6 [IQR 4-13] vs 5 [IQR 3-8] days; P < .001). Similarly, patients with CHD had higher median post-transplantation costs than myocarditis or cardiomyopathy ($415K [IQR $201K-503K] vs $354K [IQR $179K-390K] vs $284K [IQR $145K-319K]; P < .001) that persisted after adjusting for patient-level factors (adjusted cost ratio 1.4 [95% CI 1.4-1.5], CHD vs cardiomyopathy) and was primarily driven by longer LOS. More than 50% were readmitted during the first year after transplantation, although readmission rates were similar across transplant indications (P = .42).Conclusions: Children with CHD, particularly single-ventricle patients, require substantially greater hospital resource utilization and have significantly worse outcomes during the first year after heart transplantation compared with other indications. Further work is aimed at identifying modifiable pre-transplantation risk factors, such as pre-transplantation conditioning with VAD support and cardiac rehabilitation, to improve post-transplantation outcomes and reduce resource utilization in this complex population. AD - Division of Pediatric Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania Division of Pediatric Oncology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania Healthcare Analytics Unit, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania AN - 134150630. Language: English. Entry Date: 20190318. Revision Date: 20190809. Publication Type: journal article AU - Burstein, Danielle S. AU - Li, Yimei AU - Getz, Kelly D. AU - Huang, Yuan-Shung V. AU - Rossano, Joseph W. AU - O'Connor, Matthew J. AU - Lin, Kimberly Y. AU - Aplenc, Richard DB - ccm DO - 10.1016/j.cardfail.2018.11.014 DP - EBSCOhost IS - 1 KW - Heart Transplantation -- Mortality Resource Databases -- Economics Resource Databases -- Trends Health Information Systems -- Economics Patient Attitudes Health Facility Costs -- Trends Health Information Systems -- Trends Heart Failure -- Mortality Child, Preschool Health Resource Utilization -- Economics Mortality -- Trends Heart Failure -- Economics Heart Transplantation -- Trends Hospitalization -- Economics Female Retrospective Design Heart Failure -- Therapy Heart Transplantation -- Economics Infant Adolescence Health Resource Utilization -- Trends Male Human Child Validation Studies Comparative Studies Evaluation Research Multicenter Studies Scales N1 - research. Journal Subset: Biomedical; Double Blind Peer Reviewed; Peer Reviewed; USA. Instrumentation: Work Environment Scale (WES) (Moos et al). NLM UID: 9442138. PMID: NLM30485789. PY - 2019 SN - 1071-9164 SP - 27-35 ST - Mortality, Resource Utilization, and Inpatient Costs Vary Among Pediatric Heart Transplant Indications: A Merged Data Set Analysis From the United Network for Organ Sharing and Pediatric Health Information Systems Databases T2 - Journal of Cardiac Failure TI - Mortality, Resource Utilization, and Inpatient Costs Vary Among Pediatric Heart Transplant Indications: A Merged Data Set Analysis From the United Network for Organ Sharing and Pediatric Health Information Systems Databases UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=134150630&site=ehost-live&scope=site VL - 25 ID - 1446 ER - TY - JOUR AB - To compare the motor development of patients with hypoplastic left heart syndrome (HLHS) and other types of univentricular heart (UVH) with peers who are healthy at the ages of 16 and 52 weeks.Motor development was assessed with the Alberta Infant Motor Scale (AIMS).Both the 23 patients with HLHS and the 13 patients with UVH had lower total AIMS scores in both observations than the controls. At the age of 52 weeks, patients with HLHS had significantly lower scores in all 4 AIMS subscales, whereas patients with UVH had lower scores only in the prone and standing subscales.Motor development of patients with HLHS or UVH is delayed during the first year of life, especially in the prone and standing subscales. AD - Children's Hospital (Ms Rajantie and Drs Lönnqvist, Sarajuuri, and Jokinen), University of Helsinki, Helsinki, Finland; Department of Neurology (Ms Laurila), Helsinki University Hospital, Helsinki, Finland; Office of the Ombudsman for Children (Ms Pollari), Jyväskylä, Finland; Faculty of Sport and Health Sciences (Dr Mälkiä), University of Jyväskylä, Jyväskylä, Finland. AN - 115803340. Language: English. Entry Date: 20180326. Revision Date: 20180326. Publication Type: Article AU - Rajantie, Irmeli AU - Laurila, Maarit AU - Pollari, Kirsi AU - Lönnqvist, Tuula AU - Sarajuuri, Anne AU - Jokinen, Eero AU - Mälkiä, Esko DA - Winter2013 DB - ccm DO - 10.1097/PEP.0b013e3182a31704 DP - EBSCOhost IS - 4 KW - Infant Development Motor Skills Motor Activity -- Physiology Therapeutic Exercise -- Methods Heart Ventricle -- Abnormalities Hypoplastic Left Heart Syndrome Human Comparative Studies Neuropsychological Tests Heart Defects, Congenital -- Rehabilitation Scales Female Male Infant N1 - research; tables/charts. Journal Subset: Allied Health; Peer Reviewed; USA. Instrumentation: Alberta Infant Motor Scale (AIMS). NLM UID: 8912748. PY - 2013 SN - 0898-5669 SP - 444-450 ST - Motor Development of Infants With Univentricular Heart at the Ages of 16 and 52 Weeks T2 - Pediatric Physical Therapy TI - Motor Development of Infants With Univentricular Heart at the Ages of 16 and 52 Weeks UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=115803340&site=ehost-live&scope=site VL - 25 ID - 1542 ER - TY - JOUR AB - Aim Patients with congenital heart disease are at risk of neurodevelopmental deficits. Impairments in motor and behavioural function occur frequently, but no information is available concerning the coexistence of deficits in these two developmental domains. This study explored the occurrence of motor and behavioural deficits and their coexistence in children with surgically corrected congenital heart disease. Methods Outcome was assessed in 95 children with congenital heart disease who had undergone cardiopulmonary bypass surgery. Their mean age was 9.6 years (SD 2.5). Motor function was assessed with the Zurich Neuromotor Assessment and behaviour with the Strength and Difficulties Questionnaire. Results Children with congenital heart disease performed poorer in all motor domains compared with the reference population (all p ≤ 0.001). Behaviour was affected in the domains 'emotional symptoms' and 'hyperactivity/inattention' (both p < 0.01), and 54% of the children with motor abnormalities showed behavioural deficits. Children with coexistent abnormalities in behaviour and motor function had higher rates of remedial school services and therapeutic support. Conclusion Children with congenital heart disease are at risk of long-term motor and behavioural problems, and there is a high rate of coexistence of problems in both domains. Early and longitudinal assessment of all developmental domains is necessary to provide adequate early support. ©2014 Foundation Acta Pædiatrica. Published by John Wiley & Sons Ltd. AD - B. Latal, Child Development Center, University Children's Hospital Zurich, Children Research Center Zurich, Steinwiesstrasse 75, 8032 Zurich, Switzerland AU - Liamlahi, R. AU - Von Rhein, M. AU - Bührer, S. AU - Valsangiacomo Buechel, E. R. AU - Knirsch, W. AU - Landolt, M. A. AU - Latal, B. DB - Embase Medline DO - 10.1111/apa.12639 IS - 7 KW - aortic coarctation aortic valve stenosis aortopulmonary septal defect article atrioventricular septal defect attention disturbance behavior disorder cardiopulmonary bypass child congenital heart disease controlled study cor triatriatum coronary artery fistula emotional disorder Fallot tetralogy female functional assessment great vessels transposition heart atrium septum defect heart left ventricle double inlet heart single ventricle heart ventricle septum defect human hyperactivity inferior cava vein major clinical study male motor dysfunction motor performance outcome assessment priority journal pulmonary valve atresia pulmonary valve stenosis pulmonary vein malformation questionnaire risk factor school child school health service Strength and Difficulties Questionnaire tricuspid valve atresia Zurich Neuromotor Assessment LA - English M3 - Article N1 - L53114668 2014-05-02 2014-06-25 PY - 2014 SN - 1651-2227 0803-5253 SP - 752-758 ST - Motor dysfunction and behavioural problems frequently coexist with congenital heart disease in school-age children T2 - Acta Paediatrica, International Journal of Paediatrics TI - Motor dysfunction and behavioural problems frequently coexist with congenital heart disease in school-age children UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L53114668 http://dx.doi.org/10.1111/apa.12639 VL - 103 ID - 957 ER - TY - JOUR AB - Magnetic resonance imaging can be used for prepro-cedural assessment of complex anatomy for radiofre-quency (RF) ablations, e.g., in a univentricular heart. This case report features the treatment of a young patient with a functionally univentricular heart who suf-fered from persistent sudden onset tachycardia with wide complexes that required RF ablation as treatment. © 2012 Baishideng. AD - T. Reiter, Department of In-ternal Medicine I, University Hospital Wuerzburg, 97080 Wuerz-burg, Germany AU - Reiter, T. AU - Ritter, O. AU - Nordbeck, P. AU - Beer, M. AU - Bauer, W. R. C1 - aggrenox medikinet DB - Embase DO - 10.4330/wjc.v4.i8.260 IS - 8 KW - acetylsalicylic acid plus dipyridamole adenosine ajmaline gadolinium isoprenaline methylphenidate adolescent angina pectoris article attention deficit disorder case report dizziness electrocardiography follow up Fontan procedure heart ejection fraction cardiac index heart single ventricle heart ventricle septum defect human magnetic resonance angiography male nuclear magnetic resonance imaging radiofrequency ablation tachycardia tricuspid valve atresia aggrenox medikinet 3T Trio LA - English M3 - Article N1 - L365756458 2012-10-09 2012-10-15 http://www.wjgnet.com/1949-8462/pdf/v4/i8/260.pdf PY - 2012 SN - 1949-8462 SP - 260-263 ST - MRI-guided ablation of wide complex tachycardia in a univentricular heart T2 - World Journal of Cardiology TI - MRI-guided ablation of wide complex tachycardia in a univentricular heart UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L365756458 http://dx.doi.org/10.4330/wjc.v4.i8.260 VL - 4 ID - 1049 ER - TY - JOUR AB - Ventricular volumes and ejection fraction are often used in clinical decision making in patients with congenital heart disease (CHD). The referral diagnosis, radiation exposure and image quality of functional cardiac computed tomography (CT) in a relatively large cohort of patients of CHD has not been reported. This is a retrospective evaluation of functional CT studies performed in CHD patients from three institutions (1/2007–3/2013). Patient and scanner characteristics, radiation dose estimates and image quality were compared. Two hundred ninety-eight functional CT studies were evaluated. The most common referral diagnosis were tetralogy of Fallot (33 %), transposition complexes (24 %) single ventricle heart disease (15 %), and left sided obstruction (15 %). The reason for cardiac CT was presence of pacemaker (60 %), need for detailed coronary artery imaging (18 %), metallic artifact in CMR (12 %), evaluation of prosthetic valve function (4 %), and claustrophobia or BMI too large for the available MR scanner (6 %). 266 (89.3 %) scans allowed quantification of ventricular function, 25 (8.4 %) scans allowed qualitative assessment of function, and 7 (2.3 %) of the scans were non-diagnostic for functional analysis. Median DLP was 399 mGy cm (186, 614), and median effective dose was 5.5 mSv (2.6, 8.5). Radiation dose and image quality varied across institutions. Cardiac CT function imaging can be performed in patients with congenital heart disease when CMR is contraindicated or has poor image quality. Radiation dose and image quality varies across institutions. AD - B.K. Han, c/o The Children’s Heart Clinic, Children’s Hospitals and Clinics of Minnesota, 2530 Chicago Ave South, Suite 500, Minneapolis, MN, United States AU - Han, B. K. AU - Hlavacek, A. M. AU - Kay, W. A. AU - Pham, T. D. N. AU - Grant, K. AU - Garberich, R. F. AU - Lesser, J. R. AU - Raman, S. V. DB - Embase Medline DO - 10.1007/s10554-015-0775-y IS - 2 KW - adolescent adult age distribution article body height body mass body weight cardiac imaging cardiovascular computer assisted tomography cardiovascular magnetic resonance child claustrophobia comparative study computed tomography scanner computer assisted tomography congenital heart disease controlled study dosimetry Fallot tetralogy female heart left ventricle function heart left ventricle outflow tract obstruction heart valve prosthesis heart ventricle failure human image processing image quality major clinical study male cardiac rhythm management device patient referral radiation dose retrospective study risk assessment sex difference supraventricular tachycardia Somatom Definition Somatom Definition Flash Somatom Sensation 64 LA - English M3 - Article N1 - L606508158 2015-10-26 2016-02-22 PY - 2016 SN - 1573-0743 1569-5794 SP - 339-346 ST - Multi-institutional evaluation of the indications and radiation dose of functional cardiovascular computed tomography (CCT) imaging in congenital heart disease T2 - International Journal of Cardiovascular Imaging TI - Multi-institutional evaluation of the indications and radiation dose of functional cardiovascular computed tomography (CCT) imaging in congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L606508158 http://dx.doi.org/10.1007/s10554-015-0775-y VL - 32 ID - 824 ER - TY - JOUR AB - Objectives: Assess the effect of a simulation "boot camp" on the ability of pediatric nurse practitioners to identify and treat a low cardiac output state in postoperative patients with congenital heart disease. Additionally, assess the pediatric nurse practitioners' confidence and satisfaction with simulation training. Design: Prospective pre/post interventional pilot study. Setting: University simulation center. Subjects: Thirty acute care pediatric nurse practitioners from 13 academic medical centers in North America. Interventions: We conducted an expert opinion survey to guide curriculum development. The curriculum included didactic sessions, case studies, and high-fidelity simulation, based on high-complexity cases, congenital heart disease benchmark procedures, and a mix of lesion-specific postoperative complications. To cover multiple, high-complexity cases, we implemented Rapid Cycle Deliberate Practice method of teaching for selected simulation scenarios using an expert driven checklist. Measurements and Main Results: Knowledge was assessed with a pre-/posttest format (maximum score, 100%). A paired-sample t test showed a statistically significant increase in the posttest scores (mean [sd], pre test, 36.8% [14.3%] vs post test, 56.0% [15.8%]; p < 0.001). Time to recognize and treat an acute deterioration was evaluated through the use of selected high-fidelity simulation. Median time improved overall "time to task" across these scenarios. There was a significant increase in the proportion of clinically time-sensitive tasks completed within 5 minutes (pre, 60% [30/50] vs post, 86% [43/50]; p = 0.003] Confidence and satisfaction were evaluated with a validated tool ("Student Satisfaction and Self-Confidence in Learning"). Using a five-point Likert scale, the participants reported a high level of satisfaction (4.7 ± 0.30) and performance confidence (4.8 ± 0.31) with the simulation experience. Conclusions: Although simulation boot camps have been used effectively for training physicians and educating critical care providers, this was a novel approach to educating pediatric nurse practitioners from multiple academic centers. The course improved overall knowledge, and the pediatric nurse practitioners reported satisfaction and confidence in the simulation experience. AD - K.M. Brown, Department of Acute and Chronic Care, Johns Hopkins University School of Nursing, 525 N. Wolfe Street, Baltimore, MD, United States AU - Brown, K. M. AU - Mudd, S. S. AU - Hunt, E. A. AU - Perretta, J. S. AU - Shilkofski, N. A. AU - Diddle, J. W. AU - Yurasek, G. AU - Bembea, M. AU - Duval-Arnould, J. AU - McMillan, K. N. DB - Embase Medline DO - 10.1097/PCC.0000000000001532 IS - 6 KW - advanced practice nurse article atrioventricular canal clinical article clinical assessment congenital heart disease curriculum curriculum development e-mail emergency care forward heart failure heart injury high fidelity simulation training human hypertensive crisis hypoplastic left heart syndrome Likert scale North America patient satisfaction pediatric cardiology pediatric nurse practitioner postoperative care postoperative complication priority journal pulmonary hypertension registered nurse sedation task performance university hospital work experience LA - English M3 - Article N1 - L624066136 2018-10-02 2018-10-08 PY - 2018 SN - 1947-3893 1529-7535 SP - 564-571 ST - A Multi-Institutional Simulation Boot Camp for Pediatric Cardiac Critical Care Nurse Practitioners T2 - Pediatric Critical Care Medicine TI - A Multi-Institutional Simulation Boot Camp for Pediatric Cardiac Critical Care Nurse Practitioners UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L624066136 http://dx.doi.org/10.1097/PCC.0000000000001532 VL - 19 ID - 684 ER - TY - JOUR AB - This session of the Congenital Post-Graduate Course 89th Annual Meeting of the American Association for Thoracic Surgery focused on clinical research in pediatric cardiac surgery. This particular lecture concentrated on the management of clinical trials from the viewpoint of a multi-institutional trial, utilizing my experiences as the Study Co-Chair of the National Heart Lung Blood Institute (NHLBI)-funded, Pediatric Heart Network Single Ventricle Reconstruction (SVR) Trial. Preparing for the role as a Primary Investigator through coursework and reading is an important prerequisite. Study design requires thoughtful crafting of the primary question, secondary question, and subanalyses. Often the challenge is not what to include, but what to exclude, to develop a practical database. Getting your co-investigators invested in the trial is crucial, as subject enrollment is time-consuming and will determine the success or failure of your trial. © 2010 Elsevier Inc. AD - R.G. Ohye, 5144 CVC, 1500 E. Medical Center Drive/SPC 5864, Ann Arbor, MI 48109-5864, United States AU - Ohye, R. G. DB - Embase Medline DO - 10.1053/j.pcsu.2010.01.008 IS - 1 KW - article childhood disease clinical protocol clinical research clinical study clinical trial data base health care management health center heart single ventricle heart ventricle remodeling human investment pediatric surgery personal experience process design professional competence reading study design LA - English M3 - Article N1 - L358490502 2010-03-29 2010-04-13 PY - 2010 SN - 1092-9126 SP - 76-78 ST - Multi-institutional studies: Lessons learned from the pediatric heart network single ventricle reconstruction trial T2 - Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual TI - Multi-institutional studies: Lessons learned from the pediatric heart network single ventricle reconstruction trial UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L358490502 http://dx.doi.org/10.1053/j.pcsu.2010.01.008 VL - 13 ID - 1146 ER - TY - JOUR AB - Objectives: The purpose of this study was to compare the safety and efficacy of acetylsalicylic acid (ASA) and warfarin for thromboprophylaxis after the Fontan procedure. Background: Fontan surgery is the definitive palliation for children with single-ventricle physiology. Thrombosis is an important complication; the optimal thromboprophylaxis strategy has not been determined. Methods: We performed a multicenter international randomized trial of primary prophylactic anticoagulation after Fontan surgery. Patients were randomized to receive for 2 years either ASA (5 mg/kg/day, no heparin phase) or warfarin (started within 24 h of heparin lead-in; target international normalized ratio: 2.0 to 3.0). Primary endpoint (intention to treat) was thrombosis, intracardiac or embolic (all events adjudicated). At 3 months and 2 years after the Fontan procedure, transthoracic and transesophageal echocardiograms were obtained as routine surveillance. Major bleeding and death were primary adverse outcomes. Results: A total of 111 eligible patients were randomized (57 to ASA, 54 to heparin/warfarin). Baseline characteristics for each group were similar. There were 2 deaths unrelated to thrombosis or bleeding. There were 13 thromboses in the heparin/warfarin group (3 clinical, 10 routine echo) and 12 thromboses in the ASA group (4 clinical, 8 routine echo). Overall freedom from thrombosis 2 years after Fontan surgery was 19%, despite thrombosis prophylaxis. Cumulative risk of thrombosis was persistent but varying and similar for both groups (p = 0.45). Major bleeding occurred in 1 patient in each group. Conclusions: There was no significant difference between ASA and heparin/warfarin as primary thromboprophylaxis in the first 2 years after Fontan surgery. The thrombosis rate was suboptimal for both regimens, suggesting alternative approaches should be considered. (International Multi Centre Randomized Clinical Trial Of Anticoagulation In Children Following Fontan Procedures; NCT00182104) © 2011 American College of Cardiology Foundation. AD - B.W. McCrindle, Hospital for Sick Children, 555 University Avenue, Toronto, ON M5G1X8, Canada AU - Monagle, P. AU - Cochrane, A. AU - Roberts, R. AU - Manlhiot, C. AU - Weintraub, R. AU - Szechtman, B. AU - Hughes, M. AU - Andrew, M. AU - McCrindle, B. W. DB - Embase Medline DO - 10.1016/j.jacc.2011.01.061 IS - 6 KW - NCT00182104 acetylsalicylic acid heparin warfarin article bleeding child controlled study female Fontan procedure gastrointestinal hemorrhage human loading drug dose major clinical study male mortality multicenter study patient compliance preschool child priority journal randomized controlled trial risk assessment thrombosis transesophageal echocardiography treatment outcome LA - English M3 - Article N1 - L362236233 2011-08-05 2011-08-09 PY - 2011 SN - 0735-1097 1558-3597 SP - 645-651 ST - A multicenter, randomized trial comparing heparin/warfarin and acetylsalicylic acid as primary thromboprophylaxis for 2 years after the Fontan procedure in children T2 - Journal of the American College of Cardiology TI - A multicenter, randomized trial comparing heparin/warfarin and acetylsalicylic acid as primary thromboprophylaxis for 2 years after the Fontan procedure in children UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L362236233 http://dx.doi.org/10.1016/j.jacc.2011.01.061 VL - 58 ID - 1096 ER - TY - JOUR AB - Parents of children with congenitally malformed hearts can suffer from stress as a result of the medical condition of their child. In this cross-sectional study, we aimed to describe levels of parental stress, and perceived vulnerability, in parents of children who underwent major cardiac surgery, by using both generic and disease-related measures for assessment. We included parents of children who underwent open-heart surgery over the period 2002 through 2007 in the Center for congenital Anomalies Heart Amsterdam/Leiden, abbreviated to provide the acronym CAHAL. In total, we assessed 114 mothers and 82 fathers of 131 children, using the Pediatric Inventory for Parents, short form, General Health Questionnaire, Parental Stress Index-Short Form, State-Trait Anxiety Index and the Child Vulnerability Scale. Compared to the reference groups of the instruments used, parents of children with congenitally malformed hearts did not report higher generic nor disease-related stress scores, and parenting levels of stress were also comparable to reference groups. State anxiety levels, however, were higher in mothers of children with congenitally malformed hearts. Both fathers and mothers reported significantly higher rates of perceived vulnerability than did parents of healthy children. Risk factors for increased anxiety and perceived vulnerability were found in the number of surgical procedures, the time past since the last procedure, and ethnicity. Severity of the lesion did not influence parental levels of stress, but parents of children with hypoplastic left heart syndrome did report higher levels of stress than other parents. Psychosocial screening of parents of children with congenitally malformed hearts is important in order to provide appropriate counselling to those parents most in need. AD - Pediatric Department, Leiden University Medical Center, Leiden, The Netherlands. c.m.j.vrijmoetwiersma@lumc.nl AN - 19825253 AU - Vrijmoet-Wiersma, C. M. AU - Ottenkamp, J. AU - van Roozendaal, M. AU - Grootenhuis, M. A. AU - Koopman, H. M. DA - Dec DB - PubMed DO - 10.1017/s1047951109991831 DP - NLM ET - 2009/10/15 IS - 6 KW - Adolescent Adult Analysis of Variance Child Child, Preschool Cross-Sectional Studies Disease Susceptibility Female Heart Defects, Congenital/*psychology/surgery Humans Infant Male Middle Aged Parents/*psychology Risk Factors Stress, Psychological/*etiology Surveys and Questionnaires LA - eng N1 - 1467-1107 Vrijmoet-Wiersma, C M Jantien Ottenkamp, Jaap van Roozendaal, Matty Grootenhuis, Martha A Koopman, Hendrik M Journal Article Multicenter Study England Cardiol Young. 2009 Dec;19(6):608-14. doi: 10.1017/S1047951109991831. Epub 2009 Oct 14. PY - 2009 SN - 1047-9511 SP - 608-14 ST - A multicentric study of disease-related stress, and perceived vulnerability, in parents of children with congenital cardiac disease T2 - Cardiol Young TI - A multicentric study of disease-related stress, and perceived vulnerability, in parents of children with congenital cardiac disease VL - 19 ID - 187 ER - TY - JOUR AB - Parents of children with congenitally malformed hearts can suffer from stress as a result of the medical condition of their child. In this cross-sectional study, we aimed to describe levels of parental stress, and perceived vulnerability, in parents of children who underwent major cardiac surgery, by using both generic and disease-related measures for assessment. We included parents of children who underwent open-heart surgery over the period 2002 through 2007 in the Center for congenital Anomalies Heart Amsterdam/Leiden, abbreviated to provide the acronym CAHAL. In total, we assessed 114 mothers and 82 fathers of 131 children, using the Pediatric Inventory for Parents, short form, General Health Questionnaire, Parental Stress Index-Short Form, State-Trait Anxiety Index and the Child Vulnerability Scale. Compared to the reference groups of the instruments used, parents of children with congenitally malformed hearts did not report higher generic nor disease-related stress scores, and parenting levels of stress were also comparable to reference groups. State anxiety levels, however, were higher in mothers of children with congenitally malformed hearts. Both fathers and mothers reported significantly higher rates of perceived vulnerability than did parents of healthy children. Risk factors for increased anxiety and perceived vulnerability were found in the number of surgical procedures, the time past since the last procedure, and ethnicity. Severity of the lesion did not influence parental levels of stress, but parents of children with hypoplastic left heart syndrome did report higher levels of stress than other parents. Psychosocial screening of parents of children with congenitally malformed hearts is important in order to provide appropriate counselling to those parents most in need. © 2009 Cambridge University Press. AD - C. M. J. Vrijmoet-Wiersma, Pediatric Department, Leiden University Medical Center, P.O. Box 9600, 2300 RC Leiden, Netherlands AU - Vrijmoet-Wiersma, C. M. J. AU - Ottenkamp, J. AU - Van Roozendaal, M. AU - Grootenhuis, M. A. AU - Koopman, H. M. DB - Embase Medline DO - 10.1017/S1047951109991831 IS - 6 KW - adult anxiety article child child parent relation congenital heart malformation controlled study disease severity ethnicity female General Health Questionnaire human hypoplastic left heart syndrome infant major clinical study male open heart surgery parental stress preschool child risk factor school child scoring system State Trait Anxiety Inventory vulnerable population LA - English M3 - Article N1 - L50668076 2009-10-20 2010-03-15 PY - 2009 SN - 1047-9511 1467-1107 SP - 608-614 ST - A multicentric study of disease-related stress, and perceived vulnerability, in parents of children with congenital cardiac disease T2 - Cardiology in the Young TI - A multicentric study of disease-related stress, and perceived vulnerability, in parents of children with congenital cardiac disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50668076 http://dx.doi.org/10.1017/S1047951109991831 VL - 19 ID - 1175 ER - TY - JOUR AB - Aim: Multidisciplinary foetal centres have recently emerged in partnership with children's hospitals throughout the United States. The aim of this investigation was to describe the patient experience of pregnant women who were referred to our foetal centres for the evaluation of foetal congenital anomalies. Methods: A qualitative interview study of women referred to our foetal centres was conducted using semi-structured telephone interviews. The data were coded and analysed using thematic networks analysis. Results: Six themes were identified (i) foetal centre evaluations had a far-reaching impact on participants’ lives, (ii) participants appreciated both expertise and support from providers, (iii) participants recognised the need to cope with uncertainty, (iv) participants sought additional support from multiple arenas, (v) participants specifically looked to the Internet for both information and support and (vi) participants had pre-established views about pregnancy termination. Conclusion: Multidisciplinary foetal centres can provide a unified source of clinical expertise to women who are carrying a foetus with a suspected or identified congenital anomaly. Despite this diagnostic acumen, uncertainty is pervasive and patients seek support and additional information from a variety of other sources. This investigation is an important initial step in the evaluation of this emerging model of care. ©2017 Foundation Acta Pædiatrica. Published by John Wiley & Sons Ltd AD - Mary Bridge Children's Hospital, Tacoma, WA, United States Seattle Children's Hospital, Seattle, WA, United States University of Washington, Seattle, WA, United States AU - Kett, J. C. AU - Wolfe, E. AU - Vernon, M. M. AU - Woodrum, D. AU - Diekema, D. DB - Scopus DO - 10.1111/apa.13812 IS - 6 KW - Congenital anomalies Foetal care centre Foetal centre Patient experience Prenatal counselling M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2017 SP - 930-934 ST - The multidisciplinary fetal center: clinical expertise is only part of the experience T2 - Acta Paediatrica, International Journal of Paediatrics TI - The multidisciplinary fetal center: clinical expertise is only part of the experience UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85018548500&doi=10.1111%2fapa.13812&partnerID=40&md5=2be3c30a00029746a32b8a321ff8655c VL - 106 ID - 1860 ER - TY - JOUR AB - OBJECTIVE: First, to compare QOL and illness perceptions between patients with a Fontan circulation and patients with anatomically simple defects (ie, atrial septal defects [ASD] or ventricular septal defects [VSD]). Second, to explore illness perceptions as a mediator of the association between congenital heart disease (CHD) diagnosis and QOL. DESIGN: Cross-sectional observational study. SETTING: Twenty-four cardiology centers from 15 countries across five continents. PATIENTS: Four hundred thirty-five adult patients with congenital heart disease (177 Fontan and 258 ASD/VSD) ages 18-83 years. OUTCOME MEASURES: QOL and illness perceptions were assessed by the Satisfaction With Life Scale and the Brief Illness Perceptions Questionnaire, respectively. RESULTS: Patients with a Fontan circulation reported lower QOL (Wald Z = -3.59, p = <.001) and more negative perceptions of their CHD (Wald Z = -7.66, p < .001) compared with patients with ASD/VSD. After controlling for demographics, anxiety, depressive symptoms, and New York Heart Association functional class, path analyses revealed a significant mediation model, αβ = 0.15, p = .002, 95% CI = 0.06-0.25, such that CHD diagnosis was indirectly related to QOL through illness perceptions. CONCLUSIONS: The Fontan sample's more negative perceptions of CHD were likely a reflection of life with a more complex defect. Illness perceptions appear to account for unique differences in QOL between groups of varying CHD complexity. Psychosocial screening and interventions may be important treatment components for patients with CHD, particularly those with Fontan circulations. AD - Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA. University of Louisville, Louisville, Kentucky, USA. KU Leuven, Leuven, Belgium. University of Gothenburg, Gothenburg, Sweden. Amsterdam Medical Center, Amsterdam, The Netherlands. Chiba Cardiovascular Center, Chiba, Japan. Umeå University, Umeå, Sweden. National Taiwan University, Taipei, Taiwan. Adult Congenital Heart Unit, Sahlgrenska University Hospital, Gothenburg, Sweden. Frontier Lifeline Hospital, Dr. K. M. Cherian Heart Foundation, Chennai, India. Nationwide Children's Hospital, Columbus, Ohio, USA. University Hospitals Leuven and Department of Cardiovascular Sciences, Leuven, Belgium. Oregon Health & Science University, Portland, Oregon, USA. Hospital Louis Pradel, Lyon, France. Oslo University Hospital, Oslo, Norway. University Hospital Bern, University of Bern, Bern, Switzerland. University West, Trollhättan, Sweden. Hospital de Niños, Córdoba, Argentina. IRCCS Policlinico San Donato Hospital, Milan, Italy. Stanford University, Palo Alto, California, USA. Mater Dei Hospital, Msida, Malta. Monash Medical Center, Melbourne, Australia. Helen DeVos Children's Hospital, Grand Rapids, Michigan, USA. University of Alberta, Edmonton, Canada. Washington University and Barnes Jewish Heart & Vascular Center, University of Missouri, Saint Louis, Missouri, USA. Montreal Heart Institute, Montreal, Canada. Children's Hospital & Medical Center, Omaha, Nebraska, USA. AN - 29457362 AU - Holbein, C. E. AU - Fogleman, N. D. AU - Hommel, K. AU - Apers, S. AU - Rassart, J. AU - Moons, P. AU - Luyckx, K. AU - Sluman, M. A. AU - Enomoto, J. AU - Johansson, B. AU - Yang, H. L. AU - Dellborg, M. AU - Subramanyan, R. AU - Jackson, J. L. AU - Budts, W. AU - Kovacs, A. H. AU - Morrison, S. AU - Tomlin, M. AU - Gosney, K. AU - Soufi, A. AU - Eriksen, K. AU - Thomet, C. AU - Berghammer, M. AU - Alday, L. AU - Callus, E. AU - Fernandes, S. M. AU - Caruana, M. AU - Menahem, S. AU - Cook, S. C. AU - Rempel, G. R. AU - White, K. AU - Khairy, P. AU - Kutty, S. AU - Veldtman, G. C2 - PMC5993574 C6 - NIHMS933370 DA - May DB - PubMed DO - 10.1111/chd.12583 DP - NLM ET - 2018/02/20 IS - 3 KW - Adolescent Adult Aged Aged, 80 and over *Attitude to Health Child Cross-Sectional Studies Female Global Health Heart Defects, Congenital/epidemiology/*psychology Humans Male Middle Aged Morbidity/trends *Perception Quality of Life/*psychology Young Adult Fontan circulation congenital heart disease illness perceptions quality of life LA - eng N1 - 1747-0803 Holbein, Christina E Orcid: 0000-0001-9516-0952 Fogleman, Nicholas D Hommel, Kevin Apers, Silke Rassart, Jessica Moons, Philip Luyckx, Koen Sluman, Maayke A Enomoto, Junko Johansson, Bengt Yang, Hsiao-Ling Dellborg, Mikael Subramanyan, Raghavan Jackson, Jamie L Budts, Werner Kovacs, Adrienne H Morrison, Stacey Tomlin, Martha Gosney, Kathy Soufi, Alexandra Eriksen, Katrine Thomet, Corina Berghammer, Malin Alday, Luis Callus, Edward Fernandes, Susan M Caruana, Maryanne Menahem, Samuel Cook, Stephen C Rempel, Gwen R White, Kamila Khairy, Paul Kutty, Shelby Veldtman, Gruschen APPROACH-IS consortium and the International Society for Adult Congenital Heart Disease (ISACHD) T32 HD068223/HD/NICHD NIH HHS/United States Comparative Study Journal Article Multicenter Study Observational Study Congenit Heart Dis. 2018 May;13(3):392-400. doi: 10.1111/chd.12583. Epub 2018 Jan 3. PY - 2018 SN - 1747-079X (Print) 1747-079x SP - 392-400 ST - A multinational observational investigation of illness perceptions and quality of life among patients with a Fontan circulation T2 - Congenit Heart Dis TI - A multinational observational investigation of illness perceptions and quality of life among patients with a Fontan circulation VL - 13 ID - 59 ER - TY - JOUR AB - Background. Myocardial contractile dysfunction has been frequently observed in adolescents or adults with cyanotic congenital heart disease. Impaired energy metabolism may be present in such dysfunctional myocardium. Methods and Results. To evaluate the findings of myocardial free fatty acid metabolism, and its relations to ventricular wall motion and myocardial perfusion in cyanotic congenital heart disease, we performed a combined study of iodine 123-labeled 15-(p-iodophenyl)3- (R,S)-methylpentadecanoic acid (BMIPP) scintigraphy, thallium scintigraphy, and contrast cineangiography in seven patients with single right or left ventricle. The results showed that wall motion was reduced in 17 of 35 ventricular segments (49%), which were mostly identical in location to decreased BMIPP uptake. The severity of BMIPP uptake deficit correlated positively with the degree of impairment of wall motion. On the other hand, thallium uptake was abnormal only in 5 of 35 segments (14%), and the severity of the perfusion defect did not correlate with the degree of wall motion abnormality. Conclusions. Contractile dysfunction in cyanotic heart disease was primarily linked to impaired free fatty acid metabolism rather than to myocardial scar as represented by perfusion defect on thallium imaging. AD - C. Kondo, Department of Pediatric Cardiology, Heart Institute of Japan, Tokyo Women's Medical College, 8-1 Kawada-cho, Shinjuku-ku, Tokyo, Japan AU - Kondo, C. AU - Nakazawa, M. AU - Kusakabe, K. AU - Momma, K. DB - Embase Medline DO - 10.1016/S1071-3581(96)90021-5 IS - 1 KW - decanoic acid derivative iodine 123 thallium adolescent adult article child clinical article cyanotic heart disease fatty acid metabolism female heart cineventriculography heart contraction heart muscle metabolism heart perfusion heart scintiscanning heart single ventricle human human tissue male priority journal LA - English M3 - Article N1 - L26069594 1996-03-12 PY - 1996 SN - 1071-3581 SP - 30-36 ST - Myocardial dysfunction and depressed fatty acid metabolism in patients with cyanotic congenital heart disease T2 - Journal of Nuclear Cardiology TI - Myocardial dysfunction and depressed fatty acid metabolism in patients with cyanotic congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L26069594 http://dx.doi.org/10.1016/S1071-3581(96)90021-5 VL - 3 ID - 1362 ER - TY - JOUR AB - The outcome of children born with cyanotic congenital heart disease has markedly improved over the years. Follow up is recommended for most post-operated cases as complications may occur over long term. One of the complications is the development of ventricular dysfunction, often seen after a successful Fontan surgery (or one of its modifications) for single ventricle. The aim of this study was to determine the prevalence of myocardial perfusion abnormalities in the ventricular myocardium of asymptomatic patients, older than 8 years of age, who had earlier undergone either a univentricular palliation (modified Fontan procedure) or a biventricular repair for tetralogy of Fallot, more than a year ago. All eligible patients underwent screening electrocardiogram (to rule out rhythm disturbance) and echocardiography. Patients with ventricular ejection fraction of more than 50 % by echocardiography were included. Enrolled patients were subjected to gated stress-rest myocardial perfusion imaging using Technitium-99m tetrofosmin single photon emission-computerized tomography (SPECT). Ventricular ejection fraction was also calculated from gated rest study. For the Fontan group, we also analyzed data to see if the morphology of the systemic ventricle would make a difference as far as myocardial perfusion was concerned. Twenty-six patients were enrolled (11 had undergone Fontan surgery and 15 had complete repair of tetralogy of Fallot). Seven of 11 patients in the Fontan group had myocardial perfusion defects (63.6 %) as against none in the repaired tetralogy of Fallot group (p < 0.001). The ejection fraction was within normal range in both the groups; it was statistically higher in the post tetralogy of Fallot repair group (p < 0.04). There were two subgroups in the post Fontan group depending on the morphology of systemic ventricle-left (4 patients) and non-left (7 patients). Higher number and larger size of perfusion defects were present in the non-left ventricular systemic ventricle morphology as compared with left ventricular morphology, but this difference did not reach statistical significance. Myocardial perfusion defects are common in patients who have undergone univentricular repair more than one year ago in contrast to patients who had a biventricular repair for tetralogy of Fallot. In the Fontan group, the morphology of the systemic ventricle was not predictive of prevalence of perfusion defect. AD - Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, 110029, India. AN - 23064840 AU - Priyadarshini, A. AU - Saxena, A. AU - Patel, C. AU - Paul, V. K. AU - Lodha, R. AU - Airan, B. DA - Apr DB - PubMed DO - 10.1007/s00246-012-0531-1 DP - NLM ET - 2012/10/16 IS - 4 KW - Adolescent Chi-Square Distribution Child *Coronary Circulation Cross-Sectional Studies Echocardiography Electrocardiography Exercise Test Female *Fontan Procedure Humans Image Interpretation, Computer-Assisted Male Myocardial Perfusion Imaging/*methods Organophosphorus Compounds Organotechnetium Compounds Oximetry Postoperative Complications/*diagnostic imaging/epidemiology Prevalence Prospective Studies Radiopharmaceuticals Statistics, Nonparametric Tetralogy of Fallot/*physiopathology/*surgery Time Factors Tomography, Emission-Computed, Single-Photon Young Adult LA - eng N1 - 1432-1971 Priyadarshini, A Saxena, Anita Patel, Chetan Paul, Vinod K Lodha, Rakesh Airan, Balram Journal Article United States Pediatr Cardiol. 2013 Apr;34(4):786-94. doi: 10.1007/s00246-012-0531-1. Epub 2012 Oct 12. PY - 2013 SN - 0172-0643 SP - 786-94 ST - Myocardial perfusion abnormalities in patients occurring more than 1 year after successful univentricular (Fontan surgery) and biventricular repair (complete repair of tetralogy of Fallot) T2 - Pediatr Cardiol TI - Myocardial perfusion abnormalities in patients occurring more than 1 year after successful univentricular (Fontan surgery) and biventricular repair (complete repair of tetralogy of Fallot) VL - 34 ID - 299 ER - TY - JOUR AB - Ventricular dysfunction in patients after Fontan-like operations (FLOs) is a serious complication that might contribute to poor long-term results. Ischemic heart disease will have debilitating consequences on a Fontan heart. Ten patients (15.8 +/- 5.01 years) after FLO had transesophageal echocardiography and cardiac catheterization 9.3 +/- 4.2 years after surgery. Myocardial perfusion was assessed by NH3-positron emission tomography (rest/adenosine) and compared with that of 10 healthy adults (26.1 +/- 6.3 years). Ventricular function was normal in 4 and reduced in 6 patients; end systolic and end diastolic meridional wall stress was significantly elevated in the FLO group. Coronary angiography revealed no stenosis of the coronaries. Compared to normals, myocardial blood flow (MBF) at rest was higher in the FLO group (0.99 +/- 0.25 vs 0.77 +/- 0.17 ml/g/min, p <0.05), whereas MBF after vasodilatation (2.12 +/- 0.78 vs 3.10 +/- 0.85 ml/g/min, p <0.05) and coronary flow reserve (CFR) was reduced (2.5 +/- 0.88 vs 4.1 +/- 1.01, p <0.05), especially in those with impaired ventricular function. Coronary vascular resistance after vasodilatation was elevated in the FLO group (38.2 +/- 17.4 vs 24.5 +/- 8.3 mmHg/ml/g/min, p <0.05). Altered MBF, increased meridional wall stress, and impaired CFR are common findings in FLO. Attenuated CFR and reduced ventricular function are significantly correlated and may be risk factors for the long-term outcome. AD - Deutsches Herzzentrum München, Pediatric Cardiology, Lazarettstrasse 36, 80636 Munich, Germany. AN - 12545320 AU - Hauser, M. AU - Bengel, F. M. AU - Kühn, A. AU - Sauer, U. AU - Nekolla, S. G. AU - Eicken, A. AU - Schwaiger, M. AU - Hess, J. DA - Jul-Aug DB - PubMed DO - 10.1007/s00246-002-0355-5 DP - NLM ET - 2003/01/25 IS - 4 KW - Adolescent Blood Flow Velocity Cardiac Catheterization Case-Control Studies Child Cohort Studies Coronary Angiography Coronary Circulation/physiology Echocardiography, Transesophageal Electrocardiography Female Follow-Up Studies Fontan Procedure/adverse effects/*methods Heart Function Tests Hemodynamics/physiology Humans Male Myocardial Ischemia/*diagnostic imaging/etiology Probability Reference Values Risk Assessment Sensitivity and Specificity Severity of Illness Index Statistics, Nonparametric *Tomography, Emission-Computed Tricuspid Atresia/surgery Ventricular Dysfunction, Left/*diagnostic imaging/physiopathology LA - eng N1 - Hauser, M Bengel, F M Kühn, A Sauer, U Nekolla, S G Eicken, A Schwaiger, M Hess, J Comparative Study Journal Article United States Pediatr Cardiol. 2003 Jul-Aug;24(4):386-92. doi: 10.1007/s00246-002-0355-5. Epub 2003 Jan 28. PY - 2003 SN - 0172-0643 (Print) 0172-0643 SP - 386-92 ST - Myocardial perfusion and coronary flow reserve assessed by positron emission tomography in patients after Fontan-like operations T2 - Pediatr Cardiol TI - Myocardial perfusion and coronary flow reserve assessed by positron emission tomography in patients after Fontan-like operations VL - 24 ID - 474 ER - TY - JOUR AB - Objective To analyse the prevalence of fetal and infant deaths due to birth defects in Korea and those trends according to maternal age. Design Retrospective national cohort study Setting Korean Vital Statistics database of the Korean Statistical Information Service, between 2009 and 2015. Participants 2176 infant deaths and 4343 fetal deaths caused by birth defects, among 3 181 145 total live births and 43 385 fetal deaths during the study periods. Methods Infant and fetal mortality rates (IMRs and FMRs) by birth defects, from deaths caused by birth defects, were analysed. They were compared, according to maternal age groups: (I) ' 10-19 years'; (II) ' 20-29 years'; (III) ' 30-34 years'; (IV) ' 35-39 years'; and (V) ' 40-55 years'. Main outcome measures IMRs and FMRs by birth defects and comparison according to maternal age group. Results IMRs and FMRs by birth defects were 6.84 per 10 000 live births and 13.47 per 10 000 total births. The most common causes of infant deaths and fetal deaths by birth defect were anomaly of the circulatory system (51.1%, IMR 3.5) and chromosomal abnormality (33.1%, FMR 4.46), respectively. Among groups by maternal age, FMRs by birth defects were significantly higher in groups I and V compared with group III (OR 6.59, 95% CI 3.49 to 12.43; and OR 3.46, 95% CI 1.77 to 6.78, respectively). IMR and FMR by nervous system anomaly were significantly higher in group I at 3.63 (OR 2.0, 95% CI 1.97 to 2.03) and 29.84 (OR 15.04, 95% CI 3.59 to 62.96) compared with 0.32 and 1.97 in group III. Conclusion FMRs by birth defects were the highest in the extreme maternal age groups. Severe anomalies, except for chromosomal abnormality, were most prevalent in teenage pregnancies. AD - J.C. Shin, Department of Obstetrics and Gynecology, College of Medicine, Catholic University of Korea, Seoul, South Korea AU - Ko, H. S. AU - Kim, D. J. AU - Chung, Y. AU - Wie, J. H. AU - Choi, S. K. AU - Park, I. Y. AU - Park, Y. G. AU - Shin, J. C. DB - Embase Medline DO - 10.1136/bmjopen-2017-017963 IS - 11 KW - adolescent adult article cardiovascular malformation cat cry syndrome child child death chromosome aberration cohort analysis comparative study congenital diaphragm hernia congenital heart malformation congenital malformation controlled study Down syndrome Edwards syndrome Fallot tetralogy female fetus fetus death fetus mortality gestational age human hypoplastic left heart syndrome infant infant mortality kleinfelter syndrome Korea live birth major clinical study maternal age middle aged musculoskeletal system malformation nervous system malformation retrospective study sex chromosome aberration triploidy trisomy 13 trisomy 18 Turner syndrome wolff hirschorn syndrome young adult LA - English M3 - Article N1 - L623794998 2018-09-18 2018-09-20 PY - 2017 SN - 2044-6055 ST - A national cohort study evaluating infant and fetal mortality caused by birth defects in Korea T2 - BMJ Open TI - A national cohort study evaluating infant and fetal mortality caused by birth defects in Korea UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L623794998 http://dx.doi.org/10.1136/bmjopen-2017-017963 VL - 7 ID - 730 ER - TY - JOUR AB - BACKGROUND: In the United States, birth defects affect approximately 3% of all births, are a leading cause of infant mortality, and contribute substantially to childhood morbidity. METHODS: Population-based data from the National Birth Defects Prevention Network were combined to estimate the prevalence of 21 selected defects for 1999-2001, stratified by surveillance system type. National prevalence was estimated for each defect by pooling data from 11 states with active case-finding, and adjusting for the racial/ethnic distribution of US live births. We also assessed racial/ethnic variation of the selected birth defects. RESULTS: National birth defect prevalence estimates ranged from 0.82 per 10,000 live births for truncus arteriosus to 13.65 per 10,000 live births for Down syndrome. Compared with infants of non-Hispanic (NH) white mothers, infants of NH black mothers had a significantly higher birth prevalence of tetralogy of Fallot, lower limb reduction defects, and trisomy 18, and a significantly lower birth prevalence of cleft palate, cleft lip with or without cleft palate, esophageal atresia/tracheoesophageal fistula, gastroschisis, and Down syndrome. Infants of Hispanic mothers, compared with infants of NH white mothers, had a significantly higher birth prevalence of anencephalus, spina bifida, encephalocele, gastroschisis, and Down syndrome, and a significantly lower birth prevalence of tetralogy of Fallot, hypoplastic left heart syndrome, cleft palate without cleft lip, and esophageal atresia/tracheoesophageal fistula. CONCLUSIONS: This study can be used to evaluate individual state surveillance data, and to help plan for public health care and educational needs. It also provides valuable data on racial/ethnic patterns of selected major birth defects. © 2006 Wiley-Liss, Inc. AD - M.A. Canfield, Birth Defects Epidemiology and Surveillance Branch, Texas Department of State Health Services, 1100 W. 49th Street, Austin, TX 78756, United States AU - Canfield, M. A. AU - Honein, M. A. AU - Yuskiv, N. AU - Xing, J. AU - Mai, C. T. AU - Collins, J. S. AU - Devine, O. AU - Petrini, J. AU - Ramadhani, T. A. AU - Hobbs, C. A. AU - Kirby, R. S. DB - Embase Medline DO - 10.1002/bdra.20294 IS - 11 KW - anencephalus anophthalmia anus atresia article congenital malformation cause of death cleft lip cleft lip palate cleft palate congenital heart disease diaphragm hernia Down syndrome encephalocele endocardial cushion defect esophagus atresia ethnic difference Fallot tetralogy gastroschisis great vessels transposition health care planning health education Hispanic human hypoplastic left heart syndrome infant mortality intestine atresia intestine stenosis limb reduction defect microphthalmia morbidity omphalocele prevalence priority journal race difference spinal dysraphism tracheoesophageal fistula trisomy 13 trisomy 18 United States LA - English M3 - Article N1 - L44777830 2006-12-05 PY - 2006 SN - 1542-0752 1542-0760 SP - 747-756 ST - National estimates and race/ethnic-specific variation of selected birth defects in the United States, 1999-2001 T2 - Birth Defects Research Part A - Clinical and Molecular Teratology TI - National estimates and race/ethnic-specific variation of selected birth defects in the United States, 1999-2001 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L44777830 http://dx.doi.org/10.1002/bdra.20294 VL - 76 ID - 1250 ER - TY - JOUR AB - OBJECTIVE: To define the evolution of the single-ventricle (SV) heart in adult patients in terms of morbidity, mortality and quality of life. METHODS: Sixty-two patients with SV physiology and aged older than 16 years were retrospectively reviewed. Three patients (5%) were in natural history, one had received a Blalock-Taussig shunt, one a Waterstone anastomosis, one a pulmonary artery banding, three a bidirectional cavopulmonary anastomosis, eight a classic Fontan procedure and 46 a total cavopulmonary connection (TCPC). The morphology of the SV was left in 48 patients (77%), right in nine (14%) and indeterminable in five (8%). Thirty-three patients underwent magnetic resonance imaging (MRI) to assess ventricular mass (VM), ventricular systolic function, pulmonary artery branch diameter and potential thrombosis of the conduit. Cardiopulmonary exercise testing (CPTE) was carried out to evaluate exercise tolerance. The quality of life was monitored with two different specific tests, the Short Form-36 (SF-36) and the congenital heart disease-TNO/AZL adult quality of life (CHD-TAAQOL). The mean follow-up time was 8.0 ± 9.1 years. RESULTS: Two of the three patients in natural history underwent primary TCPC. Re-interventions were necessary in seven patients (11%). Three patients (5%) died during follow-up. Five patients (8%) underwent cardiac transplantation. Protein losing enteropathy appeared in six (10%), while the arrhythmic disorder was detected in 13 patients. On the MRI, the mean end-diastolic ventricular volume was 106 ± 448 ml/m(2), the mean ejection fraction (EF) was 52.3 ± 10% and VM was 56 ± 22.1 g/m(2). On CPTE, the peak of oxygen uptake (peak VO(2)) was moderately impaired in 92% of patients, while 4% presented a severely impaired and 4% a normal peak of VO(2). No correlations were found among the peak of VO(2) and the quality-of-life evaluation. CONCLUSIONS: Adult patients with SV are at high risk of reoperations and need of transplant and complications. Nevertheless, in the presence of a moderately reduced peak of VO(2) and a moderate reduction in the EF detected at the MRI, the results of the evaluation of daily quality of life are incredibly high. AD - Pediatric and Grown-up Congenital Cardiac Surgery, University of Bologna, S.Orsola-Malpighi Hospital, Bologna, Italy. emanuela.angeli3@unibo.it AN - 22538457 AU - Angeli, E. AU - Pace Napoleone, C. AU - Balducci, A. AU - Formigari, R. AU - Lovato, L. AU - Candini, L. AU - Oppido, G. AU - Gargiulo, G. DA - Dec DB - PubMed DO - 10.1093/ejcts/ezs202 DP - NLM ET - 2012/04/28 IS - 6 KW - Adolescent Adult *Blalock-Taussig Procedure/mortality Exercise Test Female Follow-Up Studies *Fontan Procedure/mortality *Heart Transplantation Heart Ventricles/*abnormalities/diagnostic imaging/surgery Humans Kaplan-Meier Estimate Magnetic Resonance Imaging Male Middle Aged Postoperative Complications/epidemiology Quality of Life Reoperation/statistics & numerical data Retrospective Studies Surveys and Questionnaires Tomography, X-Ray Computed *Ventricular Function Young Adult LA - eng N1 - 1873-734x Angeli, Emanuela Pace Napoleone, Carlo Balducci, Anna Formigari, Roberto Lovato, Luigi Candini, Lorena Oppido, Guido Gargiulo, Gaetano Evaluation Study Journal Article Germany Eur J Cardiothorac Surg. 2012 Dec;42(6):996-1002. doi: 10.1093/ejcts/ezs202. Epub 2012 Apr 26. PY - 2012 SN - 1010-7940 SP - 996-1002 ST - Natural and modified history of single-ventricle physiology in adult patients T2 - Eur J Cardiothorac Surg TI - Natural and modified history of single-ventricle physiology in adult patients VL - 42 ID - 171 ER - TY - JOUR AB - Background. Before the era of the Fontan procedure, the typical course of patients with single left ventricle (LV) consisted of heart failure and death during the second or third decade of life. Despite the advent of effective palliative therapy, ventricular dysfunction remains a significant clinical problem for these patients. Methods and Results. To investigate the causes of ventricular dysfunction in these patients and to determine whether Fontan- type repair reverses deterioration of LV function, the ventricular dimensions, volume, shape, wall stress, and systolic function were determined by echocardiography in 84 patients 0.2-35 years old with double-inlet single LV or tricuspid atresia. Measurements were obtained in 67 patients after palliation (arterial shunt or pulmonary artery band) and in 47 patients a median of 4.4 years after a Glenn (n=9) or a Fontan operation (n=38). Before a Fontan procedure, ventricular volumes were 2 to 3 times normal. Ventricular afterload, assessed as circumferential and meridional end-systolic wall stress, became abnormal after 2 years of age. With age, LV shape changed progressively from ellipsoidal to spherical, as indicated by the decrease in long axis:short axis ratio from normal (1.9) toward unity. Concomitantly, the ratio of circumferential to meridional end-systolic wall stress fell from 1.3 to unity, the ratio of a sphere at equilibrium. This age-related change in shape and load occurred in concert with progressive deterioration of LV systolic function and contractility. Aortic oxygen saturation, an indicator of pulmonary blood flow and therefore volume work in single-ventricle physiology, was inversely and independently correlated with contractility. In the group of patients in whom a Glenn or a Fontan operation was performed at <10 years of age, ventricular dimensions, volumes, and wall stress diminished and LV function and contractility improved after surgery (p<0.001). In patients undergoing surgery after 10 years of age, few had improvement of LV function after surgery. Postoperative ventricular function and contractility were inversely related to age at surgery and to aortic oxygen saturation measured before surgery. Conclusions. Although Fontan-type repair of single ventricle early in life is associated with reversal of the abnormal contractile mechanics associated with age and volume load, this capacity for recovery diminishes with age at surgery. AD - S.D. Colan, Department of Cardiology, Children's Hospital, 300 Longwood Avenue, Boston, MA 02115, United States AU - Sluysmans, T. AU - Sanders, S. P. AU - Van der Velde, M. AU - Matitiau, A. AU - Parness, I. A. AU - Spevak, P. J. AU - Mayer Jr, J. E. AU - Colan, S. D. DB - Embase Medline IS - 6 KW - adolescent adult arterial oxygen saturation article child echocardiography Fontan procedure heart left ventricle contractility heart left ventricle function heart left ventricle volume heart single ventricle hemodynamic parameters human infant major clinical study onset age palliative therapy priority journal prognosis tricuspid valve atresia LA - English M3 - Article N1 - L22361963 1992-12-18 PY - 1992 SN - 0009-7322 SP - 1753-1761 ST - Natural history and patterns of recovery of contractile function in single left ventricle after Fontan operation T2 - Circulation TI - Natural history and patterns of recovery of contractile function in single left ventricle after Fontan operation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L22361963 VL - 86 ID - 1381 ER - TY - JOUR AB - Objectives: Fetal aortic valvuloplasty (FV) aims to prevent fetal aortic valve stenosis progressing into hypoplastic left heart syndrome (HLHS), which results in postnatal univentricular (UV) circulation. Despite increasing numbers of FVs performed worldwide, the natural history of the disease in fetal life remains poorly defined. The primary aim of this study was to describe the natural history of fetal aortic stenosis, and a secondary aim was to test previously published criteria designed to identify cases of emerging HLHS with the potential for a biventricular (BV) outcome after FV.Methods: From a European multicenter retrospective study of 214 fetuses with aortic stenosis (2005-2012), 107 fetuses in ongoing pregnancies that did not undergo FV were included in this study and their natural history was reported. We examined longitudinal changes in Z-scores of aortic and mitral valve and left ventricular dimensions and documented direction of flow across the foramen ovale and aortic arch, and mitral valve inflow pattern and any gestational changes. Data were used to identify fetuses satisfying the Boston criteria for emerging HLHS and estimate the proportion of these that would have been ideal FV candidates. We applied the threshold score whereby a score of 1 was assigned to fetuses for each Z-score meeting the following criteria: left ventricular length and width > 0; mitral valve diameter > -2; aortic valve diameter > -3.5; and pressure gradient across either the mitral or aortic valve > 20 mmHg. We compared the predicted circulation with known survival and final postnatal circulation (BV, UV or conversion from BV to UV).Results: Among the 107 ongoing pregnancies there were eight spontaneous fetal deaths and 99 livebirths. Five were lost to follow-up, five had comfort care and four had mild aortic stenosis not requiring intervention. There was intention-to-treat in these 85 newborns but five died prior to surgery, before circulation could be determined, and thus 80 underwent postnatal procedures with 44 BV, 29 UV and seven BV-to-UV circulatory outcomes. Of newborns with intention-to-treat, 69/85 (81%) survived ≥ 30 days. Survival at median 6 years was superior in cases with BV circulation (P = 0.041). Those with a postnatal UV circulation showed a trend towards smaller aortic valve diameters at first scan than did the BV cohort (P = 0.076), but aortic valve growth velocities were similar in both cohorts to term. In contrast, the mitral valve diameter was significantly smaller at first scan in those with postnatal UV outcomes (P = 0.004) and its growth velocity (P = 0.008), in common with the left ventricular inlet length (P = 0.004) and width (P = 0.002), were reduced significantly by term in fetuses with UV compared with BV outcome. Fetal data, recorded before 30 completed gestational weeks, from 70 treated neonates were evaluated to identify emerging HLHS. Forty-four had moderate or severe left ventricular depression and 38 of these had retrograde flow in the aortic arch and two had left-to-right flow at atrial level and reversed a-waves in the pulmonary veins. Thus 40 neonates met the criteria for emerging HLHS and BV circulation was documented in 13 (33%). Of these 40 cases, 12 (30%) had a threshold score of 4 or 5, of which five (42%) had BV circulation without fetal intervention.Conclusions: The natural history in our cohort of fetuses with aortic stenosis and known outcomes shows that a substantial proportion of fetuses meeting the criteria for emerging HLHS, with or without favorable selection criteria for FV, had a sustained BV circulation without fetal intervention. This indicates that further work is needed to refine the selection criteria to offer appropriate therapy to fetuses with aortic stenosis. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd. AD - The Fetal Center at Children's Memorial Hermann Hospital, McGovern Medical School at The University of Texas Health Science Center at Houston (UTHealth), TX, USA Department of Paediatric and Congenital Cardiology, University of Heidelberg, Heidelberg Germany Department of Paediatric Cardiology, Children's Heart Centre, Linz Austria University of Helsinki and Helsinki University Hospital, Helsinki Finland Department of Paediatric Cardiology, University Hospital Bonn, Bonn Germany Perinatal Cardiology Clinic, 2nd Department of Obstetrics and Gynecology, Medical University of Warsaw, Warsaw Poland Department of Paediatric Cardiology, Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Gothenburg Sweden Department of Paediatric Cardiology, Sant Joan de Déu, Barcelona Spain Department of Paediatric and Congenital Cardiology, Royal Brompton and Harefield Hospital, NHS Trust, and Fetal Medicine Unit, St George's Hospital NHS Trust, London UK University Hospital and Masaryk University Brno, Brno Czech Republic Center of Fetal Cardiology, Policlinico San Donato IRCSS, Milan Italy Congenital and Paediatric Cardiac Service, Glenfield Hospital, Leicester UK Academic Medical Center, Amsterdam The Netherlands Clinic for Pediatric Cardiology, Heart Centre, University of Leipzig, Leipzig Germany Department of Pediatric Cardiology, Second University of Naples, Monaldi Hospital, Naples Italy Department of Congenital and Pediatric Cardiology, University Hospital Gasthuisberg, Leuven Belgium AN - 117899149. Language: English. Entry Date: 20170223. Revision Date: 20190711. Publication Type: journal article AU - Gardiner, H. M. AU - Kovacevic, A. AU - Tulzer, G. AU - Sarkola, T. AU - Herberg, U. AU - Dangel, J. AU - Öhman, A. AU - Bartrons, J. AU - Carvalho, J. S. AU - Jicinska, H. AU - Fesslova, V. AU - Averiss, I. AU - Mellander, M. AU - Bulock, Frances AU - Shebani, Suhair AU - Clur, Sally Ann AU - Daehnert, Ingo AU - Salvo, Giovanni Di AU - Heying, Ruth AU - Gewillig, Marc DB - ccm DO - 10.1002/uog.15876 DP - EBSCOhost IS - 3 KW - Ultrasonography, Prenatal Fetal Diseases -- Surgery Aortic Valve Stenosis -- Surgery Balloon Dilatation Infant Pregnancy Europe Fetal Heart Fetal Diseases -- Epidemiology Human Coronary Circulation Infant, Newborn Aortic Valve Stenosis -- Epidemiology Aortic Valve Stenosis Fetal Diseases Gestational Age Retrospective Design Female Pregnancy Outcomes Validation Studies Comparative Studies Evaluation Research Multicenter Studies N1 - research; tables/charts. Journal Subset: Biomedical; Europe; Peer Reviewed; UK & Ireland. NLM UID: 9108340. PMID: NLM26843026. PY - 2016 SN - 0960-7692 SP - 373-381 ST - Natural history of 107 cases of fetal aortic stenosis from a European multicenter retrospective study T2 - Ultrasound in Obstetrics & Gynecology TI - Natural history of 107 cases of fetal aortic stenosis from a European multicenter retrospective study UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=117899149&site=ehost-live&scope=site VL - 48 ID - 1519 ER - TY - JOUR AB - Plastic bronchitis is a condition in which large, rubber-like, bronchial casts develop in the tracheobronchial tree causing airway obstruction. It is an unusual disorder that occurs in various disease states. Most case reports of plastic bronchitis associated with congenital heart disease are in patients palliated with Fontan physiology. We describe a 13-year-old girl with DiGeorge syndrome and truncus arteriosus who underwent an uneventful truncal valve replacement. The child developed plastic bronchitis on postoperative day eight requiring extra-corporeal membrane oxygenation support and daily bronchoscopy to remove bronchial casts. Our patient did not have elevated systemic venous pressures or Fontan physiology. We speculate the etiology of plastic bronchitis may have been surgical trauma to the lymphatic channels surrounding the bronchi and disruption of pleural adhesions. A comprehensive evaluation revealed no specific cause and the diagnosis of idiopathic plastic bronchitis was made. AD - Department of Pediatric and Congenital Cardiothoracic Surgery, Levine Children's Hospital, Charlotte, NC, USA. Department of Pediatric Pulmonology, Levine Children's Hospital, Charlotte, NC, USA. Department of Pediatric General Surgery, Levine Children's Hospital, Charlotte, NC, USA. AN - 31214435 AU - Lang, K. AU - Champion, E. AU - Cosper, G. AU - Peeler, B. AU - Maxey, T. C2 - PMC6530730 DA - Sep DB - PubMed DO - 10.3233/pic-13062 DP - NLM ET - 2013/09/01 IS - 3 KW - Plastic bronchitis bronchial casts congenital heart disease truncal valve replacement truncus arteriosus LA - eng N1 - 2146-4626 Lang, Kristin Champion, Elizabeth Cosper, Graham Peeler, Benjamin Maxey, Thomas Case Reports J Pediatr Intensive Care. 2013 Sep;2(3):131-135. doi: 10.3233/PIC-13062. PY - 2013 SN - 2146-4618 (Print) 2146-4626 SP - 131-135 ST - A near lethal case of plastic bronchitis following truncal valve replacement T2 - J Pediatr Intensive Care TI - A near lethal case of plastic bronchitis following truncal valve replacement VL - 2 ID - 466 ER - TY - JOUR AB - OBJECTIVES: To assess health-related quality of life (HRQOL) in families of young children with complex congenital heart disease (CHD), and identify the demographic, clinical, and psychosocial factors that place these children and their mothers at greater risk of vulnerability. STUDY DESIGN: This cross-sectional study took place from June 2015 to October 2016 at The Sydney Children's Hospitals Network Cardiac Service, Australia. Mothers of a child aged 1-5 years with either single ventricle CHD or CHD requiring neonatal biventricular repair were invited to participate. Eighty-seven mothers completed a suite of validated measures, including the Pediatric Quality of Life Inventory, which assessed the outcomes of child and maternal HRQOL. RESULTS: Sixty percent of children with single ventricle CHD and 25% of children with biventricular repair had total Pediatric Quality of Life Inventory scores within the at-risk range. Lower child HRQOL was strongly associated with single ventricle CHD (β = -0.38; P < .001), physical comorbidity (β = -0.32; P = .001), feeding difficulties (β = -0.26; P = .008), and greater maternal psychological stress (β = -0.18; P = .045), accounting for 52% of the variance in child HRQOL. Lower maternal HRQOL was strongly associated with poorer family functioning (β = 0.61; P < .001), greater maternal psychological stress (β = -0.23; P = .004), child physical comorbidity (β = -0.17; P = .01), and a 'difficult' child temperament (β = -0.14; P = .01), accounting for 73% of the variance in maternal HRQOL. CONCLUSIONS: Lower HRQOL is common in young children with complex CHD, particularly single ventricle CHD. Several predictors of HRQOL are potentially modifiable, offering possible pathways for prevention and early intervention. Routine screening is a necessary first step toward developing models of care to improve HRQOL in this population. AD - Discipline of Pediatrics, School of Women's and Children's Health, UNSW Medicine, The University of New South Wales, Sydney, NSW, Australia; Heart Center for Children, The Sydney Children's Hospitals Network (Westmead and Randwick), Sydney, NSW, Australia. Heart Center for Children, The Sydney Children's Hospitals Network (Westmead and Randwick), Sydney, NSW, Australia; Discipline of Child and Adolescent Health, Sydney Medical School, The University of Sydney, NSW, Australia. Pain Management Research Institute, Royal North Shore Hospital, North Sydney, NSW, Australia; Sydney Medical School, The University of Sydney, NSW, Australia. Discipline of Pediatrics, School of Women's and Children's Health, UNSW Medicine, The University of New South Wales, Sydney, NSW, Australia; Heart Center for Children, The Sydney Children's Hospitals Network (Westmead and Randwick), Sydney, NSW, Australia. Electronic address: n.kasparian@unsw.edu.au. AN - 30366775 AU - Denniss, D. L. AU - Sholler, G. F. AU - Costa, D. S. J. AU - Winlaw, D. S. AU - Kasparian, N. A. DA - Feb DB - PubMed DO - 10.1016/j.jpeds.2018.09.037 DP - NLM ET - 2018/10/28 KW - Adult Anxiety/psychology Case-Control Studies Child, Preschool Cognition Cross-Sectional Studies Female Heart Defects, Congenital/*psychology/surgery Humans Infant Male Mothers/*psychology Quality of Life/*psychology Stress, Psychological/psychology Surveys and Questionnaires *child development *congenital heart disease *integrated care *mental health *psychological stress *psychosocial screening *quality of life LA - eng N1 - 1097-6833 Denniss, Dominique L Sholler, Gary F Costa, Daniel S J Winlaw, David S Kasparian, Nadine A Journal Article Research Support, Non-U.S. Gov't United States J Pediatr. 2019 Feb;205:21-28.e2. doi: 10.1016/j.jpeds.2018.09.037. Epub 2018 Oct 23. PY - 2019 SN - 0022-3476 SP - 21-28.e2 ST - Need for Routine Screening of Health-Related Quality of Life in Families of Young Children with Complex Congenital Heart Disease T2 - J Pediatr TI - Need for Routine Screening of Health-Related Quality of Life in Families of Young Children with Complex Congenital Heart Disease VL - 205 ID - 78 ER - TY - JOUR AD - Boston Children's Hospital, Boston, MA, USA AN - 142458412. Language: English. Entry Date: In Process. Revision Date: 20200401. Publication Type: Article. Supplement Title: 2020 Supplement 1. Journal Subset: Biomedical AU - Bucholz, Emily AU - Sleeper, Lynn AU - Goldberg, Caren AU - Pasquali, Sara AU - Anderson, Brett AU - Gaynor, J. William AU - Cnota, James Frank AU - Newburger, Jane W. DB - ccm DO - 10.1016/S0735-1097(20)31184-0 DP - EBSCOhost N1 - Peer Reviewed; USA. NLM UID: 8301365. PY - 2020 SN - 0735-1097 SP - 557-557 ST - NEIGHBORHOOD SOCIOECONOMIC STATUS AND DIFFERENCES IN TRANSPLANT-FREE SURVIVAL, NEURODEVELOPMENT, AND HEALTH-RELATED QUALITY OF LIFE IN CHILDREN WITH SINGLE VENTRICLE HEART DISEASE: RESULTS FROM THE PEDIATRIC HEART NETWORK (PHN) SINGLE VENTRICLE RECONSTRUCTION (SVR) STUDY T2 - Journal of the American College of Cardiology (JACC) TI - NEIGHBORHOOD SOCIOECONOMIC STATUS AND DIFFERENCES IN TRANSPLANT-FREE SURVIVAL, NEURODEVELOPMENT, AND HEALTH-RELATED QUALITY OF LIFE IN CHILDREN WITH SINGLE VENTRICLE HEART DISEASE: RESULTS FROM THE PEDIATRIC HEART NETWORK (PHN) SINGLE VENTRICLE RECONSTRUCTION (SVR) STUDY UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=142458412&site=ehost-live&scope=site VL - 75 ID - 1467 ER - TY - JOUR AB - A 5-year-old child with hypoplastic left heart syndrome presented with myocardial infarction 2 years after Fontan completion. Evaluation identified a thrombus in his native aortic root. Retrospective review of a prior catheterization revealed stasis in the native aortic root. Surgical thrombectomy was performed after failed medical management. The neoaortic root was revised at the time of thrombectomy in an attempt to reduce stasis in the native aortic root. This case suggests the need to identify anatomic findings that may predispose to coronary artery thrombus formation after Norwood palliation. © 2006 The Society of Thoracic Surgeons. AD - Division of Pediatric Cardiology, Medical University of South Carolina, Charleston, SC, United States Division of Cardiothoracic Surgery, Medical University of South Carolina, Charleston, SC, United States AU - Graham, E. M. AU - Shakir, H. AU - Atz, A. M. AU - Ringewald, J. M. AU - Bradley, S. M. DB - Scopus DO - 10.1016/j.athoracsur.2006.07.068 IS - 5 M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2006 SP - e29-e30 ST - Neoaortic Root Modification for Late Thrombosis After Norwood Palliation T2 - Annals of Thoracic Surgery TI - Neoaortic Root Modification for Late Thrombosis After Norwood Palliation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-33750057684&doi=10.1016%2fj.athoracsur.2006.07.068&partnerID=40&md5=1e4390ad524cb0b728638b4219ea6ba7 VL - 82 ID - 2219 ER - TY - JOUR AB - Background: Brain injury (BI) is reported in 60% of newborns with critical congenital heart disease as white matter injury (WMI) or stroke. Neurodevelopmental (ND) impairments are reported in these patients. The relationship between neonatal BI and ND outcome has not been established. Objectives: This study sought to determine the association between peri-operative BI and ND outcomes in infants with single ventricle physiology (SVP) and d-transposition of the great arteries (d-TGA). Methods: Term newborns with d-TGA and SVP had pre-operative and post-operative brain magnetic resonance imaging and ND outcomes assessed at 12 and 30 months with the Bayley Scales of Infant Development-II. BI was categorized by the brain injury severity score and WMI was quantified by volumetric analysis. Results: A total of 104 infants had follow-up at 12 months and 70 had follow-up at 30 months. At 12 months, only clinical variables were associated with ND outcome. At 30 months, subjects with moderate-to-severe WMI had significantly lower Psychomotor Development Index (PDI) scores (13 points lower) as compared with those with none or minimal WMI for d-TGA and SVP (p = 0.03 and p = 0.05, respectively) after adjusting for various factors. Quantitative WMI volume was likewise associated. Stroke was not associated with outcome. The Bland-Altman limits of agreement for PDI scores at 12 and 30 months were wide (−40.3 to 31.2) across the range of mean PDI values. Conclusions: Increasing burden of WMI is associated with worse motor outcomes at 30 months for infants with critical congenital heart disease, whereas no adverse association was seen between small strokes and outcome. These results support the utility of neonatal brain magnetic resonance imaging in this population to aid in predicting later outcomes and the importance of ND follow-up beyond 1 year of age. AD - P.S. McQuillen, Department of Pediatrics, Division of Critical Care, University of California San Francisco Benioff Children's Hospital, 1550 4th Street, RM RH-384E, San Francisco, California, United States AU - Peyvandi, S. AU - Chau, V. AU - Guo, T. AU - Xu, D. AU - Glass, H. C. AU - Synnes, A. AU - Poskitt, K. AU - Barkovich, A. J. AU - Miller, S. P. AU - McQuillen, P. S. DB - Embase Medline DO - 10.1016/j.jacc.2018.02.068 IS - 18 KW - article Bayley Scales of Infant Development brain injury clinical assessment congenital heart disease controlled study disease association female follow up great vessels transposition human injury scale major clinical study male mental disease nervous system development newborn nuclear magnetic resonance imaging postoperative period preoperative evaluation priority journal prospective study Psychomotor Development Index quantitative analysis scoring system single ventricle physiology time factor volumetry white matter injury LA - English M3 - Article N1 - L2000703471 2018-05-14 2018-06-12 PY - 2018 SN - 1558-3597 0735-1097 SP - 1986-1996 ST - Neonatal Brain Injury and Timing of Neurodevelopmental Assessment in Patients With Congenital Heart Disease T2 - Journal of the American College of Cardiology TI - Neonatal Brain Injury and Timing of Neurodevelopmental Assessment in Patients With Congenital Heart Disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2000703471 http://dx.doi.org/10.1016/j.jacc.2018.02.068 VL - 71 ID - 686 ER - TY - JOUR AB - The death rate among neonates with cardiovascular disease is 50% during the first 6 mth. with the majority dying during the first month. With early diagnosis most of these babies could be saved. In approaching the diagnosis of cardiac distress in the newborn, it is important to remember that the types of cardiovascular disease which cause symptoms and death early in life are quite different from those in older children. Lesions such as hypoplasia of the left heart, transposition of the great arteries, endocardial fibroelastosis, pulmonary atresia. mitral atresia, tricuspid atresia and t run cue arteriös us are common, not rare, causes of cardiac distress in the newborn. A classification of neonatal cardiovascular disease! into 7 pathophysiological groups is presented as a basis for an effective,practical approach to the differential diagnosis of the potentially lethal lesions. This approach is simplified further since over 90% of babies with cardiac distress have one of 3 lesions: (1) Large left-to-right shunt (characterized by the presence of massive plethora on the chest roentgenogram), (2) Large right-to-left shunt (association with intense cyanosis) or (3) Severe obstruction (including hypoplasia of the left heart, which is the most common cause of death due to cardiac distress during the first week of life). AD - G.G. Cayler, Sutter Commun. Hosp., Sacramento, Ca AU - Cayler, G. G. DB - Embase Classic Medline IS - 3 KW - artery baby cardiovascular disease catecholamine depletion cause of death child classification cyanosis death diagnosis differential diagnosis early diagnosis endocardial fibroelastosis heart heart left right shunt cardiac muscle cell heart right left shunt heart septum defect hypoplasia pulmonary valve atresia mitral valve atresia mortality newborn obstruction thorax radiography tricuspid valve atresia Wolff Parkinson White syndrome LA - English M3 - Article N1 - L288040025 1968-12-01 PY - 1968 SN - 0008-1264 SP - 188-200 ST - Neonatal cardiac distress. a practical approach to recognition. diagnosis, and management T2 - California medicine TI - Neonatal cardiac distress. a practical approach to recognition. diagnosis, and management UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L288040025 VL - 108 ID - 1409 ER - TY - JOUR AB - Background: Congenital fetal cardiac anomalies compromise the most common group of fetal structural anomalies. Several previous reports analyzed all types of fetal cardiac anomalies together without individualized neonatal morbidity outcomes based on cardiac defect. Mode of delivery in cases of fetal cardiac anomalies varies greatly as optimal mode of delivery in these complex cases is unknown.Objective: We sought to determine rates of neonatal outcomes for fetal cardiac anomalies and examine the role of attempted route of delivery on neonatal morbidity.Study Design: Gravidas with fetal cardiac anomalies and delivery >34 weeks, excluding stillbirths and aneuploidies (n = 2166 neonates, n = 2701 cardiac anomalies), were analyzed from the Consortium on Safe Labor, a retrospective cohort study of electronic medical records. Cardiac anomalies were determined using International Classification of Diseases, Ninth Revision codes and organized based on morphology. Neonates were assigned to each cardiac anomaly classification based on the most severe cardiac defect present. Neonatal outcomes were determined for each fetal cardiac anomaly. Composite neonatal morbidity (serious respiratory morbidity, sepsis, birth trauma, hypoxic ischemic encephalopathy, and neonatal death) was compared between attempted vaginal delivery and planned cesarean delivery for prenatal and postnatal diagnosis. We used multivariate logistic regression to calculate adjusted odds ratio for composite neonatal morbidity controlling for race, parity, body mass index, insurance, gestational age, maternal disease, single or multiple anomalies, and maternal drug use.Results: Most cardiac anomalies were diagnosed postnatally except hypoplastic left heart syndrome, which had a higher prenatal than postnatal detection rate. Neonatal death occurred in 8.4% of 107 neonates with conotruncal defects. Serious respiratory morbidity occurred in 54.2% of 83 neonates with left ventricular outflow tract defects. Overall, 76.3% of pregnancies with fetal cardiac anomalies underwent attempted vaginal delivery. Among patients who underwent attempted vaginal delivery, 66.1% had a successful vaginal delivery. Women with a fetal cardiac anomaly diagnosed prenatally were more likely to have a planned cesarean delivery than women with a postnatal diagnosis (31.7 vs 22.8%; P < .001). Planned cesarean delivery compared to attempted vaginal delivery was not associated with decreased composite neonatal morbidity for all prenatally diagnosed (adjusted odds ratio, 1.67; 95% confidence interval, 0.85-3.30) or postnatally diagnosed (adjusted odds ratio, 0.99; 95% confidence interval, 0.77-1.27) cardiac anomalies.Conclusion: Most fetal cardiac anomalies were diagnosed postnatally and associated with increased rates of neonatal morbidity. Planned cesarean delivery for prenatally diagnosed cardiac anomalies was not associated with less neonatal morbidity. AD - Department of Obstetrics and Gynecology, MedStar Washington Hospital Center, Washington, DC Department of Biostatistics and Bioinformatics, MedStar Health Research Institute, Hyattsville, MD Georgetown-Howard Universities Center for Clinical and Translational Science, Washington, DC Department of Obstetrics and Gynecology, MedStar Georgetown University Hospital, Washington, DC AN - 125312211. Language: English. Entry Date: 20171006. Revision Date: 20180510. Publication Type: journal article AU - Parikh, Laura I. AU - Grantz, Katherine L. AU - Iqbal, Sara N. AU - Huang, Chun-Chih AU - Landy, Helain J. AU - Fries, Melissa H. AU - Reddy, Uma M. DB - ccm DO - 10.1016/j.ajog.2017.05.049 DP - EBSCOhost IS - 4 KW - Delivery, Obstetric -- Statistics and Numerical Data Labor, Induced -- Statistics and Numerical Data Cesarean Section -- Statistics and Numerical Data Heart Defects, Congenital -- Epidemiology Infant Prospective Studies Multivariate Analysis Female Respiratory Distress Syndrome -- Epidemiology Prenatal Diagnosis Pregnancy Infant, Newborn Gestational Age Infant Mortality United States Retrospective Design Human Funding Source N1 - research. Journal Subset: Biomedical; Peer Reviewed; USA. Grant Information: HHSN267200603425C/HD/NICHD NIH HHS/United States. NLM UID: 0370476. PMID: NLM28578168. PY - 2017 SN - 0002-9378 SP - 469.e1-469.e12 ST - Neonatal outcomes in fetuses with cardiac anomalies and the impact of delivery route T2 - American Journal of Obstetrics & Gynecology TI - Neonatal outcomes in fetuses with cardiac anomalies and the impact of delivery route UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=125312211&site=ehost-live&scope=site VL - 217 ID - 1598 ER - TY - JOUR AB - Background Congenital fetal cardiac anomalies compromise the most common group of fetal structural anomalies. Several previous reports analyzed all types of fetal cardiac anomalies together without individualized neonatal morbidity outcomes based on cardiac defect. Mode of delivery in cases of fetal cardiac anomalies varies greatly as optimal mode of delivery in these complex cases is unknown. Objective We sought to determine rates of neonatal outcomes for fetal cardiac anomalies and examine the role of attempted route of delivery on neonatal morbidity. Study Design Gravidas with fetal cardiac anomalies and delivery >34 weeks, excluding stillbirths and aneuploidies (n = 2166 neonates, n = 2701 cardiac anomalies), were analyzed from the Consortium on Safe Labor, a retrospective cohort study of electronic medical records. Cardiac anomalies were determined using International Classification of Diseases, Ninth Revision codes and organized based on morphology. Neonates were assigned to each cardiac anomaly classification based on the most severe cardiac defect present. Neonatal outcomes were determined for each fetal cardiac anomaly. Composite neonatal morbidity (serious respiratory morbidity, sepsis, birth trauma, hypoxic ischemic encephalopathy, and neonatal death) was compared between attempted vaginal delivery and planned cesarean delivery for prenatal and postnatal diagnosis. We used multivariate logistic regression to calculate adjusted odds ratio for composite neonatal morbidity controlling for race, parity, body mass index, insurance, gestational age, maternal disease, single or multiple anomalies, and maternal drug use. Results Most cardiac anomalies were diagnosed postnatally except hypoplastic left heart syndrome, which had a higher prenatal than postnatal detection rate. Neonatal death occurred in 8.4% of 107 neonates with conotruncal defects. Serious respiratory morbidity occurred in 54.2% of 83 neonates with left ventricular outflow tract defects. Overall, 76.3% of pregnancies with fetal cardiac anomalies underwent attempted vaginal delivery. Among patients who underwent attempted vaginal delivery, 66.1% had a successful vaginal delivery. Women with a fetal cardiac anomaly diagnosed prenatally were more likely to have a planned cesarean delivery than women with a postnatal diagnosis (31.7 vs 22.8%; P <.001). Planned cesarean delivery compared to attempted vaginal delivery was not associated with decreased composite neonatal morbidity for all prenatally diagnosed (adjusted odds ratio, 1.67; 95% confidence interval, 0.85–3.30) or postnatally diagnosed (adjusted odds ratio, 0.99; 95% confidence interval, 0.77–1.27) cardiac anomalies. Conclusion Most fetal cardiac anomalies were diagnosed postnatally and associated with increased rates of neonatal morbidity. Planned cesarean delivery for prenatally diagnosed cardiac anomalies was not associated with less neonatal morbidity. AD - L.I. Parikh, Georgetown-Howard Universities Center for Clinical and Translational Science, Washington, DC, United States AU - Parikh, L. I. AU - Grantz, K. L. AU - Iqbal, S. N. AU - Huang, C. C. AU - Landy, H. J. AU - Fries, M. H. AU - Reddy, U. M. DB - Embase Medline DO - 10.1016/j.ajog.2017.05.049 IS - 4 KW - adult aortic valve stenosis Apgar score article body mass cesarean section cohort analysis congenital heart malformation drug use Ebstein anomaly electronic medical record Fallot tetralogy female fetus malformation gestational age great vessels transposition health insurance heart atrium septum defect heart left ventricle outflow tract obstruction heart right ventricle double outlet heart right ventricle outflow tract obstruction heart ventricle septum defect human hypoplastic left heart syndrome intermethod comparison labor induction live birth lung vein drainage anomaly major clinical study maternal disease newborn newborn death newborn morbidity newborn mortality obstetric delivery parity prenatal diagnosis priority journal pulmonary valve atresia race respiratory tract disease retrospective study vaginal delivery LA - English M3 - Article N1 - L617276253 2017-07-14 2017-10-11 PY - 2017 SN - 1097-6868 0002-9378 SP - 469.e1-469.e12 ST - Neonatal outcomes in fetuses with cardiac anomalies and the impact of delivery route T2 - American Journal of Obstetrics and Gynecology TI - Neonatal outcomes in fetuses with cardiac anomalies and the impact of delivery route UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L617276253 http://dx.doi.org/10.1016/j.ajog.2017.05.049 VL - 217 ID - 731 ER - TY - JOUR AB - Congenital heart disease (CHD) occurs in 8 per 1000 live births, with approximately one third of these neonates requiring intervention in the first month of life. Neonates with respiratory distress, cyanosis, feeding difficulties, low cardiac output, or dysmorphic syndromes commonly have CHD. Clinical suspicion increases in a symptomatic infant with a heart murmur, but the presence or absence of a murmur does not assure either the presence or absence of significant congenital heart disease. Infants suspected to have CHD may be divided into premature and term infants, as well as infants with duct-dependent pulmonary blood flow, infants with duct-dependent systemic blood flow, and infants with unrestricted pulmonary blood flow. This article will also address the specialized clinical situations of total anomalous pulmonary venous return, transposition of the great arteries, and hypoplastic left heart syndrome with intact atrial septum. Copyright © 2002 by Aspen Publishers, Inc. AD - Sibley Heart Center Cardiology, Associate Clinical Professor Pediatrics, Emory University School of Medicine, Atlanta, GA AN - 106799778. Language: English. Entry Date: 20030124. Revision Date: 20150818. Publication Type: Journal Article AU - McConnell, M. E. AU - Elixson, E. M. DB - ccm DP - EBSCOhost IS - 3 KW - Heart Defects, Congenital -- Diagnosis Heart Defects, Congenital -- Therapy Heart Defects, Congenital -- Classification Infant, Newborn Infant, Premature Ductus Arteriosus, Patent Prostaglandins -- Therapeutic Use -- In Infancy and Childhood Heart Septal Defects, Ventricular Vascular Resistance Tetralogy of Fallot Hypoplastic Left Heart Syndrome Transposition of Great Arteries Pediatric Critical Care Nursing N1 - diagnostic images; pictorial; tables/charts. Journal Subset: Core Nursing; Nursing; Peer Reviewed; USA. NLM UID: 8704517. PMID: NLM12450156. PY - 2002 SN - 0887-9303 SP - 17-25 ST - The neonate with suspected congenital heart disease T2 - Critical Care Nursing Quarterly TI - The neonate with suspected congenital heart disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106799778&site=ehost-live&scope=site VL - 25 ID - 1540 ER - TY - JOUR AB - The United States is the largest country in the 'industrialized world', and, as such, has dominated the medical literature reporting practices and outcomes of neonatal intensive care. Perhaps surprisingly, very few things about U.S. Neonatology are unique to the U.S. This chapter will attempt an impossible task - summarizing the practice of neonatology in the entire U.S. I will draw parallels to practices of my colleagues around the globe, and point out American idiosyncracies where they arise. I will end with my sense of the likely (at least in the near-term) future developments for neonatology in the U.S. © 2013 Bentham Science Publishers. AD - W. Meadow, MacLean Center for Clinical Medical Ethics, The University of Chicago, United States AU - Meadow, W. DB - Embase DO - 10.2174/157339613805289578 IS - 1 KW - antibiotic agent article congenital malformation Down syndrome ethnicity futurology gestational age history human hypoplastic left heart syndrome legal abortion meningomyelocele neonatology newborn care newborn intensive care newborn morbidity newborn mortality prenatal diagnosis priority journal trisomy 13 trisomy 18 United States ventilator LA - English M3 - Article N1 - L368517819 2013-03-20 2013-03-25 PY - 2013 SN - 1573-3963 1875-6336 SP - 32-35 ST - Neonatology in the United States: Where we are, and where we are going T2 - Current Pediatric Reviews TI - Neonatology in the United States: Where we are, and where we are going UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L368517819 http://dx.doi.org/10.2174/157339613805289578 VL - 9 ID - 1010 ER - TY - JOUR AB - OBJECTIVES: Complex neonatal surgery is considered a risk factor for neuro-developmental impairment in single-ventricle patients. Neuro-developmental outcome was compared between preschool-aged Fontan patients who underwent a Norwood procedure and single-ventricle patients not requiring neonatal surgery with cardiopulmonary bypass. METHODS: Verbal, performance and full-scale intelligence quotient (IQ) were evaluated with the Wechsler Preschool and Primary Scale of Intelligence. Cognitive functions were assessed with the German 'Kognitiver Entwicklungstest für das Kindergartenalter' (KET-KID). Risk factors for impaired neuro-development were evaluated. RESULTS: Neuro-developmental assessment was completed in 95 patients (Norwood: n = 69; non-Norwood: n = 26). Median (interquartile range) IQ and KET-KID scores were in the normal range. Except for verbal KET-KID, scores did not differ between Norwood and non-Norwood patients (verbal IQ: 98 (86-105) vs 93 (85-102), P = 0.312; performance IQ: 91 (86-100) vs 96 (86-100), P = 0.932; full-scale IQ: 93 (86-101) vs 89 (84-98), P = 0.314; KET-KID verbal: 48 (17-72) vs 25 (2-54), P = 0.020; KET-KID non-verbal: 33 (18-62) vs 45 (15-54), P = 0.771; KET-KID global: 42 (14-65) vs 28 (6-63), P = 0.208). Full-scale IQ was below average (<85 points) in 14 (20%) Norwood and 9 (35%) non-Norwood cases (P = 0.181). Global KET-KID was below average (<16th percentile) in 19 (28%) and 10 (38%) patients (P = 0.326). Smaller head circumference z-score and complications before neonatal surgery were independently associated with lower scores. CONCLUSIONS: Neuro-developmental outcome of preschool-aged Fontan patients was in the normal range. The Norwood procedure was not a risk factor for neuro-developmental impairment. Preoperative condition and patient-related factors were more important determinants than variables related to surgical palliation. AD - Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital of Schleswig-Holstein, Campus Kiel, Kiel, Germany. Department of Anesthesiology and Intensive Care Medicine, University Hospital of Schleswig-Holstein, Campus Kiel, Kiel, Germany. Department of Cardiovascular Surgery, University Hospital of Schleswig-Holstein, Campus Kiel, Kiel, Germany. DZHK (German Center for Cardiovascular Research), Partner Site Hamburg/Kiel/Lübeck, Kiel, Germany. AN - 28472306 AU - Rotermann, I. AU - Logoteta, J. AU - Falta, J. AU - Wegner, P. AU - Jung, O. AU - Dütschke, P. AU - Scheewe, J. AU - Kramer, H. H. AU - Hansen, J. H. DA - Sep 1 DB - PubMed DO - 10.1093/ejcts/ezx119 DP - NLM ET - 2017/05/05 IS - 3 KW - Child Child, Preschool Cognition/physiology Cognition Disorders/*epidemiology/etiology/physiopathology Developmental Disabilities/*epidemiology/etiology/physiopathology Female Germany/epidemiology Humans Hypoplastic Left Heart Syndrome/*surgery Incidence Male Neuropsychological Tests Norwood Procedures/*adverse effects *Risk Assessment Risk Factors Treatment Outcome Cardiopulmonary bypass Neuro-developmental outcome Univentricular heart disease LA - eng N1 - 1873-734x Rotermann, Ina Logoteta, Jana Falta, Janine Wegner, Philip Jung, Olaf Dütschke, Peter Scheewe, Jens Kramer, Hans-Heiner Hansen, Jan Hinnerk Journal Article Germany Eur J Cardiothorac Surg. 2017 Sep 1;52(3):558-564. doi: 10.1093/ejcts/ezx119. PY - 2017 SN - 1010-7940 SP - 558-564 ST - Neuro-developmental outcome in single-ventricle patients: is the Norwood procedure a risk factor? T2 - Eur J Cardiothorac Surg TI - Neuro-developmental outcome in single-ventricle patients: is the Norwood procedure a risk factor? VL - 52 ID - 205 ER - TY - JOUR AB - In a prospective study, we evaluated the perioperative application of the Neonatal Intensive Care Unit Network Neurobehavioral Scale in a cohort of newborns with congenital heart disease (CHD). Infants with CHD were found to have suboptimal neurobehavioral performance compared with healthy infants without CHD, with particular vulnerability in the Regulation and Stress subscales. Copyright © 2011 Mosby Inc. All rights reserved. AD - A. N. Massaro, George Washington University School of Medicine, Department of Neonatology, Children's National Medical Center, 111 Michigan Ave NW, Washington, DC 20010, United States AU - Massaro, A. N. AU - Glass, P. AU - Brown, J. AU - Chang, T. AU - Krishnan, A. AU - Jonas, R. A. AU - Donofrio, M. T. DB - Embase Medline DO - 10.1016/j.jpeds.2010.11.060 IS - 4 KW - Apgar score article behavior disorder birth weight cesarean section cohort analysis congenital heart disease controlled study deep hypothermic circulatory arrest female gestational age head circumference heart single ventricle human hypoplastic left heart syndrome intensive care unit major clinical study male newborn open heart surgery prenatal diagnosis priority journal risk factor LA - English M3 - Article N1 - L51228688 2011-01-16 2011-03-28 PY - 2011 SN - 0022-3476 SP - 678-681.e2 ST - Neurobehavioral abnormalities in newborns with congenital heart disease requiring open-heart surgery T2 - Journal of Pediatrics TI - Neurobehavioral abnormalities in newborns with congenital heart disease requiring open-heart surgery UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51228688 http://dx.doi.org/10.1016/j.jpeds.2010.11.060 VL - 158 ID - 1108 ER - TY - JOUR AB - Brain maturation in 1-36 month old children suffering from congenital cardiopathologies was assessed after a study of psychomotor development. The Rogers' test (Rogers et al., Developmental programming for infants and young children. Volume 2. Early intervention developmental profile, Revised edition, ESL/ELT Michigan, Ann Arbor, 1981) was applied to 65 children, of whom 21 presented with simple cardiopathologies (CpS) and 22 with complex cardiopathologies (CpC). All children were matched by age, sex and socioeconomic status to 22 healthy children in a control group (C). Mean differences between the three groups were established by applying the Kruskal-Wallis test, and mean differences between the C and CpS/CpC groups were determined using the Mann-Whitney test. The proportion of cases evaluated as "low" in each group was calculated by applying the Rogers' test, and a test of proportion differences was applied between the C and CpS/CpC groups. CpS children performed similarly to the C, whereas CpC children scored significantly lower than C children on all variables. It is highly likely that the suboptimal psychomotor performance observed in CpC children was due to compromised hemodynamics and related to subclinical immaturity of cerebral development. © 2012 Springer Science+Business Media, LLC. AD - G.A. Otero-Ojeda, Facultad de Medicina, Universidad Autónoma Del Estado de México, P. Tollocan esq. J. Carranza (s/n), 50180 Toluca, Mexico, Mexico AU - Porcayo-Mercado, M. R. AU - Otero-Ojeda, G. A. AU - Pliego-Rivero, F. B. AU - Aguirre-Pérez, D. M. AU - Ricardo-Garcell, J. DB - Embase Medline DO - 10.1007/s10880-012-9314-3 IS - 1 KW - age aortic coarctation article brain development brain maturation child childhood disease cognition congenital cardiopathology congenital heart disease controlled study emotion Fallot tetralogy female heart atrium septum defect heart hemodynamics heart single ventricle heart ventricle septum defect human hypothesis immaturity infant Kruskal Wallis test language lung vein drainage anomaly major clinical study male patent ductus arteriosus perception preschool child priority journal psychologist psychometry psychomotor development psychomotor performance rank sum test self care sex social behavior social status tricuspid valve atresia LA - English M3 - Article N1 - L52158222 2012-08-15 2013-03-29 PY - 2013 SN - 1068-9583 1573-3572 SP - 71-78 ST - Neurobehavioral assessment of children presenting diverse congenital cardiopathologies T2 - Journal of Clinical Psychology in Medical Settings TI - Neurobehavioral assessment of children presenting diverse congenital cardiopathologies UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52158222 http://dx.doi.org/10.1007/s10880-012-9314-3 VL - 20 ID - 1017 ER - TY - JOUR AB - Aim: To describe neurobehavioral patterns in neonates with congenital heart disease (CHD). Method: A cohort study describing neurobehavioral performance of neonates with CHD requiring cardiac surgery. The neonates were evaluated preoperatively and postoperatively with the Neonatal Intensive Care Unit Network Neurobehavioral Scale (NNNS) and scores were compared with published normative values. Clinical factors were obtained by chart review to assess their association with behavior. The CHD NNNS score pattern was compared with previously reported profiles in other high-risk populations. Results: NNNS evaluations were completed on 67 neonates with CHD, resulting in 97 evaluations (50 preoperative, 47 postoperative). Compared with normative values, the cohort with CHD demonstrated decreased attention, regulation, asymmetry, stress, arousal, and excitability, along with increased non-optimal reflexes, lethargy, and need for handling (p<0.05 for all). Additional clinical factors had a minimal effect on the neurobehavioral pattern. Compared with previously published patterns in high-risk neonates without CHD, the cohort with CHD demonstrated a unique pattern of behavior. Interpretation: Neonates with CHD demonstrate different neurobehavioral performance compared with typically developing neonates born at term as well as other high-risk neonates. Our experience suggests there is a unique neonatal neurobehavioral pattern in the hospitalized population with CHD. Targeted neonatal neurobehavioral evaluations may be useful in developing specific therapies to improve neurodevelopmental outcomes in neonates with CHD. What this paper adds: Neonates with congenital heart disease demonstrate different neurobehavioral performance than typically developing neonates. Evaluation of neonatal neurobehavioral performance provides an opportunity to identify neurodevelopmental variability early. Identification of neurobehavioral performance variability allows targeted interactions and therapy. AD - W.J. Hogan, Division of Pediatric Cardiology, Department of Pediatrics, Primary Children's Hospital, University of Utah, Salt Lake City, UT, United States AU - Hogan, W. J. AU - Winter, S. AU - Pinto, N. M. AU - Weng, C. AU - Sheng, X. AU - Conradt, E. AU - Wood, J. AU - Puchalski, M. D. AU - Tani, L. Y. AU - Miller, T. A. DB - Embase Medline DO - 10.1111/dmcn.13912 IS - 12 KW - aortopulmonary septal defect arousal article attention behavior disorder cardiopulmonary bypass cohort analysis congenital heart disease excitability Fallot tetralogy female genetic disorder great vessels transposition habituation heart catheterization heart left right shunt heart surgery high risk population human hypoplastic left heart syndrome lethargy major clinical study male medical record review neonatal abstinence syndrome neonatal intensive care unit nerve cell differentiation newborn premature labor prenatal diagnosis priority journal pulmonary valve atresia retrospective study stress tricuspid valve atresia LA - English M3 - Article N1 - L622113202 2018-05-17 2019-01-11 PY - 2018 SN - 1469-8749 0012-1622 SP - 1225-1231 ST - Neurobehavioral evaluation of neonates with congenital heart disease: a cohort study T2 - Developmental Medicine and Child Neurology TI - Neurobehavioral evaluation of neonates with congenital heart disease: a cohort study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L622113202 http://dx.doi.org/10.1111/dmcn.13912 VL - 60 ID - 647 ER - TY - JOUR AB - Recent advances in the treatment of children with severe congenital heart defects, such as hypoplastic left heart syndrome (HLHS) and other forms of univentricular heart (UVH), have significantly improved their survival rates. However, these children are at risk for various neurodevelopmental deficits. The aim of the present study was to assess cognitive development, expressive language, and behavior in 30-month-old children with univentricular heart. The participants were 22 children with HLHS, 14 with UVH, and 41 healthy control subjects. The Bayley Scales of Infant Development II, MacArthur Communicative Development Inventories, and Child Behavior Checklist were used for assessments. The results revealed that children with HLHS exhibited a significantly lower mean mental development index, more delays in expressive language functions, and more behavioral problems than did the control children. Two children with HLHS (9%) had mental development indexes below 50, indicating significantly delayed performance. The children with UVH differed from the control children with respect to their lower mean mental development index. These findings suggest that at the age of 30 months, neurodevelopmental deficits are especially prevalent in children with HLHS. Thus, early developmental screening, intervention, and neuropsychological follow-up until school age is recommended particularly for the children with HLHS. AD - Division of Child Neurology, Helsinki University Central Hospital, Helsinki, Finland. riina.puosi@hus.fi AN - 22014005 AU - Puosi, R. AU - Korkman, M. AU - Sarajuuri, A. AU - Jokinen, E. AU - Mildh, L. AU - Mattila, I. AU - Lönnqvist, T. DA - Nov DB - PubMed DO - 10.1017/s135561771100110x DP - NLM ET - 2011/10/22 IS - 6 KW - Chi-Square Distribution Child Behavior Disorders/*etiology Child, Preschool Cognition Disorders/*etiology Developmental Disabilities/*complications Female Heart Defects, Congenital/*complications/*pathology Heart Ventricles/*physiopathology Humans Infant Language Development Disorders/diagnosis/etiology Male Neurologic Examination Neuropsychological Tests Severity of Illness Index LA - eng N1 - 1469-7661 Puosi, Riina Korkman, Marit Sarajuuri, Anne Jokinen, Eero Mildh, Leena Mattila, Ilkka Lönnqvist, Tuula Journal Article Research Support, Non-U.S. Gov't England J Int Neuropsychol Soc. 2011 Nov;17(6):1094-103. doi: 10.1017/S135561771100110X. Epub 2011 Oct 13. PY - 2011 SN - 1355-6177 SP - 1094-103 ST - Neurocognitive development and behavioral outcome of 2-year-old children with univentricular heart T2 - J Int Neuropsychol Soc TI - Neurocognitive development and behavioral outcome of 2-year-old children with univentricular heart VL - 17 ID - 173 ER - TY - JOUR AB - OBJECTIVES: To assess the long-term neuropsychological and behavioural profile of school-aged children who were treated for univentricular heart (UVH) conditions or biventricular heart defect (BiVH) in infancy in a cross-sectional study design. METHODS: Sixty-three patients, 17 UVH (13 males, 4 females) and 46 BiVH (19 males, 27 females), were assessed at a mean age of 9.1 years (2.2 years) with an intelligence and neuropsychological test battery. Results were compared between subgroups (UVH, BiVH and a healthy control group). Associations between cognitive outcome, medical and socio-demographic factors were explored. Parents completed the Child Behavior Checklist (CBCL). RESULTS: Mean intelligence and neuropsychological scores were found within normal ranges for all diagnostic groups. Significant differences between UVH patients and controls emerged on auditory sustained and alternating attention, fine motor skills, visuospatial information processing, and to a lesser extent, memory performance. Parents of UVH patients reported more externalizing problems and school problems. Patient groups did not differ on neuropsychological outcome measures, nor on behavioural problems as rated by parents. CONCLUSIONS: After Fontan completion, patients at school age display intelligence scores within normal population-based ranges. However, they were found at risk for subtle shortcomings in attention, fine motor skills, visuospatial information processing and externalizing behaviour problems. Considerations pertaining to neurobehavioural outcome in school-aged children are discussed. AD - Department of Experimental Psychology, Ghent University, Ghent, Belgium iemke.sarrechia@gmail.com. Department of Neurology, Head, Neck and Nerve System, Ghent University Hospital, Ghent, Belgium. Department of Pediatrics, Ghent University, Ghent, Belgium. Department of Cardiac Surgery, Congenital Cardiac Surgery, Ghent University Hospital, Ghent, Belgium. Pediatric and Congenital Cardiology, University Hospital Gasthuisberg, Leuven, Belgium. Pediatric and Congenital Cardiothoracic Surgery, University Hospital Gasthuisberg, Leuven, Belgium. Department of Experimental Psychology, Ghent University, Ghent, Belgium. AN - 25694470 AU - Sarrechia, I. AU - Miatton, M. AU - De Wolf, D. AU - François, K. AU - Gewillig, M. AU - Meyns, B. AU - Vingerhoets, G. DA - Jan DB - PubMed DO - 10.1093/ejcts/ezv029 DP - NLM ET - 2015/02/20 IS - 1 KW - Case-Control Studies Child Child Behavior Disorders/diagnosis/*etiology Cross-Sectional Studies Developmental Disabilities/diagnosis/*etiology Female Heart Defects, Congenital/complications/psychology/*surgery Humans Intelligence Tests Male Neuropsychological Tests *Postoperative Complications/diagnosis Retrospective Studies Congenital heart defect Hypoplastic left heart Neurodevelopmental outcome Neuropsychology Tricuspid atresia LA - eng N1 - 1873-734x Sarrechia, Iemke Miatton, Marijke De Wolf, Daniël François, Katrien Gewillig, Marc Meyns, Bart Vingerhoets, Guy Journal Article Research Support, Non-U.S. Gov't Germany Eur J Cardiothorac Surg. 2016 Jan;49(1):167-74. doi: 10.1093/ejcts/ezv029. Epub 2015 Feb 17. PY - 2016 SN - 1010-7940 SP - 167-74 ST - Neurocognitive development and behaviour in school-aged children after surgery for univentricular or biventricular congenital heart disease T2 - Eur J Cardiothorac Surg TI - Neurocognitive development and behaviour in school-aged children after surgery for univentricular or biventricular congenital heart disease VL - 49 ID - 54 ER - TY - JOUR AB - OBJECTIVE: Adults with congenital heart disease (CHD) are at increased risk of psychological disorders and cognitive deficiencies due to structural/acquired neurological abnormalities and neurodevelopmental disorders as children. However, limited information is known about the neuropsychological functioning of adults with CHD. This study screened neuropsychological abilities and explored group differences related to cardiac disease severity and neurological risk factors in adults with CHD. DESIGN: Participants completed brief neuropsychological testing. Information about neurobehavioral and psychological symptoms, employment, education, and disability were also collected from the patient and a family member. RESULTS: Forty-eight participants with adult CHD completed neuropsychological testing. Visuospatial skills and working memory were worse than expected compared to the typical population. Frequency of neurological comorbidities (e.g., stroke, seizures) was higher in those with more severe heart disease (e.g., single ventricle or cyanotic disease), and executive functioning was weaker in those with neurological comorbidities. Those with more severe heart disease were more likely to be unemployed and to receive disability benefits, but educational attainment did not differ. Those who received disability performed worse on tasks of executive functioning. CONCLUSIONS: Findings suggest concerns about neuropsychological functioning that need to be more comprehensively assessed in adults with CHD. Understanding the cognitive limitations of this aging population can help guide access to resources, transition of care, and medical care engagement, thus improving quality of care and quality of life. AD - Department of Neuropsychology, Children's Healthcare of Atlanta, Atlanta, Georgia, USA. Department of Rehabilitation Medicine, Emory University, Atlanta, Georgia, USA. Behavioral Health, Southeast Permanente Medical Group, Tucker, Georgia, USA. Department of Internal Medicine, Division of Cardiology, Emory University, Atlanta, Georgia, USA. AN - 27957813 AU - Ilardi, D. AU - Ono, K. E. AU - McCartney, R. AU - Book, W. AU - Stringer, A. Y. DA - Mar DB - PubMed DO - 10.1111/chd.12434 DP - NLM ET - 2016/12/14 IS - 2 KW - Adolescent Adult *Cognition Cognition Disorders/diagnosis/*etiology/psychology Comorbidity Disability Evaluation Executive Function Female Heart Defects, Congenital/*complications/diagnosis/psychology Humans Insurance, Disability Male Memory, Short-Term Middle Aged Neuropsychological Tests Risk Factors Space Perception Unemployment Visual Perception Young Adult adult congenital heart disease adult transition cognitive functioning executive functioning neurodevelopmental outcomes neuropsychological outcomes LA - eng N1 - 1747-0803 Ilardi, Dawn Ono, Kim E McCartney, Rebecca Book, Wendy Stringer, Anthony Y Journal Article United States Congenit Heart Dis. 2017 Mar;12(2):166-173. doi: 10.1111/chd.12434. Epub 2016 Dec 13. PY - 2017 SN - 1747-079x SP - 166-173 ST - Neurocognitive functioning in adults with congenital heart disease T2 - Congenit Heart Dis TI - Neurocognitive functioning in adults with congenital heart disease VL - 12 ID - 176 ER - TY - JOUR AD - Division of Pediatric Cardiology, University of Michigan Congenital Heart Center, 1500 East Medical Center Drive, Ann Arbor, MI 48109, USA. cgoldberg@umich.edu AN - 17978848 AU - Goldberg, C. DA - Nov-Dec DB - PubMed DO - 10.1007/s00246-007-9004-3 DP - NLM ET - 2007/11/06 IS - 6 KW - Cardiopulmonary Bypass/adverse effects Child Child, Preschool Circulatory Arrest, Deep Hypothermia Induced/adverse effects Cognition Disorders/*diagnosis/*etiology/surgery Developmental Disabilities/*etiology *Fontan Procedure Heart Defects, Congenital/*complications Heart Ventricles/*abnormalities Humans Intraoperative Care Postoperative Care Treatment Outcome LA - eng N1 - Goldberg, C Journal Article Review United States Pediatr Cardiol. 2007 Nov-Dec;28(6):443-7. doi: 10.1007/s00246-007-9004-3. PY - 2007 SN - 0172-0643 (Print) 0172-0643 SP - 443-7 ST - Neurocognitive outcomes for children with functional single ventricle malformations T2 - Pediatr Cardiol TI - Neurocognitive outcomes for children with functional single ventricle malformations VL - 28 ID - 433 ER - TY - JOUR AB - The aim of this review is to describe the current state of knowledge related to neurodevelopmental outcomes and quality of life for children with hypoplastic left heart syndrome and to explore future questions to be answered for this group of children. AD - Pediatric Cardiology, C. S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI 48109, USA. cgoldber@umich.edu AN - 22152534 AU - Goldberg, C. S. AU - Mussatto, K. AU - Licht, D. AU - Wernovsky, G. C2 - PMC3849043 C6 - NIHMS421677 DA - Dec DB - PubMed DO - 10.1017/s104795111100165x DP - NLM ET - 2011/12/14 IS - 0 2 KW - Child *Child Behavior *Child Development Follow-Up Studies Humans Hypoplastic Left Heart Syndrome/*psychology Nervous System/*growth & development *Quality of Life LA - eng N1 - 1467-1107 Goldberg, Caren S Mussatto, Kathleen Licht, Daniel Wernovsky, Gil R01 NS072338/NS/NINDS NIH HHS/United States Journal Article Review Cardiol Young. 2011 Dec;21 Suppl 2(0 2):88-92. doi: 10.1017/S104795111100165X. PY - 2011 SN - 1047-9511 (Print) 1047-9511 SP - 88-92 ST - Neurodevelopment and quality of life for children with hypoplastic left heart syndrome: current knowns and unknowns T2 - Cardiol Young TI - Neurodevelopment and quality of life for children with hypoplastic left heart syndrome: current knowns and unknowns VL - 21 Suppl 2 ID - 337 ER - TY - JOUR AB - OBJECTIVE: Despite improved survival and neurodevelopmental outcome, children with hypoplastic left heart syndrome and other forms of univentricular heart remain at increased risk for cognitive, motor, and other neurologic deficits. METHODS: We examined 27 children with hypoplastic left heart syndrome or other forms of univentricular heart at a median age of 5.70 years (range 4.99-7.51 years) and performed brain computed tomography or magnetic resonance imaging on 20. Possible risk factors were correlated with outcome. RESULTS: Mean full-scale IQ among patients with hypoplastic left heart syndrome was 86.7; that among patients with other forms of univentricular heart was 89.1, with both differing significantly from the expected population mean (P = .015 and P = .029, respectively). Cerebral palsy was diagnosed in 1 of 7 patients with hypoplastic left heart syndrome and 2 of 20 with other forms of univentricular heart. Brain computed tomography or magnetic resonance imaging revealed ischemic changes and infarcts or atrophy in 5 of 8 patients who had undergone the Norwood procedure and in 2 of 12 of those who had not (P = .062). Abnormal computed tomographic findings correlated significantly with lower full-scale IQ (P = .045) and verbal IQ (P = .02). In the multiple linear regression model, diuresis the third day after the primary operation and cardiopulmonary bypass time in the bidirectional Glenn operation correlated significantly with the primary outcome of full-scale IQ. CONCLUSION: In children with univentricular heart, intellectual and neurologic deficits are common. Perioperative and postoperative risk factors related to the primary phase and bidirectional Glenn operation contribute to these deficits. AD - Department of Neurology, Helsinki University Central Hospital, Helsinki, Finland. AN - 17532951 AU - Sarajuuri, A. AU - Jokinen, E. AU - Puosi, R. AU - Eronen, M. AU - Mildh, L. AU - Mattila, I. AU - Valanne, L. AU - Lönnqvist, T. DA - Jun DB - PubMed DO - 10.1016/j.jtcvs.2006.12.022 DP - NLM ET - 2007/05/30 IS - 6 KW - Child Child, Preschool Cognition Disorders/diagnosis/etiology Developmental Disabilities/diagnosis/*etiology Female Humans Hypoplastic Left Heart Syndrome/*surgery Intelligence Tests Linear Models Magnetic Resonance Imaging Male Neurologic Examination *Neuropsychological Tests Psychomotor Performance/*physiology Risk Factors Statistics, Nonparametric Tomography, X-Ray Computed LA - eng N1 - 1097-685x Sarajuuri, Anne Jokinen, Eero Puosi, Riina Eronen, Marianne Mildh, Leena Mattila, Ilkka Valanne, Leena Lönnqvist, Tuula Journal Article Research Support, Non-U.S. Gov't United States J Thorac Cardiovasc Surg. 2007 Jun;133(6):1524-32. doi: 10.1016/j.jtcvs.2006.12.022. PY - 2007 SN - 0022-5223 SP - 1524-32 ST - Neurodevelopmental and neuroradiologic outcomes in patients with univentricular heart aged 5 to 7 years: related risk factor analysis T2 - J Thorac Cardiovasc Surg TI - Neurodevelopmental and neuroradiologic outcomes in patients with univentricular heart aged 5 to 7 years: related risk factor analysis VL - 133 ID - 82 ER - TY - JOUR AB - Objective: Mortality rates for children with congenital heart disease (CHD) have significantly declined, resulting in a growing population with associated neurodevelopmental disabilities. American Heart Association guidelines recommend systematic developmental screening for children with CHD. The present study describes results of inpatient newborn neurodevelopmental assessment of infants after open heart surgery. Outcome measures: We evaluated the neurodevelopment of a convenience sample of high-risk infants following cardiac surgery but before hospital discharge using an adaptation of the Newborn Behavioral Observation. Factor analysis examined relationships among assessment items and consolidated them into domains of development. Results: We assessed 237 infants at a median of 11 days (interquartile range [IQR]: 7-19 days) after cardiac surgery and median corrected age of 21 days (IQR: 13-33 days). Autonomic regulation was minimally stressed or well organized in 14% of infants. Upper and lower muscle tone was appropriate in 33% and 35%, respectively. Appropriate response to social stimulation ranged between 7% and 12% depending on task, and state regulation was well organized in 14%. The vast majority (87%) required enhanced examiner facilitation for participation. Factor analyses of assessment items aligned into four domains of development (autonomic, motor, oral motor, and attention organization). Conclusion: At discharge, postoperative infants with CHD had impairments in autonomic, motor, attention, and state regulation following cardiac surgery. Findings highlight the challenges faced by children with CHD relative to healthy peers, suggesting that neurodevelopmental follow-up and intervention should begin early in infancy. AD - S.C. Butler, Department of Psychiatry, Boston Children’s Hospital, Boston, MA, United States AU - Butler, S. C. AU - Sadhwani, A. AU - Stopp, C. AU - Singer, J. AU - Wypij, D. AU - Dunbar-Masterson, C. AU - Ware, J. AU - Newburger, J. W. DB - Embase Medline DO - 10.1111/chd.12686 IS - 2 KW - aortic coarctation article attention clinical assessment tool cohort analysis congenital heart disease controlled study cross-sectional study facilitation factor analysis Fallot tetralogy female gestational age great vessels transposition heart catheterization heart single ventricle heart ventricle septum defect high risk infant hospital discharge hospital patient human hypoplastic left heart syndrome infant major clinical study male motor performance muscle tone newborn Newborn Behavioral Observation open heart surgery postoperative period priority journal regulatory mechanism seizure stimulus response LA - English M3 - Article N1 - L624408096 2018-10-23 2019-04-17 PY - 2019 SN - 1747-0803 1747-079X SP - 236-245 ST - Neurodevelopmental assessment of infants with congenital heart disease in the early postoperative period T2 - Congenital Heart Disease TI - Neurodevelopmental assessment of infants with congenital heart disease in the early postoperative period UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L624408096 http://dx.doi.org/10.1111/chd.12686 VL - 14 ID - 624 ER - TY - JOUR AB - BACKGROUND: Despite increasing survival, patients with hypoplastic left heart syndrome (HLHS) and other forms of functionally univentricular heart defects (UVHs) remain at increased risk of long-term neurodevelopmental deficits. METHODS: A nationwide sample of 23 patients with HLHS, 13 with UVH, and 40 controls were followed prospectively until the age of 5 years, when neurologic, neuropsychological, and motor examinations and brain MRI were performed. RESULTS: The median full-scale IQ was significantly lower in patients with HLHS (97, P < .001) and patients with UVH (112, P = .024) compared with controls (121). Major neurodevelopmental impairment was found in 26% of the patients with HLHS and 23% of those with UVH, and minor neurologic dysfunction was found in 43% and 46%, respectively. MRI revealed abnormalities, mostly ischemic changes of different degrees, in 82% of the patients with HLHS and in 56% of those with UVH. Prominent changes were significantly associated with neurodevelopmental findings and parental reports of adaptive behavior. In linear regression, significant risk factors for a worse outcome were a history of clinical seizures in connection with the primary operation, a lower diameter of the neonatal ascending aorta, and several pre-, peri-, and postoperative factors related to the primary and bidirectional Glenn operations. CONCLUSIONS: Although median cognitive performance was within the normal range, neurodevelopmental and brain MRI abnormalities were found in the majority of the patients with UVH, and especially in those with HLHS, at preschool age. Both a narrowed ascending aorta and operation-related factors contributed to these findings. AD - Divisions of Child Neurology, Helsinki University Central Hospital and University of Helsinki, Helsinki, Finland. anne.sarajuuri@hus.fi AN - 23166336 AU - Sarajuuri, A. AU - Jokinen, E. AU - Mildh, L. AU - Tujulin, A. M. AU - Mattila, I. AU - Valanne, L. AU - Lönnqvist, T. DA - Dec DB - PubMed DO - 10.1542/peds.2012-0486 DP - NLM ET - 2012/11/21 IS - 6 KW - Adaptation, Psychological Aorta, Thoracic/surgery Brain/pathology Brain Ischemia/diagnosis/epidemiology Case-Control Studies Child, Preschool Cognition Disorders/diagnosis/epidemiology Cross-Sectional Studies Developmental Disabilities/*diagnosis/epidemiology Electroencephalography Epilepsy/diagnosis/epidemiology Female Follow-Up Studies Fontan Procedure Heart Arrest, Induced Heart Bypass, Right Heart Ventricles/*abnormalities/surgery Humans Hypoplastic Left Heart Syndrome/*diagnosis/epidemiology/surgery Longitudinal Studies Magnetic Resonance Imaging Male Motor Skills Disorders/diagnosis/epidemiology Neuropsychological Tests/*statistics & numerical data Norwood Procedures Prospective Studies Psychometrics Psychomotor Disorders/diagnosis/epidemiology Risk Factors LA - eng N1 - 1098-4275 Sarajuuri, Anne Jokinen, Eero Mildh, Leena Tujulin, Anna-Mari Mattila, Ilkka Valanne, Leena Lönnqvist, Tuula Journal Article Research Support, Non-U.S. Gov't United States Pediatrics. 2012 Dec;130(6):e1636-46. doi: 10.1542/peds.2012-0486. Epub 2012 Nov 19. PY - 2012 SN - 0031-4005 SP - e1636-46 ST - Neurodevelopmental burden at age 5 years in patients with univentricular heart T2 - Pediatrics TI - Neurodevelopmental burden at age 5 years in patients with univentricular heart VL - 130 ID - 283 ER - TY - JOUR AB - BACKGROUND: Although single ventricle physiology and cyanosis are known risk factors for neurodevelopmental delay (NDD), the impacts of isolated coarctation (Iso CoA) repair or arch reconstruction (AR) are less understood. METHODS: The Vineland Adaptive Behavior Scales, third edition, prospectively evaluated children without a genetic syndrome. An overall composite score, normalized to age and sex, was generated from individual domain scores. NDD was defined as a composite or domain score at least 1 SD less than the established mean. Iso CoA was repaired using a left thoracotomy, whereas AR was performed using a sternotomy and cardiopulmonary bypass. Children with a structurally normal heart and without previous surgery were used as control patients. RESULTS: Of 60 children, 50 required neonatal repair (12 for Iso CoA, 38 for AR), and 10 were control patients. From the entire cohort of children who required neonatal coarctation repair (Iso CoA + AR) composite (93.9 ± 15.9 vs 105.0 ± 7.4; P = .004) and all domain scores were significantly lower than control patients. NDD was present in 25 of 50 children after repair and in 0 of 10 control patients (P = .003). Similarly, the prevalence of NDD was significantly greater after Iso CoA repair (58.3% vs 0%; P = .005) and AR (47.3% vs 0%; P = .007) than in the control population, but no significant difference was found between the Iso CoA and AR groups (P = .74). CONCLUSIONS: In this small cohort, half of the neonates who required either Iso CoA repair or AR exhibit NDD at an intermediate-term follow-up. AD - Department of Surgery, University of Rochester Medical Center, Rochester, New York. Department of Neurology, University of Rochester Medical Center, Rochester, New York. Department of Pediatrics, University of Rochester Medical Center, Rochester, New York. Department of Surgery, University of Rochester Medical Center, Rochester, New York. Electronic address: george_alfieris@urmc.rochester.edu. AN - 31207245 AU - Simon, B. V. AU - Swartz, M. F. AU - Orie, J. M. AU - Adams, H. R. AU - Seltzer, L. E. AU - Angona, R. E. AU - Atallah-Yunes, N. H. AU - Alfieris, G. M. DA - Nov DB - PubMed DO - 10.1016/j.athoracsur.2019.04.088 DP - NLM ET - 2019/06/18 IS - 5 KW - Aorta, Thoracic/*surgery Aortic Coarctation/*surgery Arterial Occlusive Diseases/*surgery Developmental Disabilities/*epidemiology Female Humans Infant, Newborn Male Postoperative Complications/*epidemiology Prospective Studies Vascular Surgical Procedures LA - eng N1 - 1552-6259 Simon, Bartholomew V Swartz, Michael F Orie, Jennifer M Adams, Heather R Seltzer, Laurie E Angona, Ron E Atallah-Yunes, Nader H Alfieris, George M Journal Article Netherlands Ann Thorac Surg. 2019 Nov;108(5):1416-1422. doi: 10.1016/j.athoracsur.2019.04.088. Epub 2019 Jun 14. PY - 2019 SN - 0003-4975 SP - 1416-1422 ST - Neurodevelopmental Delay After the Neonatal Repair of Coarctation and Arch Obstruction T2 - Ann Thorac Surg TI - Neurodevelopmental Delay After the Neonatal Repair of Coarctation and Arch Obstruction VL - 108 ID - 539 ER - TY - JOUR AB - As survival after cardiac surgery continues to improve, an increasing number of patients with hypoplastic left heart syndrome are reaching school age and beyond, with growing recognition of the wide range of neurodevelopmental challenges many survivors face. Improvements in fetal detection rates, coupled with advances in fetal ultrasound and MRI imaging, are contributing to a growing body of evidence that abnormal brain architecture is in fact present before birth in hypoplastic left heart syndrome patients, rather than being solely attributable to postnatal factors. We present an overview of the contemporary data on neurodevelopmental outcomes in hypoplastic left heart syndrome, focussing on imaging techniques that are providing greater insight into the nature of disruptions to the fetal circulation, alterations in cerebral blood flow and substrate delivery, disordered brain development, and an increased potential for neurological injury. These susceptibilities are present before any intervention, and are almost certainly substantial contributors to adverse neurodevelopmental outcomes in later childhood. The task now is to determine which subgroups of patients with hypoplastic left heart syndrome are at particular risk of poor neurodevelopmental outcomes and how that risk might be modified. This will allow for more comprehensive counselling for carers, better-informed decision making before birth, and earlier, more tailored provision of neuroprotective strategies and developmental support in the postnatal period. AD - 1Paediatric Cardiology Department,Evelina Children's Hospital,London,United Kingdom. 2Division of Imaging Sciences and Biomedical Engineering,King's College London,London,United Kingdom. AN - 27821206 AU - Lloyd, D. F. AU - Rutherford, M. A. AU - Simpson, J. M. AU - Razavi, R. DA - Mar DB - PubMed DO - 10.1017/s1047951116001645 DP - NLM ET - 2016/11/09 IS - 2 KW - *Cardiac Surgical Procedures Decision Making Developmental Disabilities/*etiology Female Humans *Hypoplastic Left Heart Syndrome/diagnosis/embryology/surgery Pregnancy Ultrasonography, Prenatal Chd Fetal cardiology hypoplastic left heart syndrome neurodevelopment paediatric cardiology LA - eng N1 - 1467-1107 Lloyd, David F A Rutherford, Mary A Simpson, John M Razavi, Reza MC_U120088465/Medical Research Council/United Kingdom Journal Article England Cardiol Young. 2017 Mar;27(2):217-223. doi: 10.1017/S1047951116001645. Epub 2016 Nov 8. PY - 2017 SN - 1047-9511 SP - 217-223 ST - The neurodevelopmental implications of hypoplastic left heart syndrome in the fetus T2 - Cardiol Young TI - The neurodevelopmental implications of hypoplastic left heart syndrome in the fetus VL - 27 ID - 69 ER - TY - JOUR AB - OBJECTIVE: Increased survival in children with critical congenital heart disease (CHD) has raised interest in the neurodevelopmental sequelae of these lesions. This investigation is part of an institutional effort to examine the neurodevelopment of 5-year-old children following repair or palliation of CHD. METHODS: We performed a battery of neuropsychological tests on a sample of 243 children between 1998 and 2001. RESULTS: In the sample as a whole, mean full-scale (FSIQ), verbal (VIQ), and performance (PIQ) IQ scores were in the normal range (96.8+/-15.9, 97.8+/-14.6, and 96.3+/-17.1, respectively). Anatomic, demographic, and perioperative factors were assessed for impact on neurodevelopment. In multiple regression analysis, lower socioeconomic status (SES) and the diagnosis of velocardiofacial syndrome (VCFS) predicted a lower FSIQ (P=0.01, and P=0.001, respectively). A single ventricle diagnosis (P=0.06), longer postoperative ICU stay (P=0.08), and cumulative duration of hypothermic circulatory arrest (HCA) (P=0.09) approached significance as predictors of lower FSIQ. CONCLUSION: Children with CHD, on the whole, appear to be performing within the average range in terms of intellectual abilities. Lower SES and VCFS are associated with lower IQ scores. Trends toward worse outcomes were observed in single ventricle patients, biventricular patients with longer postrepair ICU stays, and patients subjected to longer periods of HCA. AD - Department of Cardiovascular Surgery, Children's Hospital, Boston, Mass 02115, USA. forbess@cardio.tch.harvard.edu AN - 12354716 AU - Forbess, J. M. AU - Visconti, K. J. AU - Hancock-Friesen, C. AU - Howe, R. C. AU - Bellinger, D. C. AU - Jonas, R. A. DA - Sep 24 DB - PubMed DP - NLM ET - 2002/10/02 IS - 12 Suppl 1 KW - Child Developmental Disabilities/*diagnosis Female Follow-Up Studies Heart Defects, Congenital/diagnosis/*surgery Heart Ventricles/surgery Humans Intelligence Tests Learning Male Memory Psychomotor Performance Registries Risk Factors Socioeconomic Factors Treatment Outcome LA - eng N1 - 1524-4539 Forbess, Joseph M Visconti, Karen J Hancock-Friesen, Camille Howe, Robert C Bellinger, David C Jonas, Richard A Journal Article United States Circulation. 2002 Sep 24;106(12 Suppl 1):I95-102. PY - 2002 SN - 0009-7322 SP - I95-102 ST - Neurodevelopmental outcome after congenital heart surgery: results from an institutional registry T2 - Circulation TI - Neurodevelopmental outcome after congenital heart surgery: results from an institutional registry VL - 106 ID - 446 ER - TY - JOUR AN - 11060522 AU - Freedom, R. M. DA - Nov DB - PubMed DO - 10.1067/mpd.2000.110444 DP - NLM ET - 2000/11/04 IS - 5 KW - *Child Development Developmental Disabilities *Fontan Procedure Humans Hypoplastic Left Heart Syndrome/*surgery Infant *Psychomotor Performance Treatment Outcome LA - eng N1 - Freedom, R M Comment Editorial United States J Pediatr. 2000 Nov;137(5):602-4. doi: 10.1067/mpd.2000.110444. PY - 2000 SN - 0022-3476 (Print) 0022-3476 SP - 602-4 ST - Neurodevelopmental outcome after the fontan procedure in children with the hypoplastic left heart syndrome and other forms of single ventricle pathology: challenges unresolved T2 - J Pediatr TI - Neurodevelopmental outcome after the fontan procedure in children with the hypoplastic left heart syndrome and other forms of single ventricle pathology: challenges unresolved VL - 137 ID - 467 ER - TY - JOUR AB - Neurodevelopmental impairment and impaired quality of life constitute a major source of morbidity among children with complex congenital heart disease, in particular for single-ventricle (SV) morphologies. Risk factors and quality of life determining clinical and neurodevelopmental outcome at 2 years of age are examined. In a 2-center cohort study, 48 patients with SV morphology (26 hypoplastic left heart syndrome and 22 other types of univentricular heart defect) have been examined before Fontan procedure between 2010 and 2015. Patients were assessed with the Bayley Scales of Infant and Toddler Development, Third Version (Bayley-III), and the Preschool Children Quality of Life (TAPQOL) questionnaire. A total of 44 patients underwent hybrid procedure (n = 25), Norwood procedure (n = 7), or shunt or banding procedure (n = 12) as first surgery before subsequent bidirectional cavopulmonary anastomosis (n = 48). Median cognitive, language, and motor composite scores on the Bayley-III were 100 (range 65-120), 97 (68-124), and 97 (55-124), respectively. The language composite score was significantly below the norm (P = 0.025). Risk factors for poorer neurodevelopmental outcome were prolonged mechanical ventilation, longer days of hospital stay, and more reinterventions (all P < 0.05). Parents reported a good quality of life for their children. Children undergoing Fontan procedure show a favorable development and good quality of life. More complicated postoperative course and reinterventions constitute risk factors for impaired neurodevelopment. Improving postoperative management and implementing routine follow-up assessments aremeasures to further improve the neurodevelopmental outcome of this high-risk patient population. AD - Pediatric Heart Center, University Hospital, Giessen, Germany. Electronic address: bettina.reich@paediat.med.uni-giessen.de. Pediatric Cardiology, University Children's Hospital, Zurich, Switzerland; Children's Research Center, University Children's Hospital, Zurich, Switzerland. Children's Research Center, University Children's Hospital, Zurich, Switzerland; Center for MR Research, University Children's Hospital, Zurich, Switzerland. Children's Research Center, University Children's Hospital, Zurich, Switzerland; Child Development Center, University Children's Hospital, Zurich, Switzerland. Child Development Center, SPZ Frankfurt Mitte, Frankfurt/Main, Germany. Pediatric Neurology, University Hospital, Giessen, Germany. Pediatric Heart Center, University Hospital, Giessen, Germany. AN - 29104017 AU - Reich, B. AU - Heye, K. AU - Tuura, R. AU - Beck, I. AU - Wetterling, K. AU - Hahn, A. AU - Hofmann, K. AU - Schranz, D. AU - Akintürk, H. AU - Latal, B. AU - Knirsch, W. DA - Dec 5 DB - PubMed DO - 10.1053/j.semtcvs.2017.09.014 DP - NLM ET - 2017/11/07 KW - congenital heart disease neurodevelopmental outcome risk factors LA - eng N1 - 1532-9488 Reich, Bettina Heye, Kristina Tuura, Ruth Beck, Ingrid Wetterling, Kristina Hahn, Andreas Hofmann, Karoline Schranz, Dietmar Akintürk, Hakan Latal, Beatrice Knirsch, Walter Journal Article United States Semin Thorac Cardiovasc Surg. 2017 Dec 5:S1043-0679(17)30288-5. doi: 10.1053/j.semtcvs.2017.09.014. PY - 2017 SN - 1043-0679 ST - Neurodevelopmental Outcome and Health-related Quality of Life in Children With Single-ventricle Heart Disease Before Fontan Procedure T2 - Semin Thorac Cardiovasc Surg TI - Neurodevelopmental Outcome and Health-related Quality of Life in Children With Single-ventricle Heart Disease Before Fontan Procedure ID - 97 ER - TY - JOUR AB - OBJECTIVES: The purposes of this study are to describe the quality of life and cognitive function in school-aged children who have undergone staged palliation for hypoplastic left heart syndrome (HLHS), and to identify factors that are predictive of neurodevelopmental outcome in this population. METHODS: School-aged survivors with HLHS who had undergone palliative surgery at our institution were identified and mailed a questionnaire to assess subjectively quality of life, school performance, and incidence of medical complications. A subgroup of local patients underwent standardized testing of cognitive function and neurologic examination. These patients were compared with the larger (remote) group of questionnaire respondents to determine whether results may be generalizable to the entire HLHS population. Potential predictors of neurologic and cognitive outcome were tested for their association with test scores using multivariate regression analysis. RESULTS: Questionnaire results were obtained from 115 of 138 eligible children (83%; mean age: 9.0 +/- 2.0 years). Standardized testing was performed in 28 of 34 (82%) eligible local patients (mean age: 8.6 +/- 2.1 years). The majority of parents or guardians described their child's health as good (34%) or excellent (45%) and their academic performance as average (42%) or above average (42%). One third of the children, however, were receiving some form of special education. Chronic medication usage was common (64%); the incidence of medical complications was comparable to that previously reported in children with Fontan physiology. Cognitive testing of the local group demonstrated a median full scale IQ of 86 (range: 50-116). Mental retardation (IQ: <70) was noted in 18% of patients. In multivariate analysis, only the occurrence of preoperative seizures predicted lower full scale IQ. CONCLUSIONS: Although the majority of school-aged children with HLHS had IQ scores within the normal range, mean performance for this historical cohort of survivors was lower than that in the general population. AD - Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA. AN - 10790466 AU - Mahle, W. T. AU - Clancy, R. R. AU - Moss, E. M. AU - Gerdes, M. AU - Jobes, D. R. AU - Wernovsky, G. DA - May DB - PubMed DO - 10.1542/peds.105.5.1082 DP - NLM ET - 2000/05/03 IS - 5 KW - Adolescent Child Cross-Sectional Studies Female Humans Hypoplastic Left Heart Syndrome/*physiopathology/*psychology/surgery Intelligence Tests *Life Style Male Nervous System/*growth & development *Psychomotor Performance Surveys and Questionnaires LA - eng N1 - 1098-4275 Mahle, W T Clancy, R R Moss, E M Gerdes, M Jobes, D R Wernovsky, G Journal Article United States Pediatrics. 2000 May;105(5):1082-9. doi: 10.1542/peds.105.5.1082. PY - 2000 SN - 0031-4005 SP - 1082-9 ST - Neurodevelopmental outcome and lifestyle assessment in school-aged and adolescent children with hypoplastic left heart syndrome T2 - Pediatrics TI - Neurodevelopmental outcome and lifestyle assessment in school-aged and adolescent children with hypoplastic left heart syndrome VL - 105 ID - 335 ER - TY - JOUR AB - OBJECTIVE: To characterize neurodevelopmental outcomes after fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome and determine the risk factors for adverse neurodevelopment. STUDY DESIGN: Questionnaires were mailed to families of children who underwent fetal aortic valvuloplasty from 2000 to 2012, and medical records were reviewed retrospectively. The primary outcome was the General Adaptive Composite score of the Adaptive Behavior Assessment System Questionnaire-Second Edition. Other questionnaires included the Behavior Assessment System for Children, Behavior Rating Inventory of Executive Function, Ages and Stages, and Pediatric Quality of Life Inventory. RESULTS: Among 69 eligible subjects, 52 (75%) completed questionnaires at median age of 5.5 (range 1.3-12) years; 30 (58%) had biventricular status circulation. The General Adaptive Composite mean score (92 ± 17) was lower than population norms (P < .001) and similar to published reports in patients with hypoplastic left heart syndrome without fetal intervention; scores in the single ventricular versus biventricular group were 97 ± 19 vs 89 ± 14, respectively (P = .10). On multivariable analysis, independent predictors of a lower General Adaptive Composite score were total hospital duration of stay in the first year of life (P = .001) and, when forced into the model, biventricular status (P = .02). For all other neurodevelopmental questionnaires (Behavior Assessment System for Children, Behavior Rating Inventory of Executive Function, Ages and Stages, Pediatric Quality of Life Inventory), most subscale scores for patients with biventricular and single ventricular status were similar. CONCLUSION: Children who underwent fetal aortic valvuloplasty have neurodevelopmental delay, similar to patients with hypoplastic left heart syndrome without fetal intervention. Achievement of biventricular circulation was not associated with better outcomes. We infer that innate patient factors and morbidity during infancy have the greatest effect on neurodevelopmental outcomes. AD - Department of Cardiology, Boston Children's Hospital, Boston, MA; Department of Pediatrics, Harvard Medical School, Boston, MA. Department of Psychiatry, Boston Children's Hospital, Boston, MA; Department of Psychiatry, Harvard Medical School, Boston, MA. Department of Cardiology, Boston Children's Hospital, Boston, MA. Department of Psychiatry, Harvard Medical School, Boston, MA; Developmental Medicine Center, Boston Children's Hospital, Boston, MA. Department of Obstetrics and Gynecology, Brigham and Women's Hospital, Boston, MA; Department of Obstetrics and Gynecology, Harvard Medical School, Boston, MA. AN - 28233547 AU - Laraja, K. AU - Sadhwani, A. AU - Tworetzky, W. AU - Marshall, A. C. AU - Gauvreau, K. AU - Freud, L. AU - Hass, C. AU - Dunbar-Masterson, C. AU - Ware, J. AU - Lafranchi, T. AU - Wilkins-Haug, L. AU - Newburger, J. W. C2 - PMC6343658 C6 - NIHMS1517999 DA - May DB - PubMed DO - 10.1016/j.jpeds.2017.01.034 DP - NLM ET - 2017/02/25 KW - Aortic Valve Stenosis/*complications/*surgery Balloon Valvuloplasty Cardiac Surgical Procedures/methods Child Child, Preschool Cohort Studies Female Fetus/*surgery Humans Hypoplastic Left Heart Syndrome/*complications Infant Neurodevelopmental Disorders/*epidemiology Postoperative Complications/*epidemiology Pregnancy Retrospective Studies Risk Factors *child development *congenital heart disease *fetal cardiac intervention *neurodevelopmental outcomes LA - eng N1 - 1097-6833 Laraja, Kristin Sadhwani, Anjali Tworetzky, Wayne Marshall, Audrey C Gauvreau, Kimberlee Freud, Lindsay Hass, Cara Dunbar-Masterson, Carolyn Ware, Janice Lafranchi, Terra Wilkins-Haug, Louise Newburger, Jane W P30 HD018655/HD/NICHD NIH HHS/United States Journal Article Research Support, Non-U.S. Gov't J Pediatr. 2017 May;184:130-136.e4. doi: 10.1016/j.jpeds.2017.01.034. Epub 2017 Feb 21. PY - 2017 SN - 0022-3476 (Print) 0022-3476 SP - 130-136.e4 ST - Neurodevelopmental Outcome in Children after Fetal Cardiac Intervention for Aortic Stenosis with Evolving Hypoplastic Left Heart Syndrome T2 - J Pediatr TI - Neurodevelopmental Outcome in Children after Fetal Cardiac Intervention for Aortic Stenosis with Evolving Hypoplastic Left Heart Syndrome VL - 184 ID - 115 ER - TY - JOUR AB - BACKGROUND: The purpose of the present study was to assess a broad range of neuropsychological outcome variables in children with functionally single ventricle hearts after a total cavopulmonary connection and to examine potential risk factors for impaired neurodevelopment. PATIENTS & METHOD: A total of 104 patients aged 2 to 20 years underwent follow-up standardized psychological testing, including measures of intelligence, motor function, visuospatial abilities, behavioural outcome and health-related quality of life. RESULTS: With a mean fluid intelligence score of 93.0 and a mean crystallized intelligence score of 92.3, patients scored significantly lower on tests of intelligence compared to the general population (p<0.001). Reduced motor function was found in 34% of the patients (p<0.001), impaired visuospatial abilities in 51%; parents reported significantly more behavioural problems (p<0.001). There was no difference in the self-reported quality of life compared to the general population. Risk factors for reduced fluid intelligence were deep hypothermic circulatory arrest times (p=0.03) and complications causing suboptimal brain perfusion prior to the total cavopulmonary connection (e. g. seizures; p=0.04). CONCLUSIONS: Patients with functionally single ventricle hearts palliated with a total cavopulmonary connection are at an increased risk of neurodevelopmental delays and behavioural disorders. Nevertheless, they adapt well in terms of quality of life. Early diagnostics and interventions are necessary when developmental delays are suspected. AD - German Paediatric Heart Centre Sankt Augustin, Paediatric Cardiology, Sankt Augustin, Germany. University of Mannheim, School of Social Sciences, Mannheim, Germany. Children's Hospital of Wisconsin, Cardiothoracic Surgery, Milwaukee, United States. AN - 29258158 AU - Vahsen, N. AU - Bröder, A. AU - Hraska, V. AU - Schneider, M. DA - Jan DB - PubMed DO - 10.1055/s-0043-120526 DP - NLM ET - 2017/12/20 IS - 1 KW - Adolescent Adult Child Child, Preschool Developmental Disabilities/diagnosis/*etiology Heart Defects, Congenital/*surgery Heart Ventricles Humans Postoperative Complications/diagnosis/*etiology Pulmonary Artery/*surgery *Quality of Life Treatment Outcome Young Adult LA - eng N1 - 1439-3824 Vahsen, Nicole Bröder, Arndt Hraska, Viktor Schneider, Martin Journal Article Germany Klin Padiatr. 2018 Jan;230(1):24-30. doi: 10.1055/s-0043-120526. Epub 2017 Dec 19. OP - Psychologisches Funktionsniveau bei Kindern und Jugendlichen mit univentrikulärer Zirkulation nach operativer Palliation. PY - 2018 SN - 0300-8630 SP - 24-30 ST - Neurodevelopmental Outcome in Children With Single Ventricle After Total Cavopulmonary Connection T2 - Klin Padiatr TI - Neurodevelopmental Outcome in Children With Single Ventricle After Total Cavopulmonary Connection VL - 230 ID - 56 ER - TY - JOUR AB - BACKGROUND: Little is known about the mid-term outcome and brain development in patients following the hybrid approach for hypoplastic left heart syndrome (HLHS). This study investigates neurodevelopmental outcome, quality of life (QoL) and brain MRI findings in HLHS preschoolers treated with the hybrid approach. METHODS: Twenty HLHS patients (60% males) have been examined after neonatal hybrid Stage I and comprehensive stage II operation at the Pediatric Heart Center Giessen, Germany, between 2012 and 2016. Patients were evaluated with the Bayley Scales of Infant and Toddler Development III (Bayley-III), neurological examination, the Preschool Children Quality of Life Questionnaire (TAPQOL) at age 26.5±3.6 months, and again at 39.7±3.9 months with the Pediatric Cardiac Quality of Life Inventory (PCQLI). Furthermore, brain volumetric measurements and conventional brain MRI findings (27.3±4.5 months) were analyzed and compared with six healthy controls (29.2±11.1 months, P=0.53). Children with verified genetic comorbidities were excluded. RESULTS: Mean cognitive, language, and motor composite scores on the Bayley-III were not different from healthy norms (100±15), and were 101±9.3 (P=0.48), 100±13 (P=0.93), and 98±11.7 (P=0.45), respectively. Status post stroke was the most common brain MRI abnormality, and was found in 3/19 (16%) patients, most common affecting the middle cerebral artery territory. In comparison to controls, total white matter volumes were reduced (P=0.014), and cerebrospinal fluid (CSF) volumes were increased (P=0.042) in patients. Overall health-related QoL in 2 to 3 years aged children HLHS was good, but inferior scores in the motor subscale were noted compared to healthy norms (P=0.007). However, at 3 to 4 years, parents reported comparable QoL for their children in the PCQLI to children with biventricular heart lesion. CONCLUSIONS: HLHS patients followed by hybrid approach without major complications show a favorable neurodevelopment at 2-3 years of age. Despite extensive health-related burden, the vast majority of Fontan preschoolers with HLHS showed a good health-related QoL. Nevertheless, comprehensive care and establishing routine follow-up examinations are important to recognize long-term challenges and further improve neurodevelopmental outcome of this high-risk patient population. AD - Pediatric Heart Center, University Hospital Giessen, Justus-Liebig-University, Giessen, Germany. Division of Neurology, Children's Hospital of Philadelphia, Philadelphia, PA, USA. Child Development Center, SPZ Frankfurt Mitte, Frankfurt/Main, Germany. Pediatric Neurology, University Hospital Giessen, Justus-Liebig-University, Giessen, Germany. Pediatric Heart Center Hesse, Johann-Wolfgang-Goethe University Hospital Frankfurt, Frankfurt/Main, Germany. AN - 31161076 AU - Reich, B. AU - Heye, K. N. AU - Wetterling, K. AU - Logeswaran, T. AU - Hahn, A. AU - Akintürk, H. AU - Jux, C. AU - Schranz, D. C2 - PMC6514277 DA - Apr DB - PubMed DO - 10.21037/tp.2019.04.05 DP - NLM ET - 2019/06/05 IS - 2 KW - Congenital heart disease (CHD) brain development hybrid procedure hypoplastic left heart syndrome (HLHS) neurodevelopmental outcome LA - eng N1 - 2224-4344 Reich, Bettina Heye, Kristina N Wetterling, Kristina Logeswaran, Thushiha Hahn, Andreas Akintürk, Hakan Jux, Christian Schranz, Dietmar Journal Article Transl Pediatr. 2019 Apr;8(2):94-106. doi: 10.21037/tp.2019.04.05. PY - 2019 SN - 2224-4344 (Print) 2224-4336 SP - 94-106 ST - Neurodevelopmental outcome in hypoplastic left heart syndrome after hybrid procedure T2 - Transl Pediatr TI - Neurodevelopmental outcome in hypoplastic left heart syndrome after hybrid procedure VL - 8 ID - 14 ER - TY - JOUR AB - OBJECTIVES: Patients with hypoplastic left heart syndrome are at risk for neurodevelopmental impairment. Hypoxic-ischemic brain injury during neonatal treatment might be a relevant cause. We evaluated the association between cerebral oxygenation in the perioperative course of the Norwood procedure and neurodevelopmental outcome. METHODS: Cerebral tissue oxygen saturation (ScO2) was obtained by near-infrared spectroscopy for 24 hours before and 48 hours after surgery in 43 patients. Full-scale, verbal, and performance IQ scores were evaluated with the Wechsler Preschool and Primary Scale of Intelligence at a median of 4.5 years (range, 3.5-6.8 years). Cognitive functions were assessed with the German Kognitiver Entwicklungstest für das Kindergartenalter (KET-KID). RESULTS: Mean IQ scores and KET-KID percentile ranks were in the lower-normal range (full-scale IQ, 94 ± 11; verbal IQ, 97 ± 13; performance IQ, 93 ± 9; KET-KID global, 42 ± 27; verbal, 48 ± 29; nonverbal, 37 ± 23). Scores were below average (full scale IQ <85 or KET-KID <16th percentile) in 12 cases. Mean preoperative ScO2 was lower in patients scoring below average (56.8% ± 7.1% vs 61.7% ± 5.8%; P = .028) and was correlated with full-scale IQ (r = 0.495; P = .001), verbal IQ (r = 0.524; P = .001), and performance IQ (r = 0.386; P = .012) scores, and with global (r = 0.360; P = .018) and verbal (r = 0.395, P = .009) KET-KID scores. A relationship between IQ or KET-KID scores and postoperative ScO2 was not found. Gestational age, head circumference z-score, age at surgery, and postoperative length of stay were associated with IQ and KET-KID scores. CONCLUSIONS: Neurodevelopmental outcome was in the lower-normal range. Along with innate patient factors, preoperative cerebral tissue oxygenation is likely an important determinant of cognitive development. AD - Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Kiel, Germany. Electronic address: jan.hansen@uk-sh.de. Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Kiel, Germany. Department of Anesthesiology and Intensive Care Medicine, University Hospital Schleswig-Holstein, Kiel, Germany. Department of Cardiovascular Surgery, University Hospital Schleswig-Holstein, Kiel, Germany. AN - 27085616 AU - Hansen, J. H. AU - Rotermann, I. AU - Logoteta, J. AU - Jung, O. AU - Dütschke, P. AU - Scheewe, J. AU - Kramer, H. H. DA - May DB - PubMed DO - 10.1016/j.jtcvs.2016.02.035 DP - NLM ET - 2016/04/18 IS - 5 KW - Area Under Curve Cerebrovascular Circulation Child Child, Preschool Cognition Disorders/epidemiology/etiology/physiopathology Cohort Studies Developmental Disabilities/epidemiology/*etiology/physiopathology Female Follow-Up Studies Humans Hypoplastic Left Heart Syndrome/complications/*surgery Hypoxia-Ischemia, Brain/etiology/*physiopathology/psychology Male Neuropsychological Tests Norwood Procedures/adverse effects/*methods Oxygen Consumption/*physiology Palliative Care/methods Perioperative Care/methods ROC Curve Retrospective Studies Risk Assessment Spectroscopy, Near-Infrared Survivors Time Factors Treatment Outcome hypoplastic left heart syndrome, developmental outcome, near-infrared spectroscopy LA - eng N1 - 1097-685x Hansen, Jan H Rotermann, Ina Logoteta, Jana Jung, Olaf Dütschke, Peter Scheewe, Jens Kramer, Hans-Heiner Journal Article United States J Thorac Cardiovasc Surg. 2016 May;151(5):1358-66. doi: 10.1016/j.jtcvs.2016.02.035. Epub 2016 Feb 24. PY - 2016 SN - 0022-5223 SP - 1358-66 ST - Neurodevelopmental outcome in hypoplastic left heart syndrome: Impact of perioperative cerebral tissue oxygenation of the Norwood procedure T2 - J Thorac Cardiovasc Surg TI - Neurodevelopmental outcome in hypoplastic left heart syndrome: Impact of perioperative cerebral tissue oxygenation of the Norwood procedure VL - 151 ID - 155 ER - TY - JOUR AB - INTRODUCTION: The purpose of this study was to describe the neurodevelopmental outcome of preschool survivors of Hypoplastic Left Heart Syndrome (HLHS) and Transposition of the Great Arteries (TGA) in the modern surgical era. METHODS: A cross-sectional design was used to evaluate 26 children (13 HLHS, 13 TGA), ages 3.5 to 6 years, at a Midwestern children's hospital. Measures included McCarthy Scales of Children's Abilities, Woodcock Johnson III Tests of Achievement, Developmental Test of Visual-Motor Integration, Receptive One-Word Vocabulary Test, Expressive One-Word Vocabulary Test, and Child Behavior Checklist. RESULTS: Although intelligence quotient scores fell within the average range for both groups (TGA=110.5 vs. HLHS=97), the difference between groups was clinically meaningful (effect size= .79). The HLHS group showed more problems than did the TGA group with visual-motor skills, expressive language, attention, and externalizing behavior. DISCUSSION: Results suggest that even in the modern surgical era, regular developmental screening for these patients is critical. Pediatric nurse practitioners play an important role in educating parents about the potential developmental risks to these children. AD - Medical College of Wisconsin, Department of Pediatrics, Milwaukee,Wisconsin, USA. AN - 17198894 AU - Brosig, C. L. AU - Mussatto, K. A. AU - Kuhn, E. M. AU - Tweddell, J. S. DA - Jan-Feb DB - PubMed DO - 10.1016/j.pedhc.2006.03.008 DP - NLM ET - 2007/01/03 IS - 1 KW - Analysis of Variance Child Child, Preschool Cross-Sectional Studies Developmental Disabilities/epidemiology/*etiology Female Humans Hypoplastic Left Heart Syndrome/*complications/surgery Male Mass Screening Neuropsychological Tests *Postoperative Complications/epidemiology Transposition of Great Vessels/*complications/surgery Wisconsin/epidemiology LA - eng N1 - Brosig, Cheryl L Mussatto, Kathleen A Kuhn, Evelyn M Tweddell, James S Journal Article Research Support, Non-U.S. Gov't United States J Pediatr Health Care. 2007 Jan-Feb;21(1):3-12. doi: 10.1016/j.pedhc.2006.03.008. PY - 2007 SN - 0891-5245 (Print) 0891-5245 SP - 3-12 ST - Neurodevelopmental outcome in preschool survivors of complex congenital heart disease: implications for clinical practice T2 - J Pediatr Health Care TI - Neurodevelopmental outcome in preschool survivors of complex congenital heart disease: implications for clinical practice VL - 21 ID - 438 ER - TY - JOUR AD - Division of Paediatric Cardiology, Paediatric Heart Centre, University Children's Hospital Zurich, Zurich, Switzerland. AN - 27535107 AU - Knirsch, W. AU - Liamlahi, R. AU - Dave, H. AU - Kretschmar, O. AU - Latal, B. C2 - PMC5183985 DA - Dec 20 DB - PubMed DO - 10.5761/atcs.lte.16-00106 DP - NLM ET - 2016/08/19 IS - 6 KW - Age Factors Child Behavior *Child Development Child, Preschool Cognition Female Humans Hypoplastic Left Heart Syndrome/diagnosis/physiopathology/psychology/*surgery Infant Infant Behavior Male Motor Activity Nervous System/*growth & development *Norwood Procedures/adverse effects Time Factors Treatment Outcome LA - eng N1 - 2186-1005 Knirsch, Walter Liamlahi, Rabia Dave, Hitendu Kretschmar, Oliver Latal, Beatrice Comparative Study Letter Ann Thorac Cardiovasc Surg. 2016 Dec 20;22(6):375-377. doi: 10.5761/atcs.lte.16-00106. Epub 2016 Aug 16. PY - 2016 SN - 1341-1098 (Print) 1341-1098 SP - 375-377 ST - Neurodevelopmental Outcome of Children with Hypoplastic Left Heart Syndrome at One and Four Years of Age Comparing Hybrid and Norwood Procedure T2 - Ann Thorac Cardiovasc Surg TI - Neurodevelopmental Outcome of Children with Hypoplastic Left Heart Syndrome at One and Four Years of Age Comparing Hybrid and Norwood Procedure VL - 22 ID - 370 ER - TY - JOUR AB - The neurodevelopmental outcome of hypoplastic left heart syndrome in infants remains unclear. All 11 survivors of staged surgical repair of hypoplastic left heart syndrome received standardized neurodevelopmental assessments at one regional children's hospital. Seven children (64%) had major developmental disabilities. Quality-of-life outcomes must be considered when management options for children with hypoplastic left heart syndrome are evaluated. AD - Department of Pediatrics, State University of New York at Buffalo. AN - 7532710 AU - Rogers, B. T. AU - Msall, M. E. AU - Buck, G. M. AU - Lyon, N. R. AU - Norris, M. K. AU - Roland, J. M. AU - Gingell, R. L. AU - Cleveland, D. C. AU - Pieroni, D. R. DA - Mar DB - PubMed DO - 10.1016/s0022-3476(95)70478-7 DP - NLM ET - 1995/03/01 IS - 3 KW - Cerebral Palsy/complications Developmental Disabilities/*etiology Female Follow-Up Studies Fontan Procedure Humans Hypoplastic Left Heart Syndrome/*complications/surgery Infant, Newborn Intellectual Disability/*etiology Male Motor Skills *Quality of Life Treatment Outcome LA - eng N1 - Rogers, B T Msall, M E Buck, G M Lyon, N R Norris, M K Roland, J M Gingell, R L Cleveland, D C Pieroni, D R Journal Article Research Support, Non-U.S. Gov't United States J Pediatr. 1995 Mar;126(3):496-8. doi: 10.1016/s0022-3476(95)70478-7. PY - 1995 SN - 0022-3476 (Print) 0022-3476 SP - 496-8 ST - Neurodevelopmental outcome of infants with hypoplastic left heart syndrome T2 - J Pediatr TI - Neurodevelopmental outcome of infants with hypoplastic left heart syndrome VL - 126 ID - 397 ER - TY - JOUR AB - The neurodevelopmental outcome of hypoplastic left heart syndrome in infants remains unclear. All 11 survivors of staged surgical repair of hypoplastic left heart syndrome received standardized neurodevelopmental assessments at one regional children's hospital. Seven children (64%) had major developmental disabilities. Quality-of-life outcomes must be considered when management options for children with hypoplastic left heart syndrome are evaluated. AD - B.T. Rogers, Robert Warner Rehabilitation Center, Children's Hospital, 936 Delaware Avenue, Buffalo, NY 14209, United States AU - Rogers, B. T. AU - Msall, M. E. AU - Buck, G. M. AU - Lyon, N. R. AU - Norris, M. K. AU - Roland, J. M. A. AU - Gingell, R. L. AU - Cleveland, D. C. AU - Pieroni, D. R. DB - Embase Medline DO - 10.1016/S0022-3476(95)70478-7 IS - 3 KW - article clinical article cognition developmental disorder disability human hypoplastic left heart syndrome newborn priority journal prognosis quality of life LA - English M3 - Article N1 - L25088076 1995-04-04 PY - 1995 SN - 0022-3476 SP - 496-498 ST - Neurodevelopmental outcome of infants with hypoplastic left heart syndrome T2 - Journal of Pediatrics TI - Neurodevelopmental outcome of infants with hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L25088076 http://dx.doi.org/10.1016/S0022-3476(95)70478-7 VL - 126 ID - 1369 ER - TY - JOUR AB - OBJECTIVE: To compare neurodevelopmental outcome (NDO) in patients with hypoplastic left heart syndrome (HLHS), other functional single ventricle lesions, and the standard population and to investigate predictors of NDO in the population of children with functional single ventricle (FSV). STUDY DESIGN: A time- and age-defined cohort of patients with the Fontan circulation was recruited to participate in neurodevelopmental testing, behavioral evaluation, and imaging of the central nervous system. The Wechsler Intelligence test was the primary measure of NDO. Analysis included comparison of patients with HLHS with other patients with functional single ventricles. Other potential clinical predictors of NDO were investigated. RESULTS: The mean Full Scale Wechsler Intelligence score was 101.4+/-5.4. For the HLHS subgroup the mean Full Scale Wechsler score was 93.8+/-7.3, and for the non-HLHS subgroup it was 107.0+/-7.0. Although the HLHS group had significantly lower scores than the non-HLHS subgroup, neither subgroup scored significantly different from the standard population on the Wechsler Scales. Socioeconomic status, circulatory arrest, and perioperative seizures also were predictive of neurodevelopmental outcome. CONCLUSION: Neurodevelopmental and behavioral outcome in patients who have undergone the Fontan procedure including patients with HLHS is good in the preschool and early school years, with Wechsler Intelligence scores generally in the normal range. AD - University of Michigan Congenital Heart Center and the Division of Pediatric Cardiology, the Department of Pediatrics, University of Michigan, Ann Arbor, USA. AN - 11060530 AU - Goldberg, C. S. AU - Schwartz, E. M. AU - Brunberg, J. A. AU - Mosca, R. S. AU - Bove, E. L. AU - Schork, M. A. AU - Stetz, S. P. AU - Cheatham, J. P. AU - Kulik, T. J. DA - Nov DB - PubMed DO - 10.1067/mpd.2000.108952 DP - NLM ET - 2000/11/04 IS - 5 KW - Central Nervous System/pathology Child Behavior *Child Development Child, Preschool *Developmental Disabilities Female *Fontan Procedure Humans Hypoplastic Left Heart Syndrome/*surgery Infant *Intelligence Magnetic Resonance Imaging Male Neurologic Examination *Psychomotor Performance Treatment Outcome Ventricular Dysfunction/*surgery Wechsler Scales LA - eng N1 - Goldberg, C S Schwartz, E M Brunberg, J A Mosca, R S Bove, E L Schork, M A Stetz, S P Cheatham, J P Kulik, T J M01-RR00042/RR/NCRR NIH HHS/United States Comparative Study Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S. United States J Pediatr. 2000 Nov;137(5):646-52. doi: 10.1067/mpd.2000.108952. PY - 2000 SN - 0022-3476 (Print) 0022-3476 SP - 646-52 ST - Neurodevelopmental outcome of patients after the fontan operation: A comparison between children with hypoplastic left heart syndrome and other functional single ventricle lesions T2 - J Pediatr TI - Neurodevelopmental outcome of patients after the fontan operation: A comparison between children with hypoplastic left heart syndrome and other functional single ventricle lesions VL - 137 ID - 340 ER - TY - JOUR AB - OBJECTIVE: The goal was to determine the relative effects of underlying genetic factors and current management strategies on neurodevelopmental disabilities among one-year old survivors of palliation for hypoplastic left heart syndrome. METHODS: Children who underwent staged reconstruction for hypoplastic left heart syndrome and variants were assessed at 1 year of age by using a neuromuscular examination and the Bayley Scales of Infant Development II, which provide the Mental Development Index and the Psychomotor Development Index. The effects of perioperative, operative, and genetic variables on developmental scores were evaluated. RESULTS: The median birth weight was 3.3 kg (range: 2.1-4.5 kg). Eight-three patients (94%) underwent multiple operations with cardiopulmonary bypass during the first year of life (median: 2 operations). Seven patients (8%) required extracorporeal membrane oxygenation. Twenty-five patients (28%) had a confirmed or suspected genetic syndrome. At 1 year of age, the neuromuscular examination results were abnormal or suspect for 57 patients (65%). The median Mental Development Index score was 90, and 10 patients (11%) had scores of <70 (2 SDs below the general population mean). The median Psychomotor Development Index score was 73, and 42 patients (48%) had scores of <70. In multivariate analyses, younger gestational age, the presence of a genetic syndrome, and the need for preoperative intubation had significant negative effects on neurodevelopmental outcomes. No association was found with operative factors, including duration of deep hypothermic circulatory arrest. CONCLUSIONS: At 1 year of age, there was a significant incidence of neurodevelopmental disabilities in children with hypoplastic left heart syndrome and variants; motor scores were particularly concerning. Many children had suspected or confirmed genetic syndromes, which negatively affected neurodevelopmental outcomes. Surgical variables did not affect neurologic outcomes. AD - Department of Pediatrics, Division of Cardiology, Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA. tabbutt@email.chop.edu AN - 18310195 AU - Tabbutt, S. AU - Nord, A. S. AU - Jarvik, G. P. AU - Bernbaum, J. AU - Wernovsky, G. AU - Gerdes, M. AU - Zackai, E. AU - Clancy, R. R. AU - Nicolson, S. C. AU - Spray, T. L. AU - Gaynor, J. W. DA - Mar DB - PubMed DO - 10.1542/peds.2007-1282 DP - NLM ET - 2008/03/04 IS - 3 KW - Cardiac Surgical Procedures/*adverse effects/methods Cohort Studies Developmental Disabilities/diagnosis/epidemiology/*etiology Female Follow-Up Studies Humans Hypoplastic Left Heart Syndrome/diagnosis/mortality/*surgery Incidence Infant Infant, Newborn Male Neuropsychological Tests Palliative Care/*methods Probability Registries Retrospective Studies Risk Assessment Severity of Illness Index Survival Analysis Time Factors LA - eng N1 - 1098-4275 Tabbutt, Sarah Nord, Alex S Jarvik, Gail P Bernbaum, Judy Wernovsky, Gil Gerdes, Marsha Zackai, Elaine Clancy, Robert R Nicolson, Susan C Spray, Thomas L Gaynor, J William Journal Article Research Support, Non-U.S. Gov't United States Pediatrics. 2008 Mar;121(3):476-83. doi: 10.1542/peds.2007-1282. PY - 2008 SN - 0031-4005 SP - 476-83 ST - Neurodevelopmental outcomes after staged palliation for hypoplastic left heart syndrome T2 - Pediatrics TI - Neurodevelopmental outcomes after staged palliation for hypoplastic left heart syndrome VL - 121 ID - 154 ER - TY - JOUR AB - BACKGROUND: Both congenital heart disease (CHD) and very low birth weight (VLBW) infants are very high risk for neurodevelopmental delay. We investigated neurological development at 3 years among pediatric patients with CHD after surgical intervention, very low birth weight (VLBW), and healthy controls. METHODS: We enrolled pediatric patients with CHD (n = 67), VLBW (n = 67), and healthy controls (n = 81). Infants with CHD were grouped into those with single ventricle and two ventricles, and infants with VLBW were grouped into those with birth weights of <1000 and 1000-1499 g. Neurodevelopmental outcomes at 3 years were evaluated using the Bayley Scales of Infant and Toddler Development, Third Edition. RESULTS: Compared with healthy controls, a significant deficit in the language, cognition, and motor skills scores were observed in infants with CHD and VLBW. Infants with a single ventricle exhibited significantly low scores in language and gross motor skills. No statistically significant difference was observed between the birth weight groups of <1000 and 1000-1499 g. CONCLUSION: Neurodevelopmental outcomes for infants with both CHD and VLBW showed impairment. Notably, neurodevelopmental delays in infants with a single ventricle were remarkable. Thus, because infants with both CHD and VLBW are at high risk for neurodevelopmental disorders, periodic developmental screenings and support are warranted for these children. (215 words). AU - Yoshida, T. AU - Hiraiwa, A. AU - Ibuki, K. AU - Makimoto, M. AU - Inomata, S. AU - Tamura, K. AU - Kawasaki, Y. AU - Ozawa, S. AU - Hirono, K. AU - Ichida, F. DB - Medline DO - 10.1111/ped.14160 KW - article Bayley Scales of Infant Development child cognition controlled study developmental screening female heart single ventricle human infant language major clinical study male motor performance nervous system malformation pediatric patient surgery very low birth weight LA - English M3 - Article in Press N1 - L630663476 2020-01-24 PY - 2020 SN - 1442-200X ST - Neurodevelopmental outcomes at three years for infants with CHD and VLBW T2 - Pediatrics international : official journal of the Japan Pediatric Society TI - Neurodevelopmental outcomes at three years for infants with CHD and VLBW UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L630663476 http://dx.doi.org/10.1111/ped.14160 ID - 567 ER - TY - JOUR AB - This study aimed to determine the neurodevelopmental (ND) outcome for children with hypoplastic left heart syndrome (HLHS) at early school age. English-speaking patients who underwent the Norwood procedure between 2000 and 2005 were eligible at 4-6 years of age for ND testing. Of the 72 eligible patients, 44 (61 %) agreed to participate, and 37 completed ND testing before the close of the study. Three subjects were excluded from analyses due to late stroke. The ND testing included intelligence, visual motor integration, memory and motor and language skills. Parents and teachers completed measures of behavior and attention problems. Subjects' scores and parent/teacher ratings were converted to z-scores and compared with test norms. Higher scores on child measures represent better outcomes, whereas higher scores on parent and teacher rating scales indicate more problems. The average ND performance of the tested cohort fell within one standard deviation of the test norms for all measures. However, the subjects performed significantly lower than the test norms on measures of visual-motor integration, fine motor skills, memory, and word structure (z = -0.42 to -0.54; p < 0.005). On the parent and teacher completed measures, the subjects scored higher than the test norms on attention problems (z = 0.40-0.62; p < 0.005). Although the overall ND performance of the cohort was normal, the subjects showed relative weakness in visual motor and attention skills. Ongoing developmental monitoring of these children is recommended to guide interventions that may improve individual outcomes and to assess the impact of changes in clinical management strategies on functional outcomes. AD - Department of Pediatrics, Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI, 53201, USA, cbrosig@chw.org. AN - 23503929 AU - Brosig, C. AU - Mussatto, K. AU - Hoffman, G. AU - Hoffmann, R. G. AU - Dasgupta, M. AU - Tweddell, J. AU - Ghanayem, N. C2 - PMC3982227 C6 - NIHMS563695 article to disclose. The authors have no conflicts of interest to disclose. DA - Oct DB - PubMed DO - 10.1007/s00246-013-0679-3 DP - NLM ET - 2013/03/19 IS - 7 KW - *Attention Child *Child Behavior *Child Development Child, Preschool *Cognition Developmental Disabilities/diagnosis/epidemiology/*etiology Female Follow-Up Studies Humans Hypoplastic Left Heart Syndrome/*complications/surgery Incidence Male *Motor Activity Neuropsychological Tests Norwood Procedures Retrospective Studies Risk Factors Wisconsin/epidemiology LA - eng N1 - 1432-1971 Brosig, Cheryl Mussatto, Kathleen Hoffman, George Hoffmann, Raymond G Dasgupta, Mahua Tweddell, James Ghanayem, Nancy UL1 RR031973/RR/NCRR NIH HHS/United States Journal Article Research Support, Non-U.S. Gov't Pediatr Cardiol. 2013 Oct;34(7):1597-604. doi: 10.1007/s00246-013-0679-3. Epub 2013 Mar 16. PY - 2013 SN - 0172-0643 (Print) 0172-0643 SP - 1597-604 ST - Neurodevelopmental outcomes for children with hypoplastic left heart syndrome at the age of 5 years T2 - Pediatr Cardiol TI - Neurodevelopmental outcomes for children with hypoplastic left heart syndrome at the age of 5 years VL - 34 ID - 197 ER - TY - JOUR AB - BACKGROUND: Previous studies of patients after the Fontan operation have reported IQ scores lower than population norms. In the past decade, changes have occurred both in surgical methods used and in the patient population undergoing Fontan palliation. The present study examined the impact of these changes on neurodevelopmental outcomes after Fontan. METHODS AND RESULTS: Neuropsychological tests were administered to 27 five-year-old children after Fontan. Mean age at repair was 2 years 4 months. The present sample was compared with an earlier Fontan group (EFG) of 133 patients who underwent surgery in the 1970s and 1980s. Mean age at repair for the EFG was 7 years 3 months. Compared with EFG, the present study sample was younger at Fontan (P=0.0001) and more likely to have undergone a Norwood procedure (P=0.02), a pre-Fontan bidirectional cavopulmonary anastomosis (P<0.001), and Fontan fenestration (P=0.001). Although mean full-scale, verbal, and performance IQ scores were within 1 SD (15 points) of the population mean of 100 (93+/-16, 95+/-15, and 91+/-17, respectively), mean full-scale and performance IQ scores were significantly lower than this population mean (P=0.03 and P=0.01, respectively). CONCLUSIONS: Compared with a historical cohort of Fontan patients from this institution, a staged approach to Fontan earlier in life is not detrimental to neurodevelopmental outcome. Neurodevelopmental outcomes in children after Fontan are in the normal range, but performance remains lower than the general population. AD - Department of Cardiovascular Surgery, Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA. AN - 11568043 AU - Forbess, J. M. AU - Visconti, K. J. AU - Bellinger, D. C. AU - Jonas, R. A. DA - Sep 18 DB - PubMed DO - 10.1161/hc37t1.094816 DP - NLM ET - 2001/09/25 IS - 12 Suppl 1 KW - Adolescent Adult Child Child, Preschool Cohort Studies Female Follow-Up Studies Fontan Procedure/*adverse effects/statistics & numerical data Humans Intelligence Tests/statistics & numerical data Learning Disabilities/*diagnosis/*etiology Male Neuropsychological Tests/statistics & numerical data Risk Assessment Risk Factors Time Treatment Outcome Wechsler Scales/statistics & numerical data LA - eng N1 - 1524-4539 Forbess, J M Visconti, K J Bellinger, D C Jonas, R A Clinical Trial Comparative Study Journal Article United States Circulation. 2001 Sep 18;104(12 Suppl 1):I127-32. doi: 10.1161/hc37t1.094816. PY - 2001 SN - 0009-7322 SP - I127-32 ST - Neurodevelopmental outcomes in children after the fontan operation T2 - Circulation TI - Neurodevelopmental outcomes in children after the fontan operation VL - 104 ID - 387 ER - TY - JOUR AB - OBJECTIVES: The purpose of this study was to assess the neurodevelopmental status of children after Fontan repair of functional single ventricle and to examine the relationship between cognitive function and selected patient characteristics. STUDY DESIGN: Neurodevelopmental tests including the Stanford-Binet Intelligence (IQ) scale and the Developmental Test of Visual Motor Integration (VMI) were administered to 32 children (26 months to 16 years of age) with complex single ventricle. The mean and distribution of IQ and VMI scores were compared with population norms. The relationship between test scores and patient characteristics was examined utilizing analysis of variance and correlational methods. RESULTS: The majority of children had intellectual function within the normal range (mean, 97.5 +/- 12.1). Below average VMI scores were found in 21.4% of children. There were no significant correlations between intellectual function or visual motor integration ability and preoperative oxygen saturation or age at Fontan. Children who had deep hypothermic circulatory arrest during a prior Norwood procedure tended to have a lower IQ score. CONCLUSIONS: Intellectual development in children with Fontan repair of complex heart defects is essentially within the normal range. Visual motor integration deficits may be more prevalent in these children. In our population, the duration and degree of preoperative hypoxemia had no apparent effect on cognitive function. AD - Children's Heart Institute, San Diego, California 92123, USA. AN - 9521946 AU - Uzark, K. AU - Lincoln, A. AU - Lamberti, J. J. AU - Mainwaring, R. D. AU - Spicer, R. L. AU - Moore, J. W. DA - Apr DB - PubMed DO - 10.1542/peds.101.4.630 DP - NLM ET - 1998/04/29 IS - 4 Pt 1 KW - Adolescent Child Child, Preschool Female Follow-Up Studies *Fontan Procedure Heart Arrest, Induced/adverse effects Heart Defects, Congenital/psychology/surgery Heart Ventricles/*abnormalities Humans Hypoxia/complications *Intelligence Male *Psychomotor Performance Stanford-Binet Test LA - eng N1 - Uzark, K Lincoln, A Lamberti, J J Mainwaring, R D Spicer, R L Moore, J W Journal Article Research Support, Non-U.S. Gov't United States Pediatrics. 1998 Apr;101(4 Pt 1):630-3. doi: 10.1542/peds.101.4.630. PY - 1998 SN - 0031-4005 (Print) 0031-4005 SP - 630-3 ST - Neurodevelopmental outcomes in children with Fontan repair of functional single ventricle T2 - Pediatrics TI - Neurodevelopmental outcomes in children with Fontan repair of functional single ventricle VL - 101 ID - 326 ER - TY - JOUR AB - Infants with hypoplastic left heart syndrome (HLHS) are now surviving to school age and early adulthood because of surgical palliation with either staged reconstruction or transplantation. Although in some respects these patients are doing remarkably well, recent investigations have shown that they are at risk for cognitive, neuromotor, and psychosocial problems. This article examines the neurodevelopmental outcome for children and adolescents with HLHS. In addition, hemodynamic and genetic factors unique to this patient population that may impact longer-term outcome are explored. The continued evolution of the pre-, intra- and post-operative care of the patient with HLHS has significantly improved early survival. Concomitant changes in care, such as selective cerebral perfusion and neurologic monitoring, aimed at reducing neurologic insult may also favorably impact later neurodevelopmental status. AD - Department of Pediatrics, Division of Cardiology, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, GA 30322-1062, USA. AN - 15283351 AU - Mahle, W. T. AU - Wernovsky, G. DB - PubMed DO - 10.1053/j.pcsu.2004.02.019 DP - NLM ET - 2004/07/31 KW - Adolescent Cardiac Surgical Procedures/*adverse effects Child Child, Preschool Developmental Disabilities/etiology/prevention & control Humans Hypoplastic Left Heart Syndrome/*complications/physiopathology/surgery Infant Mental Disorders/*etiology/prevention & control Nervous System Diseases/*etiology/prevention & control Patient Care/methods Risk Factors Time Factors LA - eng N1 - Mahle, William T Wernovsky, Gil Journal Article Review United States Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2004;7:39-47. doi: 10.1053/j.pcsu.2004.02.019. PY - 2004 SN - 1092-9126 (Print) 1092-9126 SP - 39-47 ST - Neurodevelopmental outcomes in hypoplastic left heart syndrome T2 - Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu TI - Neurodevelopmental outcomes in hypoplastic left heart syndrome VL - 7 ID - 298 ER - TY - JOUR AB - Purpose: Hypoplastic Left Heart Syndrome (HLHS) accounts for 7-9% of all congenital heart diseases. It is fatal unless surgically corrected. Survival has improved overtime. However, neurodevelopmental outcomes have not been known until recently. This paper discusses the neurodevelopmental outcomes of patients with HLHS and the factors that influence these outcomes. Methods: A search of the literature was performed using several databases, medical textbooks and other on-line sources to locate research related to HLHS and neurodevelopment in HLHS. Results: Mean performance IQ scores tended to be lower than mean verbal IQ scores. Mean motor scores were below normal population values indicating impairment. Growth was impaired in many of those with HLHS. Factors that had negative impact on outcomes included deep hypothermic circulatory arrest (DHCA), cardiopulmonary bypass (CPB), longer hospital length-of-stay, seizures, and lower socioeconomic status (SES). Conclusions: The studies show that children with HLHS do have neurodevelopmental deficits. Intelligence, motor development and growth are all affected. Focus during care of these patients should be spent on improving the factors discussed that have been shown to have negative impacts on outcomes. AD - Nova Southeastern University AN - 105211531. Language: English. Entry Date: 20100730. Revision Date: 20150818. Publication Type: Journal Article AU - Hagan, E. AU - Feldman, H. DB - ccm DP - EBSCOhost IS - 2 KW - Cardiac Patients -- Evaluation Child Development Disorders Hypoplastic Left Heart Syndrome -- Diagnosis Neurology Outcomes (Health Care) Aortic Valve -- Anatomy and Histology Aortic Valve -- Physiopathology Databases Embase Heart Diseases -- Familial and Genetic Human Hypoplastic Left Heart Syndrome -- Surgery Medline Mitral Valve -- Anatomy and Histology Mitral Valve -- Physiopathology Palliative Care -- Methods Psycinfo Surgery, Operative -- Methods N1 - pictorial; research. Journal Subset: Allied Health; Blind Peer Reviewed; Double Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. PY - 2010 SN - 1540-580X SP - 10p-10p ST - Neurodevelopmental outcomes in patients with hypoplastic left heart syndrome T2 - Internet Journal of Allied Health Sciences & Practice TI - Neurodevelopmental outcomes in patients with hypoplastic left heart syndrome UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105211531&site=ehost-live&scope=site VL - 8 ID - 1649 ER - TY - JOUR AB - Objectives: Neurofeedback is a technique that aims to teach a subject to regulate a brain parameter measured by a technical interface to modulate his/her related brain and cognitive activities. However, the use of neurofeedback as a therapeutic tool for psychiatric disorders remains controversial. The aim of this review is to summarize and to comment the level of evidence of electroencephalogram (EEG) neurofeedback and real-time functional magnetic resonance imaging (fMRI) neurofeedback for therapeutic application in psychiatry. Method: Literature on neurofeedback and mental disorders but also on brain computer interfaces (BCI) used in the field of neurocognitive science has been considered by the group of expert of the Neurofeedback evaluation & training (NExT) section of the French Association of biological psychiatry and neuropsychopharmacology (AFPBN). Results: Results show a potential efficacy of EEG-neurofeedback in the treatment of attentional-deficit/hyperactivity disorder (ADHD) in children, even if this is still debated. For other mental disorders, there is too limited research to warrant the use of EEG-neurofeedback in clinical practice. Regarding fMRI neurofeedback, the level of evidence remains too weak, for now, to justify clinical use. The literature review highlights various unclear points, such as indications (psychiatric disorders, pathophysiologic rationale), protocols (brain signals targeted, learning characteristics) and techniques (EEG, fMRI, signal processing). Conclusion: The field of neurofeedback involves psychiatrists, neurophysiologists and researchers in the field of brain computer interfaces. Future studies should determine the criteria for optimizing neurofeedback sessions. A better understanding of the learning processes underpinning neurofeedback could be a key element to develop the use of this technique in clinical practice. (PsycINFO Database Record (c) 2017 APA, all rights reserved) Introduction: Le neurofeedback consiste à mesurer, chez un sujet, une activité cérébrale et à traiter le signal au moyen d’une interface technique afin d’en extraire un paramètre d’intérêt qui sera présenté en temps réel au participant sous la forme d’une information visuelle ou auditive. L’objectif est d’apprendre au sujet à modifier ce paramètre et donc à moduler son activité cérébrale et cognitive. Cependant, l’utilisation du neurofeedback en pratique clinique pour la prise en charge des troubles psychiatriques reste controversée. Méthode: Cet article présente une synthèse de la 1re journée nationale sur le neurofeedback organisé par la section Neurofeedback Evaluation & Training (NExT) de l’Association franc¸ aise de psychiatrie biologique et de neuropharmacologie (AFPBN). Un état des lieux de l’utilisation du neurofeedback en électroencéphalographie (EEG) et en imagerie par résonance magnétique fonctionnelle (IRMf) est proposé. Pour intégrer l’arsenal thérapeutique, cette technique doit en effet répondre aux exigences de l’evidence based medicine. Résultats: Les études montrent une efficacité probable du neurofeedback en EEG pour le trouble du déficit de l’attention/hyperactivité (TDAH) chez les enfants. Pour les autres troubles psychiatriques, le nombre d’études est encore trop limité pour se positionner. En ce qui concerne le neurofeedback en IRMf, le niveau de preuve reste, pour l’heure, trop faible pour justifier une utilisation clinique. Les modalités d’emploi du neurofeedback, notamment en ce qui concerne les indications médicales, les protocoles d’utilisation (activité(s) cérébrale(s) ciblée(s), caractéristiques d’apprentissage) et les outils de mesure employés (EEG, IRMf, mode de traitement du signal) restent donc à clarifier. Conclusion: Le vaste champ de recherche du neurofeedback implique à la fois des psychiatres, des neurophysiologistes et des chercheurs du domaine des interfaces cerveaux-ordinateurs. Les futurs travaux devront s’attacher à déterminer les critères permettant d’optimiser les séances de neurofeedback afin de mieux comprendre ses effets, le tout dans l’optique d’une utilisation en pratique clinique dans certaines indications. L’étude des processus d’apprentissage constitue un élément clé autour duquel les futures recherches devront se focaliser. (PsycINFO Database Record (c) 2017 APA, all rights reserved) AD - Micoulaud-Franchi, J.-A., Services d'Explorations Fonctionnelles du Systeme Nerveux, Clinique du Sommeil, CHU de Bordeaux, place Amelie-Raba-Leon, 33076, Bordeaux, France AN - 2017-15475-009 AU - Arns, M. AU - Batail, J. M. AU - Bioulac, S. AU - Congedo, M. AU - Daudet, C. AU - Drapier, D. AU - Fovet, T. AU - Jardri, R. AU - Le-Van-Quyen, M. AU - Lotte, F. AU - Mehler, D. AU - Micoulaud-Franchi, J. A. AU - Purper-Ouakil, D. AU - Vialatte, F. DB - psyh DO - 10.1016/j.encep.2016.11.003 DP - EBSCOhost IS - 2 KW - Neurofeedback EEG Real-time fMRI Psychiatric disorder Electroencephalography Mental Disorders Psychiatry Functional Magnetic Resonance Imaging Neurotherapy N1 - Research Institute Brainclinics, Nijmegen, Netherlands. Other Publishers: Masson. Release Date: 20170717. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Electroencephalography; Mental Disorders; Psychiatry; Functional Magnetic Resonance Imaging; Neurotherapy. Classification: Specialized Interventions (3350). Population: Human (10). Methodology: Literature Review. Page Count: 11. Issue Publication Date: Apr, 2017. Publication History: First Posted Date: Dec 29, 2016; Accepted Date: Nov 21, 2016; First Submitted Date: Aug 29, 2016. Copyright Statement: L’Encéphale, Paris. 2016. PY - 2017 SN - 0013-7006 SP - 135-145 ST - Neurofeedback: One of today's techniques in psychiatry? T2 - L'Encéphale: Revue de psychiatrie clinique biologique et thérapeutique TI - Neurofeedback: One of today's techniques in psychiatry? UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2017-15475-009&site=ehost-live&scope=site ORCID: 0000-0002-5203-8444 ORCID: 0000-0003-4596-1502 jarthur.micoulaud@gmail.com VL - 43 ID - 1676 ER - TY - JOUR AN - 12520246 AU - Wernovsky, G. AU - Newburger, J. DA - Jan DB - PubMed DO - 10.1067/mpd.2003.mpd0354 DP - NLM ET - 2003/01/10 IS - 1 KW - Abnormalities, Multiple/physiopathology Adolescent Child *Child Development Child, Preschool Female Heart Defects, Congenital/physiopathology Heart Transplantation Humans Hypoplastic Left Heart Syndrome/*physiopathology/psychology/surgery Infant Male Treatment Outcome LA - eng N1 - Wernovsky, Gil Newburger, Jane Editorial United States J Pediatr. 2003 Jan;142(1):6-8. doi: 10.1067/mpd.2003.mpd0354. PY - 2003 SN - 0022-3476 (Print) 0022-3476 SP - 6-8 ST - Neurologic and developmental morbidity in children with complex congenital heart disease T2 - J Pediatr TI - Neurologic and developmental morbidity in children with complex congenital heart disease VL - 142 ID - 456 ER - TY - JOUR AB - OBJECTIVES: The purpose of this study is to review a single-institutional experience gained over one decade, to assess late neuropsychological outcome in patients as well as to determine Norwood operation risk factors that are related to patient and procedure and influence these results. BACKGROUND: The reports of first successful staged operation of hypoplastic left heart syndrome (HLHS) and its numerous modifications have improved the early survival also in patients with complex forms. Specifically, the identification of other specific risk factors related to patient or procedure in the first stage of palliation for HLHS may play a role in cognitive deficit. METHODS: Single-institutional, cross-sectional study of neurodevelopmental outcome. The mean age at testing was 6.9 +/- 0.3 years. There were 19 patients enrolled with completed psychological testing Standford-Binet test. RESULTS: The mean neurocognitive results were significantly below the population normative values. The mean full-scale IQ for the entire cohort was 84.1 +/- 8.2. The predictive factors are strongly associated with severe metabolic acidosis, low birth weight, hospital stay over 35 days, prolonged duration of DHA and aortic valve diameter playing an important role in the subsequent psychological outcome, especially in differential subsections of IQ scale (Tab. 2, Ref. 15). Full Text (Free, PDF) www.bmj.sk. AD - Department of Pediatric Cardiology, Children's University Hospital Bratislava and Children's Cardiac Center of Slovak Republic, Bratislava, Slovakia. bordacov@yahoo.com AN - 17694805 AU - Bordacova, L. AU - Docolomanska, D. AU - Masura, J. DB - PubMed DP - NLM ET - 2007/08/19 IS - 4-5 KW - Child Child, Preschool Cognition Disorders/diagnosis/etiology Developmental Disabilities/diagnosis/*etiology Follow-Up Studies Humans Hypoplastic Left Heart Syndrome/*psychology/surgery Infant, Newborn Intelligence Neuropsychological Tests Psychomotor Performance LA - eng N1 - Bordacova, L Docolomanska, D Masura, J Journal Article Slovakia Bratisl Lek Listy. 2007;108(4-5):203-6. PY - 2007 SN - 0006-9248 (Print) 0006-9248 SP - 203-6 ST - Neuropsychological outcome in children with hypoplastic left heart syndrome T2 - Bratisl Lek Listy TI - Neuropsychological outcome in children with hypoplastic left heart syndrome VL - 108 ID - 247 ER - TY - JOUR AB - Design: To assess the neuropsychological outcome of children with asymmetric ventricles and unilateral ventriculomegaly identified in utero. Setting: Fetal neurology clinic. Population: We assessed 21 children with asymmetric ventricles (group 1) and 20 children with unilateral ventriculomegaly (group 2) identified in utero and compared them with a group of 20 children with symmetric ventricles using a formal neuropsychological tool: the Bayley Scale of Infant Development II (BSID-II). Main outcome measures: The group of children with unilateral ventriculomegaly scored significantly lower than the control group on the mental developmental index (MDI) and on the behaviour rating scale (BRS) but not on the psychomotor index. The group of children with asymmetric ventricles did not differ significantly from the control group on either the MDI or psychomotor developmental index but differed from the latter on the BRS. Fifteen percent of the children in the asymmetric ventriculomegaly group performed two SDs below average compared with 4% of children in the asymmetrical ventricles group and none of the control. Conclusion: Our results indicate that prenatally observed unilateral ventriculomegaly is a significant risk factor for developmental delay. The mental and motor outcome of children with asymmetric ventricles is similar to that of the control group, but these children are at a significant risk for behavioural abnormalities. © 2007 The Authors. AD - T. Lerman-Sagie, Wolfson Medical Center, PO Box 5, Holon 58900, Israel AU - Sadan, S. AU - Malinger, G. AU - Schweiger, A. AU - Lev, D. AU - Lerman-Sagie, T. DB - Embase Medline DO - 10.1111/j.1471-0528.2007.01301.x IS - 5 KW - article bayley scale of infant development behavioral science controlled study female fetus echography heart single ventricle heart ventricle hypertrophy human major clinical study male medical assessment mental development neuropsychology outcome assessment prenatal diagnosis preschool child priority journal psychomotor development rating scale risk factor scoring system LA - English M3 - Article N1 - L46587970 2007-05-01 PY - 2007 SN - 1470-0328 1471-0528 SP - 596-602 ST - Neuropsychological outcome of children with asymmetric ventricles or unilateral mild ventriculomegaly identified in utero T2 - BJOG: An International Journal of Obstetrics and Gynaecology TI - Neuropsychological outcome of children with asymmetric ventricles or unilateral mild ventriculomegaly identified in utero UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L46587970 http://dx.doi.org/10.1111/j.1471-0528.2007.01301.x VL - 114 ID - 1237 ER - TY - JOUR AB - To assess long-term neuropsychological outcomes of infant heart transplant recipients.Fifty-five children (mean age, 6.4 years) with a primary diagnosis of hypoplastic left heart syndrome or other cardiac disorders participated in the study. Six areas of risk were assessed (birth; preoperative, intraoperative, and postoperative status; long-term medical complications; and socioeconomic factors). Neuropsychological evaluations included the following: Wechsler Scales of Intelligence, Wechsler Individual Achievement Test, Berry Visual Motor Integration Scale, Comprehensive Evaluation of Language Function, and Achenbach Child Behavioral Checklist.Intellectual functioning scores revealed a mean full-scale IQ of 81 ± 19. Intraoperative and postoperative variables (bypass time, hospital course, and lowest posttransplant pH), long-term medical complications (serious infections and posttransplant surgical procedures), and socioeconomic factors were associated with cognitive functioning. Visual-motor functioning was also related to bypass and total support time.Long-term outcomes reveal low-average neuropsychological functioning and are related to intrasurgical-postsurgical, long-term medical, and socioeconomic factors. The neuropsychological and psychosocial functioning of infant heart transplant recipients will be important to address as they mature during adolescence and adulthood. AD - Departments of Pediatrics, Psychology, Surg., Loma Linda Univ., Loma L. AU - Baum, M. AU - Catherin Freier, M. AU - Freeman, K. AU - Babikian, T. AU - Ashwal, S. AU - Chinnock, R. AU - Bailey, L. DB - Embase Medline DO - 10.1016/j.jpeds.2004.05.047 IS - 3 KW - article bypass surgery child cognition controlled study female heart disease heart graft human hypoplastic left heart syndrome infant infection complication long term care major clinical study male maturation neurologic examination neuropsychology postoperative complication postoperative infection priority journal risk assessment socioeconomics treatment outcome visuomotor coordination LA - English M3 - Article N1 - L39165686 2004-09-15 PY - 2004 SN - 0022-3476 SP - 365-372 ST - Neuropsychological outcome of infant heart transplant recipients T2 - Journal of Pediatrics TI - Neuropsychological outcome of infant heart transplant recipients UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L39165686 http://dx.doi.org/10.1016/j.jpeds.2004.05.047 VL - 145 ID - 1291 ER - TY - JOUR AB - OBJECTIVE: To assess long-term neuropsychological outcomes of infant heart transplant recipients. STUDY DESIGN: Fifty-five children (mean age, 6.4 years) with a primary diagnosis of hypoplastic left heart syndrome or other cardiac disorders participated in the study. Six areas of risk were assessed (birth; preoperative, intraoperative, and postoperative status; long-term medical complications; and socioeconomic factors). Neuropsychological evaluations included the following: Wechsler Scales of Intelligence, Wechsler Individual Achievement Test, Berry Visual Motor Integration Scale, Comprehensive Evaluation of Language Function, and Achenbach Child Behavioral Checklist. RESULTS: Intellectual functioning scores revealed a mean full-scale IQ of 81 +/- 19. Intraoperative and postoperative variables (bypass time, hospital course, and lowest posttransplant pH), long-term medical complications (serious infections and posttransplant surgical procedures), and socioeconomic factors were associated with cognitive functioning. Visual-motor functioning was also related to bypass and total support time. CONCLUSIONS: Long-term outcomes reveal low-average neuropsychological functioning and are related to intrasurgical-postsurgical, long-term medical, and socioeconomic factors. The neuropsychological and psychosocial functioning of infant heart transplant recipients will be important to address as they mature during adolescence and adulthood. AD - Department of Pediatrics, Division of Child Neurology, Loma Linda University, Loma Linda, CA 92350, USA. AN - 15343193 AU - Baum, M. AU - Freier, M. C. AU - Freeman, K. AU - Babikian, T. AU - Ashwal, S. AU - Chinnock, R. AU - Bailey, L. DA - Sep DB - PubMed DO - 10.1016/j.jpeds.2004.05.047 DP - NLM ET - 2004/09/03 IS - 3 KW - Child Female *Heart Transplantation Humans Infant, Newborn *Intelligence Male Neuropsychological Tests Postoperative Period Risk Factors Socioeconomic Factors Wechsler Scales LA - eng N1 - Baum, Marti Freier, M Catherin Freeman, Kim Babikian, Talin Ashwal, Stephen Chinnock, Richard Bailey, Leonard Journal Article Research Support, Non-U.S. Gov't United States J Pediatr. 2004 Sep;145(3):365-72. doi: 10.1016/j.jpeds.2004.05.047. PY - 2004 SN - 0022-3476 (Print) 0022-3476 SP - 365-72 ST - Neuropsychological outcome of infant heart transplant recipients T2 - J Pediatr TI - Neuropsychological outcome of infant heart transplant recipients VL - 145 ID - 384 ER - TY - JOUR AB - OBJECTIVES: Despite advances in perioperative management during surgical treatment of hypoplastic left heart syndrome (HLHS), patients are still at risk for adverse neurodevelopmental sequelae including cognitive dysfunction. This study aimed to assess a neuropsychological profile of HLHS patients at school age who underwent the Norwood operation between 1996 and 2003 with deep hypothermic circulatory arrest (DHCA) or antegrade selective cerebral perfusion (ASCP), respectively. METHODS: Forty HLHS patients (DHCA: n = 11 and ASCP: n = 29) were compared with healthy controls (DHCA controls: n = 10 and ASCP controls: n = 24), recruited according to age, sex and socioeconomic status. Neuropsychological assessment included non-verbal intelligence (IQ norms) and raw score measures of visual and verbal short- and long-term memory as well as executive functions, processing speed and concentration. Neuropsychological data were correlated with bypass and circulatory arrest times. RESULTS: Compared with control subjects, both patient groups had average non-verbal intelligence scores [DHCA: 102 (72-112) and ASCP: 92 (70-127)], but showed reduced long-term memory capacities and decreased executive performance as well as reduced processing speed. DHCA patients, furthermore, had a reduced visual attention span, and ASCP patients scored less on the verbal learning task than controls. The duration of DHCA and cardiopulmonary bypass (CPB) was associated with visual executive functions and short-term memory. In the DHCA group, the duration of DHCA was correlated with the time to complete the Trail Making Test A measuring visual executive functions (Spearman rank correlation, rS = 0.867, P = 0.018). In patients provided with ASCP, the cumulated CPB duration was negatively correlated with the score of the block span test measuring visual short memory (rS = -0.476, P = 0.020) as well as with the copy score of the Rey Figure assessing visual executive functions (rS = -0.399, P = 0.032). CONCLUSIONS: School-age children with HLHS who underwent the Norwood procedure either with DHCA or ASCP show cognitive impairments compared with healthy controls. Our data indicate deficits in specific cognitive domains such as memory, executive functions and processing speed rather than basic intellectual dysfunction. AD - Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Kiel, Germany. Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Kiel, Germany jan.hansen@uk-sh.de. Department of Cardiovascular Surgery, University Hospital Schleswig-Holstein, Kiel, Germany. Department of Paediatric Neurology, University Hospital Schleswig-Holstein, Kiel, Germany. Department of General Paediatrics, University Hospital Schleswig-Holstein, Kiel, Germany. AN - 25100716 AU - Bergemann, A. AU - Hansen, J. H. AU - Rotermann, I. AU - Voges, I. AU - Scheewe, J. AU - Otto-Morris, C. AU - Geiger, F. AU - Kramer, H. H. DA - May DB - PubMed DO - 10.1093/ejcts/ezu299 DP - NLM ET - 2014/08/08 IS - 5 KW - Child Circulatory Arrest, Deep Hypothermia Induced/*methods Cognition/*physiology Cognition Disorders/*etiology Female Humans Hypoplastic Left Heart Syndrome/*surgery Male Memory, Short-Term/physiology Neuropsychological Tests Norwood Procedures/*adverse effects Palliative Care/*methods Retrospective Studies Cardiopulmonary bypass Hypoplastic left heart syndrome Hypothermic circulatory arrest Norwood operation LA - eng N1 - 1873-734x Bergemann, Annabell Hansen, Jan Hinnerk Rotermann, Ina Voges, Inga Scheewe, Jens Otto-Morris, Christine Geiger, Friedemann Kramer, Hans-Heiner Journal Article Research Support, Non-U.S. Gov't Germany Eur J Cardiothorac Surg. 2015 May;47(5):803-11. doi: 10.1093/ejcts/ezu299. Epub 2014 Aug 6. PY - 2015 SN - 1010-7940 SP - 803-11 ST - Neuropsychological performance of school-aged children after staged surgical palliation of hypoplastic left heart syndrome T2 - Eur J Cardiothorac Surg TI - Neuropsychological performance of school-aged children after staged surgical palliation of hypoplastic left heart syndrome VL - 47 ID - 172 ER - TY - JOUR AB - BACKGROUND: Few studies have described the neuropsychological outcomes and frequency of structural brain or genetic abnormalities in adolescents with single ventricle who underwent the Fontan procedure. METHODS AND RESULTS: In a cross-sectional, single-center study, we enrolled 156 subjects with single ventricle, mean age 14.5±2.9 years, who had undergone the Fontan procedure. Scores in the entire cohort on a standard battery of neuropsychological tests were compared with those of normative populations or to those of a group of 111 locally recruited healthy adolescents. They also underwent brain magnetic resonance imaging and were evaluated by a clinical geneticist. Genetic abnormalities were definite in 16 subjects (10%) and possible in 49 subjects (31%). Mean Full-Scale IQ was 91.6±16.8, mean Reading Composite score was 91.9±17.2, and mean Mathematics Composite score was 92.0±22.9, each significantly lower than the population means of 100±15. Mean scores on other neuropsychological tests were similarly lower than population norms. In multivariable models, risk factors for worse neuropsychological outcomes were longer total support and circulatory arrest duration at first operation, presence of a genetic abnormality, more operations and operative complications, more catheterization complications, and seizure history. The frequency of any abnormality on magnetic resonance imaging was 11 times higher among Fontan adolescents than referents (66% versus 6%); 19 (13%) patients had evidence of a stroke, previously undiagnosed in 7 patients (40%). CONCLUSIONS: The neuropsychological deficits and high frequencies of structural brain abnormalities in adolescents who underwent the Fontan procedure highlight the need for research on interventions to improve the long-term outcomes in this high-risk group. AD - Department of Neurology, Boston Children's Hospital, Boston, MA (D.C.B., C.G.W., M.J.R.) Department of Psychiatry, Boston Children's Hospital, Boston, MA (D.C.B., M.J.R., D.R.D.M.) Department of Neurology, Harvard Medical School, Boston, MA (D.C.B., M.J.R.) Department of Psychiatry, Harvard Medical School, Boston, MA (D.C.B., D.R.D.M.). Department of Neurology, Boston Children's Hospital, Boston, MA (D.C.B., C.G.W., M.J.R.) Graduate Program for Neuroscience, Boston University, Boston, MA (C.G.W.). Department of Neurology, Boston Children's Hospital, Boston, MA (D.C.B., C.G.W., M.J.R.) Department of Radiology, Boston Children's Hospital, Boston, MA (M.J.R., R.L.R.) Department of Psychiatry, Boston Children's Hospital, Boston, MA (D.C.B., M.J.R., D.R.D.M.) Department of Neurology, Harvard Medical School, Boston, MA (D.C.B., M.J.R.). Department of Radiology, Boston Children's Hospital, Boston, MA (M.J.R., R.L.R.) Department of Radiology, Harvard Medical School, Boston, MA (R.L.R.). Department of Cardiology, Boston Children's Hospital, Boston, MA (A.E.R., C.S., C.D.M., D.B., D.W., J.W.N.) Department of Pediatrics, Harvard Medical School, Boston, MA (A.E.R., D.W., J.W.N.). Department of Cardiology, Boston Children's Hospital, Boston, MA (A.E.R., C.S., C.D.M., D.B., D.W., J.W.N.). Department of Psychiatry, Boston Children's Hospital, Boston, MA (D.C.B., M.J.R., D.R.D.M.) Department of Psychiatry, Harvard Medical School, Boston, MA (D.C.B., D.R.D.M.). Department of Cardiology, Boston Children's Hospital, Boston, MA (A.E.R., C.S., C.D.M., D.B., D.W., J.W.N.) Department of Pediatrics, Harvard Medical School, Boston, MA (A.E.R., D.W., J.W.N.) Department of Biostatistics, Harvard T. H. Chan School of Public Health, Boston, MA (D.W.). AN - 26667085 AU - Bellinger, D. C. AU - Watson, C. G. AU - Rivkin, M. J. AU - Robertson, R. L. AU - Roberts, A. E. AU - Stopp, C. AU - Dunbar-Masterson, C. AU - Bernson, D. AU - DeMaso, D. R. AU - Wypij, D. AU - Newburger, J. W. C2 - PMC4845289 DA - Dec 14 DB - PubMed DO - 10.1161/jaha.115.002302 DP - NLM ET - 2015/12/17 IS - 12 KW - Adolescent Brain/*pathology Child Cognition Disorders/*etiology Cross-Sectional Studies Female *Fontan Procedure/adverse effects Genetic Diseases, Inborn/complications/genetics Heart Ventricles/*abnormalities/surgery Humans Magnetic Resonance Imaging Male Neuroimaging Neuropsychological Tests Young Adult Fontan procedure congenital heart defects genetics LA - eng N1 - 2047-9980 Bellinger, David C Watson, Christopher G Rivkin, Michael J Robertson, Richard L Roberts, Amy E Stopp, Christian Dunbar-Masterson, Carolyn Bernson, Dana DeMaso, David R Wypij, David Newburger, Jane W UL1 RR025758/RR/NCRR NIH HHS/United States R01 HL096825/HL/NHLBI NIH HHS/United States Journal Article Research Support, N.I.H., Extramural J Am Heart Assoc. 2015 Dec 14;4(12):e002302. doi: 10.1161/JAHA.115.002302. PY - 2015 SN - 2047-9980 ST - Neuropsychological Status and Structural Brain Imaging in Adolescents With Single Ventricle Who Underwent the Fontan Procedure T2 - J Am Heart Assoc TI - Neuropsychological Status and Structural Brain Imaging in Adolescents With Single Ventricle Who Underwent the Fontan Procedure VL - 4 ID - 167 ER - TY - JOUR AB - Background: Few studies have described the neuropsychological outcomes and frequency of structural brain or genetic abnormalities in adolescents with single ventricle who underwent the Fontan procedure.Methods and Results: In a cross-sectional, single-center study, we enrolled 156 subjects with single ventricle, mean age 14.5±2.9 years, who had undergone the Fontan procedure. Scores in the entire cohort on a standard battery of neuropsychological tests were compared with those of normative populations or to those of a group of 111 locally recruited healthy adolescents. They also underwent brain magnetic resonance imaging and were evaluated by a clinical geneticist. Genetic abnormalities were definite in 16 subjects (10%) and possible in 49 subjects (31%). Mean Full-Scale IQ was 91.6±16.8, mean Reading Composite score was 91.9±17.2, and mean Mathematics Composite score was 92.0±22.9, each significantly lower than the population means of 100±15. Mean scores on other neuropsychological tests were similarly lower than population norms. In multivariable models, risk factors for worse neuropsychological outcomes were longer total support and circulatory arrest duration at first operation, presence of a genetic abnormality, more operations and operative complications, more catheterization complications, and seizure history. The frequency of any abnormality on magnetic resonance imaging was 11 times higher among Fontan adolescents than referents (66% versus 6%); 19 (13%) patients had evidence of a stroke, previously undiagnosed in 7 patients (40%).Conclusions: The neuropsychological deficits and high frequencies of structural brain abnormalities in adolescents who underwent the Fontan procedure highlight the need for research on interventions to improve the long-term outcomes in this high-risk group. AD - Department of Neurology, Boston Children's Hospital, Boston MA Department of Psychiatry, Boston Children's Hospital, Boston MA Department of Neurology, Harvard Medical School, Boston MA Department of Psychiatry, Harvard Medical School, Boston MA Graduate Program for Neuroscience, Boston University, Boston MA Department of Radiology, Boston Children's Hospital, Boston MA Department of Radiology, Harvard Medical School, Boston MA Department of Cardiology, Boston Children's Hospital, Boston MA Department of Pediatrics, Harvard Medical School, Boston MA Department of Biostatistics, Harvard T. H. Chan School of Public Health, Boston MA AN - 112039048. Language: English. Entry Date: 20160826. Revision Date: 20190517. Publication Type: journal article AU - Bellinger, David C. AU - Watson, Christopher G. AU - Rivkin, Michael J. AU - Robertson, Richard L. AU - Roberts, Amy E. AU - Stopp, Christian AU - Dunbar‐Masterson, Carolyn AU - Bernson, Dana AU - DeMaso, David R. AU - Wypij, David AU - Newburger, Jane W. DB - ccm DO - 10.1161/JAHA.115.002302 DP - EBSCOhost IS - 12 KW - Cognition Disorders -- Etiology Cardiopulmonary Bypass -- Adverse Effects Heart Ventricle -- Abnormalities Brain -- Pathology Neuroradiography Adolescence Heart Ventricle -- Surgery Child Young Adult Female Hereditary Diseases -- Complications Neuropsychological Tests Cross Sectional Studies Male Hereditary Diseases Magnetic Resonance Imaging Human Funding Source N1 - research; tables/charts. Journal Subset: Biomedical; Europe; UK & Ireland. Grant Information: R01 HL096825/HL/NHLBI NIH HHS/United States. NLM UID: 101580524. PMID: NLM26667085. PY - 2015 SN - 2047-9980 SP - 1-N.PAG ST - Neuropsychological Status and Structural Brain Imaging in Adolescents With Single Ventricle Who Underwent the Fontan Procedure T2 - Journal of the American Heart Association TI - Neuropsychological Status and Structural Brain Imaging in Adolescents With Single Ventricle Who Underwent the Fontan Procedure UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=112039048&site=ehost-live&scope=site VL - 4 ID - 1479 ER - TY - JOUR AB - Children with functionally univentricular hearts are now surviving into their third and fourth decades of life. Although survival alone is a remarkable achievement, a lot must still be done to improve the quality and duration of life after the Fontan operation. Challenges that may be faced by these patients include the impact of the Fontan operation on the liver and the density of bone, protein-losing enteropathy, and plastic bronchitis. Paediatric cardiologists are familiar with the haemodynamic issues inherent in Fontan physiology; however, training in cardiology is often not sufficient to give us a complete understanding of the pathophysiology of the complications or of the options for treatment. Collaboration with other subspecialists including gastroenterologists, endocrinologists, and pulmonologists is essential in order to provide the rigorous and nuanced care that our patients need and deserve. A clinic in which a patient can see multiple subspecialists, and in which the subspecialists, as a group, can discuss each patient, can provide a unique and valuable service for patients with a functionally univentricular heart. © 2011 Cambridge University Press. AD - D.J. Goldberg, Cardiac Center, Children's Hospital of Philadelphia, Division of Cardiology, 34th Street and Civic Center Boulevard, Philadelphia, PA 19146, United States AU - Goldberg, D. J. AU - Dodds, K. AU - Rychik, J. DB - Embase Medline DO - 10.1017/S1047951111001636 IS - SUPPL. 2 KW - article bone density bronchitis croupous bronchitis Fontan procedure heart single ventricle human liver cirrhosis patient care protein losing gastroenteropathy quality of life LA - English M3 - Article N1 - L363111230 2011-12-26 2012-01-03 PY - 2011 SN - 1047-9511 1467-1107 SP - 77-79 ST - New concepts: Development of a survivorship programme for patients with a functionally univentricular heart T2 - Cardiology in the Young TI - New concepts: Development of a survivorship programme for patients with a functionally univentricular heart UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L363111230 http://dx.doi.org/10.1017/S1047951111001636 VL - 21 ID - 1077 ER - TY - JOUR AB - Hypoplastic left heart syndrome (HLHS) was once a uniformly fatal disease, accounting the majority of neonatal deaths due to congenital heart defects. Twenty-five years of advances in the surgical and medical management of this disease have resulted in dramatic improvements in survival for these children. The goal for patients with HLHS should be survival of near 100% with a good quality of life. The advances described in this manuscript detail some of the new techniques used in the surgical and medical management. For infants undergoing staged reconstruction, the Norwood procedure is performed in the newborn period, followed by a hemi-Fontan operation at 6 months of age, and a modified Fontan operation at 1 to 2 years of age. A significant recent modification of the Norwood procedure is the placement of a right ventricle to pulmonary artery (RV-PA) conduit instead of a systemic artery to pulmonary shunt (modified Blalock-Taussig or BT shunt) as the source of pulmonary blood flow. Our recent experience with this modification demonstrated an incremental increase in survival, improved postoperative stability, and decreased inter-stage mortality. At catheterization, significant differences in hemodynamic parameters were present that were consistent with improved coronary blood flow, decreased volume load to the single right ventricle, and improved pulmonary artery growth in those patients with the RV-PA conduit. The 3rd stage in the surgical treatment of HLHS is the modified Fontan operation or cavo-pulmonary anastamosis. A new approach for Fontan completion is the placement of a stent covered with a thin layer of Goretex from the inferior vena cava to the hemi-Fontan baffle. This can be performed in the catheterization laboratory, with a decrease in post-procedure pleural effusions, lower mortality, and a shorter length of stay. The future of treatment for HLHS may eventually involve a single open-heart procedure with initial and final interventions being performed in the catheterization laboratory. Some cardiac centers perform transplantation for management of HLHS. Survival following transplantation has improved as advances in the pre- and post-operative management continue, along with new options for immunosuppression. Treatment for HLHS continues to evolve, as ongoing work has resulted in improved short and long-term survival. The future for children with this disease is encouraging as evidenced by the remarkable achievements made to date and the current worldwide interest and study of HLHS. AD - Nemours Cardiac Center, duPont Hospital for Children, Wilmington, DE 19899, USA. kmaher@nemours.org AN - 15249913 AU - Maher, K. O. AU - Gidding, S. S. AU - Baffa, J. M. AU - Pizarro, C. AU - Norwood, W. I., Jr. DA - Feb DB - PubMed DP - NLM ET - 2004/07/14 IS - 1 KW - Cardiac Surgical Procedures/*trends Child, Preschool Humans Hypoplastic Left Heart Syndrome/*surgery Infant LA - eng N1 - Maher, K O Gidding, S S Baffa, J M Pizarro, C Norwood, W I Jr Journal Article Italy Minerva Pediatr. 2004 Feb;56(1):41-9. PY - 2004 SN - 0026-4946 (Print) 0026-4946 SP - 41-9 ST - New developments in the treatment of hypoplastic left heart syndrome T2 - Minerva Pediatr TI - New developments in the treatment of hypoplastic left heart syndrome VL - 56 ID - 273 ER - TY - JOUR AB - INTRODUCTION: Despite an abundance of prior Fontan simulation articles, there have been relatively few clinical advances that are a direct result of computational methods. We address a few key limitations of previous Fontan simulations as a step towards increasing clinical relevance. Previous simulations have been limited in scope because they have primarily focused on a single energy loss parameter. We present a multi-parameter approach to Fontan modeling that establishes a platform for clinical decision making and comprehensive evaluation of proposed interventions. METHODS: Time-dependent, 3-D blood flow simulations were performed on six patient-specific Fontan models. Key modeling advances include detailed pulmonary anatomy, catheterization-derived pressures, and MRI-derived flow with respiration. The following performance parameters were used to rank patients at rest and simulated exercise from best to worst performing: energy efficiency, inferior and superior vena cava (IVC, SVC) pressures, wall shear stress, and IVC flow distribution. RESULTS: Simulated pressures were well matched to catheterization data, but low Fontan pressure did not correlate with high efficiency. Efficiency varied from 74% to 96% at rest, and from 63% to 91% with exercise. Distribution of IVC flow ranged from 88%/12% (LPA/RPA) to 53%/47%. A "transcatheter" virtual intervention demonstrates the utility of computation in evaluating interventional strategies, and is shown to result in increased energy efficiency. CONCLUSIONS: A multiparameter approach demonstrates that each parameter results in a different ranking of Fontan performance. Ranking patients using energy efficiency does not correlate with the ranking using other parameters of presumed clinical importance. As such, current simulation methods that evaluate energy dissipation alone are not sufficient for a comprehensive evaluation of new Fontan designs. AD - Mechanical and Aerospace Engineering Department, University of California, San Diego, CA 92093-0411, USA. amarsden@ucsd.edu AN - 20412482 AU - Marsden, A. L. AU - Reddy, V. M. AU - Shadden, S. C. AU - Chan, F. P. AU - Taylor, C. A. AU - Feinstein, J. A. DA - Mar-Apr DB - PubMed DO - 10.1111/j.1747-0803.2010.00383.x DP - NLM ET - 2010/04/24 IS - 2 KW - Adolescent Adult Blood Flow Velocity Child Child, Preschool *Computer Simulation Exercise Female *Fontan Procedure/methods Hemodynamics Humans Magnetic Resonance Angiography Male *Models, Cardiovascular Pulmonary Artery Respiration Tomography, X-Ray Computed Venae Cavae/pathology Young Adult LA - eng N1 - 1747-0803 Marsden, Alison L Reddy, V Mohan Shadden, Shawn C Chan, Frandics P Taylor, Charles A Feinstein, Jeffrey A Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, Non-P.H.S. United States Congenit Heart Dis. 2010 Mar-Apr;5(2):104-17. doi: 10.1111/j.1747-0803.2010.00383.x. PY - 2010 SN - 1747-079x SP - 104-17 ST - A new multiparameter approach to computational simulation for Fontan assessment and redesign T2 - Congenit Heart Dis TI - A new multiparameter approach to computational simulation for Fontan assessment and redesign VL - 5 ID - 392 ER - TY - JOUR AB - The limitations of geometry assumptions in 2-dimensional echocardiographic assessment of the single ventricle (SV) have been overcome by recent advances in 3-D echocardiography. Improved reproducibility for measuring ventricular volumes and ejection fraction using 3-D echocardiography makes it ideally suited for serial monitoring of SV systolic function and should be considered in routine echocardiography imaging protocols for SV. The moderate correlation of Doppler derived E/e' ratio with invasive ventricular end diastolic pressure in SV, suggests it might be useful in the assessment of SV diastolic function. Speckle tracking imaging is intensely studied and promises to be a simple and repeatable imaging tool for quantifying SV function. In contrast, the advances in cardiac magnetic resonance imaging techniques promise to offer insights into the pathogenesis of myocardial dysfunction in SV. Late gadolinium enhancement imaging is a robust tool in assessing macroscopic myocardial scarring and T1 mapping and stress perfusion imaging are newer modalities that might improve understanding of the mechanisms in progressive myocardial dysfunction in SV hearts. AD - Stollery Children's Hospital, University of Alberta, Edmonton, Alberta, Canada. khoo@ualberta.ca AN - 23706785 AU - Khoo, N. S. AU - Tham, E. B. AU - Kantor, P. F. DA - Jul DB - PubMed DO - 10.1016/j.cjca.2013.03.025 DP - NLM ET - 2013/05/28 IS - 7 KW - Adult Cardiac Imaging Techniques/*methods Child Echocardiography/methods Heart Defects, Congenital/*physiopathology Humans Magnetic Resonance Imaging/methods Ventricular Function/*physiology LA - eng N1 - 1916-7075 Khoo, Nee Scze Tham, Edythe B Kantor, Paul F Journal Article Review England Can J Cardiol. 2013 Jul;29(7):886-9. doi: 10.1016/j.cjca.2013.03.025. Epub 2013 May 22. PY - 2013 SN - 0828-282x SP - 886-9 ST - Newer imaging modalities in the assessment of heart function in single ventricle hearts T2 - Can J Cardiol TI - Newer imaging modalities in the assessment of heart function in single ventricle hearts VL - 29 ID - 248 ER - TY - JOUR AB - Objectives: We retrospectively analyzed lung biopsy specimens from patients who underwent the Fontan procedure to identify predictive markers of outcome. Methods: We studied the intra-acinar pulmonary arteries present in lung biopsy specimens from 17 patients undergoing the Fontan procedure. We evaluated both their morphology and their expression of endothelial nitric oxide synthase and endothelin 1. We compared these data with those of 6 patients who died of no pulmonary cause (control group). Results: Eight patients had a good surgical outcome (group 1). Their distal arteries were thin and weakly expressed endothelin 1 and endothelial nitric oxide synthase. The procedure failed in 9 patients (group 2). Their distal arteries displayed muscle extension with an increased wall thickness (P < .01 vs group 1). Their endothelin 1 expression remained low (not significant vs group 1). By contrast, endothelial nitric oxide synthase was markedly overexpressed (P < .001 vs group 1). Conclusion: Distal pulmonary arteries of patients in whom the Fontan procedure failed exhibited a markedly increased wall thickness and a clear endothelial nitric oxide synthase overexpression. In addition to giving clues to the pathogenesis of the procedure's failure, our study might help to define reliable predictive markers of its outcome. AD - Service de Chirurgie Cardiaque, UFR Biomedicale des Saints Peres, Faculté Paris V, Paris, France Hôpital Necker-Enfants Malades, Service d'Anatomo-Pathologie, UFR Biomedicale des Saints Peres, Paris, France Hopital Europeen Georges Pompidou, Laboratoire d'Immunologie Pulmonaire, UFR Biomedicale des Saints Peres, Paris, France Hôpital Necker-Enfants Malades, 149 rue de Sevres, 75015 Paris, France AU - Lévy, M. AU - Danel, C. AU - Laval, A. M. AU - Leca, F. AU - Vouhé, P. R. AU - Israël-Biet, D. DB - Scopus DO - 10.1067/mtc.2003.193 IS - 5 M3 - Article N1 - Cited By :31 Export Date: 15 June 2020 PY - 2003 SP - 1083-1090 ST - Nitric oxide synthase expression by pulmonary arteries: A predictive marker of Fontan procedure outcome? T2 - Journal of Thoracic and Cardiovascular Surgery TI - Nitric oxide synthase expression by pulmonary arteries: A predictive marker of Fontan procedure outcome? UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-19244383546&doi=10.1067%2fmtc.2003.193&partnerID=40&md5=650e603ab571a7be298a764f2ac7fe99 VL - 125 ID - 2262 ER - TY - JOUR DB - Medline IS - 37 KW - article attitude to death case report female home delivery human hypoplastic left heart syndrome male mother newborn nurse midwife nursing pregnancy psychological aspect LA - English M3 - Article N1 - L126265895 1996-09-04 PY - 1996 SN - 1522-2888 SP - 25-26 ST - No heroics, just love T2 - Midwifery today and childbirth education TI - No heroics, just love UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L126265895 ID - 1363 ER - TY - JOUR AB - Cardiovascular simulations provide a promising means to predict risk of thrombosis in grafts, devices, and surgical anatomies in adult and pediatric patients. Although the pathways for platelet activation and clot formation are not yet fully understood, recent findings suggest that thrombosis risk is increased in regions of flow recirculation and high residence time (RT). Current approaches for calculating RT are typically based on releasing a finite number of Lagrangian particles into the flow field and calculating RT by tracking their positions. However, special care must be taken to achieve temporal and spatial convergence, often requiring repeated simulations. In this work, we introduce a non-discrete method in which RT is calculated in an Eulerian framework using the advection-diffusion equation. We first present the formulation for calculating residence time in a given region of interest using two alternate definitions. The physical significance and sensitivity of the two measures of RT are discussed and their mathematical relation is established. An extension to a point-wise value is also presented. The methods presented here are then applied in a 2D cavity and two representative clinical scenarios, involving shunt placement for single ventricle heart defects and Kawasaki disease. In the second case study, we explored the relationship between RT and wall shear stress, a parameter of particular importance in cardiovascular disease. © 2013 AIP Publishing LLC. AD - Department of Mechanical and Aerospace Engineering, University of California, San Diego, CA 92093-0411, United States Cardiac Unit, Great Ormond Street Hospital for Children, Institute of Child Health, London WC1N 1EH, United Kingdom AU - Esmaily-Moghadam, M. AU - Hsia, T. AU - Marsden, A. L. C7 - 110802 DB - Scopus DO - 10.1063/1.4819142 IS - 11 M3 - Article N1 - Cited By :25 Export Date: 15 June 2020 PY - 2013 ST - A non-discrete method for computation of residence time in fluid mechanics simulations T2 - Physics of Fluids TI - A non-discrete method for computation of residence time in fluid mechanics simulations UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84888807291&doi=10.1063%2f1.4819142&partnerID=40&md5=4f77e1ef9ba0f49d93ff36857bbbc503 VL - 25 ID - 2039 ER - TY - JOUR AB - Cardiovascular simulations provide a promising means to predict risk of thrombosis in grafts, devices, and surgical anatomies in adult and pediatric patients. Although the pathways for platelet activation and clot formation are not yet fully understood, recent findings suggest that thrombosis risk is increased in regions of flow recirculation and high residence time (RT). Current approaches for calculating RT are typically based on releasing a finite number of Lagrangian particles into the flow field and calculating RT by tracking their positions. However, special care must be taken to achieve temporal and spatial convergence, often requiring repeated simulations. In this work, we introduce a non-discrete method in which RT is calculated in an Eulerian framework using the advection-diffusion equation. We first present the formulation for calculating residence time in a given region of interest using two alternate definitions. The physical significance and sensitivity of the two measures of RT are discussed and their mathematical relation is established. An extension to a point-wise value is also presented. The methods presented here are then applied in a 2D cavity and two representative clinical scenarios, involving shunt placement for single ventricle heart defects and Kawasaki disease. In the second case study, we explored the relationship between RT and wall shear stress, a parameter of particular importance in cardiovascular disease. AD - Department of Mechanical and Aerospace Engineering, University of California, San Diego, California 92093-0411, USA. AN - 24046509 AU - Esmaily-Moghadam, M. AU - Hsia, T. Y. AU - Marsden, A. L. C2 - PMC3765298 DA - Nov DB - PubMed DO - 10.1063/1.4819142 DP - NLM ET - 2013/09/21 IS - 11 LA - eng N1 - 1089-7666 Esmaily-Moghadam, Mahdi Hsia, Tain-Yen Marsden, Alison L FS/12/35/29566/British Heart Foundation/United Kingdom R21 HL102596/HL/NHLBI NIH HHS/United States Journal Article Phys Fluids (1994). 2013 Nov;25(11):110802. doi: 10.1063/1.4819142. Epub 2013 Aug 23. PY - 2013 SN - 1070-6631 (Print) 1070-6631 SP - 110802 ST - A non-discrete method for computation of residence time in fluid mechanics simulations T2 - Phys Fluids (1994) TI - A non-discrete method for computation of residence time in fluid mechanics simulations VL - 25 ID - 517 ER - TY - JOUR AB - OBJECTIVE: To describe the first clinical application of a novel tissue Doppler derived index of contractility, isovolumic acceleration (IVA), in the assessment of the ventricular myocardial force-frequency relation (FFR) in the univentricular heart (UVH). DESIGN: Prospective study. SETTING: Tertiary referral centre. INTERVENTIONS: Non-invasive assessment of the myocardial FFR by tissue Doppler echocardiography during atrial pacing. RESULTS: IVA was used to measure the FFR of the systemic ventricle in patients with structurally normal hearts and in patients with UVHs. Basal IVA of the normal hearts (mean (SD) 1.9 (0.3) m/s2) was significantly greater than that of UVHs in patients with a dominant right ventricle (RV) (1.0 (0.3) m/s2) or left ventricle (LV) (0.8 (0.7) m/s2; p < 0.05 for both). Neither the absolute nor percentage change from basal to peak values of IVA with pacing differed between the three groups. Peak force developed by the normal LV was significantly greater than that of the UVH, dominant LV group but not different from that of the UVH, dominant RV group. CONCLUSION: Contractility at basal heart rate is depressed in patients with UVH compared with the normal LV. Analysis of ventricular FFRs exposes further differences in myocardial contractility. There is no evidence that contractile function of the dominant RV is inferior to that of the dominant LV over a physiological range of heart rates. AD - Division of Cardiology, Hospital for Sick Children, Toronto, Ontario, M5G 1X8, Canada. AN - 16162630 AU - Cheung, M. M. AU - Smallhorn, J. F. AU - McCrindle, B. W. AU - Van Arsdell, G. S. AU - Redington, A. N. C2 - PMC1769147 DA - Oct DB - PubMed DO - 10.1136/hrt.2004.048207 DP - NLM ET - 2005/09/16 IS - 10 KW - Adolescent Cardiac Pacing, Artificial Child *Echocardiography, Doppler Heart Defects, Congenital/diagnostic imaging/*physiopathology Heart Rate/physiology Heart Ventricles/abnormalities Humans Myocardial Contraction/physiology Pacemaker, Artificial Prospective Studies Stroke Volume LA - eng N1 - 1468-201x Cheung, M M H Smallhorn, J F McCrindle, B W Van Arsdell, G S Redington, A N Evaluation Study Journal Article Research Support, Non-U.S. Gov't Heart. 2005 Oct;91(10):1338-42. doi: 10.1136/hrt.2004.048207. PY - 2005 SN - 1355-6037 (Print) 1355-6037 SP - 1338-42 ST - Non-invasive assessment of ventricular force-frequency relations in the univentricular circulation by tissue Doppler echocardiography: a novel method of assessing myocardial performance in congenital heart disease T2 - Heart TI - Non-invasive assessment of ventricular force-frequency relations in the univentricular circulation by tissue Doppler echocardiography: a novel method of assessing myocardial performance in congenital heart disease VL - 91 ID - 429 ER - TY - JOUR AB - We present the case histories of two children having respiratory failure due to bilateral diaphragm paralysis after cardiac surgery. In both children non-invasive positive pressure ventilation alleviated respiratory distress, improved gas exchange, and prevented the need for endotracheal intubation. Following unilateral recovery of diaphragmatic function both children were successfully weaned from non-invasive positive pressure ventilation. © 2009 Published by European Association for Cardio-Thoracic Surgery. All rights reserved. AD - L. Kovacikova, Intensive Care Unit, Pediatric Cardiac Center, Limbova 1, 833 51 Bratislava, Slovakia AU - Kovacikova, L. AU - Dobos, D. AU - Zahorec, M. DB - Embase Medline DO - 10.1510/icvts.2008.187096 IS - 1 KW - article artificial ventilation Blalock Taussig shunt breathing rate case report child chylothorax diaphragm paralysis endotracheal intubation Fontan procedure Glenn shunt heart atrioventricular node heart septum defect heart surgery human infant lung artery banding lung gas exchange male non invasive procedure parenteral nutrition peak inspiratory flow positive end expiratory pressure postoperative complication preschool child priority journal respiratory distress respiratory failure respiratory tract infection ventilator Servo-i LA - English M3 - Article N1 - L354056570 2009-02-03 http://icvts.ctsnetjournals.org/cgi/reprint/8/1/171?ck=nck PY - 2009 SN - 1569-9293 SP - 171-172 ST - Non-invasive positive pressure ventilation for bilateral diaphragm paralysis after pediatric cardiac surgery T2 - Interactive Cardiovascular and Thoracic Surgery TI - Non-invasive positive pressure ventilation for bilateral diaphragm paralysis after pediatric cardiac surgery UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L354056570 http://dx.doi.org/10.1510/icvts.2008.187096 VL - 8 ID - 1191 ER - TY - JOUR AB - Background In Fontan patients with atrial arrhythmias (AA), non-Vitamin K antagonist oral anticoagulants(NOACs) have a class III recommendation according to the Pediatric & Congenital Electrophysiology Society (PACES)/Heart Rhythm Society (HRS) guideline in 2014, due to lack of data on outcomes as opposed to evidence of harm. To address this gap in data, we investigated the safety and efficacy of NOACs in adults with a Fontan circulation in a worldwide study. Methods This is an international multicentre prospective cohort study, using data from the NOTE (non-Vitamin K antagonist oral anticoagulants for thromboembolic prevention in patients with congenital heart disease) registry. The study population comprised consecutive adults with a Fontan circulation using NOACs. Follow-up took place at 6 months and yearly thereafter. The primary endpoints were thromboembolism and major bleeding. Secondary endpoint was minor bleeding. Results From April 2014 onward, 74 patients (mean age 32±10 years (range 18-68), 54% male) with a Fontan circulation using NOACs were included. During a median follow-up of 1.2 (IQR 0.8-2.0) years, three thromboembolic events (2.9 per 100 patient-years (95% CI 0.7 to 7.6)) and three major bleedings (2.9 per 100 patient-years (95% CI 0.7 to 7.6)) occurred in five atriopulmonary Fontan and one total cavopulmonary connection Fontan patients with AA. Fifteen patients experienced minor bleeding episodes (15.8 per 100 patient-years (95% CI 9.1 to 25.2)). In patients (n=37) using Vitamin K antagonists (VKAs) prior to the initiation of NOAC, annual incidence of historical thromboembolic events and major bleeding were 2.4% (95% CI 0.4% to 7.4%) (n = 2) and 1.2% (95% CI 0.7% to 5.1%) (n = 1), respectively. Conclusions In this review of the largest Fontan cohort using NOACs with prospective follow-up, NOACs appear to be well tolerated and their efficacy and safety during short-term follow-up seem comparable to VKAs. Longer term data are required to confirm these promising short-term results. AD - H. Yang, Deparment of Cardiology, AMC, Amsterdam, Netherlands AU - Yang, H. AU - Veldtman, G. R. AU - Bouma, B. J. AU - Budts, W. AU - Niwa, K. AU - Meijboom, F. AU - Scognamiglio, G. AU - Egbe, A. C. AU - Schwerzmann, M. AU - Broberg, C. AU - Morissens, M. AU - Buber, J. AU - Tsai, S. AU - Polyzois, I. AU - Post, M. C. AU - Greutmann, M. AU - Van Dijk, A. AU - Mulder, B. J. M. AU - Aboulhosn, J. DB - Embase DO - 10.1136/openhrt-2018-000985 IS - 1 KW - acetylsalicylic acid anticoagulant agent antithrombocytic agent antivitamin K dabigatran edoxaban nonsteroid antiinflammatory agent rivaroxaban adult aged alcohol consumption article atriopulmonary connection bleeding brain ischemia cavopulmonary connection cerebrovascular accident CHA2DS2-VASc score congenital heart disease controlled study deep vein thrombosis double inlet left ventricle drug efficacy drug safety epistaxis female follow up Fontan procedure gastrointestinal hemorrhage gingiva bleeding headache heart atrium arrhythmia heart failure heart right ventricle double outlet hematoma human hypertension inferior cava vein international normalized ratio intracardiac thrombosis kidney function liver function lung embolism major clinical study male menorrhagia multicenter study observational study postoperative hemorrhage primary prevention priority journal prospective study pulmonary valve atresia risk factor secondary prevention skin bleeding superior cava vein survival analysis thromboembolism thrombosis prevention transient ischemic attack tricuspid valve atresia vasculitis vein thrombosis LA - English M3 - Article N1 - L627945506 2019-06-14 2019-06-19 PY - 2019 SN - 2053-3624 ST - Non-Vitamin K antagonist oral anticoagulants in adults with a Fontan circulation: Are they safe T2 - Open Heart TI - Non-Vitamin K antagonist oral anticoagulants in adults with a Fontan circulation: Are they safe UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L627945506 http://dx.doi.org/10.1136/openhrt-2018-000985 VL - 6 ID - 614 ER - TY - JOUR AB - Outcomes following cardiac intensive care unit (CICU) admission are influenced by many factors including initial cardiac diagnosis, surgical complexity, and burden of critical illness. Additionally, the presence of noncardiac issues may have a significant impact on outcomes and the patient experience during and following an intensive care unit stay. This review focuses on three common noncardiac areas which impact outcomes and patient experience in and beyond the CICU: feeding and growth, pain and analgesia, and anticoagulation. Growth failure and feeding dysfunction are commonly encountered in infants requiring cardiac surgery and have been associated with worse surgical and developmental outcomes. Recent studies most notably in the single ventricle population have demonstrated improved weight gain and outcomes when feeding protocols are implemented. Children undergoing cardiac surgery may experience both acute and chronic pain. Emerging research is investigating the impact of sedatives and analgesics on neurodevelopmental outcomes and quality of life. Improved pain scores and standardized management of pain and withdrawal may improve the patient experience and outcomes. Effective anticoagulation is a critical component of perioperative care but may be complicated by inflammation, multiorgan dysfunction, and patient factors. Advances in monitoring of anticoagulation and emerging therapies are reviewed. AD - Nemours Cardiac Center, AI Dupont Hospital for Children, Thomas Jefferson Medical College, Philadelphia, PA, USA david.hehir@nemours.org. Department of Anesthesiology, Baylor College of Medicine, Texas Children's Hospital, Houston, TX, USA Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, TX, USA. The Heart Institute, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH, USA. AN - 26957404 AU - Hehir, D. A. AU - Easley, R. B. AU - Byrnes, J. DA - Mar DB - PubMed DO - 10.1177/2150135115615847 DP - NLM ET - 2016/03/10 IS - 2 KW - Analgesia/methods Anticoagulants/*therapeutic use *Cardiac Surgical Procedures Child Child, Preschool Coronary Care Units Enterocolitis, Necrotizing/*epidemiology Failure to Thrive/epidemiology/therapy Feeding Methods Growth Disorders/*epidemiology/therapy Heart Defects, Congenital/*surgery Heart Ventricles Hospitalization Humans Infant Intensive Care Units, Pediatric Pain Management Pain, Postoperative/*therapy Perioperative Care Postoperative Complications/*epidemiology/therapy Quality of Life Thrombosis/drug therapy/*epidemiology/prevention & control blood coagulation/anticoagulation congenital heart disease intensive care nutrition pain LA - eng N1 - 2150-136x Hehir, David A Easley, R Blaine Byrnes, Jonathan Journal Article Review United States World J Pediatr Congenit Heart Surg. 2016 Mar;7(2):199-209. doi: 10.1177/2150135115615847. PY - 2016 SN - 2150-1351 SP - 199-209 ST - Noncardiac Challenges in the Cardiac ICU: Feeding, Growth and Gastrointestinal Complications, Anticoagulation, and Analgesia T2 - World J Pediatr Congenit Heart Surg TI - Noncardiac Challenges in the Cardiac ICU: Feeding, Growth and Gastrointestinal Complications, Anticoagulation, and Analgesia VL - 7 ID - 194 ER - TY - JOUR AB - Although aortic valve anatomy is three-dimensionally complex, careful echocardiographic investigation can yield surgically relevant information. The promise of intravascular and transesophageal imaging is in yielding the raw data of sufficient resolution to make the three-dimensional reconstruction clinically meaningful. AD - S.P. Sanders, Clinique de Genolier, A. C. Congenital Heart Disease Inst., CH-1261 Genolier, Switzerland AU - Sanders, S. P. AU - Simonds, H. M. AU - Jameson, S. M. DB - Embase DO - 10.1111/j.1540-8175.1996.tb00904.x IS - 3 KW - aortic valve disease aortic valve prolapse aortic valve stenosis article child color Doppler flowmetry Doppler echocardiography human hypoplastic left heart syndrome image reconstruction infant Marfan syndrome priority journal three-dimensional imaging Williams Beuren syndrome LA - English M3 - Article N1 - L26152359 1996-06-02 PY - 1996 SN - 0742-2822 SP - 315-323 ST - Noninvasive evaluation of aortic valve anatomy T2 - Echocardiography TI - Noninvasive evaluation of aortic valve anatomy UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L26152359 http://dx.doi.org/10.1111/j.1540-8175.1996.tb00904.x VL - 13 ID - 1361 ER - TY - JOUR AB - We report a case of severe hyperthermia in a 6-month-old boy with a single ventricle, dextrocardia, asplenia, and transposition of the great arteries, during induction of anesthesia on three separate occasions. To our knowledge, this is the first case reported of repeated intraoperative hyperthermia not related to malignant hyperthermia, infection, neuroendocrine tumor, or iatrogenic causes (e.g., anticholinergic blockade or warming devices). The severe hyperthermia may be secondary to the medications given before and during induction and/or the stress of the induction. Among the induction medications given during the three episodes, fentanyl is the most likely contributing drug. Human data indicate that opioids increase the sweating threshold and decrease the vasoconstriction and shivering thresholds. The medications could cause a widening in the thermoregulation interthreshold and the stress could induce nonshivering thermogenesis. AD - J.S. Jahr, Department of Anesthesiology, University of California, Davis Medical Center, 4150 'V' St., Sacramento, CA 95817, United States AU - Leung, W. K. AU - Jahr, J. S. AU - Hotz, J. AU - Pollock, M. DB - Embase Medline DO - 10.1016/S0952-8180(98)00059-2 IS - 5 KW - acetylsalicylic acid altizide plus spironolactone amoxicillin anesthetic agent cefuroxime digoxin erythromycin etomidate fentanyl furosemide methylprednisolone midazolam neuromuscular blocking agent pancuronium paracetamol rocuronium sulfafurazole anesthesia complication anesthesia induction article cardiovascular disease case report chronic otitis media congenital heart disease general anesthesia human hyperthermia infant inhalational drug administration intraoperative period intravenous drug administration male pediatric anesthesia pediatric surgery priority journal respiratory tract infection surgical stress thermogenesis LA - English M3 - Article N1 - L28361628 1998-08-24 PY - 1998 SN - 0952-8180 SP - 427-431 ST - Nonmalignant hyperthermia on induction of anesthesia in a pediatric patient undergoing Bi-directional glenn procedure T2 - Journal of Clinical Anesthesia TI - Nonmalignant hyperthermia on induction of anesthesia in a pediatric patient undergoing Bi-directional glenn procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L28361628 http://dx.doi.org/10.1016/S0952-8180(98)00059-2 VL - 10 ID - 1348 ER - TY - JOUR AB - We report a case of severe hyperthermia in a 6-month-old boy with a single ventricle, dextrocardia, asplenia, and transposition of the great arteries, during induction of anesthesia on three separate occasions. To our knowledge, this is the first case reported of repeated intraoperative hyperthermia not related to malignant hyperthermia, infection, neuroendocrine tumor, or iatrogenic causes (e.g., anticholinergic blockade or warming devices). The severe hyperthermia may be secondary to the medications given before and during induction and/or the stress of the induction. Among the induction medications given during the three episodes, fentanyl is the most likely contributing drug. Human data indicate that opioids increase the sweating threshold and decrease the vasoconstriction and shivering thresholds. The medications could cause a widening in the thermoregulation interthreshold and the stress could induce nonshivering thermogenesis. AD - Department of Anesthesiology, University of California, Davis Medical Center, Sacramento 95817, USA. AN - 9702626 AU - Leung, W. K. AU - Jahr, J. S. AU - Hotz, J. AU - Pollock, M. DA - Aug DB - PubMed DO - 10.1016/s0952-8180(98)00059-2 DP - NLM ET - 1998/08/14 IS - 5 KW - Adjuvants, Anesthesia/adverse effects Anastomosis, Surgical/*methods Anesthesia, General/*adverse effects Anesthetics, Intravenous/adverse effects Body Temperature Regulation/drug effects Dextrocardia/surgery Fentanyl/adverse effects Fever/*etiology Heart Defects, Congenital/*surgery Heart Ventricles/abnormalities/surgery Humans Infant *Intraoperative Complications Male Narcotics/adverse effects Pulmonary Artery/*surgery Shivering/drug effects Spleen/abnormalities Stress, Physiological/physiopathology Sweating/drug effects Transposition of Great Vessels/surgery Vasoconstriction/drug effects Vena Cava, Superior/*surgery LA - eng N1 - Leung, W K Jahr, J S Hotz, J Pollock, M Case Reports Journal Article United States J Clin Anesth. 1998 Aug;10(5):427-31. doi: 10.1016/s0952-8180(98)00059-2. PY - 1998 SN - 0952-8180 (Print) 0952-8180 SP - 427-31 ST - Nonmalignant hyperthermia on induction of anesthesia in a pediatric patient undergoing bidirectional Glenn procedure T2 - J Clin Anesth TI - Nonmalignant hyperthermia on induction of anesthesia in a pediatric patient undergoing bidirectional Glenn procedure VL - 10 ID - 516 ER - TY - JOUR AB - Objective Transanal endorectal pull-through (ERPT) is the most popular technique to treat Hirschsprung disease (HD). Still, there is limited knowledge on long-term bowel function. This cross-sectional, multicenter study assessed long-term bowel function in a large HD population and examined predictors of poor outcome. Methods Patients older than four years or their parents filled out a validated questionnaire on bowel function. Clinical details were recorded retrospectively from medical records. Results 73/200 (37%) patients reported absolutely no impaired bowel function, meaning no constipation, fecal accidents, stoma, appendicostomy or need for enemas. Seven (4%) had a stoma, and 33 (17%) used antegrade or rectal colonic enemas. Most disarrangements of fecal control and constipation were significantly less common in older age group, but abnormal defecation frequency and social problems remained unchanged. Syndromic patients (n = 31) experienced frequent fecal accidents (46%) more often than nonsyndromic (14%, P < 0.001). Having a syndrome (adjusted OR 5.6, 95% CI 2.1–15, P = 0.001) or a complete transanal ERPT (adjusted OR 2.4, 95% CI 1.1–5.7, P = 0.038) was significantly associated with poor outcome defined as having a stoma, an appendicostomy, daily fecal accidents or need of regular rectal wash outs. Conclusion A significant number of HD patients experience bowel problems many years after definite surgery. Fecal control was significantly better in older than younger HD patients, but some continued to have considerable bowel problems also as adults. A total transanal ERPT was associated with poorer outcome. Long-term follow-up of HD patients is warranted. Prognosis Study: Level II. AD - K. Bjørnland, Department of Paediatric Surgery, Oslo University Hospital Rikshospitalet, Postboks 4950, Oslo, Norway AU - Bjørnland, K. AU - Pakarinen, M. P. AU - Stenstrøm, P. AU - Stensrud, K. J. AU - Neuvonen, M. AU - Granström, A. L. AU - Graneli, C. AU - Pripp, A. H. AU - Arnbjörnsson, E. AU - Emblem, R. AU - Wester, T. AU - Rintala, R. J. DB - Embase Medline DO - 10.1016/j.jpedsurg.2017.01.001 IS - 9 KW - botulinum toxin enema abdominal discomfort age anastomosis leakage antibiotic therapy article child clinical outcome congenital central hypoventilation syndrome constipation cross-sectional study defecation Down syndrome enterocolitis enterostomy feces incontinence female Hirschsprung disease human hypoplastic left heart syndrome intestine function intestine obstruction lactose intolerance major clinical study male medical record review multicenter study muscle resection postoperative complication predictive value priority journal pull through operation retrospective study sex difference social problem stoma treatment outcome Wilson disease wound dehiscence wound infection LA - English M3 - Article N1 - L614111681 2017-01-24 2019-10-31 PY - 2017 SN - 1531-5037 0022-3468 SP - 1458-1464 ST - A Nordic multicenter survey of long-term bowel function after transanal endorectal pull-through in 200 patients with rectosigmoid Hirschsprung disease T2 - Journal of Pediatric Surgery TI - A Nordic multicenter survey of long-term bowel function after transanal endorectal pull-through in 200 patients with rectosigmoid Hirschsprung disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L614111681 http://dx.doi.org/10.1016/j.jpedsurg.2017.01.001 VL - 52 ID - 733 ER - TY - JOUR AB - This study provides Rorschach data for 88 adults aged 18–65 years from the general population in England. The sample was matched as closely as possible with census data on the variables of gender, age, marital status, ethnicity, geographical location, occupation, and level of education. The Rorschach was administered according to the Comprehensive System by five experienced psychologists. Participants also completed a measure of psychological distress called the CORE. Interscorer reliability was found to be excellent for all variables apart from the six cognitive special scores, for which it was fair. Rorschach data are presented for Comprehensive System variables and compared with Exner’s (2007) sample of 450 nonpatient adults in the United States and with the international reference sample (Meyer, Erdberg, & Shaffer, 2007). (PsycINFO Database Record (c) 2018 APA, all rights reserved) AD - Carstairs, Kari, 7 Mayfield Road, Bromley, United Kingdom, BR1 2HB AN - 2018-58103-001 AU - Carstairs, Kari AU - Hartley, Sarah AU - Peden, Andrew AU - Woods, Justine McCarthy AU - van Graan, Andre AU - Andronikof, Anne AU - Fontan, Patrick DB - psyh DO - 10.1027/1192-5604/a000103 DP - EBSCOhost IS - 2 KW - England Rorschach Comprehensive System normative study Ethnic Identity Geography Marital Status Rorschach Test Test Norms Distress Educational Attainment Level Occupational Status N1 - Carstairs Psychological Associates Limited, Kent, United Kingdom. Other Publishers: Hogrefe & Huber Publishers. Release Date: 20181119. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Ethnic Identity; Geography; Marital Status; Rorschach Test; Test Norms. Minor Descriptor: Distress; Educational Attainment Level; Occupational Status. Classification: Research Methods & Experimental Design (2260). Population: Human (10); Male (30); Female (40). Location: England. Age Group: Adulthood (18 yrs & older) (300); Young Adulthood (18-29 yrs) (320); Thirties (30-39 yrs) (340); Middle Age (40-64 yrs) (360); Aged (65 yrs & older) (380). Tests & Measures: CORE-Short Form A; Rorschach Test DOI: 10.1037/t03306-000. Methodology: Empirical Study; Quantitative Study. Page Count: 30. Issue Publication Date: 2018. Publication History: Accepted Date: Apr 20, 2018; Revised Date: Mar 3, 2018; First Submitted Date: Dec 12, 2017. Copyright Statement: Hogrefe Publishing. 2018. PY - 2018 SN - 1192-5604 2151-206X SP - 105-134 ST - A normative study in England with the Rorschach comprehensive system T2 - Rorschachiana TI - A normative study in England with the Rorschach comprehensive system UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2018-58103-001&site=ehost-live&scope=site kari@carstairspsych.co.uk VL - 39 ID - 1671 ER - TY - JOUR AB - Objective: The introduction of right ventricle to pulmonary artery (RV-PA) conduit in the Norwood procedure for hypoplastic left heart syndrome resulted in a higher survival rate in many centers. A higher diastolic aortic pressure and a higher mean coronary perfusion pressure were suggested as the hemodynamic advantage of this source of pulmonary blood flow. The main objective of this study was the comparison of two models of Norwood physiology with different types of pulmonary blood flow sources and their hemodynamics. Method: Based on anatomic details obtained from echocardiographic assessment and angiographic studies, two three-dimensional computer models of post-Norwood physiology were developed. The finite-element method was applied for computational hemodynamic simulations. Norwood physiology with RV-PA 5-mm conduit and Blalock-Taussig shunt (BTS) 3.5-mm shunt were compared. Right ventricle work, wall stress, flow velocity, shear rate stress, energy loss and turbulence eddy dissipation were analyzed in both models. Results: The total work of the right ventricle after Norwood procedure with the 5-mm RV-PA conduit was lower in comparison to the 3.5-mm BTS while establishing an identical systemic blood flow. The Qp/Qs ratio was higher in the BTS group. Conclusions: Hemodynamic performance after Norwood with the RV-PA conduit is more effective than after Norwood with BTS. Computer simulations of complicated hemodynamics after the Norwood procedure could be helpful in establishing optimal post-Norwood physiology. © 2011 European Association for Cardio-Thoracic Surgery. AD - T. Mroczek, Department of Pediatric Cardiac Surgery, Collegium Medicum, Jagiellonian University Ul. Wielicka 265, 30 663 Krakow, Poland AU - Mroczek, T. AU - Małota, Z. AU - Wójcik, E. AU - Nawrat, Z. AU - Skalski, J. DB - Embase Medline DO - 10.1016/j.ejcts.2011.03.033 IS - 6 KW - angiography article biological model Blalock Taussig shunt blood pressure measurement computer simulation controlled study Doppler flowmetry echocardiography flow rate heart cycle heart hemodynamics heart right ventricle heart right ventricle pressure human hypoplastic left heart syndrome intermethod comparison lung blood flow major clinical study newborn Norwood procedure priority journal right ventricle to pulmonary artery conduit shear stress systemic circulation systole wall stress LA - English M3 - Article N1 - L51406339 2011-05-07 2011-12-13 PY - 2011 SN - 1010-7940 1873-734X SP - 1412-1418 ST - Norwood with right ventricle-to-pulmonary artery conduit is more effective than Norwood with Blalock-Taussig shunt for hypoplastic left heart syndrome: Mathematic modeling of hemodynamics T2 - European Journal of Cardio-thoracic Surgery TI - Norwood with right ventricle-to-pulmonary artery conduit is more effective than Norwood with Blalock-Taussig shunt for hypoplastic left heart syndrome: Mathematic modeling of hemodynamics UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51406339 http://dx.doi.org/10.1016/j.ejcts.2011.03.033 VL - 40 ID - 1081 ER - TY - JOUR AB - This report describes the case of a child with Kabuki syndrome, hypoplastic left heart syndrome, and right-sided partial anomalous pulmonary venous drainage to the inferior vena cava. The child underwent successful Norwood and Glenn palliative procedures. At the age of 9 months, the genetic condition was recognized. This represents the first report describing this combination of findings for Kabuki syndrome. © 2008 Springer Science+Business Media, LLC. AD - C. J. McMahon, Department of Paediatric Cardiology, Our Lady's Hospital for Sick Children, Crumlin, Dublin 12, Ireland AU - Shahdadpuri, R. AU - Lynch, S. A. AU - Murchan, H. AU - McMahon, C. J. DB - Embase Medline DO - 10.1007/s00246-008-9197-0 IS - 4 KW - angiocardiography aortic coarctation aortic valve stenosis article Blalock Taussig shunt cardiomegaly case report developmental disorder face dysmorphia genetic analysis Glenn shunt growth retardation heart catheterization heart right ventricle hypertrophy human hypoplastic left heart syndrome infant inferior cava vein Kabuki makeup syndrome levocardia lung vein drainage anomaly mitral valve stenosis Norwood procedure patent ductus arteriosus phenotype prenatal diagnosis systolic heart murmur thorax radiography transthoracic echocardiography LA - English M3 - Article N1 - L50068693 2008-08-05 PY - 2008 SN - 0172-0643 SP - 820-822 ST - A novel constellation of cardiac findings for Kabuki syndrome: Hypoplastic left heart syndrome and partial anomalous pulmonary venous drainage T2 - Pediatric Cardiology TI - A novel constellation of cardiac findings for Kabuki syndrome: Hypoplastic left heart syndrome and partial anomalous pulmonary venous drainage UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50068693 http://dx.doi.org/10.1007/s00246-008-9197-0 VL - 29 ID - 1205 ER - TY - JOUR AB - Objectives This study aimed to analyze the surface stress generated by a novel curved balloon and assess its efficacy for treating angular lesions associated with congenital heart disease. Background Obstructions at the anastomosis of aortopulmonary shunts and cavopulmonary connections may occur postoperatively. Catheter interventions are often performed for such lesions; however, acute angulation may cause balloon slippage or inappropriate stress on the vessel wall. Methods We dilated the curved balloon in a curved vessel model and measured the resultant wall stress and its distribution. Clinical evaluations were performed using this balloon in angled lesions. Results In the curved vessel model, curved balloons generated uniform stress on the lesser and greater curvatures (curved type, lesser/greater=0.343MPa/0.327MPa; P=0.61), whereas straight balloons caused disproportionate stress (straight type, lesser/greater=0.358MPa/0.254MPa; P=0.19). However, the difference in average stress was not statistically significant. Furthermore, the stress was uniform along the entire length of the curved balloon, but differed between the mid and end portions of the straight balloon. Curved balloon dilations were performed for 10 lesions in 7 patients. The curved balloon conformed well to the angulated lesion without slipping. The median percent change in the minimal lumen diameter (MLD) was 64% (range, 0-206%). In 5 lesions, MLD increased by ≥50%. Oxygen saturation increased by 5% (0-9%). Conclusions Although further clinical evaluation is necessary, this novel curved balloon may be a reasonable alternative in angled lesions, providing better conformability and preventing excessive stress to the vessel wall adjacent to the stenosis. (J Interven Cardiol 2014;27:408-413) © 2014, Wiley Periodicals, Inc. AD - H. Kise, Cardiovascular Center, Showa University, Northern Yokohama Hospital, 35-1 Chigasaki-chuo, Tsuzuki-ku, Yokohama 224-8503, Japan AU - Kise, H. AU - Miyata, A. AU - Tomita, H. AU - Fujii, T. AU - Fujimoto, K. AU - Kiguchi, H. AU - Oyama, N. AU - Soga, T. AU - Hata, Y. AU - Hirata, M. AU - Itoh, A. AU - Ishino, K. AU - Hasebe, Y. AU - Hoshiai, M. DB - Embase Medline DO - 10.1111/joic.12122 IS - 4 KW - Blalock Taussig shunt aortopulmonary shunt artery catheter cavopulmonary connection systemic pulmonary shunt elastomer heparin polyamide angiography aortography artery diameter artery lesion article ascending aorta balloon dilatation blood vessel wall brachiocephalic trunk child clinical article clinical evaluation congenital heart disease coronary artery anomaly curved balloon catheter cyanosis disease association heart ventricle septum defect hospital discharge human in vitro study in-stent restenosis infant lung hemorrhage minimal lumen diameter occlusion balloon catheter oxygen desaturation oxygen saturation phlebography postoperative angled lesion postoperative complication preschool child priority journal pulmonary valve atresia subclavian artery surface stress tricuspid valve atresia vascular guide wire wall stress GOKU curve GOKU straight Radifocus LA - English M3 - Article N1 - L373630144 2014-08-06 2014-08-13 PY - 2014 SN - 1540-8183 0896-4327 SP - 408-413 ST - Novel curved balloon catheter for dilating postoperative angled lesions associated with congenital heart disease T2 - Journal of Interventional Cardiology TI - Novel curved balloon catheter for dilating postoperative angled lesions associated with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L373630144 http://dx.doi.org/10.1111/joic.12122 VL - 27 ID - 935 ER - TY - JOUR AB - The Fontan procedure is employed as the final-stage palliation in single-ventricle congenital heart patients and results in diversion of venous blood directly to the pulmonary arteries. Fontan patients have been known to suffer from postoperative systemic venous hypertension, which in turn is associated with pleural effusions and protein losing enteropathy, leading to a decreased duration and quality of life. Despite the ongoing debate on its benefits, a circular fenestration hole (typically 4 mm) establishing a venous shunt to the common atrium is traditionally employed to relieve venous pressure in the Fontan conduit and improve early postoperative Fontan hemodynamics. However, these improvements come at the cost of reduced oxygen saturation due to excessive right-to-left shunting if the fenestration is permanent. The ideal "selective" fenestration would therefore limit or eliminate shunt flow at tolerable systemic venous pressures and allow increased flow at high pressures. The objective of this study is to introduce new fenestration designs that exhibit these desirable pressure-flow characteristics. Novel plus-shaped and S-shaped fenestration designs with leaflets are introduced as alternatives to the traditional circular fenestration, each having identical effective orifice areas at the fully open states. In vitro steady leakage flow tests were performed for physiological flow-driving pressures in order to obtain pressure-drop versus flow-rate characteristics. In addition, the leaflet opening kinematics of the plus-shaped fenestration was investigated computationally using finite element simulation. Fluid-structure interaction analysis was performed to determine leaflet displacements and pressure-flow characteristics at low pressures. Further, a lumped parameter model of the single-ventricle circuit was created to simulate pulsatile flow conditions For the plus-shaped fenestration, the flow rate was found to increase nonlinearly with increased driving systemic venous pressures at high physiological-pressure drops which did not cause the leaflets to fully open, and linearly for low driving pressures. These results indicate that leaflets of the plus-shaped fenestration design activated passively after a critical systemic venous pressure threshold. This feature is ideal for minimizing undesirable excessive venous shunting. A large variability in shunting flow rate may be obtained by changing the shape, thickness, size, and material of the fenestration to suit requirements of the patient, which can further limit shunt flow in a controlled manner. AD - Biomedical Engineering Department, Carnegie Mellon University, Pittsburgh, PA, USA. AN - 23305575 AU - Albal, P. G. AU - Menon, P. G. AU - Kowalski, W. AU - Undar, A. AU - Turkoz, R. AU - Pekkan, K. DA - Jan DB - PubMed DO - 10.1111/aor.12011 DP - NLM ET - 2013/01/12 IS - 1 KW - Adolescent Cardiac Output Child Child, Preschool Equipment Design Fontan Procedure/*methods Heart Defects, Congenital/*physiopathology/*surgery Hemodynamics Humans Hypertension/*physiopathology In Vitro Techniques Infant Infant, Newborn Palliative Care Polytetrafluoroethylene Pulsatile Flow LA - eng N1 - 1525-1594 Albal, Priti G Menon, Prahlad G Kowalski, William Undar, Akif Turkoz, Riza Pekkan, Kerem Journal Article United States Artif Organs. 2013 Jan;37(1):66-75. doi: 10.1111/aor.12011. PY - 2013 SN - 0160-564x SP - 66-75 ST - Novel fenestration designs for controlled venous flow shunting in failing Fontans with systemic venous hypertension T2 - Artif Organs TI - Novel fenestration designs for controlled venous flow shunting in failing Fontans with systemic venous hypertension VL - 37 ID - 374 ER - TY - JOUR AB - Patients with a Fontan circulation have impaired exercise capacity. Cardiac rehabilitation (CR) has shown promise in enhancing peak exercise parameters in this population, but an improvement in submaximal exercise has not been consistently demonstrated. We assessed the hypothesis that participation in CR will be associated with more efficient oxygen extraction and ventilation during submaximal exercise. In this prospective study, pediatric Fontans completed two 60 min CR sessions per week for 12 weeks. Cardiopulmonary exercise testing and stress echocardiography were performed at baseline and last CR session, and then compared with a paired sample t test. Ten pediatric Fontans completed the study. Five had tricuspid atresia and five had hypoplastic left heart syndrome. No serious adverse events occurred during CR sessions. Peak indexed oxygen consumption increased by a mean of 3.7 mL/kg/min (95% CI 1.5-5.9; p = 0.004), and peak oxygen pulse increased by a mean of 0.9 mL/beat (95% CI 0.4-1.4; p = 0.004). The peak respiratory exchange ratio did not change significantly. The significant difference in oxygen pulse became evident during submaximal exercise without a corresponding difference in echocardiographic stroke volume. Indexed oxygen consumption at ventilatory anaerobic threshold increased by a mean of 3.0 mL/kg/min (95% CI - 0.07 to 6.0; p = 0.055). The slope for the volume of expired ventilation to volume of carbon dioxide production improved by a mean of 4.5 (95% CI - 8.4 to - 0.6; p = 0.03). We observed significant improvements in both submaximal and peak exercise performance in pediatric Fontans undergoing CR with no serious adverse events. These changes appeared to be mediated, at least in part, by more efficient oxygen extraction and ventilation. AD - Cincinnati Children's Hospital Medical Center, Heart Institute, 3333 Burnet Avenue, MLC 2003, Cincinnati, OH, 45229-3026, USA. Samuel.Wittekind@cchmc.org. Cincinnati Children's Hospital Medical Center, Heart Institute, 3333 Burnet Avenue, MLC 2003, Cincinnati, OH, 45229-3026, USA. Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, GA, USA. AN - 29523922 AU - Wittekind, S. AU - Mays, W. AU - Gerdes, Y. AU - Knecht, S. AU - Hambrook, J. AU - Border, W. AU - Jefferies, J. L. DA - Jun DB - PubMed DO - 10.1007/s00246-018-1854-3 DP - NLM ET - 2018/03/11 IS - 5 KW - Adolescent Cardiac Rehabilitation/*methods Child Echocardiography, Stress/methods Exercise Test/methods Exercise Tolerance/*physiology Female Fontan Procedure/methods/*rehabilitation Humans Hypoplastic Left Heart Syndrome/physiopathology/surgery Male Oxygen Consumption/physiology Prospective Studies Respiratory Function Tests Tricuspid Atresia/surgery Cardiac rehabilitation Congenital heart disease Exercise testing Exercise therapy Fontan circulation LA - eng N1 - 1432-1971 Wittekind, Samuel Mays, Wayne Gerdes, Yvette Knecht, Sandra Hambrook, John Border, William Jefferies, John Lynn Journal Article United States Pediatr Cardiol. 2018 Jun;39(5):1023-1030. doi: 10.1007/s00246-018-1854-3. Epub 2018 Mar 9. PY - 2018 SN - 0172-0643 SP - 1023-1030 ST - A Novel Mechanism for Improved Exercise Performance in Pediatric Fontan Patients After Cardiac Rehabilitation T2 - Pediatr Cardiol TI - A Novel Mechanism for Improved Exercise Performance in Pediatric Fontan Patients After Cardiac Rehabilitation VL - 39 ID - 31 ER - TY - JOUR AB - We report a 9-year-old boy, with childhood cerebral X-linked adrenoleukodystrophy (CCALD), presenting with an episode of loss of consciousness, fixed gaze, hypotonia and vomit with spontaneous resolution and post-ictal sleep. Behavioural impairment has been observed since the previous five months. Magnetic Resonance Images (MRI) revealed isolated bilateral, symmetric, confluent temporal white matter lesions involving also corticospinal tracts, with sparing of the parieto-occipital and frontal white matter. This report outlines an atypical neuroradiological localization of X-linked adrenoleukodystrophy and neuropsychological findings not specifically related to the brain involvement seen at the MRI. AD - E.C. Turco, Child Neuropsychiatry Unit, Mother and Child Department, University-Hospital of Parma, Via Gramsci 14, Parma (PR), Italy AU - Turco, E. C. AU - Ormitti, F. AU - Andreolli, A. AU - Barsacchi, M. AU - Facini, C. AU - Pisani, F. DB - Embase Medline DO - 10.1016/j.braindev.2017.10.005 IS - 3 KW - anticonvulsive agent very long chain fatty acid adrenoleukodystrophy anticonvulsant therapy article case report child childhood cerebral X linked adrenoleukodystrophy childhood disease clinical article consciousness disorder deterioration diet therapy electroencephalogram epilepsy executive function human hyperactivity intelligence quotient male medical history muscle hypotonia neurologic examination neuropsychological test nuclear magnetic resonance imaging priority journal pulmonary artery stenosis school child tricuspid valve atresia vomiting Wechsler intelligence scale for children white matter lesion Wisconsin Card Sorting Test LA - English M3 - Article N1 - L619216219 2018-12-06 PY - 2018 SN - 1872-7131 0387-7604 SP - 238-241 ST - A novel temporal pattern of childhood cerebral X-linked adrenoleukodystrophy T2 - Brain and Development TI - A novel temporal pattern of childhood cerebral X-linked adrenoleukodystrophy UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L619216219 http://dx.doi.org/10.1016/j.braindev.2017.10.005 VL - 40 ID - 696 ER - TY - JOUR AB - Objective: To evaluate the ability to diagnose structural fetal anomalies during or soon after an extended nuchal translucency (NT) examination. Methods: The study population included all women who had a routine NT examination in the ultrasound division of one of three centers. Also included in the study were women referred to these centers following an abnormal NT examination. The sonographers were instructed to pay attention to fetal anomalies while performing the NT examination. Each examination was initially attempted transabdominally. Failure to obtain adequate views transabdominally was an indication for a transvaginal examination. When a structural fetal anomaly was detected or suspected, a full fetal anomaly scan was performed. When a diagnosis could not be established, fetal anatomy scan was repeated after 14. weeks of gestation. Fetal cardiac scanning was performed transvaginally, immediately or within 3. days after an increased NT was observed. When fetal anomalies were diagnosed the patients were informed about the possibilities of terminating the pregnancy or continuing the work-up and follow-up. Overall, ascertainment of fetal outcome was available in 85% of the study population. Results: We performed 4467 NT examinations during the study period and additional 123 fetal cardiac scanning following an abnormal NT examination. Overall, we performed 365 fetal cardiac scanning between 11 and 14. weeks of gestation. The fetal anomalies detected included the following: three skeletal anomalies, seven brain anomalies, four urinary system anomalies, four abdominal anomalies, two facial anomalies, and 13 cardiac anomalies. Six of the 13 cardiac anomalies were found in the atrioventricular canal. One third of the patients (11/33) elected to discontinue the pregnancy a short time after the detection of the congenital anomaly (until 14. weeks of gestation) and half of the patients (16\33) asked for termination of pregnancy later. More than 60% of the patients (20/33) with congenital anomalies detected following the NT examination refused to have chorionic villous sampling (CVS) or amniocentesis. Conclusion: The opportunity to scan the fetal anatomy in the early stages of pregnancy, when the NT examination is performed, justifies the approach of extended NT examination. © 2013 Elsevier Ireland Ltd. AD - Department of Obstetrics and Gynecology, Rambam Medical Center, Haifa, Israel AU - Goldstein, I. AU - Weizman, B. AU - Nizar, K. AU - Weiner, Z. DB - Scopus DO - 10.1016/j.earlhumdev.2013.12.008 IS - 2 KW - Nuchal translucency Structural fetal anomalies M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2014 SP - 87-91 ST - The nuchal translucency examination leading to early diagnosis of structural fetal anomalies T2 - Early Human Development TI - The nuchal translucency examination leading to early diagnosis of structural fetal anomalies UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84893699750&doi=10.1016%2fj.earlhumdev.2013.12.008&partnerID=40&md5=61fa5d38861f7eacc4f296240d5b05d0 VL - 90 ID - 1997 ER - TY - JOUR AB - Background: Few data are available regarding requirements of sedation and analgesia in children during extracorporeal life support. Aims: The aim of this study was to evaluate if children with functionally univentricular hearts on extracorporeal life support after first-stage palliation surgery have higher requirement of analgesics and sedatives compared with children without extracorporeal life support using a goal-directed nurse-driven analgesia and sedation protocol. Methods: This prospective observational matched case-control pilot study was conducted at a cardiac pediatric intensive care unit of a tertiary referral center. Seventeen patients with functionally univentricular hearts including hypoplastic left heart syndrome who were on extracorporeal life support after first-stage palliation surgery were enrolled from July 2012 to January 2017. Seventeen matched patients served as controls. Doses of morphine, midazolam, clonidine, and muscle relaxants as well as sedation scores (COMFORT behavior scale and the nurse interpretation of sedation scale) were assessed according to a nurse-driven protocol every 8 hours up to 120 hours after first-stage palliation surgery. Results: Sedation scores were equal in the extracorporeal life support group and in the control group at most points in time. There was no significant difference in cumulative doses of morphine and midazolam. However, children of the extracorporeal life support group received higher doses of midazolam and morphine at some points in time. Conclusion: A nurse-driven protocol for analgesia and sedation of children with extracorporeal life support is feasible. Patients with extracorporeal life support do not need deeper sedation levels and have not higher cumulative sedation requirements than children without extracorporeal life support. AD - J. Michel, Department of Pediatric Cardiology, Pulmology and Pediatric Intensive Care Medicine, University Children's Hospital Tuebingen, Tuebingen, Germany AU - Michel, J. AU - Hofbeck, M. AU - Gerbig, I. AU - Icheva, V. AU - Heimberg, E. AU - Jost, W. AU - Schlensak, C. AU - Kumpf, M. AU - Blumenstock, G. AU - Neunhoeffer, F. DB - Embase Medline DO - 10.1111/pan.13274 IS - 12 KW - blood pump medos clonidine midazolam morphine muscle relaxant agent analgesia article case control study clinical article controlled study coronary care unit extracorporeal oxygenation female heart single ventricle human infant male nurse attitude observational study palliative therapy pediatric intensive care unit pediatrics pilot study priority journal prospective study sedation tertiary care center deltastream LA - English M3 - Article N1 - L618897101 2017-10-27 2018-08-01 PY - 2017 SN - 1460-9592 1155-5645 SP - 1261-1270 ST - Nurse-driven analgesia and sedation in pediatric patients with univentricular hearts requiring extracorporeal life support after first-stage palliation surgery: A pilot study T2 - Paediatric Anaesthesia TI - Nurse-driven analgesia and sedation in pediatric patients with univentricular hearts requiring extracorporeal life support after first-stage palliation surgery: A pilot study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L618897101 http://dx.doi.org/10.1111/pan.13274 VL - 27 ID - 725 ER - TY - JOUR AB - PURPOSE OF REVIEW: Growth failure secondary to feeding problems after complex neonatal cardiac surgery is well documented, but not well understood. The purpose of this review is to describe feeding and growth pattern in children with congenital heart defects. RECENT FINDINGS: Nearly half of the infants with univentricular heart defects require supplementation with nasogastric or gastrostomy tube at discharge from neonatal surgery. Feeding challenges contribute to parental stress, and persist beyond infancy. These infants are 'stunted' with both weight and height being below normal. Nearly a quarter of these infants meet the definition of 'failure to thrive' in the first year of life. Short stature is a significant problem for many of these children, and has an impact on neurodevelopmental outcomes. A structured nutritional program can have a positive impact on growth in the interstage period prior to the superior cavopulmonary connection. SUMMARY: Optimizing nutritional intake has been targeted as a key component of the National Pediatric Cardiology Quality Improvement Collaborative. This initiative has enabled the development of best practices that have the potential to mitigate poor growth in children with congenital heart defects. AD - School of Nursing, University of Pennsylvania, USA. AN - 23370229 AU - Medoff-Cooper, B. AU - Ravishankar, C. DA - Mar DB - PubMed DO - 10.1097/HCO.0b013e32835dd005 DP - NLM ET - 2013/02/02 IS - 2 KW - Child Energy Intake Enteral Nutrition Failure to Thrive/*etiology/physiopathology Heart Defects, Congenital/*complications/*physiopathology/surgery Humans Length of Stay Malnutrition/*complications Norwood Procedures *Nutritional Support/methods/standards Parents/psychology Preoperative Care Risk Factors Stress, Psychological LA - eng N1 - 1531-7080 Medoff-Cooper, Barbara Ravishankar, Chitra Journal Article Review United States Curr Opin Cardiol. 2013 Mar;28(2):122-9. doi: 10.1097/HCO.0b013e32835dd005. PY - 2013 SN - 0268-4705 SP - 122-9 ST - Nutrition and growth in congenital heart disease: a challenge in children T2 - Curr Opin Cardiol TI - Nutrition and growth in congenital heart disease: a challenge in children VL - 28 ID - 4 ER - TY - JOUR AB - Growth failure is a widespread health problem in infants with congenital heart disease, specifically infants with single ventricle heart lesions, including hypoplastic left heart syndrome. Optimizing nutrition delivery for neonates undergoing cardiac surgery is challenging given environmental and physiologic barriers. Concomitant with nutrition delivery challenges, wide practice variations exist among clinicians. Malnutrition and poor growth are associated with poor wound healing, increased infection risk, prolonged hospitalizations, and long-term neurodevelopmental disability, including worse school performance. The cause of early postnatal growth failure is multifactorial and related to the delay in onset of enteral feedings, increased metabolic stress of cardiac surgery, cyanosis, low cardiac output, increased energy expenditure, altered splanchnic perfusion, and gastrointestinal morbidities. In addition, cardiac infants are susceptible to unique complications, including chylothorax, vocal cord dysmotility, and swallowing dysfunction. © 2015 by the American Academy of Pediatrics. All rights reserved. AD - Department of Pediatrics, Medical University of South Carolina, Charleston, SC, United States AU - Fogg, K. L. AU - Zyblewski, S. C. DB - Scopus DO - 10.1542/neo.16-3-e144 IS - 3 M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2015 SP - e144-e151 ST - Nutrition and growth in neonates undergoing cardiac surgery T2 - NeoReviews TI - Nutrition and growth in neonates undergoing cardiac surgery UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84923932271&doi=10.1542%2fneo.16-3-e144&partnerID=40&md5=a7bcb84f57b5e070480c7f27c742455c VL - 16 ID - 1963 ER - TY - JOUR AD - R.D. Harris, Department of Radiology, Dartmouth-Hitchcock Clinic, Lebanon, NH 03756, United States AU - Harris, R. D. DB - Embase IS - 2 KW - adult Alagille syndrome amniocentesis article autopsy case report CATCH 22 syndrome chromosome 22q chromosome deletion cleft palate clinical feature clubfoot congenital heart malformation congenital malformation developmental disorder differential diagnosis DiGeorge syndrome face dysmorphia Fallot tetralogy family history female fetus echography fluorescence in situ hybridization Goldenhar syndrome heart ventricle septum defect heterozygote human hypoplasia hypoplastic left heart syndrome incidence kidney malformation mental disease newborn newborn death palatopharyngeal incompetence patent ductus arteriosus polydactyly priority journal recurrence risk short stature Smith Lemli Opitz syndrome syndactyly syndrome VACTERL thymus disease thymus hypoplasia velocardiofacial syndrome vertebra malformation LA - English M3 - Article N1 - L40734822 2005-06-09 PY - 2005 SN - 0894-8771 SP - 107-108+125-126 ST - Obstetrics T2 - Ultrasound Quarterly TI - Obstetrics UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L40734822 VL - 21 ID - 1276 ER - TY - JOUR AB - The vast majority of congenital diaphragmatic cases present with severe respiratory distress in the immediate neonatal period. Obstructive jaundice secondary to CDH is a rare presenting symptom, previously unreported with Morgagni type hernias which comprise 2% of all CDHs. Although the safety of minimally invasive repair for CDH has been shown previously, there has been little data on its safety for patients with complex congenital cardiac disease. In this case report, we present an 18-month old infant with Fontan circulation presenting with jaundice secondary to Morgagni hernia who ultimately underwent laparoscopic repair. A brief discussion of the safety of laparoscopic surgery in patients post-Fontan is included. AD - Section of Pediatric Surgery, Department of Surgery, University of Michigan, Ann Arbor USA Section of Pediatric Gastroenterology, Department of Pediatrics, University of Michigan, Ann Arbor USA Section of Pediatric Surgery, Department of Surgery, University of Michigan, Ann Arbor, MI, USA C.S. Mott Children's Hospital, University of Michigan, 1540 E. Hospital Dr., SPC 4211, Ann Arbor, MI, 48109-0344, USA AN - 110861350. Language: English. Entry Date: 20160902. Revision Date: 20170929. Publication Type: journal article AU - Halaweish, Ihab AU - Ralls, Mathew AU - Siddiqui, Sabina AU - Dickinson, Chris AU - Jarboe, Marcus AU - Jarboe, Marcus D. DB - ccm DO - 10.1007/s00383-015-3804-y DP - EBSCOhost IS - 12 KW - Cardiopulmonary Bypass Cholestasis -- Complications Hernia, Diaphragmatic, Congenital -- Complications Hernia, Diaphragmatic, Congenital -- Surgery Male Laparoscopy Treatment Outcomes Infant Clinical Assessment Tools Questionnaires N1 - case study. Journal Subset: Biomedical; Continental Europe; Europe. Instrumentation: Infant Characteristics Questionnaire (ICQ) (Bates et al); Behavior Rating Inventory of Executive Function (BRIEF). NLM UID: 8609169. PMID: NLM26500076. PY - 2015 SN - 0179-0358 SP - 1199-1202 ST - Obstructive jaundice secondary to Morgagni hernia in an infant with Fontan circulation T2 - Pediatric Surgery International TI - Obstructive jaundice secondary to Morgagni hernia in an infant with Fontan circulation UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=110861350&site=ehost-live&scope=site VL - 31 ID - 1500 ER - TY - JOUR AB - The vast majority of congenital diaphragmatic cases present with severe respiratory distress in the immediate neonatal period. Obstructive jaundice secondary to CDH is a rare presenting symptom, previously unreported with Morgagni type hernias which comprise 2% of all CDHs. Although the safety of minimally invasive repair for CDH has been shown previously, there has been little data on its safety for patients with complex congenital cardiac disease. In this case report, we present an 18-month old infant with Fontan circulation presenting with jaundice secondary to Morgagni hernia who ultimately underwent laparoscopic repair. A brief discussion of the safety of laparoscopic surgery in patients post-Fontan is included. AD - Section of Pediatric Surgery, Department of Surgery, University of Michigan, Ann Arbor, MI, USA. Section of Pediatric Gastroenterology, Department of Pediatrics, University of Michigan, Ann Arbor, MI, USA. Section of Pediatric Surgery, Department of Surgery, University of Michigan, Ann Arbor, MI, USA. marjarbo@med.umich.edu. C.S. Mott Children's Hospital, University of Michigan, 1540 E. Hospital Dr., SPC 4211, Ann Arbor, MI, 48109-0344, USA. marjarbo@med.umich.edu. AN - 26500076 AU - Halaweish, I. AU - Ralls, M. AU - Siddiqui, S. AU - Dickinson, C. AU - Jarboe, M. D. DA - Dec DB - PubMed DO - 10.1007/s00383-015-3804-y DP - NLM ET - 2015/10/27 IS - 12 KW - *Fontan Procedure Hernias, Diaphragmatic, Congenital/*complications/*surgery Humans Infant Jaundice, Obstructive/*complications Laparoscopy Male Treatment Outcome Diaphragmatic Fontan Hernia Jaundice Morgagni Pediatric LA - eng N1 - 1437-9813 Halaweish, Ihab Ralls, Mathew Siddiqui, Sabina Dickinson, Chris Jarboe, Marcus D Case Reports Journal Article Germany Pediatr Surg Int. 2015 Dec;31(12):1199-202. doi: 10.1007/s00383-015-3804-y. Epub 2015 Oct 25. PY - 2015 SN - 0179-0358 SP - 1199-202 ST - Obstructive jaundice secondary to Morgagni hernia in an infant with Fontan circulation T2 - Pediatr Surg Int TI - Obstructive jaundice secondary to Morgagni hernia in an infant with Fontan circulation VL - 31 ID - 493 ER - TY - JOUR AB - Objectives: Peripheral pulmonary artery stenosis (PPS) is a heterogeneous disease associated with numerous clinical or syndromic disorders including Williams syndrome. Previous studies focusing on patients with Williams syndrome have documented spontaneous improvement in branch pulmonary artery (PA) stenosis and right ventricular pressure. The goal of this study is to identify angiographic evidence of spontaneous occlusion or loss of segmental PAs in patients with PPS. Methods: Patients undergoing serial catheterization for PPS at Boston Children's Hospital between 1999 and 2014 were selected. Patients with structural heart disease such as tetralogy of Fallot, truncus arteriosus, transposition of the great arteries, or single ventricle were excluded. Hemodynamic data were extracted from catheterization reports, all available angiograms were reviewed, and evidence of segmental PA loss was documented based on defined angiographic criteria. Results: Forty-two patients were included in the study. Each patient underwent an average of 5 catheterizations over the 15-year study period. The average RV pressure at initial catheterization was near systemic levels. Evidence of segmental PA loss was present in 40% of patients. Almost 30% of patients with vessel loss demonstrated loss at their initial catheterization, and 65% of patients experienced loss of multiple vessels over time. Patients with vessel loss tended to be older at initial catheterization and have higher average RV pressures at their most recent catheterization than those without vessel loss. Conclusions: Progressive segmental PA loss in patients with systemic arteriopathy and PPS has not been well described previously. In this cohort, segmental PA loss occurred in 40% of patients, resulting in persistently elevated PA pressures relative to patients who did not develop vessel loss. These findings highlight the importance of surveillance in this population of patients with systemic arteriopathy and severe PPS. AD - L. Bergersen, Department of Cardiology, 300 Longwood Avenue, Boston, MA, United States AU - Morray, B. H. AU - Bergersen, L. AU - Lock, J. E. AU - Marshall, A. C. DB - Embase Medline DO - 10.1111/chd.12211 IS - 2 KW - adolescent Alagille syndrome angiography aortic stenosis artery disease article child clinical article disease association familial disease female heart right ventricle hypertension heart right ventricle pressure hemodynamics human hypertension male moyamoya disease outcome assessment peripheral pulmonary artery stenosis priority journal pulmonary artery catheterization pulmonary artery stenosis retrospective study systemic arteriopathy venous return Williams Beuren syndrome LA - English M3 - Article N1 - L53285495 2014-08-14 2015-05-05 PY - 2015 SN - 1747-0803 1747-079X SP - E60-E67 ST - Occult progressive pulmonary arterial occlusion associated with right ventricular hypertension in patients with systemic arteriopathy T2 - Congenital Heart Disease TI - Occult progressive pulmonary arterial occlusion associated with right ventricular hypertension in patients with systemic arteriopathy UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L53285495 http://dx.doi.org/10.1111/chd.12211 VL - 10 ID - 872 ER - TY - JOUR AB - We tested the hypothesis that later completion of the Fontan procedure is associated with improved exercise capacity in the current period of staged single-ventricle palliation. We performed a retrospective study, in Fontan patients, of exercise stress test data from April 2003 through March 2011. Patients were included if they had received staged palliations in accordance with current surgical strategy, defined as the performance of a superior cavopulmonary connection at ≤1 year of age, followed in subsequent years by Fontan completion. Patients with a pacemaker or respiratory exchange ratio <1 were excluded. Early and late Fontan groups were created on the basis of whether Fontan completion had been performed at <4 or ≥ 4 years of age. The primary predictor variable was age at Fontan completion, and the primary marker of exercise performance was the percentage of predicted maximum oxygen consumption. During the study period, 55 patients were identified (mean age, 11.7 ± 2.8 yr). Older age at Fontan completion correlated positively with higher percentages of predicted maximum oxygen consumption (R=0.286, P=0.034). Patients in whom Fontan completion was performed at ≥4 years of age had higher percentages of predicted maximum oxygen consumption than did those in whom completion was at <4 years of age (84.4 ± 21.5 vs 72.9 ± 18.1; P=0.041). Later Fontan completion might be associated with improved exercise capacity in patients palliated in accordance with contemporary surgical strategy. AN - 26413015 AU - Bolin, E. H. AU - Maskatia, S. A. AU - Tate, A. L. AU - Petit, C. J. C2 - PMC4567125 DA - Aug DB - PubMed DO - 10.14503/thij-14-4178 DP - NLM ET - 2015/09/29 IS - 4 KW - Age Factors Child, Preschool Exercise Test *Exercise Tolerance Female *Fontan Procedure Humans Hypoplastic Left Heart Syndrome/diagnosis/metabolism/physiopathology/*surgery Infant Male *Oxygen Consumption Palliative Care Recovery of Function Retrospective Studies Time Factors Treatment Outcome Fontan procedure/adverse effects heart defects, congenital/surgery hemodynamics/physiology hypoplastic left heart syndrome longitudinal studies oxygen consumption/physiology physical exertion postoperative complications pulmonary circulation tricuspid atresia/surgery vascular resistance LA - eng N1 - 1526-6702 Bolin, Elijah H Maskatia, Shiraz A Tate, Amanda L Petit, Christopher J Comparative Study Journal Article Tex Heart Inst J. 2015 Aug 1;42(4):333-40. doi: 10.14503/THIJ-14-4178. eCollection 2015 Aug. PY - 2015 SN - 0730-2347 (Print) 0730-2347 SP - 333-40 ST - Older Age at Completion of Fontan Procedure Is Associated with Improved Percentage of Predicted Maximum Oxygen Uptake T2 - Tex Heart Inst J TI - Older Age at Completion of Fontan Procedure Is Associated with Improved Percentage of Predicted Maximum Oxygen Uptake VL - 42 ID - 126 ER - TY - JOUR AB - OBJECTIVES: The aim of this study was to evaluate the outcome in a series of patients with Ebstein's anomaly and a failing right ventricle (RV) undergoing tricuspid valve (TV) repair and bidirectional Glenn cavopulmonary anastomosis (BDG). MATERIALS AND METHOD: Between January 2006 and September 2013, 11 consecutive patients diagnosed with severe forms of Ebstein's anomaly and a failing RV underwent TV surgery and BDG. The mean age was 16.5 ± 7 years. Most frequently found symptoms were cyanosis, dyspnea, and arrhythmias. The azygos or hemiazygos veins were left open. The TV was repaired using the "peacock tail" technique, which consisted of total detachment of the anterior and posterior leaflets of the TV and rotation in both directions reimplanting them to the true annulus. The mean follow-up was 3.8 ± 2.4 years (range three months to six years). RESULTS: Hospital mortality was 9% (one patient). TV repair was possible in 10 patients. None of the patients had AV block postoperatively. At one year after surgery, the indexed RV and RA diameter were reduced significantly versus the preoperative data (p = 0.003 and p < 0.001). The mean TVR and indexed TV area were 1.2 ± 0.42 and 1.6 ± 0.6 (mm/m2), significantly lower than preoperatively (p = 0.001 and p = 0.008, respectively). The mean NYHA functional class, SaO2 , and cardiothoracic ratio were significantly improved. CONCLUSIONS: The peacock tail technique for TV repair in combination with BDG in patients with Ebstein's malformation and depressed RV function results in TV preservation, a low incidence of recurrent regurgitation, favorable functional status and RV function, and resolution of cyanosis. AD - Division of Cardiac Surgery, University Hospital Center of Tirana, Tirana, Albania. AN - 24762038 AU - Prifti, E. AU - Baboci, A. AU - Esposito, G. AU - Kajo, E. AU - Dado, E. AU - Vanini, V. DA - May DB - PubMed DO - 10.1111/jocs.12321 DP - NLM ET - 2014/04/26 IS - 3 KW - Adolescent Adult Cardiac Surgical Procedures/*methods Child Ebstein Anomaly/*complications/surgery Female Fontan Procedure/methods Humans Male Reconstructive Surgical Procedures/*methods Treatment Outcome Tricuspid Valve/abnormalities/*surgery Ventricular Dysfunction, Right/*complications/surgery Young Adult LA - eng N1 - 1540-8191 Prifti, Edvin Baboci, Arben Esposito, Giampiero Kajo, Efrosina Dado, Elona Vanini, Vittorio Journal Article United States J Card Surg. 2014 May;29(3):383-9. doi: 10.1111/jocs.12321. PY - 2014 SN - 0886-0440 SP - 383-9 ST - One and a half ventricle repair in association with tricuspid valve repair according to "peacock tail" technique in patients with Ebstein's malformation and failing right ventricle T2 - J Card Surg TI - One and a half ventricle repair in association with tricuspid valve repair according to "peacock tail" technique in patients with Ebstein's malformation and failing right ventricle VL - 29 ID - 305 ER - TY - JOUR AB - Background: Pulmonary artery stenting without a long vascular sheath has a special significance, particularly for children with low body weight. Using only a short sheath often permits the implantion of a stent of the correct size; moreover, it improves access to peripherally located stenoses. The aim of this paper is to present the results of a balloon expandable stent implantation into pulmonary arteries without using a long vascular sheath. Methods: The subjects were divided into two groups. The first group (28 patients, mean age 3.2 years) comprised patients with a single-ventricle heart after bi-directional Glenn procedure (Fontan procedure). The second group (22 patients, mean age 8.3 years) consisted of patients with a two-ventricle heart. Patients were retrospectively analyzed with regard to stenosis size widening and change in trans-stenotic pressure gradient after stenting. Results: In our data, no statistically significant differences between the two groups in terms of the number of complications and incorrect stent position following implantation were noted (3% and 4%). Good treatment results, with a decrease in trans-stenotic pressure gradient in the first group of 3.2 mm and in the second group of 13.4 mm of mercury, and a widening of the stenosis, were obtained in most cases in both groups (97% and 96%). The average change of the vessel's diameter was in the first group 4.2 mm and in the second 5.4 mm. Conclusions: The obtained results suggest that pulmonary artery stenting with a short vascular sheath has numerous advantages and can be successfully performed in children. In the case of single-ventricle hearts after a Glenn procedure, it may indeed be the method of choice. © 2010 Via Medica. AD - K. W. Michalak, Department of Cardiology, Polish Mother's Memorial Hospital, Research Institute, Rzgowska 281/289, 93-338 Łódź, Poland AU - Moszura, T. AU - Michalak, K. W. AU - Dryzek, P. AU - Moll, J. A. AU - Moll, J. AU - Sysa, A. DB - Embase Medline IS - 2 KW - article blood vessel diameter child clinical article controlled study Fallot tetralogy female Fontan procedure Glenn shunt heart single ventricle human hypoplastic left heart syndrome infant lung artery pressure lung edema male Norwood procedure percutaneous transluminal angioplasty pulmonary artery stenosis pulmonary artery stenting stent Williams Beuren syndrome Coroflex Genesis stent Palmaz P-308 stent LA - English M3 - Article N1 - L358675524 2010-04-29 2010-05-06 PY - 2010 SN - 1897-5593 SP - 149-156 ST - One center experience in pulmonary artery stenting without long vascular sheath T2 - Cardiology Journal TI - One center experience in pulmonary artery stenting without long vascular sheath UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L358675524 VL - 17 ID - 1154 ER - TY - JOUR AN - 107786737. Language: English. Entry Date: 20150520. Revision Date: 20150712. Publication Type: Journal Article DB - ccm DP - EBSCOhost IS - 5 KW - Pediatrics Privacy and Confidentiality Echocardiography Hepatitis B Papillomavirus Vaccine Milk, Human Immunization Perinatal Care Heart Rate Enterocolitis, Necrotizing Middle Ear Ventilation Pregnancy Female Body Mass Index Stroke Spinal Puncture Intubation, Intratracheal Clinical Competence Parenting Child Development Breast Feeding Appointments and Schedules Constipation Surgery, Operative Eating Disorders Pulmonary Embolism Aortic Diseases Cholestasis Hypoplastic Left Heart Syndrome N1 - abstract. Journal Subset: Biomedical; Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. Special Interest: Pediatric Care. NLM UID: 0376422. PY - 2015 SN - 0031-4005 SP - 932-944 ST - ONLINE ARTICLES T2 - Pediatrics TI - ONLINE ARTICLES UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=107786737&site=ehost-live&scope=site VL - 135 ID - 1550 ER - TY - JOUR AB - The use of amiodarone is associated with many organ-specific side effects, of which thyroid gland dysfunction constitute up to 35% of them. Amiodarone-induced thyrotoxicosis can occur in either of two forms, type 1 or type 2, depending on whether there was a positive history of thyroid dysfunction prior to the use of amiodarone therapy or not, respectively. A unique underlying pathophysiology discerns one type from another, which is why early differentiation is pivotal to the rapid institution of appropriate therapy that is contingent on an accurate and correct diagnosis. We report a case of a 15-year-old adolescent with type 2 amiodarone-induced thyrotoxicosis, a rare presentation of thyrotoxicosis in children. AD - A.E. Al-Agha, Pediatric Endocrinology, King Abdulaziz University Hospital, Pediatric Department, P.O.Box 80215, Jeddah, Saudi Arabia AU - Milyani, A. A. AU - Al-Agha, A. E. DB - Embase IS - 3 KW - septostomy balloon catheter amiodarone antinuclear antibody double stranded DNA antibody parietal cell antibody prednisone smooth muscle antibody steroid thyroglobulin antibody thyroid peroxidase antibody adolescent article case report clinical article color Doppler flowmetry congenital heart disease drug dose reduction drug induced disease euthyroidism follow up Fontan procedure free liothyronine index free thyroxine index hemodynamics human hyperactivity male paroxysmal tachycardia pathophysiology steroid therapy supraventricular tachycardia thorax pain thyroid crisis thyroid disease thyroid function test thyrotoxicosis thyrotropin blood level LA - English M3 - Article N1 - L2001650042 2019-04-05 2019-04-10 PY - 2018 SN - 0971-9032 SP - 192-195 ST - The onset of asymptomatic amiodarone-induced thyrotoxicosis in an adolescent male T2 - Current Pediatric Research TI - The onset of asymptomatic amiodarone-induced thyrotoxicosis in an adolescent male UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2001650042 VL - 22 ID - 710 ER - TY - JOUR AD - J.H. Moller, University of Minnesota Hospital, Box 288, Minneapolis, MN 55455, United States AU - Powell, C. B. AU - Stone, F. M. AU - Atkins, D. L. AU - Watson, D. G. AU - Moller, J. H. DB - Embase Medline DO - 10.1016/S0002-9149(97)00069-6 IS - 8 KW - aortic arch interruption aortopulmonary septal defect article DiGeorge syndrome Down syndrome Goldenhar syndrome great vessels transposition heart right ventricle double outlet heart single ventricle heart ventricle septum defect human infant major clinical study mitral valve atresia patent ductus arteriosus priority journal surgical approach surgical mortality tricuspid valve atresia United States Williams Beuren syndrome LA - English M3 - Article N1 - L27217080 1997-06-15 PY - 1997 SN - 0002-9149 SP - 1147-1148 ST - Operative mortality and frequency of coexistent anomalies in interruption of the aortic arch T2 - American Journal of Cardiology TI - Operative mortality and frequency of coexistent anomalies in interruption of the aortic arch UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L27217080 http://dx.doi.org/10.1016/S0002-9149(97)00069-6 VL - 79 ID - 1357 ER - TY - JOUR AB - ObJective: The concept of a lateral tunnel for the Fontan operation is now widely accepted. Most lateral tunnels are constructed intraatrially with the use of aortic crossclamping. Construction of extracardiac lateral tunnels with the use of homografts or other nonviable tubes eliminates aortic crossclamping but lacks growth potential in length or width. The native pericardium, which is 'sealed' posteriorly along the pulmonary artery, atrium, and inferior vena cava, could be turned down onto the right atrium to form a viable extracardiac lateral tunnel. Methods: We designed and successfully constructed extracardiac lateral tunnels using viable autologous pericardium, pedicled on its lateral blood supply, in 19 patients aged 9 months to 5 years. All patients had a previous Glenn shunt; five patients had dextrocardia and a midline inferior vena cava. The patients' inferior vena cava-right atrial connection was opened transversely and the right atrial opening was sutured to its back wall, keeping the eustachian valve in the inferior vena cava. The underside of the right pulmonary artery was opened longitudinally; its inferior edge was sewn to the adjacent pericardial reflection. Any 'pocket' or depressions in the posterior pericardium along the pulmonary veins were closed with running suture. Two incisions were made in the right pericardium down to the phrenic nerve parallel to the inferior vena caval and pulmonary arterial openings. This pedicled pericardium was trimmed and sewn as a roof to the upper edges of the inferior vena cava and pulmonary artery openings and then sewn longitudinally along the unopened right atrial wall, completing the viable extracardiac lateral tunnel. Although no fenestrations were used, these could be made during construction, or more significantly, owing to the lack of thick walled structures, in the catheterization laboratory in the postoperative period. Results: All 19 patients had respiratory/cardiac pulsations in the pulmonary arteries owing to the compressible lateral tunnel. At follow-up of up to 2 1/4 years, all tunnels are growing and no obstructions have occurred. Conclusion: The viable autologous pericardial extracardiac lateral tunnel can be constructed without cardiac ischemia, can be fenestrated in the postoperative period, and forms a compressible, nonthrombogenic conduit capable of growth, which can be constructed early in infancy. AD - Division of Cardiothoracic Surgery, Loma Linda University Medical Center, 11234 Anderson St., Loma Linda, CA 92354, United States AU - Gundry, S. R. AU - Razzouk, A. J. AU - Del Rio, M. J. AU - Shirali, G. AU - Bailey, L. L. AU - Reddy, V. M. AU - Drinkwater, D. C. AU - Lamberti, J. AU - Mayer J.E, Jr. DB - Scopus DO - 10.1016/S0022-5223(97)70043-6 IS - 4 M3 - Article N1 - Cited By :34 Export Date: 15 June 2020 PY - 1997 SP - 552-559 ST - The optimal fontan connection: A growing extracardiac lateral tunnel with pedicled pericardium T2 - Journal of Thoracic and Cardiovascular Surgery TI - The optimal fontan connection: A growing extracardiac lateral tunnel with pedicled pericardium UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0030779122&doi=10.1016%2fS0022-5223%2897%2970043-6&partnerID=40&md5=4dab79b0081993d1f76da40db43770b8 VL - 114 ID - 2298 ER - TY - JOUR AB - For children with hypoplastic left heart syndrome, contemporary management over three stages includes a window of high risk for sudden death between the initial stage of palliation, the Norwood operation itself, and the second stage, creation of the bidirectional superior cavopulmonary connection. The risk is highest at a time when patients have been discharged from the hospital to grow and prepare for the second stage, and has persisted despite the remarkable improvements in immediate postoperative and hospital survival after the initial surgery. Potential contributing factors to the increased vulnerability to sudden death between the stages include the limited circulatory reserve inherent in the parallel circulations supported by a functionally univentricular heart, the reliance on a prosthetic shunt which is susceptible to thrombosis, and congenital or acquired anatomical cardiovascular abnormalities such as aortic atresia, residual obstruction in the aortic arch, tricuspid valvar insufficiency, or right ventricular dysfunction. © 2006 Cambridge University Press. AD - N.S. Ghanayem, Department of Pediatric Critical Care, Children's Hospital of Wisconsin, 9000 West Wisconsin Avenue, Milwaukee, WI 53226, United States AU - Ghanayem, N. S. AU - Tweddell, J. S. AU - Hoffman, G. M. AU - Mussatto, K. AU - Jaquiss, R. D. B. DB - Embase DO - 10.1017/S1047951105002349 IS - SUPPL. 1 KW - oxygen aggressiveness arterial oxygen saturation article body growth body weight child child health care elective surgery Fontan procedure home monitoring human hypoplastic left heart syndrome pulmonary arteriovenous fistula observational study palliative therapy risk assessment treatment outcome body weight gain LA - English M3 - Article N1 - L44375394 2006-10-10 PY - 2006 SN - 1047-9511 1467-1107 SP - 61-66 ST - Optimal timing of the second stage of palliation for hypoplastic left heart syndrome facilitated through home monitoring, and the results of early cavopulmonary anastomosis T2 - Cardiology in the Young TI - Optimal timing of the second stage of palliation for hypoplastic left heart syndrome facilitated through home monitoring, and the results of early cavopulmonary anastomosis UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L44375394 http://dx.doi.org/10.1017/S1047951105002349 VL - 16 ID - 1264 ER - TY - JOUR AB - The Fontan operation is a palliative surgical procedure performed on children, born with congenital heart defects that have yielded only a single functioning ventricle. The total cavo-pulmonary connection (TCPC) is a common variant of the Fontan procedure, where the superior vena cava (SVC) and inferior vena cava (IVC) are routed directly into the pulmonary arteries (PA). Due to the limited pumping energy available, optimized hemodynamics, in turn, minimized power loss, inside the TCPC pathway is required for the best optimal surgical outcomes. To complement ongoing efforts to optimize the anatomical geometric design of the surgical Fontan templates, here, we focused on the characterization of power loss changes due to the temporal variations in between SVC and IVC flow waveforms. An experimentally validated pulsatile computational fluid dynamics solver is used to quantify the effect of phase-shift between SVC and IVC inflow waveforms and amplitudes on internal energy dissipation. The unsteady hemodynamics of two standard idealized TCPC geometries are presented, incorporating patient-specific real-time PC-MRI flow waveforms of "functional" Fontan patients. The effects of respiration and pulsatility on the internal energy dissipation of the TCPC pathway are analyzed. Optimization of phase-shift between caval flows is shown to lead to lower energy dissipation up to 30% in these idealized models. For physiological patient-specific caval waveforms, the power loss is reduced significantly (up to 11%) by the optimization of all three major harmonics at the same mean pathway flow (3 L/min). Thus, the hemodynamic efficiency of single ventricle circuits is influenced strongly by the caval flow waveform quality, which is regulated through respiratory dependent physiological pathways. The proposed patient-specific waveform optimization protocol may potentially inspire new therapeutic applications to aid postoperative hemodynamics and improve the well being of the Fontan patients. AD - Department of Biomedical Engineering, Carnegie Mellon University, Pittsburgh, PA 15219, USA. AN - 20459200 AU - Dur, O. AU - DeGroff, C. G. AU - Keller, B. B. AU - Pekkan, K. DA - Mar DB - PubMed DO - 10.1115/1.4000954 DP - NLM ET - 2010/05/13 IS - 3 KW - Animals Blood Flow Velocity Computer Simulation Energy Transfer/physiology Heart Bypass, Right/*methods Humans *Models, Cardiovascular Pulmonary Artery/*physiology/*surgery Vena Cava, Inferior/*physiology/*surgery Vena Cava, Superior/*physiology/*surgery LA - eng N1 - 1528-8951 Dur, Onur DeGroff, Curt G Keller, Bradley B Pekkan, Kerem Journal Article Research Support, Non-U.S. Gov't United States J Biomech Eng. 2010 Mar;132(3):031012. doi: 10.1115/1.4000954. PY - 2010 SN - 0148-0731 SP - 031012 ST - Optimization of inflow waveform phase-difference for minimized total cavopulmonary power loss T2 - J Biomech Eng TI - Optimization of inflow waveform phase-difference for minimized total cavopulmonary power loss VL - 132 ID - 510 ER - TY - JOUR AB - Tissue-engineered vascular grafts hold great promise in many clinical applications, especially in pediatrics wherein growth potential is critical. A continuing challenge, however, is identification of optimal scaffold parameters for promoting favorable neovessel development. In particular, given the countless design parameters available, including those related to polymeric microstructure, material behavior, and degradation kinetics, the number of possible scaffold designs is almost limitless. Advances in computationally modeling the growth and remodeling of native blood vessels suggest that similar simulations could help reduce the search space for candidate scaffold designs in tissue engineering. In this study, we meld a computational model of in vivo neovessel formation with a surrogate management framework to identify optimal scaffold designs for use in the extracardiac Fontan circulation while comparing the utility of different objective functions. We show that evolving luminal radius and graft compliance can be matched to that of the native vein by the end of the simulation period with judicious combinations of scaffold parameters, although the inability to match these metrics at all times reveals constraints engendered by current materials. We emphasize further that there is yet a need to examine additional metrics, and combinations thereof, when seeking to optimize functionality and reduce the potential for adverse outcomes. Tissue-engineered vascular grafts have considerable promise for treating myriad conditions, and multiple designs are now in FDA-approved trials. Nevertheless, the search continues for the optimal design of the underlying polymeric scaffold. We present a novel melding of a computational model of vascular adaptation and a formal method of optimization that can aid in identifying optimal design parameters, with potential to save development time and costs while improving clinical outcomes. © Copyright 2019, Mary Ann Liebert, Inc., publishers. AD - Department of Biomedical Engineering, Yale University, 55 Prospect Street, New Haven, CT 06520, United States Regenerative Medicine Program, Nationwide Children's Hospital, Columbus, OH, United States Departments of Pediatrics and Bioengineering, Stanford University, Stanford, CA, United States Vascular Biology and Therapeutics Program, Yale School of Medicine, New Haven, CT, United States AU - Szafron, J. M. AU - Ramachandra, A. B. AU - Breuer, C. K. AU - Marsden, A. L. AU - Humphrey, J. D. DB - Scopus DO - 10.1089/ten.tec.2019.0086 IS - 10 KW - computational modeling inflammation mechanobiology optimization scaffold M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2019 SP - 561-570 ST - Optimization of Tissue-Engineered Vascular Graft Design Using Computational Modeling T2 - Tissue Engineering - Part C: Methods TI - Optimization of Tissue-Engineered Vascular Graft Design Using Computational Modeling UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85073184595&doi=10.1089%2ften.tec.2019.0086&partnerID=40&md5=6776dd74ecfcc478a1bb9001b0ad27a1 VL - 25 ID - 1733 ER - TY - JOUR AB - We have studied the neurodevelopmental outcome of 104 consecutive unselected children who underwent open-heart surgery from 1987 through 1989. Survivors had formal neurologic and psychometric examinations after 2 years of age. Mean IQ was 90, and 78% had scores above 70. Cerebral palsy occurred in 22%. Deep hypothermia for longer than 45 minutes was associated with IQ less than 85 (P < .001) and later cerebral palsy (P = .02). Those less than 1 month old at operation had a median IQ of 96, and 25% had cerebral palsy. Median IQ for survivors of hypoplastic left heart syndrome was 66, only one had an IQ above 70, and 57% had cerebral palsy. Median IQ for transposition of great arteries was 109, only one was less than 85, and all had normal neurologic examinations. Those between 1 and 6 months of age at operation had a median IQ of 93, with 64% above 85, and 5% had cerebral palsy. Those older than 6 months had a median IQ of 99, with 70% above 85, and 13% had cerebral palsy. For infants less than 1 month old at operation, a strong association existed between outcome, type of lesion, and duration of deep hypothermia (P < .01), although not in all cases. In those older than 1 month at operation, no association existed between outcome and any study variable. Although the majority of children have an uneventful outcome after open-heart surgery, a significant morbidity exists. This is related to several factors, including type of lesion and duration of hypothermia, particularly in neonates; preoperative congenital and acquired lesions; and possible perioperative cerebrovascular events. AD - Section of Child Neurology, Baylor College of Medicine, Houston, TX, 77030, U AN - 8745386 AU - Miller, G. AU - Tesman, J. R. AU - Ramer, J. C. AU - Baylen, B. G. AU - Myers, J. L. DA - Jan DB - PubMed DO - 10.1177/088307389601100112 DP - NLM ET - 1996/01/01 IS - 1 KW - Age Factors *Cardiac Surgical Procedures/mortality/psychology Cardiopulmonary Bypass Child, Preschool Heart Defects, Congenital/*surgery Humans Infant Infant, Newborn Neurologic Examination Psychometrics Retrospective Studies Survival Rate Treatment Outcome LA - eng N1 - Miller, G Tesman, J R Ramer, J C Baylen, B G Myers, J L Comparative Study Journal Article Research Support, Non-U.S. Gov't United States J Child Neurol. 1996 Jan;11(1):49-53. doi: 10.1177/088307389601100112. PY - 1996 SN - 0883-0738 (Print) 0883-0738 SP - 49-53 ST - Outcome after open-heart surgery in infants and children T2 - J Child Neurol TI - Outcome after open-heart surgery in infants and children VL - 11 ID - 434 ER - TY - JOUR AB - Objective - To derive accurate survival figures in the current surgical era for counselling in early pregnancy after the diagnosis of fetal hypoplastic left heart syndrome. Setting - A tertiary referral centre for paediatric cardiology and cardiac surgery. Design - A retrospective study of the outcome in all cases of hypoplastic left heart syndrome presenting in fetal life between mid-1993 and the end of 1996. Patients - The diagnosis was made in 30 fetuses. In four of 12 identified before 24 weeks' gestation the mothers chose to terminate the pregnancy. There was an intention to treat in 24 of the remaining fetuses. Main outcome measure - Survival to six months of postnatal life. Results - Of the 24 infants, five were not offered Norwood stage I because of trisomy 18 (n = 2), unfavourable cardiac anatomy (n = 2), or neurological impairment (n = 1). One further infant did not survive to cardiac surgery after gastrointestinal surgery. Of the remaining 18 patients, eight had features that were considered to increase the risk of surgical repair. Of the 18 patients who underwent Norwood stage 1, there were nine survivors. There was a survival rate of 70% in infants undergoing surgery with no complicating features, a 50% survival of the all surgical candidates, and 37.5% survival from an intention to treat position. Conclusions - At the initial diagnosis of fetal hypoplastic left heart syndrome, the overall survival appears to be less than 40%. Evaluation must include detailed extracardiac and intracardiac assessment to predict the risks of surgical treatment. Prenatal counselling can be modified as pregnancy advances, depending on the detection or exclusion of complicating factors. AD - L.D. Allan, Department of Pediatric Cardiology, Babies Hospital 2N, Columbia Presbyterian Medical Center, 3959 Broadway, New York, NY 10032, United States AU - Allan, L. D. AU - Apfel, H. D. AU - Printz, B. F. DB - Embase Medline IS - 4 KW - article clinical article counseling echocardiography fetus first trimester pregnancy human hypoplastic left heart syndrome infant outcomes research prenatal diagnosis priority journal quality of life retrospective study survival LA - English M3 - Article N1 - L28197753 1998-05-17 PY - 1998 SN - 1355-6037 SP - 371-374 ST - Outcome after prenatal diagnosis of the hypoplastic left heart syndrome T2 - Heart TI - Outcome after prenatal diagnosis of the hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L28197753 VL - 79 ID - 1351 ER - TY - JOUR AB - BACKGROUND: From June 1995 to January 2003, 49 consecutive neonates of less than 2,500 g underwent early surgery for congenital heart disease. A retrospective analysis was performed to evaluate the early to medium term outcome. METHODS: Major cardiac surgery for congenital heart defects included a complete correction in 31 patients (group I) and a palliative procedure in 18 patients (group II). Mean age at operation was 15.2 days (1 day-90 days) and mean weight was 2,190 g (1,300 g-2,500 g). Twenty-four children (49%) were born prematurely. All neonates were critically ill and 47% were already ventilated preoperatively. Heart defects included mainly ventricular septal defect (10), tetralogy of Fallot complexes (8), aortic coarctation (8), transposition complexes (7), single ventricle anomalies (4), pulmonary atresia with intact septum (4), interrupted aortic arch (3), totally anomalous pulmonary venous return (3), and common atrioventricular septal defect (2). RESULTS: Overall surgical mortality was 18%: 4 neonates died after definitive repair and 5 after palliation; representing, respectively, 13% and 28% of each group. Postoperative morbidity occurred in half of the patients (53%). Age, weight, prematurity, type of first surgical procedure, and use of cardiopulmonary bypass did not influence the early outcome. After a mean follow-up of 2.82 years (2 months to 6 years), survival was 87% in the correction group and 54% in the palliation group. All children were in NYHA class I-II. Freedom from reintervention at 18 months was 68% after correction versus 8% after palliation. CONCLUSIONS: Cardiac surgery for congenital malformations in critically ill, low weight neonates can be achieved with acceptable mortality, at the cost of an increased morbidity. Early outcome seems independent of age, weight, prematurity, use of extracorporeal perfusion, and type of first intervention. Moreover, primary correction appears to result in an early survival benefit, remaining constant over time. AD - Department of Cardiac Surgery, University Hospital of Gent, Ghent, Belgium. thierry.bove@ugent.be AN - 15223425 AU - Bové, T. AU - François, K. AU - De Groote, K. AU - Suys, B. AU - De Wolf, D. AU - Verhaaren, H. AU - Matthys, D. AU - Moerman, A. AU - Poelaert, J. AU - Vanhaesebroeck, P. AU - Van Nooten, G. DA - Jul DB - PubMed DO - 10.1016/j.athoracsur.2003.12.066 DP - NLM ET - 2004/06/30 IS - 1 KW - Birth Weight Cardiac Surgical Procedures/mortality/*statistics & numerical data Cardiopulmonary Bypass/statistics & numerical data Critical Illness Female Gestational Age Heart Defects, Congenital/mortality/*surgery Hospital Mortality Humans *Infant, Low Birth Weight Infant, Newborn Infant, Premature Intellectual Disability/epidemiology Male Palliative Care Postoperative Complications/epidemiology Reoperation Retrospective Studies Risk Factors Treatment Outcome LA - eng N1 - 1552-6259 Bové, Thierry François, Katrien De Groote, Katya Suys, Bert De Wolf, Daniel Verhaaren, Hendrik Matthys, Dirk Moerman, Annelies Poelaert, Jan Vanhaesebroeck, Piet Van Nooten, Guido Comparative Study Journal Article Netherlands Ann Thorac Surg. 2004 Jul;78(1):181-7. doi: 10.1016/j.athoracsur.2003.12.066. PY - 2004 SN - 0003-4975 SP - 181-7 ST - Outcome analysis of major cardiac operations in low weight neonates T2 - Ann Thorac Surg TI - Outcome analysis of major cardiac operations in low weight neonates VL - 78 ID - 481 ER - TY - JOUR AB - Little is known about the outcomes of extremely low birth weight (ELBW) preterm infants with congenital heart defects (CHDs). The aim of this study was to assess the mortality, morbidity, and early childhood outcomes of ELBW infants with isolated CHD compared with infants with no congenital defects. Participants were 401-1,000 g infants cared for at National Institute of Child Health and Human Development Neonatal Research Network centers between January 1, 1998, and December 31, 2005. Neonatal morbidities and 18-22 months' corrected age outcomes were assessed. Neurodevelopmental impairment (NDI) was defined as moderate to severe cerebral palsy, Bayley II mental or psychomotor developmental index <70, bilateral blindness, or hearing impairment requiring aids. Poisson regression models were used to estimate relative risks for outcomes while adjusting for gestational age, small-for-gestational-age status, and other variables. Of 14,457 ELBW infants, 110 (0.8 %) had isolated CHD, and 13,887 (96 %) had no major birth defect. The most common CHD were septal defects, tetralogy of Fallot, pulmonary valve stenosis, and coarctation of the aorta. Infants with CHD experienced increased mortality (48 % compared with 35 % for infants with no birth defect) and poorer growth. Surprisingly, the adjusted risks of other short-term neonatal morbidities associated with prematurity were not significantly different. Fifty-seven (52 %) infants with CHD survived to 18-22 months' corrected age, and 49 (86 %) infants completed follow-up. A higher proportion of surviving infants with CHD were impaired compared with those without birth defects (57 vs. 38 %, p = 0.004). Risk of death or NDI was greater for ELBW infants with CHD, although 20 % of infants survived without NDI. © 2012 Springer Science+Business Media, LLC. AD - Department of Pediatrics, Children's Hospital of Michigan and Hutzel Women's Hospital, Wayne State University, 3901 Beaubien, Detroit MI 48201, United States Statistics and Epidemiology Unit, RTI International, Research Triangle Park NC 27709, United States Department of Pediatrics, University of Iowa, Iowa City IA 52242, United States Department of Pediatrics, Emory University, School of Medicine and Children's Healthcare of Atlanta, Atlanta GA 30303, United States Department of Pediatrics, Women and Infants' Hospital, Brown University, Providence RI 02905, United States Department of Pediatrics, Rainbow Babies and Children's Hospital, Case Western Reserve University, Cleveland OH 44106, United States Statistics and Epidemiology Unit, RTI International, Rockville MD 20852, United States Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda MD 20852, United States Department of Pediatrics, Duke University, Durham NC 27710, United States AU - Pappas, A. AU - Shankaran, S. AU - Hansen, N. I. AU - Bell, E. F. AU - Stoll, B. J. AU - Laptook, A. R. AU - Walsh, M. C. AU - Das, A. AU - Bara, R. AU - Hale, E. C. AU - Newman, N. S. AU - Boghossian, N. S. AU - Murray, J. C. AU - Cotten, C. M. AU - Adams-Chapman, I. AU - Hamrick, S. AU - Higgins, R. D. DB - Scopus DO - 10.1007/s00246-012-0375-8 IS - 8 KW - Congenital heart defects Extremely low birth weight Neurodevelopmental follow-up M3 - Article N1 - Cited By :20 Export Date: 15 June 2020 PY - 2012 SP - 1415-1426 ST - Outcome of extremely preterm infants (<1,000 g) with congenital heart defects from the national institute of child health and human development neonatal research network T2 - Pediatric Cardiology TI - Outcome of extremely preterm infants (<1,000 g) with congenital heart defects from the national institute of child health and human development neonatal research network UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84878251956&doi=10.1007%2fs00246-012-0375-8&partnerID=40&md5=13dc4a19cf7aef67a06d09c9f3d995a0 VL - 33 ID - 2079 ER - TY - JOUR AB - BACKGROUND: Multiple systemic-to-pulmonary artery shunts often represent a deviation from the initial management strategy for cyanotic congenital heart disease. This study analysed the outcome in patients undergoing a second shunt. METHODS: Between 1965 and 1998, 80 patients required a second shunt. Patient age ranged from 11 days to 11.2 years at the initial shunt, and 4 months to 25.6 years at the second shunt. The interval between shunts ranged from 11 days to 19.6 years. RESULTS: The patient survival rates at 1, 6, 12, 60 and 120 months following the second shunt were 95.0 +/- 2.4%, 92.5+/-3.0%, 87.4+/-3.7%. 82.4+/-4.5% and 74.8+/-5.8%, respectively. Although excess pulmonary flow was not encountered after the second shunt, mode and incidence of morbidity were otherwise similar to the first one. Between 9 months and 7.5 years after the second shunt, 24 patients underwent successful definitive repair. Multivariable analysis identified pulmonary atresia (p=0.027) and a short (<1 year) interval between the two shunts (p=0.034) as the independent risk factors for long-term shunt failure. Single ventricle physiology (p=0.002) and a central approach for the second shunt (p=0.016) were independent risk factors for lack of application, or failure of intracardiac definitive repair. CONCLUSIONS: A significant limitation in longevity and quality of life is common in patients requiring a second shunt, especially those associated with pulmonary atresia, the single ventricle physiology heart, or ungraftably hypoplastic contralateral branch pulmonary artery to the first shunt. Since excess pulmonary blood flow leading to congenital heart failure and/or ventricular diastolic dysfunction is unlikely, all patients who preclude definitive repair due to decreased pulmonary blood flow even after the first shunt should be shunted again. AD - Division of Cardiovascular Surgery, Keio University, Tokyo, Japan. AN - 10836217 AU - Aeba, R. AU - Katogi, T. AU - Takeuchi, S. AU - Kawada, S. DA - Feb DB - PubMed DP - NLM ET - 2000/06/03 IS - 1 KW - *Blood Vessel Prosthesis Implantation Child Child, Preschool Female Heart Defects, Congenital/mortality/*surgery Hospital Mortality Humans Infant Infant, Newborn Male Postoperative Complications/mortality/*surgery Pulmonary Artery/abnormalities/*surgery Reoperation Survival Analysis LA - eng N1 - Aeba, R Katogi, T Takeuchi, S Kawada, S Journal Article Italy J Cardiovasc Surg (Torino). 2000 Feb;41(1):23-30. PY - 2000 SN - 0021-9509 (Print) 0021-9509 SP - 23-30 ST - Outcome of patients with cyanotic congenital heart disease undergoing a second systemic-to-pulmonary artery shunt T2 - J Cardiovasc Surg (Torino) TI - Outcome of patients with cyanotic congenital heart disease undergoing a second systemic-to-pulmonary artery shunt VL - 41 ID - 494 ER - TY - JOUR AB - Background: Pacemaker implantation in patients with single ventricle is associated with poor outcomes. Objective: The purpose of this study was to determine the reasons for the poor outcomes of pacemaker implantation. Methods: We performed a retrospective chart review of patients with single ventricle who had undergone permanent pacemaker implantation. Patients were categorized into 3 groups based on the site of pacing and the proportion of ventricular pacing (VP) as follows: (1) atrial pacing group with atrial pacing only (n = 11); (2) low VP group with low daily VP proportion (<50%; n = 12); and (3) high VP group with high daily VP proportion (≥50%; n = 15). Pacing leads were placed at the epicardium in all patients. Results: No patients in the atrial pacing or low VP groups died, whereas the survival rate in the high VP group was 58.9% and 39.3% at 10 and 20 years, respectively, after pacemaker implantation. Among the post-Fontan patients, plasma brain natriuretic peptide (BNP) levels significantly increased with the proportion of VP: 11.7, 20.3, and 28.4 pg/mL in the atrial pacing, low VP, and high VP groups, respectively (P = 0.04). In the high VP group, the plasma BNP level was significantly lower in patients with an apical pacing lead than in those with a nonapical pacing lead (27.0 pg/mL vs 82.8 pg/mL, respectively; P = .03). Conclusion: A higher proportion of VP was associated with poor outcome and higher plasma BNP levels, probably due to ventricular dyssynchrony. In epicardial ventricular pacing, apical pacing is better to avoid the increase in ventricular stress and plasma BNP level. AD - Y. Kodama, Department of Pediatric Cardiology, Fukuoka Children's Hospital, Kashiiteriha 5-1-1, Higashi-ku, Fukuoka, Japan AU - Kodama, Y. AU - Kuraoka, A. AU - Ishikawa, Y. AU - Nakamura, M. AU - Ushinohama, H. AU - Sagawa, K. AU - Umemoto, S. AU - Hashimoto, T. AU - Sakamoto, I. AU - Ohtani, K. AU - Ide, T. AU - Tsutsui, H. AU - Ishikawa, S. DB - Embase Medline DO - 10.1016/j.hrthm.2019.06.019 IS - 12 KW - brain natriuretic peptide apical pacing article cardiac resynchronization therapy child epicardial ventricular pacing epicardium female Fontan procedure heart atrium pacing heart pacing heart single ventricle heart ventricle function Holter monitoring human major clinical study male medical record review pacemaker implantation preschool child priority journal protein blood level treatment outcome LA - English M3 - Article N1 - L2003463510 2019-11-12 2019-11-26 PY - 2019 SN - 1556-3871 1547-5271 SP - 1870-1874 ST - Outcome of patients with functional single ventricular heart after pacemaker implantation: What makes it poor, and what can we do? T2 - Heart Rhythm TI - Outcome of patients with functional single ventricular heart after pacemaker implantation: What makes it poor, and what can we do? UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2003463510 http://dx.doi.org/10.1016/j.hrthm.2019.06.019 VL - 16 ID - 583 ER - TY - JOUR AB - Objective: Total cavopulmonary connection (TCPC) is rarely performed for a functional single ventricle in children with Down syndrome; therefore, the postsurgical outcomes are not well known. We evaluated mortality and related factors after TCPC in children with Down syndrome. Methods: Between January 2004 and March 2010 we identified 8 patients with Down syndrome among 235 patients who had undergone TCPC. The preoperative clinical course, preoperative data, and postoperative clinical course were evaluated. In addition, clinical parameters and postoperative clinical course were compared between children with Down syndrome (n = 8) and a non-Down syndrome group (n = 227). Results: The median age at the time of TCPC was 4.1 years (range, 3.4-5.5 years), and the preoperative mean pulmonary artery pressure was 13.9 ± 1.81 mm Hg. We observed respiratory complications in 2 patients, surgical site infection in 3 patients, and chylothorax in 2 patients. No significant difference was observed in preoperative data and mortality rate (1 of 8 patients [12.5%] in the Down syndrome group; 5 of 227 patients [2.2%] in the non-Down syndrome group) between the groups of children with and without Down syndrome. However, when the postoperative clinical course was examined, the durations of intensive care unit stay (P =.009) and hospital stay (P =.007) were found to be significantly prolonged in the Down syndrome group. Conclusions: Patients with Down syndrome tend to show prolonged recovery after TCPC. However, as opposed to previous reports, the mortality rate of patients undergoing TCPC is lower with no significant difference from that of children without Down syndrome. Copyright © 2013 by The American Association for Thoracic Surgery. AD - T. Furukawa, Department of Pediatrics, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan AU - Furukawa, T. AU - Park, I. S. AU - Yoshikawa, T. AU - Nishimura, T. AU - Takahashic, Y. AU - Ando, M. AU - Wada, N. DB - Embase Medline DO - 10.1016/j.jtcvs.2013.02.017 IS - 6 KW - article artificial ventilation cavopulmonary connection child chylothorax clinical article disease course Down syndrome female follow up heterotaxy syndrome hospitalization human hypoplastic left heart syndrome intensive care unit lung artery pressure male mortality oxygen saturation preoperative care preschool child priority journal protein losing gastroenteropathy pulmonary hypertension pulmonary vascular disease retrospective study sudden death surgical infection total cavopulmonary connection LA - English M3 - Article N1 - L52500558 2013-03-22 2013-11-28 PY - 2013 SN - 0022-5223 1097-685X SP - 1349-1352 ST - Outcome of univentricular repair in patients with Down syndrome T2 - Journal of Thoracic and Cardiovascular Surgery TI - Outcome of univentricular repair in patients with Down syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52500558 http://dx.doi.org/10.1016/j.jtcvs.2013.02.017 VL - 146 ID - 980 ER - TY - JOUR AB - Purpose: Survival of neonatal and pediatric patients undergoing extracorporeal membrane oxygenation (ECMO) ≥ 21 days has not been well described. We hypothesized that patients would have poor survival and increased long-term complications. Methods: Retrospective, single center, review and case analysis. Tertiary-care university children’s hospital including neonatal, pediatric and cardiac intensive care units. After institutional review board approval, the charts of all patients < 18 years of age undergoing ECMO for ≥ 21 continuous days were performed, and they were compared to comparative patients undergoing shorter runs. Overall survival, incidence of complications, and post-discharge recovery were recorded. Results: Overall survival was 36% in patients undergoing ≥ 21 days of ECMO (N = 14). 5/8 patients with cardiopulmonary failure from acquired etiologies survived versus 0/6 patients with congenital anomalies. 1/5 survivors achieved complete recovery with no neurologic deficits. The remaining survivors suffer from multiple medical and neurodevelopmental morbidities. Conclusion: ECMO support for ≥ 21 days is associated with poor survival, particularly in neonates with congenital anomalies. Long-term outcomes for survivors ought to be carefully weighed and discussed with parents given the high incidence of neurologic morbidities in this population. AD - C.J. Hunter, Division of Pediatric Surgery, Ann and Robert H. Lurie Children’s Hospital of Chicago, 211 E Chicago Avenue, Box 63, Chicago, IL, United States AU - Ares, G. J. AU - Buonpane, C. AU - Helenowski, I. AU - Reynolds, M. AU - Hunter, C. J. DB - Embase Medline DO - 10.1007/s00383-019-04443-y IS - 3 KW - above knee amputation adult article bacterial pneumonia brain hemorrhage brain ischemia cardiopulmonary arrest child clinical article clinical outcome congenital malformation convalescence coronary care unit developmental delay diaphragm hernia extracorporeal oxygenation female heart failure human hypoplastic left heart syndrome learning disorder leg ischemia lung dysplasia lung hemorrhage male medical ethics medical record review mental disease middle aged morbidity myositis necrotizing pneumonia overall survival pediatric intensive care unit persistent pulmonary hypertension pertussis preschool child priority journal retrospective study school child superinfection tension pneumothorax thoracostomy thrombosis virus pneumonia LA - English M3 - Article N1 - L626168276 2019-02-05 2019-05-14 PY - 2019 SN - 1437-9813 0179-0358 SP - 321-328 ST - Outcomes and associated ethical considerations of long-run pediatric ECMO at a single center institution T2 - Pediatric Surgery International TI - Outcomes and associated ethical considerations of long-run pediatric ECMO at a single center institution UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L626168276 http://dx.doi.org/10.1007/s00383-019-04443-y VL - 35 ID - 621 ER - TY - JOUR AB - Background: Mortality and morbidity after surgical repair for complex congenital heart defects and severe cardiopulmonary failure on extracorporeal membrane oxygenation (ECMO) support remain high despite significant advances in medical management and technological improvements. We report on outcomes and factors after using ECMO in our surgical pediatric population including short- and long-term survival. Methods: A total of 45 neonatal and pediatric patients were identified who needed ECMO in our department between January 2008 and December 2016. In 41 cases (91%) a vaECMO (ECLS) was implemented, whereas 4 patients (9%) received vvECMO treatment for respiratory failure. In 33 cases vaECMO was implanted following cardiac surgery for congenital heart disease (CHD), whereas in 8 patients ECMO was utilized by means of extracorporeal cardiopulmonary resuscitation (eCPR) following refractory cardiac arrest. The primary endpoint of the present study was survival to discharge and long-term survival free from neurological impairments. Univariate and bivariate analysis was performed to address predictors for outcome. Kaplan-Meier survival analysis was used to address mid- and long-term survival. Results: Median [IQR] duration of ECMO support was 3 [2, 5] days (range, 1–17 days). Median age at ECMO implantation was 128 [14, 1,813] days, median weight of patients was 5.4 [3.3, 12] kg. Totally 10 patients included in this study were diagnosed with concomitant genetic conditions. A total of 20 (44%) patients were successfully weaned off ECMO (survived >24 h after ECMO explantation), whereas 15 (33%) of them survived to discharge. Single ventricle (SV) repair was performed in 14, biventricular repair in 19 patients. Neonates (<30 days of age), female patients, patients with genetic conditions, SV repair patients, and eCPR patient cohort showed lower odds of survival on ECMO. Failed myocardial recovery (P=0.001), profound circulatory failure despite a high dose of catecholamines (P<0.001), neurological impairment pre-ECMO and post-ECMO (P=0.04 and P<0.001, respectively), and severe pulmonary failure despite high respiratory pressure settings were most common mortality reasons. Conclusions: ECMO provides efficient therapy opportunities for life-threatening conditions. Nevertheless, neonates and pediatric patients who underwent ECMO were at high risk for cerebrovascular events and poor survival. Appropriate patient selection using predictors of outcome reducing complications might improve outcomes of this patient cohort. AD - F. Azizov, Department of Cardiothoracic Surgery, University Hospital of Cologne, Cologne, Germany AU - Azizov, F. AU - Merkle, J. AU - Fatullayev, J. AU - Eghbalzadeh, K. AU - Djordjevic, I. AU - Weber, C. AU - Saenko, S. AU - Kroener, A. AU - Zeriouh, M. AU - Sabashnikov, A. AU - Bennink, G. AU - Wahlers, T. DB - Embase DO - 10.21037/jtd.2018.11.107 KW - activated partial thromboplastin time arterial switch operation article bivariate analysis child childhood mortality clinical article computer assisted tomography creatinine clearance disease association elective surgery electroencephalography electronic medical record extracorporeal oxygenation female glomerulus filtration rate heart arrest heart failure heart single ventricle heart surgery hospital mortality human infant long term survival lung insufficiency male neonatal intensive care unit newborn newborn mortality outcome assessment patient selection pediatric intensive care unit pediatric patient platelet count postoperative complication postoperative period practice guideline prothrombin time quality of life respiratory failure resuscitation survival analysis urea nitrogen blood level LA - English M3 - Article N1 - L627537917 2019-05-14 2019-05-17 PY - 2019 SN - 2077-6624 2072-1439 SP - S871-S888 ST - Outcomes and factors associated with early mortality in pediatric and neonatal patients requiring extracorporeal membrane oxygenation for heart and lung failure T2 - Journal of Thoracic Disease TI - Outcomes and factors associated with early mortality in pediatric and neonatal patients requiring extracorporeal membrane oxygenation for heart and lung failure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L627537917 http://dx.doi.org/10.21037/jtd.2018.11.107 VL - 11 ID - 643 ER - TY - JOUR AB - OBJECTIVES: We investigated the results of a revision of a previous Fontan connection to total cavopulmonary connection (TCPC) in patients with failing Fontan circulation. METHODS: From July 1998 to April 2013, 21 patients who had failing Fontan circulation underwent revision of the previous Fontan operation to TCPC. The median age at TCPC conversion was 17.9 years (range, 4.6-38.1 years) and the median interval between initial Fontan operation and TCPC was 13.8 years (range, 2.1-25.4 years). There were 37 indications for Fontan revision in 21 patients. The indications were huge right atrium (n = 15), atrial arrhythmia (n = 8), intra-atrial thrombi (n = 6), protein-losing enteropathy (PLE) (n = 3) and more than mild atrioventricular valve regurgitation (n = 5). The previous Fontan operation was revised to extracardiac conduit replacement (n = 20) and intra-atrial lateral tunnel (n = 1). Concomitant surgery for atrial arrhythmia was performed in 8 patients. Fenestration was performed in 7 patients. The median follow-up duration was 7.1 years (range, 0.3-13.4 years). RESULTS: There were no operative deaths and two late deaths occurred 7.9 and 8.1 years after operation. Actuarial 5- and 10-year survival rates were 92.3 and 83.1%, respectively. Postoperative complications included bleeding (n = 3), deep sternal infection (n = 1) and prolonged pleural effusion for more than 2 weeks (n = 5). During follow-up, atrial arrhythmia recurred in 6 patients, PLE recurred in 2 patients and pleural effusion recurred in 2 patients. All patients were classified as New York Heart Association Class I (n = 15) or Class II (n = 4). CONCLUSIONS: Fontan conversion to TCPC in patients with failing Fontan circulation can be performed with low risk of morbidity and mortality. The procedure confers better quality of life and is functional for patients with failed Fontan circulation. AD - Division of Cardiovascular Surgery, Department of Thoracic and Cardiovascular Surgery, Severance Cardiovascular Hospital, Yonsei University Health System, Seoul, Republic of Korea. Department of Thoracic and Cardiovascular Surgery, College of Medicine, Chungbuk National University, Cheongju, Republic of Korea babymedi@naver.com. AN - 27001674 AU - Park, H. K. AU - Shin, H. J. AU - Park, Y. H. C2 - PMC4986739 DA - Jul DB - PubMed DO - 10.1093/icvts/ivw062 DP - NLM ET - 2016/03/24 IS - 1 KW - Adolescent Adult Arrhythmias, Cardiac/etiology/surgery Child Child, Preschool Female Fontan Procedure/*adverse effects Heart Defects, Congenital/*surgery Humans Male Postoperative Complications/epidemiology/surgery Pulmonary Artery/surgery Quality of Life Reoperation Retrospective Studies Shock/etiology/physiopathology/*surgery Survival Rate Treatment Outcome Young Adult *Fontan operation LA - eng N1 - 1569-9285 Park, Han Ki Shin, Hong Ju Park, Young Hwan Journal Article Interact Cardiovasc Thorac Surg. 2016 Jul;23(1):14-7. doi: 10.1093/icvts/ivw062. Epub 2016 Mar 21. PY - 2016 SN - 1569-9293 (Print) 1569-9285 SP - 14-7 ST - Outcomes of Fontan conversion for failing Fontan circulation: mid-term results T2 - Interact Cardiovasc Thorac Surg TI - Outcomes of Fontan conversion for failing Fontan circulation: mid-term results VL - 23 ID - 160 ER - TY - JOUR AB - Objectives To describe the experience of a single, tertiary care institution in the care of patients with Fontan physiology undergoing anesthesia for noncardiac surgery. Background The Fontan procedure was developed in 1971 to palliate patients with univentricular cardiac physiology leading to long-term survival of these patients, who may now present as adults for noncardiac surgery. Methods We retrospectively reviewed the medical records of Fontan patients 16 years and older who underwent general anesthesia for noncardiac surgery at Mayo Clinic in Rochester, Minnesota. Preoperative data, perioperative course, intraoperative and postoperative hemodynamic, pulmonary, cardiovascular, and renal complications were described. Results Thirty-nine general anesthetics were administered to 31 patients for noncardiac surgery after Fontan palliation. Perioperative complications occurred in 12 of the 39 (31%) noncardiac surgeries, and there was one postoperative death that occurred on day 13 after ventral hernia repair. The two patients who had complications that did not resolve (long-term dialysis and death) had ejection fractions well below the mean for the group (22% and 28%). Conclusion It may be more appropriate for Fontan patients to undergo anesthesia for noncardiac surgery in a tertiary institution, particularly patients with an ejection fraction of <30%. Intraoperative arterial blood pressure monitoring and overnight admission are likely appropriate for most cases. © 2012 Blackwell Publishing Ltd. AD - Department of Anesthesiology, Mayo Clinic, Rochester, MN, United States Department of Cardiovascular Surgery, Mayo Clinic, Rochester, MN, United States Department of Internal Medicine, Mayo Clinic, Rochester, MN, United States Department of Cardiovascular Disease, Mayo Clinic, Rochester, MN, United States Department of Anesthesiology and Pain Medicine, Seattle Children's Hospital, University of Washington School of Medicine, 4800 Sand Point Way NE, Seattle, WA 98105, United States AU - Rabbitts, J. A. AU - Groenewald, C. B. AU - Mauermann, W. J. AU - Barbara, D. W. AU - Burkhart, H. M. AU - Warnes, C. A. AU - Oliver Jr, W. C. AU - Flick, R. P. DB - Scopus DO - 10.1111/pan.12020 IS - 2 KW - congenital heart disease single ventricle univentricular M3 - Article N1 - Cited By :28 Export Date: 15 June 2020 PY - 2013 SP - 180-187 ST - Outcomes of general anesthesia for noncardiac surgery in a series of patients with Fontan palliation T2 - Paediatric Anaesthesia TI - Outcomes of general anesthesia for noncardiac surgery in a series of patients with Fontan palliation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84872078242&doi=10.1111%2fpan.12020&partnerID=40&md5=a216e59eb768b5548b21e11ddd6bbdfa VL - 23 ID - 2064 ER - TY - JOUR AB - Aim: To study the influence of a prenatal diagnosis on parental choice of treatment for infants born with hypoplastic left heart syndrome (HLHS). Methods: Retrospective review of medical records of infants admitted to a paediatric tertiary hospital 1983-2004 with a diagnosis of HLHS. Results: A total of 201 infants had HLHS diagnosed by echocardiography after birth with 129 subsequently undergoing surgery and 72 non-surgical management (compassionate/comfort care). When the diagnosis of HLHS was made prenatally, 68 of 71 (96%) infants underwent surgery whereas when the diagnosis was made post-natally 61 of 130 (47%) infants underwent surgery. Prenatal diagnosis was significantly associated with a parental choice of surgery (P < 0.001). Among 72 infants managed non-surgically, compassionate care was chosen by parents for 49 (68%) despite being fit for surgery, 20 (28%) were deemed unfit for surgery and in three (4%) the reasons for non-surgical management were unknown. Of the total 178 infants considered fit for surgery, 68 (38%) had had a prenatal diagnosis and 110 (62%) did not. Of 20 considered unfit for surgery, none had had a prenatal diagnosis. A prenatal diagnosis was significantly associated with fitness for surgery (P < 0.001). Conclusions: Approximately half of parents chose non-surgical management (compassionate care) of their infant if the diagnosis was post-natal. Although most parents of infants born with a prenatal diagnosis of HLHS chose surgical management of their infant, this is the likely influence of termination of pregnancy for this condition. Lack of prenatal diagnosis compromised fitness for surgery. © 2008 The Authors. AD - J. Tibballs, Intensive Care Unit, Royal Children's Hospital, Flemington Road, Melbourne, VIC 3052, Australia AU - Tibballs, J. AU - Cantwell-Bartl, A. DB - Embase Medline DO - 10.1111/j.1440-1754.2007.01265.x IS - 6 KW - article decision making heart surgery human hypoplastic left heart syndrome infant major clinical study outcome assessment parental attitude patient care prenatal diagnosis priority journal LA - English M3 - Article N1 - L351677479 2008-06-03 PY - 2008 SN - 1034-4810 1440-1754 SP - 321-324 ST - Outcomes of management decisions by parents for their infants with hypoplastic left heart syndrome born with and without a prenatal diagnosis T2 - Journal of Paediatrics and Child Health TI - Outcomes of management decisions by parents for their infants with hypoplastic left heart syndrome born with and without a prenatal diagnosis UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L351677479 http://dx.doi.org/10.1111/j.1440-1754.2007.01265.x VL - 44 ID - 1206 ER - TY - JOUR AB - INTRODUCTION: Fontan operation is a palliative procedure used in patients with univentricular heart (UVH). There have been numerous modifications to the technique. The appropriate strategy for treating patients with UVH in Thailand is still a major concern. OBJECTIVE: To describe the outcomes of patients after straightforward Fontan operation at advanced-age and evaluate their post-surgical cardiac performance. MATERIAL AND METHOD: A cross sectional study was performed in post-Fontan operation patients at Rajvithi Hospital between 2000 and 2009. Post-operative cardiac performance was analyzed using the echocardiogram and treadmill exercise stress test (EST). RESULTS: Thirty-nine patients were enrolled in the present study. The mean age was 11.7 years and the mean age at operation was 7.4 years. The predominant systemic chamber was morphologic left ventricle. Straightforward Fontan operation was performed in 60% of cases. Mean of Fontan index, mean of McGoon ratio, and mean of Nakata index were 2.63, 2.32, and 414.15 sqmm/sqm, respectively. Mean of the PVR and PAP were 1.98 U/m2 and 11.05 mmHg. Eighteen percent of patients died in the early post-operative period. Most of the patients died from septicemia. The mean EFwas 58.43%. Systolic dysfunction in 17.9% of the cases was abnormal, whereas diastolic dysfunction was present in half of the cases. Thirty-nine percent had MPI abnormality. During EST three cases developed arrhythmias and 3 cases had hypotension. Patients were categorized into 3 groups (Group 1: normal systolic and diastolic functions, Group 2: diastolic dysfunction, Group 3: impairment of both systole and diastole). Pre-operative cardiac catheterization parameters, surgical data and cardiac performance showed no statistical significance. However, there was a significant correlation between those with abnormal cardiac performance and arrhythmias or hypotension during EST (p = 0.003). CONCLUSION: The selection of suitable cases and good pre-operative evaluation could decrease the morbidity and mortality in patients undergoing the Fontan procedure. This study also found a correlation between abnormal cardiac performance and transient cardiac arrhythmia during exercise. The evaluation of cardiac performance and EST remains to be performed for following-up of patients who have undergone the Fontan operation, even for the asymptomatic cases. AN - 25391195 AU - Prachasilchai, P. AU - Promphan, W. AU - Katanyuwong, P. AU - Layangool, T. AU - Sangtawesin, C. AU - Kirawittaya, T. AU - Mokarapong, P. DA - Jun DB - PubMed DP - NLM ET - 2014/11/14 KW - Adolescent Arrhythmias, Cardiac/surgery Brugada Syndrome Cardiac Catheterization/methods Cardiac Conduction System Disease Child Child, Preschool Cross-Sectional Studies Diastole Echocardiography Female Fontan Procedure/adverse effects/*methods Heart Heart Conduction System/abnormalities Heart Ventricles/surgery Humans Male Postoperative Period Systole Thailand LA - eng N1 - Prachasilchai, Pimpak Promphan, Worakan Katanyuwong, Poomiporn Layangool, Thanarat Sangtawesin, Chaisit Kirawittaya, Thawatchai Mokarapong, Pirapat Journal Article Thailand J Med Assoc Thai. 2014 Jun;97 Suppl 6:S205-12. PY - 2014 SN - 0125-2208 (Print) 0125-2208 SP - S205-12 ST - Outcomes of straightforward extracardiac Fontan operation in advanced-age group T2 - J Med Assoc Thai TI - Outcomes of straightforward extracardiac Fontan operation in advanced-age group VL - 97 Suppl 6 ID - 321 ER - TY - JOUR AB - Background: The Fontan-Kreutzer procedure (FK) was widely performed in the past, but in the long-term generated many complications resulting in univentricular circulation failure. The conversion to total cavopulmonary connection (TCPC) is one of the options for treatment. Objective: To evaluate the results of conversion from FK to TCPC. Methods: A retrospective review of medical records for patients who underwent the conversion of FK to TCPC in the period of 1985 to 2016. Significance p < 0,05. Results: Fontan-type operations were performed in 420 patients during this period: TCPC was performed in 320, lateral tunnel technique in 82, and FK in 18. Ten cases from the FK group were elected to conversion to TCPC. All patients submitted to Fontan Conversion were included in this study. In nine patients the indication was due to uncontrolled arrhythmia and in one, due to protein-losing enteropathy. Death was observed in the first two cases. The average intensive care unit (ICU) length of stay (LOS) was 13 days, and the average hospital LOS was 37 days. A functional class by New York Heart Association (NYHA) improvement was observed in 80% of the patients in NYHA I or II. Fifty-seven percent of conversions due to arrhythmias had improvement of arrhythmias; four cases are cured. Conclusions: The conversion is a complex procedure and requires an experienced tertiary hospital to be performed. The conversion has improved the NYHA functional class despite an unsatisfactory resolution of the arrhythmia. AD - G.C. Fernandes, Rua Pintassilgo, 516, apt. 98, Vila Uberabinha, São Paulo, SP, Brazil AU - Fernandes, G. C. AU - da Silva, G. V. R. AU - Caneo, L. F. AU - Tanamati, C. AU - Turquetto, A. L. R. AU - Jatene, M. B. DB - Embase Medline DO - 10.5935/abc.20180256 IS - 2 KW - adult article blood clotting disorder bradycardia cardiovascular surgery cavopulmonary connection child cryoablation female Fontan procedure heart arrhythmia heart left ventricle volume heart transplantation heart ventricle function hospitalization human intensive care unit ischemia lateral tunnel technique length of stay major clinical study male middle aged mortality operation duration protein losing gastroenteropathy quality of life retrospective study survival rate univentricular circulation failure LA - English M3 - Article N1 - L2001587919 2019-03-12 2019-03-15 PY - 2019 SN - 1678-4170 0066-782X SP - 130-135 ST - Outcomes of the conversion of the fontan-kreutzer operation to a total cavopulmonary connection for the failing univentricular circulation T2 - Arquivos Brasileiros de Cardiologia TI - Outcomes of the conversion of the fontan-kreutzer operation to a total cavopulmonary connection for the failing univentricular circulation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2001587919 http://dx.doi.org/10.5935/abc.20180256 VL - 112 ID - 629 ER - TY - JOUR AB - The number of children now surviving surgical interventions because of congenital cardiac disease continues to increase. Indeed, it is estimated that, by the end of 2005, there will be more adults than children with congenital cardiac malformations. The complex challenges facing this growing population are only now becoming apparent, including the continued hazard for mortality, the risk of reoperation, and newly-emerging late sequels of surgery. In particular, children with severe lesions requiring intervention in the neonatal period are surviving at levels thought to be highly improbable no more than two decades ago. Heralded by the availability of prostaglandin E1 in the late 1970s, improvements in technology, such as echocardiography, and color Doppler in particular, intensive care and intraoperative support, have recently allowed surgeons to reconstruct the hearts of many children with otherwise lethal defects. Much of the published literature has focused on the cardiac sequels in this population. Increasing attention has recently been given, nonetheless, to the neurodevelopmental outcomes in this population of patients. © 2005 Cambridge University Press. AD - G. Wernovsky, Division of Pediatric Cardiology, The Children's Hospital of Philadelphia, 34th Street, Philadelphia, PA 19104, United States AU - Wernovsky, G. DB - Embase Medline DO - 10.1017/S1047951105001162 IS - SUPPL. 1 KW - article cardiopulmonary bypass central nervous system disease child cognition congestive heart failure disease severity heart arrhythmia heart surgery human hypoplastic left heart syndrome learning disorder mortality perioperative period postoperative period reoperation seizure cerebrovascular accident surgical technique survival LA - English M3 - Article N1 - L44604541 2006-11-13 PY - 2005 SN - 1047-9511 1467-1107 SP - 132-133 ST - Outcomes regarding the central nervous system in children with complex congenital cardiac malformations T2 - Cardiology in the Young TI - Outcomes regarding the central nervous system in children with complex congenital cardiac malformations UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L44604541 http://dx.doi.org/10.1017/S1047951105001162 VL - 15 ID - 1283 ER - TY - JOUR AB - Oxidation reduction potential (ORP) or Redox is the ratio of activity between oxidizers and reducers. Oxidative stress (OS) can cause cellular injury and death, and is important in the regulation of immune response to injury or disease. In the present study, we investigated changes in the redox system as a function of cardiopulmonary bypass (CPB) in pediatric patients. 664 plasma samples were collected from 162 pediatric patients having cardiac surgery of various CPB times. Lower ORP values at 12 h post-CPB were associated with poor survival rate (mean ± SD 167 ± 20 vs. 138 ± 19, p = 0.005) and higher rate of thrombotic complications (153 ± 21 vs. 168 ± 20, p < 0.008). Similarly, patients who developed infections had lower ORP values at 6 h (149 ± 19 vs. 160 ± 22, p = 0.02) and 12 h (156 ± 17 vs. 168 ± 21, p = 0.004) post-CPB. Patients that developed any post-operative complication also had lower 6 h (149 ± 17 vs. 161 ± 23, p = 0.002) and 12 h (157 ± 18 vs. 170 ± 21, p = 0.0007) post-CPB ORP values. Free hemoglobin and IL-6, IL-10, and CRP were not associated with ORP levels. However, higher haptoglobin levels preoperatively were protective against decreases in ORP. Decreased ORP is a marker for poor outcome and predictive of post-operative thrombosis, infection, and other complications in critically ill pediatric cardiac surgery patients. These results suggest that redox imbalance and OS may contribute to the risk of complications and poor outcome in pediatric CBP patients. Haptoglobin may be a marker for increased resilience to OS in this population. AD - A.E. Schmidt, Department of Pathology and Laboratory Medicine, University of Rochester Medical Center, 601 Elmwood Ave, Box 608, Rochester, NY, United States AU - Schmidt, A. E. AU - Gore, E. AU - Henrichs, K. F. AU - Conley, G. AU - Dorsey, C. AU - Bjugstad, K. B. AU - Refaai, M. A. AU - Blumberg, N. AU - Cholette, J. M. DB - Embase Medline DO - 10.1007/s00246-017-1755-x IS - 2 KW - NCT00693498 C reactive protein haptoglobin hemoglobin interleukin 10 interleukin 6 adolescent article bleeding cardiopulmonary bypass child complete heart block controlled study cyanotic heart disease female heart single ventricle heart surgery human hypoxia infant junctional ectopic tachycardia major clinical study male newborn oxidation reduction potential pediatric surgery postoperative complication predictive value preoperative period surgical infection survival rate thrombosis LA - English M3 - Article N1 - L619064692 2017-11-08 2018-08-13 PY - 2018 SN - 1432-1971 0172-0643 SP - 299-306 ST - Oxidation Reduction Potential (ORP) is Predictive of Complications Following Pediatric Cardiac Surgery T2 - Pediatric Cardiology TI - Oxidation Reduction Potential (ORP) is Predictive of Complications Following Pediatric Cardiac Surgery UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L619064692 http://dx.doi.org/10.1007/s00246-017-1755-x VL - 39 ID - 706 ER - TY - JOUR AB - Objective: The type of congenital heart disease, early diagnosis, and treatment modality all play an important role in the morbidity and mortality of these diseases. This study examined the relationship between congenital heart disease and oxidative stress in children with cyanotic and acyanotic congenital heart disease. Methods: In this case-controlled cross-sectional study, the study groups consisted of 29 patients with cyanotic heart disease, 30 patients with acyanotic heart disease, and a control group of 32 healthy individuals. For all groups, the total oxidant status (TOS), total antioxidant status (TAS), and oxidative stress index (OSI) were calculated. Of the cyanotic group, 12 were diagnosed with tetralogy of Fallot, 7 with transposition of the great arteries, 4 with tricuspid atresia, 4 with double outlet right ventricle and 2 with truncus arteriosus. In the acyanotic group 19 patients had ventricular septal defect (VSD), 5 atrial septal defect (ASD) and 6 patent ductus arteriosus (PDA). Statistical analyses were performed using Chi-square, Mann-Whitney U and Kruskal-Wallis tests. Results: The plasma TAS, TOS, and OSI were significantly higher in the cyanotic group than in the acyanotic group (p<0.0001, p<0.01, and p<0.01, respectively) and controls (p<0.0001, p<0.0001, and p<0.0001, respectively). A comparison between the acyanotic and control groups showed no statistical differences. Conclusion: The level of oxidative stress in patients with cyanotic congenital heart disease was significantly higher than in the acyanotic and control groups, which were similar. ©Copyright 2009 by AVES Yayincilik Ltd. AD - A. Çakmak, Harran University School of Medicine, Department of Pediatrics, 63100, Şanliurfa, Turkey AU - Ercan, S. AU - Çakmak, A. AU - Kösecik, M. AU - Erel, Ö DB - Embase Medline IS - 6 KW - antioxidant antioxidant activity arterial trunk article chi square test child controlled study cross-sectional study cyanotic heart disease Fallot tetralogy female heart atrium septum defect heart disease heart right ventricle double outlet heart ventricle septum defect human infant Kruskal Wallis test major clinical study male morbidity mortality newborn oxidative stress patent ductus arteriosus preschool child rank sum test statistical analysis statistical significance tricuspid valve atresia LA - English M3 - Article N1 - L358102081 2010-02-22 http://www.anakarder.com/eng/sayilar/57/486-490.pdf PY - 2009 SN - 1302-8723 SP - 486-490 ST - The oxidative state of children with cyanotic and acyanotic congenital heart disease T2 - Anadolu Kardiyoloji Dergisi TI - The oxidative state of children with cyanotic and acyanotic congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L358102081 VL - 9 ID - 1164 ER - TY - JOUR AB - Introduction: As survival of previously considered as lethal congenital heart disease forms is the case in our days, issues regarding quality of life including sport and daily activities emerge. In patients with Fontan circulation, there is no pump to propel blood into the pulmonary arteries since the systemic veins are directly connected to the pulmonary arteries. The complex hemodynamics of Fontan circulation include atrial function, peripheral muscle pump, integrity of the atrioventricular valve, absence of restrictive, or obstructive pulmonary lung function. Therefore, thoracic mechanics are of particular importance within the complex hemodynamics of Fontan circulation. Methods: To understand the physiology of respiratory muscles, the aim of this study was to examine the matching of auxiliary respiratory muscle oxygen delivery and utilization during incremental exercise in young male Fontan patients (n = 22, age = 12.04 ± 2.51) and healthy Controls (n = 10, age = 14.90 ± 2.23). All subjects underwent a cardiopulmonary exercise test (CPET) to exhaustion whereas respiratory muscle oxygenation was measured non-invasively using a near-infrared spectrometer (NIRS). Results: CPET revealed significantly lower peak power output, oxygen uptake and breath activity in Fontan patients. The onset of respiratory muscle deoxygenation was significantly earlier. The matching of local muscle perfusion to oxygen demand was significantly worse in Fontans between 50 and 90% V.O2peak . Findings: The results indicate that (a) there is high strain on respiratory muscles during incremental cycling exercise and (b) auxiliary respiratory muscles are worse perfused in patients who underwent a Fontan procedure compared to healthy Controls. This might be indicative of a more general skeletal muscle strain and worse perfusion in Fontan patients rather than a localized-limited to thoracic muscles phenomenon. AD - Department for Sport and Health Sciences, Teaching and Educational Center, Technical University Munich, Munich, Germany. Department of Pediatric Cardiology and Congenital Heart Defects, German Heart Centre, Munich, Germany. Department for Sport and Health Sciences, Chair of Preventive Pediatrics, Technical University Munich, Munich, Germany. AN - 30972311 AU - Stöcker, F. AU - Neidenbach, R. AU - Fritz, C. AU - Oberhoffer, R. M. AU - Ewert, P. AU - Hager, A. AU - Nagdyman, N. C2 - PMC6443956 DB - PubMed DO - 10.3389/fped.2019.00096 DP - NLM ET - 2019/04/12 KW - Chd Fontan Nirs muscle oxygenation pediatrics respiratory muscles LA - eng N1 - 2296-2360 Stöcker, Fabian Neidenbach, Rhoia Fritz, Celina Oberhoffer, Renate M Ewert, Peter Hager, Alfred Nagdyman, Nicole Journal Article Front Pediatr. 2019 Mar 26;7:96. doi: 10.3389/fped.2019.00096. eCollection 2019. PY - 2019 SN - 2296-2360 (Print) 2296-2360 SP - 96 ST - Oxygen Availability in Respiratory Muscles During Exercise in Children Following Fontan Operation T2 - Front Pediatr TI - Oxygen Availability in Respiratory Muscles During Exercise in Children Following Fontan Operation VL - 7 ID - 42 ER - TY - JOUR AB - OBJECTIVES: To evaluate whether the degree of hypoxemia following stage-I and stage-II palliative surgeries predicts neurodevelopmental outcomes at 14 months of age in children with single ventricle congenital heart disease (SVCHD). DESIGN: We analyzed longitudinal data from two Pediatric Heart Network (PHN) randomized controlled trials, with a total of 328 subjects. Oxygen saturations, measured via pulse oximetry, at time of discharge from stage-I and stage-II surgeries were the primary predictors of interest, and Bayley Scales of Infant Development-II (BSID-II) scores at 14 months old were the primary outcome measure. Relevant covariates from previously-published PHN studies were also included in regression models. RESULTS: Oxygen saturations at time of discharge from stage-I and stage-II surgeries were not related to BSID-II scores. Having one or more oxygen saturation measurements below 80% was also not associated with BSID-II scores, and neither was change in oxygen saturations over time. These relationships were not altered by inclusion of relevant covariates. CONCLUSIONS: In this large cohort of children with SVCHD, oxygen saturations post-stage-I and post-stage-II palliation surgeries as measured via pulse oximetry were not associated with neurodevelopmental outcomes at 14 months of age. The relationship between oxygen saturations and neurodevelopment in SVCHD is likely complex, and neurodevelopment is known to be affected by a number of factors. Pulse oximetry may also be an insufficient proxy for cerebral oxygen delivery. Clinically, pulse oximetry readings during the interstage and post-stage-II surgery periods are not a reliable predictor of future neurodevelopmental risk. AD - Pediatric Neurology, University of Colorado School of Medicine and Children's Hospital, Aurora, Colorado. Department of Biostatistics and Informatics, Colorado School of Public Health, University of Colorado, Aurora, Colorado. Heart Institute, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, Colorado. Pediatric Pulmonary Medicine, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, Colorado. AN - 30919581 AU - Wolfe, K. R. AU - Brinton, J. AU - Di Maria, M. V. AU - Meier, M. AU - Liptzin, D. R. DA - Jun DB - PubMed DO - 10.1002/ppul.24275 DP - NLM ET - 2019/03/29 IS - 6 KW - Female Heart Defects, Congenital/complications/*surgery Humans Hypoxia Infant Infant, Newborn Longitudinal Studies Male Neurodevelopmental Disorders/*diagnosis/prevention & control *Oximetry Oxygen/*adverse effects/*therapeutic use Patient Discharge Postoperative Period Psychomotor Disorders/*diagnosis/prevention & control Regression Analysis Retrospective Studies Risk Treatment Outcome Univentricular Heart/complications/*surgery *blood oxygenation *neurodevelopment *pediatric heart network *pulse oximetry *single ventricle LA - eng N1 - 1099-0496 Wolfe, Kelly R Orcid: 0000-0003-4754-4577 Brinton, John Di Maria, Michael V Meier, Maxene Liptzin, Deborah R Orcid: 0000-0002-3667-1856 Journal Article Multicenter Study Randomized Controlled Trial Research Support, Non-U.S. Gov't United States Pediatr Pulmonol. 2019 Jun;54(6):922-927. doi: 10.1002/ppul.24275. Epub 2019 Mar 28. PY - 2019 SN - 1099-0496 SP - 922-927 ST - Oxygen saturations and neurodevelopmental outcomes in single ventricle heart disease T2 - Pediatr Pulmonol TI - Oxygen saturations and neurodevelopmental outcomes in single ventricle heart disease VL - 54 ID - 76 ER - TY - JOUR AB - Cardiovascular function accounts for endurance performance and, by a rapid increase in oxygen supply at the onset of exercise, contributes to short-lasting intensive exertion prevailing in many kinds of sports activities. This study determined the time for oxygen uptake (V̇O2) to reach one half of its asymptotic level above rest, and the respective oxygen deficit following the onset of a constant-load exercise corresponding to 80% to 90% of the maximal oxygen uptake (V̇O2max) in 35 children after surgical closure of a ventricular septal defect, after corrective surgery for tetralogy of Fallot, or after the Senning, Mustard, or Fontan procedures. In addition, V̇O2max and maximal Mood lactate were determined. The half-time of V̇O2 response and oxygen deficit was lowest in patients after closure of a ventricular septal defect, whereas children had the most unfavorable V̇O2 transient kinetics after Fontan operation or with a transannular patch after correction of tetralogy of Fallot. A negative correlation was evaluated between half-time of V̇O2 response and V̇O2max, whereas maximal blood lactate varied independently of V̇O2max. It is concluded that many children with decreased V̇O2max after cardiac surgery also have unfavorable V̇O2 transient kinetics. In addition to being less qualified for endurance performance, they are also less prepared for short-lasting intensive energy expenditure. Therefore, the primary aim of training in these children is to improve the economy of motion of the respective tasks. © 1994. AD - Department of Paediatric Cardiology, University of Freiburg, Freiburg, Germany AU - Gildein, P. AU - Mocellin, R. AU - Kaufmehl, K. DB - Scopus DO - 10.1016/0002-9149(94)90091-4 IS - 2 M3 - Article N1 - Cited By :17 Export Date: 15 June 2020 PY - 1994 SP - 166-169 ST - Oxygen uptake transient kinetics during constant-load exercise in children after operations of ventricular septal defect, tetralogy of Fallot, transposition of the great arteries, or tricuspid valve atresia T2 - The American Journal of Cardiology TI - Oxygen uptake transient kinetics during constant-load exercise in children after operations of ventricular septal defect, tetralogy of Fallot, transposition of the great arteries, or tricuspid valve atresia UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0028237584&doi=10.1016%2f0002-9149%2894%2990091-4&partnerID=40&md5=1c177ed861d6e1dd0c58641da231e74a VL - 74 ID - 2307 ER - TY - JOUR AN - 104250053. Language: English. Entry Date: 20130312. Revision Date: 20150711. Publication Type: Journal Article AU - Overgaard, D. O. AU - Schrader, A. M. S. AU - Hoist, K. H. L. Lisby AU - King, C. A. T. AU - Friis, R. F. C. Christensen AU - Friis, H. F. J. Jensen AU - Moons, P. M. DB - ccm DO - 10.1016/S1474-5151(11)60062-5 DP - EBSCOhost IS - s KW - Outcomes (Health Care) Heart Ventricle -- Abnormalities Heart Defects, Congenital Functional Status Human Denmark Cross Sectional Studies Descriptive Research Surveys Adolescence Adult Middle Age Functional Assessment Quality of Life Health Status Scales Short Form-36 Health Survey (SF-36) Questionnaires Comparative Studies N1 - abstract; research. Supplement Title: Apr2011 Supplement 1. Journal Subset: Continental Europe; Europe; Nursing; Peer Reviewed. Instrumentation: Short Form-36 Health Survey (SF-36); Hospital Anxiety and Depression Scale (HADS); Linear Analogue Scale (LAS); 13-item Sense of Coherence Scale. NLM UID: 101128793. PY - 2011 SN - 1474-5151 SP - S16-S16 ST - P146 Patient-reported outcomes in patients with single ventricle physiology in Denmark T2 - European Journal of Cardiovascular Nursing TI - P146 Patient-reported outcomes in patients with single ventricle physiology in Denmark UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104250053&site=ehost-live&scope=site VL - 10 ID - 1650 ER - TY - JOUR AB - This is a report of a unique DCD paediatric heart transplant whereby normothermic regional perfusion was used to assess DCD heart function after death followed by ex situ heart perfusion of the graft during transportation from donor to recipient hospitals. The DCD donor was a 9-year-old boy weighing 84 kg. The recipient was 7-year-old boy with failing Fontan circulation and weighed 23 kg. It was an ABO-compatible heart transplantation. The DCD heart was reperfused and assessed using normothermic regional perfusion followed by portable ex situ heart perfusion during transportation. The orthotopic heart transplantation was successful with good graft function and no evidence of rejection on endomyocardial biopsy at 30 days post-transplant. At 1-year follow-up, excellent graft function is maintained, and he is attending school with a good quality of life. DCD heart transplantation in children is a promising solution to reducing paediatric waiting times. The case demonstrates the feasibility of using normothermic regional perfusion in the donor and ex situ heart perfusion during graft transportation. This combination allowed a functional assessment whilst minimizing warm ischaemia resulting in a successful outcome. More research and long-term follow-up are needed in order to benefit from the huge potential that paediatric DCD heart transplantation has to offer. AD - Department of Cardiopulmonary Transplantation, Freeman Hospital, Newcastle Upon Tyne, UK. Department of Cardiothoracic Surgery and Transplantation, Freeman Hospital, Newcastle Upon Tyne, UK. Department of Cardiac Anaesthesia and Intensive Care, Freeman Hospital, Newcastle Upon Tyne, UK. Department of Cardiothoracic Transplantation, Royal Papworth Hospital, Cambridgeshire, UK. Department of Cardiothoracic Surgery, Institute of Cellular Medicine, Newcastle University, Newcastle Upon Tyne, UK. AN - 31273913 AU - Khushnood, A. AU - Butt, T. A. AU - Jungschleger, J. AU - Henderson, P. AU - Smith, J. H. AU - De Rita, F. AU - Nassar, M. AU - Page, A. AU - Messer, S. AU - Large, S. AU - Dark, J. H. AU - Parry, G. AU - Reinhardt, Z. AU - Hasan, A. DA - Sep DB - PubMed DO - 10.1111/petr.13536 DP - NLM ET - 2019/07/06 IS - 6 KW - *donation after circulatory determined death *heart transplantation *normothermic regional perfusion *paediatric LA - eng N1 - 1399-3046 Khushnood, Abbas Orcid: 0000-0001-9258-5605 Butt, Tanveer A Jungschleger, Jerome Henderson, Paul Smith, Jonathan H De Rita, Fabrizio Nassar, Mohamed Page, Aravinda Messer, Simon Large, Stephen Dark, John H Parry, Gareth Reinhardt, Zdenka Hasan, Asif Case Reports Denmark Pediatr Transplant. 2019 Sep;23(6):e13536. doi: 10.1111/petr.13536. Epub 2019 Jul 4. PY - 2019 SN - 1397-3142 SP - e13536 ST - Paediatric donation after circulatory determined death heart transplantation using donor normothermic regional perfusion and ex situ heart perfusion: A case report T2 - Pediatr Transplant TI - Paediatric donation after circulatory determined death heart transplantation using donor normothermic regional perfusion and ex situ heart perfusion: A case report VL - 23 ID - 514 ER - TY - JOUR AB - Pulmonary haemorrhage is an uncommon symptom in paediatrics with the etiology varying among the series by age, location, and the diagnostic tests employed. Once airway protection and volume resuscitation are ensured, localization of the anatomic site of bleeding, isolation of the involved airway, control of haemorrhage and treatment of the underlying cause of becomes essential. In localized persistent bleeding, airway control may be achieved by lung isolation with double lumen endotracheal tube and synchronous independent lung ventilation. AD - Department of Paediatric Critical Care, Narayana Health Bommasandra, Bengaluru, Karnataka, India AU - Murkute, A. AU - Angadi, U. AU - Jain, P. AU - Sharique, T. AU - Hegde, R. DB - Scopus DO - 10.4103/0972-5229.142180 IS - 10 KW - Double lumen endotracheal tube Independent lung ventilation Pulmonary haemorrhage M3 - Article N1 - Export Date: 15 June 2020 PY - 2014 SP - 694-696 ST - Paediatric pulmonary haemorrhage: Independent lung ventilation as effective strategy in management T2 - Indian Journal of Critical Care Medicine TI - Paediatric pulmonary haemorrhage: Independent lung ventilation as effective strategy in management UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84908689838&doi=10.4103%2f0972-5229.142180&partnerID=40&md5=10503022005b297ee888524d6e107b2b VL - 18 ID - 2001 ER - TY - JOUR AB - Background: Postnatal depression (PND) is one of the most serious complications following delivery in developed countries today. Thus, early screening strategies by first-line healthcare workers are of primary importance. Pain following childbirth has been proposed as a possible risk-marker for later depressive disorder. We tested this assumption and explored the possible link between pain and overestimation of PND risk in routine clinical screenings. Methods: We assessed 320 women between the third and fifth day after delivery as well as at 8 weeks post-partum (PP). Midwives were asked to evaluate the risk of later PND upon discharge from the maternity unit; additionally, pain measurements were obtained using the Visual Analogic Scale (VAS) over the same time period. A stepwise logistic regression analysis was performed to identify the risk markers linked to a positive depressive disorder diagnosis (according to the MINIDSM- IV) at 8 weeks PP. Results and discussion: Multivariate risk analysis showed no statistical link between physical pain shortly after childbirth and subsequent PND diagnosis at 8 weeks PP. However, VAS measurements for pain were significantly higher for women that the midwives estimated to be at risk for PND (|Z| = 2.78, p = 0.005), suggesting the routine clinical screening for PND is susceptible for false-positives. Psychiatrists should encourage midwives to have an empathetic approach, to increase the detection as well as treatment of mental and physical suffering in early postpartum. At the same time, adequate education programmes for early PND screening should be proposed to non-psychiatric staffs to demonstrate that women at risk of PND often show minimal physical symptoms. (PsycINFO Database Record (c) 2016 APA, all rights reserved) AD - Jardri, Renaud, Department of Pediatric Psychiatry, Lille University Hospital Centre, Fontan Hospital, F-59037, Lille, France AN - 2010-23864-006 AU - Jardri, Renaud AU - Maron, Michel AU - Delion, Pierre AU - Thomas, Pierre DB - psyh DO - 10.3109/0167482X.2010.521271 DP - EBSCOhost IS - 4 KW - pain confounding factors postnatal depression screening risk assessment health promotion Adult Confounding Factors (Epidemiology) Depression, Postpartum Diagnostic and Statistical Manual of Mental Disorders Early Diagnosis Female Follow-Up Studies France Humans Medical Staff Multivariate Analysis Observer Variation Pain Measurement Pain Perception Parturition Postpartum Period Risk Factors Surveys and Questionnaires Young Adult Screening Postpartum Depression N1 - Perinatal Psychiatry Unit, Departments of Adult and Pediatric Psychiatry, Lille University Medical Centre, Fontan Hospital, Lille, France. Other Publishers: Bohn, Scheltema and Holkema; Elsevier Science; Parthenon Publishing; Taylor & Francis. Release Date: 20101213. Correction Date: 20150928. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Health Promotion; Screening; Risk Assessment. Minor Descriptor: Pain; Postpartum Depression. Classification: Promotion & Maintenance of Health & Wellness (3365). Population: Human (10); Female (40). Location: France. Age Group: Adulthood (18 yrs & older) (300). Tests & Measures: Mini International Neuropsychiatric Interview—French Version; Visual Analogue Scale. Methodology: Empirical Study; Quantitative Study. References Available: Y. Page Count: 4. Issue Publication Date: Nov, 2010. Publication History: Accepted Date: Sep 1, 2010; First Submitted Date: Jun 24, 2010. Copyright Statement: Informa UK, Ltd. 2010. PY - 2010 SN - 0167-482X 1743-8942 SP - 252-255 ST - Pain as a confounding factor in postnatal depression screening T2 - Journal of Psychosomatic Obstetrics & Gynecology TI - Pain as a confounding factor in postnatal depression screening UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2010-23864-006&site=ehost-live&scope=site ORCID: 0000-0003-4596-1502 renaud.jardri@chru-lille.fr VL - 31 ID - 1692 ER - TY - JOUR AB - The hybrid pathway for the management of patients with hypoplastic left heart syndrome was pioneered at our institution and is the preferred approach compared with the traditional Norwood pathway. Patients undergoing this surgery are generally \6 months of age, and pain management in this age group after surgery for complex congenital heart disease (CHD) may be particularly challenging. We retrospectively reviewed our painmanagement strategy after stage 1 hybrid procedure (HS1) and evaluated its efficacy, especially in the setting of early tracheal extubation. We retrospectively reviewed the records of patients receiving fentanyl analgesia after HS1 palliation for single-ventricle anatomy between June 2008 and August 2011. In addition to demographic data, we also recorded the mode of analgesia, total fentanyl administered during the first 48 postoperative hours, and total hours of fentanyl use. Other data collected included pain scores, adverse effects, time of tracheal extubation, and use of adjunctive medications, such as dexmedetomidine. Nursecontrolled analgesia (NCA) with fentanyl was used in 21 of the 33 patients in the study cohort, with the remainder receiving a continuous fentanyl infusion. NCA-fentanyl was the method of choice in 12 of the 13 patients whose tracheas were extubated in the operating room versus 9 of 20 patients who received postoperative mechanical ventilation and tracheal intubation (p = 0.0093). During the first and second 24 h after surgery, fentanyl requirements were lower in patients whose tracheas were extubated (11.8 ± 7.6 vs. 20.6 ± 18.1 and 6.6 ± 10.3 vs. 24.3 ± 20.4 lg/kg, respectively). Adverse effects were noted in 3 of the 33 patients (9%) and included one episode each of respiratory depression requiring reintubation of the trachea, pruritus, and excessive sedation. Dexmedetomidine was used as an adjunct medication in 5 patients and resulted in decreased fentanyl use (6.3 ± 1.3 vs. 19 ± 15.9 lg/kg in the first 24 postoperative hours and 7.9 ± 3.5 vs. 19 ± 20.3 lg/kg in the second 24 postoperative hours). Fentanyl administered by way of continuous infusion or NCA provided effective postoperative analgesia with a limited adverse effect profile after HS1 surgery in neonates with complex CHD. Fentanyl requirements were lower in patients who achieved early tracheal extubation as well as those who received dexmedetomidine. © Springer Science+Business Media, LLC 2012. AD - A.N. Naguib, Department of Anesthesiology, Nationwide Children's Hospital, Ohio State University, Columbus, OH, United States AU - Naguib, A. N. AU - Dewhirst, E. AU - Winch, P. D. AU - Simsic, J. AU - Galantowicz, M. AU - Tobias, J. D. DB - Embase Medline DO - 10.1007/s00246-012-0233-8 IS - 7 KW - dexmedetomidine fentanyl analgesia article artificial ventilation clinical article clinical effectiveness cohort analysis continuous infusion demography endotracheal intubation extubation female heart single ventricle heart surgery human hypoplastic left heart syndrome infant male newborn nurse controlled analgesia postoperative analgesia postoperative pain postoperative period pruritus respiration depression retrospective study sedation side effect stage 1 hybrid procedure therapy effect LA - English M3 - Article N1 - L51870340 2012-02-27 2012-11-09 PY - 2012 SN - 0172-0643 1432-1971 SP - 1104-1108 ST - Pain management after surgery for single-ventricle palliation using the hybrid approach T2 - Pediatric Cardiology TI - Pain management after surgery for single-ventricle palliation using the hybrid approach UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51870340 http://dx.doi.org/10.1007/s00246-012-0233-8 VL - 33 ID - 1039 ER - TY - JOUR AB - From March 1982 to December 1983, five patients with a mean age 7 years (4 months-16 years) underwent a palliative Mustard operation for complex cardiac anomalies. The diagnoses were: univentricular heart with pulmonary vascular obstructive disease (PVOD) (2 cases); criss-cross heart with intact atrial septum, ventricular septal defect (VSD), transposition of the great arteries (TGA) and moderate pulmonary stenosis; isolated atrioventricular discordance with VSD; TGA, diminutive right ventricle and multiple VSDs. Cardiac catheterization showed unfavourable direction of flow: the oxygen saturation in the aorta was lower than in pulmonary artery, with a mean difference of 13.5% (range 6% to 30%). In no case was anatomical or physiological repair considered advisable because of the unfavourable intracardiac anatomy or the presence of PVOD. In two patients the atrial rerouting was accompanied by pulmonary artery banding. There were no hospital or late deaths in a mean follow-up of 36 months (range 29 to 50 months). The mean post-operative arterial oxygen saturation was 95.4% (range 92% to 99%). AD - Department of Pediatric Cardiology, Ospedale Bambino Gesù, Rome, Italy. AN - 2483339 AU - Corno, A. F. AU - Parisi, F. AU - Marino, B. AU - Ballerini, L. AU - Marcelletti, C. DB - PubMed DO - 10.1016/1010-7940(87)90030-3 DP - NLM ET - 1987/01/01 IS - 3 KW - Adolescent Evaluation Studies as Topic Female Follow-Up Studies Heart Defects, Congenital/mortality/*surgery Humans Infant Male Methods Outcome and Process Assessment, Health Care *Palliative Care Quality of Life LA - eng N1 - Corno, A F Parisi, F Marino, B Ballerini, L Marcelletti, C Journal Article Germany Eur J Cardiothorac Surg. 1987;1(3):144-7. doi: 10.1016/1010-7940(87)90030-3. PY - 1987 SN - 1010-7940 (Print) 1010-7940 SP - 144-7 ST - Palliative Mustard operation: an expanded horizon T2 - Eur J Cardiothorac Surg TI - Palliative Mustard operation: an expanded horizon VL - 1 ID - 453 ER - TY - JOUR AB - OBJECTIVE: We sought to compare perceptions of functional health status between children who had undergone a Fontan procedure and their parents. METHODS: Fontan procedure survivors 10 to 18 years of age were included in the study if the child completed the Child Health Questionnaire (CHQ) and the parent completed the parent form to assess the child's functional health status. Comparisons were made between raw domain scores for the parent- and child-completed CHQs. RESULTS: Between March 2003 and April 2004, 1078 Fontan survivors were screened. Of the 546 eligible and consented patients, 354 were 10-18 years of age and 328 parent/child pairs completed the CHQs. Parents reported significantly lower scores (worse functioning) for their children than the children reported for themselves in the domains of physical functioning (P < .01), impact on school or activities from emotional and behavioral problems (P < .01), impact on school or activities from physical health issues (P < .01), general behavior (P < .01), mental health (P < .01), self-esteem (P < .01), and general health perceptions (P < .01). No significant differences were noted for the domains of bodily pain, family cohesiveness, or family activities. For the physical functioning domain, factors contributing to lower scores for parent versus child reports included pulmonary artery anomalies and fenestration at the time of the Fontan operation. Lower parent-reported scores also were associated with more noncardiac health problems in the child. CONCLUSIONS: Parents' perceptions of the functional health status of their children after the Fontan procedure were worse than the children's perceptions. AD - Department of Cardiothoracic, Primary Children's Medical Center and University of Utah, Salt Lake City, Utah 84113, USA. linda.lambert@imail.org AN - 19841109 AU - Lambert, L. M. AU - Minich, L. L. AU - Newburger, J. W. AU - Lu, M. AU - Pemberton, V. L. AU - McGrath, E. A. AU - Atz, A. M. AU - Xu, M. AU - Radojewski, E. AU - Servedio, D. AU - McCrindle, B. W. C2 - PMC2891533 C6 - NIHMS207116 DA - Nov DB - PubMed DO - 10.1542/peds.2008-1697 DP - NLM ET - 2009/10/21 IS - 5 KW - *Activities of Daily Living Adolescent *Attitude to Health Child Female *Fontan Procedure/psychology *Health Status Heart Defects, Congenital/surgery Humans Male Parents/*psychology Quality of Life Surveys and Questionnaires LA - eng N1 - 1098-4275 Lambert, Linda M Minich, L LuAnn Newburger, Jane W Lu, Minmin Pemberton, Victoria L McGrath, Ellen A Atz, Andrew M Xu, Mingfen Radojewski, Elizabeth Servedio, Darlene McCrindle, Brian W Pediatric Heart Network Investigators HL068288/HL/NHLBI NIH HHS/United States HL068285/HL/NHLBI NIH HHS/United States U01 HL068269/HL/NHLBI NIH HHS/United States U10 HL109816/HL/NHLBI NIH HHS/United States U01 HL068279/HL/NHLBI NIH HHS/United States U01 HL068288-09/HL/NHLBI NIH HHS/United States U01 HL068290/HL/NHLBI NIH HHS/United States HL068279/HL/NHLBI NIH HHS/United States U01 HL068288/HL/NHLBI NIH HHS/United States U10 HL068270/HL/NHLBI NIH HHS/United States U01 HL068281/HL/NHLBI NIH HHS/United States U01 HL068270/HL/NHLBI NIH HHS/United States HL068281/HL/NHLBI NIH HHS/United States U01 HL068292/HL/NHLBI NIH HHS/United States HL068269/HL/NHLBI NIH HHS/United States HL068270/HL/NHLBI NIH HHS/United States U10 HL109778/HL/NHLBI NIH HHS/United States HL068290/HL/NHLBI NIH HHS/United States U01 HL068285/HL/NHLBI NIH HHS/United States HL068292/HL/NHLBI NIH HHS/United States Comparative Study Journal Article Multicenter Study Research Support, N.I.H., Extramural Pediatrics. 2009 Nov;124(5):e942-9. doi: 10.1542/peds.2008-1697. Epub 2009 Oct 19. PY - 2009 SN - 0031-4005 (Print) 0031-4005 SP - e942-9 ST - Parent- versus child-reported functional health status after the Fontan procedure T2 - Pediatrics TI - Parent- versus child-reported functional health status after the Fontan procedure VL - 124 ID - 180 ER - TY - JOUR AB - Parents of children diagnosed with a complex congenital heart anomaly such as hypoplastic left heart syndrome (HLHS) are faced with the challenge of choosing the best treatment option for their children. Parents must decide among options of compassionate care, palliative reconstructive surgery, or neonatal cardiac transplantation, and additionally with antenatal diagnosis must decide regarding continuation of pregnancy. With improved survival rates yet persistent morbidities following the Norwood surgical approach for HLHS, controversy remains whether parents should be counseled on all treatment options as evidenced by research that articulates the varied opinions of health care providers. The parents' perspective is less well researched. In this study, interview data from parents who chose the Norwood-Sano surgical option were analyzed to provide insight into the various treatment options they were given following diagnosis of HLHS in their children and how they came to choose the surgical palliation option. AD - Alberta Health Services, Northern Alberta Neonatal Intensive Care Program, Edmonton, Alberta, Canada. AN - 21102175 AU - Ellinger, M. K. AU - Rempel, G. R. DA - Dec DB - PubMed DO - 10.1097/ANC.0b013e3181fc7c5d DP - NLM ET - 2010/11/26 IS - 6 KW - Adult Aortic Valve/surgery *Decision Making Female Heart Valve Prosthesis Implantation/*psychology Humans Hypoplastic Left Heart Syndrome/*psychology/*surgery Infant, Newborn Male Parent-Child Relations Parents/*psychology Pericardium/transplantation Pulmonary Artery/surgery Surveys and Questionnaires Young Adult LA - eng N1 - 1536-0911 Ellinger, Marcia K Rempel, Gwen R Journal Article Research Support, Non-U.S. Gov't United States Adv Neonatal Care. 2010 Dec;10(6):316-22; quiz 323-4. doi: 10.1097/ANC.0b013e3181fc7c5d. PY - 2010 SN - 1536-0903 SP - 316-22; quiz 323-4 ST - Parental decision making regarding treatment of hypoplastic left heart syndrome T2 - Adv Neonatal Care TI - Parental decision making regarding treatment of hypoplastic left heart syndrome VL - 10 ID - 315 ER - TY - JOUR AB - To evaluate the moderating influence of parental variables on changes in health-related quality of life (HRQoL) in adolescents with Tetralogy of Fallot (ToF) or a Fontan circulation after participation in standardized exercise training. A multicenter randomized controlled trail in which 56 patients, aged 10-15, were randomly allocated (stratified by age, gender, and congenital heart disease) to a 12-week period with either: (a) 3 times per week standardized exercise training or (b) care-as-usual (randomization ratio 2:1). Adolescents and their parents filled in online questionnaires at baseline and at 12-week follow-up. In this randomized controlled trail, primary analyses involved influence of parental mental health and parental social support for exercise on changes in the TNO/AZL Child Quality of Life Questionnaire Child Form at follow-up. Secondary analyses concerned comparing levels of parental characteristics with normative data. Compared with controls, adolescents in the exercise group reported a decrease in social functioning when their parents had more anxiety/insomnia or severe depression themselves. Adolescents also reported a decrease in social functioning when their parents showed poorer overall mental health themselves. Parents reported comparable or even better mental health compared with normative data. The effect of a standardized exercise program on HRQoL changes in adolescents with ToF or a Fontan circulation is moderated by parental mental health, more specifically by parental anxiety/insomnia and severe depression. The trial registration number of this article is NTR2731 ( www.trialregister.nl ). AD - Department of Child and Adolescent Psychiatry/Psychology, Erasmus Medical Center - Sophia Children's Hospital, P.O. Box 2060, 3000 CB, Rotterdam, The Netherlands, k.dulfer@erasmusmc.nl. AN - 25077662 AU - Dulfer, K. AU - Duppen, N. AU - Van Dijk, A. P. AU - Kuipers, I. M. AU - Van Domburg, R. T. AU - Verhulst, F. C. AU - Van der Ende, J. AU - Helbing, W. A. AU - Utens, E. M. DA - Jan DB - PubMed DO - 10.1007/s00246-014-0961-z DP - NLM ET - 2014/08/01 IS - 1 KW - Adolescent Case-Control Studies Child *Exercise Therapy Female Heart Defects, Congenital/*rehabilitation/surgery Humans Male *Mental Health *Parent-Child Relations Parents/*psychology *Quality of Life Social Support Surveys and Questionnaires Tetralogy of Fallot/rehabilitation/surgery Treatment Outcome LA - eng N1 - 1432-1971 Dulfer, Karolijn Duppen, Nienke Van Dijk, Arie P J Kuipers, Irene M Van Domburg, Ron T Verhulst, Frank C Van der Ende, Jan Helbing, Willem A Utens, Elisabeth M W J Journal Article Multicenter Study Randomized Controlled Trial Research Support, Non-U.S. Gov't United States Pediatr Cardiol. 2015 Jan;36(1):33-40. doi: 10.1007/s00246-014-0961-z. Epub 2014 Jul 31. PY - 2015 SN - 0172-0643 SP - 33-40 ST - Parental mental health moderates the efficacy of exercise training on health-related quality of life in adolescents with congenital heart disease T2 - Pediatr Cardiol TI - Parental mental health moderates the efficacy of exercise training on health-related quality of life in adolescents with congenital heart disease VL - 36 ID - 146 ER - TY - JOUR AB - To evaluate the moderating influence of parental variables on changes in health-related quality of life (HRQoL) in adolescents with Tetralogy of Fallot (ToF) or a Fontan circulation after participation in standardized exercise training. A multicenter randomized controlled trail in which 56 patients, aged 10-15, were randomly allocated (stratified by age, gender, and congenital heart disease) to a 12-week period with either: (a) 3 times per week standardized exercise training or (b) care-as-usual (randomization ratio 2:1). Adolescents and their parents filled in online questionnaires at baseline and at 12-week follow-up. In this randomized controlled trail, primary analyses involved influence of parental mental health and parental social support for exercise on changes in the TNO/AZL Child Quality of Life Questionnaire Child Form at follow-up. Secondary analyses concerned comparing levels of parental characteristics with normative data. Compared with controls, adolescents in the exercise group reported a decrease in social functioning when their parents had more anxiety/insomnia or severe depression themselves. Adolescents also reported a decrease in social functioning when their parents showed poorer overall mental health themselves. Parents reported comparable or even better mental health compared with normative data. The effect of a standardized exercise program on HRQoL changes in adolescents with ToF or a Fontan circulation is moderated by parental mental health, more specifically by parental anxiety/insomnia and severe depression. The trial registration number of this article is NTR2731 (www.trialregister.nl). © 2014 Springer Science+Business Media New York. AD - E. M. W. J. Utens, Department of Child and Adolescent Psychiatry/Psychology, Erasmus Medical Center - Sophia Children's Hospital, P.O. Box 2060, Rotterdam, 3000 CB, Netherlands AU - Dulfer, K. AU - Duppen, N. AU - Van Dijk, A. P. J. AU - Kuipers, I. M. AU - Van Domburg, R. T. AU - Verhulst, F. C. AU - Van der Ende, J. AU - Helbing, W. A. AU - Utens, E. M. W. J. DB - Embase DO - 10.1007/s00246-014-0961-z KW - human adolescent congenital heart malformation mental health parent congenital heart disease exercise quality of life child aerobic exercise follow up questionnaire social interaction social support randomization gender patient Fallot tetralogy commercial phenomena registration secondary analysis United States LA - English M3 - Article in Press N1 - L53271257 2014-08-14 PY - 2014 SN - 0172-0643 1432-1971 ST - Parental Mental Health Moderates the Efficacy of Exercise Training on Health-Related Quality of Life in Adolescents with Congenital Heart Disease T2 - Pediatric Cardiology TI - Parental Mental Health Moderates the Efficacy of Exercise Training on Health-Related Quality of Life in Adolescents with Congenital Heart Disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L53271257 http://dx.doi.org/10.1007/s00246-014-0961-z ID - 912 ER - TY - JOUR AB - To evaluate the moderating influence of parental variables on changes in health-related quality of life (HRQoL) in adolescents with Tetralogy of Fallot (ToF) or a Fontan circulation after participation in standardized exercise training. A multicenter randomized controlled trail in which 56 patients, aged 10–15, were randomly allocated (stratified by age, gender, and congenital heart disease) to a 12-week period with either: (a) 3 times per week standardized exercise training or (b) care-as-usual (randomization ratio 2:1). Adolescents and their parents filled in online questionnaires at baseline and at 12-week follow-up. In this randomized controlled trail, primary analyses involved influence of parental mental health and parental social support for exercise on changes in the TNO/AZL Child Quality of Life Questionnaire Child Form at followup. Secondary analyses concerned comparing levels of parental characteristics with normative data. Compared with controls, adolescents in the exercise group reported a decrease in social functioning when their parents had more anxiety/insomnia or severe depression themselves. Adolescents also reported a decrease in social functioning when their parents showed poorer overall mental health themselves. Parents reported comparable or even better mental health compared with normative data. The effect of a standardized exercise program on HRQoL changes in adolescents with ToF or a Fontan circulation is moderated by parental mental health, more specifically by parental anxiety/insomnia and severe depression. The trial registration number of this article is NTR2731 (www.trialregister.nl). AD - E.M.W.J. Utens, Department of Child and Adolescent Psychiatry/Psychology, Erasmus Medical Center - Sophia Children’s Hospital, P.O. Box 2060, Rotterdam, Netherlands AU - Dulfer, K. AU - Duppen, N. AU - Van Dijk, A. P. J. AU - Kuipers, I. M. AU - Van Domburg, R. T. AU - Verhulst, F. C. AU - Van der Ende, J. AU - Helbing, W. A. AU - Utens, E. M. W. J. DB - Embase Medline DO - 10.1007/s00246-014-0961-z IS - 1 KW - NTR2731 adolescent anxiety article child congenital heart disease controlled study depression disease severity exercise Fallot tetralogy female human insomnia major clinical study male mental health multicenter study parental mental health quality of life questionnaire randomized controlled trial social status social support TNO/AZL Child Quality of Life Questionnaiore Child Form LA - English M3 - Article N1 - L603592971 2015-04-16 2015-04-24 PY - 2015 SN - 1432-1971 0172-0643 SP - 33-40 ST - Parental mental health moderates the efficacy of exercise training on health-related quality of life in adolescents with congenital heart disease T2 - Pediatric Cardiology TI - Parental mental health moderates the efficacy of exercise training on health-related quality of life in adolescents with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L603592971 http://dx.doi.org/10.1007/s00246-014-0961-z VL - 36 ID - 857 ER - TY - JOUR AB - Objectives. We examined parental preferences for locus of service delivery for their teenager's congenital heart disease (CHD) and the influence of disease severity, sociodemographic factors, and insurance on these preferences. Methods. A consecutive sample of parents of teenagers followed in a pediatric cardiology clinic completed a mailed questionnaire. Disease severity was classified as low (≤1 cardiovascular procedure), moderate (>1 cardiovascular procedure), and high (cyanosis or single ventricle physiology). Results. Eighty-six of 148 parents responded (58%): 40, low severity; 36, moderate severity; and 10, high severity of illness. Parents preferred using primary care providers (PCPs) as a point of first contact for all 11 of 11 general health concerns and 5 of 7 potential cardiovascular-related concerns: chest pain (52%), syncope (73%), seeming seriously ill (79%), sports physical examination (79%), and endocarditis prophylactic antibiotics (94%). Increasing disease severity was significantly associated with preferring cardiologists for 6 of 7 cardiovascular-related concerns. Overall, 58% of parents viewed their care as a PCP- cardiologist comanagement model versus a cardiologist-dominated model. Lower family income (odds ratio [OR]: 1.5; confidence interval [CI]: 1.0-2.2) and severity of illness (OR: 2.1; CI: 1.0-4.4.) were associated with a comanagement model of health care versus a cardiologist-dominated model. Conclusions. This study suggests that the majority of parents of teenagers with CHD prefer to use their teenager's PCP for all routine health care needs and many cardiovascular health needs. Severity of illness and family income are positively associated with greater preference for cardiologist care. AD - M.R. Miller, Agency Health Care Policy/Research, Quality Measurement/Improvement Ctr., 2101 E Jefferson St, Rockville, MD 20852, United States AU - Miller, M. R. AU - Forrest, C. B. AU - Kan, J. S. DB - Embase Medline DO - 10.1542/peds.106.2.264 IS - 2 I KW - adolescent article cardiovascular disease chronic disease congenital heart disease disease severity health insurance human major clinical study medical specialist parental behavior patient care primary health care priority journal LA - English M3 - Article N1 - L30636393 2000-09-03 PY - 2000 SN - 0031-4005 SP - 264-269 ST - Parental preferences for primary and specialty care collaboration in the management of teenagers with congenital heart disease T2 - Pediatrics TI - Parental preferences for primary and specialty care collaboration in the management of teenagers with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L30636393 http://dx.doi.org/10.1542/peds.106.2.264 VL - 106 ID - 1332 ER - TY - JOUR AB - We present a case of a fetal diagnosis of tricuspid atresia (TA). The pregnant woman and her husband requested that the baby be treated with only palliative care. The cardiologist did not think it would be appropriate to withhold life-prolonging surgery once the infant was born. The neonatologist argued that outcomes for TA are similar to those for hypoplastic left heart syndrome, and the standard practice at the institution was to allow parents to choose surgery or end-of-life care for those infants. The team requested an ethics consultation to assist in determining whether forgoing life-prolonging interventions in this case would be ethically supportable. In this article, we ask a pediatric intensivist, a pediatric cardiologist, and a neonatologist to discuss the ethics of withholding life-sustaining treatment of a baby with TA. AD - Naval Medical Center San Diego and the University of California San Diego School of Medicine, San Diego, California. Lurie Children's Hospital, Chicago, Illinois. Medical College of Wisconsin, Milwaukee, Wisconsin; and. Department of Pediatrics, Children's Mercy Kansas City, Kansas City, Missouri jlantos@cmh.edu. AN - 27940784 AU - Kon, A. A. AU - Patel, A. AU - Leuthner, S. AU - Lantos, J. D. DA - Nov DB - PubMed DO - 10.1542/peds.2016-1730 DP - NLM ET - 2016/12/13 IS - 5 KW - Cardiac Surgical Procedures/adverse effects/methods Decision Making Female Gestational Age Humans Male Parents/*psychology Pregnancy *Pregnancy Outcome Treatment Refusal/*ethics Tricuspid Atresia/*diagnostic imaging/surgery Ultrasonography, Prenatal/*methods Withholding Treatment/*ethics Young Adult LA - eng N1 - 1098-4275 Kon, Alexander A Patel, Angira Leuthner, Steven Lantos, John D Case Reports Journal Article Review United States Pediatrics. 2016 Nov;138(5):e20161730. doi: 10.1542/peds.2016-1730. Epub 2016 Oct 7. PY - 2016 SN - 0031-4005 ST - Parental Refusal of Surgery in an Infant With Tricuspid Atresia T2 - Pediatrics TI - Parental Refusal of Surgery in an Infant With Tricuspid Atresia VL - 138 ID - 308 ER - TY - JOUR AB - Hypoplastic left heart syndrome (HLHS) is a complex heart malformation that requires life-saving treatments. Parents experience numerous challenges as they learn to parent a child with complex care requirements. The following research question guided this qualitative study: Is the parenting process among parents of a child with HLHS characterized by exaggerated vigilant parental action, and if so, how does this influence parental response? Situated within a larger program of pediatric cardiology research, this study included data from two grounded theory studies with parents of children with HLHS. This secondary analysis involved a thematic content analysis using sensitizing concepts of uncertainty, protectiveness, support, and mastery of complex care. Transcribed data from 55 interviews with 24 mothers and 17 fathers of young children with HLHS were analyzed for relevant and recurring themes. In mastering skills required to care for their child with HLHS, parents contrasted what was in their hands with what was out of their hands. Vigilant parental actions were evident as parents became skilled at providing complex care. Parents said they were sometimes excessive in their vigilant actions. In retrospect they viewed this vigilance as appropriate in some situations but exaggerated in other situations. Understanding parents' vigilant actions in response to their child's complex care can guide health care providers' interactions with families. Long-term follow up, both clinically and through research, is needed to assess the long-term consequences of exaggerated vigilant parental action on the child, parent, and family, and to determine and evaluate appropriate and timely intervention. AN - 26281274 AU - Meakins, L. AU - Ray, L. AU - Hegadoren, K. AU - Rogers, L. G. AU - Rempel, G. R. DA - Jan-Feb DB - PubMed DP - NLM ET - 2015/08/19 IS - 1 KW - Adaptation, Psychological Adult *Attention Caregivers/*psychology Female Humans Hypoplastic Left Heart Syndrome/*nursing/psychology Interviews as Topic Male Middle Aged Parents/*psychology Qualitative Research Social Support Uncertainty LA - eng N1 - Meakins, Leanne Ray, Lynne Hegadoren, Kathleen Rogers, Laura G Rempel, Gwen R Journal Article Research Support, Non-U.S. Gov't United States Pediatr Nurs. 2015 Jan-Feb;41(1):31-41, 50. PY - 2015 SN - 0097-9805 (Print) 0097-9805 SP - 31-41, 50 ST - Parental Vigilance in Caring for Their Children with Hypoplastic Left Heart Syndrome T2 - Pediatr Nurs TI - Parental Vigilance in Caring for Their Children with Hypoplastic Left Heart Syndrome VL - 41 ID - 137 ER - TY - JOUR AB - OBJECTIVE: To evaluate the experiences of parenting a child with hypoplastic left heart syndrome after the child has been discharged home from hospital. DESIGN: A study of the parents' experiences using face-to-face interviews and psychometric measures with parents whose child had survived stage surgery. SETTING: Parents were interviewed within the home environment or within the hospital if that was their choice. SUBJECTS: A total of 29 parents (16 mothers and 13 fathers) of surviving children. Intervention A semi-structured face-to-face interview plus psychometric tests (parent demographics, Maslach Burnout Inventory, Impact on Family Scale, and the Psychological Check List - Civilian). Measurements and main results The parents' experience in supporting a child with hypoplastic left heart syndrome is one of stress, of commitment, and of love. Although parents experienced joy in their child, they were also subjected to anxiety with four parents test positive to post-traumatic stress disorder and hypervigilance while monitoring their child's condition. Parents lived with many difficulties, and demands. AD - Private Practice,Melbourne,Victoria,Australia. AN - 28583211 AU - Cantwell-Bartl, A. M. AU - Tibballs, J. DA - Sep DB - PubMed DO - 10.1017/s1047951117000270 DP - NLM ET - 2017/06/07 IS - 7 KW - Adaptation, Psychological Adolescent Anxiety/*diagnosis Child Child, Preschool Female Humans Hypoplastic Left Heart Syndrome/*surgery Income Infant Interviews as Topic Love Male Parenting/*psychology Parents/*psychology Psychiatric Status Rating Scales Retrospective Studies Stress Disorders, Post-Traumatic/*diagnosis Young Adult Hypoplastic left heart syndrome adaptation grief parents stress LA - eng N1 - 1467-1107 Cantwell-Bartl, Annie M Tibballs, James Orcid: 0000-0002-4433-004x Journal Article England Cardiol Young. 2017 Sep;27(7):1341-1348. doi: 10.1017/S1047951117000270. Epub 2017 Jun 6. PY - 2017 SN - 1047-9511 SP - 1341-1348 ST - Parenting a child at home with hypoplastic left heart syndrome: experiences of commitment, of stress, and of love T2 - Cardiol Young TI - Parenting a child at home with hypoplastic left heart syndrome: experiences of commitment, of stress, and of love VL - 27 ID - 106 ER - TY - JOUR AB - PURPOSE. This study described the role normalization played for parents related to their young children with hypoplastic left heart syndrome. DESIGN AND METHODS. The data source for this secondary analysis was 30 interviews with 16 parents. RESULTS. Three themes were identified: normalization, parental perception of child vulnerability, and optimistic appraisal. Parents engaged in a balancing act between worrying about their children's vulnerability and marveling at their children's survival, using normalization as a coping strategy. PRACTICE IMPLICATIONS. Nurses can collaborate with parents to determine strategies to restore a sense of normalcy to simultaneously promote child independence and parent well-being. AD - Alberta Children's Hospital, Calgary, Canada. AN - 21702878 AU - Lee, A. AU - Rempel, G. R. DA - Jul DB - PubMed DO - 10.1111/j.1744-6155.2011.00289.x DP - NLM ET - 2011/06/28 IS - 3 KW - Adaptation, Psychological Adult Age Factors Alberta Cardiac Surgical Procedures/methods Caregivers/*psychology Child, Preschool Cohort Studies Continuity of Patient Care Disabled Children/psychology Female Follow-Up Studies Humans Hypoplastic Left Heart Syndrome/diagnosis/surgery/*therapy Infant Long-Term Care Male Middle Aged *Parent-Child Relations *Parenting Risk Factors Treatment Outcome Vulnerable Populations Young Adult LA - eng N1 - 1744-6155 Lee, Anra Rempel, Gwen R Canadian Institutes of Health Research/Canada Journal Article Research Support, Non-U.S. Gov't United States J Spec Pediatr Nurs. 2011 Jul;16(3):179-89. doi: 10.1111/j.1744-6155.2011.00289.x. Epub 2011 May 31. PY - 2011 SN - 1539-0136 SP - 179-89 ST - Parenting children with hypoplastic left heart syndrome: finding a balance T2 - J Spec Pediatr Nurs TI - Parenting children with hypoplastic left heart syndrome: finding a balance VL - 16 ID - 157 ER - TY - JOUR AB - OBJECTIVE: Parents of infants with congenital heart disease (CHD) experience increased parenting stress levels, potentially interfering with parenting practices and bear adverse family outcomes. Condition severity has been linked to parenting stress. The current study aimed to explore parenting stress trajectories over infancy in parents of infants with complex CHD, and to compare them by post-operative cardiac physiology. DESIGN: Data from a larger prospective cohort study was analyzed using longitudinal mixed-effects regression modeling. SETTING: Cardiac intensive care unit and outpatient clinic of a 480-bed children's hospital in the American North-Atlantic region. PARTICIPANTS: Parents of infants with complex CHD (n = 90). MEASURES: Parenting stress was measured via the parenting stress index-long form over four time points during infancy. RESULTS: Parents of infants with a single-ventricle heart experienced a decrease in total stress over time. Parents of infants with a biventricular heart experienced a decrease in attachment-related stress, and an increase in stress related to infant temperament over time. Parenting stress trajectories over time significantly differed between groups on infant temperamental subscales. CONCLUSIONS: Findings highlight stressful and potentially risky periods for parents of infants with complex CHD, and introduce additional illness-related and psychosocial/familial aspects to the parenting stress concept. Early intervention may promote parental adaptive coping and productive parenting practices in this population. AD - Department of Nursing, University of Haifa, Haifa, Israel. Center for Biostatistics and Health Data Science, Virginia Tech, Blacksburg, VA, USA. School of Nursing, University of Pennsylvania, Philadelphia, PA, USA. The Cardiac Center, The Children's Hospital of Philadelphia, Philadelphia, PA, USA. AN - 31657114 AU - Golfenshtein, N. AU - Hanlon, A. L. AU - Deatrick, J. A. AU - Medoff-Cooper, B. C2 - PMC6952575 C6 - NIHMS1055168 DA - Nov DB - PubMed DO - 10.1111/chd.12858 DP - NLM ET - 2019/10/28 IS - 6 KW - congenital heart disease infancy longitudinal design parenting stress uni-ventricular heart LA - eng N1 - 1747-0803 Golfenshtein, Nadya Orcid: 0000-0003-1875-5981 Hanlon, Alexandra L Deatrick, Janet A Medoff-Cooper, Barbara R01 NR002093/NR/NINR NIH HHS/United States Journal Article Congenit Heart Dis. 2019 Nov;14(6):1113-1122. doi: 10.1111/chd.12858. Epub 2019 Oct 27. PY - 2019 SN - 1747-079X (Print) 1747-079x SP - 1113-1122 ST - Parenting stress trajectories during infancy in infants with congenital heart disease: Comparison of single-ventricle and biventricular heart physiology T2 - Congenit Heart Dis TI - Parenting stress trajectories during infancy in infants with congenital heart disease: Comparison of single-ventricle and biventricular heart physiology VL - 14 ID - 89 ER - TY - JOUR AB - AIM: To report a grounded theory study to describe the process of parenting young children who have survived hypoplastic left heart syndrome to inform parent-focused interventions. BACKGROUND: Technological advances in paediatric cardiology worldwide have improved the survival rates for young children with hypoplastic left heart syndrome who undergo staged surgical palliation. These children, however, are at risk for life-threatening complications and parents are charged with the responsibility to monitor their children at home with minimal support and guidance from healthcare professionals once home. DESIGN: A constructivist grounded theory study. METHOD: The study was conducted in 2006-2008. Participants were 25 parents (15 mothers, 10 fathers) and 28 grandparents (17 grandmothers, 11 grandfathers) of 15 young children (6 months-4·5 years) who had undergone the Sano surgical approach for hypoplastic left heart syndrome. The 53 interviews were digitally recorded, transcribed and analysed using open and focused coding, constant comparative analysis and memoing. FINDINGS: A process of Parenting under Pressure emerged that was characterized by four overlapping and re-emerging phases: (1) realizing and adjusting to the inconceivable; (2) growing increasingly attached; (3) watching for and accommodating the unexpected; and (4) encountering new challenges. CONCLUSIONS: In-depth understanding of the phases of Parenting under Pressure provides direction for nurses to support parents of children who survive hypoplastic left heart syndrome. Interventions that help carers of children with complex health conditions move through the phases of our Parenting under Pressure process may help them safeguard the survival of their children, and their own survival as parents as they manage multiple demands. AD - Faculty of Nursing, University of Alberta, Edmonton, Alberta, Canada. gwen.rempel@ualberta.ca AN - 22616855 AU - Rempel, G. R. AU - Ravindran, V. AU - Rogers, L. G. AU - Magill-Evans, J. DA - Mar DB - PubMed DO - 10.1111/j.1365-2648.2012.06044.x DP - NLM ET - 2012/05/24 IS - 3 KW - Adaptation, Psychological Adult Aged Caregivers/*psychology Child, Preschool Family/psychology Female Humans Hypoplastic Left Heart Syndrome/*psychology/surgery Infant Male Middle Aged Parenting/*psychology Parents/*psychology Qualitative Research Stress, Psychological LA - eng N1 - 1365-2648 Rempel, Gwen R Ravindran, Vinitha Rogers, Laura G Magill-Evans, Joyce Journal Article Research Support, Non-U.S. Gov't England J Adv Nurs. 2013 Mar;69(3):619-30. doi: 10.1111/j.1365-2648.2012.06044.x. Epub 2012 May 22. PY - 2013 SN - 0309-2402 SP - 619-30 ST - Parenting under pressure: a grounded theory of parenting young children with life-threatening congenital heart disease T2 - J Adv Nurs TI - Parenting under pressure: a grounded theory of parenting young children with life-threatening congenital heart disease VL - 69 ID - 184 ER - TY - JOUR AB - Aim. To report a grounded theory study to describe the process of parenting young children who have survived hypoplastic left heart syndrome to inform parent-focused interventions. Background. Technological advances in paediatric cardiology worldwide have improved the survival rates for young children with hypoplastic left heart syndrome who undergo staged surgical palliation. These children, however, are at risk for life-threatening complications and parents are charged with the responsibility to monitor their children at home with minimal support and guidance from healthcare professionals once home. Design. A constructivist grounded theory study. Method. The study was conducted in 2006-2008. Participants were 25 parents (15 mothers, 10 fathers) and 28 grandparents (17 grandmothers, 11 grandfathers) of 15 young children (6 months-4·5 years) who had undergone the Sano surgical approach for hypoplastic left heart syndrome. The 53 interviews were digitally recorded, transcribed and analysed using open and focused coding, constant comparative analysis and memoing. Findings. A process of Parenting under Pressure emerged that was characterized by four overlapping and re-emerging phases: (1) realizing and adjusting to the inconceivable; (2) growing increasingly attached; (3) watching for and accommodating the unexpected; and (4) encountering new challenges. Conclusions. In-depth understanding of the phases of Parenting under Pressure provides direction for nurses to support parents of children who survive hypoplastic left heart syndrome. Interventions that help carers of children with complex health conditions move through the phases of our Parenting under Pressure process may help them safeguard the survival of their children, and their own survival as parents as they manage multiple demands. AD - Gwen R. Rempel PhD RN 
Assistant Professor 
Faculty of Nursing, University of Alberta, Edmonton, Alberta, Canada 
and Department of Pediatrics, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada Vinitha Ravindran PhD RN 
College of Nursing, Christian Medical College, Vellore, Tamil Nadu, India Laura G. Rogers MScRS OT(C) 
Research Associate 
Faculty of Nursing, University of Alberta, Edmonton, Alberta, Canada Joyce Magill‐Evans PhD OT(C) 
Professor 
Faculty of Rehabilitation Medicine, University of Alberta, Edmonton, Alberta, Canada AN - 85479833. Language: English. Entry Date: 20130215. Revision Date: 20190412. Publication Type: Article AU - Rempel, Gwen R. AU - Ravindran, Vinitha AU - Rogers, Laura G. AU - Magill‐Evans, Joyce DB - ccm DO - 10.1111/j.1365-2648.2012.06044.x DP - EBSCOhost IS - 3 KW - Child, Medically Fragile Parents -- Psychosocial Factors Hypoplastic Left Heart Syndrome Home Nursing Parenting Caregiver Burden Grounded Theory Human Grandparents Constant Comparative Method Hypoplastic Left Heart Syndrome -- Surgery Interviews Data Analysis Software Theoretical Sample Thematic Analysis Adult Educational Status Employment Status Income Male Female Infant Child, Preschool Aged Middle Age Family Coping Parent-Infant Relations Intensive Care Units, Pediatric Uncertainty Adaptation, Psychological Parental Role Inpatients Caregiver Support Patient Education Cross Sectional Studies Convenience Sample Funding Source N1 - research; tables/charts. Special Interest: Pediatric Care. Grant Information: University of Alberta Hospital Foundation and the University of Alberta.. PY - 2013 SN - 0309-2402 SP - 619-630 ST - Parenting under Pressure: a grounded theory of parenting young children with life-threatening congenital heart disease T2 - Journal of Advanced Nursing (John Wiley & Sons, Inc.) TI - Parenting under Pressure: a grounded theory of parenting young children with life-threatening congenital heart disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=85479833&site=ehost-live&scope=site VL - 69 ID - 1532 ER - TY - JOUR AB - BACKGROUND: The time point at which parents receive a diagnosis of congenital heart disease (CHD) has changed over the years due in part to advances in fetal ultrasound. However, CHD remains undiscovered until after birth in some cases. The psychological effect of time of diagnosis on parents' experiences has not been well researched. AIM: To explore parents' experiences at the time of diagnosis of complex CHD and to compare if experiences differ when receiving an antenatal versus postnatal diagnosis. METHOD: Descriptive and thematic analysis of primary mixed qualitative and quantitative data, collected in 2012-2013, from an online survey of parents ( n =22) whose infants had undergone stage one surgery for a functionally univentricular heart. FINDINGS: Four themes emerged: parents' understanding of the condition at the time of diagnosis; parents' emotions at the time of diagnosis; sources of support at the time of diagnosis; and additional sources of information after the diagnosis. CONCLUSION: There are implications for practice in terms of who provides the diagnosis and, more importantly, how well it is explained. Professionals need to assess parents' emotional status, information needs and level of understanding irrespective of time of diagnosis, so that support is individualised, sensitive and time appropriate. AD - Children's ward, Gloucestershire Hospitals NHS Foundation Trust, Gloucestershire, England. Advanced clinical practice, Department of Nursing and Midwifery, Institute of Health and Society, University of Worcester, Worcester, England. AN - 30358337 AU - Reid, A. AU - Gaskin, K. DA - Nov 8 DB - PubMed DO - 10.7748/ncyp.2018.e1078 DP - NLM ET - 2018/10/26 IS - 6 KW - Adult Child, Preschool Female Heart Defects, Congenital/*diagnosis Humans Infant Infant, Newborn Life Change Events Male Middle Aged Parents/*psychology Pregnancy Prenatal Diagnosis/methods/*psychology Qualitative Research Surveys and Questionnaires *Time Factors Ultrasonography/methods United Kingdom child health congenital abnormalities heart diseases research was conducted between July and September 2015 LA - eng N1 - 2046-2344 Reid, Annette Gaskin, Kerry Journal Article England Nurs Child Young People. 2018 Nov 8;30(6):19-25. doi: 10.7748/ncyp.2018.e1078. Epub 2018 Oct 22. PY - 2018 SN - 2046-2336 SP - 19-25 ST - Parents' experiences of receiving an antenatal versus postnatal diagnosis of complex congenital heart disease T2 - Nurs Child Young People TI - Parents' experiences of receiving an antenatal versus postnatal diagnosis of complex congenital heart disease VL - 30 ID - 528 ER - TY - JOUR AB - PURPOSE: The aim of the study was to explore the literature related to transitions in healthcare between the hospital and home that caregivers experience with a child who has a congenital heart defect (CHD), specifically related to hypoplastic left heart syndrome (HLHS). DESIGN AND METHODS: A systematic literature review was conducted searching OVID Medline, CINAHL, and PubMed to discover the caregivers' perceptions on their transitions between hospital care and home care of their child with a CHD. Articles included those with focus on the transitions of caregivers between hospital and home care for children with CHD. Excluded articles were studies focused on adolescents, transition to adult healthcare, mortality results, other diseases associated with CHDs, comparison of CHD treatments, feasibility studies, differences in care between hospitals, home monitoring, and comparison of videoconference and telephone home communication. RESULTS: Ten articles were selected. Many parents voiced their concerns with feeding their child, learning medical skills and knowledge, reported a disrupted relationship between parents and their child, and identified stress and anxiety associated with taking care of a child with a CHD. PRACTICE IMPLICATIONS: There were limited studies on caregivers' transitions with a child with HLHS, but there also was limited focus on the caregivers' experiences with transitions between hospital and home care for their child with any CHD. Research on the transition experience between hospital care and home care for caregivers of children born with a CHD, and a specific focus on HLHS from the caregivers' viewpoint, would provide insight into the perspective of caregivers during the numerous transitions. AD - University of Virginia School of Nursing, Charlottesville, VA, USA. AN - 28627006 AU - March, S. DA - Jul DB - PubMed DO - 10.1111/jspn.12185 DP - NLM ET - 2017/06/20 IS - 3 KW - Adaptation, Psychological Adolescent Adult Caregivers/*psychology Child Child, Preschool Female *Home Care Services Humans Hypoplastic Left Heart Syndrome/*therapy Infant Infant, Newborn Male Middle Aged Parents/*psychology *Patient Discharge Qualitative Research Stress, Psychological *Transitional Care *caregiver *congenital heart *discharge *home care *hospital care *hypoplastic left heart syndrome *parents *transition LA - eng N1 - 1744-6155 March, Sarita Orcid: 0000-0003-3900-5047 Journal Article Research Support, Non-U.S. Gov't Review United States J Spec Pediatr Nurs. 2017 Jul;22(3). doi: 10.1111/jspn.12185. Epub 2017 Jun 18. PY - 2017 SN - 1539-0136 ST - Parents' perceptions during the transition to home for their child with a congenital heart defect: How can we support families of children with hypoplastic left heart syndrome? T2 - J Spec Pediatr Nurs TI - Parents' perceptions during the transition to home for their child with a congenital heart defect: How can we support families of children with hypoplastic left heart syndrome? VL - 22 ID - 123 ER - TY - JOUR AB - Aim The aim of this study was to explore parental preparedness for discharge and their experiences of going home with their infant after the first-stage surgery for a functionally univentricular heart. BACKGROUND: Technological advances worldwide have improved outcomes for infants with a functionally univentricular heart over the last 3 decades; however, concern remains regarding mortality in the period between the first and second stages of surgery. The implementation of home monitoring programmes for this group of infants has improved this initial inter-stage survival; however, little is known about parents' experiences of going home, their preparedness for discharge, and parents' recognition of deterioration in their fragile infant. METHOD: This study was conducted in 2011-2013; eight sets of parents were consulted in the research planning stage in September, 2011, and 22 parents with children aged 0-2 years responded to an online survey during November, 2012-March, 2013. Description of categorical data and deductive thematic analysis of the open-ended questions were undertaken. RESULTS: Not all parents were taught signs of deterioration or given written information specific to their baby. The following three themes emerged from the qualitative data: mixed emotions about going home, knowledge and preparedness, and support systems. CONCLUSIONS: Parents are not adequately prepared for discharge and are not well equipped to recognise deterioration in their child. There is a role for greater parental education through development of an early warning tool to address the gap in parents' understanding of signs of deterioration, enabling appropriate contact and earlier management by clinicians. AD - 1Department of Nursing,Midwifery and Paramedic Science,Institute of Health and Society,University of Worcester,Henwick Grove,Worcester,United Kingdom. 2Department of Cardiac Surgery,Birmingham Children's Hospital,Steelhouse Lane,Birmingham,United Kingdom. 3Cardiac Unit,Birmingham Children's Hospital,Steelhouse Lane,Birmingham,United Kingdom. AN - 27431411 AU - Gaskin, K. L. AU - Barron, D. J. AU - Daniels, A. DA - Oct DB - PubMed DO - 10.1017/s1047951116001062 DP - NLM ET - 2016/07/20 IS - 7 KW - Adult *Cardiac Surgical Procedures Child, Preschool Female Health Knowledge, Attitudes, Practice Home Care Services/*standards Humans Hypoplastic Left Heart Syndrome/*nursing/surgery Infant Infant, Newborn Male Middle Aged Parents/*education/*psychology *Patient Discharge United Kingdom Young Adult Chd Hypoplastic left heart syndrome family heart defects infants parents patient discharge LA - eng N1 - 1467-1107 Gaskin, Kerry L Barron, David J Daniels, Amanda Journal Article England Cardiol Young. 2016 Oct;26(7):1414-24. doi: 10.1017/S1047951116001062. Epub 2016 Jul 19. PY - 2016 SN - 1047-9511 SP - 1414-24 ST - Parents' preparedness for their infants' discharge following first-stage cardiac surgery: development of a parental early warning tool T2 - Cardiol Young TI - Parents' preparedness for their infants' discharge following first-stage cardiac surgery: development of a parental early warning tool VL - 26 ID - 351 ER - TY - JOUR AB - We report an antenatally diagnosed fetal coarctation delivered prematurely and confirmed to have coarctation as well as additional nonobstructing anomalous left pulmonary artery branch (aLPA) from right pulmonary artery (RPA) and significant dysmorphic features. The baby underwent an uncomplicated arch repair, but had numerous multisystem and growth related issues prompting a diagnosis of Kabuki syndrome (KS) at 1 year of age. While coarctation and hypoplastic left heart syndrome are observed in this syndrome, this is the second reported case of aLPA in KS and the first with the forme fruste of left-sided obstruction as well as aLPA in this group of patients. (Echocardiography 2012;29:E145-E147) This case report describes an unusual constellation of aortic coarctation diagnosed antenatally, an unusual anatomic variant of pulmonary artery anatomy wherein the left lower lung was supplied by an anomalous accessory left pulmonary artery arising from the right pulmonary artery and clinical diagnosis of Kabuki syndrome in early childhood. © 2012, Wiley Periodicals, Inc. AD - A.H. Bhat, Division of Pediatric Cardiology, Seattle Children's Hospital, University of Washington, 4800 Sand Point Way NE, Seattle, WA 98105, United States AU - Bhat, A. H. AU - Davenport, J. AU - Cocalis, M. DB - Embase Medline DO - 10.1111/j.1540-8175.2011.01651.x IS - 6 KW - prostaglandin SOS protein aortic coarctation aortic valve aortic arch surgery article brachydactyly case report cesarean section clinodactyly cryptorchism echocardiography fetus echography gestational age heart catheterization HELLP syndrome human hypoplastic left heart syndrome infant inguinal hernia joint laxity Kabuki makeup syndrome male mitral valve Noonan syndrome premature labor prenatal diagnosis priority journal pulmonary artery malformation transthoracic echocardiography LA - English M3 - Article N1 - L51863399 2012-02-21 2012-07-24 PY - 2012 SN - 0742-2822 1540-8175 SP - E145-E147 ST - Partial anomalous left pulmonary artery along with aortic coarctation in an infant with kabuki syndrome T2 - Echocardiography TI - Partial anomalous left pulmonary artery along with aortic coarctation in an infant with kabuki syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51863399 http://dx.doi.org/10.1111/j.1540-8175.2011.01651.x VL - 29 ID - 1054 ER - TY - JOUR AB - There are established associations between advanced paternal age and offspring risk for psychiatric and developmental disorders. These are commonly attributed to genetic mutations, especially de novo single nucleotide variants (dnSNVs), that accumulate with increasing paternal age. However, the actual magnitude of risk from such mutations in the male germline is unknown. Quantifying this risk would clarify the clinical significance of delayed paternity. Using parent-child trio whole-exome-sequencing data, we estimate the relationship between paternal-age-related dnSNVs and risk for five disorders: autism spectrum disorder (ASD), congenital heart disease, neurodevelopmental disorders with epilepsy, intellectual disability and schizophrenia (SCZ). Using Danish registry data, we investigate whether epidemiologic associations between each disorder and older fatherhood are consistent with the estimated role of dnSNVs. We find that paternal-age-related dnSNVs confer a small amount of risk for these disorders. For ASD and SCZ, epidemiologic associations with delayed paternity reflect factors that may not increase with age. AD - E.B. Robinson, Stanley Center for Psychiatric Research, Broad Institute of MIT and Harvard, Cambridge, MA, United States AU - Taylor, J. L. AU - Debost, J. C. P. G. AU - Morton, S. U. AU - Wigdor, E. M. AU - Heyne, H. O. AU - Lal, D. AU - Howrigan, D. P. AU - Bloemendal, A. AU - Larsen, J. T. AU - Kosmicki, J. A. AU - Weiner, D. J. AU - Homsy, J. AU - Seidman, J. G. AU - Seidman, C. E. AU - Agerbo, E. AU - McGrath, J. J. AU - Mortensen, P. B. AU - Petersen, L. AU - Daly, M. J. AU - Robinson, E. B. DB - Embase Medline DO - 10.1038/s41467-019-11039-6 IS - 1 KW - article atrioventricular septal defect autism congenital blood vessel malformation congenital heart disease de novo single nucleotide variant effect size epilepsy father gene mutation genetic association genetic risk heart atrium septum defect heart left ventricle outflow tract obstruction heart right ventricle outflow tract obstruction heart single ventricle heart ventricle septum defect human ICD-10 incidence intellectual impairment medical ethics mental disease morbidity null hypothesis paternal age phenotype progeny schizophrenia single nucleotide polymorphism tricuspid valve disease whole exome sequencing whole genome sequencing LA - English M3 - Article N1 - L628451602 2019-07-19 2019-07-29 PY - 2019 SN - 2041-1723 ST - Paternal-age-related de novo mutations and risk for five disorders T2 - Nature Communications TI - Paternal-age-related de novo mutations and risk for five disorders UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L628451602 http://dx.doi.org/10.1038/s41467-019-11039-6 VL - 10 ID - 586 ER - TY - JOUR AB - Aortic arch reconstruction plays an important role in the success of the Norwood procedure (NP) for hypoplastic left heart syndrome (HLHS). This study investigated the cardiac specimens to determine the etiology of distal aortic arch obstruction after the NP for HLHS and to locate coarctation of the aorta in HLHS untreated by surgery. This study examined 17 cardiac specimens: 9 that had NP and 8 not treated by surgery. The findings after NP showed frequent failure to resect the coarctation segment completely and failure to extend the augmentation patch into the descending aorta. Five (62.5%) of the eight hearts not treated by surgery had significant periductal coarctation of the aorta. After NP for nine patients, three (33%) had residual coarctation of the aorta. To minimize the risk of recurrent or persistent aortic arch obstruction after NP and to improve the long- and short-term outcome, the ductal tissue and the coarctation segment encircling the aortic lumen should be resected. The distal wall incision should be extended at least 5 mm beyond the distal aspect of the ductal tissue. These steps could avoid major aortic arch obstruction, promote growth of the native aortic tissue, and avoid ventricular dysfunction. © 2010 Springer Science+Business Media, LLC. AD - S. Sivanandam, Department of Pediatrics, University of Minnesota, MMC 94, 420 Delaware St, SE, Minneapolis, MN 55455, United States AU - Sivanandam, S. AU - MacKey-Bojack, S. M. AU - Moller, J. H. DB - Embase Medline DO - 10.1007/s00246-010-9840-4 IS - 2 KW - aortic arch syndrome aortic coarctation aortic stenosis aortic arch surgery article controlled study descending aorta disease severity fetus heart right ventricle hypertrophy heart ventricle septum defect histopathology human human tissue hypoplasia hypoplastic left heart syndrome infant mitral valve atresia mitral valve stenosis Norwood procedure outcome assessment pulmonary vein malformation recurrent disease risk reduction LA - English M3 - Article N1 - L51163221 2011-03-14 2011-03-24 PY - 2011 SN - 0172-0643 SP - 189-192 ST - Pathology of the aortic arch in hypoplastic left heart syndrome: Surgical implications T2 - Pediatric Cardiology TI - Pathology of the aortic arch in hypoplastic left heart syndrome: Surgical implications UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51163221 http://dx.doi.org/10.1007/s00246-010-9840-4 VL - 32 ID - 1117 ER - TY - JOUR AB - Abstract Background On recognising poor growth following neonatal palliation with a systemic-to-pulmonary shunt, we sought to determine how patient- and procedure-related factors impact growth, paying attention to the role of the primary cardiologist in this process. Methods In a retrospective review, neonates (133 patients) receiving modified systemic-to-pulmonary artery shunts from 2002 to 2009 were studied and outpatient visits were reviewed. Patients with single- and two-ventricle circulations after shunt takedown were compared using weight-for-age z-score. Results Single-ventricle patients had a higher weight-for-age z-score at neonatal surgery than two-ventricle patients (-0.4 ± 1.0 compared with -1.2 ± 0.9, with p < 0.001), but they had a greater drop in the weight-for-age z-score to the first outpatient visit (-1.1 ± 0.7 compared with -0.8 ± 0.7, with p =0.02). After the first outpatient visit, the weight-for-age z-score was not significantly different between single-ventricle and two-ventricle patients. From multivariate analysis, a lower number of nutritional interventions by cardiologists was significantly associated with poor growth (p =0.03). Poor growth was not associated with race, use of feeding tube, exclusive formula use, or proximity to surgical centre. Conclusion The significant drop in the weight-for-age z-score from neonatal surgery to first outpatient visit suggests that these patients may receive inadequate nutrition. The poorest performers received the least number of outpatient changes to their diet. This finding underscores the critical role of the primary cardiologist in optimising weight gain through adjustments in nutrition. © 2012 Cambridge University Press. AD - W.T. Mahle, Department of Cardiology, Children's Healthcare of Atlanta, Atlanta, GA, 30322-1062, United States AU - McCrary, A. W. AU - Clabby, M. L. AU - Mahle, W. T. DB - Embase Medline DO - 10.1017/S1047951112001382 IS - 4 KW - article artificial milk breast milk cardiologist child behavior child growth child nutrition clinical practice comparative study controlled study cyanotic heart disease diet therapy female human infant major clinical study male medical record review nasogastric tube newborn outpatient care palliative therapy pediatric cardiology race scoring system single ventricle cyanotic heart disease stomach tube systemic pulmonary shunt two ventricle cyanotic heart disease weight for age z score body weight gain LA - English M3 - Article N1 - L369329244 2013-07-24 2013-08-07 PY - 2013 SN - 1047-9511 1467-1107 SP - 499-506 ST - Patient and practice factors affecting growth of infants with systemic-to-pulmonary shunt T2 - Cardiology in the Young TI - Patient and practice factors affecting growth of infants with systemic-to-pulmonary shunt UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L369329244 http://dx.doi.org/10.1017/S1047951112001382 VL - 23 ID - 992 ER - TY - JOUR AB - OBJECTIVES: Data on patient-reported outcomes (PROs) in patients with single-ventricle physiology (SVP) are scarce. We sought (1) to describe the perceived health status, quality of life, symptoms of anxiety and depression, and sense of coherence in adult survivors with SVP, (2) to compare PROs across functional classes, and (3) to compare PROs between patients and controls. METHODS: A case-control study in two adult congenital heart programmes with 62 adult survivors with SVP were matched to 172 healthy controls. A wide range of PROs were measured using validated questionnaires. The treating physician classified patients according to the Ability Index. RESULTS: Patients with SVP have a good functional status. Patients in Ability Index class I consistently reported the best scores, similar to those of healthy controls. Negative associations were found between functional class and outcomes of perceived health and quality of life. For patients in Ability Index class II and III, PROs were poorer. CONCLUSIONS: PROs in patients with SVP are generally good. AD - The Heart Centre, Copenhagen University Hospital, Copenhagen, Denmark. dorthe@overgaard.mail.dk AN - 22094965 AU - Overgaard, D. AU - Schrader, A. M. AU - Lisby, K. H. AU - King, C. AU - Christensen, R. F. AU - Jensen, H. F. AU - Idorn, L. AU - Søndergaard, L. AU - Moons, P. DB - PubMed DO - 10.1159/000333112 DP - NLM ET - 2011/11/19 IS - 1 KW - Adolescent Adult Anxiety Disorders/etiology Case-Control Studies Cross-Sectional Studies Depressive Disorder/etiology Female Health Status Heart Ventricles/*abnormalities/surgery Humans Male Patient Satisfaction Perception Quality of Life Self Concept Surveys and Questionnaires *Survivors Ventricular Dysfunction/*psychology Ventricular Function/*physiology Young Adult LA - eng N1 - 1421-9751 Overgaard, Dorthe Schrader, Anne-Marie Lisby, Karen H King, Catriona Christensen, Rie F Jensen, Helena F Idorn, Lars Søndergaard, Lars Moons, Philip Comparative Study Journal Article Switzerland Cardiology. 2011;120(1):36-42. doi: 10.1159/000333112. Epub 2011 Nov 17. PY - 2011 SN - 0008-6312 SP - 36-42 ST - Patient-reported outcomes in adult survivors with single-ventricle physiology T2 - Cardiology TI - Patient-reported outcomes in adult survivors with single-ventricle physiology VL - 120 ID - 200 ER - TY - JOUR AB - This study used a retrospective analysis of adults with single-ventricle physiology to ascertain the predictive power of cardiopulmonary stress-testing parameters in determining patients at increased risk of suffering from adverse clinical outcomes. We found that the specific parameters of percent of maximum predicted heart rate achieved and maximum oxygen consumption were significantly correlated with adverse clinical outcomes in patients with single-ventricle congenital heart disease. AD - Ahmanson/UCLA Adult Congenital Heart Disease Center, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA. AN - 21553266 AU - Bauer, B. S. AU - Aboulhosn, J. A. AU - Williams, R. J. AU - Child, J. S. DA - Oct DB - PubMed DO - 10.1007/s00246-011-0008-7 DP - NLM ET - 2011/05/10 IS - 7 KW - Adult Blood Pressure Diagnosis, Differential Disease Progression Echocardiography Electrocardiography Exercise Test/*methods Female Follow-Up Studies Heart Defects, Congenital/*diagnosis/metabolism/physiopathology Heart Ventricles/*abnormalities/diagnostic imaging/physiopathology Humans Male Middle Aged Oxygen Consumption Prognosis Retrospective Studies Severity of Illness Index Young Adult LA - eng N1 - 1432-1971 Bauer, Brenton S Aboulhosn, Jamil A Williams, Ryan J Child, John S Comparative Study Journal Article United States Pediatr Cardiol. 2011 Oct;32(7):891-5. doi: 10.1007/s00246-011-0008-7. Epub 2011 May 8. PY - 2011 SN - 0172-0643 SP - 891-5 ST - Patients with single-ventricle physiology: prognostic implications of stress testing T2 - Pediatr Cardiol TI - Patients with single-ventricle physiology: prognostic implications of stress testing VL - 32 ID - 451 ER - TY - JOUR AB - AIMS: To assess perceptions of child behaviour and parenting stress among the parents of young children with hypoplastic left heart syndrome (HLHS) and other forms of functionally univentricular heart defects (UVH). METHODS: As part of our prospective nation-wide neurodevelopmental follow-up study, the parents of 23 patients with HLHS, 14 with UVH and 46 healthy controls at the mean age of 18 months received the questionnaires Child Behavior Checklist and Parenting Stress Index. RESULTS: The reported level of total parenting stress was significantly higher among the mothers (mean score 241 vs 205, p < 0.001) and fathers (235 vs 202, p = 0.003) of patients with HLHS compared with those of controls. The parents of patients with HLHS reported significantly more total (mean T score 52 vs 45, p = 0.005) and internalizing (51 vs 41, p < 0.001) behaviour problems than the controls, but among the syndrome scales, a significant difference was only found in somatic complaints. The parents of patients with UVH did not report more parenting stress or emotional problems than the controls. CONCLUSION: Hypoplastic left heart syndrome, a severe congenital heart defect, increases parenting stress. The reported emotional maladjustment in affected children might in part be owing to somatic complaints. AD - Children's Hospital, University of Helsinki, Finland. anne.sarajuuri@hus.fi AN - 22040350 AU - Sarajuuri, A. AU - Lönnqvist, T. AU - Schmitt, F. AU - Almqvist, F. AU - Jokinen, E. DA - Mar DB - PubMed DO - 10.1111/j.1651-2227.2011.02509.x DP - NLM ET - 2011/11/02 IS - 3 KW - Case-Control Studies Child Behavior Disorders/*etiology Female Follow-Up Studies Heart Ventricles/*abnormalities Humans Hypoplastic Left Heart Syndrome/complications/*psychology Infant *Infant Behavior Male Parenting/*psychology Perception Prospective Studies Stress, Psychological/*etiology Surveys and Questionnaires LA - eng N1 - 1651-2227 Sarajuuri, A Lönnqvist, T Schmitt, F Almqvist, F Jokinen, E Journal Article Research Support, Non-U.S. Gov't Norway Acta Paediatr. 2012 Mar;101(3):252-7. doi: 10.1111/j.1651-2227.2011.02509.x. Epub 2011 Nov 29. PY - 2012 SN - 0803-5253 SP - 252-7 ST - Patients with univentricular heart in early childhood: parenting stress and child behaviour T2 - Acta Paediatr TI - Patients with univentricular heart in early childhood: parenting stress and child behaviour VL - 101 ID - 152 ER - TY - JOUR AB - Objective: To define the pattern of congenital heart disease (CHD) among neonates referred for echocardiography. Setting: Department of Pediatrics, Qatif Central Hospital. Design: A Prospective longitudinal and hospital based study. Method: All neonates with suspected CHD referred for echocardiography were reviewed for one year from March 2011. Result: Echocardiography was requested for 289 (9.5%) neonates, male to female ratio 1:1.1, mean birth weight 2.909 kg (range 0.605-5.150 kg); most of them were full-term (86%). One hundred forty-six (50.5%) neonates underwent echocardiography within the first 24 hours (group I). Among those neonates referred for echocardiography, 245 (84.8%) had heart murmur, 9 (3.1%) had cyanosis, 5 (1.7%) had questionable cardiomegaly, 13 (4.5%) had congenital anomalies, and 17 (5.9%) had various anomalies. Heart murmur was detected in 257 (88.9%) during routine neonatal examination. Sixty-four (2.1%) neonates were diagnosed to have CHD, 8 (12.5%) of them have critical congenital heart disease (CCHD). The most common CHD was ventricular septal defect (VSD) 32 (43.2%) followed by secundum atrial septal defect 31 (41.9%). Among 23 neonates with congenital anomalies referred for echocardiography, 10 (43.5%) had Down syndrome. Some neonates had more than one anomaly. Conclusion: Most neonates suspected to have CHD were referred for echocardiography during the first 24 hours. Heart murmur was the most common indication of referral for echocardiography and most of the cases with CHD and CCHD presented with heart murmur alone. Almost all cases of CCHD were diagnosed within the first 72 hours. The most common CHD was VSD followed by secundum ASD. Down syndrome was the most common congenital anomaly referred for echocardiography and 30% of them have CHD. AU - Khawahur, H. A. AU - Sowaiket, H. A. AU - Saffar, T. A. AU - Alzaher, G. AU - Darwich, A. AU - Qatary, K. A. DB - Embase DO - 10.12816/0004482 IS - 2 KW - acrocephalosyndactyly aortic coarctation article atrioventricular septal defect birth weight cardiomegaly cleft lip palate congenital heart disease cyanosis Down syndrome echocardiography Fallot tetralogy female heart atrium septum defect heart murmur heart ventricle septum defect human hypoplastic left heart syndrome leprechaunism longitudinal study major clinical study male meningomyelocele newborn newborn care pediatrics prematurity prospective study prune belly syndrome pulmonary valve stenosis skeleton malformation thorax radiography tricuspid valve atresia Philip CX 60 VIVID E7 VIVID E9 LA - English M3 - Article N1 - L373226775 2014-06-12 2014-08-19 PY - 2014 SN - 1012-8298 SP - 86-89 ST - The pattern of congenital heart disease among neonates referred for echocardiography T2 - Bahrain Medical Bulletin TI - The pattern of congenital heart disease among neonates referred for echocardiography UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L373226775 http://dx.doi.org/10.12816/0004482 VL - 36 ID - 931 ER - TY - JOUR AB - Purpose The purpose of this study was to explore parents' experiences of one specific timepoint in their infant's journey: the transition from hospital to home, following the first stage of their infant's cardiac surgery for complex congenital heart disease. Design and Methods A prospective longitudinal mixed methods study, underpinned with Middle Range Transition Theory (Meleis, Sawyer, Im, Hilfinger Messias, & Schumacher, 2000). Face to face and telephone interviews were conducted and self-report forms completed by parents at four-time points: before discharge (T0), 2 weeks after discharge (T1), 8 weeks after discharge (T2) and after stage two surgery (T3). Interviews were transcribed verbatim before inductive thematic analysis. Results Parents were recruited over a 15-month period from 2013 to 2015. Twelve mothers and 4 fathers took part. The infants had functionally univentricular heart (left n = 10, right n = 1) and a systemic shunt dependent lesion, tetralogy of Fallot (n = 1). Dynamic constructivist and constructionist social processes occurred for all parents, involving physical, physiological, psychological and cognitive elements within four ‘patterns of experience’, two of which ‘safety and security’ and ‘love and support’ are presented in this paper. Implications Parental support is essential; parents need to be engaged in discharge planning process and given the opportunity to express their needs to ensure that discharge care is truly patient and family centered. Conclusions Transition from hospital to home was complex and multi-faceted, with unanticipated physical and emotional transitions superimposed upon those that were expected. AD - Children's Cardiac Unit, Birmingham Women's and Children's NHS Foundation Trust, Birmingham Children's Hospital, Birmingham, UK Centre for Technology Enhanced Research, Coventry University, Coventry, UK Advancing Clinical Practice, Allied Health & Social Sciences Department, University of Worcester, Worcester, UK AN - 130462046. Language: English. Entry Date: 20180712. Revision Date: 20190515. Publication Type: Article AU - Gaskin, Kerry L. DB - ccm DO - 10.1016/j.pedn.2017.11.013 DP - EBSCOhost KW - Patient Discharge Parental Attitudes Heart Defects, Congenital -- Surgery Human Prospective Studies Interviews Self Report Thematic Analysis Tetralogy of Fallot Discharge Planning Family Centered Care Infant N1 - research. Journal Subset: Core Nursing; Double Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Nursing; Peer Reviewed; USA. NLM UID: 8607529. PY - 2018 SN - 0882-5963 SP - e23-e32 ST - Patterns of Transition Experience for Parents Going Home from Hospital with their Infant after First Stage Surgery for Complex Congenital Heart Disease T2 - Journal of Pediatric Nursing TI - Patterns of Transition Experience for Parents Going Home from Hospital with their Infant after First Stage Surgery for Complex Congenital Heart Disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=130462046&site=ehost-live&scope=site VL - 41 ID - 1573 ER - TY - JOUR AB - Pediatric Cardiac Care Consortium is a registry of cardiac catheterizations, surgical operations, and autopsies performed for infants, children, and adults with congenital heart disease. Four examples of use of PCCC data to evaluate variability in morphology, management, and outcomes for the procedures are described. Consideration is given to the following clinical problems: (1) the experience with surgical heart block in operative closure of perimembranous VSD, (2) the transition away from atrial baffle operations to the arterial switch operation for simple transposition of the great arteries, (3) the experience of planned 3 stage palliation of hypoplastic left heart syndrome, and (4) the identification of a high risk combination of cardiovascular anomalies in Williams syndrome. Analysis of registry outcomes allows ongoing quality improvement at a cardiac center to consider not only its own experience but that of the overall group. The PCCC data can be used to personalize management of rare congenital cardiac anomalies and combinations of anomalies. The PCCC registry allows longitudinal consideration of issues such as staged repairs and incidence of unplanned reoperation. In future years, the PCCC can facilitate investigations into the etiology of congenital heart disease. © 2009 Springer Science+Business Media, LLC. AD - L. A. Pyles, MMC 94, 420 Delaware St., Minneapolis, MN 55455, United States AU - Pyles, L. A. AU - Hills, C. M. AU - Larson, V. E. AU - Moller, J. H. DB - Embase Medline DO - 10.1007/s12265-009-9091-z IS - 2 KW - adolescent adult article child care decision support system evidence based medicine heart block heart function human hypoplastic left heart syndrome major clinical study priority journal Williams Beuren syndrome LA - English M3 - Article N1 - L50453591 2009-05-19 PY - 2009 SN - 1937-5387 1937-5395 SP - 219-224 ST - Pediatric cardiac care consortium: An instrument for evidence-based clinical decision support T2 - Journal of Cardiovascular Translational Research TI - Pediatric cardiac care consortium: An instrument for evidence-based clinical decision support UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50453591 http://dx.doi.org/10.1007/s12265-009-9091-z VL - 2 ID - 1181 ER - TY - JOUR AB - Pediatric heart transplantation has undergone major changes over the past two decades, marked by a substantial improvement in survival, reduction in posttransplant complications, and enhancement in quality of life for transplant recipients. Actuarial survival has improved substantially in the last decade. Indications for pediatric heart transplant have changed as surgery for complex congenital heart lesions has evolved. There are now left and right ventricular assist devices that are suitable for use in infants as a bridge to transplantation. New immunosuppressive agents have reduced the risk of rejection while minimizing side effects and strategies to reduce the risk of graft coronary disease are beginning to show promise. Finally, true long-term survival for children after heart transplant has now been demonstrated and quality of life is excellent. © 2006 Elsevier Inc. All rights reserved. AD - D. Bernstein, Departments of Pediatrics and Cardiothoracic Surgery, Stanford University, Stanford, CA, United States AU - Alkhaldi, A. AU - Chin, C. AU - Bernstein, D. DB - Embase Medline DO - 10.1053/j.sempedsurg.2006.03.011 IS - 3 KW - aciclovir antivirus agent atorvastatin azathioprine calcineurin inhibitor calcium channel blocking agent clonidine corticosteroid cyclosporine daclizumab diltiazem dipeptidyl carboxypeptidase inhibitor diuretic agent everolimus hydroxymethylglutaryl coenzyme A reductase inhibitor interleukin 2 derivative lymphocyte antibody methylprednisolone monoclonal antibody mycophenolate mofetil OKT 3 pravastatin prednisone rapamycin rituximab steroid tacrolimus article artificial ventilation blood group ABO system cardiac graft rejection congenital heart malformation donor fibrosing alveolitis glomerulosclerosis heart assist device heart transplantation human hypertension immunosuppressive treatment infection ischemic heart disease kidney disease lymphoproliferative disease microangiopathy nephrotoxicity patient selection postoperative complication priority journal quality of life surgical technique survival vascular disease LA - English M3 - Article N1 - L43963393 2006-08-06 PY - 2006 SN - 1055-8586 SP - 188-198 ST - Pediatric cardiac transplantation T2 - Seminars in Pediatric Surgery TI - Pediatric cardiac transplantation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L43963393 http://dx.doi.org/10.1053/j.sempedsurg.2006.03.011 VL - 15 ID - 1257 ER - TY - JOUR AB - Heart transplantation in pediatric patients generally arises as a treatment option of last resort, that is, the indication is for patients with heart failure of various etiologies, with potential or actual end-organ dysfunction, in whom there are no reasonable, long-term options for life-prolonging therapy. The concept of heart failure is complex in a pediatric population, particularly those with congenital heart disease. While heart failure may refer simply to systolic dysfunction leading to low cardiac output, it can also encompass: diastolic dysfunction in restrictive cardiomyopathy; single ventricle physiology without an option for stable palliation. A good candidate should have a predicted life expectancy less than the median lifetime of a transplanted heart. Significant improvement in survival has been observed over time with 1- and 5-year survival approximately 90% and 80% in the contemporary era. AD - C. Chin, Division of Cardiology, The Heart Institute, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave-MLC 2003, Cincinnati, Ohio, United States AU - Ryan, T. D. AU - Chin, C. DB - Embase Medline DO - 10.1053/j.sempedsurg.2017.07.012 IS - 4 KW - article artificial ventilation cardiopulmonary exercise test clinical protocol congenital heart disease extracorporeal oxygenation heart catheterization heart transplantation hospital admission human immunosuppressive treatment laboratory test patient care patient selection pediatrics postoperative period practice guideline priority journal social psychology survival rate treatment contraindication treatment indication treatment planning LA - English M3 - Article N1 - L618044869 2017-09-04 2017-10-12 PY - 2017 SN - 1532-9453 1055-8586 SP - 206-212 ST - Pediatric cardiac transplantation T2 - Seminars in Pediatric Surgery TI - Pediatric cardiac transplantation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L618044869 http://dx.doi.org/10.1053/j.sempedsurg.2017.07.012 VL - 26 ID - 736 ER - TY - JOUR AB - We use warm induction and reperfusion with amino acid enhancement on all neonates and preoperatively stressed patients to induce arrest without contracture and to replete high-energy substrates. Blood cardioplegia is used in all age groups. We then employ hypothermia in the majority of patients (all neonates), ranging from deep (18 degrees C), to moderate (26 degrees to 28 degrees C), with warm or near normothermia used in only simpler anatomic repairs. The clear benefits of hypothermia in conferring additional cardiac and systemic ischemic protection along with the relatively greater ease of cooling and rewarming in the pediatric patient warrant its continued use in the majority of open-heart cases. Calcium levels are maintained in the 0.3 to 0.5 mM/L range during the conduct of the operation and reperfusion phase. Before removal from bypass, calcium is administered through either bolus or continuous drip technique to provide a normocalcemic level of 1 to 1.2 mM/L. This close attention is particularly important in the neonate to avoid contracture injuries and to maximize cardiac function, and is warranted if citrate-phosphate-dextrose (CPD)-containing transfusion or prime components are used. Preoperative evaluation for aortopulmonary collaterals with coil embolization is routinely performed, and is particularly important in single ventricle physiology where preserved myocardial function is so vital to a favorable outcome. The opportunity to perform some surgeries off bypass, such as on the RV outflow tract, may be used whenever it represents a viable alternative in very young or ill patients. Similarly, the majority of Glenn shunts, for example, are performed using a caval-RA shunt without formal cardiopulmonary bypass.(ABSTRACT TRUNCATED AT 250 WORDS) AD - Division of Cardiothoracic Surgery, University of California, Los Angeles Medical Center 90024. AN - 8481452 AU - Drinkwater, D. C. AU - Laks, H. DA - Apr DB - PubMed DP - NLM ET - 1993/04/01 IS - 2 KW - Cardiopulmonary Bypass Child Heart Arrest, Induced/*methods Heart Defects, Congenital/physiopathology/*surgery Humans Myocardial Reperfusion LA - eng N1 - Drinkwater, D C Laks, H Journal Article United States Semin Thorac Cardiovasc Surg. 1993 Apr;5(2):168-75. PY - 1993 SN - 1043-0679 (Print) 1043-0679 SP - 168-75 ST - Pediatric cardioplegic techniques T2 - Semin Thorac Cardiovasc Surg TI - Pediatric cardioplegic techniques VL - 5 ID - 430 ER - TY - JOUR AB - Advances in prenatal imaging allow early detection of single-ventricle congenital heart disease, which may enhance prenatal care and maximize care options and decision making. Boston Children's Hospital's Advanced Fetal Care Center and fetal cardiology program provide prenatal counseling and care for single-ventricle congenital heart disease. Key points for optimal prenatal counseling and education include explanation of the diagnosis, delivery, the first surgery, cardiac neurodevelopmental issues, feeding and growth issues, quality of life and long-term care, family stressors, and fetal cardiac intervention. Such counseling and education help families make the difficult decisions required in this situation. AD - Boston Children's Hospital Cincinnati Children's Hospital Medical Center Nurse practitioner, Department of Cardiology, Boston Children's Hospital Clinical nurse specialist, cardiovascular intensive care unit, Boston Children's Hospital AN - 109512521. Language: English. Entry Date: 20150924. Revision Date: 20191029. Publication Type: Article AU - Beke, Dorothy M. AU - Staveski, Sandra AU - Lafranchi, Terra AU - Lincoln, Patricia DB - ccm DO - 10.4037/ccn2015247 DP - EBSCOhost IS - 5 KW - Prenatal Care Counseling -- In Pregnancy Hypoplastic Left Heart Syndrome -- Therapy Hypoplastic Left Heart Syndrome -- Ultrasonography -- In Utero Hypoplastic Left Heart Syndrome -- Surgery Ultrasonography, Prenatal Fetus Female Pregnancy Medical Records Record Review Information Resources Patient Education Pregnancy, High Risk Delivery, Obstetric Infant, Newborn Quality of Life Long Term Care Family -- Psychosocial Factors Patient Orientation Intensive Care Units, Neonatal Decision Making, Patient Expectant Parents -- Psychosocial Factors Abortion, Induced Hypoplastic Left Heart Syndrome -- Prognosis Expectant Mothers N1 - case study; tables/charts. Journal Subset: Blind Peer Reviewed; Core Nursing; Double Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Nursing; Peer Reviewed; USA. Special Interest: Obstetric Care; Pediatric Care; Perioperative Care. NLM UID: 8207799. PY - 2015 SN - 0279-5442 SP - 53-61 ST - Pediatric Cardiovascular Surgery. Prenatal Counseling and Care for Single-Ventricle Heart Disease: One Center's Model for Care T2 - Critical Care Nurse TI - Pediatric Cardiovascular Surgery. Prenatal Counseling and Care for Single-Ventricle Heart Disease: One Center's Model for Care UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=109512521&site=ehost-live&scope=site VL - 35 ID - 1444 ER - TY - JOUR AB - Nurses caring for children with congenital heart disease face unique challenges, especially when caring for neonates diagnosed with hypoplastic left heart syndrome (HLHS). The treatment options for these neonates present difficult choices for the child's decision makers and are not without significant life-altering consequences. In order to assist in the decision-making process, nurses as patient and family advocates should acknowledge the unique role they play in the informed consent process, while simultaneously identifying specific ethical principles that are components of this process. By incorporating the principles of autonomy, beneficence, and veracity into specific nursing interventions, nurses can assist families in making informed decisions regarding a treatment option that is best for the child as well as the family. AN - 106869989. Language: English. Entry Date: 20030926. Revision Date: 20150818. Publication Type: Journal Article. Journal Subset: Core Nursing AU - Zeigler, V. L. DB - ccm DP - EBSCOhost IS - 1 KW - Decision Making, Ethical Decision Making, Family Hypoplastic Left Heart Syndrome -- Therapy Autonomy Beneficence Consent Heart Transplantation Infant, Newborn Information Resources Nursing Role Palliative Care Parents Truth Disclosure N1 - Nursing; Peer Reviewed; USA. NLM UID: 7505804. PMID: NLM12630511. PY - 2003 SN - 0097-9805 SP - 65-69 ST - Pediatric ethics, issues, & commentary. Ethical principles and parental choice: treatment options for neonates with hypoplastic left heart syndrome T2 - Pediatric Nursing TI - Pediatric ethics, issues, & commentary. Ethical principles and parental choice: treatment options for neonates with hypoplastic left heart syndrome UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106869989&site=ehost-live&scope=site VL - 29 ID - 1517 ER - TY - JOUR AB - Pediatric heart transplantation (pHTx) represents a small (14%) but very important and particular part in the field of cardiac transplantation. This treatment has lifelong impact on children. To achieve the best short and especially long-term survival with adequate quality of life, which is of crucial importance for this young patient population, one has to realize and understand the differences with adult HTx. Indication for transplantation, waitlist management including ABO incompatible (ABOi) transplantation and immunosuppression differ. Although young transplant recipients are ultimately likely to be considered for re-transplantation. One has to distinguish between myopathy and complex congenital heart disease (CHD). The differences in anatomy and physiology make the surgical procedure much more complex and create unique challenges. These recipients need a well-organized and educated team with pediatric cardiologists and intensivists, including a high skilled surgeon, which is dedicated to pHTx. Therefore, these types of transplants are best concentrated in specialized centers to achieve promising outcome. AD - M. Schweiger, Department for Congenital Cardiovascular Surgery, University Children's Hospital, Zurich, Switzerland AU - Schweiger, M. AU - Stiasny, B. AU - Dave, H. AU - Cavigelli-Brunner, A. AU - Balmer, C. AU - Kretschmar, O. AU - Bürki, C. AU - Klauwer, D. AU - Hübler, M. DB - Embase DO - 10.3978/j.issn.2072-1439.2015.01.38 IS - 3 KW - inotropic agent article blood group ABO incompatibility cardiac allograft vasculopathy cardiomyopathy congenital heart disease donor selection Fontan procedure geographic distribution graft recipient heart failure heart transplantation high risk population human life expectancy long term care morbidity mortality Norwood procedure patient selection pediatrics practice guideline psychosocial care resuscitation risk factor surgical technique survival rate treatment contraindication ventricular assist device LA - English M3 - Article N1 - L604105903 2015-05-06 2015-05-08 PY - 2015 SN - 2077-6624 2072-1439 SP - 552-559 ST - Pediatric heart transplantation T2 - Journal of Thoracic Disease TI - Pediatric heart transplantation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L604105903 http://dx.doi.org/10.3978/j.issn.2072-1439.2015.01.38 VL - 7 ID - 885 ER - TY - JOUR AB - Heart transplantation has become the standard of care for patients with end-stage heart failure. The efficacy and therapeutic advantages of transplantation in the pediatric population have not been fully determined. Between March 1985 and September 1986, nine pediatric heart transplantations were performed; the ages ranged from 39 days to 19 years; weight ranged from 2.3 to 100 kg. The underlying disease was acquired cardiomyopathy (four patients); cardiomyopathy caused by congenital mitral valve disease (two patients); unresectable fibroma of the left ventricle in a newborn (one patient); hypoplastic left heart syndrome (one patient); and hypertrophic obstructive cardiomyopathy (one patient). Initial immunosuppression therapy consisted of cyclosporine, prednisone, and antithymocyte globulin. Recently, newer protocols have evolved through experience. Seven patients survived the perioperative period and had follow-up from 1 to 19 months, for a total of 41 transplant months. Rejection occurred at a rate of 1.4 episodes per month in children compared with 0.8 episodes per month in our adult patients. There was no statistical difference in the number, severity, or timing of rejection episodes in the pediatric versus adult population. Major complications included cyclosporine-induced seizures in two patients, mild hypertension in two, five infectious episodes (three bacterial and two viral), and three late deaths. All children who survived are in New York Heart Association functional class I with no developmental delays. This series is heavily weighted with children (33% less than age 1 year). Early results demonstrate that pediatric and infant heart transplantation is technically practical. Improved results are to be expected with additional experience and further modification of adult protocols to assure patient growth and minimize the high infection rate. AD - Pediatric Heart Institute, St. Christopher's Hospital for Children, Philadelphia, PA 19133. AN - 3320304 AU - Dunn, J. M. AU - Cavarocchi, N. C. AU - Balsara, R. K. AU - Kolff, J. AU - McClurken, J. AU - Badellino, M. M. AU - Vieweg, C. AU - Donner, R. M. DA - Nov-Dec DB - PubMed DP - NLM ET - 1987/11/01 IS - 6 KW - Adolescent Adult Biopsy Cardiomyopathies/pathology/surgery Child Graft Rejection/drug effects *Heart Transplantation Humans Immunosuppressive Agents/*therapeutic use Infant Infant, Newborn Infections/etiology Kidney Diseases/etiology Methods Myocardium/pathology Postoperative Complications/mortality Quality of Life LA - eng N1 - Dunn, J M Cavarocchi, N C Balsara, R K Kolff, J McClurken, J Badellino, M M Vieweg, C Donner, R M Comparative Study Journal Article United States J Heart Transplant. 1987 Nov-Dec;6(6):334-42. PY - 1987 SN - 0887-2570 (Print) 0887-2570 SP - 334-42 ST - Pediatric heart transplantation at St. Christopher's Hospital for Children T2 - J Heart Transplant TI - Pediatric heart transplantation at St. Christopher's Hospital for Children VL - 6 ID - 465 ER - TY - JOUR AB - Trisomy 13 and 18 are associated with congenital heart disease. Cardiac palliation has been reported in the literature, but is not usually done in this population. Thus, a multi-disciplinary team may experience controversy in formulating a care plan that includes cardiac intervention. Our objective was to determine differences in recommendations for cardiac intervention in this population between physicians specializing in pediatric cardiac critical care, neonatology, and genetics. A web-based survey was performed between April 2007 and August 2008. This survey evaluated surgical and transcatheter cardiac palliations that had been performed for individuals with trisomy 13 or 18 at the respondent's institution, the respondent's recommendations for cardiac intervention in hypothetical symptomatic patients with trisomy 13 or trisomy 18 and the influence of parental preference on these recommendations. Eight hundred fifty-nine responses were obtained from a primarily academic practice setting (59%). Cardiologists were most likely to recommend intervention; low risk interventions were recommended by 32% of cardiologists, 7% of neonatologists and 20% of geneticists. Parental request to intervene resulted in a 3 fold increased in the likelihood of all specialist recommending intervention. Counseling of families frequently occurred by multiple sub specialists (50%) and there was frequently (71%) a difference in opinion. Individuals with trisomy 13 or 18 are receiving cardiac intervention at many institutions. Cardiologists were more likely than geneticists or neonatologists to recommend intervention on all heart lesions other than single ventricle palliation which no specialists recommended. Parental wishes that 'everything be done' significantly influenced all specialists' recommendations. AD - Nationwide Children's Hospital and Research Institute, Columbus USA AN - 64590404. Language: English. Entry Date: 20111021. Revision Date: 20191120. Publication Type: Article AU - Yates, Andrew AU - Hoffman, Timothy AU - Shepherd, Edward AU - Boettner, Bethany AU - McBride, Kim DB - ccm DO - 10.1007/s10897-011-9373-x DP - EBSCOhost IS - 5 KW - Trisomy 13 -- Complications Trisomy 18 -- Complications Heart Defects, Congenital -- Etiology Heart Defects, Congenital -- Therapy Attitude of Health Personnel Human Questionnaires Pediatrics Specialties, Medical Parental Attitudes Data Analysis Software Analysis of Variance Post Hoc Analysis Male Female Descriptive Statistics Infant, Newborn Ethics, Medical N1 - questionnaire/scale; research; tables/charts. Journal Subset: Biomedical; Continental Europe; Europe; Peer Reviewed. Special Interest: Pediatric Care; Psychiatry/Psychology. NLM UID: 9206865. PY - 2011 SN - 1059-7700 SP - 495-509 ST - Pediatric Sub-specialist Controversies in the Treatment of Congenital Heart Disease in Trisomy 13 or 18 T2 - Journal of Genetic Counseling TI - Pediatric Sub-specialist Controversies in the Treatment of Congenital Heart Disease in Trisomy 13 or 18 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=64590404&site=ehost-live&scope=site VL - 20 ID - 1485 ER - TY - JOUR AB - Trisomy 13 and 18 are associated with congenital heart disease. Cardiac palliation has been reported in the literature, but is not usually done in this population. Thus, a multi-disciplinary team may experience controversy in formulating a care plan that includes cardiac intervention. Our objective was to determine differences in recommendations for cardiac intervention in this population between physicians specializing in pediatric cardiac critical care, neonatology, and genetics. A web-based survey was performed between April 2007 and August 2008. This survey evaluated surgical and transcatheter cardiac palliations that had been performed for individuals with trisomy 13 or 18 at the respondent's institution, the respondent's recommendations for cardiac intervention in hypothetical symptomatic patients with trisomy 13 or trisomy 18 and the influence of parental preference on these recommendations. Eight hundred fifty-nine responses were obtained from a primarily academic practice setting (59%). Cardiologists were most likely to recommend intervention; low risk interventions were recommended by 32% of cardiologists, 7% of neonatologists and 20% of geneticists. Parental request to intervene resulted in a 3 fold increased in the likelihood of all specialist recommending intervention. Counseling of families frequently occurred by multiple sub specialists (50%) and there was frequently (71%) a difference in opinion. Individuals with trisomy 13 or 18 are receiving cardiac intervention at many institutions. Cardiologists were more likely than geneticists or neonatologists to recommend intervention on all heart lesions other than single ventricle palliation which no specialists recommended. Parental wishes that "everything be done" significantly influenced all specialists' recommendations. AD - Nationwide Children's Hospital and Research Institute, Columbus, OH, USA. Andrew.Yates@Nationwidechildrens.org AN - 21590470 AU - Yates, A. R. AU - Hoffman, T. M. AU - Shepherd, E. AU - Boettner, B. AU - McBride, K. L. DA - Oct DB - PubMed DO - 10.1007/s10897-011-9373-x DP - NLM ET - 2011/05/19 IS - 5 KW - *Chromosomes, Human, Pair 13 *Chromosomes, Human, Pair 18 Cohort Studies Female Heart Defects, Congenital/genetics/*therapy Humans Male *Pediatrics Physicians/*psychology *Trisomy Workforce LA - eng N1 - 1573-3599 Yates, Andrew R Hoffman, Timothy M Shepherd, Edward Boettner, Bethany McBride, Kim L Journal Article United States J Genet Couns. 2011 Oct;20(5):495-509. doi: 10.1007/s10897-011-9373-x. Epub 2011 May 18. PY - 2011 SN - 1059-7700 SP - 495-509 ST - Pediatric sub-specialist controversies in the treatment of congenital heart disease in trisomy 13 or 18 T2 - J Genet Couns TI - Pediatric sub-specialist controversies in the treatment of congenital heart disease in trisomy 13 or 18 VL - 20 ID - 362 ER - TY - JOUR AN - 106428194. Language: English. Entry Date: 20060421. Revision Date: 20150711. Publication Type: Journal Article DB - ccm DP - EBSCOhost IS - 1 KW - Adrenocortical Hyperfunction Antibiotics Asthma Cytomegalovirus Infections Decision Making, Patient Disaster Planning Hypoplastic Left Heart Syndrome Infant, Low Birth Weight Infant, Newborn Infant, Very Low Birth Weight Lactation Lyme Disease Meningitis Neonatal Abstinence Syndrome Obesity Placenta Resuscitation -- In Infancy and Childhood Schools Scleroderma, Systemic Sleep Apnea Syndromes -- Drug Therapy Thrombosis Respiration, Artificial -- Psychosocial Factors N1 - abstract. Journal Subset: Biomedical; Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. NLM UID: 0376422. PY - 2006 SN - 0031-4005 SP - 192-203 ST - PeDIATRICS electronic pages T2 - Pediatrics TI - PeDIATRICS electronic pages UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106428194&site=ehost-live&scope=site VL - 117 ID - 1623 ER - TY - JOUR AB - Patients with a Fontan circulation are at risk of a sedentary lifestyle. Given the direct relationship between physical activity and health, promotion of physical activity has the potential to improve outcomes, including quality of life (QOL). This study aimed to describe self-reported physical activity levels in adult Fontan patients and examine associations between physical activity, perceived health status and QOL. The sample consisted of 177 Fontan patients (M(age) = 27.5 ± 7.6 years, 52% male) who reported their physical activity, perceived health status, and QOL as part of the cross-sectional Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease - International Study. Descriptive statistics and univariate analyses of variance with planned contrasts were computed to describe physical activity characteristics. Mediation analyses tested whether perceived health status variables mediated the association between physical activity and QOL. Forty-six percent of patients were sedentary while only 40% met international physical activity guidelines. Higher physical activity was associated with younger age, lower NYHA class, higher perceived general health, and greater QOL. Patients who commuted by walking and engaged in sports reported better perceived health and QOL. Mediation analyses revealed that perceived general health but not NYHA functional class mediated the association between physical activity and QOL (αβ = 0.22, 95% confidence interval = 0.04 to 0.49). In conclusion, Fontan patients likely benefit from regular physical activity, having both higher perceived general health and functional capacity; greater perceived health status may contribute to enhanced QOL. In conclusion, these data support the pivotal role of regular physical activity for Fontan patients. AD - Department of Child and Adolescent Psychiatry and Behavioral Sciences, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia. KU Leuven Department of Public Health and Primary Care, KU Leuven - University of Leuven, Leuven, Belgium; Institute of Health and Care Sciences, University of Gothenburg, Gothenburg, Sweden; Department of Paediatrics and Child Health, University of Cape Town, Cape Town, South Africa. Peter Munk Cardiac Centre, University Health Network, University of Toronto, Toronto, Canada; Knight Cardiovascular Institute, Oregon Health & Science University, Portland, Oregon. School Psychology and Development in Context, KU Leuven - University of Leuven, Leuven, Belgium; UNIBS, University of the Free State Bloemfontein, Bloemfontein, South Africa. KU Leuven Department of Public Health and Primary Care, KU Leuven - University of Leuven, Leuven, Belgium. Pediatric Cardiology, Frontier Lifeline Hospital (Dr. K. M. Cherian Heart Foundation), Chennai, India. Department of Congenital Heart Disease, Louis Pradel Hospital, Hospices civils de Lyon, Lyon, France. Adult Congenital Heart Disease Center, Oslo University Hospital - Rikshospitalet, Oslo, Norway. Center for Biobehavioral Health, Nationwide Children's Hospital, Columbus, Ohio. Department of Adult Congenital Heart Disease, Chiba Cardiovascular Center, Chiba, Japan. Stanford University School of Medicine, Department of Pediatrics and Medicine, Division of Pediatric Cardiology and Cardiovascular Medicine, Palo Alto, California. Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden. Division of Cardiology, Hospital de Niños, Córdoba, Argentina. Adult Congenital Heart Unit, Sahlgrenska University Hospital/Östra, Gothenburg, Sweden; Institute of Medicine, The Sahlgrenska Academy at University of Gothenburg, Gothenburg, Sweden. Institute of Health and Care Sciences, University of Gothenburg, Gothenburg, Sweden; Department of Health Sciences, University West, Trollhättan, Sweden. Monash Heart, Monash Medical Centre, Monash University, Melbourne, Australia. Department of Cardiology, Mater Dei Hospital, Birkirkara, Malta. Adult Congenital Heart Disease Center, University of Nebraska Medical Center/ Children's Hospital and Medical Center, Omaha, Nebraska. Division of Cardiology, Stollery Children's Hospital, University of Alberta, Edmonton, Canada. Center for Congenital Heart Disease, Department of Cardiology, Inselspital - Bern University Hospital, University of Bern, Bern, Switzerland. Division of Congenital and Structural Cardiology, University Hospitals Leuven, Leuven, Belgium; KU Leuven Department of Cardiovascular Sciences, KU Leuven - University of Leuven, Leuven, Belgium. Adult Congenital Heart Disease Center, Washington University and Barnes Jewish Heart & Vascular Center, University of Missouri, Saint Louis, Missouri. Department of Cardiology, Academic Medical Center, Amsterdam, the Netherlands. Clinical Psychology Service, IRCCS Policlinico San Donato, Milan, Italy. Adult Congenital Heart Disease Center, Helen DeVos Children's Hospital, Grand Rapids, Michigan. Adult Congenital Heart Center, Montreal Heart Institute, Université de Montréal, Montreal, Canada. The University of Texas Southwestern Medical Center, Dallas, Texas. Electronic address: ari.cedars@utsouthwestern.edu. AN - 31030969 AU - Holbein, C. E. AU - Veldtman, G. R. AU - Moons, P. AU - Kovacs, A. H. AU - Luyckx, K. AU - Apers, S. AU - Chidambarathanu, S. AU - Soufi, A. AU - Eriksen, K. AU - Jackson, J. L. AU - Enomoto, J. AU - Fernandes, S. M. AU - Johansson, B. AU - Alday, L. AU - Dellborg, M. AU - Berghammer, M. AU - Menahem, S. AU - Caruana, M. AU - Kutty, S. AU - Mackie, A. S. AU - Thomet, C. AU - Budts, W. AU - White, K. AU - Sluman, M. A. AU - Callus, E. AU - Cook, S. C. AU - Khairy, P. AU - Cedars, A. DA - Jul 1 DB - PubMed DO - 10.1016/j.amjcard.2019.03.039 DP - NLM ET - 2019/04/30 IS - 1 KW - Adult Cross-Sectional Studies *Exercise Female *Fontan Procedure *Health Status Heart Defects, Congenital/physiopathology/*psychology/surgery Humans Male *Quality of Life Sedentary Behavior *Self Concept Self Report Young Adult LA - eng N1 - 1879-1913 Holbein, Christina E Veldtman, Gruschen R Moons, Philip Kovacs, Adrienne H Luyckx, Koen Apers, Silke Chidambarathanu, Shanti Soufi, Alexandra Eriksen, Katrine Jackson, Jamie L Enomoto, Junko Fernandes, Susan M Johansson, Bengt Alday, Luis Dellborg, Mikael Berghammer, Malin Menahem, Samuel Caruana, Maryanne Kutty, Shelby Mackie, Andrew S Thomet, Corina Budts, Werner White, Kamila Sluman, Maayke A Callus, Edward Cook, Stephen C Khairy, Paul Cedars, Ari APPROACH-IS consortium and the International Society for Adult Congenital Heart Disease (ISACHD) T32 HD068223/HD/NICHD NIH HHS/United States Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't United States Am J Cardiol. 2019 Jul 1;124(1):144-150. doi: 10.1016/j.amjcard.2019.03.039. Epub 2019 Apr 10. PY - 2019 SN - 0002-9149 SP - 144-150 ST - Perceived Health Mediates Effects of Physical Activity on Quality of Life in Patients With a Fontan Circulation T2 - Am J Cardiol TI - Perceived Health Mediates Effects of Physical Activity on Quality of Life in Patients With a Fontan Circulation VL - 124 ID - 24 ER - TY - JOUR AB - OBJECTIVES: Siblings of children with chronic medical conditions endorse a lower quality of life compared to age-matched peers. Caregiver and sibling-self report of adjustment are often discordant. Congenital heart disease significantly affects family life. To date, there have been no studies addressing the functioning of siblings of children with hypoplastic left heart syndrome, one of the most severe forms of congenital heart disease. The goal of this study was to assess the impact of hypoplastic left heart syndrome on sibling's quality of life as well as the caregiver's perception of this effect. STUDY DESIGN: Cross-sectional study using a web-based survey distributed via various listservs targeted towards families of children with hypoplastic left heart syndrome. Employed the Sibling Perception Questionnaire, designed to assess sibling and caregiver perceptions of adjustment to chronic illness. A Negative Adjustment Composite Score was calculated for each respondent, with higher values representing more negative adjustment. RESULTS: Thirty-five caregivers responded. Majority of caregivers were female (74%), white (86%) and college educated (54%). Thirty-two siblings participated, ranging in age from 7 to 30 years of age (12.5 ± 6.3). Most children with hypoplastic left heart syndrome (73%) had undergone the third stage of palliation. Forty-two caregiver-sibling pairs were examined. Caregiver Negative Adjustment Composite Scores were significantly higher than sibling scores, with caregivers reporting more adjustment problems (2.4 ± 0.4) than siblings (2.3 ± 0.3, P < .05). Sibling age was correlated with worse caregiver and sibling scores (r 0.35, P < .05). CONCLUSIONS: Caregivers of children with hypoplastic left heart syndrome perceive their siblings as struggling more than the children self-report. Siblings tend to report worse adjustment as they get older. These data suggest that programs should include support for the entire family through all ages to optimize quality of life. AD - Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania, USA. Nationwide Children's Hospital, Columbus, Ohio, USA. Children's National Medical Center, Washington, Washington, D.C., USA. AN - 30019479 AU - Caris, E. C. AU - Dempster, N. AU - Wernovsky, G. AU - Miao, Y. AU - Moore-Clingenpeel, M. AU - Neely, T. AU - Fonseca, R. AU - Miller-Tate, H. AU - Allen, R. AU - Fichtner, S. AU - Stewart, J. AU - Cua, C. L. DA - Jul DB - PubMed DO - 10.1111/chd.12619 DP - NLM ET - 2018/07/19 IS - 4 KW - Adaptation, Psychological/*physiology Adolescent Adult Caregivers/*psychology Child Child, Preschool Cross-Sectional Studies Female Follow-Up Studies Humans Hypoplastic Left Heart Syndrome/*psychology Infant Male *Perception Prospective Studies *Quality of Life Self Report Siblings/*psychology Surveys and Questionnaires Young Adult congenital heart disease hypoplastic left heart syndrome siblings survey LA - eng N1 - 1747-0803 Caris, Elizabeth C Orcid: 0000-0002-2138-4328 Dempster, Nicole Wernovsky, Gil Miao, Yongjie Moore-Clingenpeel, Melissa Neely, Trent Fonseca, Rachel Miller-Tate, Holly Allen, Robin Fichtner, Samantha Stewart, Jamie Cua, Clifford L Orcid: 0000-0002-9172-3874 Journal Article United States Congenit Heart Dis. 2018 Jul;13(4):528-532. doi: 10.1111/chd.12619. Epub 2018 Jul 17. PY - 2018 SN - 1747-079x SP - 528-532 ST - Perception scores of siblings and parents of children with hypoplastic left heart syndrome T2 - Congenit Heart Dis TI - Perception scores of siblings and parents of children with hypoplastic left heart syndrome VL - 13 ID - 48 ER - TY - JOUR AB - Background: To determine the feasibility and clinical result of selective embolization of hepatoduodenal or paratracheal lymphatics in Fontan patients with protein-losing enteropathy (PLE) or plastic bronchitis (PB). Methods: Dilated lymph vessels in periportal (PLE) or paratracheal (PB) position were percutaneously punctured with a 22G Chiba needle. Intralymphatic position was confirmed by water soluble contrast injection with drainage to hepatoduodenal or tracheal fistulae. After flushing with 10% glucose solution, occlusion of hepatoduodenal or paratreacheal lymphatics was effected by injection of 1–4 cc mixture 4/1 of Lipiodol/n-butyl cyanoacrylate (n-BCA; Histoacryl). Results: Seven patients with proven PLE were treated with periportal lymphatic embolization 10.7 (range: 6.6–13.5) years after the Fontan operation. The Fontan operation was performed at a median age of 3.7 (range: 2.9–5.7) years and PLE started a median of 3.1 (range: 0.9–4.7) years later. Five patients required a second procedure 2–8 months later. Complications were limited (spillage of glue in portal branch, transient cholangitis, and caustic duodenal bleeding). Six of seven patients reported significant improvement in quality of life and normalization of albumin levels after limited follow-up (p <.01). One patient (Fontan at 2.9 years; age 16.4 years) had PB for 2 years. Selective transthoracic cone-beam-directed puncture of left and right paratracheal lymphatics with n-BCA embolization of distal lymphatic fistulae resulted in lasting absence of tracheal casts (11 months). Conclusions: Embolization of periportal/peritracheal lymphatics is a promising technique in Fontan patients with PLE/PB. Larger series are required to determine incidence and reasons of success/failure, with long-term results and effects on liver function. AD - M. Gewillig, Department of Paediatric and Congenital Cardiology, University Hospitals Leuven, Leuven, Belgium AU - Maleux, G. AU - Storme, E. AU - Cools, B. AU - Heying, R. AU - Boshoff, D. AU - Louw, J. J. AU - Frerich, S. AU - Malekzadeh-Milanii, S. AU - Hubrechts, J. AU - Brown, S. C. AU - Gewillig, M. DB - Embase Medline DO - 10.1002/ccd.28501 IS - 7 KW - Chiba needle albumin adolescent article artificial embolization bronchitis child cholangitis clinical article computer assisted tomography duodenum bleeding feasibility study fistula Fontan procedure human liver parenchyma protein losing gastroenteropathy quality of life retrospective study LA - English M3 - Article N1 - L2003509313 2019-11-12 PY - 2019 SN - 1522-726X 1522-1946 SP - 996-1002 ST - Percutaneous embolization of lymphatic fistulae as treatment for protein-losing enteropathy and plastic bronchitis in patients with failing Fontan circulation T2 - Catheterization and Cardiovascular Interventions TI - Percutaneous embolization of lymphatic fistulae as treatment for protein-losing enteropathy and plastic bronchitis in patients with failing Fontan circulation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2003509313 http://dx.doi.org/10.1002/ccd.28501 VL - 94 ID - 584 ER - TY - JOUR AB - Background - Plastic bronchitis is a potentially fatal disorder occurring in children with single-ventricle physiology, and other diseases, as well, such as asthma. In this study, we report findings of abnormal pulmonary lymphatic flow, demonstrated by MRI lymphatic imaging, in patients with plastic bronchitis and percutaneous lymphatic intervention as a treatment for these patients. Methods and Results - This is a retrospective case series of 18 patients with surgically corrected congenital heart disease and plastic bronchitis who presented for lymphatic imaging and intervention. Lymphatic imaging included heavy T2-weighted MRI and dynamic contrast-enhanced magnetic resonance lymphangiogram. All patients underwent bilateral intranodal lymphangiogram, and most patients underwent percutaneous lymphatic intervention. In 16 of 18 patients, MRI or lymphangiogram or both demonstrated retrograde lymphatic flow from the thoracic duct toward lung parenchyma. Intranodal lymphangiogram and thoracic duct catheterization was successful in all patients. Seventeen of 18 patients underwent either lymphatic embolization procedures or thoracic duct stenting with covered stents to exclude retrograde flow into the lungs. One of the 2 patients who did not have retrograde lymphatic flow did not undergo a lymphatic interventional procedure. A total of 15 of 17(88%) patients who underwent an intervention had significant symptomatic improvement at a median follow-up of 315 days (range, 45-770 days). The most common complication observed was nonspecific transient abdominal pain and transient hypotension. Conclusions - In this study, we demonstrated abnormal pulmonary lymphatic perfusion in most patients with plastic bronchitis. Interruption of the lymphatic flow resulted in significant improvement of symptoms in these patients and, in some cases, at least temporary resolution of cast formation. © 2016 American Heart Association, Inc. AD - Division of Cardiology, Division of Pulmonary Medicine, United States Department of Radiology, Children's Hospital of PhiladelphiaPA, United States Division of Interventional Radiology, Hospital of the University of Pennsylvania, Philadelphia, United States AU - Dori, Y. AU - Keller, M. S. AU - Rome, J. J. AU - Gillespie, M. J. AU - Glatz, A. C. AU - Dodds, K. AU - Goldberg, D. J. AU - Goldfarb, S. AU - Rychik, J. AU - Itkin, M. DB - Scopus DO - 10.1161/CIRCULATIONAHA.115.019710 IS - 12 KW - bronchitis heart defects, congenital lymphatic vessels lymphography M3 - Article N1 - Cited By :84 Export Date: 15 June 2020 PY - 2016 SP - 1160-1170 ST - Percutaneous lymphatic embolization of abnormal pulmonary lymphatic flow as treatment of plastic bronchitis in patients with congenital heart disease T2 - Circulation TI - Percutaneous lymphatic embolization of abnormal pulmonary lymphatic flow as treatment of plastic bronchitis in patients with congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84957894686&doi=10.1161%2fCIRCULATIONAHA.115.019710&partnerID=40&md5=a2b53b2b59be24bc017cfb702587a671 VL - 133 ID - 1915 ER - TY - JOUR AB - Percutaneous transluminal pulmonary valvuloplasty may be indicated in not only isolated pulmonary valve stenosis, but also complex congenital heart diseases. Because palliative surgery for increasing pulmonary blood flow entails a risk of scar formation and immediate postoperative complications, catheter intervention is preferred, if possible. However, an acute-angled, twisted, or tortuous access route or a small valve orifice occasionally makes it difficult for the catheter to reach or cross the target. We succeeded in performing this intervention for such a complex stenosis effectively and safely in a patient with tricuspid atresia, ventricular septal defect (VSD), and severe pulmonary valve stenosis, thereby evading surgery. In previous reports, the catheter for this cardiac anomaly was accessed via the femoral vein. In the present case, the catheter was advanced through the femoral artery via the aorta, left ventricle, VSD, and right ventricle to the pulmonary valve, using a micro-catheter in a telescopic manner, in combination with a coronary balloon dilatation catheter. This maneuver, which has not been reported previously, made it much easier to perform the procedure as compared to the femoral vein approach, despite the acute turn and the pinhole orifice. Moreover, reported complications of the femoral vein approach, including bradycardia, hypotension, and valve regurgitation, were not observed in this case. We conclude that the femoral artery approach can be a safe and effective alternative in patients for whom a more conventional procedure has been unsuccessful. © 2013 Wiley Periodicals, Inc. Copyright © 2013 Wiley Periodicals, Inc. AD - T. Torigoe, Department of Pediatrics, Niigata City General Hospital, 463-7 Shumoku, Chuo-ku, Niigata 950-1197, Japan AU - Torigoe, T. AU - Sato, S. AU - Kanazawa, H. DB - Embase Medline DO - 10.1002/ccd.25193 IS - 5 KW - beraprost oxygen article balloon catheter Blalock Taussig shunt case report child clinical effectiveness computer assisted tomography coronary balloon dilatation catheter craniofacial synostosis disease severity femoral artery follow up guide wire heart right ventricle outflow tract heart ventricle septum defect home oxygen therapy human male mental deficiency microcatheter oxygen desaturation oxygen saturation patient safety percutaneous transluminal angioplasty balloon percutaneous transluminal pulmonary valvuloplasty pulmonary valve atresia pulmonary valve stenosis pulmonary valvuloplasty pulmonary valvuloplasty catheter school child sheath removal surgical approach systemic pulmonary shunt tricuspid valve atresia Carnelian Pixie ER Maverick XL Progreat Ryujin plus OTW Slalom Thrill Sprinter Legend RX LA - English M3 - Article N1 - L52826581 2013-10-24 2014-05-15 PY - 2014 SN - 1522-726X 1522-1946 SP - 774-777 ST - Percutaneous transluminal pulmonary valvuloplasty in a child with tricuspid atresia, ventricular septal defect, and severe pulmonary valve stenosis: Usefulness of the Femoral Artery Approach T2 - Catheterization and Cardiovascular Interventions TI - Percutaneous transluminal pulmonary valvuloplasty in a child with tricuspid atresia, ventricular septal defect, and severe pulmonary valve stenosis: Usefulness of the Femoral Artery Approach UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52826581 http://dx.doi.org/10.1002/ccd.25193 VL - 83 ID - 946 ER - TY - JOUR AB - OBJECTIVE: The anatomic and physiologic constraints for pediatric cavopulmonary assist differ markedly from adult Fontan circulations owing to smaller vessel sizes and risk of elevated pulmonary resistance. In this study, hemodynamic and hemolysis performance of a catheter-based viscous impeller pump (VIP) to power the Fontan circulation is assessed at a pediatric scale (∼15 kg) and performance range (0-30 mm Hg). METHODS: Computer simulation and mock circulation studies were conducted to assess the hydraulic performance, acute hemodynamic response to different levels VIP support, and the potential for vena caval collapse. Computational fluid dynamics simulations were used to estimate VIP hydraulic performance, shear rates, and potential for hemolysis. Hemolysis was quantified in a mock loop with fresh bovine blood. RESULTS: A VIP augmented 4-way total cavopulmonary connection flow at pediatric scales and restored systemic pressures and flows to biventricular values, without causing flow obstruction or suction. VIP generated flows up to 4.1 L/min and pressure heads of up to 38 mm Hg at 11,000 rpm. Maximal shear rate was 160 Pa, predicting low hemolysis risk. Observed hemolysis was low with plasma free hemoglobin of 11.4 mg · dL(-1) · h(-1). CONCLUSIONS: A VIP will augment Fontan cavopulmonary flow in the proper pressure and flow ranges, with low hemolysis risk under more stringent pediatric scale and physiology compared with adult scale. This technology may be developed to simultaneously reduce systemic venous pressure and improve cardiac output after stage 2 or 3 Fontan repair. It may serve to compress surgical staging, lessening the pathophysiologic burden of repair. AD - Department of Bioengineering, Cardiovascular Innovation Institute, University of Louisville, Louisville, KY, USA. AN - 22421403 AU - Giridharan, G. A. AU - Koenig, S. C. AU - Kennington, J. AU - Sobieski, M. A. AU - Chen, J. AU - Frankel, S. H. AU - Rodefeld, M. D. C2 - PMC3525746 C6 - NIHMS360281 DA - Jan DB - PubMed DO - 10.1016/j.jtcvs.2012.01.082 DP - NLM ET - 2012/03/17 IS - 1 KW - Animals Cattle Child, Preschool Computer Simulation Feasibility Studies Fontan Procedure/adverse effects/*instrumentation *Heart-Assist Devices/adverse effects *Hemodynamics Hemolysis Humans Hydrodynamics Materials Testing Models, Cardiovascular Prosthesis Design Stress, Mechanical Time Factors LA - eng N1 - 1097-685x Giridharan, Guruprasad A Koenig, Steven C Kennington, Jeffrey Sobieski, Michael A Chen, Jun Frankel, Steven H Rodefeld, Mark D R01 HL098353/HL/NHLBI NIH HHS/United States R01 HL098353-01A1/HL/NHLBI NIH HHS/United States R01HL098353/HL/NHLBI NIH HHS/United States Evaluation Study Journal Article Research Support, N.I.H., Extramural J Thorac Cardiovasc Surg. 2013 Jan;145(1):249-57. doi: 10.1016/j.jtcvs.2012.01.082. Epub 2012 Mar 14. PY - 2013 SN - 0022-5223 (Print) 0022-5223 SP - 249-57 ST - Performance evaluation of a pediatric viscous impeller pump for Fontan cavopulmonary assist T2 - J Thorac Cardiovasc Surg TI - Performance evaluation of a pediatric viscous impeller pump for Fontan cavopulmonary assist VL - 145 ID - 253 ER - TY - JOUR AB - Background The birth prevalence of congenital heart defects (CHDs) has decreased in Canada and Europe. Recommended intake of folic acid in pregnancy is a suggestive risk-reducing factor for CHDs. We investigated the association between periconceptional intake of folic acid supplements and infant risk of CHDs. Methods Information on maternal intake of folic acid supplements before and during pregnancy in the Medical Birth Registry of Norway 1999-2009 was updated with information on CHD diagnoses from national health registers and the Cardiovascular Diseases in Norway Project. The association between folic acid intake and infant risk of CHD was estimated as relative risk (RR) with binomial log linear regression. Results Among 517784 non-chromosomal singleton births, 6200 children were identified with CHD and 1153 with severe CHD. For all births, 18.4% of the mothers initiated folic acid supplements before pregnancy and 31.6% during pregnancy. The adjusted RR for severe CHD was 0.99 [95% confidence interval [CI] 0.86, 1.13] comparing periconceptional intake of folic acid with no intake. Specifically, RR for conotruncal defects was 0.99 [95% CI 0.80, 1.22], atrioventricular septal defects 1.19 [95% CI 0.78, 1.81], left ventricular outflow tract obstructions 1.02 [95% CI 0.78, 1.32], and right ventricular outflow tract obstructions 0.97 [95% CI 0.72, 1.29]. Birth prevalence of septal defects was higher in the group exposed to folic acid supplements with RR 1.19 [95% CI 1.10, 1.30]. Conclusions Periconceptional folic acid supplement use showed no association with severe CHDs in the newborn. An unexpected association with an increased risk of septal defects warrants further investigation. AD - E. Leirgul, Department of Global Public Health and Primary Care, University of Bergen, PO box 7804, Bergen, Norway AU - Leirgul, E. AU - Gildestad, T. AU - Nilsen, R. M. AU - Fomina, T. AU - Brodwall, K. AU - Greve, G. AU - Vollset, S. E. AU - Holmstrøm, H. AU - Tell, G. S. AU - Øyen, N. DB - Embase Medline DO - 10.1111/ppe.12212 IS - 5 KW - aortic coarctation aortic valve stenosis article atrioventricular septal defect Canada cardiovascular disease cause of death congenital heart malformation controlled study diet supplementation disease severity Ebstein anomaly Europe Fallot tetralogy folic acid supplementation great vessels transposition heart atrium septum defect heart left ventricle outflow tract obstruction heart right ventricle double outlet heart septum defect interventricular septum heart ventricle septum defect human hypoplastic left heart syndrome lung vein drainage anomaly major clinical study Norway patent ductus arteriosus phenotype prevalence protein synthesis pulmonary valve atresia pulmonary valve stenosis risk factor risk reduction LA - English M3 - Article N1 - L605351929 2015-07-30 2015-08-19 PY - 2015 SN - 1365-3016 0269-5022 SP - 391-400 ST - Periconceptional Folic Acid Supplementation and Infant Risk of Congenital Heart Defects in Norway 1999-2009 T2 - Paediatric and Perinatal Epidemiology TI - Periconceptional Folic Acid Supplementation and Infant Risk of Congenital Heart Defects in Norway 1999-2009 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L605351929 http://dx.doi.org/10.1111/ppe.12212 VL - 29 ID - 851 ER - TY - JOUR AB - Objective: Significant depression in cerebral oxygen saturation has been observed in patients with hypoplastic left heart syndrome (HLHS) undergoing Norwood operations. We monitored cerebral oxygen saturation with near-infrared spectroscopy before and after this procedure. Patients with transposition of great arteries (TGA) before and after arterial switch operation were also studied to elucidate whether post-cardiopulmonary bypass (CPB) changes in cerebral oxygen saturation are related to CPB or hemodynamic alterations inherent in single-ventricle physiology. Methods: We monitored 33 patients with HLHS and 20 with TGA 24 hours before and 48 hours after CPB. In addition to cerebral oxygen saturation, routine measurements of oxygen transport and delivery were performed. Results: Preoperatively, cerebral oxygen saturation was higher in patients with HLHS than with TGA (61% ± 7% vs 56% ± 8%, P = .04). After CPB, cerebral oxygen saturation was markedly depressed in both groups but increased toward end of monitoring (HLHS vs TGA minimal value 42% ± 12% vs 54% ± 11%, P < .001, value 48 hours after CPB 62% ± 7% vs 80% ± 8%, P < .0001). Routine measures of oxygen delivery, such as arterial and central venous oxygen saturations, were similar at minimal cerebral oxygen saturation and 48 hours after CPB. Conclusions: Depression of cerebral oxygen saturation is prevalent among neonates with congenital heart disease regardless of whether univentricular or biventricular circulation is present, suggesting that cerebral desaturation is mainly induced by CPB's effect on cerebral blood flow. Routine measures of oxygen delivery fail to indicate cerebral desaturation. Copyright © 2011 by The American Association for Thoracic Surgery. AD - H.-H. Kramer, Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital of Schleswig-Holstein, Campus Kiel, Arnold-Heller-Strasse 3, Haus 9, 24105 Kiel, Germany AU - Uebing, A. AU - Furck, A. K. AU - Hansen, J. H. AU - Nufer, E. AU - Scheewe, J. AU - Dütschke, P. AU - Jung, O. AU - Kramer, H. H. DB - Embase Medline DO - 10.1016/j.jtcvs.2011.01.036 IS - 3 KW - furosemide nitroprusside sodium phentolamine mesylate prostaglandin E1 adverse outcome arterial switch operation article brain oxygen tension cardiovascular surgery child clinical article female great vessels transposition hemodynamics human hypoplastic left heart syndrome low drug dose male near infrared spectroscopy Norwood procedure oxygen saturation preoperative evaluation priority journal school child tissue oxygenation LA - English M3 - Article N1 - L51338588 2011-03-30 2011-08-26 PY - 2011 SN - 0022-5223 1097-685X SP - 523-530 ST - Perioperative cerebral and somatic oxygenation in neonates with hypoplastic left heart syndrome or transposition of the great arteries T2 - Journal of Thoracic and Cardiovascular Surgery TI - Perioperative cerebral and somatic oxygenation in neonates with hypoplastic left heart syndrome or transposition of the great arteries UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51338588 http://dx.doi.org/10.1016/j.jtcvs.2011.01.036 VL - 142 ID - 1093 ER - TY - JOUR AB - OBJECTIVES: Neonates with hypoplastic left heart syndrome have significant hemodynamic threats to cerebral perfusion and are at risk of reduced neurodevelopmental performance. We hypothesized that cerebral hypoxia, detectable by near-infrared spectroscopy in the early postoperative period, would be related to later neurodevelopmental performance. METHODS: The study population was a sequential cohort of patients who had undergone stage 1 palliation of hypoplastic left heart syndrome under standard conditions, including neonatal perioperative monitoring with cerebral near-infrared spectroscopy, and who had undergone a neurodevelopmental assessment at age 4 to 5 years. The neonatal demographic and 48-hour perioperative hemodynamic parameters, including cerebral oxygen saturation, were tested for their relationship to 4 domains of neurodevelopmental performance, including visual-motor integration in childhood in univariate and multivariate models. The neurodevelopmental scores were classified as low if less than 85 (-1 standard deviation) and abnormal if less than 70 (-2 standard deviations). RESULTS: For the 51 patients in the surgical cohort, the early survival was 94%, the cumulative survival was 86%, and the neurodevelopmental assessment was completed by 21 (48%) of the survivors, without evidence of an ascertainment bias. At the test age of 56.3 ± 5.5 months, the composite neurodevelopmental index, constructed from equally weighted measures in 4 domains, was 97.6 ± 9.6, not different from the age-based norms, with 3 of 21 in the low range and none abnormal. The mean visual-motor integration was 93.4 ± 14, slightly less than the population norm (P < .05), with 2 of 21 having low scores and 1 abnormal scores. In patients with low to abnormal visual-motor integration, the perioperative stage 1 palliation cerebral oxygenation saturation was significantly lower (63.6 ± 8.1 vs 67.8 ± 8.1, P < .05). Two patients had discrete embolic strokes after their initial hospitalization; the occurrence of late stroke reduced the visual-motor integration performance but was not related to the early cerebral oxygen saturation. Nonlinear relationships of cerebral oxygen saturation to the neurodevelopmental measures found cerebral oxygen saturation thresholds of 49% to 62%. The hours at a cerebral oxygen saturation less than 45% and 55% were related to low visual-motor integration and neurodevelopmental index scores in the univariate and multivariate models. A multivariate model of age and weight at stage 1 palliation, cerebral oxygen saturation, arterial oxygen saturation, cardiopulmonary bypass and deep hypothermic circulatory arrest times, and later stroke predicted visual-motor integration to an important degree (R(2) = 0.53, P < .001). The actual and predicted visual-motor integration and neurodevelopmental index were normal when a cerebral oxygen saturation less than 45% and other risk conditions were avoided. CONCLUSIONS: Neurodevelopmental performance was related to demographic, neonatal perioperative physiologic, and later factors. Perioperative cerebral oxygenation assessed by near-infrared spectroscopy can detect hypoxic-ischemic conditions associated with injury and reduced neurodevelopmental performance and was the most significant physiologic factor identified. These data suggest that efforts to avoid cerebral hypoxia are likely to improve the outcomes in this high-risk population. AD - Children's Hospital of Wisconsin, Milwaukee, Wis; Medical College of Wisconsin, Milwaukee, Wis. Electronic address: ghoffman@mcw.edu. AN - 23317941 AU - Hoffman, G. M. AU - Brosig, C. L. AU - Mussatto, K. A. AU - Tweddell, J. S. AU - Ghanayem, N. S. DA - Nov DB - PubMed DO - 10.1016/j.jtcvs.2012.12.060 DP - NLM ET - 2013/01/16 IS - 5 KW - Age Factors Attention *Cardiac Surgical Procedures/adverse effects *Cerebrovascular Circulation Chi-Square Distribution Child *Child Development Child Language Child, Preschool Cognition Female *Hemodynamics Humans Hypoplastic Left Heart Syndrome/blood/diagnosis/physiopathology/*surgery Hypoxia-Ischemia, Brain/blood/diagnosis/*etiology/physiopathology/psychology Infant, Newborn Linear Models Male Multivariate Analysis Nervous System/*growth & development Neuropsychological Tests Nonlinear Dynamics Oxygen/*blood Predictive Value of Tests Psychomotor Performance Risk Factors Spectroscopy, Near-Infrared Time Factors Treatment Outcome 19 21 41 Cpb Cvp Dhca Mri Nd Ndi Nirs S1p Sd SaO(2) SvO(2) Vmi arterial oxygen saturation cardiopulmonary bypass central venous pressure deep hypothermic circulatory arrest magnetic resonance imaging near-infrared spectroscopy neurodevelopmental neurodevelopmental index rSO(2)C regional cerebral oxygen saturation stage 1 palliation standard deviation venous oxygen saturation visual-motor integration LA - eng N1 - 1097-685x Hoffman, George M Brosig, Cheryl L Mussatto, Kathleen A Tweddell, James S Ghanayem, Nancy S Journal Article Research Support, Non-U.S. Gov't United States J Thorac Cardiovasc Surg. 2013 Nov;146(5):1153-64. doi: 10.1016/j.jtcvs.2012.12.060. Epub 2013 Jan 12. PY - 2013 SN - 0022-5223 SP - 1153-64 ST - Perioperative cerebral oxygen saturation in neonates with hypoplastic left heart syndrome and childhood neurodevelopmental outcome T2 - J Thorac Cardiovasc Surg TI - Perioperative cerebral oxygen saturation in neonates with hypoplastic left heart syndrome and childhood neurodevelopmental outcome VL - 146 ID - 296 ER - TY - JOUR AB - Optimizing oxygen delivery to the brain is one of the main goals in children with congenital heart defects after surgery. It has been shown that cerebral oxygen saturation (cSO2) is depressed within the first day after neonatal cardiopulmonary bypass surgery. However, peri-operative cerebral oxygen metabolism has not yet been assessed in previous studies. The aim of this study was to describe the peri-operative changes in cerebral oxygen metabolism in neonates with congenital heart defects following cardiopulmonary bypass surgery. Prospective observational cohort study. PICU of a tertiary referral center. Fourteen neonates with hypoplastic left heart syndrome (HLHS) undergoing Norwood procedure and 14 neonates with transposition of great arteries (TGA) undergoing arterial switch operation (ASO) were enrolled. Pediatric heart surgery. We measured non-invasively regional cSO2 and microperfusion (rcFlow) using tissue spectrometry and laser Doppler flowmetry before and after surgery. Cerebral fractional tissue oxygen extraction (cFTOE), the arterio-cerebral difference in oxygen content (acDO2) and approximated cerebral metabolic rate of oxygen (aCMRO2) were calculated. According to the postsurgical hemodynamics, arterial saturation (aSO2) normalized immediately after surgery in the TGA group, whereas HLHS patients still were cyanotic. cSO2 significantly increased in TGA group over 48 h after ASO (p = 0.004) and was significantly higher compared to HLHS group after Norwood procedure. cFTOE as a risk marker for brain injury was elevated before surgery (TGA group 0.37 ± 0.10, HLHS group 0.42 ± 0.12) and showed a slight decrease after ASO (p = 0.35) but significantly decreased in patients after Norwood procedure (p = 0.02). Preo-peratively, acDO2 was significantly higher in patients with HLHS compared to patients with TGA (7.7 ± 2.5 vs. 5.2 ± 1.6 ml/dl, p = 0.005), but normalized in the posto-perative course. Before surgery, the aCMRO2 was slightly higher in the HLHS group (5.1 ± 1.5 vs. 3.9 ± 2.5 AU, p = 0.14), but significantly decreased after Norwood procedure (- 1.6 AU, p = 0.009). There was no difference in rcFlow between both groups and between the points in time prior and after surgery. Neonates undergoing cardiac surgery suffer from peri-operative changes in hemodynamics and cerebral hypoxemic stress. The cerebral oxygen metabolism seems to be more affected in cyanotic children with functionally univentricular hearts compared to post-operative acyanotic patients. Additional stress factors must be avoided to achieve the best possible neurological outcome. AD - Department of Pediatric Cardiology, Pulmology and Pediatric Intensive Care Medicine, University Children's Hospital Tübingen, Hoppe-Seyler-Str. 1, 72076, Tuebingen, Germany. felix.neunhoeffer@med.uni-tuebingen.de. Department of Pediatric Cardiology, Pulmology and Pediatric Intensive Care Medicine, University Children's Hospital Tübingen, Hoppe-Seyler-Str. 1, 72076, Tuebingen, Germany. Department of Thoracic and Cardiovascular Surgery, University Hospital Tübingen, Hoppe-Seyler-Str. 1, 72076, Tuebingen, Germany. Department of Pediatric Neurosurgery, University Hospital Tübingen, Hoppe-Seyler-Str. 1, 72076, Tuebingen, Germany. AN - 30167749 AU - Neunhoeffer, F. AU - Hofbeck, M. AU - Schlensak, C. AU - Schuhmann, M. U. AU - Michel, J. DA - Dec DB - PubMed DO - 10.1007/s00246-018-1952-2 DP - NLM ET - 2018/09/01 IS - 8 KW - Arterial Switch Operation/adverse effects/methods Brain/blood supply Cardiopulmonary Bypass/adverse effects/methods Cerebrovascular Circulation/*physiology Child Cohort Studies Female Humans Hypoplastic Left Heart Syndrome/*physiopathology/surgery Infant Infant, Newborn Laser-Doppler Flowmetry Male Norwood Procedures/adverse effects/methods Oxygen/blood/*metabolism Prospective Studies Spectrum Analysis Transposition of Great Vessels/*physiopathology/surgery Treatment Outcome Arterial switch operation Cerebral autoregulation Congenital heart defect Norwood LA - eng N1 - 1432-1971 Neunhoeffer, Felix Hofbeck, Michael Schlensak, Christian Schuhmann, Martin Ulrich Michel, Jörg Journal Article Observational Study United States Pediatr Cardiol. 2018 Dec;39(8):1681-1687. doi: 10.1007/s00246-018-1952-2. Epub 2018 Aug 25. PY - 2018 SN - 0172-0643 SP - 1681-1687 ST - Perioperative Cerebral Oxygenation Metabolism in Neonates with Hypoplastic Left Heart Syndrome or Transposition of the Great Arteries T2 - Pediatr Cardiol TI - Perioperative Cerebral Oxygenation Metabolism in Neonates with Hypoplastic Left Heart Syndrome or Transposition of the Great Arteries VL - 39 ID - 22 ER - TY - JOUR AB - Plastic bronchitis is potentially a life-threatening complication of long-standing surgically palliated single ventricle congenital heart disease. Patients can present with hypoxia requiring urgent bronchoscopy for removal of bronchial casts. Perioperative care for these patients is challenging and anesthesia is associated with significant cardiac risk. As more surgically corrected single ventricle patients survive to adulthood, these patients are expected to present more frequently. This report details the perioperative management of 2 Fontan patients with hypoxia and significant plastic bronchitis disease burden. © The Author(s) 2013. AD - R.J. Ing, Department of Anesthesiology, 13123 East 16th Avenue, Box B090, Aurora, CO 80045, United States AU - Singhal, N. R. AU - Da Cruz, E. M. AU - Nicolarsen, J. AU - Schwartz, L. I. AU - Merritt, G. R. AU - Barrett, C. AU - Twite, M. D. AU - Ing, R. J. DB - Embase Medline DO - 10.1177/1089253213475879 IS - 1 KW - epinephrine alteplase antibiotic agent bronchodilating agent ceftriaxone dopamine enalapril fentanyl heparin hydrocortisone sodium succinate hypertensive agent ketamine methylprednisolone midazolam noradrenalin propofol sevoflurane sildenafil vasopressin agitated depression airway obstruction article artificial ventilation bronchitis bronchoscopy case report child coughing croupous bronchitis disease association drug dose titration echocardiography electrocardiography endotracheal intubation extracorporeal oxygenation female fever first degree atrioventricular block Fontan procedure hematothorax histopathology human hypoplastic left heart syndrome hypotension hypoxemia hypoxia intensive care unit laryngeal mask male oxygen therapy perioperative period physical examination physiotherapy pleura effusion pulse oximetry resuscitation school child septal occluder shock surgical patient tachypnea thorax radiography tracheobronchitis tricuspid valve atresia upper respiratory tract infection LMA LA - English M3 - Article N1 - L368425866 2013-03-08 2013-03-12 PY - 2013 SN - 1089-2532 1940-5596 SP - 55-60 ST - Perioperative management of shock in two fontan patients with plastic bronchitis T2 - Seminars in Cardiothoracic and Vascular Anesthesia TI - Perioperative management of shock in two fontan patients with plastic bronchitis UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L368425866 http://dx.doi.org/10.1177/1089253213475879 VL - 17 ID - 1011 ER - TY - JOUR AB - Objectives: To evaluate the value of perioperative cerebral near-infrared spectroscopy monitoring using variability analysis in the prediction of neurodevelopmental outcomes in neonates undergoing surgery for congenital heart disease. Design: Retrospective cohort study. Setting: Urban, academic, tertiary-care children's hospital. Patients: Neonates undergoing surgery with cardiopulmonary bypass for congenital heart disease. Interventions: Perioperative monitoring of continuous cerebral tissue oxygenation index by near-infrared spectroscopy and subsequent neurodevelopmental testing at 6, 15, and 21 months of age. Measurements and Main Results: We developed a new measure, cerebral tissue oxygenation index variability, using the root mean of successive squared differences of averaged 1-minute cerebral tissue oxygenation index values for both the intraoperative and first 24-hours postoperative phases of monitoring. There were 62 neonates who underwent cerebral tissue oxygenation index monitoring during surgery for congenital heart disease and 44 underwent subsequent neurodevelopmental testing (12 did not survive until testing and six were lost to follow-up). Among the 44 monitored patients who underwent neurodevelopmental testing, 20 (45%) had abnormal neurodevelopmental indices. Patients with abnormal neurodevelopmental indices had lower postoperative cerebral tissue oxygenation index variability when compared with patients with normal indices (p = 0.01). Adjusting for class of congenital heart disease and duration of deep hypothermic circulatory arrest, lower postoperative cerebral tissue oxygenation index variability was associated with poor neurodevelopmental outcome (p = 0.02). Conclusions: We found reduced postoperative cerebral tissue oxygenation index variability in neonatal survivors of congenital heart disease surgery with poor neurodevelopmental outcomes. We hypothesize that reduced cerebral tissue oxygenation index variability may be a surrogate for impaired cerebral metabolic autoregulation in the immediate postoperative period. Further research is needed to investigate clinical implications of this finding and opportunities for using this measure to drive therapeutic interventions. AD - M.C. Spaeder, Division of Pediatric Critical Care, University of Virginia School of Medicine, Charlottesville, VA, United States AU - Spaeder, M. C. AU - Klugman, D. AU - Skurow-Todd, K. AU - Glass, P. AU - Jonas, R. A. AU - Donofrio, M. T. DB - Embase Medline DO - 10.1097/PCC.0000000000001056 IS - 3 KW - aortic arch anomaly aortic coarctation aortic occlusion article brain tissue cardiopulmonary bypass cerebral tissue oxygenation index variability clinical outcome cohort analysis comparative study congenital heart disease controlled study deep hypothermic circulatory arrest evaluation study Fallot tetralogy heart right ventricle double outlet heart single ventricle heart surgery heart ventricle septum defect human hypoplastic aortic arch hypoplastic left heart syndrome lung vein drainage anomaly major clinical study mental disease near infrared spectroscopy near infrared spectroscopy probe nerve cell differentiation nervous system parameters neurological general equipment neuromonitoring newborn newborn monitoring newborn surgery perioperative monitoring postoperative period priority journal retrospective study surgical patient survival tissue oxygenation NIRO-200 LA - English M3 - Article N1 - L614021994 2017-01-13 2017-03-16 PY - 2017 SN - 1947-3893 1529-7535 SP - 213-218 ST - Perioperative Near-Infrared Spectroscopy Monitoring in Neonates with Congenital Heart Disease: Relationship of Cerebral Tissue Oxygenation Index Variability with Neurodevelopmental Outcome T2 - Pediatric Critical Care Medicine TI - Perioperative Near-Infrared Spectroscopy Monitoring in Neonates with Congenital Heart Disease: Relationship of Cerebral Tissue Oxygenation Index Variability with Neurodevelopmental Outcome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L614021994 http://dx.doi.org/10.1097/PCC.0000000000001056 VL - 18 ID - 767 ER - TY - JOUR AB - Cardiopulmonary bypass (CPB) produces inflammation and oxidative stress, which contribute to postoperative complications after cardiac surgery. F 2-Isoprostanes (F2-IsoPs) are products of lipid oxidative injury and represent the most accurate markers of oxidative stress. In adults undergoing cardiac surgery, CPB is associated with elevated IsoPs. The relationship between F2-IsoPs and perioperative end-organ function in infants with singleventricle physiology, however, has not been well studied. This study prospectively enrolled 20 infants (ages 3-12 months) with univentricular physiology undergoing elective stage 2 palliation (bidirectional cavopulmonary anastomosis). Blood samples were collected before the surgical incision (T0), 30 min after initiation of CPB (T1), immediately after separation from CPB (T2), and 24 h postoperatively (T3). Plasma F2-IsoP levels were measured at each time point and correlated with indices of pulmonary function and other relevant clinical variables. Plasma F2-IsoPs increased significantly during surgery, with highest levels seen immediately after separation from CPB (p<0.001). After separation from CPB, increased F2-IsoP was associated with lower arterial pH (ρ = -0.564; p = 0.012), higher partial pressure of carbon dioxide (PaCO2; ρ = 0.633; p = 0.004), and decreased lung compliance (ρ = -0.783; p ≤ 0.001). After CPB, F2-IsoPs did not correlate with duration of CPB, arterial lactate, or immediate postoperative outcomes. In infants with singleventricle physiology, CPB produces oxidative stress, as quantified by elevated F 2-IsoP levels. Increased F2-IsoP levels correlated with impaired ventilation in the postoperative period. The extent to which F 2-IsoPs and other bioactive products of lipid oxidative injury might predict or contribute to organ-specific stress warrants further investigation. © Springer Science+Business Media, LLC 2012. AD - E. Albers, Division of Pediatric Cardiology, Vanderbilt University Medical Center, 2200 Children's Way, Nashville, TN 37232-6602, United States AU - Albers, E. AU - Donahue, B. S. AU - Milne, G. AU - Saville, B. R. AU - Wang, W. AU - Bichell, D. AU - McLaughlin, B. DB - Embase Medline DO - 10.1007/s00246-012-0166-2 IS - 4 KW - biological marker carbon dioxide isoprostane derivative article bidirectional cavopulmonary anastomosis blood sampling cardiopulmonary bypass cavopulmonary connection clinical article concentration (parameter) disease marker female heart single ventricle human infant lung compliance lung function lung injury lung perfusion male outcome assessment oxidative stress palliative therapy perioperative period pulmonary dysfunction reoperation LA - English M3 - Article N1 - L51859466 2012-02-21 2012-07-18 PY - 2012 SN - 0172-0643 1432-1971 SP - 562-568 ST - Perioperative plasma F2-isoprostane levels correlate with markers of impaired ventilation in infants with single-ventricle physiology undergoing stage 2 surgical palliation on the cardiopulmonary bypass T2 - Pediatric Cardiology TI - Perioperative plasma F2-isoprostane levels correlate with markers of impaired ventilation in infants with single-ventricle physiology undergoing stage 2 surgical palliation on the cardiopulmonary bypass UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51859466 http://dx.doi.org/10.1007/s00246-012-0166-2 VL - 33 ID - 1066 ER - TY - JOUR AB - OBJECTIVE: Historical cohort studies have reported adverse neurodevelopment following cardiac surgery during early infancy. Advances in surgical techniques and perioperative care have coincided with updating of neurodevelopmental assessment tools. We aimed to determine perioperative risk factors for impaired neurodevelopment at 2 years following surgery for congenital heart disease (CHD) in early infancy. DESIGN AND PATIENTS: We undertook a prospective longitudinal study of 153 full-term infants undergoing surgery for CHD before 2 months of age. Infants were excluded if they had a genetic syndrome associated with neurodevelopmental impairment. OUTCOME MEASURES: Predefined perioperative parameters were recorded and infants were classified according to cardiac anatomy. At 2 years, survivors were assessed using the Bayley Scales of Infant Development-III. RESULTS: At 2 years, 130 children (98% of survivors) were assessed. Mean cognitive, language and motor scores were 93.4±13.6, 93.6±16.1 and 96.8±12.5 respectively (100±15 norm). Twenty (13%) died and 12 (9%) survivors had severe impairment (score <70), mostly language (8%). The lowest scores were in infants born with single ventricle physiology with obstruction to the pulmonary circulation who required a neonatal systemic-to-pulmonary artery shunt. Additional risk factors for impairment included reduced gestational age, postoperative elevation of lactate or S100B and repeat cardiac surgery. CONCLUSIONS: In the modern era of infant cardiac surgery and perioperative care, children continue to demonstrate neurodevelopmental delays. The use of updated assessment tools has revealed early language dysfunction and relative sparing of motor function. Ongoing follow-up is critical in this high-risk population. AD - Newborn Intensive Care, The Royal Children's Hospital, Melbourne, Australia. Neonatal Research Group, Murdoch Children's Research Institute, Melbourne, Australia. Department of Paediatrics, The University of Melbourne, Australia. Department of Paediatric Intensive Care, Starship Children's Hospital, Auckland, New Zealand. Department of Pediatric Critical Care, Texas Children's Hospital, Houston, Texas, USA. Department of Cardiac Surgery, The Royal Children's Hospital, Melbourne, Australia. Department of Cardiac Surgery, Starship Children's Hospital, Auckland, New Zealand. Clinical Epidemiology and Biostatistics Unit, Murdoch Children's Research Institute, Melbourne, Australia. AN - 27272973 AU - Gunn, J. K. AU - Beca, J. AU - Hunt, R. W. AU - Goldsworthy, M. AU - Brizard, C. P. AU - Finucane, K. AU - Donath, S. AU - Shekerdemian, L. S. DA - Nov DB - PubMed DO - 10.1136/archdischild-2015-309449 DP - NLM ET - 2016/06/09 IS - 11 KW - Cardiac Surgical Procedures/*adverse effects/mortality Child, Preschool Cognition Disorders/etiology/mortality Developmental Disabilities/*etiology/mortality Female Heart Defects, Congenital/mortality/*surgery Humans Infant Infant, Newborn Intraoperative Care Longitudinal Studies Male Postoperative Complications/etiology/mortality Prospective Studies Psychomotor Disorders/etiology/mortality Risk Factors *Cardiac Surgery *Intensive Care *Neonatology *Neurodevelopment *Outcomes research LA - eng N1 - 1468-2044 Gunn, Julia K Beca, John Hunt, Rodney W Goldsworthy, Michelle Brizard, Christian P Finucane, Kirsten Donath, Susan Shekerdemian, Lara S Journal Article Research Support, Non-U.S. Gov't England Arch Dis Child. 2016 Nov;101(11):1010-1016. doi: 10.1136/archdischild-2015-309449. Epub 2016 Jun 6. PY - 2016 SN - 0003-9888 SP - 1010-1016 ST - Perioperative risk factors for impaired neurodevelopment after cardiac surgery in early infancy T2 - Arch Dis Child TI - Perioperative risk factors for impaired neurodevelopment after cardiac surgery in early infancy VL - 101 ID - 424 ER - TY - JOUR AB - Objective: Genetic aetiology remains unknown in up to 50% of patients with persistent hyperinsulinaemic hypoglycaemia (HH). Several syndromes are associated with HH. We report Rubinstein-Taybi syndrome (RSTS) as one of the possible causes of persistent HH. Early diagnosis and treatment of HH is crucial to prevent hypoglycaemic brain injury. Design: Four RSTS patients with HH were retrospectively analysed. Methods: Genetic investigations included next-generation sequencing-based gene panels and exome sequencing. Clinical characteristics, metabolic profile during hypoglycaemia and treatment were reviewed. Results: Disease-related EP300 or CREBBP variants were found in all patients, no pathogenic variants were found in a panel of genes associated with non-syndromic HH. Two patients had classic manifestations of RSTS, three had choanal atresia or stenosis. Diagnosis of HH varied from 1 day to 18 months of age. One patient was unresponsive to treatment with diazoxide, octreotide and nifedipine, but responded to sirolimus. All required gastrostomy feeding. Conclusions: Given the rarity of RSTS (1:125 000) and HH (1:50 000), our ob servations indicate an association between these two conditions. We therefore recommend that clinicians should be vigilant in screening for HH in symptomatic infants with RSTS. In children with an apparent syndromic form of HH, RSTS should be considered in the differential diagnosis. AD - S. Kummer, Department of General Paediatrics, Neonatology and Paediatric Cardiology, Medical Faculty, University Children's Hospital Düsseldorf, Düsseldorf, Germany AU - Welters, A. AU - El-Khairi, R. AU - Dastamani, A. AU - Bachmann, N. AU - Bergmann, C. AU - Gilbert, C. AU - Clement, E. AU - Hurst, J. A. AU - Quercia, N. AU - Wasserman, J. D. AU - Meissner, T. AU - Shah, P. AU - Kummer, S. DB - Embase Medline DO - 10.1530/EJE-19-0119 IS - 2 KW - stomach tube 6 fluorodopa f 18 acarbose barium C peptide chlorothiazide crebbp protein diazoxide E1A associated p300 protein fatty acid glucose infusion fluid insulin ketone nifedipine octreotide protein rapamycin unclassified drug article bradycardia case report cataract choana atresia cleft palate clinical article clinical feature cyanosis developmental delay disease course disease severity drug withdrawal dysplasia electromyography face dysmorphia fatty acid blood level feeding difficulty female fluoroscopy Fontan procedure gastroesophageal reflux gastrostomy genetic analysis genetic association genetic screening genetic variation gestational age glucose blood level glycemic control heart atrium septum defect heterozygote high calorie diet human hypertrichosis hypervolemia insulin blood level insulin release intermittent strabismus laryngomalacia long philtrum low drug dose male multicystic dysplastic kidney nerve conduction nervousness newborn high throughput sequencing nuclear magnetic resonance imaging oral glucose tolerance test patent ductus arteriosus pericardial effusion persistent hyperinsulinemic hypoglycemia of infancy positron emission tomography-computed tomography priority journal protein intolerance ptosis (eyelid) retrospective study Rubinstein syndrome sepsis somnolence speech delay stomach fundoplication tachycardia teratoma tonic clonic seizure treatment response tricuspid valve atresia vomiting whole exome sequencing LA - English M3 - Article N1 - L2002250121 2019-07-17 2019-07-19 PY - 2019 SN - 1479-683X 0804-4643 SP - 121-128 ST - Persistent hyperinsulinaemic hypoglycaemia in children with Rubinstein-Taybi syndrome T2 - European Journal of Endocrinology TI - Persistent hyperinsulinaemic hypoglycaemia in children with Rubinstein-Taybi syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2002250121 http://dx.doi.org/10.1530/EJE-19-0119 VL - 181 ID - 635 ER - TY - JOUR AD - U. Peleg, Dept. Otolaryngol./Head/Neck Surgery, Shaare Zedek Medical Center, P.O. Box 3235, Jerusalem 91031, Israel AU - Peleg, U. AU - Schwartz, S. AU - Sirota, G. AU - Hochman, I. AU - Cohen, D. AU - Picard, E. C1 - coumadin DB - Embase Medline IS - 2 KW - acetylcysteine antibiotic agent azithromycin bronchodilating agent deoxyribonuclease diuretic agent mucolytic agent oxygen steroid tissue plasminogen activator warfarin article atelectasis Blalock Taussig shunt bronchitis bronchoscopy cardiopulmonary arrest case report childhood disease chylothorax coughing cyanosis cyanotic heart disease death diaphragm paralysis dyspnea female Fontan procedure heart catheterization heart single ventricle heart surgery human human cell hypoxia physiotherapy pneumonia preschool child psychomotor retardation respiratory distress respiratory failure surgical technique thorax radiography tricuspid valve atresia coumadin LA - English M3 - Article N1 - L40260075 2005-03-07 PY - 2005 SN - 1565-1088 SP - 122-124 ST - Persistent plastic bronchitis in a child after cardiac surgery T2 - Israel Medical Association Journal TI - Persistent plastic bronchitis in a child after cardiac surgery UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L40260075 VL - 7 ID - 1281 ER - TY - JOUR AB - Although most arrhythmias in children have a benign outcome, recurrent arrhythmic events have a significant impact on quality of life. Electrophysiology studies with ablation have good short-term success and an acceptably low complication rate. The long-term outlook for this patient group is incompletely defined, however. Factors that require special consideration in children include the need to limit the size, depth and number of lesions, and the radiation exposure incurred during fluoroscopy-guided catheters manipulation. The use of cryoablation seems promising in limiting lesion size. Three-dimensional (3D) mapping systems clearly have been shown to reduce the radiation dose. In congenital heart disease, these advances in technology have helped to further our understanding of the mechanisms underlying arrhythmias. It is anticipated that earlier intervention and newer operative techniques will reduce the incidence of postoperative arrhythmias in the future. For those patients who still develop tachyarrythmias after cardiac surgery, the use of three-dimensional systems and other new technologies permits more efficient intervention in the electrophysiology laboratory. AD - A. Pflaumer, Royal Children’s Hospital Melbourne, 50 Flemington Road, Parkville, Melbourne, VIC, Australia AU - Pflaumer, A. DB - Embase Medline DO - 10.1111/j.1445-5994.2012.02899.x KW - article atrial switch operation atrioventricular nodal reentry tachycardia catheter ablation child computer assisted tomography congenital heart disease coronary artery obstruction cryoablation electrophysiology fluoroscopy Fontan procedure guiding catheter heart arrhythmia heart atrium flutter heart surgery human invasive procedure nuclear magnetic resonance imaging paroxysmal supraventricular tachycardia postoperative complication priority journal radiation dose radiation exposure supraventricular tachycardia surgical technique tachycardia induced cardiomyopathy three-dimensional imaging LA - English M3 - Article N1 - L617301051 2017-07-21 2017-07-26 PY - 2012 SN - 1445-5994 1444-0903 SP - 70-76 ST - Perspectives in ablation of arrhythmias in children and patients with congenital heart disease T2 - Internal Medicine Journal TI - Perspectives in ablation of arrhythmias in children and patients with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L617301051 http://dx.doi.org/10.1111/j.1445-5994.2012.02899.x VL - 42 ID - 1074 ER - TY - JOUR AB - In children with structurally normal hearts, the mechanisms of arrhythmias are usually the same as in the adult patient. Some arrhythmias are particularly associated with young age and very rarely seen in adult patients. Arrhythmias in structural heart disease may be associated either with the underlying abnormality or result from surgical intervention. Chronic haemodynamic stress of congenital heart disease (CHD) might create an electrophysiological and anatomic substrate highly favourable for re-entrant arrhythmias.As a general rule, prescription of antiarrhythmic drugs requires a clear diagnosis with electrocardiographic documentation of a given arrhythmia. Risk-benefit analysis of drug therapy should be considered when facing an arrhythmia in a child. Prophylactic antiarrhythmic drug therapy is given only to protect the child from recurrent supraventricular tachycardia during this time span until the disease will eventually cease spontaneously. In the last decades, radiofrequency catheter ablation is progressively used as curative therapy for tachyarrhythmias in children and patients with or without CHD. Even in young children, procedures can be performed with high success rates and low complication rates as shown by several retrospective and prospective paediatric multi-centre studies. Three-dimensional mapping and non-fluoroscopic navigation techniques and enhanced catheter technology have further improved safety and efficacy even in CHD patients with complex arrhythmias.During last decades, cardiac devices (pacemakers and implantable cardiac defibrillator) have developed rapidly. The pacing generator size has diminished and the pacing leads have become progressively thinner. These developments have made application of cardiac pacing in children easier although no dedicated paediatric pacing systems exist. © 2013 The Author. AD - J. Brugada, Cardiology Department, Hospital Sant Joan de Déu-Hospital Clínic, University of Barcelona, 08036 Barcelona, Spain AU - Brugada, J. AU - Blom, N. AU - Sarquella-Brugada, G. AU - Blomstrom-Lundqvist, C. AU - Deanfield, J. AU - Janousek, J. AU - Abrams, D. AU - Bauersfeld, U. AU - Brugada, R. AU - Drago, F. AU - De Groot, N. AU - Happonen, J. M. AU - Hebe, J. AU - Yen Ho, S. AU - Marijon, E. AU - Paul, T. AU - Pfammatter, J. P. AU - Rosenthal, E. DB - Embase Medline DO - 10.1093/europace/eut082 IS - 9 KW - acebutolol adenosine amiodarone antiarrhythmic agent atenolol azimilide beta adrenergic receptor blocking agent bisoprolol digitalis digoxin diltiazem dofetilide esmolol flecainide ibutilide lidocaine magnesium sulfate pindolol procainaimide propafenone propranolol sotalol unclassified drug verapamil vernakalant antiarrhythmic activity article atrioventricular block atrioventricular conduction atrioventricular nodal reentry tachycardia atrioventricular reentry tachycardia av nodal conduction block bidirectional ventricular tachycardia blurred vision bradycardia Brugada syndrome cardiac resynchronization therapy cardioversion catecholaminergic polymorphic ventricular tachycardia catheter ablation channelopathy child care childhood mortality clinical effectiveness congenital heart block congenital heart disease congenital heart malformation consensus coronary artery cryoablation deep sedation dose response drug dose reduction drug excretion drug half life drug withdrawal Ebstein anomaly ectopic atrial tachycardia electrical equipment epicardial pacemaker evidence based practice exercise eye toxicity Fallot tetralogy fascicular ventricular tachycardia fetus heart arrhythmia follow up Fontan procedure genetics great vessels transposition heart accessory conduction pathway heart arrhythmia atrial fibrillation heart atrium flutter heart atrium septum defect heart electrophysiology heart failure heart muscle conduction disturbance heart muscle conduction system heart pacing heart right ventricle dysplasia heart supraventricular arrhythmia heart ventricle arrhythmia heart ventricle tachycardia human hyperactivity hypertrophic cardiomyopathy hypotension implantable cardioverter defibrillator irritability junctional ectopic tachycardia late postoperative arrhythmia lifestyle liver toxicity long QT syndrome lung toxicity mental disease narrow QRS tachycardia neurotoxicity nonhuman pathophysiology permanent junctional reciprocating tachycardia practice guideline prevalence primary prevention priority journal psychological aspect QRS complex QRS tachycardia QT interval QT prolongation radiation exposure radiofrequency ablation reentry tachycardia safety secondary prevention sedation short QT syndrome side effect sinus node skin toxicity supraventricular tachycardia systematic review tachycardia thromboembolism thyroid disease torsade des pointes treatment indication Valsalva maneuver ventricular outflow tract ventricular tachycardia wide qrs tachycardia Wolff Parkinson White syndrome LA - English M3 - Article N1 - L369729978 2013-09-11 2013-09-18 PY - 2013 SN - 1099-5129 1532-2092 SP - 1337-1382 ST - Pharmacological and non-pharmacological therapy for arrhythmias in the pediatric population: EHRA and AEPC-Arrhythmia Working Group joint consensus statement T2 - Europace TI - Pharmacological and non-pharmacological therapy for arrhythmias in the pediatric population: EHRA and AEPC-Arrhythmia Working Group joint consensus statement UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L369729978 http://dx.doi.org/10.1093/europace/eut082 VL - 15 ID - 990 ER - TY - JOUR AB - Pediatric pharmacotherapy is often challenging due to the paucity of available clinical data on the safety and efficacy of drugs that are commonly used in children This quandary is even more prevalent in children with rare diseases. Although extrapolations for dosing and administration are often made from available adult data with similar disease states, this translation becomes even more problematic in rare pediatric diseases. Understanding of rare disease pathophysiology is typically poor, and few, if any, effective therapies have been studied and identified. One condition that illustrates these issues is plastic bronchitis, a rare, most often pediatric disease that is characterized by the production of obstructive bronchial airway casts. This illness primarily occurs in children with congenital heart disease, often after palliative surgery. Plastic bronchitis is a highly clinically relevant and therapeutically challenging problem with a high mortality rate, and, a generally accepted effective pharmacotherapy regimen has yet to be identified. Furthermore, the disease is ill defined, which makes timely identification and treatment of children with plastic bronchitis difficult. The pharmacotherapies currently used to manage this disease are largely anecdotal and vary between the use of macrolide antibiotics, mucolytics, bronchodilators, and inhaled fibrinolytics in a myriad of combinations. The purpose of this review is 2-fold: first, to highlight the dilemma of treating plastic bronchitis, and second, to bring attention to the continuing need for studies of drug therapies used in children so safe and effective drug regimens can be established, particularly for rare diseases. © 2013 Pharmacotherapy Publications, Inc. AD - Department of Clinical, Social and Administrative Sciences, University of Michigan, 428 Church Street, Ann Arbor, MI 48109-1065, United States Division of Pediatric Cardiology, Department of Pediatrics and Communicable Diseases, University of Michigan, Ann Arbor, MI, United States Department of Pharmacy, University of Michigan Hospitals and Health Centers, Ann Arbor, MI, United States AU - Brooks, K. AU - Caruthers, R. L. AU - Schumacher, K. R. AU - Stringer, K. A. DB - Scopus DO - 10.1002/phar.1290 IS - 9 KW - Adverse effects Antiinflammatory agents Congenital heart disease Fibrinolysis Fontan procedure Pulmonary disease M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 2013 SP - 922-934 ST - Pharmacotherapy challenges of fontan-associated plastic bronchitis: A rare pediatric disease T2 - Pharmacotherapy TI - Pharmacotherapy challenges of fontan-associated plastic bronchitis: A rare pediatric disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84885355069&doi=10.1002%2fphar.1290&partnerID=40&md5=9519600521b64a01aed287d7efd39b8d VL - 33 ID - 2037 ER - TY - JOUR AB - OBJECTIVES: To determine physical activity levels in paediatric patients who underwent the Fontan procedure, and their relationship to functional status and exercise capacity. STUDY DESIGN: We studied 147 patients (ages 7-18 years) at a median of 8.1 years after Fontan, as part of the Pediatric Heart Network cross-sectional study of Fontan survivors. Assessment included medical history, self-reported physical activity, parent-completed Child Health Questionnaire (CHQ), cardiopulmonary exercise testing and physical activity level measured by accelerometry (MTI Actigraph). RESULTS: Measured time spent in moderate and vigorous activity was markedly below normal at all ages, particularly in females, and was not significantly related to self-reported activity levels, or to maximum Vo2, Vo2 at anaerobic threshold or maximum work rate on exercise testing. Lower measured activity levels were significantly related to lower perceived general health but not to self-esteem, physical functioning, social impact of physical limitations or overall physical or psychosocial health summary scores. Reduced exercise capacity was more strongly related than measured activity levels to lower scores in general health, self-esteem and physical functioning. CONCLUSIONS: Physical activity levels are reduced after Fontan, independent of exercise capacity, and are associated with lower perceived general health but not other aspects of functional status. AD - University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada. brian.mccrindle@sickkids.ca AN - 17307794 AU - McCrindle, B. W. AU - Williams, R. V. AU - Mital, S. AU - Clark, B. J. AU - Russell, J. L. AU - Klein, G. AU - Eisenmann, J. C. C2 - PMC2066169 DA - Jun DB - PubMed DO - 10.1136/adc.2006.105239 DP - NLM ET - 2007/02/20 IS - 6 KW - Adolescent *Attitude to Health Child Exercise/psychology Exercise Test/methods Female Fontan Procedure/*rehabilitation Health Status Indicators Heart Defects, Congenital/surgery Humans Male *Motor Activity Postoperative Period Self Concept LA - eng N1 - 1468-2044 McCrindle, Brian W Williams, Richard V Mital, Seema Clark, Bernard J Russell, Jennifer L Klein, Gloria Eisenmann, Joey C U01 HL068279/HL/NHLBI NIH HHS/United States U01 HL068290/HL/NHLBI NIH HHS/United States U01 HL068288/HL/NHLBI NIH HHS/United States U01 HL068270/HL/NHLBI NIH HHS/United States U01 HL068292/HL/NHLBI NIH HHS/United States Journal Article Multicenter Study Research Support, N.I.H., Extramural Arch Dis Child. 2007 Jun;92(6):509-14. doi: 10.1136/adc.2006.105239. Epub 2007 Feb 16. PY - 2007 SN - 0003-9888 (Print) 0003-9888 SP - 509-14 ST - Physical activity levels in children and adolescents are reduced after the Fontan procedure, independent of exercise capacity, and are associated with lower perceived general health T2 - Arch Dis Child TI - Physical activity levels in children and adolescents are reduced after the Fontan procedure, independent of exercise capacity, and are associated with lower perceived general health VL - 92 ID - 257 ER - TY - JOUR AB - Objectives: To determine physical activity levels in paediatric patients who underwent the Fontan procedure, and their relationship to functional status and exercise capacity.Study Design: We studied 147 patients (ages 7-18 years) at a median of 8.1 years after Fontan, as part of the Pediatric Heart Network cross-sectional study of Fontan survivors. Assessment included medical history, self-reported physical activity, parent-completed Child Health Questionnaire (CHQ), cardiopulmonary exercise testing and physical activity level measured by accelerometry (MTI Actigraph).Results: Measured time spent in moderate and vigorous activity was markedly below normal at all ages, particularly in females, and was not significantly related to self-reported activity levels, or to maximum Vo2, Vo2 at anaerobic threshold or maximum work rate on exercise testing. Lower measured activity levels were significantly related to lower perceived general health but not to self-esteem, physical functioning, social impact of physical limitations or overall physical or psychosocial health summary scores. Reduced exercise capacity was more strongly related than measured activity levels to lower scores in general health, self-esteem and physical functioning.Conclusions: Physical activity levels are reduced after Fontan, independent of exercise capacity, and are associated with lower perceived general health but not other aspects of functional status. AD - University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada. brian.mccrindle@sickkids.ca AN - 106128121. Language: English. Entry Date: 20070803. Revision Date: 20161118. Publication Type: journal article AU - McCrindle, B. W. AU - Williams, R. V. AU - Mital, S. AU - Clark, B. J. AU - Russell, J. L. AU - Klein, G. AU - Eisenmann, J. C. AU - McCrindle, Brian W. AU - Williams, Richard V. AU - Mital, Seema AU - Clark, Bernard J. AU - Russell, Jennifer L. AU - Klein, Gloria AU - Eisenmann, Joey C. DB - ccm DP - EBSCOhost IS - 6 KW - Attitude to Health Cardiopulmonary Bypass -- Rehabilitation Physical Activity Adolescence Child Data Analysis Software Exercise Test -- Methods Exercise Tolerance Exercise -- Psychosocial Factors Female Functional Status Funding Source Health Status Indicators Heart Defects, Congenital -- Surgery Interviews Linear Regression Male Pearson's Correlation Coefficient Postoperative Period Questionnaires Self Concept Human N1 - research; tables/charts. Journal Subset: Biomedical; Europe; Peer Reviewed; UK & Ireland. Instrumentation: Physical Activity Questionnaire; Congenital Heart Adolescent or Teenage (CHAT) questionnaire; Child Health Questionnaire (CHQ) Parent Report form (CHQ-PF50). Grant Information: U01 HL068270/HL/NHLBI NIH HHS/United States. NLM UID: 0372434. PMID: NLM17307794. PY - 2007 SN - 0003-9888 SP - 509-514 ST - Physical activity levels in children and adolescents are reduced after the Fontan procedure, independent of exercise capacity, and are associated with lower perceived general health T2 - Archives of Disease in Childhood TI - Physical activity levels in children and adolescents are reduced after the Fontan procedure, independent of exercise capacity, and are associated with lower perceived general health UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106128121&site=ehost-live&scope=site VL - 92 ID - 1560 ER - TY - JOUR AB - Objective: A physically active lifestyle can help maintain positive physical and psychosocial health outcomes among adults with congenital heart disease (CHD). This study explored the physical activity perceptions and behaviors among young adults with CHD. Design: This was a cross-sectional, mixed-methods study that included objectively measured physical activity assessment (accelerometer), individual semistructured interviews, and psychosocial questionnaires. Results: Fifteen participants (67% male; 21 ± 3 years old) with moderate (n = 10) or complex (n = 5) CHD were recruited from an outpatient adult CHD clinic. Participants accumulated 26 ± 16 minutes of moderate-to-vigorous physical activity per day, and reported a high quality of life, moderate self-efficacy for exercise, and low cardiac-focused anxiety. Qualitative data indicated that participants reported more positive perceptions toward activity if their family members encouraged physical activity participation, including siblings that engaged in physical activity alongside participants. Participants described parents as supportive rather than overprotective. Activity precautions were perceived by participants as being instructions from cardiologists rather than restrictions by parents. Participants described some physical limitations compared to peers, but managed challenges by either working within their limitations or choosing activities that met their expectations and/or in which they could fully participate. Participants often described childhood physical activity in the context of school, physical education, and organized sports. Whereas physical activity in childhood was viewed as recreational, the cardiac health-promoting aspects became more prominent in adulthood. Activities performed during one's employment were considered sufficient to meet physical activity recommendation levels, and participants reported limited time and/or energy to participate in activity outside of work. Conclusions: The influence of family appeared to help participants adopt a positive perception toward activity participation in childhood that was carried forward to young adulthood. Future clinical work should target adolescents with CHD with less social supports and/or negative perceptions toward physical activity. AD - B.W. McCrindle, Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, ON, Canada AU - McKillop, A. AU - McCrindle, B. W. AU - Dimitropoulos, G. AU - Kovacs, A. H. DB - Embase Medline DO - 10.1111/chd.12553 IS - 2 KW - accelerometer wGT3X adult anxiety assessment aortic coarctation article atrioventricular septal defect behavior assessment bicuspid aortic valve cardiac anxiety Cardiac Anxiety Questionnaire clinical article congenital heart disease cross-sectional study employment exercise Fallot tetralogy family attitude female health promotion human hypoplastic left heart syndrome male parental attitude physical activity physical education priority journal quality of life Readiness to Change questionnaire recreation Self Efficacy for Exercise Scale semi structured interview sport visual analog scale young adult LA - English M3 - Article N1 - L619415991 2017-11-30 2018-07-02 PY - 2018 SN - 1747-0803 1747-079X SP - 232-240 ST - Physical activity perceptions and behaviors among young adults with congenital heart disease: A mixed-methods study T2 - Congenital Heart Disease TI - Physical activity perceptions and behaviors among young adults with congenital heart disease: A mixed-methods study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L619415991 http://dx.doi.org/10.1111/chd.12553 VL - 13 ID - 697 ER - TY - JOUR AB - Background: Physical activity is important for the health of children after the Fontan procedure. Parents uncertain about physical activity have children who are more sedentary. To understand parent uncertainty, we examined agreement regarding activity restrictions from parents, cardiologists, and medical charts. Methods: Activity restriction information for 64 children (25 female), 6 to 11 years, with a Fontan circulation was obtained via cardiologist and parent questionnaire. Each child's medical chart was reviewed for relevant physical activity information. κ Statistics calculated agreement between sources beyond chance. Results: Cardiologist, parent, and medical chart reports of the child's activity restrictions often disagree. Regardless of the cardiologist reported restrictions, 70% of parents reported that their child's activity was restricted (κ = 0.04, P = .25). Parents and cardiologists report the same body contact restrictions (κ = 0.16) but differ for exertion and competition (κ < .05) restrictions. Medical charts reflect competitive sport (κ = 0.26) but not body contact or exertion restrictions. Conclusions: Parent feelings of uncertainty about physical activity are supported by these results indicating that reports of activity restrictions after the Fontan procedure vary significantly by source. Parent and cardiologist reports agree only for body contact restrictions due to anticoagulation therapy. Exertion and body contact restrictions are not reflected in clinic letters to primary care providers. Ensuring that activity restrictions are accurately conveyed will enable parents and primary care providers to confidently encourage the active lifestyles that are so important for these children. © 2009 Mosby, Inc. All rights reserved. AD - B.W. McCrindle, Hospital for Sick Children, Faculty of Medicine, University of Toronto, Toronto, Ont., Canada AU - Longmuir, P. E. AU - McCrindle, B. W. DB - Embase Medline DO - 10.1016/j.ahj.2009.02.014 IS - 5 KW - anticoagulant therapy article cardiologist caregiver child female Fontan procedure human lifestyle major clinical study male medical record parent parent counseling physical activity priority journal questionnaire school child sport LA - English M3 - Article N1 - L354457827 2009-05-05 PY - 2009 SN - 0002-8703 1097-6744 SP - 853-859 ST - Physical activity restrictions for children after the Fontan operation: Disagreement between parent, cardiologist, and medical record reports T2 - American Heart Journal TI - Physical activity restrictions for children after the Fontan operation: Disagreement between parent, cardiologist, and medical record reports UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L354457827 http://dx.doi.org/10.1016/j.ahj.2009.02.014 VL - 157 ID - 1182 ER - TY - JOUR AB - OBJECTIVE: To investigate longer-term physical and neurodevelopmental outcomes of patients with hypoplastic left heart syndrome (HLHS) compared with other patients with functionally single-ventricle circulation surviving beyond the age of 10 years. DESIGN: A retrospective, observational study from a UK tertiary centre for paediatric cardiology. RESULTS: 58 patients with HLHS and 44 non-HLHS patients with single-ventricle physiology were included. Subjective reduction in exercise tolerance was reported in 72% (95% CI 61% to 84%) of patients with HLHS and 45% (31% to 60%) non-HLHS patients. Compared with non-HLHS patients, educational concerns were reported more frequently in patients with HLHS, 41% (29% to 54%) vs 23% (10% to 35%), as was a diagnosis of a behaviour disorder (autism or attention deficit hyperactivity disorder) 12% (4% to 21%) vs 0%, and referral to other specialist services 67% (55% to 79%) vs 48% (33% to 63%). CONCLUSIONS: Within a group of young people with complex congenital heart disease, those with HLHS are likely to have worse physical, psychological and educational outcomes. AD - King's College London School of Medicine, London, UK. Department of Paediatric Neurology, Evelina London Children's Hospital, Guy's and St Thomas' NHS Fiundation Trust, London, UK. Department of Congenital Heart Disease, Evelina London Children's Hospital, Guy's and St Thomas' NHS Fiundation Trust, London, UK. AN - 25480924 AU - Davidson, J. AU - Gringras, P. AU - Fairhurst, C. AU - Simpson, J. DA - May DB - PubMed DO - 10.1136/archdischild-2014-306449 DP - NLM ET - 2014/12/07 IS - 5 KW - Adolescent Child Child Behavior Disorders/*physiopathology/psychology *Child Development Female Heart Ventricles/*abnormalities Humans Hypoplastic Left Heart Syndrome/*physiopathology/psychology Male Psychomotor Performance/*physiology Referral and Consultation Retrospective Studies Tertiary Care Centers United Kingdom Cardiac Surgery Cardiology Neurodevelopment Patient perspective LA - eng N1 - 1468-2044 Davidson, Joseph Gringras, Paul Fairhurst, Charlie Simpson, John Journal Article Observational Study England Arch Dis Child. 2015 May;100(5):449-53. doi: 10.1136/archdischild-2014-306449. Epub 2014 Dec 5. PY - 2015 SN - 0003-9888 SP - 449-53 ST - Physical and neurodevelopmental outcomes in children with single-ventricle circulation T2 - Arch Dis Child TI - Physical and neurodevelopmental outcomes in children with single-ventricle circulation VL - 100 ID - 107 ER - TY - JOUR AB - The aim of this paper is to clarify two important aspects about patients affected by congenital heart disease. Their functional status plays a dominant role in the definition of quality of life related to health status, because of its implication in working and recreational activities. In the first part, we explain their cardiovascular adaptation on exercise, based on pathology (Tetralogy of Fallot, transposition of great arteries, univentricular heart). In the second part, we explain the risk of sudden death from congenital heart disease due to exercise, because of electrical cardiac instability and/or the structural abnormalities of the cardiovascular parietal walls. AD - Cardiology Department, University of Padua, Padua, Italy. AN - 22134469 AU - Dal Bianco, L. AU - Russo, G. AU - Bagato, F. AU - Resta, R. AU - Daliento, L. DA - Dec DB - PubMed DP - NLM ET - 2011/12/03 IS - 6 KW - Adolescent Death, Sudden, Cardiac/*etiology/prevention & control *Exercise Heart Defects, Congenital/mortality/*physiopathology/surgery Heart Septal Defects, Ventricular/physiopathology Heart Ventricles/physiopathology Humans Quality of Life Risk Assessment Risk Factors Survival Rate Tetralogy of Fallot/physiopathology Transposition of Great Vessels/physiopathology LA - eng N1 - Dal Bianco, L Russo, G Bagato, F Resta, R Daliento, L Journal Article Review Italy Minerva Cardioangiol. 2011 Dec;59(6):555-67. PY - 2011 SN - 0026-4725 (Print) 0026-4725 SP - 555-67 ST - Physical exercise in young patients with congenital heart disease T2 - Minerva Cardioangiol TI - Physical exercise in young patients with congenital heart disease VL - 59 ID - 114 ER - TY - JOUR AB - Until the development in 1980 by William Norwood of a staged palliative surgical procedure for hypoplastic left heart syndrome (HPLHS), there was no treatment for that condition. With medical developments in the 1980s, parents had the option of the Norwood procedure, transplantation or comfort care for a child born with HPLHS. With an improvement in the survival rate for the Norwood procedure from an initial 30% to now better than 80%, some physicians believe that comfort care should no longer be an option. If, however, medically sophisticated parents, who know the neurological and motor skills impairments that accompany HPLHS, object to the surgery, they are allowed to opt for comfort care. This two-pronged approach to medical treatment seems to violate the norms on equity and fairness in the care of the patient. Parents need to be informed about long-term neurological and motor skill development as well as survival rates to give informed consent. AD - SJ Walsh Professor of Bioethics, Boston College, Chestnut Hill, MA 02467, USA. john.paris@bc.edu AN - 22678145 AU - Paris, J. J. AU - Moore, M. P. AU - Schreiber, M. D. DA - Oct DB - PubMed DO - 10.1038/jp.2012.72 DP - NLM ET - 2012/06/09 IS - 10 KW - Child *Counseling *Decision Making Humans Hypoplastic Left Heart Syndrome/mortality/*surgery/therapy Infant *Informed Consent Norwood Procedures Palliative Care Parents/*psychology Physicians Treatment Outcome LA - eng N1 - 1476-5543 Paris, J J Moore, M P Schreiber, M D Journal Article United States J Perinatol. 2012 Oct;32(10):748-51. doi: 10.1038/jp.2012.72. Epub 2012 Jun 7. PY - 2012 SN - 0743-8346 SP - 748-51 ST - Physician counseling, informed consent and parental decision making for infants with hypoplastic left-heart syndrome T2 - J Perinatol TI - Physician counseling, informed consent and parental decision making for infants with hypoplastic left-heart syndrome VL - 32 ID - 369 ER - TY - JOUR AB - Mechanical circulatory support offers an alternative therapeutic treatment for patients with dysfunctional single ventricle physiology. An intravascular axial flow pump is being developed as a cavopulmonary assist device for these patients. This study details the development of a new rotating impeller geometry. We examined the performance of 8 impeller geometries with blade stagger or twist angles varying from 100° to 800° using computational methods. A refined range of blade twist angles between 300° and 400° was then identified, and 4 additional geometries were evaluated. Generally, the impeller designs produced 4-26. mmHg for flow rates of 1-4. L/min for 6000-8000 RPM. A data regression analysis was completed and found the impeller with 400° of blade twist to be the superior performer. A hydraulic test was conducted on a prototype of the 400° impeller, which generated measurable pressure rises of 7-28. mmHg for flow rates of 1-4. L/min at 6000-8000 RPM. The findings of the numerical model and experiment were in reasonable agreement within approximately 20%. These results support the continued development of an axial-flow, mechanical cavopulmonary assist device as a new clinical therapeutic option for Fontan patients. © 2016 IPEM. AD - BioCirc Research Laboratory, School of Biomedical Engineering, Science and Health Systems, Drexel University, Philadelphia, PA, United States D. K. Engineering, Richmond, VA, United States Department of Pediatrics, St. Christopher's Hospital for Children, Drexel University College of Medicine, Philadelphia, PA, United States AU - Chopski, S. G. AU - Fox, C. S. AU - McKenna, K. L. AU - Riddle, M. L. AU - Kafagy, D. H. AU - Stevens, R. M. AU - Throckmorton, A. L. DB - Scopus DO - 10.1016/j.medengphy.2016.03.010 IS - 7 KW - Blood pump CFD Computational fluid dynamics Heart pump Mechanical circulatory assistance Pediatric circulatory support Rotary blood pump M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2016 SP - 622-632 ST - Physics-driven impeller designs for a novel intravascular blood pump for patients with congenital heart disease T2 - Medical Engineering and Physics TI - Physics-driven impeller designs for a novel intravascular blood pump for patients with congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84992305667&doi=10.1016%2fj.medengphy.2016.03.010&partnerID=40&md5=5c992b7c2453bc6cdf290c9497b287cf VL - 38 ID - 1900 ER - TY - JOUR AB - When biventricular repair of congenitally corrected transposition of the great arteries (ccTGA) is possible, complete anatomic (left ventricle to the aorta, right ventricle to the pulmonary artery, and associated atrial baffle operation) or physiologic (left ventricle to pulmonary artery, right ventricle to aorta) connections are complex and associated with significant long-term problems such as arrhythmias, subaortic obstruction, valvar dysfunction, ventricular-to-pulmonary artery conduit changes, and deteriorating ventricular function. Recent advances have supported physiologic, anatomic, and single-ventricle repairs. The purpose of this article is to review the natural history of ccTGA, compare and contrast the physiologic and anatomic repairs, and reintroduce a new concept of physiologic repair for patients with ccTGA, ventricular septal defect, and pulmonary stenosis using left ventricular unloading techniques via a bidirectional shunt. AD - Division of Cardiovascular-Thoracic Surgery, Children's Memorial Hospital, Chicago, IL 60614, USA. AN - 12740768 AU - Mavroudis, C. AU - Backer, C. L. DB - PubMed DO - 10.1053/pcsu.2003.50015 DP - NLM ET - 2003/05/13 KW - Adaptation, Physiological/*physiology Adolescent Adult Age Factors Aged Anastomosis, Surgical Cardiac Surgical Procedures/*methods Cardiovascular Physiological Phenomena Child Child, Preschool Female Heart Defects, Congenital/diagnosis/mortality/surgery Heart Function Tests Hemodynamics Humans Infant Infant, Newborn Male Middle Aged Pregnancy Prognosis *Quality of Life Survival Analysis Transposition of Great Vessels/diagnosis/*mortality/*surgery Treatment Outcome Vascular Surgical Procedures/methods LA - eng N1 - Mavroudis, Constantine Backer, Carl L Comparative Study Journal Article Review United States Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2003;6:16-26. doi: 10.1053/pcsu.2003.50015. PY - 2003 SN - 1092-9126 (Print) 1092-9126 SP - 16-26 ST - Physiologic versus anatomic repair of congenitally corrected transposition of the great arteries T2 - Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu TI - Physiologic versus anatomic repair of congenitally corrected transposition of the great arteries VL - 6 ID - 512 ER - TY - JOUR AB - Whereas multiple and different cardiac abnormalities may be accompanied by hypoplasia of the left heart, the postnatal pathophysiologic changes which accompany these abnormalities are very similar. In the fetus, hypoplasia of the left ventricle does not result in any complications, but the post-natal physiologic changes lead to severe hemodynamic and circulatory complications which in the absence of treatment are fatal. The major physiologic changes are those related to the decrease in pulmonary vascular resistance, spontaneous constriction or closure of the ductus arteriosus, and restriction of the obligatory left-to-right shunt which must exist through an interatrial communication. The clinical features are closely related to the dominance and severity of the physiologic changes and the balance between the systemic and pulmonary vascular resistance. Presentation may be with respiratory distress, congestive heart failure, vascular collapse, or less frequently with severe cyanosis. The presence of mild hypoxemia, normal or reduced Pace, and very low pH are characteristic features of infants with hypoplastic left heart syndrome. Early post-natal detection of these infants may be enhanced by screening all newborns discharged from the nursery with measurement of systemic arterial oxygen saturations by a pulse volume oximeter. AD - A. Rosenthal, Department of Pediatrics, CS Mott Children 's Hospital, University Michigan Medical Center, Ann Arbor, MI 48109-0204, United States AU - Rosenthal, A. DB - Embase DO - 10.1016/1058-9813(95)00145-X IS - 1 KW - arterial oxygen saturation clinical feature collapse congestive heart failure cyanosis ductus arteriosus early diagnosis heart hemodynamics human hypoplastic left heart syndrome hypoxemia lung vascular resistance newborn pathophysiology perinatal period pH priority journal pulse oximetry respiratory arrest screening short survey LA - English M3 - Short Survey N1 - L26130373 1996-05-12 PY - 1996 SN - 1058-9813 SP - 19-22 ST - Physiology, diagnosis and clinical profile of the hypoplastic left heart syndrome T2 - Progress in Pediatric Cardiology TI - Physiology, diagnosis and clinical profile of the hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L26130373 http://dx.doi.org/10.1016/1058-9813(95)00145-X VL - 5 ID - 1364 ER - TY - JOUR AB - Neonates have low levels of antithrombin. Inadequate anticoagulation during cardiopulmonary bypass (CPB) due to low antithrombin activity may result in a poor preservation of the coagulation system during bypass. We hypothesize that antithrombin replacement to neonates prior to CPB will preserve the hemostatic system and result in less postoperative bleeding. A randomized, double-blinded, placebo-controlled pilot study of antithrombin replacement to neonates prior to CPB was conducted. Preoperative antithrombin levels determined the dose of recombinant antithrombin or placebo to be given. Antithrombin levels were measured following the dosing of the antithrombin/placebo, after initiation of bypass, near the completion of bypass, and upon intensive care unit admission. Eight subjects were enrolled. No subject had safety concerns. Mediastinal exploration occurred in two antithrombin subjects and one placebo subject. Antithrombin activity levels were significantly higher in the treated group following drug administration; levels continued to be higher than preoperatively but not different from the placebo group at all other time points. Total heparin administration was less in the antithrombin group; measurements of blood loss were similar in both groups. A single dose of recombinant antithrombin did not maintain 100% activity levels throughout the entire operation. Although no safety concerns were identified in this pilot study, a larger trial is necessary to determine clinical efficacy. AD - R.A. Niebler, Section of Critical Care, Department of Pediatrics, Medical College of Wisconsin, PO Box 1997 (MS 681), Milwaukee, WI, United States AU - Niebler, R. A. AU - Woods, K. J. AU - Murkowski, K. AU - Ghanayem, N. S. AU - Hoffman, G. AU - Mitchell, M. E. AU - Punzalan, R. C. AU - Scott, J. P. AU - Simpson, P. AU - Tweddell, J. S. DB - Embase Medline DO - 10.1111/aor.12642 IS - 1 KW - NCT01158729 antithrombin heparin placebo recombinant antithrombin recombinant protein unclassified drug aorta anomaly aortic arch interruption arterial switch operation article blood clotting bypass surgery cardiopulmonary bypass clinical article clinical effectiveness controlled study disease control double blind procedure drug efficacy feasibility study great vessels transposition human hypoplastic left heart syndrome intensive care newborn pilot study postoperative hemorrhage priority journal randomized controlled trial risk reduction LA - English M3 - Article N1 - L607220730 2015-12-17 2016-02-11 PY - 2016 SN - 1525-1594 0160-564X SP - 80-85 ST - A Pilot Study of Antithrombin Replacement Prior to Cardiopulmonary Bypass in Neonates T2 - Artificial Organs TI - A Pilot Study of Antithrombin Replacement Prior to Cardiopulmonary Bypass in Neonates UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L607220730 http://dx.doi.org/10.1111/aor.12642 VL - 40 ID - 832 ER - TY - JOUR AB - A review of outcomes of hypoplastic left heart syndrome at a pediatric hospital 1983-2004 identified 154 deaths among 206 children (75%). Of 134 surgically treated, 82 (62%) died: 68 (83%) in intensive care, six (7%) in the operating theatre, three (4%) during transport from home, two (2%) on the cardiac ward, one (1%) at home, one (1%) in the emergency department, and one (1%) elsewhere. Seventy died after stage one Norwood surgery (median age: 13 days), three died after second stage surgery (ages: 3, 4, and 20 months), two died after third stage surgery (ages: 1.2 and 3.5 years), two died after heart transplantation (ages: 4.6 and 15 years), and five died after non-Norwood surgery. All 72 infants treated without surgery died: 68 died at a median age 3 days; 57 (79%) in intensive care, three (4%) on the ward, and 12 (17%) elsewhere. Discussion involves strategies to support parents and staff at diagnosis, and before and after a child's death. AD - Pediatric Intensive Care Unit, The Royal Children's Hospital and Department of Social Work, The University of Melbourne, Parkville, Victoria, Australia. AN - 18681243 AU - Cantwell-Bartl, A. M. AU - Tibballs, J. DA - Summer DB - PubMed DP - NLM ET - 2008/08/07 IS - 2 KW - Adolescent Age Distribution Bereavement Cardiac Surgical Procedures/mortality Child Child, Preschool *Counseling *Decision Making Humans Hypoplastic Left Heart Syndrome/*mortality/surgery/*therapy Infant Infant, Newborn Parents/*psychology Professional-Family Relations Retrospective Studies Victoria/epidemiology LA - eng N1 - Cantwell-Bartl, Annie M Tibballs, James Journal Article United States J Palliat Care. 2008 Summer;24(2):76-84. PY - 2008 SN - 0825-8597 (Print) 0825-8597 SP - 76-84 ST - Place, age, and mode of death of infants and children with hypoplastic left heart syndrome: implications for medical counselling, psychological counselling, and palliative care T2 - J Palliat Care TI - Place, age, and mode of death of infants and children with hypoplastic left heart syndrome: implications for medical counselling, psychological counselling, and palliative care VL - 24 ID - 401 ER - TY - JOUR AB - Biomarkers of heart failure in adults have been extensively studied. However, biomarkers to monitor the progression of heart failure in children with univentricular physiology are less well understood. We proposed that as mediators of diverse pathophysiology, miRNAs contained within circulating microvesicles could serve as biomarkers for the presence and progression of heart failure in univentricular patients. To test this, we studied the association of heart failure with elevations in specific miRNAs isolated from circulating microvesicles in a cohort of children with univentricular heart disease and heart failure. We conducted a single site cross-sectional observational study of 71 children aged 1 month-7 years with univentricular heart disease and heart failure. We demonstrated that levels of miR129-5p isolated from plasma microvesicles were inversely related to the degree of clinical heart failure as assessed by Ross score. We then showed that miR129-5p levels are downregulated in HL1 cells and human embryonic stem cell-derived cardiomyocytes exposed to oxidative stress. We demonstrated that bone morphogenetic protein receptor 2, which has been implicated in the development of pulmonary vascular disease, is a target of miR129-5p, and conversely regulated in response to oxidative stress in cell culture. Levels of miR129-5p were inversely related to the degree of clinical heart failure in patients with univentricular heart disease. This study demonstrates that miR129-5p is a sensitive and specific biomarker for heart failure in univentricular heart disease independent of ventricular morphology or stage of palliation. Further study is warranted to understand the targets affected by miR129-5p with the development of heart failure in patients with univentricular physiology. AD - Department of Pediatrics and Cardiovascular Research Institute, University of California San Francisco, San Francisco, CA, United States of America. Department of Pediatrics and the Mindich Child Health and Development Institute, Icahn School of Medicine at Mount Sinai, New York, NY, United States of America. AN - 28859128 AU - Ramachandran, S. AU - Lowenthal, A. AU - Ritner, C. AU - Lowenthal, S. AU - Bernstein, H. S. C2 - PMC5578659 of stock in this company, however, Merck & Co. provided no funding for this study. H.S.B. was an employee of the University of California when this study was conducted and analyzed, and during the initial preparation of this manuscript. Neither affiliation alters our adherence to PLOS ONE policies on sharing data and materials. DB - PubMed DO - 10.1371/journal.pone.0183624 DP - NLM ET - 2017/09/01 IS - 8 KW - Biomarkers/*blood Cell-Derived Microparticles/metabolism/pathology Child Child, Preschool Cross-Sectional Studies Disease Progression Female Heart Failure/*blood/physiopathology Heart Ventricles/metabolism/*physiopathology Humans Infant Infant, Newborn Male MicroRNAs/*blood LA - eng N1 - 1932-6203 Ramachandran, Sweta Lowenthal, Alexander Ritner, Carissa Lowenthal, Shiri Bernstein, Harold S Orcid: 0000-0002-0739-2508 Journal Article PLoS One. 2017 Aug 31;12(8):e0183624. doi: 10.1371/journal.pone.0183624. eCollection 2017. PY - 2017 SN - 1932-6203 SP - e0183624 ST - Plasma microvesicle analysis identifies microRNA 129-5p as a biomarker of heart failure in univentricular heart disease T2 - PLoS One TI - Plasma microvesicle analysis identifies microRNA 129-5p as a biomarker of heart failure in univentricular heart disease VL - 12 ID - 57 ER - TY - JOUR AU - Chaudhari, M. AU - Stumper, O. DB - Scopus IS - 7 M3 - Article N1 - Cited By :34 Export Date: 15 June 2020 PY - 2004 SP - 801 ST - Plastic bronchitis after Fontan operation: Treatment with stent fenestration of the Fontan circuit T2 - Heart TI - Plastic bronchitis after Fontan operation: Treatment with stent fenestration of the Fontan circuit UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-3042592353&partnerID=40&md5=bf21e070080a58a2ad9f8d2b503bf3ac VL - 90 ID - 2253 ER - TY - JOUR AB - Fibrinous bronchitis, also known as plastic bronchitis or pseudomembranous bronchitis, usually manifests clinically with non-productive cough and dyspnea. Prior to the expectoration of plaque formations, patients have a history of viral or other respiratory disease. Plastic bronchitis is an extremely rare and fatal complication associated with Fontan procedure. The condition is characterized by the formation of inspissated bronchial casts which may cause life-threatening airway obstructions. Although the pathogenesis of this condition remains unclear, it is believed to involve elevated pulmonary venous pressure, increased central venous pressure, and endobronchial lymphatic leakage. A 9-year-old male patient with Down syndrome underwent Fontan procedure 29 months earlier due to complete endocardial cushion defect and single ventricular physiology. The patient presented to the emergency department due to sudden-onset respiratory distress and fever (38.5 °C). Bilateral diffuse sibilant rhonchi, secretory rales, and intercostal retractions were noted during pulmonary system examination. Posterior-anterior chest x-ray showed cardiomegaly (cardiothoracic ratio=0.6) and bilateral diffuse infiltration. While under treatment, the patient experienced sudden-onset cough with expectoration of rubbery sputum in the form of branching bronchi-shaped casts. Samples of the inspissated sputum were sent to the Pathology and Microbiology departments for examination. The pathology report indicated fibrinoid material composed of a small number of inflammatory cells and bacterial plaques. Normal bacterial flora was identified in microbiological culture. Clinical presentation can vary from mild clinical findings to life-threatening symptoms. As in our patient, the diagnosis is made clinically, based on expectoration of bronchial casts or their detection during bronchoscopy. As our patient responded well to medical treatment, we proceeded with clinical follow-up. Plastic bronchitis is a very rare entity, and carries a poorer prognosis when it develops after congenital heart disease, as in our case. AD - Ege University Faculty of Medicine, Department of Pediatrics, Division of Pediatric Cardiology, İzmir, Turkey. AN - 137649146. Language: English. Entry Date: 20190725. Revision Date: 20190725. Publication Type: Article AU - Doğan, Eser AU - Aydın, Derya AU - Ülger, Zülal AU - Levent, Ertürk DB - ccm DO - 10.4274/jpr.galenos.2018.75768 DP - EBSCOhost IS - 2 KW - Bronchitis -- Diagnosis Postoperative Complications -- Diagnosis Cardiopulmonary Bypass -- Adverse Effects Child Male Sudden Onset Respiratory Distress Syndrome Fever Physical Examination Radiography, Thoracic Bronchoscopy Bronchitis -- Pathology Bronchitis -- Therapy Bronchitis -- Prognosis Treatment Outcomes N1 - case study; diagnostic images; pictorial. Journal Subset: Biomedical. Special Interest: Pediatric Care. PY - 2019 SN - 2147-9445 SP - 166-168 ST - Plastic Bronchitis Following Fontan Procedure: A Case Report T2 - Journal of Pediatric Research TI - Plastic Bronchitis Following Fontan Procedure: A Case Report UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=137649146&site=ehost-live&scope=site VL - 6 ID - 1453 ER - TY - JOUR AB - Plastic bronchitis is a rare and underdiagnosed disease characterized by the formation and expectoration of bronchial casts of amorphous material, which can be potentially fatal. It is more frequent in pediatric population. Symptoms can range from chronic cough and dyspnea to respiratory failure depending on the area of the compromised airway. Casts are classified as type I when constituted by inflammatory cells and accompany diseases such as asthma and pneumonias; and type II when acellular and are associated with congenital heart diseases following procedures such as Fontan. We report the case of a male schoolchild with a history of complex congenital heart disease, treated with palliative surgery, evaluated in the emergency department for cough and respiratory distress. The mother referred expulsion of gelatinous material after coughing. During clinical evaluation, expulsion of bronchial casts was evidenced, suggesting a plastic bronchitis. He underwent a diagnostic and therapeutic bronchoscopy and received initial treatment with respiratory therapy, nebulized hypertonic saline solution, mucolytics, dornase alpha and nebulized heparin. The hospitalization revealed a stenosis of the right pulmonary artery, which was corrected with stent. The patient progressed satisfactorily with improvement of cough and expectoration. He was discharged with combined treatment, nebulized medications and those concerning his underlying disease. AD - Department of Pediatric, Pediatric Pulmonology Service, Fundación Valle del Lili University Hospital, Cali, Colombia. Faculty of Health Sciences, Universidad Icesi, Cali, Colombia. Department of Pediatric, Fundación Valle del Lili University Hospital, Cali, Colombia. Department of Pathology and Laboratory Medicine, Fundación Valle del Lili University Hospital, Cali, Colombia. Clinical Research Center, Fundación Valle del Lili University Hospital, Cali, Colombia. Department of Internal Medicine, Pulmonology Service, Interventional Pulmonology, Fundación Valle del Lili University Hospital, Cali, Colombia. AN - 31245273 AU - Grizales, C. L. AU - González, L. M. AU - Castrillon, M. A. AU - Sua, L. F. AU - Lores, J. AU - Aguirre, M. AU - Fernández-Trujillo, L. C2 - PMC6582060 DB - PubMed DO - 10.1016/j.rmcr.2019.100876 DP - NLM ET - 2019/06/28 KW - Bronchial casts Congenital heart disease Fontan Plastic bronchitis LA - eng N1 - 2213-0071 Grizales, Clara L González, Lina M Castrillon, Maria A Sua, Luz F Lores, Juliana Aguirre, Marisol Fernández-Trujillo, Liliana Case Reports Respir Med Case Rep. 2019 Jun 8;28:100876. doi: 10.1016/j.rmcr.2019.100876. eCollection 2019. PY - 2019 SN - 2213-0071 (Print) 2213-0071 SP - 100876 ST - Plastic bronchitis: A case report T2 - Respir Med Case Rep TI - Plastic bronchitis: A case report VL - 28 ID - 26 ER - TY - JOUR AB - Plastic bronchitis is a rare disorder characterized by the formation of branching mucoid bronchial casts. Several pathophysiologic conditions are associated with development of these intrabronchial casts, including congenital heart defects. The management of plastic bronchitis presents an unusual and interesting bronchoscopic challenge. We describe a patient who underwent a Fontan procedure for correction of a congenital heart defect and subsequently developed respiratory distress secondary to plastic bronchitis on two occasions. In both cases, endoscopic intervention was required to remove these casts. A review of the literature, including the proposed etiologies, diagnosis, and current medical and surgical management, is also undertaken. AD - Department of Otolaryngology and Communication Sciences, Medical College of Wisconsin, 9200 West Wisconsin Avenue, Milwaukee 53226, USA. AN - 12697358 AU - Ishman, S. AU - Book, D. T. AU - Conley, S. F. AU - Kerschner, J. E. DA - May DB - PubMed DO - 10.1016/s0165-5876(03)00004-1 DP - NLM ET - 2003/04/17 IS - 5 KW - Bronchitis/complications/*etiology/surgery Bronchoscopy Child, Preschool *Fontan Procedure Heart Defects, Congenital/*surgery Humans Laryngoscopy Male *Postoperative Complications/surgery Recurrence Respiratory Insufficiency/etiology LA - eng N1 - Ishman, Stacey Book, David T Conley, Stephen F Kerschner, Joseph E Case Reports Journal Article Ireland Int J Pediatr Otorhinolaryngol. 2003 May;67(5):543-8. doi: 10.1016/s0165-5876(03)00004-1. PY - 2003 SN - 0165-5876 (Print) 0165-5876 SP - 543-8 ST - Plastic bronchitis: an unusual bronchoscopic challenge associated with congenital heart disease repair T2 - Int J Pediatr Otorhinolaryngol TI - Plastic bronchitis: an unusual bronchoscopic challenge associated with congenital heart disease repair VL - 67 ID - 496 ER - TY - JOUR AB - BACKGROUND: L transposition of the great arteries (l-tga) is an uncommon congenital cardiovascular malformation that occurs from abnormal looping of the primitive cardiac tube. Little is known about risk factors for the development of l-tga. METHODS: This study is a case-control study of 36 cases of l-tga compared to 3,495 population-based live born infant controls in the Baltimore Washington Infant Study (1981-1989 births). Extensive personal and occupational exposure data from parental interviews were available for all subjects. A geographic information system was used to identify potential environmental exposures, for example, hazardous waste sites prioritized for cleanup (National Priority List sites). Adjusted odds ratios (OR) and 95% confidence intervals (CI) were calculated using exact parameter estimates from logistic regression. RESULTS: Most infants with l-tga had multiple cardiovascular anomalies, with single ventricle in 47%. Over 75% of all cases of l-tga occur in two regions of contiguous cases. Within these regions, the case control OR of l-tga are 13.4 (95%CI, 4.7-37.8) Both areas are characterized by release of toxic chemicals into air and by hazardous waste sites. Parental exposures to hair dye, smoking and laboratory chemicals are also associated with case status. CONCLUSIONS: L-tga, a congenital cardiovascular malformation due to very early abnormalities in embryogenesis, is associated strongly with residency in two small regions of Maryland and District of Columbia (DC), and with other parental personal or occupational exposures. Additional research is needed to identify the components of spatial and other associations that constitute etiologic risk factors. © 2003 Wiley-Liss, Inc. AD - K.S. Kuehl, Department of Cardiology, CNMC, 111 Michigan Ave., NW, Washington, DC 20010, United States AU - Kuehl, K. S. AU - Loffredo, C. A. DB - Embase Medline DO - 10.1002/bdra.10015 IS - 3 KW - chemical compound hair dye article calculation case control study confidence interval controlled study disease association embryo development environmental exposure environmental factor geographic information system geography great vessels transposition hazardous waste human infant interview logistic regression analysis major clinical study occupational exposure population research priority journal risk assessment risk factor smoking United States LA - English M3 - Article N1 - L37238057 2003-10-20 PY - 2003 SN - 1542-0752 SP - 162-167 ST - Population-based study of L-transposition of the great arteries: Possible associations with environmental factors T2 - Birth Defects Research Part A - Clinical and Molecular Teratology TI - Population-based study of L-transposition of the great arteries: Possible associations with environmental factors UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L37238057 http://dx.doi.org/10.1002/bdra.10015 VL - 67 ID - 1306 ER - TY - JOUR AB - OBJECTIVE: Exercise training is recommended for its possible favorable effects on Fontan pathophysiology. This study aimed to elucidate the impact of pediatric exercise capacity trajectory, which may mimic the effect of exercise training, on late adult Fontan pathophysiology. METHODS: Since 1990, 97 Fontan patients had consecutively undergone two serial cardiopulmonary exercise tests (CPX1 and CPX2) during childhood (ages 8 ± 2 and 14 ± 2 years) and one during adulthood (CPX3; age 23 ± 5 years). The changes in peak oxygen uptake (PVO(2): % of normal value) from CPX1 to CPX2 (1-dPVO(2)) and from CPX2 to CPX3 (2-dPVO(2)) were calculated, and then the patients were divided into four subgroups according the 1-dPVO(2) and 2-dPVO(2). RESULTS: In their adulthood, when compared with groups with negative 1-dPVO(2), the central venous pressure, plasma brain natriuretic peptide level, and renal resistive index were lower, whereas liver synthetic function, body fat-free percentage, and PVO(2) were higher in those with positive 1-dPVO(2) (p < 0.05-0.0001). However, these favorable associations of 2-d-PVO(2) with adult Fontan pathophysiology were not observed, except for the PVO(2). After CPX3, 13 unexpected events occurred, and the risk was 76% lower in the groups having positive 1-dPVO(2) than in those with negative 1-dPVO(2) (hazard ratio, 0.24; 95% confidence interval, 0.09-0.62; p = 0.0035). CONCLUSIONS: A positive exercise capacity trajectory during childhood predicts better adult Fontan pathophysiology, including better prognosis. Thus, prescription of exercise could be a promising strategy in the management of pediatric Fontan patients. AD - Departments of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan; Adult Congenital Heart Disease, National Cerebral and Cardiovascular Center, Osaka, Japan. Electronic address: hohuchi@ncvc.go.jp. Departments of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan. Preventive Medicine and Epidemiologic Informatics, Center for Cerebral and Cardiovascular Center, National Cerebral and Cardiovascular Center, Osaka, Japan. AN - 30029798 AU - Ohuchi, H. AU - Negishi, J. AU - Miike, H. AU - Toyoshima, Y. AU - Morimoto, H. AU - Fukuyama, M. AU - Iwasa, T. AU - Sakaguchi, H. AU - Miyazaki, A. AU - Shiraishi, I. AU - Kurosaki, K. AU - Nakai, M. DA - Jan 1 DB - PubMed DO - 10.1016/j.ijcard.2018.06.067 DP - NLM ET - 2018/07/22 KW - Adolescent Adult Child Child, Preschool Exercise Test Exercise Therapy/*methods/psychology Exercise Tolerance/*physiology Female Follow-Up Studies *Fontan Procedure *Habits Heart Defects, Congenital/physiopathology/*rehabilitation/surgery Humans Male Patient Compliance/*psychology Postoperative Period Prognosis Retrospective Studies Young Adult Adulthood Childhood Exercise capacity Fontan operation Morbidity LA - eng N1 - 1874-1754 Ohuchi, Hideo Negishi, Jun Miike, Hikari Toyoshima, Yuka Morimoto, Hitomi Fukuyama, Midori Iwasa, Toru Sakaguchi, Heima Miyazaki, Aya Shiraishi, Isao Kurosaki, Kenichi Nakai, Michikazu Journal Article Netherlands Int J Cardiol. 2019 Jan 1;274:80-87. doi: 10.1016/j.ijcard.2018.06.067. Epub 2018 Jul 4. PY - 2019 SN - 0167-5273 SP - 80-87 ST - Positive pediatric exercise capacity trajectory predicts better adult Fontan physiology rationale for early establishment of exercise habits T2 - Int J Cardiol TI - Positive pediatric exercise capacity trajectory predicts better adult Fontan physiology rationale for early establishment of exercise habits VL - 274 ID - 33 ER - TY - JOUR AB - Few conditions make even the most experienced pediatric anesthesiologists take pause. Pulmonary hypertension is one such condition due to the associated high perioperative morbidity and mortality. Much is written about the intraoperative management of pediatric pulmonary hypertension. This article will instead focus on postoperative care and review the evidence in support of a risk stratification approach for the post-anesthetic disposition of these patients. The total risk for post-anesthetic adverse events includes the patient's baseline risk factors and the incremental risks imposed by the procedure and anesthetic. A proposal with recommendations to guide practitioners and a table summarizing relevant factors are provided. Last, the readers' attention is drawn to the heterogeneity of pulmonary hypertensive disease. Pulmonary arterial hypertension (precapillary) differs significantly from pulmonary venous hypertension (postcapillary); the anesthetic management for one may be relatively contraindicated in the other. Their dissimilarities justify the need to distinguish them for study and research endeavors. © SAGE Publications. AD - Eastern Virginia Medical School, Norfolk, VA, United States Children's Hospital of the King's Daughters, 601 Children's Lane, Norfolk, VA 23507, United States University of Texas Medical Branch, Galveston, TX, United States Driscoll Children's Hospital, Corpus Christi, TX, United States Children's Hospital Colorado, Aurora, CO, United States University of Colorado, Denver, Aurora, CO, United States AU - Chau, D. F. AU - Gangadharan, M. AU - Hartke, L. P. AU - Twite, M. D. DB - Scopus DO - 10.1177/1089253215593179 IS - 1 KW - cardiac anesthesia children hypertension pediatric intensive care perioperative mortality postoperative care postoperative complications pulmonary artery pressure M3 - Article N1 - Cited By :8 Export Date: 15 June 2020 PY - 2016 SP - 63-73 ST - The post-anesthetic care of pediatric patients with pulmonary hypertension T2 - Seminars in Cardiothoracic and Vascular Anesthesia TI - The post-anesthetic care of pediatric patients with pulmonary hypertension UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84958231070&doi=10.1177%2f1089253215593179&partnerID=40&md5=1164e2c1b8bff8c8e6130eacb57f04e1 VL - 20 ID - 1921 ER - TY - JOUR AB - Background: While therapeutic hypothermia (TH) is an effective neuroprotective therapy for neonatal hypoxic-ischemic encephalopathy, TH has not been demonstrated to improve outcome in other pediatric populations. Patients with acquired or congenital heart disease (CHD) are at high risk of both cardiac arrest and neurodevelopmental impairments, and therapies are needed to improve neurologic outcome. The primary goal of our study was to compare safety/efficacy outcomes in post-arrest CHD patients treated with TH versus controls not treated with TH. Methods: Patients with CHD treated during the first 18 months after initiation of a post-arrest TH protocol (temperature goal: 33.5 °C) were compared to historical and contemporary post-arrest controls not treated with TH. Post-arrest data, including temperature, safety measures (e.g. arrhythmia, bleeding), neurodiagnostic data (EEG, neuroimaging), and survival were compared. Results: Thirty arrest episodes treated with TH and 51 control arrest episodes were included. The groups did not differ in age, duration of arrest, post-arrest lactate, or use of ECMO-CPR. The TH group's post-arrest temperature was significantly lower than control's (33.6 ± 0.2 °C vs 34.7 ± 0.5 °C, p < 0.001). There was no difference between the groups in safety/efficacy measures, including arrhythmia, infections, chest-tube output, or neuroimaging abnormalities, nor in hospital survival (TH 61.5% vs control 59.1%, p = NS). Significantly more controls had seizures than TH patients (26.1% vs. 4.0%, p = 0.04). Almost all seizures were subclinical and occurred more than 24 h post-arrest. Conclusion: Our data show that pediatric CHD patients who suffer cardiac arrest can be treated effectively and safely with TH, which may decrease the incidence of seizures. AD - J.S. Soul, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA, United States AU - Cheng, H. H. AU - Rajagopal, S. K. AU - Sansevere, A. J. AU - McDavitt, E. AU - Wigmore, D. AU - Mecklosky, J. AU - Andren, K. AU - Williams, K. A. AU - Danehy, A. AU - Soul, J. S. DB - Embase Medline DO - 10.1016/j.resuscitation.2018.02.022 KW - fosphenytoin sodium levetiracetam midazolam phenobarbital topiramate article brain ventricle dilatation child child safety childhood disease clinical article clinical effectiveness congenital heart disease controlled study electroencephalogram encephalomalacia female heart arrest heart single ventricle heart stroke volume human hypoxic ischemic encephalopathy induced hypothermia infant infection male neuroimaging neuroprotection post treatment survival priority journal protocol compliance resuscitation seizure sinus bradycardia subdural hematoma LA - English M3 - Article N1 - L2000602809 2018-04-05 2019-01-11 PY - 2018 SN - 1873-1570 0300-9572 SP - 83-89 ST - Post-arrest therapeutic hypothermia in pediatric patients with congenital heart disease T2 - Resuscitation TI - Post-arrest therapeutic hypothermia in pediatric patients with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2000602809 http://dx.doi.org/10.1016/j.resuscitation.2018.02.022 VL - 126 ID - 687 ER - TY - JOUR AB - PURPOSE: PRES is an uncommon complication of organ transplantation. Little is known about its clinical course and risk factors. We sought to examine the natural history and risk factors for PRES in pediatric heart transplant (HTx) recipients. METHODS: 14-center international retrospective study of 46 pediatric PRES cases between 2000-2018, compared with 1372 HTx recipients without PRES between 2000-2018. Demographic and clinical data were entered into a central database. PRES diagnosis was based on a combination of seizures, headache, altered mental status or focal neurological deficits, and required confirmation, either by MRI or resolution of neurological symptoms after CNI withdrawal. Factors associated with PRES were identified by Cox Proportional Hazard multivariable analysis. RESULTS: Median onset of symptoms was 16 days (R, 2-2468 d) after HTx; 23 (50%) developed PRES in the first 2 weeks, 32 (70%) in the first month, and 40 (87%) within the first year after HTx. Symptoms at presentation included seizures (40/46, 87%), altered mental status (27/46, 59%), headache (18/46, 39%), focal neurological deficits (16/46), and lasted for median of 4 days (R 1-1772d), with almost all recipients (44/46, 96%) making a full recovery. MRI was obtained in 43/46, showing a vasogenic pattern of edema characteristic of PRES in 37 recipients (80%). Recipients with PRES tended to be older (9.1 y vs 5.9 y, p=0.06). Hypertension (SBP>95%ile) was present in 40/44 (91%) recipients with PRES at presentation, with severe hypertension (SBP>99%ile plus 5mmHG) in 37 (85%). Though all cases occurred in the setting of CNI use (TAC 40, CSA 6), levels were supratherapeutic in only 13/42 (31%). Cox analysis revealed a 5.3x increased hazard for PRES with Glenn (CI 1.54,18.01; p=0.008) and a 3.8x increased hazard with Fontan (1.08,13.00; p=0.038), while age, underlying cardiac diagnosis, BMI, ischemic time and type of CNI did not appear to contribute significantly. CONCLUSION: 2/3 of pediatric PRES cases occurred in the first month after HTx with seizures as the most common presenting symptom. An overwhelming majority of patients were severely hypertensive, whereas less than a third had supratherapeutic CNI levels. Almost all recipients recovered fully within days. Recipients with pre-existing Glenn or Fontan have a higher rate of developing PRES after HTx. AU - Kemna, M. S. AU - Shaw, D. AU - Ameduri, R. AU - Azeka, E. AU - Bradford, T. AU - Jorgensen, N. AU - Lin, K. Y. AU - Menteer, J. AU - Moller, T. AU - Reardon, L. AU - Schumacher, K. AU - Shih, R. AU - Stendahl, G. AU - West, S. AU - Wisotzkey, B. AU - Zangwill, S. DB - Medline DO - 10.1016/j.healun.2020.01.1270 IS - 4 KW - article body mass child controlled study demography drug overdose drug withdrawal female headache heart graft heart muscle ischemia history human major clinical study male mental health multicenter study nuclear magnetic resonance imaging posterior reversible encephalopathy syndrome remission retrospective study risk factor seizure surgery systolic blood pressure LA - English M3 - Article N1 - L631930964 2020-06-05 PY - 2020 SN - 1557-3117 SP - S66-S67 ST - Posterior Reversible Encephalopathy Syndrome (PRES) after Pediatric Heart Transplantation: A Multicenter Study T2 - The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation TI - Posterior Reversible Encephalopathy Syndrome (PRES) after Pediatric Heart Transplantation: A Multicenter Study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L631930964 http://dx.doi.org/10.1016/j.healun.2020.01.1270 VL - 39 ID - 560 ER - TY - JOUR AB - Identification of risk factors for PRES after organ transplant can improve early detection and avoid permanent neurological injury. High calcineurin-inhibitor levels and hypertension are recognized risk factors for PRES in adult transplant recipients. Limited data exist regarding PRES after pediatric HTx, with studies limited to case reports. We performed a retrospective review of 128 pediatric HTx recipients to identify risk factors for PRES. Seven of 128 (5.5%) recipients developed PRES at a median of 10 days (5-57) after HTx. The median age of recipients with PRES was 10.0 yr (5.7-19.0), compared to 1.4 yr (0.0-19.8) for recipients without PRES (p = 0.010). Fewer than half of recipients with PRES had elevated post-transplant calcineurin-inhibitor levels (n = 3) and/or preceding severe hypertension (n = 3). Four of seven who developed PRES (57%) had pretransplant Glenn or Fontan physiology (G/F). G/F was a significant risk factor for PRES (RR 4.99, 95% CI: 1.19-21.0, p = 0.036). Two recipients (29%), both with severe PRES, had residual neurological symptoms. In summary, PRES occurred in 5.5% of pediatric HTx recipients and presented early after HTx. All recipients with PRES were > 5 yr. Patients with pretransplant G/F were at increased risk, a risks factor not previously described. AU - Eilers, B. AU - Albers, E. AU - Law, Y. AU - Mike McMullan, D. AU - Shaw, D. AU - Kemna, M. DB - Embase Medline DO - 10.1111/petr.12702 IS - 4 KW - calcineurin inhibitor cyclosporine methylprednisolone tacrolimus thymocyte antibody adolescent amnesia aortic stenosis article brain edema child clinical article congestive cardiomyopathy controlled study coronary artery disease drug dose reduction drug megadose drug substitution drug withdrawal endocardial cushion defect female Fontan procedure Glenn shunt great vessels transposition heart transplantation heart ventricle septum defect human hypertension hypoplastic left heart syndrome language disability male motor dysfunction observational study posterior reversible encephalopathy syndrome preschool child pulmonary valve atresia retrospective study school child seizure LA - English M3 - Article N1 - L610302594 2016-05-19 PY - 2016 SN - 1399-3046 1397-3142 SP - 552-558 ST - Posterior reversible encephalopathy syndrome after pediatric heart transplantation: Increased risk for children with preexisting Glenn/Fontan physiology T2 - Pediatric Transplantation TI - Posterior reversible encephalopathy syndrome after pediatric heart transplantation: Increased risk for children with preexisting Glenn/Fontan physiology UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L610302594 http://dx.doi.org/10.1111/petr.12702 VL - 20 ID - 805 ER - TY - JOUR AB - Background: As the surgical treatment of scoliosis after a Fontan procedure is very challenging due to the risk of various perioperative complications, case reports are scarce. We herein describe three patients who were successfully treated for scoliosis following a Fontan procedure and discuss their clinical and radiological outcomes. Methods: We retrospectively reviewed three cases of scoliosis treated by posterior spinal fusion after a Fontan procedure. Results: Mean preoperative major curve Cobb angle was 83.7°, mean surgical time was 233.0 min, and mean blood loss was 1167 g. The mean correction rate of the major curve was 48.0%. Surgical outcome as evaluated by Scoliosis Research Society-22 patient questionnaires revealed acceptable results without any severe complications. Conclusions: Corrective surgery for scoliosis after a Fontan procedure becomes a stronger option if cardiac insufficiency is prevented during the perioperative period and a conservative plan is carried out with minimal invasiveness and operation time. AD - J. Takahashi, Department of Orthopaedic Surgery, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano, Japan AU - Uehara, M. AU - Takahashi, J. AU - Akazawa, Y. AU - Kuraishi, S. AU - Ikegami, S. AU - Futatsugi, T. AU - Oba, H. AU - Kato, H. DB - Embase Medline DO - 10.1016/j.jos.2017.11.004 IS - 2 KW - adolescent article child clinical article Cobb angle conservative treatment disease severity female Fontan procedure forced vital capacity human major clinical study male mental health operation duration outcome assessment perioperative period peroperative complication posterior spinal fusion postoperative period retrospective study risk factor school child scoliosis self concept spine fusion surgical patient LA - English M3 - Article N1 - L621100919 2018-03-15 2018-10-05 PY - 2018 SN - 1436-2023 0949-2658 SP - 294-298 ST - Posterior spinal fusion for scoliosis after Fontan procedure: A case series T2 - Journal of Orthopaedic Science TI - Posterior spinal fusion for scoliosis after Fontan procedure: A case series UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L621100919 http://dx.doi.org/10.1016/j.jos.2017.11.004 VL - 23 ID - 700 ER - TY - JOUR AB - An increasing number of children with congenital heart disease survive to adulthood, but many adults require surgical intervention and can present complex management challenges in the perioperative period. This review will address common considerations that surgeons, anesthesiologists, and intensivists are likely to face in caring for this growing population. AD - B. Maxwell, Department of Anesthesiology and Critical Care Medicine, Johns Hopkins Medical Institutions, 1800 Orleans Street, Baltimore, MD, United States AU - Maxwell, B. AU - Steppan, J. DB - Embase Medline DO - 10.1177/1089253214562915 IS - 2 KW - air embolism article assisted circulation Blalock Taussig shunt cardiac patient cardiac surgeon comorbidity congenital heart disease echocardiography Fontan procedure heart arrhythmia heart catheterization heart right ventricle heart single ventricle intensivist lung artery pressure palliative therapy postoperative care preoperative evaluation psychological aspect pulmonary artery stenosis reperfusion injury right ventricle to pulmonary artery conduit social aspect surgeon LA - English M3 - Article N1 - L604791174 2015-06-16 2015-06-23 PY - 2015 SN - 1940-5596 1089-2532 SP - 154-162 ST - Postoperative care of the adult with congenital heart disease T2 - Seminars in Cardiothoracic and Vascular Anesthesia TI - Postoperative care of the adult with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L604791174 http://dx.doi.org/10.1177/1089253214562915 VL - 19 ID - 863 ER - TY - JOUR AB - Life-threatening problems occur in the neonate and infant after cardiac surgery because of the interplay of diminished cardiac output (CO), increased metabolic demand, inflammatory responses to cardiopulmonary bypass, and maladaptive responses to stress. Therefore, the postoperative management of patients with complex congenital heart defects is directed at optimization of oxygen delivery to maintain end-organ function and promote wound healing. Traditionally, assessment of circulation in the postoperative congenital heart patient has depended on indirect assessment of CO using parameters such as blood pressure, pulses, capillary refill, and urine output. Because of the limitations of indirect and observer-dependent assessment of CO, we rely on objective measures of tissue oxygen levels for the complex postoperative patient. We have found that continuous monitoring of the mixed venous saturation (SvO2) allows for identification of acute changes in systemic oxygen delivery and frequently precedes other indicators of decreased CO. The postoperative patient can be expected to have a period of decreasing CO, and the need for intervention should be anticipated because critical low output syndrome will develop in a subset of patients. Strategies for postoperative care are developed based on the diagnosis and procedure, but optimizing SvO2 is a consistent goal. A uniform approach to airway maintenance, vascular access, and drug infusions, all universal concerns during the perioperative period, minimizes the potential for these predictable and necessary interventions to result in morbidity or mortality. Management of the postoperative single ventricle patient targets stabilization of the systemic vascular resistance through the use of vasodilators to improve systemic perfusion and simplify ventilator management. Management of any individual patient should be driven by objective analysis of available data and must include efforts to re-evaluate the treatment plan as well as to identify unanticipated problems. Copyright © 2002 by W.B. Saunders Company. AD - J.S. Tweddell, Division of Cardiothoracic Surgery, Children's Hospital of Wisconsin, 9000 W. Wisconsin Ave., Milwaukee, WI 53226, United States AU - Tweddell, J. S. AU - Hoffman, G. M. DB - Embase Medline DO - 10.1053/pcsu.2002.31499 KW - epinephrine amrinone beta adrenergic receptor blocking agent dobutamine dopamine glyceryl trinitrate milrinone nitroprusside sodium noradrenalin phenoxybenzamine phentolamine vasodilator agent article blood pressure cardiopulmonary bypass child circulation congenital heart disease data analysis drug antagonism drug infusion forward heart failure heart output heart single ventricle heart surgery human inflammation metabolism newborn patient care patient monitoring perfusion perioperative period postoperative care postoperative complication pulse rate surgical mortality surgical stress surgical technique tissue oxygenation treatment planning urine volume vascular access vascular resistance ventilator wound healing LA - English M3 - Article N1 - L38202157 2004-02-25 PY - 2002 SN - 1092-9126 SP - 187-205 ST - Postoperative management in patients with complex congenital heart disease T2 - Pediatric Cardiac Surgery Annual TI - Postoperative management in patients with complex congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L38202157 http://dx.doi.org/10.1053/pcsu.2002.31499 VL - 5 ID - 1312 ER - TY - JOUR AB - BACKGROUND: Mortality is a basic measure for quality and safety in anesthesia. There are few anesthesia-related mortality data available for pediatric practice. Our objective for this study was to determine the incidence of 24-hour and 30-day mortality after anesthesia and to determine the incidence and nature of anesthesia-related mortality in pediatric practice at a large tertiary institution. METHODS: Children ≤18 years old who had an anesthetic between January 1, 2003, and August 30, 2008, at the Royal Children's Hospital, Melbourne, Australia, were included for this study. Data were analyzed by merging a database for every anesthetic performed with an accurate electronic record of mortality of children who had ever been a Royal Children's Hospital patient. Cases of children dying within ≤30 days and 24 hours of an anesthetic were identified and the patient history and anesthetic record examined. Anesthesia-related death was defined as those cases whereby a panel of 3 senior anesthesiologists all agreed that anesthesia or factors under the control of the anesthesiologist more likely than not influenced the timing of death. RESULTS: During this 68-month period, 101,885 anesthetics were administered to 56,263 children. The overall 24-hour mortality from any cause after anesthesia was 13.4 per 10,000 anesthetics delivered and 30-day mortality was 34.5 per 10,000 anesthetics delivered. The incidence of death was highest in children ≤30 days old. Patients undergoing cardiac surgery had a higher incidence of 24-hour and 30-day mortality than did those undergoing noncardiac surgery. From 101,885 anesthetics there were 10 anesthesia-related deaths. The incidence of anesthesia-related death was 1 in 10,188 or 0.98 cases per 10,000 anesthetics performed (95%confidence interval, 0.5 to 1.8). In all 10 cases, preexisting medical conditions were identified as being a significant factor in the patient's death. Five of these cases (50%) involved children with pulmonary hypertension. CONCLUSIONS: Anesthesia-related mortality is higher in children with heart disease and in particular those with pulmonary hypertension. The lack of anesthetic-related deaths in children who did not have major comorbidities reinforces the safety of pediatric anesthesia in healthy children. Copyright © 2011 International Anesthesia Research Society. AD - Christchurch Hospital, Riccarton Avenue, Private Bag 4710, Christchurch, New Zealand Royal Children's Hospital, University of Melbourne, Melbourne, VIC, Australia AU - Van Der Griend, B. F. AU - Lister, N. A. AU - McKenzie, I. M. AU - Martin, N. AU - Ragg, P. G. AU - Sheppard, S. J. AU - Davidson, A. J. DB - Scopus DO - 10.1213/ANE.0b013e318213be52 IS - 6 M3 - Article N1 - Cited By :121 Export Date: 15 June 2020 PY - 2011 SP - 1440-1447 ST - Postoperative mortality in children after 101,885 anesthetics at a tertiary pediatric hospital T2 - Anesthesia and Analgesia TI - Postoperative mortality in children after 101,885 anesthetics at a tertiary pediatric hospital UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-79958159213&doi=10.1213%2fANE.0b013e318213be52&partnerID=40&md5=1da85958d883638ba6a4c26701af57f5 VL - 112 ID - 2139 ER - TY - JOUR AB - The necessity for postoperative inhaled nitric oxide (NO) therapy and predictive factors for that need were retrospectively analysed in 457 paediatric patients at risk of pulmonary hypertensive events following open-heart surgery for congenital heart disease. Inhaled NO was given postoperatively to 46% of the study group and to 23% of all patients undergoing open-heart surgery during the study period. Factors associated with increased need for postoperative NO were age <1 year, Down's syndrome, preoperative pulmonary hypertension and increased pulmonary vascular resistance. Using a multivariate model based on these factors, 73% of the patients who were given NO were identified. Thus, in a setting with unrestricted access to NO therapy, almost half of the patients with cardiac lesions that commonly give rise to postoperative pulmonary hypertension were given postoperative NO. Seventy-three percent of postoperative NO treatment was associated with a relatively small number of pre- and perioperative patient-related risk factors. AD - P.O. Laitinen, Hospital for Children/Adolescents, University of Helsinki, Stenbäckinkatu 11, FI-00290 Helsinki, Finland AU - Laitinen, P. O. AU - Räsänen, J. AU - Sairanen, H. DB - Embase Medline DO - 10.1080/14017430050142152 IS - 2 KW - nitric oxide age aortopulmonary septal defect arterial trunk article cardiomyopathy cardiopulmonary bypass child congenital heart disease controlled study Down syndrome female great vessels transposition heart atrium septum defect heart single ventricle heart ventricle septum defect human infant lung vascular resistance major clinical study male multivariate analysis open heart surgery postoperative care prediction preoperative evaluation priority journal pulmonary hypertension retrospective study risk factor LA - English M3 - Article N1 - L32241984 2001-04-10 PY - 2000 SN - 1401-7431 SP - 149-153 ST - Postoperative nitric oxide therapy in children with congenital heart disease. Can the need be predicted? T2 - Scandinavian Cardiovascular Journal TI - Postoperative nitric oxide therapy in children with congenital heart disease. Can the need be predicted? UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L32241984 http://dx.doi.org/10.1080/14017430050142152 VL - 34 ID - 1329 ER - TY - JOUR AB - Objective: The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) was established in 2008 to improve outcomes of hypoplastic left heart syndrome (HLHS) during the interstage period. They evaluated changes in patient variables and practice variation between early and late eras. Design: Data including demographic, operative, discharge, and follow-up variables from the first 100 patients (6/2008–1/2010) representing 18 centers were compared with the most recent 100 patients (1/2014–11/2014) from these same centers. Results: Prenatal diagnosis increased from 69% to 82% (P =.05). There were no differences in gestational age or weight at Norwood. A composite of any preoperative risk factor occurred more frequently in the early era (59% vs. 34%, P <.01). While mean age at Norwood was similar (8.3 vs. 6.6 days, P =.2), the standard deviation was significantly lower in the recent era (10.4–6.4 days, P =.04). Use of RV-PA conduit increased (67%–84%, P <.01). Rates of complete discharge communication with both the primary care physician (31%–97%, P <.01) and primary cardiologist (44%–97%, P <.01) increased substantially. There were limited changes in feeding strategies. Use of home monitoring program increased (76%–99%, P <.01) with all participants in the late era monitoring both oxygen saturation and weight. Conclusions: Among NPC-QIC centers contributing patients to both eras, there were significant changes in preoperative risk factors, surgical strategy, discharge communication, and interstage care. Further study is required to determine an association between these changes and decreased mortality. AD - W.F. Carlo, Department of Pediatrics, Division of Pediatric Cardiology, University of Alabama at Birmingham, Birmingham, AL, United States AU - Carlo, W. F. AU - Cnota, J. F. AU - Dabal, R. J. AU - Anderson, J. B. DB - Embase Medline DO - 10.1111/chd.12442 IS - 3 KW - article body weight cohort analysis controlled study doctor patient relationship follow up gestational age home monitoring hospital discharge human hypoplastic left heart syndrome infant infant care major clinical study Norwood procedure oxygen saturation practice guideline prenatal diagnosis preoperative period priority journal right ventricle to pulmonary artery conduit risk factor trend study LA - English M3 - Article N1 - L616624933 2017-06-09 2017-06-16 PY - 2017 SN - 1747-0803 1747-079X SP - 315-321 ST - Practice trends over time in the care of infants with hypoplastic left heart syndrome: A report from the National Pediatric Cardiology Quality Improvement Collaborative T2 - Congenital Heart Disease TI - Practice trends over time in the care of infants with hypoplastic left heart syndrome: A report from the National Pediatric Cardiology Quality Improvement Collaborative UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L616624933 http://dx.doi.org/10.1111/chd.12442 VL - 12 ID - 759 ER - TY - JOUR AB - Trisomy 18 (T18) is a genetic disorder with cardiac lesions in up to 90% of patients. Cardiac surgery is not frequently offered because of the overall poor prognosis, although this has recently been challenged. Our study aimed to explore the practices and attitudes of Canadian pediatric cardiologists managing T18 patients. We administered a survey to pediatric cardiologists attending the Canadian Cardiovascular Congress, Canadian Pediatric Cardiology Association Business Meeting. There were 30 respondents. Most (67%) supported comfort care for affected patients with a heart lesion. None supported palliative surgery for those with complex heart lesions. Of 30 respondents, 16 (53%) counsel families prenatally, and none would present the option of a single ventricle surgical track for complex heart disease. In a hypothetical situation in which their own child was born with T18, 67% would choose comfort care with medical treatment of heart failure, and none would choose palliative surgery. Being a parent was associated with a higher likelihood of choosing termination (14 of 20 vs 6 of 9; P = 0.046) or comfort care (14 of 20 vs 6 of 9; P = 0.036). Qualitative data suggest support for comfort care, while recognizing the need for individualization and shared decision-making, within the context of institution-specific policies. Canadian pediatric cardiologists surveyed support comfort care and medical treatment but not surgical treatment for T18 patients with cardiac lesions. They place primacy on nonmaleficence, yet also recognize the emerging need for individualized shared decision-making in these cases. AD - A.A. Young, IWK Health Centre, 5850/5980 University Ave, Halifax, Nova Scotia, Canada AU - Young, A. A. AU - Simpson, C. AU - Warren, A. E. DB - Embase Medline DO - 10.1016/j.cjca.2016.11.007 IS - 4 KW - adult aged article Canadian cardiac patient cardiologist clinical article female heart injury human individualization long term survival male medical practice palliative therapy patient care patient comfort physician attitude prognosis shared decision making trisomy 18 LA - English M3 - Article N1 - L614641055 2017-03-07 2017-04-06 PY - 2017 SN - 0828-282X SP - 548-551 ST - Practices and Attitudes of Canadian Cardiologists Caring for Patients With Trisomy 18 T2 - Canadian Journal of Cardiology TI - Practices and Attitudes of Canadian Cardiologists Caring for Patients With Trisomy 18 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L614641055 http://dx.doi.org/10.1016/j.cjca.2016.11.007 VL - 33 ID - 760 ER - TY - JOUR AB - Many factors will determine the quality of life at long-term follow-up after the Fontan operation. We tried to predict the long-term outcome of modified Fontan operation based on the combined assessment of various preoperative data. The preoperative condition was evaluated by a scoring system in which multilevel scores (from zero to 8 points) were given to a total of 14 morphological and physiological factors according to the contribution of each factor to mortality. The major factors were the association of the extracardiac total anomalous pulmonary venous connection, pulmonary arterial vascular resistance, mean pulmonary arterial pressure, PA index, ejection fraction of the main ventricle, and creatinine clearance. Points for each factor were determined according to surgical mortality, with zero points indicating no increased risk. The total score, i.e., the sum of the points for each item, in each patient was analyzed in relation to the patient's quality of life over a long-term follow-up, as evaluated by questionnaires. Twenty-six of 29 patients with scores of 5 or less showed normal or almost normal life long after the operation, whereas 5 of 10 with higher scores had restricted physical and daily activity. In conclusion, good preoperative condition predicts a good quality of life long after the Fontan operation. In addition, the concept of taking into account all possible risk factors as a comprehensive indicator may be useful in predicting long-term results. © 1994, The Japanese Circulation Society. All rights reserved. AD - Department of Pediatric Cardiology, Heart Institute of Japan, Tokyo Women's Medical College, 8-1 Kawada-cho, Shinjuku-ku, Tokyo, 162, Japan Departments of Pediatric Cardiology and Pediatric Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical College, Tokyo, Japan AU - Nakazawa, M. AU - Park, I. AU - Imai, Y. AU - Momma, K. AU - Sawatari, K. DB - Scopus DO - 10.1253/jcj.58.646 IS - 8 KW - Long-term follow-up Modified Fontan procedure Quality of life M3 - Article N1 - Cited By :8 Export Date: 15 June 2020 PY - 1994 SP - 646-652 ST - Prediction of quality of life at long-term follow-up after fontan operation by scoring risk factors T2 - JAPANESE CIRCULATION JOURNAL TI - Prediction of quality of life at long-term follow-up after fontan operation by scoring risk factors UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0028122524&doi=10.1253%2fjcj.58.646&partnerID=40&md5=41cc3b0af18e714b76a72e874d815846 VL - 58 ID - 2309 ER - TY - JOUR AB - Many factors will determine the quality of life at long-term follow-up after the Fontan operation. We tried to predict the long-term outcome of modified Fontan operation based on the combined assessment of various preoperative data. The preoperative condition was evaluated by a scoring system in which multilevel scores (from zero to 8 points) were given to a total of 14 morphological and physiological factors according to the contribution of each factor to mortality. The major factors were the association of the extracardiac total anomalous pulmonary venous connection, pulmonary arterial vascular resistance, mean pulmonary arterial pressure, PA index, ejection fraction of the main ventricle, and creatinine clearance. Points for each factor were determined according to surgical mortality, with zero points indicating no increased risk. The total score, i.e., the sum of the points for each item, in each patient was analyzed in relation to the patient's quality of life over a long-term follow-up, as evaluated by questionnaires. Twenty-six of 29 patients with scores of 5 or less showed normal or almost normal life long after the operation, whereas 5 of 10 with higher scores had restricted physical and daily activity. In conclusion, good preoperative condition predicts a good quality of life long after the Fontan operation. In addition, the concept of taking into account all possible risk factors as a comprehensive indicator may be useful in predicting long-term results. AD - Department of Pediatric Cardiology, Heart Institute of Japan, Tokyo. AN - 7967006 AU - Park, I. AU - Nakazawa, M. AU - Imai, Y. AU - Sawatari, K. AU - Momma, K. DA - Aug DB - PubMed DO - 10.1253/jcj.58.646 DP - NLM ET - 1994/08/01 IS - 8 KW - Adolescent Adult Child Follow-Up Studies *Fontan Procedure/mortality Humans Prognosis *Quality of Life Risk Factors LA - eng N1 - Park, I Nakazawa, M Imai, Y Sawatari, K Momma, K Journal Article Japan Jpn Circ J. 1994 Aug;58(8):646-52. doi: 10.1253/jcj.58.646. PY - 1994 SN - 0047-1828 (Print) 0047-1828 SP - 646-52 ST - Prediction of quality of life at long-term follow-up after Fontan operation by scoring risk factors T2 - Jpn Circ J TI - Prediction of quality of life at long-term follow-up after Fontan operation by scoring risk factors VL - 58 ID - 417 ER - TY - JOUR AB - Neurodevelopmental outcomes after the Norwood procedure for single right ventricular lesions are worse than those in the normal population. It would be valuable to identify which patients at the time of Norwood discharge are at greatest risk for neurodevelopmental impairment later in childhood. As such, this study sought to construct and validate a model to predict poor neurodevelopmental outcome using variables readily available to the clinician. Using data from the 14 month neurodevelopmental outcome of the Single-Ventricle Reconstruction (SVR) trial, a classification and regression tree (CART) analysis model was developed to predict severe neurodevelopmental impairment, defined as a Psychomotor Development Index (PDI) score lower than 70 on the Bayley Scales of Infant Development-II. The model then was validated using data from subjects enrolled in the Infant Single Ventricle (ISV) trial. The PDI scores were lower than 70 for 138 (44 %) of 313 subjects. Predictors of a PDI lower than 70 were post-Norwood intensive care unit (ICU) stay longer than 46 days, genetic syndrome or other anomalies, birth weight less than 2.7 kg, additional cardiac surgical procedures, and use of five or more medications at hospital discharge. Using these risk factors, the CART model correctly identified 75 % of SVR subjects with a PDI lower than 70. When the CART model was applied to 70 subjects from the ISV trial, the correct classification rate was 67 %. This model of variables from the Norwood hospitalization can help to identify infants at risk for neurodevelopmental impairment. However, given the overall high prevalence of neurodevelopmental impairment and the fact that nearly one third of severely affected children would not have been identified by these risk factors, close surveillance and assessment for early intervention services are warranted for all infants after the Norwood procedure. AD - Children's Healthcare of Atlanta and Emory University School of Medicine, 1405 Clifton Road NE, Atlanta, GA 30322, USA. MahleW@kidsheart.com AN - 22864647 AU - Mahle, W. T. AU - Lu, M. AU - Ohye, R. G. AU - William Gaynor, J. AU - Goldberg, C. S. AU - Sleeper, L. A. AU - Pemberton, V. L. AU - Mussatto, K. A. AU - Williams, I. A. AU - Sood, E. AU - Krawczeski, C. D. AU - Lewis, A. AU - Mirarchi, N. AU - Scheurer, M. AU - Pasquali, S. K. AU - Pinto, N. AU - Jacobs, J. P. AU - McCrindle, B. W. AU - Newburger, J. W. C2 - PMC3505274 C6 - NIHMS398697 DA - Feb DB - PubMed DO - 10.1007/s00246-012-0450-1 DP - NLM ET - 2012/08/07 IS - 2 KW - *Child Development Developmental Disabilities/diagnosis/*epidemiology/etiology Female Humans Hypoplastic Left Heart Syndrome/mortality/*surgery Incidence Infant Male *Models, Neurological Neuropsychological Tests *Norwood Procedures Postoperative Complications Prognosis Risk Assessment/*methods Risk Factors Survival Rate/trends United States/epidemiology LA - eng N1 - 1432-1971 Mahle, William T Lu, Minmin Ohye, Richard G William Gaynor, J Goldberg, Caren S Sleeper, Lynn A Pemberton, Victoria L Mussatto, Kathleen A Williams, Ismee A Sood, Erica Krawczeski, Catherine Dent Lewis, Alan Mirarchi, Nicole Scheurer, Mark Pasquali, Sara K Pinto, Nelangi Jacobs, Jeffrey P McCrindle, Brian W Newburger, Jane W K08 HL103631/HL/NHLBI NIH HHS/United States HL068288/HL/NHLBI NIH HHS/United States HL068285/HL/NHLBI NIH HHS/United States U10 HL109781/HL/NHLBI NIH HHS/United States U01 HL068269/HL/NHLBI NIH HHS/United States U10 HL109816/HL/NHLBI NIH HHS/United States UL1 TR000062/TR/NCATS NIH HHS/United States P30 HD018655/HD/NICHD NIH HHS/United States U01 HL068279/HL/NHLBI NIH HHS/United States U01 HL068290/HL/NHLBI NIH HHS/United States HL068279/HL/NHLBI NIH HHS/United States U01 HL068288/HL/NHLBI NIH HHS/United States U10 HL068270/HL/NHLBI NIH HHS/United States U01 HL068281/HL/NHLBI NIH HHS/United States U01 HL068270/HL/NHLBI NIH HHS/United States HL085057/HL/NHLBI NIH HHS/United States UL1 TR000055/TR/NCATS NIH HHS/United States HL068281/HL/NHLBI NIH HHS/United States U01 HL068292/HL/NHLBI NIH HHS/United States HL068269/HL/NHLBI NIH HHS/United States HL068270/HL/NHLBI NIH HHS/United States U01 HL085057/HL/NHLBI NIH HHS/United States HL068290/HL/NHLBI NIH HHS/United States U01 HL068285/HL/NHLBI NIH HHS/United States HL068292/HL/NHLBI NIH HHS/United States Comparative Study Journal Article Randomized Controlled Trial Research Support, N.I.H., Extramural Pediatr Cardiol. 2013 Feb;34(2):327-33. doi: 10.1007/s00246-012-0450-1. Epub 2012 Aug 3. PY - 2013 SN - 0172-0643 (Print) 0172-0643 SP - 327-33 ST - A predictive model for neurodevelopmental outcome after the Norwood procedure T2 - Pediatr Cardiol TI - A predictive model for neurodevelopmental outcome after the Norwood procedure VL - 34 ID - 381 ER - TY - JOUR AB - Background: Postnatal depression is a major public health problem. The aim of this study is to validate the use of the Edinburgh Postnatal Depression Scale (EPDS) in the early postpartum, and to identify the markers for risk of postnatal depression. Methods: 815 women filled out an EPDS and general information questionnaire between the third and the fifth day postpartum. The women with an EPDS score of >8 and a randomized control group from those with scores of <8 were contacted 8 weeks postpartum. 363 women therefore had a structured diagnostic interview by telephone at 8 weeks postpartum (MINI-DSM-IV), without knowledge of their EPDS scores, to screen for a major or minor depressive episode. Results: The sensitivity of EPDS was measured as 0.82 [0.78-0.86], with a positivity threshold of 9.5/30. For an estimated prevalence for all depressive episodes of 16.1%, the positive predictive value of EPDS was measured as 42.8% [39.1-46.5%]. Multivariate risk analysis using logistical regression identified the following as risk markers for postnatal depression: previous history of depression (postnatal or other), unemployment, premature delivery or stopping breast-feeding in the first month for nonmedical reasons. Conclusion: The use of EPDS between the third and fifth day postpartum is valid. An EPDS score of N10 should be completed by a clinical assessment and suitable management. The risk markers identified here are clinical indices that can be used for first-line early screening by non-psychiatric health workers. (PsycINFO Database Record (c) 2019 APA, all rights reserved) AD - Jardri, Renaud, Hopital Michel Fontan, Centre Hospitalier Universitaire de Lille, 59 037, Lille, France AN - 2006-07896-020 AU - Jardri, Renaud AU - Pelta, Jerome AU - Maron, Michel AU - Thomas, Pierre AU - Delion, Pierre AU - Codaccioni, Xavier AU - Goudemand, Michel DB - psyh DO - 10.1016/j.jad.2006.03.009 DP - EBSCOhost IS - 1-3 KW - postnatal depression Edinburgh Postnatal Depression Scale predictive validity risk analysis Adolescent Adult Breast Feeding Depression, Postpartum Depressive Disorder, Major Female Humans Obstetric Labor, Premature Personality Inventory Pregnancy Psychometrics Recurrence Reproducibility of Results Risk Factors Unemployment Postpartum Depression Screening Tests Test Validity Risk Assessment At Risk Populations N1 - Hopital Michel Fontan, Centre Hospitalier Universitaire de Lille, Lille, France. Release Date: 20060626. Correction Date: 20190211. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Postpartum Depression; Predictive Validity; Screening Tests; Test Validity; Risk Assessment. Minor Descriptor: At Risk Populations. Classification: Clinical Psychological Testing (2224); Affective Disorders (3211). Population: Human (10); Female (40). Location: France. Age Group: Adolescence (13-17 yrs) (200); Adulthood (18 yrs & older) (300); Young Adulthood (18-29 yrs) (320); Thirties (30-39 yrs) (340); Middle Age (40-64 yrs) (360). Tests & Measures: Edinburgh Postnatal Depression Scale DOI: 10.1037/t01756-000; Mini International Neuropsychiatric Interview DOI: 10.1037/t18597-000. Methodology: Empirical Study; Quantitative Study. References Available: Y. Page Count: 8. Issue Publication Date: Jul, 2006. PY - 2006 SN - 0165-0327 1573-2517 SP - 169-176 ST - Predictive validation study of the Edinburgh Postnatal Depression Scale in the first week after delivery and risk analysis for postnatal depression T2 - Journal of Affective Disorders TI - Predictive validation study of the Edinburgh Postnatal Depression Scale in the first week after delivery and risk analysis for postnatal depression UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2006-07896-020&site=ehost-live&scope=site ORCID: 0000-0003-4596-1502 r.jardri@free.fr VL - 93 ID - 1695 ER - TY - JOUR AB - Purpose: Our aim was to investigate the factors associated with mother–child separation at discharge, after joint hospitalization in psychiatric mother–baby units (MBUs) in France and Belgium. Because parents with postpartum psychiatric disorders are at risk of disturbed parent–infant interactions, their infants have an increased risk of an unstable early foundation. They may be particularly vulnerable to environmental stress and have a higher risk of developing some psychiatric disorders in adulthood. Methods: This prospective longitudinal study of 1,018 women with postpartum psychiatric disorders, jointly admitted with their infant to 16 French and Belgian psychiatric mother–baby units (MBUs), used multifactorial logistic regression models to assess the risk factors for mother–child separation at discharge from MBUs. Those factors include some infant characteristics associated with personal vulnerability, parents’ pathology and psychosocial context. Results: Most children were discharged with their mothers, but 151 (15 %) were separated from their mothers at discharge. Risk factors independently associated with separation were: (1) neonatal or infant medical problems or complications; (2) maternal psychiatric disorder; (3) paternal psychiatric disorder; (4) maternal lack of good relationship with others; (5) mother receipt of disability benefits; (6) low social class. Conclusions: This study highlights the existence of factors other than maternal pathology that lead to decisions to separate mother and child for the child’s protection in a population of mentally ill mothers jointly hospitalized with the baby in the postpartum period. (PsycINFO Database Record (c) 2019 APA, all rights reserved) AD - Glangeaud-Freudenthal, Nine M.-C., INSERM, UMRS 953, Maternite de Port Royal, 53 Avenue de l'Observatoire, 75014, Paris, France AN - 2013-09378-005 AU - Glangeaud-Freudenthal, Nine M. C. AU - Sutter-Dallay, Anne-Laure AU - Thieulin, Anne-Claire AU - Dagens, Véronique AU - Zimmermann, Marie-Agathe AU - Debourg, Alain AU - Amzallag, Corinne AU - Cazas, Odile AU - Cammas, Rafaële AU - Klopfert, Marie-Emmanuelle AU - Rainelli, Christine AU - Tielemans, Pascale AU - Mertens, Claudine AU - Maron, Michel AU - Nezelof, Sylvie AU - Poinso, François DB - psyh DO - 10.1007/s00127-012-0527-4 DP - EBSCOhost IS - 4 KW - infant foster care postpartum psychiatric disorders mother child separation hospitalization risk factors Adult Belgium Depression, Postpartum Female Foster Home Care France Humans Infant Welfare Infant, Newborn Interpersonal Relations Mental Disorders Mother-Child Relations Mothers Postnatal Care Psychiatric Status Rating Scales Social Class Foster Care Postpartum Psychosis Child Care N1 - INSERM, UMRS 953, Maternite de Port Royal, Paris, France. Release Date: 20130715. Correction Date: 20190121. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Foster Care; Hospitalization; Mental Disorders; Postpartum Psychosis; Risk Factors. Minor Descriptor: Child Care. Classification: Psychological Disorders (3210); Community & Social Services (3373). Population: Human (10); Female (40); Inpatient (50). Location: Belgium; France. Age Group: Adolescence (13-17 yrs) (200); Adulthood (18 yrs & older) (300); Young Adulthood (18-29 yrs) (320); Thirties (30-39 yrs) (340); Middle Age (40-64 yrs) (360). Tests & Measures: Marce Clinical Checklist-French Version. Methodology: Empirical Study; Longitudinal Study; Prospective Study; Quantitative Study. References Available: Y. Page Count: 9. Issue Publication Date: Apr, 2013. Publication History: First Posted Date: Jun 16, 2012; Accepted Date: May 22, 2012; First Submitted Date: Mar 10, 2011. Copyright Statement: Springer-Verlag. 2012. Sponsor: Francophone Marcé Society. Recipients: No recipient indicated PY - 2013 SN - 0933-7954 1433-9285 SP - 553-561 ST - Predictors of infant foster care in cases of maternal psychiatric disorders T2 - Social Psychiatry and Psychiatric Epidemiology: The International Journal for Research in Social and Genetic Epidemiology and Mental Health Services TI - Predictors of infant foster care in cases of maternal psychiatric disorders UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2013-09378-005&site=ehost-live&scope=site francois.poinso@ap-hm.fr snezelof@chu-besancon.fr m-maron@chru-lille.fr claudine.mertens@sint-camillus.be pascaletielemans@clairsvallons.be christine.rainelli@ch-esquirol-limoges.fr marie-emmanuelle.klopfert@chjt.be rcammas@ch-maison-blanche.fr odile.cazas@pbr.aphp.fr corinne.amzallag@chicreteil.fr alain.debourg@hopital-levesinet.fr Marie-Agathe.Zimmermann@chru-strasbourg.fr v.dagens@th-roussel.fr alsutter@ch-perrens.fr nine.glangeaud@inserm.fr VL - 48 ID - 1687 ER - TY - JOUR AB - Background: Congenital cardiac magnetic resonance is a limited resource because of scanner and physician availability. Missed appointments decrease scheduling efficiency, have financial implications and represent missed care opportunities. Objective: To characterize the rate of missed appointments and identify modifiable predictors. Materials and methods: This single-center retrospective study included all patients with outpatient congenital or pediatric cardiac MR appointments from Jan. 1, 2014, through Dec. 31, 2015. We identified missed appointments (no-shows or same-day cancellations) from the electronic medical record. We obtained demographic and clinical factors from the medical record and assessed socioeconomic factors by U.S. Census block data by patient ZIP code. Statistically significant variables (P<0.05) were included into a multivariable analysis. Results: Of 795 outpatients (median age 18.5 years, interquartile range 13.4–27.1 years) referred for congenital cardiac MR, a total of 91 patients (11.4%) missed appointments; 28 (3.5%) missed multiple appointments. Reason for missed appointment could be identified in only 38 patients (42%), but of these, 28 (74%) were preventable or could have been identified prior to the appointment. In multivariable analysis, independent predictors of missed appointments were referral by a non-cardiologist (adjusted odds ratio [AOR] 5.8, P=0.0002), referral for research (AOR 3.6, P=0.01), having public insurance (AOR 2.1, P=0.004), and having scheduled cardiac MR from November to April (AOR 1.8, P=0.01). Conclusion: Demographic factors can identify patients at higher risk for missing appointments. These data may inform initiatives to limit missed appointments, such as targeted education of referring providers and patients. Further data are needed to evaluate the efficacy of potential interventions. AD - J.C. Lu, Department of Pediatrics and Communicable Diseases, Division of Pediatric Cardiology, University of Michigan Health System, University of Michigan Congenital Heart Center, C. S. Mott Children’s Hospital, 1540 E. Hospital Drive, Ann Arbor, MI, United States AU - Lu, J. C. AU - Lowery, R. AU - Yu, S. AU - Ghadimi Mahani, M. AU - Agarwal, P. P. AU - Dorfman, A. L. DB - Embase Medline DO - 10.1007/s00247-017-3851-8 IS - 8 KW - adolescent adult aortic arch anomaly article cardiologist cardiomyopathy cardiovascular magnetic resonance congenital heart disease electronic medical record Fallot tetralogy female great vessels transposition heart single ventricle high risk patient human lung insufficiency major clinical study male outpatient patient attendance patient care patient referral pediatric cardiology pediatric hospital priority journal pulmonary valve stenosis retrospective study LA - English M3 - Article N1 - L615665335 2017-05-01 2017-07-04 PY - 2017 SN - 1432-1998 0301-0449 SP - 911-916 ST - Predictors of missed appointments in patients referred for congenital or pediatric cardiac magnetic resonance T2 - Pediatric Radiology TI - Predictors of missed appointments in patients referred for congenital or pediatric cardiac magnetic resonance UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L615665335 http://dx.doi.org/10.1007/s00247-017-3851-8 VL - 47 ID - 744 ER - TY - JOUR AN - 106525911. Language: English. Entry Date: 20051014. Revision Date: 20150711. Publication Type: Journal Article AU - Catlin, A. DB - ccm DP - EBSCOhost IS - 2 KW - Perinatal Care -- Ethical Issues Fathers Female Hypoplastic Left Heart Syndrome -- Therapy Infant, Newborn Intensive Care Units, Neonatal -- Ethical Issues Multiple Offspring -- Ethical Issues Pregnancy Pregnancy, Multiple -- Ethical Issues Serial Publications Sexism Substance Abuse, Perinatal -- Ethical Issues N1 - brief item. Journal Subset: Core Nursing; Nursing; USA. NLM UID: 101126037. PY - 2005 SN - 1527-3369 SP - 59-59 ST - Preface T2 - Newborn & Infant Nursing Reviews TI - Preface UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106525911&site=ehost-live&scope=site VL - 5 ID - 1655 ER - TY - JOUR AB - BACKGROUND AND AIMS: The fetal diagnosis of complex congenital heart disease (CHD) leads to a crisis for the affected couple. How much more so when a decision is made to terminate the pregnancy? In making that decision do the parents understand the anomaly, its consequences and possible outcome and does that information influence their decision process? METHODS AND SUBJECTS: Questionnaires were developed. They were forwarded to parents seen in a private obstetric ultrasound practice, diagnosed with, or specifically referred, because of the diagnosis of a complex cardiac anomaly in the fetus. The diagnosis was subsequently confirmed by ultrasound examination in the presence of a paediatric cardiologist, in all but one patient. Once confirmed, both the obstetric ultrasonologist and cardiologist explained the anomaly, both working off each other, the obstetrician acting as the parent's advocate, asking and "demanding" explanations in lay terms, clarifying the nature of the anomaly, its possible consequences in the newborn and childhood, the need for intervention, and the short- and long-term risks, concentrating on quality of life issues. The parents were given the opportunity to meet with the cardiologist separately to clarify the diagnosis and management, before coming to a final decision to continue or terminate the pregnancy. RESULTS: Over a 3-year period, 40 such patients were included in the study, of which 13 opted for termination. The anomalies were complex and included hypoplastic left heart syndrome, univentricular heart and atrioventricular septal defect. The three autopsies carried out confirmed the prenatal diagnosis. Completed questionnaires were received from nine couples, two being lost to follow up and two refusing to complete the questionnaire despite telephone contact. All nine responses stated the explanation given was "very clear" or "clear", the detail covered was "just right", and their understanding of the diagnosis was "good " to "excellent". Eight stated they were very distressed following the diagnosis, and seven experienced great difficulty in arriving at a decision. In contrast, two stated that the decision was "easy" or "very easy". The reasons for termination included concern for the welfare of the affected child, other siblings in the family and because of the distress of the parents themselves. Intellectual disability was given as an additional reason in the three who had associated chromosomal abnormalities. CONCLUSIONS: Although the numbers were small, it would appear that the above approach used by an obstetric ultrasonologist and cardiologist, working together, explaining complex congenital heart disease in lay terms and focusing on the need for intervention, functional outcome and quality of life issues, provided the parents with a reasonable understanding of the anomaly. Nevertheless, the decision to terminate, very difficult for nearly all the couples, seemed to be based on the perceived distress of the affected infant/child, the siblings and parents themselves. While three couples were grateful for an opportunity to respond, two refused, suggesting the need for more detailed follow up and support for those parents who decide to terminate an apparently wanted pregnancy. AD - Paediatric Cardiology Unit, Monash Medical Centre, Melbourne, Australia. menahems@cryptic.rch.unimelb.edu.au AN - 12932895 AU - Menahem, S. AU - Grimwade, J. DA - Aug DB - PubMed DO - 10.1016/s0378-3782(03)00078-1 DP - NLM ET - 2003/08/23 IS - 1-2 KW - *Abortion, Eugenic Adult Comprehension *Decision Making Female *Heart Defects, Congenital/diagnosis/psychology Humans Parents/*psychology Patient Acceptance of Health Care/*psychology Pregnancy *Prenatal Diagnosis Surveys and Questionnaires Empirical Approach Genetics and Reproduction LA - eng N1 - Menahem, Samuel Grimwade, James Journal Article Ireland Early Hum Dev. 2003 Aug;73(1-2):71-8. doi: 10.1016/s0378-3782(03)00078-1. PY - 2003 SN - 0378-3782 (Print) 0378-3782 SP - 71-8 ST - Pregnancy termination following prenatal diagnosis of serious heart disease in the fetus T2 - Early Hum Dev TI - Pregnancy termination following prenatal diagnosis of serious heart disease in the fetus VL - 73 ID - 354 ER - TY - JOUR AB - Management of thoracopagus conjoined twins is predominantly determined by the associated complex cardiovascular abnormalities seen in the majority of these cases. Magnetic resonance imaging (MRI), including three-dimensional contrast-enhanced magnetic resonance angiography (MRA) provides a useful complement to conventional cardiac echocardiography or catheterization for detailed evaluation of the extra-cardiac anatomy. We report a pair of female thoracopagus conjoined twins with a shared six-chambered heart. The MRA findings are highlighted. © Springer-Verlag 2004. AD - Department of Pediatrics, Wilford Hall Medical Center, 2200 Berquist Dr, Lackland AFB, TX 78236, United States Department of Radiology, Wilford Hall Medical Center, 2200 Berquist Dr, Lackland AFB, TX 78236, United States AU - McAdams, R. M. AU - Milhoan, K. A. AU - Hall, B. H. AU - Richardson, R. G. DB - Scopus DO - 10.1007/s00247-004-1202-z IS - 10 KW - Conjoined twins MR Thoracopagus M3 - Article N1 - Cited By :9 Export Date: 15 June 2020 PY - 2004 SP - 816-819 ST - Prenatal and postnatal imaging thoracopagus conjoined twins with a shared six-chamber heart T2 - Pediatric Radiology TI - Prenatal and postnatal imaging thoracopagus conjoined twins with a shared six-chamber heart UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-5744228407&doi=10.1007%2fs00247-004-1202-z&partnerID=40&md5=0c2b205109a9cbc575a2236f837d8c35 VL - 34 ID - 2250 ER - TY - JOUR AB - Advances in prenatal imaging allow early detection of single-ventricle congenital heart disease, which may enhance prenatal care and maximize care options and decision making. Boston Children's Hospital's Advanced Fetal Care Center and fetal cardiology program provide prenatal counseling and care for single-ventricle congenital heart disease. Key points for optimal prenatal counseling and education include explanation of the diagnosis, delivery, the first surgery, cardiac neurodevelopmental issues, feeding and growth issues, quality of life and long-term care, family stressors, and fetal cardiac intervention. Such counseling and education help families make the difficult decisions required in this situation. AD - Terra Lafranchi is a nurse practitioner in the Department of Cardiology at Boston Children's Hospital. She is the fetal cardiology coordinator and also provides longitudinal pediatric cardiac care.Patricia Lincoln is a clinical nurse specialist in the cardiovascular intensive care unit at Boston Children's Hospital. terra.lafranchi@cardio.chboston.org. Terra Lafranchi is a nurse practitioner in the Department of Cardiology at Boston Children's Hospital. She is the fetal cardiology coordinator and also provides longitudinal pediatric cardiac care.Patricia Lincoln is a clinical nurse specialist in the cardiovascular intensive care unit at Boston Children's Hospital. AN - 26427975 AU - Lafranchi, T. AU - Lincoln, P. DA - Oct DB - PubMed DO - 10.4037/ccn2015247 DP - NLM ET - 2015/10/03 IS - 5 KW - Counseling/*methods Delivery, Obstetric Female Fetus Heart Diseases/*congenital/diagnosis/*therapy *Hospitals Humans Intensive Care Units Pregnancy Prenatal Care/*methods *Prenatal Diagnosis Prognosis LA - eng N1 - 1940-8250 Lafranchi, Terra Lincoln, Patricia Journal Article United States Crit Care Nurse. 2015 Oct;35(5):53-61. doi: 10.4037/ccn2015247. PY - 2015 SN - 0279-5442 SP - 53-61 ST - Prenatal Counseling and Care for Single-Ventricle Heart Disease: One Center's Model for Care T2 - Crit Care Nurse TI - Prenatal Counseling and Care for Single-Ventricle Heart Disease: One Center's Model for Care VL - 35 ID - 70 ER - TY - JOUR AB - Objectives: To evaluate the prenatal features, associated anomalies and the postnatal management in fetuses with prenatally detected double-outlet right ventricle (DORV). Methods: Retrospective analysis of prenatal, pediatric and cardiosurgical records for all fetuses with prenatally diagnosed DORV at a single tertiary referral center. Results: 21 cases were detected prenatally with DORV (average gestational age 23(3/7) weeks). Fetal karyotyping was available in all cases revealing 3 cases with trisomy 18 and one fetus with trisomy 21. 10/21 (47.6%) cases had additional cardiac anomalies only, one fetus (4.8%) had extra-cardiac anomalies only. 8/21 cases (38.1%) presented with both additional cardiac- and extra-cardiac anomalies. A total of 13/21 (61.9%) fetuses were live-born. Average gestational age at delivery was 37(3/7) gestational weeks. There were four Rastelli maneuvers, one Fontan intervention and three children with resection of a coarctation of the aorta among the group of the surviving infants. Conclusions: Prenatal, sonographic detection of DORV is feasible. The majority of the cases shows a normal karyotype. Outcome strongly depends on the presence and severity of additional anomalies of the fetus. Surgical intervention in DORV can lead to a favorable outcome in simple DORV. DORV not aggravated by additional defects seems to be no contraindication for a vaginal delivery. AD - Department of Prenatal Medicine, University of Schleswig-Holstein Medical Center. Luebeck, 23538, Germany Department of Prenatal Medicine, University of Schleswig-Holstein Medical Center , Luebeck, 23538 , Germany. AN - 104618501. Language: English. Entry Date: 20120511. Revision Date: 20170411. Publication Type: journal article AU - Hartge, D. R. AU - Niemeyer, L. AU - Axt-Fliedner, R. AU - Krapp, M. AU - Gembruch, U. AU - Germer, U. AU - Weichert, J. AU - Hartge, David Rafael AU - Niemeyer, Linda AU - Axt-Fliedner, Roland AU - Krapp, Martin AU - Gembruch, Ulrich AU - Germer, Ute AU - Weichert, Jan DB - ccm DO - 10.3109/14767058.2011.561387 DP - EBSCOhost IS - 1 KW - Transposition of Great Arteries -- Ultrasonography Ultrasonography, Prenatal Chromosomes Transposition of Great Arteries Transposition of Great Arteries -- Surgery Down Syndrome -- Complications Down Syndrome Female Gestational Age Human Infant, Newborn Karyotyping Male Pregnancy Pregnancy Outcomes Retrospective Design Chromosome Disorders N1 - case study; research. Journal Subset: Biomedical; Europe; UK & Ireland. Special Interest: Obstetric Care. NLM UID: 101136916. PMID: NLM21410422. PY - 2012 SN - 1476-7058 SP - 58-63 ST - Prenatal detection and postnatal management of double outlet right ventricle (DORV) in 21 singleton pregnancies T2 - Journal of Maternal-Fetal & Neonatal Medicine TI - Prenatal detection and postnatal management of double outlet right ventricle (DORV) in 21 singleton pregnancies UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104618501&site=ehost-live&scope=site VL - 25 ID - 1546 ER - TY - JOUR AB - An online survey for parents of children with congenital heart disease (CHD) was developed to study parents' experiences at the time of diagnosis. The survey was distributed to online support groups. A total of 841 responses from parents of children with CHD were received during a 4-week period. The current study examined those respondents (211 [25 %]) who reported their child's diagnosis as hypoplastic left heart syndrome (HLHS). Among these, 138 (65 %) reported receiving the diagnosis prenatally. 32 % of those receiving a prenatal diagnosis reported that after they declined to terminate the pregnancy, termination was mentioned again by their physicians. Parents who had termination mentioned again after their initial decline reported significantly lower optimism regarding their child's life expectancy than those who did not have it mentioned again (66 vs. 94 %, p < 0.001); were more likely to interpret the term "rare" to mean "little or no chance of survival" (34 vs. 13 %, p = 0.01); and were more likely to change pediatric cardiologists (PCs) (43 vs. 12 %, p < 0.001). Similarly, 22 % of respondents receiving a prenatal diagnosis reported feeling pressure to terminate the pregnancy by the PC. Those who felt pressure to terminate reported lower optimism about their child's life expectancy than respondents who did not feel pressure (48 vs. 88 %, p < 0.001) and were more likely to choose a new PC (48 vs. 17 %, p < 0.001). In our cohort of parents, when termination of pregnancy was mentioned after the parents declined it, or if the parents felt pressure to terminate, the parents perceived a lower chance of survival, felt less optimistic about their child's life expectancy, and were more likely to choose another PC for long-term follow-up care. Our study could not determine whether repeated discussions of the possibility for termination of pregnancy independently impacts parental optimism regarding prognosis or whether those who counsel with repeated discussions of termination tend to have more guarded notions of the prognosis of children with HLHS. Further study is warranted to identify the implications of counseling patterns on parental perceptions and decisions regarding termination of pregnancy. AD - California Heart Connection, Irvine, CA, USA. debhilton@aol.com AN - 22618584 AU - Hilton-Kamm, D. AU - Chang, R. K. AU - Sklansky, M. DA - Dec DB - PubMed DO - 10.1007/s00246-012-0366-9 DP - NLM ET - 2012/05/24 IS - 8 KW - Abortion, Induced/*psychology *Counseling *Decision Making Female Humans Hypoplastic Left Heart Syndrome/*diagnostic imaging Internet Parents/*psychology Pregnancy *Prenatal Diagnosis Surveys and Questionnaires Ultrasonography LA - eng N1 - 1432-1971 Hilton-Kamm, Debra Chang, Ruey-Kang Sklansky, Mark Journal Article United States Pediatr Cardiol. 2012 Dec;33(8):1402-10. doi: 10.1007/s00246-012-0366-9. Epub 2012 May 23. PY - 2012 SN - 0172-0643 SP - 1402-10 ST - Prenatal diagnosis of hypoplastic left heart syndrome: impact of counseling patterns on parental perceptions and decisions regarding termination of pregnancy T2 - Pediatr Cardiol TI - Prenatal diagnosis of hypoplastic left heart syndrome: impact of counseling patterns on parental perceptions and decisions regarding termination of pregnancy VL - 33 ID - 268 ER - TY - JOUR AB - Background: Naphthalene is one of the abused inhalants. It has been associated with acute and chronic health problems. To the authors' knowledge, prenatal exposure to naphthalene has never been discussed in humans. Case: The authors discuss a case of naphthalene-addicted pregnant women with multiple fetal anomalies. At 15 weeks gestation, ultrasound screening demostrated multiple fetal anomalies: anencephaly, scoliosis, diffuse subcutaneous edema, flexion contracture of lower extremities, and hypoplastic left ventricle. Four weeks later obstetrical ultrasonography revealed that there was no fetal cardiac activity. The patient had a medical abortion. Conclusion: A stronger knowledge basis regarding naphthalene-related fetal anomaly is required to ensure accurate direct link, however the probability of naphthalene-related fetal anomaly must be considered. AD - F.K. Boynukalin, Cadde 109/2 - Bahçelievler, 06500 Ankara, Turkey AU - Boynukalin, F. K. AU - Baykal, C. DB - Embase Medline DO - 10.12891/ceog16602014 IS - 2 KW - naphthalene adult anencephalus article autopsy case report crown rump length drug dependence edema female fetus fetus echography fetus malformation flexion contracture gestational age heart left ventricle human hypoplastic left heart syndrome leg medical abortion menstrual cycle multiple malformation syndrome nausea and vomiting physical examination pleura effusion pregnant woman prenatal diagnosis prenatal drug exposure scoliosis young adult LA - English M3 - Article N1 - L372869978 2014-04-29 2014-05-07 PY - 2014 SN - 0390-6663 SP - 217-218 ST - Prenatal diagnosis of multiple fetal anomalies in naphthalene-addicted pregnant women: A case report T2 - Clinical and Experimental Obstetrics and Gynecology TI - Prenatal diagnosis of multiple fetal anomalies in naphthalene-addicted pregnant women: A case report UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L372869978 http://dx.doi.org/10.12891/ceog16602014 VL - 41 ID - 926 ER - TY - JOUR AB - OBJECTIVES: To review our 13-year experience with prenatally detected hypoplastic left heart syndrome (HLHS) of which management remains controversial. MATERIAL AND METHODS: Retrospective study of the management and outcome in all cases of HLHS diagnosed prenatally in a tertiary referral center for pediatric cardiology and cardiac surgery between January 1988 and July 2001. RESULTS: The diagnosis of HLHS was made in 32 fetuses. One mother had two pregnancies associated with HLHS. In 16 cases parents opted for termination of pregnancy and in five for compassionate care. Four fetuses died in utero, and seven patients received a palliative reconstructive Norwood procedure. In seven fetuses, associated anomalies were detected: three chromosomal and structural and four only structural. In six fetuses, other associated intracardiac anomalies were detected. Of seven infants operated, six had no associated anomalies and only one is alive at an age of 17 months. CONCLUSION: The low percentage of intention to treat among patients in our center (34%) is in accordance with the percentage found in another study from the UK (36.2%), but differs significantly from reported series across the Atlantic (67%). Prenatal diagnosis of the HLHS provides opportunities not only for getting patients in optimal preoperative condition when surgery is offered, but also for in-depth counseling of the parents on this severe malformation. A minority of parents faced with the difficult decision of possible termination of pregnancy, compassionate care or the Norwood strategy, choose surgical treatment which might be based on socioreligious differences and the interpretation of the long-term quality of life. AD - Children's Heart Center, Wilhelmina Children's Hospital, UMC Utrecht, The Netherlands. AN - 12756482 AU - Verheijen, P. M. AU - Lisowski, L. A. AU - Plantinga, R. F. AU - Hitchcock, J. F. AU - Bennink, G. B. AU - Stoutenbeek, P. AU - Meijboom, E. J. DA - May DB - PubMed DO - 10.1007/s00059-003-2439-2 DP - NLM ET - 2003/05/21 IS - 3 KW - *Echocardiography Female Follow-Up Studies Humans Hypoplastic Left Heart Syndrome/*diagnostic imaging/mortality/surgery Infant Infant, Newborn Male Pregnancy Retrospective Studies Survival Analysis Syndrome *Ultrasonography, Prenatal LA - eng N1 - Verheijen, Paul M Lisowski, Lukas A Plantinga, Rutger F Hitchcock, J François Bennink, Ger B W E Stoutenbeek, Philip Meijboom, Erik J Journal Article Research Support, Non-U.S. Gov't Germany Herz. 2003 May;28(3):250-6. doi: 10.1007/s00059-003-2439-2. PY - 2003 SN - 0340-9937 (Print) 0340-9937 SP - 250-6 ST - Prenatal diagnosis of the fetus with hypoplastic left heart syndrome management and outcome T2 - Herz TI - Prenatal diagnosis of the fetus with hypoplastic left heart syndrome management and outcome VL - 28 ID - 252 ER - TY - JOUR AB - We describe two siblings of consanguineous parents with a prenatal diagnosis of a currently unique form of congenital cardiac disease characterized by superior-inferior atrial and ventricular arrangement, concordant atrioventricular and ventriculo-arterial connections with normal arterial relationships, and a bizarre topography of the ventricular outlets, with the arterial poles being displaced posterior-inferiorly within the thorax. The abnormally low position of the aortic arch resulted in elongation and stretching of the airways, with severe compression of the trachea and left main bronchus in the surviving sibling. The finding of the same rare abnormality in a son and a daughter born to consanguineous parents supports a single gene disorder with a recessive mode of inheritance. © 2008 Cambridge University Press. AD - Fetal Cardiac Program, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, ON M5G 1X8, Canada Department of Obstetrics and Gynecology, Mount Sinai Hospital, University of Toronto, 555 University Avenue, Toronto, ON M5G 1X8, Canada Department of Surgery, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, ON M5G 1X8, Canada Department of Diagnostic Imaging, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, ON M5G 1X8, Canada AU - Jaeggi, E. AU - Chitayat, D. AU - Golding, F. AU - Kim, P. AU - Yoo, S. J. DB - Scopus DO - 10.1017/S1047951108002308 IS - 3 KW - Fetal echocardiography Malposition Superior-inferior ventricles M3 - Article N1 - Cited By :8 Export Date: 15 June 2020 PY - 2008 SP - 337-342 ST - Prenatal diagnosis of topsy-turvy heart T2 - Cardiology in the Young TI - Prenatal diagnosis of topsy-turvy heart UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-44649097536&doi=10.1017%2fS1047951108002308&partnerID=40&md5=88a33f15bf85193f5227faf4afdc6b67 VL - 18 ID - 2194 ER - TY - JOUR AB - Purpose To determine long-term outcomes of fetuses with neck masses (NM), including functional and cosmetic results. Methods A retrospective review was performed of all fetuses evaluated for NM from November 2001 to March 2014. Quality of life (QOL) was evaluated using the validated PedsQL™ questionnaire. Results Of 35 fetuses evaluated, 9 died perinatally and 1 died late from tracheostomy complications. NM ranged from 4 to 20 cm (mean, 9.1 cm); 18 were delivered by EXIT. Of 25 surviving patients, 22 had mass resection, 7 requiring more than one procedure. Surviving patients with lymphatic malformations (LM) had the highest incidence of moderate and severe disfigurement and a higher rate of persistent/recurrent disease (100% vs. 31%, p = 0.002) and cranial nerve dysfunction (50% vs. 0%, p = 0.005) compared to those with non-LM diagnoses. Of 9 children attending school, 78% achieve grades of A/B's. QOL for 13 patients revealed a mean score of 83/100 for physical and 78/100 for psychosocial functioning. Median follow-up was 6 years (7 months-17 years). Conclusion Unlike those with teratoma or other lesions, children with congenital cervicofacial LM are at high-risk for persistent disease, nerve dysfunction and moderate-severe disfigurement. There is substantial perinatal morbidity for fetuses with neck masses, but for those surviving, the long-term functional and cognitive outcomes are good. AD - D.L. Cass, Texas Children's Fetal Center, Texas Children's Hospital, Houston, TX, United States AU - Sheikh, F. AU - Akinkuotu, A. AU - Olutoye, O. O. AU - Pimpalwar, S. AU - Cassady, C. I. AU - Fernandes, C. J. AU - Ruano, R. AU - Lee, T. C. AU - Cass, D. L. DB - Embase Medline DO - 10.1016/j.jpedsurg.2015.02.035 IS - 7 KW - airway obstruction article clinical article controlled study fetus fetus heart follow up gestational age great vessels transposition heart failure hemangioendothelioma human hypoplastic left heart syndrome long term care lymphatic malformation male neck tumor premature labor prenatal diagnosis priority journal quality of life questionnaire recurrent disease retrospective study sclerotherapy teratoma thymus cyst vocal cord disorder LA - English M3 - Article N1 - L603619472 2015-04-14 2016-01-12 PY - 2015 SN - 1531-5037 0022-3468 SP - 1210-1213 ST - Prenatally diagnosed neck masses: Long-term outcomes and quality of life T2 - Journal of Pediatric Surgery TI - Prenatally diagnosed neck masses: Long-term outcomes and quality of life UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L603619472 http://dx.doi.org/10.1016/j.jpedsurg.2015.02.035 VL - 50 ID - 884 ER - TY - JOUR AB - OBJECTIVE: To describe preschool neurodevelopmental outcomes of children with complex congenital heart disease (CHD), who were evaluated as part of a longitudinal cardiac neurodevelopmental follow-up program, as recommended by the American Heart Association and the American Academy of Pediatrics, and identify predictors of neurodevelopmental outcomes in these children. STUDY DESIGN: Children with CHD meeting the American Heart Association/American Academy of Pediatrics high-risk criteria for neurodevelopmental delay were evaluated at 4-5 years of age. Testing included standardized neuropsychological measures. Parents completed measures of child functioning. Scores were compared by group (single ventricle [1V]; 2 ventricles [2V]; CHD plus known genetic condition) to test norms and classified as: normal (within 1 SD of mean); at risk (1-2 SD from mean); and impaired (>2 SD from mean). RESULTS: Data on 102 patients were analyzed. Neurodevelopmental scores did not differ based on cardiac anatomy (1V vs 2V); both groups scored lower than norms on fine motor and adaptive behavior skills, but were within 1 SD of norms. Patients with genetic conditions scored significantly worse than 1V and 2V groups and test norms on most measures. CONCLUSIONS: Children with CHD and genetic conditions are at greatest neurodevelopmental risk. Deficits in children with CHD without genetic conditions were mild and may not be detected without formal longitudinal testing. Parents and providers need additional education regarding the importance of developmental follow-up for children with CHD. AD - Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI; Herma Heart Center, Children's Hospital of Wisconsin, Milwaukee, WI. Electronic address: cbrosig@chw.org. Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI; Herma Heart Center, Children's Hospital of Wisconsin, Milwaukee, WI. Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI. AN - 28081891 AU - Brosig, C. L. AU - Bear, L. AU - Allen, S. AU - Hoffmann, R. G. AU - Pan, A. AU - Frommelt, M. AU - Mussatto, K. A. C2 - PMC5368010 C6 - NIHMS842367 DA - Apr DB - PubMed DO - 10.1016/j.jpeds.2016.12.044 DP - NLM ET - 2017/01/14 KW - Age Factors Child, Preschool Cohort Studies Developmental Disabilities/diagnosis/epidemiology/*etiology Female Follow-Up Studies Heart Defects, Congenital/*complications/*diagnosis Humans Infant Infant, Newborn Intellectual Disability/diagnosis/epidemiology/*etiology Logistic Models Longitudinal Studies Male Multivariate Analysis Neuropsychological Tests Risk Assessment Severity of Illness Index Sex Factors United States/epidemiology *assessment and surveillance *child development *congenital heart disease/defects *developmental follow-up *developmental outcomes LA - eng N1 - 1097-6833 Brosig, Cheryl L Bear, Laurel Allen, Sydney Hoffmann, Raymond G Pan, Amy Frommelt, Michele Mussatto, Kathleen A UL1 TR000055/TR/NCATS NIH HHS/United States UL1 TR001436/TR/NCATS NIH HHS/United States Journal Article Research Support, N.I.H., Extramural J Pediatr. 2017 Apr;183:80-86.e1. doi: 10.1016/j.jpeds.2016.12.044. Epub 2017 Jan 9. PY - 2017 SN - 0022-3476 (Print) 0022-3476 SP - 80-86.e1 ST - Preschool Neurodevelopmental Outcomes in Children with Congenital Heart Disease T2 - J Pediatr TI - Preschool Neurodevelopmental Outcomes in Children with Congenital Heart Disease VL - 183 ID - 159 ER - TY - JOUR AB - BACKGROUND: Children with a single systemic right ventricle, such as in hypoplastic left heart syndrome (HLHS), frequently experience reduced exercise capacity. Elucidating the causes could help with optimising treatment strategies. METHODS: Prospective data from 10 consecutive symptomatic patients with HLHS undergoing clinical cardiac magnetic resonance with catheterisation (XMR) were analysed. Mean age 8.6years (range 3.5-11.6years), mean time since Fontan completion 5.5years. MR-compatible catheters were placed in the systemic right ventricle and branch pulmonary arteries to record pressures at rest, with dobutamine infusion at 10mcg/kg/min and at 20mcg/kg/min. Cine short-axis stacks of the ventricle were performed at each condition and used to construct pressure-volume loops. RESULTS: Compared to rest, cardiac index increased with low-dose dobutamine (p<0.01) with no further rise at peak stress despite a further, albeit, blunted rise in heart rate (p=0.002). A fall in stroke volume occurred (p=0.014) despite good contractility (74% increase, p=0.045) and a well-coupled ventriculo-arterial ratio. End-diastolic pressure and early active relaxation, markers of diastolic function, were normal at rest. However, preload fell at peak stress (p<0.008) while pulmonary vascular resistance (PVR) was low throughout. This group of HLHS patients demonstrated a fall in SV at peak stress, coinciding with a fall in preload. CONCLUSIONS: Markers of systolic and diastolic function remained normal. Failure to adequately fill the ventricle implies a ceiling of maximal flow through the Fontan circuit despite low PVR. AD - Division of Imaging Sciences and Biomedical Engineering, King's College London, St. Thomas' Hospital, London SE1 7EH, United Kingdom. Division of Imaging Sciences and Biomedical Engineering, King's College London, St. Thomas' Hospital, London SE1 7EH, United Kingdom. Electronic address: reza.razavi@kcl.ac.uk. AN - 28043677 AU - Wong, J. AU - Pushparajah, K. AU - de Vecchi, A. AU - Ruijsink, B. AU - Greil, G. F. AU - Hussain, T. AU - Razavi, R. C2 - PMC5267632 DA - Mar 1 DB - PubMed DO - 10.1016/j.ijcard.2016.12.087 DP - NLM ET - 2017/01/04 KW - Blood Pressure/physiology Cardiac Catheterization Cardiac Output/*physiology Child Child, Preschool Dobutamine Exercise Test Exercise Tolerance/*physiology Female Humans Hypoplastic Left Heart Syndrome/*physiopathology Magnetic Resonance Imaging Male Vascular Resistance/physiology Catheterisation Congenital heart disease Fontan procedure Hypoplastic left heart syndrome LA - eng N1 - 1874-1754 Wong, James Pushparajah, Kuberan de Vecchi, Adelaide Ruijsink, Bram Greil, Gerald F Hussain, Tarique Razavi, Reza Journal Article Int J Cardiol. 2017 Mar 1;230:439-446. doi: 10.1016/j.ijcard.2016.12.087. Epub 2016 Dec 22. PY - 2017 SN - 0167-5273 (Print) 0167-5273 SP - 439-446 ST - Pressure-volume loop-derived cardiac indices during dobutamine stress: a step towards understanding limitations in cardiac output in children with hypoplastic left heart syndrome T2 - Int J Cardiol TI - Pressure-volume loop-derived cardiac indices during dobutamine stress: a step towards understanding limitations in cardiac output in children with hypoplastic left heart syndrome VL - 230 ID - 128 ER - TY - JOUR AB - Background Children with a single systemic right ventricle, such as in hypoplastic left heart syndrome (HLHS), frequently experience reduced exercise capacity. Elucidating the causes could help with optimising treatment strategies. Methods Prospective data from 10 consecutive symptomatic patients with HLHS undergoing clinical cardiac magnetic resonance with catheterisation (XMR) were analysed. Mean age 8.6 years (range 3.5–11.6 years), mean time since Fontan completion 5.5 years. MR-compatible catheters were placed in the systemic right ventricle and branch pulmonary arteries to record pressures at rest, with dobutamine infusion at 10 mcg/kg/min and at 20 mcg/kg/min. Cine short-axis stacks of the ventricle were performed at each condition and used to construct pressure–volume loops. Results Compared to rest, cardiac index increased with low-dose dobutamine (p < 0.01) with no further rise at peak stress despite a further, albeit, blunted rise in heart rate (p = 0.002). A fall in stroke volume occurred (p = 0.014) despite good contractility (74% increase, p = 0.045) and a well-coupled ventriculo-arterial ratio. End-diastolic pressure and early active relaxation, markers of diastolic function, were normal at rest. However, preload fell at peak stress (p < 0.008) while pulmonary vascular resistance (PVR) was low throughout. This group of HLHS patients demonstrated a fall in SV at peak stress, coinciding with a fall in preload. Conclusions Markers of systolic and diastolic function remained normal. Failure to adequately fill the ventricle implies a ceiling of maximal flow through the Fontan circuit despite low PVR. AD - R. Razavi, Division of Imaging Sciences, King's College London, 4th Floor Lambeth Wing, St. Thomas’ Hospital, London, United Kingdom AU - Wong, J. AU - Pushparajah, K. AU - de Vecchi, A. AU - Ruijsink, B. AU - Greil, G. F. AU - Hussain, T. AU - Razavi, R. DB - Embase Medline DO - 10.1016/j.ijcard.2016.12.087 KW - dobutamine age article blood flow cardiovascular magnetic resonance cardiovascular parameters child clinical article Fontan procedure heart catheterization heart function heart left ventricle filling heart muscle contractility heart muscle relaxation heart output heart preload heart pressure volume loop heart rate heart right ventricle heart stress heart stroke volume heart ventricle enddiastolic pressure human hypoplastic left heart syndrome low drug dose lung vascular resistance pharmacologic stress testing preschool child prospective study pulmonary artery rest school child LA - English M3 - Article N1 - L614076515 2017-01-19 2017-02-17 PY - 2017 SN - 1874-1754 0167-5273 SP - 439-446 ST - Pressure–volume loop-derived cardiac indices during dobutamine stress: a step towards understanding limitations in cardiac output in children with hypoplastic left heart syndrome T2 - International Journal of Cardiology TI - Pressure–volume loop-derived cardiac indices during dobutamine stress: a step towards understanding limitations in cardiac output in children with hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L614076515 http://dx.doi.org/10.1016/j.ijcard.2016.12.087 VL - 230 ID - 768 ER - TY - JOUR AB - Objective: To determine the frequence and profile of congenital heart defects in Down syndrome patients referred to a pediatric cardiologic center, considering the age of referral, gender, type of heart disease diagnosed by transthoracic echocardiography and its association with pulmonary hypertension at the initial diagnosis. Methods: Cross-sectional study with retrospective data collection of 138 patients with Down syndrome from a total of 17,873 records. Descriptive analysis of the data was performed, using Epi-Info version 7. Results: Among the 138 patients with Down syndrome, females prevailed (56.1%) and 112 (81.2%) were diagnosed with congenital heart disease. The most common lesion was ostium secundum atrial septal defect, present in 51.8%, followed by atrioventricular septal defect, in 46.4%. Ventricular septal defects were present in 27.7%, while tetralogy of Fallot represented 6.3% of the cases. Other cardiac malformations corresponded to 12.5%. Pulmonary hypertension was associated with 37.5% of the heart diseases. Only 35.5% of the patients were referred before six months of age. Conclusions: The low percentage of referral until six months of age highlights the need for a better tracking of patients with Down syndrome in the context of congenital heart disease, due to the high frequency and progression of pulmonary hypertension. AD - S. S. Mattos, Rua General Abreu e Lima, 233/201, Rosarinho, CEP 52041-040 - Recife/PE, Brazil AU - Mourato, F. A. AU - Villachan, L. R. R. AU - Mattos, S. S. DB - Embase Medline DO - 10.1590/0103-0582201432218913 IS - 2 KW - arteriovenous shunt article atriopulmonary connection atrioventricular septal defect cardiovascular malformation congenital heart disease controlled study cross-sectional study descriptive research disease course disease severity Down syndrome Fallot tetralogy female heart atrium septum defect heart ventricle septum defect human incidence lung artery pressure major clinical study male patent ductus arteriosus pediatric cardiology prevalence pulmonary hypertension retrospective study transthoracic echocardiography LA - English Polish M3 - Article N1 - L373768976 2014-08-28 2014-08-30 PY - 2014 SN - 0103-0582 SP - 159-163 ST - Prevalence and profile of congenital heart disease and pulmonary hypertension in Down syndrome in a pediatric cardiology service T2 - Revista Paulista de Pediatria TI - Prevalence and profile of congenital heart disease and pulmonary hypertension in Down syndrome in a pediatric cardiology service UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L373768976 http://dx.doi.org/10.1590/0103-0582201432218913 VL - 32 ID - 938 ER - TY - JOUR AB - BACKGROUND: Major birth defects result in high infant mortality and morbidity. It is important to evaluate the burden of birth defects and trends for future intervention and public health improvement. Using the New York State (NYS) Congenital Malformations Registry data, we examined the prevalence and trends of birth defects among children in NYS during 25 years of surveillance. METHODS: Children who had any of the 21 selected birth defects and were born to NYS residents between 1983 and 2007 were selected. The prevalence of each defect was characterized by demographic and birth factors, and the prevalence ratio was calculated. Live births of NYS residents for the same birth year period were used as the denominators for calculating the prevalence. The prevalence trends of birth defects were analyzed by maternal age and race/ethnicity. RESULTS: Compared with non-Hispanic whites, we detected 33%, 21%, and 37% higher prevalence of encephalocele, lower limb deficiencies and omphalocele among non-Hispanic blacks, respectively, and 22% higher prevalence of gastroschisis among Hispanics. Increasing trends of gastroschisis and Down syndrome among non-Hispanic blacks and decreasing trends of spina bifida and limb deficiencies were observed in NYS. CONCLUSION: The findings from this study suggest the existence of racial disparities among children with selected birth defects in NYS. The increasing trends of gastroschisis and Down syndrome observed in NYS are consistent with nationwide trends. © 2013 Wiley Periodicals, Inc. AD - Y. Wang, Congenital Malformations Registry, Center for Environmental Health, New York State Department of Health, Empire State Plaza - Corning Tower, Albany, NY 12237, United States AU - Kim, K. AU - Wang, Y. AU - Kirby, R. S. AU - Druschel, C. M. DB - Embase Medline DO - 10.1002/bdra.23160 IS - 10 KW - anencephalus anophthalmia article atrioventricular septal defect cleft face cohort analysis congenital malformation diaphragm hernia disease surveillance Down syndrome encephalocele esophagus atresia ethnicity Fallot tetralogy follow up gastroschisis great vessels transposition human hypoplastic left heart syndrome intestine atresia limb defect live birth major clinical study maternal age microphthalmia omphalocele prevalence priority journal race difference spinal dysraphism United States LA - English M3 - Article N1 - L52716277 2013-08-07 2013-10-30 PY - 2013 SN - 1542-0752 1542-0760 SP - 619-627 ST - Prevalence and trends of selected congenital malformations in New York state, 1983 to 2007 T2 - Birth Defects Research Part A - Clinical and Molecular Teratology TI - Prevalence and trends of selected congenital malformations in New York state, 1983 to 2007 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52716277 http://dx.doi.org/10.1002/bdra.23160 VL - 97 ID - 989 ER - TY - JOUR AB - OBJECTIVE: The presence of attention-deficit-hyperactivity disorder (ADHD) symptoms in children with congenital heart disease (CHD) was investigated. METHODS: Swanson, Nolan and Pelham teacher and parent rating scales, version 4 (SNAP-IV), commonly used for assessing symptoms of ADHD, were completed by parents and counselors of children who attended a CHD summer camp. Mean scores (n = 51) were compared with two comparison groups without CHD: patients with ADHD (n = 75) and patients without ADHD (n = 41). Parent scores were also compared to previously published parent normative data. RESULTS: Patients with CHD were reported to have elevated SNAP-IV scores by parents and counselors (11.8%). Parent ratings of inattention were significantly greater in CHD subjects when compared to the comparison group without ADHD (P < 0.001), and similar to the ADHD-positive comparison group. Regarding parent ratings of hyperactivity and impulsivity, the CHD group was significantly lower than the ADHD-positive controls (P = 0.024) but greater than the ADHD-negative controls (P < 0.001). CONCLUSION: ADHD symptoms are more prevalent in children with CHD. Parent ratings of inattention and hyperactivity symptoms in CHD patients are similar to ratings in children diagnosed with ADHD. There is a trend towards a greater prevalence of inattention symptoms in patients with cyanosis or single ventricle physiology. AD - UC Irvine School of Medicine, Los Angeles, CA, USA. erlendhansen@gmail.com AN - 22882233 AU - Hansen, E. AU - Poole, T. A. AU - Nguyen, V. AU - Lerner, M. AU - Wigal, T. AU - Shannon, K. AU - Wigal, S. B. AU - Batra, A. S. DA - Dec DB - PubMed DO - 10.1111/j.1442-200X.2012.03711.x DP - NLM ET - 2012/08/14 IS - 6 KW - Adolescent Attention Deficit Disorder with Hyperactivity/complications/*epidemiology California/epidemiology Child Female Follow-Up Studies Heart Defects, Congenital/*complications/epidemiology Humans Male Prevalence Retrospective Studies Surveys and Questionnaires LA - eng N1 - 1442-200x Hansen, Erlend Poole, Tiffany A Nguyen, Van Lerner, Marc Wigal, Tim Shannon, Kevin Wigal, Sharon B Batra, Anjan S Comparative Study Journal Article Australia Pediatr Int. 2012 Dec;54(6):838-43. doi: 10.1111/j.1442-200X.2012.03711.x. Epub 2012 Nov 21. PY - 2012 SN - 1328-8067 SP - 838-43 ST - Prevalence of ADHD symptoms in patients with congenital heart disease T2 - Pediatr Int TI - Prevalence of ADHD symptoms in patients with congenital heart disease VL - 54 ID - 291 ER - TY - JOUR AB - The extracardiac conduit (ECC) modification of the Fontan procedure has been theorized to reduce the risk of sinus node dysfunction and atrial arrhythmia compared with the intraatrial lateral tunnel (ILT) Fontan. This study aimed to compare the prevalence of early and late arrhythmias in patients who underwent ECC and ILT Fontan from a similar era with long-term follow-up at a single institution. A retrospective cohort study was conducted of all patients who underwent ECC or ILT Fontan from 1995 to 2005 at The Children's Hospital of Philadelphia. Bradyarrhythmias (including sinus node dysfunction), tachyarrhythmias, and pacemaker burden prevalence was determined throughout early (<30 days) and late (>30 days) postoperative periods. Of 434 patients undergoing the Fontan procedure during the study period, a total of 87 and 106 patients who underwent ECC and ILT Fontan, respectively, met the inclusion criteria. There were no significant differences in risk of sinus node dysfunction or tachyarrhythmia in both early and late postoperative periods. Although the overall risk of late postoperative pacemaker therapy was lower for the ECC cohort (4.9% vs 15.7%, p = 0.03), when adjusting for follow-up time, no significant difference was observed (odds ratio 3.1, 95% confidence interval 0.6 to 15.2, p = 0.16). In conclusion, the overall prevalence of late postoperative arrhythmias observed after contemporary Fontan modifications is low. Intra-atrial reentrant tachycardia, a potentially fatal complication of the atriopulmonary Fontan operation was infrequently encountered in both ECC and ILT Fontan cohorts. Pacemaker use was higher in the ILT group, although this difference may be explained by differences in follow-up time. Despite the low prevalence of arrhythmias after contemporary Fontan modifications, ongoing surveillance is warranted as the onset of arrhythmias may emerge after longer follow-up time. © 2014 Elsevier Inc. All rights reserved. AD - J.J. Lasa, Division of Cardiology, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, United States AU - Lasa, J. J. AU - Glatz, A. C. AU - Daga, A. AU - Shah, M. DB - Embase Medline DO - 10.1016/j.amjcard.2013.12.025 IS - 7 KW - article atriopulmonary connection bradycardia cardiovascular risk cavopulmonary connection child cohort analysis comparative study complete heart block extracardiac conduit female follow up Fontan procedure heart arrhythmia heart atrium arrhythmia heart transplantation heart ventricle tachycardia Holter monitoring human implanted heart pacemaker intraatrial lateral tunnel junctional ectopic tachycardia major clinical study male medical record review postoperative period prevalence priority journal retrospective study risk reduction school child sinoatrial nodal reentry tachycardia sinus node disease surgical technique tachycardia univariate analysis LA - English M3 - Article N1 - L52996898 2014-02-18 2014-03-24 PY - 2014 SN - 0002-9149 1879-1913 SP - 1184-1188 ST - Prevalence of arrhythmias late after the fontan operation T2 - American Journal of Cardiology TI - Prevalence of arrhythmias late after the fontan operation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52996898 http://dx.doi.org/10.1016/j.amjcard.2013.12.025 VL - 113 ID - 918 ER - TY - JOUR AB - OBJECTIVE: We sought to determine the prevalence of attention-deficit/hyperactivity disorder in a population of children who underwent neonatal heart surgery involving repair of the aortic arch for Norwood Stage I, interrupted aortic arch, and combined repair of aortic coarctation with ventricular septal defect. METHODS: Children between the ages of 5 and 16 were surveyed using the ADHD-IV and the Child Heath Questionnaire-50. Classification as attention-deficit/hyperactivity disorder was defined for this study as either a parent-reported diagnosis of attention-deficit/hyperactivity disorder or ADHD-IV inattention score of ⩾93 percentile. RESULTS: Of the 134 surveys, 57 (43%) were returned completed. A total of 25 (44%) children either had a diagnosis of attention-deficit/hyperactivity disorder and/or ADHD-IV inattention score ⩾93 percentile. Eleven of the 13 (85%) children with interrupted aortic arch, 3 of the 7 (42.9%) children with combined coarctation/ventricular septal defect repair, and 9 of the 33 (27.3%) children with hypoplastic left-heart syndrome were classified as having attention-deficit/hyperactivity disorder. Only 7 of the 25 (28%) children received medical treatment for this condition. Quality of life indicators in the Child Heath Questionnaire-50 Questionnaire were highly correlated with the ADHD-IV scores. CONCLUSION: The risks for the development of attention-deficit/hyperactivity disorder are multifactorial but are significantly increased in this post-surgical population. This study revealed a low treatment rate for attention-deficit/hyperactivity disorder, and a significant impact on the quality of life in these children. AD - 1Medical University of South Carolina,College of Health Professions,Charleston,United States of America. 2Medical University of South Carolina,College of Medicine,Charleston,United States of America. 3Medical University of South Carolina,Division of Cardiothoracic Surgery,Charleston,United States of America. AN - 24775274 AU - Sistino, J. J. AU - Atz, A. M. AU - Simpson, K. N. AU - Ellis, C. AU - Ikonomidis, J. S. AU - Bradley, S. M. DA - Apr DB - PubMed DO - 10.1017/s1047951114000547 DP - NLM ET - 2014/04/30 IS - 4 KW - Adolescent Aorta, Thoracic/surgery Aortic Diseases/*complications/surgery Attention Deficit Disorder with Hyperactivity/*complications/diagnosis/*epidemiology Child Child, Preschool Cross-Sectional Studies Female Humans Hypoplastic Left Heart Syndrome/*complications/surgery Infant, Newborn Male Norwood Procedures Parents Prevalence Quality of Life Risk Factors South Carolina/epidemiology Surveys and Questionnaires neonatal cardiac surgery LA - eng N1 - 1467-1107 Sistino, Joseph J Atz, Andrew M Simpson, Kit N Ellis, Charles Ikonomidis, John S Bradley, Scott M Journal Article England Cardiol Young. 2015 Apr;25(4):663-9. doi: 10.1017/S1047951114000547. Epub 2014 Apr 28. PY - 2015 SN - 1047-9511 SP - 663-9 ST - The prevalence of attention-deficit/hyperactivity disorder following neonatal aortic arch repair T2 - Cardiol Young TI - The prevalence of attention-deficit/hyperactivity disorder following neonatal aortic arch repair VL - 25 ID - 271 ER - TY - JOUR AB - Background: Birth defects (BD) constitute an important public health issue as they are the main cause of infant death. Their prevalence in Europe for 2008-2012 was 25.6 per 1000 newborns. To date, there are no population-based studies for the Russian Federation. The aim of the present study is to estimate the prevalence of BD, its forms, and changes over time in the Russian Arctic city of Monchegorsk (Murmansk County) for the period 1973-2011. Methods: The Murmansk County Birth Register and the Kola Birth Register were the primary sources of information, covering 30448 pregnancy outcomes in Monchegorsk (Murmansk County, Russia) during the study period. Results: The total perinatal prevalence of BD was 36.1/1000 live births (LB) and stillborn (SB) (95% CI = 34.0-38.2). After exclusions of minor malformations according to the European Surveillance of Congenital Anomalies guidelines, it decreased to 26.5/1000 LB plus SB (95% CI = 24.6-28.3). The perinatal prevalence of BD that are obligatory to report in Russia was 7.3/1000 LB plus SB (95% CI = 6.4-8.3). There was a significant positive time-trend in total perinatal prevalence of birth defects across the study period (p < 0.001 for trend). Prevalence of all BD increased from 23.5/1000 to 46.3/1000 (LB plus SB), while that excluding minor defects rose from 17.7/1000 to 35.7/1000 (LB plus SB). The most prevalent group of defects was malformations of the musculoskeletal system, which represented 35.4% of all BD. The most prominent increase was observed for the urinary system, rising from 0.2/1000 to 19.1/1000 (LB plus SB). Conclusions: The observed perinatal prevalence of BD in Monchegorsk increased two-fold during the 38-year study period. Further investigations to identify the underlying bases for the observed progressive growth in BD are recommended. AD - V.A. Postoev, Department of Community Medicine, UiT-The Arctic University of Norway, Tromsø, Norway AU - Postoev, V. A. AU - Nieboer, E. AU - Grjibovski, A. M. AU - Odland, J. Ø DB - Embase Medline DO - 10.1186/1742-4755-12-3 IS - 1 KW - anencephalus anophthalmia anus atresia Arctic article bladder exstrophy cleft lip cleft palate congenital hydrocephalus congenital malformation controlled study diaphragm hernia Down syndrome encephalocele epispadias esophagus atresia gastroschisis great vessels transposition human hypoplastic left heart syndrome hypospadias kidney agenesis limb malformation live birth major clinical study microtia newborn omphalocele pregnancy outcome prevalence Russian Federation spinal dysraphism stillbirth trend study LA - English M3 - Article N1 - L612321585 2016-10-04 2016-10-05 PY - 2015 SN - 1742-4755 ST - Prevalence of birth defects in an Arctic Russian setting from 1973 to 2011: A register-based study T2 - Reproductive Health TI - Prevalence of birth defects in an Arctic Russian setting from 1973 to 2011: A register-based study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L612321585 http://dx.doi.org/10.1186/1742-4755-12-3 VL - 12 ID - 899 ER - TY - JOUR AB - Children with acquired and congenital heart disease (CHD) are increasingly surviving to adulthood. Our aim was to determine the prevalence of coronary artery disease (CAD) risk factors in children at known risk for early CAD or with severe CHD. We recruited children (8–19 years) at risk for early CAD—Kawasaki disease (KD, N = 36) and coarctation (69) or severe CHD: > 1 cardiopulmonary bypass surgery (60), single ventricle (15), prosthetic valves (13). Anthropometric measurements, blood pressure, and fasting lipid data were compared with summaries from National Health and Nutrition Examination Survey (NHANES) publications (1999–2012). Relative risk (RR) ratios were calculated based on age classification and pooled to obtain overall RR. Of 174 subjects, 106 were male (61%) and 138 (79%) had CHD. Compared to NHANES data, hypertension and low HDL were higher in the study cohort [RR 11.7 (CI 6.34–21.6), p < 0.001; and 1.79 (CI 1.36–2.35). p < 0.001] and obesity and elevated total cholesterol were lower [RR 0.59 (CI 0.37–0.96), p = 0.03; and 0.42 (CI 0.19–0.95), p = 0.04]. Elevated non-HDL was similar between groups. Age category had similar RR for all CAD risk factors. Eight subjects had metabolic syndrome. Risk factors were similar between KD versus CHD subgroups. Both coarctation and non-coarctation subjects had increased RR for hypertension. Hypertension is the most common risk factor for children at risk of early CAD and severe CHD. Metabolic syndrome is rare. These patients should be screened and treated for hypertension and current recommendations for universal lipid screening are adequate for follow-up. AD - A.L. Ware, Division of Pediatric Cardiology, Department of Pediatrics, University of Utah, Salt Lake City, UT, United States AU - Ware, A. L. AU - Young, P. C. AU - Weng, C. AU - Presson, A. P. AU - Minich, L. L. A. AU - Menon, S. C. DB - Embase Medline DO - 10.1007/s00246-017-1750-2 IS - 2 KW - heart valve prosthesis glucose article body height body mass body weight cardiovascular risk cholesterol blood level congenital heart disease coronary artery disease disease severity family history female glucose blood level heart single ventricle human hypercholesterolemia hypertension insulin dependent diabetes mellitus major clinical study male metabolic syndrome X mucocutaneous lymph node syndrome obesity prevalence puberty risk assessment risk factor smoking systolic blood pressure waist circumference LA - English M3 - Article N1 - L618921707 2017-10-30 2018-08-13 PY - 2018 SN - 1432-1971 0172-0643 SP - 261-267 ST - Prevalence of Coronary Artery Disease Risk Factors and Metabolic Syndrome in Children with Heart Disease T2 - Pediatric Cardiology TI - Prevalence of Coronary Artery Disease Risk Factors and Metabolic Syndrome in Children with Heart Disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L618921707 http://dx.doi.org/10.1007/s00246-017-1750-2 VL - 39 ID - 705 ER - TY - JOUR AB - Background & Aims: Fontan surgery is used to treat a variety of congenital heart malformations, and may lead to advanced chronic liver disease in the long-term. This study examines the prevalence, characteristics and predictors of liver nodules in patients following Fontan surgery. Methods: This was a prospective, cross-sectional, observational study conducted at 8 European centres. Consecutive patients who had undergone Fontan surgery underwent blood tests, abdominal ultrasonography (US), transient elastography (Fibroscan®), echocardiography, haemodynamic assessments, and abdominal MRI/CT scan. The primary outcome measure was liver nodules detected in the MRI/CT scan. Predictors of liver nodules were identified by multivariate logistic regression. Results: One hundred and fifty-two patients were enrolled (mean age 27.3 years). The mean time elapsed from surgery to inclusion was 18.3 years. Liver nodule prevalences were 29.6% (95% CI 23–37%) on US and 47.7% (95% CI 39–56%) on MRI/CT. Nodules were usually hyperechoic (76.5%), round-shaped (>80%), hyperenhancing in the arterial phase (92%) and located in the liver periphery (75%). The sensitivity and specificity of US were 50% (95% CI 38–62%) and 85.3% (95% CI 75–92%), respectively. Inter-imaging test agreement was low (adjusted kappa: 0.34). In the multivariate analysis, time since surgery >10 years was the single independent predictor of liver nodules (odds ratio 4.18; p = 0.040). Hepatocellular carcinoma was histologically diagnosed in 2 of the 8 patients with hypervascular liver nodules displaying washout. Conclusion: While liver nodules are frequent in Fontan patients, they may go unnoticed in US. Liver nodules are usually hyperechoic, hypervascular and predominantly peripheral. This population is at risk of hepatocellular carcinoma, the diagnosis of which requires confirmatory biopsy. Lay summary: Fontan surgery is the standard of care for many patients with univentricular congenital cardiopathies. Recent advances have improved the survival of Fontan patients, and nowadays most of them reach adulthood. In this setting, Fontan-associated liver disease (FALD) is increasingly recognised, and has become a significant prognostic factor. Liver nodules are considered a component of FALD yet their prevalence, imaging features and predictors have hardly been evaluated. In this study, we observed that liver nodules are frequent, typically hyperechoic, hypervascular and predominantly peripheral in patients with FALD. This population is at risk of hepatocellular carcinoma, the diagnosis of which must be confirmed by biopsy. AD - A. Albillos, Servicio de Gastroenterología y Hepatología, Hospital Universitario Ramón y Cajal, Ctra Colmenar Viejo Km 9.100, Madrid, Spain AU - Téllez, L. AU - Rodríguez de Santiago, E. AU - Minguez, B. AU - Payance, A. AU - Clemente, A. AU - Baiges, A. AU - Morales-Arraez, D. AU - La Mura, V. AU - Llop, E. AU - Garrido, E. AU - Garrido-Lestache, E. AU - Tasayco, S. AU - Bruno, O. AU - Prieto, R. AU - Montserrat, S. AU - Pons, M. AU - Olavarría, A. AU - Dos, L. AU - Legendre, A. AU - Jesús del Cerro, M. AU - Bañares, R. AU - García-Pagán, J. C. AU - Rautou, P. E. AU - Albillos, A. AU - Aguilera, L. AU - Romera, R. AU - Rincón, D. AU - Fuente, M. Á AU - Merino, X. AU - Chessa, M. AU - Triolo, M. AU - Ronot, M. AU - Vilgrain, V. AU - Chassing, C. AU - Hernández-Gea, V. AU - Garcia-Criado, M. A. AU - Darnell, A. AU - Belmonte, E. AU - Turon, F. AU - Ferrusquia, J. AU - Magaz, M. DB - Embase Medline DO - 10.1016/j.jhep.2019.10.027 IS - 4 KW - adolescent adult alcohol consumption article ascites atrioventricular septal defect body mass chronic liver disease congenital heart malformation criss cross atrioventricular relationship cross-sectional study diagnostic accuracy echocardiography echography female Fontan procedure gallstone hemodynamics human liver biopsy liver cell carcinoma liver nodule major clinical study male mitral valve atresia nuclear magnetic resonance imaging observational study priority journal prospective study protein losing gastroenteropathy pulmonary valve atresia splenomegaly surgical patient transient elastography tricuspid valve atresia x-ray computed tomography young adult LA - English M3 - Article N1 - L2004601596 2020-01-17 2020-03-13 PY - 2020 SN - 1600-0641 0168-8278 SP - 702-710 ST - Prevalence, features and predictive factors of liver nodules in Fontan surgery patients: The VALDIG Fonliver prospective cohort T2 - Journal of Hepatology TI - Prevalence, features and predictive factors of liver nodules in Fontan surgery patients: The VALDIG Fonliver prospective cohort UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2004601596 http://dx.doi.org/10.1016/j.jhep.2019.10.027 VL - 72 ID - 555 ER - TY - JOUR AB - Objective: To assess the prevalence, pattern and outcome of congenital heart disease (CHD) among the babies born in Bhabha Atomic Research Centre (BARC) Hospital, Mumbai and to compare the pattern of CHD with the study done in the same hospital in year 1999. Methods: A prospective, longitudinal study was conducted from January 2006 through December 2011. Babies presenting with murmur were closely followed up till the diagnosis and further to assess the outcome. 2-D Echocardiography was used as a diagnostic tool. Results: The overall prevalence of CHD was 13.28 per 1,000 live births. Ventricular septal defect (VSD) was the commonest CHD (42.86 %) followed by Atrial septal defect (ASD) (25.71 %) giving the prevalence of 5.69 and 3.41 per 1,000 live births respectively. Tetrology of Fallot's (TOF) was the main cyanotic CHD (8.57 %) with the prevalence of 1.13 per 1,000 live births. VSD and TOF were prevalent in males. ASD was prevalent in females. Associated structural anomalies were detected in 22.86 % cases; Down's syndrome being the commonest.Spontaneous closure rate of 71.4 % in muscular VSD and 50 % in perimembranous VSD was observed. All small sized VSD's closed spontaneously and all large sized VSD's required surgical intervention. Spontaneous closure was observed in 44.44 % of ASD cases. Overall, device closure was required in 17.14 % and 25.71 % underwent open heart surgery. Conclusions: The pattern of CHD has remained almost the same compared to the previous study. The outcome was excellent due to early diagnosis, regular follow up and timely intervention. © 2012 Dr. K C Chaudhuri Foundation. AD - S.P. Sawant, Department of Pediatrics, Bhabha Atomic Research Centre Hospital, Anushakti Nagar, Mumbai, Maharashtra 400094, India AU - Sawant, S. P. AU - Amin, A. S. AU - Bhat, M. DB - Embase Medline DO - 10.1007/s12098-012-0910-x IS - 4 KW - aortic coarctation article congenital heart disease Down syndrome Fallot tetralogy female follow up great vessels transposition heart atrium septum defect heart murmur heart ventricle septum defect human India live birth longitudinal study lung vein drainage anomaly major clinical study male newborn outcome assessment patent ductus arteriosus prevalence prospective study pulmonary valve stenosis tricuspid valve atresia two dimensional echocardiography LA - English M3 - Article N1 - L52318498 2012-11-28 2013-05-09 PY - 2013 SN - 0019-5456 0973-7693 SP - 286-291 ST - Prevalence, pattern and outcome of congenital heart disease in Bhabha Atomic Research Centre Hospital, Mumbai T2 - Indian Journal of Pediatrics TI - Prevalence, pattern and outcome of congenital heart disease in Bhabha Atomic Research Centre Hospital, Mumbai UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52318498 http://dx.doi.org/10.1007/s12098-012-0910-x VL - 80 ID - 1008 ER - TY - JOUR AB - Background-After modifications in our perioperative management protocol, we have observed a decrease in sudden circulatory collapse after the Norwood operation. The current study examines early outcomes after the Norwood operation in our unit in an attempt to identify variables that may have altered the risk of unexpected circulatory collapse. Methods and Results-We studied 105 consecutive neonates who underwent a Norwood operation in our institution. Our treatment protocol has changed in the past 3 years to include the use of alpha-blockade with phenoxybenzamine (POB) for systemic afterload reduction and selective cerebral perfusion. Forty-eight infants had selective cerebral perfusion. Forty-two infants received POB. Sixty patients had hypoplastic left heart syndrome. There was no difference in age, diagnosis, number of neonates with weight <2.5 kg, aortic size diameter <2 mm, highest preoperative lactate level, and shunt size indexed to body weight among patients with or without use of POB. Twenty-five infants had circulatory collapse during the first 72 hours. Twelve of them could be explained by technical issues. Thirteen others who appeared clinically stable had early sudden circulatory collapse without an apparent cause. Sixteen out of 25 neonates died. Of those with technical problems, 8 out of 12 died. Based on the hazard function, 3 incremental risk factors for early circulatory collapse were technical issue at operation (P<0.001), longer cross-clamp time (P<0.007), and no use of POB (P<0.002). For a technically successful operation, freedom from circulatory collapse at 72 hours is 95% with the use of POB versus 69% without (P<0.002). Diagnosis, aortic size, atrioventricular valve function, birth weight, age at operation, and total circulatory arrest time and were not predictive of early sudden circulatory collapse. Conclusion-Recent changes in our treatment protocol have resulted in a decrease incidence of sudden circulatory collapse after the Norwood operation. Optimal surgical technique is the most important predictor of early survival. The use of aggressive afterload reduction with POB reduced the risk of early sudden arrest. AD - G.S. Van Arsdell, Division of Cardiovascular Surgery, Hospital for Sick Children, 555 University Avenue, Toronto, Ont. M5G 1X8, Canada AU - De Oliveira, N. C. AU - Ashburn, D. A. AU - Khalid, F. AU - Burkhart, H. M. AU - Adatia, I. T. AU - Holtby, H. M. AU - Williams, W. G. AU - Van Arsdell, G. S. DB - Embase Medline DO - 10.1161/01.CIR.0000138399.30587.8e IS - 11 SUPPL. KW - alpha adrenergic receptor blocking agent phenoxybenzamine article body weight brain perfusion cardiovascular risk circulation clinical protocol controlled study correlation analysis disease association female heart afterload heart death human hypoplastic left heart syndrome infant major clinical study male newborn Norwood procedure outcomes research patient satisfaction patient selection postoperative complication prediction preoperative care priority journal shock surgical technique treatment outcome LA - English M3 - Article N1 - L39263712 2004-10-11 PY - 2004 SN - 0009-7322 SP - II133-II138 ST - Prevention of early sudden circulatory collapse after the norwood operation T2 - Circulation TI - Prevention of early sudden circulatory collapse after the norwood operation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L39263712 http://dx.doi.org/10.1161/01.CIR.0000138399.30587.8e VL - 110 ID - 1289 ER - TY - JOUR AB - Reoperations for congenital cardiac defects are associated with an increased surgical risk due to adhesions. We compared the capability of a polytetrafluoroethylene (PTFE) membrane, synthetic polyethyleneglycol hydrogel (PEG), and a combination of them to prevent postoperative pericardial adhesions in patients with hypoplastic left heart syndrome (HLHS). Eighteen consecutive patients with HLHS were included. At the end of the Norwood I operation the cranial and the caudal half of the heart of each patient was randomized to receive a PTFE membrane, a synthetic PEG, a combination of them, or no treatment (control). Tenacity and density of adhesions, epicardial visibility, and adhesions between the heart and the sternum were analyzed semiquantitatively at a subsequent bidirectional Glenn operation. The PTFE membrane significantly decreased adhesion formation between the heart and the sternum (P<0.001). However, the PTFE membrane, with or without synthetic PEG, impaired epicardial visibility (P<0.05) when compared to synthetic PEG or controls. Synthetic PEG alone did not significantly reduce the formation of pericardial adhesions. Tenacity and density of adhesions were not affected by any of the treatment modalities. The PTFE membrane significantly decreases postoperative adhesions between the heart and the sternum, but impairs epicardial visibility. Synthetic PEG does not prevent formation of pericardial adhesions. © 2011 Published by European Association for Cardio-Thoracic Surgery. All rights reserved. AD - J. T. Salminen, Departments of Paediatric Cardiac Surgery, Hospital for Children and Adolescents, University of Helsinki, P.O.B. 281, 00029 Helsinki, Finland AU - Salminen, J. T. AU - Mattila, I. P. AU - Puntila, J. T. AU - Sairanen, H. I. DB - Embase Medline DO - 10.1510/icvts.2010.241448 IS - 2 KW - macrogol politef article child clinical article clinical effectiveness endoprosthesis feasibility study Glenn shunt heart surgery human hydrogel hypoplastic left heart syndrome Norwood procedure patient safety pericardium adhesion postoperative complication priority journal randomized controlled trial risk reduction surgical approach surgical technique tissue adhesion LA - English M3 - Article N1 - L361210871 2011-02-10 2011-02-17 http://icvts.ctsnetjournals.org/cgi/reprint/12/2/270?ck=nck PY - 2011 SN - 1569-9293 1569-9285 SP - 270-272 ST - Prevention of postoperative pericardial adhesions in children with hypoplastic left heart syndrome T2 - Interactive Cardiovascular and Thoracic Surgery TI - Prevention of postoperative pericardial adhesions in children with hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L361210871 http://dx.doi.org/10.1510/icvts.2010.241448 VL - 12 ID - 1114 ER - TY - JOUR AB - Infants with functionally single ventricle who require a modified Blalock-Taussig shunt can have stenosis at the ductal insertion site on the pulmonary artery (PA), the so-called ductal-associated PA coarctation. We hypothesized that central PA plasty with resection of the ductal tissue on the PA during the first palliation would prevent PA coarctation and obtain well-balanced PA for Fontan operation. We performed this strategy on 40 consecutive patients (from 1998 to 2012, Age 38.4 ± 24.7 day [17 neonates], body weight 3.5 ± 0.8 kg, heterotaxy n = 27). The mean Nakata index (mm2/m2) before bidirectional cavopulmonary shunt was 230.7 ± 101.7 without pulmonary stenosis. The PA diameter ratio and lung perfusion ratio (nonshunt side and/or shunt side) were 0.93 ± 0.25 and 0.9 ± 0.2, respectively. This strategy may improve clinical outcomes on functional single ventricle patients with increased risk of stenosis from pulmonary coarctation, with avoidance of an unbalanced pulmonary vascular bed. AD - K. Sakamoto, Mt. Fuji Shizuoka Children's Hospital, Department of Cardiovascular Surgery, 860 Urushiyama, Aoi-ku, Shizuoka, Shizuoka, Japan AU - Sakamoto, K. DB - Embase DO - 10.1053/j.optechstcvs.2020.04.004 KW - adult aortic coarctation artery diameter artery reconstruction article avoidance behavior body weight cavopulmonary connection clinical article clinical outcome collateral artery controlled study female Fontan procedure heart single ventricle human infant lung perfusion male newborn outcome assessment palliative therapy prevention pulmonary valve atresia pulmonary valve stenosis LA - English M3 - Article in Press N1 - L2005978526 2020-05-26 PY - 2020 SN - 1532-8627 1522-2942 ST - Primary Central Pulmonary Artery Plasty for Ductal-Associated Pulmonary Artery Coarctation T2 - Operative Techniques in Thoracic and Cardiovascular Surgery TI - Primary Central Pulmonary Artery Plasty for Ductal-Associated Pulmonary Artery Coarctation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2005978526 http://dx.doi.org/10.1053/j.optechstcvs.2020.04.004 ID - 577 ER - TY - JOUR AB - The Family Management Style Framework (FMSF) was used as a conceptual basis for secondary data analysis of 55 previously conducted interviews with mothers and fathers of children with a lethal congenital condition from two surgical treatment eras. The directed content analysis was guided by a coding structure developed from family management dimensions identified in prior research of family response to childhood chronic conditions. Results indicated that application of the FMSF was helpful in differentiating families and their processes of family management at the onset of their infant's illness through to surviving the first surgery and going home. The dimensions of Illness View and Child Identity were central to the parents' capacity to manage their baby's illness demands within their family context. Applying a robust family framework to a complex neonatal condition at illness onset provides compelling direction for clinical interventions and their rigorous evaluation. AD - University of Alberta, Edmonton, Alberta, Canada. gwen.rempel@ualberta.ca AN - 22223497 AU - Rempel, G. R. AU - Blythe, C. AU - Rogers, L. G. AU - Ravindran, V. DA - Feb DB - PubMed DO - 10.1177/1074840711427143 DP - NLM ET - 2012/01/10 IS - 1 KW - *Adaptation, Psychological Adult Canada Cardiac Surgical Procedures/*psychology/rehabilitation Caregivers/*psychology Decision Making Female Humans Hypoplastic Left Heart Syndrome/psychology/*surgery Infant, Newborn Interviews as Topic Male Middle Aged Models, Psychological Norwood Procedures/psychology/rehabilitation Palliative Care/*psychology Parents/psychology LA - eng N1 - 1552-549x Rempel, Gwen R Blythe, Catriona Rogers, Laura G Ravindran, Vinitha Comparative Study Journal Article Research Support, Non-U.S. Gov't United States J Fam Nurs. 2012 Feb;18(1):35-64. doi: 10.1177/1074840711427143. Epub 2012 Jan 5. PY - 2012 SN - 1074-8407 SP - 35-64 ST - The process of family management when a baby is diagnosed with a lethal congenital condition T2 - J Fam Nurs TI - The process of family management when a baby is diagnosed with a lethal congenital condition VL - 18 ID - 402 ER - TY - JOUR AB - Background. At the present time, the Fontan procedure is indicated for patients with univentricular cardiac pathology. In Mexico, few tertiary centers are performing Fontan surgery in the pediatric population. The objective was to analyze the results of patients with congenital heart disease with univentricular physiology who have undergone Fontan surgery since 1980 in the Hospital Infantil de Mexico Dr. Federico Gomez. Methods. We present a retrospective analysis of pediatric patients undergoing Fontan surgery. We included variables related to preoperative mortality and morbidity, those related to surgery and to the postoperative period. A bivariate analysis with the comparison of averages and odds ratio was performed. Kaplan-Meier survival curves were constructed. Results. From 1983 to 2012, 53 Fontan surgeries have been performed. Of the total, comprehensive data of 32 patients were gathered. The most common congenital heart anomaly was tricuspid atresia IB (39.4%); according to type of surgery, in 48% of patients extracardiac Fontan was performed followed by intracardiac Fontan (24%). In 87% of patients fenestration was performed. The most common complications were acute renal failure, neurological problems and pleural effusion. Overall survival was 65.6%; 90.9% of patients died within the first month of surgery. Patients have been reported with >20 years of survival (average actuarial survival 14.5 years). Variables associated with mortality were younger age at surgery, type of Fontan performed, surgical technique complications, lack of fenestration and acute renal failure. Conclusions. Evolution of Fontan surgery during the past 30 years has been satisfactory. In patients undergoing this procedure, mortality and quality of life have shown improvements. AD - G. Hernández-morales, Departamento de Cirugía Cardiovascular, Hospital Infantil de México Federico Gómez, Centro Médico Nacional Siglo XXI, Instituto Mexicano del Seguro Social, México, DF, Mexico AU - Bolio-Cerdán, A. AU - Ruiz-González, S. AU - Romero-Cárdenas, P. AU - Hernández-Morales, G. AU - Villasís-Keever, M. A. DB - Embase IS - 2 KW - acute kidney failure article cardiomyopathy cardiovascular mortality clinical article Fontan procedure human morbidity neurologic disease overall survival pleura effusion postoperative period prognosis retrospective study tricuspid valve atresia LA - English Spanish M3 - Article N1 - L368859251 2013-05-14 2013-05-23 http://www.medigraphic.com/pdfs/bmhim/hi-2013/hi132k.pdf PY - 2013 SN - 1665-1146 SP - 151-158 ST - Prognosis of children with cardiomyopathy submitted to fontan surgery: 30 years of experience at the Hospital Infantil de México Federico Gómez T2 - Boletin Medico del Hospital Infantil de Mexico TI - Prognosis of children with cardiomyopathy submitted to fontan surgery: 30 years of experience at the Hospital Infantil de México Federico Gómez UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L368859251 VL - 70 ID - 999 ER - TY - JOUR AB - Objective:Neonatologists provide antenatal counseling to support shared decision-making for complicated pregnancies. Poor or ambiguous prognostication can lead to inappropriate treatment and parental distress. We sought to evaluate the accuracy of antenatal prognosticaltion.Study Design:A retrospective cohort was assembled from a prospectively populated database of all outpatient neonatology consultations. On the basis of the written consultation, fetuses were characterized by diagnosis groups (multiple anomalies or genetic disorders, single major anomaly and obstetric complications), assigned to five prognostic categories (I=survivable, IIA=uncertain but likely survivable, II=uncertain, IIB=uncertain but likely non-survivable, III non-survivable) and two final outcome categories (fetal demise/in-hospital neonatal death or survival to hospital discharge). When possible, status at last follow-up was recorded for those discharged from the hospital. Prognostic accuracy was assessed using unweighted, multi-level likelihood ratios (LRs).Results:The final cohort included 143 fetuses/infants distributed nearly evenly among the three diagnosis groups. Over half (64%) were assigned an uncertain prognosis, but most of these could be divided into 'likely survivable' or 'likely non-survivable' subgroups. Overall survival for the entire cohort was 62% (89/143). All but one of the fetuses assigned a non-survivable prognosis suffered fetal demise or died before hospital discharge. The neonatologist's antenatal prognosis accurately predicted the probability of survival by prognosis group (LR I=4.56, LR IIA=10.53, LR II=4.71, LR IIB=0.099, LR III=0.040). The LRs clearly differentiated between fetuses with high and low probability of survival. Eleven fetuses (7.7%) had misalignment between the predicted prognosis and outcome. Five died before discharge despite being given category I or IIA prognoses, whereas six infants with category IIB or III prognoses survived to discharge, though some of these were discharged to hospice care.Conclusions:The neonatologist's antenatal prognosis accurately predicted fetal-neonatal outcome. Infants with non-survivable or uncertain but likely poor prognoses had a very low probability of survival, whereas those with good or uncertain prognoses had a high probability of survival. There were few cases of prognostic failure with most occurring in fetuses with one major or multiple anomalies. The few cases of prognostic failure suggest a need for caution. Honest disclosure of prognostic uncertainty and shared decision-making with families utilizing their personal values is critical in the antenatal encounter. AD - S. Kukora, Division of Neonatal-Perinatal Medicine, Department of Pediatrics and Communicable Diseases, Mott Children's Hospital, University of Michigan Medical Center, 8-621 CandW Mott Hospital, 1540 E Hospital Drive, SPC 4254, Ann Arbor, MI, United States AU - Kukora, S. AU - Gollehon, N. AU - Weiner, G. AU - Laventhal, N. DB - Embase Medline DO - 10.1038/jp.2016.171 IS - 1 KW - accuracy adult article bladder obstruction Caucasian cause of death central nervous system disease cohort analysis congenital diaphragm hernia consultation controlled study data base female fetus follow up galactosialidosis galactosialidosis type 1 genetic disorder hospice care hospital discharge hospitalization human hypoplastic left heart syndrome infant labor complication major clinical study meningomyelocele mucopolysaccharidosis type 7 neonatology newborn newborn death oligohydramnios outpatient population research prenatal care probability prognosis prognostic accuracy prospective study resuscitation retrospective study survival rate LA - English M3 - Article N1 - L612477722 2016-10-07 2017-02-14 PY - 2017 SN - 1476-5543 0743-8346 SP - 27-31 ST - Prognostic accuracy of antenatal neonatology consultation T2 - Journal of Perinatology TI - Prognostic accuracy of antenatal neonatology consultation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L612477722 http://dx.doi.org/10.1038/jp.2016.171 VL - 37 ID - 776 ER - TY - JOUR AB - In recent years, the surgical treatment and operative procedure for congenital heart disease (CHD) have been greatly improved. The surgical results of hypoplastic left heart syndrome (HLHS) have been improved by modified operative procedure and intervention. With proper management of the right ventricle outflow tract in tetralogy of Fallot (TOF), right ventricle heart function can be improved after operation. The surgical repair of pulmonary venous obstruction after operation of total abnormal pulmonary venous connection (TAPVC), and the renewed operative procedure of transposition of the great arteries (TGA) and congenital corrected transposition of the great arteries (ccTGA) facilitate the rational surgical treatment of complex CHD. With improvement of operative procedure for CHD, the operative successful rate is increased, the incidence of complications is decreased, and the quality of life and long-term surgical outcomes are improved. AD - Z.-W. Xu, Department of Cardiovascular and Thoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine\, Shanghai 200127, China AU - Xu, Z. W. DB - Embase DO - 10.3969/j.issn.1674-8115.2011.09.003 IS - 9 KW - article congenital heart disease Fallot tetralogy great vessels transposition heart function heart right ventricle outflow tract heart surgery human hypoplastic left heart syndrome lung vein drainage anomaly pulmonary vein obstruction pediatric surgery quality of life treatment outcome LA - English Chinese M3 - Article N1 - L362635615 2011-10-04 2011-10-07 http://xuebao.shsmu.edu.cn/EN/article/downloadArticleFile.do?attachType=PDF&id=9233 PY - 2011 SN - 1674-8115 SP - 1226-1230 ST - Progress of surgical treatment of pediatric congenital heart disease T2 - Journal of Shanghai Jiaotong University (Medical Science) TI - Progress of surgical treatment of pediatric congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L362635615 http://dx.doi.org/10.3969/j.issn.1674-8115.2011.09.003 VL - 31 ID - 1089 ER - TY - JOUR AB - Cardiac and vascular biomarkers are used in the diagnosis, assessment, and prognosis of a variety of cardiovascular diseases in adults and children. For children, cardiac biomarkers are most often used to assess cardiomyopathy or congenital cardiovascular malformations or for monitoring during cardio-pulmonary bypass and pre- and post-heart transplantation. Because the risk for cardiovascular events in adulthood can be tracked from childhood, interest is high in identifying biomarkers of vascular inflammation in children that might also predict atherosclerotic events later in life. Here, we review the most common and promising biomarkers and comment on their current and potential uses in monitoring and predicting cardiovascular dysfunction. © 2011 Elsevier Ireland Ltd. AD - Department of Pediatrics, University of Miami Miller School of Medicine, Miami, FL, United States Division of Pediatric Clinical Research, University of Miami School of Medicine, Miami, FL, United States Department of Epidemiology and Public Health, University of Miami Miller School of Medicine, Miami, FL, United States AU - Wilkinson, J. D. AU - Diamond, M. AU - Miller, T. L. DB - Scopus DO - 10.1016/j.ppedcard.2011.06.006 IS - 1 KW - Atherosclerosis Biomarkers Cardiomyopathy Child Congenital heart disease Heart failure M3 - Article N1 - Cited By :6 Export Date: 15 June 2020 PY - 2011 SP - 25-34 ST - The promise of cardiovascular biomarkers in assessing children with cardiac disease and in predicting cardiovascular events in adults T2 - Progress in Pediatric Cardiology TI - The promise of cardiovascular biomarkers in assessing children with cardiac disease and in predicting cardiovascular events in adults UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-79961171435&doi=10.1016%2fj.ppedcard.2011.06.006&partnerID=40&md5=5383ec726080635e7ca2651623f8851b VL - 32 ID - 2124 ER - TY - JOUR AB - For most women, having a baby is a very exciting and happy period of life, but it is also known as a time of increased vulnerability. Although under-diagnosed, postnatal depression (PND) remains the most frequent complication after giving birth nowadays, and constitutes a major public-health issue. PND has been linked to negative short-/long-term effects on children’s physical and psychological health, and on early brain development. In France, systematic pediatric assessments are required at 8 days, and at 9 and 24 months of an infant’s life, placing these health professionals at the heart of a dyadic assessment and a potential PND secondary prevention program. Based on this general framework, we implemented monthly psychiatrists-pediatricians sessions in the North of France to discuss mother’s and child’s evaluation, mother-child’s interactions and to guide management of the dyads by pediatricians during 'as usual' follow-up. The aim of this 2-year follow-up naturalistic cohort was to provide preliminary evidence that a multidisciplinary primary-care program could significantly impact early PND risk assessment and children’s health development. (PsycINFO Database Record (c) 2020 APA, all rights reserved) AD - Jardri, R., Service de Psychiatrie Perinatale, Hopital Fontan, CHU de Lille, CS 70001, 59037, Lille, France, Cedex AN - 2019-75712-016 AU - Brunais, E. AU - Leroy, A. AU - Touchard, A. AU - Pelta, J. AU - Tanguy, A. AU - Guedeney, A. AU - Delion, P. AU - Jardri, R. AU - Medjkane, F. DB - psyh DO - 10.1016/j.encep.2019.03.003 DP - EBSCOhost IS - 6 KW - postnatal depression psychiatrist-pediatrician collaborations depression prevention Pediatricians Postpartum Depression Prevention Psychiatrists Collaboration Counseling Professional Consultation N1 - CHU Lille, Fontan Hospital, Psychiatry Department, Perinatal Psychiatry Unit, Lille, France. Institutional Authors: GRoup of REsearch of North PAediatricians. Other Publishers: Masson. Release Date: 20200102. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Letter. Language: English. Major Descriptor: Pediatricians; Postpartum Depression; Prevention; Psychiatrists. Minor Descriptor: Collaboration; Counseling; Professional Consultation. Classification: Affective Disorders (3211). Population: Human (10); Female (40). Location: France. Age Group: Childhood (birth-12 yrs) (100); Neonatal (birth-1 mo) (120); Adulthood (18 yrs & older) (300). Methodology: Empirical Study; Longitudinal Study; Quantitative Study. Page Count: 2. Issue Publication Date: Dec, 2019. Copyright Statement: L'Encéphale, Paris. 2019. PY - 2019 SN - 0013-7006 SP - 533-534 ST - Promoting psychiatrist-pediatrician collaborations on postnatal depression prevention T2 - L'Encéphale: Revue de psychiatrie clinique biologique et thérapeutique TI - Promoting psychiatrist-pediatrician collaborations on postnatal depression prevention UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2019-75712-016&site=ehost-live&scope=site renaud.jardri@chru-lille.fr VL - 45 ID - 1658 ER - TY - JOUR AB - Background: E-type prostaglandins (PGE1) can effectively maintain the patency of the ductus arteriosus in neonates. Its use, therefore can be life saving in infants born with ductus dependent congenital heart disease. Although PGE1 is available for over two decades in western world, it has been introduced in India only since April, 1995. Objective: To assess the efficacy of PGE1 at our center. Setting: Hospital based. Method: 65 infants with ductus dependent congenital heart disease were included. Age at time of starting PGE1 infusion ranged from 18 hours to 39 days. Forty two of these were more than a week of age, 19 were more than 14 days, and two were above one month. PGE1 was started in an initial dose of 0.05 μg/kg/min, decreased to 0.005-0.01 μg/kg/min for maintenance. The indications for use of PGE1 were to increase pulmonary blood flow in 33 cases with pulmonary atresia, tricuspid atresia or critical pulmonic stenosis (Group I); to increase systemic blood flow in 15 cases with coarctation of aorta, hypoplastic left heart and interruption of aortic arch (Group II); to improve mixing in 13 cases of transposition of great arteries (Group III) and for improving the left ventricular volumes by keeping the duct open in 4 cases of transposition of great arteries with intact ventricular septum (Group IV). The efficacy of the drug was assessed by a rise in PaO2 and SaO2% determined for Groups I and III, and by appearance of lower limbs pulses in Group II. Left ventricular volumes were serially measured by echocardiography in Group IV cases. Results: The drug was successful in 62 of the 65 cases. There were two failures. One was a 39 days old baby with Ebstein's anomaly of tricuspid valve and pulmonary atresia and other was an eight days old baby with coarctation of aorta and renal failure. In addition, PGE1 could not be continued in another baby due to development of a linear skin rash locally. Side effects included apnea in 5 (9%) of 56 spontaneously breathing patients. Necrotizing enterocolitis, hyperpyrexia and jitteriness was seen in one case each. Six patients died. Two were related to PGE1 one due to failure, another due to its side effects. Definitive procedure were performed in 51 cases electively. PGE1 was used upto 13 days with sustained benefit. Conclusion: PGE1 is an effective drug for keeping the ductus open in infants with ductus dependent congenital heart disease. It can be used for neonates beyond the first week of life with efficacy. Apnea is a major side effect and close monitoring is essential. AD - A. Saxena, Department of Cardiology, Cardiothoracic Center, All India Inst. of Med. Sci., New Delhi 110029, India AU - Saxena, A. AU - Sharma, M. AU - Kothari, S. S. AU - Juneja, R. AU - Reddy, S. C. B. AU - Sharma, R. AU - Bhan, A. AU - Venugopal, P. C1 - prostin vr(Upjohn) C2 - Upjohn DB - Embase Medline IS - 11 KW - prostaglandin E1 aortic arch interruption aortic coarctation apnea article congenital heart disease drug efficacy drug indication female great vessels transposition heart left ventricle volume human hyperpyrexia hypoplastic left heart syndrome India infant intravenous drug administration pulmonary valve atresia lung blood flow major clinical study male necrotizing enterocolitis nervousness newborn patent ductus arteriosus pulmonary valve stenosis rash tricuspid valve atresia prostin vr LA - English M3 - Article N1 - L28541396 1998-12-22 PY - 1998 SN - 0019-6061 SP - 1063 ST - Prostaglandin E1 in infants with congenital heart disease: Indian experience T2 - Indian Pediatrics TI - Prostaglandin E1 in infants with congenital heart disease: Indian experience UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L28541396 VL - 35 ID - 1345 ER - TY - JOUR AB - Fontan and Baudet described the procedure in 1971 and was very useful to improve the quality of life in many complex cyanotic heart diseases. It has gone through various modifications since then to improve the outcome. The mortality was reported as 2.1% and survival rate of at 5 years, 10 years, 15 years and 20 years were 91%, 80%, 73%and 69% respectively. Though it was a useful palliative procedure to improve the quality of life, it has complications which may affect the morbidity and mortality like Protein loosing enteropathy,reduced exercise capacity thromboembolism. The patients with protein losing enteropathy present with Pleural effusion, ascites, and edema and they need periodic replacement of Albumin and frequent reviews and close follow up in the management to reduce the mortality and to improve the quality of life. These complications we come across and they need our support in the management at district level hospitals. AD - R. Vijayaraghavan, Department of Paediatrics, Hospital Tawau, Malaysia AU - Vijayaraghavan, R. AU - Ying, T. W. DB - Embase Medline IS - 3 KW - albumin calcium protein sildenafil warfarin article ascites case report clinical article computer assisted tomography cyanotic heart disease dietary supplement echocardiography edema enteropathy exercise feces analysis follow up Fontan procedure Glenn shunt heart atrium heart catheterization heart ventricle septum defect human hypoalbuminemia hypocalcemia lung artery pressure lung vein drainage anomaly male morbidity mortality newborn outcome assessment patent ductus arteriosus pleura effusion protein losing gastroenteropathy quality of life survival rate thoracocentesis thromboembolism transesophageal echocardiography tricuspid valve disease LA - English M3 - Article N1 - L622893492 2018-07-10 2018-07-17 PY - 2018 SN - 0300-5283 SP - 175-176 ST - Protein loosing enteropathy after fontan procedure T2 - Medical Journal of Malaysia TI - Protein loosing enteropathy after fontan procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L622893492 VL - 73 ID - 682 ER - TY - JOUR AB - Protein-losing enteropathy is a relatively uncommon complication of Fontan procedures for palliation of complex congenital cardiac disease. However, the relative infrequency of protein-losing enteropathy belies the tremendous medical, psychosocial and financial burdens it places upon afflicted patients, their families and the healthcare system that supports them. Unfortunately, because of the complexity and rarity of this disease process, the pathogenesis and pathophysiology of protein-losing enteropathy remain poorly understood, and attempts at treatment seldom yield long-term success. The most comprehensive analyses of protein-losing enteropathy in this patient population are now over a decade old, and re-evaluation of the prevalence and progress in treatment of this disease is needed. This report describes a single institution experience with the evaluation, management, and treatment of protein-losing enteropathy in patients with congenital cardiac disease in the current era, follows with a comprehensive review of protein-losing enteropathy, focused upon what is known and not known about the pathophysiology of protein-losing enteropathy in this patient population, and concludes with suggestions for prevention and treatment. AD - Division of Pediatric Cardiology, University of California-San Francisco, 505 Parnassus Avenue, San Francisco, CA 94143-4144, USA. jeffery.meadows@ucsf.edu AN - 21349233 AU - Meadows, J. AU - Jenkins, K. DA - Aug DB - PubMed DO - 10.1017/s1047951111000102 DP - NLM ET - 2011/02/26 IS - 4 KW - Academic Medical Centers Adolescent Cardiac Catheterization Cardiac Surgical Procedures/methods Child Child, Preschool Cohort Studies Combined Modality Therapy Drug Therapy, Combination Female Fontan Procedure/*adverse effects/methods Heart Defects, Congenital/diagnosis/*surgery Humans Infant Infant, Newborn Kaplan-Meier Estimate Male Prognosis Protein-Losing Enteropathies/etiology/mortality/*prevention & control/*therapy Retrospective Studies Risk Assessment Severity of Illness Index Survival Analysis Treatment Outcome Young Adult LA - eng N1 - 1467-1107 Meadows, Jeffery Jenkins, Kathy Journal Article England Cardiol Young. 2011 Aug;21(4):363-77. doi: 10.1017/S1047951111000102. Epub 2011 Feb 25. PY - 2011 SN - 1047-9511 SP - 363-77 ST - Protein-losing enteropathy: integrating a new disease paradigm into recommendations for prevention and treatment T2 - Cardiol Young TI - Protein-losing enteropathy: integrating a new disease paradigm into recommendations for prevention and treatment VL - 21 ID - 398 ER - TY - JOUR AB - OBJECTIVE: Hypoplastic left heart syndrome is a single ventricle defect. While staged surgical palliative treatments have revolutionised care, patients with hypoplastic left heart syndrome continue to have significant morbidity and mortality. In 2017, the National Pediatric Cardiology Quality Improvement Collaborative recommended all single ventricle patients to receive a prenatal palliative care consult. This study aimed to elucidate provider perspectives on the implementation of prenatal palliative care consults for families expecting a child with hypoplastic left heart syndrome. METHODS: An online survey was administered to obstetric and paediatric providers of relevant disciplines to assess their experience with palliative care involvement in hypoplastic left heart syndrome cases. RESULTS: Nearly, all physicians (97%) and most registered nurses (79%) agreed that the initial palliative care consult for patients with hypoplastic left heart syndrome should occur during the prenatal period. Respondents also indicated that prenatal palliative care consults should also be offered in a variety of other CHD conditions. Participants believed positive aspects of this new referral protocol included an expanded support network for families, decreased family stress during the postnatal period, increased patient education about what to expect during the postnatal period, and continuity of care. CONCLUSION: Multidisciplinary healthcare professionals believe that prenatal palliative care consults provide a variety of benefits for patients and families with hypoplastic left heart syndrome. Additional, multi-centre research is necessary to evaluate whether prenatal palliative care consults should become standard of care for families expecting a child with a single ventricle defect. AD - School of Medicine, Oregon Health & Science University, Portland, OR, USA. Bridges Palliative Care Program, Doernbecher Children's Hospital, Portland, OR, USA. Pediatric Cardiology, Doernbecher Children's Hospital, Portland, OR, USA. AN - 32146916 AU - Lowenstein, S. AU - Macauley, R. AU - Perko, K. AU - Ronai, C. DA - Mar DB - PubMed DO - 10.1017/s1047951120000128 DP - NLM ET - 2020/03/10 IS - 3 KW - Hypoplastic left heart syndrome goals of care palliative care prenatal diagnosis single ventricle defect LA - eng N1 - 1467-1107 Lowenstein, Sarah Macauley, Robert Perko, Kathleen Ronai, Christina Orcid: 0000-0002-1503-7100 Journal Article England Cardiol Young. 2020 Mar;30(3):377-382. doi: 10.1017/S1047951120000128. PY - 2020 SN - 1047-9511 SP - 377-382 ST - Provider perspective on the role of palliative care in hypoplastic left heart syndrome T2 - Cardiol Young TI - Provider perspective on the role of palliative care in hypoplastic left heart syndrome VL - 30 ID - 206 ER - TY - JOUR AB - BACKGROUND: Previous studies suggest that trichloroethylene (TCE) is a selective cardiac teratogen. We tested the hypothesis that the odds of maternal residence close to TCE-emitting sites would be greater among infants with congenital heart defects (CHDs) than among infants without CHDs. METHODS: We conducted a case-control study of 4025 infants, identified from hospital and birth records, born from 1997 to 1999 to Milwaukee, Wisconsin mothers. A geographic information system was used to calculate distances between maternal residences and TCE sites. We used classification tree analysis to determine appropriate values by which to dichotomously categorize mothers by TCE exposure (exposed: residence within 1.32 miles of at least one TCE site) and age (older: ≥38 years), and logistic regression to test for CHD risk factors. RESULTS: The proportion of mothers who were both older and had presumed TCE exposure was more than six-fold greater among case infants than among control infants (3.3% [8/245] versus 0.5% [19/3780]). When adjusted for other variables, CHD risk was over three-fold greater among infants of older, exposed mothers compared to infants of older, nonexposed mothers (adjusted OR, 3.2; 95% CI, 1.2-8.7). Older maternal age, alcohol use, chronic hypertension, and preexisting diabetes were each associated with CHDs (adjusted ORs, 1.9, 2.1, 2.8, 4.1; 95% CIs, 1.1-3.5, 1.1-4.2, 1.2-6.7, 1.5-11.2, respectively), but residence close to TCE sites alone was not. CONCLUSIONS: Our findings suggest that maternal age and TCE exposure interact to increase CHD risk, although the mechanism by which this occurs is unknown. A prospective study is underway to confirm this finding. © 2004 Wiley-Liss, Inc. AD - J.S. Yauck, Department of Pediatrics, Birth Defects Research Center, Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI 53226, United States AU - Yauck, J. S. AU - Malloy, M. E. AU - Blair, K. AU - Simpson, P. M. AU - McCarver, D. G. DB - Embase Medline DO - 10.1002/bdra.20060 IS - 10 KW - teratogenic agent trichloroethylene adult age alcohol consumption aortic coarctation article chronic disease congenital heart malformation controlled study diabetes mellitus disease association Ebstein anomaly environmental exposure Fallot tetralogy great vessels transposition heart atrium septum defect heart ventricle septum defect human hypertension hypoplastic left heart syndrome infant pulmonary valve atresia major clinical study maternal age mitral valve atresia mitral valve stenosis priority journal progeny pulmonary valve stenosis residential home risk assessment risk factor tricuspid valve atresia LA - English M3 - Article N1 - L39473590 2004-11-22 PY - 2004 SN - 1542-0752 SP - 808-814 ST - Proximity of residence to trichloroethylene-emitting sites and increased risk of offspring congenital heart defects among older women T2 - Birth Defects Research Part A - Clinical and Molecular Teratology TI - Proximity of residence to trichloroethylene-emitting sites and increased risk of offspring congenital heart defects among older women UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L39473590 http://dx.doi.org/10.1002/bdra.20060 VL - 70 ID - 1288 ER - TY - JOUR AB - This infant was cyanosed from the age of 3 months. He had a large heart with a small pulmonary conus and an ECG showing left axis deviation. The diagnosis of tricuspid atresia with oulmonarv stenosis was considered and operation attempted. The infant died and was found to have tricuspid atresia with an interauricular septal defect and an aorta opening from the left ventricle; there was also a communication between this ventricle and a rudimentary right ventricle which opened into the pulmonary artery. In addition there was partial dextrocardia. AD - W.G. Cokhr, Dept. of Path., Dept. of Pediat., Bowman Gray School of Med., Winston-Salem AU - Cokhr, W. G. AU - Bhalr, J. D. AU - Gross, F. B. DB - Embase Classic Medline IS - 5 KW - aorta bean dextrocardia diagnosis electrocardiogram embryology heart heart atrium septum defect heart left ventricle heart right ventricle infant interpersonal communication pulmonary artery pulmonary valve stenosis stenosis tricuspid valve atresia LA - English M3 - Article N1 - L280918626 1951-12-01 PY - 1951 SN - 0002-922X SP - 671-674 ST - Pseudo trilocular biatrial bean without pulmonary stenosis. Report of a case and discussion of the related embryology T2 - American Journal of Diseases of Children TI - Pseudo trilocular biatrial bean without pulmonary stenosis. Report of a case and discussion of the related embryology UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L280918626 VL - 81 ID - 1426 ER - TY - JOUR AB - BACKGROUND AND OBJECTIVES: Mental health outcomes for survivors of critical congenital heart disease (CHD) remain under-investigated. We sought to examine psychiatric disorders and psychosocial functioning in adolescents with single ventricle CHD and to explore whether patient-related risk factors predict dysfunction. METHODS: This cohort study recruited 156 adolescents with single ventricle CHD who underwent the Fontan procedure and 111 healthy referents. Participants underwent comprehensive psychiatric evaluation including a clinician-rated psychiatric interview and parent- and self-report ratings of anxiety, disruptive behavior, including attention-deficit/hyperactivity disorder (ADHD), and depressive symptoms. Risk factors for dysfunction included IQ, medical characteristics, and concurrent brain abnormalities. RESULTS: Adolescents with single ventricle CHD had higher rates of lifetime psychiatric diagnosis compared with referents (CHD: 65%, referent: 22%; P < .001). Specifically, they had higher rates of lifetime anxiety disorder and ADHD (P < .001 each). The CHD group scored lower on the primary psychosocial functioning measure, the Children's Global Assessment Scale, than referents (CHD median [interquartile range]: 62 [54-66], referent: 85 [73-90]; P < .001). The CHD group scored worse on measures of anxiety, disruptive behavior, and depressive symptoms. Genetic comorbidity did not impact most psychiatric outcomes. Risk factors for anxiety disorder, ADHD, and lower psychosocial functioning included lower birth weight, longer duration of deep hypothermic circulatory arrest, lower intellectual functioning, and male gender. CONCLUSIONS: Adolescents with single ventricle CHD display a high risk of psychiatric morbidity, particularly anxiety disorders and ADHD. Early identification of psychiatric symptoms is critical to the management of patients with CHD. AD - Departments of Psychiatry, david.demaso@childrens.harvard.edu. Cardiology, and. Departments of Psychiatry. Neurology, Boston Children's Hospital, Boston, Massachusetts. Neurology, and. Department of Biostatistics, Harvard T.H. Chan School of Public Health, Boston, Massachusetts. Pediatrics, Harvard Medical School, Boston, Massachusetts; and. AN - 28148729 AU - DeMaso, D. R. AU - Calderon, J. AU - Taylor, G. A. AU - Holland, J. E. AU - Stopp, C. AU - White, M. T. AU - Bellinger, D. C. AU - Rivkin, M. J. AU - Wypij, D. AU - Newburger, J. W. C2 - PMC5330395 C6 - NIHMS863977 conflicts of interest to disclose. DA - Mar DB - PubMed DO - 10.1542/peds.2016-2241 DP - NLM ET - 2017/02/06 IS - 3 KW - Adolescent Anxiety Disorders/*complications/diagnosis Attention Deficit and Disruptive Behavior Disorders/*complications/diagnosis Birth Weight Case-Control Studies Circulatory Arrest, Deep Hypothermia Induced Cohort Studies Depression/*complications/diagnosis Female Fontan Procedure Heart Defects, Congenital/complications/*psychology/surgery Humans Intellectual Disability Male Risk Factors Sex Factors Ventricular Dysfunction/complications/*psychology/surgery LA - eng N1 - 1098-4275 DeMaso, David R Calderon, Johanna Taylor, George A Holland, Jennifer E Stopp, Christian White, Matthew T Bellinger, David C Rivkin, Michael J Wypij, David Newburger, Jane W R01 HL096825/HL/NHLBI NIH HHS/United States U54 HD090255/HD/NICHD NIH HHS/United States Journal Article Pediatrics. 2017 Mar;139(3):e20162241. doi: 10.1542/peds.2016-2241. Epub 2017 Feb 1. PY - 2017 SN - 0031-4005 (Print) 0031-4005 ST - Psychiatric Disorders in Adolescents With Single Ventricle Congenital Heart Disease T2 - Pediatrics TI - Psychiatric Disorders in Adolescents With Single Ventricle Congenital Heart Disease VL - 139 ID - 11 ER - TY - JOUR AB - Objective: The purpose of the study was to evaluate coping and psychological functioning of parents of children prenatally or postnatally diagnosed with congenital heart disease. Study Design: Parents of 10 infants prenatally diagnosed by fetal echocardiogram and 7 infants postnatally diagnosed with severe congenital heart disease completed the Brief Symptom Inventory (BSI) at the time of diagnosis, at the time of birth and 6 months after birth. Prenatal and postnatal groups were compared to each other and to BSI norms. Result: Although both groups scored higher than test norms at the time of diagnosis, they did not differ significantly from each other. Six months after birth, the postnatal group scores did not differ from test norms, but the prenatal group scores were still significantly higher than test norms. The severity of the child's heart lesion at diagnosis was related to parental distress levels; parents with children with more severe lesions had higher BSI scores. Conclusion: Results suggest the need to provide parents with psychological support, regardless of the timing of diagnosis. Parents of children with more severe lesions may be at risk for higher levels of psychological distress, particularly over time. AD - C.L. Brosig, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, United States AU - Brosig, C. L. AU - Whitstone, B. N. AU - Frommelt, M. A. AU - Frisbee, S. J. AU - Leuthner, S. R. DB - Embase Medline DO - 10.1038/sj.jp.7211807 IS - 11 KW - aortic coarctation aortic stenosis article Brief Symptom Inventory childhood disease clinical article congenital heart disease controlled study coping behavior disease severity distress syndrome echocardiography Fallot tetralogy female great vessels transposition heart single ventricle high risk population human hypoplastic left heart syndrome infant lung vein drainage anomaly male parental attitude prenatal diagnosis psychosocial care pulmonary valve stenosis statistical significance LA - English M3 - Article N1 - L350020348 2007-11-01 PY - 2007 SN - 0743-8346 1476-5543 SP - 687-692 ST - Psychological distress in parents of children with severe congenital heart disease: The impact of prenatal versus postnatal diagnosis T2 - Journal of Perinatology TI - Psychological distress in parents of children with severe congenital heart disease: The impact of prenatal versus postnatal diagnosis UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L350020348 http://dx.doi.org/10.1038/sj.jp.7211807 VL - 27 ID - 1222 ER - TY - JOUR AB - Aims: We studied a population of patients with surgically corrected congenital cardiac disease to determine whether limitations in activity, impaired cardiac performance, and perception of body image have effects on psychological symptoms. Methods: We undertook medical examinations, and carried out standardized interviews, in 361 patients aged between 14 and 45 years with surgically corrected congenital cardiac disease. From this data, findings from 343 patients were suitable for analysis. Subjectively reported limitations in activity were classified according to the system proposed by the New York Heart Association, while cardiopulmonary capacity was used as the indicator of cardiac performance. The Brief Symptom Inventory was used for assessing psychological symptoms, such as somatization, obsession-compulsion, interpersonal sensitivity, depression, anxiety, hostility, phobic anxiety, paranoid ideation, and psychoticism. The Body Image Questionnaire was used to depict attitudes towards body image, which is assessed on the two subscales of rejection of the body and vitality. Multivariate regression analyses were conducted separately for females and males, taking into account age and socio-economic position. Results: Impairments of everyday activities had only a few substantial associations with psychological symptoms. No significant effects of cardiac functional capacity as a standardized physiological measure emerged. Psychological symptoms were strongly influenced by perceptions of body image, particularly if they rejected it, this holding particularly for males. There were no gender differences in terms of psychological symptoms. Conclusions: Limitations of activity, and impaired cardiac performance, have only minor effects on psychological symptoms in patients with surgically corrected congenital cardiac disease. The perception of body image was the strongest predictor, especially if patients rejected their body as a result of disfigurement or perceived deficiency. © 2006 Cambridge University Press. AD - S. Geyer, Medical Sociology Unit, Medizinische Hochschule Hannover, 30635 Hannover, Germany AU - Geyer, S. AU - Norozi, K. AU - Zoege, M. AU - Kempa, A. AU - Buchhorn, R. AU - Wessel, A. DB - Embase Medline DO - 10.1017/S1047951106001442 IS - 6 KW - adolescent adult age anxiety aortic coarctation article body image Brief Symptom Inventory cardiopulmonary function congenital heart disease controlled study daily life activity depression Fallot tetralogy female great vessels transposition heart left right shunt heart performance heart single ventricle heart surgery hostility human interview major clinical study male mental disease multivariate analysis obsessive compulsive disorder paranoia patent ductus arteriosus phobia postoperative period prediction psychosis questionnaire regression analysis sex difference socioeconomics somatization LA - English M3 - Article N1 - L44782594 2006-12-07 PY - 2006 SN - 1047-9511 1467-1107 SP - 540-548 ST - Psychological symptoms in patients after surgery for congenital cardiac disease T2 - Cardiology in the Young TI - Psychological symptoms in patients after surgery for congenital cardiac disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L44782594 http://dx.doi.org/10.1017/S1047951106001442 VL - 16 ID - 1245 ER - TY - JOUR AB - OBJECTIVE: To evaluate the psychosocial status of mothers and fathers of infants with hypoplastic left heart syndrome while in the PICU. DESIGN: A retrospective study combining interviews and psychometric testing of parents. SETTING: Tertiary hospital PICU. SUBJECTS: Twenty-nine parents (16 mothers and 13 fathers) of surviving children. INTERVENTION: A semistructured face-to-face interview was conducted to explore parental experiences, and a Structured Clinical Interview for Diagnosis-Clinical Version (posttraumatic stress disorder module) was conducted to determine the possibility of an acute stress disorder or a posttraumatic stress disorder. MEASUREMENT AND MAIN RESULTS: All parents reported multiple stresses which commenced with their infant's diagnosis and endured throughout their infant's time in PICU. The Structured Clinical Interview for Diagnosis revealed that acute stress disorder or posttraumatic stress disorder developed in 24 parents (83%). Of 18 parents whose infants were diagnosed with hypoplastic left heart syndrome in utero, eight of nine mothers (88%) and six of nine fathers (66%) had posttraumatic stress disorder. Of 11 parents whose infants were diagnosed with hypoplastic left heart syndrome postbirth, six of seven mothers had acute stress disorder and one mother had posttraumatic stress disorder, and of four fathers, two fathers had acute stress disorder and one father had posttraumatic stress disorder. The prevalence of parental stress-related disorder was not different between mothers and fathers (p = 0.85). Only five parents were free of traumatic stress-related illness. Parents also experienced losses. Many parents were marginalized from their infant's care by the environment of PICU. Fifty percent of mothers experienced difficulties with parental-infant bonding. Ten parents (34%) began the process of adaptation to their infant's hypoplastic left heart syndrome and were assisted by the support and sensitivity of staff or had discovered other resources. CONCLUSIONS: All parents of surviving infants with hypoplastic left heart syndrome in PICU, irrespective of timing of diagnosis, experienced numerous stresses and losses, and the majority exhibited clinical levels of traumatic stress. Receiving the diagnosis itself is very traumatic and is compounded by the environment of the PICU which alienates parents from their infants and interferes with parent-infant bonding. Parental adaptation to this situation can be assisted by staff. AD - All authors: Paediatric Intensive Care Unit, Royal Children's Hospital, Melbourne, Victoria, Australia. AN - 23965635 AU - Cantwell-Bartl, A. M. AU - Tibballs, J. DA - Nov DB - PubMed DO - 10.1097/PCC.0b013e31829b1a88 DP - NLM ET - 2013/08/24 IS - 9 KW - Adaptation, Psychological Adolescent Child Child, Preschool Fathers/*psychology Female Health Facility Environment Humans Hypoplastic Left Heart Syndrome/*diagnosis/surgery Infant Infant, Newborn Intensive Care Units, Pediatric Interviews as Topic Male Mother-Child Relations Mothers/*psychology Object Attachment Pregnancy Prenatal Diagnosis/psychology Psychiatric Status Rating Scales Retrospective Studies Stress Disorders, Post-Traumatic/*etiology/psychology Stress Disorders, Traumatic, Acute/*etiology/psychology Truth Disclosure Young Adult LA - eng N1 - Cantwell-Bartl, Annie M Tibballs, James Journal Article United States Pediatr Crit Care Med. 2013 Nov;14(9):869-75. doi: 10.1097/PCC.0b013e31829b1a88. PY - 2013 SN - 1529-7535 (Print) 1529-7535 SP - 869-75 ST - Psychosocial experiences of parents of infants with hypoplastic left heart syndrome in the PICU T2 - Pediatr Crit Care Med TI - Psychosocial experiences of parents of infants with hypoplastic left heart syndrome in the PICU VL - 14 ID - 134 ER - TY - JOUR AB - The aim was to describe the psychological functioning of parents of school-age children with heart disease (HD) in a large-scale, transnational evaluation of parent dyads across the spectrum of cardiac diagnoses and a range of psychosocial domains. Parents of children with HD attending routine out-patient cardiology follow-up visits completed questionnaires assessing their mental health, coping, and family functioning. Parents (1197 mothers and 1053 fathers) of 1214 children (mean age: 12.6 years; S.D. 3.0 years; median time since last surgery: 8.9 years) with congenital or acquired HD from three centers each in the UK and the USA participated (80% response rate). Parents of children with milder HD demonstrated few differences from healthy norms and had significantly lower scores on measures of illness-related stress and post-traumatic stress than parents of children with single ventricle conditions or cardiomyopathy. Parents in these latter two diagnostic sub-groups had significantly higher levels of anxiety and depression than healthy norms but did not differ on other measures of family functioning and coping skills. There were few differences between parents from the UK and the USA. Agreement between mothers and fathers within a dyad was highest for the measure of frequency of illness-related stressors (ICC = 0.67) and lowest for anxiety (ICC = 0.12).Conclusion: Our results suggest two different pathways for the long-term psychological well-being of parents of children with HD: on the one hand, more complex HD is associated with poorer long-term psychosocial outcomes; in contrast, there are also grounds for optimism, particularly for parents of children with less complex conditions, with better psychological outcomes noted for some groups of parents compared to previously reported early psychosocial outcomes. Future work needs to identify factors other than disease severity which might explain poorer (or better) functioning in some parents of children with more complex HD. What is Known: • Parents of children with congenital heart disease report elevated levels of anxiety, depression, and stress after cardiac surgery in infancy. • Maternal mental health problems can have an adverse impact on the psychological adjustment of the child with congenital heart disease. What is New: • Parents of children with milder forms of heart disease do not differ from healthy norms in the longer term and psychological outcomes are better than might be expected from early findings. • More complex diagnoses, particularly functional single ventricle conditions and cardiomyopathy, are associated with poorer long-term psychosocial outcomes for parents. AD - Critical Care and Cardiorespiratory Department, Great Ormond Street Hospital for Children NHS Foundation Trust, Great Ormond Street, London, WC1N 3JH, UK. jo.wray@gosh.nhs.uk. Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA. Department of Pediatrics, Division of Behavioral Medicine and Clinical Psychology, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH, USA. Department of Pediatric Cardiology, Royal Brompton and Harefield NHS Foundation Trust, London, UK. Critical Care and Cardiorespiratory Department, Great Ormond Street Hospital for Children NHS Foundation Trust, Great Ormond Street, London, WC1N 3JH, UK. Department of Pediatrics, Divisions of Pediatric Cardiology and Critical Care Medicine, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA. AN - 30232593 AU - Wray, J. AU - Cassedy, A. AU - Ernst, M. M. AU - Franklin, R. C. AU - Brown, K. AU - Marino, B. S. DA - Dec DB - PubMed DO - 10.1007/s00431-018-3250-7 DP - NLM ET - 2018/09/21 IS - 12 KW - *Adaptation, Psychological Adolescent Adult Child Child, Preschool Cross-Sectional Studies Female Heart Diseases/*psychology Humans Male Mental Disorders/*epidemiology/etiology Middle Aged Parenting/*psychology Parents/*psychology Stress, Psychological/psychology Surveys and Questionnaires United Kingdom United States Heart disease Parents Psychological functioning LA - eng N1 - 1432-1076 Wray, Jo Orcid: 0000-0002-4769-1211 Cassedy, Amy Ernst, Michelle M Franklin, Rodney C Brown, Katherine Marino, Bradley S 5-K23-HD048637-05/Eunice Kennedy Shriver National Institute of Child Health and Human Development/ 0465467/American Heart Association/ Journal Article Multicenter Study Germany Eur J Pediatr. 2018 Dec;177(12):1811-1821. doi: 10.1007/s00431-018-3250-7. Epub 2018 Sep 19. PY - 2018 SN - 0340-6199 SP - 1811-1821 ST - Psychosocial functioning of parents of children with heart disease-describing the landscape T2 - Eur J Pediatr TI - Psychosocial functioning of parents of children with heart disease-describing the landscape VL - 177 ID - 10 ER - TY - JOUR AB - The aim was to describe the psychological functioning of parents of school-age children with heart disease (HD) in a large-scale, transnational evaluation of parent dyads across the spectrum of cardiac diagnoses and a range of psychosocial domains. Parents of children with HD attending routine out-patient cardiology follow-up visits completed questionnaires assessing their mental health, coping, and family functioning. Parents (1197 mothers and 1053 fathers) of 1214 children (mean age: 12.6 years; S.D. 3.0 years; median time since last surgery: 8.9 years) with congenital or acquired HD from three centers each in the UK and the USA participated (80% response rate). Parents of children with milder HD demonstrated few differences from healthy norms and had significantly lower scores on measures of illness-related stress and post-traumatic stress than parents of children with single ventricle conditions or cardiomyopathy. Parents in these latter two diagnostic sub-groups had significantly higher levels of anxiety and depression than healthy norms but did not differ on other measures of family functioning and coping skills. There were few differences between parents from the UK and the USA. Agreement between mothers and fathers within a dyad was highest for the measure of frequency of illness-related stressors (ICC = 0.67) and lowest for anxiety (ICC = 0.12). Conclusion: Our results suggest two different pathways for the long-term psychological well-being of parents of children with HD: on the one hand, more complex HD is associated with poorer long-term psychosocial outcomes; in contrast, there are also grounds for optimism, particularly for parents of children with less complex conditions, with better psychological outcomes noted for some groups of parents compared to previously reported early psychosocial outcomes. Future work needs to identify factors other than disease severity which might explain poorer (or better) functioning in some parents of children with more complex HD.What is Known:• Parents of children with congenital heart disease report elevated levels of anxiety, depression, and stress after cardiac surgery in infancy.• Maternal mental health problems can have an adverse impact on the psychological adjustment of the child with congenital heart disease.What is New:• Parents of children with milder forms of heart disease do not differ from healthy norms in the longer term and psychological outcomes are better than might be expected from early findings.• More complex diagnoses, particularly functional single ventricle conditions and cardiomyopathy, are associated with poorer long-term psychosocial outcomes for parents. AD - J. Wray, Critical Care and Cardiorespiratory Department, Great Ormond Street Hospital for Children NHS Foundation Trust, Great Ormond Street, London, United Kingdom AU - Wray, J. AU - Cassedy, A. AU - Ernst, M. M. AU - Franklin, R. C. AU - Brown, K. AU - Marino, B. S. DB - Embase Medline DO - 10.1007/s00431-018-3250-7 IS - 12 KW - adolescent adult anxiety article cardiomyopathy child coping behavior cross-sectional study depression disease severity family functioning father female heart disease heart single ventricle human major clinical study male mental health mother parent parental stress posttraumatic stress disorder priority journal questionnaire social psychology United Kingdom United States LA - English M3 - Article N1 - L624013041 2018-09-27 2019-01-11 PY - 2018 SN - 1432-1076 0340-6199 SP - 1811-1821 ST - Psychosocial functioning of parents of children with heart disease—describing the landscape T2 - European Journal of Pediatrics TI - Psychosocial functioning of parents of children with heart disease—describing the landscape UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L624013041 http://dx.doi.org/10.1007/s00431-018-3250-7 VL - 177 ID - 649 ER - TY - JOUR AB - AIMS AND OBJECTIVES: To investigate parents' experiences, coping ability and quality of life while monitoring their sick child with hypoplastic left heart syndrome at home. BACKGROUND: Interstage home monitoring for children with hypoplastic left heart syndrome reduces interstage mortality between Norwood stages I and II. Little is known about the psychosocial impact of interstage home monitoring. DESIGN: Prospective mixed-method study. METHODS: This study assessed the psychosocial impact on parents during interstage home monitoring. This contains for quantitative assessment the Short Form Health Survey questionnaire and the Impact of Family Scale administered one and five weeks following discharge before and after stage II. For qualitative assessment, semi-structured interviews focussing on the postdischarge coping strategies were conducted twice, five weeks after hospital discharge before and after stage II. RESULTS: Ten infants (eight males) with hypoplastic left heart syndrome (n = 7) or other types of univentricular heart malformations (n = 3), and their parents (nine mother/father two-parent households, one single mother) were included. There were no interstage deaths. Mental Health Composite Summary scores were low in both parents (mothers: 40·45 ± 9·07; fathers: 40·58 ± 9·69) and lowest for the item 'vitality' (mothers: 37·0 ± 19·46; fathers: 43·12 ± 25·9) before and after stage II. Impact of Family Scale values showed higher daily and social burdens for mothers. 'Becoming a family' was the most important task as coping strategy to equilibrate the fragile emotional balance. The parents judged interstage home monitoring as a protective intervention. CONCLUSIONS: Although psychosocial burden before and after stage II remains high, becoming a family is an essential experience for parents and confirms their parenthood. RELEVANCE TO CLINICAL PRACTICE: Healthcare professionals must be aware of parents' needs during this vulnerable interstage period and to provide psychosocial and nursing support. AD - Division of Cardiology, Pediatric Heart Center, University Children's Hospital, Zurich, Switzerland. Children's Research Center, University Children's Hospital, Zurich, Switzerland. Nursing and MTTB, University Hospital Zürich and Institute of Nursing Science, University of Basel, Basel, Switzerland. Division of Neonatology and Intensive Care, Pediatric Heart Center, University Children's Hospital, Zurich, Switzerland. Division of Cardiovascular Surgery, Pediatric Heart Center, University Children's Hospital, Zurich, Switzerland. AN - 28000391 AU - Stoffel, G. AU - Spirig, R. AU - Stiasny, B. AU - Bernet, V. AU - Dave, H. AU - Knirsch, W. DA - Nov DB - PubMed DO - 10.1111/jocn.13694 DP - NLM ET - 2016/12/22 IS - 21-22 KW - *Adaptation, Psychological Adult Female Humans Hypoplastic Left Heart Syndrome/physiopathology/*psychology/therapy Infant Infant, Newborn Male Monitoring, Physiologic/psychology Parents/*psychology Prospective Studies Qualitative Research *Quality of Life Surveys and Questionnaires Young Adult family hypoplastic left heart syndrome interstage monitoring psychosocial impact quality of life LA - eng N1 - 1365-2702 Stoffel, Gaby Spirig, Rebecca Stiasny, Brian Bernet, Vera Dave, Hitendu Knirsch, Walter Journal Article England J Clin Nurs. 2017 Nov;26(21-22):3363-3370. doi: 10.1111/jocn.13694. Epub 2017 Feb 14. PY - 2017 SN - 0962-1067 SP - 3363-3370 ST - Psychosocial impact on families with an infant with a hypoplastic left heart syndrome during and after the interstage monitoring period - a prospective mixed-method study T2 - J Clin Nurs TI - Psychosocial impact on families with an infant with a hypoplastic left heart syndrome during and after the interstage monitoring period - a prospective mixed-method study VL - 26 ID - 104 ER - TY - JOUR AB - Aims and objectives: To investigate parents’ experiences, coping ability and quality of life while monitoring their sick child with hypoplastic left heart syndrome at home. Background: Interstage home monitoring for children with hypoplastic left heart syndrome reduces interstage mortality between Norwood stages I and II. Little is known about the psychosocial impact of interstage home monitoring. Design: Prospective mixed‐method study. Methods: This study assessed the psychosocial impact on parents during interstage home monitoring. This contains for quantitative assessment the Short Form Health Survey questionnaire and the Impact of Family Scale administered one and five weeks following discharge before and after stage II. For qualitative assessment, semi‐structured interviews focussing on the postdischarge coping strategies were conducted twice, five weeks after hospital discharge before and after stage II. Results: Ten infants (eight males) with hypoplastic left heart syndrome (n = 7) or other types of univentricular heart malformations (n = 3), and their parents (nine mother/father two‐parent households, one single mother) were included. There were no interstage deaths. Mental Health Composite Summary scores were low in both parents (mothers: 40·45 ± 9·07; fathers: 40·58 ± 9·69) and lowest for the item ‘vitality’ (mothers: 37·0 ± 19·46; fathers: 43·12 ± 25·9) before and after stage II. Impact of Family Scale values showed higher daily and social burdens for mothers. ‘Becoming a family’ was the most important task as coping strategy to equilibrate the fragile emotional balance. The parents judged interstage home monitoring as a protective intervention. Conclusions Although psychosocial burden before and after stage II remains high, becoming a family is an essential experience for parents and confirms their parenthood. Relevance to clinical practice Healthcare professionals must be aware of parents’ needs during this vulnerable interstage period and to provide psychosocial and nursing support. (PsycInfo Database Record (c) 2020 APA, all rights reserved) AD - Knirsch, Walter, Division of Cardiology, Pediatric Heart Center, University Children’s Hospital, Steinwiesstrasse 75, 8032, Zurich, Switzerland AN - 2017-07152-001 AU - Stoffel, Gaby AU - Spirig, Rebecca AU - Stiasny, Brian AU - Bernet, Vera AU - Dave, Hitendu AU - Knirsch, Walter DB - psyh DO - 10.1111/jocn.13694 DP - EBSCOhost IS - 21-22 KW - family hypoplastic left heart syndrome infant interstage monitoring psychosocial impact quality of life Adaptation, Psychological Adult Female Humans Infant, Newborn Male Monitoring, Physiologic Parents Prospective Studies Qualitative Research Surveys and Questionnaires Young Adult Cardiovascular Disorders Monitoring Nursing N1 - Division of Cardiology, Pediatric Heart Center, University Children’s Hospital, Zurich, Switzerland. Other Publishers: Blackwell Publishing. Release Date: 20170216. Correction Date: 20200611. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Cardiovascular Disorders; Monitoring; Nursing; Quality of Life. Minor Descriptor: Family. Classification: Cardiovascular Disorders (3295); Health & Mental Health Services (3370). Population: Human (10); Male (30); Female (40). Location: Switzerland. Age Group: Childhood (birth-12 yrs) (100); Infancy (2-23 mo) (140); Adulthood (18 yrs & older) (300); Young Adulthood (18-29 yrs) (320); Thirties (30-39 yrs) (340). Tests & Measures: Impact on Family Scale DOI: 10.1037/t06338-000; 36-Item Short Form Health Survey DOI: 10.1037/t07023-000. Methodology: Empirical Study; Interview; Quantitative Study. References Available: Y. Page Count: 8. Issue Publication Date: Nov, 2017. Publication History: Accepted Date: Dec 10, 2016. Copyright Statement: John Wiley & Sons Ltd. 2016. Sponsor: Anna Müller Grocholski Foundation. Other Details: (Reachout II). Recipients: No recipient indicated PY - 2017 SN - 0962-1067 1365-2702 SP - 3363-3370 ST - Psychosocial impact on families with an infant with a hypoplastic left heart syndrome during and after the interstage monitoring period—A prospective mixed-method study T2 - Journal of Clinical Nursing TI - Psychosocial impact on families with an infant with a hypoplastic left heart syndrome during and after the interstage monitoring period—A prospective mixed-method study UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2017-07152-001&site=ehost-live&scope=site walter.knirsch@kispi.uzh.ch VL - 26 ID - 1662 ER - TY - JOUR AB - The purpose of the current study was to assess the psychosocial outcomes of preschool-aged survivors (ages 3-6 years) of hypoplastic left heart syndrome (HLHS; n=13) and transposition of the great arteries (TGA; n=13). Parents completed the following measures: Pediatric Quality of Life Inventory, Impact on the Family Scale, Parenting Stress Index, Parent Behavior Checklist, and Child Behavior Checklist. Quality of life scores did not differ from those of healthy controls. Parents of children with HLHS reported more negative impact of the child's illness on the family and more parenting stress than parents of children with TGA. Parents of both groups of children were more permissive in their parenting style than parents of healthy controls. Children with HLHS had higher rates of attention and externalizing behavior problems than children with TGA. The results highlight the need for practitioners working with these children and families to ask about parental stress, family functioning, and behavioral expectations for the child in the context of routine medical/cardiac follow-up. AD - Department of Pediatrics, Medical College of Wisconsin, and Children's Hospital of Wisconsin, Herma Heart Center, Milwaukee, WI 53226, USA. cbrosig@mcw.edu AN - 17486393 AU - Brosig, C. L. AU - Mussatto, K. A. AU - Kuhn, E. M. AU - Tweddell, J. S. DA - Jul-Aug DB - PubMed DO - 10.1007/s00246-006-0013-4 DP - NLM ET - 2007/05/09 IS - 4 KW - Child Child, Preschool *Family Health Female Humans Hypoplastic Left Heart Syndrome/psychology/*surgery Male Parenting/psychology Stress, Psychological Transposition of Great Vessels/psychology/*surgery LA - eng N1 - Brosig, C L Mussatto, K A Kuhn, E M Tweddell, J S Journal Article Research Support, Non-U.S. Gov't United States Pediatr Cardiol. 2007 Jul-Aug;28(4):255-62. doi: 10.1007/s00246-006-0013-4. Epub 2007 May 5. PY - 2007 SN - 0172-0643 (Print) 0172-0643 SP - 255-62 ST - Psychosocial outcomes for preschool children and families after surgery for complex congenital heart disease T2 - Pediatr Cardiol TI - Psychosocial outcomes for preschool children and families after surgery for complex congenital heart disease VL - 28 ID - 215 ER - TY - JOUR AB - Objective: To evaluate the psychosocial status of mothers and fathers in response to their infant's diagnosis of hypoplastic left heart syndrome. Design: A study on interviews with parents whose children had survived staged surgery. Setting: Tertiary hospital paediatric ICU. Subjects: A total of 29 parents (16 mothers and 13 fathers) of surviving children. Intervention: A semi-structured face-to-face interview was conducted to explore experiences of parents in response to their infant's diagnosis, their interaction with the doctor delivering the diagnosis, their deliberation about staged surgery, and their reasons for this choice. Measurement and Main Results: All parents were devastated about their infant's diagnosis, and most (83%) of them said that the time of the diagnosis and the aftermath was the worst time of their lives. Parents reported helpful and unhelpful communication at this time. Although all parents in this study chose surgery for their infant, when faced with the choice, 17 of them made an immediate decision to protect their infant's life, 8 were initially unsure when their infant was diagnosed in utero, and 4 were unsure when the infant was diagnosed after birth. Parents also experienced loss and other stressors. Conclusions: All parents of the infants diagnosed with hypoplastic left heart syndrome experienced intense loss and stressors. Physicians need to be sensitive to the needs and thinking of the parents when discussing treatment options before surgery. The nature of the relationship with the doctor at this time can support parents or be a further source of stress. © © Cambridge University Press 2014. AD - Honorary Research Fellow, Paediatric Intensive Care Unit, Royal Children's Hospital, Melbourne, VIC 3052, Australia AU - Cantwell-Bartl, A. M. AU - Tibballs, J. DB - Scopus DO - 10.1017/S1047951114001590 IS - 6 KW - adaptation hypoplastic left heart syndrome Intensive care parents psychological stress M3 - Article N1 - Cited By :6 Export Date: 15 June 2020 PY - 2014 SP - 1065-1073 ST - Psychosocial responses of parents to their infant's diagnosis of hypoplastic left heart syndrome T2 - Cardiology in the Young TI - Psychosocial responses of parents to their infant's diagnosis of hypoplastic left heart syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84945469068&doi=10.1017%2fS1047951114001590&partnerID=40&md5=e54a054d03586dccb0d41919ed4dc871 VL - 25 ID - 1989 ER - TY - JOUR AB - OBJECTIVE: To evaluate the psychosocial status of mothers and fathers in response to their infant's diagnosis of hypoplastic left heart syndrome. DESIGN: A study on interviews with parents whose children had survived staged surgery. SETTING: Tertiary hospital paediatric ICU. SUBJECTS: A total of 29 parents (16 mothers and 13 fathers) of surviving children. INTERVENTION: A semi-structured face-to-face interview was conducted to explore experiences of parents in response to their infant's diagnosis, their interaction with the doctor delivering the diagnosis, their deliberation about staged surgery, and their reasons for this choice. MEASUREMENT AND MAIN RESULTS: All parents were devastated about their infant's diagnosis, and most (83%) of them said that the time of the diagnosis and the aftermath was the worst time of their lives. Parents reported helpful and unhelpful communication at this time. Although all parents in this study chose surgery for their infant, when faced with the choice, 17 of them made an immediate decision "to protect their infant's life", 8 were initially unsure when their infant was diagnosed in utero, and 4 were unsure when the infant was diagnosed after birth. Parents also experienced loss and other stressors. CONCLUSIONS: All parents of the infants diagnosed with hypoplastic left heart syndrome experienced intense loss and stressors. Physicians need to be sensitive to the needs and thinking of the parents when discussing treatment options before surgery. The nature of the relationship with the doctor at this time can support parents or be a further source of stress. AD - Paediatric Intensive Care Unit,Royal Children's Hospital,Melbourne,Victoria,Australia. AN - 25215886 AU - Cantwell-Bartl, A. M. AU - Tibballs, J. DA - Aug DB - PubMed DO - 10.1017/s1047951114001590 DP - NLM ET - 2014/09/13 IS - 6 KW - *Adaptation, Psychological Adult Attitude to Health Child Communication Fathers/*psychology Female Humans Hypoplastic Left Heart Syndrome/epidemiology/*surgery Intensive Care Units Interviews as Topic Male Mothers/*psychology Perception Retrospective Studies *Stress, Psychological Tertiary Care Centers Intensive care adaptation hypoplastic left heart syndrome parents psychological stress LA - eng N1 - 1467-1107 Cantwell-Bartl, Annie M Orcid: 0000-0002-4132-0743 Tibballs, James Orcid: 0000-0002-4433-004x Journal Article England Cardiol Young. 2015 Aug;25(6):1065-73. doi: 10.1017/S1047951114001590. Epub 2014 Sep 12. PY - 2015 SN - 1047-9511 SP - 1065-73 ST - Psychosocial responses of parents to their infant's diagnosis of hypoplastic left heart syndrome T2 - Cardiol Young TI - Psychosocial responses of parents to their infant's diagnosis of hypoplastic left heart syndrome VL - 25 ID - 132 ER - TY - JOUR AB - OBJECTIVE: The aim of this study was to explore the acute psychosocial risk in families with infants undergoing surgery for a congenital heart disease and, secondarily, to explore the psychosocial impact of antenatal versus post-natal diagnoses. METHOD: The study sample comprised 39 caregivers (28 mothers) of 29 children diagnosed with a congenital heart disease and requiring surgery within the first 4 weeks of life. Psychosocial risk was measured using the Psychosocial Assessment Tool, which was adapted to include four novel items examining infant risk factors, namely, sleeping, feeding, crying, and bonding difficulties. Parents' psychosocial risk was measured within 4 weeks after their child's surgery and stratified into a three-tiered framework: Universal, Targeted, and Clinical risk. RESULTS: Of the total sample, 61.5% of parents were classified as Universal, that is, at lowest risk; 35.9% as Targeted, and 2.6% as Clinical. The within-family parent total Psychosocial Assessment Tool score correlations were non-significant, and there were no differences between families of infants who received post-natal versus antenatal diagnosis or single ventricle versus biventricular repair. Linear regression found that a higher parent education significantly predicted a lower total Psychosocial Assessment Tool score. CONCLUSIONS: Findings indicate that, although the majority of parents adapt to the acute stress of surgery for a serious cardiac illness in their infant, the remaining 38.5% report an increased psychosocial risk associated with higher rates of emotional distress, which may impact on the parental quality of life and capacity for optimal parenting. The distribution of psychosocial risk in parents of children undergoing surgery for a congenital heart disease is consistent with that described for parents of children with other serious paediatric diagnoses. AD - 1Critical Care and Neurosciences,Murdoch Children's Research Institute,Melbourne,Australia. 5The Parenting Research Centre,Melbourne,Australia. AN - 23803336 AU - Hearps, S. J. AU - McCarthy, M. C. AU - Muscara, F. AU - Hearps, S. J. AU - Burke, K. AU - Jones, B. AU - Anderson, V. A. DA - Aug DB - PubMed DO - 10.1017/s1047951113000760 DP - NLM ET - 2013/06/28 IS - 4 KW - Adult Female Heart Defects, Congenital/*surgery Humans Infant Infant, Newborn Linear Models Male Parent-Child Relations Parenting/*psychology Parents/*psychology Prenatal Diagnosis Quality of Life/*psychology Risk Assessment Stress, Psychological/*psychology Surveys and Questionnaires Young Adult LA - eng N1 - 1467-1107 Hearps, Simone J McCarthy, Maria C Muscara, Frank Hearps, Stephen J C Burke, Kylie Jones, Bryn Anderson, Vicki A Journal Article Research Support, Non-U.S. Gov't England Cardiol Young. 2014 Aug;24(4):632-9. doi: 10.1017/S1047951113000760. Epub 2013 Jun 27. PY - 2014 SN - 1047-9511 SP - 632-9 ST - Psychosocial risk in families of infants undergoing surgery for a serious congenital heart disease T2 - Cardiol Young TI - Psychosocial risk in families of infants undergoing surgery for a serious congenital heart disease VL - 24 ID - 49 ER - TY - JOUR AB - Objective: The aim of this study was to explore the acute psychosocial risk in families with infants undergoing surgery for a congenital heart disease and, secondarily, to explore the psychosocial impact of antenatal versus post-natal diagnoses. Method: The study sample comprised 39 caregivers (28 mothers) of 29 children diagnosed with a congenital heart disease and requiring surgery within the first 4 weeks of life. Psychosocial risk was measured using the Psychosocial Assessment Tool, which was adapted to include four novel items examining infant risk factors, namely, sleeping, feeding, crying, and bonding difficulties. Parents' psychosocial risk was measured within 4 weeks after their child's surgery and stratified into a three-tiered framework: Universal, Targeted, and Clinical risk. Results: Of the total sample, 61.5% of parents were classified as Universal, that is, at lowest risk; 35.9% as Targeted, and 2.6% as Clinical. The within-family parent total Psychosocial Assessment Tool score correlations were non-significant, and there were no differences between families of infants who received post-natal versus antenatal diagnosis or single ventricle versus biventricular repair. Linear regression found that a higher parent education significantly predicted a lower total Psychosocial Assessment Tool score. Conclusions: Findings indicate that, although the majority of parents adapt to the acute stress of surgery for a serious cardiac illness in their infant, the remaining 38.5% report an increased psychosocial risk associated with higher rates of emotional distress, which may impact on the parental quality of life and capacity for optimal parenting. The distribution of psychosocial risk in parents of children undergoing surgery for a congenital heart disease is consistent with that described for parents of children with other serious paediatric diagnoses. © Cambridge University Press 2013. AD - S.J. Hearps, Department of Pediatrics, Peking University First Hospital, Beijing 100034, China AU - Hearps, S. J. AU - McCarthy, M. C. AU - Muscara, F. AU - Hearps, S. J. C. AU - Burke, K. AU - Jones, B. AU - Anderson, V. A. DB - Embase DO - 10.1017/S1047951113000760 IS - 4 KW - adult article congenital heart disease correlational study disease severity educational status emotional stress environmental factor female human male parental attitude parental stress pediatric surgery perinatal period prenatal diagnosis psychologic assessment psychosocial risk quality of life risk social psychology LA - English M3 - Article N1 - L373426505 2014-07-10 2014-07-15 PY - 2014 SN - 1467-1107 1047-9511 SP - 632-639 ST - Psychosocial risk in families of infants undergoing surgery for a serious congenital heart disease T2 - Cardiology in the Young TI - Psychosocial risk in families of infants undergoing surgery for a serious congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L373426505 http://dx.doi.org/10.1017/S1047951113000760 VL - 24 ID - 933 ER - TY - JOUR AB - Congenital heart disease (CHD), the most common inborn defect, affects approximately 1% of all newborns worldwide. Advances in its diagnosis and treatment have led to a dramatic improvement in patients' quality of life and long-term survival prospects. However, recently it has been realised that many of these patients are affected by ongoing and life-long cardiac issues, namely residual and progressive haemodynamic lesions, arrhythmia and sudden cardiac death, as well as the development of chronic heart failure and pulmonary arterial hypertension (PAH) - all of which merit tertiary care. Unfortunately, many patients with CHD are lost to follow-up, due to the assumption that their initial response to surgical and or catheter intervention in childhood led to cure. Furthermore, there are many patients with undiagnosed or unoperated CHD in the developing world coming to medical attention during adulthood. Our article focuses on advances in the management of PAH associated with CHD, a common association with an adverse impact on quality of life and survival prospects that affects approximately 10% of patients with CHD. Much of the recent progress in PAH-CHD has focused on the extreme end of the disease spectrum, namely on Eisenmenger syndrome. Herein we discuss this progress and future directions for this emerging cardiovascular field. AD - NIHR Cardiovascular BRU, Royal Brompton Hospital and the National Heart and Lung Institute, Imperial College, London, UK. Electronic address: M.Gatzoulis@rbht.nhs.uk. Children's Hospital, University of Geneva, Geneva, Switzerland. BACH Pulmonary Hypertension Service, Departments of Paediatrics, Medicine and Surgery, Boston, USA. Department of Experimental, Diagnostic and Specialty Medicine-DIMES, Bologna University Hospital, Bologna, Italy. AN - 25443244 AU - Gatzoulis, M. A. AU - Beghetti, M. AU - Landzberg, M. J. AU - Galiè, N. DA - Dec 15 DB - PubMed DO - 10.1016/j.ijcard.2014.09.024 DP - NLM ET - 2014/12/03 IS - 2 KW - Animals Eisenmenger Complex/diagnosis/physiopathology/therapy Forecasting Heart Defects, Congenital/*diagnosis/*physiopathology/therapy Hemodynamics/physiology Humans Hypertension, Pulmonary/*diagnosis/*physiopathology/therapy Congenital heart disease Eisenmenger syndrome Fontan circulation Left-to-right shunts Pulmonary arterial hypertension LA - eng N1 - 1874-1754 Gatzoulis, Michael A Beghetti, Maurice Landzberg, Michael J Galiè, Nazzareno Journal Article Research Support, Non-U.S. Gov't Review Netherlands Int J Cardiol. 2014 Dec 15;177(2):340-7. doi: 10.1016/j.ijcard.2014.09.024. Epub 2014 Sep 28. PY - 2014 SN - 0167-5273 SP - 340-7 ST - Pulmonary arterial hypertension associated with congenital heart disease: recent advances and future directions T2 - Int J Cardiol TI - Pulmonary arterial hypertension associated with congenital heart disease: recent advances and future directions VL - 177 ID - 542 ER - TY - JOUR AB - Background: Stent implant is considered the treatment of choice for most cases of congenital or postoperative pulmonary artery branch stenosis in patients with congenital heart defects. However, there are still doubts on the clinical effectiveness and the incidence of complications of this technique that need to be elucidated. Methods: This multicenter study has a descriptive, observational and retrospective design. The procedures were performed between January 2005 and April 2015. Twenty-five patients with pulmonary artery branch stenosis underwent stent implant and were followed-up both clinically and by different imaging tests. Persistent clinical and/or anatomic improvement defined clinical effectiveness. Results: Average age was 9.48 years (2 months-34 years), mean weight was 27.54 kg (3-104 kg) and 44% were women. The most frequent congenital heart defects in the treated cohort were tetralogy of Fallot and its variants, followed by single ventricle heart physiology after bidirectional Glenn shunt and/or Fontan-Kreutzer procedures. Right ventricular systolic and pulmonary artery pressure decreased significantly after the intervention (from 68.35 mmHg to 45.8 mmHg and from 47.4 mm Hg to 32 mm Hg, respectively) (p < 0.0001). The initial minimum diameter of the treated pulmonary artery branch increased significantly after the procedure (from 3.98 mm to 9.82 mm, p < 0.0001). The incidence of complications was 8% (n = 2) and the clinical effectiveness of the procedure was good in 22 patients (88%). Conclusions: Stent implant in pulmonary artery branch stenosis is a safe and feasible though challenging technique, with significant clinical and anatomic improvement. The procedure represents an alternative to surgery and decreases the need for reinterventions in patients with congenital heart defects. AD - A. Peirone, Department of Pediatric Cardiology, Hospital Universitario Privado de Córdoba, Naciones Unidas 346, Córdoba, Argentina AU - Peirone, A. AU - Contreras, A. AU - Cabrera, M. AU - Guadagnoli, A. F. AU - Gentiletti, A. AU - Schroh, A. AU - Laghezza, L. AU - Juaneda, I. AU - Díaz, J. AU - Kreutzer, C. DB - Embase DO - 10.7775/rac.v84.i1.7789 IS - 1 KW - arterial stent adult angioplasty arterial switch operation arterial trunk artery diameter article bare metal stent Blalock Taussig shunt child congenital heart disease coronary stent coronary stenting Fallot tetralogy female follow up Fontan procedure Glenn shunt human hypoplastic left heart syndrome infant lung artery banding lung artery pressure lung perfusion male medical device complication multicenter study Noonan syndrome nuclear magnetic resonance imaging percutaneous transluminal angioplasty peripheral artery stent pulmonary artery pulmonary valve stenosis self expanding nitinol stent Williams Beuren syndrome Advanta V12 CP Express Liberte NuMed Palmaz Genesis Sentinol LA - English M3 - Article N1 - L609972731 2016-04-25 2016-05-09 PY - 2016 SN - 1850-3748 0034-7000 SP - 20-24 ST - Pulmonary artery branch stent implantation in congenital heart disease: A 10-year multicenter experience T2 - Revista Argentina de Cardiologia TI - Pulmonary artery branch stent implantation in congenital heart disease: A 10-year multicenter experience UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L609972731 http://dx.doi.org/10.7775/rac.v84.i1.7789 VL - 84 ID - 828 ER - TY - JOUR AB - From clinical experiences with the Fontan operation in six cases, a few practical contrivances and operative steps are described. We stress that meticulous care should be taken not to cause any stenotic complication in the outflow tract toward the pulmonary artery, particularly by traction of the right atrial appendage only to achieve a direct anastomosis. Conduit repair would be necessitated by cases in order to expand the indicative criteria vertically as well as horizontally. For conduit material, we used consecutively nonvalved polytetrafluoroethylene that was reinforced by handmade stainless steel wire ring (Gore-Tex, Inc) or by spirally built-in stent (IMPRA, Inc). The latter was eventually useful in obtaining natural curving of the conduit without kinking or compression. Some contrivances in anastomosing a conduit were also proposed to achieve an excellent result. We believe these practical contrivances will serve for expansion of the indicative criteria and promise improved operative outcome. AN - 3829748 AU - Nawa, S. AU - Matsuki, T. AU - Shimizu, A. AU - Kurita, A. AU - Tago, M. AU - Murakami, T. AU - Senoo, Y. AU - Teramoto, S. DA - Apr DB - PubMed DO - 10.1378/chest.91.4.552 DP - NLM ET - 1987/04/01 IS - 4 KW - Adolescent Adult *Blood Vessel Prosthesis Child Heart Defects, Congenital/surgery Humans Methods Polytetrafluoroethylene Prosthesis Design Pulmonary Artery/*surgery Stainless Steel LA - eng N1 - Nawa, S Matsuki, T Shimizu, A Kurita, A Tago, M Murakami, T Senoo, Y Teramoto, S Journal Article United States Chest. 1987 Apr;91(4):552-7. doi: 10.1378/chest.91.4.552. PY - 1987 SN - 0012-3692 (Print) 0012-3692 SP - 552-7 ST - Pulmonary artery connection in the Fontan procedure. Flexible polytetrafluoroethylene conduit for expansion T2 - Chest TI - Pulmonary artery connection in the Fontan procedure. Flexible polytetrafluoroethylene conduit for expansion VL - 91 ID - 484 ER - TY - JOUR AB - PURPOSE: To assess pulmonary artery (PA) size, flow variables, and wall shear stress (WSS) in patients after Fontan operation at a young age. MATERIALS AND METHODS: Flow in the branch PA was obtained with phase contrast velocity-encoded cardiovascular magnetic resonance imaging in 14 patients before and after low-dose dobutamine stress (7.5 microg/kg/min) and in 17 healthy controls at rest. RESULTS: At rest, stroke index, total flow, average, and peak flow rate were all statistically significantly lower in patients than in controls (P<0.001). With stress-testing, all variables increased in patients (P<0.001), apart from stroke index, which did not change. At rest, branch PA area did not differ between patients and controls. Distensibility was lower in patients than in controls (P<0.001). With stress-testing, area and distensibility did not change. At rest, WSS was lower in patients than in controls (P<0.001). WSS increased with stress-testing (P<0.001), but not to the same levels as during resting conditions of the control group. CONCLUSION: PA size is normal long-term after Fontan operation at a young age. Flow variables, distensibility, and WSS are significantly lower compared to healthy controls, and do not show adequate reactions with stress-testing, which is suggestive of pulmonary artery endothelial and/or vascular dysfunction. AD - Department of Pediatrics, Division of Cardiology, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands. AN - 18972351 AU - Robbers-Visser, D. AU - Helderman, F. AU - Strengers, J. L. AU - van Osch-Gevers, L. AU - Kapusta, L. AU - Pattynama, P. M. AU - Bogers, A. J. AU - Krams, R. AU - Helbing, W. A. DA - Nov DB - PubMed DO - 10.1002/jmri.21544 DP - NLM ET - 2008/10/31 IS - 5 KW - Adolescent Child *Dobutamine Exercise Test Female *Fontan Procedure Heart Ventricles/*abnormalities/pathology/*surgery Humans Magnetic Resonance Imaging/*methods Male Organ Size Prognosis Pulmonary Artery/*pathology Treatment Outcome Young Adult LA - eng N1 - Robbers-Visser, Daniëlle Helderman, Frank Strengers, Jan L van Osch-Gevers, Lennie Kapusta, Livia Pattynama, Peter M Bogers, Ad J Krams, Rob Helbing, Willem A Clinical Trial Journal Article United States J Magn Reson Imaging. 2008 Nov;28(5):1101-7. doi: 10.1002/jmri.21544. PY - 2008 SN - 1053-1807 (Print) 1053-1807 SP - 1101-7 ST - Pulmonary artery size and function after Fontan operation at a young age T2 - J Magn Reson Imaging TI - Pulmonary artery size and function after Fontan operation at a young age VL - 28 ID - 301 ER - TY - JOUR AB - Right ventricular size in cases of pulmonary atresia is determined by blood flow into and out of the right ventricle. A hypoplastic or enlarged right ventricle can result. In cases of an intact septum, elevated right ventricular pressure results in retrogated flow of blood from a blind-ending right ventricle into the coronary artery circulation via enlarged myocardial sinusoids. In some cases, no connection exists between the coronary arteries and the aorta. Stress sestamibi myocardial perfusion scan is helpful in determining if myocardium is at risk for ischemia or infarction in cases in which coronary circulation is influenced by right ventricular-coronary artery communications. AD - D.J. Fisher, Diagnostic Radiology, University of Washington, Mail Stop 357115, Seattle, WA 98195, United States AU - Fisher, D. J. AU - Ferency, S. L. AU - Rosenbaum, D. M. AU - McCloskey, J. P. DB - Embase Medline DO - 10.1097/00003072-199708000-00013 IS - 8 KW - methoxy isobutyl isonitrile technetium tc 99m adolescent angiocardiography article case report coronary artery blood flow Fontan procedure heart infarction heart muscle ischemia heart right ventricle hypertrophy heart scintiscanning interventricular septum human pulmonary valve atresia male LA - English M3 - Article N1 - L28063259 1998-03-05 PY - 1997 SN - 0363-9762 SP - 560-561 ST - Pulmonary atresia with intact septum: Findings on exercise stress sestamibi scan T2 - Clinical Nuclear Medicine TI - Pulmonary atresia with intact septum: Findings on exercise stress sestamibi scan UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L28063259 http://dx.doi.org/10.1097/00003072-199708000-00013 VL - 22 ID - 1355 ER - TY - JOUR AB - Background. Pulmonary atresia with ventricular septal defect (PA-VSD) is one of the most common cardiac defects associated with DiGeorge syndrome. The pattern of the pulmonary circulation determines the complexity of this type of heart disease. The aim of this study was to establish the prevalence of DiGeorge syndrome with deletion 22q11 in patients with simple and complex PA-VSD. Methods. Since 1993 we have studied 128 consecutive patients affected by PA-VSD. In 90 of our patients the PA-VSD was considered "simple" (group I), because it was not associated with any other cardiac defects. In the other 38 children the PA-VSD was considered "complex" (group II) owing to the presence of heterotaxia, tricuspid atresia, a double-inlet left ventricle, transposition of the great arteries and congenitally corrected transposition of the great arteries. Results. In group I, 38 patients (42%) had genetic syndromes or major extracardiac anomalies; deletion 22q11 was detected in 31% of cases. Major aortopulmonary collateral arteries were present in 50% of group I patients and in 57% of those with deletion 22q11. In group II, 10 patients (26%) had genetic syndromes or major extracardiac anomalies but none had deletion 22q11 (p<0.005); in no case was the presence of major aortopulmonary collateral arteries observed (p<0.005). Conclusions. PA-VSD is an anatomically and morphogenetically heterogeneous disease: in the setting of DiGeorge syndrome or velocardiofacial syndrome, PA-VSD is associated with a peculiar cardiac pattern and is due to deletion 22, whereas in case of nonsyndromic PA-VSD or when this disease is associated with different syndromes or with other types of cardiac defects, it is due to other morphogenetic mechanisms. AD - B. Marino, Divisione di Cardiologia, Osped. Pediat. Bambino Gesù, Piazza S. Onofrio 4, 00165 Roma, Italy AU - Anaclerio, S. AU - Marino, B. AU - Carotti, A. AU - Digilio, M. C. AU - Toscano, A. AU - Gitto, P. AU - Giannotti, A. AU - Di Donato, R. AU - Dallapiccola, B. DB - Embase Medline IS - 5 KW - anatomy aorta article child chromosome 22q chromosome deletion collateral circulation controlled study DiGeorge syndrome disease association female genetic disorder great vessels transposition heart disease heart left ventricle heart ventricle septum defect human infant pulmonary valve atresia lung circulation major clinical study male morphogenesis newborn prevalence pulmonary artery tricuspid valve atresia velocardiofacial syndrome LA - English M3 - Article N1 - L32536891 2001-06-28 PY - 2001 SN - 1129-471X SP - 384-387 ST - Pulmonary atresia with ventricular septal defect: Prevalence of deletion 22q11 in the different anatomic patterns T2 - Italian Heart Journal TI - Pulmonary atresia with ventricular septal defect: Prevalence of deletion 22q11 in the different anatomic patterns UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L32536891 VL - 2 ID - 1322 ER - TY - JOUR AB - Cardiac and pulmonary pathophysiologies are closely interdependent, which makes the management of patients with congenital heart disease (CHD) all the more complex. Pulmonary complications of CHD can be structural due to compression causing airway malacia or atelectasis of the lung. Surgical repair of CHD can also result in structural trauma to the respiratory system, e.g., chylothorax, subglottic stenosis, or diaphragmatic paralysis. Disruption of the Starling forces in the pulmonary vascular system in certain types of CHD lead to alveolar-capillary membrane damage and pulmonary oedema. This in turn results in poorly compliant lungs with a restrictive lung function pattern that can deteriorate to cause hypoxemia. The circulation post single ventricle palliative surgery (the so called "Fontan circulation") poses a unique spectrum of pulmonary pathophysiology with restrictive lung function and a low pulmonary blood flow state that predisposes to thromboembolic complications and plastic bronchitis. As the population of patients surviving post CHD repair increases, the incidence of pulmonary complications has also increased and presents a unique cohort in both the paediatric and adult clinics. AD - Division of Pulmonary Medicine, The Children's Hospital of Philadelphia, USA. AN - 22208788 AU - Healy, F. AU - Hanna, B. D. AU - Zinman, R. DA - Mar DB - PubMed DO - 10.1016/j.prrv.2011.01.007 DP - NLM ET - 2012/01/03 IS - 1 KW - Adult Child Heart Defects, Congenital/*complications/*surgery Humans *Postoperative Complications Respiratory Tract Diseases/*etiology/*physiopathology LA - eng N1 - 1526-0550 Healy, F Hanna, B D Zinman, R Journal Article Review England Paediatr Respir Rev. 2012 Mar;13(1):10-5. doi: 10.1016/j.prrv.2011.01.007. Epub 2011 Mar 5. PY - 2012 SN - 1526-0542 SP - 10-5 ST - Pulmonary complications of congenital heart disease T2 - Paediatr Respir Rev TI - Pulmonary complications of congenital heart disease VL - 13 ID - 238 ER - TY - JOUR AB - OBJECTIVES: To review the pulmonary findings of the first 51 patients who presented to our interdisciplinary single-ventricle clinic after undergoing the Fontan procedure. STUDY DESIGN: We performed an Institutional Review Board-approved retrospective review of 51 patients evaluated following the Fontan procedure. Evaluation included history, physical examination, pulmonary function testing, and 6-minute walk. Descriptive statistics were used to describe the population and testing data. RESULTS: Sixty-one percent of the patients had a pulmonary concern raised during the visit. Three patients had plastic bronchitis. Abnormal lung function testing was present in 46% of patients. Two-thirds (66%) of the patients had significant desaturation during the 6-minute walk test. Patients who underwent a fenestrated Fontan procedure and those who underwent unfenestrated Fontan were compared in terms of saturation and 6-minute walk test results. Sleep concerns were present in 45% of the patients. CONCLUSIONS: Pulmonary morbidities are common in patients after Fontan surgery and include plastic bronchitis, abnormal lung function, desaturations with walking, and sleep concerns. Abnormal lung function and obstructive sleep apnea may stress the Fontan circuit and may have implications for cognitive and emotional functioning. A pulmonologist involved in the care of patients after Fontan surgery can assist in screening for comorbidities and recommend interventions. AD - Pediatric Pulmonary Medicine, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, CO. Electronic address: deborah.liptzin@childrenscolorado.org. Heart Institute, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, CO. Digestive Health Institute, Children's Hospital Colorado and Section of Pediatric Gastroenterology, Hepatology and Nutrition, University of Colorado School of Medicine, Aurora, CO. Pediatric Neurology, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, CO. Pediatric Pulmonary Medicine, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, CO. AN - 29747936 AU - Liptzin, D. R. AU - Di Maria, M. V. AU - Younoszai, A. AU - Narkewicz, M. R. AU - Kelly, S. L. AU - Wolfe, K. R. AU - Veress, L. A. DA - Aug DB - PubMed DO - 10.1016/j.jpeds.2018.03.050 DP - NLM ET - 2018/05/12 KW - Adolescent Child Child, Preschool Exercise Test Female *Fontan Procedure Humans Incidence Lung Diseases/*diagnosis/epidemiology/etiology Male Medical History Taking Physical Examination Postoperative Care/*methods Postoperative Complications/*diagnosis/epidemiology Respiratory Function Tests Retrospective Studies Young Adult *altitude *congenital *executive function *heart defects *hypoplastic left heart syndrome *neuropsychology *sleep apnea syndromes *sternotomy LA - eng N1 - 1097-6833 Liptzin, Deborah R Di Maria, Michael V Younoszai, Adel Narkewicz, Michael R Kelly, Sarah L Wolfe, Kelly R Veress, Livia A Journal Article United States J Pediatr. 2018 Aug;199:140-143. doi: 10.1016/j.jpeds.2018.03.050. Epub 2018 May 7. PY - 2018 SN - 0022-3476 SP - 140-143 ST - Pulmonary Screening in Subjects after the Fontan Procedure T2 - J Pediatr TI - Pulmonary Screening in Subjects after the Fontan Procedure VL - 199 ID - 68 ER - TY - JOUR AB - The surgical problem of the severely ill infant with obstruction to pulmonary blood flow is distinct from that of the older and larger child, by virtue of the urgent necessity for operation, the additional difficulties of anaesthesia and exposure of the heart and great vessels, and, particularly, the limitations placed upon the surgeon by the size of the vessels and the desirability of not compromising a later corrective or palliative operation. Pulmonary valvulotomies or infundibulotomies were performed upon 18 infants less than one year of age for relief of severe symptôme associated with the following entities: pulmonary valve stenosis or atresia, transposition of the great vessels with ventricular septal defect and pulmonary stenosis, tetralogy of Fallot with infundibular stenosis or pulmonary atresia, tricuspid atresia with ventricular septal defect and pulmonary stenosis, and tricuspid stenosis with pulmonary stenosis. In each of the entities in which a valvulotome could be passed without undue difficulty through a ventricular out-flow tract into the pulmonary artery, a satisfactory increase in pulmonary blood flow was achieved, with relief of symptoms in the 13 survivors. A sternal-splitting incision is recommended as the approach of choice in these infante, utilizing pulmonary valvulotomy or infundibulotomy for the cases in which a suitable anatomical situation can be demonstrated at the operating table. In the cases of tetralogy of Fallot and tricuspid atresia with pulmonary outflow tract and valve atresia, an anastomosis between the main pulmonary artery and the ascending aorta may be constructed from an anterior approach and probably can be effectively controlled if an open-heart operation is undertaken later for correction of the complex. AD - M. Weinberg Jr., Depts of Surg. and Cardiophysiol, Cook County Hosp., Chicago, IL, United States AU - Weinberg Jr, M. AU - Bicoff, J. P. AU - Bucheleres, H. G. AU - Aguetsson, M. H. AU - Behravesh, M. AU - Andersen, J. H. AU - Fell, E. H. AU - Gasul, B. M. DB - Embase Classic Medline IS - 4 KW - anastomosis anesthesia ascending aorta atresia child exposure Fallot tetralogy great blood vessel heart heart surgery heart ventricle septum defect incision infant pulmonary valve atresia lung blood flow morality obstruction pulmonary artery pulmonary valve stenosis stenosis surgeon survivor tricuspid valve atresia tricuspid valve stenosis valvuloplasty LA - English M3 - Article N1 - L280668284 1962-12-01 PY - 1962 SN - 0022-5223 SP - 433-442 ST - Pulmonary valvulotomy and infundibulotomy in infants T2 - Journal of Thoracic and Cardiovascular Surgery TI - Pulmonary valvulotomy and infundibulotomy in infants UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L280668284 VL - 44 ID - 1418 ER - TY - JOUR AB - It is well known that Down’s syndrome is a strong risk factor for mortality after Fontan operations. We performed two lung biopsies in a Down’s syndrome patient who underwent staged Fontan operations. The pathological findings revealed severe pulmonary arterial hypertrophy and a quantitative real-time polymerase chain reaction revealed the overexpression of endothelin and a decline in the eNOS level at the Fontan operation. Although the preoperative hemodynamic studies revealed that all of the criteria for Fontan had been fulfilled, the patient died of acute cardiac insufficiency, 35 days after the Fontan operation. AD - M. Aoki, Department of Cardiovascular Surgery, University of Toyama, 2630 Sugitani, Toyama, Japan AU - Aoki, M. AU - Hirono, K. AU - Higuma, T. AU - Suzuki, Y. AU - Nakayama, K. AU - Yamashita, A. AU - Fukahara, K. AU - Ichida, F. AU - Emoto, N. AU - Yoshimura, N. DB - Embase Medline DO - 10.1007/s11748-017-0809-6 IS - 5 KW - LightCycler96 system polymerase chain reaction system endothelial nitric oxide synthase endothelin politef RNA acute heart failure aortic clamping article atrioventricular septal defect cardiopulmonary bypass case report cesarean section child clinical article congenital heart malformation disease association Down syndrome echocardiography Fallot tetralogy fetus echography Fontan procedure gene overexpression Glenn shunt heart catheterization heart ventricle septum defect human human tissue hypothermia lung artery pressure lung biopsy lung disease lung vascular resistance male operation duration preoperative evaluation preschool child pulmonary coarctation pulmonary vascular disease real time polymerase chain reaction reverse transcription polymerase chain reaction treatment failure LA - English M3 - Article N1 - L617740634 2017-08-16 2018-12-20 PY - 2018 SN - 1863-6713 1863-6705 SP - 299-302 ST - Pulmonary vascular disease in a failed fontan patient with down’s syndrome T2 - General Thoracic and Cardiovascular Surgery TI - Pulmonary vascular disease in a failed fontan patient with down’s syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L617740634 http://dx.doi.org/10.1007/s11748-017-0809-6 VL - 66 ID - 670 ER - TY - JOUR AB - BACKGROUND: The role, interplay, and relative importance of the multifactorial hemodynamic and myocardial mechanisms causing dysfunction of the Fontan circulation remain incompletely understood. METHODS AND RESULTS: Using an MRI catheterization technique, we performed a differential analysis of pulmonary vascular resistance and aortopulmonary collateral blood flow in conjunction with global ventricular pump function, myocontractility (end-systolic pressure-volume relation), and diastolic compliance (end-diastolic pressure-volume relation) in 10 patients with a Fontan circulation at rest and during dobutamine stress. Pulmonary and ventricular pressures were measured invasively and synchronized with velocity-encoded MRI-derived pulmonary and aortic blood flows and cine MRI-derived ventricular volumes. Pulmonary vascular resistance and end-systolic and end-diastolic pressure-volume relations were then determined. Aortopulmonary collateral flow was calculated as the difference between aortic and pulmonary flow. Compared to rest, dobutamine caused a small increase in mean pulmonary pressures (P<0.05). Collateral flow was significantly augmented (P<0.001) and contributed importantly to an increase in pulmonary flow (P<0.01). Pulmonary vascular resistance decreased significantly (P<0.01). Dobutamine did not increase stroke volumes significantly despite slightly enhanced contractility (end-systolic pressure-volume relation). Active early relaxation (τ) was inconspicuous, but the end-diastolic pressure-volume relation shifted upward, indicating reduced compliance. CONCLUSIONS: In patients with a Fontan circulation, aortopulmonary collateral flow contributes substantially to enhanced pulmonary flow during stress. Our data indicate that pulmonary vascular response to augmented cardiac output was adequate, but decreased diastolic compliance was identified as an important component of ventricular dysfunction. AD - Department of Congenital Heart Disease and Pediatric Cardiology, Deutsches Herzzentrum Berlin and The Charité, Medical University Berlin, Berlin, Germany AN - 20631032 AU - Schmitt, B. AU - Steendijk, P. AU - Ovroutski, S. AU - Lunze, K. AU - Rahmanzadeh, P. AU - Maarouf, N. AU - Ewert, P. AU - Berger, F. AU - Kuehne, T. DA - Sep DB - PubMed DO - 10.1161/circimaging.109.931592 DP - NLM ET - 2010/07/16 IS - 5 KW - Adolescent Adult *Collateral Circulation Compliance *Dobutamine Echocardiography, Doppler Exercise Tolerance Fontan Procedure/*adverse effects Humans *Magnetic Resonance Imaging, Cine Myocardial Contraction Predictive Value of Tests *Pulmonary Circulation Treatment Outcome *Vascular Resistance Ventricular Dysfunction, Left/diagnosis/*etiology/physiopathology *Ventricular Function Ventricular Pressure Young Adult LA - eng N1 - 1942-0080 Schmitt, Boris Steendijk, Paul Ovroutski, Stanislav Lunze, Karsten Rahmanzadeh, Pedram Maarouf, Nizar Ewert, Peter Berger, Felix Kuehne, Titus Journal Article Research Support, Non-U.S. Gov't United States Circ Cardiovasc Imaging. 2010 Sep;3(5):623-31. doi: 10.1161/CIRCIMAGING.109.931592. Epub 2010 Jul 14. PY - 2010 SN - 1941-9651 SP - 623-31 ST - Pulmonary vascular resistance, collateral flow, and ventricular function in patients with a Fontan circulation at rest and during dobutamine stress T2 - Circ Cardiovasc Imaging TI - Pulmonary vascular resistance, collateral flow, and ventricular function in patients with a Fontan circulation at rest and during dobutamine stress VL - 3 ID - 375 ER - TY - JOUR AB - Pulmonary vascular disease develops early in untreated single ventricle patients with increased pulmonary flow. Pulmonary artery (PA) banding is done at a young age in these patients in order to protect the lung vasculature and maintain low pulmonary artery pressures (PAP) and pulmonary vascular resistance (PVR). This also enables future completion of the single ventricle palliation. Pulmonary venous hypertension (PVH) secondary to left sided obstruction if present in addition in this setting contributes to the pulmonary arterial hypertension (PAH) but involves an element of reversibility of the PAH if the obstruction is relieved. We present two cases of single ventricle both of who re-presented late with PAH and PVH (secondary to mitral valve obstruction) and underwent delayed PA banding at 9.5 and 4.5 years of age respectively. Both patients however had different outcomes. The patient undergoing PA banding at 9.5 years successfully underwent a cavo-pulmonary shunt at the age of 12 years. The patient with PA banding at 4.5 years however, has residual PAH that presently precludes a cavo-pulmonary shunt. AD - Department of Pediatric Cardiology, Amrita Institute of Medical Sciences and Research Center, Kochi, Kerala, 682 041, India AU - Kalantre, A. AU - Sunil, G. S. AU - Kumar, R. K. DB - Scopus DO - 10.4103/0974-2069.177517 IS - 2 KW - Delayed pulmonary artery banding hemodynamics pulmonary venous hypertension (PVH) single ventricle M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2016 SP - 147-152 ST - Pulmonary venous hypertension may allow delayed palliation of single ventricle physiology with pulmonary hypertension T2 - Annals of Pediatric Cardiology TI - Pulmonary venous hypertension may allow delayed palliation of single ventricle physiology with pulmonary hypertension UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84968877322&doi=10.4103%2f0974-2069.177517&partnerID=40&md5=4fb0c8c4a93ce1ed02fcfa4a9a3a616a VL - 9 ID - 1909 ER - TY - JOUR AB - The characteristic depressed hemodynamic state and gradually declining circulatory function in Fontan patients necessitates alternative postoperative management strategies incorporating a system level approach. In this study, the single-ventricle Fontan circulation is modeled by constructing a practical in vitro bench-top pulsatile pediatric flow loop which demonstrates the ability to simulate a wide range of clinical scenarios. The aim of this study is to illustrate the utility of a novel single-ventricle flow loop to study mechanical cardiac assist to Fontan circulation to aid postoperative management and clinical decision-making of single ventricle patients. Two different pediatric ventricular assist devices, Medos and Pediaflow Gen-0, are anastomosed in two nontraditional configurations: systemic venous booster (SVB) and pulmonary arterial booster (PAB). Optimum ventricle assist device strategy is analyzed under normal and pathological (pulmonary hypertension) conditions. Our findings indicate that the Medos ventricular assist device in SVB configuration provided the highest increase in pulmonary (46%) and systemic (90%) venous flow under normal conditions, whereas for the hypertensive condition, highest pulmonary (28%) and systemic (55%) venous flow augmentation were observed for the Pediaflow ventricular assist device inserted as a PAB. We conclude that mechanical cardiac assist in the Fontan circulation effectively results in flow augmentation and introduces various control modalities that can facilitate patient management. Assisted circulation therapies targeting single-ventricle circuits should consider disease state specific physiology and hemodynamics on the optimal configuration decisions. AD - Biomedical Engineering Department, Carnegie Mellon University, Pittsburgh, PA 15219, USA. AN - 20021470 AU - Dur, O. AU - Lara, M. AU - Arnold, D. AU - Vandenberghe, S. AU - Keller, B. B. AU - DeGroff, C. AU - Pekkan, K. DA - Nov DB - PubMed DO - 10.1111/j.1525-1594.2009.00951.x DP - NLM ET - 2009/12/22 IS - 11 KW - Equipment Design Fontan Procedure/*instrumentation/methods *Heart-Assist Devices Hemodynamics Pediatrics/*instrumentation Pulsatile Flow LA - eng N1 - 1525-1594 Dur, Onur Lara, Mikhail Arnold, Dorian Vandenberghe, Stijn Keller, Bradley B DeGroff, Curt Pekkan, Kerem Evaluation Study Journal Article United States Artif Organs. 2009 Nov;33(11):967-76. doi: 10.1111/j.1525-1594.2009.00951.x. PY - 2009 SN - 0160-564x SP - 967-76 ST - Pulsatile in vitro simulation of the pediatric univentricular circulation for evaluation of cardiopulmonary assist scenarios T2 - Artif Organs TI - Pulsatile in vitro simulation of the pediatric univentricular circulation for evaluation of cardiopulmonary assist scenarios VL - 33 ID - 302 ER - TY - JOUR AB - Background Shunt occlusion is a major cause of death in children with single ventricle. We evaluated whether one daily measurement of oxygen saturation at home could detect life-threatening shunt dysfunction. Methods A total of 28 infants were included. Parents were instructed to measure saturation once daily and if less than or equal to 70% repeat the measurement. Home monitoring was defined as positive when a patient was admitted to Queen Silvia Children's Hospital because of saturation less than or equal to 70% on repeated measurement at home. A shunt complication was defined as arterial desaturation and a narrowing of the shunt that resulted in an intervention to relieve the obstruction or in death. Parents' attitude towards the method was investigated using a questionnaire. Results A shunt complication occurred out of hospital eight times in eight patients. Home monitoring was positive in five out of eight patients. In two patients, home monitoring was probably life saving; in one of them, the shunt was replaced the same day and the other had an emergency balloon dilatation of the shunt. In three out of eight patients, home monitoring was negative; one had an earlier stage II and survived, but two died suddenly at home from thrombotic shunt occlusion. On seven occasions in three patients home monitoring was positive but there was no shunt complication. The method was well accepted by the parents according to the results of the questionnaire. Conclusion Home monitoring of oxygen saturation has the potential to detect some of the life-threatening shunt obstructions between stages I and II in infants with single-ventricle physiology. © Cambridge University Press 2012. AD - M. Mellander, Department of Paediatric Cardiac Surgery, Queen Silvia Children's Hospital, Sahlgrenska University Hospital, 41685 Gothenburg, Sweden AU - Öhman, A. AU - Strömvall-Larsson, E. AU - Nilsson, B. AU - Mellander, M. DB - Embase Medline DO - 10.1017/S1047951112000352 IS - 1 KW - acetylsalicylic acid low molecular weight heparin sotalol warfarin article balloon dilatation bradycardia cardiopulmonary bypass cause of death child clinical article emergency surgery heart arrhythmia home monitoring hospitalization human infant lung blood flow oxygen desaturation oxygen saturation patient attitude pericardial effusion postoperative complication postoperative thrombosis pulse oximetry quality of life questionnaire sensitivity and specificity surgical mortality survival rate thrombotic shunt occlusion LA - English M3 - Article N1 - L368274154 2013-02-13 2013-02-22 PY - 2013 SN - 1047-9511 1467-1107 SP - 75-81 ST - Pulse oximetry home monitoring in infants with single-ventricle physiology and a surgical shunt as the only source of pulmonary blood flow T2 - Cardiology in the Young TI - Pulse oximetry home monitoring in infants with single-ventricle physiology and a surgical shunt as the only source of pulmonary blood flow UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L368274154 http://dx.doi.org/10.1017/S1047951112000352 VL - 23 ID - 1019 ER - TY - JOUR AB - PURPOSE: No valid, reliable, and publicly available patient-reported outcome measure (PROM) exists to assess the pediatric heart failure (HF) experience. We sought to understand this experience in order to develop a PROM that can reliably capture and track outcomes for adolescent patients. METHODS: Semi-structured individual or group interviews were conducted at two pediatric HF and transplant centers in the US. Patient ages 12-21 who had a record of symptomatic HF in the past 2 years were eligible to participate. Caregivers of participants were also eligible. Symptom experiences and their impact on daily activities were explored. Transcripts were analyzed by members of a multidisciplinary team using thematic analysis. RESULTS: 16 patients and 9 caregivers participated. 22 completed individual interviews in-person or by phone, and 3 participated in a web-based focus group. Patient participants were 31% male, and median age was 16.5 years. 31% (n=5) had HF from cardiomyopathy and 69% (n=11) had HF due to congenital heart disease, with 7 having single ventricle physiology. 5 participants were interviewed post-transplant. Preliminary analysis revealed consistent themes across interviews. Common symptoms included fatigue, shortness of breath, and chest discomfort. Symptoms limited ability to perform daily tasks (e.g. climbing stairs at school), participate in certain extracurricular activities (e.g. sports, dance) and keep up with peers. Participants described social and emotional impacts from being treated differently by others due to surgical scars, medical devices, or functional limitations. They also reported burden of frequent medical visits and treatments, and expressed worry and uncertainty about the need for future medical interventions. Caregivers reported similar impacts on patient function and wellbeing, and they further described social exclusion by peers and anxiety about the future (e.g. limited life expectancy or career limitations). CONCLUSION: The unique personal and social impacts of HF symptoms on adolescents and young adults may necessitate development of unique items for PRO measurement in this population. These findings will be used to draft a developmentally-relevant PROM that will subsequently be tested in this population and validated for use in clinical care and regulatory evaluation of new medical products. AU - Chen, C. AU - Ridgeway, J. L. AU - Bocell, F. D. AU - Tanenbaum, M. L. AU - Hood, K. K. AU - Behnken, E. AU - Schmidt, J. AU - Hanes, S. J. AU - Saha, A. AU - Caldwell, B. AU - Tarver, M. AU - Peiris, V. AU - Almond, C. S. AU - Johnson, J. N. DB - Medline DO - 10.1016/j.healun.2020.01.280 IS - 4 KW - adolescent adult anxiety article cardiomyopathy career caregiver case report child clinical article clinical evaluation congenital heart disease daily life activity dancing dyspnea fatigue female genetic transcription heart single ventricle human interview life expectancy male medical device multidisciplinary team outcome assessment patient-reported outcome scar social exclusion sport stair climbing surgery thematic analysis thorax pain uncertainty wellbeing young adult LA - English M3 - Article N1 - L631930577 2020-06-05 PY - 2020 SN - 1557-3117 SP - S452 ST - Qualitative Exploration of the Pediatric Heart Failure Experience for Development of a Patient-Reported Outcome Measure T2 - The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation TI - Qualitative Exploration of the Pediatric Heart Failure Experience for Development of a Patient-Reported Outcome Measure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L631930577 http://dx.doi.org/10.1016/j.healun.2020.01.280 VL - 39 ID - 559 ER - TY - JOUR AB - Pediatric ventricular assist device (VAD) implantation outcomes are increasingly promising for children with dilated cardiomyopathy and advanced decompensated heart failure (ADHF). VAD placement in patients with clinical features such as complex congenital cardiac anatomy, small body size, or major comorbidities remains problematic. These comorbidities have been traditionally prohibitive for VAD consideration leaving these children as a treatment-orphaned population. Here we describe the quality bundle surrounding these patients with ADHF considered high risk for VAD implantation at our institution. Over a 7-year period, a quality bundle aimed at the peri-operative care for children with high-risk features undergoing VAD implantation was incrementally implemented at a tertiary children’s hospital. Patients were considered high risk if they were neonates (< 30 days), had single-ventricle physiology, non-dilated cardiomyopathy, biventricular dysfunction, or significant comorbidities. The quality improvement bundle evolved to include (1) structured team-based peri-operative evaluation, (2) weekly VAD rounds addressing post-operative device performance, (3) standardized anticoagulation strategies, and (4) a multidisciplinary system for management challenges. These measures aimed to improve communication, standardize management, allow for ongoing process improvement, and incorporate principles of a high-reliability organization. Between January 2010 and December 2017, 98 patients underwent VAD implantation, 48 (49%) of which had high-risk comorbidities and a resultant cohort survival-to-transplant rate of 65%. We report on the evolution of a quality improvement program to expand the scope of VAD implantation to patients with high-risk clinical profiles. This quality bundle can serve as a template for future large-scale collaborations to improve outcomes in these treatment-orphaned subgroups. AD - C. Knoll, Division of Pediatric Cardiology, Lucile Packard Children’s Hospital Stanford, Palo Alto, CA, United States AU - Knoll, C. AU - Chen, S. AU - Murray, J. M. AU - Dykes, J. C. AU - Yarlagadda, V. V. AU - Rosenthal, D. N. AU - Almond, C. S. AU - Maeda, K. AU - Shin, A. Y. DB - Embase Medline DO - 10.1007/s00246-019-02123-1 IS - 6 KW - ventricular assist device anticoagulant agent adolescent adult advanced decompensated heart failure anticoagulant therapy article child cohort analysis comorbidity congestive cardiomyopathy controlled study female health care quality heart failure heart single ventricle heart ventricle failure high risk patient human implantation infant interpersonal communication major clinical study male newborn observational study patient assessment pediatric hospital perioperative period preschool child reliability retrospective study risk assessment school child standardization survival time tertiary care center LA - English M3 - Article N1 - L627749746 2019-05-23 2019-11-05 PY - 2019 SN - 1432-1971 0172-0643 SP - 1159-1164 ST - A Quality Bundle to Support High-Risk Pediatric Ventricular Assist Device Implantation T2 - Pediatric Cardiology TI - A Quality Bundle to Support High-Risk Pediatric Ventricular Assist Device Implantation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L627749746 http://dx.doi.org/10.1007/s00246-019-02123-1 VL - 40 ID - 602 ER - TY - JOUR AB - PURPOSE OF REVIEW: The National Pediatric Quality Improvement Collaborative (NPCQIC) was established to improve outcomes and quality of life in children with hypoplastic left heart syndrome and other single ventricle lesions requiring a Norwood operation. The NPCQIC consists of a network of providers and families collecting longitudinal data, conducting research, and using quality improvement science to decrease variations in care, develop and spread best practices, and decrease mortality. RECENT FINDINGS: Initial descriptive investigation of the collaborative data found interstage care process variations, different surgical strategies, diverse feeding practices, and variable ICU approaches between centers and within sites. Analysis and evaluation of these practice variations have allowed centers to learn from each other and implement change to improve processes. There has been an improvement in performance measures and most importantly, a 39.7% reduction in mortality. SUMMARY: The NPCQIC has shown, in a rare disease such as hypoplastic left heart syndrome that a network based on multicenter collaboration, patient (parent) engagement, and quality improvement science can facilitate change in practices and improvement in outcomes. AD - aChildren's National Medical Center, Washington DC bCincinnati Children's Hospital, Cincinnati, Ohio cSister's By Heart dChildren's Hospital & Medical Center, Omaha, Nebraska, USA. AN - 26208236 AU - Clauss, S. B. AU - Anderson, J. B. AU - Lannon, C. AU - Lihn, S. AU - Beekman, R. H. AU - Kugler, J. D. AU - Martin, G. R. DA - Oct DB - PubMed DO - 10.1097/mop.0000000000000263 DP - NLM ET - 2015/07/25 IS - 5 KW - Advisory Committees Child Child, Preschool Cooperative Behavior Humans Hypoplastic Left Heart Syndrome/mortality/*surgery Infant *Norwood Procedures/standards Outcome and Process Assessment, Health Care *Pediatrics/standards Practice Guidelines as Topic Program Development Program Evaluation Quality Indicators, Health Care Quality of Life Treatment Outcome LA - eng N1 - 1531-698x Clauss, Sarah B Anderson, Jeffrey B Lannon, Carole Lihn, Stacey Beekman, Robert H Kugler, John D Martin, Gerard R Journal Article Review United States Curr Opin Pediatr. 2015 Oct;27(5):555-62. doi: 10.1097/MOP.0000000000000263. PY - 2015 SN - 1040-8703 SP - 555-62 ST - Quality improvement through collaboration: the National Pediatric Quality improvement Collaborative initiative T2 - Curr Opin Pediatr TI - Quality improvement through collaboration: the National Pediatric Quality improvement Collaborative initiative VL - 27 ID - 140 ER - TY - JOUR AB - OBJECTIVE: To determine the health-related quality of life at 4 years of age in children who had undergone cardiac surgery for congenital heart disease in early infancy. METHODS: A prospective cohort study of infants undergoing cardiac surgery at 6 weeks of age or younger from July 2000 to June 2005 at the Stollery Children's Hospital. The quality of life was assessed using the Pediatric Quality of Life Inventory, version 4.0, generic core scales, and compared with normative values for the same age. The association between the perioperative variables and health-related quality of life was explored. RESULTS: A total of 242 infants underwent complex heart surgery during the study period. Of the 166 eligible survivors, 130 were included. No significant differences were present between the children with single ventricle versus biventricular repairs, except for lower physical health summary scores in the single ventricle patients (P = .007). Compared with the normative data, the children with biventricular repair had lower total Pediatric Quality of Life Inventory, version 4.0, scores (P = .001) and psychosocial health summary scores (P < .001). The children with single ventricle repair also had lower physical health summary scores (P = .003). Older age at surgery and markers of postoperative low cardiac output syndrome were associated with worse health-related quality of life, and greater socioeconomic status was associated with better quality of life. CONCLUSIONS: At 4 years of age, health-related quality of life was significantly lower in children who had undergone surgery for congenital heart disease in early infancy. An association was found between age at surgery and postoperative low cardiac output and socioeconomic status and quality of life. AD - Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada. Gonzalo.Guerra@albertahealthservices.ca AN - 22507845 AU - Garcia Guerra, G. AU - Robertson, C. M. AU - Alton, G. Y. AU - Joffe, A. R. AU - Dinu, I. A. AU - Nicholas, D. AU - Ross, D. B. AU - Rebeyka, I. M. DA - Feb DB - PubMed DO - 10.1016/j.jtcvs.2012.03.050 DP - NLM ET - 2012/04/18 IS - 2 KW - Age Factors Canada Cardiac Output, Low/etiology/psychology *Cardiac Surgical Procedures/adverse effects/psychology Child, Preschool Health Status Heart Defects, Congenital/psychology/*surgery Humans Infant Infant, Newborn Linear Models Mental Health Multivariate Analysis Prospective Studies *Quality of Life Risk Assessment Risk Factors Socioeconomic Factors Surveys and Questionnaires Time Factors Treatment Outcome LA - eng N1 - 1097-685x Garcia Guerra, Gonzalo Robertson, Charlene M T Alton, Gwen Y Joffe, Ari R Dinu, Irina A Nicholas, David Ross, David B Rebeyka, Ivan M Western Canadian Complex Pediatric Therapies Follow-up Group Journal Article Multicenter Study Research Support, Non-U.S. Gov't United States J Thorac Cardiovasc Surg. 2013 Feb;145(2):482-488.e2. doi: 10.1016/j.jtcvs.2012.03.050. Epub 2012 Apr 14. PY - 2013 SN - 0022-5223 SP - 482-488.e2 ST - Quality of life 4 years after complex heart surgery in infancy T2 - J Thorac Cardiovasc Surg TI - Quality of life 4 years after complex heart surgery in infancy VL - 145 ID - 75 ER - TY - JOUR AB - Objective: To determine the health-related quality of life at 4 years of age in children who had undergone cardiac surgery for congenital heart disease in early infancy. Methods: A prospective cohort study of infants undergoing cardiac surgery at 6 weeks of age or younger from July 2000 to June 2005 at the Stollery Children's Hospital. The quality of life was assessed using the Pediatric Quality of Life Inventory, version 4.0, generic core scales, and compared with normative values for the same age. The association between the perioperative variables and health-related quality of life was explored. Results: A total of 242 infants underwent complex heart surgery during the study period. Of the 166 eligible survivors, 130 were included. No significant differences were present between the children with single ventricle versus biventricular repairs, except for lower physical health summary scores in the single ventricle patients (P = .007). Compared with the normative data, the children with biventricular repair had lower total Pediatric Quality of Life Inventory, version 4.0, scores (P = .001) and psychosocial health summary scores (P < .001). The children with single ventricle repair also had lower physical health summary scores (P = .003). Older age at surgery and markers of postoperative low cardiac output syndrome were associated with worse health-related quality of life, and greater socioeconomic status was associated with better quality of life. Conclusions: At 4 years of age, health-related quality of life was significantly lower in children who had undergone surgery for congenital heart disease in early infancy. An association was found between age at surgery and postoperative low cardiac output and socioeconomic status and quality of life. Copyright © 2013 by The American Association for Thoracic Surgery. AD - G. Garcia Guerra, Department of Pediatrics, University of Alberta, 3A3.07 Stollery Children's Hospital, 8440-112 St, Edmonton, AB T6G 2B7, Canada AU - Garcia Guerra, G. AU - Robertson, C. M. T. AU - Alton, G. Y. AU - Joffe, A. R. AU - Dinu, I. A. AU - Nicholas, D. AU - Ross, D. B. AU - Rebeyka, I. M. DB - Embase Medline DO - 10.1016/j.jtcvs.2012.03.050 IS - 2 KW - article biventricular repair cohort analysis disease association female follow up forward heart failure heart output heart surgery human infancy infant major clinical study male mental health Pediatric Quality of Life Inventory version 4.0 perioperative period postoperative period priority journal prospective study quality of life rating scale single ventricle repair Student t test LA - English M3 - Article N1 - L51960426 2012-04-17 2013-01-29 PY - 2013 SN - 0022-5223 1097-685X SP - 482-488.e2 ST - Quality of life 4 years after complex heart surgery in infancy T2 - Journal of Thoracic and Cardiovascular Surgery TI - Quality of life 4 years after complex heart surgery in infancy UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51960426 http://dx.doi.org/10.1016/j.jtcvs.2012.03.050 VL - 145 ID - 1021 ER - TY - JOUR AB - BACKGROUND: After the Fontan procedure patients are at risk for reduced quality of life (QoL) and cognitive function. We aimed to assess these important factors in Danish Fontan patients and to compare the results with a group of healthy controls. METHODS: All Fontan patients living in Denmark were identified and invited to participate. QoL was evaluated using the Pediatric Quality of Life Inventory (PedsQL) version 4.0 generic core module in patients <16 years and the Short Form 36 questionnaire (SF-36) in patients ≥16 years. Cognitive function was evaluated in all patients ≥6 years using the Quick Test of Cognitive Speed. To evaluate if QoL correlated with exercise capacity, patients performed a symptom-limited bicycle test. RESULTS: 158 of 179 eligible patients (88%) consented to participate. Median age was 13.9 years (IQR: 10.2-19.3). PedsQL scores increased with age but were significantly lower among patients than among controls. SF-36 physical scores were significantly lower in patients compared to controls while psychosocial scores were similar. Cognitive speed was significantly reduced in patients at all ages compared to controls. No significant difference in PedsQL-/SF-36 scores or cognitive speed was found between hypoplastic left heart syndrome (HLHS) and non-HLHS Fontan patient. PedsQL-/SF-36 scores in patients ≥10 years correlated significantly to cognitive speed but not to peak exercise capacity. CONCLUSION: QoL is reduced in Fontan children compared to their healthy counterparts whereas in patients ≥16 years only physical, but not psychosocial QoL is reduced. Cognitive speed was significantly lower in patients at all ages compared to controls. AD - Department of Cardiology, Rigshospitalet, Copenhagen, Denmark. Electronic address: lars.idorn@rh.regionh.dk. AN - 23632112 AU - Idorn, L. AU - Jensen, A. S. AU - Juul, K. AU - Overgaard, D. AU - Nielsen, N. P. AU - Sørensen, K. AU - Reimers, J. I. AU - Søndergaard, L. DA - Oct 9 DB - PubMed DO - 10.1016/j.ijcard.2013.04.008 DP - NLM ET - 2013/05/02 IS - 4 KW - Adolescent Child Child, Preschool Cognition Disorders/diagnosis/*epidemiology/*psychology Cross-Sectional Studies Denmark/epidemiology Female Follow-Up Studies Fontan Procedure/*psychology Humans Male Population Surveillance/*methods Quality of Life/*psychology Cognitive function Congenital heart disease Fontan patients Quality of life LA - eng N1 - 1874-1754 Idorn, Lars Jensen, Annette S Juul, Klaus Overgaard, Dorthe Nielsen, Niels P Sørensen, Keld Reimers, Jesper I Søndergaard, Lars Journal Article Research Support, Non-U.S. Gov't Netherlands Int J Cardiol. 2013 Oct 9;168(4):3230-5. doi: 10.1016/j.ijcard.2013.04.008. Epub 2013 Apr 28. PY - 2013 SN - 0167-5273 SP - 3230-5 ST - Quality of life and cognitive function in Fontan patients, a population-based study T2 - Int J Cardiol TI - Quality of life and cognitive function in Fontan patients, a population-based study VL - 168 ID - 112 ER - TY - JOUR AB - OBJECTIVE: To evaluate the quality of life in patients with univentricular heart and to determine the impact of sociodemographic and clinical characteristics. DESIGN AND SETTING: Retrospective, cross sectional study conducted in a regional paediatric cardiology centre. PATIENTS: The health records of 89 survivors with univentricular heart (median age 21 years; range 17-49 years) were reviewed. Sixty seven answered the Duke questionnaire. Sociodemographic and clinical variables were similar in the responders and non-responders. The impact of sociodemographic and clinical variables on individual Duke's measures was assessed. RESULTS: The Duke scores of adults with univentricular heart were similar to the normal population. Cyanosis predicted a worse score for physical (p = 0.05) and perceived health measures (p = 0.02). A higher educational level predicted a better score for physical (p = 0.004), mental (p = 0.01), and general health measures (p = 0.02). Orthopaedic problems worsened the social score (p = 0.05). Psychosocial problems worsened the pain score (p = 0.04). In comparison with the other anatomical types, mitral atresia worsened the perceived health score (p = 0.02). Patients younger than 23 years scored better for almost all health and dysfunction measures. CONCLUSIONS: Despite repeated interventions and other disease related everyday stresses, a selected group of adults with univentricular heart had a satisfying quality of life. AD - Service de Cardiologie Pédiatrique and Institut de Cardiologie Congénitale des Adultes, Hôpital Necker-Enfants Malades, 149 rue de Sèvres, 75743 Paris Cedex 15, France. AN - 11410565 AU - Saliba, Z. AU - Butera, G. AU - Bonnet, D. AU - Bonhoeffer, P. AU - Villain, E. AU - Kachaner, J. AU - Sidi, D. AU - Iserin, L. C2 - PMC1729833 DA - Jul DB - PubMed DO - 10.1136/heart.86.1.69 DP - NLM ET - 2001/06/19 IS - 1 KW - Adolescent Adult Cross-Sectional Studies Female *Health Status Heart Defects, Congenital/*psychology Heart Ventricles/abnormalities Humans Male Middle Aged *Quality of Life Retrospective Studies Surveys and Questionnaires LA - eng N1 - 1468-201x Saliba, Z Butera, G Bonnet, D Bonhoeffer, P Villain, E Kachaner, J Sidi, D Iserin, L Journal Article Research Support, Non-U.S. Gov't Heart. 2001 Jul;86(1):69-73. doi: 10.1136/heart.86.1.69. PY - 2001 SN - 1355-6037 (Print) 1355-6037 SP - 69-73 ST - Quality of life and perceived health status in surviving adults with univentricular heart T2 - Heart TI - Quality of life and perceived health status in surviving adults with univentricular heart VL - 86 ID - 208 ER - TY - JOUR AB - OBJECTIVE: To get an impression of the quality of life (QOL) and sexual well-being in the Fontan population, and to generate hypotheses for future research. METHODS: For this cross-sectional pilot study, questionnaires regarding health-related QOL, sexual function and fertility/pregnancy were completed by 21 patients with a Fontan circulation >16 years old, followed at the University Medical Center Groningen, the Netherlands. Semi-structured qualitative interviews were conducted in 8 patients. RESULTS: Fontan patients scored significantly lower on general health than their healthy peers (t(19)=-3.0, P = .008), whereas their scores on other QOL domains and sexual well-being were comparable to normal values. During childhood, most patients experienced physical limitations and the feeling of being an outsider, and frequently faced bullying. Regarding sexual well-being, large interindividual differences were noted. Four interviewed patients (25-30 years) reported a good sexual well-being, whereas the other interviewed patients (33-47 years) reported erectile dysfunction, low self-esteem and avoidance of sexual intercourse. Both the QOL domains mental health and role restrictions due to emotional problems were associated with female avoidance (P = .083, respectively, P = .089) and dyspareunia (P = ns respectively P = .094). In males, role restrictions due to physical problems and health change were related to sexual dissatisfaction (P = .056) respectively nonsensuality (P = .025). CONCLUSIONS: Overall, Fontan patients have a relatively preserved quality of life and sexual wellbeing but face more social isolation and bullying during childhood/adolescence than their healthy peers. Sexual problems were mainly associated with physical limitations in males and with psychosocial limitations in females. Finally, sexual dysfunction was more common in older Fontan patients, and future research has to clarify whether progressive attrition of the Fontan circulation affects the patients' QOL and sexual well-being. AD - Department of Pediatric Cardiology, Center for Congenital Heart Diseases, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. Wenckebach Institute, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. Center for Congenital Heart Diseases, Department of Cardiology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. Center for Congenital Heart Diseases, Department of Cardiothoracic Surgery, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. Department of Obstetrics and Gynecology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. AN - 29532606 AU - Wolff, D. AU - van de Wiel, H. B. M. AU - de Muinck Keizer, M. E. AU - van Melle, J. P. AU - Pieper, P. G. AU - Berger, R. M. F. AU - Ebels, T. AU - Weijmar Schultz, W. C. M. DA - Mar DB - PubMed DO - 10.1111/chd.12576 DP - NLM ET - 2018/03/14 IS - 2 KW - Adolescent Adult Child Cross-Sectional Studies Female *Fontan Procedure *Health Status Heart Defects, Congenital/physiopathology/*psychology/surgery Humans Male Middle Aged Pilot Projects Postoperative Period *Quality of Life Sexual Behavior/*psychology Surveys and Questionnaires Young Adult Fontan physiology congenital heart disease quality of life sexual functioning special populations LA - eng N1 - 1747-0803 Wolff, Djoeke Orcid: 0000-0002-4572-7268 van de Wiel, Henricus B M de Muinck Keizer, Mirthe E van Melle, Joost P Pieper, Petronella G Berger, Rolf M F Ebels, Tjark Weijmar Schultz, Willebrord C M Journal Article United States Congenit Heart Dis. 2018 Mar;13(2):319-326. doi: 10.1111/chd.12576. Epub 2018 Mar 12. PY - 2018 SN - 1747-079x SP - 319-326 ST - Quality of life and sexual well-being in patients with a Fontan circulation: An explorative pilot study with a mixed method design T2 - Congenit Heart Dis TI - Quality of life and sexual well-being in patients with a Fontan circulation: An explorative pilot study with a mixed method design VL - 13 ID - 51 ER - TY - JOUR AB - AIM: To measure quality of life in children with hypoplastic left heart syndrome and their families. METHODS: A questionnaire exploring socioeconomic status, structure and function of networks and psychological well-being was completed by the families of all 18 patients older than 2 years (age range 2.7-10.6). The results were compared with those of 180 healthy Swedish children matched for age and sex. RESULTS: There were no significant differences between the groups in any of the aspects of socioeconomic status. Study group parents had not more available time for their child (p < 0.05) and more separations/divorces (p < 0.01). The patients had lower self-esteem (p < 0.05), more psychosomatic symptoms (p < 0.01) [corrected] and lower peer acceptance (p < 0.01) than control children. CONCLUSION: With regard to psychological well-being, quality of life was significantly lower in children with hypoplastic left heart syndrome than in healthy controls. AD - Department of Pediatric Cardiology, The Queen Silvia Children's Hospital, Göteborg, University, Göteborg, Sweden. mats.mellander@vgregion.se AN - 17187604 AU - Mellander, M. AU - Berntsson, L. AU - Nilsson, B. DA - Jan DB - PubMed DO - 10.1111/j.1651-2227.2007.00039.x DP - NLM ET - 2006/12/26 IS - 1 KW - Cardiac Surgical Procedures/adverse effects Child Child, Preschool Developmental Disabilities Female Humans Hypoplastic Left Heart Syndrome/*psychology/surgery Male Postoperative Complications *Quality of Life Socioeconomic Factors Surveys and Questionnaires Sweden LA - eng N1 - Mellander, M Berntsson, L Nilsson, B Journal Article Research Support, Non-U.S. Gov't Norway Acta Paediatr. 2007 Jan;96(1):53-7. doi: 10.1111/j.1651-2227.2007.00039.x. PY - 2007 SN - 0803-5253 (Print) 0803-5253 SP - 53-7 ST - Quality of life in children with hypoplastic left heart syndrome T2 - Acta Paediatr TI - Quality of life in children with hypoplastic left heart syndrome VL - 96 ID - 182 ER - TY - JOUR AB - Reports an error in 'Quality of life in children with hypoplastic left heart syndrome' by M. Mellander, L. Berntsson and B. Nilsson (Acta Paediatrica, 2007[Jan], Vol 96[1], 53-57). In the original article, the following error was published. In the abstract, the word 'not' should have been deleted from the sentence: Study group parents had not more available time for their child (p < 0.05) and more separations/divorces (p < 0.01). The correct abstract is published in the erratum. (The following abstract of the original article appeared in record [rid]2008-07779-002[/rid]). Aim: To measure quality of life in children with hypoplastic left heart syndrome and their families. Methods: A questionnaire exploring socioeconomic status, structure and function of networks and psychological well-being was completed by the families of all 18 patients older than 2 years (age range 2.7-10.6). The results were compared with those of 180 healthy Swedish children matched for age and sex. Results: There were no significant differences between the groups in any of the aspects of socioeconomic status. Study group parents had not more available time for their child (p < 0.05) and more separations/divorces (p < 0.01). The patients had lower self-esteem (p < 0.05), more psychosomatic symptoms (p < 0.001) and lower peer acceptance (p < 0.01) than control children. Conclusion: With regard to psychological well-being, quality of life was significantly lower in children with hypoplastic left heart syndrome than in healthy controls. (PsycINFO Database Record (c) 2016 APA, all rights reserved) AN - 2008-07782-014 AU - Mellander, M. AU - Berntsson, L. AU - Nilsson, B. DB - psyh DP - EBSCOhost IS - 2 KW - quality of life hypoplastic left heart syndrome children Heart Disorders Pediatrics Syndromes N1 - Acta Paediatrica Scandinavica (Sweden). Partial author list: First Author & Affiliation: Mellander, M.; Department of Paediatric Cardiology, Queen Silva Children's Hospital, Goteborg University, Goteborg, Sweden. Other Publishers: Almqvist & Wiksell Periodical Co.; Scandinavian University Press; Taylor & Francis. Release Date: 20090518. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Erratum/Correction. Language: English. Major Descriptor: Heart Disorders; Pediatrics; Quality of Life; Syndromes. Classification: Cardiovascular Disorders (3295). Population: Human (10). References Available: Y. Page Count: 1. Issue Publication Date: Feb, 2007. PY - 2007 SN - 0803-5253 1651-2227 SP - 324-324 ST - 'Quality of life in children with hypoplastic left heart syndrome': Erratum T2 - Acta Paediatrica TI - 'Quality of life in children with hypoplastic left heart syndrome': Erratum UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2008-07782-014&site=ehost-live&scope=site VL - 96 ID - 1694 ER - TY - JOUR AB - OBJECTIVE: To measure quality of life in children with pulmonary atresia and intact ventricular septum, and make comparisons with a healthy group of children from the general Swedish population. An additional aim was to compare quality of life in two subgroups, children undergoing biventricular and univentricular repair, the latter by establishment of the Fontan circulation. METHODS: Quality of life was measured using a mailed questionnaire, taking into consideration the three spheres of personal, interpersonal, and external living conditions. In all, 52 children received the questionnaire, and the answers were compared with those of a random sample of 1856 healthy Swedish children. RESULTS: We received responses from 42 children and parents. The overall differences between groups were small, and no difference was found when comparing the children undergoing univentricular as opposed to biventricular surgical repair. In terms of personal quality of life, we found a higher level of psychosomatic complaints, and lower satisfaction with their own and parent-child activities, in those with the congenital anomaly than in their normal peers. In terms of the interpersonal sphere, more time was available for those with malformed hearts, but the experience of support from the relatives was significantly lower. CONCLUSIONS: Overall, quality of life was equal between children with pulmonary atresia and intact ventricular septum and the children in the reference group. The personal psychological conditions were less favourable for children with pulmonary atresia and intact ventricular septum. The perceived lack of support from relatives was also higher in these families. No difference was found when comparing the children undergoing univentricular and biventricular surgical repair. AD - Pediatric and Adolescent Medical Care, Skaraborg Hospital, Skövde, Sweden. britt-mari.ekman-joelsson@vgregion.se AN - 15679997 AU - Ekman-Joelsson, B. M. AU - Berntsson, L. AU - Sunnegårdh, J. DA - Dec DB - PubMed DO - 10.1017/s1047951104006067 DP - NLM ET - 2005/02/01 IS - 6 KW - Adolescent Adult Child Child, Preschool Heart Septal Defects, Ventricular/psychology/surgery Humans Infant Pulmonary Atresia/*psychology/surgery *Quality of Life Surveys and Questionnaires LA - eng N1 - Ekman-Joelsson, Britt-Marie Berntsson, Leeni Sunnegårdh, Jan Comparative Study Journal Article Research Support, Non-U.S. Gov't England Cardiol Young. 2004 Dec;14(6):615-21. doi: 10.1017/S1047951104006067. PY - 2004 SN - 1047-9511 (Print) 1047-9511 SP - 615-21 ST - Quality of life in children with pulmonary atresia and intact ventricular septum T2 - Cardiol Young TI - Quality of life in children with pulmonary atresia and intact ventricular septum VL - 14 ID - 145 ER - TY - JOUR AB - Comments on an article, Ethical Issues in Hypoplastic Left Heart Syndrome by D. Isaacs et al. (2013). The results of Isaacs et al.'s study showed that children with congenital heart disease rated their quality of life (QoL) significantly lower than their classmates. The reduction was related to ongoing care: cardiac interventions, school absence, regular medications and non cardiac morbidities were all independently associated with reduced QoL. (PsycINFO Database Record (c) 2016 APA, all rights reserved) AD - Isaacs, David AN - 2014-48655-020 AU - Isaacs, David DB - psyh DO - 10.1111/jpc.12748_3 DP - EBSCOhost IS - 11 KW - congenital heart disease quality of life ethical issues Bioethics Congenital Disorders Heart Disorders N1 - Children's Hospital at Westmead, Westmead, NSW, Australia. Other Publishers: Blackwell Publishing. Release Date: 20150921. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Comment/Reply. Language: English. Major Descriptor: Bioethics; Congenital Disorders; Heart Disorders; Quality of Life. Classification: Cardiovascular Disorders (3295). Population: Human (10). References Available: Y. Page Count: 2. Issue Publication Date: Nov, 2014. Copyright Statement: The Authors. Journal of Paediatrics and Child Health—Paediatrics and Child Health Division (Royal Australasian College of Physicians). 2014. PY - 2014 SN - 1034-4810 1440-1754 SP - 929-930 ST - Quality of life in congenital heart disease T2 - Journal of Paediatrics and Child Health TI - Quality of life in congenital heart disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2014-48655-020&site=ehost-live&scope=site david.isaacs@health.nsw.gov.au VL - 50 ID - 1685 ER - TY - JOUR AB - BACKGROUND: Treatment of electrophysiologic (EP) disease in pediatric patients has improved; however, the effects on quality of life (QOL) are unknown. OBJECTIVE: The purpose of this study was to compare QOL within EP disease groups and to other congenital heart diseases, to evaluate the effects of cardiac rhythm devices on QOL, and to identify drivers of QOL in EP disease. METHODS: Cross-sectional study of patient/parent proxy-reported Pediatric Cardiac Quality of Life Inventory scores (Total, Disease Impact, Psychosocial Impact) in subjects aged 8 to 18 years from 11 centers with congenital complete heart block (CCHB), ventricular tachycardia (VT), supraventricular tachycardia (SVT), and long QT syndrome (LQTS). QOL was compared between EP disease groups and congenital heart disease groups [bicuspid aortic valve (BAV), tetralogy of Fallot (TOF), and Fontan]. General linear modeling was used to perform group comparisons and to identify predictors of QOL variation. RESULTS: Among 288 patient-parent pairs, mean age was 12.8 ± 3.0 years. CCHB (μ = 83) showed higher patient Total QOL than other EP disease cohorts (P ≤ .02; LQTS μ = 73; SVT μ = 74). SVT (μ = 75) and LQTS (μ = 75) had lower patient Total scores than BAV (μ = 81; P ≤ .008). Patient/parent-proxy QOL scores for all EP disease groups were not different than TOF and higher than Fontan. The presence of a cardiac rhythm device was associated with lower QOL scores in LQTS (μ = 66 vs μ = 76; P < .01). Predictors of lower patient/parent-proxy QOL included EP disease type (P ≤ .03), increased medical care utilization (P ≤ .04), and no parental college degree (P ≤ .001). CONCLUSION: Given the significant variation in QOL in EP disease type, stratification by EP disease type and increased medical care utilization may allow for targeted interventions to improve QOL. AD - The Heart Institute, Department of Pediatrics, Division of Pediatric Cardiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Electronic address: Richard.czosek@cchmc.org. Department of Biostatistics, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Critical Care and Cardiorespiratory Division, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, United Kingdom. Miami Children's Hospital, Miami, Florida. Department of Cardiology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts. Herma Heart Center, Children's Hospital of Wisconsin, Milwaukee Wisconsin. Department of Pediatrics, University of Texas Southwestern Medical School, Dallas, Texas. Division of Pediatric Cardiology, UCSF Benioff Children's Hospital, and Department of Pediatrics, UCSF School of Medicine, San Francisco, California. Phoenix Children's Hospital and Arizona Pediatric Cardiology Consultants/Pediatrix, University of Arizona School of Medicine-Phoenix, Phoenix, Arizona. Department of Paediatric Cardiology, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom. Department of Pediatrics, Stanford University and Lucile Packard Children's Hospital, Palo Alto, California. The Heart Institute, Department of Pediatrics, Division of Pediatric Cardiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. AN - 25602174 AU - Czosek, R. J. AU - Cassedy, A. E. AU - Wray, J. AU - Wernovsky, G. AU - Newburger, J. W. AU - Mussatto, K. A. AU - Mahony, L. AU - Tanel, R. E. AU - Cohen, M. I. AU - Franklin, R. C. AU - Brown, K. L. AU - Rosenthal, D. AU - Drotar, D. AU - Marino, B. S. DA - May DB - PubMed DO - 10.1016/j.hrthm.2015.01.022 DP - NLM ET - 2015/01/21 IS - 5 KW - Adolescent *Arrhythmias, Cardiac/congenital/diagnosis/psychology/therapy Child Cross-Sectional Studies *Defibrillators/psychology/statistics & numerical data Female Heart Defects, Congenital/*psychology Heart Rate Humans Linear Models Male *Pacemaker, Artificial/psychology/statistics & numerical data Pediatrics/methods *Quality of Life United Kingdom United States Arrhythmia Channelopathy Electrophysiology Pediatrics Quality of life cardiac rhythm devices LA - eng N1 - 1556-3871 Czosek, Richard J Cassedy, Amy E Wray, Jo Wernovsky, Gil Newburger, Jane W Mussatto, Kathleen A Mahony, Lynn Tanel, Ronn E Cohen, Mitchell I Franklin, Rodney C Brown, Kate L Rosenthal, David Drotar, Dennis Marino, Bradley S Comparative Study Journal Article Multicenter Study United States Heart Rhythm. 2015 May;12(5):899-908. doi: 10.1016/j.hrthm.2015.01.022. Epub 2015 Jan 17. PY - 2015 SN - 1547-5271 SP - 899-908 ST - Quality of life in pediatric patients affected by electrophysiologic disease T2 - Heart Rhythm TI - Quality of life in pediatric patients affected by electrophysiologic disease VL - 12 ID - 120 ER - TY - JOUR AB - Objectives Improved treatments for patients with congenital heart disease (CHD) have led to a growing interest in long-term functional outcomes such as health-related quality of life (HRQoL). Studies on HRQoL in children with CHD have contradicting results. Therefore, we compared HRQoL of children with CHD with that of current healthy peers and stratify CHD cases by severity and diagnostic subgroups. Methods We included 514 patients (191 girls) aged 7-17 (12.9±3.1) years who were recruited at our institution between July 2014 and May 2017. The self-reported and age-adapted KINDL questionnaire was used to assess HRQoL. Patient data were compared with that of a recent control group of 734 healthy children (346 girls, 13.4±2.1 years). Results Patients with CHD scored at least as high as healthy peers in HRQoL (CHD: 78.6±9.8; healthy: 75.6±10.1; P<0.001). After correction for sex and age, patients with CHD presented a 2.3-point higher HRQoL (P<0.001). The sex-specific and age-specific analyses showed that there were no differences between boys with and without CHD in childhood (P=0.255), but in adolescence, boys with CHD had on average 3.9-point higher scores (P=0.001), whereas girls with CHD had statistically higher HRQoL perception than healthy girls in childhood (4.2 points; P=0.003) and adolescence (4.2 points; P=0.005). There were no differences between the severity classes or diagnostic subgroups in the total HRQoL score or in the six subdomains. Conclusion The high HRQoL in young patients with CHD suggests that they can cope well with their disease burden. This holds true for all severity classes and diagnostic subgroups. AD - B. Reiner, Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Munich, Germany AU - Reiner, B. AU - Oberhoffer, R. AU - Ewert, P. AU - Müller, J. DB - Embase Medline DO - 10.1136/archdischild-2017-314211 IS - 2 KW - adolescence adolescent article atrioventricular septal defect child childhood clinical outcome congenital heart disease developmental stage disease severity Fallot tetralogy female groups by age heart single ventricle heart ventricle septum defect human long term care major clinical study male patient coding priority journal quality of life quality of life assessment school child self esteem LA - English M3 - Article N1 - L622811110 2018-07-05 2019-11-29 PY - 2019 SN - 1468-2044 0003-9888 SP - 124-128 ST - Quality of life in young people with congenital heart disease is better than expected T2 - Archives of Disease in Childhood TI - Quality of life in young people with congenital heart disease is better than expected UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L622811110 http://dx.doi.org/10.1136/archdischild-2017-314211 VL - 104 ID - 630 ER - TY - JOUR AB - To assess the health-related quality of life (QoL) in children with congenital heart diseases (CHD) with a validated questionnaire in comparison with control children. We prospectively recruited 282 children with CHD aged from 8 to 18 years in two tertiary care centers (France and Belgium) and 180 same-age controls in randomly selected French schools. Children’s QoL was self-reported with the KIDSCREEN-52 questionnaire and reported by parents with the KIDSCREEN-27. QoL scores of each dimension were compared between CHD and controls and between the classes of disease severity. Both centers were comparable for most demographic and clinical data. Age- and gender-adjusted self-reported QoL scores were lower in CHD children than in controls for physical well-being (mean ± SEM 45.97 ± 0.57 vs 50.16 ± 0.71, p < 0.0001), financial resources (45.72 ± 0.70 vs 48.85 ± 0.87, p = 0.01), peers/social support (48.01 ± 0.72 vs 51.02 ± 0.88, p = 0.01), and autonomy in the multivariate analysis (47.63 ± 0.69 vs 49.28 ± 0.85, p = 0.04). Parents-reported scores were lower in CHD children for physical (p < 0.0001), psychological well-being (p = 0.04), peers/social support (p < 0.0001), and school environment (p < 0.0001) dimensions. Similarly, the disease severity had an impact on physical well-being (p < 0.001), financial resources (p = 0.05), and peers/social support (p = 0.01) for self-reported dimensions, and on physical well-being (p < 0.001), psychological well-being (p < 0.01), peers/social support (p < 0.001), and school environment (p < 0.001) for parents-reported dimensions. However, in multivariate analysis on self-reported QoL, disease severity was significantly associated with the self-perception dimension only. Self-reported QoL of CHD children was similar to that of same-age healthy children in seven of 10 dimensions, but parents-reported QoL was impaired in four of five dimensions. AD - P. Amedro, Physiology and Experimental Biology of Heart and Muscles Laboratory - PHYMEDEXP, UMR CNRS 9214 – INSERM U1046, Montpellier University, Montpellier, France AU - Amedro, P. AU - Dorka, R. AU - Moniotte, S. AU - Guillaumont, S. AU - Fraisse, A. AU - Kreitmann, B. AU - Borm, B. AU - Bertet, H. AU - Barrea, C. AU - Ovaert, C. AU - Sluysmans, T. AU - De La Villeon, G. AU - Vincenti, M. AU - Voisin, M. AU - Auquier, P. AU - Picot, M. C. DB - Embase Medline DO - 10.1007/s00246-015-1201-x IS - 8 KW - NCT01202916) anticoagulant agent beta adrenergic receptor blocking agent calcium channel blocking agent dipeptidyl carboxypeptidase inhibitor diuretic agent adolescent adult article artificial heart pacemaker assessment of humans Belgium catecholaminergic polymorphic ventricular tachycardia child clinical assessment congenital heart disease congestive cardiomyopathy controlled study coronary artery anomaly cross-sectional study demography disease severity female financial management France heart single ventricle heart surgery heart ventricle septum defect human hypertrophic cardiomyopathy implantable cardioverter defibrillator KIDSCREEN 27 KIDSCREEN 52 long QT syndrome major clinical study male multicenter study multivariate analysis parent patient autonomy prospective study psychological well-being quality of life school scoring system self report social support LA - English M3 - Article N1 - L604642291 2015-06-08 2015-12-01 PY - 2015 SN - 1432-1971 0172-0643 SP - 1588-1601 ST - Quality of Life of Children with Congenital Heart Diseases: A Multicenter Controlled Cross-Sectional Study T2 - Pediatric Cardiology TI - Quality of Life of Children with Congenital Heart Diseases: A Multicenter Controlled Cross-Sectional Study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L604642291 http://dx.doi.org/10.1007/s00246-015-1201-x VL - 36 ID - 840 ER - TY - JOUR AB - OBJECTIVES: We sought to assess quality of life of pediatric cardiac extracorporeal membrane oxygenation survivors. We hypothesized that these patients would have decreased quality of life when compared to that of a general U.S. population sample. DESIGN: Cross-sectional study. SETTING: Patient homes and Children's Hospital Boston. PATIENTS: Cardiac extracorporeal membrane oxygenation survivors currently 5-18 yrs old. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Quality of life was assessed by parent proxy report using the Child Health Questionnaire Parent Form 50 and was compared to that of a general U.S. population sample and other cardiac populations. Factors associated with lower quality of life were sought. Physical summary scores for 41 cardiac extracorporeal membrane oxygenation survivors were lower than the mean of the general population sample (42.4 ± 16.4 vs. 53.0 ± 8.8; p < .001) but similar to those of children with Fontan physiology or an automatic implantable cardioverter defibrillator. Psychosocial summary scores in extracorporeal membrane oxygenation patients were not different from those of the general population (48.2 ± 11.8 vs. 51.2 ± 9.1; p = .11) or of other cardiac samples. Postcardiotomy extracorporeal membrane oxygenation, more noncardiac operations, total intensive care and hospital days, noncardiac medical conditions, medications, and the need for physical, occupational, or speech therapy were associated with low physical summary scores. More noncardiac operations, noncardiac medical conditions, and the need for special education, physical, occupational, or speech therapy were associated with low psychosocial summary scores. CONCLUSIONS: In pediatric cardiac extracorporeal membrane oxygenation survivors, the physical component of health-related quality of life is lower than that of the general population but similar to that of patients with complex cardiac disease, whereas psychosocial quality of life is similar to that of the general population and of other pediatric cardiac populations. AD - Department of Cardiology, Children's Hospital Boston, Harvard Medical School, Boston, MA, USA. jmcostello@childrensmemorial.org AN - 22067987 AU - Costello, J. M. AU - O'Brien, M. AU - Wypij, D. AU - Shubert, J. AU - Salvin, J. W. AU - Newburger, J. W. AU - Laussen, P. C. AU - Arnold, J. H. AU - Fynn-Thompson, F. AU - Thiagarajan, R. R. DA - Jul DB - PubMed DO - 10.1097/PCC.0b013e318238ba21 DP - NLM ET - 2011/11/10 IS - 4 KW - Adolescent Boston Child Child, Preschool Cross-Sectional Studies *Extracorporeal Membrane Oxygenation Female Heart Diseases/*therapy Humans Male Proxy *Quality of Life Surveys and Questionnaires LA - eng N1 - Costello, John M O'Brien, Molly Wypij, David Shubert, Joana Salvin, Joshua W Newburger, Jane W Laussen, Peter C Arnold, John H Fynn-Thompson, Francis Thiagarajan, Ravi R Journal Article Research Support, Non-U.S. Gov't United States Pediatr Crit Care Med. 2012 Jul;13(4):428-34. doi: 10.1097/PCC.0b013e318238ba21. PY - 2012 SN - 1529-7535 (Print) 1529-7535 SP - 428-34 ST - Quality of life of pediatric cardiac patients who previously required extracorporeal membrane oxygenation T2 - Pediatr Crit Care Med TI - Quality of life of pediatric cardiac patients who previously required extracorporeal membrane oxygenation VL - 13 ID - 133 ER - TY - JOUR AB - Children with long QT syndrome (LQTS) live with the risk of sudden death, activity restrictions, and the need for daily medications. We sought to evaluate the quality of life (QOL), self-perception, and behavior of patients with LQTS as perceived by both patients and their parents and identify predictors of lower QOL. QOL (Pediatric QOL Inventory [PedsQL] and Pediatric Cardiac Quality of Life Inventory [PCQLI]), self-perception, and behavioral inventories were completed by patients with LQTS and their parents. Comparison of PedsQL scores was made to published data for healthy children using t tests, and PCQLI scores were compared with those of patients with differing complexity of congenital heart disease. Mixed modeling was used for multivariable analysis. Sixty-one patients with LQTS were evaluated (age 13.6 ± 3.0 years; male 49%). Compared with healthy children, the PedsQL Total, Psychosocial, and Physical Health Summary scores were significantly lower for patients with LQTS and parent proxy reports (p ≤0.001). In general, PCQLI scores of patients with LQTS and parents were similar to those of patients with tetralogy of Fallot (p ≥0.2), lower than those of patients with bicuspid aortic valve (p ≤0.02), and higher than those of patients with single ventricle (p ≤0.03). Lower patient and parent PCQLI scores were associated with internalizing problems. For parents, the presence of a cardiac device and medication side effects were additionally associated with lower PCQLI scores. In conclusion, patients with LQTS and their parents report lower QOL than normal children secondary to physical and psychosocial factors. Increasing focus on the psychological well-being of these patients is needed in an effort to improve their QOL. AD - Division of Pediatric Cardiology, Department of Pediatrics, The Heart Institute at Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Electronic address: Richard.czosek@cchmc.org. Division of Pediatric Cardiology, Department of Pediatrics, Children's National Medical Center, Washington, DC. Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio; Division of Biostatistics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Division of Cardiology, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. Division of Pediatric Cardiology, Department of Pediatrics, the UCSF Benioff Children's Hospital, San Francisco, California. Department of Pediatrics, The Heart Program at Nicklaus Children's Hospital, Florida International University Herbert Wertheim College of Medicine, Miami, Florida. Cardiorespiratory Department, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom; Department of Pediatric Cardiology, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom. Division of Cardiology, Department of Pediatrics, Ann & Robert F. Lurie Children's Hospital of Chicago, Chicago, Illinois. AN - 26721659 AU - Czosek, R. J. AU - Kaltman, J. R. AU - Cassedy, A. E. AU - Shah, M. J. AU - Vetter, V. L. AU - Tanel, R. E. AU - Wernovksy, G. AU - Wray, J. AU - Marino, B. S. DA - Feb 15 DB - PubMed DO - 10.1016/j.amjcard.2015.11.051 DP - NLM ET - 2016/01/02 IS - 4 KW - Adolescent Child Cross-Sectional Studies Electrocardiography Emotions/physiology Female Follow-Up Studies Humans Long QT Syndrome/physiopathology/*psychology Male *Quality of Life *Self Concept Surveys and Questionnaires LA - eng N1 - 1879-1913 Czosek, Richard J Kaltman, Jonathan R Cassedy, Amy E Shah, Maully J Vetter, Victoria L Tanel, Ronn E Wernovksy, Gil Wray, Jo Marino, Bradley S Journal Article Multicenter Study United States Am J Cardiol. 2016 Feb 15;117(4):605-610. doi: 10.1016/j.amjcard.2015.11.051. Epub 2015 Dec 7. PY - 2016 SN - 0002-9149 SP - 605-610 ST - Quality of Life of Pediatric Patients With Long QT Syndrome T2 - Am J Cardiol TI - Quality of Life of Pediatric Patients With Long QT Syndrome VL - 117 ID - 83 ER - TY - JOUR AB - The question "what makes a good quality of life?" is a philosophical one which could be thought immune to scientific investigations. However, over the last few decades there has been great progress in developing tools to quantify quality of life (QoL) to make comparisons between different health states, evaluate the effectiveness of medical interventions, and describe the life trajectories of individuals or groups. Using a series of vignettes, we explore and review the biomedical literature to demonstrate how QoL is affected by chronic health conditions in childhood, and how it evolves as individuals pass into adulthood. Individuals experiencing serious chronic illnesses generally have reduced health-related QoL: their health status has significant repercussions of their everyday life, but scores are usually much better than healthy individuals expect, and better than physicians predict. Global QoL is more than a health status concept. QoL is a complex relationship between objectivity and subjectivity; it requires substantial and valid facts, and it defines itself by an interpretation of health within different schemes of values: societal, medical, and those of the subject themselves. QoL is dynamic; purely physical influences diminish as individuals age, and psychosocial factors become much more important. Resilience frequently allows adaptation to adverse health states, leading to acceptable QoL for most children with disabilities. © 2011 Mosby, Inc. All rights reserved. AD - A. Payot, Department of Pediatrics, University of Montral, Montreal, QC, Canada AU - Payot, A. AU - Barrington, K. J. DB - Embase Medline DO - 10.1016/j.cppeds.2010.10.008 IS - 4 KW - dopamine anxiety Arnold Chiari malformation article attention deficit disorder auditory screening brain ventricle dilatation central venous catheter cerebral palsy cesarean section chest infection child health care childhood disease chronic disease clubfoot cognitive defect colostomy constipation coping behavior depression extremely low birth weight family counseling functional status handicapped child health care cost hearing aid human hypoplastic left heart syndrome infant disease infection risk intelligence quotient intestine perforation job finding life expectancy lung dysplasia meningomyelocele motivation motor development musculoskeletal stiffness necrotizing enterocolitis neonatology patent ductus arteriosus physiotherapy posttraumatic stress disorder prematurity quality of life questionnaire respiratory arrest resuscitation school admission scoring system self esteem sepsis tissue perfusion trisomy 13 trisomy 18 unilateral hearing loss urinary tract infection very low birth weight visual analog scale visual impairment LA - English M3 - Article N1 - L361507867 2011-04-04 2011-04-06 PY - 2011 SN - 1538-5442 SP - 91-101 ST - The quality of life of young children and infants with chronic medical problems: Review of the literature T2 - Current Problems in Pediatric and Adolescent Health Care TI - The quality of life of young children and infants with chronic medical problems: Review of the literature UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L361507867 http://dx.doi.org/10.1016/j.cppeds.2010.10.008 VL - 41 ID - 1105 ER - TY - JOUR AB - Background: Quality of life (QOL) in adolescents and adults who have undergone the Fontan procedure and are living with only 1 ventricle is presumed to be diminished. Objectives: This study aimed to compare QOL, health status, and prevalence of depression in adolescents/adults after the Fontan procedure with healthy counterparts and to identify predictors of QOL in the Fontan group. Methods: Using a comparative, cross-sectional design, 54 adolescents and adults with single ventricle congenital heart disease who have undergone the Fontan procedure were compared with 66 age-matched healthy counterparts. Quality of life, health status, depression, and social support were measured using the Satisfaction With Life Scale, Short Form Survey Version 2, Patient Health Questionnaire Depression Module, and Multidimensional Scale of Perceived Social Support. Clinical variables were abstracted from medical records. Predictors of QOL were determined using multiple linear regression. Results: Adolescents and adults in the Fontan group reported lower physical health status (mean [SD] = 46.5 [9.3] vs mean [SD] = 55.9 [5.1], P < .001) and were more depressed (mean [SD] = 7.3 [5.9] vs mean [SD] = 4.5 [4.3], P < .004) than their healthy counterparts. There were no differences in QOL, mental health status, or social support between the 2 groups. Functional status (New York Heart Association class), depression, and social support accounted for 55% of the variance in QOL in the Fontan group. Conclusions: Despite lower levels of physical health, the QOL of Fontan patients was comparable with that of their healthy counterparts; this finding contradicts previous proxy reports, self-reports, and assumptions that QOL is lower in patients with complex single ventricle congenital heart disease. However, because Fontan patients were more depressed than their healthy counterparts, the need for early screening and detection is warranted. (PsycINFO Database Record (c) 2019 APA, all rights reserved) AD - Pike, Nancy A., School of Nursing, University of California, Los Angeles, Factor Bldg, Room 3-938, Los Angeles, CA, US, 90095-6919 AN - 2013-06756-007 AU - Pike, Nancy A. AU - Evangelista, Lorraine S. AU - Doering, Lynn V. AU - Eastwood, Jo-Ann AU - Lewis, Alan B. AU - Child, John S. DB - psyh DO - 10.1097/JCN.0b013e31822ce5f6 DP - EBSCOhost IS - 6 KW - quality of life health status depression adolescent development social support Adolescent Adult Cross-Sectional Studies Female Fontan Procedure Humans Male Retrospective Studies Surveys and Questionnaires Young Adult Age Differences Health Behavior Depression (Emotion) N1 - School of Nursing, University of California, Los Angeles, Los Angeles, CA, US. Release Date: 20140519. Correction Date: 20190912. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Adolescent Development; Age Differences; Health Behavior; Quality of Life; Social Support. Minor Descriptor: Depression (Emotion). Classification: Developmental Psychology (2800). Population: Human (10); Male (30); Female (40). Location: US. Age Group: Adolescence (13-17 yrs) (200); Adulthood (18 yrs & older) (300); Young Adulthood (18-29 yrs) (320); Thirties (30-39 yrs) (340); Middle Age (40-64 yrs) (360). Tests & Measures: 36-Item Short Form Health Survey-Version 2; SF-36 Health Survey; Satisfaction With Life Scale DOI: 10.1037/t01069-000; Multidimensional Scale of Perceived Social Support—Military Peers Items Addition DOI: 10.1037/t03506-000; Patient Health Questionnaire-9 DOI: 10.1037/t06165-000. Methodology: Empirical Study; Longitudinal Study; Retrospective Study; Quantitative Study. References Available: Y. Page Count: 8. Issue Publication Date: Nov-Dec, 2012. Copyright Statement: Wolters Kluwer Health | Lippincott Williams & Wilkins. 2012. PY - 2012 SN - 0889-4655 1550-5049 SP - 539-546 ST - Quality of life, health status, and depression: Comparison between adolescents and adults after the Fontan procedure with healthy counterparts T2 - Journal of Cardiovascular Nursing TI - Quality of life, health status, and depression: Comparison between adolescents and adults after the Fontan procedure with healthy counterparts UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2013-06756-007&site=ehost-live&scope=site ORCID: 0000-0003-1708-4186 npike@sonnet.ucla.edu VL - 27 ID - 1672 ER - TY - JOUR AB - BACKGROUND: Quality of life (QOL) in adolescents and adults who have undergone the Fontan procedure and are living with only 1 ventricle is presumed to be diminished. OBJECTIVES: This study aimed to compare QOL, health status, and prevalence of depression in adolescents/adults after the Fontan procedure with healthy counterparts and to identify predictors of QOL in the Fontan group. METHODS: Using a comparative, cross-sectional design, 54 adolescents and adults with single ventricle congenital heart disease who have undergone the Fontan procedure were compared with 66 age-matched healthy counterparts. Quality of life, health status, depression, and social support were measured using the Satisfaction With Life Scale, Short Form Survey Version 2, Patient Health Questionnaire Depression Module, and Multidimensional Scale of Perceived Social Support. Clinical variables were abstracted from medical records. Predictors of QOL were determined using multiple linear regression. RESULTS: Adolescents and adults in the Fontan group reported lower physical health status (mean [SD] = 46.5 [9.3] vs mean [SD] = 55.9 [5.1], P < .001) and were more depressed (mean [SD] = 7.3 [5.9] vs mean [SD] = 4.5 [4.3], P < .004) than their healthy counterparts. There were no differences in QOL, mental health status, or social support between the 2 groups. Functional status (New York Heart Association class), depression, and social support accounted for 55% of the variance in QOL in the Fontan group. CONCLUSIONS: Despite lower levels of physical health, the QOL of Fontan patients was comparable with that of their healthy counterparts; this finding contradicts previous proxy reports, self-reports, and assumptions that QOL is lower in patients with complex single ventricle congenital heart disease. However, because Fontan patients were more depressed than their healthy counterparts, the need for early screening and detection is warranted. AD - School of Nursing, University of California, Los Angeles, CA 90095-6919, USA. npike@sonnet.ucla.edu AN - 21912272 AU - Pike, N. A. AU - Evangelista, L. S. AU - Doering, L. V. AU - Eastwood, J. A. AU - Lewis, A. B. AU - Child, J. S. C2 - PMC3460064 C6 - NIHMS384268 DA - Nov-Dec DB - PubMed DO - 10.1097/JCN.0b013e31822ce5f6 DP - NLM ET - 2011/09/14 IS - 6 KW - Adolescent Adult Cross-Sectional Studies Depression/*epidemiology/*etiology Female Fontan Procedure/*adverse effects *Health Status Humans Male *Quality of Life Retrospective Studies Surveys and Questionnaires Young Adult LA - eng N1 - 1550-5049 Pike, Nancy A Evangelista, Lorraine S Doering, Lynn V Eastwood, Jo-Ann Lewis, Alan B Child, John S R01 HL093466/HL/NHLBI NIH HHS/United States Comparative Study Journal Article J Cardiovasc Nurs. 2012 Nov-Dec;27(6):539-46. doi: 10.1097/JCN.0b013e31822ce5f6. PY - 2012 SN - 0889-4655 (Print) 0889-4655 SP - 539-46 ST - Quality of life, health status, and depression: comparison between adolescents and adults after the Fontan procedure with healthy counterparts T2 - J Cardiovasc Nurs TI - Quality of life, health status, and depression: comparison between adolescents and adults after the Fontan procedure with healthy counterparts VL - 27 ID - 158 ER - TY - JOUR AB - OBJECTIVES. Mortality rates in pediatric patients with heart disease have decreased dramatically in recent decades, resulting in an increasing number of survivors with morbidities that impact quality of life. The purpose of this study was to assess and compare how heart disease affects the quality of life of the pediatric cardiac patient from the perspectives of the patient, parent, and health care provider. METHOD. Individual focus groups were conducted with children (8-12 years of age) with heart disease, adolescents (13-18 years of age) with heart disease, parents of children with heart disease, parents of adolescents with heart disease, and health care providers of pediatric patients with heart disease. A structured focus group technique was used to develop a list of potential items that might affect quality of life. Participants chose the 5 most important items from the list. These items were then categorized into preidentified dimensions (physical, psychological, social, school, and other). The percentages of the total votes for all items were calculated and distributions of responses across dimensions within group and within dimension across groups were reported and compared qualitatively. RESULTS. Patient and parent groups identified similar items as important. Providers identified different items. The physical limitation item received the largest percentage of total votes in all groups (9%-20%). Analysis across dimension revealed that those items related to the physical dimension received the highest percentage of total votes among all groups (30.2%-51.2%). Analysis within dimension revealed that patients endorsed items in the physical dimension more frequently than parents or providers. Within the psychological dimension, the children selected the fewest items relative to all other groups (7.2% vs 21.3%-37.8%), whereas health care providers endorsed these items more frequently than patients or parents (child or parent of child groups 7.2%-21.3% vs health care provider group 28.8%; adolescent or parent of adolescent groups 29.6% vs health care provider group 37.8%). Differences were noted between the child and adolescent groups in the psychological (child versus adolescent: 7.2% vs 29.6%) and school (child versus adolescent: 11.2% vs 2.1%) dimensions. CONCLUSIONS. Patients and parents generally agreed on how heart disease affects the quality of life of children and adolescents, whereas health care providers had a different opinion. Copyright © 2009 by the American Academy of Pediatrics. AD - B. S. Marino, Cincinnati Children's Hospital Medical Center, Division of Cardiology, 3333 Burnet Ave, ML 2003, Cincinnati, OH 45229, United States AU - Marino, B. S. AU - Tomlinson, R. S. AU - Drotar, D. AU - Claybon, E. S. AU - Aguirre, A. AU - Ittenbach, R. AU - Welkom, J. S. AU - Helfaer, M. A. AU - Wernovsky, G. AU - Shea, J. A. DB - Embase Medline DO - 10.1542/peds.2008-2572 IS - 4 KW - adolescent aortic coarctation aortic stenosis aortic regurgitation arterial trunk article calculation cardiac patient cardiomyopathy child complete heart block congenital heart disease congestive cardiomyopathy Fallot tetralogy female health care personnel heart arrhythmia heart atrium septum defect heart right ventricle double outlet heart ventricle septum defect heart ventricle tachycardia human hypoplastic left heart syndrome information processing major clinical study male mitral valve regurgitation pediatrics priority journal psychological aspect pulmonary valve atresia pulmonary valve stenosis qualitative analysis quality of life school child social aspect supraventricular tachycardia tricuspid valve atresia LA - English M3 - Article N1 - L354628901 2009-06-16 http://pediatrics.aappublications.org/cgi/reprint/123/4/e708 PY - 2009 SN - 0031-4005 1098-4275 SP - e708-e715 ST - Quality-of-life concerns differ among patients, parents, and medical providers in children and adolescents with congenital and acquired heart disease T2 - Pediatrics TI - Quality-of-life concerns differ among patients, parents, and medical providers in children and adolescents with congenital and acquired heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L354628901 http://dx.doi.org/10.1542/peds.2008-2572 VL - 123 ID - 1186 ER - TY - JOUR AB - Objectives To examine racial/ethnic-specific survival of children with major birth defects in the US. Study design We pooled data on live births delivered during 1999-2007 with any of 21 birth defects from 12 population-based birth defects surveillance programs. We used the Kaplan-Meier method to calculate cumulative survival probabilities and Cox proportional hazards models to estimate mortality risk. Results For most birth defects, there were small-to-moderate differences in neonatal (<28 days) survival among racial/ethnic groups. However, compared with children born to non-Hispanic white mothers, postneonatal infant (28 days to <1 year) mortality risk was significantly greater among children born to non-Hispanic black mothers for 13 of 21 defects (hazard ratios [HRs] 1.3-2.8) and among children born to Hispanic mothers for 10 of 21 defects (HRs 1.3-1.7). Compared with children born to non-Hispanic white mothers, a significantly increased childhood (≤8 years) mortality risk was found among children born to Asian/Pacific Islander mothers for encephalocele (HR 2.6), tetralogy of Fallot, and atrioventricular septal defect (HRs 1.6-1.8) and among children born to American Indian/ Alaska Native mothers for encephalocele (HR 2.8), whereas a significantly decreased childhood mortality risk was found among children born to Asian/Pacific Islander mothers for cleft lip with or without cleft palate (HR 0.6). Conclusion Children with birth defects born to non-Hispanic black and Hispanic mothers carry a greater risk of mortality well into childhood, especially children with congenital heart defect. Understanding survival differences among racial/ethnic groups provides important information for policy development and service planning. AD - Y. Wang, Division of Data Analysis and Research, Office of Primary Care and Health System Management, New York State Department of Health, Empire State Plaza, Corning Tower, Room 1695, Albany, NY, United States AU - Wang, Y. AU - Liu, G. AU - Canfield, M. A. AU - Mai, C. T. AU - Gilboa, S. M. AU - Meyer, R. E. AU - Anderka, M. AU - Copeland, G. E. AU - Kucik, J. E. AU - Nembhard, W. N. AU - Kirby, R. S. DB - Embase Medline DO - 10.1016/j.jpeds.2014.12.025 IS - 4 KW - Alaska Native American Indian anencephalus aortic coarctation aortic valve stenosis arm malformation article Asian atrioventricular septal defect child childhood mortality cleft lip cleft palate congenital malformation diaphragm hernia Down syndrome encephalocele esophagus atresia ethnic difference Fallot tetralogy gastroschisis Hispanic human hypoplastic left heart syndrome infant mortality intestine atresia leg malformation live birth major clinical study newborn mortality omphalocele overall survival Pacific Islander population research priority journal pylorus stenosis race difference spinal dysraphism survival survival rate tracheoesophageal fistula United States LA - English M3 - Article N1 - L601970400 2015-02-06 2015-07-09 PY - 2015 SN - 1097-6833 0022-3476 SP - 819-826.e2 ST - Racial/ethnic differences in survival of United States children with birth defects: A population-based study T2 - Journal of Pediatrics TI - Racial/ethnic differences in survival of United States children with birth defects: A population-based study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L601970400 http://dx.doi.org/10.1016/j.jpeds.2014.12.025 VL - 166 ID - 882 ER - TY - JOUR AB - In 15 patients with tricuspid atresia and one with tricuspid stenosis the left ventricular ejection fraction was measured by equilibrium gated radionuclide angiography and the results compared with those from a control group of 16 patients. The patients with tricuspid atresia had a significantly depressed ejection fraction. Those who had a surgical shunt or who had had pulmonary artery banding had significantly lower ejection fractions than the remainder. There was no significant correlation between the ejection fraction and age, the arterial oxygen saturation, or the haemoglobin concentration. Five patients were also studied during isometric exercise; three had an abnormal response. Volume overload of the ventricle is identified as one cause of the dysfunction, but other factors may be important. Radionuclide angiography offers a non-invasive method of studying ventricular function in this condition. AD - Department of Pediatric Cardiology, Guy's Hospital, London SE1 9RT AU - Baker, E. J. AU - Jones, O. D. H. AU - Joseph, M. C. DB - Embase Medline IS - 5 KW - radioisotope adult child congenital disorder diagnosis heart heart left ventricle ejection fraction human major clinical study priority journal scintiangiography tricuspid valve atresia tricuspid valve stenosis LA - English M3 - Article N1 - L15208181 1985-01-16 PY - 1984 SN - 0007-0769 SP - 572-574 ST - Radionuclide measurement of left ventricular ejection fraction in tricuspid atresia T2 - British Heart Journal TI - Radionuclide measurement of left ventricular ejection fraction in tricuspid atresia UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L15208181 VL - 52 ID - 1397 ER - TY - JOUR AB - Children with single-ventricle disease experience high mortality and complex care. In other life-limiting childhood illnesses, paediatric palliative care may mitigate maternal stress. We hypothesised that early palliative care in the single-ventricle population may have the same benefit for mothers. In this pilot randomised trial of early palliative care, mothers of infants with prenatal single-ventricle diagnoses completed surveys measuring depression, anxiety, coping, and quality of life at a prenatal visit and neonatal discharge. Infants were randomised to receive early palliative care - structured evaluation, psychosocial/spiritual, and communication support before surgery - or standard care. Among 56 eligible mothers, 40 enrolled and completed baseline surveys; 38 neonates were randomised, 18 early palliative care and 20 standard care; and 34 postnatal surveys were completed. Baseline Beck Depression Inventory-II and State-Trait Anxiety Index scores exceeded normal pregnant sample scores (mean 13.76±8.46 versus 7.0±5.0 and 46.34±12.59 versus 29.8±6.35, respectively; p=0.0001); there were no significant differences between study groups. The early palliative care group had a decrease in prenatal to postnatal State-Trait Anxiety Index scores (-7.6 versus 0.3 in standard care, p=0.02), higher postnatal Brief Cope Inventory positive reframing scores (p=0.03), and a positive change in PedsQL Family Impact Module communication and family relationships scores (effect size 0.46 and 0.41, respectively). In conclusion, these data show that mothers of infants with single-ventricle disease experience significant depression and anxiety prenatally. Early palliative care resulted in decreased maternal anxiety, improved maternal positive reframing, and improved communication and family relationships. AD - 1Department of Pediatrics,Division of Pediatric Cardiology,Ward Family Heart Center,Children's Mercy Kansas City,Missouri,United States of America. 2Department of Pediatrics,Palliative Care,University of Michigan,Ann Arbor,Michigan,United States of America. 3Department of Pediatrics,Division of Pediatric Cardiology,University of Michigan,Ann Arbor,Michigan,United States of America. 4Department of Pediatrics,School of Medicine,Divisions of Pediatric Cardiology and Critical Care Medicine,Washington University,St. Louis,Missouri,United States of America. 5Department of Internal Medicine,Division of Geriatric Medicine and Palliative Care,University of Michigan,Ann Arbor,Michigan,United States of America. AN - 29316996 AU - Hancock, H. S. AU - Pituch, K. AU - Uzark, K. AU - Bhat, P. AU - Fifer, C. AU - Silveira, M. AU - Yu, S. AU - Welch, S. AU - Donohue, J. AU - Lowery, R. AU - Aiyagari, R. DA - Apr DB - PubMed DO - 10.1017/s1047951117002761 DP - NLM ET - 2018/01/11 IS - 4 KW - *Adaptation, Psychological Adult Female Follow-Up Studies Heart Defects, Congenital/diagnosis/psychology/*surgery Heart Ventricles/*abnormalities/diagnostic imaging/surgery Humans Infant, Newborn Mother-Child Relations/*psychology Mothers/*psychology Palliative Care/methods/*psychology Pregnancy Psychiatric Status Rating Scales Stress, Psychological/diagnosis/psychology/*therapy Time Factors Chd hypoplastic left heart syndrome paediatric palliative care parental stress LA - eng N1 - 1467-1107 Hancock, Hayley S Pituch, Ken Uzark, Karen Bhat, Priya Orcid: 0000-0002-8768-8818 Fifer, Carly Silveira, Maria Yu, Sunkyung Welch, Suzanne Donohue, Janet Lowery, Ray Aiyagari, Ranjit Journal Article Randomized Controlled Trial England Cardiol Young. 2018 Apr;28(4):561-570. doi: 10.1017/S1047951117002761. Epub 2018 Jan 10. PY - 2018 SN - 1047-9511 SP - 561-570 ST - A randomised trial of early palliative care for maternal stress in infants prenatally diagnosed with single-ventricle heart disease T2 - Cardiol Young TI - A randomised trial of early palliative care for maternal stress in infants prenatally diagnosed with single-ventricle heart disease VL - 28 ID - 21 ER - TY - JOUR AB - The patient, a 'blue baby', had been examined in reveral cardiac centers during his youth, and open heart intervention had been tried without success. The clinical impression at the time of his last hospitalization was tetralogy of Fallot, with secondary polycythemia. On the fifth day he lapsed into coma and died six days later at age 32. At autopsy, a single ventricle with a common atrioventricular valve was demonstrated. There was no rudimentary outlet chamber, but a small muscular band on the left lateral wall was interpreted as a rudimentary ventricular septum. The great arteries were in normal position. Concomitant pulmonary stenosis was of the infundibular type. Since this particular combination of congential anomalies did not appear in the 60 cases analyzed by Van Praagh et al, it would seem to be unique. Congenital cardiac defects, particularly in cyanotic children, are often associated with mental retardation, but this man was a productive free lance writer until his last illness. AD - R.J. Kositchek, Dept. Int. Med., St. John's Hosp., Santa Monica, CA, United States AU - Kositchek, R. J. AU - Schreiner, B. J. DB - Embase Classic Medline IS - 6 KW - artery autopsy baby child coma erythrocytosis Fallot tetralogy general aspects of disease heart heart atrioventricular valve heart single ventricle interventricular septum hospitalization human juvenile mental deficiency patient pulmonary valve stenosis LA - English M3 - Article N1 - L292080676 1971-12-01 PY - 1971 SN - 0003-3197 SP - 364-374 ST - A rare case of cor triloculare biatriatum T2 - Angiology TI - A rare case of cor triloculare biatriatum UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L292080676 VL - 22 ID - 1403 ER - TY - JOUR AB - Objective: Atypical development, behavioral difficulties, and academic underachievement are common morbidities in children with a history of congenital heart defects and impact quality of life. Language and social-cognitive deficits have been described, which are associated with autism spectrum disorders. The current study aimed to assess the rates of autism spectrum disorders in a large sample of children with a history of congenital heart defects and to assess medical, behavioral, and individual factors that may be associated with the risk of autism spectrum disorders. Design: Participants included 195 children with a history of congenital heart defects, who are followed in a large-scale longitudinal study. Measures included behavioral data from 4-year-old neurodevelopmental evaluations and parent-report data from a later annual follow-up. Results: Using established cutoffs on an autism spectrum disorder screener, children with congenital heart defects showed higher rates of “possible” autism spectrum disorders than national rates, (Chi-square Test of Equal Proportions), all Ps <.05. A stepwise variable selection method was used to create a “best prediction model” and multivariable logistic regression was used to identify variables predicting diagnostic status. Factors associated with diagnostic risk included medical (delayed sternal closure, prematurity, positive genetic findings), behavioral (cognitive, language, attention issues), and individual (socioeconomic, cultural/racial) variables. ROC analyses identified a cutoff of 7 to maximize sensitivity/specificity based on parent-reported diagnosis. Conclusions: Risk of autism spectrum disorder screening status in children with congenital heart defects was higher than expected from population rates. Findings highlight the need for referral to a specialist to assess the presence and severity of social-communication issues and congenital heart defects population-specific screening thresholds for children with concern for autism spectrum disorders. AD - J.L. Bean Jaworski, Baystate Medical Center, United States AU - Bean Jaworski, J. L. AU - Flynn, T. AU - Burnham, N. AU - Chittams, J. L. AU - Sammarco, T. AU - Gerdes, M. AU - Bernbaum, J. C. AU - Clancy, R. R. AU - Solot, C. B. AU - Zackai, E. H. AU - McDonald-McGinn, D. M. AU - Gaynor, J. W. DB - Embase Medline DO - 10.1111/chd.12461 IS - 4 KW - aortic coarctation article attention autism child cognition congenital heart malformation controlled study cultural factor Fallot tetralogy female great vessels transposition heart ventricle septum defect human hypoplastic left heart syndrome language longitudinal study major clinical study male prediction prematurity prevalence priority journal race receiver operating characteristic risk factor sensitivity and specificity socioeconomics LA - English M3 - Article N1 - L614869222 2017-03-22 2017-08-17 PY - 2017 SN - 1747-0803 1747-079X SP - 421-429 ST - Rates of autism and potential risk factors in children with congenital heart defects T2 - Congenital Heart Disease TI - Rates of autism and potential risk factors in children with congenital heart defects UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L614869222 http://dx.doi.org/10.1111/chd.12461 VL - 12 ID - 742 ER - TY - JOUR AB - A rational approach to the surgical management of tricuspid atresia with diminished blood flow to the lungs is as follows: A cava-pulmonary artery shunt is performed as the initial operation whenever possible. In the newborn infant and in patients in whom the pulmonary artery is less than one-half the diameter of the superior vena cava where the two vessels cross, the right pulmonary artery is anastomosed end-to-side to the ascending aorta. Subsequently, when the right pulmonary artery has enlarged, it is detached from the aorta and anastomosed to the superior vena cava. Closure of the outflow from the right ventricle to the left can be expected in tricuspid atresia after the first year of life, and unless the ductus remains patent, a systemic artery-pulmonary artery shunt on the left side will be required to supplement the cavapulmonary artery shunt. This shunt will also be required as a preliminary step to replacing the ascending aorta-right pulmonary artery shunt with a cava - right pulmonary artery shunt if blood flow to the left lung is by bronchial collateral arteries only. Banding of the pulmonary artery is indicated in those patients with excessive blood flow to the lungs. In the present series of 19 patients with tricuspid atresia. there were 17 with diminished flow to the lungs and two with excessive flow. Eight patients with small pulmonary arteries had a shunt made between the ascending aorta and right pulmonary artery. Five patients were less than 2 wk of age. All survived operation; one died a year postoperatively of unknown causes. Two shunts have been replaced with cava-pulmonary artery shunts. Ten patients had a shunt made between the superior vena cava and the right pulmonary artery 1 to 8 yr ago. There has been no operative or late mortality. A supplemental systemic artery-pulmonary artery shunt on the left side has been established in 3 patients. Two patients have had operative enlargement of the interatrial communication. Excessive blood flow to the lungs was present in 2 patients. Neither patient survived banding of the pulmonary artery. AD - W.W.L. Glenn, Dept. of Surg., Yale Univ. Sch. of Med., New Haven, Conn AU - Glenn, W. W. L. DB - Embase Classic Medline IS - 4 SUPPL. 2 KW - aorta artery artery bypass ascending aorta blood flow cardiovascular surgery heart right ventricle interpersonal communication lung mortality newborn patent patient pulmonary artery superior cava vein surgery tricuspid valve atresia LA - English M3 - Article N1 - L288040245 1968-12-01 PY - 1968 SN - 0009-7322 SP - 62-67 ST - Rational approach to the surgical management of tricuspid atresia T2 - Circulation TI - Rational approach to the surgical management of tricuspid atresia UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L288040245 VL - 37 ID - 1410 ER - TY - JOUR AB - Hypoplastic left heart syndrome (HLHS) is a fatal congenital heart disease in which the left side of the heart is underdeveloped, impairing the systemic circulation. Underdeveloped left ventricle exerts biomechanical stress on the right ventricle that can progress into heart failure. Genome-wide transcriptome changes have been identified at early stages in the right ventricle (RV) of infants with HLHS, although the molecular mechanisms remain unknown. Here, we demonstrate that the RNA binding protein Rbfox2, which is mutated in HLHS patients, is a contributor to transcriptome changes in HLHS patient RVs. Our results indicate that majority of transcripts differentially expressed in HLHS patient hearts have validated Rbfox2 binding sites. We show that Rbfox2 regulates mRNA levels of targets with 3'UTR binding sites contributing to aberrant gene expression in HLHS patients. Strikingly, the Rbfox2 nonsense mutation identified in HLHS patients truncates the protein, impairs its subcellular distribution and adversely affects its function in RNA metabolism. Overall, our findings uncover a novel role for Rbfox2 in controlling transcriptome in HLHS. AU - Verma, S. K. AU - Deshmukh, V. AU - Nutter, C. A. AU - Jaworski, E. AU - Jin, W. AU - Wadhwa, L. AU - Abata, J. AU - Ricci, M. AU - Lincoln, J. AU - Martin, J. F. AU - Yeo, G. W. AU - Kuyumcu-Martinez, M. N. DB - Medline DO - 10.1038/srep30896 KW - messenger RNA RBFOX2 protein, human repressor protein RNA splicing factor alternative RNA splicing genetics human hypoplastic left heart syndrome metabolism newborn pathology stop codon LA - English M3 - Article N1 - L622858830 2018-07-11 PY - 2016 SN - 2045-2322 SP - 30896 ST - Rbfox2 function in RNA metabolism is impaired in hypoplastic left heart syndrome patient hearts T2 - Scientific reports TI - Rbfox2 function in RNA metabolism is impaired in hypoplastic left heart syndrome patient hearts UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L622858830 http://dx.doi.org/10.1038/srep30896 VL - 6 ID - 797 ER - TY - JOUR AB - Congenital heart disease (CHD) affects a considerable number of children and adults worldwide. This implicates not only developmental disorders, high mortality, and reduced quality of life but also, high costs for the healthcare systems. CHD refers to a variety of heart and vascular malformations which could be very challenging to reconstruct the malformed region surgically, especially when the patient is an infant or a child. Advanced technology and research have offered a better mechanistic insight on the impact of CHD in the heart and vascular system of infants, children, and adults and identified potential therapeutic solutions. Many artificial materials and devices have been used for cardiovascular surgery. Surgeons and the medical industry created and evolved the ball valves to the carbon-based leaflet valves and introduced bioprosthesis as an alternative. However, with research further progressing, contracting tissue has been developed in laboratories and tissue engineering (TE) could represent a revolutionary answer for CHD surgery. Development of engineered tissue for cardiac and aortic reconstruction for developing bodies of infants and children can be very challenging. Nevertheless, using acellular scaffolds, allograft, xenografts, and autografts is already very common. Seeding of cells on surface and within scaffold is a key challenging factor for use of the above. The use of different types of stem cells has been investigated and proven to be suitable for tissue engineering. They are the most promising source of cells for heart reconstruction in a developing body, even for adults. Some stem cell types are more effective than others, with some disadvantages which may be eliminated in the future. AD - A.O.J. Fakoya, Department of Anatomical Sciences, University of Medicine and Health Sciences, Basseterre, Saint Kitts and Nevis AU - Mantakaki, A. AU - Fakoya, A. O. J. AU - Sharifpanah, F. DB - Embase DO - 10.7717/peerj.5805 IS - 10 KW - tissue scaffold biomaterial aortic coarctation article bone marrow cell line cardiac scaffold cardiac stem cell congenital heart disease Ebstein anomaly embryonic stem cell extracellular matrix health care system heart atrium septum defect heart ventricle septum defect human hypoplastic left heart syndrome induced pluripotent stem cell lung vein drainage anomaly nonhuman patent ductus arteriosus pulmonary valve atresia pulmonary valve stenosis stem cell transplantation survival rate tissue engineering tricuspid valve atresia LA - English M3 - Article N1 - L624540283 2018-11-02 2018-11-07 PY - 2018 SN - 2167-8359 ST - Recent advances and challenges on application of tissue engineering for treatment of congenital heart disease T2 - PeerJ TI - Recent advances and challenges on application of tissue engineering for treatment of congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L624540283 http://dx.doi.org/10.7717/peerj.5805 VL - 2018 ID - 724 ER - TY - JOUR AB - Rationale and Objectives: This study aimed to describe prenatal and postnatal imaging features and outcomes of neonates with neonatal lymphatic disorders (NLDs). Materials and Methods: An institutional review board-approved search of the radiology database for patients with NLD identified five patients. Inclusion criteria include prenatal imaging (fetal magnetic resonance [MR] imaging and ultrasound) and postnatal three-dimensional T2 Sampling Perfection with Application optimized Contrasts using different flip angle Evolution (SPACE) and dynamic contrast-enhanced MR lymphangiography within 6 months of life. Chart review was undertaken to evaluate morbidity and mortality. Results: Prenatal finding of “nutmeg lung” or fetal pulmonary lymphatic disorder was identified in all five patients on fetal MR imaging, and in four of five patients on fetal ultrasound. Postnatal dynamic contrast-enhanced MR lymphangiography demonstrated abnormal lymphatic flow to the lungs in four of five patients, but absent in the single patient with coexisting hypoplastic left heart syndrome (HLHS). Dermal backflow was seen in one patient, also the only patient with prenatal body wall edema. Three patients with lymphatic flow to the lungs only were classified as neonatal chylothorax. The patient with dermal backflow and perfusion to the lungs was diagnosed with central lymphatic flow disorder (CLFD). The HLHS patient with normal lymphatic perfusion maintained the HLHS diagnosis. Of the five patients, the patient with CLFD and the one with HLHS expired because of respiratory distress. Conclusions: NLDs can be recognized on prenatal and postnatal imaging and may be primary, as in neonatal chylothorax or CLFD, or secondary. In this small series, “nutmeg lung” was present in all patients. Prenatal imaging demonstrates that body wall edema may correlate with postnatal dermal backflow, which, in our small cohort, carried a poor prognosis. AD - D.M. Biko, Department of Radiology, Children's Hospital of Philadelphia, 34th and Civic Center Blvd, Philadelphia, PA, United States AU - Biko, D. M. AU - Johnstone, J. A. AU - Dori, Y. AU - Victoria, T. AU - Oliver, E. R. AU - Itkin, M. C1 - gadavist(Bayer HealthCare,United States) lipiodol(Guerbet,United States) C2 - Bayer HealthCare(United States) Guerbet(United States) DB - Embase Medline DO - 10.1016/j.acra.2018.02.020 IS - 11 KW - nuclear magnetic resonance scanner gadobutrol iodinated poppyseed oil article central lymphatic flow disorder chylothorax clinical article conservative treatment contrast enhancement fetus echography health care human hypoplastic left heart syndrome lung lymph flow lymphangiography lymphatic flow disorder lymphatic system disease newborn newborn disease nuclear magnetic resonance imaging perinatal period pleura effusion prenatal diagnosis priority journal respiratory distress retrospective study gadavist lipiodol LA - English M3 - Article N1 - L2000570628 2018-03-30 2019-09-20 PY - 2018 SN - 1878-4046 1076-6332 SP - 1446-1450 ST - Recognition of Neonatal Lymphatic Flow Disorder: Fetal MR Findings and Postnatal MR Lymphangiogram Correlation T2 - Academic Radiology TI - Recognition of Neonatal Lymphatic Flow Disorder: Fetal MR Findings and Postnatal MR Lymphangiogram Correlation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2000570628 http://dx.doi.org/10.1016/j.acra.2018.02.020 VL - 25 ID - 655 ER - TY - JOUR AB - We describe the successful use of recombinant factor VIIa (rFVIIa) in the control of massive haemoptysis in a 17-year-old patient with a Fontan circulation. The patient was intubated and ventilated in the ICU with deteriorating gas exchange. Conventional methods to control the haemoptysis were ineffective, and rFVIIa was successfully administered as a rescue therapy. rFVIIa is a powerful pro-thrombotic agent, which is only licensed in haemophiliacs with acquired inhibitors to anticoagulation. It has been used off-license in the treatment of massive haemorrhage, although a Cochrane review did not show any significant benefit; however, it may have a role as a rescue therapy where alternatives options have been exhausted after careful risk-benefit analysis. © Cambridge University Press 2017. AD - Critical Care, Guy's and St Thomas' NHS Foundation Trust, St Thomas' Hospital, Westminster Bridge Road, London, SE1 7EH, United Kingdom Adult Congenital Cardiology, St Thomas' Hospital, Guys and St Thomas' NHS Foundation Trust, London, United Kingdom AU - Mason, T. W. AU - Retter, A. AU - Morgan, G. J. DB - Scopus DO - 10.1017/S1047951117001007 IS - 1 KW - factor VIIa Fontan haemoptysis rFVIIa M3 - Article N1 - Export Date: 15 June 2020 PY - 2018 SP - 144-146 ST - Recombinant factor VIIa as a rescue therapy in severe haemoptysis in a patient with a Fontan circulation T2 - Cardiology in the Young TI - Recombinant factor VIIa as a rescue therapy in severe haemoptysis in a patient with a Fontan circulation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85038437121&doi=10.1017%2fS1047951117001007&partnerID=40&md5=a6a60fbf15ff8d84679d3dfbf2069f89 VL - 28 ID - 1837 ER - TY - JOUR AB - The population of adults living with congenital heart disease (CHD) has grown rapidly in recent decades due to major advances in surgical and medical care. Although the benefits of physical activity (PA) and exercise are well recognised in the management of chronic diseases, the therapeutic role of exercise for the CHD population has been under-studied and under-recognised. In fact, people living with complex CHD have traditionally been advised against participation in moderate or vigorous PA due to safety concerns. However, a small but growing body of literature has demonstrated that PA and exercise are safe and beneficial for the vast majority of people with CHD following appropriate screening. Exercise training (ET) is a potent therapy to improve well-being and cardiorespiratory fitness, which likely has important implications for prognosis. This has led to ET becoming an increasingly important adjunct to medical therapy in the management of this group who frequently have significant exercise limitation and complex medical issues. However, there is currently limited evidence-based guidance that has been published regarding appropriate exercise prescription in adults with CHD. This article aims to provide an overview of different congenital heart conditions, their impact on the exercise response, and to provide considerations and recommendations for ET in adolescents and adults living with CHD based on the available literature. AD - Discipline of Exercise and Sport Science, Faculty of Medicine and Health, The University of Sydney, Camperdown, New South Wales, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia; Sydney Medical School, The University of Sydney, Camperdown, New South Wales, Australia. School of Physiotherapy and Exercise Science, Curtin University, Bentley, Western Australia, Australia; Allied Health Department, Fiona Stanley Hospital, Murdoch, Western Australia, Australia. Sydney Medical School, The University of Sydney, Camperdown, New South Wales, Australia; The Heart Centre for Children, The Children's Hospital at Westmead, Westmead, New South Wales, Australia. Priority Research Centre for Physical Activity and Nutrition, School of Education, University of Newcastle, Callaghan Campus, Newcastle, New South Wales, Australia. Discipline of Exercise and Sport Science, Faculty of Medicine and Health, The University of Sydney, Camperdown, New South Wales, Australia. Department of Cardiology, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia; Sydney Medical School, The University of Sydney, Camperdown, New South Wales, Australia. Department of Cardiac Surgery, The Royal Children's Hospital, Melbourne, Victoria, Australia; Department of Paediatrics, University of Melbourne, Parkville, Victoria, Australia; Heart Research, Clinical Sciences, Murdoch Children's Research Institute, Melbourne, Victoria, Australia. Department of Cardiology, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia; Sydney Medical School, The University of Sydney, Camperdown, New South Wales, Australia. Electronic address: rachael.cordina@sydney.edu.au. AN - 32201288 AU - Tran, D. AU - Maiorana, A. AU - Ayer, J. AU - Lubans, D. R. AU - Davis, G. M. AU - Celermajer, D. S. AU - d'Udekem, Y. AU - Cordina, R. DA - Mar 19 DB - PubMed DO - 10.1016/j.pcad.2020.03.002 DP - NLM ET - 2020/03/24 KW - Aerobic training Eisenmenger's syndrome Exercise prescription Fontan circulation Physical activity Pulmonary hypertension Resistance training Tetralogy of Fallot Transposition of the great arteries LA - eng N1 - 1873-1740 Tran, Derek Maiorana, Andrew Ayer, Julian Lubans, David R Davis, Glen M Celermajer, David S d'Udekem, Yves Cordina, Rachael Journal Article Review United States Prog Cardiovasc Dis. 2020 Mar 19:S0033-0620(20)30059-1. doi: 10.1016/j.pcad.2020.03.002. PY - 2020 SN - 0033-0620 ST - Recommendations for exercise in adolescents and adults with congenital heart disease T2 - Prog Cardiovasc Dis TI - Recommendations for exercise in adolescents and adults with congenital heart disease ID - 289 ER - TY - JOUR AB - During the period 1981-1986, 1605 infants presenting a major congenital heart defect (CHD) were identified in Sweden. Using the personal identification numbers of the mothers, 1507 of them could be linked to the Medical Birth Registry and two controls were selected for each infant. For this total of 4521 infants, 2686 postoccurrence sibs born during the period 1981-1989 were identified from the Medical Birth Registry. The tendency to have one or more sibs was higher among cases than among controls and was correlated to the life status of the proband. This tendency did not vary according to the type of CHD when controlling for life status. The prevalence at birth of CHD was almost four times higher among sibs of CHD infants than among sibs of normal infants. The tendency to have a sib with an extracardiac malformation did not differ between CHD probands and normal probands. The importance of only including births occurring after the proband when evaluating recurrence risks is stressed. AD - P. Pradat, Department of Embryology, Biskopsgatan 7, S-223 62 Lund, Sweden AU - Pradat, P. DB - Embase Medline IS - 2 KW - aortic coarctation artery malformation article congenital heart malformation controlled study endocardial cushion defect female heart septum defect human hypoplastic left heart syndrome infant major clinical study male mitral valve disease recurrence risk register sibling Sweden tricuspid valve disease LA - English M3 - Article N1 - L24138842 1994-05-23 PY - 1994 SN - 0741-0395 SP - 131-140 ST - Recurrence risk for major congenital heart defects in Sweden: A registry study T2 - Genetic Epidemiology TI - Recurrence risk for major congenital heart defects in Sweden: A registry study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L24138842 VL - 11 ID - 1373 ER - TY - JOUR AB - Most maternal cardiac disease in Western societies is now congenital in origin. This relates to the significant improvements in congenital cardiac surgery during the last years. Some patients will present for the first time in pregnancy with symptoms and learn that they have congenital heart disease. So all patients should have a detailed evaluation and appropriate counseling before pregnancy. Ebstein anomaly is a rare and complex congenital heart disorder occurring in 1 per 200 000 live births and, first described by a German physician Wilhelm Ebstein in 1866 in a report titled, “Concerning a very rare case of insufficiency of the tricuspid valve caused by a congenital malformation. This anomaly accounts for 0.5%-0.7% of cases of congenital heart disease. The average life duration of patients with Ebstein’s anomaly is 25-30 years. The malformation consists of apical displacement of the tricuspid valve with resultant regurgitation and enlargement of the right heart chambers, resulting in arrhythmias and heart failure. The common cardiac anomalies associated with the condition are atrial septal defect (ASD) in 90% of patients, anatomic or functional tricuspid atresia in 30%, Wolff-Parkinson-White (WPW) syndrome in 15% and less commonly ventricular septal defect (VSD), pulmonic stenosis or atresia and mitral valve prolapse. Survival into adulthood is common and patients present with cyanosis, dyspnea and palpitations. © 2014 The Author(s). AD - Cardiovascular Research Center, Tabriz University of Medical Sciences, Tabriz, Iran AU - Ranjbar, A. AU - Parizad, R. AU - Tabrizi, M. T. DB - Scopus DO - 10.15296/ijwhr.2014.41 IS - 4 KW - Box Shaped Heart Ebstein Anomaly Pregnancy Recurrent Abortion M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2014 SP - 265-267 ST - Recurrent abortion in a patient with ebstein anomaly T2 - International Journal of Women's Health and Reproduction Sciences TI - Recurrent abortion in a patient with ebstein anomaly UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84969255757&doi=10.15296%2fijwhr.2014.41&partnerID=40&md5=0e77e998bbff52ab51d7ddd5d869e754 VL - 2 ID - 1991 ER - TY - JOUR AB - OBJECTIVE: The objective of this article is to describe a case of recurrent delirium after cardiac surgery in an infant. DESIGN: Case report. The institutional review board at Children's Hospital Los Angeles waived the need for informed consent. SETTING: Cardiothoracic intensive care unit in a freestanding children's hospital. PATIENT: A male infant with hypoplastic left heart syndrome who developed delirium on consecutive admissions to the cardiothoracic intensive care unit after cardiac surgery. INTERVENTION: Pharmacologic intervention using the atypical antipsychotic olanzapine. MEASUREMENTS AND MAIN RESULTS: The symptoms of delirium resolved with the initiation and continuation of olanzapine on both occasions. CONCLUSION: Delirium is a common, but often unrecognized, diagnosis in the intensive care unit. Its early recognition and treatment may prevent unnecessary use of narcotics and benzodiazepines, decrease length of stay and may improve long-term neurocognitive function. This case report describes an infant who developed discrete, consecutive episodes of delirium following surgery for congenital heart disease. Both episodes were treated effectively with olanzapine. AD - Department of Pediatrics and Anesthesiology Critical Care Medicine, Children's Hospital, Los Angeles, CA, USA. kmadden@chla.usc.edu AN - 21336229 AU - Madden, K. AU - Turkel, S. AU - Jacobson, J. AU - Epstein, D. AU - Moromisato, D. Y. DA - Nov DB - PubMed DO - 10.1097/PCC.0b013e31820ac2bf DP - NLM ET - 2011/02/22 IS - 6 KW - Delirium/*drug therapy/etiology/physiopathology Humans Hypoplastic Left Heart Syndrome/physiopathology/*surgery Infant, Newborn Intensive Care Units, Pediatric Male Outcome Assessment, Health Care Postoperative Complications/*drug therapy Recurrence LA - eng N1 - Madden, Kevin Turkel, Susan Jacobson, Julienne Epstein, David Moromisato, David Y Case Reports Journal Article United States Pediatr Crit Care Med. 2011 Nov;12(6):e413-5. doi: 10.1097/PCC.0b013e31820ac2bf. PY - 2011 SN - 1529-7535 (Print) 1529-7535 SP - e413-5 ST - Recurrent delirium after surgery for congenital heart disease in an infant T2 - Pediatr Crit Care Med TI - Recurrent delirium after surgery for congenital heart disease in an infant VL - 12 ID - 427 ER - TY - JOUR AB - The results of the Transfusion Requirements in Pediatric Intensive Care Unit study suggest that a red blood cell transfusion is not required in stable or stabilized pediatric intensive care unit children as long as their hemoglobin level is >7 g/dL. Subgroup analyses suggest that this recommendation is also adequate for stable critically ill children with a high severity of illness, respiratory dysfunction, acute lung injury, sepsis, neurological dysfunction, severe head trauma, or severe trauma, and during the postoperative period, for noncyanotic patients older than 28 days. A small randomized clinical trial suggests that a hemoglobin level of 9 g/dL is safe in the postoperative care of children with single-ventricle physiology undergoing cavopulmonary connection. Although there is consensus that blood is clearly indicated for the treatment of hemorrhagic shock, the clinical determinants that should prompt pediatric intensivists to prescribe a red blood cell transfusion to unstable PICU children are not well characterized. AD - Division of Pediatric Critical Care, Department of Pediatrics, Sainte-Justine Hospital, Université de Montréal, Montréal, Quebec, Canada. jacques_lacroix@ssss.gouv.qc.ca AN - 22818542 AU - Lacroix, J. AU - Demaret, P. AU - Tucci, M. DA - Aug DB - PubMed DO - 10.1053/j.semperi.2012.04.002 DP - NLM ET - 2012/07/24 IS - 4 KW - Child Critical Illness/*therapy Decision Making Erythrocyte Indices Erythrocyte Transfusion/*standards Hemoglobins Humans Intensive Care Units, Pediatric Pediatrics/*methods Practice Guidelines as Topic Severity of Illness Index LA - eng N1 - 1558-075x Lacroix, Jacques Demaret, Pierre Tucci, Marisa 130770/Canadian Institutes of Health Research/Canada 84300/Canadian Institutes of Health Research/Canada Journal Article Research Support, Non-U.S. Gov't Review United States Semin Perinatol. 2012 Aug;36(4):225-31. doi: 10.1053/j.semperi.2012.04.002. PY - 2012 SN - 0146-0005 SP - 225-31 ST - Red blood cell transfusion: decision making in pediatric intensive care units T2 - Semin Perinatol TI - Red blood cell transfusion: decision making in pediatric intensive care units VL - 36 ID - 322 ER - TY - JOUR AB - AIM: A growing number of patients with Fontan circulation are reaching adulthood, and there is increasing concern about their physical performance and quality of life. This study compared self-reported exercise and measured activity with quality of life in patients after Fontan palliation and healthy controls. METHODS: Physical exercise during an average school week was reported by 30 Fontan circulation patients aged eight to 20 years, and 25 healthy controls, followed by accelerometer recordings over seven days. All subjects and their parents answered a questionnaire on quality of life. RESULTS: Patients reported spending less time exercising each week than the controls (114 ± 66 minutes vs. 228 ± 147 minutes, p < 0.001). However, the overall measured activity and moderate-to-vigorous activity was similar for patients and controls. Patients reported a lower quality of life score than the controls (70.9 ± 9.9 vs. 85.7 ± 8.0, p < 0.001). CONCLUSION: In spite of similar measured total activity, Fontan patients reported less time engaged in regular physical exercise than healthy controls and their quality of life was lower than the controls. We speculate that promoting structured regular physical exercise could improve the quality of life of Fontan patients. AD - Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden. eva.rylander-hedlund@karolinska.se. Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden. Department of Physiotherapy, Karolinska University Hospital, Stockholm, Sweden. Division of Physiotherapy, Department of Neurobiology Care Sciences and Society, Karolinska Institutet, Stockholm, Sweden. AN - 27515293 AU - Hedlund, E. R. AU - Lundell, B. AU - Villard, L. AU - Sjöberg, G. DA - Nov DB - PubMed DO - 10.1111/apa.13544 DP - NLM ET - 2016/08/16 IS - 11 KW - Accelerometry/instrumentation/methods Adolescent Case-Control Studies Child *Exercise/physiology/psychology Exercise Tolerance/*physiology Female Fontan Procedure/adverse effects/psychology/*rehabilitation Humans Linear Models Long Term Adverse Effects Male *Quality of Life Self Report Surveys and Questionnaires Survival Analysis Sweden Tricuspid Atresia/*surgery Young Adult *Accelerometer *Fontan circulation *Fontan palliation *Physical exercise LA - eng N1 - 1651-2227 Hedlund, Eva R Lundell, Bo Villard, Li Sjöberg, Gunnar Journal Article Norway Acta Paediatr. 2016 Nov;105(11):1322-1328. doi: 10.1111/apa.13544. Epub 2016 Sep 8. PY - 2016 SN - 0803-5253 SP - 1322-1328 ST - Reduced physical exercise and health-related quality of life after Fontan palliation T2 - Acta Paediatr TI - Reduced physical exercise and health-related quality of life after Fontan palliation VL - 105 ID - 80 ER - TY - JOUR AB - Objective: Enteral feeding is associated with decreased infection rates, decreased mechanical ventilation, decreased hospital length of stay, and improved wound healing. Enteral feeding difficulties are common in congenital heart disease. Our objective was to develop experience-based newborn feeding guidelines for the initiation and advancement of enteral feeding in the cardiothoracic intensive care unit. Design: This is a retrospective analysis of a quality improvement project. Setting: This quality improvement project was performed in a cardiothoracic intensive care unit. Patients: Newborns admitted to the cardiothoracic intensive care unit for cardiac surgery from January 2011 to May 2015 were retrospectively reviewed. Intervention: Newborn feeding guidelines for the initiation and advancement of enteral feeding were implemented in January 2012. Outcome measures: Guideline compliance and clinical variables before and after guideline implementation were reviewed. Results: Compliance with the guidelines increased from 83% in 2012 to 100% in the first two quarters of 2015. Preguidelines (January 2011–December 2011): 45 newborns underwent cardiac surgery; 8 deaths prior to discharge; 1 patient discharged from NICU, therefore, N = 36. Postguidelines (January 2012–May 2015): 131 newborns with 12 deaths, 12 admitted from home, 8 in the NICU, 3 on the floor preop, and 3 back transferred, therefore, N = 93. No difference in feeding preop (post 75% vs pre 69%; P =.5) or full po feeds at discharge (post 78% vs pre 89%; P =.2). Mesenteric ischemia was not statistically different postguidelines (post 6% vs pre 14%; P =.14). Length of hospital stay decreased postguidelines (post 27 + 17 d vs pre 34 + 42 d; P <.001). Conclusions: Implementation of experience-based newborn feeding guidelines for initiation and advancement of enteral feeding in the cardiothoracic intensive care unit was successful in reducing practice variation supported by increasing guideline compliance. Percentage of patient's full oral feeding at discharge did not change. Length of hospital stay was reduced although cannot be fully attributed to feeding guideline implementation. AD - J.M. Simsic, The Heart Center at Nationwide Children's Hospital, Columbus, OH, United States AU - Simsic, J. M. AU - Carpenito, K. R. AU - Kirchner, K. AU - Peters, S. AU - Miller-Tate, H. AU - Joy, B. AU - Galantowicz, M. DB - Embase Medline DO - 10.1111/chd.12435 IS - 3 KW - aortic arch anomaly aortic coarctation article cardiac surgery intensive care unit child nutrition congenital heart disease controlled study enteric feeding heart surgery hospital admission hospital discharge human hypoplastic left heart syndrome length of stay lung vein drainage anomaly major clinical study mesenteric ischemia neonatal intensive care unit newborn newborn death postoperative period practice guideline preoperative care priority journal protocol compliance pulmonary valve atresia retrospective study total quality management tricuspid valve atresia LA - English M3 - Article N1 - L613688431 2016-12-19 2017-06-15 PY - 2017 SN - 1747-0803 1747-079X SP - 275-281 ST - Reducing variation in feeding newborns with congenital heart disease T2 - Congenital Heart Disease TI - Reducing variation in feeding newborns with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L613688431 http://dx.doi.org/10.1111/chd.12435 VL - 12 ID - 756 ER - TY - JOUR AB - OBJECTIVE: Corticosteroids attenuate an inflammatory reaction in pediatric heart surgery. Inflammation is a source of free oxygen radicals. Children with a cyanotic heart defect are prone to increased radical stress during heart surgery. The authors hypothesized that high-dose methylprednisolone reduces inflammatory reaction and thereby also oxidative stress in infants with a univentricular heart defect undergoing the bidirectional Glenn procedure. DESIGN: A double-blind, placebo-controlled, randomized clinical trial. SETTING: Operating room and pediatric intensive care unit of a university hospital. PARTICIPANTS: The study comprised 29 infants undergoing the bidirectional Glenn procedure with or without aortic arch or pulmonary arterial repair. INTERVENTIONS: After anesthesia induction, the patients received intravenously either 30 mg/kg of methylprednisolone (n = 15) or the same volume of saline as placebo (n = 14). MEASUREMENTS AND MAIN RESULTS: Plasma interleukin-6, interleukin-8, interleukin-10 (biomarkers of inflammation), and 8-hydroxydeoxyguanosine concentrations (a biomarker of oxidative stress) were measured at the following 4 time points: preoperatively, during cardiopulmonary bypass, after protamine administration, and 6 hours postoperatively. The study parameters did not differ between the study groups preoperatively. Methylprednisolone reduced the proinflammatory cytokines interleukin-6 and interleukin-8 and increased the anti-inflammatory cytokine interleukin-10 postoperatively. Despite reduced inflammation, there were no differences in 8-hydroxydeoxyguanosine between the methylprednisolone and placebo groups. CONCLUSIONS: The proinflammatory reaction and increase in free radical stress were not interrelated during congenital heart surgery in cyanotic infants with a univentricular heart defect undergoing the bidirectional Glenn procedure. High-dose methylprednisolone was ineffective in attenuating free radical stress. AD - Division of Anaesthesiology, Department of Anaesthesiology, Intensive Care and Pain Medicine, Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland. Electronic address: juho.keski-nisula@hus.fi. Department of Anesthesiology, Perioperative and Pain Medicine, Stanford University School of Medicine, Stanford, CA. Department of Pediatric Nephrology and Transplantation, Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland. Department of Neonatology, Pediatric Research Center, Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland. Division of Anaesthesiology, Department of Anaesthesiology, Intensive Care and Pain Medicine, Kirurginen sairaala, University of Helsinki and Helsinki University Hospital, Helsinki, Finland. AN - 32037273 AU - Keski-Nisula, J. AU - Arvola, O. AU - Jahnukainen, T. AU - Andersson, S. AU - Pesonen, E. DA - Jun DB - PubMed DO - 10.1053/j.jvca.2019.10.015 DP - NLM ET - 2020/02/11 IS - 6 KW - bidirectional Glenn procedure congenital heart defect infant inflammation methylprednisolone radical stress LA - eng N1 - 1532-8422 Keski-Nisula, Juho Arvola, Oiva Jahnukainen, Timo Andersson, Sture Pesonen, Eero Journal Article United States J Cardiothorac Vasc Anesth. 2020 Jun;34(6):1542-1547. doi: 10.1053/j.jvca.2019.10.015. Epub 2019 Oct 18. PY - 2020 SN - 1053-0770 SP - 1542-1547 ST - Reduction of Inflammation by High-Dose Methylprednisolone Does not Attenuate Oxidative Stress in Children Undergoing Bidirectional Glenn Procedure With or Without Aortic Arch or Pulmonary Arterial Repair T2 - J Cardiothorac Vasc Anesth TI - Reduction of Inflammation by High-Dose Methylprednisolone Does not Attenuate Oxidative Stress in Children Undergoing Bidirectional Glenn Procedure With or Without Aortic Arch or Pulmonary Arterial Repair VL - 34 ID - 389 ER - TY - JOUR AB - Background: The Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Risk Model adjusts not only for procedure and age group pairings but also for additional patient factors, including the binary presence or absence of a chromosomal abnormality (CA), syndrome (S), and/or a noncardiac congenital anatomic abnormality (NCAA). This analysis refines case-mix adjustment by adding more granular adjustment for individual conditions (CA, S, and NCAA), consistent with a hypothesis that associated risk of mortality differs between individual conditions. Methods: CA/S corresponding to the same condition were merged to a single condition code. Odds ratios were estimated for all CA/S. For CA/S associated with at least 10 deaths in neonates and infants and at least 10 deaths in children and adults, odds ratios were estimated for the effect of the CA/S separately in neonates/infants and in children/adults. In addition to these condition/age interactions, condition/age/procedure interactions were explored (eg, effect of Down syndrome was estimated based on age and procedure subgroups, including atrioventricular canal repair and single-ventricle palliation). Bayesian modeling was used to create 5 maximally homogeneous groups of CA/S from 81 candidate CA/S variables. A standard logistic regression model then incorporated indicator variables for the 5 categories of CAs/Ss, 7 unique NCAAs, and all other covariates in the previously published Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Model. Results: Analysis included 107,062 operations in 100 centers (2010 to 2015). Operative Mortality was 3,629 (3.4%). In the development sample, the C statistics of the original nonaugmented model and the augmented model were 0.872 and 0.875, respectively. Conclusions: The Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Risk Model has been augmented by addition of covariates representing individual CAs, Ss, and NCAAs. AD - J.P. Jacobs, Johns Hopkins All Children's Hospital, 600 Fifth St S, Fifth Flr, Rm 5216, Saint Petersburg, FL, United States AU - Jacobs, J. P. AU - O'Brien, S. M. AU - Hill, K. D. AU - Kumar, S. R. AU - Austin, E. H. AU - Gaynor, J. W. AU - Gruber, P. J. AU - Jonas, R. A. AU - Pasquali, S. K. AU - Pizarro, C. AU - St. Louis, J. D. AU - Meza, J. AU - Thibault, D. AU - Shahian, D. M. AU - Mayer, J. E. AU - Jacobs, M. L. DB - Embase Medline DO - 10.1016/j.athoracsur.2019.01.069 IS - 2 KW - acrocephalosyndactyly adult age Alagille syndrome article Bayes theorem cat cry syndrome child chromosome aberration congenital disorder congenital rubella syndrome Costello syndrome de Lange syndrome DiGeorge syndrome Down syndrome Edwards syndrome Ellis van Creveld syndrome fetal alcohol syndrome Goldenhar syndrome heart surgery heterotaxy syndrome Holt Oram syndrome human Hunter syndrome infant Jacobsen syndrome Kabuki makeup syndrome Kartagener syndrome Klinefelter syndrome Loeys Dietz syndrome long QT syndrome major clinical study medical society monosomy X mortality risk newborn Noonan syndrome omphalocele priority journal Rubinstein syndrome short QT syndrome situs inversus Smith Lemli Opitz syndrome surgical mortality syndrome trisomy trisomy 13 Turner syndrome Williams Beuren syndrome Wolf Hirschhorn syndrome Wolff Parkinson White syndrome LA - English M3 - Article N1 - L2002186861 2019-07-02 2019-07-29 PY - 2019 SN - 1552-6259 0003-4975 SP - 558-566 ST - Refining The Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Risk Model With Enhanced Risk Adjustment for Chromosomal Abnormalities, Syndromes, and Noncardiac Congenital Anatomic Abnormalities T2 - Annals of Thoracic Surgery TI - Refining The Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Risk Model With Enhanced Risk Adjustment for Chromosomal Abnormalities, Syndromes, and Noncardiac Congenital Anatomic Abnormalities UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2002186861 http://dx.doi.org/10.1016/j.athoracsur.2019.01.069 VL - 108 ID - 604 ER - TY - JOUR AB - The first successful total right heart bypass via atriopulmonary anastomosis (APA) were reported in 1971 for patients with tricuspid atresia. At the Children's Hospital of Buenos Aires, the cohort of such procedures started in July, when the first fenestrated right heart by pass was performed, with the interposition of a homograft between the right atrial appendage and the main pulmonary artery. In the second patient, instead of placing a homograft, the APA was achieved with the patient's own pulmonary root harvested from the outflow tract of the right ventricle. These techniques were soon replaced in 1978 with the development of the direct valveless posterior APA. Since the very beginning the principle was that the right atrium only functions as a pathway rather than a pump (reason why no inferior vena cava valves were ever used), and the diastolic properties of the systemic ventricle regulate the only real "pump" of this system. The late hemodynamic problems inherent of the APA diminished with modern surgical techniques like the lateral tunnel (LT) or the extracardiac conduit (EC). In spite of the improvement in prognosis and quality of life that the modern techniques have brought for univentricular hearts (UH), with the passing of time, deterioration of this system is frequently seen, due to chronic low cardiac output, elevated central venous pressure making heart transplantation the final stage of treatment. Progressive increase in pulmonary vascular resistances and ventricular dysfunction result in a decline in quality of life and survival. However, the timing of this occurrence is variable, and many survivors enjoy today a satisfactory clinical status. The challenge is to develop a better solution for UH, but in the mean time the Fontan Kreutzer palliation represents the best and only surgical option. It is undoubtedly one of the triumphs of cardiac surgery in congenital heart disease. AD - Congenital Heart Surgery, Posadas National Hospital and Austral University Hospital , Buenos Aires , Argentina. Cardiac Catheterization Laboratories, Pittsburgh Children's Hospital , Pittsburgh, PA , USA. Pediatric Cardiac Surgery, Bazterrica Clinic , Buenos Aires , Argentina. AN - 24400290 AU - Kreutzer, C. AU - Kreutzer, J. AU - Kreutzer, G. O. C2 - PMC3866802 DA - Dec 18 DB - PubMed DO - 10.3389/fped.2013.00045 DP - NLM ET - 2014/01/09 KW - Apa Fontan Kreutzer atriopulmonary anastomosis cardiac surgery heart disease univentricular hearts LA - eng N1 - 2296-2360 Kreutzer, Christián Kreutzer, Jacqueline Kreutzer, Guillermo O Journal Article Review Front Pediatr. 2013 Dec 18;1:45. doi: 10.3389/fped.2013.00045. PY - 2013 SN - 2296-2360 (Print) 2296-2360 SP - 45 ST - Reflections on five decades of the fontan kreutzer procedure T2 - Front Pediatr TI - Reflections on five decades of the fontan kreutzer procedure VL - 1 ID - 524 ER - TY - JOUR AB - Hypoplastic left heart syndrome (HLHS), the most severe and common form of single ventricle congenital heart lesions, is characterized by hypoplasia of the mitral valve, left ventricle (LV), and all LV outflow structures. While advances in surgical technique and medical management have allowed survival into adulthood, HLHS patients have severe morbidities, decreased quality of life, and a shortened lifespan. The single right ventricle (RV) is especially prone to early failure because of its vulnerability to chronic pressure overload, a mode of failure distinct from ischemic cardiomyopathy encountered in acquired heart disease. As these patients enter early adulthood, an emerging epidemic of RV failure has become evident. Regenerative medicine strategies may help preserve or boost RV function in children and adults with HLHS by promoting angiogenesis and mitigating oxidative stress. Rescuing a RV in decompensated failure may also require the creation of new, functional myocardium. Although considerable hurdles remain before their clinical translation, stem cell therapy and cardiac tissue engineering possess revolutionary potential in the treatment of pediatric and adult patients with HLHS who currently have very limited long-term treatment options. AD - 1 Department of Cardiac Surgery, University of Michigan , Ann Arbor, Michigan. 2 Department of Pediatric Cardiology, University of Michigan , Ann Arbor, Michigan. AN - 27245633 AU - Chery, J. AU - Wong, J. AU - Huang, S. AU - Wang, S. AU - Si, M. S. DA - Dec DB - PubMed DO - 10.1089/ten.TEB.2016.0136 DP - NLM ET - 2016/06/02 IS - 6 KW - Heart Ventricles Humans *Hypoplastic Left Heart Syndrome Myocardium Quality of Life Regenerative Medicine *congenital heart *heart failure *stem cells *tissue engineering LA - eng N1 - 1937-3376 Chery, Josue Wong, Joshua Huang, Shan Wang, Shuyun Si, Ming-Sing Journal Article Review United States Tissue Eng Part B Rev. 2016 Dec;22(6):459-469. doi: 10.1089/ten.TEB.2016.0136. Epub 2016 Jun 28. PY - 2016 SN - 1937-3368 SP - 459-469 ST - Regenerative Medicine Strategies for Hypoplastic Left Heart Syndrome T2 - Tissue Eng Part B Rev TI - Regenerative Medicine Strategies for Hypoplastic Left Heart Syndrome VL - 22 ID - 98 ER - TY - JOUR AB - Adolescents with single ventricle heart disease (SVHD) show autonomic, mood, and cognitive deficits, indicating aberrations in brain areas that regulate these functions. However, the gray matter integrity in autonomic, mood, and cognitive control sites is unclear. We examined regional brain gray matter changes, using high-resolution T1-weighted images (3.0-T magnetic resonance scanner) with voxel based morphometry procedures, as well as mood and cognitive functions in SVHD (n=18; age, 15.7±1.1years; male, 10) and controls (n=31; age, 16.0±1.1years; male, 17). High-resolution T1-weighted images were realigned, gray matter tissue type partitioned, normalized to a common space, smoothed, and compared between groups (analysis of covariance; covariates, age and gender). The mood and cognitive scores were compared between groups using independent samples t-tests. SVHD subjects showed significantly altered mood and cognitive functions over controls. Significantly reduced gray matter emerged in multiple brain areas, including the thalamus, caudate nuclei, putamen, insula, prefrontal, post-central and precentral gyrus, occipital gyrus, para-hippocampal gyrus, temporal gyrus, and cerebellar sites in SVHD over controls. SVHD subjects show compromised gray matter integrity in autonomic, mood and cognitive control sites. The findings indicate that frequent deficits found in SVHD subjects have a brain structural basis in the condition. AD - Department of Anesthesiology, University of California, Los Angeles, CA, USA. Department of Anesthesiology, University of California, Los Angeles, CA, USA; Department of Radiological Sciences, University of California, Los Angeles, CA, USA; Department of Bioengineering, University of California, Los Angeles, CA, USA; Brain Research Institute, University of California, Los Angeles, CA, USA. Electronic address: rkumar@mednet.ucla.edu. UCLA School of Nursing, University of California, Los Angeles, CA, USA. Division of Pediatric Cardiology, Children's Hospital, Los Angeles, CA, USA. Division of Pediatric Cardiology, University of California, Los Angeles, CA, USA. UCLA School of Nursing, University of California, Los Angeles, CA, USA. Electronic address: npike@sonnet.ucla.edu. AN - 29222023 AU - Singh, S. AU - Kumar, R. AU - Roy, B. AU - Woo, M. A. AU - Lewis, A. AU - Halnon, N. AU - Pike, N. C2 - PMC5801206 C6 - NIHMS926030 DA - Feb 5 DB - PubMed DO - 10.1016/j.neulet.2017.12.011 DP - NLM ET - 2017/12/10 KW - Adolescent Affective Symptoms/physiopathology *Brain Mapping Cerebral Cortex/pathology Cognition/physiology Cognitive Dysfunction/pathology/*physiopathology Female Gray Matter/*pathology/physiopathology Heart Diseases/*complications/pathology Humans Magnetic Resonance Imaging/methods Male *Cognition *Congenital heart disease *Gray matter *Magnetic resonance imaging *Voxel based morphometry article to disclose. LA - eng N1 - 1872-7972 Singh, Sadhana Kumar, Rajesh Roy, Bhaswati Woo, Mary A Lewis, Alan Halnon, Nancy Pike, Nancy R01 NR013930/NR/NINR NIH HHS/United States R01 NR016463/NR/NINR NIH HHS/United States Journal Article Research Support, N.I.H., Extramural Neurosci Lett. 2018 Feb 5;665:156-162. doi: 10.1016/j.neulet.2017.12.011. Epub 2017 Dec 6. PY - 2018 SN - 0304-3940 (Print) 0304-3940 SP - 156-162 ST - Regional brain gray matter changes in adolescents with single ventricle heart disease T2 - Neurosci Lett TI - Regional brain gray matter changes in adolescents with single ventricle heart disease VL - 665 ID - 58 ER - TY - JOUR AB - BACKGROUND: Regional low-flow perfusion of the brain is a bypass technique commonly used during stage 1 reconstruction in neonates with hypoplastic left heart syndrome and related variants. The neurodevelopmental outcome of these children is unknown. METHODS: Twenty-nine infants (22 boys, 7 girls) with hypoplastic left heart syndrome or variant requiring single ventricle palliation and aortic arch reconstruction were studied between 1999 and 2004. Mental Developmental Index (MDI) and Psychomotor Developmental Index were assessed using Bayley Scales of Infant Development and correlated with intraoperative and perioperative variables. Results are reported as mean +/- standard deviation. RESULTS: Average age at stage 1 operation and at bidirectional Glenn was 7 +/- 8 days and 6.0 +/- 2 months, respectively. The MDI was in the low average range (87.7 +/- 13.2). The Psychomotor Developmental Index was in the mildly delayed range (75.2 +/- 14.5). Regional low-flow perfusion was used in 31% (9 of 29 patients), with an average circulatory arrest time of 23.5 +/- 13.4 minutes. Deep hypothermia and circulatory arrest was used as the primary operative strategy in 69% of patients (20 of 29 patients), with an average circulatory arrest time of 44.3 +/- 15.3 minutes (p = 0.003). No differences in MDI or Psychomotor Developmental Index scores were observed between the regional low-flow perfusion and non-regional low-flow perfusion groups (MDI, 88.0 +/- 12.1 versus 87.6 +/- 14.0; p = 0.93, respectively; Psychomotor Developmental Index, 75.5 +/- 15.1 versus 75.0 +/- 14.6; p = 0.93, respectively). Lowest operative temperature (<16 degrees C) and birth order (<2 versus >3) significantly related to MDI (89.6 versus 72.8; p = 0.047). CONCLUSIONS: At 1 year of age, neurodevelopmental outcomes of patients undergoing stage 1 using regional low-flow perfusion were similar to outcomes observed in children exposed to circulatory arrest. The association of birth order and MDI suggests that early intervention may benefit these patients. AD - Department of Cardiovascular Surgery, Children's Hospital, Boston, Massachusetts 02115, USA. AN - 17126136 AU - Visconti, K. J. AU - Rimmer, D. AU - Gauvreau, K. AU - del Nido, P. AU - Mayer, J. E., Jr. AU - Hagino, I. AU - Pigula, F. A. DA - Dec DB - PubMed DO - 10.1016/j.athoracsur.2006.06.069 DP - NLM ET - 2006/11/28 IS - 6 KW - Cardiac Surgical Procedures/adverse effects Cerebrovascular Circulation *Circulatory Arrest, Deep Hypothermia Induced/adverse effects Developmental Disabilities/*diagnosis/etiology Female Heart Defects, Congenital/surgery Humans Hypoplastic Left Heart Syndrome/*surgery Infant Infant, Newborn Male Neuropsychological Tests *Perfusion/adverse effects/methods Retrospective Studies Treatment Outcome LA - eng N1 - 1552-6259 Visconti, Karen J Rimmer, David Gauvreau, Kimberlee del Nido, Pedro Mayer, John E Jr Hagino, Ikou Pigula, Frank A Comparative Study Journal Article Netherlands Ann Thorac Surg. 2006 Dec;82(6):2207-11; discussion 2211-3. doi: 10.1016/j.athoracsur.2006.06.069. PY - 2006 SN - 0003-4975 SP - 2207-11; discussion 2211-3 ST - Regional low-flow perfusion versus circulatory arrest in neonates: one-year neurodevelopmental outcome T2 - Ann Thorac Surg TI - Regional low-flow perfusion versus circulatory arrest in neonates: one-year neurodevelopmental outcome VL - 82 ID - 391 ER - TY - JOUR AB - To determine whether systolic regional wall deformation and motion, which may be used as an index of mechanical function, change with surgical intervention in patients with a functional single ventricle, a noninvasive magnetic resonance tagging technique was used to examine 33 such patients at all stages of Fontan reconstruction. The systolic motion of the intersection points was tracked to determine regional twist and radial shortening. Finite strain analysis was applied to the grid lines, and principal E1 strains were derived. The results were as follows. 1) Fontan and prebidirectional cavopulmonary anastomosis (hemiFontan) groups had the highest compressive strains, and regional heterogeneity of strain was least in the Fontan group. 2) Fontan patients had endocardial/epicardial strain different from the other surgical subgroups as well as the normal left ventricle, while the pre- and post-hemiFontan groups had basal/apical short-axis strain different from Fontan patients and normal subjects. 3) Functional single left ventricles had a different strain distribution across wall regions and surgical subgroup from functional single right ventricles. 4) Contrary to the normal human adult studied by the same method, which twists uniformly counterclockwise, 31 of 33 single ventricles, regardless of ventricular morphology or surgical subgroup, twisted counterclockwise in one region, clockwise in another, and met at a "transition zone" of no twist, which had the highest strains of all regions. 5) Radial contraction was greatest in the superior walls and least at the inferior walls of single-ventricle patients regardless of morphology. In the Fontan group, the inferior walls moved paradoxically. In conclusion, markedly different strain characteristics are noted at each stage of Fontan reconstruction, across various wall regions, and between ventricular morphological groups. Differences in regional wall motion were demonstrated in functional single ventricles throughout Fontan reconstruction regardless of morphology, and differences in associated strains and radial contraction were noted across various wall regions and surgical subgroups. These may play an important role in the energetics of the heart and the long-term viability of the single ventricle. AD - Department of Radiology, Hospital of the University of Pennsylvania, Philadelphia 19104, USA. AN - 7573511 AU - Fogel, M. A. AU - Gupta, K. B. AU - Weinberg, P. M. AU - Hoffman, E. A. DA - Sep DB - PubMed DO - 10.1152/ajpheart.1995.269.3.H1132 DP - NLM ET - 1995/09/01 IS - 3 Pt 2 KW - Child Child, Preschool *Fontan Procedure Heart/*physiopathology Humans Image Processing, Computer-Assisted Infant Infant, Newborn Magnetic Resonance Imaging/*methods Motion Myocardium/pathology Prospective Studies Stress, Mechanical Ventricular Function, Left Ventricular Function, Right LA - eng N1 - Fogel, M A Gupta, K B Weinberg, P M Hoffman, E A R01-HL-29886/HL/NHLBI NIH HHS/United States Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S. United States Am J Physiol. 1995 Sep;269(3 Pt 2):H1132-52. doi: 10.1152/ajpheart.1995.269.3.H1132. PY - 1995 SN - 0002-9513 (Print) 0002-9513 SP - H1132-52 ST - Regional wall motion and strain analysis across stages of Fontan reconstruction by magnetic resonance tagging T2 - Am J Physiol TI - Regional wall motion and strain analysis across stages of Fontan reconstruction by magnetic resonance tagging VL - 269 ID - 415 ER - TY - JOUR AB - Objective The aim of the study is to determine the number and indications of unplanned hospital readmissions and emergency room visits for children with hypoplastic left heart syndrome (HLHS) or transposition of great arteries (TGA). Design This retrospective chart review involved children with HLHS or TGA who underwent neonatal surgical repair in a single Children's Hospital. Statistical analysis (SPSS 22.0) included chi-square test, t-test, and binary logistic regression. Results Our study cohort (n=117) comprised 28 children with HLHS and 89 with TGA who were followed for a mean (standard deviation, SD) duration of 4.7 (3.2) years. Unplanned readmissions (n=153) occurred in 46 (39.3%) children, a mean (SD) number of 1.3 (2.8) for a median (interquartile range, IQR) of 8 (2-28) days per patient. Indications were respiratory (57.5%), gastrointestinal (18.3%), and cardiac (12.4%) in nature. On binary regression, with birth weight, race, and gestational age as covariates, among children with HLHS, increasing gestational age tended to be associated with a lower risk of readmissions (odds ratio [OR] 0.138; 95% confidence interval [CI]: 0.018-1.031, p=0.054]. The mean (SD) number of emergency room visits (n=261) was 3.2 (9.7) in 42 (35.9%) infants for minor illnesses (64%) or gastrointestinal (13.8%) problems. Conclusion The majority of children with HLHS and TGA have unplanned readmissions. Home-care surveillance programs may reduce health-care utilization in this population. AD - Division of Pediatric Cardiology, Children's Hospital of Michigan, 3901 Beaubien Blvd, Detroit, MI 48201, United States AU - Soliman, A. AU - Aggarwal, S. AU - Natarajan, G. DB - Scopus DO - 10.1055/s-0035-1549215 IS - 12 KW - hypoplastic left heart syndrome rehospitalization transposition of great arteries M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2015 SP - 1133-1138 ST - Rehospitalization Following Neonatal Cardiac Surgery: Risk Factors and Outcomes T2 - American Journal of Perinatology TI - Rehospitalization Following Neonatal Cardiac Surgery: Risk Factors and Outcomes UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84943398090&doi=10.1055%2fs-0035-1549215&partnerID=40&md5=d090133181338dcaeb5d9694a5bc2d9b VL - 32 ID - 1946 ER - TY - JOUR AB - Milrinone reduces the risk of low cardiac output syndrome for some pediatric patients after congenital heart surgery. Data from adults undergoing cardiac surgery suggest an association between milrinone and an increased risk of postoperative arrhythmias. We tested the hypothesis that milrinone is an independent risk factor for tachyarrhythmias after congenital heart surgery. Subjects undergoing congenital heart surgery at our institution were consecutively enrolled for 38 months, through September 2010. The data were prospectively collected, including a review of full-disclosure telemetry and the medical records. Within 38 months, 603 enrolled subjects underwent 724 operative procedures. The median age was 5.5 months (range 0.0 to 426), the median weight was 6.0 kg (range 0.7 to 108), and the cohort was 45% female. The overall arrhythmia incidence was 50%, most commonly monomorphic ventricular tachycardia (n = 85, 12%), junctional ectopic tachycardia (n = 69, 10%), accelerated junctional rhythm (n = 58, 8%), and atrial tachyarrhythmias (including atrial fibrillation, atrial flutter, and ectopic or chaotic atrial tachycardia, n = 58, 8%). Multivariate logistic regression analysis demonstrated that independent of age <1 month, the use of cardiopulmonary bypass, the duration of cardiopulmonary bypass, Risk Adjusted classification for Congenital Heart Surgery, version 1, score >3, and the use of epinephrine or dopamine, milrinone use on admission to the cardiac intensive care unit remained independently associated with an increase in the odds of postoperative tachyarrhythmia resulting in an intervention (odds ratio 2.8, 95% confidence interval 1.3 to 6.0, p = 0.007). In conclusion, milrinone use is an independent risk factor for clinically significant tachyarrhythmias in the early postoperative period after congenital heart surgery. © 2011 Elsevier Inc. All rights reserved. AD - A.H. Smith, Department of Pediatrics, Monroe Carell Jr. Children's Hospital at Vanderbilt, Vanderbilt University, Nashville, TN, United States AU - Smith, A. H. AU - Owen, J. AU - Borgman, K. Y. AU - Fish, F. A. AU - Kannankeril, P. J. DB - Embase Medline DO - 10.1016/j.amjcard.2011.07.023 IS - 11 KW - adenosine epinephrine amiodarone dopamine milrinone aortic coarctation article cardiopulmonary bypass cardiovascular risk cardioversion child clinical classification cohort analysis congenital heart disease continuous infusion controlled study disease association Fallot tetralogy female forward heart failure heart arrhythmia heart atrium arrhythmia atrial fibrillation heart atrium flutter heart atrium septum defect heart surgery heart ventricle septum defect human hypoplastic left heart syndrome incidence infant intensive care unit loading drug dose major clinical study male monomorphic ventricular tachycardia postoperative period preschool child priority journal prospective study risk assessment risk reduction scoring system supraventricular tachycardia tachycardia telemetry treatment duration LA - English M3 - Article N1 - L51603336 2011-09-07 2011-11-22 PY - 2011 SN - 0002-9149 1879-1913 SP - 1620-1624 ST - Relation of milrinone after surgery for congenital heart disease to significant postoperative tachyarrhythmias T2 - American Journal of Cardiology TI - Relation of milrinone after surgery for congenital heart disease to significant postoperative tachyarrhythmias UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51603336 http://dx.doi.org/10.1016/j.amjcard.2011.07.023 VL - 108 ID - 1082 ER - TY - JOUR AB - BACKGROUND: Patients with single ventricle (SV) may often undergo aortic reconstruction that creates a stiff large vessel, increasing afterload and affecting exercise performance. The objective of this study was to determine the relationship of pulse wave velocity (PWV) and distensibility in reconstructed and normal aortic arches after Fontan with exercise variables. METHODS: PWV and distensibility of the descending aorta at the level of the diaphragm (DAo) were calculated with real-time exercise cardiac magnetic resonance in 48 patients with SV after Fontan (18 after aortic reconstruction; 30 without aortic reconstruction) and compared with metabolic exercise stress test variables. RESULTS: PWV was greater in the reconstructed group than in the non-reconstructed group (median 4.4 m/s [range: 2.3 to 9.8 m/s] versus 3.6 [range: 2.6 to 6.3 m/s], respectively, p = 0.003). Statistically significant inverse correlations were found between PWV and end-diastolic, end-systolic, and stroke volumes at rest and at exercise in the reconstructed group. In addition, inverse correlations also existed in the reconstructed group between distensibility of the DAo and the exercise variables such as peak oxygen pulse (R = 0.56, p = 0.02), peak oxygen consumption (R = 0.63, p = 0.008), oxygen consumption at ventilatory anaerobic threshold (R = 0.48, p = 0.04), and peak work (R = 0.54, p = 0.02). Similar correlations were not seen in patients with non-reconstructed aortas. CONCLUSIONS: Patients with SV with reconstructed aortas have increased aortic stiffness, increasing afterload on the ventricle. Native DAo stiffness distal to the reconstruction is inversely correlated with exercise performance, presumably to decrease impedance mismatch to maintain homogeneity of the aortic wall. This information suggests a possible mechanism for decreased exercise performance in patients with SV with aortic reconstructions. AD - Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania. Electronic address: bikod@email.chop.edu. Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania; Division of Cardiothoracic Surgery, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania; Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. Cardiovascular Fluid Mechanics Laboratory, Wallace H. Coulter Department of Biomedical Engineering, Georgia Institute of Technology, Atlanta, Georgia. AN - 30959013 AU - Biko, D. M. AU - Gaynor, J. W. AU - Partington, S. L. AU - Harris, M. A. AU - Whitehead, K. K. AU - Trusty, P. AU - Yoganathan, A. P. AU - Fogel, M. A. DA - Aug DB - PubMed DO - 10.1016/j.athoracsur.2019.03.019 DP - NLM ET - 2019/04/09 IS - 2 KW - Adolescent Adult Aorta, Thoracic/diagnostic imaging/*physiopathology/surgery Blood Flow Velocity/*physiology Cardiac Surgical Procedures/methods Cardiac Volume/*physiology Child Exercise/*physiology Exercise Test Female Follow-Up Studies Heart Defects, Congenital/diagnosis/*physiopathology/surgery Heart Ventricles/abnormalities/diagnostic imaging/*physiopathology Humans Magnetic Resonance Imaging, Cine Male Prospective Studies Reconstructive Surgical Procedures/methods Vascular Stiffness/*physiology Young Adult LA - eng N1 - 1552-6259 Biko, David M Gaynor, J William Partington, Sara L Harris, Matthew A Whitehead, Kevin K Trusty, Phillip Yoganathan, Ajit P Fogel, Mark A Journal Article Research Support, N.I.H., Extramural Netherlands Ann Thorac Surg. 2019 Aug;108(2):574-580. doi: 10.1016/j.athoracsur.2019.03.019. Epub 2019 Apr 5. PY - 2019 SN - 0003-4975 SP - 574-580 ST - Relationship of Aortic Stiffness to Exercise and Ventricular Volumes in Single Ventricles T2 - Ann Thorac Surg TI - Relationship of Aortic Stiffness to Exercise and Ventricular Volumes in Single Ventricles VL - 108 ID - 506 ER - TY - JOUR AB - BACKGROUND: After the Fontan procedure, patients are at risk for suboptimal health status related to their complex healthcare experience, physiological limitations, medical complications, and guarded long-term prognosis. METHODS AND RESULTS: In the Pediatric Heart Network cross-sectional study of Fontan survivors 6 to 18 years of age, parents completed the Child Health Questionnaire, and scores were related in multivariable analysis to patient and medical characteristics obtained from medical record review. For 537 patients (mean age at study, 11.9 years; 60% male) with a median age at Fontan of 2.8 years (range, 0.7 to 14.6 years), parent-reported patient morbidities included deficits in vision in 33%, speech in 27%, and hearing in 7%, as well as problems with attention in 46%, learning in 43%, development in 24%, behavior in 23%, anxiety in 17%, and depression in 8%. Child Health Questionnaire summary scores were significantly lower than the US population sample for Physical Functioning (mean Z score, -0.47+/-1.19; P<0.001) and Psychosocial Functioning (-0.28+/-1.08; P<0.001). Parent-reported medical conditions and long-term and current medical problems explained the greatest amount of variation in the Physical Functioning scores. Parent-reported patient conditions, including behavior, learning, anxiety, and attention problems and depression, explained the greatest amount of variation in the Psychosocial Functioning scores. Lower family income had a negative impact on both Physical and Psychosocial Functioning. CONCLUSIONS: There are deficits in health status in children and adolescents after the Fontan procedure. Strategies to address this problem might emphasize coordinated and effective prevention, detection, and management of noncardiac and psychosocial conditions, as well as specific targeting of patients from low-income households. AD - University of Toronto, Hospital for Sick Children, Toronto, Canada. brian.mccrindle@sickkids.ca AN - 16490823 AU - McCrindle, B. W. AU - Williams, R. V. AU - Mitchell, P. D. AU - Hsu, D. T. AU - Paridon, S. M. AU - Atz, A. M. AU - Li, J. S. AU - Newburger, J. W. DA - Feb 28 DB - PubMed DO - 10.1161/circulationaha.105.576660 DP - NLM ET - 2006/02/24 IS - 8 KW - Adolescent Child Child, Preschool Cross-Sectional Studies Female *Fontan Procedure *Health Status Heart Defects, Congenital/complications/epidemiology/surgery Humans Infant Male Morbidity Quality of Life Surveys and Questionnaires LA - eng N1 - 1524-4539 McCrindle, Brian W Williams, Richard V Mitchell, Paul D Hsu, Daphne T Paridon, Stephen M Atz, Andrew M Li, Jennifer S Newburger, Jane W Pediatric Heart Network Investigators U01 HL068269/HL/NHLBI NIH HHS/United States U01 HL068270/HL/NHLBI NIH HHS/United States U01 HL068279/HL/NHLBI NIH HHS/United States U01 HL068281/HL/NHLBI NIH HHS/United States U01 HL068285/HL/NHLBI NIH HHS/United States U01 HL068288/HL/NHLBI NIH HHS/United States U01 HL068290/HL/NHLBI NIH HHS/United States U01 HL068292/HL/NHLBI NIH HHS/United States Journal Article Research Support, N.I.H., Extramural United States Circulation. 2006 Feb 28;113(8):1123-9. doi: 10.1161/CIRCULATIONAHA.105.576660. Epub 2006 Feb 20. PY - 2006 SN - 0009-7322 SP - 1123-9 ST - Relationship of patient and medical characteristics to health status in children and adolescents after the Fontan procedure T2 - Circulation TI - Relationship of patient and medical characteristics to health status in children and adolescents after the Fontan procedure VL - 113 ID - 222 ER - TY - JOUR AB - Despite hypothesized concerns about deterioration beginning in adolescence, longitudinal data and associated factors regarding standardized assessment of physical functioning are not available for Fontan patients. Parents who participated in the Fontan Cross-Sectional Study completed the Child Health Questionnaire at 2 time points for 245 subjects ages 6-18 years. Associations between change in Physical Functioning Summary Score and baseline patient, medical, and laboratory characteristics (mean age 9.5 ± 1.7 years) and follow-up patient and medical characteristics (mean age 16.2 ± 1.6 years) were determined by regression analyses. During a mean of 6.7 ± 0.4 years, a small (not clinically important) but statistically significant decrease in score from 46.2 ± 11.7 to 44.5 ± 12.1 (p < 0.03) was noted. Subjects with higher baseline scores had a greater decrease in score (r = -0.48; p < 0.001). A multivariable model of patient and medical characteristics (R(2) = 0.11) showed that a greater decrease in score was significantly associated with interim development of asthma (n = 13; parameter estimate [PE] -6.6; p < 0.05) or other chronic respiratory, lung, or breathing problems (n = 13; PE -12.5; p < 0.001) and the presence of protein-losing enteropathy at any time (n = 12; PE -9.4; p = 0.006). Change in score was not significantly associated with baseline laboratory measures of exercise capacity and ventricular characteristics and function. Therefore, although physical functioning may be stable during adolescence for many Fontan patients, deterioration occurs in some in association with respiratory conditions and protein-losing enteropathy. Further longitudinal study is necessary to better understand the relationship between clinical morbidities and functional health status as these patients transition into adulthood. AD - The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, ON, M5G 1X8, Canada, brian.mccrindle@sickkids.ca. AN - 24264999 AU - McCrindle, B. W. AU - Zak, V. AU - Breitbart, R. E. AU - Mahony, L. AU - Shrader, P. AU - Lai, W. W. AU - Burns, K. M. AU - Colan, S. D. AU - Williams, R. V. AU - Goldberg, D. AU - Hill, K. D. AU - Khaikin, S. AU - Atz, A. M. C2 - PMC3959245 C6 - NIHMS543554 DA - Apr DB - PubMed DO - 10.1007/s00246-013-0831-0 DP - NLM ET - 2013/11/23 IS - 4 KW - Adolescent Child Child, Preschool Cross-Sectional Studies Echocardiography/*methods Exercise Test Female Follow-Up Studies Fontan Procedure/*methods *Health Status Heart Defects, Congenital/diagnosis/epidemiology/*surgery Heart Ventricles/*abnormalities Humans Magnetic Resonance Imaging, Cine/*methods Male Morbidity/trends Postoperative Period Prognosis *Quality of Life Retrospective Studies Surveys and Questionnaires United States/epidemiology Young Adult LA - eng N1 - 1432-1971 McCrindle, Brian W Zak, Victor Breitbart, Roger E Mahony, Lynn Shrader, Peter Lai, Wyman W Burns, Kristin M Colan, Steven D Williams, Richard V Goldberg, David Hill, Kevin D Khaikin, Svetlana Atz, Andrew M Pediatric Heart Network Investigators HL068288/HL/NHLBI NIH HHS/United States HL068285/HL/NHLBI NIH HHS/United States U10 HL109781/HL/NHLBI NIH HHS/United States U01 HL068269/HL/NHLBI NIH HHS/United States U01 HL068279/HL/NHLBI NIH HHS/United States U10 HL109743/HL/NHLBI NIH HHS/United States U01 HL068290/HL/NHLBI NIH HHS/United States HL068279/HL/NHLBI NIH HHS/United States U01 HL068288/HL/NHLBI NIH HHS/United States U10 HL068270/HL/NHLBI NIH HHS/United States U01 HL068281/HL/NHLBI NIH HHS/United States U01 HL068270/HL/NHLBI NIH HHS/United States U10 HL109777/HL/NHLBI NIH HHS/United States HL068281/HL/NHLBI NIH HHS/United States U01 HL068292/HL/NHLBI NIH HHS/United States HL068269/HL/NHLBI NIH HHS/United States HL068270/HL/NHLBI NIH HHS/United States U10 HL109778/HL/NHLBI NIH HHS/United States HL068290/HL/NHLBI NIH HHS/United States U01 HL068285/HL/NHLBI NIH HHS/United States HL068292/HL/NHLBI NIH HHS/United States Comparative Study Journal Article Multicenter Study Research Support, N.I.H., Extramural Pediatr Cardiol. 2014 Apr;35(4):632-40. doi: 10.1007/s00246-013-0831-0. Epub 2013 Nov 22. PY - 2014 SN - 0172-0643 (Print) 0172-0643 SP - 632-40 ST - The relationship of patient medical and laboratory characteristics to changes in functional health status in children and adolescents after the Fontan procedure T2 - Pediatr Cardiol TI - The relationship of patient medical and laboratory characteristics to changes in functional health status in children and adolescents after the Fontan procedure VL - 35 ID - 242 ER - TY - JOUR AB - OBJECTIVE: Two strategies for surgical management are used for infants with hypoplastic left heart syndrome (HLHS), primary heart transplantation and the Norwood procedure. We sought to determine how these 2 surgical approaches influence neurodevelopmental outcomes at school age. METHODS: A multicenter, cross-sectional study of neurodevelopmental outcomes among school-aged children (>8 years of age) with HLHS was undertaken between July 2003 and September 2004. Four centers enrolled 48 subjects, of whom 47 completed neuropsychologic testing. Twenty-six subjects (55%) had undergone the Norwood procedure and 21 (45%) had undergone transplantation, with an intention-to-treat analysis. The mean age at testing was 12.4 +/- 2.5 years. Evaluations included the Wechsler Abbreviated Scale of Intelligence, Clinical Evaluation of Language Fundamentals, Wechsler Individual Achievement Test, and Beery-Buktenica Developmental Test of Visual-Motor Integration. RESULTS: The mean neurocognitive test results were significantly below population normative values. The mean full-scale IQ for the entire cohort was 86 +/- 14. In a multivariate model, there was no association of surgical strategy with any measure of developmental outcome. A longer hospital stay, however, was associated significantly with lower verbal, performance, and full-scale IQ scores. Aortic valve atresia was associated with lower math achievement test scores. CONCLUSIONS: Neurodevelopmental deficits are prevalent among school-aged children with HLHS, regardless of surgical approach. Complications that result in prolonged hospitalization at the time of the initial operation are associated with neurodevelopmental status at school age. AD - Children's Healthcare of Atlanta, Atlanta, GA 30322-1062, USA. mahlew@kidsheart.com AN - 16361221 AU - Mahle, W. T. AU - Visconti, K. J. AU - Freier, M. C. AU - Kanne, S. M. AU - Hamilton, W. G. AU - Sharkey, A. M. AU - Chinnock, R. E. AU - Jenkins, K. J. AU - Isquith, P. K. AU - Burns, T. G. AU - Jenkins, P. C. DA - Jan DB - PubMed DO - 10.1542/peds.2005-0575 DP - NLM ET - 2005/12/20 IS - 1 KW - Adolescent Cardiac Surgical Procedures/adverse effects Child Developmental Disabilities/*diagnosis/etiology Female Heart Transplantation/adverse effects Humans Hypoplastic Left Heart Syndrome/*surgery Male *Neuropsychological Tests Palliative Care Psychometrics LA - eng N1 - 1098-4275 Mahle, William T Visconti, Karen J Freier, M Catherin Kanne, Stephen M Hamilton, William G Sharkey, Angela M Chinnock, Richard E Jenkins, Kathy J Isquith, Peter K Burns, Thomas G Jenkins, Pamela C Comparative Study Journal Article Multicenter Study Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S. United States Pediatrics. 2006 Jan;117(1):e90-7. doi: 10.1542/peds.2005-0575. Epub 2005 Dec 15. PY - 2006 SN - 0031-4005 SP - e90-7 ST - Relationship of surgical approach to neurodevelopmental outcomes in hypoplastic left heart syndrome T2 - Pediatrics TI - Relationship of surgical approach to neurodevelopmental outcomes in hypoplastic left heart syndrome VL - 117 ID - 212 ER - TY - JOUR AB - The Fontan operation is the anticipated palliative strategy for children born with single-ventricle type of congenital heart disease. As a result of important circulatory limitations, a series of end-organ complications are now increasingly recognized. Elevated central venous pressure and impaired cardiac output are the hallmarks of cavo-pulmonary flow, which result in a cascade of pathophysiological consequences. The Fontan circulation likely impacts all organ systems in an indolent and relentless manner, with progressive decline in functionality likely to occur in many. Liver fibrosis, altered bone density, decreased muscle mass, renal dysfunction, lymphatic insufficiency, and a host of other conditions are present. Standardized screening and evaluation of survivors as they grow through childhood and beyond is indicated and can be facilitated through dedicated multidisciplinary clinical programs. Invasive assessment at specific milestones can provide important actionable information to optimize individual status. More detailed characterization and understanding of these end-organ complications is necessary to contribute to the goal of achieving a normal duration and quality of life for these unique individuals. AD - Single Ventricle Survivorship Program, The Children's Hospital of Philadelphia, Philadelphia, PA. Electronic address: Rychik@email.chop.edu. AN - 27060041 AU - Rychik, J. DB - PubMed DO - 10.1053/j.pcsu.2015.11.006 DP - NLM ET - 2016/04/10 IS - 1 KW - *Fontan Procedure Heart Defects, Congenital/complications/*physiopathology/*surgery Heart Ventricles/*abnormalities Humans Liver Diseases/etiology/pathology Protein-Losing Enteropathies/diagnosis/etiology Venous Pressure/physiology LA - eng N1 - Rychik, Jack Journal Article Review United States Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2016;19(1):37-43. doi: 10.1053/j.pcsu.2015.11.006. PY - 2016 SN - 1092-9126 (Print) 1092-9126 SP - 37-43 ST - The Relentless Effects of the Fontan Paradox T2 - Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu TI - The Relentless Effects of the Fontan Paradox VL - 19 ID - 52 ER - TY - JOUR AB - The care of patients who refuse homologous transfusions has challenged cardiac surgery teams to refine blood conservation techniques and question standard transfusion practices. We cared for a newborn child with hypoplastic left heart syndrome (HLHS) whose parents refused to give consent to care for the child that involved the transfusion of homologous blood. A Norwood Stage I procedure was planned with the understanding that transfusions would be avoided, if possible. A court order was obtained that specified the conditions under which the attending physicians would transfuse the newborn. The birth weight of the patient was 4.25 kg. A low prime cardiopulmonary bypass (CPB) circuit and aggressive blood conservation techniques that included modified ultrafiltration (MUF) allowed the completion of the repair and CPB portion of the operation without the use of blood. The lowest hematocrit during CPB was 20%. After an unsuccessful attempt to separate from CPB, blood was transfused. Recovery was consistent for HLHS patients following Norwood Stage I. However, at 1 month postoperatively, the patient did require a shunt reduction for pulmonary overcirculation. Norwood Stage II repair was completed at age 4 months without donor blood. The key to a successful outcome is a well-thought-out plan by the surgeon, anesthesiologist and perfusionist. This plan should include careful monitoring of the patient's oxygenation and cardiovascular status. AD - R.C. Groom, Cardiac Surgery Department, Maine Medical Center, Perfusion Services, 22 Bramhall Street, Portland, ME 04102, United States AU - Forest, R. J. AU - Groom, R. C. AU - Quinn, R. AU - Donnelly, J. AU - Clark, C. DB - Embase Medline DO - 10.1191/0267659102pf564oa IS - 3 KW - epinephrine dopamine milrinone protamine sulfate adult article birth weight blood autotransfusion cardiopulmonary bypass case report clinical practice female health care planning hematocrit human hypoplastic left heart syndrome informed consent lung circulation oxygenation oxygenator patient care patient compliance patient monitoring postoperative period priority journal religion standardization surgical technique treatment outcome ultrafiltration D901 LA - English M3 - Article N1 - L34467130 2002-05-15 PY - 2002 SN - 0267-6591 SP - 221-225 ST - Repair of hypoplastic left heart syndrome of a 4.25-kg Jehovah's witness T2 - Perfusion TI - Repair of hypoplastic left heart syndrome of a 4.25-kg Jehovah's witness UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L34467130 http://dx.doi.org/10.1191/0267659102pf564oa VL - 17 ID - 1314 ER - TY - JOUR AB - OBJECTIVE: Early failure of the Fontan-type circulation is a potentially fatal complication. We review our experience with cardiac transplantation in children presenting with end-stage heart failure in this scenario. METHODS: We performed a retrospective review. Between 1985 and 2003, 6 children aged less than 16 years were referred for cardiac transplantation. The indication for cardiac transplantation was end-stage cardiac failure early after the completion of the Fontan-type operation. RESULTS: All 6 patients listed for transplantation underwent cardiac transplantation; the median interval to transplantation from the operation was 36 days (range, 6-180 days). Four patients had undergone the Fontan procedure, and 2 had one-and-a-half-ventricle repair. All 6 patients were ventilated and inotrope dependent, with varying degrees of multiorgan dysfunction. One patient was bridged to transplantation with extracorporeal membrane oxygenation. The median age at transplantation was 7.1 years (range, 3-12.5 years), and weight was 18.9 kg (range, 11-35 kg). One patient died on the operating table (graft failure and hemorrhage). In 5 survivors the median intensive care unit stay was 10 days (range, 8-61 days). On follow-up of 6 to 81 months, there have been no subsequent deaths, and the quality of life in survivors is good. CONCLUSION: Rescue cardiac transplantation in the setting of an early failure of the Fontan-type circulation and end-stage cardiac failure is an effective treatment option and can be performed with acceptable early mortality and encouraging short-term to medium-term results. AD - Department of Paediatric Cardiology, Freeman Hospital, Newcastle upon Tyne, United Kingdom. milind.chaudhari@nuth.northy.nhs.uk AN - 15678054 AU - Chaudhari, M. AU - Sturman, J. AU - O'Sullivan, J. AU - Smith, J. AU - Wrightson, N. AU - Parry, G. AU - Bolton, D. AU - Haynes, S. AU - Hamilton, L. AU - Hasan, A. DA - Feb DB - PubMed DO - 10.1016/j.jtcvs.2004.06.030 DP - NLM ET - 2005/01/29 IS - 2 KW - Coronary Circulation/physiology Female Follow-Up Studies *Fontan Procedure Heart Failure/congenital/*physiopathology/*surgery *Heart Transplantation Humans Infant Infant Welfare Infant, Newborn Male Postoperative Complications/etiology/mortality/surgery Quality of Life Reoperation Retrospective Studies Time Factors Treatment Outcome LA - eng N1 - Chaudhari, Milind Sturman, Jon O'Sullivan, John Smith, Jon Wrightson, Neil Parry, Gareth Bolton, David Haynes, Simon Hamilton, Leslie Hasan, Asif Journal Article United States J Thorac Cardiovasc Surg. 2005 Feb;129(2):416-22. doi: 10.1016/j.jtcvs.2004.06.030. PY - 2005 SN - 0022-5223 (Print) 0022-5223 SP - 416-22 ST - Rescue cardiac transplantation for early failure of the Fontan-type circulation in children T2 - J Thorac Cardiovasc Surg TI - Rescue cardiac transplantation for early failure of the Fontan-type circulation in children VL - 129 ID - 425 ER - TY - JOUR AD - Department of Anesthesiology and Pain Medicine, Children's Hospital, Regional Medical Center, Seattle, WA, United States Division of Pediatric Cardiology, Department of Pediatrics, Children's Hospital and Regional Medical Center, Seattle, WA, United States Section of Pediatric Cardiac Surgery, Department of Surgery, Children's Hospital and Regional Medical Center, Seattle, WA, United States AU - Pieters, B. AU - Johnston, T. A. AU - Jones, T. K. AU - Cohen, G. AU - Jonmarker, C. DB - Scopus DO - 10.1053/j.jvca.2006.12.009 IS - 6 KW - anesthesia children congenital heart disease hypoplastic left-heart syndrome infants intensive care surgery M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2007 SP - 880-882 ST - Resistant Hypoxemia in an Infant With a Right Ventricle-to-Pulmonary Artery (Sano) Shunt T2 - Journal of Cardiothoracic and Vascular Anesthesia TI - Resistant Hypoxemia in an Infant With a Right Ventricle-to-Pulmonary Artery (Sano) Shunt UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-36749043711&doi=10.1053%2fj.jvca.2006.12.009&partnerID=40&md5=60e82114a6520716accd1756ad5de1fc VL - 21 ID - 2203 ER - TY - JOUR AB - Fontan palliation allows patients with "single ventricle" circulation to reach adulthood with an acceptable quality of life, although exercise tolerance is significantly reduced. To assess whether controlled respiratory training (CRT) increases cardiorespiratory performance. 16 Adolescent Fontan patients (age 17. 5 ± 3.8 years) were enrolled. Patients were divided into CRT group (n = 10) and control group (C group, n = 6). Maximal cardiopulmonary test (CPT) was repeated at the end of CRT in the CRT group and after an average time of 3 months in the C group. In the CRT group a CPT endurance was also performed before and after CRT. In the CRT group there was a significant improvement in cardiovascular and respiratory response to exercise after CRT. Actually, after accounting for baseline values, the CRT group had decreased breathing respiratory reserve (- 15, 95% CI -22.3 to - 8.0, p = 0.001) and increased RR peak (+ 4.8, 95% CI 0.7-8.9, p = 0.03), VE peak (+ 13.7, 95% CI 5.6-21.7, p = 0.004), VO(2) of predicted (+ 8.5, 95% CI 0.1-17.0, p = 0.05), VO(2) peak (+ 4.3, 95% CI 0.3 to 8.2, p = 0.04), and VO(2) workslope (+ 1.7, 95% CI 0.3-3.1, p = 0.02) as compared to the control group. Moreover, exercise endurance time increased from 8.45 to 17.7 min (p = 0.01). CRT improves cardiorespiratory performance in post-Fontan patients leading to a better aerobic capacity. AD - Clinical Physiology Institute, CNR, Via Moruzzi 1, 56124, Pisa, Italy. Fondazione G. Monasterio, Regione Toscana, Pisa, Italy. Clinical Physiology Institute, CNR, Via Moruzzi 1, 56124, Pisa, Italy. pingi@ifc.cnr.it. National Institute of Diabetes and Digestive and Kidney Disease, National Institutes of Health, Phoenix, AZ, USA. Pediatric Cardiology, Brotzu Hospital, Cagliari, Italy. Institute of Life Sciences, Scuola Superiore Sant'Anna, Pisa, Italy. AN - 29349618 AU - Ait Ali, L. AU - Pingitore, A. AU - Piaggi, P. AU - Brucini, F. AU - Passera, M. AU - Marotta, M. AU - Cadoni, A. AU - Passino, C. AU - Catapano, G. AU - Festa, P. DA - Apr DB - PubMed DO - 10.1007/s00246-018-1808-9 DP - NLM ET - 2018/01/20 IS - 4 KW - Adolescent Cardiorespiratory Fitness/*physiology Exercise/physiology Exercise Test/methods Exercise Tolerance/*physiology Female Fontan Procedure/*rehabilitation Heart Defects, Congenital/surgery Humans Male Oxygen Consumption/physiology Pilot Projects Quality of Life Respiratory Therapy/*methods Spirometry/methods Treatment Outcome Young Adult Congenital heart disease Controlled respiratory training Ergospirometry Fontan circulation LA - eng N1 - 1432-1971 Ait Ali, Lamia Pingitore, Alessandro Orcid: 0000-0002-4049-184x Piaggi, Paolo Brucini, Fabio Passera, Mirko Marotta, Marco Cadoni, Alessandra Passino, Claudio Catapano, Giosuè Festa, Pierluigi Journal Article United States Pediatr Cardiol. 2018 Apr;39(4):695-704. doi: 10.1007/s00246-018-1808-9. Epub 2018 Jan 19. PY - 2018 SN - 0172-0643 SP - 695-704 ST - Respiratory Training Late After Fontan Intervention: Impact on Cardiorespiratory Performance T2 - Pediatr Cardiol TI - Respiratory Training Late After Fontan Intervention: Impact on Cardiorespiratory Performance VL - 39 ID - 179 ER - TY - JOUR AB - In this study, three patients presenting with early or late postoperative Fontan complications were identified to suffer from restriction of the native atrial septum. This caused significant obstruction to pulmonary venous return and elevated systemic venous pressure. Dobutamine stress testing was used in one patient to identify this lesion. Transcatheter stenting was performed in the other two patients. Patients improved after relief of the obstruction. AD - The Heart Unit,Birmingham Children's Hospital,Birmingham,United Kingdom. AN - 26175163 AU - Penford, G. AU - Quandt, D. AU - Stumper, O. DA - Mar DB - PubMed DO - 10.1017/s1047951115001262 DP - NLM ET - 2015/07/16 IS - 3 KW - Atrial Septum/*physiopathology/surgery Child Child, Preschool Exercise Test Female Fontan Procedure/*adverse effects Humans Hypoplastic Left Heart Syndrome/etiology/*surgery Male Postoperative Complications/*surgery Protein-Losing Enteropathies/etiology/therapy Pulmonary Veins/physiopathology/surgery Reoperation Treatment Outcome Venous Pressure Fontan procedure hypoplastic left heart syndrome protein-losing enteropathy stent LA - eng N1 - 1467-1107 Penford, Gemma Quandt, Daniel Stumper, Oliver Case Reports Journal Article Research Support, Non-U.S. Gov't England Cardiol Young. 2016 Mar;26(3):574-8. doi: 10.1017/S1047951115001262. Epub 2015 Jul 15. PY - 2016 SN - 1047-9511 SP - 574-8 ST - Restrictive atrial septum after the Fontan procedure T2 - Cardiol Young TI - Restrictive atrial septum after the Fontan procedure VL - 26 ID - 441 ER - TY - JOUR AB - OBJECTIVE: From October 1989 to June 1998, 60 patients have undergone the Norwood procedure (stage I) at our institution. The results of the staged surgical reconstruction and risk factors were analysed. Typical hypoplastic left heart syndrome (HLHS) and complex lesions with aortic hypoplasia were compared with each other. PATIENTS: Typical HLHS: N = 48, median age 15 days (5-190 days), median weight 3.6 kg (2.6-5.3 kg). Complex lesions (dominant left ventricle): N = 12, median age 59 days (10-884 days), median weight 3.4 kg (2.4-12 kg). RESULTS: Typical HLHS: The stage-I hospital survival was 73% (35/48). It improved from 60% (95% confidence interval: 49-71%) during 1989-1994 to 91% (95% CI: 81-100%) during 1997-1998. Seven patients were lost late. The right ventricular end diastolic diameter (P = 0.015), shortening fraction (P = 0.027), and the presence of an obstructed pulmonary venous return (P = 0.0032) were significant risk factors. 23 children underwent stage-II operation with four (17%) deaths. All survivors experienced an improvement of their statomotoric development. Stage-III operation was performed in 13 patients with no hospital death. Follow up after stage-III procedure was 7 months to 7 years. 4 year actuarial survival, including hospital mortality and deaths at subsequent stages, improved from 28% (95% CI: 18-38%) during 1989-1994 to 58% (95% CI: 48-68%) during 1994-1997. No patient had signs of myocardial insufficiency. Complex lesions: Stage-I hospital survival was 83% (10/12) with no late death. Stage-II was performed in 8 and stage-III in 6 patients with no death respectively. CONCLUSION: In typical HLHS the results have improved over time. Both size and function of the right ventricle determined significantly stage-I survival. An early operation prevents the natural progression of pulmonary blood flow and may weaken all three risk factors. Patients with complex lesions seemed to have better chances of surviving the early postoperative period. The multistage reconstructions have become a realistic option for patients with HLHS and similar lesions, regardless of the morphologic subtype or diminutiveness of the aorta, and lead to an acceptable quality of life. AD - Department of Thoracic and Cardiovascular Surgery, Heart Center NRW, Ruhr University of Bochum (Klinik für Thorax- und Kardiovaskularchirurgie, Ruhr Universität Bochum), Bad Oeynhausen, Germany. AN - 10485407 AU - Breymann, T. AU - Kirchner, G. AU - Blanz, U. AU - Cherlet, E. AU - Knobl, H. AU - Meyer, H. AU - Körfer, R. AU - Thies, W. R. DA - Aug DB - PubMed DO - 10.1016/s1010-7940(99)00155-4 DP - NLM ET - 1999/09/15 IS - 2 KW - Abnormalities, Multiple/diagnosis/mortality/*surgery Angiography Aorta, Thoracic/*abnormalities/diagnostic imaging Aortic Diseases/congenital/diagnosis/mortality/*surgery Blood Vessel Prosthesis Implantation Cardiac Surgical Procedures/methods/mortality Child, Preschool Echocardiography, Doppler Follow-Up Studies *Heart Bypass, Right Heart Ventricles/abnormalities/diagnostic imaging Hospital Mortality Humans Hypoplastic Left Heart Syndrome/diagnosis/mortality/*surgery Infant Infant, Newborn Pulmonary Artery/abnormalities/diagnostic imaging Pulmonary Atresia/diagnosis/mortality/*surgery Retrospective Studies Risk Factors Survival Rate Treatment Outcome LA - eng N1 - Breymann, T Kirchner, G Blanz, U Cherlet, E Knobl, H Meyer, H Körfer, R Thies, W R Comparative Study Journal Article Germany Eur J Cardiothorac Surg. 1999 Aug;16(2):117-24. doi: 10.1016/s1010-7940(99)00155-4. PY - 1999 SN - 1010-7940 (Print) 1010-7940 SP - 117-24 ST - Results after Norwood procedure and subsequent cavopulmonary anastomoses for typical hypoplastic left heart syndrome and similar complex cardiovascular malformations T2 - Eur J Cardiothorac Surg TI - Results after Norwood procedure and subsequent cavopulmonary anastomoses for typical hypoplastic left heart syndrome and similar complex cardiovascular malformations VL - 16 ID - 442 ER - TY - JOUR AB - OBJECTIVES The purpose of this study was to examine the outcome after the Fontan operation in patients with congenitally corrected transposition of great arteries with ventricular septal defect and pulmonary stenosis (ccTGA-VSD-PS). METHODS Patient- and procedure-related variables were analysed in 23 patients with ccTGA-VSD-PS operated between April 2003 and April 2015. RESULTS The mean age was 14.07 ± 6.38 years (range 4-23, median 11 years), with 82% patients being male (19/23). Dextrocardia was present in 52% (12/23) of patients and left superior vena cava was present in 26% (6/23) of patients. Most patients underwent extracardiac Fontan (n = 18), whereas in 5 patients lateral tunnel Fontan was performed. All patients received polytetrafluoroethylene grafts of size 18-22 mm for extracardiac Fontan. In 8 patients, conduits were fenestrated to reduce the intraconduit pressure. The mean hospital stay was 15.7 ± 11.24 days (5-60, median 14 days). The most common cause for prolonged hospital stay was pleural effusion in 5 patients (21.7%). One 7-year old patient developed conduit thrombosis, intracranial bleed, seizures and died. The mean follow-up was 46.4.4 ± 32.2 months (range 8-142, median 42 months) and was available for 21 patients (91.3%). There was 1 mid-term non-cardiac death after 3 years of operation. Of the total, 85.7% (18/21) patients in follow-up are in NYHA class I, whereas 3 patients are in class II. The actuarial event-free survival rate was 81.8 ± 13.2% at 10 years. CONCLUSIONS In ccTGA-VSD-PS patients with non-routable VSD and in those with difficult options for biventricular repair, the Fontan approach provides satisfactory mid-term palliation. © The Author 2015. AD - Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, 110029, India AU - Talwar, S. AU - Bansal, A. AU - Choudhary, S. K. AU - Kothari, S. S. AU - Juneja, R. AU - Saxena, A. AU - Airan, B. DB - Scopus DO - 10.1093/icvts/ivv316 IS - 2 KW - Anatomical repair Corrected transposition Fontan operation M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2016 SP - 188-193 ST - Results of Fontan operation in patients with congenitally corrected transposition of great arteries T2 - Interactive Cardiovascular and Thoracic Surgery TI - Results of Fontan operation in patients with congenitally corrected transposition of great arteries UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84960392462&doi=10.1093%2ficvts%2fivv316&partnerID=40&md5=6e271570a52d2c80d05121516c2797fd VL - 22 ID - 1923 ER - TY - JOUR AB - Objectives: Patients with unoperated functional single ventricle (FSV) rarely survive into adulthood with good functional status and there are few reports about surgical results of adult patients with FSV. This study retrospectively reviews our experience with surgery in adult patients with FSV. Methods: From January 2008 to September 2017, 65 adult patients with FSV underwent surgery in our hospital. Twenty underwent Blalock-Taussig shunt or bidirectional Glenn procedures in other hospitals prior, and four were lost to follow-up. Finally, 41 patients were included in this study. Results: The early postoperative mortality was 7.3% (3/41). Postoperative systemic arterial oxygen saturation (SpO2, 83.7% ± 4.8%) was significantly higher than preoperative SpO2 (77.9% ± 10.1%, P <.01). The mean follow-up time was 3.9 ± 3.1 years (range 1-11 years). There was only one case of late mortality and massive hemoptysis was the cause of death. Right ventricular morphology and severe atrioventricular valve regurgitation (AVVR) were the risk factors of postoperative death for patients with pulmonary stenosis(PS). There was no death case in patients who received Fontan procedure. In a follow-up, we found the grade of AVVR was reduced and the grade of heart function (New York Heart Association) was improved. Conclusions: Adult patients with FSV can also undergo surgery. The mortality was acceptable and late results were satisfactory. After surgery, oxygen saturations increased, grades of AVVR decreased and the heart functions improved. Right ventricular morphology and severe AVVR were risk factors for patients with PS. AD - J.-M. Chen, Department of Cardiovascular Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, Guangdong, China AU - Yang, J. AU - He, B. C. AU - Chen, J. M. AU - Cen, J. Z. AU - Wen, S. S. AU - Xu, G. AU - Zhuang, J. DB - Embase Medline DO - 10.1111/jocs.14306 IS - 12 KW - adult arterial oxygen saturation article Blalock Taussig shunt clinical article disease severity female follow up Glenn shunt heart function heart right ventricle heart single ventricle heart valve regurgitation hemoptysis human male medical history New York Heart Association class outcome assessment patient satisfaction postoperative complication postoperative period preoperative evaluation pulmonary valve stenosis retrospective study risk factor surgical mortality survival rate young adult LA - English M3 - Article N1 - L2003548502 2019-12-12 2019-12-16 PY - 2019 SN - 1540-8191 0886-0440 SP - 1556-1562 ST - Results of surgery on adults with functional single ventricle without prior cardiac surgery in childhood T2 - Journal of Cardiac Surgery TI - Results of surgery on adults with functional single ventricle without prior cardiac surgery in childhood UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2003548502 http://dx.doi.org/10.1111/jocs.14306 VL - 34 ID - 585 ER - TY - JOUR AB - We report a case where endovascular stenting as part of the treatment of complex cardiovascular disease led to airway compression. Using a novel technique, this was successfully reversed. Simultaneous inflations of two balloons—one in the obstructed bronchus and one in the aorta combined with external compression of the chest—resulted in compression the stent. This report illustrates that by thinking “out of the box” and bearing in mind spatial relationships inside the chest, it is possible to diminish the anterior–posterior diameters of a stent. This intervention proved to be successful with relief of the left bronchial compression and improvement of chronic airway infection. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc. AD - Fetal & Pediatric Cardiology, University Hospitals Leuven, Leuven, Belgium Pediatric Cardiology, University of the Free State, Bloemfontein, South Africa AU - Brown, S. C. AU - Proesmans, M. AU - Gewillig, M. DB - Scopus DO - 10.1002/ccd.26396 IS - 1 KW - angioplasty balloon compression obstruction stent M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2016 SP - 103-106 ST - Reversing left bronchus obstruction by compression of a pulmonary artery stent T2 - Catheterization and Cardiovascular Interventions TI - Reversing left bronchus obstruction by compression of a pulmonary artery stent UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84978900524&doi=10.1002%2fccd.26396&partnerID=40&md5=8078316f804bc611ce8450188a91c433 VL - 88 ID - 1899 ER - TY - JOUR AB - PURPOSE OF REVIEW: We aim to review select literature pertaining to congenital heart disease (CHD)-induced right ventricular (RV) function and failure. RECENT FINDINGS: We review recent findings pertaining to children and adults with repaired tetralogy of Fallot (rTOF), systemic RV and hypoplastic left heart syndrome (HLHS). We emphasize pathophysiological mechanisms contributing to RV dysfunction in these conditions, the risk factors for adverse outcomes and the continuing challenges in treating these patients. We discuss how recent pathology findings, as well as developments in imaging and computer modeling have broadened our understanding of the pathophysiology of these conditions. We further review developments in the molecular and cellular basis of RV failure; and in particular, the RV molecular response to stress in repaired tetralogy of Fallot (rTOF). We highlight some of the genetic complexities in HLHS and how these may influence the long-term outcomes in these patients. SUMMARY: Recent literature has led to new understandings in the pathology, pathophysiology, risk factors for adverse outcomes, molecular and genetic basis for RV dysfunction and failure in CHD. Although these findings provide new therapeutic targets, the treatment of RV failure at this time remains limited. AD - Division of Cardiology, Labatt Family Heart Centre, Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada. Department of Pediatrics (Cardiology), Stanford University, Stanford, California, USA. AN - 31356354 AU - Friedberg, M. K. AU - Reddy, S. DA - Oct DB - PubMed DO - 10.1097/mop.0000000000000804 DP - NLM ET - 2019/07/30 IS - 5 KW - Heart Defects, Congenital/*physiopathology Heart Failure/*physiopathology Humans Ventricular Dysfunction, Right/*physiopathology LA - eng N1 - 1531-698x Friedberg, Mark K Reddy, Sushma Journal Article Review United States Curr Opin Pediatr. 2019 Oct;31(5):604-610. doi: 10.1097/MOP.0000000000000804. PY - 2019 SN - 1040-8703 SP - 604-610 ST - Right ventricular failure in congenital heart disease T2 - Curr Opin Pediatr TI - Right ventricular failure in congenital heart disease VL - 31 ID - 46 ER - TY - JOUR AB - BACKGROUND: Patients with Ebstein malformation (EM) and severe RV dilatation and dysfunction have increased operative risk. Early results with right ventricular unloading utilizing the bidirectional cavopulmonary shunt (BCPS) during repair of EM have been encouraging. We report our experience of the 1.5-ventricle repair strategy for this difficult group of patients. METHODS: Between July 1999 and January 2013, 62 patients with severe EM underwent BCPS at the time of tricuspid repair. Median age was 21.5 years (range, 9 months to 57 years), 51.6% were male, and 72.5% were children. Severe RV dilatation was present in all patients; severe RV dysfunction was present in 72.5% (n=45) and moderate to severe RV dysfunction in 22.5% (n=14). Mean RV systolic pressure was 32.7±0.7 mm Hg and mean PA pressure was 15.6±2.1 mm Hg. Mean preoperative left ventricular ejection fraction (LVEF) was 0.536±0.071; it was less than 40% in 10 patients (16.1%). New York Heart Association class III/IV heart failure was present in 43 patients (69.3%) preoperatively and 20 patients (32.2%) were initially referred for heart transplant evaluation. Prior EM surgery occurred in 35.4% (n=22; 8 prior valve repair, 8 prior valve replacement, Blalock-Taussig shunt in 4, atrial septal defect (ASD) closure in 2). RESULTS: Tricuspid repair was performed in 51.6% (n=32, 5 had re-repair). Bioprosthetic valve replacement was performed in 48.4% (n=30, 8 had rereplacement). The BCPS was a planned procedure in 53 patients (85.5%) because of RV dysfunction; BCPS was added after unsuccessful weaning from bypass in 7 (11.2%), and in the early postoperative period due to hemodynamic instability in 2. Concomitant procedures included ASD closure in 48.3%, maze in 38.7%, and mitral valve repair in 6.4%. Postoperative extracorporeal membrane oxygenation support was needed in 8 patients. Delayed chest closure was performed in 25.8%. Early mortality was 1.6% (n=1). Mean mechanical ventilation time was 69.7 hours. Mean intensive care unit and hospital stays were 5.4±3.5 and 10.7±3.5 days, respectively. Mean follow-up was 3.6±2.6 years (maximum, 12.8 years). Patients (n=10) with low preoperative LVEF (0.362±0.035) improved to 0.517±0.042 postoperatively (p=0.001). There was 1 late death in a patient with cystic fibrosis. Late reintervention was needed in 5 patients (8%). Late follow-up was available in 95% (n=59); all were acyanotic and 88% were in New York Heart Association functional class I/II. CONCLUSIONS: Concomitant BCPS is a useful adjunct in repair of advanced EM with severe RV dilatation and dysfunction. Operation can be performed with low early mortality. Intermediate-term survival and quality of life is good to excellent, and transplantation can be delayed or avoided in the majority. AD - Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota. Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota. Electronic address: jdearani@mayo.edu. Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota. Department of Anesthesiology, Mayo Clinic, Rochester, Minnesota. Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota. AN - 24811983 AU - Raju, V. AU - Dearani, J. A. AU - Burkhart, H. M. AU - Grogan, M. AU - Phillips, S. D. AU - Ammash, N. AU - Pike, R. P. AU - Johnson, J. N. AU - O'Leary, P. W. DA - Jul DB - PubMed DO - 10.1016/j.athoracsur.2014.03.009 DP - NLM ET - 2014/05/09 IS - 1 KW - Adolescent Adult Blalock-Taussig Procedure Child Child, Preschool Ebstein Anomaly/*complications/mortality/surgery Female Follow-Up Studies Fontan Procedure/*methods Heart Failure/etiology/mortality/*surgery Heart Transplantation/*methods Humans Infant Male Middle Aged Retrospective Studies Survival Rate/trends Time Factors Treatment Outcome United States/epidemiology Young Adult LA - eng N1 - 1552-6259 Raju, Vijayakumar Dearani, Joseph A Burkhart, Harold M Grogan, Martha Phillips, Sabrina D Ammash, Naser Pike, Roxann P Johnson, Jonathan N O'Leary, Patrick W Journal Article Netherlands Ann Thorac Surg. 2014 Jul;98(1):167-73; discussion 173-4. doi: 10.1016/j.athoracsur.2014.03.009. Epub 2014 May 6. PY - 2014 SN - 0003-4975 SP - 167-73; discussion 173-4 ST - Right ventricular unloading for heart failure related to Ebstein malformation T2 - Ann Thorac Surg TI - Right ventricular unloading for heart failure related to Ebstein malformation VL - 98 ID - 359 ER - TY - JOUR AB - BACKGROUND: Congenital heart disease (CHD) that causes right atrial dilatation is commonly associated with atrial flutter and/or fibrillation (AFl/F). To reduce late recurrence of AFl/F in patients undergoing repair of CHD, we utilized a concomitant right-sided maze procedure. METHODS: From 1993 to 2003, 99 patients with CHD and associated AFl/F underwent a concomitant right-sided maze procedure at the time of CHD repair. Ages ranged from 9 to 72 years (median, 43 years). Atrial flutter and/or fibrillation was paroxysmal in 81 and chronic in 18; duration ranged from less than 1 month to 39.5 years (median, 2.9 years). Primary cardiac diagnoses were Ebstein anomaly (n = 47), other congenital tricuspid regurgitation (n = 19), univentricular heart (n = 11), isolated atrial septal defect (ASD, n = 8), tetralogy of Fallot (n = 8), and other (n = 6). RESULTS: Other concomitant procedures included tricuspid valve repair or replacement (n = 70), ASD closure (n = 39), and pulmonary valve procedures (n = 18). There were 6 early deaths. At hospital dismissal, 83 patients were free of AFl/F and 63 were in sinus rhythm. Follow-up in 87 of the 93 early survivors extended up to 8 years (mean, 2.7 years). There were 4 late deaths, all from noncardiac causes. Of the 83 known late survivors, 77 (93%) were free of AFl/F. Eighty-two of the 83 survivors were in New York Heart Association class I or II. CONCLUSIONS: In patients with AFl/F associated with CHD, a concomitant right-sided maze procedure at the time of intracardiac repair is effective in reducing late recurrent AFl/F. Most patients enjoy an excellent quality of life. AD - Division of Cardiovascular Surgery, Mayo Clinic and Foundation, Rochester, Minnesota 55905, USA. AN - 16631672 AU - Stulak, J. M. AU - Dearani, J. A. AU - Puga, F. J. AU - Zehr, K. J. AU - Schaff, H. V. AU - Danielson, G. K. DA - May DB - PubMed DO - 10.1016/j.athoracsur.2005.10.046 DP - NLM ET - 2006/04/25 IS - 5 KW - Adolescent Adult Aged Cardiac Surgical Procedures Child Comorbidity Ebstein Anomaly/epidemiology/surgery Female Heart Defects, Congenital/*epidemiology/surgery Heart Septal Defects, Atrial/surgery Humans Male Middle Aged Tachycardia/*epidemiology/*surgery Tricuspid Valve Insufficiency/epidemiology/surgery LA - eng N1 - 1552-6259 Stulak, John M Dearani, Joseph A Puga, Francisco J Zehr, Kenton J Schaff, Hartzell V Danielson, Gordon K Journal Article Netherlands Ann Thorac Surg. 2006 May;81(5):1780-4; discussion 1784-5. doi: 10.1016/j.athoracsur.2005.10.046. PY - 2006 SN - 0003-4975 SP - 1780-4; discussion 1784-5 ST - Right-sided Maze procedure for atrial tachyarrhythmias in congenital heart disease T2 - Ann Thorac Surg TI - Right-sided Maze procedure for atrial tachyarrhythmias in congenital heart disease VL - 81 ID - 420 ER - TY - JOUR AB - BACKGROUND AND OBJECTIVE: Children with congenital heart disease (CHD) are at risk for developmental delay (DD). Changes in cognitive, language, and motor skills in early childhood have not been described. We report the results of a structured approach using longitudinal testing to identify problems and ensure early intervention in accordance with published guidelines. METHODS: Bayley Scales of Infant Development, Third Edition, were used to assess cognitive, language, and motor skills in 99 children with CHD. Subjects were evaluated 3 to 6 times in the first 3 years of life. DD was defined as scores >1 SD below the population mean. RESULTS: Cardiac anatomy was single ventricle (1V) in 34 subjects and 2 ventricles (2V) in 65. Medical comorbidities were present in 21% and genetic syndromes in 19%. Most subjects (75%) had DD in ≥1 area at ≥1 assessments. Subjects with 1V anatomy had equivalent outcomes to those with 2V. Cognitive and language scores declined in subjects with genetic syndromes but were stable and within the average range for subjects with 1V and 2V. Motor scores improved for subjects with 1V and 2V but remained low for those with genetic syndromes. In addition to age, need for supplemental tube feeding, longer cardiopulmonary bypass time, and shorter time since last hospitalization were significant predictors of developmental outcomes. CONCLUSIONS: DDs in young children with CHD are both common and dynamic. Providers should encourage longitudinal surveillance for children with CHD because exposure to risk and prevalence of DD change over time. AD - Herma Heart Center, Children's Hospital of Wisconsin, Milwaukee, Wisconsin; and. AN - 24488746 AU - Mussatto, K. A. AU - Hoffmann, R. G. AU - Hoffman, G. M. AU - Tweddell, J. S. AU - Bear, L. AU - Cao, Y. AU - Brosig, C. C2 - PMC3934337 DA - Mar DB - PubMed DO - 10.1542/peds.2013-2309 DP - NLM ET - 2014/02/04 IS - 3 KW - Child, Preschool Developmental Disabilities/*diagnosis/*epidemiology Female Follow-Up Studies Heart Defects, Congenital/*diagnosis/*epidemiology Humans Infant Longitudinal Studies Male Prevalence Risk Factors assessment and surveillance child development congenital heart disease and defects developmental follow-up developmental outcomes LA - eng N1 - 1098-4275 Mussatto, Kathleen A Hoffmann, Raymond G Hoffman, George M Tweddell, James S Bear, Laurel Cao, Yumei Brosig, Cheryl UL1 TR000055/TR/NCATS NIH HHS/United States 8UL1TR000055/TR/NCATS NIH HHS/United States Journal Article Research Support, N.I.H., Extramural Pediatrics. 2014 Mar;133(3):e570-7. doi: 10.1542/peds.2013-2309. Epub 2014 Feb 2. PY - 2014 SN - 0031-4005 (Print) 0031-4005 SP - e570-7 ST - Risk and prevalence of developmental delay in young children with congenital heart disease T2 - Pediatrics TI - Risk and prevalence of developmental delay in young children with congenital heart disease VL - 133 ID - 177 ER - TY - JOUR AB - BACKGROUND: Congenital cardiac anomalies are associated with immunologic perturbations. Surgical thymectomy, thoracic duct manipulation, and protein- losing enteropathy (PLE), a condition related to stressed Fontan hemodynamics, presumably contribute to low peripheral absolute lymphocyte counts (ALCs) and quantitative immunoglobulins. Clinical significance of lymphopenia and hypogammaglobulinemia in single-ventricle survivors requires additional study. OBJECTIVE: Although immunologic laboratory anomalies are common in this population, we hypothesize that clinically significant immunodeficiency requiring intervention is rarely required. METHODS: A retrospective chart review of the immunologic parameters of patients enrolled in the Single Ventricle Survivorship Program (SVSP) at the Children's Hospital of Philadelphia was performed. RESULTS: The age range of the 178 SVSP patients was 3 to 26 years, with a median of 10.8 years. Most of the SVSP patients had some degree of lymphopenia. In the non-PLE group, the range of ALCs varied from 530 to 5322 cells/μL, with 17 patients without PLE maintaining an ALC of less than 1000 cells/μL. Among those with PLE, the median ALC and the IgG level were lower (672 cells/μL and 200 mg/dL, respectively) than in those without (1610 cells/μL and 868 mg/dL, respectively). Despite lymphopenia in the majority, few were severely clinically affected: 24% had delayed clearance of cutaneous viral infections, 63% had atopy, and 1 died of EBV-associated Hodgkin lymphoma. Immunoglobulin replacement was clinically indicated for 3 patients, 1 of whom had common variable immunodeficiency. Four patients with normal splenic function were treated with daily antibiotic prophylaxis. CONCLUSIONS: Patients with repaired single-ventricle physiology often demonstrate T-cell lymphopenia and hypogammaglobulinemia. A significant portion of patients without PLE also have lymphopenia. The most common clinical manifestation was delayed clearance of cutaneous viral infections, but significant systemic opportunistic infections were not seen despite laboratory abnormalities and lack of antimicrobial prophylaxis. AD - Division of Allergy & Immunology, The Children's Hospital of Philadelphia, Pa. Division of Cardiology, The Children's Hospital of Philadelphia, Pa. Section of Immunology, Allergy, Rheumatology, Baylor College of Medicine, Tex Children's Hospital, Houston, Tex. Division of Allergy & Immunology, The Children's Hospital of Philadelphia, Pa. Electronic address: heimallj@email.chop.edu. AN - 26897303 AU - Morsheimer, M. M. AU - Rychik, J. AU - Forbes, L. AU - Dodds, K. AU - Goldberg, D. J. AU - Sullivan, K. AU - Heimall, J. R. DA - May-Jun DB - PubMed DO - 10.1016/j.jaip.2015.11.034 DP - NLM ET - 2016/02/22 IS - 3 KW - Adolescent Adult Agammaglobulinemia/*etiology Child Child, Preschool Female Fontan Procedure/*adverse effects Heart Defects, Congenital/*surgery Humans Lymphopenia/*etiology Male Protein-Losing Enteropathies/etiology Risk Factors Survivors Young Adult *Fontan *Hypogammaglobulinemia *Protein-losing enteropathy (PLE) *Secondary immunodeficiency *Single ventricle *T-cell lymphopenia *Warts LA - eng N1 - 2213-2201 Morsheimer, Megan M Rychik, Jack Forbes, Lisa Dodds, Kathryn Goldberg, David J Sullivan, Kathleen Heimall, Jennifer R Journal Article Research Support, Non-U.S. Gov't United States J Allergy Clin Immunol Pract. 2016 May-Jun;4(3):491-6. doi: 10.1016/j.jaip.2015.11.034. Epub 2016 Feb 18. PY - 2016 SP - 491-6 ST - Risk Factors and Clinical Significance of Lymphopenia in Survivors of the Fontan Procedure for Single-Ventricle Congenital Cardiac Disease T2 - J Allergy Clin Immunol Pract TI - Risk Factors and Clinical Significance of Lymphopenia in Survivors of the Fontan Procedure for Single-Ventricle Congenital Cardiac Disease VL - 4 ID - 170 ER - TY - JOUR AB - Background: Congenital heart disease (CHD) is the most common birth defect group and a significant contributor to neonatal and infant death. CHD with single ventricle anatomy, including hypoplastic left heart syndrome (HLHS), tricuspid atresia (TA), and various double-inlet ventricle (DIV) malformations, is the most complex with the highest mortality. Prenatal risk factors associated with HLHS have been studied, but such data for DIV are lacking. Methods: We analyzed DIV cases and nonmalformed controls in the National Birth Defects Prevention Study, a case-control, multicenter population-based study of birth defects. Random forest analysis identified potential predictor variables for DIV, which were included in multivariable models to estimate effect magnitude and directionality. Results: Random forest analysis identified pre-pregnancy diabetes, history of maternal insulin use, maternal total lipid intake, paternal race, and intake of several foods and nutrients as potential predictors of DIV. Logistic regression confirmed pre-pregnancy diabetes, maternal insulin use, and paternal race as risk factors for having a child with DIV. Additionally, higher maternal total fat intake was associated with a reduced risk. Conclusions: Maternal pre-pregnancy diabetes and history of insulin use were associated with an increased risk of having an infant with DIV, while maternal lipid intake had an inverse association. These novel data provide multiple metabolic pathways for investigation to identify better the developmental etiologies of DIV and suggest that public health interventions targeting diabetes prevention and management in women of childbearing age could reduce CHD risk. © 2019 Wiley Periodicals, Inc. AD - Division of Pediatric Cardiology, Department of Pediatrics, Stanford University School of Medicine, Palo Alto, CA, United States Stanford Cardiovascular Institute, Stanford University School of Medicine, Palo Alto, CA, United States Division of Neonatal and Developmental Medicine, Department of Pediatrics, Stanford University School of Medicine, Palo Alto, CA, United States Division of Medical Genetics, Department of Pediatrics, University of Utah, Salt Lake City, UT, United States Division of Cardiovascular Medicine, Department of Internal Medicine, Stanford University School of Medicine, Palo Alto, CA, United States AU - Paige, S. L. AU - Yang, W. AU - Priest, J. R. AU - Botto, L. D. AU - Shaw, G. M. AU - Collins, R. T., II AU - the National Birth Defects Prevention, Study DB - Scopus DO - 10.1002/bdr2.1501 IS - 11 KW - case-control studies congenital heart disease double-inlet ventricle M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2019 SP - 640-648 ST - Risk factors associated with the development of double-inlet ventricle congenital heart disease T2 - Birth Defects Research TI - Risk factors associated with the development of double-inlet ventricle congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85063573191&doi=10.1002%2fbdr2.1501&partnerID=40&md5=579670349c8499c0184dc8d0921f539c VL - 111 ID - 1742 ER - TY - JOUR AB - Purpose: To assess the incidence and severity of adverse events (AE) associated with cardiovascular magnetic resonance (CMR) in a large cohort of patients with congenital heart disease and to identify independent risk factors for their occurrence. Methods: AEs were prospectively recorded from October 2002 through December 2004 and graded by 3 independent observers for severity, preventability, and attributability. The rate of adverse events was analyzed for each candidate variable using Fisher's exact test and independent predictors were identified by multiple logistic regression analysis. Results: There were 22 AEs among 1334 CMR studies (1.6%); 14 (63.5%) minor, 7 (32%) moderate, and 1 (4.5%) major. General anesthesia (GA) was used in 274 studies (20.5%) with 12 AEs (4.4%, p < 0.001). There were 7 AEs (6.3%, p = 0.001) in 112 studies on hospitalized patients, 5 AEs (5.2%, p = 0.018) in 97 patients under 1 year of age, and 3 AEs (2.2%, p = 0.479) in 134 patients with functional single ventricle. The highest rate of AEs was noted in inpatients under GA (10.4%, p < 0.001); most were in the intensive care unit. Use of anesthesia (OR 3.91 [95% CI 1.46, 10.48] p = 0.007) and inpatient status (OR 3.56 [95% CI 1.16, 10.89], p = 0.026) were independent predictors of AEs. Conclusions: CMR in patients with congenital heart disease has a low rate of AEs. Use of GA and examinations on hospitalized patients are independent risk factors for AEs with the most acutely ill patients at highest risk. Copyright © 2007 Informa Healthcare USA, Inc. AD - T. Geva, Department of Cardiology, Children's Hospital Boston, 300 Longwood Avenue, Boston, MA 02115, United States AU - Dorfman, A. L. AU - Odegard, K. C. AU - Powell, A. J. AU - Laussen, P. C. AU - Geva, T. C2 - Berlex(United States) DB - Embase Medline DO - 10.1080/10976640701545305 IS - 5 KW - adenosine epinephrine anesthetic agent diphenhydramine dopamine gadolinium pentetate glycopyrronium ipratropium bromide salbutamol steroid adolescent adult aged anxiety article bronchospasm child congenital heart disease controlled study disease severity dysphagia extravasation female Fisher exact test general anesthesia heat hospital patient human hypotension hypoxia incidence intensive care unit lung edema major clinical study male multivariate logistic regression analysis nausea nuclear magnetic resonance imaging pneumothorax priority journal prophylaxis prospective study rash risk factor side effect tachycardia tachypnea unspecified side effect urticaria vomiting LA - English M3 - Article N1 - L47463907 2007-09-01 PY - 2007 SN - 1097-6647 1532-429X SP - 793-798 ST - Risk factors for adverse events during cardiovascular magnetic resonance in congenital heart disease T2 - Journal of Cardiovascular Magnetic Resonance TI - Risk factors for adverse events during cardiovascular magnetic resonance in congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L47463907 http://dx.doi.org/10.1080/10976640701545305 VL - 9 ID - 1229 ER - TY - JOUR AB - OBJECTIVES: As the surgical results for Fontan candidates improve, much attention has been given to the long-term cognitive outcomes. This study aimed to assess the neurocognitive outcomes after Fontan completion, and to determine the factors associated with an extremely low intelligence quotient (IQ <70). METHODS: A total of 70 local eligible school-aged patients were enrolled in the study (hypoplastic left heart syndrome, 17; right atrial isomerism, 15 and others, 38). Fontan operations were performed at a median age of 1.8 (range, 0.5-8.9) years (primary Fontan, 4 of 70 [6%]). The Wechsler Intelligence Test was taken at a median age of 9 (range, 5.1-14.4) years for the purpose of neurocognitive evaluation. Patients' data were collected from medical records, and a retrospective analysis of potential predictors for an IQ <70 was performed. RESULTS: The median full-scale IQ (FSIQ) for the entire cohort was 85 (range, 43-118). Of the lower order composites, the perceptual reasoning index and the working memory index were significantly lower in low-FSIQ patients (P < 0.05). Overall, 15 of 70 (21%) of the cohort had an IQ <70. Univariate analysis identified three significant risk factors for FSIQ <70 (15 of 70, 21%): body weight <2.5 kg at initial palliative surgery (P < 0.05), low 5-min Apgar score <4 (P < 0.05) and inter-stage events requiring cardiopulmonary resuscitation (CPR) (P < 0.05). No other patient-specific factors (e.g. cardiac morphology) or modifiable surgical factors (e.g. the use of hypothermic cardiac arrest) were associated with FSIQ <70. CONCLUSIONS: Low body weight (<2.5 kg) at initial operation, low 5-min Apgar score (<4) and inter-stage CPR were significant risk factors for impaired neurocognitive outcomes. An evolving strategy for preventing inter-stage CPR may improve cognitive outcomes. AD - Department of Cardiovascular Surgery, Mt. Fuji Shizuoka Children's Hospital, Shizuoka, Japan. AN - 23423918 AU - Sugimoto, A. AU - Ota, N. AU - Ibuki, K. AU - Miyakoshi, C. AU - Murata, M. AU - Tosaka, Y. AU - Yamazaki, T. AU - Sakamoto, K. DA - Sep DB - PubMed DO - 10.1093/ejcts/ezt062 DP - NLM ET - 2013/02/21 IS - 3 KW - Analysis of Variance Child Child, Preschool Cohort Studies Fontan Procedure/*adverse effects/methods Heart Defects, Congenital/*surgery Humans Infant Intellectual Disability/*etiology Intelligence Tests Postoperative Complications/etiology Retrospective Studies Risk Factors Adverse effects Fontan procedure Hypoplastic left heart syndrome Neurobehavioural manifestations Right atrial isomerism LA - eng N1 - 1873-734x Sugimoto, Ai Ota, Noritaka Ibuki, Keijiro Miyakoshi, Chisato Murata, Masaya Tosaka, Yuko Yamazaki, Toru Sakamoto, Kisaburo Journal Article Germany Eur J Cardiothorac Surg. 2013 Sep;44(3):454-61; discussion 461. doi: 10.1093/ejcts/ezt062. Epub 2013 Feb 19. PY - 2013 SN - 1010-7940 SP - 454-61; discussion 461 ST - Risk factors for adverse neurocognitive outcomes in school-aged patients after the Fontan operation T2 - Eur J Cardiothorac Surg TI - Risk factors for adverse neurocognitive outcomes in school-aged patients after the Fontan operation VL - 44 ID - 350 ER - TY - JOUR AB - OBJECTIVES: This study evaluated the various risk factors for chylothorax and persistent serous effusions (>7 days) after congenital heart surgery and developed equations to calculate the probability of their occurrence. METHODS: We performed a retrospective review of different medical databases at the University Hospital of Erlangen between January 2014 and December 2016. Full model regression analysis was used to identify risk factors, and prediction algorithms were set up to calculate probabilities. Discriminative power of the models was checked with the help of C-statistics. RESULTS: Of 745 operations on 667 patients, 68 chylothoraxes (9.1%) and 125 persistent pleural effusions (16.8%) were diagnosed. Lowest temperature [P = 0.043; odds ratio (OR) 0.899], trisomy 21 (P = 0.001; OR 5.548), a higher vasoactive inotropic score on the day of surgery (P = 0.001; OR 1.070) and use of an assist device (P = 0.001; OR 5.779) were significantly associated with chylothorax. Risk factors for persistent serous effusions were a given or possible involvement of the aortic arch during the operation (P = 0.000; OR 3.982 and 2.905), univentricular hearts (P = 0.019; OR 2.644), a higher number of previous heart operations (P = 0.014; OR 1.436), a higher vasoactive inotropic score 72 h after surgery (P = 0.019; OR 1.091), a higher central venous pressure directly after surgery (P = 0.046; OR 1.076) and an aortic cross-clamp time >86 min (P = 0.023; OR 2.223), as well as use of an assist device (P = 0.002; OR 10.281). The prediction models for both types of effusions proved to have excellent discriminative power. CONCLUSIONS: Persistent serous effusion is associated with a higher vasoactive inotropic score 72 h after surgery, an aortic cross-clamp time >86 min and elevated central venous pressure directly after surgery, which, in combination, potentially indicate cardiac stress. The developed logistic algorithm helps to estimate future likelihood. © 2019 The Author(s). Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. AD - Department of Pediatric Cardiology, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Loschgestrasse 15, Erlangen, 91054, Germany Department of Pediatric Cardiac Surgery, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Erlangen, Germany Department of Pediatric Cardiac Surgery, University Heart Centre Hamburg, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany AU - Raatz, A. AU - Schöber, M. AU - Zant, R. AU - Cesnjevar, R. AU - Rüffer, A. AU - Purbojo, A. AU - Dittrich, S. AU - Alkassar, M. DB - Scopus DO - 10.1093/ejcts/ezz203 IS - 6 KW - Cardiac insufficiency Cardiac surgery Chylothorax Congenital heart disease Persistent effusions Risk factor M3 - Article N1 - Export Date: 15 June 2020 PY - 2019 SP - 1162-1169 ST - Risk factors for chylothorax and persistent serous effusions after congenital heart surgery T2 - European Journal of Cardio-thoracic Surgery TI - Risk factors for chylothorax and persistent serous effusions after congenital heart surgery UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85075223029&doi=10.1093%2fejcts%2fezz203&partnerID=40&md5=9e744c10bcfa4960991bc3baf735ef74 VL - 56 ID - 1723 ER - TY - JOUR AB - Objective: To determine the risk factors for adverse neurodevelopmental outcomes in school-age children after full flow open-heart surgery for congenital heart disease. Methods: The outcome was assessed in 117 children without a genetic comorbidity at a mean age of 10.4 ± 2.5 years. Intelligence was assessed using the Raven's Progressive Matrices and neuromotor function using the Zurich Neuromotor Assessment. Risk factors were retrieved from detailed chart review. Results: The mean intelligence score was 89 ± 16, significantly lower than the norm (P < .001). Cerebral palsy was diagnosed in 10% of patients. Poor neuromotor performance (less than p10) was present in 15% to 20% of the children, depending on the motor task (all P < .001). Pure motor and static balance performance was also significantly impaired when patients with cerebral palsy were excluded (P < .01). Intelligence was only related to socioeconomic status (P = .006), and neuromotor outcome was related to the length of hospital stay and postoperative neurologic abnormalities (P < .03). The extracorporeal circulation time was related to adaptive fine motor performance (P = .05). All other variables were not related to outcome. Conclusions: Children without a genetic comorbidity are at risk of long-term intellectual and motor impairments also after full-flow cardiac repair. Surgery-related parameters play a less important role for adverse outcomes than postoperative complications. Our findings stress the importance of specialized follow-up assessments for all children with CHD undergoing open heart surgery. Copyright © 2012 by The American Association for Thoracic Surgery. AD - M. Von Rhein, Child Development Center, University Children's Hospital of Zurich, Steinwiesstrasse 75, Zurich CH-8032, Switzerland AU - Von Rhein, M. AU - Dimitropoulos, A. AU - Valsangiacomo Buechel, E. R. AU - Landolt, M. A. AU - Latal, B. DB - Embase Medline DO - 10.1016/j.jtcvs.2012.02.005 IS - 3 KW - adolescent age aortic coarctation aortic stenosis article body equilibrium cardiopulmonary bypass cerebral palsy child clinical assessment tool cohort analysis congenital heart disease cor triatriatum coronary artery fistula extracorporeal circulation Fallot tetralogy female heart arrhythmia heart atrium septum defect human intellectual impairment intelligence test length of stay lung vein drainage anomaly major clinical study male medical record review mitral valve stenosis morbidity motoneuron motor dysfunction motor performance neurologic disease open heart surgery outcome assessment preschool child priority journal pulmonary valve atresia pulmonary valve stenosis raven progressive matrices and neuromotor function risk factor school child socioeconomics tricuspid valve atresia zurich neuromotor assessment LA - English M3 - Article N1 - L51903746 2012-03-14 2012-09-13 PY - 2012 SN - 0022-5223 1097-685X SP - 577-583 ST - Risk factors for neurodevelopmental impairments in school-age children after cardiac surgery with full-flow cardiopulmonary bypass T2 - Journal of Thoracic and Cardiovascular Surgery TI - Risk factors for neurodevelopmental impairments in school-age children after cardiac surgery with full-flow cardiopulmonary bypass UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51903746 http://dx.doi.org/10.1016/j.jtcvs.2012.02.005 VL - 144 ID - 1045 ER - TY - JOUR AB - BACKGROUND: Injured phrenic nerve secondary to cardiac surgeries is the most common cause of diaphragmatic paralysis (DP) in infants. The aim of this study was to determine the risk factors for DP caused by congenital heart defect corrective surgeries in pediatrics. METHODS: This cross-sectional study, conducted in a 2-year period (2006-2008), included 451 children with congenital heart diseases admitted to the Pediatric Cardiac Surgery Ward of Imam Khomeini Hospital. The diaphragmatic function was examined via fluoroscopy, and the frequency of DP and its relevant parameters were evaluated. RESULTS: Of the 451 patients, comprising 268 males and 183 females at an age range of 3 days to 204 months (28.2 ± 33.4 months), 25 (5.5%) infants (60% male and 40% female, age range = 15 days to 132 months, 41.2 ± 28.1 months) had DP as follows: 48% unilateral right-sided and 36% unilateral left-sided. Additionally, 68% had cyanotic congenital heart disease and 84% had DP following total correction surgery. The highest prevalence rates of DP resulting in phrenic hemiparesis were observed after arterial switch operation, Fontan procedure, and Blalock-Taussig shunt surgery, respectively. Thirteen (52%) of the 25 DP patients underwent surgical diaphragmatic plication because of severe respiratory distress and dependency on mechanical ventilation, and most of the cases of plication underwent arterial switch operation. The rate of mortality was 24% (6 patients). CONCLUSION: DP with a prevalence of 5.5% was one of the most common complications secondary to cardiac surgeries in the infants included in the present study. Effective factors were age, weight, cyanotic congenital heart defects, and previous cardiac surgery. Diaphragmatic plication improved prognosis in severe cases. AD - Imam Khomeini Complex, Tehran University of Medical Sciences, Tehran, Iran. Baharlo Hospital, Tehran University of Medical Sciences, Tehran, Iran. Maternal-Fetal and Neonatal Research Center, Tehran University of Medical Sciences, Tehran, Iran. Valiasr Hospital, Breastfeeding Research Center, Tehran, Iran. AN - 26697086 AU - Akbariasbagh, P. AU - Mirzaghayan, M. R. AU - Akbariasbagh, N. AU - Shariat, M. AU - Ebrahim, B. C2 - PMC4685369 DA - Jul 3 DB - PubMed DP - NLM ET - 2015/12/24 IS - 3 KW - Cardiac surgical procedures Diaphragmatic Infant Phrenic nerve newborn LA - eng N1 - 2008-2371 Akbariasbagh, Parvin Mirzaghayan, Mohammad Reza Akbariasbagh, Naseredin Shariat, Mamak Ebrahim, Bita Journal Article J Tehran Heart Cent. 2015 Jul 3;10(3):134-9. PY - 2015 SN - 1735-5370 (Print) 1735-5370 SP - 134-9 ST - Risk Factors for post-Cardiac Surgery Diaphragmatic Paralysis in Children with Congenital Heart Disease T2 - J Tehran Heart Cent TI - Risk Factors for post-Cardiac Surgery Diaphragmatic Paralysis in Children with Congenital Heart Disease VL - 10 ID - 339 ER - TY - JOUR AB - Objective: Prolonged mechanical ventilation (PMV) is closely associated with higher morbidity and mortality after total cavopulmonary surgery. The aim of the present study was to identify the clinical risk factors for PMV. Design: A retrospective case-control study. Setting: Fuwai Hospital. Participants: The study comprised 504 patients who underwent total cavopulmonary surgery from 2010 to 2018. Interventions: None. Measurements and Main Results: The definition of PMV was derived from the Cox regression model for predicting postoperative length of hospital stay. Least absolute shrinkage and selection operator regression, logistic regression, and Cox regression were applied to identify predictors for PMV. Patients with mechanical ventilation time >9 hours were identified as having PMV. Independent predictors of PMV included age, intraoperative maximum vasoactive-inotropic score, minimal temperature during cardiopulmonary bypass, postoperative prothrombin time, alkaline phosphatase and total bilirubin levels, and postoperative fluid balance. These predictors also were achieved in the Cox regression for predicting the duration of mechanical ventilation. Patients with PMV were associated with increased blood transfusions, more consumption of vasopressin and antipulmonary hypertension medication, higher incidence of reintubation, more renal replacement treatment, longer intensive care unit stay, greater hospitalization costs, and more specialist visits. Conclusions: Age at surgery, maximal vasoactive-inotropic score and minimal temperature during cardiopulmonary bypass, postoperative prothrombin time, alkaline phosphatase and total bilirubin levels, and postoperative fluid balance were demonstrated to be independent predictors of PMV. Adopting a comprehensive strategy of perioperative management that targets the identified risk factors might significantly lower the risk of PMV and improve in-hospital outcomes, and furthermore, patients with PMV might need more specialist visits. AD - F. Yan, Department of Anesthesiology, Fuwai Hospital, National Center of Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, 167 Beilishi Road, Xicheng District, Beijing, China AU - Luo, Q. AU - Su, Z. AU - Jia, Y. AU - Liu, Y. AU - Wang, H. AU - Zhang, L. AU - Li, Y. AU - Wu, X. AU - Liu, Q. AU - Yan, F. DB - Embase Medline DO - 10.1053/j.jvca.2019.10.043 IS - 4 KW - alkaline phosphatase bilirubin fresh frozen plasma inotropic agent levosimendan vasopressin article artificial ventilation atrioventricular septal defect blood transfusion body surface cardiopulmonary bypass case control study cavopulmonary connection child controlled study erythrocyte transfusion estimated glomerular filtration rate fluid balance follow up health care cost heart ejection fraction heart left ventricle heart left ventricle enddiastolic diameter heart right ventricle heart right ventricle double outlet heterotaxy syndrome hospital mortality hospitalization cost human hypervolemia intensive care unit length of stay lung artery pressure major clinical study postoperative period preoperative evaluation preschool child priority journal prothrombin time pulmonary hypertension pulmonary valve atresia renal replacement therapy retrospective study risk factor risk reduction thrombocyte transfusion total cavopulmonary connection surgery treatment duration tricuspid valve atresia LA - English M3 - Article N1 - L2004691073 2020-01-31 2020-03-10 PY - 2020 SN - 1532-8422 1053-0770 SP - 940-948 ST - Risk Factors for Prolonged Mechanical Ventilation After Total Cavopulmonary Connection Surgery: 8 Years of Experience at Fuwai Hospital T2 - Journal of Cardiothoracic and Vascular Anesthesia TI - Risk Factors for Prolonged Mechanical Ventilation After Total Cavopulmonary Connection Surgery: 8 Years of Experience at Fuwai Hospital UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2004691073 http://dx.doi.org/10.1053/j.jvca.2019.10.043 VL - 34 ID - 556 ER - TY - JOUR AB - The aim of our project was to study possible etiological factors in the origin of congenital heart defects (CHDs) because in the majority of patients the underlying causes are unclear. Cases with different CHD entities as homogeneously as possible were planned for evaluation in the population-based large data set of the Hungarian Case Control Surveillance of Congenital Abnormalities. Dead or surgically corrected 302 live-born cases with different types of left-ventricular outflow tract obstructive defects (LVOT, i.e., valvular aortic stenosis 76, hypoplastic left heart syndrome 76, coarctation of the aorta 113, and other congenital anomalies of aorta 32) were compared with 469 matched controls, 38,151 controls without any defects, and 20,750 malformed controls with other isolated defects. Medically recorded pregnancy complications and chronic diseases were evaluated based on prenatal maternity logbooks, whereas acute diseases, drug treatments, and folic acid/multivitamin supplementation were analyzed both on the basis of retrospective maternal information and medical records. The results of the study showed the role of maternal diabetes in the origin of LVOT in general, while panic disorder was associated with a higher risk of hypoplastic left heart syndrome and ampicillin treatment with a higher risk of coarctation of the aorta (COA). High doses of folic acid had a protective effect regarding the manifestation of LVOT, particularly COA. In conclusion, only a minor portion of causes was shown in our study; thus, further studies are needed to understand better the underlying causal factors in the origin of LVOT. © 2013 Springer Science+Business Media New York. AD - A. Vereczkey, Versys Clinics, Human Reproduction Institute, Madarász Viktor utca 47-49, Budapest 1138, Hungary AU - Csáky-Szunyogh, M. AU - Vereczkey, A. AU - Kósa, Z. AU - Gerencsér, B. AU - Czeizel, A. E. DB - Embase Medline DO - 10.1007/s00246-013-0749-6 IS - 1 KW - ampicillin cotrimoxazole etacrynic acid folic acid insulin magnesium citrate methyldopa paracetamol ritodrine acute disease anemia aortic coarctation aortic stenosis subvalvular aortic stenosis aortic valve stenosis article bicuspid aortic valve cardiovascular risk child death chromosome aberration chronic disease controlled study cytomegalovirus infection disease association eclampsia edema environmental exposure heart left ventricle outflow tract heart left ventricle outflow tract obstruction heart ventricle septum defect human hydramnios hypertrophic cardiomyopathy hypoplastic left heart syndrome imminent abortion insulin dependent diabetes mellitus kidney disease major clinical study maternal age maternal diabetes mellitus maternal disease maternal hypertension nausea and vomiting newborn panic placenta disorder population based case control study preeclampsia pregnancy complication pregnancy diabetes mellitus pregnancy outcome premature labor teratogenesis two dimensional echocardiography vitamin supplementation LA - English M3 - Article N1 - L52676724 2013-07-17 2014-02-12 PY - 2014 SN - 0172-0643 1432-1971 SP - 108-120 ST - Risk factors in the origin of congenital left-ventricular outflow-tract obstruction defects of the heart: A population-based case-control study T2 - Pediatric Cardiology TI - Risk factors in the origin of congenital left-ventricular outflow-tract obstruction defects of the heart: A population-based case-control study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52676724 http://dx.doi.org/10.1007/s00246-013-0749-6 VL - 35 ID - 942 ER - TY - JOUR AB - Background: Injured phrenic nerve secondary to cardiac surgeries is the most common cause of diaphragmatic paralysis (DP) in infants. The aim of this study was to determine the risk factors for DP caused by congenital heart defect corrective surgeries in pediatrics. Methods: This cross-sectional study, conducted in a 2-year period (2006–2008), included 451 children with congenital heart diseases admitted to the Pediatric Cardiac Surgery Ward of Imam Khomeini Hospital. The diaphragmatic function was examined via fluoroscopy, and the frequency of DP and its relevant parameters were evaluated. Results: Of the 451 patients, comprising 268 males and 183 females at an age range of 3 days to 204 months (28.2 ± 33.4 months), 25 (5.5%) infants (60% male and 40% female, age range = 15 days to 132 months, 41.2 ± 28.1 months) had DP as follows: 48% unilateral right-sided and 36% unilateral left-sided. Additionally, 68% had cyanotic congenital heart disease and 84% had DP following total correction surgery. The highest prevalence rates of DP resulting in phrenic hemiparesis were observed after arterial switch operation, Fontan procedure, and Blalock–Taussig shunt surgery, respectively. Thirteen (52%) of the 25 DP patients underwent surgical diaphragmatic plication because of severe respiratory distress and dependency on mechanical ventilation, and most of the cases of plication underwent arterial switch operation. The rate of mortality was 24% (6 patients). Conclusion: DP with a prevalence of 5.5% was one of the most common complications secondary to cardiac surgeries in the infants included in the present study. Effective factors were age, weight, cyanotic congenital heart defects, and previous cardiac surgery. Diaphragmatic plication improved prognosis in severe cases. AD - B. Ebrahim, Breastfeeding Research Center, Valiasr Hospital, Imam Khomeini Complex, Keshavarz Blvd, Tehran, Iran AU - Akbariasbagh, P. AU - Mirzaghayan, M. R. AU - Akbariasbagh, N. AU - Shariat, M. AU - Ebrahim, B. DB - Embase IS - 3 KW - arterial switch operation article artificial ventilation Blalock Taussig shunt child child health care childhood mortality congenital heart disease controlled study cross-sectional study cyanotic heart disease descriptive research diaphragm movement diaphragm paralysis female fluoroscopy Fontan procedure heart surgery human infant Iran major clinical study male phrenic nerve paralysis prevalence respiratory distress risk assessment risk factor surgical diaphragmatic plication thorax surgery LA - English M3 - Article N1 - L605461084 2015-08-06 2015-08-12 PY - 2015 SN - 2008-2371 1735-8620 SP - 134-139 ST - Risk factors of post-cardiac surgery diaphragmatic paralysis in children with congenital heart disease T2 - Journal of Tehran University Heart Center TI - Risk factors of post-cardiac surgery diaphragmatic paralysis in children with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L605461084 VL - 10 ID - 890 ER - TY - JOUR AB - BACKGROUND: More children with congenital heart disease (CHD) are surviving to adulthood, and CHD is associated with risk factors for dementia. We compared the risk of dementia in CHD adults to that of the general population. METHODS: In this cohort study, we used medical registries and a medical record review covering all Danish hospitals to identify adults with CHD diagnosed between 1963 and 2012. These individuals with CHD were followed from January 1, 1981, 30 years of age, or date of first CHD registration (index date for matched members of the general population cohort) until hospital diagnosis of dementia, death, emigration, or end of study (December 31, 2012). For each individual with CHD, we identified 10 members of the general population utilizing the Danish Civil Registration System matched on sex and birth year. We computed cumulative incidences and hazard ratios (HRs) of dementia, adjusting for sex and birth year. RESULTS: The cumulative incidence of dementia was 4% by 80 years of age in 10 632 adults with CHD (46% male). The overall HR comparing adults with CHD with the general population cohort was 1.6 (95% confidence interval [CI], 1.3-2.0). The HR among individuals with CHD without extracardiac defects was 1.4 (95% CI, 1.1-1.8). Adults with mild-to-moderate CHD had an HR of 1.5 (95% CI, 1.1-2.0), whereas the HR was 2.0 (95% CI, 1.2-3.3) for severe CHD, including univentricular hearts. The HR for early onset dementia (<65 years of age) was 2.6 (95% CI, 1.8-3.8), whereas the late-onset HR was 1.3 (95% CI, 1.0-1.8). CONCLUSIONS: CHD was associated with an increased risk of dementia compared with the general population, in particular for early onset dementia. Further understanding of dementia risk in the population with CHD is a potential target for future investigation. © 2018 American Heart Association, Inc. AD - Department of Clinical Epidemiology, Aarhus University Hospital, Olof Palmes Allé 43-45, Aarhus N, DK-8200, Denmark Departments of Cardiology, Aarhus University Hospital, Denmark Departments of Radiology, Aarhus University Hospital, Denmark Departments of Health Research and Policy (Epidemiology) and Neurology and Neurological Sciences, Stanford UniversityCA, United States Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH, United States AU - Bagge, C. N. AU - Henderson, V. W. AU - Laursen, H. B. AU - Adelborg, K. AU - Olsen, M. AU - Madsen, N. L. DB - Scopus DO - 10.1161/CIRCULATIONAHA.117.029686 IS - 18 KW - Cohort study Congenital heart disease Dementia Epidemiology Long-term outcome M3 - Article N1 - Cited By :16 Export Date: 15 June 2020 PY - 2018 SP - 1912-1920 ST - Risk of dementia in adults with congenital heart disease: Population-based cohort study T2 - Circulation TI - Risk of dementia in adults with congenital heart disease: Population-based cohort study UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85048859827&doi=10.1161%2fCIRCULATIONAHA.117.029686&partnerID=40&md5=9a1c9a3cfb17b5936e6c44b863dc6d38 VL - 137 ID - 1838 ER - TY - JOUR AD - K.G. Meighen, James Whitcomb Riley Hosp. Children, 702 Barnhill Dr., Indianapolis, IN 46202-5200, United States AU - Bealke, J. M. AU - Meighen, K. G. DB - Embase Medline DO - 10.1176/appi.psy.46.3.254 IS - 3 KW - chloral hydrate chlorpromazine dexamethasone diphenhydramine haloperidol hypnotic sedative agent lorazepam mercaptopurine methotrexate morphine sulfate phenytoin risperidone vincristine acute lymphoblastic leukemia aggression agitation article brain infarction case report clinical feature computer assisted tomography congenital heart disease critical illness drug response electroencephalogram feeding disorder fever Fontan procedure heart single ventricle human hypertension hypoxia irritability male mood disorder nuclear magnetic resonance imaging preschool child seizure tachycardia tachypnea LA - English M3 - Article N1 - L40594316 2005-05-12 PY - 2005 SN - 0033-3182 SP - 254-258 ST - Risperidone treatment of three seriously medically ill children with secondary mood disorders T2 - Psychosomatics TI - Risperidone treatment of three seriously medically ill children with secondary mood disorders UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L40594316 http://dx.doi.org/10.1176/appi.psy.46.3.254 VL - 46 ID - 1277 ER - TY - JOUR AB - Exercise testing is a valuable tool in the management of pediatric patients with heart disease. It can be used to help determine the need for medical or surgical interventions and can be used to determine the efficacy of these interventions. Common settings in which exercise testing may be helpful include evaluation of exercise tolerance in repaired or unrepaired congenital heart disease, assessment of heart failure symptoms, diagnosis and management of dysrhythmias, determination of cardiac, as opposed to respiratory causes of chest pain and prescription of exercise rehabilitation programs. This manuscript will review the indications, techniques, and uses of exercise stress testing in the pediatric population with an emphasis on the development of the exercise physiology lab as a source of high-quality data that can be used to improve patient outcomes. © 2005 Elsevier Ireland Ltd. All rights reserved. AD - Exercise Physiology Laboratory, Golisano Children's Hosp. at Strong, United States Division of Pediatric Cardiology, Box 631, Golisano Children's Hosp. at Strong, 601 Elmwood Ave, Rochester, NY 14642, United States AU - Connuck, D. M. DB - Scopus DO - 10.1016/j.ppedcard.2004.12.004 IS - 1 KW - Congenital heart disease Exercise testing Outcomes Pediatrics M3 - Article N1 - Cited By :19 Export Date: 15 June 2020 PY - 2005 SP - 45-52 ST - The role of exercise stress testing in pediatric patients with heart disease T2 - Progress in Pediatric Cardiology TI - The role of exercise stress testing in pediatric patients with heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-16844374512&doi=10.1016%2fj.ppedcard.2004.12.004&partnerID=40&md5=ed3ca440ce9d6593404279e850ae5916 VL - 20 ID - 2243 ER - TY - JOUR AB - Background: Cardiovascular Magnetic Resonance (CMR) of patients with congenital heart disease (CHD) has become routine clinical practice. However, existing CMR protocols focus predominantly on patients with ischemic heart disease, and information is limited on the types of patient with CHD who benefit from CMR investigation, and in what ways. Therefore the aim of this study was to answer the questions: What type of patients were studied by CMR in a centre specializing in paediatric and adult CHD management? What questions were asked, which protocols were used and were the questions successfully answered? To answer these questions, we conducted a cohort study of all 362 patients that received routine clinical CMR during 2007 at the Department of Paediatric Cardiology and Congenital Heart Disease at the Deutsches Herzzentrum München. Results: Underlying diagnosis was in 33% Fallot's tetralogy, 17% aortic coarctation, 8% Ebstein's disease, 6% Marfan's disease, 4% single ventricle with Fontan-like circulation, and 32% others. Median age was 26 years (7 days - 75 years). Ventricular volumes were assessed in 67% of the patients; flow in 74%; unknown anatomy only in 9%; specific individual morphology of known anatomy in 83%; myocardial fibrosis in 8%; stress-induced myocardial perfusion defects in 1%. Only in 3% of the cases the question could not be fully answered. Conclusion: Contrary to common belief, routine CMR of patients with CHD was not requested to address global anatomical questions so much as to clarify specific questions of morphology and function of known anatomy. The CMR protocols used differed markedly from those widely used in patients with ischemic heart disease. © 2008 Fratz et al; licensee BioMed Central Ltd. AD - S. Fratz, Department of Paediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München an der Technischen Universität München, Munich, Germany AU - Fratz, S. AU - Hess, J. AU - Schuhbaeck, A. AU - Buchner, C. AU - Hendrich, E. AU - Martinoff, S. DB - Embase Medline DO - 10.1186/1532-429X-10-46 IS - 1 KW - adolescent adult adult disease aged aortic coarctation article child childhood disease congenital heart disease Ebstein anomaly Fallot tetralogy heart muscle fibrosis heart muscle perfusion heart single ventricle heart ventricle volume human infant major clinical study Marfan syndrome morphology newborn nuclear magnetic resonance imaging practice guideline preschool child priority journal school child LA - English M3 - Article N1 - L355084827 2009-09-08 PY - 2008 SN - 1097-6647 1532-429X ST - Routine clinical cardiovascular magnetic resonance in paediatric and adult congenital heart disease: Patients, protocols, questions asked and contributions made T2 - Journal of Cardiovascular Magnetic Resonance TI - Routine clinical cardiovascular magnetic resonance in paediatric and adult congenital heart disease: Patients, protocols, questions asked and contributions made UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L355084827 http://dx.doi.org/10.1186/1532-429X-10-46 VL - 10 ID - 1194 ER - TY - JOUR AB - BACKGROUND: Cardiovascular magnetic resonance (CMR) of patients with congenital heart disease (CHD) has become routine clinical practice. However, existing CMR protocols focus predominantly on patients with ischemic heart disease, and information is limited on the types of patient with CHD who benefit from CMR investigation, and in what ways. Therefore the aim of this study was to answer the questions: What type of patients were studied by CMR in a centre specializing in paediatric and adult CHD management? What questions were asked, which protocols were used and were the questions successfully answered? To answer these questions, we conducted a cohort study of all 362 patients that received routine clinical CMR during 2007 at the Department of Paediatric Cardiology and Congenital Heart Disease at the Deutsches Herzzentrum München. RESULTS: Underlying diagnosis was in 33% Fallot's tetralogy, 17% aortic coarctation, 8% Ebstein's disease, 6% Marfan's disease, 4% single ventricle with Fontan-like circulation, and 32% others. Median age was 26 years (7 days - 75 years). Ventricular volumes were assessed in 67% of the patients; flow in 74%; unknown anatomy only in 9%; specific individual morphology of known anatomy in 83%; myocardial fibrosis in 8%; stress-induced myocardial perfusion defects in 1%. Only in 3% of the cases the question could not be fully answered. CONCLUSION: Contrary to common belief, routine CMR of patients with CHD was not requested to address global anatomical questions so much as to clarify specific questions of morphology and function of known anatomy. The CMR protocols used differed markedly from those widely used in patients with ischemic heart disease. AD - Department of Paediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München an der Technischen Universität München, Munich, Germany. fratz@dhm.mhn.de AN - 18928522 AU - Fratz, S. AU - Hess, J. AU - Schuhbaeck, A. AU - Buchner, C. AU - Hendrich, E. AU - Martinoff, S. AU - Stern, H. C2 - PMC2579426 DA - Oct 17 DB - PubMed DO - 10.1186/1532-429x-10-46 DP - NLM ET - 2008/10/22 IS - 1 KW - Adolescent Adult Aged Child Child, Preschool Clinical Protocols Cohort Studies Coronary Circulation Fibrosis Heart Defects, Congenital/*pathology/physiopathology Heart Ventricles/pathology Humans Infant Infant, Newborn *Magnetic Resonance Imaging/methods Middle Aged Myocardium/*pathology Patient Selection Predictive Value of Tests Retrospective Studies Young Adult LA - eng N1 - 1532-429x Fratz, Sohrab Hess, John Schuhbaeck, Annika Buchner, Christine Hendrich, Eva Martinoff, Stefan Stern, Heiko Journal Article J Cardiovasc Magn Reson. 2008 Oct 17;10(1):46. doi: 10.1186/1532-429X-10-46. PY - 2008 SN - 1097-6647 (Print) 1097-6647 SP - 46 ST - Routine clinical cardiovascular magnetic resonance in paediatric and adult congenital heart disease: patients, protocols, questions asked and contributions made T2 - J Cardiovasc Magn Reson TI - Routine clinical cardiovascular magnetic resonance in paediatric and adult congenital heart disease: patients, protocols, questions asked and contributions made VL - 10 ID - 342 ER - TY - JOUR AB - Background. Although excellent survival following the Norwood procedure for palliation of hypoplastic left heart syndrome (HLHS) is being achieved by some, most centers, especially the ones with small surgical volume and limited experience, continue to struggle with initial results. Survivors often showed evidence of significant neurologic injury. The early postoperative care is labor-intensive as attempts are made to balance the systemic and pulmonary circulation for these infants. We report our experience with routine use of mechanical circulatory assist to support the increased cardiac output requirements present following Norwood procedure. Methods. Eighteen consecutive infants undergoing Norwood operation for HLHS (Oregon Health & Science University [OHSU] 13; University of Louisville [UL] 5) were placed on a ventricular assist device (VAD) immediately following modified ultrafiltration in the operating room using the cardiopulmonary bypass (CPB) cannulas that were in the right atrium and the neoaorta. VAD flows were maintained at approximately 200 mL·kg-1·min-1 and the patients were transported to the intensive care unit (ICU). Patients operated at OHSU also received neurodevelopmental testing before their Glenn procedure, approximately 4 to 6 months following their Norwood operation. Results. All patients were stable on VAD support and no attempt was made to balance the systemic and pulmonary circulation. The ventilator was manipulated to achieve systemic Pa02 between 30 and 45 mm Hg and PaC02 between 35 and 45 mm Hg. Evidence of hypoperfusion (increasing lactates) was managed by increasing the VAD flow. Lactates normalized [< 2 mmol/L]) by 1.8 ± 1.1 days following surgery. Average time of VAD support was 3.1 ± 1.0 (range, 2 to 5 days) and average time until chest closure was 3.4 ± 1.5 (range, 2 to 8 days). There were two cases of postoperative bleeding (11.1%) requiring reexploration and one case of mediastinitis (5.5%) in a patient who has now gone on to successful Glenn. Sixteen of the eighteen patients survived (hospital survival mean 89% with a 95% confidence interval of 63.9% to 98.1%; 12/13 OHSU [92.3%]; 4/5 UL [80%]). Neurodevelopmental testing using the Mullen Scales of Early Learning and the Vineland Adaptive Behavior Scale were normal for all infants tested. Conclusions. Routine postoperative use of VAD can support the increased cardiac output demands of infants following Norwood operation and results in a stable postoperative convalescence that does not require aggressive ventilator or inotrope manipulation. Although not a panacea, this strategy can simplify postoperative management, lead to excellent hospital survival, and possibly augment cerebral oxygen delivery, resulting in improved neurologic outcomes for this challenging group of patients. © 2004 by The Society of Thoracic Surgeons. AD - R.M. Ungerleider, Oregon Health and Science University, Doernbecher Children's Hospital, Mail Code L353, 3181 SW Sam Jackson Park Rd, Portland, OR 97239, United States AU - Ungerleider, R. M. AU - Shen, I. AU - Yeh Jr, T. AU - Schultz, J. AU - Butler, R. AU - Silberbach, M. AU - Giacomuzzi, C. AU - Heller, E. AU - Studenberg, L. AU - Mejak, B. AU - You, J. AU - Farrel, D. AU - McClure, S. AU - Austin, E. H. DB - Embase Medline DO - 10.1016/S0003-4975(03)01365-1 IS - 1 KW - lactic acid derivative aorta arterial carbon dioxide tension article assisted circulation blood flow cannula cardiopulmonary bypass child behavior clinical article confidence interval controlled study follow up heart assist device heart output heart right atrium hemodynamic monitoring hospital human hypoplastic left heart syndrome infant intensive care lung circulation mediastinitis neurologic examination neurology operating room oxygen tension perfusion postoperative care postoperative hemorrhage priority journal statistical significance surgical patient survival rate systemic circulation treatment outcome ultrafiltration United States ventilator LA - English M3 - Article N1 - L38129977 2004-02-17 PY - 2004 SN - 0003-4975 SP - 18-22 ST - Routine mechanical ventricular assist following the Norwood procedure - Improved neurologic outcome and excellent hospital survival T2 - Annals of Thoracic Surgery TI - Routine mechanical ventricular assist following the Norwood procedure - Improved neurologic outcome and excellent hospital survival UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L38129977 http://dx.doi.org/10.1016/S0003-4975(03)01365-1 VL - 77 ID - 1297 ER - TY - JOUR AB - The purpose of this study is to describe the process of parenting a child with life-threatening heart disease. Despite advances in pediatric cardiac sciences, hypoplastic left heart syndrome remains difficult and controversial to treat. The Norwood surgical approach is a developing technology, and little is known about how mothers and fathers experience parenting a child who survives this treatment. Constructivist grounded theory informed this Canadian study that involved multiple interactive interviews with 9 mothers and 7 fathers of children with hypoplastic left heart syndrome who were at various stages of treatment. Extraordinary parenting in a context of uncertainty was evident as parents simultaneously safeguarded their child's precarious survival as well as their own survival. As technologically advanced treatment contributes to the survival of children with complex health conditions, health care professionals must consider how to promote and support parenting strategies that benefit the new survivors of technology as well as their families. AD - Faculty of Nursing, University of Alberta, Edmonton, Canada. AN - 17582024 AU - Rempel, G. R. AU - Harrison, M. J. DA - Jul DB - PubMed DO - 10.1177/1049732307303164 DP - NLM ET - 2007/06/22 IS - 6 KW - Adult Alberta Cardiac Surgical Procedures/rehabilitation Child Disabled Children/*rehabilitation Female Home Nursing/*psychology Humans Hypoplastic Left Heart Syndrome/*surgery Interviews as Topic Male Narration *Parent-Child Relations Parenting/*psychology Qualitative Research Tape Recording LA - eng N1 - Rempel, Gwen R Harrison, Margaret J Journal Article Research Support, Non-U.S. Gov't United States Qual Health Res. 2007 Jul;17(6):824-37. doi: 10.1177/1049732307303164. PY - 2007 SN - 1049-7323 (Print) 1049-7323 SP - 824-37 ST - Safeguarding precarious survival: parenting children who have life-threatening heart disease T2 - Qual Health Res TI - Safeguarding precarious survival: parenting children who have life-threatening heart disease VL - 17 ID - 288 ER - TY - JOUR AB - PURPOSE: To determine the safety and feasibility of an inpatient exercise training program for a group of pediatric heart transplantation candidates on multiple inotropic support. BACKGROUND: Children with end-stage heart disease often require heart transplantation. Currently, no data exist on the safety and feasibility of an inpatient exercise training program in pediatric patients awaiting heart transplantation while on inotropic support. METHODS: Twenty ambulatory patients (11 male; age, 13.6 ± 3.2 years) were admitted, listed, and subsequently enrolled into an exercise training program while awaiting heart transplantation. Patient diagnoses consisted of dilated cardiomyopathy (n = 15), restrictive cardiomyopathy (n = 1), and failing single-ventricle physiology (n = 4). Inotropic support consisted of a combination of dobutamine, dopamine, or milrinone. Exercise sessions were scheduled three times a week lasting from 30 to 60 minutes and consisted of aerobic and musculoskeletal conditioning. RESULTS: Over 6.2 ± 4.2 months, 1,251 of a possible 1,508 exercise training sessions were conducted, with a total of 615 hours (26.3 ± 2.7 min/session) dedicated to low-intensity aerobic exercise. Reasons for noncompliance included a change in medical status, staffing, or patient cooperation. Two adverse episodes (seizures) occurred, neither of which resulted in termination from the program. No adverse episodes of hypotension or significant complex arrhythmias occurred. No complication of medication administration or loss of intravenous access occurred. CONCLUSION: Data from this study indicate that pediatric patients on inotropic support as a result of systemic ventricular or biventricular heart failure can safely participate in exercise training programs with relatively moderate to high compliance. © 2007 Lippincott Williams & Wilkins, Inc. AD - M.G. McBride, Division of Cardiology, Children's Hospital of Philadelphia, 34th and Civic Center Boulevard, Philadelphia, PA 19104, United States AU - McBride, M. G. AU - Binder, T. J. AU - Paridon, S. M. DB - Embase Medline DO - 10.1097/01.HCR.0000281766.59781.e8 IS - 4 KW - dobutamine dopamine inotropic agent milrinone adolescent adult aerobic exercise article child childhood disease clinical article congestive cardiomyopathy exercise female health status heart failure heart rehabilitation heart transplantation hospital patient human infant inotropism male patient compliance priority journal restrictive cardiomyopathy risk benefit analysis safety seizure LA - English M3 - Article N1 - L47222191 2007-07-01 PY - 2007 SN - 1932-7501 1932-751X SP - 219-222 ST - Safety and feasibility of inpatient exercise training in pediatric heart failure: A preliminary report T2 - Journal of Cardiopulmonary Rehabilitation and Prevention TI - Safety and feasibility of inpatient exercise training in pediatric heart failure: A preliminary report UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L47222191 http://dx.doi.org/10.1097/01.HCR.0000281766.59781.e8 VL - 27 ID - 1233 ER - TY - JOUR AB - Objective: We aimed to investigate the incidence and causes of readmission of infants with single-ventricle and shunt-dependent biventricular CHD following routine, outpatient cardiac catheterisation.Background Cardiac catheterisation is commonly performed in patients with single-ventricle and shunt-dependent biventricular CHD for haemodynamic assessment and surgical planning. Best practices for post-procedural care in this population are unknown, and substantial variation exists between centres. Outpatient catheterisation reduces parental anxiety and decreases cost. Our institutional strategy is to discharge patients following a 4- to 6-hour post-procedure observation period. Methods: Retrospective cohort study using the Society of Thoracic Surgeons Database identified patients <1 year of age with pre-stage II single-ventricle CHD or shunt-dependent biventricular CHD who underwent cardiac catheterisation between 2007 and 2015. Readmission was defined as admission to the hospital within 48 hours after discharge after catheterisation. Results: A total of 92 patients were included in the analysis. Among them, 62 patients (67%) were discharged after a 4- to 6-hour observation period with only 3% readmission, 18 patients (20%) stayed for a 23-hour observation period, and 12 patients (13%) were admitted for >23 hours. There were no differences in baseline characteristics between discharged and admitted patients. Patients who underwent intervention were more likely to be admitted. Patients with hypoplastic left heart syndrome did not have major adverse events or readmissions. No intra- or peri-procedural deaths occurred. Conclusions: Outpatient cardiac catheterisation may be a safe option for infants with single-ventricle and shunt-dependent biventricular CHD, with low readmission rates and minimal morbidity. AD - J.N. Colombo, Department of Pediatrics, Division of Pediatric Cardiology, University of Virginia Children's Hospital, University of Virginia, Charlottesville, VA, United States AU - Colombo, J. N. AU - Spaeder, M. C. AU - Hainstock, M. R. DB - Embase Medline DO - 10.1017/S1047951118001567 IS - 12 KW - article child care cohort analysis congenital heart disease controlled study disease severity female heart catheterization heart hemodynamics heart septum defect heart single ventricle high risk patient hospital discharge hospital readmission human hypoplastic left heart syndrome infant major clinical study male morbidity outcome assessment outpatient care patient safety retrospective study treatment planning LA - English M3 - Article N1 - L624435861 2018-10-24 2019-03-22 PY - 2018 SN - 1467-1107 1047-9511 SP - 1444-1451 ST - Safety of outpatient cardiac catheterisation in infants with single-ventricle or shunt-dependent biventricular congenital heart disease T2 - Cardiology in the Young TI - Safety of outpatient cardiac catheterisation in infants with single-ventricle or shunt-dependent biventricular congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L624435861 http://dx.doi.org/10.1017/S1047951118001567 VL - 28 ID - 651 ER - TY - JOUR AB - Background Expanded use of pediatric ventricular assist devices (VADs) has decreased mortality in children awaiting heart transplantation. Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs), a National Heart, Lung, and Blood Institute–sponsored North American database, provides a platform to understand this emerging population. Methods Between September 2012 and September 2016, patients aged younger than 19 years who underwent VAD implantation were enrolled in Pedimacs. FDA approved durable devices as well as temporary support devices were included. The second annual report updates the current Pedimacs data. Patients implanted with temporary devices are included in Pedimacs and this analysis includes this group of paracorporeal continuous flow VADs. Results Over the 4 years, 42 hospitals implanted 432 devices in 364 patients less than 19 yrs of age. Diagnoses included cardiomyopathy in 223 (61%), myocarditis in 41 (11%), and congenital heart disease in 77 (21%), of which 48 had single-ventricle physiology. At implant, 87% were at Intermacs patient profile 1 or 2. The age distribution of children (59% male) supported on VAD included 69 (19%) aged younger than 1 year, 66 (18%) aged 1 to 6 years, 56 (15%) aged 6 to 10 years, and 173 (48%) aged 11 to 19 years. Median follow-up was 2.2 months (range, 1 day to 41.5 months). Median (interquartile) age at implant was 1.7 (0.3–10.0) years for paracorporeal continuous-flow pumps (n = 60), 1.7 (0.4–5.3) years for paracorporeal pulsatile pumps (n = 105), and 15.0 (11.3–16.9) years for implantable continuous-flow pumps (n = 174). Support strategies included LVAD in 293 (80%), biventricular device in 55 (15%), and total artificial heart in 8 (2%). Nearly 50% of patients underwent transplantation within 6 months, with overall mortality of 19%. Adverse event burden continues to be high. Conclusions Pedimacs constitutes the largest longitudinal pediatric VAD registry. Preimplant data across centers will be helpful at creating shared protocols with which to improve outcomes. Adverse events continue to be the major challenge, especially among the young critically ill children with complex congenital disease. AD - E.D. Blume, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, United States AU - Blume, E. D. AU - VanderPluym, C. AU - Lorts, A. AU - Baldwin, J. T. AU - Rossano, J. W. AU - Morales, D. L. S. AU - Cantor, R. S. AU - Miller, M. A. AU - St. Louis, J. D. AU - Koehl, D. AU - Sutcliffe, D. L. AU - Eghtesady, P. AU - Kirklin, J. K. AU - Rosenthal, D. N. DB - Embase Medline DO - 10.1016/j.healun.2017.06.017 IS - 1 KW - biventricular assist device left ventricular assist device adolescent adult article assisted circulation cardiomyopathy cause of death child congenital heart disease controlled study female follow up heart transplantation human infant longitudinal study male mechanical circulatory support mortality myocarditis preschool child priority journal quality of life school child young adult LA - English M3 - Article N1 - L618682936 2017-10-13 2018-01-16 PY - 2018 SN - 1557-3117 1053-2498 SP - 38-45 ST - Second annual Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) report: Pre-implant characteristics and outcomes T2 - Journal of Heart and Lung Transplantation TI - Second annual Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) report: Pre-implant characteristics and outcomes UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L618682936 http://dx.doi.org/10.1016/j.healun.2017.06.017 VL - 37 ID - 713 ER - TY - JOUR AB - Background: Large databases and population registers are increasingly used to examine adverse birth outcomes, congenital heart anomalies, in particular, following antidepressant exposures in pregnancy. Yet many studies have failed to account for other characteristics of the women who were prescribed antidepressants. Objective: To examine the characteristics of women who are prescribed selective serotonin reuptake inhibitors (SSRIs) in pregnancy and women who are not, associations between SSRIs prescribed in pregnancy and congenital heart anomalies, and the association between social and lifestyle characteristics of pregnant women and congenital heart anomalies. Method: Using data from The Health Improvement Network primary care database in the United Kingdom between January 1, 1990, and January 31, 2011, we set up a comparative study including 4 cohorts of children of women with and without different antidepressant exposures before and during pregnancy. 5,154 women were receiving SSRIs before pregnancy, 2,776 were receiving SSRIs during pregnancy, 992 were receiving other antidepressants during pregnancy, and 200,213 were receiving no antidepressants before or during pregnancy. Our primary outcome was congenital heart anomalies. Results: Less than 1% of children had a record of congenital heart anomalies within 5 years of birth, and there were no significant differences related to antidepressant exposure in pregnancy (women not prescribed antidepressants versus women prescribed SSRIs in first trimester: odds ratio [OR] = 1.00; 95% CI, 0.65-1.52); however, independent of antidepressant prescribing, diabetes (OR = 2.23; 95% CI, 1.79-2.77), increasing age (OR = 1.01; 95% CI, 1.00-1.02), alcohol problem (OR = 2.58; 95% CI, 1.55-4.29, illicit drug problems (OR = 1.89; 95% CI, 1.09-3.25), and obesity (OR = 1.38; 95% CI, 1.13-1.69) were associated with an increased risk of having a child with congenital heart anomalies. Conclusions: There was no difference in congenital heart anomalies in children born to women with different antidepressant prescribing exposure status. However, we confirmed an increased risk of congenital heart anomalies in children of older women and in children of women with diabetes, a body mass index above 30 kg/m2, and a history of alcohol and illicit drug problems independent of the prescription of antidepressants. Future research in this field must account for these characteristics. On the basis of existing evidence, advising women to stop antidepressant treatment in pregnancy may be counterproductive. AD - I. Petersen, Department of Primary Care and Population Health, University College London, Rowland Hill St, London, United Kingdom AU - Petersen, I. AU - Evans, S. J. AU - Gilbert, R. AU - Marston, L. AU - Nazareth, I. DB - Embase Medline DO - 10.4088/JCP.14m09241 IS - 1 KW - amitriptyline anticonvulsive agent antidepressant agent anxiolytic agent citalopram clomipramine dosulepin escitalopram fluoxetine hypnotic agent illicit drug lofepramine neuroleptic agent paroxetine psychotropic agent serotonin uptake inhibitor sertraline venlafaxine adult aging alcoholism aortic arch anomaly aortic coarctation aortic stenosis subvalvular aortic stenosis aortic valve disease aortic regurgitation aortic valve stenosis article atrioventricular septal defect bicuspid aortic valve body mass child cohort analysis congenital heart malformation controlled study cyanotic heart disease depression dextrocardia diabetes mellitus drug dependence Ebstein anomaly Fallot tetralogy female first trimester pregnancy great vessels transposition heart atrium septum defect heart ventricle septum defect human hypoplastic left heart syndrome independent variable lifestyle major clinical study male medical history medical record mitral valve regurgitation obesity patent foramen ovale pregnant woman prenatal drug exposure prescription primary medical care priority journal pulmonary artery malformation pulmonary artery stenosis pulmonary valve atresia pulmonary valve disease pulmonary valve stenosis risk assessment social aspect tricuspid valve regurgitation United Kingdom LA - English M3 - Article N1 - L608293401 2016-02-17 2016-02-23 PY - 2016 SN - 0160-6689 SP - e36-e42 ST - Selective serotonin reuptake inhibitors and congenital heart anomalies: Comparative cohort studies of women treated before and during pregnancy and their children T2 - Journal of Clinical Psychiatry TI - Selective serotonin reuptake inhibitors and congenital heart anomalies: Comparative cohort studies of women treated before and during pregnancy and their children UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L608293401 http://dx.doi.org/10.4088/JCP.14m09241 VL - 77 ID - 834 ER - TY - JOUR AB - Aim: To assess together with their parents, the quality-of-life of the teenage patients who had undergone open heart surgery because of cyanotic heart disease during their infancy and to compare their self-perception to that of physically healthy control group. Material and Method: This study includes 53 patients who had undergone operation for cyanotic congenital heart disease in Istanbul University, Institute of Cardiology, between 1989-1994. The relatives and the patients were informed with a consent form approved by Istanbul University, Cerrahpaşa Medical Faculty, Clinical Studies, Ethics Commitee (Decree no. D-005, Date: 11-10-2009). Group 1 was the healthy control group (15 patients, median age: 19 years, 8 months), Group 2 included patients with single ventricle who had undergone Fontan procedure (20 patients, median age: 19 years, 6 months; 10 of them had tricuspid atresia, five of them had pulmonary atresia with intact ventricular septum, three of them had double inlet left ventricle, one of them had double outlet right ventricle, one of them had ventricular septal defect and pulmonary atresia), group 3 included patients who had undergone operation for congenital cyanotic heart disease (33 patients, median age: 19 years, 2 months; 18 of them had transposition of the great arteries, 10 of them had tetralogy of Fallot, two of them had complete atrioventricular canal defect and pulmonary stenosis, two of them had double outlet right ventricle, one of them had pentalogy of Fallot). In this study, the quality of life and self perception scales were used. Quality of life scale was filled by patients, healthy adolescents and their parents separately. Self perception scale was only filled by patients and healthy adolescents. The comparison of age and self perception scores between the groups was carried out with unidirectional Analysis of variance (ANOVA) and the multicomparisons with LSD (Least Significant Difference). Since the quality of life variable does not have a normal distribution, the comparions were carried out with Kruskal-Wallis nonparametric ANOVA test and the multicomparisons with Dunn's test. Results: There was not a significant difference between the groups in terms of age and sex. In the quality of life scale (QOLS), for the replies to related questions, a significant difference was determined between the groups. The replies of group 2 and 3 for about quality of life of both children and the parents were far more negative when compared to those of the control group. When the groups were compared via multiple comparison tests, there was no significant difference between group 2 and 3. The replies of the parents regarding their children's quality of life, were statistically more negative than those of their children (p<0.001). Within the self perception scale, between group 1 and 3, there were significant differences about the social acceptance, behaviours in relationships and general self perception (p=0.03, p=0.03 and p=0.01; respectively). The most significant difference that was detected between group 1 and 2, was about the behaviour in relationships (p=0.04). Conclusions: With the help of developing technology, and surgical experience, many complex cyanotic heart diseases are treated successfully and most patients reach puberty. However the responses for quality of life and self perception paremeters of this group of patients are more negative than those of the control group. Therefore, these adolescents and especially their parents may need psychosocial support. AD - C. E. Yildiz, Istanbul Üniversitesi Kardiyoloji Enstitüsü, Kalp ve Damar Cerrahisi Anabilim Dali, Istanbul, Turkey AU - Yildiz, C. E. AU - Zahmacioǧlu, O. AU - Koca, B. AU - Oktay, V. AU - Gökalp, S. AU - Eroǧlu, A. G. AU - Çetin, G. AU - Öztunç, F. DB - Embase DO - 10.4274/tpa.46.78 IS - 3 KW - adolescent adult article controlled study cyanotic heart disease endocardial cushion defect fallot pentalogy Fallot tetralogy Fontan procedure great vessels transposition heart left ventricle double inlet heart right ventricle double outlet heart single ventricle heart ventricle septum defect human human relation major clinical study open heart surgery pediatric surgery pulmonary valve atresia pulmonary valve stenosis quality of life rating scale self concept social behavior social interaction tricuspid valve atresia Turkey (republic) university hospital LA - English Turkish M3 - Article N1 - L362761852 2011-10-25 2011-10-27 PY - 2011 SN - 1308-6278 1306-0015 SP - 220-227 ST - Self perception and quality of life of adolescents who had undergone open-heart surgery due to cyanotic congenital heart disease in their infancy T2 - Turk Pediatri Arsivi TI - Self perception and quality of life of adolescents who had undergone open-heart surgery due to cyanotic congenital heart disease in their infancy UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L362761852 http://dx.doi.org/10.4274/tpa.46.78 VL - 46 ID - 1122 ER - TY - JOUR AB - AIMS: The aim of this study is to compare self-reported health-related quality-of-life (HRQoL) with the objective of exercise performance in patients with congenital heart disease (CHD) according to diagnosis. METHODS AND RESULTS: 564 patients (255 females, 14-73 years) with various CHD (62 shunt, 66 left heart obstruction, 33 PS/PR, 47 Ebstein, 96 Fallot, 98 TGA after atrial switch, 38 other TGA, 31 Fontan, 32 palliated/native cyanotic, 61 others) and a group of 53 healthy controls (18 females, 14-57 years) completed a QoL questionnaire (SF-36) and performed a symptom-limited cardiopulmonary exercise test. Despite several limitations at exercise (P = 1.30 x 10(-33)), patients only reported reductions in HRQoL concerning physical functioning (P = 4.41 x 10(-15)) and general health (P = 6.17 x 10(-5)) and not psychosocial aspects. This could be confirmed in all diagnostic subgroups. Correlation to peak oxygen uptake was found in physical functioning (r = 0.435, P = 1.72 x 10(-27)) and general health (r = 0.275, P = 3.79 x 10(-11)). However, there was severe overestimation of physical functioning in most patients when compared with actual exercise test results. CONCLUSION: Patients with CHD rate their HRQoL impaired only in physical functioning and general health and not in any psychosocial aspect. Self-estimated physical functioning poorly predicts actual exercise capacity. AD - Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, München, Germany. AN - 19066211 AU - Gratz, A. AU - Hess, J. AU - Hager, A. DA - Feb DB - PubMed DO - 10.1093/eurheartj/ehn531 DP - NLM ET - 2008/12/11 IS - 4 KW - Adolescent Adult Aged Attitude to Health Exercise Test/methods Exercise Tolerance/*physiology Female Health Status Indicators Heart Defects, Congenital/*physiopathology/psychology/rehabilitation Humans Male Middle Aged Oxygen Consumption/physiology Quality of Life/psychology Self Concept Surveys and Questionnaires Young Adult LA - eng N1 - 1522-9645 Gratz, Alexander Hess, John Hager, Alfred Journal Article England Eur Heart J. 2009 Feb;30(4):497-504. doi: 10.1093/eurheartj/ehn531. Epub 2008 Dec 9. PY - 2009 SN - 0195-668x SP - 497-504 ST - Self-estimated physical functioning poorly predicts actual exercise capacity in adolescents and adults with congenital heart disease T2 - Eur Heart J TI - Self-estimated physical functioning poorly predicts actual exercise capacity in adolescents and adults with congenital heart disease VL - 30 ID - 364 ER - TY - JOUR AB - PURPOSE: Today, more patients with congenital heart disease (CHD) reach adulthood. There are conflicting findings concerning the relationship between quality of life (QoL) or health state for adults with CHD and the complexity of their CHD. The aim of the study was, firstly, to compare the reported health status and health perception of adult patients with CHD and, secondly, to investigate what variables influenced the patients' health status and health perception. METHODS: Data from 1435 patients completing the EQ-5D questionnaire, which includes reported health status and health perception, were analyzed. RESULTS: Valid EQ-5D data were reported by 1274 patients, showing overall results indicating a good health status. Problems were most frequently reported in the dimension "pain/discomfort" (31.9%) and "anxiety/depression" (29.8%). Higher occurrence of problems were reported by patients with complex disease i.e. single ventricle (p<0.001) and by female patients (p<0.0001). Symptomatic patients reported a lower health status (p<0.0001) and a lower perceived health on EQ-VAS (p<0.0001). Of the asymptomatic patients, 20.5% nevertheless reported problems in "pain/discomfort" and 22.2% in the "anxiety/depression" dimension. CONCLUSION: The health status of adults with CHD is influenced by symptoms, NYHA-classification, age and gender. Adults with CHD report a lower occurrence of problems in comparison to previously published results from a general population, but the importance of actively asking about the patient's experience is demonstrated by the high degree of asymptomatic patients reporting problems on EQ-5D. AD - The Sahlgrenska Academy at University of Gothenburg, Institute of Health and Care Sciences, Box 457, SE-40530 Gothenburg, Sweden. malin.berghammer@gu.se AN - 22051437 AU - Berghammer, M. AU - Karlsson, J. AU - Ekman, I. AU - Eriksson, P. AU - Dellborg, M. DA - May 25 DB - PubMed DO - 10.1016/j.ijcard.2011.10.002 DP - NLM ET - 2011/11/05 IS - 3 KW - Adolescent Adult Aged Aged, 80 and over Female *Health Status Heart Defects, Congenital/*diagnosis/epidemiology/psychology Humans Longitudinal Studies Male Middle Aged Registries/*standards Self Report/*standards Young Adult LA - eng N1 - 1874-1754 Berghammer, Malin Karlsson, Jan Ekman, Inger Eriksson, Peter Dellborg, Mikael Journal Article Research Support, Non-U.S. Gov't Netherlands Int J Cardiol. 2013 May 25;165(3):537-43. doi: 10.1016/j.ijcard.2011.10.002. Epub 2011 Nov 1. PY - 2013 SN - 0167-5273 SP - 537-43 ST - Self-reported health status (EQ-5D) in adults with congenital heart disease T2 - Int J Cardiol TI - Self-reported health status (EQ-5D) in adults with congenital heart disease VL - 165 ID - 312 ER - TY - JOUR AB - Background: Sertraline, a selective serotonin reuptake inhibitor (SSRI), is the most commonly prescribed therapy for maternal depression. Epidemiologic studies have linked SSRI exposure with decreased fetal growth, altered autonomic regulation, and cardiac malformations. We hypothesized that SSRI exposure decreases left-ventricular (LV) volumes and increases adult sympathetic nervous system activation, resulting in increased adult heart rates.Methods:C57BL/6 mice received saline or sertraline (5 or 15 mg/kg/day i.p.) on postnatal days 1-14. Adult phenotypes were assessed at 5 mo.Results:Sertraline-exposed mice had smaller LV internal diameters in diastole (control 4.0 ± 0.1 mm, SSRI 3.7 ± 0.1 mm, P < 0.05), decreased stroke volumes (control 46 ± 2.6, SSRI 37 ± 2.3, P < 0.05), higher heart rates (control 530 ± 13 beats per minute (bpm), SSRI 567 ± 6 bpm, P <0.05), and increased urinary excretion of noradrenaline (control 174 ± 29.4 ng/ml, SSRI 276 ± 35.1 ng/ml, P < 0.05). These changes were associated with increased cerebral serotonin transporter (5-HTT) expression.Conclusion:Neonatal sertraline exposure causes long-term changes in cardiac morphology and physiology. We speculate that early-life SSRI exposure impairs cardiomyocyte growth and central serotonin signaling, leading to a small left heart syndrome in adult mice. Copyright © 2013 International Pediatric Research Foundation, Inc. AD - Department of Pediatrics, University of Iowa Carver, College of Medicine, Iowa City, IA, United States AU - Haskell, S. E. AU - Hermann, G. M. AU - Reinking, B. E. AU - Volk, K. A. AU - Peotta, V. A. AU - Zhu, V. AU - Roghair, R. D. DB - Scopus DO - 10.1038/pr.2012.183 IS - 3 M3 - Article N1 - Cited By :16 Export Date: 15 June 2020 PY - 2013 SP - 286-293 ST - Sertraline exposure leads to small left heart syndrome in adult mice T2 - Pediatric Research TI - Sertraline exposure leads to small left heart syndrome in adult mice UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84874040296&doi=10.1038%2fpr.2012.183&partnerID=40&md5=3a4fd9c72db7cf74e95c83ea5cebba46 VL - 73 ID - 2061 ER - TY - JOUR AB - We describe an unusual case of a patient with polysplenia, inferior vena cava (IVC) interruption, and unrecognized congenital portosystemic venous connection (CPVC) in whom severe cyanosis developed in the acute postoperative stage after a Kawashima operation and required urgent surgical division of the CPVC. It has been stressed recently that CPVCs and other abdominal venous abnormalities have been underdiagnosed in patients with polysplenia and IVC interruption, and we think that a routine evaluation of abdominal venous connections should be mandatory before considering the Kawashima operation for these patients. © 2012 The Society of Thoracic Surgeons. AD - Y. Nakamura, Department of Cardiovascular Surgery, Chiba Children's Hospital, 579-1, Heta, Midori, Chiba 266-0007, Japan AU - Nakamura, Y. AU - Aoki, M. AU - Hagino, I. AU - Nakajima, H. DB - Embase Medline DO - 10.1016/j.athoracsur.2011.08.058 IS - 4 KW - contrast medium nitric oxide accessory spleen ambient air arterial oxygen saturation article case report central venous pressure computer assisted tomography congenital portosystemic shunt contrast enhancement cyanosis disease severity echocardiography Fontan procedure heart atrium pressure heart catheterization heart left right shunt heart right ventricle double outlet liver vein human infant inferior cava vein internal jugular vein Kawashima operation kidney vein laparotomy lung artery pressure lung blood flow male newborn postoperative period priority journal pulmonary valve stenosis splenic vein superior cava vein surgical technique thorax surgery LA - English M3 - Article N1 - L364521941 2012-04-04 2012-04-13 PY - 2012 SN - 0003-4975 1552-6259 SP - 1315-1317 ST - Severe cyanosis after kawashima operation resulting from congenital portosystemic shunt T2 - Annals of Thoracic Surgery TI - Severe cyanosis after kawashima operation resulting from congenital portosystemic shunt UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L364521941 http://dx.doi.org/10.1016/j.athoracsur.2011.08.058 VL - 93 ID - 1062 ER - TY - JOUR AB - Potocki-Lupski syndrome (PTLS) is a recently described microduplication syndrome associated with duplication 17p11.2, including the RAI1 gene. Features of PTLS include hypotonia, feeding difficulties, failure to thrive, developmental delay and behavioral abnormalities including autistic spectrum disorder, anxiety, and inattention. Cardiovascular anomalies were not recognized as a feature of duplication 17p11.2 until 2007 when noted in over 50% of a clinically characterized cohort. We report a patient with hypoplastic left heart syndrome whose diagnosis of PTLS was delayed until a genetic evaluation at age 4 years because of severe expressive language impairment. We suggest that array comparative genomic hybridization be performed in infants with severe congenital heart defects. AD - Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas, USA. AN - 21271655 AU - Sanchez-Valle, A. AU - Pierpont, M. E. AU - Potocki, L. C2 - PMC3092288 C6 - NIHMS255756 DA - Feb DB - PubMed DO - 10.1002/ajmg.a.33844 DP - NLM ET - 2011/01/29 IS - 2 KW - Abnormalities, Multiple Child Chromosome Disorders Chromosome Duplication Comparative Genomic Hybridization/*methods Humans Hypoplastic Left Heart Syndrome/*pathology Male Smith-Magenis Syndrome/genetics/pathology LA - eng N1 - 1552-4833 Sanchez-Valle, Amarilis Pierpont, Mary Ella Potocki, Lorraine K08 HD001149-04/HD/NICHD NIH HHS/United States K08 HD001149-05/HD/NICHD NIH HHS/United States M01 RR000188/RR/NCRR NIH HHS/United States M01RR00188/RR/NCRR NIH HHS/United States Case Reports Journal Article Research Support, N.I.H., Extramural Am J Med Genet A. 2011 Feb;155A(2):363-6. doi: 10.1002/ajmg.a.33844. Epub 2011 Jan 13. PY - 2011 SN - 1552-4825 (Print) 1552-4825 SP - 363-6 ST - The severe end of the spectrum: Hypoplastic left heart in Potocki-Lupski syndrome T2 - Am J Med Genet A TI - The severe end of the spectrum: Hypoplastic left heart in Potocki-Lupski syndrome VL - 155a ID - 412 ER - TY - JOUR AB - BACKGROUND: Kleefstra syndrome arises from haploinsufficiency of EHMT1 caused by either microdeletions at 9q34.3 or intragenic mutations. Patients with Kleefstra syndrome have multisystem involvement including intellectual disability, hypotonia, and characteristic facial features. METHODS: We report on the severe neonatal presentation of the first case of Kleefstra syndrome associated with hypoplastic left heart syndrome and multicystic renal disease in a patient with a 9q34.3 microdeletion. RESULTS: Array-CGH analysis revealed a 2.1 Mb deletion at 9q34.3, including EHMT1 and NOTCH1. CONCLUSION: Kleefstra syndrome is a multisystem disorder with a high frequency of congenital heart disease and less frequently, renal defects. Mortality has rarely been documented, particularly in infancy. Based on the present case and the extant literature, a routine echocardiogram and renal ultrasound should be ordered in all cases of Kleefstra syndrome. The cardiac changes seen in this patient could be the result of the haploinsufficiency of EHMT1, NOTCH1, or their combined effect. AD - University of Arkansas for Medical Sciences, Department of Pediatrics, Little Rock, Arkansas; University of Arkansas for Medical Sciences, Division of Cardiology, Little Rock, Arkansas. AN - 25380126 AU - Campbell, C. L. AU - Collins, R. T., 2nd AU - Zarate, Y. A. DA - Dec DB - PubMed DO - 10.1002/bdra.23324 DP - NLM ET - 2014/11/08 IS - 12 KW - Adult Chromosome Deletion Chromosomes, Human, Pair 9/*genetics Comparative Genomic Hybridization Craniofacial Abnormalities/diagnosis/*genetics Female Heart Defects, Congenital/diagnosis/*genetics Histone-Lysine N-Methyltransferase/*genetics Humans Hypoplastic Left Heart Syndrome/*genetics Infant, Newborn Intellectual Disability/diagnosis/*genetics Multicystic Dysplastic Kidney/diagnosis/genetics Mutation/genetics Receptor, Notch1/*genetics *Sequence Deletion Young Adult 9q34.3 microdeletion Kleefstra syndrome chromosomal microarray hypoplastic left heart syndrome multicystic renal disease LA - eng N1 - 1542-0760 Campbell, Candace L Collins, R Thomas 2nd Zarate, Yuri A Case Reports Journal Article United States Birth Defects Res A Clin Mol Teratol. 2014 Dec;100(12):985-90. doi: 10.1002/bdra.23324. Epub 2014 Nov 7. PY - 2014 SN - 1542-0752 SP - 985-90 ST - Severe neonatal presentation of Kleefstra syndrome in a patient with hypoplastic left heart syndrome and 9q34.3 microdeletion T2 - Birth Defects Res A Clin Mol Teratol TI - Severe neonatal presentation of Kleefstra syndrome in a patient with hypoplastic left heart syndrome and 9q34.3 microdeletion VL - 100 ID - 297 ER - TY - JOUR AD - Department of Pediatric Cardiology and Pulmonology, Heinrich Heine University, Moorenstrasse 5, 40225, Duesseldorf, Germany. Alexander.Kovacevic@med.uni-duesseldorf.de AN - 22383164 AU - Kovacevic, A. AU - Schmidt, K. G. AU - Schaper, J. AU - Messing-Juenger, M. AU - Hoehn, T. DA - Oct DB - PubMed DO - 10.1007/s00381-012-1726-8 DP - NLM ET - 2012/03/03 IS - 10 KW - Child, Preschool Craniocerebral Trauma/surgery Fontan Procedure/*methods Heart Ventricles/surgery Hematoma, Epidural, Cranial/surgery Humans Male Neurosurgery/*methods Tomography, X-Ray Computed Wound Healing/*physiology LA - eng N1 - 1433-0350 Kovacevic, Alexander Schmidt, Klaus G Schaper, Joerg Messing-Juenger, Martina Hoehn, Thomas Case Reports Journal Article Germany Childs Nerv Syst. 2012 Oct;28(10):1815-7. doi: 10.1007/s00381-012-1726-8. Epub 2012 Mar 1. PY - 2012 SN - 0256-7040 SP - 1815-7 ST - Severely impaired wound healing in a Fontan patient after neurosurgery: a novel topic in univentricular heart physiology? T2 - Childs Nerv Syst TI - Severely impaired wound healing in a Fontan patient after neurosurgery: a novel topic in univentricular heart physiology? VL - 28 ID - 498 ER - TY - JOUR AB - Single-ventricle congenital heart disease (SVCHD) requires multiple palliative surgical procedures that leave visible surgical scars and physical deficits, which can alter body-image and self-esteem. This study aimed to compare sex and age differences in body-image, self-esteem, and body mass index (BMI) in adolescents and adults with SVCHD after surgical palliation with those of a healthy control group. Using a comparative, cross-sectional design, 54 adolescent and adult (26 male and 28 female) patients, age 15–50 years, with SVCHD were compared with 66 age-matched healthy controls. Body-image and self-esteem were measured using the Multidimensional Body-Self Relations Questionnaire–Appearance Scale and Rosenberg Self-Esteem Scale. Height and weight were collected from retrospective chart review, and BMI was calculated. Female adolescents and adult patients with SVCHD reported lower body image compared with males patients with SVCHD and healthy controls (p = 0.003). Specific areas of concern were face (p = 0.002), upper torso or chest (p = 0.002), and muscle tone (p = 0.001). Patients with SVCHD who were \21 years of age had lower body image compared with healthy controls (p = 0.006). Self-esteem was comparable for both patients with SVCHD and healthy peers. There were no sex differences in BMI; BMI was higher in subjects[21 years of age (p = 0.01). Despite the similarities observed in self-esteem between the two groups, female patients with SVCHD\21 years of age reported lower perceived body-image. Our findings support the need to recognize poor psychological adjustment related to low self-esteem in patients with SVCHD; female patients warrant increased scrutiny. Strategies to help patients with SVCHD cope with nonmodifiable aspects of body-image during the difficult adolescent–to–young adult years may potentially enhance self-esteem and decrease psychological distress. AD - School of Nursing, University of California, Factor Building Room 3-938, Los Angeles, CA 90095-6919, USA. npike@sonnet.ucla.edu AN - 22314368 AU - Pike, N. A. AU - Evangelista, L. S. AU - Doering, L. V. AU - Eastwood, J. A. AU - Lewis, A. B. AU - Child, J. S. C2 - PMC3396742 C6 - NIHMS384265 DA - Jun DB - PubMed DO - 10.1007/s00246-012-0198-7 DP - NLM ET - 2012/02/09 IS - 5 KW - Adolescent Adult Age Factors Analysis of Variance *Body Image *Body Mass Index Case-Control Studies Cross-Sectional Studies Female Heart Defects, Congenital/*psychology/*surgery Humans Male *Palliative Care *Self Concept Sex Factors Surveys and Questionnaires Treatment Outcome LA - eng N1 - 1432-1971 Pike, Nancy A Evangelista, Lorraine S Doering, Lynn V Eastwood, Jo-Ann Lewis, Alan B Child, John S R01 HL093466/HL/NHLBI NIH HHS/United States Comparative Study Journal Article Research Support, Non-U.S. Gov't Pediatr Cardiol. 2012 Jun;33(5):705-12. doi: 10.1007/s00246-012-0198-7. PY - 2012 SN - 0172-0643 (Print) 0172-0643 SP - 705-12 ST - Sex and age differences in body-image, self-esteem, and body mass index in adolescents and adults after single-ventricle palliation T2 - Pediatr Cardiol TI - Sex and age differences in body-image, self-esteem, and body mass index in adolescents and adults after single-ventricle palliation VL - 33 ID - 266 ER - TY - JOUR AB - BACKGROUND: The combination of pulmonary valve atresia and ventricular septal defect accounts for about 2% of cases of congenital heart disease. Most of the cases have intracardiac anatomy of Tetralogy Fallot and present with cyanosis in neonatal age. OBJECTIVES: To report a case of a newborn with rare combination of pulmonary atresia, ventricular septal defect and single ventricle physiology presenting with shock very early following birth. METHODS AND RESULTS: We describe a newborn infant who developed shock with severe metabolic acidosis and respiratory distress several hours following birth. Cardiac ultrasound showed pulmonary atresia and ventricular septal defect with single ventricle physiology. Cardiac cathetrisation revealed major aorto-pulmonary collaterals with an excessive pulmonary blood flow. Resuscitative measures resulted in hemodynamic stability. However, due to unfavorable prognosis, a decision not to undertake surgical palliation was made. Withdrawal of intensive care led to rapid demise of the patient. CONCLUSIONS: We report a case of a newborn with pulmonary atresia, ventricular septal defect and single ventricle physiology in whom an excessive flow through major aorto-pulmonary collaterals led to shock and death very early following birth (Fig. 1, Ref. 5). Full Text (Free, PDF) www.bmj.sk. AD - L. Kovacikova, Intensive Care Unit, Pediatric Cardiac Center, Bratislava, Slovakia. AU - Kovacikova, L. AU - Skrak, P. AU - Zahorec, M. AU - Masura, J. DB - Medline IS - 5 KW - article case report collateral circulation congenital malformation heart septum defect heart ventricle human lung male newborn pulmonary valve atresia shock vascularization LA - English M3 - Article N1 - L352239592 2008-09-03 PY - 2008 SN - 0006-9248 SP - 202-203 ST - Shock--manifestation of pulmonary atresia with aorto-pulmonary collaterals and single ventricle physiology in a 2-day neonate T2 - Bratislavské lekárske listy TI - Shock--manifestation of pulmonary atresia with aorto-pulmonary collaterals and single ventricle physiology in a 2-day neonate UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L352239592 VL - 109 ID - 1199 ER - TY - JOUR AB - The case reports of 492 infants with critical congenital cardiac disease treated with prostaglandin E1 (PGE1) were reviewed to determine the nature and incidence of intercurrent medical events. 43% of the infants had at least one such event, but only half of these were related to PGE1 and the majority required only minor changes in management. Cardiovascular events were the most common (18% incidence), with cutaneous vasodilation and edema occurring more frequently during intraaortic infusion than during i.v. infusion. Central nervous system events were reported in 16% of the patients. Respiratory depression as reported in 12%, and was particularly common in infants weighing less than 2.0 kg at birth (42%). Hematologic, infectious and renal events appeared for the most part to be unrelated to PGE1. The overall mortality (excluding 19 patients with hypoplastic left-heart syndrome) was 31%; the mortality for the patients with critical coarctation or interruption of the aortic arch was nearly twice that for the cyanotic infants (50% vs 27%). No deaths was attributed to PGE1 administration. During infusion of PGE1, arterial blood pressure and respiratory activity should be monitored carefully and appropriate supportive steps taken if hypotension or respiratory depression occurs. The development of fever or jitteriness may require reduction of the infusion rate and, in view of the possible increased incidence of infections, the prophylactic use of antibiotics is recommended. AD - Div. Cardiol., Child. Hosp., Los Angeles, CA AU - Lewis, A. B. AU - Freed, M. D. AU - Heymann, M. A. DB - Embase Medline DO - 10.1161/01.CIR.64.5.893 IS - 5 KW - prostaglandin E1 adverse drug reaction congenital heart malformation drug therapy edema heart hypoplastic left heart syndrome intoxication intraarterial drug administration intravenous drug administration major clinical study nervous system neurotoxicity newborn patent ductus arteriosus respiration depression respiratory system therapy vasodilatation LA - English M3 - Article N1 - L11028084 1981-12-29 PY - 1981 SN - 0009-7322 SP - 893-898 ST - Side effects of therapy with prostaglandin E1 in infants with critical congenital heart disease T2 - Circulation TI - Side effects of therapy with prostaglandin E1 in infants with critical congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L11028084 http://dx.doi.org/10.1161/01.CIR.64.5.893 VL - 64 ID - 1399 ER - TY - JOUR AB - OBJECTIVE: The population of children who received right ventricle-to-pulmonary artery shunt (RV-PA) at Norwood procedure (NP) is growing, but limited data are available regarding the long-term outcome. The aim of this study was to present operative outcomes and mid-term hemodynamics and to assess the impact of the residual aortic obstruction on the results in patients undergoing three-staged surgery with RV-PA application at NP. METHODS: Between June 2001 and June 2009, 229 children with hypoplastic left heart syndrome (HLHS) and variants underwent NP with RV-PA; 172 have proceeded to stage II and 95 to stage III. The medical records (clinical data, echocardiographic records, cardiac catheterization reports, electrocardiograms, and surgical notes) were retrospectively reviewed. RESULTS: The later era of NP was associated with significantly better outcome (2001-2004: 1- and 5-years' survival were 64.3% and 59.8%, respectively; 2005-2009: 1- and 4-years' survival were 93.1% and 86.9%, respectively) (p<0.001). There was no association between long-term survival and diagnosis (HLHS/HLHS variant) (p=0.39). The incidence of depressed ventricular function and moderate or severe systemic atrioventricular valve regurgitation among the children who required balloon aortoplasty (BA) before stage II surgery was significantly higher than in children without aortic arch obstruction (p=0.027, p=0.008, respectively). In midterm follow-up, BA had no significant influence on the actuarial survival (p=0.089). No ventricular arrhythmias were noticed at any stage. CONCLUSIONS: The RV-PA shunt is a safe technique that does not seem to impair systolic or electrical ventricular function; its outcomes continue to improve with growing experience. Combined cardiologic interventional and surgical procedures are required to optimize the outcomes. AD - Department of Cardiac Surgery, Klinikum Grosshadern, Ludwig Maximilians University, Munich, Germany. mijanusz@cyf-kr.edu.pl AN - 21295990 AU - Januszewska, K. AU - Kozlik-Feldmann, R. AU - Kordon, Z. AU - Urschel, S. AU - Netz, H. AU - Reichart, B. AU - Malec, E. DA - Aug DB - PubMed DO - 10.1016/j.ejcts.2010.12.023 DP - NLM ET - 2011/02/08 IS - 2 KW - Aortic Valve Stenosis/*complications/physiopathology/therapy Cardiac Catheterization Catheterization/methods Epidemiologic Methods Female Fontan Procedure/adverse effects/methods Heart Ventricles/surgery Hemodynamics Humans Hypoplastic Left Heart Syndrome/physiopathology/*surgery Infant Infant, Newborn Male Norwood Procedures/adverse effects/*methods Pulmonary Artery/surgery Treatment Outcome Tricuspid Valve Insufficiency/etiology Ventricular Dysfunction/etiology LA - eng N1 - 1873-734x Januszewska, Katarzyna Kozlik-Feldmann, Rainer Kordon, Zbigniew Urschel, Simon Netz, Heinrich Reichart, Bruno Malec, Edward Evaluation Study Journal Article Multicenter Study Germany Eur J Cardiothorac Surg. 2011 Aug;40(2):508-13. doi: 10.1016/j.ejcts.2010.12.023. Epub 2011 Feb 4. PY - 2011 SN - 1010-7940 SP - 508-13 ST - Significance of the residual aortic obstruction in multistage repair of hypoplastic left heart syndrome T2 - Eur J Cardiothorac Surg TI - Significance of the residual aortic obstruction in multistage repair of hypoplastic left heart syndrome VL - 40 ID - 255 ER - TY - JOUR AB - Background: Major depression is characterized by (i) a high lifetime prevalence of 16–17% in the general population; (ii) a high frequency of treatment resistance in around 20–30% of cases; (iii) a recurrent or chronic course; (iv) a negative impact on the general functioning and quality of life; and (v) a high level of comorbidity with various psychiatric and non-psychiatric disorders, high occurrence of completed suicide, significant burden along with the personal, societal, and economic costs. In this context, there is an important need for the development of a network of expert centers for treatment-resistant depression (TRD), as performed under the leadership of the Fondation FondaMental. Methods: The principal mission of this national network is to establish a genuine prevention, screening, and diagnosis policy for TRD to offer a systematic, comprehensive, longitudinal, and multidimensional evaluation of cases. A shared electronic medical file is used referring to a common exhaustive and standardized set of assessment tools exploring psychiatric, non-psychiatric, metabolic, biological, and cognitive dimensions of TRD. This is paralleled by a medico-economic evaluation to examine the global economic burden of the disease and related health-care resource utilization. In addition, an integrated biobank has been built by the collection of serum and DNA samples for the measurement of several biomarkers that could further be associated with the treatment resistance in the recruited depressed patients. A French observational long-term follow-up cohort study is currently in progress enabling the extensive assessment of resistant depressed patients. In those unresponsive cases, each expert center proposes relevant therapeutic options that are classically aligned to the international guidelines referring to recognized scientific societies. discussion: This approach is expected to improve the overall clinical assessments and to provide evidence-based information to those clinicians most closely involved in the management of TRD thereby facilitating treatment decisions and choice in everyday clinical practice. This could contribute to significantly improve the poor prognosis, the relapsing course, daily functioning and heavy burden of TRD. Moreover, the newly created French network of expert centers for TRD will be particularly helpful for a better characterization of sociodemographic, clinical, neuropsychological, and biological markers of treatment resistance required for the further development of personalized therapeutic strategies in TRD. (PsycINFO Database Record (c) 2018 APA, all rights reserved) AD - Yrondi, Antoine AN - 2017-54240-001 AU - Yrondi, Antoine AU - Bennabi, Djamila AU - Haffen, Emmanuel AU - Garnier, Marion AU - Bellivier, Frank AU - Bourgerol, Thierry AU - Camus, Vincent AU - D'Amato, Thierry AU - Doumy, Olivier AU - Haesebaert, Frédéric AU - Holtzmann, Jérôme AU - Lançon, Christophe AU - Vignaud, Philippe AU - Moliere, Fanny AU - Nieto, Isabel AU - Richieri, Raphaëlle Marie AU - Domenech, Philippe AU - Rabu, Corentin AU - Mallet, Luc AU - Yon, Liova AU - Schmitt, Laurent AU - Stephan, Florian AU - Vaiva, Guillaume AU - Walter, Michel AU - Llorca, Pierre-Michel AU - Courtet, Philippe AU - Leboyer, Marion AU - El-Hage, Wissam AU - Aouizerate, Bruno DB - psyh DO - 10.3389/fpsyt.2017.00244 DP - EBSCOhost KW - treatment-resistant depression depressive disorder innovative strategies disease management Innovation Major Depression Treatment Resistant Depression N1 - Service de Psychiatrie et de Psychologie Medicale de l’adulte, Centre Expert Depression Resistante FondaMental, CHRU de Toulouse, Hopital Purpan, Toulouse, France. Other Publishers: Frontiers Research Foundation. Release Date: 20180917. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Disease Management; Innovation; Major Depression; Treatment Resistant Depression. Classification: Affective Disorders (3211). Population: Human (10). Location: France. Tests & Measures: Functional Analysis Screening Tool; Mood Disorder Questionnaire; Big Five Inventory; Post Traumatic Stress Disorder Check List Scale; Clinical Global Impression Severity/Improvement Scale; Client Service Receipt Inventory; Child Trauma Questionnaire; Measure of Suicidal Ideation; State-Trait Anxiety Inventory for Adults DOI: 10.1037/t06496-000; Medication Adherence Rating Scale DOI: 10.1037/t15526-000; Suicide Intent Scale DOI: 10.1037/t15303-000; Columbia-Suicide Severity Rating Scale DOI: 10.1037/t52667-000; Quick Inventory of Depressive Symptomatology--Self Report Version DOI: 10.1037/t49873-000; EQ-5D-5L DOI: 10.1037/t63848-000; Global Assessment of Functioning Scale; Pittsburgh Sleep Quality Index DOI: 10.1037/t05178-000; Young Mania Rating Scale DOI: 10.1037/t20936-000; Lam Employment Absence and Productivity Scale DOI: 10.1037/t00404-000; Social Phobia Inventory. ArtID: 244. Issue Publication Date: Nov 24, 2017. Publication History: First Posted Date: Nov 24, 2017; Accepted Date: Nov 6, 2017; First Submitted Date: Aug 29, 2017. Copyright Statement: This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. Yrondi, Bennabi, Haffen, Garnier, Bellivier, Bourgerol, Camus, D’Amato, Doumy, Haesebaert, Holtzmann, Lanc¸on, Vignaud, Moliere, Nieto, Richieri, Domenech, Rabu, Mallet, Yon, Schmitt, Stephan, Vaiva, Walter, Llorca, Courtet, Leboyer, El-Hage and Aouizerate. 2017. Sponsor: AstraZeneca. Other Details: FondaMental Network. Recipients: No recipient indicated Sponsor: Janssen. Recipients: No recipient indicated Sponsor: Lilly. Recipients: No recipient indicated Sponsor: Lundbeck Foundation. Recipients: No recipient indicated Sponsor: Otsuka. Recipients: No recipient indicated Sponsor: Sanofi. Recipients: No recipient indicated Sponsor: Servier. Recipients: No recipient indicated Sponsor: Takeda. Recipients: No recipient indicated PY - 2017 SN - 1664-0640 ST - Significant need for a French network of expert centers enabling a better characterization and management of treatment-resistant depression (Fondation FondaMental) T2 - Frontiers in Psychiatry TI - Significant need for a French network of expert centers enabling a better characterization and management of treatment-resistant depression (Fondation FondaMental) UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2017-54240-001&site=ehost-live&scope=site ORCID: 0000-0002-6813-9012 ORCID: 0000-0002-4091-518X antoineyrondi@gmail.com VL - 8 ID - 1665 ER - TY - JOUR AB - One of the longest living survivors of the Blalock-Taussig shunt is a patient with a complex form of cyanotic congenital heart disease, including a single ventricle (anatomically right), d-loop, anterior aorta, pulmonary valvular and subvalvular stenosis and anomalous pulmonary venous return to a common atrial chamber. A Blalock shunt was performed at age 21 months, a Potts anastomosis at age 9 years, and an 8 mm Dacron shunt was added between the ascending aorta and the pulmonary artery at age 24 years. The patient's arterial oxygen saturation level of 86 percent and his relative well-being and active life at age 26 years suggest that sequential aortopulmonary shunting with increasing age is desirable in some patients with complicated heart disease and severe polycythemia. © 1973 Dun · Donnelley Publishing Corporation 666 Fifth Avenue, New York, New York 10019 All rights reserved. AD - Sections of Cardiovascular and Thoracic Surgery, Cardiology and Pediatric Cardiology, University of Chicago, Chicago, IL, United States the Johns Hopkins Hospital, Baltimore, MD, United States AU - Anagnostopoulos, C. E. AU - Coleman, P. G. AU - Taussig, H. B. AU - Resnekov, L. AU - Cassels, D. E. DB - Scopus DO - 10.1016/S0002-9149(73)80017-7 IS - 6 M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 1973 SP - 855-859 ST - Single ventricle and pulmonary stenosis. Surgical management in a patient over a period of 25 years T2 - The American Journal of Cardiology TI - Single ventricle and pulmonary stenosis. Surgical management in a patient over a period of 25 years UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0015842220&doi=10.1016%2fS0002-9149%2873%2980017-7&partnerID=40&md5=4ff1461d58bd8438f2e524276f48bafa VL - 32 ID - 2337 ER - TY - JOUR AB - PURPOSE OF REVIEW: Among the most frequently encountered congenital heart malformations are those with so-called single ventricle physiology, in which there is only one ventricle to pump blood to the pulmonary and systemic vascular beds, respectively. Long-term survival is possible, based on the principle of right heart bypass, whereby the ventricle pumps blood only to the systemic circuit, whereas pulmonary blood flow occurs passively. Such a circulatory system is achieved in a series of staged reconstructive operations, each of which was formerly accompanied by very high rates of major morbidity and mortality. Current approaches to single ventricle physiology as well as areas of controversy will be reviewed. RECENT FINDINGS: The development of a number of inventive operations, combined with a greater understanding of the physiologic requirements for success after single ventricle reconstruction has resulted in dramatic improvements in outcomes. The identification and modification of risk factors as well as the recent development of catheter-based intervention offer the real prospect of significant continued improvement. SUMMARY: Advances in the care of children with single ventricle hearts have resulted in remarkably improved prognosis, with the expectation of continued improvement in not only survival but also quality of life. AD - Arkansas Children's Hospital & University of Arkansas for Medical Sciences, Little Rock, AR 72202, USA. rjaquiss@uams.edu AN - 19225294 AU - Jaquiss, R. AU - Imamura, M. DA - Mar DB - PubMed DO - 10.1097/HCO.0b013e328323d85a DP - NLM ET - 2009/02/20 IS - 2 KW - Child *Fontan Procedure Heart Defects, Congenital/*surgery Heart Ventricles/*abnormalities Hemodynamics Humans LA - eng N1 - 1531-7080 Jaquiss, Robert D B Imamura, Michiaki Journal Article Review United States Curr Opin Cardiol. 2009 Mar;24(2):113-8. doi: 10.1097/HCO.0b013e328323d85a. PY - 2009 SN - 0268-4705 SP - 113-8 ST - Single ventricle physiology: surgical options, indications and outcomes T2 - Curr Opin Cardiol TI - Single ventricle physiology: surgical options, indications and outcomes VL - 24 ID - 218 ER - TY - JOUR AB - PURPOSE OF REVIEW: Among the most frequently encountered congenital heart malformations are those with so-called single ventricle physiology, in which there is only one ventricle to pump blood to the pulmonary and systemic vascular beds, respectively. Long-term survival is possible, based on the principle of right heart bypass, whereby the ventricle pumps blood only to the systemic circuit, whereas pulmonary blood flow occurs passively. Such a circulatory system is achieved in a series of staged reconstructive operations, each of which was formerly accompanied by very high rates of major morbidity and mortality. Current approaches to single ventricle physiology as well as areas of controversy will be reviewed. RECENT FINDINGS: The development of a number of inventive operations, combined with a greater understanding of the physiologic requirements for success after single ventricle reconstruction has resulted in dramatic improvements in outcomes. The identification and modification of risk factors as well as the recent development of catheter-based intervention offer the real prospect of significant continued improvement. SUMMARY: Advances in the care of children with single ventricle hearts have resulted in remarkably improved prognosis, with the expectation of continued improvement in not only survival but also quality of life. AD - Arkansas Children's Hospital & University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA. AN - 105474982. Language: English. Entry Date: 20090417. Revision Date: 20150711. Publication Type: Journal Article AU - Jaquiss, R. D. B. AU - Imamura, M. DB - ccm DO - 10.1097/HCO.0b013e328323d85a DP - EBSCOhost IS - 2 KW - Heart Ventricle -- Physiology Outcomes (Health Care) Cardiology Child Heart Diseases -- Familial and Genetic Heart Diseases -- Surgery Hemodynamics Infant, Newborn Lung Diseases -- Surgery Lung -- Physiopathology Palliative Care -- Methods Surgery, Operative -- Methods N1 - review. Journal Subset: Biomedical; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. NLM UID: 8608087. PMID: NLM19225294. PY - 2009 SN - 0268-4705 SP - 113-118 ST - Single ventricle physiology: surgical options, indications and outcomes T2 - Current Opinion in Cardiology TI - Single ventricle physiology: surgical options, indications and outcomes UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105474982&site=ehost-live&scope=site VL - 24 ID - 1644 ER - TY - JOUR AB - Objective. There is a paucity of information regarding appropriate management of children with Down's syndrome and a functional single ventricle. We report the results of bidirectional superior cavopulmonary shunts in six patients with Down's syndrome with a functional single ventricle. Methods. Between January 1991 and December 2004, we identified six patients with Down's syndrome among 263 who had undergone bidirectional superior cavopulmonary shunts (BCPSs). There were four males and two females. The age at BCPS ranged from 1 to 12 years (mean 4.3 ± 3.9 years), and body weight varied between 8.2 and 29.4 kg (mean 13.8 ± 7.8 kg). All six patients had an unbalanced complete atrioventricular septal defect, with right ventricular hypoplasia present in five and left ventricular hypoplasia in one. Results. There were no operative deaths, but one case required takedown of the BCPS. Except for this case, postoperative courses were generally uneventful. The median duration of follow-up was 46 months (range 12-80 months). Only two of five survivors after BCPS underwent a subsequent Fontan procedure, and one of these patients died of pulmonary hypertension post-operatively. The remaining three patients appeared to have significant risk factors for the Fontan procedure due to severe common atrioventricular valve regurgitation or persistent pulmonary vascular obstructive disease, including one who has completely dropped out from the Fontan track. Conclusion. Down's syndrome is a risk factor in patients with functionally single ventricle due to persistent pulmonary hypertension and airway obstruction. These results show that single ventricle repair in patients with Down's syndrome is accompanied with difficulties, and patient selection for the Fontan procedure should be done carefully. © 2008 The Japanese Association for Thoracic Surgery. AD - N. Wada, Department of Cardiovascular Surgery, Sakakibara Heart Institute, Japan Promotion Society for Cardiovascular Diseases, 3-16-1 Asahi-cho, Fuchu, Tokyo 183-0003, Japan AU - Wada, N. AU - Takahashi, Y. AU - Ando, M. AU - Park, I. S. AU - Sasaki, T. DB - Embase Medline DO - 10.1007/s11748-007-0201-z IS - 3 KW - article cavopulmonary connection child clinical article controlled study Down syndrome female Fontan procedure heart atrioventricular valve heart single ventricle human lung hemodynamics male postoperative care pulmonary hypertension pulmonary vascular obstructive disease risk factor treatment outcome LA - English M3 - Article N1 - L351414883 2008-04-02 PY - 2008 SN - 1863-6705 SP - 104-108 ST - Single ventricle repair in children with Down's syndrome T2 - General Thoracic and Cardiovascular Surgery TI - Single ventricle repair in children with Down's syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L351414883 http://dx.doi.org/10.1007/s11748-007-0201-z VL - 56 ID - 1218 ER - TY - JOUR AB - OBJECTIVES: The objectives of this study were to describe the approach to stenting arterial shunts in adult congenital patients with single-ventricle physiology and to assess the medium-term clinical and haemodynamic outcomes following stent insertion. BACKGROUND: Adult patients with single-ventricle physiology and pulmonary blood flow dependent on a surgically placed arterial shunt who did not progress to venous palliation are extremely challenging to manage. Progressive cyanosis secondary to narrowing of the shunt has a marked impact on exercise tolerance and results in intolerable well-being for these patients. Stenting arterial shunts in adult patients is one method that can help improve pulmonary blood flow. There is very limited information in the literature about this patient. METHODS: This is a retrospective study for arterial shunts stenting conducted between 2008 and 2016. The peripheral oxygen saturations, the NYHA status, the haemoglobin, and the degree of atrio-ventricular valve regurgitation as assessed on transthoracic echo were compared before and 6-12 months after procedures. RESULTS: There was a short-term improvement in oxygen saturations; the pre-procedure mean was 75.8 (SD 2.55)% (range 70-85%) and post-procedure mean was 83 (SD 2.52)% (range 78-87%), with a p value of 0.04. Haemoglobin level decreased from a pre-procedure mean of 22.06-20.28 g/L 6 months post procedure (range 18.1-24.4 to 13-23.3 g/L), with a p value of 0.44. NYHA class decreased from a mean of 3.2-2.2 post procedure. Left atrial volume for four of the cases did not change (22.6-76.6 ml [mean 48.4 ml] to 29.6-72.9 ml [mean 52 ml], p value: 0.83). CONCLUSIONS: Stenting stenotic arterial shunts is a useful method to gain a medium-term improvement on the oxygen saturation and clinical symptoms, and may act as a useful intermediate step for further management plans. AD - Department of Congenital Cardiology,Leeds General Infirmary,Leeds,UK. AN - 30309400 AU - Kasem, M. AU - Bentham, J. AU - Thomson, J. DA - Dec DB - PubMed DO - 10.1017/s1047951118001464 DP - NLM ET - 2018/10/13 IS - 12 KW - Adolescent Adult Blalock-Taussig Procedure Cardiac Catheterization/*methods Coronary Disease/complications/diagnostic imaging/*surgery Databases, Factual Female Heart Defects, Congenital/complications/*surgery Heart Ventricles/abnormalities/surgery Humans Male Middle Aged Retrospective Studies Stents Treatment Outcome Young Adult Arterial shunt Blalock–Taussig shunt central shunt stenting LA - eng N1 - 1467-1107 Kasem, Mohamed Bentham, James Thomson, John Journal Article England Cardiol Young. 2018 Dec;28(12):1431-1435. doi: 10.1017/S1047951118001464. Epub 2018 Oct 12. PY - 2018 SN - 1047-9511 SP - 1431-1435 ST - Single-centre experience in stenting arterial shunts for adult CHD patients with single-ventricle physiology and pulmonary blood flow dependent on arterial shunts T2 - Cardiol Young TI - Single-centre experience in stenting arterial shunts for adult CHD patients with single-ventricle physiology and pulmonary blood flow dependent on arterial shunts VL - 28 ID - 92 ER - TY - JOUR AB - OBJECTIVES: Favourable outcomes in the repair of Ebstein's anomaly are predicated on tricuspid valve competence, right ventricular function and presence of arrhythmia. We report our experience with a single-stage, three-fold repair of Ebstein's anomaly, namely, cone reconstruction of the tricuspid valve supplemented by bidirectional cavopulmonary anastomosis and right atrial electrocautery maze. METHODS: From 2010 to 2014, 37 consecutive patients with Ebstein's anomaly, median age 17.3 (9.1-56.2) years, underwent this single-stage, three-fold surgical procedure. The principal elements of the procedure include (i) cone reconstruction of the tricuspid valve, limited plication at the level of the displaced valve, insertion of a homemade annuloplasty ring, defect repair and reduction atrioplasty supplemented by (ii) right atrial electrocautery maze and (iii) bidirectional cavopulmonary anastomosis. Postoperatively, all patients were followed up regularly for a mean period of 2.3 (1-4) years by clinical, electrocardiographic and echocardiographic examinations. RESULTS: The in-hospital mortality rate was 2.7% (1 patient) with no late deaths. Mean cardiopulmonary bypass time was 110 ± 18.3 min and aortic cross-clamp time was 48.5 ± 7.4 min. Echocardiographic examination showed significant improvement of valve regurgitation (P < 0.0001). NYHA functional class was I in 77.8% of the survivors and II in 22.2%. The cardiothoracic ratio decreased significantly (P < 0.05). No deleterious effects of the Glenn shunts have been reported. Sinus rhythm has remained stable in 31 patients (86.1%) during the follow-up period. CONCLUSIONS: Single-stage, three-fold repair for the management of Ebstein's anomaly offers good outcome in terms of low mortality and morbidity rates. It can achieve a durable valve-sparing repair, good functional mid-term outcomes and good quality of life among survivors. AD - Department of Cardiovascular and Thoracic Surgery, Ain-Shams University Hospital, Faculty of Medicine, Cairo, Egypt. AN - 27702829 AU - Mostafa, E. A. AU - El Midany, A. A. AU - Elnahas, Y. AU - Helmy, A. AU - Mansour, S. A. DA - Feb 1 DB - PubMed DO - 10.1093/icvts/ivw333 DP - NLM ET - 2016/10/06 IS - 2 KW - Adolescent Adult *Cardiac Valve Annuloplasty Child Cohort Studies Ebstein Anomaly/mortality/physiopathology/*surgery Echocardiography Electrocardiography Female *Fontan Procedure Heart Atria/surgery Hospital Mortality Humans Male Middle Aged Tricuspid Valve/surgery Ventricular Function, Right Young Adult *Cone reconstruction of tricuspid valve *Congenital heart disease *Ebstein's anomaly *One-and-a-half ventricular repair LA - eng N1 - 1569-9285 Mostafa, Ezzeldin A El Midany, Ashraf A H Elnahas, Yasser Helmy, Ahmed Mansour, Sherif A Journal Article England Interact Cardiovasc Thorac Surg. 2017 Feb 1;24(2):245-250. doi: 10.1093/icvts/ivw333. PY - 2017 SN - 1569-9285 SP - 245-250 ST - Single-stage, three-fold repair for Ebstein's anomaly T2 - Interact Cardiovasc Thorac Surg TI - Single-stage, three-fold repair for Ebstein's anomaly VL - 24 ID - 110 ER - TY - JOUR AB - OBJECTIVES: Favourable outcomes in the repair of Ebstein's anomaly are predicated on tricuspid valve competence, right ventricular function and presence of arrhythmia. We report our experience with a single-stage, three-fold repair of Ebstein's anomaly, namely, cone reconstruction of the tricuspid valve supplemented by bidirectional cavopulmonary anastomosis and right atrial electrocautery maze. METHODS: From 2010 to 2014, 37 consecutive patients with Ebstein's anomaly, median age 17.3 (9.1-56.2) years, underwent this singlestage, three-fold surgical procedure. The principal elements of the procedure include (i) cone reconstruction of the tricuspid valve, limited plication at the level of the displaced valve, insertion of a homemade annuloplasty ring, defect repair and reduction atrioplasty supplemented by (ii) right atrial electrocautery maze and (iii) bidirectional cavopulmonary anastomosis. Postoperatively, all patients were followed up regularly for a mean period of 2.3 (1-4) years by clinical, electrocardiographic and echocardiographic examinations. RESULTS: The in-hospital mortality rate was 2.7% (1 patient) with no late deaths. Mean cardiopulmonary bypass time was 110 ± 18.3 min and aortic cross-clamp time was 48.5 ± 7.4 min. Echocardiographic examination showed significant improvement of valve regurgitation (P < 0.0001). NYHA functional class was I in 77.8% of the survivors and II in 22.2%. The cardiothoracic ratio decreased significantly (P < 0.05). No deleterious effects of the Glenn shunts have been reported. Sinus rhythm has remained stable in 31 patients (86.1%) during the follow-up period. CONCLUSIONS: Single-stage, three-fold repair for the management of Ebstein's anomaly offers good outcome in terms of low mortality and morbidity rates. It can achieve a durable valve-sparing repair, good functional mid-term outcomes and good quality of life among survivors. © The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. AD - Department of Cardiovascular and Thoracic Surgery, Ain-Shams University Hospital, Faculty of Medicine, Ramses Str. Abbassia, Cairo, 11588, Egypt AU - Mostafa, E. A. AU - El Midany, A. A. H. AU - Elnahas, Y. AU - Helmy, A. AU - Mansour, S. A. DB - Scopus DO - 10.1093/icvts/ivw333 IS - 2 KW - Cone reconstruction of tricuspid valve Congenital heart disease Ebstein's anomaly One-and-a-half ventricular repair M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2017 SP - 245-250 ST - Single-stage, three-fold repair for Ebstein's anomaly T2 - Interactive Cardiovascular and Thoracic Surgery TI - Single-stage, three-fold repair for Ebstein's anomaly UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85016207631&doi=10.1093%2ficvts%2fivw333&partnerID=40&md5=f5f833a0e0b5e328825a2c3305de2ca2 VL - 24 ID - 1876 ER - TY - JOUR AB - PURPOSE OF REVIEW: Patients with single-ventricle, shunt-dependent physiology are at increased risk for interstage death due to the inherent instability of parallel circulation. Enhanced surveillance and early identification of deteriorating physiology via interstage home monitoring result in significant reduction in mortality. These programs are an important focus of improving outcomes for patients with single-ventricle heart disease. RECENT FINDINGS: In the multi-institutional Pediatric Heart Network Single-Ventricle Reconstruction Trial, interstage mortality was 12%, highlighting the continued opportunity to improve on this metric. A number of single-center series have demonstrated significant benefit of interstage monitoring on survival and growth. The focus on interstage monitoring by the National Pediatric Cardiology Quality Improvement Collaborative of the Joint Council on Congenital Heart Disease should improve our understanding of patients at greatest risk and help establish best practices for interstage care. In addition, a number of pilot projects utilizing newer communication technologies seek to improve the connection between program and patient. SUMMARY: Interstage home monitoring programs are a model of collaborative care that improves outcomes. Continued research in this area will refine the elements of home monitoring programs and continue to guide improved results. In addition, this model may serve as a template for the care of other populations of medically complex infants. AD - Division of Pediatric Cardiology, Children's Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee, Wisconsin 53226, USA. dhehir@mcw.edu AN - 23337893 AU - Hehir, D. A. AU - Ghanayem, N. S. DA - Mar DB - PubMed DO - 10.1097/HCO.0b013e32835dceaf DP - NLM ET - 2013/01/23 IS - 2 KW - Heart Defects, Congenital/mortality/*surgery Heart Ventricles/*abnormalities Home Care Services, Hospital-Based/*organization & administration Humans Length of Stay Monitoring, Ambulatory/*methods *Norwood Procedures Palliative Care Parents/psychology Postoperative Complications/*prevention & control Risk Factors Stress, Psychological/prevention & control LA - eng N1 - 1531-7080 Hehir, David A Ghanayem, Nancy S Journal Article United States Curr Opin Cardiol. 2013 Mar;28(2):97-102. doi: 10.1097/HCO.0b013e32835dceaf. PY - 2013 SN - 0268-4705 SP - 97-102 ST - Single-ventricle infant home monitoring programs: outcomes and impact T2 - Curr Opin Cardiol TI - Single-ventricle infant home monitoring programs: outcomes and impact VL - 28 ID - 210 ER - TY - JOUR AB - Purpose Of Review: Patients with single-ventricle, shunt-dependent physiology are at increased risk for interstage death due to the inherent instability of parallel circulation. Enhanced surveillance and early identification of deteriorating physiology via interstage home monitoring result in significant reduction in mortality. These programs are an important focus of improving outcomes for patients with single-ventricle heart disease.Recent Findings: In the multi-institutional Pediatric Heart Network Single-Ventricle Reconstruction Trial, interstage mortality was 12%, highlighting the continued opportunity to improve on this metric. A number of single-center series have demonstrated significant benefit of interstage monitoring on survival and growth. The focus on interstage monitoring by the National Pediatric Cardiology Quality Improvement Collaborative of the Joint Council on Congenital Heart Disease should improve our understanding of patients at greatest risk and help establish best practices for interstage care. In addition, a number of pilot projects utilizing newer communication technologies seek to improve the connection between program and patient.Summary: Interstage home monitoring programs are a model of collaborative care that improves outcomes. Continued research in this area will refine the elements of home monitoring programs and continue to guide improved results. In addition, this model may serve as a template for the care of other populations of medically complex infants. AD - Division of Pediatric Cardiology, Children's Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee, Wisconsin 53226, USA aDivision of Pediatric Cardiology bDivision of Pediatric Critical Care Medicine, Children's Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee, Wisconsin, USA. AN - 108028336. Language: English. Entry Date: 20130802. Revision Date: 20160208. Publication Type: journal article. Journal Subset: Biomedical AU - Hehir, D. A. AU - Ghanayem, N. S. AU - Hehir, David A. AU - Ghanayem, Nancy S. DB - ccm DO - 10.1097/HCO.0b013e32835dceaf DP - EBSCOhost IS - 2 KW - Heart Defects, Congenital -- Surgery Heart Surgery -- Methods Heart Ventricle -- Abnormalities Home Health Care -- Administration Monitoring, Physiologic -- Methods Postoperative Complications -- Prevention and Control Heart Defects, Congenital -- Mortality Palliative Care Parents -- Psychosocial Factors Risk Factors Stress, Psychological -- Prevention and Control N1 - Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. NLM UID: 8608087. PMID: NLM23337893. PY - 2013 SN - 0268-4705 SP - 97-102 ST - Single-ventricle infant home monitoring programs: outcomes and impact T2 - Current Opinion in Cardiology TI - Single-ventricle infant home monitoring programs: outcomes and impact UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=108028336&site=ehost-live&scope=site VL - 28 ID - 1530 ER - TY - JOUR AB - Sirenomelia, also known as "mermaid malformation/syndrome," is a rare, serious congenital anomaly characterized by variable degrees of fusion of the lower limbs and associated with severe malformations of vertebral, genitourinary, cardiovascular system and single umbilical artery. The first pregnancy of a 25-year-old woman resulted in one twin born by Cesarian section at 32 weeks' gestation, who was referred to our hospital with cyanosis, a congenital anomaly and respiratory distress. On physical examination, there was no urogenital region and anal fissure and gender was indeterminate. The arms were in adduction and wrist in flexion position with four fingers on the right hand and two fingers on the left hand. There was a single lower extremity with a webbed single foot and two toes consistent with sirenomelia type IV radiologically. Abdominal ultrasonography showed urogenital system agenesis and echocardiography detected hypoplastic left heart. However, the patient died 4 hours after birth. The other twin was followed for 1 week for nutrition and respiratory support and was then discharged without any problems. AD - R. Ozdemir, Division of Neonatology, Department of Pediatrics, Inonu University School of Medicine, Malatya, Turkey AU - Turgut, H. AU - Ozdemir, R. AU - Gokce, I. AU - Karakurt, C. AU - Karadag, A. DB - Embase DO - 10.1515/bjmg-2017-0001 IS - 1 KW - adduction anus fissure article assisted ventilation case report cesarean section clinical article cyanosis disease association echocardiography echography gestational age heart arrest human hypoplastic left heart syndrome karyotype 46,XY newborn respiratory distress resuscitation sirenomelia LA - English M3 - Article N1 - L618385278 2017-09-26 2017-09-28 PY - 2017 SN - 1311-0160 SP - 91-94 ST - Sirenomelia associated with hypoplastic left heart in a newborn T2 - Balkan Journal of Medical Genetics TI - Sirenomelia associated with hypoplastic left heart in a newborn UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L618385278 http://dx.doi.org/10.1515/bjmg-2017-0001 VL - 20 ID - 782 ER - TY - JOUR AB - BACKGROUND AND OBJECTIVES: The care of children with complex congenital heart disease creates emotional and financial hardships for their families. We evaluated the social impact on families of patients with complex congenital heart disease (CHD) who underwent single ventricle repair. DESIGN AND SETTING: Cross-sectional survey conducted at the pediatric cardiology outpatient department at Prince Sultan Cardiac Center-Qassim (PSCC-Q). PATIENTS AND METHODS: All patients diagnosed and treated for complex CHD of single ventricle pathophysiology and seen in the pediatric cardiology at PSCC-Q were eligible for the study. Families of these patients completed a questionnaire conducted by one interviewer. The Impact on Family Scale (IFS) questionnaire of Stein and Riessman was instituted. Patients were divided into two groups according to the cardiac diagnosis and the requirement for medical or surgical management. The first group included patients with CHD who do not need any medical or surgical intervention, .e.g. tiny VSD or small ASD. The second group included patients with complex CHD with single ventricle pathophysiology who underwent Glenn and/or Fontan procedures. The mean impact on family scores was compared among the different groups by two sample t test analysis. RESULTS: Families of 41 children with CHD were interrogated during the study period from September 2011 to February 2012. Patients were divided into two groups. Group one (20 patients, 49%) with simple CHD and group two (21 patients, 51%) with complex CHD who are managed in the univentricular tract. Families of children who underwent single ventricle repair had significantly higher IFS (mean and standard deviation of 62 [7]) than families with minor heart disease (mean of 51 [4]) (P=.005). CONCLUSION: Families of patients who underwent single ventricle repair have significant social impact because of their child illness. A supporting public group should be initiated and encouraged. AD - A. Al Mesned, Pediatric Cardiology, Prince Sultan Cardiac Center Qassim, Othman ibn Affan Street, Buriadah, Qassim 896, Saudi Arabia AU - Almesned, S. AU - Al-Akhfash, A. AU - Al Mesned, A. DB - Embase Medline DO - 10.5144/0256-4947.2013.140 IS - 2 KW - article child clinical article congenital heart disease controlled study cross-sectional study emotion Fontan procedure Glenn shunt heart atrium septum defect heart single ventricle heart ventricle septum defect human Impact on Family Scale mitral valve disease assessment of humans priority journal psychological aspect pulmonary valve stenosis social aspect LA - English M3 - Article N1 - L368819269 2013-05-08 2013-05-15 http://www.annsaudimed.net/files.php?force&file=2012_0568_OA_486500697.pdf PY - 2013 SN - 0256-4947 0975-4466 SP - 140-143 ST - Social impact on families of children with complex congenital heart disease T2 - Annals of Saudi Medicine TI - Social impact on families of children with complex congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L368819269 http://dx.doi.org/10.5144/0256-4947.2013.140 VL - 33 ID - 1013 ER - TY - JOUR AB - BACKGROUND AND OBJECTIVES: The care of children with complex congenital heart disease creates emo.tional and financial hardships for their families. We evaluated the social impact on families of patients with complex congenital heart disease (CHD) who underwent single ventricle repair. DESIGN AND SETTING: Cross-sectional survey conducted at the pediatric cardiology outpatient department at Prince Sultan Cardiac Center-Qassim (PSCC-Q). PATIENTS AND METHODS: All patients diagnosed and treated for complex CHD of single ventricle patho.physiology and seen in the pediatric cardiology at PSCC-Q were eligible for the study. Families of these patients completed a questionnaire conducted by one interviewer. The Impact on Family Scale (IFS) questionnaire of Stein and Riessman was instituted. Patients were divided into two groups according to the cardiac diagnosis and the requirement for medical or surgical management. The first group included patients with CHD who do not need any medical or surgical intervention, .e.g. tiny VSD or small ASD. The second group included patients with complex CHD with single ventricle pathophysiology who underwent Glenn and/or Fontan procedures. The mean impact on family scores was compared among the different groups by two sample t test analysis. RESULTS: Families of 41 children with CHD were interrogated during the study period from September 2011 to February 2012. Patients were divided into two groups. Group one (20 patients, 49%) with simple CHD and group two (21 patients, 51%) with complex CHD who are managed in the univentricular tract. Families of chil.dren who underwent single ventricle repair had significantly higher IFS (mean and standard deviation of 62 [7]) than families with minor heart disease (mean of 51 [4]) (P=.005). CONCLUSION: Families of patients who underwent single ventricle repair have significant social impact be.cause of their child illness. A supporting public group should be initiated and encouraged. AD - Pediatric Cardiology, Prince Sultan Cardiac Center Qassim, Buriadah, Qassim 896, Saudi Arabia. AN - 23563001 AU - Almesned, S. AU - Al-Akhfash, A. AU - Mesned, A. A. C2 - PMC6078613 DA - Mar-Apr DB - PubMed DO - 10.5144/0256-4947.2013.140 DP - NLM ET - 2013/04/09 IS - 2 KW - Case-Control Studies Child Child, Preschool Chronic Disease *Cost of Illness Cross-Sectional Studies Family/*psychology Female Fontan Procedure Health Surveys Heart Defects, Congenital/economics/*psychology/surgery Humans Infant Male Saudi Arabia Social Support Socioeconomic Factors Surveys and Questionnaires LA - eng N1 - 0975-4466 Almesned, Sulaiman Al-Akhfash, Ali Mesned, Abdulrahman Al Journal Article Ann Saudi Med. 2013 Mar-Apr;33(2):140-3. doi: 10.5144/0256-4947.2013.140. PY - 2013 SN - 0256-4947 (Print) 0256-4947 SP - 140-3 ST - Social impact on families of children with complex congenital heart disease T2 - Ann Saudi Med TI - Social impact on families of children with complex congenital heart disease VL - 33 ID - 224 ER - TY - JOUR AB - For pediatric rare diseases, the number of patients available to support traditional research methods is often inadequate. However, patients who have similar diseases cluster "virtually" online via social media. This study aimed to (1) determine whether patients who have the rare diseases Fontan-associated protein losing enteropathy (PLE) and plastic bronchitis (PB) would participate in online research, and (2) explore response patterns to examine social media's role in participation compared with other referral modalities. A novel, internetbased survey querying details of potential pathogenesis, course, and treatment of PLE and PB was created. The study was available online via web and Facebook portals for 1 year. Apart from 2 study-initiated posts on patient-run Facebook pages at the study initiation, all recruitment was driven by study respondents only. Response patterns and referral sources were tracked. A total of 671 respondents with a Fontan palliation completed a valid survey, including 76 who had PLE and 46 who had PB. Responses over time demonstrated periodic, marked increases as new online populations of Fontan patients were reached. Of the responses, 574 (86%) were from the United States and 97 (14%) were international. The leading referral sources were Facebook, internet forums, and traditional websites. Overall, social media outlets referred 84% of all responses, making it the dominant modality for recruiting the largest reported contemporary cohort of Fontan patients and patients who have PLE and PB. The methodology and response patterns from this study can be used to design research applications for other rare diseases. Copyright © 2014 by the American Academy of Pediatrics. AD - K.R. Schumacher, Congenital Heart Center, C.S. Mott Children's Hospital, University of Michigan, 1540 E Hospital Dr, Ann Arbor, MI 48109-4204, United States AU - Schumacher, K. R. AU - Stringer, K. A. AU - Donohue, J. E. AU - Yu, S. AU - Shaver, A. AU - Caruthers, R. L. AU - Zikmund-Fisher, B. J. AU - Fifer, C. AU - Goldberg, C. AU - Russell, M. W. DB - Embase Medline DO - 10.1542/peds.2013-2966 IS - 5 KW - access to information African American article Asian bronchitis Caucasian child childhood disease cohort analysis comparative study controlled study croupous bronchitis feasibility study female Fontan procedure geographic distribution health survey Hispanic human Internet major clinical study male medical research online system Pacific Islander patient attitude patient history of surgery patient participation patient referral priority journal protein losing gastroenteropathy rare disease self report social media social network study design United States validation process LA - English M3 - Article N1 - L373021174 2014-05-15 2014-05-23 PY - 2014 SN - 1098-4275 0031-4005 SP - e1345-e1353 ST - Social media methods for studying rare diseases T2 - Pediatrics TI - Social media methods for studying rare diseases UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L373021174 http://dx.doi.org/10.1542/peds.2013-2966 VL - 133 ID - 929 ER - TY - JOUR AB - The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD) is the largest congenital and pediatric cardiac surgical clinical data registry in the world. With more than 400,000 total operations from nearly all centers performing pediatric and congenital heart operations in North America, the STS CHSD is an unparalleled platform for clinical investigation, outcomes research, and quality improvement activities in this subspecialty. In 2015, several major original publications reported analyses of data in the CHSD pertaining to specific diagnostic and procedural groups, age-defined cohorts, or the entire population of patients in the database. Additional publications reported the most recent development, evaluation, and application of metrics for quality measurement and reporting of pediatric and congenital heart operation outcomes. This use of the STS CHSD for outcomes research and for quality measurement continues to expand as database participation and the available wealth of data in it continue to grow. This article reviews outcomes research and quality improvement articles published in 2015 based on STS CHSD data. AD - M.L. Jacobs, Johns Hopkins Hospital, 1800 Orleans St, Sheikh Zayed Tower, Ste 7107, Baltimore, MD, United States AU - Jacobs, M. L. AU - Jacobs, J. P. AU - Pasquali, S. K. AU - Hill, K. D. AU - Hornik, C. AU - O'Brien, S. M. AU - Shahian, D. M. AU - Habib, R. H. AU - Edwards, F. H. DB - Embase Medline DO - 10.1016/j.athoracsur.2016.07.015 IS - 3 KW - article clinical outcome congenital heart disease data base heart surgery heterotaxy syndrome human hypoplastic left heart syndrome lung vein drainage anomaly medical research medical society mortality risk outcomes research palliative therapy prenatal diagnosis priority journal publication surgical mortality thoracic surgeon total quality management Williams Beuren syndrome LA - English M3 - Article N1 - L612633105 2016-10-14 2016-10-26 PY - 2016 SN - 1552-6259 0003-4975 SP - 688-695 ST - The Society of Thoracic Surgeons Congenital Heart Surgery Database: 2016 Update on Research T2 - Annals of Thoracic Surgery TI - The Society of Thoracic Surgeons Congenital Heart Surgery Database: 2016 Update on Research UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L612633105 http://dx.doi.org/10.1016/j.athoracsur.2016.07.015 VL - 102 ID - 794 ER - TY - JOUR AB - Background: We aimed to explore the sociodemographic and psychiatric correlates of psychotic symptoms in a large general population sample. Methods: The French Mental Health in the General Population cross-sectional survey interviewed 38,694 individuals using the Mini International Neuropsychiatric Interview. We looked for associations between the presence of lifetime psychotic symptoms, sociodemographic characteristics (including migrant status over three generations) and clinical characteristics. We then looked for associations regarding only hallucinations, delusional symptoms, and the co-occurrence of both hallucinations and delusional symptoms. To test the psychosis continuum hypothesis, associations with sociodemographic characteristics were compared with the characterized psychotic disorders' associations. Results: We found that 22.3% of the population declared psychotic symptoms without psychotic disorders, including 5.7% who declared hallucinations, 20.5% delusional symptoms, 4.0% both hallucinations and delusional symptoms, and 2.8% characterized psychotic disorders. The presence of psychotic symptoms was associated with young age, migrant status (over three generations), secondary education level, low-income level and never-married and separated marital status. Hallucinations, delusional symptoms and the co-occurrence of both hallucinations and delusional symptoms showed the same correlates, and hallucinations were also associated with elementary education level. Characterized psychotic disorders showed the same correlates. Concerning clinical outcomes, the presence of psychotic symptoms, hallucinations and delusional symptoms was associated with all non-psychotic disorders, i.e., bipolar, depressive, alcohol use, generalized anxiety, social phobia, panic and post-traumatic stress disorders and dysthymia (except dysthymia, which was not associated with hallucinations). Conclusions: Our results indicate that psychotic symptoms are associated with broad psychopathologies and support the continuum model of psychosis. (PsycINFO Database Record (c) 2019 APA, all rights reserved) AD - Pignon, Baptiste, Hopital Fontan, CHRU de Lille, F-59037, Lille, France, Cedex AN - 2017-30087-001 AU - Pignon, Baptiste AU - Schürhoff, Franck AU - Szöke, Andrei AU - Geoffroy, Pierre A. AU - Jardri, Renaud AU - Roelandt, Jean-Luc AU - Rolland, Benjamin AU - Thomas, Pierre AU - Vaiva, Guillaume AU - Amad, Ali DB - psyh DO - 10.1016/j.schres.2017.06.053 DP - EBSCOhost KW - Psychotic symptoms Hallucinations Delusional symptoms Psychosis continuum Sociodemographic correlates Clinical correlates Adolescent Adult Age Factors Aged Community Health Planning Cross-Sectional Studies Delusions Female France Health Surveys Humans Male Mental Health Middle Aged Mood Disorders Psychiatric Status Rating Scales Psychotic Disorders Socioeconomic Factors Young Adult Psychiatric Symptoms Psychosis Demographic Characteristics N1 - CHRU de Lille, Pole de Psychiatrie, Hopital Fontan, Lille, France. Release Date: 20170713. Correction Date: 20190523. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Psychiatric Symptoms; Psychosis. Minor Descriptor: Delusions; Demographic Characteristics; Hallucinations. Classification: Schizophrenia & Psychotic States (3213). Population: Human (10); Male (30); Female (40). Location: France. Age Group: Adulthood (18 yrs & older) (300); Young Adulthood (18-29 yrs) (320); Thirties (30-39 yrs) (340); Middle Age (40-64 yrs) (360). Tests & Measures: Mini International Neuropsychiatric Interview DOI: 10.1037/t18597-000. Methodology: Empirical Study; Quantitative Study. Page Count: 7. Issue Publication Date: Mar, 2018. Publication History: First Posted Date: Jul 8, 2017; Accepted Date: Jun 27, 2017; Revised Date: Jun 26, 2017; First Submitted Date: Dec 12, 2016. Copyright Statement: All rights reserved. Elsevier B.V. 2017. PY - 2018 SN - 0920-9964 1573-2509 SP - 336-342 ST - Sociodemographic and clinical correlates of psychotic symptoms in the general population: Findings from the MHGP survey T2 - Schizophrenia Research TI - Sociodemographic and clinical correlates of psychotic symptoms in the general population: Findings from the MHGP survey UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2017-30087-001&site=ehost-live&scope=site ORCID: 0000-0002-8666-3635 ORCID: 0000-0003-2973-7981 ORCID: 0000-0003-0526-3136 baptistepignon@yahoo.fr VL - 193 ID - 1667 ER - TY - JOUR AB - INTRODUCTION: Performed for many years in clinical settings, pleural and lung ultrasound (PLUS) has emerged to be an invaluable tool to diagnose underlying conditions of respiratory failure, to monitor disease progression and to ensure appropriate therapeutic intervention. PLUS basically relies on the analysis of two prevalent ultrasound artefacts: A-lines and B-lines. A-lines are hyperechoic reverberation artefacts of the pleural line. A-lines combined with lung sliding show that lungs are well aerated. B-lines are vertical hyperechoic reverberation artefacts arising from pleural line extending to the bottom of the screen. The prevalence of B-lines indicates a pathologic parenchyma. Since PLUS is readily available, easily affordable, and biologically non-invasive, it is especially suitable for bedside clinical care in critically ill and unstable adult patients. Several authors have recently proposed PLUS for application in critically ill neonates and children. We report a case in which PLUS was used to clinically monitor a complex lung lesion during treatment of a child with congenital heart disease suffering from severe lung injury. CASE DESCRIPTION: A 1-year-old male with hypoplastic left heart syndrome underwent bidirectional Glenn procedure and systemic-to-left pulmonary artery shunt for heart palliation. After surgery, he developed a severe acute respiratory distress syndrome (ARDS) and extra-corporeal membrane oxygenation (ECMO) treatment was started. PLUS was performed daily to monitor the disease's progression and response to treatment during lung rest. As B-lines were decreasing and A-lines were becoming visible, we were able to monitor the improving aeration of the injured lung. The ultrasound showed high consistency with traditional imaging. DISCUSSION: Due to its non-ionizing nature, low cost, easy availability, easy repeatability and real-time results, PLUS is a feasible and beneficial bedside imaging technique for critically ill and unstable adult and pediatric patients. A reliable monitoring of ongoing treatments is certainly helpful to provide appropriate intervention, correctly schedule chest X-ray and CT-scan, and optimize ECMO weaning. The present case suggests that PLUS may be a successful and useful tool for monitoring lung diseases in children with CHD with severe post-operative complex lung injury. AD - Pediatric Intensive Care Unit, Emergency Department, "A. Gemelli" Medical School, Catholic University of the Sacred Heart, Rome, Italy. AN - 24850798 AU - Biasucci, D. G. AU - Ricci, Z. AU - Conti, G. AU - Cogo, P. DA - Dec DB - PubMed DO - 10.1002/ppul.23059 DP - NLM ET - 2014/05/23 IS - 12 KW - Acute Lung Injury/*diagnostic imaging/etiology *Extracorporeal Membrane Oxygenation Humans Hypoplastic Left Heart Syndrome/surgery Infant Infant, Newborn Lung/*diagnostic imaging Male Pleura/*diagnostic imaging *Postoperative Complications Respiration Disorders/etiology/therapy Ultrasonography Ecmo congenital heart diseases heart surgery lung ultrasound mechanical ventilation pediatric critical care respiratory failure LA - eng N1 - 1099-0496 Biasucci, Daniele G Ricci, Zaccaria Conti, Giorgio Cogo, Paola Case Reports Journal Article United States Pediatr Pulmonol. 2014 Dec;49(12):E147-50. doi: 10.1002/ppul.23059. Epub 2014 May 22. PY - 2014 SN - 1099-0496 SP - E147-50 ST - Sonographic dynamic assessment of lung injury in a child with hypoplastic left heart syndrome undergoing extracorporeal membrane oxygenation T2 - Pediatr Pulmonol TI - Sonographic dynamic assessment of lung injury in a child with hypoplastic left heart syndrome undergoing extracorporeal membrane oxygenation VL - 49 ID - 365 ER - TY - JOUR AB - BACKGROUND: The purpose of this study was to define the prevalence of specific sequelae after extracardiac Fontan operation. METHODS: Sixty-five consecutive patients undergoing extracardiac Fontan operation were studied for mortality, Fontan failure, systemic ventricular function, supraventricular arrhythmias, thromboembolism, and growth potential. Age was 3 to 31 years (mean +/- standard deviation, 9.4 +/- 1.8; median, 7 years). The conduits were constructed of polytetrafluoroethylene (n = 50), and "viable" in situ pericardium (n = 15). The patients underwent serial echocardiogram, dynamic radionuclide studies, and cardiac catheterization. RESULTS: Operative mortality was 3%, and the incidence of conduit thrombosis was 4.6%. There was paradoxic filling of the right lung after femoral injection of the radiotracer in all cases of conduit obstruction. Perioperative and late postoperative supraventricular arrhythmias were observed in 9.2% and 4.7% of patients, respectively. Risk factors for supraventricular arrhythmias included systemic ventricular dysfunction (p = 0.000), heterotaxy syndrome (p = 0.008), systemic venous anomalies (p = 0.015), and previous bidirectional Glenn operation (p = 0.017). At a mean follow-up of 77 +/- 2 months (range, 8 to 79 months), there were no late deaths (actuarial survival at 79 months, 96.9% +/- 0.02%). Serial echocardiograms demonstrated evidence of growth of the viable tunnels. Postoperatively, there was transient depression of ejection fraction in all patients (p = 0.000). CONCLUSIONS: Supraventricular arrhythmias after extracardiac Fontan are more common in patients with heterotaxy syndrome, bilateral superior venae cavae, systemic ventricular dysfunction, and those undergoing completion Fontan operation. The viable tunnel may emerge as an optimal alternative by virtue of reduction of supraventricular arrhythmias, elimination of the need for anticoagulation, and addressing the issue of growth potential in selected patients. AD - Department of Cardiothoracic and Vascular Surgery, Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India. ujjwalchow@rediffmail.com AN - 16039223 AU - Chowdhury, U. K. AU - Airan, B. AU - Kothari, S. S. AU - Talwar, S. AU - Saxena, A. AU - Singh, R. AU - Subramaniam, G. K. AU - Pradeep, K. K. AU - Patel, C. D. AU - Venugopal, P. DA - Aug DB - PubMed DO - 10.1016/j.athoracsur.2005.02.024 DP - NLM ET - 2005/07/26 IS - 2 KW - Adolescent Adult Arrhythmias, Cardiac/*epidemiology/etiology Child Child, Preschool Fontan Procedure/*adverse effects/methods/mortality Heart Defects, Congenital/surgery Humans Organ Size/*physiology Prevalence Risk Factors Thromboembolism/*epidemiology/etiology Ventricular Dysfunction/*epidemiology/etiology Ventricular Function LA - eng N1 - 1552-6259 Chowdhury, Ujjwal K Airan, Balram Kothari, Shyam Sundar Talwar, Sachin Saxena, Anita Singh, Rajvir Subramaniam, Ganapathy K Pradeep, Kizakke K Patel, Chetan D Venugopal, Panangipalli Journal Article Netherlands Ann Thorac Surg. 2005 Aug;80(2):665-72. doi: 10.1016/j.athoracsur.2005.02.024. PY - 2005 SN - 0003-4975 SP - 665-72 ST - Specific issues after extracardiac fontan operation: ventricular function, growth potential, arrhythmia, and thromboembolism T2 - Ann Thorac Surg TI - Specific issues after extracardiac fontan operation: ventricular function, growth potential, arrhythmia, and thromboembolism VL - 80 ID - 418 ER - TY - JOUR AB - Background Diabetes mellitus is one of the most common medical problems among pregnant women. Now, gestational diabetes mellitus (GDM) is increasing and amounts to 17% in Asian women but only in 4% of American and European women. In southern India, the prevalence of GDM is 17% in urban women, 13.8% in semiurban, and 9.8% in rural women. Aim The aim of the study is to find the cardiovascular abnormalities in infants born to a diabetic mother (IDM) and the association between infant's heart lesion and diabetes mellitus in pregnant mother in a tertiary care center. Materials and Methods All babies born to both pregestational and gestational diabetic mother were included. Information regarding maternal type of diabetes, treatment regimen, maternal glycemic control status, antenatal ultrasonogram, baby's sex, birth weight, gestational age, and clinical features were collected. Echocardiogram was done for all the babies. Results Out of the 100 IDM, 28 babies had cardiac disease, 5 babies had cyanotic heart disease, and 23 babies had acyanotic heart disease. Out of 100 diabetic mothers, 66 were on meal plan, 21 were on oral hypoglycemic agent (OHA) metformin, and 13 were on insulin therapy. Among 66 babies whose mother was on the meal plan, 8 had cardiac abnormality. Among the 21 mothers on OHA, 7 babies had cardiac abnormality and all the 13 babies of mothers who were on insulin had cardiac abnormality which was a significant finding among the IDM. Conclusion Maternal diabetes is a significant risk factor for heart disease in the newborn. Careful evaluation and early diagnosis of heart diseases in this high-risk group is of great value. Both pregestational and gestational diabetic mothers should monitor their blood sugar and maintain it in a normal range at the time of conception and early in pregnancy to reduce the risk of congenital heart disease in IDM. AD - A.S. Arul, Department of Cardiology, Tirunelveli Medical College, Tamilnadu Dr MGR Medical University, A 67, 5th Cross Street Maharaja Nagar, Tirunelveli, Tamilnadu, India AU - Arul, A. S. AU - Babu Kandha Kumar, A. S. AU - Kiruthiga, K. AU - Priya, M. K. AU - Neveythaa, S. A. DB - Embase DO - 10.1055/s-0039-1697074 IS - 3 KW - insulin metformin oral antidiabetic agent arterial trunk article asymmetrical septal hypertrophy birth weight cardiovascular malformation clinical feature congenital heart disease cyanotic heart disease diabetic diet early diagnosis echocardiography female gestational age glucose blood level glycemic control great vessels transposition heart atrium septum defect heart murmur heart ventricle septum defect high risk infant human hypoplastic left heart syndrome infant insulin treatment large for gestational age major clinical study male maternal diabetes mellitus neonatal intensive care unit newborn patent ductus arteriosus patent foramen ovale priority journal risk factor risk reduction small for date infant tertiary care center treatment outcome tricuspid valve atresia LA - English M3 - Article N1 - L630324347 2019-12-31 2020-01-08 PY - 2019 SN - 2455-7854 SP - 124-128 ST - Spectrum of Cardiovascular Abnormalities in Infants Born to Diabetic Mother in a Tertiary Care Center T2 - Indian Journal of Cardiovascular Disease in Women - WINCARS TI - Spectrum of Cardiovascular Abnormalities in Infants Born to Diabetic Mother in a Tertiary Care Center UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L630324347 http://dx.doi.org/10.1055/s-0039-1697074 VL - 4 ID - 645 ER - TY - JOUR AB - Background: Children's health programs in Sub-Saharan Africa have always been oriented primarily to infectious diseases and malnutrition. We are witnessing in the early 21st century an epidemiological transition marked by the decline of old diseases and the identification of new diseases including heart disease. Therefore, it is necessary to describe the spectrum of these diseases in order to better prepare health workers to these new challenges. Methods: We conducted a cross-sectional study focused on heart disease diagnosed by echocardiography in children seen from January 2006 to December 2014 in a pediatric hospital of Yaounde. We collected sociodemographic data and the types of heart disease from registers, patients files as well as the electronic database of echocardiographic records. Results: A total of 2,235 patients underwent echocardiographic examination during the study period including 1,666 subjects with heart disease. Congenital cardiopathies were found in 1,230 (73.8%) patients and acquired abnormalities in 429 (25.8%). Seven children (0.4%) had a combination of both types. Congenital heart defects (CHD) were dominated by ventricular septal defect (VSD). Acquired heart disease was mostly rheumatic valvulopathies. Dyspnea on exertion was the most frequent presenting complaint (87.6%). Discovery of a heart murmur was the principal clinical finding on physical examination (81.4%). The median age was 9 months for congenital heart disease and 132 months for acquired heart disease. Conclusions: As infectious diseases recede and the diagnostic facilities are improving, pediatric heart diseases occupy a more important position in the spectrum of pediatric diseases in our context. However, the ability to evoke the diagnosis remains unsatisfactory by the majority of health personnel and therefore needs to be improved. Apart from congenital heart diseases, the impact of acquired heart diseases, rheumatic valvulopathy being the highest ranking, is remarkable in pediatrics. Awareness of health personnel for better management of child tonsillitis is more than ever a necessity. This preventive attitude of rheumatic heart disease is the main attitude available in our disadvantaged economic environment. AD - D. Chelo, Mother and Child Center of the Chantal Biya Foundation, Pediatric Cardiology Unit, P.O. Box 1936, Yaounde, Cameroon AU - Chelo, D. AU - Nguefack, F. AU - Menanga, A. P. AU - Um, S. N. AU - Gody, J. C. AU - Tatah, S. A. AU - Ndombo, P. O. K. DB - Embase DO - 10.3978/j.issn.2223-3652.2015.11.04 IS - 1 KW - acquired heart disease adolescent article awareness Cameroon child childhood disease clinical feature congenital heart disease controlled study cross-sectional study dyspnea echocardiography endocardial cushion defect Fallot tetralogy female great vessels transposition heart atrium septum defect heart disease heart murmur heart muscle fibrosis heart ventricle septum defect human infant lung vein drainage anomaly major clinical study male mitral valve regurgitation mixed cardiomyopathy patent ductus arteriosus patient attitude physical examination pulmonary valve stenosis rheumatic heart disease tricuspid valve atresia valvular heart disease LA - English M3 - Article N1 - L613217525 2016-11-18 2016-12-06 PY - 2016 SN - 2223-3660 2223-3652 SP - 10-19 ST - Spectrum of heart diseases in children: An echocardiographic study of 1,666 subjects in a pediatric hospital, Yaounde, Cameroon T2 - Cardiovascular Diagnosis and Therapy TI - Spectrum of heart diseases in children: An echocardiographic study of 1,666 subjects in a pediatric hospital, Yaounde, Cameroon UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L613217525 http://dx.doi.org/10.3978/j.issn.2223-3652.2015.11.04 VL - 6 ID - 837 ER - TY - JOUR AB - Background: Adolescents and young adults with congenital heart disease (CHD) are often restricted from physical activity and sports participation, which may have adverse effects. Objectives: To determine the amount of physical activity, type of sports participation, and reasons for sports restrictions, and to evaluate the effect of sports participation on quality of life (QoL) in a cohort of patients with CHD. Methods: Individuals with CHD aged 13-30 years were recruited at outpatient visits or via mailings. They completed a questionnaire addressing physical activity, sports participation, sports restrictions, and QoL (Pediatric Quality of Life Inventory). We also reviewed the patient's medical record. Results: Of the 177 patients who responded (mean age 20 years), 31% have mild CHD, 40% have moderate CHD, and 29% have severe CHD. In the cohort, 52% participate in competitive sports, 25% recreational sports, and 23% no sports. Among patients with severe CHD, 29% participate in competitive sports that would be restricted by published guidelines (36th Bethesda Conference). After controlling for age, sex, CHD severity, residual hemodynamic disease, and comorbidities, participation in competitive sports and increased frequency of physical activity are independently associated with a higher QoL (P = .003 and P = .001, respectively). In an identical model, competitive sports participation and frequency of physical activity are associated with higher maximum predicted oxygen consumption (VO2) (n = 40; P = .002 and .02) and slightly lower body mass index (BMI) (P = .02 and .01). All findings were similar when analyses were stratified by recruitment method. Conclusions: Patients with CHD commonly participate in competitive sports, and such participation is associated with higher QoL, improved exercise capacity, and lower BMI. AD - P.N. Dean, Division of Cardiology, Children's National Health System, 111 Michigan Avenue, N.W., Washington, DC, United States AU - Dean, P. N. AU - Gillespie, C. W. AU - Greene, E. A. AU - Pearson, G. D. AU - Robb, A. S. AU - Berul, C. I. AU - Kaltman, J. R. DB - Embase Medline DO - 10.1111/chd.12221 IS - 2 KW - adolescent adult article assessment of humans body mass cohort analysis congenital heart disease disease severity female Fontan procedure human major clinical study male medical record review outpatient oxygen consumption Pediatric Quality of Life Inventory physical activity practice guideline prediction priority journal quality of life sedentary lifestyle sport LA - English M3 - Article N1 - L603849863 2015-04-23 2015-05-05 PY - 2015 SN - 1747-0803 1747-079X SP - 169-179 ST - Sports participation and quality of life in adolescents and young adults with congenital heart disease T2 - Congenital Heart Disease TI - Sports participation and quality of life in adolescents and young adults with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L603849863 http://dx.doi.org/10.1111/chd.12221 VL - 10 ID - 874 ER - TY - JOUR AD - R.K. Retnamma, Department of Cardiac Anaesthesia, Amrita Institute of Medical Sciences, Anaesthesia Office, Edappaly, Cochin, Kerala, India AU - Retnamma, R. K. AU - Nair, S. G. AU - Sunil, G. S. AU - Benedict, R. DB - Embase Medline DO - 10.4103/0971-9784.91486 IS - 1 KW - epinephrine glyceryl trinitrate artery compression article artificial ventilation case report clinical feature coronary artery compression human hypoplastic left heart syndrome infant intensive care unit lung artery banding mitral valve atresia outcome assessment oxygen saturation patent foramen ovale ST segment depression tachycardia LA - English M3 - Article N1 - L364254947 2012-02-22 2012-02-28 PY - 2012 SN - 0971-9784 0974-5181 SP - 64-66 ST - ST segment depression following pulmonary artery banding T2 - Annals of Cardiac Anaesthesia TI - ST segment depression following pulmonary artery banding UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L364254947 http://dx.doi.org/10.4103/0971-9784.91486 VL - 15 ID - 1071 ER - TY - JOUR AB - Background: Validated protocols for diagnostic testing and management of pregnant women with cardiovascular disease (CVD) do not exist. Our objective was to establish a prospective standardized protocol for the clinical evaluation of pregnant women with CVD. Methods: The Standardized Outcomes in Reproductive Cardiovascular Care (STORCC) initiative prospectively enrolled pregnant women with CVD into a standardized diagnostic testing and assessment protocol. Detailed cardiac and obstetric data were collected during the antepartum, intrapartum, and postpartum periods. Each woman was assigned a STORCC color code of perceived risk at a monthly multidisciplinary conference. Results: In 250 pregnancies of 207 women with CVD, the standardized care protocol was followed in 136 and routine care in 114. The median age of the subjects was 32 years, and the most common form of heart disease was congenital heart disease (77%). Women enrolled in standardized care protocol had high compliance with second- and third-trimester visits (93%) and postpartum visits (76%). Maternal cardiac complications occurred in 10%. The STORCC cardiac and obstetric color codes predicted adverse outcomes within each respective category (P = .02, .01). Conclusions: The STORCC protocol for prospective diagnostic testing and follow-up of pregnant women with CVD was successfully established, and compliance was high. The strength of a standardized testing and care protocol as well as detailed classification of labor and delivery characteristics allows for robust analyses into specific questions regarding testing protocols, and mode and timing of delivery. © 2019 AD - Department of Cardiology, Boston Children's Hospital, and Department of Pediatrics, Boston, MA, United States Department of Medicine, Division of Cardiovascular Medicine, Brigham and Women's Hospital, Boston, MA, United States Department of Anesthesiology, Perioperative, and Pain Medicine, Brigham and Women's Hospital, Boston, MA, United States Department of Obstetrics and Gynecology, Division of Maternal Fetal Medicine, Brigham and Women's Hospital, Boston, MA, United States AU - Valente, A. M. AU - Landzberg, M. J. AU - Gauvreau, K. AU - Egidy-Assenza, G. AU - Barker, N. AU - Partington, S. AU - Morgan, R. B. AU - Harmon, A. J. AU - Hickey, K. AU - Mullen, M. P. AU - Carabuena, J. M. AU - O'Gara, P. AU - Economy, K. E. AU - on behalf of the, Storcc Investigators DB - Scopus DO - 10.1016/j.ahj.2019.07.015 M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2019 SP - 112-120 ST - Standardized outcomes in reproductive cardiovascular care: The STORCC initiative T2 - American Heart Journal TI - Standardized outcomes in reproductive cardiovascular care: The STORCC initiative UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85071978006&doi=10.1016%2fj.ahj.2019.07.015&partnerID=40&md5=ec94dc608cfd2a9acf161c62cf4daf9a VL - 217 ID - 1730 ER - TY - JOUR AB - During the last 20 years, pediatric cardiac surgery has been characterized by important changes, with reductions in surgical mortality and the achievement of complete repair at an earlier age, thus avoiding multiple procedures and strongly ameliorating the global outcome of these patients. In this review, we describe the actual trends in the surgical treatment of cardiac malformations. We analyze two groups of patients: in the first group (septal defects, tetralogy of Fallot, transposition of the great arteries, aortic stenosis and coarctation) the indications are well established and the goal is represented by a lessening of the surgical trauma and post-operative morbidity, with stable results in the follow-up. In the second group (univentricular heart, pulmonary atresia and intact ventricular septum, double discordance, conduit, hypoplastic left heart syndrome), the lesions are still considered complex and submitted to ongoing experimental and clinical research, in order to improve the post-surgical history of these diseases. AD - Division of Pediatric Cardiac Surgery, IRCCS Giannina Gaslini, Genoa, Italy. luciozannini@ospedale-gaslini.ge.it AN - 17255808 AU - Zannini, L. AU - Borini, I. DA - Jan DB - PubMed DO - 10.2459/01.JCM.0000247427.44204.0d DP - NLM ET - 2007/01/27 IS - 1 KW - Aortic Valve Stenosis/congenital/surgery Cardiac Surgical Procedures/*methods/trends Heart Defects, Congenital/*surgery Heart Septal Defects/surgery Humans Hypoplastic Left Heart Syndrome/surgery Infant Infant, Newborn Pediatrics/*methods/trends Pulmonary Atresia/surgery Tetralogy of Fallot/surgery Transposition of Great Vessels/surgery LA - eng N1 - Zannini, Lucio Borini, Italo Journal Article Review United States J Cardiovasc Med (Hagerstown). 2007 Jan;8(1):3-6. doi: 10.2459/01.JCM.0000247427.44204.0d. PY - 2007 SN - 1558-2027 (Print) 1558-2027 SP - 3-6 ST - State of the art of cardiac surgery in patients with congenital heart disease T2 - J Cardiovasc Med (Hagerstown) TI - State of the art of cardiac surgery in patients with congenital heart disease VL - 8 ID - 330 ER - TY - JOUR AB - In patients with a functionally univentricular heart, the Fontan strategy achieves separation of the systemic and pulmonary circulation and reduction of ventricular volume overload. Contemporary modifications of surgical techniques have significantly improved survival. However, the resulting Fontan physiology is associated with high morbidity. In this review, we discuss the state of the art of the Fontan strategy by assessing survival and risk factors for mortality. Complications of the Fontan circulation, such as cardiac arrhythmia, thromboembolism, and protein-losing enteropathy, are discussed. Common surgical and catheter-based interventions following Fontan completion are outlined. We describe functional status measurements such as quality of life and developmental outcomes in the contemporary Fontan patient. The current role of drug therapy in the Fontan patient is explored. Furthermore, we assess the current use and outcomes of mechanical circulatory support in the Fontan circulation and novel surgical innovations. Despite large improvements in outcomes for contemporary Fontan patients, a large burden of disease exists in this patient population. Continued efforts to improve outcomes are warranted. Several remaining challenges in the Fontan field are outlined. AD - Department of Pediatrics, Division of Pediatric Cardiology, Erasmus MC-Sophia Children's Hospital, Rotterdam, Netherlands. Netherlands Heart Institute, Utrecht, Netherlands. Department of Cardiothoracic Surgery, Erasmus MC, Rotterdam, Netherlands. AN - 30002816 AU - van der Ven, J. P. G. AU - van den Bosch, E. AU - Bogers, Ajcc AU - Helbing, W. A. C2 - PMC6024235 DB - PubMed DO - 10.12688/f1000research.13792.1 DP - NLM ET - 2018/07/14 KW - Congenital heart defects Fontan Procedure Morbidity Mortality Pediatrics Re-interventions Single ventricle Total cavopulmonary connection competing interests were disclosed. LA - eng N1 - 2046-1402 van der Ven, Jelle P G Orcid: 0000-0002-9794-6980 van den Bosch, Eva Bogers, Ad J C C Helbing, Willem A Journal Article Review F1000Res. 2018 Jun 27;7:F1000 Faculty Rev-935. doi: 10.12688/f1000research.13792.1. eCollection 2018. PY - 2018 SN - 2046-1402 (Print) 2046-1402 ST - State of the art of the Fontan strategy for treatment of univentricular heart disease T2 - F1000Res TI - State of the art of the Fontan strategy for treatment of univentricular heart disease VL - 7 ID - 7 ER - TY - JOUR AB - and purpose Static balloon atrial septostomy is a widely accepted intervention for children with CHD. Successful surgical palliation is creating increasing numbers of adult CHD patients who need subsequent left heart intervention requiring transseptal access. In these patients, the interatrial septum is usually thick and fibrotic because of a previous open heart surgery or catheter intervention, and conventional transseptal puncture may be unsuccessful. Static balloon atrial septostomy to access the left atrium may facilitate intervention via the interatrial septum in such situations. The purpose of this study was to investigate the usefulness and the safety of static balloon atrial septostomy, and the evolution of an iatrogenic atrial septal defect post procedure in adult CHD.Methods We retrospectively reviewed six procedures in five adults with CHD and collected demographic characteristics, details of the procedures, clinical outcome, and size changes of the iatrogenic atrial septal defect.Results The mean age at the time of the procedure was 35 years. The intended primary interventions were pulmonary vein isolation, stenting for pulmonary vein obstruction, and catheter ablation for focal atrial tachycardia. All static balloon atrial septostomies were effective, and the left heart interventions were successfully achieved via transseptal sheaths. There were no major complications associated with the static balloon atrial septostomy. There were no adverse clinical outcomes related to iatrogenic atrial septal defect, and the size of the defects regressed over time in all cases.Conclusions Static balloon atrial septostomy can be a safe and useful technique in adult CHD patients needing left heart procedures. The thick interatrial septum found in postoperative patients may reduce the risk of persistent iatrogenic atrial septal defect. AD - T. Fujii, Cardiovascular Center, Showa University Northern Yokohama Hospital, 35-1 Chigasakichuo, Tuzuki-ku, Yokohama, Japan AU - Fujii, T. AU - Tomita, H. AU - Hata, Y. AU - Sasaki, T. AU - Asada, D. AU - Tarui, S. AU - Miyahara, Y. AU - Ishino, K. AU - Soga, T. DB - Embase Medline DO - 10.1017/S1047951118001063 IS - 10 KW - balloon catheter Conquest balloon Jackal balloon adult age distribution article cardiovascular procedure catheter ablation clinical article clinical effectiveness clinical outcome congenital heart disease demography disease severity human pulmonary vein isolation pulmonary vein obstruction retrospective study risk reduction safety static balloon atrial septostomy supraventricular tachycardia Sterling balloon LA - English M3 - Article N1 - L623048724 2018-07-20 2019-04-17 PY - 2018 SN - 1467-1107 1047-9511 SP - 1116-1121 ST - Static balloon atrial septostomy for the purpose of left heart intervention in postoperative adult CHD T2 - Cardiology in the Young TI - Static balloon atrial septostomy for the purpose of left heart intervention in postoperative adult CHD UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L623048724 http://dx.doi.org/10.1017/S1047951118001063 VL - 28 ID - 662 ER - TY - JOUR AB - This numerical study examined the performance of an intravascular axial flow blood pump for mechanical hemodynamic support of patients in the setting of Fontan failure, which presently has few treatment options. Three anatomically accurate geometries of the total cavopulmonary connection (TCPC) were generated using patients' magnetic resonance imaging data. These patient-specific geometries, as well as an idealized version with cylindrical vessels, were computationally analyzed with and without a pump in the inferior vena cava. Pressure flow characteristics, energy gain calculations, and blood damage analyses were performed for each model. The pump produced pressures of 1-14mmHg for 1500-4000 revolutions per minute, flow rates of 1-4L/min, and pulmonary artery pressures of 8-24mmHg. Comparison of pump performance among the four models showed minimal intermodel differences (<5% deviation) in the pressure rise generated by the pump, the IVC pressure, and the energy imparted to the system by the pump. Blood damage analysis showed maximum fluid scalar stress values of 372Pa or less, and the blood damage index was less than 2% in all of the models. These results suggest that this axial flow blood pump performs consistently in a variety of TCPC vessel geometries with low risk of blood trauma. © 2012, Copyright the Authors. Artificial Organs. © 2012, International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc. AD - Department of Mechanical and Nuclear Engineering, School of Engineering, Virginia Commonwealth University, United States Division of Pediatric Cardiology, Children's Hospital of Richmond and School of Medicine, Virginia Commonwealth University, Richmond, VA, United States AU - Downs, E. A. AU - Moskowitz, W. B. AU - Throckmorton, A. L. DB - Scopus DO - 10.1111/j.1525-1594.2012.01510.x IS - 11 KW - Artificial right ventricle Blood pump Cavopulmonary assist device Computational fluid dynamics (CFD) Fontan physiology Mechanical cavopulmonary assist Pediatric circulatory support Single ventricle physiology Total cavopulmonary connection M3 - Article N1 - Cited By :11 Export Date: 15 June 2020 PY - 2012 SP - 972-980 ST - Steady Flow Analysis of Mechanical Cavopulmonary Assistance in MRI-Derived Patient-Specific Fontan Configurations T2 - Artificial Organs TI - Steady Flow Analysis of Mechanical Cavopulmonary Assistance in MRI-Derived Patient-Specific Fontan Configurations UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84868599364&doi=10.1111%2fj.1525-1594.2012.01510.x&partnerID=40&md5=f64ca765f977eac3a794b65f0422c683 VL - 36 ID - 2081 ER - TY - JOUR AB - Objectives To evaluate ascending aortic strain (AAS) with cardiac magnetic resonance (CMR) in a large consecutive series of patients with different types of cardiovascular disease (CVD). Methods Two-dimensional phase-contrast gradient-echo sequences of the ascending aorta were retrospectively reviewed in 1027 patients (726 males, 301 females). Aortic lumen area was segmented using a semi-automatic approach to calculate AAS values. Subgroup analysis was performed for patients with normal CMR, tetralogy of Fallot (ToF), and ischemic heart disease (IHD). Multivariate and post-hoc analyses were performed to evaluate the effect of age, gender, and CVD on AAS values. Shapiro-Wilk, three- and two-way ANOVA, Mann-Whitney U, and Spearman correlation statistics were used. Results Multivariate analysis showed significant differences in AAS among decades of age (p < 0.001), genders (p = 0.006) and CVD subgroups (p < 0.001) without interaction among these factors. A gender-related difference (higher AAS in females) was significant in ToF (p = 0.008), while an AAS reduction during aging was observed in all CVD subgroups. Post-hoc analysis showed a significantly lower AAS in ToF and IHD patients compared to subjects with normal CMR (p < 0.001). Conclusion Differences in age, gender, and CVD independently affect AAS. The lower AAS observed in ToF fosters its assessment during follow-up in adulthood. Future studies on causes and clinical implications of a higher AAS in females affected by ToF are warranted. © 2017 Elsevier B.V. AD - Postgraduate School in Radiodiagnostics, Università degli Studi di Milano, Via Festa del Perdono 7, Milan, 20122, Italy Unit of Radiology, IRCCS Policlinico San Donato, Via Morandi 30, San Donato Milanese, Milan, 20097, Italy PhD Course in Integrative Biomedical Research, Department of Biomedical Science for Health, Università degli Studi di Milano, Via Mangiagalli, 31, Milano, 20133, Italy Department of Biomedical Sciences for Health, Università degli Studi di Milano, Via Morandi 30, San Donato Milanese20097, Italy AU - Scarabello, M. AU - Codari, M. AU - Secchi, F. AU - Cannaò, P. M. AU - Alì, M. AU - Di Leo, G. AU - Sardanelli, F. DB - Scopus DO - 10.1016/j.ejrad.2017.12.002 KW - Aging Ascending aorta Ischemic heart disease Magnetic resonance imaging Tetralogy of Fallot M3 - Article N1 - Export Date: 15 June 2020 PY - 2018 SP - 34-39 ST - Strain of ascending aorta on cardiac magnetic resonance in 1027 patients: Relation with age, gender, and cardiovascular disease T2 - European Journal of Radiology TI - Strain of ascending aorta on cardiac magnetic resonance in 1027 patients: Relation with age, gender, and cardiovascular disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85038815444&doi=10.1016%2fj.ejrad.2017.12.002&partnerID=40&md5=f9c2087d667d55092f0dd57c7a18c0a0 VL - 99 ID - 1825 ER - TY - JOUR AB - Plastic bronchitis, a rare complication after Fontan palliation, carries a high morbidity and mortality risk. Heart transplantation is an effective treatment option, but casts may occur in the early post-operative period. We present a case series detailing peri-operative management strategies to minimize morbidity and mortality related to plastic bronchitis in patients undergoing heart transplantation. Patient 1 received no treatment pre-, intra-, or post-transplant for prevention of bronchial casts and developed severe respiratory acidosis 18 h following transplant. Emergent bronchoscopy was performed and a large obstructive cast was removed. The patient recovered and received inhaled tissue plasminogen activator (tPA) for 5 days. Patient 2 received inhaled tPA before, during, and for 5 days after transplantation and no bronchial casts developed. Patient 3 underwent intraoperative bronchoscopy just prior to implantation revealing no casts. The patient underwent non-urgent, preemptive bronchoscopy on post-transplant days 1, 3, and 4, removing several partially obstructive bronchial blood clots/casts, with no casts thereafter. Heart transplantation results in eventual resolution of plastic bronchitis. Residual bronchial casts can still be problematic in the peri-operative period. Airway clearance with inhaled tPA or bronchoscopy may prevent the need for prolonged mechanical ventilation and reduce post-operative morbidity in this unique population. AD - J.J. Parent, Division of Pediatric Cardiology, Indiana University School of Medicine – Riley Hospital, Indiana University Health, 705 Riley Hospital Drive, RR 127, Indianapolis, IN, United States AU - Parent, J. J. AU - Darragh, R. K. AU - Gossett, J. G. AU - Ryan, T. D. AU - Villa, C. R. AU - Lorts, A. AU - Jefferies, J. L. AU - Towbin, J. A. AU - Chin, C. DB - Embase Medline DO - 10.1007/s00246-017-1568-y IS - 5 KW - budesonide dornase alfa salbutamol tissue plasminogen activator article breathing exercise bronchitis bronchoscopy case report child female heart transplantation human lung clearance male morbidity outpatient care plastic bronchitis postoperative hemorrhage preschool child quality of life respiratory acidosis risk factor school child treatment duration LA - English M3 - Article N1 - L614133163 2017-01-25 2017-06-09 PY - 2017 SN - 1432-1971 0172-0643 SP - 1077-1079 ST - Strategies to Prevent Cast Formation in Patients with Plastic Bronchitis Undergoing Heart Transplantation T2 - Pediatric Cardiology TI - Strategies to Prevent Cast Formation in Patients with Plastic Bronchitis Undergoing Heart Transplantation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L614133163 http://dx.doi.org/10.1007/s00246-017-1568-y VL - 38 ID - 751 ER - TY - JOUR AB - OBJECTIVE: The study examined parent stress and health-related quality of life (HRQOL) among families of children with congenital heart disease (CHD) referred for psychological services. METHODS: Parents of 54 children (85% boys) aged 3 to 13 (M(age)  = 7.48, SD = 2.38) completed measures to assess parenting stress (Parenting Stress Index - Short Form; Pediatric Inventory for Parents) and the PedsQL Family Impact Module. Medical information was retrieved from medical record review. RESULTS: Half of parents of children with single ventricle anatomy had clinically significant levels of parenting stress. Parents of children with single ventricle anatomy reported more frequent illness-related stress and more difficulty dealing with illness-related stress than parents of children with two ventricle anatomy. Younger gestational age at birth and referral for attention or behavior problems were associated with greater likelihood of parent at-risk psychosocial functioning. CONCLUSIONS: Among children referred for psychological services, many parents report significant stress and significant negative impact of the child's medical condition on the family. Results underscore the need to consider assessing parent psychosocial functioning and providing additional support for parents of children with CHD. AD - Department of Psychology, Marquette University, Milwaukee, Wisconsin, USA. Department of Psychological Sciences, Kent State University, Kent, Ohio, USA. Department of Pediatrics, Medical College of Wisconsin, Herma Heart Center, Children's Hospital of Wisconsin, Milwaukee, Wisconsin, USA. AN - 29071790 AU - Kaugars, A. AU - Shields, C. AU - Brosig, C. DA - Jan DB - PubMed DO - 10.1111/chd.12547 DP - NLM ET - 2017/10/27 IS - 1 KW - Adolescent Behavioral Medicine/*methods Child Child, Preschool Female Heart Defects, Congenital/*psychology Humans Male Parenting/*psychology Parents/*psychology *Quality of Life *Referral and Consultation Retrospective Studies Stress, Psychological/psychology/*rehabilitation Surveys and Questionnaires children congenital heart disease health-related quality of life parent stress parents LA - eng N1 - 1747-0803 Kaugars, Astrida Orcid: 0000-0003-0538-2768 Shields, Clarissa Brosig, Cheryl Journal Article United States Congenit Heart Dis. 2018 Jan;13(1):72-78. doi: 10.1111/chd.12547. Epub 2017 Oct 25. PY - 2018 SN - 1747-079x SP - 72-78 ST - Stress and quality of life among parents of children with congenital heart disease referred for psychological services T2 - Congenit Heart Dis TI - Stress and quality of life among parents of children with congenital heart disease referred for psychological services VL - 13 ID - 12 ER - TY - JOUR AB - BACKGROUND: We hypothesize that dobutamine-induced stress impacts intracardiac hemodynamic parameters and that this may be linked to decreased exercise capacity in Fontan patients. Therefore, the purpose of this study was to assess the effect of pharmacologic stress on intraventricular kinetic energy (KE), viscous energy loss (EL) and vorticity from four-dimensional (4D) Flow cardiovascular magnetic resonance (CMR) imaging in Fontan patients and to study the association between stress response and exercise capacity. METHODS: Ten Fontan patients underwent whole-heart 4D flow CMR before and during 7.5 μg/kg/min dobutamine infusion and cardiopulmonary exercise testing (CPET) on the same day. Average ventricular KE, EL and vorticity were computed over systole, diastole and the total cardiac cycle (vorticity_vol(avg cycle), KE(avg cycle,) EL(avg cycle)). The relation to maximum oxygen uptake (VO(2) max) from CPET was tested by Pearson's correlation or Spearman's rank correlation in case of non-normality of the data. RESULTS: Dobutamine stress caused a significant 88 ± 52% increase in KE (KE(avg cycle): 1.8 ± 0.5 vs 3.3 ± 0.9 mJ, P < 0.001), a significant 108 ± 49% increase in EL (EL(avg cycle): 0.9 ± 0.4 vs 1.9 ± 0.9 mW, P < 0.001) and a significant 27 ± 19% increase in vorticity (vorticity_vol(avg cycle): 3441 ± 899 vs 4394 ± 1322 mL/s, P = 0.002). All rest-stress differences (%) were negatively correlated to VO(2) max (KE(avg cycle): r = - 0.83, P = 0.003; EL(avg cycle): r = - 0.80, P = 0.006; vorticity_vol(avg cycle): r = - 0.64, P = 0.047). CONCLUSIONS: 4D flow CMR-derived intraventricular kinetic energy, viscous energy loss and vorticity in Fontan patients increase during pharmacologic stress and show a negative correlation with exercise capacity measured by VO(2) max. AD - Department of Pediatrics division of Pediatric Cardiology, Leiden University Medical Center, Leiden, the Netherlands. Netherlands Heart Institute, Utrecht, The Netherlands. Department of Radiology Feinberg School of Medicine, Northwestern University, Chicago, USA. Department of Radiology, Leiden University Medical Center, Leiden, the Netherlands. Department of Cardiothoracic Surgery, Leiden University Medical Center, Leiden, the Netherlands. Department of Cardiology, Leiden University Medical Center, Leiden, the Netherlands. Department of Pediatrics division of Pediatric Cardiology, Academic Medical Center, Amsterdam, the Netherlands. Department of Pediatrics, division of Pediatric Cardiology, Erasmus Medical Center, Rotterdam, the Netherlands. Department of Pediatrics division of Pediatric Cardiology, Radboud university Medical Center, Nijmegen, the Netherlands. Department of Pediatrics division of Pediatric Cardiology, Leiden University Medical Center, Leiden, the Netherlands. a.roest@lumc.nl. AN - 31340834 AU - Kamphuis, V. P. AU - Elbaz, M. S. M. AU - van den Boogaard, P. J. AU - Kroft, L. J. M. AU - Lamb, H. J. AU - Hazekamp, M. G. AU - Jongbloed, M. R. M. AU - Blom, N. A. AU - Helbing, W. A. AU - Roest, A. A. W. AU - Westenberg, J. J. M. C2 - PMC6657113 DA - Jul 25 DB - PubMed DO - 10.1186/s12968-019-0553-4 DP - NLM ET - 2019/07/26 IS - 1 KW - Adolescent Adrenergic beta-1 Receptor Agonists/*administration & dosage Coronary Circulation/*drug effects Dobutamine/*administration & dosage *Exercise Test Exercise Tolerance/*drug effects Female *Fontan Procedure Heart Defects, Congenital/*diagnostic imaging/physiopathology/*surgery Hemodynamics/*drug effects Humans Image Interpretation, Computer-Assisted *Magnetic Resonance Imaging, Cine Male Myocardial Perfusion Imaging/*methods Oxygen Consumption/*drug effects Predictive Value of Tests Prospective Studies Time Factors Treatment Outcome Young Adult *4D flow CMR *Flow *Fontan *Kinetic energy: energy loss *Vorticity LA - eng N1 - 1532-429x Kamphuis, Vivian P Elbaz, Mohammed S M van den Boogaard, Pieter J Kroft, Lucia J M Lamb, Hildo J Hazekamp, Mark G Jongbloed, Monique R M Blom, Nico A Helbing, Willem A Roest, Arno A W Orcid: 0000-0002-0153-5934 Westenberg, Jos J M Comparative Study Journal Article Research Support, Non-U.S. Gov't J Cardiovasc Magn Reson. 2019 Jul 25;21(1):43. doi: 10.1186/s12968-019-0553-4. PY - 2019 SN - 1097-6647 (Print) 1097-6647 SP - 43 ST - Stress increases intracardiac 4D flow cardiovascular magnetic resonance -derived energetics and vorticity and relates to VO(2)max in Fontan patients T2 - J Cardiovasc Magn Reson TI - Stress increases intracardiac 4D flow cardiovascular magnetic resonance -derived energetics and vorticity and relates to VO(2)max in Fontan patients VL - 21 ID - 37 ER - TY - JOUR AB - Purpose: Kabuki syndrome is a multiple congenital malformation syndrome, with characteristic facial features, mental retardation, and skeletal and congenital heart anomalies. However, the cardiac anomalies are not well described in the Korean population. We analyzed the cardiac anomalies and clinical features of Kabuki syndrome in a single tertiary center. Methods: A retrospective analysis was conducted for a total of 13 patients with Kabuki syndrome. Results: The median age at diagnosis of was 5.9 years (range, 9 days to 11 years and 8 months). All patients showed the characteristic facial dysmorphisms and congenital anomalies in multiple organs, and the diagnosis was delayed by 5.9 years (range, 9 days to 11 years and 5 months) after the first visit. Noncardiac anomalies were found in 84% of patients, and congenital heart diseases were found in 9 patients (69%). All 9 patients exhibited left-side heart anomalies, including hypoplastic left heart syndrome in 3, coarctation of the aorta in 4, aortic valve stenosis in 1, and mitral valve stenosis in 1. None had right-side heart disease or isolated septal defects. Genetic testing in 10 patients revealed 9 novel MLL2 mutations. All 11 patients who were available for follow-up exhibited developmental delays during the median 4 years (range, 9 days to 11 years 11 months) of follow-up. The leading cause of death was hypoplastic left heart syndrome. Conclusion: Pediatric cardiologist should recognize Kabuki syndrome and the high prevalence of left heart anomalies with Kabuki syndrome. Genetic testing can be helpful for early diagnosis and counseling. AD - E.J. Bae, Department of Pediatrics, Seoul National University Children’s Hospital, 101 Daehak-ro, Jongno-gu, Seoul, South Korea AU - Yoon, J. K. AU - Ahn, K. J. AU - Kwon, B. S. AU - Kim, G. B. AU - Bae, E. J. AU - Noh, C. I. AU - Ko, J. M. DB - Embase DO - 10.3345/kjp.2015.58.7.256 IS - 7 KW - mixed lineage leukemia protein angiography aortic coarctation aortic valve stenosis article child clinical article computer assisted tomography congenital heart malformation disease association echocardiography electrocardiography face dysmorphia female follow up gene mutation genetic analysis heart catheterization human hypoplastic left heart syndrome infant Kabuki makeup syndrome male mitral valve stenosis multiple organ failure newborn prevalence retrospective study thorax radiography LA - English M3 - Article N1 - L605345844 2015-07-30 2015-08-03 PY - 2015 SN - 2092-7258 1738-1061 SP - 256-262 ST - The strong association of left-side heart anomalies with Kabuki syndrome T2 - Korean Journal of Pediatrics TI - The strong association of left-side heart anomalies with Kabuki syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L605345844 http://dx.doi.org/10.3345/kjp.2015.58.7.256 VL - 58 ID - 889 ER - TY - JOUR AB - OBJECTIVES: Neonates with single ventricle congenital heart disease are at risk for structural cerebral abnormalities. Little is known about the further evolution of cerebral abnormalities until Fontan procedure. METHODS: Between August 2012 and July 2015, we conducted a prospective cross-sectional two centre study using cerebral magnetic resonance imaging (MRI) and neuro-developmental outcome assessed by the Bayley-III. Forty-seven children (31 male) were evaluated at a mean age of 25.9 ± 3.4 months with hypoplastic left heart syndrome (25) or other single ventricle (22). RESULTS: Cerebral MRI was abnormal in 17 patients (36.2%) including liquor space enlargements (10), small grey (9) and minimal white (5) matter injuries. Eight of 17 individuals had combined lesions. Median (range) cognitive composite score (CCS) (100, 65-120) and motor composite score (MCS) (97, 55-124) were comparable to the reference data, while language composite score (LCS) (97, 68-124) was significantly lower ( P  = 0.040). Liquor space enlargement was associated with poorer performance on all Bayley-III subscores (CCS: P  = 0.02; LCS: P  = 0.002; MCS: P  = 0.013). The number of re-operations [odds ratio (OR) 2.2, 95% confidence interval (CI) 1.1-4.3] ( P  = 0.03) and re-interventions (OR 2.1, 95% CI 1.1-3.8) ( P  = 0.03) was associated with a higher rate of overall MRI abnormalities. CONCLUSIONS: Cerebral MRI abnormalities occur in more than one third of children with single ventricle, while the neuro-developmental status is less severely affected before Fontan procedure. Liquor space enlargement is the predominant MRI finding associated with poorer neuro-developmental status, warranting further studies to determine aetiology and further evolution until school-age. AD - Pediatric Cardiology, Pediatric Heart Center, University Children's Hospital Zurich, Switzerland. Children's Research Center, University Children's Hospital Zurich, Switzerland. Diagnostic Imaging, MR center, University Children's Hospital Zurich, Switzerland. Child Development Center, University Children's Hospital Zurich, Switzerland. Pediatric Heart Center, University Hospital Giessen, Germany. Pediatric Neurology, University Hospital Giessen, Germany. Child Development Center, Frankfurt/Main, Germany. AN - 28013288 AU - Knirsch, W. AU - Mayer, K. N. AU - Scheer, I. AU - Tuura, R. AU - Schranz, D. AU - Hahn, A. AU - Wetterling, K. AU - Beck, I. AU - Latal, B. AU - Reich, B. DA - Apr 1 DB - PubMed DO - 10.1093/ejcts/ezw399 DP - NLM ET - 2016/12/26 IS - 4 KW - Abnormalities, Multiple/*diagnostic imaging/psychology Brain/*abnormalities/diagnostic imaging Child, Preschool Cross-Sectional Studies Female Fontan Procedure Heart Defects, Congenital/*diagnosis/psychology/surgery Heart Ventricles/abnormalities Humans Hypoplastic Left Heart Syndrome/diagnosis/psychology/surgery Infant Magnetic Resonance Imaging Male Neurodevelopmental Disorders/*etiology Neuroimaging/methods Neuropsychological Tests *Congenital heart disease *mri *Neuroimaging *Outcome LA - eng N1 - 1873-734x Knirsch, Walter Mayer, Kristina Nadine Scheer, Ianina Tuura, Ruth Schranz, Dietmar Hahn, Andreas Wetterling, Kristina Beck, Ingrid Latal, Beatrice Reich, Bettina Journal Article Multicenter Study Germany Eur J Cardiothorac Surg. 2017 Apr 1;51(4):740-746. doi: 10.1093/ejcts/ezw399. PY - 2017 SN - 1010-7940 SP - 740-746 ST - Structural cerebral abnormalities and neurodevelopmental status in single ventricle congenital heart disease before Fontan procedure T2 - Eur J Cardiothorac Surg TI - Structural cerebral abnormalities and neurodevelopmental status in single ventricle congenital heart disease before Fontan procedure VL - 51 ID - 121 ER - TY - JOUR AB - OBJECTIVES: Neonates with single ventricle congenital heart disease are at risk for structural cerebral abnormalities. Little is known about the further evolution of cerebral abnormalities until Fontan procedure. METHODS: Between August 2012 and July 2015, we conducted a prospective cross-sectional two centre study using cerebral magnetic resonance imaging (MRI) and neuro-developmental outcome assessed by the Bayley-III. Forty-seven children (31 male) were evaluated at a mean age of 25.9 ± 3.4 months with hypoplastic left heart syndrome (25) or other single ventricle (22). RESULTS: Cerebral MRI was abnormal in 17 patients (36.2%) including liquor space enlargements (10), small grey (9) and minimal white (5) matter injuries. Eight of 17 individuals had combined lesions. Median (range) cognitive composite score (CCS) (100, 65-120) and motor composite score (MCS) (97, 55-124) were comparable to the reference data, while language composite score (LCS) (97, 68-124) was significantly lower (P = 0.040). Liquor space enlargement was associated with poorer performance on all Bayley-III subscores (CCS: P = 0.02; LCS: P = 0.002; MCS: P = 0.013). The number of re-operations [odds ratio (OR) 2.2, 95% confidence interval (CI) 1.1-4.3] (P = 0.03) and reinterventions (OR 2.1, 95% CI 1.1-3.8) (P = 0.03) was associated with a higher rate of overall MRI abnormalities. CONCLUSIONS: Cerebral MRI abnormalities occur in more than one third of children with single ventricle, while the neurodevelopmental status is less severely affected before Fontan procedure. Liquor space enlargement is the predominant MRI finding associated with poorer neuro-developmental status, warranting further studies to determine aetiology and further evolution until school-age. © The Author 2016. AD - Pediatric Cardiology, Pediatric Heart Center, University Children's Hospital, Zurich, Switzerland Children's Research Center, University Children's Hospital, Zurich, Switzerland Diagnostic Imaging, MR center, University Children's Hospital, Zurich, Switzerland Child Development Center, University Children's Hospital, Zurich, Switzerland Pediatric Heart Center, University Hospital, Giessen, Germany Pediatric Neurology, University Hospital, Giessen, Germany Child Development Center, Frankfurt/Main, Germany AU - Knirsch, W. AU - Mayera, K. N. AU - Scheer, I. AU - Tuura, R. AU - Schranz, D. AU - Hahn, A. AU - Wetterling, K. AU - Beck, I. AU - Latal, B. AU - Reich, B. C7 - ezw371 DB - Scopus DO - 10.1093/ejcts/ezw399 IS - 4 KW - Congenital heart disease MRI Neuroimaging Outcome M3 - Article N1 - Cited By :10 Export Date: 15 June 2020 PY - 2017 SP - 740-746 ST - Structural cerebral abnormalities and neurodevelopmental status in single ventricle congenital heart disease before Fontan procedure T2 - European Journal of Cardio-thoracic Surgery TI - Structural cerebral abnormalities and neurodevelopmental status in single ventricle congenital heart disease before Fontan procedure UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85017336082&doi=10.1093%2fejcts%2fezw399&partnerID=40&md5=c746b5f7548aa93e057bfb20974d4c39 VL - 51 ID - 1865 ER - TY - JOUR AB - BACKGROUND: Ventricular-ventricular interaction is known to occur in normal human heart. To determine whether it plays a role in the function of single right ventricles, systemic right ventricles were compared with and without a left ventricle mechanically coupled to it. METHODS AND RESULTS: A noninvasive magnetic resonance tagging technique (spatial modulation of magnetization [SPAMM]) that lays intersecting stripes down on the myocardium was used to examine 18 patients with systemic right ventricles: 7 with a single right ventricle who have undergone the Fontan procedure (age, 38.8 +/- 8.9 months) and 11 with transposition of the great arteries who have undergone an atrial inversion operation (age, 16.3 +/- 3.9 years). The motion of the intersection points was tracked through systole to determine regional twist and radial shortening. Shortening rates also were evaluated. Finite strain analysis was applied to the grid lines using Delaunay triangulation, and the two-dimensional strain tensor and principal E1 strains were derived for the various anatomic regions. Basal and apical short-axis planes through the ventricular wall were categorized into four distinct regions spaced equally around the circumference of the slice. We observed the following results. (1) Strain was greatest and heterogeneity of strain was least in patients with transposition of the great arteries who were status post atrial inversion operation (six of eight regions). Marked differences were noted in the distribution of strain within a given region, from endocardium to epicardium, and from atrioventricular valve to apical plane between patient subtypes and those with a normal left ventricle. (2) Contrary to the normal subject studied by the use of the same method, for both patient subtypes, there was counterclockwise twist in one region, clockwise twist in the posterior or inferior wall, and a transition zone of no twist at which the two regions of twist met. Normal human adult left ventricles studied in short-axis twist uniformly counterclockwise as viewed from apex to base. (3) Radial inward motion was greatest in the superior wall of both types of systemic right ventricle. The inferior walls of Fontan patients and the posterior (ie, septal) walls of patients with transposition of the great arteries, status post atrial inversion, moved paradoxically in systole. The shortening rate at the atrioventricular valve of patients with transposition of the great arteries, status post atrial inversion, was significantly lower than at the apex or in Fontan patients. CONCLUSIONS: Marked differences in regional wall motion and strain were demonstrated in systemic right ventricles, depending on whether a left ventricle was present to augment its function. Ventricular-ventricular interaction appears to play an important role in affecting the biomechanics of systemic right ventricles. These observations were markedly different from those in the normal systemic left ventricle. These techniques demonstrate tools with which we can begin to evaluate surgical outcomes using regional myocardial mechanics and may provide a clue to single right ventricle failure. AD - Department of Pediatrics, Children's Hospital of Philadelphia, PA 19104, USA. AN - 7600654 AU - Fogel, M. A. AU - Weinberg, P. M. AU - Fellows, K. E. AU - Hoffman, E. A. DA - Jul 15 DB - PubMed DO - 10.1161/01.cir.92.2.219 DP - NLM ET - 1995/07/15 IS - 2 KW - Adolescent Child, Preschool Fontan Procedure Heart Defects, Congenital/*physiopathology/surgery Humans Hypoplastic Left Heart Syndrome/physiopathology/surgery Image Processing, Computer-Assisted Magnetic Resonance Imaging/methods Myocardial Contraction/*physiology Prospective Studies Stress, Mechanical Transposition of Great Vessels/physiopathology/surgery Ventricular Function, Left/*physiology Ventricular Function, Right/*physiology LA - eng N1 - Fogel, M A Weinberg, P M Fellows, K E Hoffman, E A R01-HL-29886/HL/NHLBI NIH HHS/United States Comparative Study Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S. United States Circulation. 1995 Jul 15;92(2):219-30. doi: 10.1161/01.cir.92.2.219. PY - 1995 SN - 0009-7322 (Print) 0009-7322 SP - 219-30 ST - A study in ventricular-ventricular interaction. Single right ventricles compared with systemic right ventricles in a dual-chamber circulation T2 - Circulation TI - A study in ventricular-ventricular interaction. Single right ventricles compared with systemic right ventricles in a dual-chamber circulation VL - 92 ID - 150 ER - TY - JOUR AB - OBJECTIVES: The study aims to describe substance use, dental hygiene, and physical activity in adult survivors with single ventricle physiology (SVP) and to compare the behaviors with matched controls, while the patients are particularly at risk for general health problems. DESIGN: The present study is part of a larger research project on long-term outcomes in adult patients with SVP. A cross-sectional, case-control study including 59 patients out of 83 eligible patients participated in the study (response rate 71%). The patients were matched to 172 healthy controls. A questionnaire was mailed to the patients. Nonresponders did not differ significantly from the included participants on age, gender, or physical function. SETTING: The study was conducted at the Heart Center of Copenhagen University Hospital or the Department of Cardiology, Aarhus University Hospital. RESULTS: In these patients, 85% report alcohol consumption (92% in controls; odds ratio [OR] = 0.91; P = 0.575); 26% admit "binge drinking" (41% in controls; OR = 0.56; P = 0.041); 20% are cigarette smokers (36% in controls; OR = 0.59; P = 0.100); 12% have used cannabis over the past year (15% in controls; OR = 0.80; P = 0.596); 20% have had no dental visits during the last year (25% in controls; OR = 1.07; P = 0.684); 46% are not flossing their teeth (32% in controls; OR = 1.32; P = 0.239); and 39% are not physically active (24% in controls; OR = 1.63; P = 0.069). CONCLUSIONS: While in general there was no significant differences in overall health behaviors between SVP patients and controls, SVP patients are less physically active and are less likely to binge drink. AD - The Heart Center, Rigshospitalet, Copenhagen University Hospital, Skejby, Denmark. AN - 23663529 AU - Overgaard, D. AU - Schrader, A. M. AU - Lisby, K. H. AU - King, C. AU - Christensen, R. F. AU - Jensen, H. F. AU - Moons, P. DA - Jan-Feb DB - PubMed DO - 10.1111/chd.12086 DP - NLM ET - 2013/05/15 IS - 1 KW - Adolescent Adult Age Factors Binge Drinking/epidemiology Case-Control Studies Cross-Sectional Studies Denmark Female *Health Behavior Heart Defects, Congenital/diagnosis/*epidemiology Heart Ventricles/*abnormalities Humans Logistic Models Male Marijuana Smoking/epidemiology *Motor Activity Odds Ratio *Oral Hygiene Risk Factors Substance-Related Disorders/*epidemiology Surveys and Questionnaires Young Adult Congenital Heart Disease Endocarditis Health Behavior “Binge Drinking” LA - eng N1 - 1747-0803 Overgaard, Dorthe Schrader, Anne-Marie Lisby, Karen H King, Catriona Christensen, Rie F Jensen, Helena F Moons, Philip Journal Article United States Congenit Heart Dis. 2014 Jan-Feb;9(1):75-82. doi: 10.1111/chd.12086. Epub 2013 May 13. PY - 2014 SN - 1747-079x SP - 75-82 ST - Substance use, dental hygiene, and physical activity in adult patients with single ventricle physiology T2 - Congenit Heart Dis TI - Substance use, dental hygiene, and physical activity in adult patients with single ventricle physiology VL - 9 ID - 355 ER - TY - JOUR AB - Arrhythmia is a prevalent complication of the atriopulmonary Fontan procedure, most commonly macro-reentrant tachycardia within the right atrium as a consequence of long-term morphological and haemodynamic changes coupled with surgical intervention. We describe a patient with a long history of supraventricular arrhythmia following the atriopulmonary Fontan procedure identified as atrioventricular nodal reentry tachycardia at electrophysiological assessment. Despite anatomical and technical difficulties related to right atrial morphological changes and loss of the tricuspid annulus with its associated anatomical landmarks, the arrhythmia was successfully ablated using a combined antegrade and retrograde mapping approach. © 2006 Oxford University Press. AD - Department of Cardiac Electrophysiology, St. Bartholomew's Hospital, 60 Dominion House, Bartholomew Close, West Smithfield, London EC1A 7BE, United Kingdom AU - Abrams, D. J. AU - Earley, M. J. AU - Sporton, S. C. AU - Schilling, R. J. DB - Scopus DO - 10.1093/europace/eul096 IS - 10 KW - Atrioventricular nodal reentry tachycardia Fontan Tricuspid atresia M3 - Article N1 - Cited By :6 Export Date: 15 June 2020 PY - 2006 SP - 907-910 ST - Successful ablation of atrioventricular nodal reentry tachycardia following the atriopulmonary Fontan procedure T2 - Europace TI - Successful ablation of atrioventricular nodal reentry tachycardia following the atriopulmonary Fontan procedure UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-33749663795&doi=10.1093%2feuropace%2feul096&partnerID=40&md5=19657632c629b0bfe2985b35e4bf4119 VL - 8 ID - 2222 ER - TY - JOUR AB - Plastic bronchitis is a rare disorder, characterized by formation of thick fibrinous bronchial casts which can obstruct the airway and present as a life threatening emergency (1). It is more common in the pediatric population after corrective or palliative surgery for congenital heart disease like fontan procedure but has rarely been reported in adults as well (1). Pregnancy is a relative contraindication for bronchoscopy. Bronchoscopy in the pregnant patient poses significant risks as manipulation of the airway can lead to impaired oxygenation and ventilation. In addition, the drugs used during this procedure to provide sedation can have a direct impact on the developing fetus (2). In spite of these risks bronchoscopy should not be withheld in an emergent situation as it can be a lifesaving measure. We report a case of successful bronchoscopy using Propofol as the sedating agent in a pregnant female with plastic bronchitis who presented with respiratory distress. AD - University at Buffalo, Department of Pulmonary Critical Care Medicine, Buffalo, NY, USA. University at Buffalo, Department of Pulmonary Critical Care Medicine, Buffalo, NY, USA; Buffalo General Medical Center, Department of Critical Care Medicine, Buffalo, NY, USA. University at Buffalo, Department of Pulmonary Critical Care Medicine, Buffalo, NY, USA; Veteran Affairs Western New York Health Care System-Buffalo Division, Department of Pulmonary and Critical Care Medicine, Buffalo, NY, USA. AN - 27054088 AU - Patil, M. H. AU - Siddiqi, A. AU - Jeffrey Mador, M. C2 - PMC4802819 DB - PubMed DO - 10.1016/j.rmcr.2016.03.003 DP - NLM ET - 2016/04/08 KW - Bronchoscope Plastic bronchitis Pregnancy Propofol LA - eng N1 - 2213-0071 Patil, Monali H Siddiqi, Attiya Jeffrey Mador, M Case Reports Respir Med Case Rep. 2016 Mar 11;18:8-9. doi: 10.1016/j.rmcr.2016.03.003. eCollection 2016. PY - 2016 SN - 2213-0071 (Print) 2213-0071 SP - 8-9 ST - Successful bronchoscopy in a pregnant patient with plastic bronchitis T2 - Respir Med Case Rep TI - Successful bronchoscopy in a pregnant patient with plastic bronchitis VL - 18 ID - 463 ER - TY - JOUR AB - PURPOSE: Plastic bronchitis is the occlusion of the major bronchial airways by a firm, gelatinous mucoid cast. It is a rare condition, which while classically described in asthma and sickle cell disease has greater mortality in patients with congenital heart disease. The management of this disease is obscure given the lack of clinical data regarding treatment therapies. METHODS: We describe a case of an 11-year-old female status after Fontan surgery who presented with respiratory distress secondary to atelectasis of the right lung. RESULTS: A bronchoscopy was performed demonstrating an obstructing bronchial cast with successful extraction. The plastic bronchitis continued to recur and she was placed on multiple inhaled mucolytics as well as inhaled tissue plasminogen activator with temporary resolution. Further evaluation of the etiology of her casts revealed that she had elevated pulmonary arterial pressures. Repeated bronchoscopic removal of the casts was utilized as well as continuation of the aggressive airway clearance. Ultimately fenestration of her Fontan was performed along with treatment of pulmonary vasodilators sildenafil and bosentan. Although there was improvement of the cast formation, her airway clearance could only be weaned to four times a day therapy with which she was discharged home after a 3-month hospitalization. She continues to remain on this therapy and has not required hospitalization since the initial incident over 1 year ago. CONCLUSIONS: Plastic bronchitis in a patient with Fontan physiology presents a treatment dilemma that may require comprehensive therapy in severe cases such as described. AD - Miller Children's Hospital, 2801 Atlantic Ave. Ground Floor, Long Beach, CA, USA. pcdo78.pedspulm@gmail.com AN - 22430124 AU - Do, P. AU - Randhawa, I. AU - Chin, T. AU - Parsapour, K. AU - Nussbaum, E. DA - Aug DB - PubMed DO - 10.1007/s00408-012-9384-x DP - NLM ET - 2012/03/21 IS - 4 KW - Antihypertensive Agents/therapeutic use Bosentan Bronchitis/*drug therapy/etiology/*surgery *Bronchoscopy Child Combined Modality Therapy Drug Therapy, Combination Expectorants/*therapeutic use Female Fontan Procedure/*adverse effects Heart Defects, Congenital/*surgery Humans Piperazines/therapeutic use Purines/therapeutic use Sildenafil Citrate Sulfonamides/therapeutic use Sulfones/therapeutic use Tissue Plasminogen Activator/*therapeutic use Treatment Outcome Vasodilator Agents/therapeutic use LA - eng N1 - 1432-1750 Do, Paul Randhawa, Inderpal Chin, Terry Parsapour, Kourosh Nussbaum, Eliezer Case Reports Journal Article Review United States Lung. 2012 Aug;190(4):463-8. doi: 10.1007/s00408-012-9384-x. Epub 2012 Mar 20. PY - 2012 SN - 0341-2040 SP - 463-8 ST - Successful management of plastic bronchitis in a child post Fontan: case report and literature review T2 - Lung TI - Successful management of plastic bronchitis in a child post Fontan: case report and literature review VL - 190 ID - 414 ER - TY - JOUR AB - For many decades, the primary treatment of acute respiratory failure has been mechanical ventilation. Intubation and mechanical ventilation carry complications such as upper respiratory system trauma, barotrauma, volutrauma, oxytrauma, atelectrauma, and nosocomial infection, and they increase mortality. Noninvasive mechanical ventilation (NIMV) applied to a specially selected group of patients has more advantages than invasive ventilation, especially the decreased number of complications. We present a three-year-old girl who had prolonged invasive mechanical ventilation (IMV) and weaning failure due to severe tracheal bleeding after Fontan operation. She could not be extubated for 20 days after admission to our Pediatric Intensive Care Unit (PICU) because of life-threatening airway bleedings from within the endotracheal tube. Owing to the severe bleedings during endotracheal aspirations, it was decided to change her mechanical ventilation to NIMV. NIMV was performed successfully for 12 days without difficulty, and she was discharged from the hospital on the 55th postoperative day with normal clinical findings. Noninvasive ventilation has an increasing use in critically ill children. NIMV should be considered in prolonged mechanical ventilation and weaning failure from IMV in critically ill children. AD - Division of Pediatric Intensive Care Unit, Department of Pediatrics, Ankara University School of Medicine, Ankara, Turkey. dryamanayhan@yahoo.com.tr. AN - 24827960 AU - Yaman, A. AU - Kendirli, T. AU - Ödek, Ç AU - Karadeniz, C. AU - Uçar, T. AU - Göllü, G. AU - Eyileten, Z. AU - İnce, E. DA - Jan-Feb DB - PubMed DP - NLM ET - 2014/05/16 IS - 1 KW - Child, Preschool Female *Fontan Procedure Humans Noninvasive Ventilation/*methods Postoperative Complications Respiration, Artificial Ventilator Weaning LA - eng N1 - Yaman, Ayhan Kendirli, Tanıl Ödek, Çağlar Karadeniz, Cem Uçar, Tayfun Göllü, Gülnur Eyileten, Zeynep İnce, Erdal Case Reports Journal Article Turkey Turk J Pediatr. 2014 Jan-Feb;56(1):111-4. PY - 2014 SN - 0041-4301 (Print) 0041-4301 SP - 111-4 ST - Successful noninvasive mechanical ventilation in a child on prolonged and life-threatening invasive mechanical ventilation after Fontan operation T2 - Turk J Pediatr TI - Successful noninvasive mechanical ventilation in a child on prolonged and life-threatening invasive mechanical ventilation after Fontan operation VL - 56 ID - 447 ER - TY - JOUR AB - CLINICAL CASE: This case report documents outpatient cardiac rehabilitation (CR) in a 28-year-old woman born with transposition of the great vessels, tricuspid atresia, hypoplastic right ventricle, and an atrial septal defect. Surgical procedures were performed during childhood to correct these defects. In 2006, she underwent the following procedures: Fontan revision with a graft to an extracardiac total cavopulmonary connection; ASD creation; right atrial reduction; bidirectional Glenn shunt; right atrial and modified left atrial Maze procedures; and placement of an epicardial dual-chamber anti-tachycardia pacemaker. The patient was referred to CR because of postoperative complaints of fatigue, dyspnea on exertion, and low exercise tolerance. At intake, she underwent a cardiopulmonary stress test, measurement of percentage body fat, and completed the Medical Outcomes Survey 36-Item Short-Form Health Survey, Diet Intake Survey, and Center for Epidemiologic Studies Depression Scale. After completing 36 sessions, all outcomes demonstrated improvement with the exception of percentage dietary fat intake. DISCUSSION: Despite increasing numbers of patients with congenital heart disease (CHD) surviving into adulthood, exercise prescription in this population remains poorly delineated. In this case, possible physiologic limitations to exercise included diminished cardiac output secondary to low pressures and flow rates in the pulmonary arteries and veins because of the absence of a functioning right ventricle, limited chronotropic response, and severe deconditioning. SUMMARY: This case presents an adult patient who underwent surgical procedures to avoid heart transplantation. Despite severe CHD with many surgical procedures, and what is effectively a 2-chambered heart, she was able to successfully complete CR, graduate to home exercise, return to independent home living, and pursue her master's degree. AD - Helen Hayes Hospital, West Haverstraw, NY 10993, USA. lichtman@helenhayeshosp.org AN - 18277831 AU - Lichtman, S. W. AU - Caravano, M. AU - Schneyman, M. AU - Howell, B. AU - King, M. L. DA - Jan-Feb DB - PubMed DO - 10.1097/01.HCR.0000311509.16226.b8 DP - NLM ET - 2008/02/19 IS - 1 KW - Adult *Exercise Therapy Female Fontan Procedure Heart Defects, Congenital/*rehabilitation/surgery Heart Septal Defects, Atrial/surgery Heart Ventricles/*abnormalities/surgery Humans Postoperative Complications/*rehabilitation Transposition of Great Vessels/surgery Tricuspid Atresia/*surgery LA - eng N1 - Lichtman, Steven W Caravano, Michelle Schneyman, Michael Howell, Barbara King, Marjorie L Case Reports Journal Article United States J Cardiopulm Rehabil Prev. 2008 Jan-Feb;28(1):48-51. doi: 10.1097/01.HCR.0000311509.16226.b8. PY - 2008 SN - 1932-7501 (Print) 1932-7501 SP - 48-51 ST - Successful outpatient cardiac rehabilitation in an adult patient post-surgical repair for tricuspid valve atresia and hypoplastic right ventricle: a case study T2 - J Cardiopulm Rehabil Prev TI - Successful outpatient cardiac rehabilitation in an adult patient post-surgical repair for tricuspid valve atresia and hypoplastic right ventricle: a case study VL - 28 ID - 376 ER - TY - JOUR AB - We report successful selective local intra-arterial thrombolytic therapy for thromboembolic occlusion of right middle cerebral artery in a patient with asplenia syndrome and unrepaired cyanotic congenital heart disease. © 2006 the Authors; Journal compilation © 2006 Blackwell Publishing, Inc. AD - K. Higashi, Department of Pediatrics, Chiba Cardiovascular Center, 575 Tsurumai, Ichihara, Chiba 290-0512, Japan AU - Higashi, K. AU - Honda, T. AU - Tateno, S. AU - Kawasoe, Y. AU - Niwa, K. AU - Matsuda, S. AU - Ono, J. I. C1 - plasvata(Asahi Kasei,Japan) C2 - Asahi Kasei(Japan) DB - Embase Medline DO - 10.1111/j.1747-0803.2006.00061.x IS - 6 KW - acetylsalicylic acid digoxin dipeptidyl carboxypeptidase inhibitor diuretic agent dopamine enalapril fibrinolytic agent heparin tissue plasminogen activator warfarin adolescent aortic regurgitation aphasia article asplenia Blalock Taussig shunt cardiomegaly carotid arteriography case report computer assisted tomography cyanotic heart disease disease severity drug selectivity dyspnea electrocardiography guide wire heart failure heart single ventricle hemiparesis hospital discharge human internal carotid artery pulmonary valve atresia lung vein drainage anomaly male middle cerebral artery occlusion pericardial effusion peripheral edema physical examination priority journal retina artery occlusion thorax radiography treatment outcome two dimensional echocardiography valvular heart disease plasvata LA - English M3 - Article N1 - L44756303 2006-12-06 PY - 2006 SN - 1747-079X 1747-0803 SP - 343-345 ST - Successful selective intra-arterial thrombolytic therapy for embolic stroke in a patient with asplenia syndrome and unrepaired cyanotic congenital heart disease T2 - Congenital Heart Disease TI - Successful selective intra-arterial thrombolytic therapy for embolic stroke in a patient with asplenia syndrome and unrepaired cyanotic congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L44756303 http://dx.doi.org/10.1111/j.1747-0803.2006.00061.x VL - 1 ID - 1249 ER - TY - JOUR AB - Plastic bronchitis is a rare and often fatal complication of single-ventricle surgical palliation after total cavopulmonary connection. Although lymphatic abnormalities have been postulated to play a role in the disease process, the etiology and pathophysiology of this complication remain incompletely understood. Here we report on the etiology of plastic bronchitis in a child with total cavopulmonary connection as demonstrated by magnetic resonance (MR) lymphangiography. We also report on a new treatment of this disease. The patient underwent noncontrast T2-weighted MR lymphatic mapping and dynamic contrast MR lymphangiography with bi-inguinal intranodal contrast injection to determine the anatomy and flow pattern of lymph in his central lymphatic system. The MRI scan demonstrated the presence of a dilated right-sided peribronchial lymphatic network supplied by retrograde lymphatic flow through a large collateral lymphatic vessel originating from the thoracic duct. After careful analysis of the MRI scans we performed selective lymphatic embolization of the pathologic lymphatic network and supplying vessel. This provided resolution of plastic bronchitis for this patient. Five months after the procedure, the patient remains asymptomatic off respiratory medications. Copyright © 2014 by the American Academy of Pediatrics. AD - Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA, United States Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA, United States Division of Interventional Radiology, Hospital of the University of Pennsylvania, Philadelphia, PA, United States AU - Dori, Y. AU - Keller, M. S. AU - Rychik, J. AU - Itkin, M. DB - Scopus DO - 10.1542/peds.2013-3723 IS - 2 KW - Fontan Lymphatics Plastic bronchitis Single ventricle M3 - Article N1 - Cited By :61 Export Date: 15 June 2020 PY - 2014 SP - e590-e595 ST - Successful treatment of plastic bronchitis by selective lymphatic embolization in a Fontan patient T2 - Pediatrics TI - Successful treatment of plastic bronchitis by selective lymphatic embolization in a Fontan patient UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84905281494&doi=10.1542%2fpeds.2013-3723&partnerID=40&md5=c20e942f1ffc84f4baa97eeaa75aa732 VL - 134 ID - 1988 ER - TY - JOUR AB - OBJECTIVE: To report the first successful use of extracorporeal membrane oxygenation for acute respiratory distress syndrome secondary to 2009 pH1N1 influenza A infection in a child status post the Fontan operation for hypoplastic left heart syndrome. DESIGN: Individual case report. SETTING: Pediatric intensive care unit. PATIENT: We describe a 7-yr-old boy with a history of the Fontan operation for hypoplastic left heart syndrome admitted with acute respiratory distress syndrome secondary to 2009 pH1N1 influenza A infection. INTERVENTION: Cannulation for venoarterial extracorporeal membrane oxygenation. MEASUREMENTS AND MAIN RESULTS: In this patient with a history of complex congenital heart disease and repair, extracorporeal membrane oxygenation was a successful rescue therapy for refractory pH1N1-induced respiratory failure. CONCLUSION: Extracorporeal membrane oxygenation can be successfully applied for refractory respiratory failure, even in the setting of significant underlying comorbidity. With emerging data to support the role of extracorporeal membrane oxygenation in improving mortality for refractory hypoxemia secondary to pH1N1, it is prudent to strongly consider the use of extracorporeal support in patients with underlying diseases or comorbidities that may have previously precluded them from being candidates for this therapy. AD - Department of Pediatrics, Duke Children's Hospital, Duke University Medical Center, Durham, NC, USA. niyati.gandhi@duke.edu AN - 21116209 AU - Gandhi, N. N. AU - Hartman, M. E. AU - Williford, W. L. AU - Peters, M. A. AU - Cheifetz, I. M. AU - Turner, D. A. DA - Nov DB - PubMed DO - 10.1097/PCC.0b013e3181fe3189 DP - NLM ET - 2010/12/01 IS - 6 KW - Child Extracorporeal Membrane Oxygenation/*standards Humans *Hypoplastic Left Heart Syndrome Hypoxia/*etiology *Influenza A Virus, H1N1 Subtype Influenza, Human/*complications/virology Intensive Care Units, Pediatric Male North Carolina Respiratory Distress Syndrome, Adult LA - eng N1 - Gandhi, Niyati N Hartman, Mary E Williford, Walter L Peters, Michelle A Cheifetz, Ira M Turner, David A Case Reports Journal Article United States Pediatr Crit Care Med. 2011 Nov;12(6):e398-401. doi: 10.1097/PCC.0b013e3181fe3189. PY - 2011 SN - 1529-7535 (Print) 1529-7535 SP - e398-401 ST - Successful use of extracorporeal membrane oxygenation for pH1N1-induced refractory hypoxemia in a child with hypoplastic left heart syndrome T2 - Pediatr Crit Care Med TI - Successful use of extracorporeal membrane oxygenation for pH1N1-induced refractory hypoxemia in a child with hypoplastic left heart syndrome VL - 12 ID - 361 ER - TY - JOUR AB - Although SCD is relatively uncommon, its psychosocial impact is devastating. This article has reviewed the potential causes of SCD in infants, children, and adolescents. Many patients who die from SCD have identifiable cardiac disease and are known to have been at risk; however, the existence of other cardiac abnormalities, such as hypertrophic cardiomyopathy or long QT syndrome, may not be known, and SCD may be the first symptom. The authors' contention is that many of the patients in this latter group (e.g., patients who have hypertrophic cardiomyopathy or LQLTS but who have no symptoms) can be screened with a careful, accurate, and detailed history, including family history and review of systems, and physical examination. Any patient with a positive family history, positive review of systems, or positive physical examination should receive further in-depth evaluation, such as an BCG and echocardiogram. These studies permit the detection of most, if not all, of the entities potentially associated with SCD in the pediatric population. AD - Medical College of Wisconsin, 9000 West Wisconsin Avenue, Milwaukee, WI 53201, United States AU - Berger, S. AU - Dhala, A. AU - Friedberg, D. Z. DB - Scopus DO - 10.1016/S0031-3955(05)70114-0 IS - 2 M3 - Article N1 - Cited By :48 Export Date: 15 June 2020 PY - 1999 SP - 221-234 ST - Sudden cardiac death in infants, children, and adolescents T2 - Pediatric Clinics of North America TI - Sudden cardiac death in infants, children, and adolescents UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0032943785&doi=10.1016%2fS0031-3955%2805%2970114-0&partnerID=40&md5=6a809dc6d98bb3f85a8d976e7d98e5c9 VL - 46 ID - 2283 ER - TY - JOUR AB - Around 20,000 neonatal deaths occur each year, many from congenital heart defects such as hypoplastic left heart syndrome. Nurses are on the frontline of caring for families experiencing neonatal loss. Careful spiritual and cultural assessment, attention to beliefs, focusing on relationship, and helping families create legacy can assist with grieving and making meaning out of loss. AU - Holston, J. T. DB - Medline DO - 10.1097/CNJ.0000000000000118 IS - 1 KW - bereavement case report child death Christianity decision making empathy family female grief health personnel attitude human hypoplastic left heart syndrome infant male newborn newborn intensive care nurse attitude nursing nursing staff psychology terminal care LA - English M3 - Article N1 - L604451064 2015-05-26 PY - 2015 SN - 0743-2550 SP - 18-25 ST - Supporting families in neonatal loss: relationship and faith key to comfort T2 - Journal of Christian nursing : a quarterly publication of Nurses Christian Fellowship TI - Supporting families in neonatal loss: relationship and faith key to comfort UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L604451064 http://dx.doi.org/10.1097/CNJ.0000000000000118 VL - 32 ID - 886 ER - TY - JOUR AB - Background Short-term continuous-flow ventricular assist devices (STCF-VADs) are increasingly being used in the pediatric population. However, little is known about the outcomes in patients supported with these devices. Methods All pediatric patients supported with a STCF-VAD, including the Thoratec PediMag or CentriMag, or the Maquet RotaFlow, between January 2005 and May 2014, were included in this retrospective single-center study. Results Twenty-seven patients (15 girls [56%]) underwent 33 STCF-VAD runs in 28 separate hospital admissions. The STCF-VAD was implanted 1 time in 23 patients (85%), 2 times in 2 patients (7%), and 3 times in 2 patients (7%). Implantation occurred most commonly in the context of congenital heart disease in 14 runs (42.2%), cardiomyopathy in 11 (33%), and after transplant in 6 (18%). The median age at implantation was 1.7 (interquartile range [IQR] 0.1, 4.1) years, and median weight was 8.9 kg (IQR 3.7, 18 kg). Patients were supported for a median duration of 12 days (IQR 6, 23 days) per run; the longest duration was 75 days. Before implantation, 15 runs (45%) were supported by extracorporeal membrane oxygenation (ECMO). After implantation, an oxygenator was required in 20 runs (61%) and continuous renal replacement therapy in 21 (64%). Overall, 7 runs (21%) resulted in weaning for recovery, 14 (42%) converted to a long-term VAD, 4 (12%) resulted in direct transplantation, 3 (9%) were converted to ECMO, and 5 (15%) runs resulted in death on the device or within 1 month after decannulation. The most common complication was bleeding requiring reoperation in 24% of runs. In addition, 18% of runs were associated with neurologic events and 15% with a culture-positive infection. Hospital discharge occurred in 19 of 28 STCF-VAD admissions (67%). In follow-up, with a median duration of 9.2 months (IQR 2.3, 38.3 months), 17 patients (63%) survived. Conclusions STCF-VADs can successfully bridge most pediatric patients to recovery, long-term device, or transplant, with an acceptable complication profile. Although these devices are designed for short-term support, longer support is possible and may serve as an alternative approach to patients not suitable for the current long-term devices. © 2016 International Society for Heart and Lung Transplantation. AD - University of Alberta, Division of Pediatric Cardiology, 8440-112 St NW, Edmonton, AB T6G2B7, Canada Division of Pediatric Cardiac Surgery, Stollery Children's Hospital, Edmonton, AB, Canada Division of Pediatric Critical Care, Divisions of Cardiac Surgery, Canada Stollery Children's Hospital, Pediatric Cardiac Critical Care, University of Alberta, Edmonton, AB, Canada AU - Conway, J. AU - Al-Aklabi, M. AU - Granoski, D. AU - Islam, S. AU - Ryerson, L. AU - Anand, V. AU - Guerra, G. AU - Mackie, A. S. AU - Rebeyka, I. AU - Buchholz, H. DB - Scopus DO - 10.1016/j.healun.2016.01.1224 IS - 5 KW - CentriMag continuous-flow pediatric patients PediMag RotaFlow short-term support ventricular assist device M3 - Article N1 - Cited By :21 Export Date: 15 June 2020 PY - 2016 SP - 603-609 ST - Supporting pediatric patients with short-term continuous-flow devices T2 - Journal of Heart and Lung Transplantation TI - Supporting pediatric patients with short-term continuous-flow devices UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84961226703&doi=10.1016%2fj.healun.2016.01.1224&partnerID=40&md5=04daf9b734e20a28ec3c5d8d7772fcae VL - 35 ID - 1910 ER - TY - JOUR AB - The highest mortality from congenital cardiovascular anomalies occurs during infancy» and 80% of such patients die during the first year of life« usually because of anoxaemia or pulmonary congestion from cardiac failure. Surgical treatment provides palliation or complete cure in many such patients. In a series of 400 consecutive operations there were 288 survivors (72%). The commonest lesions (332 patients) were patent ductus arteriosus, tetralogy of Fallet, complete transposition of the great vessels, ventricular septal defect, coarctation of the aorta, tricuspid atresia, and total anomaly of pulmonary venous drainage. During the same period of this study 154 autopsies were performed on patients with congenital cardiovascular anomalies of which theoretically 70 could have been treated surgically. The authors stress the need for an aggressive diagnostic and therapeutic effort in infants with cardiovascular anomalies. AU - Cooley, D. DB - Embase Classic Medline IS - 9 KW - aorta autopsy cardiovascular disease cardiovascular malformation congenital disorder diagnosis great blood vessel heart failure heart surgery heart ventricle septum defect infancy infant lung congestion mortality newborn palliative therapy patent ductus arteriosus patient surgery survivor tricuspid valve atresia LA - English M3 - Article N1 - L280668792 1962-12-01 PY - 1962 SN - 0098-7484 SP - 912-917 ST - Surgery in the newborn for congenital cardiovascular lesions. Report of 400 consecutive operations T2 - JAMA (Chicago, Ill.) TI - Surgery in the newborn for congenital cardiovascular lesions. Report of 400 consecutive operations UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L280668792 VL - 182 ID - 1419 ER - TY - JOUR AB - A series of 33 patients were seen. Twenty-four died, and. on the basis of pathological findings in these cases, a simple classification was established. Incomplete obstruction of the tricuspid valve (stenosis) was associated with an intact ventricular septum and stenosis or atresia of the pulmonary outflow. The ductus arteriosus was usually patent. Complete obstruction of the tricuspid valve (atresia) was associated with a ventricular septal defect. The pulmonary outflow was usually normal and the ductus closed. The clinical features of this disease are reviewed in detail, and a method that will assist in differentiating tricuspid atresia from stenosis on the basis of ECG findings is outlined. The surgical management of tricuspid atreeia and stenosis ie emphaeized and, for reasons given, a palliative operation in the form of a partial venoue bypass of the right side of the heart is the preferred operation in most cases. In a few cases a systemic artery to pulmonary artery shunt is indicated, and, rarely, in cases of increased pulmonary blood flow, banding of the main pulmonary artery is required. Of particular interest were a few cases of tricuspid stenosis with associated pulmonary atreeia or stenosis, in which valvu lotomy may have been effective in increasing the blood flow to the lungs. For tricuspid atresia at the present time there appears to be little hope of developing a corrective procedure. Even should a satisfactory prosthetic valve become available, the underdeveloped right ventricle would probably prevent the proper functioning of the valve. The results of 197 shunt operations (1-74 systemic artery to pulmonary artery and 23 systemic vein to pulmonary artery) are reviewed. The immediate operative mortality is approximately the same in both groups. It is anticipated that the long-term results may be better with the vena cava-pulmonary artery anastomosis, as the work of the heart is lessened by this procedure. The technique of performance of the vena cava-pulmonary artery shunt in the small infant is given in some detail. It cannot be too strongly emphasized that meticulous attention to minute details in the selection of patients for operation, the performance of the operative procedure, and in the pre- and postoperative care of the patient with tricuspid valve obstruction is absolutely essential to the successful outcome of a vena cava to pulmonary artery anastomosis. (XVIII, 9). AD - R.K. Bopp, Depts of Surg.. Ped., Pathol. and Radio 1, Yale Univ., Sch. of Med., New Haven AU - Bopp, R. K. AU - Larsen, P. B. AU - Caddell, J. L. AU - Patrick, J. R. AU - Hipona, F. A. AU - Glenn, W. W. L. DB - Embase Classic Medline IS - 1 KW - artery artery anastomosis artery bypass atresia blood flow cava vein classification clinical feature ductus arteriosus electrocardiogram heart heart right ventricle heart surgery interventricular septum heart ventricle septum defect hope infant lung lung blood flow obstruction patent patient postoperative care pulmonary artery shunting stenosis surgery surgical mortality tricuspid valve tricuspid valve atresia tricuspid valve stenosis vein LA - English M3 - Article N1 - L281361088 1962-12-01 PY - 1962 SP - 97-113 ST - SURGICAL CONSIDERATIONS for TREATMENT of CONGENITAL TRICUSPID ATRESIA and STENOSIS; with PARTICULAR REFERENCE to VENA CAVA-PULMONARY ARTERY ANASTOMOSIS T2 - J. THORAC. CARDIOV. SURG. TI - SURGICAL CONSIDERATIONS for TREATMENT of CONGENITAL TRICUSPID ATRESIA and STENOSIS; with PARTICULAR REFERENCE to VENA CAVA-PULMONARY ARTERY ANASTOMOSIS UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L281361088 VL - 43 ID - 1420 ER - TY - JOUR AB - Ebstein's malformation is a congenital anomaly of the tricuspid valve and right ventricle. Surgical repair of Ebstein's anomaly improves functional class and exercise tolerance, eliminates right-to-left intracardiac shunting (if present), and reduces the incidence of supraventricular tachyarrhythmias. As a result, quality of life and survival are improved. Because of the variable degree of malformation present, repair is predicated on favorable anatomic factors, most importantly the arrangement of the anterior leaflet of the tricuspid valve. When anatomic derangements threaten a durable tricuspid valve repair, valve replacement with protection of the conduction tissue and right coronary artery should be performed. The vast majority of patients can undergo a biventricular repair. The application of the bidirectional cavopulmonary anastomosis is reserved for patients with poor right ventricular function. Freedom from reoperation after tricuspid valve repair is similar when compared with valve replacement. In the current era, overall early mortality after surgical repair in children and adults has fallen to less than 3% in experienced centers. Surgical treatment of the symptomatic neonate remains a significant challenge, with approaches that include either a biventricular or single ventricle algorithm. AD - Division of Cardiovascular Surgery, Mayo Clinic and Foundation, Rochester, MN 55905, USA. AN - 17434001 AU - Stulak, J. M. AU - Dearani, J. A. AU - Danielson, G. K. DB - PubMed DO - 10.1053/j.pcsu.2007.01.007 DP - NLM ET - 2007/04/17 KW - Adolescent Adult Age Factors Aged Bioprosthesis *Cardiac Surgical Procedures/methods Child Child, Preschool Ebstein Anomaly/complications/epidemiology/physiopathology/*surgery Heart Valve Prosthesis Implantation Humans Infant Middle Aged Minnesota/epidemiology Retrospective Studies Risk Factors Survival Analysis Suture Techniques Tachycardia, Supraventricular/etiology/physiopathology/prevention & control/surgery Treatment Outcome Ventricular Dysfunction, Left/etiology/physiopathology/prevention & control Ventricular Dysfunction, Right/etiology/physiopathology/prevention & control LA - eng N1 - Stulak, John M Dearani, Joseph A Danielson, Gordon K Journal Article United States Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2007:105-11. doi: 10.1053/j.pcsu.2007.01.007. PY - 2007 SN - 1092-9126 (Print) 1092-9126 SP - 105-11 ST - Surgical management of Ebstein's anomaly T2 - Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu TI - Surgical management of Ebstein's anomaly ID - 395 ER - TY - JOUR AB - Heterotaxy syndrome encompasses a wide range of anatomical variants including poor pulmonary vascular development. Surgical outcome remains poor in this difficult subgroup. Between April 1996 and November 2004, 27 patients with visceral heterotaxy were enrolled in this study. The median age at presentation was 25 days. There were 11 patients with asplenia and 16 with polysplenia. There were 5 deaths (4 in patients with asplenia, 1 in a patient with polysplenia). All patients with asplenia were scheduled for single-ventricle repair, and 5 completed a modified Fontan procedure. Of the 16 patients with polysplenia, 8 completed 2-ventricle repair and 6 had a modified Fontan. In polysplenia, actuarial survival was 93.8% at 4 months, and remained constant thereafter. In asplenia, survival was 81.8% at 1 month, and 53.0% at 1 year. A significant correlation was found between nonconfluent pulmonary arteries and mortality. The medium-term result in polysplenia was satisfactory, but not in asplenia. Nonconfluent pulmonary arteries carry a high risk of mortality, and a strategy to create pulmonary vascular confluence and satisfactory pulmonary blood flow is mandatory. AD - K. Takeuchi, Department of Cardiovascular Surgery, National Children's Hospital, National Center for Child Health and Development, 2-10-1, Ohkura, Setagaya-ku, Tokyo 157-8535, Japan AU - Takeuchi, K. AU - Murakami, A. AU - Hirata, Y. AU - Kitahori, K. AU - Doi, Y. AU - Takamoto, S. DB - Embase Medline DO - 10.1177/021849230601400610 IS - 6 KW - accessory spleen article asplenia child correlation analysis female Fontan procedure heart surgery heterotaxy syndrome high risk population human infant lung artery banding pulmonary valve atresia lung blood flow lung blood vessel male outcome assessment patient satisfaction pulmonary valve stenosis risk assessment surgical mortality survival time LA - English M3 - Article N1 - L44883680 2007-01-03 PY - 2006 SN - 0218-4923 SP - 489-494 ST - Surgical outcome of heterotaxy syndrome in a single institution T2 - Asian Cardiovascular and Thoracic Annals TI - Surgical outcome of heterotaxy syndrome in a single institution UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L44883680 http://dx.doi.org/10.1177/021849230601400610 VL - 14 ID - 1247 ER - TY - JOUR AB - OBJECTIVE: The study objective was to describe the surgical pathway progression through adolescence of an inception cohort of neonates with aortic valve atresia managed initially with surgical palliation or primary transplantation, comparing survival and self-reported health-related quality of life. METHODS: From 1994 to 2000, 565 neonates with aortic atresia were admitted to 26 Congenital Heart Surgeons' Society hospitals and followed annually for vital status. Initial management included surgical palliation (n = 453) and primary cardiac transplantation (n = 68). PedsQL health-related quality of life questionnaires were sent cross-sectionally to a subgroup of 198 patients alive at previous follow-up, with 80 responses. RESULTS: Risk of death was initially high for both treatment strategies. However, compared with initial surgical palliation, survival with primary transplantation, including wait-list mortality, was greater and persisted long-term (65% vs 40% at 15 years; P = .002). Survival after secondary transplantation (48% at 9 years) was lower than after primary transplantation (74%). Health-related quality of life total score was lower overall than that of the general adolescent population (71 ± 16 vs 84 ± 13; P = .0001; normal = 100), but similar to that of adolescents with chronic diseases. It was similar in the surgical palliation and primary transplantation groups (70 ± 16 vs 75 ± 15; P = .3). Patients who received surgical palliation reported more symptoms (76 ± 15 vs 63 ± 18; P = .02). CONCLUSIONS: Patients receiving primary heart transplantation for aortic atresia in 1994 to 2000 experienced better survival, fewer symptoms, and equivalent quality of life compared with those undergoing initial surgical palliation. Notwithstanding the limited availability of neonatal and infant donor hearts, primary transplantation may be considered for those neonates with risk factors predictive of exceptionally poor survival after surgical palliation. AD - Department of Surgery, Beth Israel Deaconess Medical Center, Boston, Mass. Department of Cardiology, Hospital for Sick Children, Toronto, Ontario, Canada. Department of Thoracic and Cardiovascular Surgery, Cleveland Clinic, Cleveland, Ohio; Department of Quantitative Health Sciences, Cleveland Clinic, Cleveland, Ohio. Department of Quantitative Health Sciences, Cleveland Clinic, Cleveland, Ohio. Division of Cardiothoracic Surgery, University of Alabama at Birmingham, Birmingham, Ala. Department of Cardiothoracic Surgery, Loma Linda University, Loma Linda, Calif. Division of Cardiac Surgery, Johns Hopkins, Baltimore, Md. Division of Pediatric Cardiovascular Surgery, Montreal Children's Hospital, Montreal, Quebec, Canada. Johns Hopkins University, Baltimore, Md. Department of Thoracic and Cardiovascular Surgery, Cleveland Clinic, Cleveland, Ohio. Electronic address: petterg@ccf.org. AN - 31740116 AU - Stackhouse, K. A. AU - McCrindle, B. W. AU - Blackstone, E. H. AU - Rajeswaran, J. AU - Kirklin, J. K. AU - Bailey, L. L. AU - Jacobs, M. L. AU - Tchervenkov, C. I. AU - Jacobs, J. P. AU - Pettersson, G. B. DA - Apr DB - PubMed DO - 10.1016/j.jtcvs.2019.08.104 DP - NLM ET - 2019/11/20 IS - 4 KW - Adolescent *Aortic Valve Child Child, Preschool Cohort Studies Female *Heart Transplantation Heart Valve Diseases/mortality/*surgery Heart Valve Prosthesis Implantation Humans Infant Infant, Newborn Male Norwood Procedures *Palliative Care Quality of Life Survival Rate Treatment Outcome *Fontan *aortic atresia *neonatal *quality of life *surgical palliation *survival *transplantation LA - eng N1 - 1097-685x Stackhouse, Kathryn A McCrindle, Brian W Blackstone, Eugene H Rajeswaran, Jeevanantham Kirklin, James K Bailey, Leonard L Jacobs, Marshall L Tchervenkov, Christo I Jacobs, Jeffrey P Pettersson, Gösta B Congenital Heart Surgeons’ Society Journal Article United States J Thorac Cardiovasc Surg. 2020 Apr;159(4):1451-1461.e7. doi: 10.1016/j.jtcvs.2019.08.104. Epub 2019 Sep 25. PY - 2020 SN - 0022-5223 SP - 1451-1461.e7 ST - Surgical palliation or primary transplantation for aortic valve atresia T2 - J Thorac Cardiovasc Surg TI - Surgical palliation or primary transplantation for aortic valve atresia VL - 159 ID - 360 ER - TY - JOUR AB - Objective: The study objective was to describe the surgical pathway progression through adolescence of an inception cohort of neonates with aortic valve atresia managed initially with surgical palliation or primary transplantation, comparing survival and self-reported health-related quality of life. Methods: From 1994 to 2000, 565 neonates with aortic atresia were admitted to 26 Congenital Heart Surgeons' Society hospitals and followed annually for vital status. Initial management included surgical palliation (n = 453) and primary cardiac transplantation (n = 68). PedsQL health-related quality of life questionnaires were sent cross-sectionally to a subgroup of 198 patients alive at previous follow-up, with 80 responses. Results: Risk of death was initially high for both treatment strategies. However, compared with initial surgical palliation, survival with primary transplantation, including wait-list mortality, was greater and persisted long-term (65% vs 40% at 15 years; P =.002). Survival after secondary transplantation (48% at 9 years) was lower than after primary transplantation (74%). Health-related quality of life total score was lower overall than that of the general adolescent population (71 ± 16 vs 84 ± 13; P =.0001; normal = 100), but similar to that of adolescents with chronic diseases. It was similar in the surgical palliation and primary transplantation groups (70 ± 16 vs 75 ± 15; P =.3). Patients who received surgical palliation reported more symptoms (76 ± 15 vs 63 ± 18; P =.02). Conclusions: Patients receiving primary heart transplantation for aortic atresia in 1994 to 2000 experienced better survival, fewer symptoms, and equivalent quality of life compared with those undergoing initial surgical palliation. Notwithstanding the limited availability of neonatal and infant donor hearts, primary transplantation may be considered for those neonates with risk factors predictive of exceptionally poor survival after surgical palliation. © 2019 The American Association for Thoracic Surgery AD - Department of Surgery, Beth Israel Deaconess Medical Center, Boston, Mass, United States Department of Cardiology, Hospital for Sick Children, Toronto, Ontario, Canada Department of Thoracic and Cardiovascular Surgery, Cleveland Clinic, Cleveland, OH, United States Department of Quantitative Health Sciences, Cleveland Clinic, Cleveland, OH, United States Division of Cardiothoracic Surgery, University of Alabama at Birmingham, Birmingham, Ala, United Kingdom Department of Cardiothoracic Surgery, Loma Linda University, Loma Linda, Calif, United States Division of Cardiac Surgery, Johns Hopkins, Baltimore, Md, United States Division of Pediatric Cardiovascular Surgery, Montreal Children's Hospital, Montreal, Quebec, Canada Johns Hopkins University, Baltimore, Md, United States AU - Stackhouse, K. A. AU - McCrindle, B. W. AU - Blackstone, E. H. AU - Rajeswaran, J. AU - Kirklin, J. K. AU - Bailey, L. L. AU - Jacobs, M. L. AU - Tchervenkov, C. I. AU - Jacobs, J. P. AU - Pettersson, G. B. AU - Congenital Heart Surgeons, Society DB - Scopus DO - 10.1016/j.jtcvs.2019.08.104 IS - 4 KW - aortic atresia Fontan neonatal quality of life surgical palliation survival transplantation M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2020 SP - 1451-1461.e7 ST - Surgical palliation or primary transplantation for aortic valve atresia T2 - Journal of Thoracic and Cardiovascular Surgery TI - Surgical palliation or primary transplantation for aortic valve atresia UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85075364999&doi=10.1016%2fj.jtcvs.2019.08.104&partnerID=40&md5=3742b458572d776b4d3f48992031f0a4 VL - 159 ID - 1703 ER - TY - JOUR AB - Peripheral pulmonary artery stenosis is a relatively rare form of congenital heart disease typically associated with genetic syndromes, such as Williams or Alagille syndromes. However, some patients present with severe stenosis without associated syndromes. The purpose of the study was to review our surgical experience in such patients. This was a retrospective review of 30 patients who underwent surgical repair for peripheral pulmonary artery stenosis. Concomitant anatomical diagnoses in 20 patients (67%) included supravalvar aortic stenosis (n = 8), tetralogy of Fallot (n = 4), d-transposition of the great arteries (n = 2), truncus arteriosus (n = 2), hypoplastic left heart syndrome (n = 2), ventricular septal defect (n = 1), and patent ductus arteriosus (n = 1). Additional medical diagnoses in 15 patients (50%) included elastin arteriopathy (n = 9), pulmonary artery calcinosis (n = 1), arterial tortuosity syndrome (n = 1), DiGeorge syndrome (n = 1), and Noonan syndrome (n = 1). Median age at surgery was 3.6 years (interquartile range 1.6–7.4 years). Seventeen patients (57%) had prior cardiac operations, and 16 patients (53%) previously underwent percutaneous intervention. With surgery, mean right ventricle-to-aortic systolic pressure ratio decreased from 0.95 ± 0.2 to 0.28 ± 0.08 (P< 0.0001). Median duration of cardiopulmonary bypass was 369 minutes. There was 100% survival to hospital discharge, with no mortality at mean follow-up of 2.3 years. No patient required reoperation, while 4 underwent balloon dilation. Freedom from pulmonary artery catheter-based reintervention was 95% and 80% at 12 and 36 months, respectively. Patients with peripheral pulmonary artery stenosis without either Williams or Alagille syndrome can successfully undergo surgical repair with a significant reduction in right ventricle-to-aortic pressure ratios. AD - R.D. Mainwaring, Stanford University School of Medicine, 300 Pasteur Drive, Falk CVRC, Stanford, CA, United States AU - Martin, E. AU - Mainwaring, R. D. AU - Collins, R. T. AU - MacMillen, K. L. AU - Hanley, F. L. DB - Embase Medline DO - 10.1053/j.semtcvs.2020.01.003 KW - Alagille syndrome aortic pressure artery disease balloon dilatation calcinosis cardiopulmonary bypass child clinical article conference paper congenital heart disease controlled study DiGeorge syndrome Fallot tetralogy female follow up great vessels transposition heart right ventricle heart ventricle septum defect hospital discharge human hypoplastic left heart syndrome male mortality Noonan syndrome patent ductus arteriosus preschool child pulmonary artery catheter pulmonary artery stenosis pulmonary valve reoperation retrospective study supravalvular aortic stenosis surgery survival systolic blood pressure Williams Beuren syndrome elastin endogenous compound LA - English M3 - Article in Press N1 - L2004926229 2020-02-24 PY - 2020 SN - 1532-9488 1043-0679 ST - Surgical Repair of Peripheral Pulmonary Artery Stenosis in Patients Without Williams or Alagille Syndromes T2 - Seminars in Thoracic and Cardiovascular Surgery TI - Surgical Repair of Peripheral Pulmonary Artery Stenosis in Patients Without Williams or Alagille Syndromes UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2004926229 http://dx.doi.org/10.1053/j.semtcvs.2020.01.003 ID - 574 ER - TY - JOUR AB - Background. Pulmonary vein stenosis (PVS), both congenital and acquired, is challenging to treat surgically with uncertain long-term results. We reviewed an 11-year surgical experience in 52 children. Methods. From 2002 to 2012, 52 children age 0 days to 13 years (mean 1.9 years, median 11.7 months) weighing 2.2 to 32.5 kg (mean 9.3 kg, median 7.6 kg) had surgical relief of PVS. Based on clinical characteristics or complexity, 33 (63%) had a sutureless pericardial well repair and 19 (37%) had a more standard patch repair. There were no signi ficant differences in clinical characteristics between the 2 techniques. Twenty children (38%) had prior anomalous pulmonary vein repair and 8 had primary pulmonary vein stenosis; 26 (50%) had other operations at the time of PVS relief. Results. There were 2 hospital deaths (10.5%) in the "standard" group and 5 (15.2%) in the sutureless group (p > 0.99). Despite postoperative evidence of PVS relief by echocardiogram or cardiac cath in all patients, at 5 years, actuarial freedom from PVS recurrence or death in the hospital survivors was 67% in the standard group and 58% in the sutureless group. Most recurrences or deaths occurred within 6 months of operation. Heterotaxy, single ventricle anatomy, bilateral disease, and previous anomalous pulmonary vein repair were not predictors of failure. Conclusions. Surgical treatment of pulmonary vein stenosis remains a challenging problem with nontrivial early mortality and ongoing risk for recurrence or death regardless of surgical technique employed. Clearly, development of methods for earlier intervention or detection and improved surgical techniques are warranted. AD - K.R. Kanter, Pediatric Cardiac Surgery, Emory University, School of Medicine, 1405 Clifton Rd, Atlanta, GA, United States AU - Kanter, K. R. AU - Kirshbom, P. M. AU - Kogon, B. E. DB - Embase Medline DO - 10.1016/j.athoracsur.2014.05.082 IS - 5 KW - adolescent article cardiopulmonary bypass catheterization child echocardiography follow up Fontan procedure Glenn shunt heart catheterization heart single ventricle heart transplantation hospital discharge human hypoplastic left heart syndrome infant infant mortality lung vein drainage anomaly major clinical study newborn Norwood procedure patient history of surgery patient satisfaction postoperative period priority journal pulmonary vein malformation pulmonary vein obstruction recurrent disease reoperation retrospective study surgical mortality survivor LA - English M3 - Article N1 - L53295021 2014-08-26 2015-02-18 PY - 2014 SN - 1552-6259 0003-4975 SP - 1687-1691 ST - Surgical repair of pulmonary venous stenosis: A word of caution T2 - Annals of Thoracic Surgery TI - Surgical repair of pulmonary venous stenosis: A word of caution UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L53295021 http://dx.doi.org/10.1016/j.athoracsur.2014.05.082 VL - 98 ID - 961 ER - TY - JOUR AB - The results of operation in all patients with univentricular heart and an obstructed anterior subaortic outlet chamber who were operated on utilizing extracorporeal circulation at the Mayo Clinic from 1973 through 1983 were reviewed. Ten of the 18 patients died during the immediate postoperative period and there was one late death. Factors significantly related to operative and immediate postoperative mortality were age at operation, cardiothoracic ratio on X-ray examination, degree of ST depression on electrocardiogram and pressure gradient across the outlet foramen at catheterization. Autopsy in eight cases revealed significant hypertrophy of ventricular myocardium and a small outlet foramen that was considered stenotic relative to either body surface area or aortic root area. The ventricular myocardium showed histologic changes of chronic ischemia that predated the surgical procedure. AN - 6541233 AU - Barber, G. AU - Hagler, D. J. AU - Edwards, W. D. AU - Puga, F. J. AU - Danielson, G. K. AU - McGoon, D. C. AU - Driscoll, D. J. DA - Oct DB - PubMed DO - 10.1016/s0735-1097(84)80405-2 DP - NLM ET - 1984/10/01 IS - 4 KW - Adolescent Adult Aortic Stenosis, Subvalvular/*complications/pathology/surgery Cardiac Catheterization Cardiomyopathy, Hypertrophic/*complications Child Child, Preschool Echocardiography Electrocardiography Heart Ventricles/*abnormalities/pathology/surgery Humans Methods Myocardium/pathology Pressure LA - eng N1 - Barber, G Hagler, D J Edwards, W D Puga, F J Danielson, G K McGoon, D C Driscoll, D J Journal Article United States J Am Coll Cardiol. 1984 Oct;4(4):771-8. doi: 10.1016/s0735-1097(84)80405-2. PY - 1984 SN - 0735-1097 (Print) 0735-1097 SP - 771-8 ST - Surgical repair of univentricular heart (double inlet left ventricle) with obstructed anterior subaortic outlet chamber T2 - J Am Coll Cardiol TI - Surgical repair of univentricular heart (double inlet left ventricle) with obstructed anterior subaortic outlet chamber VL - 4 ID - 406 ER - TY - JOUR AB - Background: Tetralogy of Fallot, or double-outlet right ventricle with atrioventricular (AV) septal defect (TOF/DORV-AVSD), is rare, with limited long-term data available. We report our institutional experience and outcome over a 50-year period. Methods: From January 1961 to January 2011, 73 patients (50 males [68%]), with a mean age of 6.8 ± 4.4 years (range, 1 month to 35 years), underwent surgical repair of TOF/DORV-AVSD. Symptoms included cyanosis in 50 (69%) and heart failure in 12 (17%). Down syndrome was present in 25 (34%). Rastelli type A, B, and C was seen in 12%, 7%, and 81% of patients, respectively. Moderate or more common AV valve (AVV) regurgitation was present in 40%. Forty-nine patients (67%) had previous palliation, including 36 with a systemic-to-pulmonary arterial shunt. Results: Surgical management included two-ventricle complete repair (CR) in 35 (48%) and single-ventricle (SV) palliation in 38 (52%). Overall, early mortality was 31% for CR and 34% for SV; after 1990, mortality was 6% for CR and 14% for SV. Repair before 1990 (p = 0.008) and the presence of significant common AVV regurgitation (p = 0.016) were univariate risk factors for early death in both groups. Median follow-up was 9.8 years (maximum, 32 years). Late mortality rate was 12% in CR (n = 6) and 18% (n = 9) in SV (p = 0.95). The presence of significant right AVV regurgitation was associated with late death (p = 0.02). Overall survival at 1, 5, and 15 years was 92%, 77%, and 77% in CR, and 83%, 79%, 70% in SV (p = 0.9). Freedom from reoperation at 1, 5, and 15 years was 95%, 85%, 67% in CR and 96%, 91%, 82% in SV (p = 0.1). Reoperations were most common for right ventricular outflow tract pathology, Fontan revision, and AVV intervention. Right AVV regurgitation (p = 0.018) and repair before 1990 (p = 0.041) were risk factors for late reoperation in both groups. Conclusions: Complete repair of TOF/DORV-AVSD is standard of care and associated with low early mortality rate in the current era, with reasonable long-term outcome. SV palliation continues to have significant risk. The presence of AVV regurgitation is a significant risk factor for death and reoperation. © 2013 The Society of Thoracic Surgeons. AD - H.M. Burkhart, Mayo Clinic College of Medicine, Division of Cardiovascular Surgery, 200 First St SW, Rochester, MN 55905, United States AU - Raju, V. AU - Burkhart, H. M. AU - Rigelman Hedberg, N. AU - Eidem, B. W. AU - Li, Z. AU - Connolly, H. AU - Schaff, H. V. AU - Dearani, J. A. DB - Embase Medline DO - 10.1016/j.athoracsur.2013.02.016 IS - 6 KW - adult article atrioventricular septal defect cardiovascular mortality cardiovascular risk cardiovascular surgery child disease severity Down syndrome Fallot tetralogy female follow up heart right ventricle double outlet heart right ventricle outflow tract heart single ventricle heart valve regurgitation human infant major clinical study male overall survival palliative therapy preschool child priority journal reoperation school child LA - English M3 - Article N1 - L52544058 2013-04-22 2013-06-06 PY - 2013 SN - 0003-4975 1552-6259 SP - 2079-2085 ST - Surgical strategy for atrioventricular septal defect and tetralogy of fallot or double-outlet right ventricle T2 - Annals of Thoracic Surgery TI - Surgical strategy for atrioventricular septal defect and tetralogy of fallot or double-outlet right ventricle UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52544058 http://dx.doi.org/10.1016/j.athoracsur.2013.02.016 VL - 95 ID - 997 ER - TY - JOUR AB - It is anticipated that as many as 10-20% of patients alive with anatomical congenital heart lesions may eventually develop heart failure. Most of these patients have undergone previous palliative or corrective surgeries. The Fontan procedure, although it has helped many patients with single-ventricle physiology to become hemodynamically functional, it is associated with  protein-losing entropathy, intractable atrial arryhythmia and systemic ventricular dysfunction. In patients with transposition of the great arteries, physiological or intra-atrial repair techniques (Mustard and Senning) or anatomical correction (arterial switch) have been used. The majority of these patients are clinically well throughout their adult life but, as a consequence of the potential for right ventricular failure, some may develop cardiac insufficiency. Although long-term survival and quality of life in children and adults with complex congenital heart disease have remarkably improved due to advances in operative techniques and perioperative management, as well as the increasing experience of congenital heart surgeons, a growing number of these patients eventually develop end-stage heart failure and will require another treatment. Surgical options for treatment of these patients are limited. Heart transplantation has become a well-established treatment option for children as well as adults with end-stage congenital heart  disease. Because of organ donor shortage, another option is mechanical circulatory assist device implantation, either as a bridge to transplantation, or as a permanent therapy. AD - Department of Cardiothoracic and Vascular Surgery, Deutsches Herzzentrum Berlin, Germany. AN - 23888229 AU - Delmo Walter, E. M. AU - Hetzer, R. C2 - PMC3722338 DB - PubMed DP - NLM ET - 2013/07/28 IS - 2 KW - atrial switch end-stage heart failure heart transplantation mechanical circulatory assist devices single ventricle physiology LA - eng N1 - 2037-0512 Delmo Walter, E M Hetzer, R Journal Article HSR Proc Intensive Care Cardiovasc Anesth. 2013;5(2):81-4. PY - 2013 SN - 2037-0504 (Print) 2037-0504 SP - 81-4 ST - Surgical treatment concepts for end-stage congenital heart diseases T2 - HSR Proc Intensive Care Cardiovasc Anesth TI - Surgical treatment concepts for end-stage congenital heart diseases VL - 5 ID - 404 ER - TY - JOUR AB - BACKGROUND: There remains controversy regarding the appropriate surgical treatment of coarctation of the aorta associated with intracardiac anomalies in neonates and infants. Furthermore, the relative benefits of one versus two-stage repair, and subclavian flap aortoplasty versus end-to-end anastomosis for some of these lesions, remain controversial. The purpose of this paper is to review our experience with two-stage repair using subclavian flap aortoplasty and to seek an appropriate procedure. METHODS AND RESULT: From June 1996 to November 1999, thirteen patients underwent subclavian flap aortoplasty in our department. The age range was 16 to 101 days (mean 52 days), and the body weight range was 1.9 to 4.5 kg (mean 3.0 kg). Anatomic diagnosis was coarctation with ventricular septal defect (six patients), double outlet right ventricle (two patients), atrioventricular canal defect (one patient), tricuspid atresia (two patients), mitral atresia (one patient), and single atrium and subaortic stenosis (one patient). There was one hospital death in our series due to the progression of pulmonary hypertension 3 months after the operation. The mean follow up for remaining twelve patients was 28 months (range 7 approximately 48 months). There was one reoperation for recurrent coarctation. Three patients underwent pulmonary artery plasty in a second operation because of right pulmonary artery stenosis. We performed the definitive operation for six patients with coarctation with ventricular septal defect and two patients with double outlet right ventricle, and we performed a bidirectional cavopulmonary shunt for four univentricular hearts who are candidates for the Fontan operation. Two patients required Damus-Kaye-Stansel procedure to release restrictive bulboventricular foramen. Three patients underwent a modified Fontan operation after these palliations. In our series, the intraoperative mortality rate for subclavian flap aortoplasty was 0% and the post operative mortality rate was 7.7% (1/13). Ten patients underwent the final operation successfully, and further two patients are considered good candidates for the final operation. The overall mortality was 7.7% (1/13). CONCLUSION: Two-stage repair appears to offer a good prognosis for neonates and infants with a coarctation complex. Subclavian flap aortoplasty showed the lowest rate of restenosis. However, late mortality may be associated with the progression of pulmonary vascular disease and the presence of associated severe cardiac anomalies. Although Fontan candidates need staged operations, if biventricular repair is feasible, one-stage repair would be a reasonable procedure considering the progression of the pulmonary vascular disease and the distortion of the pulmonary artery due to pulmonary artery banding. It would appear to improve the quality of life of those children if a one-stage operation can be performed with reasonable risk and good midterm outcome. AD - Department of Surgery II, Division of Cardiovascular Surgery, Nippon Medical School, Tokyo, Japan. yamauchi_hitoshi/surg2@nms.ac.jp AN - 11116241 AU - Yamauchi, H. AU - Fujii, M. AU - Iwaki, H. AU - Ishii, Y. AU - Saji, Y. AU - Maruyama, Y. AU - Yamashita, H. AU - Uchikoba, Y. AU - Fukazawa, R. AU - Okubo, T. AU - Ogawa, S. AU - Tanaka, S. DA - Dec DB - PubMed DO - 10.1272/jnms.67.455 DP - NLM ET - 2000/12/16 IS - 6 KW - Aorta Aortic Coarctation/complications/*surgery Cardiovascular Surgical Procedures/methods Female Follow-Up Studies Heart Defects, Congenital/complications/*surgery Humans Infant Infant, Newborn Male Retrospective Studies Surgical Flaps Treatment Outcome LA - eng N1 - Yamauchi, H Fujii, M Iwaki, H Ishii, Y Saji, Y Maruyama, Y Yamashita, H Uchikoba, Y Fukazawa, R Okubo, T Ogawa, S Tanaka, S Journal Article Japan J Nippon Med Sch. 2000 Dec;67(6):455-8. doi: 10.1272/jnms.67.455. PY - 2000 SN - 1345-4676 (Print) 1345-4676 SP - 455-8 ST - Surgical treatment of coarctation complex in neonates and infants T2 - J Nippon Med Sch TI - Surgical treatment of coarctation complex in neonates and infants VL - 67 ID - 544 ER - TY - JOUR AB - Mortality during the first year of life in patients with congenital cardiovascular disease is approximately 60% without surgical treatment. Operation was undertaken in a consecutive series of 1,050 infants in this age group between 1954 and 1970, with survival of 721 Among the anomalies were patent ductus arteriös us, coarctation, aortic stenosis, pulmonary stenosis, aortic vascular ring, total anomalous pulmonary venous return, tricuspid atresia, tetralogy of Fallot, transposition of the great vessels, ventricular septal defect, pulmonary valve atresia and miscellaneous anomalies. Corrective operations were performed when feasible. A palliative operation was chosen for patients with complex anomalies such as transposition of the great vessels, deferring total correction until a more favorable age for open heart surgery was reached. While two staged procedures were used for several anomalies in this series, the authors recently adopted a more aggressive attitude toward total correction during infancy. To achieve open heart conditions they are employing induced hypothermia by surface cooling, temporary venous inflow occlusion and rewarming by partial cardiopulmonary bypass. AD - D.A. Cooley, Div. Surg., Texas Heart Inst., Houston, TX, United States AU - Cooley, D. A. AU - Mailman, G. L. AU - Wukasch, D. C. DB - Embase Classic Medline IS - 3 KW - aggression aortic stenosis cardiopulmonary bypass cardiovascular disease congenital heart disease cooling cryoanesthesia Fallot tetralogy great blood vessel groups by age hand malformation heart heart surgery heart ventricle septum defect induced hypothermia infancy infant lung vein drainage anomaly mortality occlusion open heart surgery patent patient pulmonary valve atresia pulmonary valve stenosis surgery survival tricuspid valve atresia vascular ring warming LA - English M3 - Article N1 - L293113502 1972-12-01 PY - 1972 SP - 232-237 ST - Surgical treatment of congenital heart disease during infancy: One and two stage techniques T2 - J.Cardiovasc.Surg. (Torino) TI - Surgical treatment of congenital heart disease during infancy: One and two stage techniques UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L293113502 VL - 13 ID - 1402 ER - TY - JOUR AB - The surgical treatment of congenital heart disease with cyanosis is briefly discussed. Among these anomalies the authors include: atrial septal defect associated with valvular pulmonary stenosis; tetralogy of Fallot; complete transposition of the great vessels; total pulmonary vein transposition (total anomalous venous return); tricuspid valve atresia or stenosis; Ebstein's anomaly, and truncus arteriosus peristens. The surgical management of each of these anomalies is discussed, from total correction to temporary or permanent palliation. In this discussion, special emphasis is placed on the common fields which radiologist and surgeon must have in the treatment of these patients. Good teamwork between the cardiologist, the radiologist and the surgeon is an essential condition for the success of these surgical procedures. This holds even more in cases involving emergency operations, such as the making of an atria I septal defect in patients with transpositions of the great vessels or the blind splitting of the pulmonary artery valve in severely cyanotic infants. The emergency operation performed by the surgeon in such cases could not save lives if he were not supported by a cardiologist and radiologist prepared to perform emergency diagnostic procedures at any hour of the day or night. AD - H. Van Der, Dept. Thor. Surg., Univ. Hosp., Groningen, Netherlands AU - Van Der, H. AU - Heide, J. N. DB - Embase Classic IS - 2 KW - arterial trunk cardiologist congenital heart disease cyanosis diagnosis diagnostic procedure Ebstein anomaly emergency emergency surgery Fallot tetralogy great blood vessel heart atrium heart atrium septum defect infant night palliative therapy patient pulmonary artery pulmonary valve stenosis pulmonary vein radiologist stenosis surgeon surgery surgical technique teamwork tricuspid valve atresia venous return LA - English M3 - Article N1 - L291073538 1970-12-01 PY - 1970 SP - 184-188 ST - The surgical treatment of congenital heart disease with cyanosis T2 - Radiol.Clin.Biol. TI - The surgical treatment of congenital heart disease with cyanosis UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L291073538 VL - 39 ID - 1404 ER - TY - JOUR AB - Cyanotic heart disease results from an avascular lung variously combined with atrial or ventricular septal defects. Exploration is urged in every case where there is any chance at all for benefit. Pre-operative preparation includes: premedication with atropine for infants or atropine and morphine for older children, intratracheal anaesthesia with ether-oxygen at a very light plane, and a continuous drip of saline or dextrose through a cannula in the saphenous vein at the ankle. The Brock type of pulmonary valvulotomy is indicated in pulmonic valvular stenosis with or without complicating septal defects. Shunt operations of the Blalock-Taussig or Potts-Smith type are done for pulmonic olus infundibular stenosis, small pulmonary artery, ventricular septal defect, and tricuspid atresia. Surgical approach has been through antero- or posterolateral incisions through the right or left fourth interspace. Exploration of the heart always includes adequate opening of the pericardium to visualize the base of the heart. Certain anomalies are marked by decreased pulmonary blood flow. Tight pulmonary stenosis occasionally favours a persistent foramen ovale, right-to-left shunting of blood and cyanosis. The Brock operation has been done in 17 cases with no deaths. Intracardiac pressure readings at time of surgery are helpful in confirming the diagnosis and establishing the immediate effects of' surgery. Some infundibular stenoses have also been incised and dilated successfully. Shunt operations are done in the tetralogy of Fallet, the Potts-Smith in infants and the Blalock-Taussig in older children. Valvulotomy has been done in 14 patients aged 3 months to 25 years with two deaths and the results have not been as satisfactory as with the shunt operations. In tricuspid atresia symptoms are more severe and tend to occur earlier in infancy; cyanosis becomes worse as the ductus arteriosus closes. The Potts-Smith operation was done in 12 patients aged 8 days to 3 yr. with 3 deaths. Shunt operations have also been used in pseudotruncus, single ventricle plus pulmonary stenosis, and combined interatrial and interventricular septal defects. Anomalies with increased pulmonary blood flow, such as the Eisenmenger complex, Taussig-Bing heart (transposition of aorta and left-sided pulmonary artery), and single ventricle without pulmonary stenosis are not amenable to surgery at present. Complete transposition of the great vessels baffles surgical repair because the coronary arteries almost always arise from the trunk leaving the right ventricle. The patients who have survived longest with this defect have had high ventricular septal defects or an aortic window which made oxygenated blood available to the coronary system. BIalock and Hanlon have attempted to produce a similar situation by creating interatrial septal defects plus a right subclavian-pulmonary anastomosis. Creation of artificial pulmonary stenosis has been proposed to reduce pulmonary blood flow in the Eisenmenger complex and in the single ventricle without pulmonary stenosis, but this increases the work of the right ventricle. AD - E.H. Fell, 122 Sth Michigan Ave., Chicago, IL, United States AU - Fell, E. H. AU - Fornay, R. A. DB - Embase Classic KW - atropine BCG vaccine ether glucose morphine oxygen sodium chloride aged anesthesia ankle aorta aortopulmonary septal defect Blalock Taussig shunt blood cannula cardiovascular malformation cerebrospinal fluid child coronary artery cyanosis cyanotic heart disease death diagnosis ductus arteriosus Eisenmenger complex great blood vessel heart heart atrium septum defect heart right ventricle heart single ventricle heart valve stenosis heart ventricle septum defect incision infancy infant intracardiac drug administration lung lung blood flow patient pericardium premedication pulmonary artery pulmonary valve stenosis reading saphenous vein shunting stenosis surgery surgical approach tricuspid valve atresia valvuloplasty LA - English M3 - Article N1 - L280760733 1954-12-01 PY - 1954 SN - 0031-3955 SP - 197-213 ST - Surgical treatment of congenital, cardiovascular anomalies accompanied by cyanosis T2 - Pediatric Clinics of North America TI - Surgical treatment of congenital, cardiovascular anomalies accompanied by cyanosis UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L280760733 VL - FEBRUARY ID - 1425 ER - TY - JOUR AB - The surgical treatment of congenital heart disease with cyanosis is briefly discussed. Among these anomalies the authors include: atrial septal defect associated with valvular pulmonary stenosis; tetralogy of Fallot; complete transposition of the great vessels; total pulmonary vein transposition (total anomalous venous 'return); tricuspid valve atresia or stenosis; Ebstein's anomaly, and truncus arteriosus peristens. The surgical management of each of these anomalies is discussed* from total correction to temporary or permanent palliation. In this discussion, special emphasis is placed on the common fields which radiologist and surgeon must have in the treatment of these patients. Good teamwork between the cardiologist, the radiologist and the surgeon is an essential condition for the success of these surgical procedures. This holds even more in cases involving emergency operations, such as the making of an atrial septal defect in patients with transpositions of the great vessels or the blind splitting of the pulmonary artery valve in severely cyanotic infants. The emergency operation performed by the surgeon in such cases could not save lives if he were not supported by a cardiologist and radiologist prepared to perform emergency diagnostic procedures at any hour of the day or night. AD - J.N. Van Homan Der Heide, Dept Thor. Surg, Univ. Hosp., Gronîngen AU - Van Homan Der Heide, J. N. DB - Embase Classic IS - 2 KW - acid tritium arterial trunk cardiologist chromosome congenital heart disease cyanosis diagnosis diagnostic procedure Ebstein anomaly emergency emergency surgery Fallot tetralogy great blood vessel heart atrium septum defect heart disease infant night palliative therapy patient pulmonary artery pulmonary valve stenosis pulmonary vein radiologist stenosis surgeon surgery surgical technique teamwork tricuspid valve atresia venous return LA - English M3 - Article N1 - L291090799 1970-12-01 PY - 1970 SP - 140-167 ST - The surgical treatment of congential heart disease with cyanesis T2 - Radiol.Clin. TI - The surgical treatment of congential heart disease with cyanesis UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L291090799 VL - 39 ID - 1405 ER - TY - JOUR AB - Introduction: Surveillance and management guidelines for Fontan patients are lacking due to the paucity of evidence in the literature of screening efficacy on outcome measures. Methods: The Fontan Working Group within the New England Congenital Cardiology Association designed an electronic survey to assess surveillance practices for patients with Fontan procedures among New England congenital cardiologists and to explore variability in screening low-risk vs high-risk Fontan patients across regional programs. Results: Fifty-six cardiologists representing 12 regional programs responded to the survey, comprising ~40% of the total New England congenital cardiac physicians. The majority of desired testing and consultation was available within 50 miles of the patient's home institution with some limitations of cardiac catheterization and cardiac magnetic resonance imaging availability. Surveillance and screening were less frequent in low-risk Fontan patients compared to high-risk Fontan patients. Counseling practices were similar for both low-risk and high-risk Fontan patients. Aspirin monotherapy was recommended by 82% of providers for low-risk Fontan patients, while anticoagulation regimens were more varied for the high-risk population. Practitioners with ≤15 years of experience were more likely to provide quality of life testing in both low-risk and high-risk Fontan patients. There were no other major differences in testing frequencies by years of practice, quaternary vs nonquaternary care facility, or the number of Fontan patients in a practice. Conclusion: This survey provides insight into regional practices of screening and surveillance of Fontan patients. These data may be used to design future research studies and evidence-based guidelines to streamline the approach to manage these complex patients. AD - B.T. Davey, Connecticut Children’s, Hartford, CT, United States AU - Davey, B. T. AU - Toro-Salazar, O. H. AU - Gauthier, N. AU - Valente, A. M. AU - Elder, R. W. AU - Wu, F. M. AU - Berman, N. AU - Pollack, P. AU - Lee, J. H. AU - Rathod, R. H. DB - Embase Medline DO - 10.1111/chd.12854 IS - 6 KW - acetylsalicylic acid anticoagulant agent beta adrenergic receptor blocking agent warfarin adolescent adult article cardiologist cardiovascular magnetic resonance child clinical practice congenital heart disease consultation disease surveillance evidence based medicine female Fontan procedure health care availability health care facility health care survey health program heart catheterization heart ventricle failure high risk patient Holter monitoring human low risk patient major clinical study male patient counseling practice guideline priority journal quality of life screening test United States work experience LA - English M3 - Article N1 - L2003525800 2019-11-12 PY - 2019 SN - 1747-0803 1747-079X SP - 1013-1023 ST - Surveillance and screening practices of New England congenital cardiologists for patients after the Fontan operation T2 - Congenital Heart Disease TI - Surveillance and screening practices of New England congenital cardiologists for patients after the Fontan operation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2003525800 http://dx.doi.org/10.1111/chd.12854 VL - 14 ID - 589 ER - TY - JOUR AB - Termination of pregnancy for fetal anomaly is legal in the UK with no upper limit, if two doctors, in good faith, agree “there is a substantial risk that if the child were born it would suffer from such physical or mental abnormalities as to be seriously handicapped”. This is Clause E of the Human Fertlisation and Embryology Act. The most commonly sighted Clause is C, which states “the pregnancy has not exceeded its twenty-fourth week and that the continuance of the pregnancy would involve risk, greater than if the pregnancy were terminated, of injury to the physical or mental health of the pregnant woman”. This study aimed to investigate health professionals’ views on gestational age and acceptable termination of pregnancy for fetal anomaly (TOPFA). We undertook a questionnaire survey of UK health professionals working in fetal medicine, obstetrics and gynaecology and neonatology. A study pack consisting of a self-completion questionnaire, an invitation letter, participant information sheet, and a stamped addressed return envelope, were sent to health professionals. We used four fetal anomalies as case study examples in the questionnaire: isolated cleft lip, hypoplastic left heart, spina bifida and trisomy 21. These anomalies were chosen as they differed in terms of the type of anomaly, the type of impairment, and the perceived severity. Forty-one study packs were returned. For anomalies deemed less serious, later gestational ages were an important consideration when deciding acceptable TOPFA. The prognosis of an anomaly was considered an important factor in deciding whether a TOPFA was acceptable alongside gestational age. Clause C of the current UK legislation, which allows a legal termination prior to 24 weeks gestational age if continuing with the pregnancy would impact the mental health of the mother, was deemed a reasonable option for termination when parents are requesting a TOPFA. For each case study example, health professionals responded that TOPFA at ‘25 weeks and over’ was acceptable (cleft lip n = 1; hypoplastic left heart n = 19; spina bifida n = 13 and Trisomy 21 n = 10). Professionals also distinguished between their personal and professional views. These findings offer new insight into how gestational age considerations influence professionals’ conceptualisation of acceptable TOPFA. AD - L. Crowe, Institute of Health & Society, Baddiley-Clark Building, Newcastle University, United Kingdom AU - Crowe, L. AU - Graham, R. H. AU - Robson, S. C. AU - Rankin, J. DB - Embase Medline DO - 10.1016/j.ejmg.2018.05.011 IS - 9 KW - article cleft lip disease severity female fetus malformation gestational age gynecology health personnel attitude health practitioner health survey human hypoplastic left heart syndrome law male medical information mental health neonatology pregnancy termination prognosis questionnaire spinal dysraphism trisomy 21 United Kingdom LA - English M3 - Article N1 - L2000820152 2018-06-07 2018-12-27 PY - 2018 SN - 1878-0849 1769-7212 SP - 493-498 ST - A survey of health professionals’ views on acceptable gestational age and termination of pregnancy for fetal anomaly T2 - European Journal of Medical Genetics TI - A survey of health professionals’ views on acceptable gestational age and termination of pregnancy for fetal anomaly UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2000820152 http://dx.doi.org/10.1016/j.ejmg.2018.05.011 VL - 61 ID - 666 ER - TY - JOUR AB - Despite improvement in surgical and medical management of children with congenital and acquired heart disease, cardiac transplantation remains an important therapeutic option for infants and children with end-stage heart disease. Ultimate survival in patients who are listed for transplantation is a function of both mortality while awaiting transplantation and survival after transplantation. Survival of heart transplantation is affected by the severity of illness before transplantation, the unique pathophysiology of certain defects, and the availability of donor hearts. Outcome following listing for transplantation is best studied with the use of recent modifications in statistical methods of competing outcomes analysis. By this analysis a predicted mortality while waiting among all pediatric patients is 20% at 1 year, with 67% undergoing transplantation, 10% still on the list awaiting transplant, and 3% removed from the list. Among infants, most of them with hypoplastic left heart syndrome, 60% will have transplantation by 6 months after listing, with 27% of patients dying while waiting. In infants the major risk factors for death while waiting are the need for inotropic support at listing, smaller size, and recipient blood type. In older children risk factors for death while waiting are Status 1 at listing and a need for mechanical ventilation. Intermediate-term survival after transplant is excellent in all age groups with 86% alive at 6 months, 84% at 1 year, and 73% at 5 years. Survival after transplant in infants is comparable to survival in older children, although the early mortality after transplantation is greater. Infants who have recently undergone sternotomy or received organs from donors who did not die of closed head trauma are more likely to die early after transplant. Among older children risk factors for death after transplantation include the need for a mechanical support device or a younger age in patients greater than 1 year of age. Death following transplantation is primarily related to early graft failure in infants, whereas rejection, infection, and sudden death account for the majority of deaths in older children. Although improved immunosuppressive agents promise to lead to even better survival rates after transplantation, greater access to donors is essential if overall survival is to be improved. AD - Division of Pediatric Cardiology, Department of Pediatrics, University of Arkansas for Medical Sciences and Arkansas Children's Hospital, Little Rock, AR, USA AN - 10856691 AU - Morrow, W. R. AU - Frazier, E. AU - Naftel, D. C. DA - Jun 1 DB - PubMed DO - 10.1016/s1058-9813(00)00041-2 DP - NLM ET - 2000/06/17 IS - 2 LA - eng N1 - Morrow, WR Frazier, E Naftel, DC Journal Article Netherlands Prog Pediatr Cardiol. 2000 Jun 1;11(2):99-105. doi: 10.1016/s1058-9813(00)00041-2. PY - 2000 SN - 1058-9813 (Print) 1058-9813 SP - 99-105 ST - Survival after listing for cardiac transplantation in children T2 - Prog Pediatr Cardiol TI - Survival after listing for cardiac transplantation in children VL - 11 ID - 492 ER - TY - JOUR AB - BACKGROUND: Some pediatric patients referred for heart transplant (HTx) are sub-optimal candidates. Their outcomes without HTx are presumed to be dismal, but have not been well described. Knowledge about their outcomes is critical when weighing the risks between a high-risk transplant and "terminal" palliation. METHODS: We retrospectively reviewed all HTx referrals from January 2005 to July 2013. We excluded those who were listed for HTx, or who were denied HTx due to being "too well," seeking only those who were in need of but not suitable for HTx. End-points included mortality and length of survival. RESULTS: Of 212 referrals, 39 (19%) (age 0 to 19 years, median 3.5 years) were denied HTx for reasons other than being too well. Twenty-eight (72%) had palliated congenital heart disease. Overall mortality during the follow-up period was 38% (n = 15) with a median follow-up time of 195 days (8 to 2,832 days). Ten patients received subsequent cardiac surgery with 1 death (10%) and median follow-up of 2.6 years. Mortality risk was not influenced by age, weight, growth failure, congenital heart disease or single-ventricle physiology. Mechanical ventilation (hazard ratio 6.31, p = 0.001) and inotrope dependence (hazard ratio 4.79, p = 0.006) were associated with the highest risk of mortality. Quality of life was measured with the PedsQL cardiac module and completed by 11 of 16 eligible patients with an overall average score of 70.2 ± 23.9. CONCLUSIONS: An advanced heart failure program can achieve satisfactory results for pediatric patients who are not suitable candidates for HTx. For some children, high-risk palliative surgery can result in better outcome than high-risk HTx. Mortality was related to the degree of heart failure at presentation rather than underlying heart disease. AD - Division of Cardiology. Electronic address: asabati@chla.usc.edu. Division of Cardiology. Department of Cardiothoracic Surgery. Clinical Investigation Center at the Saban Research Institute; Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, California. AN - 25765370 AU - Sabati, A. A. AU - Szmuszkovicz, J. R. AU - Herrington, C. AU - Hermes, M. AU - Mahmoud, H. A. AU - Phei Wee, C. AU - Starnes, V. A. AU - Menteer, J. DA - Jul DB - PubMed DO - 10.1016/j.healun.2015.01.006 DP - NLM ET - 2015/03/15 IS - 7 KW - Adolescent California/epidemiology Child Child, Preschool Female Follow-Up Studies Heart Failure/*mortality/psychology/therapy Heart Transplantation Heart-Assist Devices Humans Infant Infant, Newborn Male Prognosis *Quality of Life Retrospective Studies Survival Rate/trends Time Factors Treatment Outcome Young Adult Pediatric heart failure alloimmunization congenital heart disease congenital heart surgery panel reactive antibodies pediatric heart transplant LA - eng N1 - 1557-3117 Sabati, Arash Alen Szmuszkovicz, Jacqueline R Herrington, Cynthia Hermes, Mark Mahmoud, Hesham A Phei Wee, Choo Starnes, Vaughn A Menteer, JonDavid Journal Article Research Support, Non-U.S. Gov't United States J Heart Lung Transplant. 2015 Jul;34(7):906-11. doi: 10.1016/j.healun.2015.01.006. Epub 2015 Jan 16. PY - 2015 SN - 1053-2498 SP - 906-11 ST - Survival and quality of life for children with end-stage heart failure who are not candidates for cardiac transplant T2 - J Heart Lung Transplant TI - Survival and quality of life for children with end-stage heart failure who are not candidates for cardiac transplant VL - 34 ID - 124 ER - TY - JOUR AB - BACKGROUND: Despite the increasing application of cardiac transplantation in infants, reported survival rates vary, and risk factors for death are poorly understood. METHODS AND RESULTS: To examine early survival and risk factors for death in infants (< 1 year of age) undergoing cardiac transplantation, 141 infants (36 < 1 months of age) underwent primary cardiac transplantation between January 1, 1993, and January 1, 1995, at 23 centers in the Pediatric Heart Transplant Study (PHTS). Diagnoses were hypoplastic left heart syndrome (66%), other congenital heart disease (17%), cardiomyopathy (14%), and other (3%). Actuarial survival after cardiac transplantation was 84% at 1 month, 70% at 1 year, and 69% at 2 years, with the greatest hazard for death within the first 3 months. The principal cause of death was early graft failure in 20 patients (52% of deaths), infection in 10 (26% of deaths), and rejection in 4 (10%). On the basis of multivariate analysis, risk factors for early mortality were history of previous sternotomy (P = .0003), nonidentical blood type donor (P = .01), recipient non-blood group A (P = .02), and donor cause of death other than closed head trauma (P = .04). Diagnosis at listing, waiting time (mean, 1.3 months), graft ischemic time (mean, 228 minutes; range, 68 to 479 minutes), and recipient ventilatory or inotropic support at listing were not predictive for mortality after transplant. CONCLUSIONS: The higher mortality rate observed with infant heart transplantation is due to a higher mortality within the first month after transplantation as a result of early graft failure. Strategies to improve donor heart function at implantation would have the greatest impact on survival after infant cardiac transplantation. AD - Washington University, St Louis, Mo, USA. canter@al@kids.wustl.edu AN - 9236438 AU - Canter, C. AU - Naftel, D. AU - Caldwell, R. AU - Chinnock, R. AU - Pahl, E. AU - Frazier, E. AU - Kirklin, J. AU - Boucek, M. AU - Morrow, R. DA - Jul 1 DB - PubMed DO - 10.1161/01.cir.96.1.227 DP - NLM ET - 1997/07/01 IS - 1 KW - Actuarial Analysis Cause of Death Graft Rejection Heart Transplantation/*mortality Humans Infant Multivariate Analysis Risk Factors Survival Rate LA - eng N1 - Canter, C Naftel, D Caldwell, R Chinnock, R Pahl, E Frazier, E Kirklin, J Boucek, M Morrow, R Journal Article United States Circulation. 1997 Jul 1;96(1):227-31. doi: 10.1161/01.cir.96.1.227. PY - 1997 SN - 0009-7322 (Print) 0009-7322 SP - 227-31 ST - Survival and risk factors for death after cardiac transplantation in infants. A multi-institutional study. The Pediatric Heart Transplant Study T2 - Circulation TI - Survival and risk factors for death after cardiac transplantation in infants. A multi-institutional study. The Pediatric Heart Transplant Study VL - 96 ID - 521 ER - TY - JOUR AB - OBJECTIVE: Multicenter longitudinal outcome data for Fontan patients surviving into adulthood are lacking. The aim of this study was to better understand contemporary outcomes in Fontan survivors by collecting follow-up data in a previously well-characterized cohort. DESIGN: Baseline data from the Fontan Cross-Sectional Study (Fontan 1) were previously obtained in 546 Fontan survivors aged 11.9 ± 3.4 years. We assessed current transplant-free survival status in all subjects 6.8 ± 0.4 years after the Fontan 1 study. Anatomic, clinical, and surgical data were collected along with socioeconomic status and access to health care. RESULTS: Thirty subjects (5%) died or underwent transplantation since Fontan 1. Subjects with both an elevated (>21 pg/mL) brain natriuretic peptide and a low Child Health Questionnaire physical summary score (<44) measured at Fontan 1 were significantly more likely to die or undergo transplant than the remainder, with a hazard ratio of 6.2 (2.9-13.5). Among 516 Fontan survivors, 427 (83%) enrolled in this follow-up study (Fontan 2) at 18.4 ± 3.4 years of age. Although mean scores on functional health status questionnaires were lower than the general population, individual scores were within the normal range in 78% and 88% of subjects for the Child Health Questionnaire physical and psychosocial summary score, and 97% and 91% for the SF-36 physical and mental aggregate score, respectively. Since Fontan surgery, 119 (28%) had additional cardiac surgery; 55% of these (n = 66) in the interim between Fontan 1 and Fontan 2. A catheter intervention occurred in 242 (57%); 32% of these (n = 78) after Fontan 1. Arrhythmia requiring treatment developed in 118 (28%) after Fontan surgery; 58% of these (n = 68) since Fontan 1. CONCLUSIONS: We found 95% interim transplant-free survival for Fontan survivors over an average of 7 years of follow-up. Continued longitudinal investigation into adulthood is necessary to better understand the determinants of long-term outcomes and to improve functional health status. AD - Department of Pediatrics, Medical University of South Carolina, Charleston, SC, USA. AN - 24934522 AU - Atz, A. M. AU - Zak, V. AU - Mahony, L. AU - Uzark, K. AU - Shrader, P. AU - Gallagher, D. AU - Paridon, S. M. AU - Williams, R. V. AU - Breitbart, R. E. AU - Colan, S. D. AU - Kaltman, J. R. AU - Margossian, R. AU - Pasquali, S. K. AU - Allen, K. AU - Lai, W. W. AU - Korsin, R. AU - Marino, B. S. AU - Mirarchi, N. AU - McCrindle, B. W. C2 - PMC4414014 C6 - NIHMS649071 DA - Jan-Feb DB - PubMed DO - 10.1111/chd.12193 DP - NLM ET - 2014/06/18 IS - 1 KW - Adolescent Biomarkers/blood Canada Disease-Free Survival Female *Fontan Procedure/adverse effects/mortality Health Services Accessibility Health Status Heart Defects, Congenital/diagnosis/mortality/physiopathology/*surgery Heart Transplantation Humans Kaplan-Meier Estimate Longitudinal Studies Male Natriuretic Peptide, Brain/blood Proportional Hazards Models Prospective Studies Risk Assessment Risk Factors Socioeconomic Factors Surveys and Questionnaires Time Factors Treatment Outcome United States Young Adult Congenital Fontan Procedure Functional Health Status Heart Defects Pediatrics LA - eng N1 - 1747-0803 Atz, Andrew M Zak, Victor Mahony, Lynn Uzark, Karen Shrader, Peter Gallagher, Dianne Paridon, Stephen M Williams, Richard V Breitbart, Roger E Colan, Steven D Kaltman, Jonathan R Margossian, Renee Pasquali, Sara K Allen, Kerstin Lai, Wyman W Korsin, Rosalind Marino, Bradley S Mirarchi, Nicole McCrindle, Brian W Pediatric Heart Network Investigators K08 HL103631/HL/NHLBI NIH HHS/United States HL068288/HL/NHLBI NIH HHS/United States HL068285/HL/NHLBI NIH HHS/United States U01 HL068269/HL/NHLBI NIH HHS/United States U01 HL068279/HL/NHLBI NIH HHS/United States U01 HL068290/HL/NHLBI NIH HHS/United States HL068279/HL/NHLBI NIH HHS/United States U01 HL068288/HL/NHLBI NIH HHS/United States U10 HL068270/HL/NHLBI NIH HHS/United States U01 HL068281/HL/NHLBI NIH HHS/United States U01 HL068270/HL/NHLBI NIH HHS/United States HL068281/HL/NHLBI NIH HHS/United States U01 HL068292/HL/NHLBI NIH HHS/United States HL068269/HL/NHLBI NIH HHS/United States HL068270/HL/NHLBI NIH HHS/United States U10 HL109778/HL/NHLBI NIH HHS/United States HL068290/HL/NHLBI NIH HHS/United States U01 HL068285/HL/NHLBI NIH HHS/United States HL068292/HL/NHLBI NIH HHS/United States Journal Article Multicenter Study Research Support, N.I.H., Extramural Congenit Heart Dis. 2015 Jan-Feb;10(1):E30-42. doi: 10.1111/chd.12193. Epub 2014 Jun 17. PY - 2015 SN - 1747-079X (Print) 1747-079x SP - E30-42 ST - Survival data and predictors of functional outcome an average of 15 years after the Fontan procedure: the pediatric heart network Fontan cohort T2 - Congenit Heart Dis TI - Survival data and predictors of functional outcome an average of 15 years after the Fontan procedure: the pediatric heart network Fontan cohort VL - 10 ID - 156 ER - TY - JOUR AB - Objective: Multicenter longitudinal outcome data for Fontan patients surviving into adulthood are lacking. The aim of this study was to better understand contemporary outcomes in Fontan survivors by collecting follow-up data in a previously well-characterized cohort. Design: Baseline data from the Fontan Cross-Sectional Study (Fontan 1) were previously obtained in 546 Fontan survivors aged 11.9 ± 3.4years. We assessed current transplant-free survival status in all subjects 6.8 ± 0.4years after the Fontan 1 study. Anatomic, clinical, and surgical data were collected along with socioeconomic status and access to health care. Results: Thirty subjects (5%) died or underwent transplantation since Fontan 1. Subjects with both an elevated (>21pg/mL) brain natriuretic peptide and a low Child Health Questionnaire physical summary score (<44) measured at Fontan 1 were significantly more likely to die or undergo transplant than the remainder, with a hazard ratio of 6.2 (2.9-13.5). Among 516 Fontan survivors, 427 (83%) enrolled in this follow-up study (Fontan 2) at 18.4 ± 3.4years of age. Although mean scores on functional health status questionnaires were lower than the general population, individual scores were within the normal range in 78% and 88% of subjects for the Child Health Questionnaire physical and psychosocial summary score, and 97% and 91% for the SF-36 physical and mental aggregate score, respectively. Since Fontan surgery, 119 (28%) had additional cardiac surgery; 55% of these (n = 66) in the interim between Fontan 1 and Fontan 2. A catheter intervention occurred in 242 (57%); 32% of these (n = 78) after Fontan 1. Arrhythmia requiring treatment developed in 118 (28%) after Fontan surgery; 58% of these (n = 68) since Fontan 1. Conclusions: We found 95% interim transplant-free survival for Fontan survivors over an average of 7years of follow-up. Continued longitudinal investigation into adulthood is necessary to better understand the determinants of long-term outcomes and to improve functional health status. AD - A.M. Atz, Department of Pediatrics, Division of Cardiology, Medical University of South Carolina, MSC 915, Children's Hospital, Charleston, SC, United States AU - Atz, A. M. AU - Zak, V. AU - Mahony, L. AU - Uzark, K. AU - Shrader, P. AU - Gallagher, D. AU - Paridon, S. M. AU - Williams, R. V. AU - Breitbart, R. E. AU - Colan, S. D. AU - Kaltman, J. R. AU - Margossian, R. AU - Pasquali, S. K. AU - Allen, K. AU - Lai, W. W. AU - Korsin, R. AU - Marino, B. S. AU - Mirarchi, N. AU - McCrindle, B. W. DB - Embase Medline DO - 10.1111/chd.12193 IS - 1 KW - brain natriuretic peptide dipeptidyl carboxypeptidase inhibitor adult article catheter cross-sectional study follow up Fontan procedure health care access health status heart arrhythmia heart surgery heart transplantation human major clinical study medical record review priority journal protein losing gastroenteropathy questionnaire Short Form 36 social status survival treatment outcome LA - English M3 - Article N1 - L53199345 2014-06-26 2019-12-19 PY - 2015 SN - 1747-0803 1747-079X SP - E30-E42 ST - Survival data and predictors of functional outcome an average of 15years after the fontan procedure: The pediatric heart network fontan cohort T2 - Congenital Heart Disease TI - Survival data and predictors of functional outcome an average of 15years after the fontan procedure: The pediatric heart network fontan cohort UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L53199345 http://dx.doi.org/10.1111/chd.12193 VL - 10 ID - 878 ER - TY - JOUR AB - BACKGROUND: Children who require prolonged intensive care after cardiac surgery are at risk of high mortality. The long-term survival and functional outcome of these children have not been studied in detail. METHODS: Children who stayed in intensive care for >28 days after cardiac surgery from 1997 to 2012 were studied in a single institution. A total of 116 patients were identified; 107 (92%) were <1 year of age and 63 (54%) had univentricular physiology. RESULTS: The incidence of children requiring prolonged intensive care increased from 1.01/100 undergoing cardiac surgery in 1997 to 2000 to 2.66/100 in 2009 to 2012 (P trend = .002). This increase coincided with an increase in the number of children with hypoplastic left heart syndrome having prolonged intensive care during the same period (0.13/100 in 1997-2000 to 1.0/100 in 2009-2012; P trend = .001). Survival to pediatric intensive care unit (PICU) discharge was 74% (95% confidence interval [CI], 65-82) and 51% (95% CI, 41-59) at 3 years. Factors associated with mortality were univentricular repair (hazard ratio [HR], 2.12; 95% CI, 1.21-3.70; P = .008) and acute renal failure (HR, 3.01; 95% CI, 1.77-5.12; P < .001), but era did not influence mortality (1997-2005 vs 2006-2012; log-rank P = .66). Among PICU survivors, 3-year survival in those who did not need early reoperation was 81% (95% CI, 66-90), compared with 58% (95% CI, 42-71) in those who required early reoperation (log-rank P = .01). Among survivors, 36% had either moderate or severe disability and 13% had poor quality of life. CONCLUSIONS: The incidence of children requiring prolonged intensive care after cardiac surgery has increased in our institution. Our data suggest that the long-term outcome for most of these children is poor, especially after univentricular repair. AD - Intensive Care Unit, Royal Children's Hospital, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia. Electronic address: siva.namachivayam@rch.org.au. Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia. Intensive Care Unit, Royal Children's Hospital, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia. Department of Cardiology, Royal Children's Hospital, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia. Intensive Care Unit, Royal Children's Hospital, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia. AN - 27262362 AU - Namachivayam, S. P. AU - d'Udekem, Y. AU - Millar, J. AU - Cheung, M. M. AU - Butt, W. DA - Oct DB - PubMed DO - 10.1016/j.jtcvs.2016.05.006 DP - NLM ET - 2016/06/06 IS - 4 KW - *Cardiac Surgical Procedures Female Heart Defects, Congenital/mortality/*surgery Hospital Mortality Humans Infant Infant, Newborn Intensive Care Units, Pediatric/*statistics & numerical data Length of Stay/statistics & numerical data Male Quality of Life Survival Rate Treatment Outcome *functional outcome *pediatric cardiac surgery *prolonged intensive care *survival status LA - eng N1 - 1097-685x Namachivayam, Siva P d'Udekem, Yves Millar, Johnny Cheung, Michael M Butt, Warwick Journal Article United States J Thorac Cardiovasc Surg. 2016 Oct;152(4):1104-1112.e3. doi: 10.1016/j.jtcvs.2016.05.006. Epub 2016 May 10. PY - 2016 SN - 0022-5223 SP - 1104-1112.e3 ST - Survival status and functional outcome of children who required prolonged intensive care after cardiac surgery T2 - J Thorac Cardiovasc Surg TI - Survival status and functional outcome of children who required prolonged intensive care after cardiac surgery VL - 152 ID - 183 ER - TY - JOUR AB - Background: Management of patients with hypoplastic left heart syndrome has benefited from advancements in medical and surgical care. Outcomes have improved, although survival and long-term functional and cognitive deficits remain a concern. Methods and Results: This is a cohort study of all consecutive patients with hypoplastic left heart syndrome undergoing surgical palliation at a single center. We aimed to examine demographic and perioperative factors from each surgical stage for their association with survival and neurocognitive outcomes. A total of 117 consecutive patients from 1996 to 2010 underwent surgical palliation. Seventy patients (60%) survived to the Fontan stage and 68 patients (58%) survived to undergo neurocognitive assessment at a mean (SD) age of 56.6 months (6.4 months). Full-scale, performance, and verbal intelligence quotient, as well as visual-motor integration mean (SD) scores were 86.7 (16.1), 86.3 (15.8), 88.8 (17.2), and 83.2 (14.8), respectively. On multivariable analysis, older age at Fontan, sepsis peri-Norwood, lowest arterial partial pressure of oxygen postbidirectional cavopulmonary anastomosis, and presence of neuromotor disability pre-Fontan were strongly associated with lower scores for all intelligence quotient domains. Older age at Fontan and sepsis peri-Norwood remained associated with lower scores for all intelligence quotient domains in a subgroup analysis excluding patients with disability pre-Fontan or with chromosomal abnormalities. Conclusions: Older age at Fontan and sepsis are among independent predictors of poor neurocognitive outcomes for patients with hypoplastic left heart syndrome. Further studies are required to identify the appropriate age range for Fontan completion, balancing a lower risk of acute and long-term hemodynamic complications while optimizing long-term neurocognitive outcomes. AD - J. Atallah, Department of Pediatrics, University of Alberta, Edmonton, AB, Canada AU - Atallah, J. AU - Garcia Guerra, G. AU - Joffe, A. R. AU - Bond, G. Y. AU - Islam, S. AU - Ricci, M. F. AU - AlAklabi, M. AU - Rebeyka, I. M. AU - Robertson, C. M. T. DB - Embase Medline DO - 10.1161/JAHA.119.013632 IS - 4 KW - age arterial pressure article Blalock Taussig shunt cavopulmonary connection child clinical outcome cognition cognition assessment cohort analysis epilepsy extracorporeal oxygenation female follow up Fontan procedure full scale intelligence quotient score functional behavior human hypoplastic left heart syndrome intelligence quotient long term survival major clinical study male mental performance mortality rate motor dysfunction neurologic examination Norwood procedure outcome assessment overall survival palliative therapy perception deafness performance intelligence quotient score perioperative period predictive value preschool child priority journal risk factor sepsis survival rate treatment outcome verbal intelligence quotient score visual impairment visuomotor coordination LA - English M3 - Article N1 - L2004375535 2020-03-16 2020-03-25 PY - 2020 SN - 2047-9980 ST - Survival, Neurocognitive, and Functional Outcomes After Completion of Staged Surgical Palliation in a Cohort of Patients With Hypoplastic Left Heart Syndrome T2 - Journal of the American Heart Association TI - Survival, Neurocognitive, and Functional Outcomes After Completion of Staged Surgical Palliation in a Cohort of Patients With Hypoplastic Left Heart Syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2004375535 http://dx.doi.org/10.1161/JAHA.119.013632 VL - 9 ID - 565 ER - TY - JOUR AB - It is proposed that the term 'hypoplastic left heart syndrome' be used for all cases of malformations of the left heart in children in which there are hypoplasia of the left ventricle and hypertrophy of the right ventricle. In the course of 9 yr., from 1949 to 1957, 101 such cases have been observed. There were 15 cases of atresia of the aortic valves (2 cases with mitral atresia and 13 with mitral hypoplasia), 5 cases of mitral atresia, 4 cases of mitral stenosis, and 6 of atresia and 71 of hypoplasia of the aortic arch. Most of the patients also showed defects of the atrial or ventricular septa, patent ductus arteriosus or such malformations. In 95 cases the duration of survival was between 72 hr. and 12 months, in 3 cases up to 6 yr. and in 3 other cases up to 13 yr. In 37 children the malformations of the heart were associated with malformations in other parts of the body (Meckel's diverticula, multiple spleens, skeletal anomalies, mongolism etc.). The pathophysiology, the clinical picture, the diagnosis and the differential diagnosis, the treatment, the prognosis and the post-mortem findings are discussed. The clinical diagnosis of the 'syndrome' is not too difficult as a rule, but an accurate determination of the existing anatomical defects is usually impossible. Conservative treatment gives unsatisfactory results, the surgical possibilities are limited. In these cases it is always to be recommended to carry out cardiac catheterization and angiocardiography, so that the abnormal circulatory conditions will be known, and the possibility that the patient may benefit from the great advances made in the field of open-heart surgery can be considered. AD - J.A. Noonan, Dept. of Ped., Harvard Med. Sch., Boston, MA, United States AU - Noonan, J. A. AU - Nadas, A. S. DB - Embase Classic KW - angiocardiography aortic arch aortic valve atresia child congenital malformation conservative treatment diagnosis differential diagnosis Down syndrome heart heart catheterization heart left ventricle heart right ventricle interventricular septum hypertrophy hypoplasia hypoplastic left heart syndrome Meckel diverticulum mitral valve atresia mitral valve stenosis open heart surgery patent ductus arteriosus pathophysiology patient pediatric cardiology prognosis skeleton malformation spleen survival LA - English M3 - Article N1 - L281037293 1958-12-01 PY - 1958 SN - 0031-3955 SP - 1029-1056 ST - Symposium on pediatric cardiology. The hypoplastic left heart syndrome. An analysis of 101 cases T2 - Pediatric Clinics of North America TI - Symposium on pediatric cardiology. The hypoplastic left heart syndrome. An analysis of 101 cases UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L281037293 VL - NOV. NR. ID - 1423 ER - TY - JOUR AB - Systolic and diastolic function were assessed at preoperative cardiac catheterization in 6 patients with classic tricuspid atresia and were compared with those in control subjects. All patients had systemic-to-pulmonary shunts. Left ventricular end-systolic and end-diastolic volumes and masses were significantly larger in the patient group; stroke volume and the ratio of volume to mass were also higher, but not significantly. Mean end-diastolic, peak systolic and end-systolic pressures were similar. Mean stress at end-systole and peak systole were significantly higher in the patient group. Operant chamber stiffness tended to be lower than normal, but was not statistically significant, and myocardial stiffness was normal for the patient group also. Indexes of systolic function were compared. The rate-corrected mean velocity of fiber shortening in the patient group was lower and its relation with stress at end-systole was variable with 4 abnormal patients. The ratio of stress at end-systole/end-systolic volume was significantly lower in patients. The duration of hospital stay did not correlate with end-diastolic volume or any parameter. Thus, this study found abnormalities of load-dependent and independent indexes of systolic function with normal diastolic function in young preoperative patients with tricuspid atresia. AD - Department of Paediatrics and Cardiovascular Surgery, University of British Columbia, British Columbia's Children's Hospital, Vancouver, Canada. AN - 8296762 AU - Sandor, G. G. AU - Patterson, M. W. AU - LeBlanc, J. G. DA - Feb 1 DB - PubMed DO - 10.1016/0002-9149(94)90236-4 DP - NLM ET - 1994/02/01 IS - 4 KW - Cardiac Catheterization Cardiac Surgical Procedures/methods Child Child, Preschool Diastole/*physiology Humans Myocardial Contraction/physiology Systole/*physiology Treatment Outcome Tricuspid Valve/*abnormalities/*surgery LA - eng N1 - Sandor, G G Patterson, M W LeBlanc, J G Journal Article Research Support, Non-U.S. Gov't United States Am J Cardiol. 1994 Feb 1;73(4):292-7. doi: 10.1016/0002-9149(94)90236-4. PY - 1994 SN - 0002-9149 (Print) 0002-9149 SP - 292-7 ST - Systolic and diastolic function in tricuspid valve atresia before the Fontan operation T2 - Am J Cardiol TI - Systolic and diastolic function in tricuspid valve atresia before the Fontan operation VL - 73 ID - 428 ER - TY - JOUR AB - Systolic and diastolic function were assessed at preoperative cardiac catheterization in 6 patients with classic tricuspid atresia and were compared with those in control subjects. All patients had systemic-to- pulmonary shunts. Left ventricular end-systolic and end-diastolic volumes and masses were significantly larger in the patient group; stroke volume and the ratio of volume to mass were also higher, but not significantly. Mean end- diastolic, peak systolic and end-systolic pressures were similar. Mean stress at end-systole and peak systole were significantly higher in the patient group. Operant chamber stiffness tended to be lower than normal, but was not statistically significant, and myocardial stiffness was normal for the patient group also. Indexes of systolic function were compared. The rate- corrected mean velocity of fiber shortening in the patient group was lower and its relation with stress at end-systole was variable with 4 abnormal patients. The ratio of stress at end-systole/end-systolic volume was significantly lower in patients. The duration of hospital stay did not correlate with end-diastolic volume or any parameter. Thus, this study found abnormalities of load-dependent and independent indexes of systolic function with normal diastolic function in young preoperative patients with tricuspid atresia. AD - G.G.S. Sandor, Division of Cardiology, 4480 Oak Street, Vancouver, BC V6H 3V4, Canada AU - Sandor, G. G. S. AU - Patterson, M. W. H. AU - LeBlanc, J. G. DB - Embase Medline DO - 10.1016/0002-9149(94)90236-4 IS - 4 KW - article child clinical article controlled study Fontan procedure heart catheterization heart ejection fraction heart function heart left ventricle enddiastolic volume heart left ventricle volume heart stroke volume hospitalization human preschool child priority journal tricuspid valve atresia LA - English M3 - Article N1 - L24050085 1994-02-23 PY - 1994 SN - 0002-9149 SP - 292-297 ST - Systolic and diastolic function in tricuspid valve atresia before the Fontan operation T2 - American Journal of Cardiology TI - Systolic and diastolic function in tricuspid valve atresia before the Fontan operation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L24050085 http://dx.doi.org/10.1016/0002-9149(94)90236-4 VL - 73 ID - 1376 ER - TY - JOUR AB - We report a highly unusual case of a 22-year-old woman with single-ventricle anatomy and a history of Fontan palliation who developed takotsubo cardiomyopathy. To our knowledge, takotsubo cardiomyopathy has not previously been described in a patient with single-ventricle anatomy, or more generally in any patient with congenital heart disease. The patient presented at our clinic for urgent examination after a 2-day history of chest pain that had begun upon the death of her dog. Invasive evaluation was refused by the patient; instead, she underwent electrocardiogram-gated coronary computed tomographic angiography, to rule out obstructive coronary disease. A physician who suspects takotsubo cardiomyopathy in a patient should look for new-onset chest pain and dyspnea coupled with these features: immediate onset of segmental akinesia in the midventricular or apical aspects of the heart, elevation of brain natriuretic peptide level 12 to 24 hours after onset of akinesia, elevation of troponin level 24 to 48 hours after onset, and disappearance of the segmental akinesia a few days after onset. AN - 27047288 AU - Derk, G. R. AU - Aboulhosn, J. AU - Reardon, L. C. C2 - PMC4810588 DA - Feb DB - PubMed DO - 10.14503/thij-14-4151 DP - NLM ET - 2016/04/06 IS - 1 KW - Diagnosis, Differential Echocardiography Electrocardiography Female Heart Defects, Congenital/complications/*diagnosis/physiopathology Heart Ventricles/*abnormalities/diagnostic imaging/physiopathology Humans Takotsubo Cardiomyopathy/complications/*diagnosis/physiopathology Tomography, X-Ray Computed Ventricular Function/physiology Young Adult Apical-ballooning syndrome Fontan procedure cardiomyopathies/diagnosis coronary vasospasm/complications heart disease, congenital single ventricle stress, psychological/complications takotsubo cardiomyopathy/diagnosis/epidemiology/therapy treatment outcome ventricular dysfunction/etiology/radiography LA - eng N1 - 1526-6702 Derk, Gwendolyn Rose Aboulhosn, Jamil Reardon, Leigh C Case Reports Journal Article Research Support, Non-U.S. Gov't Tex Heart Inst J. 2016 Feb 1;43(1):61-4. doi: 10.14503/THIJ-14-4151. eCollection 2016 Feb. PY - 2016 SN - 0730-2347 (Print) 0730-2347 SP - 61-4 ST - Takotsubo Cardiomyopathy in a 22-Year-Old Single-Ventricle Patient T2 - Tex Heart Inst J TI - Takotsubo Cardiomyopathy in a 22-Year-Old Single-Ventricle Patient VL - 43 ID - 186 ER - TY - JOUR AB - Ongoing technological advances in pediatrics are improving the survival rates among babies born with life-threatening anomalies. For these neonates, surgeries like brain shunts, trachea, gut and heart reconstruction, and organ transplants are replacing palliative care. Although parents and health care professionals alike are celebrating the successes, advancing technology also raises issues for everyone involved. This paper incorporates Dunst and Trivette's Effective Help-giving framework and the Calgary Family Intervention Model to recommend nursing care that moves beyond life-saving highly technical surgical procedures and responds to the challenges parents face with their children with complex congenital heart disease, for example, who have 'beat the odds.' Copyright © 2004 by Elsevier Science (USA). AD - Faculty of Nursing, University of Alberta, Third Floor Clinical Sciences Building, Edmonton AB, Canada, T6G 2G3; grrempel@ualberta.ca AN - 106565848. Language: English. Entry Date: 20050121. Revision Date: 20150819. Publication Type: Journal Article AU - Rempel, G. R. DB - ccm DP - EBSCOhost IS - 1 KW - Hypoplastic Left Heart Syndrome Parents Pediatric Nursing Professional-Family Relations Technology, Medical Caregiver Burden Collaboration Coping Decision Making, Family Grief Heart Transplantation -- In Infancy and Childhood Hypoplastic Left Heart Syndrome -- Mortality Hypoplastic Left Heart Syndrome -- Nursing Hypoplastic Left Heart Syndrome -- Psychosocial Factors Hypoplastic Left Heart Syndrome -- Surgery Infant Infant, Newborn Information Needs Models, Theoretical Nursing Interventions Parents -- Psychosocial Factors Survival -- In Infancy and Childhood N1 - tables/charts. Journal Subset: Core Nursing; Double Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Nursing; Peer Reviewed; USA. NLM UID: 8607529. PMID: NLM14963866. PY - 2004 SN - 0882-5963 SP - 13-24 ST - Technological advances in pediatrics: challenges for parents and nurses T2 - Journal of Pediatric Nursing TI - Technological advances in pediatrics: challenges for parents and nurses UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106565848&site=ehost-live&scope=site VL - 19 ID - 1605 ER - TY - JOUR AB - The Fontan surgery involves the creation a conduit between the inferior vena cava and the right pulmonary artery. This conduit has a small fenestration that shunts the blood from right to left in case the pulmonary blood flow is limited; namely, if the pulmonary vascular resistance (PVR) is increased then the shunt is increased. Bacteria may bypass the pulmonary circulation and easily get access to the systemic circulation (bacteremia). We report the case of a patient that underwent Fontan surgery in 2010 and remained in a high-altitude city for 7 years, during this time he was asymptomatic until 2017 when he developed a brain abscess due to Streptococcus gordonii, a pathogen of dental plaque. Since high-altitude may raise PVR in response to reduction in the partial pressure of oxygen, we conclude that the long-term outcome of increased altitude on Fontan hemodynamics can lead to the shunt of teeth flora and consequently leading to severe infections. AD - J.D.C. Sierra, Pediatric Cardiology Hospitalist, Fundación Cardioinfantil, Cl. 163a #13B-60, Bogotá, Colombia AU - Sierra, J. D. C. AU - Mestra, C. F. AU - Nieto, J. C. G. AU - Dumit, M. R. AU - García-Torres, A. DB - Embase DO - 10.21037/atm.2018.02.05 IS - 7 KW - C reactive protein cefepime ceftriaxone erythromycin furosemide penicillin derivative spironolactone vancomycin warfarin altitude antibiotic sensitivity article bacterium identification brain abscess case report child clinical article computer assisted tomography craniotomy dental pulp capping depersonalization deterioration disease duration disorientation echocardiography erythrocyte sedimentation rate fever Fontan procedure general anesthesia headache hemodynamics human incubation time international normalized ratio leukocyte count lung vascular resistance male medical history minimum inhibitory concentration physical examination protein blood level risk factor school child Streptococcus gordonii tooth extraction tooth infection tooth plaque tricuspid valve atresia vomiting LA - English M3 - Article N1 - L621691717 2018-04-20 2018-04-30 PY - 2018 SN - 2305-5847 2305-5839 ST - Teeth infection may "shunt" through Fontan in high-altitude conditions T2 - Annals of Translational Medicine TI - Teeth infection may "shunt" through Fontan in high-altitude conditions UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L621691717 http://dx.doi.org/10.21037/atm.2018.02.05 VL - 6 ID - 694 ER - TY - JOUR AB - We repaired a case of pulmonary atresia with intact ventricular septum in which the blood supply to the left anterior descending coronary artery depended on the right ventricle. At the time of a bidirectional Glenn operation, total cardiopulmonary bypass with venous drainage from the right atrium was performed in order to evaluate the safety of right ventricular decompression required for a planned Fontan operation. We confirmed the dependence of the coronary perfusion on the right ventricle by demonstrating transient depression of the ST segment in the epicardial electrocardiogram during temporary decompression of the right ventricle. To prevent ischemic myocardial damage, we then performed an extracardiac Fontan operation with a temporary venous shunt and without cardiopulmonary bypass. AD - S. Iwai, Department of Cardiovascular Surgery, Osaka Medical Center, Research Institute for Maternal and Child Health, 840 Murodo-cho, Izumi, Osaka 594-1101, Japan AU - Iwai, S. AU - Kishimoto, H. AU - Kawata, H. DB - Embase Medline DO - 10.1007/s11748-005-0060-4 IS - 7 KW - angiography article cardiopulmonary bypass case report coronary artery blood flow decompression echocardiography electrocardiogram Fontan procedure heart muscle injury heart muscle ischemia heart right ventricle interventricular septum human infant left anterior descending coronary artery pulmonary valve atresia male LA - English M3 - Article N1 - L41105066 2005-08-30 PY - 2005 SN - 1344-4964 SP - 400-403 ST - Temporary decompression of the right ventricle to assess the right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum T2 - Japanese Journal of Thoracic and Cardiovascular Surgery TI - Temporary decompression of the right ventricle to assess the right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L41105066 http://dx.doi.org/10.1007/s11748-005-0060-4 VL - 53 ID - 1275 ER - TY - JOUR AB - Although the accumulation of gas is the most common cause of an expanding interpleural space, the presence of other structures or substances (hydrothorax, gastrothorax, hemothorax, urohemothorax, pyothorax, and chylothorax) under pressure may be sufficient to cause hemodynamic and respiratory compromise. We present two pediatric patients that developed hemodynamic and respiratory effects secondary to a chylothorax. The first patient presented in respiratory distress and cardiovascular collapse 4 weeks after a Fontan procedure. Placement of a chest tube resulted in the release of chyle under pressure and prompt resolution of hemodynamic and respiratory symptoms. The second patient was a 2100 g neonate who developed a chylothorax during an episode of sepsis following gastroschisis repair. On two separate occasions, the development of the chylothorax was associated with tachycardia, oliguria, and increased requirements during mechanical ventilation. Chest tube placement resulted in the release of chyle under pressure and resolution of the symptoms. These two cases demonstrate that chylothorax like pneumothorax can have deleterious effects on hemodynamic and respiratory function. AD - Department of Anesthesiology, University of Missouri, Columbia, MO 65212, USA. AN - 17474958 AU - Wheeler, A. D. AU - Tobias, J. D. DA - May DB - PubMed DO - 10.1111/j.1460-9592.2006.02153.x DP - NLM ET - 2007/05/04 IS - 5 KW - Blood Pressure Chest Tubes Child, Preschool Chylothorax/complications/*diagnosis/*therapy Drainage/methods Female Heart Rate Humans Infant, Newborn Pleural Effusion/etiology/therapy Postoperative Complications/diagnosis/therapy Respiratory Insufficiency/etiology/therapy Sepsis/complications LA - eng N1 - Wheeler, Adam D Tobias, Joseph D Case Reports Journal Article France Paediatr Anaesth. 2007 May;17(5):488-91. doi: 10.1111/j.1460-9592.2006.02153.x. PY - 2007 SN - 1155-5645 (Print) 1155-5645 SP - 488-91 ST - Tension chylothorax in two pediatric patients T2 - Paediatr Anaesth TI - Tension chylothorax in two pediatric patients VL - 17 ID - 455 ER - TY - JOUR AB - BACKGROUND: The influence of ventricular assist device (VAD) use in the care of children with end-stage heart failure is growing rapidly through increasing numbers, new devices, expanding indications, and improving outcomes. Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs), a National Institutes of Health-sponsored United States database, provides a platform to understand this emerging population. METHODS: Between September 19, 2012, and December 31, 2017, 30 hospitals implanted 508 devices in 423 patients aged younger than 19 years. This past year was one of evolution for the database as its management was transitioned to The Society of Thoracic Surgery; therefore, this report does not include data from institutions not under contract by August 1, 2018. RESULTS: Of the 423 patients, the diagnosis was cardiomyopathy in 261 (62%), myocarditis in 48 (11%), other in 28 (7%), and congenital heart disease (CHD) in 86 (20%), with 52 of these patients having single-ventricle physiology. The two most common support strategies included left VAD in 342 (81%) and biventricular assist device in 64 (15%). Positive outcome (alive on device or bridge to transplantation/recovery) was 80% at 6 months (overall mortality of 20%). The patient cohort for implantable continuous-flow (IC) pumps (n = 197; age at implant, 13.4 ± 3.8 years; 19% Interagency Registry for Mechanically Assisted Circulatory Support [Intermacs] profile 1, 21% intubated at implant, and 12% with CHD) was significantly different from the paracorporeal continuous-flow (PC) pump cohort (n = 79; age, 3.9 ± 5.2 years; 49% Intermacs profile 1, 86% intubated at implant, and 38% with CHD) and the paracorporeal pulsatile (PP) pump cohort (n = 121; age, 3.3 ± 3.9 years; 41% Intermacs profile 1, 77% intubated at implant, and 21% with CHD). Consistent with their cohort composition, device type positive outcomes at 6 months were PC, 63%; PP, 77%; and IC, 92%. Parametric hazard modeling showed an early hazard for death was associated with Intermacs profile 1, biventricular assist device, percutaneous devices, PC devices, small-volume institutions, low age, and low weight, whereas a constant hazard was associated with intubation and liver dysfunction at time of implant. CONCLUSIONS: IC VADs are the most common VAD type placed in children. The positive outcomes for the IC VADs exceed 90% at 6 months, which may represent our field's maturation in both patient selection and timing of implantation. The PP/PC devices are currently limited to supporting our most challenging patients, those weighing less than 20 kg and those with CHD. The introduction of new devices and our communities' commitment to shared learning and improvement will lead to more pediatric lives saved by VAD support and will also focus on improving the quality of life of children supported with VADs. AD - The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Electronic address: david.morales@cchmc.org. Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. Department of Cardiology, Children's Hospital Boston, Boston, Massachusetts. The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Kirklin Institute for Research in Surgical Outcomes, Birmingham, Alabama. Division of Cardiac Surgery, Children's Mercy Hospital, Kansas City, Missouri. Division of Cardiology, Children's Health Dallas, Dallas, Texas. Division of Cardiac Surgery, Texas Children's Hospital, Houston, Texas. Division of Cardiology, Stanford University, Palo Alto, California. AN - 30817920 AU - Morales, D. L. S. AU - Rossano, J. W. AU - VanderPluym, C. AU - Lorts, A. AU - Cantor, R. AU - St Louis, J. D. AU - Koeh, D. AU - Sutcliffe, D. L. AU - Adachi, I. AU - Kirklin, J. K. AU - Rosenthal, D. N. AU - Blume, E. D. DA - Apr DB - PubMed DO - 10.1016/j.athoracsur.2019.01.038 DP - NLM ET - 2019/03/01 IS - 4 KW - Adolescent Child Child, Preschool Female Heart Failure/diagnosis/*surgery Heart Transplantation/methods/*mortality Heart-Assist Devices/*statistics & numerical data Hospitals, Pediatric Humans Male Pediatrics Preoperative Care/methods *Registries Risk Assessment Societies, Medical Survival Rate *Thoracic Surgery Treatment Outcome United States LA - eng N1 - 1552-6259 Morales, David L S Rossano, Joseph W VanderPluym, Christina Lorts, Angela Cantor, Ryan St Louis, James D Koeh, Devin Sutcliffe, David L Adachi, Iki Kirklin, James K Rosenthal, David N Blume, Elizabeth D Pedimacs Investigators Journal Article Multicenter Study Netherlands Ann Thorac Surg. 2019 Apr;107(4):993-1004. doi: 10.1016/j.athoracsur.2019.01.038. Epub 2019 Feb 26. PY - 2019 SN - 0003-4975 SP - 993-1004 ST - Third Annual Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) Report: Preimplant Characteristics and Outcomes T2 - Ann Thorac Surg TI - Third Annual Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) Report: Preimplant Characteristics and Outcomes VL - 107 ID - 13 ER - TY - JOUR AB - Background: The influence of ventricular assist device (VAD) use in the care of children with end-stage heart failure is growing rapidly through increasing numbers, new devices, expanding indications, and improving outcomes. Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs), a National Institutes of Health–sponsored United States database, provides a platform to understand this emerging population. Methods: Between September 19, 2012, and December 31, 2017, 30 hospitals implanted 508 devices in 423 patients aged younger than 19 years. This past year was one of evolution for the database as its management was transitioned to The Society of Thoracic Surgery; therefore, this report does not include data from institutions not under contract by August 1, 2018. Results: Of the 423 patients, the diagnosis was cardiomyopathy in 261 (62%), myocarditis in 48 (11%), other in 28 (7%), and congenital heart disease (CHD) in 86 (20%), with 52 of these patients having single-ventricle physiology. The two most common support strategies included left VAD in 342 (81%) and biventricular assist device in 64 (15%). Positive outcome (alive on device or bridge to transplantation/recovery) was 80% at 6 months (overall mortality of 20%). The patient cohort for implantable continuous-flow (IC) pumps (n = 197; age at implant, 13.4 ± 3.8 years; 19% Interagency Registry for Mechanically Assisted Circulatory Support [Intermacs] profile 1, 21% intubated at implant, and 12% with CHD) was significantly different from the paracorporeal continuous-flow (PC) pump cohort (n = 79; age, 3.9 ± 5.2 years; 49% Intermacs profile 1, 86% intubated at implant, and 38% with CHD) and the paracorporeal pulsatile (PP) pump cohort (n = 121; age, 3.3 ± 3.9 years; 41% Intermacs profile 1, 77% intubated at implant, and 21% with CHD). Consistent with their cohort composition, device type positive outcomes at 6 months were PC, 63%; PP, 77%; and IC, 92%. Parametric hazard modeling showed an early hazard for death was associated with Intermacs profile 1, biventricular assist device, percutaneous devices, PC devices, small-volume institutions, low age, and low weight, whereas a constant hazard was associated with intubation and liver dysfunction at time of implant. Conclusions: IC VADs are the most common VAD type placed in children. The positive outcomes for the IC VADs exceed 90% at 6 months, which may represent our field's maturation in both patient selection and timing of implantation. The PP/PC devices are currently limited to supporting our most challenging patients, those weighing less than 20 kg and those with CHD. The introduction of new devices and our communities’ commitment to shared learning and improvement will lead to more pediatric lives saved by VAD support and will also focus on improving the quality of life of children supported with VADs. AD - D.L.S. Morales, The Heart Institute, Cincinnati Children's Hospital Medical Center, The University of Cincinnati College of Medicine, 3333 Burnet Ave, MLC 2004, Cincinnati, OH, United States AU - Morales, D. L. S. AU - Rossano, J. W. AU - VanderPluym, C. AU - Lorts, A. AU - Cantor, R. AU - St. Louis, J. D. AU - Koeh, D. AU - Sutcliffe, D. L. AU - Adachi, I. AU - Kirklin, J. K. AU - Rosenthal, D. N. AU - Blume, E. D. DB - Embase Medline DO - 10.1016/j.athoracsur.2019.01.038 IS - 4 KW - biventricular assist device left ventricular assist device adolescent adult article assisted circulation bleeding brain hemorrhage brain ischemia cardiogenic shock cardiomyopathy cause of death cerebrovascular accident child cohort analysis congenital heart disease device malfunction extracorporeal oxygenation hospitalization human implantation infection liver dysfunction major clinical study mechanical circulatory support medical device complication mortality multiple organ failure myocarditis neurologic disease postoperative period preschool child priority journal register young adult LA - English M3 - Article N1 - L2001710885 2019-03-26 2019-03-29 PY - 2019 SN - 1552-6259 0003-4975 SP - 993-1004 ST - Third Annual Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) Report: Preimplant Characteristics and Outcomes T2 - Annals of Thoracic Surgery TI - Third Annual Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) Report: Preimplant Characteristics and Outcomes UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2001710885 http://dx.doi.org/10.1016/j.athoracsur.2019.01.038 VL - 107 ID - 618 ER - TY - JOUR AB - A 12 year-old boy who had received modified Fontan procedure was scheduled for cholecystectomy. Anesthesia was maintained with thoracic epidural anesthesia (Th8-9) and 02-N20-sevoflurane under mechanical ventilation through the endotracheal tube. Transeshophageal echo cardiography and mixed venous oxygen saturation (Svoz) were used for hemodynamic monitoring. The combined use of epidural anesthesia and volatile anesthetics decreased central venous pressure, left ventricular end diastolic volume, left ventricular ejection fraction and Sv̄o 2. These hemodynamic problems were easily solved by infusion therapy and a low dose of dopamine. On the other hand, there were also some hemodynamic benefits such as inhibition of tachycardia and suppression of an increase in pulmonary vascular resistance due to surgical stress. Moreover, patient returned to normal spontaneous breathing with complete analgesia during the early phase after surgery. CVP and Sv̄o 2 increased to preoperative values in the recovery room. From these results, we conclude that satisfactory results can be obtained with epidural anesthesia for upper abdominal surgery after Fontan procedure. AD - Department of Anesthesiology, Kitasato University, School of Medicine, SagamUiara, 228, Japan AU - Arai, M. AU - Kanai, A. AU - Matsuzaki, S. AU - Takenaka, T. AU - Kato, S. DB - Scopus IS - 2 M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 1997 SP - 274-275 ST - Thoracic epidural anesthesia for cholecystectomy in a patient after fontan procedure T2 - Japanese Journal of Anesthesiology TI - Thoracic epidural anesthesia for cholecystectomy in a patient after fontan procedure UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0030936182&partnerID=40&md5=f4753240208683609f8479f0c9535aad VL - 46 ID - 2294 ER - TY - JOUR AB - This is a cross-section of the author's experience with over 1,200 cases. Tumors: neuroblastoma is the most common; easily accessible parts only should be cut away, and the rest irradiated. Other tumors discussed include teratomas, lung cysts, and duplications of the gastrointestinal tract. In infantile lobar emphysema lobectomy is the treatment of choice. Compression of trachea or oesophagus by a double aortic arch, a right aortic arch with left ligament or an aberrant subclavian artery are removed by dividing the respective vessels; in tracheomalacia the thymus is removed and the aortic arch is tacked to the sternum thus stretching the soft trachea. Patent ductus arteriosus is a prosaic subject that may turn serious only in combination with other anomalies. An end-to-end anastomosis is sound therapy for most cases of coarctation of the aorta with the exception of a small group of infants mostly with other anomalies who are critically ill. Their operational risk is 40%. Congenital diaphragmatic hernia should be promptly operated upon, preferably in 2 stages; the need for very gentle elevation of the intrapulmonary pressure is stressed. The apparent fall in the recovery rate is explained by the fact that children who would have previously died are operated on within 24 hrs after delivery. Oesophageal hiatus hernia should be operated on after failure of conservative treatment. An anomalous left coronary artery should only be ligated when the flow in it is reversed and drains off the myocardium into the pulmonary artery. In the contrary event ligation would be fatal, and the pulmonary artery is to be banded. In ventricular septal defect it is essential to determine the pulmonary vascular resistance before deciding whether the defect should be closed or the pulmonary artery banded first. Both aortic and pulmonary stenosis that have symptoms in the early months are almost always fatal even with surgery. In cases of pulmonary or tricuspid atresia as well as in Fallot's tetralogy a Blalock anastomosis is preferred. True transposition of the great vessels is handled by making a larger interatrial opening when the communication is insufficient, in the contrary case the pulmonary artery is banded. In most critical conditions the hyperbaric chamber provides the surgeon the 'short but golden hour' for unhurried work. Oesophageal fistula has in the author's hands 100% survival for babies over 7 pounds, for those smaller a staged approach with gastostomy yields better results. AD - R.E. Gross, Surg. Serv., Child. Hosp., Harvard Med. Sch., Boston, MA, United States AU - Gross, R. E. DB - Embase Classic Medline IS - 2 KW - anastomosis aorta aortic arch baby child compression congenital diaphragm hernia conservative treatment critically ill patient emphysema end to end anastomosis esophagus esophagus fistula Fallot tetralogy gastrointestinal tract great blood vessel cardiac muscle heart ventricle septum defect hiatus hernia infant interpersonal communication left coronary artery ligament ligation lobectomy lung artery pressure lung cyst lung vascular resistance neuroblastoma patent ductus arteriosus pulmonary artery pulmonary valve stenosis risk sternum stretching subclavian artery surgeon surgery survival teratoma therapy thorax surgery thymus trachea tracheomalacia tricuspid valve atresia neoplasm LA - English M3 - Article N1 - L281225474 1964-12-01 PY - 1964 SN - 0022-5223 SP - 152-176 ST - Thoracic surgery for infants T2 - Journal of Thoracic and Cardiovascular Surgery TI - Thoracic surgery for infants UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L281225474 VL - 48 ID - 1416 ER - TY - JOUR AB - Purpose: Congenital diaphragmatic hernia (CDH) has traditionally been repaired via a laparotomy. More and more reports on thoracoscopic repair are being published. The aim of this study was to evaluate our series of thoracoscopic CDH repair and compare this group to an open repair group treated during the same period in the same institute. Patients and Methods: Between June 2006 and December 2008, 49 children with posterolateral CDH were admitted, of whom 23 (47%) were operated thoracoscopically and 23 (47%) using an open repair, depending on the discretion of the attending surgeon and the clinical condition of the patient. Three patients (6%) with CDH were not treated because of associated anomalies (twice Cornelia de Lange syndrome and once hypoplastic left heart syndrome). Six thoracoscopic operations (26%) were converted to open surgery. Nine defects (39%) were closed thoracoscopically without a patch. In 8 (35%) patients, a patch was used. We used a patch in 20 open procedures (87%). Results: Three (33%) of the 9 thoracoscopic repairs without patch and 1 (12%) of the 8 with a patch developed a recurrence. All these recurrences were repaired thoracoscopically. The 3 recurrences from the thoracoscopic primary repair were repaired using a patch. In the open group, 3 patients (13%) developed a recurrence, of whom 2 were repaired thoracoscopically. Mean operative time was significantly longer in the thoracoscopic patch repair group (158 minutes), when compared to the open repair group (125 minutes). Conclusion: As in open repair, it seems wise to use large patches liberally, not only to reconstruct the dome of the diaphragm but also to avoid undue tension on the repair and prevent recurrences. The thoracoscopic approach is also considered feasible in case of a recurrence from either a thoracoscopic or open repair. © 2010 Elsevier Inc. All rights reserved. AD - R. Keijzer, Department of Pediatric Surgery, ErasmusMC-Sophia, PO Box 2060, 3000 CB, Rotterdam, Netherlands AU - Keijzer, R. AU - van de Ven, C. AU - Vlot, J. AU - Sloots, C. AU - Madern, G. AU - Tibboel, D. AU - Bax, K. DB - Embase Medline DO - 10.1016/j.jpedsurg.2010.02.017 IS - 5 KW - article brain infarction child chylothorax clinical article congenital diaphragm hernia controlled study de Lange syndrome female hematothorax human hypoplastic left heart syndrome length of stay male operation duration priority journal pulmonary hypertension recurrence risk risk reduction safety surgical mortality suture thoracoscopic patch repair thoracoscopy LA - English M3 - Article N1 - L358727975 2010-05-07 2010-05-18 PY - 2010 SN - 0022-3468 SP - 953-957 ST - Thoracoscopic repair in congenital diaphragmatic hernia: patching is safe and reduces the recurrence rate T2 - Journal of Pediatric Surgery TI - Thoracoscopic repair in congenital diaphragmatic hernia: patching is safe and reduces the recurrence rate UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L358727975 http://dx.doi.org/10.1016/j.jpedsurg.2010.02.017 VL - 45 ID - 1144 ER - TY - JOUR AB - While many congenital heart defects can be treated without significant long term sequelae, some achieve successful palliation as their definitive endpoints. The single-ventricle defect is one such defect and leaves the child with only one operational ventricle, requiring the systemic and the pulmonary circulations to be placed in series through several operations performed during early childhood. Numerical simulations may be used to investigate these hemodynamic conditions and their relation to post-operative sequelae; however, they rely heavily on boundary condition prescription. In this study, we investigate the impact of hemodynamic input data uncertainties on simulation results. Imaged-based patient-specific models of the multi-branched pulmonary arteries and superior vena cava were built for five cavopulmonary connection (i.e. Glenn) patients. Magnetic resonance imaging and catheterization data were acquired for each patient prior to their Fontan surgery. Inflow and outflow boundary conditions were constructed to match available clinical data and resulted in the development of a framework to incorporate these types of clinical data into patient-specific simulations. Three-dimensional computational fluid dynamics simulations were run and hemodynamic indicators were computed. Power loss was low (and efficiency very high) and a linear correlation was found between power loss and cardiac index among the five patients. Other indicators such as low wall shear stress were considered to better characterize these patients. Flow was complex and oscillatory near the anastomosis, and laminar in the smaller branches. While common trends were seen among patients, results showed differences among patients, especially in the 3D maps, strengthening the importance of patient-specific simulations. A sensitivity analysis was performed to investigate the impact of input data (clinical and modeling) to construct boundary conditions on several indicators. Overall, the sensitivity of the output indicators to the input data was small but non-negligible. The sensitivity of commonly used hemodynamic indicators to compare patients is discussed in this context. Power efficiency was much more sensitive to pressure variation than power loss. To increase the precision of such indicators, mean flow split between right and left lungs needs to be measured with more accuracy with higher priority than refining the model of how the flow is distributed on average among the smaller branches. Although ± 10% flow split imprecision seemed reasonable in terms of patient comparison, this study suggests that the common practice of imposing a right pulmonary artery/left pulmonary artery flow split of 55%/45% when performing patient specific simulations should be avoided. This study constitutes a first step towards understanding the hemodynamic differences between pre- and post Fontan surgery, predicting these differences, and evaluating surgical outcomes based on preoperative data. AD - Institute for Computational and Mathematical Engineering, Stanford University, Stanford, CA 94305, USA. AN - 22168738 AU - Troianowski, G. AU - Taylor, C. A. AU - Feinstein, J. A. AU - Vignon-Clementel, I. E. DA - Nov DB - PubMed DO - 10.1115/1.4005377 DP - NLM ET - 2011/12/16 IS - 11 KW - Biomechanical Phenomena Child, Preschool Computer Simulation Fontan Procedure/*statistics & numerical data Heart Defects, Congenital/pathology/*physiopathology/*surgery Hemodynamics Humans Imaging, Three-Dimensional Models, Anatomic Models, Cardiovascular Pulmonary Artery/pathology/physiopathology/surgery Vena Cava, Superior/pathology/physiopathology/surgery LA - eng N1 - 1528-8951 Troianowski, G Taylor, C A Feinstein, J A Vignon-Clementel, I E Journal Article United States J Biomech Eng. 2011 Nov;133(11):111006. doi: 10.1115/1.4005377. PY - 2011 SN - 0148-0731 SP - 111006 ST - Three-dimensional simulations in Glenn patients: clinically based boundary conditions, hemodynamic results and sensitivity to input data T2 - J Biomech Eng TI - Three-dimensional simulations in Glenn patients: clinically based boundary conditions, hemodynamic results and sensitivity to input data VL - 133 ID - 457 ER - TY - JOUR AB - We first report the case of a 14-year-old boy, previously treated with a modified Fontan operation at the age of 2, who underwent an intra-arterial thrombolysis for an ischemic stroke with a favorable outcome. No controlled randomized trials on thrombolysis among childhood stroke have been conducted yet. This and other similar cases suggest that thrombolysis may also be safe and beneficial in pediatric patients and those cases may benefit from a prompt access to specialized stroke centers. © 2011-IOS Press and the authors. All rights reserved. AD - M. Bianco, Neurology Department, San Raffaele Scientific Institute, Via Olgettina, 58, 20132 Milan, Italy AU - Bianco, M. AU - Simionato, F. AU - Giusti, C. AU - Corea, F. AU - Giua, A. AU - Martinelli, V. AU - Comi, G. AU - Sessa, M. DB - Embase DO - 10.3233/JPN-2012-0516 IS - 4 KW - alteplase adolescent article ataxic aphasia blood clot lysis brain ischemia case report Caucasian cavopulmonary connection child computer assisted tomography Fontan procedure general anesthesia hemiparesis hemiplegia human Italy magnetic resonance angiography male middle cerebral artery occlusion preschool child LA - English M3 - Article N1 - L364620966 2012-04-23 2012-04-25 PY - 2011 SN - 1304-2580 1305-0613 SP - 497-500 ST - Thrombolysis with rt-PA for an ischemic stroke in boy treated with Fontan operation T2 - Journal of Pediatric Neurology TI - Thrombolysis with rt-PA for an ischemic stroke in boy treated with Fontan operation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L364620966 http://dx.doi.org/10.3233/JPN-2012-0516 VL - 9 ID - 1079 ER - TY - JOUR AB - Objectives: To describe the incidence of thrombotic complications across all 3 stages of single ventricle palliation and the association between thromboprophylaxis use and thrombotic risk. Study design: Two separate cross-sectional studies were performed that included 195 patients born between 2003-2008 and 162 patients who underwent Fontan after 2000. Results: The incidence of thrombotic complications was 40% and 28% after initial palliation and superior cavopulmonary connection (SCPC), respectively; 5-year freedom from thrombotic complications after Fontan was 79%. Thromboprophylaxis was initiated for 70%, 46%, and 94% of patients after initial palliation, SCPC, and Fontan, respectively. Thromboprophylaxis with enoxaparin (vs no thromboprophylaxis) was associated with a reduction in risk of thrombotic complications after initial palliation (hazard ratio [HR] 0.5, P = .05) and SCPC (HR 0.2, P = .04). Thromboprophylaxis with warfarin was associated with a reduction in thrombotic complications after Fontan (HR 0.27, P = .05 vs acetylsalicylic acid; HR 0.18, P = .02 vs no thromboprophylaxis). Thrombotic complications were associated with increased mortality after initial palliation (HR 5.5, P < .001) and SCPC (HR 12.5, P < .001). Three patients experienced major bleeding complications without permanent sequelae (2 enoxaparin, 1 warfarin). Conclusions: Given the negative impact of thrombotic complications on survival, the low risk of serious bleeding complications, and the association between thromboprophylaxis and lowered thrombotic complication risk across all 3 palliative stages, routine use of thromboprophylaxis from the initial palliation to the early post-Fontan period in this population may be indicated. © 2012 Mosby, Inc. AD - B.W. McCrindle, Hospital for Sick Children, 555 University Avenue, Toronto, ON M5G 1X8, Canada AU - Manlhiot, C. AU - Brandão, L. R. AU - Kwok, J. AU - Kegel, S. AU - Menjak, I. B. AU - Carew, C. L. AU - Chan, A. K. AU - Schwartz, S. M. AU - Sivarajan, V. B. AU - Caldarone, C. A. AU - Van Arsdell, G. S. AU - McCrindle, B. W. DB - Embase Medline DO - 10.1016/j.jpeds.2012.03.004 IS - 3 KW - acetylsalicylic acid enoxaparin warfarin article bleeding brain hematoma female Fontan procedure human infant initial thrombosis palliation major clinical study male mortality newborn portocaval shunt priority journal risk reduction single ventricle heart palliation superior cavopulmonary connection thrombosis thrombosis prevention LA - English M3 - Article N1 - L51964970 2012-04-19 2012-09-05 PY - 2012 SN - 0022-3476 1097-6833 SP - 513-519.e3 ST - Thrombotic complications and thromboprophylaxis across all three stages of single ventricle heart palliation T2 - Journal of Pediatrics TI - Thrombotic complications and thromboprophylaxis across all three stages of single ventricle heart palliation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51964970 http://dx.doi.org/10.1016/j.jpeds.2012.03.004 VL - 161 ID - 1046 ER - TY - JOUR AB - A coronary artery embolism is an uncommon non-atherosclerotic cause of acute myocardial infarction. We present a case of coronary artery embolism in a patient with past history of hypoplastic left heart syndrome that was initially palliated via a modified Blalock–Taussig shunt followed by a Fontan anastomosis and Damus–Kaye–Stansel type anastomosis of the pulmonary valve to the ascending hypoplastic aorta. In this case we demonstrate usefulness of cardiac CT to delineate the patient's complex anatomy, identify the presence and etiology of a thromboembolic event, as well as assess for appropriate response to anticoagulation therapy. © 2016 Society of Cardiovascular Computed Tomography AD - Non-Invasive Cardiovascular Imaging Program, Division of Cardiovascular Medicine and Department of Cardiology, Brigham and Women's Hospital, Harvard Medical School, United States AU - Keraliya, A. R. AU - Murphy, D. J. AU - Steigner, M. L. AU - Blankstein, R. DB - Scopus DO - 10.1016/j.jcct.2016.03.003 IS - 3 KW - Cardiac CT Coronary artery embolism Hypoplastic left heart syndrome M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2016 SP - 263-264 ST - Thrombus in hypoplastic aorta: An uncommon cause of acute myocardial infarction T2 - Journal of Cardiovascular Computed Tomography TI - Thrombus in hypoplastic aorta: An uncommon cause of acute myocardial infarction UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84961843567&doi=10.1016%2fj.jcct.2016.03.003&partnerID=40&md5=4506109cef4594daa38d1b0af03956eb VL - 10 ID - 1902 ER - TY - JOUR AB - Tissue engineering holds great promise for the advancement of cardiovascular surgery as well as other medical fields. Tissue-engineered vascular grafts have the ability to grow and remodel and could therefore make great advances for pediatric cardiovascular surgery. In 2001, we began a human clinical trial evaluating these grafts in patients with a univentricular physiology. Herein, we report the long-term results of patients who underwent implantation of tissue-engineered vascular grafts as extracardiac total cavopulmonary conduits. Tissue-engineered vascular grafts seeded with autologous bone marrow mononuclear cells were implanted in 25 patients with univentricular physiology. The graft is composed of a woven fabric of poly-l-lactide acid or polyglycolic acid and a 50:50 poly (l-lactic-co-ε-caprolactone) copolymer. Patients were followed up with postoperatively in a multidisciplinary clinic. Median patient age at operation was 5.5 years and the mean follow-up period was 11.1 years. There was no graft-related mortality during the follow-up period. There was also no evidence of aneurysmal formation, graft rupture, graft infection, or calcification. Seven (28%) patients had asymptomatic graft stenosis and underwent successful balloon angioplasty. Stenosis is the primary complication of the tissue-engineered vascular graft. Avoidance of anticoagulation therapy would improve patients' quality of life. Tissue-engineered vascular grafts have feasibility in pediatric cardiovascular surgery. AD - Tissue Engineering Program and Surgical Research, Nationwide Children's Hospital, Columbus, Ohio. Department of Cardiovascular Surgery, Tokyo Women's Medical University, Tokyo, Japan. Tissue Engineering Program and Surgical Research, Nationwide Children's Hospital, Columbus, Ohio; Department of Cardiothoracic Surgery, The Heart Center, Nationwide Children's Hospital, Columbus, Ohio. Electronic address: toshiharu.shinoka@nationwidechildrens.org. AN - 29427773 AU - Sugiura, T. AU - Matsumura, G. AU - Miyamoto, S. AU - Miyachi, H. AU - Breuer, C. K. AU - Shinoka, T. C2 - PMC6380348 C6 - NIHMS1012140 DA - Summer DB - PubMed DO - 10.1053/j.semtcvs.2018.02.002 DP - NLM ET - 2018/02/11 IS - 2 KW - Adolescent Age Factors Angioplasty, Balloon *Bioprosthesis *Blood Vessel Prosthesis Blood Vessel Prosthesis Implantation/adverse effects/*instrumentation Child Child, Preschool Computed Tomography Angiography Feasibility Studies Female Follow-Up Studies Fontan Procedure/adverse effects/*instrumentation Graft Occlusion, Vascular/diagnostic imaging/physiopathology/therapy Heart Defects, Congenital/diagnostic imaging/physiopathology/*surgery Humans Infant Male Polyesters/chemistry Prosthesis Design Quality of Life Stem Cell Transplantation/adverse effects/*methods Time Factors Tissue Engineering/*methods *Tissue Scaffolds Treatment Outcome Vascular Patency Vena Cava, Inferior/diagnostic imaging/physiopathology/*surgery Young Adult *Fontan surgery *congenital heart disease *pediatric cardiac surgery *tissue-engineering *univentricular physiology from Gunze Ltd. (Kyoto, Japan). The other authors report no conflicts of interest. LA - eng N1 - 1532-9488 Sugiura, Tadahisa Matsumura, Goki Miyamoto, Shinka Miyachi, Hideki Breuer, Christopher K Shinoka, Toshiharu R01 HL098228/HL/NHLBI NIH HHS/United States R01 HL128847/HL/NHLBI NIH HHS/United States Clinical Trial Journal Article Video-Audio Media Semin Thorac Cardiovasc Surg. 2018 Summer;30(2):175-179. doi: 10.1053/j.semtcvs.2018.02.002. Epub 2018 Feb 7. PY - 2018 SN - 1043-0679 (Print) 1043-0679 SP - 175-179 ST - Tissue-engineered Vascular Grafts in Children With Congenital Heart Disease: Intermediate Term Follow-up T2 - Semin Thorac Cardiovasc Surg TI - Tissue-engineered Vascular Grafts in Children With Congenital Heart Disease: Intermediate Term Follow-up VL - 30 ID - 25 ER - TY - JOUR AB - Only 27 cases (including those of the authors) of type III total anomalous venous drainage have been reported. The authors describe 3 cases in detail and suggest that this diagnosis can be made on a combination of X-ray and clinical features. In this type the pulmonary venous return is below the diaphragm, i.e. into the portal vein, ductus venosus or inferior vena cava. In each of the cases reported the correct diagnosis was suggested by the radiologist. It is the apparent discrepancy of a normal-sized heart in a cyanotic newborn with both clinical and X-ray signs of congestive failure which permits the correct diagnosis to be entertained. The pulmonary congestion and oedema probably occur as a result of obstruction to pulmonary venous blood flow due to the small cross-sectional area of the common pulmonary vein. Total anomalous pulmonary venous return below the diaphragm forms an entity quite different from total communication in the thorax, since most of the latter show obvious cardiomegaly. The differential diagnosis includes interstitial pneumonitis, cor triatriatum and hypoplastic left heart syndrome as well as other types of total anomalous pulmonary venous return. Seaman - New York, N.Y. (XIV, 18). AD - G.B.C. Harris, Dept. of Radiol., Child. Hosp. Med. Center, Harvard Med. Sch, Boston, MA, United States AU - Harris, G. B. C. AU - Neuhauser, E. B. D. AU - Giedion, A. DB - Embase Classic Medline IS - 3 KW - cardiomegaly clinical feature cor triatriatum diagnosis diaphragm differential diagnosis ductus venosus edema heart hypoplastic left heart syndrome inferior cava vein interpersonal communication interstitial pneumonia lung congestion lung vein drainage anomaly newborn obstruction patient hepatic portal vein pulmonary vein radiologist sailor thorax United States vein blood flow venous return X ray LA - English M3 - Article N1 - L280887846 1960-12-01 PY - 1960 SN - 0092-5381 SP - 436-441 ST - Total anomalous pulmonary venons return below the diaphragm. The roentgen appearances in three patients diagnosed during life T2 - American journal of roentgenology TI - Total anomalous pulmonary venons return below the diaphragm. The roentgen appearances in three patients diagnosed during life UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L280887846 VL - 84 ID - 1421 ER - TY - JOUR AB - The development of durable ventricular assist devices (VADs) has improved mortality rates and quality of life in patients with end stage heart failure. While the use of VADs has increased dramatically in recent years, there is limited experience with VAD implantation in patients with complex congenital heart disease (CHD), despite the fact that the number of patients with end stage CHD has grown due to improvements in surgical and medical care. VAD use has been limited in patients with CHD and end stage heart failure due to anatomic (systemic right ventricle, single ventricle, surgically altered anatomy, valve dysfunction, etc.) and physiologic constraints (diastolic dysfunction). The total artificial heart (TAH), which has right and left sided pumps that can be arranged in a variety of orientations, can accommodate the anatomic variation present in CHD patients. This review provides an overview of the potential use of the TAH in patients with CHD. AD - Cincinnati Children's Hospital Medical Center, Heart InstituteCincinnati, OH, USA. AN - 28536530 AU - Villa, C. R. AU - Morales, D. L. S. C2 - PMC5422510 DB - PubMed DO - 10.3389/fphys.2017.00131 DP - NLM ET - 2017/05/26 KW - bridge to transplantation congenital heart disease mechanical circulatory support pediatrics total artificial heart LA - eng N1 - 1664-042x Villa, Chet R Morales, David L S Journal Article Review Front Physiol. 2017 May 9;8:131. doi: 10.3389/fphys.2017.00131. eCollection 2017. PY - 2017 SN - 1664-042X (Print) 1664-042x SP - 131 ST - The Total Artificial Heart in End-Stage Congenital Heart Disease T2 - Front Physiol TI - The Total Artificial Heart in End-Stage Congenital Heart Disease VL - 8 ID - 527 ER - TY - JOUR AB - Background. There is a paucity of information to guide the management of the child with Down's syndrome and congenital heart disease in whom biventricular repair is precluded. Methods. Through the cardiology and cardiovascular surgery databases of The Hospital for Sick Children and Toronto Congenital Cardiac Centre for Adults, we identified patients with trisomy 21 and ventricular hypoplasia who had undergone a Fontan procedure (or modification). Results. Of 533 patients who had undergone a Fontan operation between 1976 and 1997, 4 had trisomy 21. All 4 patients had unbalanced complete atrioventricular septal defect with right ventricular hypoplasia in 3 and left ventricular hypoplasia in 1. Three patients survived, and 1 died of endocarditis. The 3 survivors have done well in the short term and medium term without complications related to the pulmonary vasculature. Conclusions. We suggest that in appropriately selected patients with trisomy 21 and ventricular hypoplasia who are unsuitable for two or one and a half ventricle repair, the Fontan procedure is not contraindicated and provides short, term and medium-term benefit. AD - I. Adatia, Critical Care Medicine/Cardiology, Hospital for Sick Children, 555 University Ave, Toronto, Ont. M5G 1X8, Canada AU - Campbell, R. M. AU - Adatia, I. AU - Gow, R. M. AU - Webb, G. D. AU - Williams, W. G. AU - Freedom, R. M. DB - Embase Medline DO - 10.1016/S0003-4975(98)00463-9 IS - 2 KW - article case report child congenital heart disease Down syndrome endocarditis Fontan procedure heart atrium septum defect human hypoplastic left heart syndrome lung artery banding priority journal treatment outcome trisomy 21 LA - English M3 - Article N1 - L28410689 1998-09-23 PY - 1998 SN - 0003-4975 SP - 523-526 ST - Total cavopulmonary anastomosis (Fontan) in children with Down's syndrome T2 - Annals of Thoracic Surgery TI - Total cavopulmonary anastomosis (Fontan) in children with Down's syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L28410689 http://dx.doi.org/10.1016/S0003-4975(98)00463-9 VL - 66 ID - 1349 ER - TY - JOUR AB - The status of pulmonary circulation is of utmost importance to the success of the modified Fontan operation. Pulmonary artery distortion, hypoplasia of the total pulmonary vascular bed, and elevated pulmonary vascular resistance are all risk factors for adverse outcome after modified Fontan operations. In cases of irreparable acquired atresia of one or the other branch pulmonary artery, clinicians are forced to contemplate the possibility of total cavopulmonary connection to one lung. The combined experience of the authors with 12 cases suggests that the likelihood of operative survival following Fontan's operation to one lung is predicted based on the usual hemodynamic parameters: pulmonary artery pressure and flow, ventricular end diastolic pressure, transpulmonary gradient, and pulmonary vascular resistance. In this series, there were no operative mortalities among patients undergoing Fontan's operation to one lung, all of whom meet the usual criteria for hemodynamic acceptability. There may, however, be a higher incidence of protein-losing enteropathy than in Fontan patients with normal pulmonary vascular beds. All possible means of resuscitating the lost elements of the pulmonary vascular bed and re-establishing pulmonary artery continuity should be attempted to minimize pulmonary vascular capacitance of patients undergoing Fontan's operation. It is clear, however, that the presence of only one pulmonary artery does not in and of itself preclude satisfactory outcome. © 2004 Elsevier Inc. All rights reserved. AD - M.L. Jacobs, St. Christopher's Hosp. for Children, Eric Ave. Front St., Philadelphia, PA 19134, United States AU - Jacobs, M. L. AU - Schneider, D. J. AU - Pourmoghadam, K. K. AU - Pizarro, C. AU - Norwood, W. I. C1 - aspirin lasix DB - Embase Medline DO - 10.1053/j.pcsu.2004.02.008 KW - acetylsalicylic acid captopril digoxin enalapril furosemide octreotide oxygen prednisone spironolactone article child Fontan procedure heart ventricle enddiastolic pressure hemodynamic parameters human hypoplastic left heart syndrome lung artery pressure lung blood flow lung circulation lung hypoplasia lung vascular resistance patient satisfaction pulmonary valve atresia risk factor surgical mortality survival treatment outcome tricuspid valve atresia aspirin lasix LA - English M3 - Article N1 - L38678727 2004-06-08 PY - 2004 SN - 1092-9126 SP - 72-79 ST - Total cavopulmonary connection to one lung T2 - Pediatric Cardiac Surgery Annual TI - Total cavopulmonary connection to one lung UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L38678727 http://dx.doi.org/10.1053/j.pcsu.2004.02.008 VL - 7 ID - 1294 ER - TY - JOUR AB - Background: Fenestration in the modified Fontan operation allows right-to-left shunting, which reduces the Fontan pathway pressure and improves cardiac output. However, on account of the fact that persistent right-to-left shunting results in cyanosis and paradoxical emboli, fenestration closure is recommended after recovery from the Fontan operation. Methods: This study recruited 3 patients who underwent the transcatheter closure of the Fontan fenestration with the ASD-Amplatzer because of severe cyanosis and significant intracardiac shunts. Results: Fenestration closure was performed at a mean age of 8 yr (6-12 yr) and average of 15 months after the Fontan operation. Aortic O2 saturation increased by an average of 17.6% (9-26%). During more than a two-year period of follow-up (mean: 27 months), two patients had complete occlusion on echocardiography and the other one had a small residual shunt. One of these patients had atrial flutter during the follow-up. Conclusion: The transcatheter closure of the Fontan fenestration is a safe and feasible technique that is effective in elevating systemic O2 saturation and well-being and confers acceptable growth and development in children. AD - R. Emamzadegan, Shahid Rajaei Hospital, Iran University of Medical Sciences, Valiye-Asr Street, Tehran, Iran AU - Meraji, S. M. AU - Davari, P. N. AU - Aarabi, M. Y. AU - Shahmohammadi, A. A. AU - Mortezaeian, H. AU - Emamzadegan, R. DB - Embase IS - 1 KW - oxygen aorta arterial oxygen saturation article case report catheter child clinical effectiveness cyanosis disease severity female fenestration follow up Fontan procedure great vessels transposition heart atrium flutter heart catheterization heart right ventricle double outlet heart septum defect heart single ventricle heart ventricle septum defect human male patient safety pulmonary valve stenosis transthoracic echocardiography tricuspid valve atresia ASD-Amplatzer LA - English M3 - Article N1 - L352197094 2008-08-29 PY - 2008 SN - 1735-8620 SP - 39-42 ST - Transcatheter closure of fenestration after modified fontan operation in children T2 - Journal of Tehran University Heart Center TI - Transcatheter closure of fenestration after modified fontan operation in children UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L352197094 VL - 3 ID - 1193 ER - TY - JOUR AB - BACKGROUND: Almost all reports on cardiac regeneration therapy have referred to adults, and only a few have focused on transcoronary infusion of cardiac progenitor cells using the stop-flow technique in children. METHODS: Intracoronary autologous cardiosphere-derived cell (CDC) transfer was conducted at Okayama University as a phase 1 clinical trial for seven patients with hypoplastic left heart syndrome between January 2011 and December 2012, and as a phase 2 clinical trial for 34 patients with single-ventricle physiology between July 2013 and March 2015. RESULTS: A total of 41 patients with single-ventricle physiology underwent transcoronary infusion of CDC with the stop-flow technique. The median age was 33 months (range, 5-70 months) and the median weight was 10.1 kg (range, 4.1-16.0 kg). Transient adverse events occurred during the procedure, including ST-segment elevation or depression, hypotension, bradycardia, and coronary artery vasospasm. All patients completely recovered. There were no major procedure-related adverse events. In this study, transcoronary infusion of CDC using the stop-flow technique was successfully completed in all patients. CONCLUSION: Transcoronary infusion of CDC using the stop-flow technique in children is a feasible and safe procedure. AD - Department of Pediatrics, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan. Department of Pediatric Cardiology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan. Department of Cardiovascular Surgery, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan. Department of Regenerative Medicine, Center for Innovative Clinical Medicine, Okayama University Hospital, Okayama, Japan. AN - 29266671 AU - Eitoku, T. AU - Baba, K. AU - Kondou, M. AU - Kurita, Y. AU - Fukushima, Y. AU - Hirai, K. AU - Ohtsuki, S. AU - Ishigami, S. AU - Sano, S. AU - Oh, H. DA - Mar DB - PubMed DO - 10.1111/ped.13485 DP - NLM ET - 2017/12/22 IS - 3 KW - Cardiac Catheterization/adverse effects/*methods Child Child, Preschool Coronary Angiography/methods Coronary Vessels/surgery Female Humans Hypoplastic Left Heart Syndrome/*therapy Infant Male Stem Cell Transplantation/adverse effects/*methods Stroke Volume Transplantation, Autologous/methods Treatment Outcome congenital heart disease regeneration therapy stop-flow technique temporary occlusion balloon transcoronary infusion LA - eng N1 - 1442-200x Eitoku, Takahiro Orcid: 0000-0002-8761-4464 Baba, Kenji Kondou, Maiko Kurita, Yoshihiko Fukushima, Yousuke Hirai, Kenta Ohtsuki, Shinichi Ishigami, Shuta Sano, Shunji Oh, Hidemasa Clinical Trial, Phase I Clinical Trial, Phase II Journal Article Australia Pediatr Int. 2018 Mar;60(3):240-246. doi: 10.1111/ped.13485. PY - 2018 SN - 1328-8067 SP - 240-246 ST - Transcoronary cell infusion with the stop-flow technique in children with single-ventricle physiology T2 - Pediatr Int TI - Transcoronary cell infusion with the stop-flow technique in children with single-ventricle physiology VL - 60 ID - 77 ER - TY - JOUR AB - Abstract: Background: Almost all reports on cardiac regeneration therapy have referred to adults, and only a few have focused on transcoronary infusion of cardiac progenitor cells using the stop‐flow technique in children. Methods: Intracoronary autologous cardiosphere‐derived cell (CDC) transfer was conducted at Okayama University as a phase 1 clinical trial for seven patients with hypoplastic left heart syndrome between January 2011 and December 2012, and as a phase 2 clinical trial for 34 patients with single‐ventricle physiology between July 2013 and March 2015. Results: A total of 41 patients with single‐ventricle physiology underwent transcoronary infusion of CDC with the stop‐flow technique. The median age was 33 months (range, 5–70 months) and the median weight was 10.1 kg (range, 4.1–16.0 kg). Transient adverse events occurred during the procedure, including ST‐segment elevation or depression, hypotension, bradycardia, and coronary artery vasospasm. All patients completely recovered. There were no major procedure‐related adverse events. In this study, transcoronary infusion of CDC using the stop‐flow technique was successfully completed in all patients. Conclusion: Transcoronary infusion of CDC using the stop‐flow technique in children is a feasible and safe procedure. AD - Department of Pediatrics, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan Department of Pediatric Cardiology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan Department of Cardiovascular Surgery, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan Department of Regenerative Medicine, Center for Innovative Clinical Medicine, Okayama University Hospital, Okayama, Japan AN - 128680352. Language: English. Entry Date: 20180413. Revision Date: 20190301. Publication Type: Article AU - Eitoku, Takahiro AU - Baba, Kenji AU - Kondou, Maiko AU - Kurita, Yoshihiko AU - Fukushima, Yousuke AU - Hirai, Kenta AU - Ohtsuki, Shinichi AU - Ishigami, Shuta AU - Sano, Shunji AU - Oh, Hidemasa DB - ccm DO - 10.1111/ped.13485 DP - EBSCOhost IS - 3 KW - Cell Therapy -- In Infancy and Childhood Angioplasty, Balloon -- Methods -- In Infancy and Childhood Heart Defects, Congenital Angioplasty, Balloon -- Adverse Effects Human Hypoplastic Left Heart Syndrome Coronary Vasospasm -- Etiology Bradycardia -- Etiology Hypotension -- Etiology Depression -- Etiology Treatment Outcomes Infant N1 - diagnostic images; research; tables/charts. Journal Subset: Asia; Biomedical; Peer Reviewed. NLM UID: 100886002. PY - 2018 SN - 1328-8067 SP - 240-246 ST - Transcoronary cell infusion with the stop‐flow technique in children with single‐ventricle physiology T2 - Pediatrics International TI - Transcoronary cell infusion with the stop‐flow technique in children with single‐ventricle physiology UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=128680352&site=ehost-live&scope=site VL - 60 ID - 1465 ER - TY - JOUR AB - Objectives Our aim was to assess midterm safety and clinical outcomes of intracoronary infusion of cardiosphere-derived cells (CDCs) after staged palliation in patients with hypoplastic left heart syndrome (HLHS). Methods In this prospective, controlled study, 14 consecutive patients with HLHS who were undergoing 2- or 3-stage surgical palliations were assigned to receive intracoronary CDC infusion 1 month after cardiac surgery (n = 7), followed by 7 patients allocated to a control group with standard care alone. The primary end point was to assess procedural feasibility and safety; the secondary end point was to evaluate cardiac function and heart failure status through 36-month follow-up. Results No complications, including tumor formation, were reported within 36 months after CDC infusion. Echocardiography showed significantly greater improvement in right ventricular ejection fraction (RVEF) in infants receiving CDCs than in controls at 36 months (+8.0% ± 4.7% vs +2.2% ± 4.3%; P =.03). These cardiac function improvements resulted in reduced brain natriuretic peptide levels (P =.04), lower incidence of unplanned catheter interventions (P =.04), and higher weight-for-age z score (P =.02) at 36 months relative to controls. As independent predictors of treatment responsiveness, absolute changes in RVEF at 36 months were negatively correlated with age, weight-for-age z score, and RVEF at CDC infusion. Conclusions Intracoronary CDC infusion after staged procedure in patients with HLHS is safe and improves RVEF, which persists during 36-month follow-up. This therapeutic strategy may enhance somatic growth and reduce incidence of heart failure. AD - H. Oh, Department of Regenerative Medicine, Center for Innovative Clinical Medicine, Okayama University Hospital, 2-5-1 Shikata-cho, Kita-ku, Okayama, Japan AU - Tarui, S. AU - Ishigami, S. AU - Ousaka, D. AU - Kasahara, S. AU - Ohtsuki, S. AU - Sano, S. AU - Oh, H. DB - Embase Medline DO - 10.1016/j.jtcvs.2015.06.076 IS - 5 KW - brain natriuretic peptide aorta article body growth carcinogenesis cardiac stem cell child clinical article controlled study echocardiography event free survival extracorporeal oxygenation female Fontan procedure functional status heart ejection fraction heart function heart right ventricle enddiastolic volume heart right ventricle endsystolic volume heart single ventricle heart ventricle function human hypoplastic left heart syndrome lung vascular resistance male mean arterial pressure preschool child priority journal quality of life stem cell transplantation LA - English M3 - Article N1 - L605415483 2015-08-04 2015-12-09 PY - 2015 SN - 1097-685X 0022-5223 SP - 1198-1207 ST - Transcoronary infusion of cardiac progenitor cells in hypoplastic left heart syndrome: Three-year follow-up of the Transcoronary Infusion of Cardiac Progenitor Cells in Patients with Single-Ventricle Physiology (TICAP) trial T2 - Journal of Thoracic and Cardiovascular Surgery TI - Transcoronary infusion of cardiac progenitor cells in hypoplastic left heart syndrome: Three-year follow-up of the Transcoronary Infusion of Cardiac Progenitor Cells in Patients with Single-Ventricle Physiology (TICAP) trial UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L605415483 http://dx.doi.org/10.1016/j.jtcvs.2015.06.076 VL - 150 ID - 846 ER - TY - JOUR AB - PURPOSE: Children with hypoplastic left heart syndrome (HLHS) experience numerous vulnerabilities during transitions from hospital to home during their first year of life. This paper examines the parents' responses to the situations they experience during the initial interstage transition as described through illness blogs. DESIGN AND METHODS: A qualitative descriptive design through inductive theme development using thematic analysis was performed for this study. Text data were used from publically available blogs written by parents of HLHS children on their feelings and experiences during transitions between hospital- and home-care during the interstage period. RESULTS: Six blogs were analyzed. Overall, the lack of difference in normalcy for these parents and their families was apparent. Major themes included: thoughts and feelings when discharged from the hospital, difficulties with hospital readmissions, protecting their child from infection, and developing a support system. CONCLUSIONS: Parents looked forward to going home and learned all they could about properly caring for their child before going home. They were disappointed and frustrated when returning to the hospital for either emergency or scheduled admissions and were not prepared to see their child in the intensive care unit environment again. Many parents isolated themselves and avoided the hospital as much as possible to avoid their child developing an infection. All parents had support systems through family and/or friends and found an unexpected sense of support through their blog families. AD - Levine Children's Hospital, Carolinas Medical Center, Charlotte, North Carolina. Department of Acute and Specialty Care, School of Nursing, University of Virginia, Charlottesville, Virginia. Department of Pediatrics, University of Virginia School of Medicine, Charlottesville, Virginia. AN - 30098124 AU - March, S. AU - Keim-Malpass, J. DA - Jul DB - PubMed DO - 10.1111/jspn.12225 DP - NLM ET - 2018/08/12 IS - 3 KW - Adaptation, Psychological Adult Blogging Child, Preschool Female Home Care Services/*organization & administration Humans Hypoplastic Left Heart Syndrome/diagnosis/*nursing/surgery Infant Male Narration Parent-Child Relations Parents/*psychology *Patient Discharge Prognosis Qualitative Research Risk Assessment Transitional Care/*organization & administration *blog analysis *congenital heart defect *hypoplastic left heart syndrome *interstage *transitions in care LA - eng N1 - 1744-6155 March, Sarita Orcid: 0000-0003-3900-5047 Keim-Malpass, Jessica Orcid: 0000-0002-7035-8556 Lanford Award/University of Virginia/International Journal Article United States J Spec Pediatr Nurs. 2018 Jul;23(3):e12225. doi: 10.1111/jspn.12225. Epub 2018 Aug 11. PY - 2018 SN - 1539-0136 SP - e12225 ST - Transition experiences between hospital- and home-care for parents of children with hypoplastic left heart syndrome T2 - J Spec Pediatr Nurs TI - Transition experiences between hospital- and home-care for parents of children with hypoplastic left heart syndrome VL - 23 ID - 64 ER - TY - JOUR AB - Purpose: Children with hypoplastic left heart syndrome (HLHS) experience numerous vulnerabilities during transitions from hospital to home during their first year of life. This paper examines the parents' responses to the situations they experience during the initial interstage transition as described through illness blogs. Design and methods: A qualitative descriptive design through inductive theme development using thematic analysis was performed for this study. Text data were used from publically available blogs written by parents of HLHS children on their feelings and experiences during transitions between hospital‐ and home‐care during the interstage period. Results: Six blogs were analyzed. Overall, the lack of difference in normalcy for these parents and their families was apparent. Major themes included: thoughts and feelings when discharged from the hospital, difficulties with hospital readmissions, protecting their child from infection, and developing a support system. Conclusions: Parents looked forward to going home and learned all they could about properly caring for their child before going home. They were disappointed and frustrated when returning to the hospital for either emergency or scheduled admissions and were not prepared to see their child in the intensive care unit environment again. Many parents isolated themselves and avoided the hospital as much as possible to avoid their child developing an infection. All parents had support systems through family and/or friends and found an unexpected sense of support through their blog families. AD - Levine Children's Hospital, Carolinas Medical Center, Charlotte North Carolina Department of Acute and Specialty Care, School of Nursing, University of Virginia, Charlottesville Virginia Department of Pediatrics, University of Virginia School of Medicine, Charlottesville Virginia AN - 132601347. Language: English. Entry Date: 20181029. Revision Date: 20190701. Publication Type: Article AU - March, Sarita AU - Keim‐Malpass, Jessica DB - ccm DO - 10.1111/jspn.12225 DP - EBSCOhost IS - 3 KW - Parents -- Psychosocial Factors Transitional Care Acute Care Home Health Care Hypoplastic Left Heart Syndrome -- Surgery Human Male Female Infant Mothers Fathers United States Europe Descriptive Research Qualitative Studies Purposive Sample Thematic Analysis Blogs -- Evaluation Nursing Theory Parental Attitudes Quality of Health Care Patient Discharge Readmission Parent-Child Relations Funding Source N1 - research; tables/charts. Journal Subset: Core Nursing; Nursing; Peer Reviewed; USA. Grant Information: The study was supported by University of Virginia.. NLM UID: 101142025. PY - 2018 SN - 1539-0136 SP - N.PAG-N.PAG ST - Transition experiences between hospital‐ and home‐care for parents of children with hypoplastic left heart syndrome T2 - Journal for Specialists in Pediatric Nursing TI - Transition experiences between hospital‐ and home‐care for parents of children with hypoplastic left heart syndrome UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=132601347&site=ehost-live&scope=site VL - 23 ID - 1535 ER - TY - JOUR AB - Objectives: We previously reported common knowledge deficits and lack of transition readiness in 13- 25-year-olds with congenital or acquired heart disease. The aims of this study were to re-evaluate transition readiness in this cohort at follow-up and to examine the relationship between changes in transition readiness and quality of life (QOL). Study design: In this prospective cohort study, patients completed the Transition Readiness Assessment and the Pediatric Quality of Life Inventory using an e-tablet, web-based format at a routine follow-up visit. Changes from initial to follow-up scores were evaluated. Results: Sixty-five percent of patients (106 of 164) completed follow-up assessments at a median age of 18.7 years (IQR, 16.5-21.2 years) at a median follow-up of 1 year. The average perceived knowledge deficit score (percent of items with no knowledge) at follow-up was 18.0 ± 15.2%, which decreased from 24.7 ± 16.5% (P < .0001). On a 100-point scale, the mean score for self-efficacy increased from 71.4 ± 17.0 to 76.7 ± 18.2 (P = .0004) and for self-management increased from 47.9 ± 18.4 to 52.0 ± 20.7 (P = .004). Although physical QOL did not change, the mean psychosocial QOL score increased significantly (P = .02). A decrease in the knowledge deficit score at follow-up was significantly associated with an increased psychosocial QOL score (P = .03). An increase in the self-efficacy score was associated with an increase in psychosocial QOL score (P = .04), especially social QOL (P = .02). Conclusions: Although deficits in knowledge and self-management skills persist, transition readiness assessment and recognition of deficits can improve transition readiness with improved psychosocial QOL. AD - K. Uzark, University of Michigan Mott Children's Hospital L2110 Women's, SPC 5202, 1500 East Medical Center Drive, Ann Arbor, MI, United States AU - Uzark, K. AU - Afton, K. AU - Yu, S. AU - Lowery, R. AU - Smith, C. AU - Norris, M. D. DB - Embase Medline DO - 10.1016/j.jpeds.2019.04.060 KW - adolescent adult article cohort analysis disease severity female follow up heart disease heart single ventricle human knowledge male Pediatric Quality of Life Inventory priority journal prospective study quality of life self care self concept social psychology young adult LA - English M3 - Article N1 - L2002081638 2019-06-13 PY - 2019 SN - 1097-6833 0022-3476 SP - 73-78 ST - Transition Readiness in Adolescents and Young Adults with Heart Disease: Can We Improve Quality of Life? T2 - Journal of Pediatrics TI - Transition Readiness in Adolescents and Young Adults with Heart Disease: Can We Improve Quality of Life? UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2002081638 http://dx.doi.org/10.1016/j.jpeds.2019.04.060 VL - 212 ID - 597 ER - TY - JOUR AB - Children and adolescents with cardiac disease (CCD) have significant morbidity and lower quality of life. However, there are no broadly applicable tools similar to the frailty score as described in the elderly, to define functional phenotype in terms of physical capability and psychosocial wellbeing in CCD. The purpose of this study is to investigate the domains of the frailty in CCD. We prospectively recruited CCD (8-17.5 years old, 70% single ventricle, 27% heart failure, 12% pulmonary hypertension; NYHA classes I, II and III) and age and gender matched healthy controls (total n = 56; CCD n = 34, controls n = 22; age 12.6 ± 2.6 years; 39.3% female). We measured the five domains of frailty: slowness, weakness, exhaustion, body composition and physical activity using developmentally appropriate methods. Age and gender-based population norms were used to obtain Z scores and percentiles for each measurement. Two-tailed t-tests were used to compare the two groups. The CCD group performed significantly worse in all five domains of frailty compared to healthy controls. Slowness: 6-min walk test with Z score -3.9 ± 1.3 vs -1.4 ± 1.3, p < 0.001; weakness: handgrip strength percentile 18.9 ± 20.9 vs 57.9 ± 26.0, p < 0.001; exhaustion: multidimensional fatigue scale percentile 63.7 ± 13.5 vs 83.3 ± 14.4, p < 0.001; body composition: height percentile 43.4 ± 29.5 vs 71.4 ± 25.2, p < 0.001, weight percentile 46.0 ± 36.0 vs 70.9 ± 24.3, p = 0.006, BMI percentile 48.4 ± 35.5 vs 66.9 ± 24.2, p = 0.04, triceps skinfold thickness 41.0 ± 24.0 vs 54.4 ± 22.1, p = 0.04; physical activity: pediatric activity questionnaire score 2 ± 0.6 vs 2.7 ± 0.6, p < 0.001. The domains of frailty can be quantified in children using developmentally appropriate methods. CCD differ significantly from controls in all five domains, supporting the concept of quantifying the domains of frailty. Larger longitudinal studies are needed to study frailty in CCD and examine if it predicts adverse health outcomes.Clinical Trial Registration: The ClinicalTrials.gov identification number is NCT02999438. https://clinicaltrials.gov/ct2/show/NCT02999438. AD - Department of Child Health, University of Missouri Health Care, Columbia, MO, USA. panchangams@health.missouri.edu. University of Missouri-Columbia, 500 N Keene St, Suite 207, Columbia, MO, 65201, USA. panchangams@health.missouri.edu. The Ward Family Heart Center, Children's Mercy Hospital, Kansas City, MO, USA. UMKC School of Medicine, Kansas City, MO, USA. AN - 32377892 AU - Panchangam, C. AU - White, D. A. AU - Goudar, S. AU - Birnbaum, B. AU - Malloy-Walton, L. AU - Gross-Toalson, J. AU - Reid, K. J. AU - Shirali, G. AU - Parthiban, A. C2 - PMC7223568 DA - May 6 DB - PubMed DO - 10.1007/s00246-020-02354-7 DP - NLM ET - 2020/05/08 KW - Cardiomyopathy Children with heart disease Congenital heart disease Fontan Frailty Heart failure Pulmonary hypertension Quality and outcomes LA - eng N1 - 1432-1971 Panchangam, Chaitanya Orcid: 0000-0001-6515-0526 White, David A Goudar, Suma Birnbaum, Brian Malloy-Walton, Lindsey Gross-Toalson, Jami Reid, Kimberly J Shirali, Girish Parthiban, Anitha Journal Article Pediatr Cardiol. 2020 May 6:1-11. doi: 10.1007/s00246-020-02354-7. PY - 2020 SN - 0172-0643 (Print) 0172-0643 SP - 1-11 ST - Translation of the Frailty Paradigm from Older Adults to Children with Cardiac Disease T2 - Pediatr Cardiol TI - Translation of the Frailty Paradigm from Older Adults to Children with Cardiac Disease ID - 382 ER - TY - JOUR AB - Transparency-sharing data or information about outcomes, processes, protocols, and practices-may be the most powerful driver of health care improvement. In this special article, the development and growth of transparency within the National Pediatric Cardiology Quality Improvement Collaborative is described. The National Pediatric Cardiology Quality Improvement Collaborative transparency journey is guided by equal numbers of clinicians and parents of children with congenital heart disease working together in a Transparency Work Group. Activities are organized around four interrelated levels of transparency (individual, organizational, collaborative, and system), each with a specified purpose and aim. A number of Transparency Work Group recommendations have been operationalized. Aggregate collaborative performance is now reported on the public-facing web site. Specific information that the Transparency Work Group recommends centers provide to parents has been developed and published. Almost half of National Pediatric Cardiology Quality Improvement Collaborative centers participated in a pilot of transparently sharing their outcomes achieved with one another. Individual centers have also begun successfully implementing recommended transparency activities. Despite progress, barriers to full transparency persist, including health care organization concerns about potential negative effects of disclosure on reputation and finances, and lack of reliable definitions, data, and reporting standards for fair comparisons of centers. The National Pediatric Cardiology Quality Improvement Collaborative's transparency efforts have been a journey that continues, not a single goal or destination. Balanced participation of clinicians and parents has been a critical element of the collaborative's success on this issue. Plans are in place to guide implementation of additional transparency recommendations across all four levels, including extension of the activities beyond the collaborative to support transparency efforts in national cardiology and cardiac surgery societies. AD - Sisters by Heart. Division of Cardiology, Children's Hospital & Medical Center, Omaha, Neb, USA. Independent Consultant, Boston, Mass, USA. James M. Anderson Center for Health Systems Excellence, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA. Department of Cardiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA. AN - 26554878 AU - Lihn, S. L. AU - Kugler, J. D. AU - Peterson, L. E. AU - Lannon, C. M. AU - Pickles, D. AU - Beekman, R. H., 3rd DA - Nov-Dec DB - PubMed DO - 10.1111/chd.12314 DP - NLM ET - 2015/11/12 IS - 6 KW - Cardiology/*standards Child Evidence-Based Medicine/*standards Heart Defects, Congenital/*therapy Humans Interdisciplinary Communication Parents/*psychology Pediatrics/*standards Quality Improvement/*standards Registries *Societies, Medical Congenital Heart Disease Hypoplastic Left Heart Syndrome Public Reporting Quality Improvement Collaborative Transparency LA - eng N1 - 1747-0803 Lihn, Stacey L Kugler, John D Peterson, Laura E Lannon, Carole M Pickles, Diane Beekman, Robert H 3rd Journal Article Review United States Congenit Heart Dis. 2015 Nov-Dec;10(6):572-80. doi: 10.1111/chd.12314. Epub 2015 Nov 11. PY - 2015 SN - 1747-079x SP - 572-80 ST - Transparency in a Pediatric Quality Improvement Collaborative: A Passionate Journey by NPC-QIC Clinicians and Parents T2 - Congenit Heart Dis TI - Transparency in a Pediatric Quality Improvement Collaborative: A Passionate Journey by NPC-QIC Clinicians and Parents VL - 10 ID - 231 ER - TY - JOUR AB - Infants with transposed great arteries need atrioseptostomy for adequate intracardiae mixing of the systemic and pulmonic circulations. Those with ventricular septal defect and pulmonary artery hypertension, with or without other associated lesions, usually require further surgical palliation. Postseptostomy measurements of pulmonary artery pressures at systemic levels ' were obtained in ten infants with transposition of the great arteries and ventricular septal defects. Three had. in addition, patent ductus artenosus. and two of these had aortic e-oarelation (one preductal with hypoplasia of the aortic arch). One had transposition of the great arteries, ventricular septal defect, and tricuspid atresia. Four of the patients had surgical septectomy; the remaining seven had balloon atnoseptostomy. All had pulmonary artery banding, the three ductu.s artenosus lesions were ligated; and both coarctations were repaired (one with reconstruction of the aortic arch). The only operative death was in one of the patients with transposition of the great arteries and ventricular septal defect only. When the interatnal communication is adequate in patients with transposition, ventricular septal defect, and pulmonary artery hypertension, pulmonary vascular obstructive changes may be prevented by pulmonary artery banding and surgical correction. AU - Waldhausen, J. A. DB - Embase Classic IS - 5 KW - trimetazidine aortic arch aortic sinus artery case report death great vessels transposition heart ventricle septum defect hypoplasia infant interpersonal communication lung lung artery lung artery banding lung artery pressure palliative therapy patent patient pulmonary artery aneurysm pulmonary hypertension surgery tricuspid valve atresia LA - English M3 - Article N1 - L290030244 1969-12-01 PY - 1969 SN - 0009-7322 SP - 297-307 ST - Transposition of the great arteries with ventricular septal defect. palliation by atrial septostomy and pulmonary artery banding T2 - Circulation TI - Transposition of the great arteries with ventricular septal defect. palliation by atrial septostomy and pulmonary artery banding UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L290030244 VL - 39 ID - 1406 ER - TY - JOUR AB - Infants with transposed great arteries need atrioseptostomy for adequate intracardiac mixing of the systemic and pulmonic circulations. Those with ventricular septal defect and pulmonary artery hypertension, with or without other associated lesions, usually require further surgical palliation. Postseptostomy measurements of pulmonary artery pressures at systemic levels were obtained in ten infants with transposition of the great arteries and ventricular septal defects. Three had, in addition, patent ductus arteriosus, and two of these had aortic coarctation (one preductal with hypoplasia of the aortic arch). One had transposition of the great arteries, ventricular septal defect, and tricuspid atresia. Four of the patients had surgical septectomy; the remaining seven had balloon atrioseptostomy. All had pulmonary artery banding; the three ductus arteriosus lesions were ligated; and both coarctations were repaired (one with reconstruction of the aortic arch). The only operative death was in one of the patients with transposition of the great arteries and ventricular septal defect only. When the interatrial communication is adequate in patients with transposition, ventricular septal defect, and pulmonary artery hypertension, pulmonary vascular obstructive changes may be prevented by pulmonary artery banding and surgical correction of the associated lesions. AD - J.A. Waldhausen, Dept. of Surg., Univ. of Pennsylvania Sch. of Med. AU - Waldhausen, J. A. AU - Boruchow, I. AU - Miller, W. W. AU - Rashkind, W. J. DB - Embase Classic Medline IS - 5 KW - trimetazidine aortic arch aortic coarctation artery artery ligation cardiovascular surgery death ductus arteriosus great vessels transposition heart septum defect heart ventricle septum defect hypoplasia infant interpersonal communication intracardiac drug administration lung artery lung artery banding lung artery pressure palliative therapy patent ductus arteriosus patient pulmonary hypertension surgery tricuspid valve atresia LA - English M3 - Article N1 - L290061480 1969-12-01 PY - 1969 SN - 0009-7322 SP - 215-221 ST - Transposition of the great arteries with ventricular septal defect. palliation by atrial septostomy and pulmonary artery banding T2 - Circulation TI - Transposition of the great arteries with ventricular septal defect. palliation by atrial septostomy and pulmonary artery banding UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L290061480 VL - 39 ID - 1407 ER - TY - JOUR AB - Infants with hypoplastic left heart syndrome and intact or highly restrictive atrial septum require emergent therapy to relieve pulmonary congestion. Transcatheter stenting has become, in most large centers, the mainstay of therapy for relief of left atrial hypertension. Normally, this procedure is performed with the assistance of transesophageal echocardiographic guidance. However, the transesophageal approach is untenable in neonates of low birth weight, as the transducers can be traumatic and can cause ventilatory difficulty. We present two cases in which transthoracic echocardiographic guidance was used to guide atrial perforation and stenting in low birth-weight neonates with hypoplastic left heart syndrome with intact or highly restrictive atrial septum. Both procedures were straightforward and there were no complications. Real-time biplane imaging greatly assisted in the intervention in one case. The advantages of this approach are discussed. © 2010 Wiley-Liss, Inc. AD - Lilllie Frank Abercrombie Section of Cardiology, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, United States AU - Petit, C. J. AU - Altman, C. A. AU - Pignatelli, R. H. DB - Scopus DO - 10.1002/ccd.22549 IS - 3 KW - Intracardiac echo Pediatric interventions Transeptal cath M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2010 SP - 425-430 ST - Transthoracic echocardiographic assistance for interatrial stenting in low birth-weight neonates with hypoplastic left heart syndrome and intact atrial septum T2 - Catheterization and Cardiovascular Interventions TI - Transthoracic echocardiographic assistance for interatrial stenting in low birth-weight neonates with hypoplastic left heart syndrome and intact atrial septum UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-77956791799&doi=10.1002%2fccd.22549&partnerID=40&md5=bbff80045c1a478e3feef7e1aa074937 VL - 76 ID - 2145 ER - TY - JOUR AB - Decompression of the left heart in patients supported with extracorporeal membrane oxygenation (ECMO) is often warranted to protect the myocardium and facilitate recovery. We studied the ability of standard echocardiographic parameters to predict left atrial hypertension by reviewing 3 cardiac patients supported with ECMO who subsequently underwent left atrial decompression. We found that standard echocardiographic parameters poorly predict the need for left atrial decompression on ECMO. Following a more specific diagnostic algorithm to estimate left-sided filling pressure in patients with depressed ejection fraction may significantly improve the ability of echocardiography to accurately predict left atrial hypertension and the need for decompression. © The Author(s) 2014. AD - A. W. Eckhauser, Department of Surgery, Division of Pediatric Cardiothoracic Surgery, University of Utah, Primary Children's Medical Center, 100N. Mario Capecchi Dr, Salt Lake City, UT 84113, United States AU - Eckhauser, A. W. AU - Jones, C. AU - Witte, M. K. AU - Puchalski, M. D. DB - Embase Medline DO - 10.1177/2150135113508291 IS - 1 KW - article case report child decompression surgery decompressive craniectomy extracorporeal oxygenation female heart arrest heart arrhythmia heart block heart catheterization heart graft heart muscle biopsy heart ventricle pressure heart ventricle tachycardia human Human parvovirus B19 human tissue hypertension hypoplastic left heart syndrome left atrial hypertension lung edema lung hemorrhage male mitral valve regurgitation mydriasis myocarditis polymerase chain reaction preschool child priority journal respiratory failure retrospective study school child septostomy balloon catheter thorax radiography transthoracic echocardiography LA - English M3 - Article N1 - L373179043 2014-06-06 2020-04-07 PY - 2014 SN - 2150-136X 2150-1351 SP - 67-69 ST - Transthoracic echocardiographic predictors of left atrial hypertension in patients on venoarterial extracorporeal membrane oxygenation T2 - World Journal for Pediatric and Congenital Hearth Surgery TI - Transthoracic echocardiographic predictors of left atrial hypertension in patients on venoarterial extracorporeal membrane oxygenation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L373179043 http://dx.doi.org/10.1177/2150135113508291 VL - 5 ID - 930 ER - TY - JOUR AB - A study of 6,690 transthoracic intracardiac monitoring (TIM) catheters in 5,666 pediatrics patients undergoing cardiac surgical procedures during a 10-year period demonstrates the safety of this monitoring method. The utility of TIM catheters in patients undergoing a modified Fontan procedure, those undergoing repair of tetralogy of Fallot, and those with elevated or labile pulmonary resistance is stressed. Right atrial (RA) and left atrial (LA) catheters are particularly safe; they have a low incidence of bleeding complications (RA = 0%, LA = 0.13%) and retention complications (RA = 0.15%, LA = 0.63%). Pulmonary artery (PA) catheters that are introduced through the right ventricular infundibulum have higher complication rates (1.07%); unpredictable hemodynamic compromise occurs in approximately 0.5% of such patients. PA catheters placed through the wall of the atrium have a lower complication rate - approximately equal to that for RA and LA catheters. The benefits of TIM catheters far outweigh the associated risks if care is exercised in placing and removing them, and if complications are rapidly recognized and aggressively managed. The continued use of TIM catheters in pediatric cardiac surgical patients is therefore strongly recommended. AD - Department of Cardiac Surgery, Children's Hospital, Boston, MA AU - Gold, J. P. AU - Jonas, R. A. AU - Lang, P. DB - Embase Medline DO - 10.1016/S0003-4975(10)60515-2 IS - 2 KW - case report child congenital disorder congenital heart disease diagnosis heart heart surgery human monitoring pediatrics priority journal LA - English M3 - Article N1 - L16035265 1986-10-07 PY - 1986 SN - 0003-4975 SP - 185-191 ST - Transthoracic intracardiac monitoring lines in pediatric surgical patients: A ten-year experience T2 - Annals of Thoracic Surgery TI - Transthoracic intracardiac monitoring lines in pediatric surgical patients: A ten-year experience UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L16035265 http://dx.doi.org/10.1016/S0003-4975(10)60515-2 VL - 42 ID - 1393 ER - TY - JOUR AB - A study of 6,690 transthoracic intracardiac monitoring (TIM) catheters in 5,666 pediatric patients undergoing cardiac surgical procedures during a 10-year period demonstrates the safety of this monitoring method. The utility of TIM catheters in patients undergoing a modified Fontan procedure, those undergoing repair of tetralogy of Fallot, and those with elevated or labile pulmonary resistance is stressed. Right atrial (RA) and left atrial (LA) catheters are particularly safe; they have a low incidence of bleeding complications (RA = 0%, LA = 0.13%) and retention complications (RA = 0.15%, LA = 0.63%). Pulmonary artery (PA) catheters that are introduced through the right ventricular infundibulum have higher complication rates (1.07%); unpredictable hemodynamic compromise occurs in approximately 0.5% of such patients. PA catheters placed through the wall of the atrium have a lower complication rate-approximately equal to that for RA and LA catheters. The benefits of TIM catheters far outweight the associated risks if care is exercised in placing and removing them, and if complications are rapidly recognized and aggressively managed. The continued use of TIM catheters in pediatric cardiac surgical patients is therefore strongly recommended. AN - 3741014 AU - Gold, J. P. AU - Jonas, R. A. AU - Lang, P. AU - Elixson, E. M. AU - Mayer, J. E. AU - Castaneda, A. R. DA - Aug DB - PubMed DO - 10.1016/s0003-4975(10)60515-2 DP - NLM ET - 1986/08/01 IS - 2 KW - Cardiac Catheterization/adverse effects/*methods/standards *Cardiac Surgical Procedures Cardiopulmonary Bypass Child Child, Preschool Heart Arrest, Induced Heart Defects, Congenital/surgery Heart Diseases/surgery *Hemodynamics Hemorrhage/etiology Humans Infant Infant, Newborn Intraoperative Care *Monitoring, Physiologic Pulmonary Artery/physiology Risk Vascular Resistance LA - eng N1 - Gold, J P Jonas, R A Lang, P Elixson, E M Mayer, J E Castaneda, A R Journal Article Netherlands Ann Thorac Surg. 1986 Aug;42(2):185-91. doi: 10.1016/s0003-4975(10)60515-2. PY - 1986 SN - 0003-4975 (Print) 0003-4975 SP - 185-91 ST - Transthoracic intracardiac monitoring lines in pediatric surgical patients: a ten-year experience T2 - Ann Thorac Surg TI - Transthoracic intracardiac monitoring lines in pediatric surgical patients: a ten-year experience VL - 42 ID - 489 ER - TY - JOUR AB - This case illustrates the successful use of orthotopic heart transplantation for the treatment of plastic bronchitis in a 6-year-old boy with hypoplastic left heart syndrome, which developed 2 years after Fontan procedure. Transplantation was undertaken after he failed medical management of airway obstruction. He is currently 1-year post-cardiac transplantion and has no evidence of plastic bronchitis despite weaning of an aggressive airway clearance regimen. © 2011 Springer Science+Business Media, LLC. AD - Johns Hopkins Hospital, 600N Wolfe Street, Baltimore, MD 21287, United States AU - Laubisch, J. E. AU - Green, D. M. AU - Mogayzel, P. J. AU - Reid Thompson, W. DB - Scopus DO - 10.1007/s00246-011-9989-5 IS - 8 KW - Congenital heart disease Plastic bronchitis Transplantation M3 - Article N1 - Cited By :16 Export Date: 15 June 2020 PY - 2011 SP - 1193-1195 ST - Treatment of plastic bronchitis by orthotopic heart transplantation T2 - Pediatric Cardiology TI - Treatment of plastic bronchitis by orthotopic heart transplantation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-83555174672&doi=10.1007%2fs00246-011-9989-5&partnerID=40&md5=77d98e6074c205fe8c063bfc990e903d VL - 32 ID - 2110 ER - TY - JOUR AB - The congenital heart defect of HLHS is nearly uniformly fatal without intervention. As surgeons gain experience with the techniques the success rates are improving; more infants with HLHS are being offered hope for survival and quality of life. The critical nature of this diagnosis, the relatively new treatment options, and uncertainty of the surgical outcome impose tremendous stress on the parents. Choice of treatment is difficult and should be made in collaboration with the attending physician. Although the first year of my son's life was difficult, the past 5 years have been wonderful. He continues to thrive and enjoy most of the activities other 6-year-olds enjoy. In retrospect, palliative surgery was a good option for him. As both his mother and a cardiac nurse, I recognize that he may once again require surgical intervention, but I am encouraged that medical and surgical advances are being made for children with HLHS. AN - 7539730 AU - Swanson, L. T. DA - Jun DB - PubMed DP - NLM ET - 1995/06/01 IS - 3 KW - *Decision Making Humans Hypoplastic Left Heart Syndrome/*surgery Infant, Newborn Male Mothers/*psychology *Palliative Care LA - eng N1 - Swanson, L T Case Reports Journal Article United States Crit Care Nurse. 1995 Jun;15(3):70-2, 76-9. PY - 1995 SN - 0279-5442 (Print) 0279-5442 SP - 70-2, 76-9 ST - Treatment options for hypoplastic left heart syndrome: a mother's perspective T2 - Crit Care Nurse TI - Treatment options for hypoplastic left heart syndrome: a mother's perspective VL - 15 ID - 487 ER - TY - JOUR AB - Tricuspid atresia is a rare cyanotic congenital heart disease, first described by Kreysig (1817), with an incidence of 1%to 5%. It is incompatible with longevity without surgical intervention. Glenn (1958) was successful in using a superior vena cava to right pulmonary artery anastomosis. Fontan (1958) was successful in separating the right and left cardiac circulation; this is the surgical method of choice, with death of 17% by 20 years of age. The Fontan circulation has burdens of multisystem abnormalities, including ventricular dysfunction (72%), dysrhythmias (40%) and renal, hepatic, gastrointestinal and pulmonary complications. The index case is the oldest documented report of tricuspid atresia with rudimentary right ventricle and concordant ventriculo-arterial connections, 18 years post Glen only, from two years of age. She is asymptomatic, of high intellect, never used medications and is now functioning at New York Heart Association (NYHA) I-II at 20 years of age. Her survival and function was secondary to an innate large atrial and ventricular communication with normal left ventricle, mitral, aortic and pulmonary valves, indicating Fontan may not be needed in all structural anatomic cases of tricuspid atresia. AD - S. Williams-Phillips, Andrews Memorial Hospital, TAI Wing, 27 Hope Road, Kingston 10, West Indies, Jamaica AU - Williams-Phillips, S. DB - Embase DO - 10.7727/wimj.2014.272 IS - 3 KW - hemoglobin adult article body dysmorphic disorder case report cyanosis digital clubbing electrocardiography female Fontan procedure Glenn shunt hemoglobin blood level human longevity medical decision making New York Heart Association class pulmonary artery rare disease superior cava vein transthoracic echocardiography tricuspid valve atresia urinalysis young adult LA - English M3 - Article N1 - L607844085 2016-02-03 2016-02-11 PY - 2015 SN - 0043-3144 SP - 294-297 ST - Tricuspid atresia 18 years post Glenn: Is Fontan necessary in all cases? T2 - West Indian Medical Journal TI - Tricuspid atresia 18 years post Glenn: Is Fontan necessary in all cases? UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L607844085 http://dx.doi.org/10.7727/wimj.2014.272 VL - 64 ID - 896 ER - TY - JOUR AB - Rubinstein-Taybi syndrome is a well-characterized condition causing distinctive physical characteristics, intellectual disability, and multiple congenital malformations. Cardiac abnormalities are found in a third of individuals with this condition and usually consist of isolated septal defects or patent ductus arteriosus, although more complex congenital lesions have been described. We present the first reported case of tricuspid atresia and pulmonary atresia with hypoplasia of the right ventricle in the setting of Rubenstein-Taybi syndrome. AD - Department of Pediatrics, Children's Research Institute, Milwaukee, WI, United States Human and Molecular Genetics Center, Medical College of Wisconsin, Milwaukee, WI, United States AU - Loomba, R. S. AU - Geddes, G. DB - Scopus DO - 10.4103/0974-2069.154151 IS - 2 KW - Hypoplastic right ventricle Rubinstein-Taybi syndrome tricuspid atresia M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2015 SP - 157-160 ST - Tricuspid atresia and pulmonary atresia in a child with Rubinstein-Taybi syndrome T2 - Annals of Pediatric Cardiology TI - Tricuspid atresia and pulmonary atresia in a child with Rubinstein-Taybi syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84930362024&doi=10.4103%2f0974-2069.154151&partnerID=40&md5=cd92184b112b754964eff3b49cb3d308 VL - 8 ID - 1972 ER - TY - JOUR AB - Twenty nine patients aged 15-35 years (mean 23) with tricuspid atresia and normally related great arteries were studied; 20 are alive. Ten patients who had had a Fontan operation (group 1) were compared with 10 patients with palliative shunts (9) or no surgery (1) (group 2). Patients were graded according to their ability to lead a normal life (ability index). Patients in group 1 tended to have a better ability index, a greater exercise capacity, and fewer social and extracardiac problems than those in group 2. The mean left ventricular ejection fraction measured by radionuclide angiography was the same in both groups. Arrhythmias were equally common in both groups; they appear to be age related and they occur independently of left ventricular function. AN - 3801245 AU - Warnes, C. A. AU - Somerville, J. C2 - PMC1216401 DA - Dec DB - PubMed DO - 10.1136/hrt.56.6.535 DP - NLM ET - 1986/12/01 IS - 6 KW - Adolescent Adult Arrhythmias, Cardiac/etiology Female Heart Valve Diseases/physiopathology/surgery Humans Male Postoperative Complications Quality of Life Reoperation Stroke Volume Tricuspid Valve/*abnormalities/surgery LA - eng N1 - Warnes, C A Somerville, J Journal Article Research Support, Non-U.S. Gov't Br Heart J. 1986 Dec;56(6):535-43. doi: 10.1136/hrt.56.6.535. PY - 1986 SN - 0007-0769 (Print) 0007-0769 SP - 535-43 ST - Tricuspid atresia in adolescents and adults: current state and late complications T2 - Br Heart J TI - Tricuspid atresia in adolescents and adults: current state and late complications VL - 56 ID - 449 ER - TY - JOUR AB - A case of tricuspid atresia with pulmonic stenosis, ventricular septal defect, and normally related great vessels with dextroversion was correctly diagnosed. The patient was treated successfully with a Glenn operation. The association of this complex of congenital defects with dextroversion has not been stressed adequately in the literature. It is emphasized that, in cases of dextroversion with cyanosis, tricuspid atresia should always be suspected. AD - King Paul Hosp., Athens AU - Cokkinos, D. V. AU - Plessas, S. T. AU - Tolis, G. AU - Voridis, E. M. DB - Embase Medline IS - 2 KW - child congenital heart malformation cyanosis dextrocardia diagnosis electrocardiography heart surgery heart ventricle septum defect hemoglobin rahere major clinical study pulmonary valve stenosis tricuspid valve tricuspid valve atresia LA - English M3 - Article N1 - L5080442 1975-01-01 PY - 1974 SN - 0022-5223 SP - 268-272 ST - Tricuspid atresia with dextroversion: a not very rare combination T2 - Journal of Thoracic and Cardiovascular Surgery TI - Tricuspid atresia with dextroversion: a not very rare combination UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L5080442 VL - 68 ID - 1401 ER - TY - JOUR AB - It is pointed out that tricuspid atresia is the Cinderella of the surgery of congenital heart disease. This is because it is one element in a whole range of abnormalities, some so complex as to be untreatable. Operation has consisted of a shunt procedure - an excellent palliative in very ill children; the immediate results have been reasonably good, but the late results are less so. In one group the essential lesion is atresia of the inflow tract of the right ventricle but with adequate development of the outflow tract and of the pulmonary artery. In these, an associated valvar or infundibular stenosis can be relieved with great improvement. A more important lesion, however, is stenosis (or smallness) of the ventricular septal defect through which all the blood to the lungs must pass. A logical treatment is deliberate enlargement of the defect by an open operation. An example of such an operation is reported. In this case the condition of the right ventricle prevented total cure which would have involved closure of the atrial and ventricular communications and insertion of a valve or of a prosthesis between the right atrium and right ventricle. This could be a feasible procedure for which surgeons should be alert. AD - R. Brock, Guy's Hosp., London, United Kingdom AU - Brock, R. DB - Embase Classic Medline IS - 1 KW - atresia blood child congenital heart disease heart right atrium heart right ventricle heart ventricle septum defect interpersonal communication lung prosthesis pulmonary artery stenosis surgeon surgery Trichomonas tricuspid valve atresia LA - English M3 - Article N1 - L281097351 1964-12-01 PY - 1964 SN - 0022-5223 SP - 17-25 ST - Tricuspid atresia: A step toward corrective treatment T2 - Journal of Thoracic and Cardiovascular Surgery TI - Tricuspid atresia: A step toward corrective treatment UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L281097351 VL - 47 ID - 1413 ER - TY - JOUR AD - I.E. Konstantinov, Department of Cardiac Surgery, The Royal Children's Hospital, 50 Flemington Rd, Parkville, Victoria, Australia AU - Perrier, S. L. AU - Zhu, M. Z. L. AU - Weintraub, R. G. AU - Konstantinov, I. E. DB - Embase Medline DO - 10.1016/j.jtcvs.2018.03.097 IS - 3 KW - annuloplasty ring dual chamber pacemaker politef politef implant acetylsalicylic acid diuretic agent lactic acid lisinopril oxygen arterial gas article assisted circulation atresia autism cardiopulmonary bypass case report child clinical article complete heart block echocardiography extracorporeal oxygenation Fontan procedure heart failure heart transplantation hospital discharge human hypoplastic left heart syndrome male mitral valve atresia oxygen saturation oxygen tension pH preschool child priority journal tricuspid annuloplasty tricuspid valve replacement Carpentier-Edwards Physio GORE-TEX LA - English M3 - Article N1 - L2000711911 2018-05-07 2018-08-21 PY - 2018 SN - 1097-685X 0022-5223 SP - e141-e143 ST - Tricuspid valve replacement in failing Fontan circulation with severe ventricular dysfunction: The road not taken? T2 - Journal of Thoracic and Cardiovascular Surgery TI - Tricuspid valve replacement in failing Fontan circulation with severe ventricular dysfunction: The road not taken? UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2000711911 http://dx.doi.org/10.1016/j.jtcvs.2018.03.097 VL - 156 ID - 665 ER - TY - JOUR AB - Singleton pregnancy in patients with single ventricle after the Fontan operation has been reported with significant offspring and maternal complications. We report a twin pregnancy and premature delivery, in a patient following the Fontan operation. © 2013 Wiley Periodicals, Inc. AD - The Pediatric Cardiology Department, Hadassah, Hebrew University Medical Center, Jerusalem, Israel Obstetrics and Gynecology Department, Hadassah, Hebrew University Medical Center, Jerusalem, Israel The Neonatology Unit, Shaare Zedek Medical Center, Jerusalem, Israel AU - Nir, A. AU - Elchalal, U. AU - Hammerman, C. AU - Rein, A. J. DB - Scopus DO - 10.1111/chd.12054 IS - 6 KW - Cesarean Section Fontan Pregnancy Prematurity Single Ventricle Twins M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2013 SP - E196-E198 ST - Twin pregnancy in a patient after the fontan operation: Report of a case T2 - Congenital Heart Disease TI - Twin pregnancy in a patient after the fontan operation: Report of a case UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84889564602&doi=10.1111%2fchd.12054&partnerID=40&md5=0a0c8d14f6a5d4c102f3b923126b677c VL - 8 ID - 2032 ER - TY - JOUR AB - Failing single ventricle physiology represents an ongoing challenge in mechanical assist device development, requiring pressure augmentation in the cavopulmonary circuit, reduction of systemic venous pressure, and increased cardiac output to achieve hemodynamic stabilization. To meet these requirements, we are developing a percutaneously-placed, axial flow blood pump to support ailing single ventricle physiology in adolescents and adults. We have modified the outer cage of the device to serve as both a protective and functional design component. This study examined the performance of 3 cage geometries with varying directions of filament twist using numerical simulations and hydraulic experiments. All 3 cage and pump models performed in acceptable ranges to support Fontan patients. The cage design employing filaments that are twisted in the opposite direction to the impeller blades and in the direction of the diffuser blades (against-with) demonstrated superior performance by generating a pressure rise range of 5-38 mmHg of flow rates of 0.5-6 l/min at rotational speeds of 5000-7000 rpm. The blood damage indices for all of the cages were found to be well below 2%, and the scalar stress levels were below 200 Pa. This study represents ongoing progress in the development of the impeller and cage assembly. Validation of the results will continue in experiments with blood bag evaluation as well as by particle image velocimetry measurements. AD - BioCirc Research Laboratory, Department of Mechanical and Nuclear Engineering, School of Engineering, Virginia Commonwealth University, Richmond, VA, USA. althrock@vcu.edu AN - 22661112 AU - Throckmorton, A. L. AU - Downs, E. A. AU - Hazelwood, J. A. AU - Monroe, J. O. AU - Chopski, S. G. DA - May DB - PubMed DO - 10.5301/ijao.5000105 DP - NLM ET - 2012/06/05 IS - 5 KW - Adolescent Adult Biomechanical Phenomena Blood Flow Velocity Computer Simulation Diffusion Chambers, Culture Fontan Procedure/*adverse effects Heart Defects, Congenital/physiopathology/*surgery Heart Failure/etiology/physiopathology/*therapy *Heart-Assist Devices Humans Hydrodynamics Materials Testing Models, Cardiovascular Models, Statistical Numerical Analysis, Computer-Assisted Prosthesis Design Regression Analysis Stress, Mechanical *Ventricular Function Ventricular Pressure LA - eng N1 - 1724-6040 Throckmorton, Amy L Downs, Emily A Hazelwood, John A Monroe, Jonathan O Chopski, Steven G Journal Article United States Int J Artif Organs. 2012 May;35(5):369-75. doi: 10.5301/ijao.5000105. PY - 2012 SN - 0391-3988 SP - 369-75 ST - Twisted cardiovascular cages for intravascular axial flow blood pumps to support the Fontan physiology T2 - Int J Artif Organs TI - Twisted cardiovascular cages for intravascular axial flow blood pumps to support the Fontan physiology VL - 35 ID - 454 ER - TY - JOUR AB - Failing single ventricle physiology represents an ongoing challenge in mechanical assist device development, requiring pressure augmentation in the cavopulmonary circuit, reduction of systemic venous pressure, and increased cardiac output to achieve hemodynamic stabilization. To meet these requirements, we are developing a percutaneously-placed, axial flow blood pump to support ailing single ventricle physiology in adolescents and adults. We have modified the outer cage of the device to serve as both a protective and functional design component. This study examined the performance of 3 cage geometries with varying directions of filament twist using numerical simulations and hydraulic experiments. All 3 cage and pump models performed in acceptable ranges to support Fontan patients. The cage design employing filaments that are twisted in the opposite direction to the impeller blades and in the direction of the diffuser blades (against-with) demonstrated superior performance by generating a pressure rise range of 5-38 mmHg of flow rates of 0.5-6 l/min at rotational speeds of 5000-7000 rpm. The blood damage indices for all of the cages were found to be well below 2%, and the scalar stress levels were below 200 Pa. This study represents ongoing progress in the development of the impeller and cage assembly. Validation of the results will continue in experiments with blood bag evaluation as well as by particle image velocimetry measurements. AD - BioCirc Research Laboratory, Department of Mechanical and Nuclear Engineering, School of Engineering, Virginia Commonwealth University, Richmond, VA, USA BioCirc Research Laboratory, Department of Mechanical and Nuclear Engineering, School of Engineering, Virginia Commonwealth University, Richmond, VA - USA. AN - 104459795. Language: English. Entry Date: 20121102. Revision Date: 20171209. Publication Type: journal article AU - Throckmorton, A. L. AU - Downs, E. A. AU - Hazelwood, J. A. AU - Monroe, J. O. AU - Chopski, S. G. AU - Throckmorton, Amy L. AU - Downs, Emily A. AU - Hazelwood, John A. AU - Monroe, Jonathan O. AU - Chopski, Steven G. DB - ccm DO - 10.5301/ijao.5000105 DP - EBSCOhost IS - 5 KW - Cardiopulmonary Bypass -- Adverse Effects Heart Defects, Congenital -- Surgery Heart Failure -- Therapy Heart Assist Devices Cardiovascular System Physiology Adolescence Adult Biomechanics Blood Flow Velocity Computer Simulation Cell Culture Techniques Heart Defects, Congenital -- Physiopathology Heart Failure -- Etiology Heart Failure -- Physiopathology Human Physiochemical Phenomena Materials Testing Models, Biological Models, Statistical Computing Methodologies Prosthesis Design Regression Stress, Mechanical Ventricular Pressure N1 - research. Journal Subset: Biomedical; Blind Peer Reviewed; Continental Europe; Double Blind Peer Reviewed; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed. Special Interest: Laboratory Diagnosis. NLM UID: 7802649. PMID: NLM22661112. PY - 2012 SN - 0391-3988 SP - 369-375 ST - Twisted cardiovascular cages for intravascular axial flow blood pumps to support the Fontan physiology T2 - International Journal of Artificial Organs TI - Twisted cardiovascular cages for intravascular axial flow blood pumps to support the Fontan physiology UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104459795&site=ehost-live&scope=site VL - 35 ID - 1555 ER - TY - JOUR AB - BACKGROUND: Assessment of systemic right ventricular (RV) function in patients with hypoplastic left heart syndrome is important during long-term follow-up after Fontan repair. Traditional echocardiographic parameters to evaluate systolic ventricular function are affected by loading conditions. The only generally accepted load-independent parameter of systolic function, end systolic elastance (Ees), requires invasive catheterization. Therefore, we sought to determine if parameters obtained by 2-dimensional speckle tracking (2DST) were affected by acute changes in preload and correlated with catheterization-derived indices of RV contractility in hypoplastic left heart syndrome patients after Fontan palliation. METHODS AND RESULTS: Fifty-two patients with hypoplastic left heart syndrome (median age, 6.6; range 2.9-22.2 years) were prospectively enrolled to have echocardiography and conductance catheter studies performed simultaneously. We compared traditional echo, 2-dimensional speckle tracking and catheterization-derived parameters during different states of preload at baseline and during dobutamine infusion. Global longitudinal strain (S) showed a tendency to decrease with preload reduction, whereas global longitudinal strain rate (SR) did not change (S: -17.7 ± 3.4% versus -16.9 ± 3.8%, P=0.08; SR: -1.30 ± 0.29 versus -1.34 ± 0.34 s(-1), P=0.3). S did not change with dobutamine infusion (-17.7 ± 3.4% versus -18.4 ± 3.9%, P=0.24), whereas SR increased significantly (-1.30 ± 0.29 versus -2.26 ± 0.49 s(-1), P<0.001). RV Ees correlated with SR (rs= -0.47, P<0.001), but not with S (rs=0.07, P=0.5) or other echocardiographic parameters. CONCLUSIONS: In contrast to S, SR was not affected by preload and correlated with Ees of the systemic RV. SR may be a useful noninvasive surrogate of RV contractility and suitable for follow-up of patients with hypoplastic left heart syndrome after Fontan palliation. AD - From the Department for Congenital Heart Disease and Paediatric Cardiology, University Hospital of Schleswig-Holstein, Campus Kiel, Kiel, Germany (J.S., C.P., J.H.H., M.M., C.H., G.F., K.B., H.-H.K.); Department of Pediatric, Fetal and Congenital Cardiology, Hawaii Permanente Medical Group, Kaiser Permanente Moanalua Medical Center, Honolulu, HI (C.P.); and Adult Congenital Heart Disease Centre and National Centre for Pulmonary Hypertension, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom (A.U.). jana.schlangen@pedcard.uni-kiel.de. From the Department for Congenital Heart Disease and Paediatric Cardiology, University Hospital of Schleswig-Holstein, Campus Kiel, Kiel, Germany (J.S., C.P., J.H.H., M.M., C.H., G.F., K.B., H.-H.K.); Department of Pediatric, Fetal and Congenital Cardiology, Hawaii Permanente Medical Group, Kaiser Permanente Moanalua Medical Center, Honolulu, HI (C.P.); and Adult Congenital Heart Disease Centre and National Centre for Pulmonary Hypertension, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom (A.U.). AN - 25270741 AU - Schlangen, J. AU - Petko, C. AU - Hansen, J. H. AU - Michel, M. AU - Hart, C. AU - Uebing, A. AU - Fischer, G. AU - Becker, K. AU - Kramer, H. H. DA - Nov DB - PubMed DO - 10.1161/circimaging.114.002110 DP - NLM ET - 2014/10/02 IS - 6 KW - Adolescent Cardiac Catheterization Child Child, Preschool Echocardiography, Stress Female *Fontan Procedure Humans Hypoplastic Left Heart Syndrome/diagnosis/physiopathology/*surgery Male *Myocardial Contraction Observer Variation Palliative Care Predictive Value of Tests Prospective Studies Reproducibility of Results Treatment Outcome *Ventricular Function, Right Young Adult hypoplastic left heart syndrome ventricular function LA - eng N1 - 1942-0080 Schlangen, Jana Petko, Colin Hansen, Jan H Michel, Miriam Hart, Christopher Uebing, Anselm Fischer, Gunther Becker, Kolja Kramer, Hans-Heiner Comparative Study Journal Article Research Support, Non-U.S. Gov't United States Circ Cardiovasc Imaging. 2014 Nov;7(6):880-6. doi: 10.1161/CIRCIMAGING.114.002110. Epub 2014 Sep 30. PY - 2014 SN - 1941-9651 SP - 880-6 ST - Two-dimensional global longitudinal strain rate is a preload independent index of systemic right ventricular contractility in hypoplastic left heart syndrome patients after Fontan operation T2 - Circ Cardiovasc Imaging TI - Two-dimensional global longitudinal strain rate is a preload independent index of systemic right ventricular contractility in hypoplastic left heart syndrome patients after Fontan operation VL - 7 ID - 226 ER - TY - JOUR AB - Critical aortic valve stenosis (CAS) in the newborn is treated by balloon or surgical aortic valve intervention with nearly equal success, but the subset of patients with severe left ventricular (LV) dilation and dysfunction present a significant mortality risk. We describe a two-stage surgical management approach for those infants who represent an unusually high failure risk for either aortic valvotomy or conventional stage 1 single ventricle (Norwood) palliation because of severe LV dysfunction at the time of presentation. A two-stage surgical palliation was undertaken consisting of surgical aortic valvotomy, bilateral pulmonary artery banding and atrial septectomy (stage 1), followed by patch closure of the atrial septal defect, ligation of the ductus arteriosus and removal of the pulmonary artery bands (stage 2) with prostaglandin infusion continued between stages to maintain right ventricular contribution to systemic perfusion via the ductus arteriosus. Four neonates with CAS and severely depressed LV systolic function were treated using this strategy. LV dilation resolved and systolic function improved in three patients after 2, 2 and 4 weeks, enabling stage 2. LV dysfunction did not improve in one patient who expired before conversion to biventricular circulation. Of the three who proceeded to stage 2, one infant continued to have poor biventricular diastolic function that precluded conversion, and this patient also died. The remaining two infants are now alive and well at 34 and 44 months of age. These two had the most severe LV dilation (internal dimension Z-scores of 6.9 and 7.7) and the worst systolic function (fractional shortening 4 and 10%) at presentation, and both were born prematurely (32 and 35 weeks). A two-stage surgical approach may improve the likelihood of survival in selected patients with CAS presenting with severely depressed LV systolic function. Relief of LV distention may have contributed to the improvement of LV function in these infants. AD - J.M. Hammel, Division of Cardiovascular Surgery, University of Nebraska Medical Center College of Medicine/Creighton University School of Medicine, Children's Hospital and Medical Center, Omaha, USA. AU - Hammel, J. M. AU - Duncan, K. F. AU - Danford, D. A. AU - Kutty, S. DB - Medline DO - 10.1093/ejcts/ezs197 IS - 1 KW - aortic valve aortic valve stenosis article heart left ventricle function heart surgery human infant methodology newborn pathophysiology physiology pulmonary artery retrospective study LA - English M3 - Article N1 - L366418229 2013-09-24 PY - 2013 SN - 1873-734X SP - 143-148 ST - Two-stage biventricular rehabilitation for critical aortic stenosis with severe left ventricular dysfunction T2 - European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery TI - Two-stage biventricular rehabilitation for critical aortic stenosis with severe left ventricular dysfunction UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L366418229 http://dx.doi.org/10.1093/ejcts/ezs197 VL - 43 ID - 1024 ER - TY - JOUR AB - Background - The Norwood procedure for stage 1 palliation of hypoplastic left heart syndrome is performed with either the modified Blalock-Taussig (MBTS) or the right ventricle-to-pulmonary artery (RVPA) shunt. In our institution, surgical practice changed from use of the MBTS to use of the RVPA shunt in 2002. We analyzed survival and mental and psychomotor outcomes of the 2 consecutive surgical eras. Methods and Results - Between September 1996 and July 2005, 94 neonates with hypoplastic left heart syndrome underwent the Norwood procedure. Patients were recruited as neonates and followed up prospectively. Health, mental, and psychomotor outcomes (Bayley Scales of Infant Development-II) were assessed at 2 years. The study subjects were from the Norwood-MBTS era (n=62; 1996 to 2002) or the Norwood-RVPA era (n=32; 2002 to 2005). In the MBTS era, early and 2-year mortality rates were 23% (14/62) and 52% (32/62); the mean (SD) mental and psychomotor developmental indices were 79 (18) and 67 (19). In the RVPA era, early and 2-year mortality rates were 6% (2/32) and 19% (6/32); the mean (SD) mental and psychomotor developmental indices were 85 (18) and 78 (18). The 2-year mortality rate (P=0.002) and the psychomotor developmental index (P=0.029) were improved in the more recent surgical era. On multivariable Cox regression analysis, postoperative highest serum lactate independently predicted 2-year mortality in the MBTS and RVPA eras. Conclusions - Analysis of 2 consecutive surgical eras of hypoplastic left heart syndrome patients undergoing the Norwood procedure showed a significant improvement in 2-year survival and psychomotor development in the more recent era. Adverse neurodevelopmental outcome in this patient population remains a concern. © 2008 American Heart Association. All rights reserved. AD - A. R. Joffe, Department of Pediatrics, 3A3.07 Stollery Children's Hospital, 8440 112 St., Edmonton, AB T6G 2B7, Canada AU - Atallah, J. AU - Dinu, I. A. AU - Joffe, A. R. AU - Robertson, C. M. T. AU - Sauve, R. S. AU - Dyck, J. D. AU - Ross, D. B. AU - Rebeyka, I. M. DB - Embase Medline DO - 10.1161/CIRCULATIONAHA.107.741579 IS - 14 KW - lactic acid article Blalock Taussig shunt child clinical trial controlled clinical trial controlled study female follow up human hypoplastic left heart syndrome lactate blood level major clinical study male mental development mental function mortality nerve cell differentiation Norwood procedure outcome assessment predictive validity preschool child priority journal proportional hazards model prospective study psychomotor activity psychomotor development right ventricle to pulmonary artery conduit surgical technique survival time treatment outcome LA - English M3 - Article N1 - L354748080 2009-11-11 PY - 2008 SN - 0009-7322 1524-4539 SP - 1410-1418 ST - Two-year survival and mental and psychomotor outcomes after the Norwood procedure: An analysis of the modified Blalock-Taussig shunt and right ventricle-to-pulmonary artery shunt surgical eras T2 - Circulation TI - Two-year survival and mental and psychomotor outcomes after the Norwood procedure: An analysis of the modified Blalock-Taussig shunt and right ventricle-to-pulmonary artery shunt surgical eras UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L354748080 http://dx.doi.org/10.1161/CIRCULATIONAHA.107.741579 VL - 118 ID - 1198 ER - TY - JOUR AB - OBJECTIVE: Comprehensive outcome assessment of children receiving cardiac extracorporeal life support. METHODS: From 2000 to 2004, 39 consecutive children (aged 1 day to 4.4 years) had cardiac extracorporeal life support. Neurodevelopmental follow-up of all survivors was performed more than 6 months after life support (aged 53 +/- 12 months). Developmental delay was defined as a score of less than 70 on the Bayley Scales of Infant Development II or Wechsler Preschool and Primary Scale of Intelligence. Predictor variables for mortality (at 2 years' follow-up) and delay were examined by univariate and multivariate analyses. RESULTS: Indications for extracorporeal life support were progressive low cardiac output in 14 (36%), failed weaning from cardiopulmonary bypass in 13 (33%), cardiac arrest in 9 (23%), and hypoxia in 3 (8%). Cardiac anatomy was single ventricle in 16 (41%), biventricular in 21 (54%), and myocarditis in 2 (5%). Survival was 18 (46%) at hospital discharge and 16 (41%) at 2 years. In survivors, mental score was 73 +/- 16 (normal 100 +/- 15), and 8 (50%) had mental delay. Initiating extracorporeal life support during cardiopulmonary resuscitation and duration of this resuscitation were not associated with death or mental delay. On multivariable Cox regression, lactate on admission to the pediatric intensive care unit (hazard rate 1.13; 95% confidence intervals 1.08-1.27) and single ventricle anatomy (hazard rate 3.93; 95% confidence intervals 1.62-9.49) were associated with death at 2 years. Stepwise multiple regression found time for lactate to normalize on extracorporeal life support, highest inotrope score during 120 hours of life support, and chromosomal abnormality explained 76.7% of the variance in mental score. CONCLUSION: Cardiac extracorporeal life support had a 41% 2-year survival. Potentially modifiable variables (time for lactate to normalize and highest inotrope score early during extracorporeal life support) explained 69% of mental score variance. AD - Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada. AN - 18954639 AU - Lequier, L. AU - Joffe, A. R. AU - Robertson, C. M. AU - Dinu, I. A. AU - Wongswadiwat, Y. AU - Anton, N. R. AU - Ross, D. B. AU - Rebeyka, I. M. DA - Oct DB - PubMed DO - 10.1016/j.jtcvs.2008.02.009 DP - NLM ET - 2008/10/29 IS - 4 KW - Age Factors Analysis of Variance Cardiac Surgical Procedures/methods/mortality Cardiopulmonary Bypass/adverse effects/methods Cardiopulmonary Resuscitation/methods/mortality *Cause of Death Child, Preschool Cohort Studies Confidence Intervals Developmental Disabilities/*epidemiology/etiology Extracorporeal Membrane Oxygenation/methods/*mortality Female Heart Arrest/mortality/therapy Heart Defects, Congenital/diagnosis/*mortality/surgery Humans Infant Infant, Newborn Life Support Care/methods Male Mental Disorders/*epidemiology/etiology Motor Skills Disorders/*epidemiology/etiology Multivariate Analysis Palliative Care/methods Postoperative Complications/diagnosis/therapy Proportional Hazards Models Regression Analysis Retrospective Studies Sensitivity and Specificity Survival Analysis Time Factors Treatment Outcome LA - eng N1 - 1097-685x Lequier, Laurance Joffe, Ari R Robertson, Charlene M T Dinu, Irina A Wongswadiwat, Yuttapong Anton, Natalie R Ross, David B Rebeyka, Ivan M Western Canadian Complex Pediatric Therapies Program Follow-up Group Journal Article Research Support, Non-U.S. Gov't United States J Thorac Cardiovasc Surg. 2008 Oct;136(4):976-983.e3. doi: 10.1016/j.jtcvs.2008.02.009. Epub 2008 Jun 2. PY - 2008 SN - 0022-5223 SP - 976-983.e3 ST - Two-year survival, mental, and motor outcomes after cardiac extracorporeal life support at less than five years of age T2 - J Thorac Cardiovasc Surg TI - Two-year survival, mental, and motor outcomes after cardiac extracorporeal life support at less than five years of age VL - 136 ID - 61 ER - TY - JOUR AB - AIMS: We sought to evaluate the efficacy of ultrasound contrast (UC) and low mechanical index real-time perfusion (RTP) in the haemodynamic and anatomic assessment of repaired congenital heart disease (CHD) at rest and during supine bicycle stress echocardiography (BSE). METHODS AND RESULTS: Patients with CHD (n = 51, median age 21.5 years) were prospectively studied. All had compromised image quality, 20 (39%) had arrhythmias, and 10 (20%) had pacemakers. RTP was performed at rest and during BSE using Definity and Contrast Pulse Sequencing, with assessment of Doppler pressure gradients. Diagnoses included tetralogy of Fallot (n = 27), transposition of the great arteries (TGA) atrial switch (n = 10), TGA arterial switch (n = 2), aortic valve disease (n = 4), Fontan (n = 4), and Kawasaki disease (n = 4). UC with RTP improved endocardial border definition, with increased number of left ventricular (LV) and right ventricular (RV) segments visualized at rest (P < 0.0001) and during stress. LV ejection fraction (EF) and RV fractional area change (FAC) were measurable at rest and peak stress, RV FAC correlating closely with same-day magnetic resonance EFs (r = 0.72; P < 0.001). UC enhanced Doppler signals, enabling subpulmonary ventricular systolic pressure measurements at rest and stress. In six patients, marked elevations of subpulmonary ventricular systolic pressure were detected with UC during BSE, and quantifiable ventricular dysfunction. No adverse events occurred, other than transient low back pain in one patient. CONCLUSION: UC at rest and with supine BSE enables safe and comprehensive assessment of anatomy, haemodynamics, and biventricular functional and perfusion reserve in adolescents and young adults with surgically modified CHD. AD - Joint Division of Pediatric Cardiology, University of Nebraska College of Medicine/Creighton University, Children's Hospital and Medical Center, Omaha, NE, USA. AN - 22173935 AU - Kutty, S. AU - Olson, J. AU - Danford, C. J. AU - Sandene, E. K. AU - Xie, F. AU - Fletcher, S. E. AU - Erickson, C. C. AU - Kugler, J. D. AU - Danford, D. A. AU - Porter, T. R. DA - Jun DB - PubMed DO - 10.1093/ejechocard/jer287 DP - NLM ET - 2011/12/17 IS - 6 KW - Adolescent Adult Contrast Media Echocardiography, Stress/*methods Electrocardiography Female Fluorocarbons Gadolinium DTPA Heart Defects, Congenital/diagnostic imaging/*physiopathology/surgery Hemodynamics Humans Image Interpretation, Computer-Assisted/methods Linear Models Magnetic Resonance Imaging/*methods Male Nebraska Prospective Studies Statistics, Nonparametric Supine Position LA - eng N1 - 2047-2412 Kutty, Shelby Olson, Joan Danford, Christopher J Sandene, Erin K Xie, Feng Fletcher, Scott E Erickson, Christopher C Kugler, John D Danford, David A Porter, Thomas R Journal Article Research Support, Non-U.S. Gov't England Eur Heart J Cardiovasc Imaging. 2012 Jun;13(6):500-9. doi: 10.1093/ejechocard/jer287. Epub 2011 Dec 15. PY - 2012 SN - 2047-2404 SP - 500-9 ST - Ultrasound contrast and real-time perfusion in conjunction with supine bicycle stress echocardiography for comprehensive evaluation of surgically corrected congenital heart disease T2 - Eur Heart J Cardiovasc Imaging TI - Ultrasound contrast and real-time perfusion in conjunction with supine bicycle stress echocardiography for comprehensive evaluation of surgically corrected congenital heart disease VL - 13 ID - 530 ER - TY - JOUR AB - Objective: The purpose of this study was to evaluate the role of CT angiography in different encountered congenital vascular anomalies of pediatric age groups using 64 multislice CT scanner. Subjective and methods: Through one year, a total of 15 patients having congenital extracardiac anomalies were encountered from those attending big trauma and emergency center and were studied in this work. Each patient's parent was thoroughly asked about the detailed clinical history after reviewing the referring imaging request and laboratory findings. Exclusion criteria for CT were as follows: previous allergic reactions to iodine contrast media and severe renal insufficiency. CT examination was performed using 64 multislice CT machine. Results: CTA examination was performed in a total of 15 patients. The age of the patients was ranging from 2 days to 14 yrs and 5 cases were females. Four cases came with cyanosis; one case came with follow up after operation for transposition of great arteries, while the remaining cases were suffering from medical problems such as respiratory distress or hypertension. Aortic arch hypoplasia, right sided aortic arch with mirror image or with aberrant left subclavian artery, hypoplastic left pulmonary artery with left sided aortic arch and aberrant right subclavian artery, hypoplastic left heart chambers, retrotracheal left pulmonary artery with patent ductus arteriosus (PDA), right superior partial anomalous pulmonary venous drainage (PAPVD) with ASD sinus venosum, postoperative assessment of transposition of great arteries (TGA), fibromuscular dysplasia involving the renal and common iliac arteries, and bilateral double renal arteries with ectopic right kidney were the different extracardiac vascular anomalies encountered in this work. Conclusion: CTA provides an excellent means to detect a number of extracardiac vascular anomalies and allows accurate and fast noninvasive characterization of extracardiac vascular anatomy. It is a helpful tool in establishing the primary diagnosis, defining anatomic landmarks and relationships, identifying vascular anomalies and helping in postoperative follow-up. © 2011 Egyptian Society of Radiology and Nuclear Medicine. Production and hosting by Elsevier B.V. All rights reserved. AD - Radiology Department, Faculty of Medicine, Al Azher University, Egypt AU - El Kheshen, A. M. M. DB - Scopus DO - 10.1016/j.ejrnm.2011.10.005 IS - 1 KW - Anomalies Aortic Cardiac CTA Vascular M3 - Article N1 - Export Date: 15 June 2020 PY - 2012 SP - 1-9 ST - Uncommon congenital extracardiac vascular anomalies detected on MSCT (Multi-Slice Computed Tomography) aortic angiography with 64-multislice technology T2 - Egyptian Journal of Radiology and Nuclear Medicine TI - Uncommon congenital extracardiac vascular anomalies detected on MSCT (Multi-Slice Computed Tomography) aortic angiography with 64-multislice technology UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84865763397&doi=10.1016%2fj.ejrnm.2011.10.005&partnerID=40&md5=2eeac2fa05fc203970e3e393623586dc VL - 43 ID - 2102 ER - TY - JOUR AB - OBJECTIVES: Heart disease accounts for a significant proportion of sudden unexpected deaths among children. We describe here demographic features, pathological conditions, and the frequency of premonitory symptoms in a retrospective series of cases of sudden unexpected cardiac death (SUCD) attributable to undiagnosed structural heart disease. METHODS: A chart review of autopsies involving children 0 to 17 years of age that were performed at the Hospital for Sick Children (Toronto, Ontario, Canada) between 1984 and 2003 was conducted. Cases of sudden unexpected death within 24 hours after clinical presentation with previously undetected fatal heart disease were included. Cases with multiple or thoracic trauma and chronic or multisystem disease were excluded. RESULTS: During the 20-year study period, 4926 autopsies were performed. A total of 103 cases (2.1%), involving 51 male patients and 52 female patients 1 day to 15 years of age (mean: 2.9 ± 4.2 years), were diagnosed as having SUCD. The most common diagnoses were myocarditis (n = 37 [35.9%]), hypoplastic left heart syndrome (HLHS) (n = 19 [18.4%]), dilated cardiomyopathy (DCM) (n = 16 [16.5%]), coronary artery anomalies (n = 6 [5.8%]), and aortic stenosis (n = 5 [4.9%]). There was a significant difference in the mean age of presentation between leading causes of SUCD (6.5 days for HLHS, 1.7 years for DCM, and 5.4 years for myocarditis; P < .0001). Of 103 cases, 27 (26.2%) had premonitory symptoms documented. CONCLUSION: SUCD accounted for 2.1% of all autopsies, and HLHS, DCM, and myocarditis were the 3 most common diagnoses, which presented at increasing ages. AD - Division of Pediatric Cardiology, Hospital for Sick Children, Toronto, Ontario, Canada. maria.ilina@btinternet.com AN - 21824887 AU - Ilina, M. V. AU - Kepron, C. A. AU - Taylor, G. P. AU - Perrin, D. G. AU - Kantor, P. F. AU - Somers, G. R. DA - Sep DB - PubMed DO - 10.1542/peds.2010-2307 DP - NLM ET - 2011/08/10 IS - 3 KW - Adolescent Cardiomyopathy, Dilated/complications Child Child, Preschool Coronary Vessel Anomalies/epidemiology Death, Sudden, Cardiac/epidemiology/*etiology Female Heart Diseases/*complications Humans Hypoplastic Left Heart Syndrome/complications Infant Male Myocarditis/*complications Retrospective Studies LA - eng N1 - 1098-4275 Ilina, Maria V Kepron, Charis A Taylor, Glenn P Perrin, Donald G Kantor, Paul F Somers, Gino R Journal Article United States Pediatrics. 2011 Sep;128(3):e513-20. doi: 10.1542/peds.2010-2307. Epub 2011 Aug 8. PY - 2011 SN - 0031-4005 SP - e513-20 ST - Undiagnosed heart disease leading to sudden unexpected death in childhood: a retrospective study T2 - Pediatrics TI - Undiagnosed heart disease leading to sudden unexpected death in childhood: a retrospective study VL - 128 ID - 345 ER - TY - JOUR AB - BACKGROUND: Unifocalization of major aortopulmonary collateral arteries (MAPCAs) in pulmonary atresia with ventricular septal defect and intracardiac repair has become the standard of care. However, there are no reports addressing unifocalization of MAPCAs in single-ventricle patients. It is unknown whether their pulmonary vascular bed can be reconstructed and low enough pulmonary vascular resistance achieved to allow for superior or total cavopulmonary connections. METHODS: We reviewed data on all patients with functional single ventricles and unifocalization procedures of MAPCAs. From 1997 to 2005, 14 consecutive children with various single-ventricle anatomies were operated on. RESULTS: Patients had a median of three surgical procedures (range, 1 to 5). Two patients had absent, all others diminutive central pulmonary arteries, with an average of 3.5 +/- 1.2 MAPCAs. Seven patients (50%) had bidirectional Glenn procedures, and 3 of these had Fontan procedures. Median postoperative pulmonary artery pressures measured 12.5 mm Hg (Glenn) and 14 mm Hg (Fontan), respectively. Six patients are alive today (46%), with 1 patient lost to follow-up. Three patients died early and 3 late after initial unifocalization to shunts. One other patient survived unifocalization, but was not considered a candidate for a Glenn procedure and died after high-risk two-ventricle repair. Another patient with right-ventricle-dependent coronary circulation died of sepsis late after Glenn. CONCLUSIONS: In selected patients with functional single ventricles and MAPCAs, the pulmonary vascular bed can be reconstructed sufficiently to allow for cavopulmonary connections. Venous flow to the pulmonary vasculature decreases cardiac volume load and is likely to increase life expectancy and quality of life for these patients. AD - Department of Cardiothoracic Surgery, Division of Pediatric Cardiac Surgery, Stanford University, Stanford, California 94305, USA. orx@stanford.edu AN - 16928512 AU - Reinhartz, O. AU - Reddy, V. M. AU - Petrossian, E. AU - Suleman, S. AU - Mainwaring, R. D. AU - Rosenthal, D. N. AU - Feinstein, J. A. AU - Gulati, R. AU - Hanley, F. L. DA - Sep DB - PubMed DO - 10.1016/j.athoracsur.2006.03.063 DP - NLM ET - 2006/08/25 IS - 3 KW - Abnormalities, Multiple Aorta, Thoracic/*surgery Arteriovenous Shunt, Surgical Cardiac Surgical Procedures Child Child, Preschool *Collateral Circulation Female Fontan Procedure Heart Ventricles/*abnormalities/surgery Humans Infant Infant, Newborn Male Postoperative Complications/mortality Pulmonary Artery/*surgery Pulmonary Atresia/*surgery *Pulmonary Circulation *Vascular Surgical Procedures Vena Cava, Superior/surgery LA - eng N1 - 1552-6259 Reinhartz, Olaf Reddy, V Mohan Petrossian, Edwin Suleman, Sam Mainwaring, Richard D Rosenthal, David N Feinstein, Jeffrey A Gulati, Raj Hanley, Frank L Evaluation Study Journal Article Netherlands Ann Thorac Surg. 2006 Sep;82(3):934-8; discussion 938-9. doi: 10.1016/j.athoracsur.2006.03.063. PY - 2006 SN - 0003-4975 SP - 934-8; discussion 938-9 ST - Unifocalization of major aortopulmonary collaterals in single-ventricle patients T2 - Ann Thorac Surg TI - Unifocalization of major aortopulmonary collaterals in single-ventricle patients VL - 82 ID - 290 ER - TY - JOUR AB - Background: Exercise limitation is almost universal among Fontan patients. Identifying unique clinical features in the small fraction of Fontan patients with normal exercise capacity (high-capacity Fontan [HCF]) provides potential to inform clinical strategies for those with low exercise capacity (usual Fontan). Methods: We performed a retrospective chart review of all patients with single-ventricle physiology palliated with a Fontan operation who underwent incremental cardiopulmonary exercise testing at Cincinnati Children's Hospital Medical Center from 2013 to 2018. Comparison was between patients with peak oxygen uptake < vs ≥ 80% predicted. Results: A total of 22 of 112 patients were classified as HCF (68% were female; aged 18 ± 7 years). During incremental exercise, peak oxygen uptake (86.1% ± 6.1% vs 62% ± 12.2% predicted; P < 0.001) was greater in HCF vs usual Fontan despite similar chronotropic impairment, resulting in a greater oxygen pulse in HCF. Pulmonary function, breathing reserve, and ventilatory equivalent for CO2 output slope were not different between groups. Those in the HCF group were more likely to self-report exercise ≥ 4 days/week for at least 30 minutes (77% vs 10%, P < 0.001), have normal systolic function (95% vs 74%, P = 0.003), have fewer postoperative complications (8% vs 36%, P = 0.04), and have shorter post-Fontan length of stay (8 ± 2.8 vs 12.4 ± 0.9 days, P = 0.04). Conclusions: Approximately 1 in 5 Fontan patients who undergo cardiopulmonary exercise testing have normal exercise capacity despite chronotropic impairment. This implies a better preserved stroke volume, perhaps due to greater muscle pump-mediated preload. Additionally, a complicated perioperative Fontan course is associated with eventual impaired functional capacity. AD - A.W. Powell, Cincinnati, 3333 Burnett Ave, MLC 2003, Ohio, United States AU - Powell, A. W. AU - Chin, C. AU - Alsaied, T. AU - Rossiter, H. B. AU - Wittekind, S. AU - Mays, W. A. AU - Lubert, A. AU - Veldtman, G. DB - Embase Medline DO - 10.1016/j.cjca.2019.11.006 KW - adult aerobic capacity article breathing child chronotropism complication controlled study exercise test female Fontan procedure functional status heart single ventricle heart stroke volume human length of stay lung function major clinical study male medical record review muscle phenotype postoperative complication retrospective study self report young adult carbon dioxide oxygen LA - English M3 - Article in Press N1 - L2005547819 2020-04-20 PY - 2020 SN - 0828-282X ST - The Unique Clinical Phenotype and Exercise Adaptation of Fontan Patients With Normal Exercise Capacity T2 - Canadian Journal of Cardiology TI - The Unique Clinical Phenotype and Exercise Adaptation of Fontan Patients With Normal Exercise Capacity UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2005547819 http://dx.doi.org/10.1016/j.cjca.2019.11.006 ID - 576 ER - TY - JOUR AB - OBJECTIVE: Advances in the geometric design of blood-contacting components are critically important as the use of minimally invasive, intravascular blood pumps becomes more pervasive in the treatment of adult and pediatric patients with congestive heart failure. The present study reports on the evaluation of uniquely shaped filaments and diffuser blades in the development of a protective stent for an intravascular cavopulmonary assist device for patients with a single ventricle. METHODS: We performed numeric modeling, hydraulic testing of 11 stents with an axial flow blood pump, and blood bag experiments (n = 6) of the top-performing stent geometries to measure the levels of hemolysis. A direct comparison using statistical analyses, including regression analysis and analysis of variance, was completed. RESULTS: The stent geometry with straight filaments and diffuser blades that extended to the vessel wall outperformed all other stent configurations. The pump with this particular stent was able to generate pressures of 2 to 32 mm Hg for flow rates of 0.5 to 4 L/min at 5000 to 7000 RPM. A comparison of the experimental performance data to the numeric predictions demonstrated an excellent agreement within 16%. The addition of diffuser blades to the stent reduced the flow vorticity at the pump outlet. The average and maximum normalized index of hemolysis level was 0.0056 g/100 L and 0.0064 g/100 L, respectively. CONCLUSIONS: The specialized design of the stents, which protect the vessel wall from the rotating components of the pump, proved to be advantageous by further augmenting the pressure generation of the pump, reducing the flow vorticity at the pump outlet, and enhancing flow control. AD - Department of Mechanical Engineering, Virginia Commonwealth University School of Engineering, Richmond, VA 23284, USA. althrock@vcu.edu AN - 22341416 AU - Throckmorton, A. L. AU - Carr, J. P. AU - Moskowitz, W. B. AU - Gangemi, J. J. AU - Haggerty, C. M. AU - Yoganathan, A. P. DA - Sep DB - PubMed DO - 10.1016/j.jtcvs.2011.12.061 DP - NLM ET - 2012/02/22 IS - 3 KW - Biomechanical Phenomena Blood Flow Velocity Blood Pressure Computer Simulation *Coronary Circulation *Heart-Assist Devices/adverse effects *Hemodynamics Hemolysis Humans Materials Testing Models, Cardiovascular Numerical Analysis, Computer-Assisted Prosthesis Design Regression Analysis *Stents/adverse effects Stress, Mechanical LA - eng N1 - 1097-685x Throckmorton, Amy L Carr, James P Moskowitz, William B Gangemi, James J Haggerty, Christopher M Yoganathan, Ajit P Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, Non-P.H.S. United States J Thorac Cardiovasc Surg. 2012 Sep;144(3):704-9. doi: 10.1016/j.jtcvs.2011.12.061. Epub 2012 Feb 15. PY - 2012 SN - 0022-5223 SP - 704-9 ST - Uniquely shaped cardiovascular stents enhance the pressure generation of intravascular blood pumps T2 - J Thorac Cardiovasc Surg TI - Uniquely shaped cardiovascular stents enhance the pressure generation of intravascular blood pumps VL - 144 ID - 532 ER - TY - JOUR AB - As early as 1699, Chemineau described a heart composed of 2 auricles but only 1 ventricle. The univentricular heart has since fascinated the medical community. Unique in its complexity and scope, the univentricular heart has sparked intense debates about embryology and nomenclature, challenged our understanding of cardiovascular physiology and hemodynamics, and inspired some of the most creative surgical and interventional approaches in human history. The present report provides an overview of the nomenclature and classification of the univentricular heart, epidemiology and pathological subtypes, genetic factors, physiology, clinical features, diagnostic assessment, therapy, and postoperative sequelae. Although the present report touches on issues applicable to neonates and children with univentricular hearts, the focus is on information of interest and relevance to the adult cardiologist. AD - Adult Congenital Heart Center, Montreal Heart Institute, 5000 Bélanger St, Montreal, Quebec, H1T 1C8, Canada. paul.khairy@umontreal.ca AN - 17296869 AU - Khairy, P. AU - Poirier, N. AU - Mercier, L. A. DA - Feb 13 DB - PubMed DO - 10.1161/circulationaha.105.592378 DP - NLM ET - 2007/02/14 IS - 6 KW - Adolescent Cardiac Catheterization Child Child, Preschool Cyanosis/therapy Echocardiography Electrocardiography Exercise Fontan Procedure *Heart Defects, Congenital/classification/diagnosis/pathology/surgery Humans Infant Infant, Newborn Quality of Life Terminology as Topic LA - eng N1 - 1524-4539 Khairy, Paul Poirier, Nancy Mercier, Lise-Andrée Journal Article Research Support, Non-U.S. Gov't United States Circulation. 2007 Feb 13;115(6):800-12. doi: 10.1161/CIRCULATIONAHA.105.592378. PY - 2007 SN - 0009-7322 SP - 800-12 ST - Univentricular heart T2 - Circulation TI - Univentricular heart VL - 115 ID - 45 ER - TY - JOUR AB - OBJECTIVES: Unplanned admissions to the pediatric cardiac ICU may be a large and high-risk group. Our study describes the frequency of unplanned pediatric cardiac ICU admissions, their admission data, and outcomes. DESIGN: All admissions to a pediatric cardiac ICU over 2 years were reviewed and those that were unplanned were identified for a detailed chart abstraction. Demographic, laboratory, diagnostic, and outcome data were collected. Readmission or admission for adverse event was noted. SETTING: Single, tertiary, pediatric cardiac center. PATIENTS: All patients admitted unexpectedly to the pediatric cardiac critical care unit between May 2008 and May 2010. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: There were 1,203 admissions to the cardiac ICU, and 426 (35%) were unplanned. The most common reasons for admission were new heart disease (25%), infection (19%), arrhythmia (13%), and congestive heart failure (11%). The majority of unplanned admissions (62%) occurred at night. Shock was present at admission in 18.5% of patients. Structural heart disease was present in 79%, and 39% of those were patients with single ventricle. Overall mortality among unplanned admissions was 7.3%, which is higher than that reported for elective surgical admissions. Mortality for the subset of patients readmitted within 30 days was 5.5%. Mean creatinine at admission was higher among nonsurvivors (0.7) than survivors (0.5). CONCLUSIONS: Unplanned admissions accounted for over one third of all admissions and had a high mortality rate. The majority of these occur at night, which may affect staffing models. Acute deterioration leading to unplanned admission, rather than readmission status, may be the driving factor in increased mortality. However, the risk of readmission, lower renal function, or other indices may identify patients at higher risk of an unplanned admission. Continued efforts to identify patients at risk for unplanned admissions are warranted given the outcomes in this cohort. Copyright © 2015 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies. AD - Division of Cardiology, Advocate Children's Hospital, Oak Lawn, IL, United States Department of Pediatrics, Children's National Medical Center, Washington, DC, United States Department of Pediatrics, Children's Hospital Los Angeles, Los Angeles, CA, United States Division of Cardiology, Children's National Medical Center, Washington, DC, United States Division of Critical Care, Children's National Medical Center, Washington, DC, United States AU - Penk, J. S. AU - Loke, Y. H. AU - Waloff, K. R. AU - Frank, L. H. AU - Stockwell, D. C. AU - Spaeder, M. C. AU - Berger, J. T. DB - Scopus DO - 10.1097/PCC.0000000000000316 IS - 2 KW - cardiac intensive care unit mortality outcomes pediatric critical care readmissions unplanned admissions M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2015 SP - 155-160 ST - Unplanned admissions to a pediatric cardiac critical care unit: A review of 2 years' experience T2 - Pediatric Critical Care Medicine TI - Unplanned admissions to a pediatric cardiac critical care unit: A review of 2 years' experience UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84923072558&doi=10.1097%2fPCC.0000000000000316&partnerID=40&md5=a08419d6102ba381da06b9c0cb50306e VL - 16 ID - 1965 ER - TY - JOUR AB - The ideal age to perform the Fontan procedure is still unknown. The aim of this study is to determine outcomes after Fontan surgery delayed to adolescence and adulthood in Australia and New Zealand. Patients who had undergone a Fontan procedure at 15 years of age or older were identified in the 1133 patients registered in the Australia and New Zealand Fontan Registry until December 2012. A total of 45 patients underwent the following Fontan procedure at a median age of 18.3 years (16-21 years): 24 atriopulmonary connections, 10 lateral tunnel, and 11 extracardiac conduits. Hospital mortality was 13% (6 of 45). After a mean follow-up of 15.5 ± 9 years, there were 8 late deaths. Survival rates after 10, 20, and 25 years were 79% (95% CI: 64-89), 70% (95% CI: 51-83), and 70% (95% CI: 51-83), respectively. Freedom from Fontan failure (death, heart transplantation, Fontan takedown, protein-losing enteropathy, and poor functional status) after 10 and 20 years was 63% (95% CI: 47-76) and 35% (95% CI: 19-52), respectively. Patients with a single left ventricle had a lower risk of failure (hazard ratio = 0.25, 95% CI: 0.10-0.59; P = 0.002). Arrhythmias developed in 29 patients after a median of 0.1 years (0-9.3 years) and 10 required a permanent pacemaker. Freedom from all adverse events at 10 years was 30% (95% CI: 16-45). Outcomes of the Fontan procedure in adolescents and adults are poor, with disproportionately high hospital mortality and late adverse events. The Fontan procedure should not be delayed to adolescence and adulthood and should be performed electively in childhood. AD - Y. d'Udekem, Department of Cardiac Surgery, The Royal Children's Hospital, Flemington Rd, Parkville, Melbourne, VIC, Australia AU - Forsdick, V. AU - Iyengar, A. J. AU - Carins, T. AU - Gentles, T. L. AU - Weintraub, R. G. AU - Celermajer, D. S. AU - d'Udekem, Y. DB - Embase Medline DO - 10.1053/j.semtcvs.2015.05.001 IS - 2 KW - adolescence adolescent adult adulthood article Australia Budd Chiari syndrome cerebrovascular accident complete heart block congenital heart disease controlled study female femoral vein follow up Fontan procedure functional status heart arrhythmia heart muscle ischemia heart transplantation heart ventricle fibrillation heart ventricle tachycardia human lung embolism major clinical study male New Zealand patient satisfaction post treatment survival prospective study protein losing gastroenteropathy supraventricular tachycardia surgical mortality thromboembolism treatment failure treatment outcome vein thrombosis LA - English M3 - Article N1 - L606666176 2015-11-04 2015-12-23 PY - 2015 SN - 1532-9488 1043-0679 SP - 168-174 ST - Unsatisfactory Early and Late Outcomes After Fontan Surgery Delayed to Adolescence and Adulthood T2 - Seminars in Thoracic and Cardiovascular Surgery TI - Unsatisfactory Early and Late Outcomes After Fontan Surgery Delayed to Adolescence and Adulthood UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L606666176 http://dx.doi.org/10.1053/j.semtcvs.2015.05.001 VL - 27 ID - 876 ER - TY - JOUR AB - PURPOSE OF REVIEW: The success in the management of congenital heart disease has resulted in a growing population now expected to reach adulthood. The declining mortality during childhood results in adults with decades of cardiovascular disease burden. The purpose of this review is to provide an update on some of the common issues encountered when caring for the adult with congenital heart disease through a review of recently published articles. RECENT FINDINGS: This review focuses on addressing the lack of information, gaps in clinical care, single ventricle complications, and heart failure in the adults with congenital heart disease population. SUMMARY: The advancing age of the congenital heart population comes with a substantial life cardiovascular risk. Despite this, they typically experience and report a good quality of life. Providing care to adults with congenital heart disease requires a comprehensive team to address the many complications that are known to arise. This comprehensive team should be available to all. AD - Thomas P. Graham Jr. Division of Pediatric Cardiology, the Division of Cardiovascular Medicine, and the Vanderbilt Heart and Vascular Institute, Vanderbilt University School of Medicine, Nashville, Tennessee, USA. AN - 25102108 AU - Markham, L. W. DA - Oct DB - PubMed DO - 10.1097/mop.0000000000000134 DP - NLM ET - 2014/08/08 IS - 5 KW - Adult Cardiac Care Facilities/*organization & administration Evidence-Based Practice/*organization & administration Female Health Knowledge, Attitudes, Practice Health Services Accessibility/*organization & administration Health Services Needs and Demand Heart Defects, Congenital/complications/*epidemiology/psychology Heart Failure/*prevention & control/psychology Humans Insurance, Health/statistics & numerical data Male Multicenter Studies as Topic Practice Guidelines as Topic Pregnancy Pregnancy Complications, Cardiovascular/*epidemiology/psychology Quality of Life Risk Factors Transition to Adult Care/organization & administration United States/epidemiology LA - eng N1 - 1531-698x Markham, Larry W Journal Article Review United States Curr Opin Pediatr. 2014 Oct;26(5):521-6. doi: 10.1097/MOP.0000000000000134. PY - 2014 SN - 1040-8703 SP - 521-6 ST - Update on the challenges facing the adult with congenital heart disease community: for both the patient and provider T2 - Curr Opin Pediatr TI - Update on the challenges facing the adult with congenital heart disease community: for both the patient and provider VL - 26 ID - 148 ER - TY - JOUR AB - Summary Background A central venous catheter located in the jugular or subclavian vein provides rapid, reliable vascular access for pediatric heart surgery. However, intravascular catheters are associated with vessel injury. Stenosis or thrombosis of central veins in the upper body can lead to 'superior vena cava syndrome' with markedly elevated venous pressures in the head and neck, causing facial swelling and headaches. This complication may be especially serious for patients with superior cavopulmonary (Glenn) or total cavopulmonary (Fontan) circulation. The authors hypothesized that upper body central line placement would be associated with a low risk of venous thrombosis or stenosis. Methods A three-year retrospective review of infant and univentricular cardiac procedures at a single institution was performed. Two hundred and thirty-five consecutive cardiac surgical patients <1 year of age or undergoing palliation for univentricular cardiac anatomy up to five years of age during January 2010 to December 2012 were included in this study. Upper body central lines are routinely placed by the anesthesiologist after induction of anesthesia for pediatric cardiac surgery at the study institution. The major exception is existing central venous access via an umbilical vein or femoral vein. Patients <2 years of age received a 4.0-French, 5-cm double-lumen central line [Cook Medical polyurethane, no antibiotic or heparin coating]. Those over two years of age received a 5.0-French, 8-cm triple lumen central line [Cook Medical polyurethane, no antibiotic or heparin coating]. A retrospective review of charts, hospital reports, echocardiographic studies, and cardiac catheterization studies was performed. Results The combined population of infants <1 year of age and patients <5 years of age with functional univentricular hearts totaled 235 patients who underwent 261 cardiac surgical operations. In this cohort of 261 cases, 171 size 4.0 or 5.0-French upper body central lines were inserted. A total of 158 right internal jugular vein catheters were placed. Two left internal jugular lines, two left subclavian lines, and nine right subclavian lines were placed in this population after failure to obtain right internal jugular access. Due to the small sample size (N = 13), the central lines not placed in the right internal jugular vein were excluded from further review. Two cases with right internal jugular venous lines were excluded due to death (without known stenosis or thrombosis) with the line in place. Twenty-three size 4.0- or 5.0-French right internal jugular central venous lines were placed in patients over one year of age (range 1.1-4.3 years) having modified Glenn- or Fontan-type surgery. The central lines were removed with a median of 1.4 days after insertion (range 0.7-8.2 days) for these older children, compared with a median of 4.2 days of age (range 0.3-19.3 days) for the 133 children <1 year of age. Retrospective chart review of nursing notes, progress notes, cardiology notes, discharge summaries, echocardiographic reports, and cardiac catheterization reports for all patients who received an upper body central venous line (internal jugular or subclavian) showed no definitive diagnosis of an upper body venous stenosis or thrombosis related to the central venous line. A further targeted review of echocardiographic and cardiac catheterization studies for univentricular cardiac patients failed to show stenosis or thrombosis of a vessel associated with upper body central line placement. Conclusions This study describes one institution's experience with routine upper body central venous catheter placement for neonatal and infant cardiac surgery as well as univentricular cardiac palliation (Glenn and Fontan procedures) with minimal risk of clinically significant catheter-associated vessel thrombosis or stenosis. No upper body central venous stenosis or thrombosis was detected in association with perioperative catheter placement in the upper body central venous system, primarily the right internal jugular vein in 156 cases. Right internal jugular central line placement for infant cardiac surgery can be utilized with a low risk of direct venous thrombosis or stenosis. © 2013 John Wiley & Sons Ltd. AD - J.W. Miller, Department of Anesthesiology, Division of Pediatric Cardiac Anesthesiology, Cincinnati Children's Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229, United States AU - Miller, J. W. AU - Vu, D. N. AU - Chai, P. J. AU - Kreutzer, J. H. AU - John, J. B. AU - Vener, D. F. AU - Jacobs, J. P. DB - Embase Medline DO - 10.1111/pan.12261 IS - 11 KW - polyurethan anesthesia induction article brachiocephalic vein cardiac patient central venous catheter child chylothorax Fallot tetralogy femoral vein Fontan procedure Glenn shunt heart catheterization heart single ventricle heart surgery human hypoplastic left heart syndrome infant inferior cava vein internal jugular vein major clinical study Norwood procedure palliative therapy pediatric surgery peripherally inserted central venous catheter preschool child priority journal retrospective study risk reduction stenosis subclavian vein surgical patient umbilical vein vein thrombosis LA - English M3 - Article N1 - L369975601 2013-10-16 2013-10-18 PY - 2013 SN - 1155-5645 1460-9592 SP - 980-988 ST - Upper body central venous catheters in pediatric cardiac surgery T2 - Paediatric Anaesthesia TI - Upper body central venous catheters in pediatric cardiac surgery UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L369975601 http://dx.doi.org/10.1111/pan.12261 VL - 23 ID - 982 ER - TY - JOUR AB - Background: Nurse education and training are key to providing congenital heart disease (CHD) patients with consistent high standards of care as well as enabling career progression. One approach for improving educational experience is the use of 3D patient-specific models. Objectives: To gather pilot data to assess the feasibility of using 3D models of CHD during a training course for cardiac nurses; to evaluate the potential of 3D models in this context, from the nurses' perspective; and to identify possible improvements to optimise their use for teaching. Design: A cross-sectional survey. Setting: A national training week for cardiac nurses. Participants: One hundred cardiac nurses (of which 65 pediatric and 35 adult). Methods: Nurses were shown nine CHD models within the context of a specialized course, following a lecture on the process of making the models themselves, starting from medical imaging. Participants were asked about their general learning experience, if models were more/less informative than diagrams/drawings and lesion-specific/generic models, and their overall reaction to the models. Possible differences between adult and pediatric nurses were investigated. Written feedback was subjected to content analysis and quantitative data were analyzed using nonparametric statistics. Results: Generally models were well liked and nurses considered them more informative than diagrams. Nurses found that 3D models helped in the appreciation of overall anatomy (86%), spatial orientation (70%), and anatomical complexity after treatment (66%). There was no statistically significant difference between adult and pediatric nurses' responses. Thematic analysis highlighted the need for further explanation, use of labels and use of colors to highlight the lesion of interest amongst improvements for optimizing 3D models for teaching/training purposes. Conclusion: 3D patient-specific models are useful tools for training adult and pediatric cardiac nurses and are particularly helpful for understanding CHD anatomy after repair. © 2016 Wiley Periodicals, Inc. AD - Bristol Heart Institute, School of Clinical Sciences, University of Bristol, Bristol, United Kingdom Cardiorespiratory Division, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, United Kingdom Centre for Cardiovascular Imaging, Institute of Cardiovascular Science, University College London, London, United Kingdom AU - Biglino, G. AU - Capelli, C. AU - Koniordou, D. AU - Robertshaw, D. AU - Leaver, L. K. AU - Schievano, S. AU - Taylor, A. M. AU - Wray, J. DB - Scopus DO - 10.1111/chd.12414 IS - 1 KW - 3D printing cardiovascular nursing congenital heart defects training M3 - Article N1 - Cited By :33 Export Date: 15 June 2020 PY - 2017 SP - 113-118 ST - Use of 3D models of congenital heart disease as an education tool for cardiac nurses T2 - Congenital Heart Disease TI - Use of 3D models of congenital heart disease as an education tool for cardiac nurses UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84988849795&doi=10.1111%2fchd.12414&partnerID=40&md5=7a5516d8d458156537b1f2f6a87fbd6b VL - 12 ID - 1881 ER - TY - JOUR AB - BACKGROUND: Open cardiovascular magnetic resonance (CMR) scanners offer the potential for imaging patients with claustrophobia or large body size, but at a lower 1.0 Tesla magnetic field. This study aimed to evaluate the efficacy of open CMR for evaluation of pediatric and congenital heart disease. METHODS: This retrospective, cross-sectional study included all patients ≤18 years old or with congenital heart disease who underwent CMR on an open 1.0 Tesla scanner at two centers from 2012-2014. Indications for CMR and clinical questions were extracted from the medical record. Studies were qualitatively graded for image quality and diagnostic utility. In a subset of 25 patients, signal-to-noise (SNR) and contrast-to-noise (CNR) ratios were compared to size- and diagnosis-matched patients with CMR on a 1.5 Tesla scanner. RESULTS: A total of 65 patients (median 17.3 years old, 60% male) were included. Congenital heart disease was present in 32 (50%), with tetralogy of Fallot and bicuspid aortic valve the most common diagnoses. Open CMR was used due to scheduling/equipment issues in 51 (80%), claustrophobia in 7 (11%), and patient size in 3 (5%); 4 patients with claustrophobia had failed CMR on a different scanner, but completed the study on open CMR without sedation. All patients had good or excellent image quality on black blood, phase contrast, magnetic resonance angiography, and late gadolinium enhancement imaging. There was below average image quality in 3/63 (5%) patients with cine images, and 4/15 (27%) patients with coronary artery imaging. SNR and CNR were decreased in cine and magnetic resonance angiography images compared to 1.5 Tesla. The clinical question was answered adequately in all but 2 patients; 1 patient with a Fontan had artifact from an embolization coil limiting RV volume analysis, and in 1 patient the right coronary artery origin was not well seen. CONCLUSIONS: Open 1.0 Tesla scanners can effectively evaluate pediatric and congenital heart disease, including patients with claustrophobia and larger body size. Despite minor artifacts and differences in SNR and CNR, the majority of clinical questions can be answered adequately, with some limitations with coronary artery imaging. Further evaluation is necessary to optimize protocols and image quality. AD - Department of Pediatrics and Communicable Diseases, Division of Pediatric Cardiology, University of Michigan, Ann Arbor, MI, USA. jimmyl@umich.edu. Department of Radiology, Section of Pediatric Radiology, University of Michigan, Ann Arbor, MI, USA. jimmyl@umich.edu. Departments of Pediatrics and Radiology, Stony Brook University, Stony Brook, NY, USA. james.nielsen@stonybrookmedicine.edu. Departments of Pediatrics and Radiology, Stony Brook University, Stony Brook, NY, USA. Layne.morowitz@stonybrookmedicine.edu. Department of Medicine, Division of Cardiology, Stony Brook University, Stony Brook, NY, USA. Muzammil.musani@stonybrookmedicine.edu. Department of Radiology, Section of Pediatric Radiology, University of Michigan, Ann Arbor, MI, USA. maryamg@med.umich.edu. Department of Radiology, Division of Cardiothoracic Radiology, University of Michigan, Ann Arbor, MI, USA. prachia@med.umich.edu. Department of Radiology, Division of Cardiothoracic Radiology, University of Michigan, Ann Arbor, MI, USA. elsayei@med.umich.edu. Department of Pediatrics and Communicable Diseases, Division of Pediatric Cardiology, University of Michigan, Ann Arbor, MI, USA. adamdorf@med.umich.edu. Department of Radiology, Section of Pediatric Radiology, University of Michigan, Ann Arbor, MI, USA. adamdorf@med.umich.edu. AN - 26004027 AU - Lu, J. C. AU - Nielsen, J. C. AU - Morowitz, L. AU - Musani, M. AU - Ghadimi Mahani, M. AU - Agarwal, P. P. AU - Ibrahim el, S. H. AU - Dorfman, A. L. C2 - PMC4490663 DA - May 25 DB - PubMed DO - 10.1186/s12968-015-0144-y DP - NLM ET - 2015/05/26 IS - 1 KW - Adolescent Adult Age Factors Artifacts Body Size Child Child, Preschool Coronary Vessels/*pathology Cross-Sectional Studies Equipment Design Female Heart Defects, Congenital/*pathology Humans Image Interpretation, Computer-Assisted Magnetic Resonance Angiography/adverse effects/*instrumentation Magnetic Resonance Imaging, Cine/adverse effects/*instrumentation Male Michigan Middle Aged Myocardium/*pathology New York Observer Variation Phobic Disorders/etiology Predictive Value of Tests Reproducibility of Results Retrospective Studies Signal-To-Noise Ratio Young Adult LA - eng N1 - 1532-429x Lu, Jimmy C Nielsen, James C Morowitz, Layne Musani, Muzammil Ghadimi Mahani, Maryam Agarwal, Prachi P Ibrahim, El-Sayed H Dorfman, Adam L Evaluation Study Journal Article Multicenter Study J Cardiovasc Magn Reson. 2015 May 25;17(1):39. doi: 10.1186/s12968-015-0144-y. PY - 2015 SN - 1097-6647 (Print) 1097-6647 SP - 39 ST - Use of a 1.0 Tesla open scanner for evaluation of pediatric and congenital heart disease: a retrospective cohort study T2 - J Cardiovasc Magn Reson TI - Use of a 1.0 Tesla open scanner for evaluation of pediatric and congenital heart disease: a retrospective cohort study VL - 17 ID - 281 ER - TY - JOUR AB - Background: Open cardiovascular magnetic resonance (CMR) scanners offer the potential for imaging patients with claustrophobia or large body size, but at a lower 1.0 Tesla magnetic field. This study aimed to evaluate the efficacy of open CMR for evaluation of pediatric and congenital heart disease. Methods: This retrospective, cross-sectional study included all patients ≤18 years old or with congenital heart disease who underwent CMR on an open 1.0 Tesla scanner at two centers from 2012-2014. Indications for CMR and clinical questions were extracted from the medical record. Studies were qualitatively graded for image quality and diagnostic utility. In a subset of 25 patients, signal-to-noise (SNR) and contrast-to-noise (CNR) ratios were compared to size- and diagnosis-matched patients with CMR on a 1.5 Tesla scanner. Results: A total of 65 patients (median 17.3 years old, 60% male) were included. Congenital heart disease was present in 32 (50%), with tetralogy of Fallot and bicuspid aortic valve the most common diagnoses. Open CMR was used due to scheduling/equipment issues in 51 (80%), claustrophobia in 7 (11%), and patient size in 3 (5%); 4 patients with claustrophobia had failed CMR on a different scanner, but completed the study on open CMR without sedation. All patients had good or excellent image quality on black blood, phase contrast, magnetic resonance angiography, and late gadolinium enhancement imaging. There was below average image quality in 3/63 (5%) patients with cine images, and 4/15 (27%) patients with coronary artery imaging. SNR and CNR were decreased in cine and magnetic resonance angiography images compared to 1.5 Tesla. The clinical question was answered adequately in all but 2 patients; 1 patient with a Fontan had artifact from an embolization coil limiting RV volume analysis, and in 1 patient the right coronary artery origin was not well seen. Conclusions: Open 1.0 Tesla scanners can effectively evaluate pediatric and congenital heart disease, including patients with claustrophobia and larger body size. Despite minor artifacts and differences in SNR and CNR, the majority of clinical questions can be answered adequately, with some limitations with coronary artery imaging. Further evaluation is necessary to optimize protocols and image quality. AD - J.C. Lu, Department of Pediatrics and Communicable Diseases, Division of Pediatric Cardiology, University of Michigan, Ann Arbor, MI, United States AU - Lu, J. C. AU - Nielsen, J. C. AU - Morowitz, L. AU - Musani, M. AU - Ghadimi Mahani, M. AU - Agarwal, P. P. AU - Ibrahim, E. S. H. AU - Dorfman, A. L. C1 - magnevist(Bayer,Germany) prohance(Bracco,United States) C2 - Bayer(Germany) Bracco(United States) DB - Embase Medline DO - 10.1186/s12968-015-0144-y IS - 1 KW - gadolinium pentetate meglumine gadoteridol adolescent adult article artifact bicuspid aortic valve cardiovascular magnetic resonance childhood disease claustrophobia cohort analysis congenital heart disease contrast enhancement contrast to noise ratio controlled study cross-sectional study data extraction diagnostic value embolization coil Fallot tetralogy female heart right ventricle volume human image quality magnetic resonance angiography major clinical study male medical record nuclear magnetic resonance scanner priority journal qualitative analysis retrospective study right coronary artery sedation signal noise ratio magnevist prohance 1.0 Tesla LA - English M3 - Article N1 - L604454617 2015-05-28 2015-06-08 PY - 2015 SN - 1532-429X 1097-6647 ST - Use of a 1.0 Tesla open scanner for evaluation of pediatric and congenital heart disease: A retrospective cohort study T2 - Journal of Cardiovascular Magnetic Resonance TI - Use of a 1.0 Tesla open scanner for evaluation of pediatric and congenital heart disease: A retrospective cohort study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L604454617 http://dx.doi.org/10.1186/s12968-015-0144-y VL - 17 ID - 864 ER - TY - JOUR AB - OBJECTIVE: To present a case of nebulized tissue plasminogen activator (t-PA) treatment for symptomatic plastic bronchitis in a pediatric patient years after a Fontan procedure. CASE SUMMARY: A 13-year-old boy with a history of corrected congenital heart disease was admitted to the pediatric intensive care unit after 2 weeks of worsening respiratory distress. A chest radiograph and subsequent bronchoscopy revealed extensive mucus plugging due to plastic bronchitis. Casts reaccumulated quickly after manual removal of the mucus and a regimen of aerosolized t-PA was initiated to break down the casts and prevent further cast formation over the 17-day hospital course. The treatment was successful and the patient was discharged home without evidence of bronchial casts. DISCUSSION: Plastic bronchitis is a potentially devastating condition in which pulmonary infiltrates line the bronchial tree, forming casts and prohibiting effective oxygen exchange. There are few effective treatment options for this condition. The use of aerosolized t-PA for the treatment of plastic bronchitis has been reported to be safe and effective in 4 cases but no consistent regimen, dose, or duration of treatment has been established. CONCLUSIONS: t-PA can be nebulized and inhaled for successful inhibition of bronchial cast formation. More information to determine the most effective dose and duration of therapy is needed to effectively improve the lives of people with plastic bronchitis. AD - American Family Children's Hospital, University of Wisconsin Hospital and Clinics, Madison, WI, USA. nlubcke@uwhealth.org AN - 23424228 AU - Lubcke, N. L. AU - Nussbaum, V. M. AU - Schroth, M. DA - Mar DB - PubMed DO - 10.1345/aph.1R690 DP - NLM ET - 2013/02/21 IS - 3 KW - Administration, Inhalation Adolescent Aerosols Bronchitis/diagnostic imaging/*drug therapy Humans Male Radiography Tissue Plasminogen Activator/*administration & dosage LA - eng N1 - 1542-6270 Lubcke, Nicole L Nussbaum, Vicki M Schroth, Mary Case Reports Journal Article United States Ann Pharmacother. 2013 Mar;47(3):e13. doi: 10.1345/aph.1R690. Epub 2013 Feb 19. PY - 2013 SN - 1060-0280 SP - e13 ST - Use of aerosolized tissue plasminogen activator in the treatment of plastic bronchitis T2 - Ann Pharmacother TI - Use of aerosolized tissue plasminogen activator in the treatment of plastic bronchitis VL - 47 ID - 422 ER - TY - JOUR AB - Total cavopulmonary connection (TCPC), a typical kind of Fontan procedure, is commonly used in the treatment of a functional single ventricle. The palliative cardiothoracic procedure is performed by connecting the superior vena cava and the inferior vena cava to the pulmonary arteries. Due to the difficulty of direct study in vivo, in this paper, computational fluid dynamics (CFD) was introduced to estimate the outcomes of patient-specific TCPC configuration. We mainly focused on the influence of blood pulsation and respiration. Fast Fourier transforms method was employed to separate the measured flow conditions into the rate of breath and heart beat. Blood flow performance around the TCPC connection was investigated by analyzing the results of time-varying energy losses, blood flow distribution rate, local pressure, and wall shear stress distributions. It was found that the value of energy loss including the influence of respiration was 1.5 times higher than the value of energy loss disregarding respiratory influences. The results indicated that the hemodynamic outcomes of TCPC treatment are obviously influenced by respiration. The influence of respiration plays an important role in estimating the results of TCPC treatment and thus should be included as one of the important conditions of computational haemodynamic analysis. AD - Department of Cardiothoracic Surgery, Shanghai Children's Medical Centre, Shanghai Jiao Tong University School of Medicine, 1678 Dongfang Road, Shanghai 200127, China. Australian School of Advanced Medicine, 2 Technology Place, Macquarie University, North Ryde, Sydney, NSW 2109, Australia. ASMeW Lab, Centre for Advanced Biomedical Sciences, TWIns, Waseda University, 2-2 Wakamatsucho, Shinjuku, Tokyo 162-8480, Japan. AN - 24385870 AU - Liu, J. AU - Qian, Y. AU - Sun, Q. AU - Liu, J. AU - Umezu, M. C2 - PMC3872434 DB - PubMed DO - 10.1155/2013/131597 DP - NLM ET - 2014/01/05 KW - Cardiac Surgical Procedures Child Computer Simulation Female Fontan Procedure Fourier Analysis Heart/anatomy & histology Heart Bypass, Right/*methods Heart Ventricles/surgery Hemodynamics Humans *Hydrodynamics Hypoplastic Left Heart Syndrome/*surgery Magnetic Resonance Imaging Models, Cardiovascular Pressure *Respiration Software Time Factors Ultrasonography LA - eng N1 - 1537-744x Liu, Jinlong Qian, Yi Sun, Qi Liu, Jinfen Umezu, Mitsuo Journal Article Research Support, Non-U.S. Gov't ScientificWorldJournal. 2013 Dec 9;2013:131597. doi: 10.1155/2013/131597. eCollection 2013. PY - 2013 SN - 2356-6140 (Print) 1537-744x SP - 131597 ST - Use of computational fluid dynamics to estimate hemodynamic effects of respiration on hypoplastic left heart syndrome surgery: total cavopulmonary connection treatments T2 - ScientificWorldJournal TI - Use of computational fluid dynamics to estimate hemodynamic effects of respiration on hypoplastic left heart syndrome surgery: total cavopulmonary connection treatments VL - 2013 ID - 300 ER - TY - JOUR AB - Abstract Objective To categorise records according to primary cardiac diagnosis in the United Kingdom Central Cardiac Audit Database in order to add this information to a risk adjustment model for paediatric cardiac surgery. Design Codes from the International Paediatric Congenital Cardiac Code were mapped to recognisable primary cardiac diagnosis groupings, allocated using a hierarchy and less refined diagnosis groups, based on the number of functional ventricles and presence of aortic obstruction. Setting A National Clinical Audit Database. Patients Children undergoing cardiac interventions: the proportions for each diagnosis scheme are presented for 13,551 first patient surgical episodes since 2004. Results In Scheme 1, the most prevalent diagnoses nationally were ventricular septal defect (13%), patent ductus arteriosus (10.4%), and tetralogy of Fallot (9.5%). In Scheme 2, the prevalence of a biventricular heart without aortic obstruction was 64.2% and with aortic obstruction was 14.1%; the prevalence of a functionally univentricular heart without aortic obstruction was 4.3% and with aortic obstruction was 4.7%; the prevalence of unknown (ambiguous) number of ventricles was 8.4%; and the prevalence of acquired heart disease only was 2.2%. Diagnostic groups added to procedural information: of the 17% of all operations classed as not a specific procedure, 97.1% had a diagnosis identified in Scheme 1 and 97.2% in Scheme 2. Conclusions Diagnostic information adds to surgical procedural data when the complexity of case mix is analysed in a national database. These diagnostic categorisation schemes may be used for future investigation of the frequency of conditions and evaluation of long-term outcome over a series of procedures. © 2012 Cambridge University Press. AD - K.L. Brown, Cardiac Unit, Great Ormond Street Hospital NHS Trust, London WC1N 3JH, United Kingdom AU - Brown, K. L. AU - Crowe, S. AU - Pagel, C. AU - Bull, C. AU - Muthialu, N. AU - Gibbs, J. AU - Cunningham, D. AU - Utley, M. AU - Tsang, V. T. AU - Franklin, R. DB - Embase Medline DO - 10.1017/S1047951112001369 IS - 4 KW - algorithm aortic occlusion article atrioventricular septal defect cardiovascular system examination controlled study data base disease classification Fallot tetralogy great vessels transposition heart atrium septum defect heart right ventricle double outlet heart surgery heart ventricle septum defect human lung vein drainage anomaly major clinical study clinical audit medical information patent ductus arteriosus pediatric surgery prevalence quality of life risk assessment United Kingdom LA - English M3 - Article N1 - L369329242 2013-07-24 2013-08-07 PY - 2013 SN - 1047-9511 1467-1107 SP - 491-498 ST - Use of diagnostic information submitted to the United Kingdom Central Cardiac Audit Database: Development of categorisation and allocation algorithms T2 - Cardiology in the Young TI - Use of diagnostic information submitted to the United Kingdom Central Cardiac Audit Database: Development of categorisation and allocation algorithms UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L369329242 http://dx.doi.org/10.1017/S1047951112001369 VL - 23 ID - 991 ER - TY - JOUR AB - Mechanical ventilation hampers passive venous flow to the lungs in patients with cavopulmonary connections. When feasible, early extubation may be beneficial in this patient population. In cases of required ventilatory support, understanding of cardiopulmonary interactions is paramount for the election of appropriate ventilatory strategies. Results on the use of high-frequency oscillatory ventilation in these patients remain controversial. We present the case of a child with single ventricle physiology palliated with a cavopulmonary connection who developed acute respiratory distress syndrome in whom the use of high-frequency oscillatory ventilation improved the respiratory and hemodynamic status. The reduced peak inspiratory pressures achieved with high frequency ventilation versus similar parameters of conventional mechanical ventilation may better maintain hemodynamics in patients with parenchymal lung disease and cavopulmonary connections. AD - J. Sanchez-de-Toledo, Cardiac Intensive Care Unit, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Passeig de la Vall d'Hebron 119-129, 08035 Barcelona, Spain AU - Sanchez-de-Toledo, J. AU - Vega, L. AU - Gil, L. AU - Balcells, J. AU - Abella, F. AU - Gran, F. DB - Embase IS - 8 KW - adult respiratory distress syndrome article cardiopulmonary hemodynamics case report cavopulmonary connection child female high frequency oscillatory ventilation high frequency ventilation human hypoxemia oxygenation positive end expiratory pressure preschool child LA - English M3 - Article N1 - L373746999 2014-08-22 2014-08-28 PY - 2014 SN - 1205-6626 SP - 2748-2757 ST - Use of high-frequency oscillatory ventilation in patients with acute respiratory distress syndrome and cavopulmonary connections: A case report T2 - Experimental and Clinical Cardiology TI - Use of high-frequency oscillatory ventilation in patients with acute respiratory distress syndrome and cavopulmonary connections: A case report UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L373746999 VL - 20 ID - 937 ER - TY - JOUR AB - BACKGROUND: Dexmedetomidine is a selective agonist of α2 receptors that induces hypnotic, sedative and mild analgesic effect. The aim of our study was to test the effects of dexmedetomidine in combination with opioids and benzodiazepines compared to benzodiazepine-opioids alone. METHODS: Arandomized controlled trial was conducted. Patients (children >30 days and <24 months undergoing correction of complex congenital heart diseases [CHD]) were randomized to receive 0.5 mcg/kg/h dexmedetomidine in addition to half dose of opioids and benzodiazepines (D-CASES) or standard dose opioids and benzodiazepines (CONTROLs). Primary outcome: to compare the duration of mechanical ventilation (MV) in D-CASEs and CONTROLs. Secondary outcomes: 1) the degree of sedation; 2) the onset of withdrawal symptoms; 3) the occurrence bradycardia and hypotension. RESULTS: Overall, 48 patients, 26 in CONTROLs group and 22 in D-CASEs group were ultimately included in the analysis after enrollment. The median duration of MV was 33.5 (16.7-75) hours in CONTROLs and 41.5 (23.7-71.2) hours in D-CASEs (P=0.51). Dexmedetomidine did not affect COMFORTand FLACCscales but it reduced the SOSscale in 15 D-CASEs vs. 11 CONTROLs (P=0.001). The incidence of bradycardia and hypotension and vasoactive support did not show significant differences in the two groups. CONCLUSIONS: Low dose of dexmedetomidine in combination with morphine and midazolam was safe in a high-risk cohort of CHD children after cardiac surgery and reduced the onset of withdrawal symptoms. However, it did not decrease MV time and the total amount of other sedative and analgesic drugs required in the post-operative period. AD - C. Garisto, Piazza S. Onofrio 4, Rome, Italy AU - Garisto, C. AU - Ricci, Z. AU - Tofani, L. AU - Benegni, S. AU - Pezzella, C. AU - Cogo, P. DB - Embase Medline DO - 10.23736/S0375-9393.18.12213-9 IS - 9 KW - NCT02375243 dexmedetomidine midazolam opiate aortic arch interruption aortic regurgitation article artificial ventilation bradycardia cardiopulmonary bypass clinical article comparative study congenital heart disease controlled study Fallot tetralogy female heart atrium septum defect heart surgery heart ventricle septum defect human hypoplastic left heart syndrome hypotension infant male mitral valve regurgitation newborn pulmonary artery aneurysm pulmonary valve atresia pulmonary valve stenosis pulmonary vein stenosis randomized controlled trial sedation systolic blood pressure withdrawal syndrome LA - English M3 - Article N1 - L623937971 2018-09-24 2018-09-27 PY - 2018 SN - 1827-1596 0375-9393 SP - 1053-1062 ST - Use of low-dose dexmedetomidine in combination with opioids and midazolam in pediatric cardiac surgical patients: Randomized controlled trial T2 - Minerva Anestesiologica TI - Use of low-dose dexmedetomidine in combination with opioids and midazolam in pediatric cardiac surgical patients: Randomized controlled trial UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L623937971 http://dx.doi.org/10.23736/S0375-9393.18.12213-9 VL - 84 ID - 668 ER - TY - JOUR AB - PURPOSE: Patients with Fontan circulation have increased cardio-respiratory risk during major spinal surgery. We report a case of severe scoliosis with a Cobb angle of 123.3° in a 16-year-old boy with Fontan circulation treated with single stage posterior segmental pedicle screw instrumentation and fusion. METHODS: Case report. RESULTS: The use pre-operative halo-ring traction for a duration of 6 weeks in this case lead to improvement in cobb angle from 123.3°, kyphotic angle 87.1° to cobb angle of 78.0°, kyphotic angle 57.2° (on bending and stress films). The operation was completed in 150 min, blood loss 1050 ml (25 ml/kg), and cell salvage of 490 ml. He was immediately extubated post correction, but monitored in ICU for a day. Total length of stay was 8 days without any perioperative morbidity or allogeneic blood transfusion. Final post-operative radiograph showed a cobb angle of 44.2°, kyphotic angle 22.8°. Follow up at 27 months showed solid union with no significant loss of correction. CONCLUSION: From this case experience, pre-operative halo traction is a useful surgical strategy in patients with Fontan circulation with severe kyposcoliosis to achieve adequate correction without additional osteotomies to minimize the risk of surgical correction. AD - Department of Orthopaedic Surgery, Faculty of Medicine, University of Malaya, 50603, Lembah Pantai, Kuala Lumpur, Malaysia. chrnat01@yahoo.com. Department of Orthopaedic Surgery, Faculty of Medicine, University of Malaya, 50603, Lembah Pantai, Kuala Lumpur, Malaysia. Department of Anaesthesiology, Faculty of Medicine, University of Malaya, 50603, Lembah Pantai, Kuala Lumpur, Malaysia. AN - 27028615 AU - Chan, C. Y. AU - Lim, C. Y. AU - Shahnaz Hasan, M. AU - Kwan, M. K. DA - May DB - PubMed DO - 10.1007/s00586-016-4538-9 DP - NLM ET - 2016/03/31 KW - Adolescent Fontan Procedure/*adverse effects Humans Kyphosis/pathology/surgery Male Pedicle Screws Postoperative Period Preoperative Care/methods Risk Factors Scoliosis/pathology/*surgery Spinal Fusion/adverse effects/*methods Traction/*methods Treatment Outcome *Fontan Circulation *Halo Traction *Scoliosis LA - eng N1 - 1432-0932 Chan, Chris Yin Wei Lim, Chiao Yee Shahnaz Hasan, M Kwan, Mun Keong Case Reports Journal Article Review Germany Eur Spine J. 2016 May;25 Suppl 1:245-50. doi: 10.1007/s00586-016-4538-9. Epub 2016 Mar 30. PY - 2016 SN - 0940-6719 SP - 245-50 ST - The use of pre-operative halo traction to minimize risk for correction of severe scoliosis in a patient with Fontan circulation: a case report and review of literature T2 - Eur Spine J TI - The use of pre-operative halo traction to minimize risk for correction of severe scoliosis in a patient with Fontan circulation: a case report and review of literature VL - 25 Suppl 1 ID - 349 ER - TY - JOUR AB - Purpose: Patients with Fontan circulation have increased cardio-respiratory risk during major spinal surgery. We report a case of severe scoliosis with a Cobb angle of 123.3° in a 16-year-old boy with Fontan circulation treated with single stage posterior segmental pedicle screw instrumentation and fusion. Methods: Case report. Results: The use pre-operative halo-ring traction for a duration of 6 weeks in this case lead to improvement in cobb angle from 123.3°, kyphotic angle 87.1° to cobb angle of 78.0°, kyphotic angle 57.2° (on bending and stress films). The operation was completed in 150 min, blood loss 1050 ml (25 ml/kg), and cell salvage of 490 ml. He was immediately extubated post correction, but monitored in ICU for a day. Total length of stay was 8 days without any perioperative morbidity or allogeneic blood transfusion. Final post-operative radiograph showed a cobb angle of 44.2°, kyphotic angle 22.8°. Follow up at 27 months showed solid union with no significant loss of correction. Conclusion: From this case experience, pre-operative halo traction is a useful surgical strategy in patients with Fontan circulation with severe kyposcoliosis to achieve adequate correction without additional osteotomies to minimize the risk of surgical correction. AD - C.Y.W. Chan, Department of Orthopaedic Surgery, Faculty of Medicine, University of Malaya, Lembah Pantai, Kuala Lumpur, Malaysia AU - Chan, C. Y. W. AU - Lim, C. Y. AU - Shahnaz Hasan, M. AU - Kwan, M. K. DB - Embase Medline DO - 10.1007/s00586-016-4538-9 KW - adolescent article bispectral index Blalock Taussig shunt blood transfusion bone graft case report Cobb angle computer assisted tomography echocardiography extubation follow up Fontan procedure fracture healing halo brace human intravenous anesthesia kyphosis length of stay male morbidity operation duration pedicle screw preoperative care priority journal scoliosis spine radiography traction therapy LA - English M3 - Article N1 - L609366405 2016-04-05 2016-06-14 PY - 2016 SN - 1432-0932 0940-6719 SP - 245-250 ST - The use of pre-operative halo traction to minimize risk for correction of severe scoliosis in a patient with Fontan circulation: a case report and review of literature T2 - European Spine Journal TI - The use of pre-operative halo traction to minimize risk for correction of severe scoliosis in a patient with Fontan circulation: a case report and review of literature UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L609366405 http://dx.doi.org/10.1007/s00586-016-4538-9 VL - 25 ID - 810 ER - TY - JOUR AB - Objective: To evaluate whether pulse oximetry can be used as a screening test to detect congenital heart disease (CHD) in otherwise well newborns before nursery discharge. Methods: Births at Lakeland Regional Medical Center (LRMC) who met inclusion criteria, had a single pulse oximeter reading before discharge. A normal reading was considered to be >94%. Abnormal readings were repeated on a different extremity and if still abnormal, an echocardiography was performed. The number of children with CHD and the number of echocardiograms were compared with the previous year at LRMC. Results: There were 2114 infants enrolled, 88 echocardiograms (3.8%) were performed, and 43 were abnormal (yield = 48.9%), of which 12 required management (13.6%). The control group consisted of 2851 births, producing 108 echocardiograms (3.8%), 42 of which were abnormal (38.9%), and 13 required management (12.0%). No comparisons were significant. During both years, 3 children with cyanotic CHD were born. Routine pulse oximetry failed to detect a child with total anomalous pulmonary venous return. Not a single, normal echocardiogram was generated by the study. Conclusion: Routine pulse oximetry was nearly 100% specific for detecting cyanotic CHD; as a result there was no increase in the number of echocardiograms. Routine pulse oximetry did detect a child who might not have been detected otherwise but failed to detect one that should have been detected. AD - 1600 Lakeland Hills Blvd, Lakeland, FL 33805, United States AU - Reich, J. D. AU - Miller, S. AU - Brogdon, B. AU - Casatelli, J. AU - Gompf, T. C. AU - Huhta, J. C. AU - Sullivan, K. DB - Scopus DO - 10.1067/mpd.2003.87 IS - 3 M3 - Article N1 - Cited By :73 Export Date: 15 June 2020 PY - 2003 SP - 268-272 ST - The use of pulse oximetryto detect congenital heart disease T2 - Journal of Pediatrics TI - The use of pulse oximetryto detect congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0037332410&doi=10.1067%2fmpd.2003.87&partnerID=40&md5=ae631888e18f8560dd896045928392e0 VL - 142 ID - 2263 ER - TY - JOUR AD - The Fetal Heart Program at the Children's Hospital of Philadelphia, 34th & Civic Center Boulevard, Philadelphia, PA, USA. AN - 104535320. Language: English. Entry Date: 20120727. Revision Date: 20150711. Publication Type: Journal Article. Journal Subset: Biomedical AU - Szwast, A. AU - Rychik, J. DB - ccm DP - EBSCOhost IS - 2 KW - Hypoplastic Left Heart Syndrome -- Surgery Prenatal Diagnosis -- Methods Quality of Life -- Psychosocial Factors Surgery, Reconstructive -- Methods Aortic Valve Stenosis -- Psychosocial Factors Aortic Valve Stenosis -- Surgery Female Genetic Counseling Hypoplastic Left Heart Syndrome -- Mortality Hypoplastic Left Heart Syndrome -- Psychosocial Factors Infant, Newborn Pregnancy Prenatal Diagnosis -- Equipment and Supplies Prenatal Diagnosis -- Trends Preoperative Care Surgery, Reconstructive -- Equipment and Supplies Surgery, Reconstructive -- Trends United States N1 - Europe; UK & Ireland. NLM UID: 101239345. PMID: NLM22413981. PY - 2012 SN - 1479-6678 SP - 215-225 ST - The use of reconstructive surgery to improve quality of life and survival in prenatal hypoplastic left heart syndrome T2 - Future Cardiology TI - The use of reconstructive surgery to improve quality of life and survival in prenatal hypoplastic left heart syndrome UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104535320&site=ehost-live&scope=site VL - 8 ID - 1577 ER - TY - JOUR AB - Objectives: Pediatric cardiac intensive care continues to evolve, with rapid advances in knowledge and improvement in clinical outcomes. In the past, the Board of Directors of the Pediatric Cardiac Intensive Care Society created and subsequently updated a list of sentinel references focused on the care of critically ill children with congenital and acquired heart disease. The objective of this article is to provide clinicians with a compilation and brief summary of updated and useful references that have been published since 2012. Data Selection: Pediatric Cardiac Intensive Care Society members were solicited via a survey sent out between March 20, 2017, and April 28, 2017, to provide important references that have impacted clinical care. The survey was sent to approximately 523 members. Responses were received from 45 members, of which some included multiple references. Data Extraction: Following review of the list of references, and removing editorials, references were compiled by the first and last author. The final list was submitted to members of the society's Research Briefs Committee, who ranked each publication. Data Synthesis: Rankings were compiled and the references with the highest scores included. Research Briefs Committee members ranked the articles from 1 to 3, with one being highly relevant and should be included and 3 being less important and should be excluded. Averages were computed, and the top articles included in this article. The first (K.C.U.) and last author (K.M.G.) reviewed and developed summaries of each article. Conclusions: This article contains a compilation of useful references for the critical care of children with congenital and acquired heart disease published in the last 5 years. In conjunction with the prior version of this update in 2012, this article may be used as an educational reference in pediatric cardiac intensive care. AD - K.M. Gist, Division of Pediatric Cardiology, Department of Pediatrics, University of Colorado Anschutz Medical Campus School of Medicine, Children's Hospital Colorado, Aurora, CO, United States AU - Uzark, K. C. AU - Costello, J. M. AU - DeSena, H. C. AU - Thiagajaran, R. AU - Smith-Parrish, M. AU - Gist, K. M. DB - Embase Medline DO - 10.1097/PCC.0000000000001523 IS - 6 KW - acquired heart disease acute kidney failure advanced practice provider article bleeding cardiopulmonary function childhood disease congenital heart disease coronary care unit critically ill patient extracorporeal oxygenation extubation glucose homeostasis heart arrest heart single ventricle human hypervolemia intensive care nursing nutrition palliative therapy pediatric cardiology pediatric intensive care unit perioperative period postoperative care preoperative care priority journal quality of life thrombosis thrombosis prevention LA - English M3 - Article N1 - L624066132 2018-10-02 2018-10-08 PY - 2018 SN - 1947-3893 1529-7535 SP - 553-563 ST - Useful References in Pediatric Cardiac Intensive Care: The 2017 Update T2 - Pediatric Critical Care Medicine TI - Useful References in Pediatric Cardiac Intensive Care: The 2017 Update UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L624066132 http://dx.doi.org/10.1097/PCC.0000000000001523 VL - 19 ID - 683 ER - TY - JOUR AB - We report a case of a 23-year-old male with failing Fontan circulation who was taken to the catheterization lab to better evaluate the Fontan circulation and hemodynamics. Catheterization revealed arteriovenous malformations exclusively present in the right lung leading to the consideration of placing stents to direct the inferior vena cava flow through the Fontan circuit to the right pulmonary artery (RPA), thus increasing the RPA concentration of the hepatic factor. However, comprehensive 4D-Flow MRI analyses indicated sufficient distribution of the hepatic flow between branch pulmonary arteries, and consequently no further invasive intervention to redirect hepatic flow was performed. © 2019, Springer Science+Business Media, LLC, part of Springer Nature. AD - Division of Cardiology, Heart Institute, Children’s Hospital Colorado, University of Colorado Denver, Anschutz Medical Campus, 13123 E 16th Ave, Aurora, CO 80045-2560, United States Section of Congenital Heart Surgery, Heart Institute, Children’s Hospital Colorado, University of Colorado Denver, Anschutz Medical Campus, Aurora, CO, United States AU - McLennan, D. AU - Schäfer, M. AU - Mitchell, M. B. AU - Morgan, G. J. AU - Ivy, D. AU - Barker, A. J. AU - Jacobsen, R. DB - Scopus DO - 10.1007/s00246-019-02101-7 IS - 5 KW - Flow Fontan circulation MRI M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2019 SP - 1093-1096 ST - Usefulness of 4D-Flow MRI in Mapping Flow Distribution Through Failing Fontan Circulation Prior to Cardiac Intervention T2 - Pediatric Cardiology TI - Usefulness of 4D-Flow MRI in Mapping Flow Distribution Through Failing Fontan Circulation Prior to Cardiac Intervention UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85064505028&doi=10.1007%2fs00246-019-02101-7&partnerID=40&md5=08000347db7e538c6a46dc3ddde35949 VL - 40 ID - 1747 ER - TY - JOUR AB - After Fontan operation, patients are limited in increasing cardiac output and in exercise capacity. This has been related to impaired preload or other factors leading to decreased global ventricular performance with stress. To study these factors, the stress responses of functionally univentricular hearts were assessed at rest and during low-dose dobutamine stress using cardiovascular magnetic resonance imaging. Thirty-two patients after Fontan completion at young age were included (27 with total cavopulmonary connection, 5 with atriopulmonary connection; mean age 13.3 years, range 7.5 to 22.2; 23 male patients; median follow-up after Fontan operation 8.1 years, range 5.2 to 17.8). A multiphase short-axis stack of 10 to 12 contiguous slices of the systemic ventricle was obtained at rest and during low-dose dobutamine stress cardiovascular magnetic resonance imaging (maximum 7.5 microg/kg/min). With stress-testing, heart rate, ejection fraction, and cardiac index increased adequately (p <0.001). There was an abnormal decrease in end-diastolic volume and an adequate decrease in end-systolic volume (p <0.001). Stroke volume did not change with stress testing (p = 0.15). At rest, dominant left ventricles had higher ejection fractions than dominant right ventricles (p = 0.01), but this difference disappeared with stress testing. In conclusion, a functionally univentricular heart after Fontan completion at young age has an adequate increase in ejection fraction with beta-adrenergic stimulation. However, as a result of impaired preload with stress, cardiac output can be increased only by increasing heart rate. AD - Department of Pediatric Cardiology, Erasmus Medical Center, Rotterdam, The Netherlands. AN - 18489947 AU - Robbers-Visser, D. AU - Jan Ten Harkel, D. AU - Kapusta, L. AU - Strengers, J. L. AU - Dalinghaus, M. AU - Meijboom, F. J. AU - Pattynama, P. M. AU - Bogers, A. J. AU - Helbing, W. A. DA - Jun 1 DB - PubMed DO - 10.1016/j.amjcard.2008.01.050 DP - NLM ET - 2008/05/21 IS - 11 KW - Adolescent Adult Cardiotonic Agents/administration & dosage Child Cross-Sectional Studies Dobutamine/*administration & dosage Dose-Response Relationship, Drug Echocardiography, Stress/*methods Female Follow-Up Studies Fontan Procedure/*methods Heart Defects, Congenital/diagnosis/physiopathology/*surgery Heart Rate/physiology Heart Ventricles/*abnormalities/diagnostic imaging/physiopathology Humans Injections, Intravenous Magnetic Resonance Imaging/*methods Male Myocardial Contraction/*physiology Prognosis Reproducibility of Results Retrospective Studies Severity of Illness Index LA - eng N1 - Robbers-Visser, Daniëlle Jan Ten Harkel, Derk Kapusta, Livia Strengers, Jan L Dalinghaus, Michiel Meijboom, Folkert J Pattynama, Peter M Bogers, Ad J Helbing, Willem A Comparative Study Journal Article Multicenter Study United States Am J Cardiol. 2008 Jun 1;101(11):1657-62. doi: 10.1016/j.amjcard.2008.01.050. Epub 2008 Apr 8. PY - 2008 SN - 0002-9149 (Print) 0002-9149 SP - 1657-62 ST - Usefulness of cardiac magnetic resonance imaging combined with low-dose dobutamine stress to detect an abnormal ventricular stress response in children and young adults after fontan operation at young age T2 - Am J Cardiol TI - Usefulness of cardiac magnetic resonance imaging combined with low-dose dobutamine stress to detect an abnormal ventricular stress response in children and young adults after fontan operation at young age VL - 101 ID - 293 ER - TY - JOUR AB - After Fontan operation, patients are limited in increasing cardiac output and in exercise capacity. This has been related to impaired preload or other factors leading to decreased global ventricular performance with stress. To study these factors, the stress responses of functionally univentricular hearts were assessed at rest and during low-dose dobutamine stress using cardiovascular magnetic resonance imaging. Thirty-two patients after Fontan completion at young age were included (27 with total cavopulmonary connection, 5 with atriopulmonary connection; mean age 13.3 years, range 7.5 to 22.2; 23 male patients; median follow-up after Fontan operation 8.1 years, range 5.2 to 17.8). A multiphase short-axis stack of 10 to 12 contiguous slices of the systemic ventricle was obtained at rest and during low-dose dobutamine stress cardiovascular magnetic resonance imaging (maximum 7.5 microg/kg/min). With stress-testing, heart rate, ejection fraction, and cardiac index increased adequately (p <0.001). There was an abnormal decrease in end-diastolic volume and an adequate decrease in end-systolic volume (p <0.001). Stroke volume did not change with stress testing (p = 0.15). At rest, dominant left ventricles had higher ejection fractions than dominant right ventricles (p = 0.01), but this difference disappeared with stress testing. In conclusion, a functionally univentricular heart after Fontan completion at young age has an adequate increase in ejection fraction with beta-adrenergic stimulation. However, as a result of impaired preload with stress, cardiac output can be increased only by increasing heart rate. AD - Department of Pediatric Cardiology, Erasmus Medical Center, Rotterdam, The Netherlands Department of Pediatric Cardiology, Erasmus Medical Center, Sophia Children's Hospital, Rotterdam, The Netherlands AN - 105783417. Language: English. Entry Date: 20080808. Revision Date: 20170413. Publication Type: journal article AU - Robbers-Visser, D. AU - Jan Ten Harkel, D. AU - Kapusta, L. AU - Strengers, J. L. AU - Dalinghaus, M. AU - Meijboom, F. J. AU - Pattynama, P. M. AU - Bogers, A. J. AU - Helbing, W. A. AU - Robbers-Visser, Daniëlle AU - Jan Ten Harkel, Derk AU - Kapusta, Livia AU - Strengers, Jan L. AU - Dalinghaus, Michiel AU - Meijboom, Folkert J. AU - Pattynama, Peter M. AU - Bogers, Ad J. AU - Helbing, Willem A. DB - ccm DO - 10.1016/j.amjcard.2008.01.050 DP - EBSCOhost IS - 11 KW - Heart Defects, Congenital -- Surgery Heart Surgery -- Adverse Effects -- In Infancy and Childhood Outcomes Research Cardiac Output Child, Preschool Demography Descriptive Statistics Dobutamine Equipment and Supplies Exercise Test, Cardiopulmonary Exercise Tolerance Linear Regression Magnetic Resonance Imaging Men T-Tests Univariate Statistics Human N1 - research; tables/charts. Journal Subset: Biomedical; Peer Reviewed; USA. NLM UID: 0207277. PMID: NLM18489947. PY - 2008 SN - 0002-9149 SP - 1657-1662 ST - Usefulness of cardiac magnetic resonance imaging combined with low-dose dobutamine stress to detect an abnormal ventricular stress response in children and young adults after fontan operation at young age T2 - American Journal of Cardiology TI - Usefulness of cardiac magnetic resonance imaging combined with low-dose dobutamine stress to detect an abnormal ventricular stress response in children and young adults after fontan operation at young age UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105783417&site=ehost-live&scope=site VL - 101 ID - 1585 ER - TY - JOUR AB - After the Fontan operation, patients are at a substantial risk of the development of impaired functional health status. Few early markers of suboptimal outcomes have been identified. We sought to assess the association between peripheral vascular function and functional health status in Fontan-palliated patients. Asymptomatic Fontan patients (n = 51) and age- and gender-matched healthy controls (n = 22) underwent endothelial pulse amplitude testing using a noninvasive fingertip peripheral arterial tonometry (PAT) device. Raw data were transformed into the PAT ratio, an established marker of vascular function. Cardiopulmonary exercise testing was performed using the Bruce protocol. In the Fontan cohort, 94% of patients were New York Heart Association functional class I and 88% had a B-type natriuretic peptide level of <50 pg/ml. The baseline pulse amplitude, a measure that reflects the arterial tone at rest, was greater in the Fontan patients than in the controls (median 2.74, interquartile range 1.96 to 4.13 vs median 1.86, interquartile range 1.14 to 2.79, p = 0.03). The PAT ratio, a measure of reactive hyperemia, was lower in Fontan patients (median 0.17, interquartile range -0.04 to 0.44, vs median 0.50, interquartile range 0.27 to 0.74, p = 0.002). The key parameters of exercise performance, including peak oxygen consumption (median 28.8 ml/kg/min, interquartile range 25.6 to 33.2 vs median 45.5 ml/kg/min, interquartile range 41.7 to 49.9, p <0.0001) and peak work (median 192 W, interquartile range 150 to 246 vs median 330, interquartile range 209 to 402 W, p <0.0001), were lower in Fontan patients than in the controls. The PAT ratio correlated with the peak oxygen consumption (r = 0.28, p = 0.02) and peak work (r = 0.26, p = 0.03). In conclusion, in an asymptomatic Fontan population, there is evidence of reduced basal peripheral arterial tone and vasodilator response, suggesting dysfunction of the endothelium-derived nitric oxide pathway. Vasodilator function appears to correlate with exercise performance. AD - Division of Cardiology, Department of Pediatrics, C. S. Mott Children's Hospital, University of Michigan Health System, Ann Arbor, USA. bryan.goldstein@cchmc.org AN - 21600541 AU - Goldstein, B. H. AU - Golbus, J. R. AU - Sandelin, A. M. AU - Warnke, N. AU - Gooding, L. AU - King, K. K. AU - Donohue, J. E. AU - Gurney, J. G. AU - Goldberg, C. S. AU - Rocchini, A. P. AU - Charpie, J. R. DA - Aug 1 DB - PubMed DO - 10.1016/j.amjcard.2011.03.064 DP - NLM ET - 2011/05/24 IS - 3 KW - Adolescent Adult Blood Pressure/physiology Child Child, Preschool *Disability Evaluation Echocardiography, Doppler Endothelium, Vascular/physiopathology *Exercise Test Female Fingers/blood supply *Fontan Procedure *Health Status Humans Hyperemia/physiopathology Male *Manometry Nitric Oxide/physiology Oxygen/blood Peripheral Vascular Diseases/*diagnosis/physiopathology Postoperative Complications/*diagnosis/physiopathology Prognosis Prospective Studies Pulse Quality of Life Reference Values Vascular Resistance/*physiology Young Adult LA - eng N1 - 1879-1913 Goldstein, Bryan H Golbus, Jessica R Sandelin, Angela M Warnke, Nicole Gooding, Lindsay King, Karen K Donohue, Janet E Gurney, James G Goldberg, Caren S Rocchini, Albert P Charpie, John R Journal Article Research Support, Non-U.S. Gov't United States Am J Cardiol. 2011 Aug 1;108(3):428-34. doi: 10.1016/j.amjcard.2011.03.064. Epub 2011 May 19. PY - 2011 SN - 0002-9149 SP - 428-34 ST - Usefulness of peripheral vascular function to predict functional health status in patients with Fontan circulation T2 - Am J Cardiol TI - Usefulness of peripheral vascular function to predict functional health status in patients with Fontan circulation VL - 108 ID - 352 ER - TY - JOUR AB - Background: Studies on associations between periconceptional cannabis exposure and birth defects have mainly relied on self-reported exposure. Therefore, the results may be biased due to under-reporting of the exposure. The aim of this study was to quantify the potential effects of this form of exposure misclassification. Methods: Using multivariable logistic regression, we re-analysed associations between periconceptional cannabis use and 20 specific birth defects using data from the National Birth Defects Prevention Study from 1997-2005 for 13 859 case infants and 6556 control infants. For seven birth defects, we implemented four Bayesian models based on various assumptions concerning the sensitivity of self-reported cannabis use to estimate odds ratios (ORs), adjusted for confounding and under-reporting of the exposure. We used information on sensitivity of selfreported cannabis use from the literature for prior assumptions. Results: The results unadjusted for under-reporting of the exposure showed an association between cannabis use and anencephaly (posterior OR 1.9 [95% credible interval (CRI) 1.1, 3.2]) which persisted after adjustment for potential exposure misclassification. Initially, no statistically significant associations were observed between cannabis use and the other birth defect categories studied. Although adjustment for under-reporting did not notably change these effect estimates, cannabis use was associated with esophageal atresia (posterior OR 1.7 [95% CRI 1.0, 2.9]), diaphragmatic hernia (posterior OR 1.8 [95% CRI 1.1, 3.0]), and gastroschisis (posterior OR 1.7 [95% CRI 1.2, 2.3]) after correction for exposure misclassification. Conclusions: Under-reporting of the exposure may have obscured some cannabis-birth defect associations in previous studies. However, the resulting bias is likely to be limited. AD - J. Reefhuis, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, 1600 Clifton Road N.E. MS E-86, Atlanta, GA, United States AU - Van Gelder, M. M. H. J. AU - Donders, A. R. T. AU - Devine, O. AU - Roeleveld, N. AU - Reefhuis, J. DB - Embase Medline DO - 10.1111/ppe.12140 IS - 5 KW - cannabis adult anencephalus anus atresia aortic coarctation article Bayes theorem congenital malformation cannabis use cleft lip palate cleft palate controlled study craniofacial synostosis diaphragm hernia disease association esophagus atresia Fallot tetralogy female gastroschisis great vessels transposition heart atrium septum defect heart ventricle septum defect human hypoplastic left heart syndrome hypospadias limb defect major clinical study microtia newborn population based case control study pregnancy pulmonary valve stenosis quantitative study self report sensitivity and specificity spinal dysraphism LA - English M3 - Article N1 - L603834715 2015-04-23 2019-10-31 PY - 2014 SN - 1365-3016 0269-5022 SP - 424-433 ST - Using bayesian models to assess the effects of under-reporting of cannabis use on the association with birth defects, national birth defects prevention study, 1997-2005 T2 - Paediatric and Perinatal Epidemiology TI - Using bayesian models to assess the effects of under-reporting of cannabis use on the association with birth defects, national birth defects prevention study, 1997-2005 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L603834715 http://dx.doi.org/10.1111/ppe.12140 VL - 28 ID - 974 ER - TY - JOUR AB - OBJECTIVE: Children with hypoplastic left heart syndrome are at a risk for neurodevelopmental delays. Current guidelines recommend systematic evaluation and management of neurodevelopmental outcomes with referral for early intervention services. The Single Ventricle Reconstruction Trial represents the largest cohort of children with hypoplastic left heart syndrome ever assembled. Data on life events and resource utilisation have been collected annually. We sought to determine the type and prevalence of early intervention services used from age 1 to 4 years and factors associated with utilisation of services. METHODS: Data from 14-month neurodevelopmental assessment and annual medical history forms were used. We assessed the impact of social risk and geographic differences. Fisher exact tests and logistic regression were used to evaluate associations. RESULTS: Annual medical history forms were available for 302 of 314 children. Greater than half of the children (52-69%) were not receiving services at any age assessed, whereas 20-32% were receiving two or more therapies each year. Utilisation was significantly lower in year 4 (31%) compared with years 1-3 (with a range from 40 to 48%) (p<0.001). Social risk factors were not associated with the use of services at any age but there were significant geographic differences. Significant delay was reported by parents in 18-43% of children at ages 3 and 4. CONCLUSION: Despite significant neurodevelopmental delays, early intervention service utilisation was low in this cohort. As survival has improved for children with hypoplastic left heart syndrome, attention must shift to strategies to optimise developmental outcomes, including enrolment in early intervention when merited. AD - 1Children's Hospital of Wisconsin,Herma Heart Center, Milwaukee,Wisconsin,United States of America. 2New England Research Institutes, Inc.,Watertown,Massachusetts,United States of America. 3Nemours/Alfred I. duPont Hospital for Children,Department of Pediatrics, Wilmington,Delaware,United States of America. 4The Hospital for Sick Children,Department of Pediatrics, Toronto,Ontario,Canada. 5Children's Hospital Los Angeles,Department of Pediatrics, Los Angeles,California,United States of America. 6Primary Children's Hospital,Department of Pediatrics, Salt Lake City,Utah,United States of America. 7Children's Healthcare of Atlanta,Department of Pediatrics, Atlanta,Georgia,United States of America. 8Children's Hospital of Philadelphia,Department of Pediatrics, Philadelphia,Pennsylvania,United States of America. 9University of Michigan Health System,Department of Pediatrics, Ann Arbor,Michigan,United States of America. 10Boston Children's Hospital,Department of Pediatrics, Boston,Massachusetts,United States of America. 11Medical College of Wisconsin,Department of Pediatrics, Milwaukee,Wisconsin,United States of America. 12Ann & Robert H. Lurie Children's Hospital,Department of Pediatrics, Chicago,Illinois,United States of America. 13Duke University Hospital,Department of Pediatrics, Durham,North Carolina,United States of America. 14Department of Pediatrics,New York State Psychiatric Institute,Department of Developmental Neuroscience,Morgan Stanley Children's Hospital of New York-Presbyterian,New York,New York,United States of America. 15Johns Hopkins All Children's Hospital,Johns Hopkins University,Department of Pediatrics, Saint Petersburg,Florida and Baltimore,Pediatric Heart Network, Maryland,United States of America. 16Medical University of South Carolina,Department of Pediatrics, Charleston,South Carolina,United States of America. 17National Heart, Lung, and Blood Institute,Bethesda,Pediatric Heart Network, Maryland,United States of America. AN - 28847329 AU - Mussatto, K. A. AU - Hollenbeck-Pringle, D. AU - Trachtenberg, F. AU - Sood, E. AU - Sananes, R. AU - Pike, N. A. AU - Lambert, L. M. AU - Mahle, W. T. AU - Goldberg, D. J. AU - Goldberg, C. S. AU - Dunbar-Masterson, C. AU - Otto, M. AU - Marino, B. S. AU - Bartle, B. H. AU - Williams, I. A. AU - Jacobs, J. P. AU - Zyblewski, S. C. AU - Pemberton, V. L. C2 - PMC5815511 C6 - NIHMS920009 DA - Jan DB - PubMed DO - 10.1017/s104795111700169x DP - NLM ET - 2017/08/30 IS - 1 KW - Cardiac Surgical Procedures Child Child, Preschool Early Medical Intervention/*statistics & numerical data Female Heart Defects, Congenital/*psychology/*surgery Heart Ventricles/*abnormalities/physiopathology Humans Infant Logistic Models Male Neurodevelopmental Disorders/*physiopathology Neuropsychological Tests Prospective Studies Risk Factors Chd early intervention neurodevelopmental disorders LA - eng N1 - 1467-1107 Mussatto, Kathleen A Hollenbeck-Pringle, Danielle Trachtenberg, Felicia Sood, Erica Sananes, Renee Pike, Nancy A Lambert, Linda M Mahle, William T Goldberg, David J Goldberg, Caren S Dunbar-Masterson, Carolyn Otto, Michelle Marino, Bradley S Bartle, Bronwyn H Williams, Ismee A Jacobs, Jeffrey P Zyblewski, Sinai C Pemberton, Victoria L UG1 HL135646/HL/NHLBI NIH HHS/United States U10 HL109781/HL/NHLBI NIH HHS/United States UG1 HL135685/HL/NHLBI NIH HHS/United States U01 HL068269/HL/NHLBI NIH HHS/United States U01 HL068279/HL/NHLBI NIH HHS/United States U01 HL068290/HL/NHLBI NIH HHS/United States U24 HL135691/HL/NHLBI NIH HHS/United States U01 HL068288/HL/NHLBI NIH HHS/United States U10 HL068270/HL/NHLBI NIH HHS/United States U01 HL068281/HL/NHLBI NIH HHS/United States U01 HL068270/HL/NHLBI NIH HHS/United States U10 HL109737/HL/NHLBI NIH HHS/United States U01 HL068292/HL/NHLBI NIH HHS/United States U01 HL085057/HL/NHLBI NIH HHS/United States UG1 HL135678/HL/NHLBI NIH HHS/United States U01 HL068285/HL/NHLBI NIH HHS/United States Journal Article Cardiol Young. 2018 Jan;28(1):126-133. doi: 10.1017/S104795111700169X. Epub 2017 Aug 29. PY - 2018 SN - 1047-9511 (Print) 1047-9511 SP - 126-133 ST - Utilisation of early intervention services in young children with hypoplastic left heart syndrome T2 - Cardiol Young TI - Utilisation of early intervention services in young children with hypoplastic left heart syndrome VL - 28 ID - 50 ER - TY - JOUR AB - The most common modes of medical education for congenital heart disease (CHD) rely heavily on 2-dimensional imaging. Three-dimensional (3D) printing technology allows for the creation of physical cardiac models that can be used for teaching trainees. 3D printed cardiac models were created for the following lesions: pulmonic stenosis, atrial septal defect, tetralogy of Fallot, d-transposition of the great arteries, coarctation of the aorta, and hypoplastic left heart syndrome. Medical students participated in a workshop consisting of different teaching stations. At the 3D printed station, students completed a pre- and post-intervention survey assessing their knowledge of each cardiac lesion on a Likert scale. Students were asked to rank the educational benefit of each modality. Linear regression was utilized to assess the correlation of the mean increase in knowledge with increasing complexity of CHD based on the Aristotle Basic Complexity Level. 45 medical students attended the CHD workshop. Students’ knowledge significantly improved for every lesion (p < 0.001). A strong positive correlation was found between mean increase in knowledge and increasing complexity of CHD (R2 = 0.73, p < 0.05). The 3D printed models, pathology specimens and spoken explanation were found to be the most helpful modalities. Students “strongly agreed” the 3D printed models made them more confident in explaining congenital cardiac anatomy to others (mean = 4.23, ± 0.69), and that they recommend the use of 3D models for future educational sessions (mean = 4.40, ± 0.69). 3D printed cardiac models should be included in medical student education particularly for lesions that require a complex understanding of spatial relationships. AD - K.M. Farooqi, Division of Cardiology, Department of Pediatrics, Columbia University Irving Medical Center, 3959 Broadway, CHN-2, New York, NY, United States AU - Smerling, J. AU - Marboe, C. C. AU - Lefkowitch, J. H. AU - Pavlicova, M. AU - Bacha, E. AU - Einstein, A. J. AU - Naka, Y. AU - Glickstein, J. AU - Farooqi, K. M. DB - Embase Medline DO - 10.1007/s00246-019-02146-8 IS - 6 KW - adult aortic coarctation article attitude to health biological model congenital heart disease disease severity equipment and supplies utilization Fallot tetralogy female great vessels transposition heart atrium septum defect human hypoplastic left heart syndrome male medical education medical student pulmonary valve stenosis spatial analysis teaching three dimensional printing two dimensional echocardiography workshop young adult LA - English M3 - Article N1 - L628341832 2019-07-05 2019-08-26 PY - 2019 SN - 1432-1971 0172-0643 SP - 1258-1265 ST - Utility of 3D Printed Cardiac Models for Medical Student Education in Congenital Heart Disease: Across a Spectrum of Disease Severity T2 - Pediatric Cardiology TI - Utility of 3D Printed Cardiac Models for Medical Student Education in Congenital Heart Disease: Across a Spectrum of Disease Severity UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L628341832 http://dx.doi.org/10.1007/s00246-019-02146-8 VL - 40 ID - 603 ER - TY - JOUR AB - Congenital heart disease is the leading cause of stroke in children. Warfarin therapy can be difficult to manage safely in this population because of its narrow therapeutic index, multiple drug and dietary interactions, small patient size, high-risk cardiac indications, and lack of data to support anticoagulation recommendations. We sought to describe our institution’s effort to develop a dedicated cardiac anticoagulation service to address the special needs of this population and to review the literature. In 2009, in response to Joint Commission National Patient Safety Goals for Anticoagulation, Boston Children’s Hospital created a dedicated pediatric Cardiac Anticoagulation Monitoring Program (CAMP). The primary purpose was to provide centralized management of outpatient anticoagulation to cardiac patients, to serve as a disease-specific resource to families and providers, and to devise strategies to evolve clinical care with rapidly emerging trends in anticoagulation care. Over 5 years the CAMP Service, staffed by a primary pediatric cardiology attending, a full-time nurse practitioner, and administrative assistant with dedicated support from pharmacy and nutrition, has enrolled over 240 patients ranging in age from 5 months to 55 years. The most common indications include a prosthetic valve (34 %), Fontan prophylaxis (20 %), atrial arrhythmias (11 %), cardiomyopathy (10 %), Kawasaki disease (7 %), and a ventricular assist device (2 %). A patient-centered multi-disciplinary cardiac anticoagulation clinic was created in 2012. Overall program international normalized ratio (INR) time in therapeutic range (TTR) is favorable at 67 % (81 % with a 0.2 margin) and has improved steadily over 5 years. Pediatric-specific guidelines for VKOR1 and CYP2C9 pharmacogenomics testing, procedural bridging with enoxaparin, novel anticoagulant use, and quality metrics have been developed. Program satisfaction is rated highly among families and providers. A dedicated pediatric cardiac anticoagulation program offers a safe and effective strategy to standardize anticoagulation care for pediatric cardiology patients, is associated with high patient and provider satisfaction, and is capable of evolving care strategies with emerging trends in anticoagulation. AD - C.S. Almond, Division of Pediatric Cardiology, Stanford University, The Lucile Packard Children’s Hospital, 750 Welch Road Suite 325, Palo Alto, CA, United States AU - Murray, J. M. AU - Hellinger, A. AU - Dionne, R. AU - Brown, L. AU - Galvin, R. AU - Griggs, S. AU - Mittler, K. AU - Harney, K. AU - Manzi, S. AU - VanderPluym, C. AU - Baker, A. AU - O’Brien, P. AU - O’Connell, C. AU - Almond, C. S. DB - Embase DO - 10.1007/s00246-014-1089-x IS - 4 KW - cytochrome P450 2C9 enoxaparin anticoagulation article cardiac patient cardiomyopathy child child health care clinical medicine dedicated pediatric vardiac anticoagulation program health care utilization health program heart atrium arrhythmia heart valve prosthesis human international normalized ratio mucocutaneous lymph node syndrome nurse practitioner nutrition outpatient care patient satisfaction pediatric cardiology pediatric hospital pharmacogenomics practice guideline program development standardization United States ventricular assist device LA - English M3 - Article N1 - L601298436 2015-01-20 2015-06-29 PY - 2015 SN - 1432-1971 0172-0643 SP - 842-850 ST - Utility of a Dedicated Pediatric Cardiac Anticoagulation Program: The Boston Children’s Hospital Experience T2 - Pediatric Cardiology TI - Utility of a Dedicated Pediatric Cardiac Anticoagulation Program: The Boston Children’s Hospital Experience UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L601298436 http://dx.doi.org/10.1007/s00246-014-1089-x VL - 36 ID - 881 ER - TY - JOUR AB - Context: Current guidelines recommend close monitoring of electrolytes in transgender patients using spironolactone given the risk of hyperkalemia from mineralocorticoid antagonism. In patients taking spironolactone for other conditions, the rate of hyperkalemia is low, and the utility of frequent monitoring has been questioned. Objective: We hypothesized that the rate of hyperkalemia in gender-diverse adolescents taking spironolactone is low and, when present, clinically insignificant. Design and Outcomes: A retrospective chart review of adolescents seen in a specialty gender clinic at a tertiary care pediatric hospital over 10 years identified patients prescribed spironolactone for gender transition. Study outcomes were the incidence of hyperkalemia, defined as serum potassium concentration .5.0 mmol/L, and the relationship between potassium levels and spironolactone dose and duration. Results: Records were reviewed for 85 subjects with a mean 6 SD age of 16.6 6 1.7 years. There were a total of 269 potassium measurements (80 prior to spironolactone initiation and 189 during spironolactone treatment). Six potassium measurements in five subjects were .5.0 mmol/L, indicating a rate of hyperkalemia of 2.2%. None of the subjects had symptoms of hyperkalemia, and all elevated measurements were normal when repeated. Only one subject discontinued spironolactone after an elevated potassium measurement. There was no relationship between hyperkalemia and spironolactone dose. Potassium measurements decreased with increasing treatment duration. Conclusions: Hyperkalemia in patients taking spironolactone for gender transition is rare and when present is transient and asymptomatic. In the absence of other medical comorbidities, routine electrolyte monitoring in this population may be unnecessary. AD - Y.-M. Chan, Division of Endocrinology, Department of Pediatrics, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, United States AU - Millington, K. AU - Liu, E. AU - Chan, Y. M. DB - Embase DO - 10.1210/js.2019-00030 IS - 5 KW - potassium spironolactone adolescent article asthma cohort analysis comorbidity congestive heart failure drug withdrawal eosinophilic esophagitis female gender dysphoria gender identity hemolysis human hyperkalemia hypertension hypoplastic left heart syndrome low drug dose macrophage activation syndrome major clinical study male medical record review migraine narcolepsy patient monitoring potassium blood level priority journal retrospective study risk factor transgender treatment duration LA - English M3 - Article N1 - L628380644 2019-07-09 2019-07-11 PY - 2019 SN - 2472-1972 SP - 1031-1038 ST - The utility of potassium monitoring in gender-diverse adolescents taking spironolactone T2 - Journal of the Endocrine Society TI - The utility of potassium monitoring in gender-diverse adolescents taking spironolactone UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L628380644 http://dx.doi.org/10.1210/js.2019-00030 VL - 3 ID - 617 ER - TY - JOUR AB - Objectives: Extracorporeal life support for neonatal respiratory failure has decreased, but utilization and outcome of cardiac extracorporeal life support are not well characterized. Among neonates born 1996-2000, our objects were to evaluate changes in utilization and outcome of cardiac extracorporeal life support and characterize correlates of survival. Design: Retrospective analysis of Extracorporeal Life Support Organization Registry data. Setting: Intensive care units participating in the ELSO registry. Patients: Patients placed on extracorporeal life support for center-specified "cardiac support" at ≤30 days of age from 1996 to 2000. Patients with hypoplastic left heart syndrome were also analyzed separately. Interventions: None. Measurements and Main Results: Patient characteristics and correlates of survival to discharge or transfer were analyzed by chi-square, Student's t-test, and logistic regression analysis. Neonates placed on cardiac extracorporeal life support increased from 112 in 1996 to 200 in 2000 (total n = 740). Overall survival was 34.2%: 28% for hypoplastic left heart syndrome and 35.4% for nonhypoplastic left heart syndrome. For the overall group, no significant correlations were found between survival and year on extracorporeal life support, multiple runs, or diagnosis of hypoplastic left heart syndrome. Diagnoses of transposition of the great arteries (p = .03) or persistent pulmonary hypertension of the neonate (p = .004) and extracorporeal life support at <3 days (p = .003) were associated with higher survival. Survivors had fewer mean extracorporeal life support hours (125.5 ± 121.4 vs. 159.0 ± 127.6, p = .0006). Logistic regression confirmed significant bivariate findings. A total of 118 hypoplastic left heart syndrome patients were reported from 1996 to 2000. Extracorporeal life support at >15 days was associated with improved survival among hypoplastic left heart syndrome patients (p = .03), and survivors had fewer mean extracorporeal life support hours (89.3 ± 52.3 vs. 147.5 ± 129.7, p = .015). Logistic regression showed that only greater number of hours on extracorporeal life support was independently associated with nonsurvival. Conclusions: Neonatal cardiac extracorporeal life support use increased substantially from 1996 to 2000, with survival to discharge or transfer in more than one third of patients. Hypoplastic left heart syndrome was not associated with nonsurvival. Fewer hours on extracorporeal life support, diagnoses of persistent pulmonary hypertension of the neonate and transposition of the great arteries, and extracorporeal life support at <3 days were associated with survival. Copyright © 2005 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies. AD - Division of Neonatal and Developmental Medicine, Stanford University School of Medicine, Palo Alto, CA, United States Division of Neonatology, Mayo Clinic, Rochester, MN, United States University of Michigan, ELSO Registry, Ann Arbor, MI, United States Division of Neonatal and Developmental Medicine, Stanford University School of Medicine, 750 Welch Road, Palo Alto, CA 94304, United States AU - Hintz, S. R. AU - Benitz, W. E. AU - Colby, C. E. AU - Sheehan, A. M. AU - Rycus, P. AU - Van Meurs, K. P. DB - Scopus DO - 10.1097/01.PCC.0000149135.95884.65 IS - 1 KW - Cardiac Extracorporeal life support Extracorporeal membrane oxygenation Hypoplastic left heart syndrome Infant Neonate Outcome M3 - Article N1 - Cited By :52 Export Date: 15 June 2020 PY - 2005 SP - 33-38 ST - Utilization and outcomes of neonatal cardiac extracorporeal life support: 1996-2000 T2 - Pediatric Critical Care Medicine TI - Utilization and outcomes of neonatal cardiac extracorporeal life support: 1996-2000 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-21744458964&doi=10.1097%2f01.PCC.0000149135.95884.65&partnerID=40&md5=7149b179cd4191e3b7d37e442cc87eab VL - 6 ID - 2242 ER - TY - JOUR AB - OBJECTIVE: Apolipoprotein E (APOE) genotype is a determinant of neurologic recovery after brain ischemia and traumatic brain injury. The APOE ε2 allele has been associated with worse neurodevelopmental (ND) outcome after repair of congenital heart defects (CHD) in infancy. Replication of this finding in an independent cohort is essential to validate the observed genotype-phenotype association. METHODS: The association of APOE genotype with ND outcomes was assessed in a combined cohort of patients with single-ventricle CHD enrolled in the Single Ventricle Reconstruction and Infant Single Ventricle trials. ND outcome was assessed at 14 months using the Psychomotor Development Index (PDI) and Mental Development Index (MDI) of the Bayley Scales of Infant Development-II. Stepwise multivariable regression was performed to develop predictive models for PDI and MDI scores. RESULTS: Complete data were available for 298 of 435 patients. After adjustment for preoperative and postoperative covariates, the APOE ε2 allele was associated with a lower PDI score (P = .038). Patients with the ε2 allele had a PDI score approximately 6 points lower than those without the risk allele, explaining 1.04% of overall PDI variance, because the ε2 allele was present in only 11% of the patients. There was a marginal effect of the ε2 allele on MDI scores (P = .058). CONCLUSIONS: These data validate the association of the APOE ε2 allele with adverse early ND outcomes after cardiac surgery in infants, independent of patient and operative factors. Genetic variants that decrease neuroresilience and impair neuronal repair after brain injury are important risk factors for ND dysfunction after surgery for CHD. AD - Division of Cardiothoracic Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pa. Electronic address: gaynor@email.chop.edu. Departments of Medicine (Division of Medical Genetics) and Genome Sciences, University of Washington School of Medicine, Seattle, Wash. Primary Children's Medical Center, Salt Lake City, Utah. Division of Pediatric Cardiology, Medical University of South Carolina, Charleston, SC. Department of Neurology, Boston Children's Hospital, Boston, Mass, and Department of Neurology, Harvard Medical School, Boston, Mass. Division of Medical Genetics, Department of Medicine, University of Washington School of Medicine, Seattle, Wash. Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wis. Johns Hopkins Children's Heart Institute, All Children's Hospital and Florida Hospital for Children, St Petersburg, Fla. Department of Pediatrics, Columbia University Medical Center, New York, NY. Children's Hospital Los Angeles, Los Angeles, Calif. Children's Healthcare of Atlanta, Atlanta, Ga. Ann and Robert F. Lurie Children's Hospital of Chicago, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Division of Pediatric Oncology, Duke University Medical Center, Durham, NC. Department of Cardiology, Boston Children's Hospital, Boston, Mass. Nemours Cardiac Center, Alfred I. Dupont Hospital for Children, Wilmington, Del. Division of Pediatric Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pa. Department of Pathology and Laboratory Medicine, The Children's Hospital of Philadelphia, Philadelphia, Pa. Department of Anesthesia, University of Michigan Medical School, Ann Arbor, Mich. Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. Department of Pediatrics and Communicable Diseases (Division of Pediatric Cardiology), University of Michigan Medical School, Ann Arbor, Mich. AN - 25282659 AU - Gaynor, J. W. AU - Kim, D. S. AU - Arrington, C. B. AU - Atz, A. M. AU - Bellinger, D. C. AU - Burt, A. A. AU - Ghanayem, N. S. AU - Jacobs, J. P. AU - Lee, T. M. AU - Lewis, A. B. AU - Mahle, W. T. AU - Marino, B. S. AU - Miller, S. G. AU - Newburger, J. W. AU - Pizarro, C. AU - Ravishankar, C. AU - Santani, A. B. AU - Wilder, N. S. AU - Jarvik, G. P. AU - Mital, S. AU - Russell, M. W. C2 - PMC4376113 C6 - NIHMS673829 DA - Dec DB - PubMed DO - 10.1016/j.jtcvs.2014.07.052 DP - NLM ET - 2014/10/06 IS - 6 KW - Age Factors Apolipoprotein E2/*genetics Cardiac Surgical Procedures/*adverse effects Child Development Developmental Disabilities/*genetics/physiopathology/psychology Female Genetic Predisposition to Disease Heart Defects, Congenital/*surgery Humans Infant Infant, Newborn Male Multivariate Analysis Nervous System/*growth & development Neuropsychological Tests Phenotype Predictive Value of Tests Reproducibility of Results Risk Factors LA - eng N1 - 1097-685x Gaynor, J William Kim, Daniel Seung Arrington, Cammon B Atz, Andrew M Bellinger, David C Burt, Amber A Ghanayem, Nancy S Jacobs, Jeffery P Lee, Teresa M Lewis, Alan B Mahle, William T Marino, Bradley S Miller, Stephen G Newburger, Jane W Pizarro, Christian Ravishankar, Chitra Santani, Avni B Wilder, Nicole S Jarvik, Gail P Mital, Seema Russell, Mark W HL068288/HL/NHLBI NIH HHS/United States HL068285/HL/NHLBI NIH HHS/United States U01 HL068269/HL/NHLBI NIH HHS/United States F31 MH101905/MH/NIMH NIH HHS/United States U10 HL109816/HL/NHLBI NIH HHS/United States P30 HD018655/HD/NICHD NIH HHS/United States U01 HL068279/HL/NHLBI NIH HHS/United States U10 HL109743/HL/NHLBI NIH HHS/United States U01 HL068290/HL/NHLBI NIH HHS/United States HL068279/HL/NHLBI NIH HHS/United States U01 HL068288/HL/NHLBI NIH HHS/United States U10 HL068270/HL/NHLBI NIH HHS/United States U01 HL068281/HL/NHLBI NIH HHS/United States U01 HL068270/HL/NHLBI NIH HHS/United States HL085057/HL/NHLBI NIH HHS/United States UL1 RR025758/RR/NCRR NIH HHS/United States UL1 RR 025758/RR/NCRR NIH HHS/United States HL068281/HL/NHLBI NIH HHS/United States U01 HL068292/HL/NHLBI NIH HHS/United States HL068269/HL/NHLBI NIH HHS/United States HL068270/HL/NHLBI NIH HHS/United States 1F31MH101905-01/MH/NIMH NIH HHS/United States U01 HL085057/HL/NHLBI NIH HHS/United States U10 HL109778/HL/NHLBI NIH HHS/United States HL068290/HL/NHLBI NIH HHS/United States U10 HL109673/HL/NHLBI NIH HHS/United States U01 HL068285/HL/NHLBI NIH HHS/United States HL068292/HL/NHLBI NIH HHS/United States Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't Validation Study J Thorac Cardiovasc Surg. 2014 Dec;148(6):2560-6. doi: 10.1016/j.jtcvs.2014.07.052. Epub 2014 Aug 1. PY - 2014 SN - 0022-5223 (Print) 0022-5223 SP - 2560-6 ST - Validation of association of the apolipoprotein E ε2 allele with neurodevelopmental dysfunction after cardiac surgery in neonates and infants T2 - J Thorac Cardiovasc Surg TI - Validation of association of the apolipoprotein E ε2 allele with neurodevelopmental dysfunction after cardiac surgery in neonates and infants VL - 148 ID - 407 ER - TY - JOUR AB - OBJECTIVE: The purpose of this multicenter study was to confirm the validity and reliability of the Pediatric Cardiac Quality of Life Inventory (PCQLI). METHODS: Seven centers recruited pediatric patients (8-18 years of age) with heart disease (HD) and their parents to complete the PCQLI and generic health-related quality of life (Pediatric Quality of Life Inventory [PedsQL]) and non-quality of life (Self-Perception Profile for Children [SPPC]/Self-Perception Profile for Adolescents [SPPA] and Youth Self-Report [YSR]/Child Behavior Checklist [CBCL]) tools. PCQLI construct validity was assessed through correlations of PCQLI scores between patients and parents and with severity of congenital HD, medical care utilization, and PedsQL, SPPC/SPPA, and YSR/CBCL scores. PCQLI test-retest reliability was evaluated. RESULTS: The study enrolled 1605 patient-parent pairs. Construct validity was substantiated by the association of lower PCQLI scores with Fontan palliation and increased numbers of cardiac operations, hospital admissions, and physician visits (P<.001); moderate to good correlations between patient and parent PCQLI scores (r=0.41-0.61; P<.001); and fair to good correlations between PCQLI total scores and PedsQL total (r=0.70-0.76), SPPC/SPPA global self-worth (r=0.43-0.46), YSR/CBCL total competency (r=0.28-0.37), and syndrome and Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition-oriented scale (r=-0.58 to -0.30; P<.001) scores. Test-retest reliability correlations were excellent (r=0.78-0.90; P<.001). CONCLUSIONS: PCQLI scores are valid and reliable for children and adolescents with congenital and acquired HD and may be useful for future research and clinical management. AD - Cincinnati Children's Hospital Medical Center, Department of Pediatrics, 3333 Burnet Ave, MLC 5050, Cincinnati, OH 45229, USA. bradley.marino@cchmc.org AN - 20805147 AU - Marino, B. S. AU - Tomlinson, R. S. AU - Wernovsky, G. AU - Drotar, D. AU - Newburger, J. W. AU - Mahony, L. AU - Mussatto, K. AU - Tong, E. AU - Cohen, M. AU - Andersen, C. AU - Shera, D. AU - Khoury, P. R. AU - Wray, J. AU - Gaynor, J. W. AU - Helfaer, M. A. AU - Kazak, A. E. AU - Shea, J. A. C2 - PMC3418527 C6 - NIHMS395073 DA - Sep DB - PubMed DO - 10.1542/peds.2009-2973 DP - NLM ET - 2010/09/02 IS - 3 KW - Adolescent Adult Child Cross-Sectional Studies Female *Heart Diseases/therapy Humans Male Middle Aged Prospective Studies *Quality of Life Severity of Illness Index *Surveys and Questionnaires LA - eng N1 - 1098-4275 Marino, Bradley S Tomlinson, Ryan S Wernovsky, Gil Drotar, Dennis Newburger, Jane W Mahony, Lynn Mussatto, Kathleen Tong, Elizabeth Cohen, Mitchell Andersen, Charlotte Shera, David Khoury, Philip R Wray, Jo Gaynor, J William Helfaer, Mark A Kazak, Anne E Shea, Judy A Pediatric Cardiac Quality of Life Inventory Testing Study Consortium K23 HD048637/HD/NICHD NIH HHS/United States 5-K23-HD048637/HD/NICHD NIH HHS/United States Journal Article Multicenter Study Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't Validation Study Pediatrics. 2010 Sep;126(3):498-508. doi: 10.1542/peds.2009-2973. Epub 2010 Aug 30. PY - 2010 SN - 0031-4005 (Print) 0031-4005 SP - 498-508 ST - Validation of the pediatric cardiac quality of life inventory T2 - Pediatrics TI - Validation of the pediatric cardiac quality of life inventory VL - 126 ID - 131 ER - TY - JOUR AB - Objective: To assess the utility of transesophageal echocardiography (TEE) in the detection of proximal coronary artery (CA) stenoses in children undergoing cardiac surgery Design: Longitudinal cohort study conducted between January 2000 and April 2008 Setting: Chest Diseases Hospital, Kuwait and the University of Toronto, Canada. Subjects: Fifteen patients of either sex, aged between three days to 17 years (median = 8 years, 8 female) identi.ed as having proximal CA stenosis by TEE Interventions: Pre and postoperative TEE done in all patients where CA surgery was performed Main Outcome Measures: Patients with proximal CA Doppler and color .ow patterns suggestive of proximal CA stenosis after cardiac surgery were identi.ed. The clinical, echocardiographic, angiographic and autopsy data were reviewed. Results: We identi.ed with TEE proximal CA stenoses after Ross-Konno (5/15), arterial switch repair (2/15), repair of supravalvar AS (2/15), reimplantation of ALCA (2/15), complex AV and sub-AV stenosis (1/15), isolated supravalvar PS (1/15), post-Norwood stage I (1/15) and redo of RV to PA conduit (1/15). RCA stenoses were identi.ed in nine and LCA stenoses in six cases. Six cases had anomalous origin of CA diagnosed preoperatively. Three Doppler .ow patterns were identi.ed. Wall motion abnormalities were seen in all cases. The result of TEE of CA's altered immediate surgical management in eight patients. Conclusion: TEE imaging and Doppler interrogation of the proximal CAs reliably detects stenoses and may provide important information leading to surgical revision of the CA anastomoses. We suggest routine perioperative TEE assessment of proximal CA anastomoses following surgical reimplantation. AD - M. A. Al-Qbandi, Department of Pediatrics and Congenital Heart Diseases, Chest Disease Hospital, P O BOX 4082, Safat, 13081, Kuwait AU - Al-Qbandi, M. A. AU - Smallhorn, J. DB - Embase IS - 1 KW - adolescent angiocardiography aortic regurgitation aortic valve stenosis artery reconstruction artery wall article autopsy child childhood disease clinical article cohort analysis color Doppler flowmetry congestive cardiomyopathy coronary artery anomaly coronary artery obstruction coronary artery surgery coronary stent diagnostic value Doppler echocardiography endocardial fibroelastosis female great vessels transposition heart left ventricle outflow tract obstruction hospitalized child human hypoplastic left heart syndrome infant left coronary artery longitudinal study male preschool child right coronary artery school child transesophageal echocardiography Williams Beuren syndrome LA - English M3 - Article N1 - L358388731 2010-03-11 2010-04-01 http://www.kma.org.kw/KMJ/march2010.pdf PY - 2010 SN - 0023-5776 SP - 27-37 ST - The value of transesophageal echocardiography to detect coronary artery stenosis in children T2 - Kuwait Medical Journal TI - The value of transesophageal echocardiography to detect coronary artery stenosis in children UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L358388731 VL - 42 ID - 1149 ER - TY - JOUR AB - Initial surgical strategies in neonates with single left ventricular (LV) anatomy vary based on adequacy of pulmonary and systemic blood flow. Differing myocardial responses to these strategies, as reflected in indices of systolic function, ventricular size, and mass have not been well defined. We sought to evaluate single LV myocardial response to varied physiology and initial palliation and determine whether the response is consistent and predictable. Infants with single LV physiology were divided based on neonatal palliation: no palliation/PA band (NO); BT shunt only (BT); or Norwood procedure (NP). Echo measures were obtained at presentation, early post-bidirectional Glenn (BDG), late post-BDG follow-up, and post-Fontan procedure. Measures included ejection fraction, LV mass indexed to height(2.7) and end diastolic volume indexed to body surface area, and mass/volume ratio. The cohort included 38 children (13 NO, 13 BT, 12 NP). Ejection fraction was similar but depressed in all groups at all stages. LV mass was higher in the NP group than the BT group at early post-BDG (p = 0.03) and higher than both BT and NO groups (p < 0.01) at late post-BDG, but the difference was resolved by post-Fontan follow-up. The NP group had the most remarkable remodeling in LV size from BDG to Fontan, suggesting that volume unloading is most valuable in this subgroup. Ventricular remodeling can be identified by echocardiography in children with single LV physiology, despite variable initial surgical palliative strategies. Importantly, these initial surgical strategies do not result in significant differences after Fontan palliation during early childhood. AD - Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI, 53226, USA. Department of Obstetrics and Gynecology, University of Florida, PO Box 100294, Gainesville, FL, 32610, USA. Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI, 53226, USA. ghill@chw.org. Children's Hospital of Wisconsin, 9000 W. Wisconsin Ave., Milwaukee, WI, 53226, USA. ghill@chw.org. AN - 27554256 AU - Horriat, N. L. AU - Deatsman, S. L. AU - Stelter, J. AU - Frommelt, P. C. AU - Hill, G. D. DA - Dec DB - PubMed DO - 10.1007/s00246-016-1471-y DP - NLM ET - 2016/08/25 IS - 8 KW - Echocardiography Fontan Procedure *Heart Ventricles Humans Infant Norwood Procedures Treatment Outcome Ventricular Function, Left Fontan Glenn Single ventricle LA - eng N1 - 1432-1971 Horriat, Narges L Deatsman, Sara L Stelter, Jessica Frommelt, Peter C Hill, Garick D Journal Article United States Pediatr Cardiol. 2016 Dec;37(8):1569-1574. doi: 10.1007/s00246-016-1471-y. Epub 2016 Aug 23. PY - 2016 SN - 0172-0643 SP - 1569-1574 ST - Variable Myocardial Response to Load Stresses in Infants with Single Left Ventricular Anatomy: Influence of Initial Physiology and Surgical Palliative Strategy T2 - Pediatr Cardiol TI - Variable Myocardial Response to Load Stresses in Infants with Single Left Ventricular Anatomy: Influence of Initial Physiology and Surgical Palliative Strategy VL - 37 ID - 239 ER - TY - JOUR AD - The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH; The James M. Anderson Center for Clinical Excellence, Cincinnati Children's Hospital Medical Center, Cincinnati, OH. AN - 104466818. Corporate Author: National Pediatric Cardiology Quality Improvement Collaborative. Language: English. Entry Date: 20120921. Revision Date: 20150711. Publication Type: Journal Article AU - Anderson, J. B. AU - Iyer, S. B. AU - Schidlow, D. N. AU - Williams, R. AU - Varadarajan, K. AU - Horsley, M. AU - Slicker, J. AU - Pratt, J. AU - King, E. AU - Lannon, C. DB - ccm DP - EBSCOhost IS - 1 KW - Eating Behavior Growth Hypoplastic Left Heart Syndrome -- Physiopathology Heart Ventricle -- Abnormalities Human Hypoplastic Left Heart Syndrome -- Surgery Infant Infant, Newborn Male Retrospective Design N1 - research. Journal Subset: Biomedical; Peer Reviewed; USA. Special Interest: Pediatric Care. NLM UID: 0375410. PMID: NLM22336578. PY - 2012 SN - 0022-3476 SP - 16-21.e3 ST - Variation in growth of infants with a single ventricle T2 - Journal of Pediatrics TI - Variation in growth of infants with a single ventricle UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104466818&site=ehost-live&scope=site VL - 161 ID - 1640 ER - TY - JOUR AB - Ethical issues in pediatric heart transplantation (Htx) include resource allocation, benefit, and burden assessment in high-risk recipients, and informed consent. Practice patterns and decision-making was investigated using an internet survey with 47-multiple choice items and vignette-based questions. Of 43 pediatric Htx cardiologists contacted, 28 (65%) responded. Respondents reported that an overall median 1-month survival of 73% (range 50-100%), 1-year survival of 70% (range 50-85%), 5-year survival of 50% (range 40-85%), and 10-year survival of 50% (range 25-85%) was adequate to offer Htx. Based on vignettes presented, 100% of those surveyed would offer Htx to a straightforward 12-year old with end-stage dilated cardiomyopathy and a 7-year old with hypoplastic left heart syndrome with protein losing enteropathy. Thirty percent of physicians would offer Htx to a patient status post a Fontan procedure with mutliple co-morbidities. Seventy-five percent of physicians would offer Htx despite proven non-adherence. Considerable variability exists in the practice patterns of pediatric heart transplant cardiologists with regards to decision-making while evaluating patients for listing. Disagreements among pediatric Htx cardiologists exist when there are concerns for non-adherence and associated multiple co-morbidities. Further work is needed to understand these variations and develop consensus for pediatric Htx organ allocation. AD - Northwestern Feinberg School of Medicine, Ann & Robert H. Lurie Children's Hospital of Chicago, 225 E. Chicago Avenue, Box 21, Chicago, IL, 60611-2991, USA. anpatel@luriechildrens.org. Feinberg School of Medicine, Northwestern University, Chicago, IL, USA. anpatel@luriechildrens.org. Northwestern Feinberg School of Medicine, Ann & Robert H. Lurie Children's Hospital of Chicago, 225 E. Chicago Avenue, Box 21, Chicago, IL, 60611-2991, USA. Feinberg School of Medicine, Northwestern University, Chicago, IL, USA. University of California San Francisco Benioff Children's Hospitals, San Francisco, CA, USA. AN - 30121868 AU - Patel, A. AU - Michelson, K. AU - Andrei, A. C. AU - Pahl, E. AU - Gossett, J. G. DA - Jan DB - PubMed DO - 10.1007/s00246-018-1965-x DP - NLM ET - 2018/08/20 IS - 1 KW - Adult Cardiologists/*ethics/standards Child Child, Preschool *Clinical Decision-Making Female Health Status Heart Transplantation/*ethics/mortality/psychology Humans Male Pediatrics/methods Pilot Projects *Practice Patterns, Physicians' Retrospective Studies Surveys and Questionnaires Ethics Heart transplantation Pediatric LA - eng N1 - 1432-1971 Patel, Angira Orcid: 0000-0001-6760-0340 Michelson, Kelly Andrei, Adin-Cristian Pahl, Elfriede Gossett, Jeffrey G Journal Article United States Pediatr Cardiol. 2019 Jan;40(1):101-109. doi: 10.1007/s00246-018-1965-x. Epub 2018 Aug 18. PY - 2019 SN - 0172-0643 SP - 101-109 ST - Variations in Criteria and Practices for Heart Transplantation Listing Among Pediatric Transplant Cardiologists T2 - Pediatr Cardiol TI - Variations in Criteria and Practices for Heart Transplantation Listing Among Pediatric Transplant Cardiologists VL - 40 ID - 90 ER - TY - JOUR AB - There is new and growing experience with venovenous extracorporeal life support (VV ECLS) for neonatal and pediatric patients with single-ventricle physiology and acute respiratory distress syndrome (ARDS). Outcomes in this population have been defined but could be improved; survival rates in single-ventricle patients on VV ECLS for respiratory failure are slightly higher than those in single-ventricle patients on venoarterial ECLS for cardiac failure (48 vs. 32-43%), but are lower than in patients with biventricular anatomy (58-74%). To that end, special consideration is necessary for patients with single-ventricle physiology who require VV ECLS for ARDS. Specifically, ARDS disrupts the balance between pulmonary and systemic blood flow through dynamic alterations in cardiopulmonary mechanics. This complexity impacts how to run the VV ECLS circuit and the transition back to conventional support. Furthermore, these patients have a complicated coagulation profile. Both venous and arterial thrombi carry marked risk in single-ventricle patients due to the vulnerability of the pulmonary, coronary, and cerebral circulations. Finally, single-ventricle palliation requires the preservation of low resistance across the pulmonary circulation, unobstructed venous return, and optimal cardiac performance including valve function. As such, the proper timing as well as the particular conduct of ECLS might differ between this population and patients without single-ventricle physiology. The goal of this review is to summarize the current state of knowledge of VV ECLS in the single-ventricle population in the context of these special considerations. AD - Department of Pediatrics, University of California San Francisco , San Francisco, CA , USA. Department of Pediatrics, University of California San Francisco, San Francisco, CA, USA; Cardiovascular Research Institute, University of California San Francisco, San Francisco, CA, USA. AN - 27446889 AU - Nair, A. B. AU - Oishi, P. C2 - PMC4923132 DB - PubMed DO - 10.3389/fped.2016.00066 DP - NLM ET - 2016/07/23 KW - acute respiratory distress syndrome anticoagulation cannulation congenital heart disease extracorporeal membrane oxygenation single ventricle thrombosis venovenous extracorporeal life support LA - eng N1 - 2296-2360 Nair, Alison B Oishi, Peter Journal Article Review Front Pediatr. 2016 Jun 28;4:66. doi: 10.3389/fped.2016.00066. eCollection 2016. PY - 2016 SN - 2296-2360 (Print) 2296-2360 SP - 66 ST - Venovenous Extracorporeal Life Support in Single-Ventricle Patients with Acute Respiratory Distress Syndrome T2 - Front Pediatr TI - Venovenous Extracorporeal Life Support in Single-Ventricle Patients with Acute Respiratory Distress Syndrome VL - 4 ID - 94 ER - TY - JOUR AB - The management of failing Fontan patients with mechanical circulatory support has been reported with unsatisfactory results. A 5-yearold girl, listed for heart transplantation for failing Fontan, underwent rescue extracorporeal membrane oxygenation support. As it was necessary to move the patient to the Berlin Heart Excor, a step-by-step approach was designed to obtain a gradual adaptation to this form of support. A central extracorporeal membrane oxygenation, with a Berlin Heart apical and aortic cannulae, was implanted leaving the previously positioned femoral vein cannula in parallel with the outflow apical one. The femoral vein cannula was removed when the lungs recovered from the effects of surgical trauma. Four days later, the Fontan fenestration was closed, and after 4 more days, a gradual reduction of gas support was planned without problems. Oxygenator was then removed, and 4 days later, a 30-ml Berlin Heart EXCOR ventricle replaced the centrifugal pump. This sequential approach, which has never been described before, can improve the results of the ventricular assist device support in failing Fontan patients, because it allows a gradual adaptation, even in patients with damaged lungs. AD - C.P. Napoleone, Department of Pediatric Cardiac Surgery, Regina Margherita Children's Hospital, Piazza Polonia 94, Torino, Italy AU - Napoleone, C. P. AU - Cascarano, M. T. AU - Deorsola, L. AU - Valori, A. DB - Embase Medline DO - 10.1093/icvts/ivx288 IS - 2 KW - cannula centrifugal pump oxygenator Quadrox ventricular assist device article case report cavopulmonary connection child clinical article extracorporeal oxygenation female femoral vein Fontan procedure heart hemodynamics heart transplantation human lung lung injury preschool child priority journal surgical technique treatment failure treatment indication EXCOR LA - English M3 - Article N1 - L620608810 2018-02-14 2018-02-20 PY - 2018 SN - 1569-9285 1569-9293 SP - 341-342 ST - Ventricular assist device in a failing total cavopulmonary connection: A new step-by-step approach T2 - Interactive Cardiovascular and Thoracic Surgery TI - Ventricular assist device in a failing total cavopulmonary connection: A new step-by-step approach UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L620608810 http://dx.doi.org/10.1093/icvts/ivx288 VL - 26 ID - 708 ER - TY - JOUR AB - The management of failing Fontan patients with mechanical circulatory support has been reported with unsatisfactory results. A 5-year-old girl, listed for heart transplantation for failing Fontan, underwent rescue extracorporeal membrane oxygenation support. As it was necessary to move the patient to the Berlin Heart Excor, a step-by-step approach was designed to obtain a gradual adaptation to this form of support. A central extracorporeal membrane oxygenation, with a Berlin Heart apical and aortic cannulae, was implanted leaving the previously positioned femoral vein cannula in parallel with the outflow apical one. The femoral vein cannula was removed when the lungs recovered from the effects of surgical trauma. Four days later, the Fontan fenestration was closed, and after 4 more days, a gradual reduction of gas support was planned without problems. Oxygenator was then removed, and 4 days later, a 30-ml Berlin Heart EXCOR ventricle replaced the centrifugal pump. This sequential approach, which has never been described before, can improve the results of the ventricular assist device support in failing Fontan patients, because it allows a gradual adaptation, even in patients with damaged lungs. AD - Department of Pediatric Cardiac Surgery, Regina Margherita Children's Hospital, Torino, Italy. AN - 29049683 AU - Pace Napoleone, C. AU - Cascarano, M. T. AU - Deorsola, L. AU - Valori, A. DA - Feb 1 DB - PubMed DO - 10.1093/icvts/ivx288 DP - NLM ET - 2017/10/20 IS - 2 KW - Child, Preschool Extracorporeal Membrane Oxygenation/*adverse effects Female Fontan Procedure/*adverse effects Heart Transplantation Heart Ventricles *Heart-Assist Devices Humans Postoperative Complications/*therapy *Extracorporeal membrane oxygenation *Fontan procedure *Ventricular assist device LA - eng N1 - 1569-9285 Pace Napoleone, Carlo Cascarano, Maria Teresa Deorsola, Luca Valori, Andrea Case Reports Journal Article England Interact Cardiovasc Thorac Surg. 2018 Feb 1;26(2):341-342. doi: 10.1093/icvts/ivx288. PY - 2018 SN - 1569-9285 SP - 341-342 ST - Ventricular assist device in a failing total cavopulmonary connection: a new step-by-step approach T2 - Interact Cardiovasc Thorac Surg TI - Ventricular assist device in a failing total cavopulmonary connection: a new step-by-step approach VL - 26 ID - 246 ER - TY - JOUR AB - BACKGROUND/OBJECTIVE: Total cavopulmonary connection (TCPC) has been the preferred treatment for patients with univentricular hearts. Current TCPC-techniques are the intra-atrial lateral tunnel (ILT) and the extracardiac conduit (ECC). We aimed to determine ventricular function during rest and stress, and to compare results for both techniques and for left (LV) versus right ventricular (RV) dominance. METHODS: 99 patients, aged 12.5 ± 4.0 years underwent echocardiography and magnetic resonance imaging (MRI), and 69 patients underwent stress MRI. RESULTS: Echocardiography showed impaired systolic and diastolic function. MRI parameters were comparable between ILT and ECC at rest. During dobutamine there was a decrease in end-diastolic volume (EDVi) (91 ± 21 vs. 80 ± 20 ml/m(2) p<0.001). Ejection fraction (EF) and cardiac index (CI) during dobutamine were lower for ILT patients (59 ± 11 (ILT) vs. 64 ± 7% (ECC), p=0.027 and 4.2 ± 1.0 (ILT) vs. 4.9 ± 1.0L/min/m(2) (ECC), p=0.006), whereas other parameters were comparable. TEI-index was higher in ILT-patients (0.72 ± 0.27 (ILT) vs. 0.56 ± 0.22 (ECC), p=0.002). Diastolic function was frequently impaired in patients with a dominant RV (67% (RV) vs. 39% (LV), p=0.011). Patients with dominant LV's had smaller end-systolic volume (ESVi) (40 ± 13 (LV) vs. 47 ± 16 (RV) ml/m(2), p=0.030) and higher EF (55 ± 8 (LV) vs. 49 ± 9 ml/m(2) (RV), p=0.001) and contractility (2.3 ± 0.8 (LV) vs. 1.9 ± 0.7 mmHg/ml/m(2) (RV), p=0.050) during rest and higher EF during dobutamine (63 ± 8 (LV) vs. 58 ± 10 ml/m(2) (RV), p=0.043). CONCLUSION: Ventricular function is relatively well preserved in modern-day Fontan patients. With dobutamine stress there is a decrease in EDVi. ECC patients have higher CI and EF during stress. Patients with a dominant RV have lower systolic, including impaired contractility, and diastolic function. AD - Department of Pediatrics, Division of Pediatric Cardiology, Erasmus Medical Center - Sophia Children's Hospital, Rotterdam, The Netherlands; Department of Radiology, Erasmus Medical Center, Rotterdam, The Netherlands. Department of Pediatric Cardiology, Radboud University Medical Centre - Amalia Children's Hospital, Nijmegen, The Netherlands,; Department of Pediatrics, Pediatric Cardiology Unit, Tel-Aviv Sourasky Medical Center, Tel Aviv, Israel. Department of Pediatric Cardiology, Academic Medical Center, Amsterdam, The Netherlands. Department of Pediatric Cardiology, University Medical Center Utrecht - Wilhelmina Children's Hospital, Utrecht, The Netherlands. Department of Radiology, Erasmus Medical Center, Rotterdam, The Netherlands. Department of Radiology, Leiden University Medical Center, Leiden, The Netherlands. Department of Radiology, Academic Medical Center, Amsterdam, The Netherlands. Department of Clinical Chemistry, Erasmus Medical Center, Rotterdam, The Netherlands. Department of Paediatric Cardiology, Leiden University Medical Center, Leiden, The Netherlands. Department of Pediatrics, Division of Pediatric Cardiology, Erasmus Medical Center - Sophia Children's Hospital, Rotterdam, The Netherlands; Department of Radiology, Erasmus Medical Center, Rotterdam, The Netherlands. Electronic address: w.a.helbing@erasmusmc.nl. AN - 26080280 AU - Bossers, S. S. AU - Kapusta, L. AU - Kuipers, I. M. AU - van Iperen, G. AU - Moelker, A. AU - Kroft, L. J. AU - Romeih, S. AU - de Rijke, Y. AU - Ten Harkel, A. D. AU - Helbing, W. A. DA - Oct 1 DB - PubMed DO - 10.1016/j.ijcard.2015.05.181 DP - NLM ET - 2015/06/17 KW - Adolescent Child Dobutamine/metabolism Female *Fontan Procedure *Fractional Flow Reserve, Myocardial Heart Defects, Congenital/*surgery Heart Ventricles/diagnostic imaging/*physiopathology Humans Magnetic Resonance Imaging/methods Male Stress, Physiological/physiology Ultrasonography Ventricular Function/*physiology Cardiac function Congenital heart disease Echocardiography Fontan Mri Total cavopulmonary connection LA - eng N1 - 1874-1754 Bossers, S S M Kapusta, L Kuipers, I M van Iperen, G Moelker, A Kroft, L J M Romeih, S de Rijke, Y Ten Harkel, A D J Helbing, W A Journal Article Research Support, Non-U.S. Gov't Netherlands Int J Cardiol. 2015 Oct 1;196:73-80. doi: 10.1016/j.ijcard.2015.05.181. Epub 2015 Jun 3. PY - 2015 SN - 0167-5273 SP - 73-80 ST - Ventricular function and cardiac reserve in contemporary Fontan patients T2 - Int J Cardiol TI - Ventricular function and cardiac reserve in contemporary Fontan patients VL - 196 ID - 309 ER - TY - JOUR AB - Background/objective Total cavopulmonary connection (TCPC) has been the preferred treatment for patients with univentricular hearts. Current TCPC-techniques are the intra-atrial lateral tunnel (ILT) and the extracardiac conduit (ECC). We aimed to determine ventricular function during rest and stress, and to compare results for both techniques and for left (LV) versus right ventricular (RV) dominance. Methods 99 patients, aged 12.5 ± 4.0 years underwent echocardiography and magnetic resonance imaging (MRI), and 69 patients underwent stress MRI. Results Echocardiography showed impaired systolic and diastolic function. MRI parameters were comparable between ILT and ECC at rest. During dobutamine there was a decrease in end-diastolic volume (EDVi) (91 ± 21 vs. 80 ± 20 ml/m2 p < 0.001). Ejection fraction (EF) and cardiac index (CI) during dobutamine were lower for ILT patients (59 ± 11 (ILT) vs. 64 ± 7% (ECC), p = 0.027 and 4.2 ± 1.0 (ILT) vs. 4.9 ± 1.0 L/min/m2 (ECC), p = 0.006), whereas other parameters were comparable. TEI-index was higher in ILT-patients (0.72 ± 0.27 (ILT) vs. 0.56 ± 0.22 (ECC), p = 0.002). Diastolic function was frequently impaired in patients with a dominant RV (67% (RV) vs. 39% (LV), p = 0.011). Patients with dominant LV's had smaller end-systolic volume (ESVi) (40 ± 13 (LV) vs. 47 ± 16 (RV) ml/m2, p = 0.030) and higher EF (55 ± 8 (LV) vs. 49 ± 9 ml/m2 (RV), p = 0.001) and contractility (2.3 ± 0.8 (LV) vs. 1.9 ± 0.7 mm Hg/ml/m2 (RV), p = 0.050) during rest and higher EF during dobutamine (63 ± 8 (LV) vs. 58 ± 10 ml/m2 (RV), p = 0.043). Conclusion Ventricular function is relatively well preserved in modern-day Fontan patients. With dobutamine stress there is a decrease in EDVi. ECC patients have higher CI and EF during stress. Patients with a dominant RV have lower systolic, including impaired contractility, and diastolic function. AD - W.A. Helbing, Department of Pediatrics, Division of Pediatric Cardiology, Erasmus Medical Center, Sophia Children's Hospital, PO Box 2060, CB Rotterdam, Netherlands AU - Bossers, S. S. M. AU - Kapusta, L. AU - Kuipers, I. M. AU - Van Iperen, G. AU - Moelker, A. AU - Kroft, L. J. M. AU - Romeih, S. AU - De Rijke, Y. AU - Ten Harkel, A. D. J. AU - Helbing, W. A. DB - Embase Medline DO - 10.1016/j.ijcard.2015.05.181 KW - dobutamine age distribution article child clinical effectiveness diastole drug effect echocardiography female Fontan procedure cardiac index heart left ventricle ejection fraction heart left ventricle enddiastolic volume heart left ventricle function heart right ventricle ejection fraction heart right ventricle enddiastolic volume heart right ventricle function heart single ventricle heart stress human major clinical study male multicenter study nuclear magnetic resonance imaging priority journal rest surgical approach systole LA - English M3 - Article N1 - L605256808 2015-07-28 2015-07-31 PY - 2015 SN - 1874-1754 0167-5273 SP - 73-80 ST - Ventricular function and cardiac reserve in contemporary Fontan patients T2 - International Journal of Cardiology TI - Ventricular function and cardiac reserve in contemporary Fontan patients UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L605256808 http://dx.doi.org/10.1016/j.ijcard.2015.05.181 VL - 196 ID - 858 ER - TY - JOUR AB - BACKGROUND: Patients with single ventricle physiology and Fontan circulation are at increased risk for late complications and reduced survival. The aim of the study was to investigate the correlation between ventricular geometry and systolic regional function in different underlying anatomic conditions in adolescent and adult Fontan-palliated patients. METHOD: In a retrospective cross-sectional study, we measured 2D strain, ventricular diameters, ventricular volumes, ejection fraction (EF), global and segmental wall stress, and sphericity index. The same analyses were performed in 99 age- and gender-matched healthy individuals. RESULTS: One hundred and one patients were included at a mean age of 21 (range 14-59) years. In comparison with healthy subjects, patients with Fontan circulation displayed larger ventricular volumes (153 ± 78 mL vs 116 ± 38 mL P < 0.05), reduced EF (43% ± 15% vs 55% ± 8% P < 0.05), reduced longitudinal (-13% ± 6% vs -21% ± 4% P < 0.05) and circumferential strain values (-15% ± 7% vs -22% ± 4% P < 0.05). Functionally single ventricles were more spherical (ratio of longitudinal to short-axis diameters 1.3 ± 0.3 vs 1.7 ± 0.2 P < 0.05). Circumferential strain correlated well with global wall stress and the degree of sphericity (R(2)  = 0.320), while segmental strain did not correlate with segmental wall stress. The percentage of segments with akinesia was relatively high (16 ± 16% vs 0 ± 0% P < 0.05) indicating reduced segmental contractile function. CONCLUSION: Functionally single ventricles after Fontan palliation have significantly reduced systolic regional and global function with a high intersegmental inhomogeneity. The underlying pathological mechanisms might be multifactorial, including ventricular geometry, sphericity, and regional contractile properties. Future studies are needed to investigate the role of ventricular geometry for clinical performance and outcome. AD - Department of Cardiology, Division of Cardiothoracic and Respiratory Medicine, University Hospital of North Norway, Tromsø, Norway. Department of Cardiothoracic Surgery, Stanford University School of Medicine, Stanford, California. Department of Cardiovascular Sciences, K.U. Leuven, Leuven, Belgium. Department of Medicine, Levanger Hospital, Nord-Trøndelag Hospital Trust, Levanger, Norway. Department of Cardiology, St. Olav's University Hospital, Trondheim, Norway. Department of Circulation and Medical Imaging, Norwegian University of Science and Technology, Trondheim, Norway. Division of Pediatric Cardiology, Department of Pediatrics, Stanford University School of Medicine, Stanford, California. Division of Pediatric Cardiology, Hospital for Sick Children, Toronto, Ontario, Canada. Division of Cardiovascular Medicine, Department of Medicine, Stanford University School of Medicine, Stanford, California. AN - 30350438 AU - Rösner, A. AU - Khalapyan, T. AU - Pedrosa, J. AU - Dalen, H. AU - McElhinney, D. B. AU - Friedberg, M. K. AU - Lui, G. K. DA - Dec DB - PubMed DO - 10.1111/echo.14169 DP - NLM ET - 2018/10/24 IS - 12 KW - Adolescent Adult Cross-Sectional Studies Echocardiography/*methods Female Follow-Up Studies Fontan Procedure/*methods Heart Defects, Congenital/diagnosis/*physiopathology/surgery Heart Ventricles/*diagnostic imaging/physiopathology Humans Male Middle Aged Myocardial Contraction/*physiology Retrospective Studies Systole Ventricular Function, Left/*physiology Young Adult *Fontan circulation *strain rate echocardiography *ventricular function *ventricular geometry LA - eng N1 - 1540-8175 Rösner, Assami Orcid: 0000-0001-9084-5805 Khalapyan, Tigran Pedrosa, João Dalen, Håvard McElhinney, Doff B Friedberg, Mark K Lui, George K Journal Article United States Echocardiography. 2018 Dec;35(12):2035-2046. doi: 10.1111/echo.14169. Epub 2018 Oct 23. PY - 2018 SN - 0742-2822 SP - 2035-2046 ST - Ventricular mechanics in adolescent and adult patients with a Fontan circulation: Relation to geometry and wall stress T2 - Echocardiography TI - Ventricular mechanics in adolescent and adult patients with a Fontan circulation: Relation to geometry and wall stress VL - 35 ID - 72 ER - TY - JOUR AB - OBJECTIVES: This study was designed to evaluate changes in ventricular volume, mass and cardiac function before and after creation of an atrial to pulmonary connection in patients with a univentricular atrioventricular connection. BACKGROUND: Intact systolic and diastolic performance is critical for successful establishment of an atrial dependent circulation, and few studies are available comparing cardiac performance before and after creation. METHODS: With the use of radionuclide blood pool imaging and ventricular cineangiography, 54 patients (mean age 6.4 +/- 3.4 years) were studied. Twenty-eight patients were investigated preoperatively and 36 greater than 1 year after repair and compared with a control population. RESULTS: Before operation, end-diastolic volume and wall mass were significantly increased compared with those of control subjects; however, the mass/volume ratio was normal (1.08 +/- 0.31 g/ml for the preoperative group; 0.97 +/- 0.19 for control subjects). Although end-diastolic volume returned to normal after the procedure, wall mass remained elevated and contributed to an elevated mass/volume ratio (1.20 +/- 0.38 g/ml). After the procedure, systemic vascular resistance index was significantly elevated compared with that before surgery or with that of control subjects (1,199 +/- 373, 2,120 +/- 645, 1,556 +/- 275 dynes.s.cm-5.m2: pre- and postrepair and control subjects, respectively). Radionuclide studies demonstrated that preoperative ejection fraction (52 +/- 9, 50 +/- 9, 60 +/- 8%), peak ejection (2.58 +/- 0.66, 2.95 +/- 0.81, 3.73 +/- 0.70 EDV/s) and peak filling rates (2.84 +/- 0.75, 2.75 +/- 0.79, 3.84 +/- 0.51 end-diastolic volumes [EDV/s]) were significantly reduced compared with those of control subjects and remained so after surgery. CONCLUSIONS: These data suggest that systolic and diastolic function is depressed preoperatively in these patients, remains unchanged after the creation of an atrial-dependent circulation and is associated with an increased systemic vascular resistance. Long-term issues addressing preservation of cardiac function need to be prospectively studied. AD - Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada. AN - 1527303 AU - Akagi, T. AU - Benson, L. N. AU - Green, M. AU - Ash, J. AU - Gilday, D. L. AU - Williams, W. G. AU - Freedom, R. M. DA - Oct DB - PubMed DO - 10.1016/0735-1097(92)90194-r DP - NLM ET - 1992/10/01 IS - 4 KW - Child Cineangiography Female Gated Blood-Pool Imaging Heart/diagnostic imaging Heart Atria/*surgery Heart Defects, Congenital/diagnosis/*physiopathology/*surgery Heart Ventricles/abnormalities Humans Male Myocardial Contraction/*physiology Pulmonary Artery/*surgery Vascular Resistance/physiology Ventricular Function/*physiology LA - eng N1 - Akagi, T Benson, L N Green, M Ash, J Gilday, D L Williams, W G Freedom, R M Journal Article Research Support, Non-U.S. Gov't United States J Am Coll Cardiol. 1992 Oct;20(4):920-6. doi: 10.1016/0735-1097(92)90194-r. PY - 1992 SN - 0735-1097 (Print) 0735-1097 SP - 920-6 ST - Ventricular performance before and after Fontan repair for univentricular atrioventricular connection: angiographic and radionuclide assessment T2 - J Am Coll Cardiol TI - Ventricular performance before and after Fontan repair for univentricular atrioventricular connection: angiographic and radionuclide assessment VL - 20 ID - 546 ER - TY - JOUR AB - Background Ventricular function and arrhythmia in patients with Fontan circulation in long-term follow-up are still unknown. Methods We retrospectively reviewed 48 patients who survived and were followed up for more than 15 years, among 110 patients who underwent Fontan operation in our institute from 1979 to 1992. Atriopulmonary connection was performed in 26 patients and total cavopulmonary connection in 22. The patients were categorized into right ventricle, left ventricle, and biventricle groups. Follow-up cardiac catheterization and exercise test were performed routinely every 5 years post surgery. Median age at Fontan operation was 5 years. Results Mean follow-up was 18.5 years. Cardiac index in the total cavopulmonary connection group was higher than in the atriopulmonary connection group at 10 and 15 years post surgery (p < 0.05). Ejection fraction in the left-ventricle group was higher than in the right-ventricle group. End-diastolic volume at 5, 10, and 15 years was significantly lower than at 1 year (p < 0.05). End-diastolic pressure at 10 years was significantly higher than at 1 and 5 years (p < 0.05). Beyond 15 years, 6 patients developed ventricular tachycardia. The only significant risk factors for the onset of ventricular tachycardia in a multivariate analysis were age at Fontan operation and absolute age (p < 0.05). Conclusions Long-term follow-up of patients demonstrated that postoperative ventricular systolic performance seemed to become steady. Ventricular tachycardia was detected 15 years post surgery, especially in older patients with older age at Fontan operation, possibly revealing a risk factor in the long-term postoperative period, thereby meriting further consideration. © 2011 The Society of Thoracic Surgeons. AD - Y. Nakamura, Department of Cardiovascular Surgery, National Cardiovascular Center, 5-7-1, Fujishirodai, Suita, Osaka, 565-8565, Japan AU - Nakamura, Y. AU - Yagihara, T. AU - Kagisaki, K. AU - Hagino, I. AU - Kobayashi, J. DB - Embase Medline DO - 10.1016/j.athoracsur.2010.07.055 IS - 1 KW - adolescent adult article atriopulmonary connection cardiopulmonary function cavopulmonary connection child clinical article exercise test female follow up Fontan procedure heart catheterization cardiac index heart left ventricle heart left ventricle ejection fraction heart left ventricle enddiastolic pressure heart left ventricle enddiastolic volume heart right ventricle heart ventricle function heart ventricle tachycardia human long term care male preschool child priority journal risk assessment school child survivor task performance LA - English M3 - Article N1 - L360204481 2010-12-30 2011-01-07 PY - 2011 SN - 0003-4975 SP - 172-180 ST - Ventricular performance in long-term survivors after fontan operation T2 - Annals of Thoracic Surgery TI - Ventricular performance in long-term survivors after fontan operation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L360204481 http://dx.doi.org/10.1016/j.athoracsur.2010.07.055 VL - 91 ID - 1119 ER - TY - JOUR AN - 30343077 AU - van den Bosch, E. AU - Bossers, S. S. M. AU - Robbers-Visser, D. AU - Boersma, E. AU - Roos-Hesselink, J. W. AU - Breur, Hmpj AU - Blom, N. A. AU - Kroft, L. J. M. AU - Snoeren, M. M. AU - Kapusta, L. AU - Helbing, W. A. DA - Feb DB - PubMed DO - 10.1016/j.jcmg.2018.07.025 DP - NLM ET - 2018/10/22 IS - 2 KW - Adolescent Cardiotonic Agents/*administration & dosage Child Dobutamine/*administration & dosage Female *Fontan Procedure/adverse effects/mortality Heart Defects, Congenital/*diagnostic imaging/mortality/physiopathology/*surgery Humans Magnetic Resonance Imaging/*methods Male Predictive Value of Tests Progression-Free Survival Risk Factors Time Factors *Ventricular Function, Left *Ventricular Function, Right LA - eng N1 - 1876-7591 van den Bosch, Eva Bossers, Sjoerd S M Robbers-Visser, Daniëlle Boersma, Eric Roos-Hesselink, Jolien W Breur, Hans M P J Blom, Nico A Kroft, Lucia J M Snoeren, Miranda M Kapusta, Livia Helbing, Willem A Letter Research Support, Non-U.S. Gov't United States JACC Cardiovasc Imaging. 2019 Feb;12(2):368-370. doi: 10.1016/j.jcmg.2018.07.025. Epub 2018 Oct 17. PY - 2019 SN - 1876-7591 SP - 368-370 ST - Ventricular Response to Dobutamine Stress CMR Is a Predictor for Outcome in Fontan Patients T2 - JACC Cardiovasc Imaging TI - Ventricular Response to Dobutamine Stress CMR Is a Predictor for Outcome in Fontan Patients VL - 12 ID - 439 ER - TY - JOUR AB - With use of biplane cineangiocardiograms, ventricular cavity volume measurements were determined in 17 patients with single ventricle, before total correction. There were 4 patients with a single left and 13 with a single right ventricle. Atrioventricular (A-V) valve regurgitation was evident in 6 of 13 patients with a common A-V valve. End-diastolic volume index ranged from 72 to 282 ml/m2 (average 131 ml/m2), and these values ranged from 71 to 206 percent (average 110 percent) of the sum of the normal left and right ventricular end-diastolic volumes. Ejection fraction averaged 0.55. There was no significant difference in ventricular cavity volume characteristics and ventricular function between patients with single and and single right ventricle. The single ventricle ejects blood to both the systemic and the pulmonary circulatory beds. There was a high correlation of end-diastolic volume index with pulmonary to system flow ratio (r = +0.89, p less than 0.001). This observation indicates that the major factor affecting ventricular cavity volume in single ventricle is pulmonary blood flow and a systemic to pulmonary arterial shunt should increase the ventricular cavity volume. Ventricular function of single ventricle was depressed. The ejection fraction of patients with A-V valve regurgitation averaged 0.49, which was significantly lower than that (0.58) of patients without regurgitation (p less than 0.05). A-V valve regurgitation was also a factor that increased ventricular cavity volume, although the ventricular function became more depressed. AN - 7361672 AU - Shimazaki, Y. AU - Kawashima, Y. AU - Mori, T. AU - Kitamura, S. AU - Matsuda, H. AU - Yokota, K. DA - Apr DB - PubMed DO - 10.1016/0002-9149(80)90125-3 DP - NLM ET - 1980/04/01 IS - 4 KW - Adolescent Angiocardiography Blood Circulation Child Child, Preschool Cineangiography Diastole Female Heart Ventricles/*abnormalities/anatomy & histology/surgery Humans Infant Male Pulmonary Circulation Pulmonary Valve Stenosis/physiopathology Regression Analysis LA - eng N1 - Shimazaki, Y Kawashima, Y Mori, T Kitamura, S Matsuda, H Yokota, K Journal Article United States Am J Cardiol. 1980 Apr;45(4):806-10. doi: 10.1016/0002-9149(80)90125-3. PY - 1980 SN - 0002-9149 (Print) 0002-9149 SP - 806-10 ST - Ventricular volume characteristics of single ventricle before corrective surgery T2 - Am J Cardiol TI - Ventricular volume characteristics of single ventricle before corrective surgery VL - 45 ID - 458 ER - TY - JOUR AB - Background: Viral-associated trichodysplasia of immunosuppression is an increasingly recognized entity characterized by follicular-based papules, primarily in the central part of the face, that produce variable degrees of alopecia and dysmorphic features. It has been primarily described in transplant recipients but has recently been recognized in patients receiving chemotherapy for leukemia and lymphoma. It is associated with distinctive histologic features such as dilated anagen hair follicles, absent hair papillae, and abrupt cornification of the inner root sheath. Observations: A 5-year-old boy presented with spiny follicular papules that caused thickening of the skin of the face 1 year after cardiac transplantation. He had been exposed to several immunosuppressive agents, including mycophenolate mofetil, tacrolimus, intravenous immunoglobulin, rituximab, cylcophosphamide, and prednisone. Despite the failure of multiple topical treatments, our patient's eruption improved with systemic valganciclovir therapy. Conclusions: We describe the youngest patient (to our knowledge) with viral-associated trichodysplasia of immunosuppression and discuss the characteristic clinicopathologic features. Our report supports the theory that immunosuppression is the predisposing factor to a folliculotropic papovavirus that alters follicular maturation. ©2010 American Medical Association. All rights reserved. AD - T. Benoit, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina, MSC 578, 135 Rutledge Ave, Charleston, SC 29425, United States AU - Benoit, T. AU - Bacelieri, R. AU - Morrell, D. S. AU - Metcalf, J. DB - Embase Medline DO - 10.1001/archdermatol.2010.175 IS - 8 KW - ammonium lactate cidofovir cyclophosphamide eosin hematoxylin hyalin immunoglobulin inotropic agent mycophenolate mofetil prednisone retinoic acid rituximab tacrolimus tolonium chloride triamcinolone urea valganciclovir article cardiac graft rejection case report cell nucleus child drug substitution drug withdrawal dysplasia electron microscopy erythema face fibrosis general anesthesia graft versus host reaction heart transplantation histiocyte histology histopathology human human tissue hyperkeratosis hypoplastic left heart syndrome immunosuppressive treatment Kabuki makeup syndrome keratinocyte keratosis Darier disease limb lymphocytic infiltration male papovavirus papule plasmapheresis preschool child priority journal pruritus punch biopsy skin biopsy skinfold staining thigh trunk viral associated trichodysplasia of immunosuppression LA - English M3 - Article N1 - L359405163 2010-09-01 2010-09-07 PY - 2010 SN - 1538-3652 0003-987X SP - 871-874 ST - Viral-associated trichodysplasia of immunosuppression: Report of a pediatric patient with response to oral valganciclovir T2 - Archives of Dermatology TI - Viral-associated trichodysplasia of immunosuppression: Report of a pediatric patient with response to oral valganciclovir UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L359405163 http://dx.doi.org/10.1001/archdermatol.2010.175 VL - 146 ID - 1155 ER - TY - JOUR AB - OBJECTIVE: To determine whether visual-spatial processing style is associated with psychopathology in a large sample of adolescents with critical congenital heart disease (CHD). Local (part-oriented) style was hypothesized to increase risk for internalizing (but not externalizing) forms of psychopathology. METHOD: Participants included 278 adolescents with critical CHD (dextro-transposition of the great arteries = 134, tetralogy of Fallot = 58, single-ventricle cardiac anatomy requiring the Fontan procedure = 86). Visual-spatial processing style was indexed using Copy Style Ratings from the Rey-Osterrieth Complex Figure-Developmental Scoring System. The Schedule for Affective Disorders and Schizophrenia for School-Aged Children-Present & Lifetime Version was used to determine presence/absence of diagnosable DSM-IV psychiatric disorder(s). Processing style and psychopathology were assessed concurrently. RESULTS: Thirty-three percent of the sample had a part-oriented processing style. In multivariable binary logistic regression models, part-orientation was associated with more than twice the odds of having an anxiety disorder (lifetime: OR = 2.2, p = .02, 95% CI = 1.1-4.1; current: OR = 2.7, p = .03, 95% CI = 1.1-6.5) but was not associated with an increased risk for ADHD, disruptive behavior, or mood disorders (ps > .05). CONCLUSIONS: Adolescents with critical CHD who approach complex visual-spatial materials in a local, part-oriented fashion are more likely to meet criteria for an anxiety disorder than those who approach complexity more holistically. Part-orientation may make it more difficult for individuals to judge the relative importance of isolated details and engage in more adaptive perspective-taking. AD - a Department of Psychiatry , Boston Children's Hospital, Harvard Medical School , Boston , MA , USA. b Department of Neurology , Boston Children's Hospital, Harvard Medical School , Boston , MA , USA. c Department of Cardiology , Boston Children's Hospital, Harvard Medical School , Boston , MA , USA. AN - 30585527 AU - Cassidy, A. R. AU - Bernstein, J. H. AU - Bellinger, D. C. AU - Newburger, J. W. AU - DeMaso, D. R. C2 - PMC6525045 C6 - NIHMS1514814 DA - May DB - PubMed DO - 10.1080/13854046.2018.1503333 DP - NLM ET - 2018/12/27 IS - 4 KW - Adolescent Female Heart Defects, Congenital/*psychology Humans Male Psychopathology/*methods Spatial Processing/*physiology Vision, Ocular/*physiology *adhd *anxiety *cardiac *cognitive style *pediatric LA - eng N1 - 1744-4144 Cassidy, Adam R Bernstein, Jane Holmes Bellinger, David C Newburger, Jane W DeMaso, David R M01 RR002172/RR/NCRR NIH HHS/United States P50 HL074734/HL/NHLBI NIH HHS/United States R01 HL077681/HL/NHLBI NIH HHS/United States R01 HL096825/HL/NHLBI NIH HHS/United States Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't Clin Neuropsychol. 2019 May;33(4):760-778. doi: 10.1080/13854046.2018.1503333. Epub 2018 Dec 26. PY - 2019 SN - 1385-4046 (Print) 1385-4046 SP - 760-778 ST - Visual-spatial processing style is associated with psychopathology in adolescents with critical congenital heart disease T2 - Clin Neuropsychol TI - Visual-spatial processing style is associated with psychopathology in adolescents with critical congenital heart disease VL - 33 ID - 202 ER - TY - JOUR AB - Among the most significant factors affecting quality of life in individuals with critical congenital heart disease (CCHD) are neurodevelopmental challenges, including deficits in visuospatial processing and academic achievement. Few studies have compared outcomes across CCHD subgroups, despite their significant differences in anatomy/physiology and medical/surgical courses. This study compared visuospatial processing abilities using the Developmental Scoring System for the Rey-Osterrieth Complex Figure (DSS-ROCF) across groups of adolescents with CCHD (d-transposition of the great arteries [TGA, n = 139], Tetralogy of Fallot [TOF, n = 68], single-ventricle cardiac anatomy requiring the Fontan operation [SVF, n = 145]) and a group of healthy controls (CTR, n = 111), and examined the validity of visuospatial processing in predicting concurrent academic outcomes. The CCHD subgroups were found to differ in Organization, ps < .001, Structural Accuracy, ps < .001, and Incidental Elements Accuracy scores, ps ≤ .008; the post hoc analyses show that the SVF group tended to underperform compared to the other CCHD groups. With respect to academic skills, all CCHD groups scored lower than the CTR group, ps ≤ .007; however, the CCHD groups were not different from each other, ps > .23. The regression results showed that the DSS-ROCF Style rating (reflecting integration) accounted for a small yet statistically significant portion of unique variance in "assembled" academic outcomes, over and above the variance already accounted for by DSS-ROCF Organization, p < .01. These findings support the need for comprehensive neuropsychological assessment and monitoring of children and adolescents with CCHD, as well as targeted intervention for organization and integration deficits that may increase their risk for academic underachievement. AD - a Department of Psychiatry , Boston Children's Hospital , Boston , MA , USA. AN - 28277152 AU - Bean Jaworski, J. L. AU - White, M. T. AU - DeMaso, D. R. AU - Newburger, J. W. AU - Bellinger, D. C. AU - Cassidy, A. R. C2 - PMC6131702 C6 - NIHMS1505297 DA - May DB - PubMed DO - 10.1080/09297049.2017.1283396 DP - NLM ET - 2017/03/10 IS - 4 KW - Academic Success Adolescent Child Female Heart Defects, Congenital/*complications/pathology Humans Male Quality of Life/*psychology Spatial Navigation/*physiology *Adolescence *Cardiac *Congenital heart defect *Rey–Osterrieth Complex Figure *Visuospatial LA - eng N1 - 1744-4136 Bean Jaworski, Jessica L White, Matthew T DeMaso, David R Newburger, Jane W Bellinger, David C Cassidy, Adam R R01 HL077681/HL/NHLBI NIH HHS/United States M01 RR002172/RR/NCRR NIH HHS/United States P50 HL074734/HL/NHLBI NIH HHS/United States U54 HD090255/HD/NICHD NIH HHS/United States R01 HL096825/HL/NHLBI NIH HHS/United States Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't Child Neuropsychol. 2018 May;24(4):451-468. doi: 10.1080/09297049.2017.1283396. Epub 2017 Feb 16. PY - 2018 SN - 0929-7049 (Print) 0929-7049 SP - 451-468 ST - Visuospatial processing in adolescents with critical congenital heart disease: Organization, integration, and implications for academic achievement T2 - Child Neuropsychol TI - Visuospatial processing in adolescents with critical congenital heart disease: Organization, integration, and implications for academic achievement VL - 24 ID - 100 ER - TY - JOUR AB - Long-term outcome following the Fontan operation may be affected by the amount of energy lost as blood flows through the anastomosis geometry. A method for detailed quantification of energy loss is applied to computational simulations of the flow in an atriopulmonary and a total cavopulmonary model. Five types of flow (near wall, slow recirculation, medium speed vortices, collision, and streamlined flow) are identified and their energy losses quantified. The presence of recirculation regions decreases the efficiency of the atriopulmonary model, and a region of increased energy loss is seen in the collision region in the total cavopulmonary model. However, the most significant energy loss is through wall shear stress, which is maximal in areas where there is rapid, near wall flow. © Springer Science+Business Media, Inc. 2006. AD - G.D. Mallinson, Department of Mechanical Engineering, University of Auckland, Private Bag 92019, Auckland, New Zealand AU - Moyle, K. R. AU - Mallinson, G. D. AU - Occleshaw, C. J. AU - Cowan, B. R. AU - Gentles, T. L. DB - Embase Medline DO - 10.1007/s00246-005-0918-3 IS - 3 KW - adult anastomosis article blood flow blood flow velocity energy expenditure female flow kinetics Fontan procedure hemodynamics human male model school child shear stress simulation vortex motion LA - English M3 - Article N1 - L43828669 2006-06-21 PY - 2006 SN - 0172-0643 SP - 309-315 ST - Wall shear stress is the primary mechanism of energy loss in the Fontan connection T2 - Pediatric Cardiology TI - Wall shear stress is the primary mechanism of energy loss in the Fontan connection UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L43828669 http://dx.doi.org/10.1007/s00246-005-0918-3 VL - 27 ID - 1261 ER - TY - JOUR AB - Given the current excellent early mortality rates for paediatric cardiac surgery, stakeholders believe that this important safety outcome should be supplemented by a wider range of measures. Our objectives were to prospectively measure the incidence of morbidities following paediatric cardiac surgery and to evaluate their clinical and health-economic impact over 6 months. Design The design was a prospective, multicentre, multidisciplinary mixed methods study. Setting The setting was 5 of the 10 paediatric cardiac surgery centres in the UK with 21 months recruitment. Participants Included were 3090 paediatric cardiac surgeries, of which 666 patients were recruited to an impact substudy. Results Families and clinicians prioritised: Acute neurological event, unplanned re-intervention, feeding problems, renal replacement therapy, major adverse events, extracorporeal life support, necrotising enterocolitis, postsurgical infection and prolonged pleural effusion or chylothorax. Among 3090 consecutive surgeries, there were 675 (21.8%) with at least one of these morbidities. Independent risk factors for morbidity included neonatal age, complex heart disease and prolonged cardiopulmonary bypass (p<0.001). Among patients with morbidity, 6-month survival was 88.2% (95% CI 85.4 to 90.6) compared with 99.3% (95% CI 98.9 to 99.6) with none of the morbidities (p<0.001). The impact substudy in 340 children with morbidity and 326 control children with no morbidity indicated that morbidity-related impairment in quality of life improved between 6 weeks and 6 months. When compared with children with no morbidities, those with morbidity experienced a median of 13 (95% CI 10.2 to 15.8, p<0.001) fewer days at home by 6 months, and an adjusted incremental cost of £21 292 (95% CI £17 694 to £32 423, p<0.001). Conclusions Evaluation of postoperative morbidity is more complicated than measuring early mortality. However, tracking morbidity after paediatric cardiac surgery over 6 months offers stakeholders important data that are of value to parents and will be useful in driving future quality improvement. AD - K.L. Brown, Cardiorespiratory Division, Great Ormond Street Hospital for Children, London, United Kingdom AU - Brown, K. L. AU - Pagel, C. AU - Ridout, D. AU - Wray, J. AU - Anderson, D. AU - Barron, D. J. AU - Cassidy, J. AU - Davis, P. AU - Hudson, E. AU - Jones, A. AU - McLean, A. AU - Morris, S. AU - Rodrigues, W. AU - Sheehan, K. AU - Stoica, S. AU - Tibby, S. M. AU - Witter, T. AU - Tsang, V. T. DB - Embase Medline DO - 10.1136/bmjopen-2018-028533 IS - 9 KW - age aortic arch anomaly aortic occlusion aortic regurgitation article cardiopulmonary bypass child chylothorax controlled study extracorporeal oxygenation feeding difficulty female great vessels transposition health care cost heart atrium septum defect heart single ventricle heart surgery heart ventricle septum defect human hypoplastic left heart syndrome incidence infant lung vein drainage anomaly major clinical study male morbidity necrotizing enterocolitis newborn patent ductus arteriosus pediatric surgery pleura effusion postoperative complication preschool child prospective study pulmonary valve atresia quality of life renal replacement therapy surgical infection survival rate survival time tricuspid valve disease LA - English M3 - Article N1 - L629263410 2019-09-20 2019-09-23 PY - 2019 SN - 2044-6055 ST - What are the important morbidities associated with paediatric cardiac surgery? A mixed methods study T2 - BMJ Open TI - What are the important morbidities associated with paediatric cardiac surgery? A mixed methods study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L629263410 http://dx.doi.org/10.1136/bmjopen-2018-028533 VL - 9 ID - 601 ER - TY - JOUR AB - BACKGROUND: Despite the low early mortality of Fontan procedures, Fontan patients are prone to vari-ous cardiac and extra-cardiac complications in the long term. This may influence patient perception of their health and outcome. The aim of the study was to assess the relationship of multi-organ compli-cations and physical efficiency with self-reported health-related quality of life (QOL) in adult Fontan patients. METHODS: Quality of life was assessed with the Short Form-36 questionnaire. Laboratory tests were done together with echocardiography, plethysmography, and cardiopulmonary exercise test. RESULTS: The QOL was poorer in patients than in control subjects. The physical characteristics of patients correlated with dynamic ventilatory parameters, heart rate at the peak of exercise, alanine aminotransferase and albumin level. CONCLUSIONS: Liver impairment and chronotropic incompetence during exercise are associated with poor QOL in patients after Fontan procedure. In these patient, hepatic, pulmonary and cardiac functions should be carefully monitored. (Cardiol J 2018; 25, 1: 72-80). AD - Jagiellonian University Medical College Institute of Cardiology Departament of Cardiac and Vascular Diseases. monikasmas@gmail.com. AN - 28695974 AU - Smaś-Suska, M. AU - Dłużniewska, N. AU - Weryński, P. AU - Pająk, J. AU - Płazak, W. AU - Olszowska, M. AU - Podolec, P. AU - Tomkiewicz-Pająk, L. DB - PubMed DO - 10.5603/CJ.a2017.0078 DP - NLM ET - 2017/07/12 IS - 1 KW - Adolescent Adult Child Child, Preschool Echocardiography Exercise Test Female *Fontan Procedure *Health Status Heart Defects, Congenital/psychology/*surgery Humans Male Postoperative Period *Quality of Life *Surveys and Questionnaires Young Adult *sf-36 *adult Fontan patients *exercise tolerance *multiorgan complications *pulmonary function test LA - eng N1 - 1897-5593 Smaś-Suska, Monika Dłużniewska, Natalia Weryński, Piotr Pająk, Jacek Płazak, Wojciech Olszowska, Maria Podolec, Piotr Tomkiewicz-Pająk, Lidia Journal Article Poland Cardiol J. 2018;25(1):72-80. doi: 10.5603/CJ.a2017.0078. Epub 2017 Jul 11. PY - 2018 SN - 1898-018x SP - 72-80 ST - What determines the quality of life of adult patients after Fontan procedure? T2 - Cardiol J TI - What determines the quality of life of adult patients after Fontan procedure? VL - 25 ID - 41 ER - TY - JOUR AB - Adults with congenital heart disease (CHD) represent a growing population of patients thanks to the medical and surgical advances which enable at least 85% of children to survive to adulthood. These advances may create quality-of-life (QoL) issues not previously considered. The purpose of this cross-sectional study of 124 adults with CHD was to describe their QoL as a basis for providing appropriate information, counseling, and anticipatory guidance. Thirteen patients had single ventricle physiology (SVP), 43 had cyanotic lesions with 2 ventricle repairs, and 68 had acyanotic CHD. On the basis of Sickness Impact Profile (SIP) scores, individuals with SVP had worse QoL than did those with cyanotic lesions (with 2 ventricle repairs) and acyanotic anomalies (SIP = 9.98 vs 4.61 and 3.76). SIP scores were statistically significantly different between those with SVP and those with acyanotic anomalies (P = .02). For all groups, the areas of life most affected were work and sleep and rest. Participants with SVP saw themselves as having the poorest QoL. AD - School of Nursing, Duquesne University, Pittsburgh, PA 15282, USA. Simko@duq.edu AN - 15870592 AU - Simko, L. C. AU - McGinnis, K. A. DA - May-Jun DB - PubMed DO - 10.1097/00005082-200505000-00013 DP - NLM ET - 2005/05/05 IS - 3 KW - Activities of Daily Living Adult Analysis of Variance *Attitude to Health Cross-Sectional Studies Cyanosis/*etiology Disabled Persons/classification/psychology Female Health Knowledge, Attitudes, Practice Health Status Heart Defects, Congenital/classification/*complications/*psychology Humans Male Needs Assessment New England Nurse's Role Nursing Assessment Patient Education as Topic Quality of Life/*psychology Severity of Illness Index Sickness Impact Profile Surveys and Questionnaires LA - eng N1 - Simko, Lynn Coletta McGinnis, Kathleen A Comparative Study Journal Article Research Support, Non-U.S. Gov't United States J Cardiovasc Nurs. 2005 May-Jun;20(3):206-14. doi: 10.1097/00005082-200505000-00013. PY - 2005 SN - 0889-4655 (Print) 0889-4655 SP - 206-14 ST - What is the perceived quality of life of adults with congenital heart disease and does it differ by anomaly? T2 - J Cardiovasc Nurs TI - What is the perceived quality of life of adults with congenital heart disease and does it differ by anomaly? VL - 20 ID - 377 ER - TY - JOUR AD - A.S. Teebi, Division of Clinical Genetics, Hospital for Sick Children, 555 University Ave., Toronto, Ont. M5G-1X8, Canada AU - Al-Khenaizan, S. AU - Al-Sannaa, N. AU - Teebi, A. S. DB - Embase Medline DO - 10.1046/j.1525-1470.2001.018001068.x IS - 1 KW - article case report child ectodermal dysplasia Ellis van Creveld syndrome female human hypoplastic left heart syndrome patent ductus arteriosus polydactyly priority journal LA - English M3 - Article N1 - L32163920 2001-03-06 PY - 2001 SN - 0736-8046 SP - 68-70 ST - What syndrome is this? T2 - Pediatric Dermatology TI - What syndrome is this? UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L32163920 http://dx.doi.org/10.1046/j.1525-1470.2001.018001068.x VL - 18 ID - 1325 ER - TY - JOUR AB - BACKGROUND: Families of children at the worst end of the congenital heart disease endure a significant burden which is often not clearly delineated in the clinical literature. We examined the greatest concerns of parents whose children have a Fontan circulation. METHODS: Parents (N = 107) of children in the Australian and New Zealand Fontan Registry completed online surveys with open-ended and closed questions. A qualitative method approach incorporating thematic analyses was used. RESULTS: The greatest concerns for parents of a child with a Fontan circulation were centered on fear of death for their child and psychosocial well-being, followed by lesser themes around anti-coagulation use, pregnancy and financial burdens. CONCLUSIONS: Fear of death and the psychological well-being of their children were the main parental concerns. It highlights the need to clearly communicate information on outcomes to families, and the need for family-focused psychological interventions to improve the psychosocial functioning of both parents and young people. AD - Murdoch Children's Research Institute, Melbourne, Victoria, Australia. Australian and New Zealand Fontan Advisory Committee. Cardiac Surgery Department, Royal Children's Hospital, Melbourne, Victoria, Australia. Department of Pediatrics, Faculty of Medicine, The University of Melbourne, Melbourne, Victoria, Australia. AN - 29876506 AU - du Plessis, K. AU - Peters, R. AU - King, I. AU - Robertson, K. AU - Mackley, J. AU - Maree, R. AU - Stanley, T. AU - Pickford, L. AU - Rose, B. AU - Orchard, M. AU - Stewart, H. AU - d'Udekem, Y. C2 - PMC5988481 DA - Mar DB - PubMed DO - 10.1016/j.ijcha.2018.02.008 DP - NLM ET - 2018/06/08 KW - ANZFAC, Australian and New Zealand Fontan Advisory Committee AVSD, Atrioventricular Septal Defect Congenital heart disease DILV, Double Inlet Left Ventricle DORV, Double Outlet Right Ventricle Fontan HLHS, Hypoplastic Left Heart Syndrome INR, International Normalized Ratio PLE, Protein Losing Enteropathy PTSD, Post-Traumatic Stress Disorder (PTSD) Parents of children with a Fontan circulation Qualitative research ccTGA, Congenitally Corrected Transposition of the Great Arteries LA - eng N1 - 2352-9067 du Plessis, Karin Peters, Rebecca King, Ingrid Robertson, Kirsty Mackley, Jonathan Maree, Rachel Stanley, Tracy Pickford, Louise Rose, Brian Orchard, Matthew Stewart, Helen d'Udekem, Yves Journal Article Int J Cardiol Heart Vasc. 2018 Mar 9;18:65-70. doi: 10.1016/j.ijcha.2018.02.008. eCollection 2018 Mar. PY - 2018 SN - 2352-9067 (Print) 2352-9067 SP - 65-70 ST - "Will she live a long happy life?" Parents' concerns for their children with Fontan circulation T2 - Int J Cardiol Heart Vasc TI - "Will she live a long happy life?" Parents' concerns for their children with Fontan circulation VL - 18 ID - 96 ER - TY - JOUR AB - A 13-months old boy was admitted in National Heart Foundation Hospital and Research Institute on 3 August 2011 with the diagnosis of Dextrocardia, A-V discordance, DORV, large perimembranous VSD, severe infundibular and valvular PS, bilateral SVC. He was operated on 10 August 2011. Bilateral bidirectional Glenn shunt was done off pump along with interruption of PDA. Antegrade pulmonary blood flow was minimized by tight PA banding. Baby was extubated 3 hours after surgery but had to reintubate immediately due to intense respiratory distress. Subsequent three trials of extubation failed. Chest x-ray revealed elevation of both the hemidiaphragm. Ultrasonogram of abdomen and Bronchogram along with fluoroscopy done and bilateral diaphragmatic palsy was diagnosed. Tracheostomy was done on 25th August 2011. Plication of left hemidiaphragm was done on 27th August and right hemidiaphragm plication was done on 10th September 2011. Though it took long period of time we managed to take him out of ventilator on 57th postoperative day. He was oxygen dependent for a period of time and finally he managed to take his own breath without tracheostomy tube from 67th postoperative day. After a long eventful postoperative hospital stay he was discharged home on 78th postoperative day. Discharge Chest x-ray revealed well expanded lung with flattened diaphragm. Echo revealed well functioning bilateral Glenn shunt. Tracheostomy wound healed nicely and there was no evidence of tracheal stenosis. AD - Dr AK Shamsuddin, Associate Professor & Senior Consultant, Pediatric Cardiac Surgery, National Heart Foundation Hospital and Research Institute (NHFH & RI), Sher-E-Bangladesh, Bangladesh. AN - 25178619 AU - Shamsuddin, A. K. AU - Biswas, S. K. AU - Rahman, M. Z. AU - Biswas, S. AU - Hasan, N. A. AU - Sharifuzzaman, M. DA - Jul DB - PubMed DP - NLM ET - 2014/09/03 IS - 3 KW - Fontan Procedure/*adverse effects Humans Infant Male Respiratory Paralysis/*etiology/surgery LA - eng N1 - Shamsuddin, A K Biswas, S K Rahman, M Z Biswas, S Hasan, N A Sharifuzzaman, M Case Reports Journal Article Bangladesh Mymensingh Med J. 2014 Jul;23(3):595-8. PY - 2014 SN - 1022-4742 (Print) 1022-4742 SP - 595-8 ST - A young child with bilateral diaphragmatic palsy after bilateral bidirectional Glenn shunt T2 - Mymensingh Med J TI - A young child with bilateral diaphragmatic palsy after bilateral bidirectional Glenn shunt VL - 23 ID - 450 ER -