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 amphetamine derivative serotonin uptake inhibitor aortic coarctation aortic stenosis article cardiovascular risk congenital heart disease congenital heart malformation drug exposure drug safety drug traffic fetus monitoring human hypoplastic left heart syndrome patient safety prenatal exposure smoking cessation tobacco dependence LA - English M3 - Article N1 - L368887147 2013-05-17 2013-05-29 http://english.prescrire.org/en/20338B806CD5CA10A1DF6E78CC35577F/Download.aspx PY - 2012 SN - 1167-7422 SP - 97 ST - Bupropion: Congenital heart defects T2 - Prescrire International TI - Bupropion: Congenital heart defects UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L368887147 VL - 21 ID - 1064 ER - TY - JOUR AB - Abstracts of the journal's online articles are presented including "Preserving Patient Privacy and Confidentiality in the Era of Personal Health Records" by C. Fabienne and colleagues, "Outcomes of Infants Born to Women Infected With Hepatitis B" by Dr. Sarah Schillie and colleagues, and "Cow's Milk Contamination of Human Milk Purchased via the Internet" by A. Sarah and colleagues. AN - 102711342. Language: English. Entry Date: 20150520. Revision Date: 20150525. Publication Type: Abstract 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. 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=102711342&site=ehost-live&scope=site VL - 135 ID - 1551 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 - P. Amedro, Paediatric and Congenital Cardiology Department, Arnaud De Villeneuve University Hospital, 371 Avenue du Doyen Giraud, Montpellier, France 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. DB - Embase Medline DO - 10.1016/j.ijcard.2019.02.069 KW - NCT01202916 age distribution airway obstruction article body mass child childhood clinical outcome congenital heart disease controlled study cross-sectional study exercise female forced expiratory volume forced vital capacity genetic disorder heart catheterization heart single ventricle heart surgery heart ventricle heterotaxy syndrome human lung function major clinical study male oxygen consumption priority journal quality of life risk factor LA - English M3 - Article N1 - L2001670799 2019-03-14 PY - 2019 SN - 1874-1754 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 - International Journal of Cardiology TI - Impaired pulmonary function and its association with clinical outcomes, exercise capacity and quality of life in children with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2001670799 http://dx.doi.org/10.1016/j.ijcard.2019.02.069 VL - 285 ID - 612 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. © 2019 Elsevier B.V. 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 Epidemiology Department, Montpellier University Hospital, Clinical Investigation Centre, INSERM–CIC 1411, University of Montpellier, 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 PhyMedExp, University of Montpellier, INSERM, CNRS, France 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. DB - Scopus DO - 10.1016/j.ijcard.2019.02.069 KW - Children Congenital heart disease Lung Pulmonary function Spirometry M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2019 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 - International Journal of Cardiology TI - Impaired pulmonary function and its association with clinical outcomes, exercise capacity and quality of life in children with congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85062665547&doi=10.1016%2fj.ijcard.2019.02.069&partnerID=40&md5=8ea52405f2b346e2ad10f3605ba491bd VL - 285 ID - 1746 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 - R. Aeba, Div. Cardiovascular Surg., Keio University, 35 Shinanomachi, Shinjuku, Tokyo 160-8582, Japan AU - Aeba, R. AU - Katogi, T. AU - Takeuchi, S. AU - Kawada, S. DB - Embase Medline IS - 1 KW - adolescent adult artery blood flow article child cyanotic heart disease human infant pulmonary valve atresia major clinical study multivariate analysis newborn pulmonary artery reoperation shunting survival rate LA - English M3 - Article N1 - L30192891 2000-04-19 PY - 2000 SN - 0021-9509 SP - 23-30 ST - Outcome of patients with cyanotic congenital heart disease undergoing a second systemic-to-pulmonary artery shunt T2 - Journal of Cardiovascular Surgery TI - Outcome of patients with cyanotic congenital heart disease undergoing a second systemic-to-pulmonary artery shunt UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L30192891 VL - 41 ID - 1339 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 Division of Cardiovascular Surgery, Keio University, 35 Shinanomachi, Shinjuku, Tokyo 160-8582, Japan AU - Aeba, R. AU - Katogi, T. AU - Takeuchi, S. AU - Kawada, S. DB - Scopus IS - 1 KW - Heart defects, congenital surgery Heart ventricle abnormalities Tetralogy of Fallot M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2000 SP - 23-30 ST - Outcome of patients with cyanotic congenital heart disease undergoing a second systemic-to-pulmonary artery shunt T2 - Journal of Cardiovascular Surgery TI - Outcome of patients with cyanotic congenital heart disease undergoing a second systemic-to-pulmonary artery shunt UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0034027224&partnerID=40&md5=999362f2298af7f77e71ee7130119c1f VL - 41 ID - 2276 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), VO2 of predicted (+ 8.5, 95% CI 0.1–17.0, p = 0.05), VO2 peak (+ 4.3, 95% CI 0.3 to 8.2, p = 0.04), and VO2 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 - A. Pingitore, Clinical Physiology Institute, CNR, Via Moruzzi 1, Pisa, Italy 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. DB - Embase Medline DO - 10.1007/s00246-018-1808-9 IS - 4 KW - adolescent aerobic capacity article breathing exercise cardiopulmonary test cardiorespiratory fitness clinical article clinical effectiveness controlled respiratory training controlled study endurance training female Fontan procedure human lung function test male outcome assessment patient safety prediction repeat procedure LA - English M3 - Article N1 - L620318179 2018-01-26 2018-08-13 PY - 2018 SN - 1432-1971 0172-0643 SP - 695-704 ST - Respiratory Training Late After Fontan Intervention: Impact on Cardiorespiratory Performance T2 - Pediatric Cardiology TI - Respiratory Training Late After Fontan Intervention: Impact on Cardiorespiratory Performance UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L620318179 http://dx.doi.org/10.1007/s00246-018-1808-9 VL - 39 ID - 691 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), VO2 of predicted (+ 8.5, 95% CI 0.1–17.0, p = 0.05), VO2 peak (+ 4.3, 95% CI 0.3 to 8.2, p = 0.04), and VO2 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. © 2018, Springer Science+Business Media, LLC, part of Springer Nature. AD - Clinical Physiology Institute, CNR, Via Moruzzi 1, Pisa, 56124, Italy Fondazione G. Monasterio, Regione Toscana, Pisa, Italy Pediatric Cardiology, Brotzu Hospital, Cagliari, Italy Institute of Life Sciences, Scuola Superiore Sant’Anna, Pisa, Italy National Institute of Diabetes and Digestive and Kidney Disease, National Institutes of Health, Phoenix, AZ, United States 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. DB - Scopus DO - 10.1007/s00246-018-1808-9 IS - 4 KW - Congenital heart disease Controlled respiratory training Ergospirometry Fontan circulation M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2018 SP - 695-704 ST - Respiratory Training Late After Fontan Intervention: Impact on Cardiorespiratory Performance T2 - Pediatric Cardiology TI - Respiratory Training Late After Fontan Intervention: Impact on Cardiorespiratory Performance UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85040792006&doi=10.1007%2fs00246-018-1808-9&partnerID=40&md5=644281175d1b1c1af93f3b857d80f2ca VL - 39 ID - 1810 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 > 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/mol 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 - L.N. Benson, Division of Cardiology, The Hospital for Sick Children, 555 University of Avenue, Toronto, Ont. M5G 1X8, Canada 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. DB - Embase Medline IS - 4 KW - article atrioventricular conduction controlled study diastole Fontan procedure heart left ventricle mass heart left ventricle performance human infant major clinical study priority journal scintiangiography systole LA - English M3 - Article N1 - L22336897 1992-11-20 PY - 1992 SN - 0735-1097 SP - 920-926 ST - Ventricular performance before and after fontan repair for univentricular atrioventricular connection: Angiographic and radionuclide assessment T2 - Journal of the American College of Cardiology TI - Ventricular performance before and after fontan repair for univentricular atrioventricular connection: Angiographic and radionuclide assessment UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L22336897 VL - 20 ID - 1382 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 > 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 posirepair 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 contral 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. © 1992. AD - Departments of Pediatrics and Radiology, Divisions of Cardiology. Nuclear Medicine and Cardiovascular Surgery, The Variety Club Cardiac Catheterization Laboratories, Toronto, Ont., Canada 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. DB - Scopus DO - 10.1016/0735-1097(92)90194-R IS - 4 M3 - Article N1 - Cited By :51 Export Date: 15 June 2020 PY - 1992 SP - 920-926 ST - Ventricular performance before and after fontan repair for univentricular atrioventricular connection: Angiographic and radionuclide assessment T2 - Journal of the American College of Cardiology TI - Ventricular performance before and after fontan repair for univentricular atrioventricular connection: Angiographic and radionuclide assessment UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0026482597&doi=10.1016%2f0735-1097%2892%2990194-R&partnerID=40&md5=fba13b5938b6031a90c7d49fa4e2a697 VL - 20 ID - 2316 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 - 108832314. Language: English. Entry Date: 20180117. Revision Date: 20180118. Publication Type: Article AU - Akbariasbagh, Parvin AU - Mirzaghayan, Mohammad Reza AU - Akbariasbagh, Naseredin AU - Shariat, Mamak AU - Ebrahim, Bita DB - ccm DP - EBSCOhost IS - 3 KW - Heart Defects, Congenital Heart Defects, Congenital -- Surgery Heart Surgery Phrenic Nerve Pediatric Surgery Child Female Infant Male N1 - research; tables/charts. Journal Subset: Biomedical; Double Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Middle East; Peer Reviewed. NLM UID: 101289255. PY - 2015 SN - 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=108832314&site=ehost-live&scope=site VL - 10 ID - 1493 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. © 2015, Tehran Heart Center. All rights reserved. AD - Tehran University of Medical Sciences, Imam Khomeini Complex, 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 AU - Akbariasbagh, P. AU - Mirzaghayan, M. R. AU - Akbariasbagh, N. AU - Shariat, M. AU - Ebrahim, B. DB - Scopus IS - 3 KW - Cardiac surgical procedures Diaphragmatic Infant, newborn Phrenic nerve M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2015 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84938337196&partnerID=40&md5=9eb8ea280503137fcf39f2ce84cebc7f VL - 10 ID - 1969 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 4mm) 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. © 2013, The Authors. Artificial Organs © 2013, International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc. AD - Biomedical Engineering Department, Carnegie Mellon University, Pittsburgh, United States Penn State Children's Hospital and Bioengineering, Pennsylvania State University, Hershey, PA, United States Cardiothoracic Surgery Department, Başkent University, Istanbul, Turkey Mechanical Engineering Department, Koc University, Istanbul, Turkey AU - Albal, P. G. AU - Menon, P. G. AU - Kowalski, W. AU - Undar, A. AU - Turkoz, R. AU - Pekkan, K. DB - Scopus DO - 10.1111/aor.12011 IS - 1 KW - Fenestration Fontan surgery Systemic venous hypertension Venous flow shunting M3 - Article N1 - Cited By :6 Export Date: 15 June 2020 PY - 2013 SP - 66-75 ST - Novel Fenestration Designs for Controlled Venous Flow Shunting in Failing Fontans With Systemic Venous Hypertension T2 - Artificial Organs TI - Novel Fenestration Designs for Controlled Venous Flow Shunting in Failing Fontans With Systemic Venous Hypertension UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84872391032&doi=10.1111%2faor.12011&partnerID=40&md5=ad7fde1d278d2d08d949e94800905e75 VL - 37 ID - 2074 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 - Department of Cardiovascular Surgery, V.K.V. American Bristol Hospital, Turkey Department of Pediatric Cardiology, Acibadem-Caroussel Hospital, Istanbul, Turkey Department of Cardiovascular Surgery, Acibadem-Caroussel Hospital, Istanbul, Turkey 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 - Scopus DO - 10.1097/01.mat.0000235275.65027.1d IS - 5 M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 2006 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-33748696816&doi=10.1097%2f01.mat.0000235275.65027.1d&partnerID=40&md5=2276942f2537b865bf80fa0eca95d40a VL - 52 ID - 2226 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 - Departments of Pediatrics and Cardiothoracic Surgery, Stanford University, Stanford, CA, United States AU - Alkhaldi, A. AU - Chin, C. AU - Bernstein, D. DB - Scopus DO - 10.1053/j.sempedsurg.2006.03.011 IS - 3 KW - Cardiomyopathy Congenital heart defects Hypoplastic left heart syndrome Myocarditis Pediatric heart transplant Ventricular dysfunction M3 - Article N1 - Cited By :11 Export Date: 15 June 2020 PY - 2006 SP - 188-198 ST - Pediatric cardiac transplantation T2 - Seminars in Pediatric Surgery TI - Pediatric cardiac transplantation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-33745481297&doi=10.1053%2fj.sempedsurg.2006.03.011&partnerID=40&md5=650ee24e053139ab1380829d79ed2c7b VL - 15 ID - 2227 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 - 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 - Scopus IS - 4 KW - Congenital heart disease Fetus Hypoplastic left heart syndrome M3 - Article N1 - Cited By :84 Export Date: 15 June 2020 PY - 1998 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0031959438&partnerID=40&md5=9ac617bb88f27d9ecbeebd21df7b766f VL - 79 ID - 2288 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. AU - Altmann, J. AU - Kiver, V. AU - Henrich, W. AU - Weichert, A. DB - Medline DO - 10.1515/jpm-2019-0246 IS - 1 KW - article atrioventricular block Beckwith Wiedemann syndrome cancer patient cancer prognosis cardiomyopathy case report child clinical article clinical outcome epileptic focus female fetus fetus hydrops Fontan procedure Glenn shunt heart arrhythmia heart left ventricle ejection fraction human infantile spasm Lesch Nyhan syndrome multiple cancer mutational analysis neurosurgery outcome assessment pericardial effusion pregnancy prognosis psychomotor retardation quality of life retrospective study rhabdomyoma supraventricular tachycardia tonic clonic seizure tuberous sclerosis Wolff Parkinson White syndrome LA - English M3 - Article N1 - L630122841 2019-12-18 PY - 2019 SN - 1619-3997 SP - 74-81 ST - Clinical outcome of prenatally suspected cardiac rhabdomyomas of the fetus T2 - Journal of perinatal medicine TI - Clinical outcome of prenatally suspected cardiac rhabdomyomas of the fetus UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L630122841 http://dx.doi.org/10.1515/jpm-2019-0246 VL - 48 ID - 581 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 BerlinBerlin, Germany Practice for Prenatal DiagnosisBerlin, Germany AU - Altmann, J. AU - Kiver, V. AU - Henrich, W. AU - Weichert, A. DB - Scopus DO - 10.1515/jpm-2019-0246 IS - 1 KW - Beckwith-Wiedemann syndrome cardiac rhabdomyoma cranial tuber Lesch-Nyhan disease tuberous sclerosis complex M3 - Article N1 - Export Date: 15 June 2020 PY - 2019 SP - 74-81 ST - Clinical outcome of prenatally suspected cardiac rhabdomyomas of the fetus T2 - Journal of perinatal medicine TI - Clinical outcome of prenatally suspected cardiac rhabdomyomas of the fetus UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85077173493&doi=10.1515%2fjpm-2019-0246&partnerID=40&md5=d8cd3bbe25a84e1a3d5b977327dc980a VL - 48 ID - 1719 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 - 140960027. Language: English. Entry Date: 20200104. Revision Date: 20200104. Publication Type: Article AU - Altmann, Judith AU - Kiver, Verena AU - Henrich, Wolfgang AU - Weichert, Alexander DB - ccm DO - 10.1515/jpm-2019-0246 DP - EBSCOhost IS - 1 KW - Rhabdomyoma -- Ultrasonography -- In Utero Heart Neoplasms -- Ultrasonography -- In Utero Ultrasonography, Prenatal Tuberous Sclerosis -- Complications -- In Utero Rhabdomyoma -- Complications -- In Utero Heart Neoplasms -- Complications -- In Utero Comorbidity -- Risk Factors Risk Assessment Human Retrospective Design Tertiary Health Care Abortion, Induced Tuberous Sclerosis -- Familial and Genetic Tuberous Sclerosis -- Diagnosis Mutation Postnatal Period Pericardial Effusion -- Diagnosis Extrasystole -- Diagnosis Tachycardia, Ventricular -- Diagnosis Wolff-Parkinson-White Syndrome -- Diagnosis Heart Block -- Diagnosis Cardiomyopathy, Dilated -- Diagnosis Cardiopulmonary Bypass Ventricular Ejection Fraction Epilepsy -- Diagnosis Spasms, Infantile -- Diagnosis Seizures -- Diagnosis Neurosurgery Purine-Pyrimidine Metabolism, Inborn Errors -- Complications Beckwith-Wiedemann Syndrome -- Complications Perinatal Death -- Risk Factors Fetus Treatment Outcomes N1 - diagnostic images; research; tables/charts. Journal Subset: Biomedical; Continental Europe; Double Blind Peer Reviewed; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed. NLM UID: 0361031. PY - 2020 SN - 0300-5577 SP - 74-81 ST - Clinical outcome of prenatally suspected cardiac rhabdomyomas of the fetus T2 - Journal of Perinatal Medicine TI - Clinical outcome of prenatally suspected cardiac rhabdomyomas of the fetus UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=140960027&site=ehost-live&scope=site VL - 48 ID - 1437 ER - TY - JOUR AB - BACKGROUND: Improvements in long-term survival of children undergoing the Norwood staged procedure and the arterial switch operation have resulted in the need to prepare these at-risk children for each stage of their developmental trajectory, including school readiness. This study describes and compares functional outcomes following the Norwood staged procedure and arterial switch operations. METHODS: This prospective inception cohort study comprised a sample of 73 children (71% boys) who had the Norwood staged procedure (n = 28) or the arterial switch operation (n = 45) at the age of 6 weeks or younger at the Stollery Children's Hospital, Edmonton, Alberta, between 2002 and 2005. We excluded children who had chromosomal abnormalities or cerebral palsy. When children were 18-24 months of age, parents completed the Adaptive Behavioral Assessment System II. Standard scores for the domains are mean 100, standard deviation (15); skill area scaled scores, 10 (3). Student's t-test with Bonferonni correction was used to compare groups. RESULTS: This population has greater than four times the number of children delayed on the General Adaptive Composite than the normative group. Functional outcomes were similar in the two groups other than those of home living (Norwood: 8.8 (2.8) compared with arterial switch: 11.2 (3.1), t = 3.389, p = 0.001) and self-care (Norwood: 5.9 (3.5) versus arterial switch: 8.1 (2.6), t = 3.140, p = 0.002). CONCLUSION: These survivors are at increased risk for delayed functional abilities. Self-care, necessary for independence and confidence as children reach school age, was particularly low in the Norwood group. Reasons for low self-care abilities require further study. AD - Stollery Children's Hospital, University of Alberta, Canada. gwen.alton@albertahealthservices.ca AN - 104937682. Language: English. Entry Date: 20110311. Revision Date: 20150711. Publication Type: Journal Article AU - Alton, G. Y. AU - Rempel, G. R. AU - Robertson, C. M. AU - Newburn-Cook, C. V. AU - Norris, C. M. DB - ccm DO - 10.1017/S1047951110000971 DP - EBSCOhost IS - 6 KW - Heart Surgery Child Behavior Developmental Disabilities -- Diagnosis Hypoplastic Left Heart Syndrome -- Surgery Transposition of Great Arteries -- Surgery Child, Preschool Human Infant Infant, Newborn N1 - research. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Special Interest: Pediatric Care. NLM UID: 9200019. PMID: NLM20810009. PY - 2010 SN - 1047-9511 SP - 668-675 ST - Functional outcomes after neonatal open cardiac surgery: comparison of survivors of the Norwood staged procedure and the arterial switch operation T2 - Cardiology in the Young TI - Functional outcomes after neonatal open cardiac surgery: comparison of survivors of the Norwood staged procedure and the arterial switch operation UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104937682&site=ehost-live&scope=site VL - 20 ID - 1603 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. © 2015, Springer Science+Business Media New York. AD - Pediatric and Congenital Cardiology Department, University Hospital, Montpellier, France Pediatric and Congenital Cardiology Department, St-Luc University Hospital, Brussels, Belgium Pediatric and Congenital Cardiology Department, La Timone University Hospital, Marseille, France Department of Public Health, EA 3279 Research Unit, Aix-Marseille University, Marseille, France Clinical Research and Epidemiology Unit, University Hospital, Montpellier, France Clinical Investigation Center, University Hospital, Montpellier, France Physiology and Experimental Biology of Heart and Muscles Laboratory - PHYMEDEXP, UMR CNRS 9214 – INSERM U1046, Montpellier University, Montpellier, France Clinical Investigation Center, INSERM–CIC 1411, 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 - Scopus DO - 10.1007/s00246-015-1201-x IS - 8 KW - Children Congenital heart disease Kidscreen Pediatric cardiology Quality of life M3 - Article N1 - Cited By :32 Export Date: 15 June 2020 PY - 2015 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84947862597&doi=10.1007%2fs00246-015-1201-x&partnerID=40&md5=7b2fa84017e593d666c54afba2d31015 VL - 36 ID - 1932 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). AD - P. Amedro, Paediatric and Congenital Cardiology Department, M3C Regional Reference CHD Centre, CHU Montpellier, Montpellier, 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. DB - Embase Medline DO - 10.1002/ehf2.12630 IS - 2 KW - NCT03153137 amino terminal pro brain natriuretic peptide carbon dioxide oxygen phosphodiesterase V inhibitor placebo sildenafil adolescent adult arterial oxygen saturation article cardiopulmonary exercise test clinical article clinical outcome congenital heart disease controlled study double blind procedure drug efficacy drug safety drug screening heart catheterization heart single ventricle heart stroke volume human human cell lung artery pressure lung ventilation mean arterial pressure multicenter study outcome assessment parallel design phase 3 clinical trial pressure gradient priority journal pulmonary artery occlusion pressure quality of life randomized controlled trial respiratory tract parameters scoring system Short Form 36 six minute walk test World Health Organization LA - English M3 - Article N1 - L2004429016 2020-03-19 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 Failure TI - Efficacy of phosphodiesterase type 5 inhibitors in univentricular congenital heart disease: the SV-INHIBITION study design UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2004429016 http://dx.doi.org/10.1002/ehf2.12630 VL - 7 ID - 554 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. © The Author 2012. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. AD - Pediatric and Grown-up Congenital Cardiac Surgery, University of Bologna, S.Orsola-Malpighi Hospital, Bologna, Italy Pediatric and Grown-up Congenital Cardiology, University of Bologna, S.Orsola-Malpighi Hospital, Bologna, Italy Cardio-Vascular and Thoracic Radiology, University of Bologna, S.Orsola-Malpighi Hospital, Bologna, Italy Psychological Service Pediatric and Grown-up Congenital Cardiac Surgery and Cardiology, University of Bologna, S.Orsola-Malpighi Hospital, Bologna, Italy 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. C7 - ezs202 DB - Scopus DO - 10.1093/ejcts/ezs202 IS - 6 KW - Cardiopulmonary exercise test Fontan operation Quality of life Single-ventricle physiology M3 - Article N1 - Cited By :16 Export Date: 15 June 2020 PY - 2012 SP - 996-1002 ST - Natural and modified history of single-ventricle physiology in adult patients T2 - European Journal of Cardio-thoracic Surgery TI - Natural and modified history of single-ventricle physiology in adult patients UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84873129589&doi=10.1093%2fejcts%2fezs202&partnerID=40&md5=d3c4fa804187a26916fdd71df24095a1 VL - 42 ID - 2078 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. © 2019, Springer-Verlag GmbH Germany, part of Springer Nature. AD - Division of Pediatric Surgery, Ann and Robert H. Lurie Children’s Hospital of Chicago, 211 E Chicago Avenue, Box 63, Chicago, IL 60611, United States Department of Surgery, University of Illinois at Chicago, 840 South Wood Street, Suite 376-CSN, Chicago, IL 60612, United States Feinberg School of Medicine, Northwestern University, 310 East Superior Street, Morton 4-685, Chicago, IL 60611, United States AU - Ares, G. J. AU - Buonpane, C. AU - Helenowski, I. AU - Reynolds, M. AU - Hunter, C. J. DB - Scopus DO - 10.1007/s00383-019-04443-y IS - 3 KW - Long-run Moral distress, Ethics Neonatal ECMO Neurodevelopmental outcome Pediatric ECMO M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2019 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85060759517&doi=10.1007%2fs00383-019-04443-y&partnerID=40&md5=5d3972a53fc73c464e4500c189d4546c VL - 35 ID - 1756 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. © 2012 Cambridge University Press. AD - A.M. Atz, Division of Cardiology, Department of Pediatrics, Medical University of South Carolina, Charleston, SC 29425, United States 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. DB - Embase Medline DO - 10.1017/S1047951112001175 IS - 3 KW - NCT00132782 anticoagulant agent dipeptidyl carboxypeptidase inhibitor diuretic agent adolescent adult article cardiopulmonary bypass cardiovascular parameters cavopulmonary connection child chylothorax cross-sectional study disease association echocardiography Fontan procedure heart performance heart rhythm human hypoplastic left heart syndrome major clinical study outcome assessment preschool child quality of life school child LA - English M3 - Article N1 - L368879239 2013-05-16 2013-05-27 PY - 2013 SN - 1047-9511 1467-1107 SP - 335-343 ST - Cardiac performance and quality of life in patients who have undergone the Fontan procedure with and without prior superior cavopulmonary connection T2 - Cardiology in the Young TI - Cardiac performance and quality of life in patients who have undergone the Fontan procedure with and without prior superior cavopulmonary connection UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L368879239 http://dx.doi.org/10.1017/S1047951112001175 VL - 23 ID - 994 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 - 1 Division of Cardiology, Department of Pediatrics, Medical University of South Carolina, Charleston, South Carolina, United States of America. AN - 104072395. Corporate Author: Pediatric Heart Network Investigators. Language: English. Entry Date: 20140124. Revision Date: 20161117. Publication Type: journal article AU - Atz, Andrew M. AU - Travison, Thomas G. AU - McCrindle, Brian W. AU - Mahony, Lynn AU - Glatz, Andrew C. AU - Kaza, Aditya K. AU - Breitbart, Roger E. AU - Colan, Steven D. AU - Kaltman, Jonathan R. AU - Margossian, Renee AU - Pasquali, Sara K. AU - Wang, Yanli AU - Gersony, Welton M. DB - ccm DO - 10.1017/S1047951112001175 DP - EBSCOhost IS - 3 KW - Cardiopulmonary Bypass -- Methods Heart Defects, Congenital -- Surgery Quality of Life Adolescence Child Child, Preschool Cross Sectional Studies Female Heart Defects, Congenital -- Physiopathology Heart Function Tests Human Male Treatment Outcomes N1 - research. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Special Interest: Pediatric Care. Grant Information: HL068292/HL/NHLBI NIH HHS/United States. NLM UID: 9200019. PMID: NLM22824161. PY - 2013 SN - 1047-9511 SP - 335-343 ST - Cardiac performance and quality of life in patients who have undergone the Fontan procedure with and without prior superior cavopulmonary connection T2 - Cardiology in the Young TI - Cardiac performance and quality of life in patients who have undergone the Fontan procedure with and without prior superior cavopulmonary connection UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104072395&site=ehost-live&scope=site VL - 23 ID - 1524 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. © 2012 Cambridge University Press. AD - Division of Cardiology, Department of Pediatrics, Medical University of South Carolina, Charleston, SC 29425, United States New England Research Institutes, Watertown, MA, United States Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto, Canada Department of Pediatrics, University of Texas Southwestern, Dallas, TX, United States Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA, United States Department of Surgery, University of Utah, Salt Lake City, UT, United States Department of Cardiology, Children's Hospital Boston, Boston, MA, United States National Heart Lung and Blood Institute, National Institutes of Health, Bethesda, MD, United States Department of Pediatrics, Duke University Medical Center, Durham, NC, United States Division of Pediatrics, Columbia University Medical Center, New York, NY, United States 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. DB - Scopus DO - 10.1017/S1047951112001175 IS - 3 KW - Congenital heart disease Fontan functionally univentricular heart quality of life M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 2013 SP - 335-343 ST - Cardiac performance and quality of life in patients who have undergone the Fontan procedure with and without prior superior cavopulmonary connection T2 - Cardiology in the Young TI - Cardiac performance and quality of life in patients who have undergone the Fontan procedure with and without prior superior cavopulmonary connection UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84877331349&doi=10.1017%2fS1047951112001175&partnerID=40&md5=8edb1d8b71a1f8da7c6f1f94ca51bac8 VL - 23 ID - 2044 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 - A.M. Atz, Department of Pediatrics, Division of Cardiology, Medical University of South Carolina, MSC 915, Room 601, Children's Hospital, 165 Ashley Avenue, Charleston, South Carolina, United States 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. DB - Embase Medline DO - 10.1016/j.jacc.2017.03.582 IS - 22 KW - NCT00132782 brain natriuretic peptide adolescent age distribution article cardiovascular function cardiovascular mortality cardiovascular risk child echocardiography exercise exercise test female Fontan procedure functional status health status heart ejection fraction heart single ventricle heart transplantation heart ventricle function human longitudinal study major clinical study male outcome assessment oxygen consumption priority journal quality of life assessment risk assessment survival transplant free survival LA - English M3 - Article N1 - L616527394 2017-06-05 2019-10-31 PY - 2017 SN - 1558-3597 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 TI - Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L616527394 http://dx.doi.org/10.1016/j.jacc.2017.03.582 VL - 69 ID - 750 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. © 2014 Wiley Periodicals, Inc. AD - Department of Pediatrics, Medical University of South Carolina, Charleston, SC, United States New England Research Institutes, Watertown, United States Boston Children's Hospital, Boston, MA, United States Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX, United States Department of Pediatrics, University of Michigan, Ann Arbor, MI, United States Children's Hospital of Philadelphia, Philadelphia, PA, United States Department of Pediatrics, University of Utah, Salt Lake City, UT, United States National Heart, Lung, and Blood Institute, Bethesda, MD, United States Department of Pediatrics, Duke University Medical Center, Durham, NC, United States Department of Pediatrics, Columbia University Medical Center, New York, NY, United States Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States The Hospital for Sick Children, University of Toronto, Toronto, Canada 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. AU - Pediatric Heart Network, Investigators DB - Scopus DO - 10.1111/chd.12193 IS - 1 KW - Congenital Fontan procedure Functional health status Heart defects Pediatrics M3 - Article N1 - Cited By :36 Export Date: 15 June 2020 PY - 2015 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84922604949&doi=10.1111%2fchd.12193&partnerID=40&md5=8af2599e2c0dc0252291b0c1650024c6 VL - 10 ID - 1964 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, Ï ‡ 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 - B.H. Goldstein, Heart Institute, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH, United States AU - Averin, K. AU - Hirsch, R. AU - Seckeler, M. D. AU - Whiteside, W. AU - Beekman, R. H. AU - Goldstein, B. H. DB - Embase Medline DO - 10.1136/heartjnl-2015-309042 IS - 14 KW - dipeptidyl carboxypeptidase inhibitor diuretic agent adolescent adult article child clinical article cohort analysis controlled study diastolic dysfunction feasibility study female Fontan procedure heart catheterization heart failure heart failure with preserved ejection fraction heart function test heart hemodynamics cardiac index heart left ventricle heart ventricle enddiastolic pressure human lung capillary pressure male operation duration patient safety priority journal rapid volume expansion technique school child treatment outcome LA - English M3 - Article N1 - L608795360 2016-03-10 2016-08-02 PY - 2016 SN - 1468-201X 1355-6037 SP - 1109-1114 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L608795360 http://dx.doi.org/10.1136/heartjnl-2015-309042 VL - 102 ID - 802 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, Ï ‡ 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. © 2016 Published by the BMJ Publishing Group Limited. For permission to use. AD - Heart Institute, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229, United States Department of Pediatrics (Cardiology), University of Arizona, Tucson, AZ, United States AU - Averin, K. AU - Hirsch, R. AU - Seckeler, M. D. AU - Whiteside, W. AU - Beekman, R. H. AU - Goldstein, B. H. DB - Scopus DO - 10.1136/heartjnl-2015-309042 IS - 14 M3 - Article N1 - Cited By :20 Export Date: 15 June 2020 PY - 2016 SP - 1109-1114 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84959302299&doi=10.1136%2fheartjnl-2015-309042&partnerID=40&md5=c53cdcebbeba180fabbcfc2243bde775 VL - 102 ID - 1896 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. © Journal of Thoracic Disease. All rights reserved. AD - 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 - Scopus DO - 10.21037/jtd.2018.11.107 KW - Congenital cardiac surgery (CCS) Extracorporeal membrane oxygenation (ECMO) Neonatal Pediatric and neonatal patients Pediatric intensive care unit Resuscitation M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2019 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85065163798&doi=10.21037%2fjtd.2018.11.107&partnerID=40&md5=7ee2b8b333a9a129a578765140468315 VL - 11 ID - 1776 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. © 2010 Springer Science+Business Media, LLC. AD - K. Babaoglu, Department of Pediatric Cardiology, Faculty of Medicine, Kocaeli University, Kocaeli, Turkey AU - Babaoglu, K. AU - Binnetoglu, F. K. AU - Aydoǧan, A. AU - Altun, G. AU - Gürbüz, Y. AU - Inan, N. AU - Çorapçioǧlu, F. DB - Embase Medline DO - 10.1007/s00246-010-9685-x IS - 6 KW - alanine aminotransferase albumin alkaline phosphatase alpha fetoprotein aspartate aminotransferase bilirubin bilirubin glucuronide hemoglobin protein abdominal distension adenomatosis alanine aminotransferase blood level albumin blood level alkaline phosphatase blood level angiocardiography article aspartate aminotransferase blood level bilirubin blood level case report child computer assisted tomography cyanosis Doppler ultrasonography female Fontan procedure heart atrium septum defect heart catheterization hematocrit hemoglobin blood level hepatocellular adenomatosis hepatomegaly histopathology human human tissue hypoplasia inferior cava vein leukocyte count liver biopsy liver cirrhosis liver fibrosis lung angiography lung artery pressure nuclear magnetic resonance imaging oxygen saturation partial thromboplastin time postoperative complication protein blood level prothrombin time pulmonary arteriovenous fistula pulmonary valve atresia school child scoring system staining superior cava vein platelet count transcutaneous oxygen monitoring tricuspid valve hypoplasia umbilicus venous pressure LA - English M3 - Article N1 - L50824386 2010-03-12 2010-08-26 PY - 2010 SN - 0172-0643 SP - 861-864 ST - Hepatic adenomatosis in a 7-year-old child treated earlier with a fontan procedure T2 - Pediatric Cardiology TI - Hepatic adenomatosis in a 7-year-old child treated earlier with a fontan procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50824386 http://dx.doi.org/10.1007/s00246-010-9685-x VL - 31 ID - 1160 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. © 2010 Springer Science+Business Media, LLC. AD - Department of Pediatric Cardiology, Faculty of Medicine, Kocaeli University, Kocaeli, Turkey Department of Pediatric Gastroenterology, Faculty of Medicine, Kocaeli University, Kocaeli, Turkey Department of Pathology, Faculty of Medicine, Kocaeli University, Kocaeli, Turkey Department of Radiology, Faculty of Medicine, Kocaeli University, Kocaeli, Turkey Department of Pediatric Oncology, Faculty of Medicine, Kocaeli University, Kocaeli, Turkey AU - Babaoglu, K. AU - Binnetoglu, F. K. AU - Aydoǧan, A. AU - Altun, G. AU - Gürbüz, Y. AU - Inan, N. AU - Çorapçioǧlu, F. DB - Scopus DO - 10.1007/s00246-010-9685-x IS - 6 KW - Cardiac cirrhosis Fontan procedure Hepatic adenomatosis M3 - Article N1 - Cited By :15 Export Date: 15 June 2020 PY - 2010 SP - 861-864 ST - Hepatic adenomatosis in a 7-year-old child treated earlier with a fontan procedure T2 - Pediatric Cardiology TI - Hepatic adenomatosis in a 7-year-old child treated earlier with a fontan procedure UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-77955553444&doi=10.1007%2fs00246-010-9685-x&partnerID=40&md5=821e8c8e56b043d38fc26c384758d290 VL - 31 ID - 2148 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 - 104497373. Language: English. Entry Date: 20120831. Revision Date: 20150711. Publication Type: Journal Article. Supplement Title: Dec2011 Supplement 2. Journal Subset: Biomedical AU - Backer, Carl L. DB - ccm DP - EBSCOhost IS - S2 KW - Cardiopulmonary Bypass -- Methods Heart Defects, Congenital -- Surgery Illinois Child N1 - Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Special Interest: Pediatric Care. NLM UID: 9200019. PMID: NLM22152545. PY - 2011 SN - 1047-9511 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 - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104497373&site=ehost-live&scope=site VL - 21 ID - 1568 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 - Division of Cardiovascular-Thoracic Surgery, Children's Memorial Hospital, Northwestern University Feinberg School of Medicine, 2300 Children's Plaza, Chicago, IL 60614, United States Division of Cardiology, Children's Memorial Hospital, Northwestern University Feinberg School of Medicine, Chicago, IL, United States AU - Backer, C. L. AU - Deal, B. J. AU - Mavroudis, C. AU - Franklin, W. H. AU - Stewart, R. D. DB - Scopus DO - 10.1017/S1047951105002386 IS - SUPPL. 1 KW - Arrhythmias Atrioventricular valvar atresia Cardiac failure Double inlet ventricle Functionally univentricular heart M3 - Article N1 - Cited By :45 Export Date: 15 June 2020 PY - 2006 SP - 85-91 ST - Conversion of the failed Fontan circulation T2 - Cardiology in the Young TI - Conversion of the failed Fontan circulation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-33646196300&doi=10.1017%2fS1047951105002386&partnerID=40&md5=a04c8361bdd56a379a06ebee867b81ab VL - 16 ID - 2233 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. AU - Backer, C. L. AU - Mavroudis, C. DB - Medline DO - 10.14797/mdcj-15-2-105 IS - 2 KW - adolescent adult cardiomegaly child clinical decision making complication congenital heart malformation female Fontan procedure heart arrhythmia human male middle aged mortality pathophysiology patient selection preschool child reoperation risk assessment risk factor time factor young adult LA - English M3 - Article N1 - L629028463 2019-08-29 2019-09-06 PY - 2019 SN - 1947-6108 SP - 105-110 ST - 149 Fontan Conversions T2 - Methodist DeBakey cardiovascular journal TI - 149 Fontan Conversions UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L629028463 http://dx.doi.org/10.14797/mdcj-15-2-105 VL - 15 ID - 620 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, IL, United States FLORIDA HOSPITAL FOR CHILDREN, ORLANDO, FL, United States JOHNS HOPKINS UNIVERSITY SCHOOL OF MEDICINE, BALTIMORE, MD, United States AU - Backer, C. L. AU - Mavroudis, C. DB - Scopus DO - 10.14797/mdcj-15-2-105 IS - 2 KW - arrhythmia atrial fibrillation atriopulmonary Fontan congenital heart surgery cryoablation epicardial pacemaker Fontan conversion protein-losing enteropathy tricuspid atresia M3 - Article N1 - Export Date: 15 June 2020 PY - 2019 SP - 105-110 ST - 149 Fontan Conversions T2 - Methodist DeBakey cardiovascular journal TI - 149 Fontan Conversions UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85071171620&doi=10.14797%2fmdcj-15-2-105&partnerID=40&md5=15faa1e9b77343b060362116f5197eb4 VL - 15 ID - 1754 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 - Box 22, Division of Cardiovascular-Thoracic Surgery, Children's Memorial Hospital, 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 - Scopus IS - 5 M3 - Article N1 - Cited By :40 Export Date: 15 June 2020 PY - 1991 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0025863701&partnerID=40&md5=a7ecb4295c7aa6ca0311c95d64b0490c VL - 101 ID - 2317 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 - 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 - Scopus IS - 5 M3 - Article N1 - Cited By :99 Export Date: 15 June 2020 PY - 1993 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0027160235&partnerID=40&md5=3cf97ebb077f1133d922dab7ab40f5fa VL - 105 ID - 2312 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, United Kingdom AU - Baker, E. J. AU - Jones, O. D. H. AU - Joseph, M. C. AU - Maisey, M. N. AU - Tynan, M. J. DB - Scopus IS - 5 M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 1984 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0021746802&partnerID=40&md5=3de60d1e1e47b5c0fea25471d7b52089 VL - 52 ID - 2330 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. © 2014 The Authors. AD - Division of Epidemiology and Public Health, University of Nottingham, Nottingham City Hospital, Clinical Sciences Building, Nottingham, NG5 1PB, United Kingdom Nottingham Digestive Diseases Centre, National Institute for Health Research Biomedical Research Unit, Nottingham University Hospitals National Health Service Trust, Nottingham, United Kingdom Department of Non-Communicable Disease Epidemiology, London School of Hygiene and Tropical Medicine, London, 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 - Scopus DO - 10.1111/1471-0528.12682 IS - 12 KW - Antidepressants Congenital anomaly Depression SSRIs TCAs M3 - Article N1 - Cited By :54 Export Date: 15 June 2020 PY - 2014 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84914708300&doi=10.1111%2f1471-0528.12682&partnerID=40&md5=a9abbfd4dc8a4a5c442f552da4da6464 VL - 121 ID - 1981 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. © 2011 Springer Science+Business Media, LLC. AD - Ahmanson/UCLA Adult Congenital Heart Disease Center, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, United States AU - Bauer, B. S. AU - Aboulhosn, J. A. AU - Williams, R. J. AU - Child, J. S. DB - Scopus DO - 10.1007/s00246-011-0008-7 IS - 7 KW - Adult congenital heart disease Cardiopulmonary stress testing Single ventricle physiology M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2011 SP - 891-895 ST - Patients with single-ventricle physiology: Prognostic implications of stress testing T2 - Pediatric Cardiology TI - Patients with single-ventricle physiology: Prognostic implications of stress testing UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84859631470&doi=10.1007%2fs00246-011-0008-7&partnerID=40&md5=13f58b49652276670392e175d78b5f33 VL - 32 ID - 2116 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 - J. Bauer, Department of Pediatric Cardiology, University Giessen, Feulgenstrasse 12, 35385 Giessen, Germany 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. DB - Embase Medline DO - 10.1034/j.1399-3046.2001.00023.x IS - 6 KW - azathioprine cyclosporine OKT 3 prednisolone thymocyte antibody article cardiomyopathy cause of death child congenital heart disease controlled study coronary artery disease endocardial fibroelastosis follow up graft rejection heart right ventricle failure heart transplantation human hypoplastic left heart syndrome immunosuppressive treatment infant kidney function long term care lymphoproliferative disease major clinical study morbidity neurologic disease quality of life survival treatment outcome LA - English M3 - Article N1 - L33095212 2001-12-11 PY - 2001 SN - 1397-3142 SP - 457-462 ST - Heart transplantation in children and infants: Short-term outcome and long-term follow-up T2 - Pediatric Transplantation TI - Heart transplantation in children and infants: Short-term outcome and long-term follow-up UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L33095212 http://dx.doi.org/10.1034/j.1399-3046.2001.00023.x VL - 5 ID - 1317 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 Department of Cardiovascular Surgery, University Giessen, Giessen, Germany Department of Anesthesiology, University Giessen, Giessen, Germany Department of Pathology, University Giessen, Giessen, Germany Department of Pediatric Cardiology, University Giessen, Feulgenstrasse 12, 35385 Giessen, Germany 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. DB - Scopus DO - 10.1034/j.1399-3046.2001.00023.x IS - 6 KW - Children Follow-up Heart transplantation Infants Morbidity M3 - Article N1 - Cited By :35 Export Date: 15 June 2020 PY - 2001 SP - 457-462 ST - Heart transplantation in children and infants: Short-term outcome and long-term follow-up T2 - Pediatric Transplantation TI - Heart transplantation in children and infants: Short-term outcome and long-term follow-up UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-17944378058&doi=10.1034%2fj.1399-3046.2001.00023.x&partnerID=40&md5=2c64c283715c01ddf9a93bf76e003478 VL - 5 ID - 2269 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., United States Department of Psychology, Loma Linda Univ. Graduate School, 11130 Anderson St, Ste 102, Loma L., United States AU - Baum, M. AU - Catherin Freier, M. AU - Freeman, K. AU - Babikian, T. AU - Ashwal, S. AU - Chinnock, R. AU - Bailey, L. DB - Scopus DO - 10.1016/j.jpeds.2004.05.047 IS - 3 M3 - Article N1 - Cited By :23 Export Date: 15 June 2020 PY - 2004 SP - 365-372 ST - Neuropsychological outcome of infant heart transplant recipients T2 - Journal of Pediatrics TI - Neuropsychological outcome of infant heart transplant recipients UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-4444235498&doi=10.1016%2fj.jpeds.2004.05.047&partnerID=40&md5=fd5399e85fc8f7d3ee833d0f90e662ae VL - 145 ID - 2251 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. © 2017 Wiley Periodicals, Inc. AD - Cardiothoracic Surgery, The Children's Hospital of Philadelphia, Philadelphia, PA, United States 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 - Scopus DO - 10.1111/chd.12461 IS - 4 KW - APOE genotype autism serious congenital heart defects M3 - Article N1 - Cited By :10 Export Date: 15 June 2020 PY - 2017 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85015307095&doi=10.1111%2fchd.12461&partnerID=40&md5=6433f82e9d3450522a8ad6ea3fd7c5c8 VL - 12 ID - 1856 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.R. Cassidy, Department of Psychiatry, Boston Children’s Hospital, Center for Neuropsychology, Boston, MA, United States 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. DB - Embase Medline DO - 10.1080/09297049.2017.1283396 IS - 4 KW - academic achievement accuracy adolescent adult article child concurrent validity congenital heart disease controlled study critical congenital heart disease depth perception Developmental Scoring System Developmental Scoring System for the Rey Osterrieth Complex Figure Fallot tetralogy female great vessels transposition heart single ventricle human integration major clinical study male multiple regression organization post hoc analysis predictive value Rey Osterrieth complex figure test scoring system LA - English M3 - Article N1 - L614490503 2017-02-23 2018-03-22 PY - 2018 SN - 1744-4136 0929-7049 SP - 451-468 ST - Visuospatial processing in adolescents with critical congenital heart disease: Organization, integration, and implications for academic achievement T2 - Child Neuropsychology TI - Visuospatial processing in adolescents with critical congenital heart disease: Organization, integration, and implications for academic achievement UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L614490503 http://dx.doi.org/10.1080/09297049.2017.1283396 VL - 24 ID - 685 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. (PsycINFO Database Record (c) 2018 APA, all rights reserved) AD - Cassidy, Adam R., Department of Psychiatry, Boston Children’s Hospital, Center for Neuropsychology, Boston, MA, US, 02115 AN - 2018-10595-002 AU - Bean Jaworski, Jessica L. AU - White, Matthew T. AU - DeMaso, David R. AU - Newburger, Jane W. AU - Bellinger, David C. AU - Cassidy, Adam R. DB - psyh DO - 10.1080/09297049.2017.1283396 DP - EBSCOhost IS - 4 KW - Rey–Osterrieth Complex Figure Visuospatial Cardiac Congenital heart defect Adolescence Congenital Disorders Heart Disorders Symptoms Visuospatial Ability N1 - Department of Psychiatry, Boston Children’s Hospital, Boston, MA, US. Release Date: 20180628. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Congenital Disorders; Heart Disorders; Symptoms; Visuospatial Ability. Classification: Cardiovascular Disorders (3295). Population: Human (10); Male (30); Female (40). Age Group: Adolescence (13-17 yrs) (200). Tests & Measures: Wechsler Individual Achievement Test--Second Edition DOI: 10.1037/t15173-000; Rey-Osterrieth Complex Figure Test. Methodology: Empirical Study; Quantitative Study. Page Count: 18. Issue Publication Date: May, 2018. Publication History: Accepted Date: Jan 10, 2017; First Submitted Date: Nov 16, 2016. Copyright Statement: Informa UK Limited, trading as Taylor & Francis Group. 2017. Sponsor: National Heart, Lung, and Blood Institute, US. Grant: HL77681; HL74734; HL096825. Recipients: No recipient indicated Sponsor: Farb Family Fund. Recipients: No recipient indicated Sponsor: National Center for Research Resources, US. Grant: RR02172. Recipients: No recipient indicated PY - 2018 SN - 0929-7049 1744-4136 SP - 451-468 ST - Visuospatial processing in adolescents with critical congenital heart disease: Organization, integration, and implications for academic achievement T2 - Child Neuropsychology TI - Visuospatial processing in adolescents with critical congenital heart disease: Organization, integration, and implications for academic achievement UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2018-10595-002&site=ehost-live&scope=site adam.cassidy@childrens.harvard.edu VL - 24 ID - 1664 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. © 2017 Informa UK Limited, trading as Taylor & Francis Group. AD - Department of Psychiatry, Boston Children’s Hospital, Boston, MA, United States 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. DB - Scopus DO - 10.1080/09297049.2017.1283396 IS - 4 KW - Adolescence Cardiac Congenital heart defect Rey–Osterrieth Complex Figure Visuospatial M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 2018 SP - 451-468 ST - Visuospatial processing in adolescents with critical congenital heart disease: Organization, integration, and implications for academic achievement T2 - Child Neuropsychology TI - Visuospatial processing in adolescents with critical congenital heart disease: Organization, integration, and implications for academic achievement UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85013074748&doi=10.1080%2f09297049.2017.1283396&partnerID=40&md5=06d026d5ebb789990d809106a8976a7a VL - 24 ID - 1805 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 highrisk group. AD - J.W. Newburger, Department of Cardiology, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, United States 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. DB - Embase Medline DO - 10.1161/JAHA.115.002302 IS - 12 KW - academic achievement adolescent age distribution article attention cerebrovascular accident cohort analysis controlled study cross-sectional study executive function family history female Fontan procedure genetic disorder heart arrest heart single ventricle human intelligence quotient major clinical study male Mathematics Composite score memory test morbidity neuroimaging neuropsychological test nuclear magnetic resonance imaging operation duration population research postoperative complication priority journal Reading Composite score scoring system seizure social cognition LA - English M3 - Article N1 - L611124470 2016-07-12 2016-07-20 PY - 2015 SN - 2047-9980 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://www.embase.com/search/results?subaction=viewrecord&from=export&id=L611124470 http://dx.doi.org/10.1161/JAHA.115.002302 VL - 4 ID - 843 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 - A. Benatar, Children's Heart Centre, Wilhelmina Children's Hospital, University of Utrecht, The Netherlands. AU - Benatar, A. AU - Tanke, R. AU - Roef, M. AU - Meyboom, E. J. AU - Van de Wal, H. J. DB - Medline IS - 6 KW - adolescent anastomosis article child congenital heart malformation congenital malformation diaphragm paralysis female follow up Fontan procedure heart arrhythmia heart atrium human infant inferior cava vein length of stay male methodology pericardial effusion plastic surgery pleura effusion postoperative complication preschool child pulmonary artery statistical analysis survival rate tricuspid valve LA - English M3 - Article N1 - L125127904 1995-11-07 PY - 1995 SN - 1010-7940 SP - 320-324 ST - Mid-term results of the modified Senning operation for cavopulmonary connection with autologous tissue T2 - European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery TI - Mid-term results of the modified Senning operation for cavopulmonary connection with autologous tissue UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L125127904 VL - 9 ID - 1365 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 - Oral and Maxillofacial Surgery, Leeds General Infirmary, United Kingdom AU - Benson, R. E. AU - Fabbroni, G. AU - Russell, J. L. DB - Scopus DO - 10.1016/j.bjoms.2007.12.015 IS - 1 KW - Epipalatus Palate Teratoma M3 - Article N1 - Cited By :11 Export Date: 15 June 2020 PY - 2009 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-58149348705&doi=10.1016%2fj.bjoms.2007.12.015&partnerID=40&md5=d254fd086d039d3fb3605be43d43cfa1 VL - 47 ID - 2186 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 - J.H. Hansen, Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Arnold-Heller-Str. 3, Haus 9, Kiel, Germany 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. DB - Embase Medline DO - 10.1093/ejcts/ezu299 IS - 5 KW - article bleeding brain hemorrhage brain perfusion cardiopulmonary bypass child cognitive defect controlled study deep hypothermic circulatory arrest executive function female focal epilepsy Fontan procedure forward heart failure heart arrhythmia heart tamponade human hypoplastic left heart syndrome intelligence quotient long term memory male mental health multiple organ failure necrotizing enterocolitis Norwood procedure palliative therapy peroperative complication priority journal psychologic test resuscitation school child seizure sepsis short term memory shunt occlusion thrombosis LA - English M3 - Article N1 - L604373215 2015-05-20 2015-05-29 PY - 2015 SN - 1873-734X 1010-7940 SP - 803-811 ST - Neuropsychological performance of school-aged children after staged surgical palliation of hypoplastic left heart syndrome T2 - European Journal of Cardio-thoracic Surgery TI - Neuropsychological performance of school-aged children after staged surgical palliation of hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L604373215 http://dx.doi.org/10.1093/ejcts/ezu299 VL - 47 ID - 844 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. © The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. AD - Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Kiel, Germany 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 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. C7 - ezu299 DB - Scopus DO - 10.1093/ejcts/ezu299 IS - 5 KW - Cardiopulmonary bypass Hypoplastic left heart syndrome Hypothermic circulatory arrest Norwood operation M3 - Article N1 - Cited By :11 Export Date: 15 June 2020 PY - 2015 SP - 803-811 ST - Neuropsychological performance of school-aged children after staged surgical palliation of hypoplastic left heart syndrome T2 - European Journal of Cardio-thoracic Surgery TI - Neuropsychological performance of school-aged children after staged surgical palliation of hypoplastic left heart syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84929173297&doi=10.1093%2fejcts%2fezu299&partnerID=40&md5=45b26c0bb7a52031d5c152d43112a9bd VL - 47 ID - 1934 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 - 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 - Scopus DO - 10.1542/peds.2012-0043 IS - 2 KW - Catheterization Congenital heart disease Outcomes RVU M3 - Article N1 - Cited By :14 Export Date: 15 June 2020 PY - 2013 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84873436271&doi=10.1542%2fpeds.2012-0043&partnerID=40&md5=a79ded9e66ca04dcf40034f6d07614db VL - 131 ID - 2073 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 - M. Berghammer, Sahlgrenska Academy at University of Gothenburg, Institute of Health and Care Sciences, Box 457, Gothenburg, Sweden AU - Berghammer, M. AU - Karlsson, J. AU - Ekman, I. AU - Eriksson, P. AU - Dellborg, M. DB - Embase Medline DO - 10.1016/j.ijcard.2011.10.002 IS - 3 KW - adolescent adult aged anxiety aortic arch anomaly aortic valve disease article asymptomatic disease congenital heart disease controlled study depression disease registry dyspnea edema faintness Fallot tetralogy fatigue female Health Perceptions Questionnaire health status heart palpitation human major clinical study male mitral valve disease priority journal pulmonary valve disease quality of life questionnaire self care self report symptom thorax pain tricuspid valve disease very elderly visual analog scale LA - English M3 - Article N1 - L603876448 2015-05-04 2015-05-06 PY - 2013 SN - 1874-1754 0167-5273 SP - 537-543 ST - Self-reported health status (EQ-5D) in adults with congenital heart disease T2 - International Journal of Cardiology TI - Self-reported health status (EQ-5D) in adults with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L603876448 http://dx.doi.org/10.1016/j.ijcard.2011.10.002 VL - 165 ID - 998 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. © 2011 Elsevier Ireland Ltd. AD - M. Berghammer, Sahlgrenska Academy at University of Gothenburg, Institute of Health and Care Sciences, Box 457, SE-40530 Gothenburg, Sweden AU - Berghammer, M. AU - Karlsson, J. AU - Ekman, I. AU - Eriksson, P. AU - Dellborg, M. DB - Embase Medline DO - 10.1016/j.ijcard.2011.10.002 IS - 3 KW - adolescent adult article congenital heart disease controlled study disease association disease classification dyspnea edema educational status Euro QoL 5 Dimension questionnaire faintness fatigue female health status heart palpitation human life satisfaction major clinical study male mental function perception priority journal quality of life questionnaire self care self report symptomatology thorax pain validation process visual analog scale LA - English M3 - Article N1 - L51694810 2011-11-04 2013-05-22 PY - 2013 SN - 0167-5273 1874-1754 SP - 537-543 ST - Self-reported health status (EQ-5D) in adults with congenital heart disease T2 - International Journal of Cardiology TI - Self-reported health status (EQ-5D) in adults with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51694810 http://dx.doi.org/10.1016/j.ijcard.2011.10.002 VL - 165 ID - 1000 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. © 2011 Elsevier Ireland Ltd. AD - Sahlgrenska Academy at University of Gothenburg, Institute of Health and Care Sciences, Box 457, SE-40530 Gothenburg, Sweden Centre for Health Care Sciences, Örebro University Hospital, SE-701 82 Örebro, Sweden University of Gothenburg, Centre for Person-Centred Care, Sweden Institute of Medicine, Sahlgrenska Academy at University of Gothenburg, Sahlgrenska University Hospital/ Östra, SE-41685 Gothenburg, Sweden AU - Berghammer, M. AU - Karlsson, J. AU - Ekman, I. AU - Eriksson, P. AU - Dellborg, M. DB - Scopus DO - 10.1016/j.ijcard.2011.10.002 IS - 3 KW - Adult congenital heart disease (ACHD) EQ-5D Health state Self-reported health status M3 - Article N1 - Cited By :22 Export Date: 15 June 2020 PY - 2013 SP - 537-543 ST - Self-reported health status (EQ-5D) in adults with congenital heart disease T2 - International Journal of Cardiology TI - Self-reported health status (EQ-5D) in adults with congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84877071412&doi=10.1016%2fj.ijcard.2011.10.002&partnerID=40&md5=5f9e81a4a3fc61ef9f29b4ebb92bb12a VL - 165 ID - 2075 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 - M.C. Berghammer, Research Unit/GPCC, Department of Medicine, Sahlgrenska University Hospital/Östra, Gothenburg, Sweden AU - Berghammer, M. C. AU - Brink, E. AU - Rydberg, A. M. AU - Dellborg, M. AU - Ekman, I. DB - Embase Medline DO - 10.1111/chd.12244 IS - 5 KW - adolescent adult ambivalence article attitude to life clinical article cognition confusion (uncertainty) controlled study disease control fear female Fontan procedure happiness heart single ventricle human male patient satisfaction patient worry personal experience physical mobility priority journal self concept social acceptance wisdom young adult LA - English M3 - Article N1 - L602340918 2015-02-24 2015-10-20 PY - 2015 SN - 1747-0803 1747-079X SP - 403-412 ST - Committed to Life: Adolescents' and Young Adults' Experiences of Living with Fontan Circulation T2 - Congenital Heart Disease TI - Committed to Life: Adolescents' and Young Adults' Experiences of Living with Fontan Circulation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L602340918 http://dx.doi.org/10.1111/chd.12244 VL - 10 ID - 850 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. © 2015 Wiley Periodicals, Inc. AD - Institute of Medicine, Sahlgrenska Academy, Gothenburg, Sweden Centre for Person-Centred Care, University of Gothenburg, Gothenburg, Sweden Institute of Health and Care Sciences, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden Institute of Caring, Health and Culture, University West, Trollhättan, Sweden Department of Clinical Sciences, Pediatrics, University of Umeå, Umeå, Sweden Adult Congenital Heart Unit, Sahlgrenska University Hospital/Östra, Gothenburg, Sweden AU - Berghammer, M. C. AU - Brink, E. AU - Rydberg, A. M. AU - Dellborg, M. AU - Ekman, I. DB - Scopus DO - 10.1111/chd.12244 IS - 5 KW - Adolescents and Young Adults Fontan Circulation In-Depth Interviews Lived Experience Phenomenological Hermeneutics Univentricular Heart M3 - Article N1 - Cited By :13 Export Date: 15 June 2020 PY - 2015 SP - 403-412 ST - Committed to Life: Adolescents' and Young Adults' Experiences of Living with Fontan Circulation T2 - Congenital Heart Disease TI - Committed to Life: Adolescents' and Young Adults' Experiences of Living with Fontan Circulation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84943662715&doi=10.1111%2fchd.12244&partnerID=40&md5=81711cfda3d9ec45b7417dfab524abbc VL - 10 ID - 1939 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 - 138825666. Language: English. Entry Date: 20050722. Revision Date: 20190927. Publication Type: journal article AU - Bhat, Aarti Hejmadi AU - Sahn, David J. DB - ccm DP - EBSCOhost IS - 5 KW - Heart Defects, Congenital -- Surgery Autografts Heart Septal Defects, Ventricular -- Surgery Transposition of Great Arteries -- Surgery Prostheses and Implants Adult Cardiopulmonary Bypass Reoperation -- Methods Pulmonary Artery -- Surgery Tetralogy of Fallot -- Surgery Aorta -- Surgery Death, Sudden, Cardiac -- Prevention and Control Reoperation -- Equipment and Supplies Psychosocial Adjustment to Illness Scale Questionnaires Scales N1 - review. Journal Subset: Biomedical; Peer Reviewed; USA. Instrumentation: Psychosocial Adjustment to Illness Scale (PAIS); General Health Questionnaire (GHQ); Work Environment Scale (WES) (Moos et al). NLM UID: 9000850. PMID: NLM15367842. PY - 2004 SN - 1040-8703 SP - 500-507 ST - Congenital heart disease never goes away, even when it has been 'treated': the adult with congenital heart disease T2 - Current Opinion in Pediatrics TI - Congenital heart disease never goes away, even when it has been 'treated': the adult with congenital heart disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=138825666&site=ehost-live&scope=site VL - 16 ID - 1648 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°. 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. © 2009, International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc. AD - A. L. Throckmorton, Department of Mechanical Engineering, Virginia Commonwealth University, 401 West Main Street, P.O. Box 843015, Richmond, VA 23284, United States AU - Bhavsar, S. S. AU - Kapadia, J. Y. AU - Chopski, S. G. AU - Throckmorton, A. L. DB - Embase Medline DO - 10.1111/j.1525-1594.2009.00940.x IS - 11 KW - article blood pressure blood pump computational fluid dynamics Fontan procedure heart assist device inferior cava vein lung blood flow lung circulation lung hemodynamics mathematical model pressure measurement priority journal quasi experimental study surgical technique treatment failure vascular disease LA - English M3 - Article N1 - L355704352 2009-12-29 PY - 2009 SN - 0160-564X 1525-1594 SP - 977-987 ST - Intravascular mechanical cavopulmonary assistance for patients with failing fontan physiology T2 - Artificial Organs TI - Intravascular mechanical cavopulmonary assistance for patients with failing fontan physiology UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L355704352 http://dx.doi.org/10.1111/j.1525-1594.2009.00940.x VL - 33 ID - 1169 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°. 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. © 2009, International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc. AD - Department of Mechanical Engineering, Virginia Commonwealth University, 401 West Main Street, P.O. Box 843015, Richmond, VA 23284, United States AU - Bhavsar, S. S. AU - Kapadia, J. Y. AU - Chopski, S. G. AU - Throckmorton, A. L. DB - Scopus DO - 10.1111/j.1525-1594.2009.00940.x IS - 11 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(s) M3 - Article N1 - Cited By :30 Export Date: 15 June 2020 PY - 2009 SP - 977-987 ST - Intravascular mechanical cavopulmonary assistance for patients with failing fontan physiology T2 - Artificial Organs TI - Intravascular mechanical cavopulmonary assistance for patients with failing fontan physiology UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-70450267398&doi=10.1111%2fj.1525-1594.2009.00940.x&partnerID=40&md5=d6988808c355b69bc0f7dc9d922edf06 VL - 33 ID - 2168 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 postoperative complex lung injury. AD - D.G. Biasucci, Pediatric Intensive Care Unit, Emergency Department, Catholic University of the Sacred Heart, L.go A. Gemelli 8, Roma, Italy AU - Biasucci, D. G. AU - Ricci, Z. AU - Conti, G. AU - Cogo, P. DB - Embase Medline DO - 10.1002/ppul.23059 IS - 12 KW - adult respiratory distress syndrome aortopulmonary shunt article artificial ventilation case report child disease course echography extracorporeal oxygenation Glenn shunt human hypoplastic left heart syndrome lung compliance lung gas exchange lung infarction male preschool child treatment response LA - English M3 - Article N1 - L600548370 2014-12-01 2014-12-09 PY - 2014 SN - 1099-0496 8755-6863 SP - E147-E150 ST - Sonographic dynamic assessment of lung injury in a child with hypoplastic left heart syndrome undergoing extracorporeal membrane oxygenation T2 - Pediatric Pulmonology TI - Sonographic dynamic assessment of lung injury in a child with hypoplastic left heart syndrome undergoing extracorporeal membrane oxygenation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L600548370 http://dx.doi.org/10.1002/ppul.23059 VL - 49 ID - 904 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 postoperative complex lung injury. © 2014 Wiley Periodicals, Inc. AD - Pediatric Intensive Care Unit, Emergency Department, Catholic University of the Sacred Heart, L.go A. Gemelli 8, Roma, 00168, Italy Pediatric Cardiac Intensive Care Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital, Rome, Italy AU - Biasucci, D. G. AU - Ricci, Z. AU - Conti, G. AU - Cogo, P. DB - Scopus DO - 10.1002/ppul.23059 IS - 12 KW - Congenital heart diseases ECMO Heart surgery Lung ultrasound Mechanical ventilation Pediatric critical care Respiratory failure M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2014 SP - E147-E150 ST - Sonographic dynamic assessment of lung injury in a child with hypoplastic left heart syndrome undergoing extracorporeal membrane oxygenation T2 - Pediatric Pulmonology TI - Sonographic dynamic assessment of lung injury in a child with hypoplastic left heart syndrome undergoing extracorporeal membrane oxygenation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84911477546&doi=10.1002%2fppul.23059&partnerID=40&md5=ceafb1563f6abaac64870d574a45caa5 VL - 49 ID - 2012 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 - D.M. Biko, The Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA, United States 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. DB - Embase Medline DO - 10.1016/j.athoracsur.2019.03.019 IS - 2 KW - adolescent adult anaerobic threshold aortic arch aortic reconstruction arterial stiffness article cardiac imaging cardiovascular magnetic resonance child clinical article descending aorta diaphragm end diastolic volume end systolic volume exercise exercise test female Fontan procedure heart single ventricle heart stroke volume heart volume human male oxygen consumption priority journal pulse wave pulse wave distensibility LA - English M3 - Article N1 - L2002205318 2019-07-08 2019-11-27 PY - 2019 SN - 1552-6259 0003-4975 SP - 574-580 ST - Relationship of Aortic Stiffness to Exercise and Ventricular Volumes in Single Ventricles T2 - Annals of Thoracic Surgery TI - Relationship of Aortic Stiffness to Exercise and Ventricular Volumes in Single Ventricles UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2002205318 http://dx.doi.org/10.1016/j.athoracsur.2019.03.019 VL - 108 ID - 605 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. © 2019 The Society of Thoracic Surgeons AD - Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA, United States Division of Cardiothoracic Surgery, Children's Hospital of Philadelphia, Philadelphia, PA, United States Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA, United States Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States Cardiovascular Fluid Mechanics Laboratory, Wallace H. Coulter Department of Biomedical Engineering, Georgia Institute of Technology, Atlanta, Georgia 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. DB - Scopus DO - 10.1016/j.athoracsur.2019.03.019 IS - 2 M3 - Article N1 - Export Date: 15 June 2020 PY - 2019 SP - 574-580 ST - Relationship of Aortic Stiffness to Exercise and Ventricular Volumes in Single Ventricles T2 - Annals of Thoracic Surgery TI - Relationship of Aortic Stiffness to Exercise and Ventricular Volumes in Single Ventricles UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85068219720&doi=10.1016%2fj.athoracsur.2019.03.019&partnerID=40&md5=415fcdf89cebcb1bf4f2e99f65e701ca VL - 108 ID - 1738 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. AU - Black, A. K. AU - Sadanala, U. K. AU - Mascio, C. E. AU - Hornung, C. A. AU - Keller, B. B. DB - Medline DO - 10.1089/tmj.2013.0343 IS - 9 KW - oxygen blood body weight cardiology emergency health service feasibility study female congenital heart malformation home care human infant Internet male mortality newborn organization and management palliative therapy pediatrics physiologic monitoring pilot study procedures questionnaire sudden cardiac death telemedicine utilization LA - English M3 - Article N1 - L611702577 2016-08-23 PY - 2014 SN - 1556-3669 SP - 858-867 ST - Challenges in implementing a pediatric cardiovascular home telehealth project T2 - Telemedicine journal and e-health : the official journal of the American Telemedicine Association TI - Challenges in implementing a pediatric cardiovascular home telehealth project UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L611702577 http://dx.doi.org/10.1089/tmj.2013.0343 VL - 20 ID - 911 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. © 2014 Mary Ann Liebert, Inc. AD - Kosair Charities Pediatric Heart Research Program, Cardiovascular Innovation Institute, 302 E. Muhammad Ali Boulevard, Louisville, KY 40202, United States Department of Pediatrics, University of Louisville School of Medicine, Louisville, KY, United States Department of Surgery, University of Louisville School of Medicine, Louisville, KY, United States Department of Epidemiology and Population Health, School of Public Health and Information Sciences, University of Louisville, Louisville, KY, United States Department of Medicine, University of Louisville School of Medicine, Louisville, KY, United States Department of Surgery, Childrens Hospital of Philadelphia, Philadelphia, PA, United States AU - Black, A. K. AU - Sadanala, U. K. AU - Mascio, C. E. AU - Hornung, C. A. AU - Keller, B. B. DB - Scopus DO - 10.1089/tmj.2013.0343 IS - 9 KW - congenital heart disease home health monitoring hypoplastic left heart syndrome interstage sudden death pediatrics telemedicine M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2014 SP - 858-867 ST - Challenges in implementing a pediatric cardiovascular home telehealth project T2 - Telemedicine and e-Health TI - Challenges in implementing a pediatric cardiovascular home telehealth project UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84906898965&doi=10.1089%2ftmj.2013.0343&partnerID=40&md5=7018da8b290add730948da28d50c9064 VL - 20 ID - 1985 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. © 2018 International Society for the Heart and Lung Transplantation AD - Department of Cardiology, Children's Hospital Boston, Boston, Massachusetts, United States The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, United States Department of Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States Department of Cardiac Surgery, Cincinnati Children's Hospital, Cincinnati, Ohio, United States Kirklin Institute for Research in Surgical Outcomes, Birmingham, Alabama, United States Children's Mercy Hospital, Kansas City, Missouri, United States Dallas Children's Hospital, Dallas, Texas, United States St. Louis Children's Hospital, St. Louis, Missouri, United States Stanford University, Palo Alto, California, 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. AU - for the Pedimacs, Investigators DB - Scopus DO - 10.1016/j.healun.2017.06.017 IS - 1 KW - advanced heart failure bridge to transplant children mechanical support pediatrics Pedimacs ventricular assist device M3 - Article N1 - Cited By :55 Export Date: 15 June 2020 PY - 2018 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85030680407&doi=10.1016%2fj.healun.2017.06.017&partnerID=40&md5=fb045abeb1ae1f92747a014219e4056f VL - 37 ID - 1829 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. © 2011 Bojan et al.; licensee BioMed Central Ltd. AD - M. Bojan, Anesthesiolgy and Critical Care Department, Necker-Enfants Malades Hospital, Assistance Publique-Hôpitaux de Paris, 149 rue de Sèvres, 75015 Paris, France AU - Bojan, M. AU - Gioanni, S. AU - Mauriat, P. AU - Pouard, P. DB - Embase Medline DO - 10.1186/cc10521 IS - 5 KW - epinephrine milrinone acute kidney failure article artificial ventilation cohort analysis extracorporeal oxygenation heart surgery high frequency oscillatory ventilation human infant intensive care intensive care unit length of stay major clinical study newborn outcome assessment pediatric surgery priority journal pulmonary hypertension renal replacement therapy respiratory distress retrospective study LA - English M3 - Article N1 - L51694100 2011-11-04 2011-12-20 http://ccforum.com/content/15/5/R259 PY - 2011 SN - 1364-8535 1466-609X ST - High-frequency oscillatory ventilation and short-term outcome in neonates and infants undergoing cardiac surgery: A propensity score analysis T2 - Critical Care TI - High-frequency oscillatory ventilation and short-term outcome in neonates and infants undergoing cardiac surgery: A propensity score analysis UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51694100 http://dx.doi.org/10.1186/cc10521 VL - 15 ID - 1088 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. © 2011 Bojan et al.; licensee BioMed Central Ltd. AD - Anesthesiolgy and Critical Care Department, Necker-Enfants Malades Hospital, Assistance Publique-Hôpitaux de Paris, 149 rue de Sèvres, 75015 Paris, France Anesthesiolgy and Critical Care Department, Haut-Lévêque Hospital, 1 avenue de Magellan, 33604 Pessac, France AU - Bojan, M. AU - Gioanni, S. AU - Mauriat, P. AU - Pouard, P. C7 - R259 DB - Scopus DO - 10.1186/cc10521 IS - 5 M3 - Article N1 - Cited By :15 Export Date: 15 June 2020 PY - 2011 ST - High-frequency oscillatory ventilation and short-term outcome in neonates and infants undergoing cardiac surgery: A propensity score analysis T2 - Critical Care TI - High-frequency oscillatory ventilation and short-term outcome in neonates and infants undergoing cardiac surgery: A propensity score analysis UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-80055023035&doi=10.1186%2fcc10521&partnerID=40&md5=04a3604ca2d9d3d7f3715479a7292db5 VL - 15 ID - 2114 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. AD - E.H. Bolin, Section of Pediatric Cardiology, Arkansas Children’s Hospital, University of Arkansas for Medical Sciences, 1 Children’s Way, Slot 512-3, Little Rock, AR, United States AU - Bolin, E. H. AU - Maskatia, S. A. AU - Tate, A. L. AU - Petit, C. J. DB - Embase Medline DO - 10.14503/THIJ-14-4178 IS - 4 KW - age distribution anaerobic threshold article body mass body surface cavopulmonary connection child clinical feature computer assisted tomography echocardiography exercise exercise test female fenestration Fontan procedure heart rate human lung gas exchange lung vascular resistance major clinical study male metabolic parameters mitral valve regurgitation nuclear magnetic resonance imaging oxygen consumption oxygen saturation preschool child retrospective study systolic dysfunction treadmill exercise tricuspid valve regurgitation LA - English M3 - Article N1 - L605514612 2015-08-11 2015-08-13 PY - 2015 SN - 1526-6702 0730-2347 SP - 333-340 ST - Older age at completion of fontan procedure: Is associated with improved percentage of predicted maximum oxygen uptake T2 - Texas Heart Institute Journal TI - Older age at completion of fontan procedure: Is associated with improved percentage of predicted maximum oxygen uptake UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L605514612 http://dx.doi.org/10.14503/THIJ-14-4178 VL - 42 ID - 856 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. © 2015 by the Texas Heart ® Institute, Houston. AD - Department of Pediatrics, Lillie Abercrombie Section of Pediatric Cardiology, Texas Children’s Hospital, Baylor College of Medicine, Texas Children’s Hospital, Houston, TX 77030, United States Department of Exercise Science, Texas Children’s Hospital, Houston, TX 77030, United States Section of Pediatric Cardiology, Children’s Healthcare of Atlanta, Emory University School of Medicine, Atlanta, GA, United States AU - Bolin, E. H. AU - Maskatia, S. A. AU - Tate, A. L. AU - Petit, C. J. DB - Scopus DO - 10.14503/THIJ-14-4178 IS - 4 KW - Child, preschool Congenital/ surgery Exercise test Fontan procedure/adverse effects Heart defects Hemodynamics/ physiology Hypoplastic left heart syndrome Longitudinal studies Oxygen consumption/ physiology Physical exertion Postoperative complications Pulmonary circulation Retrospective studies Tricuspid atresia/surgery Vascular resistance M3 - Article N1 - Cited By :8 Export Date: 15 June 2020 PY - 2015 SP - 333-340 ST - Older age at completion of fontan procedure: Is associated with improved percentage of predicted maximum oxygen uptake T2 - Texas Heart Institute Journal TI - Older age at completion of fontan procedure: Is associated with improved percentage of predicted maximum oxygen uptake UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84938568408&doi=10.14503%2fTHIJ-14-4178&partnerID=40&md5=0cced769cd691f71f9b3388795e180b4 VL - 42 ID - 1941 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 - Departamento de Cirugía Cardiovascular, Hospital Infantil de México Federico Gómez, Centro Médico Nacional Siglo XXI, Instituto Mexicano del Seguro Social, México, DF, Mexico Unidad de Investigación en Epidemiología Clínica, Unidad Médica de Alta Especialidad Hospital de Pediatría, 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 - Scopus IS - 2 KW - Congenital heart disease Fontan surgery Prognosis M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2013 SP - 151-158 ST - Pronóstico de niños cardiópatas sometidos a cirugía de Fontan: Experiencia de treinta años en el 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84877118970&partnerID=40&md5=d2f602c8e87dd39cb11a7e457bce8999 VL - 70 ID - 2048 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 - L. Bordacova, Department of Pediatric Cardiology, Children's University Hospital Bratislava and Children's Cardiac Center of Slovak Republic, Bratislava, Slovakia. AU - Bordacova, L. AU - Docolomanska, D. AU - Masura, J. DB - Medline IS - 4-5 KW - article child cognitive defect developmental disorder follow up human hypoplastic left heart syndrome intelligence neuropsychological test newborn preschool child psychological aspect psychomotor performance LA - English M3 - Article N1 - L47569276 2007-10-19 PY - 2007 SN - 0006-9248 SP - 203-206 ST - Neuropsychological outcome in children with hypoplastic left heart syndrome T2 - Bratislavské lekárske listy TI - Neuropsychological outcome in children with hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L47569276 VL - 108 ID - 1225 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. © 2015, Springer Science+Business Media New York. AD - Pediatric Cardiology Unit, Department of Women’s and Children’s Health, University of Padua, Via Giustiniani 3, Padua, 35128, Italy Pediatric and Congenital Cardiac Surgery Unit, Department of Thoracic, Cardiac and Vascular Sciences, University of Padua, Padua, Italy Sport Medicine Department, Hospital of Padua, Padua, Italy Department of Cardiology, Bolzano Hospital, Bolzano, Italy Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Padua, Italy 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. DB - Scopus DO - 10.1007/s00246-015-1156-y IS - 6 KW - Follow-up study Fontan procedure Quality of life M3 - Article N1 - Cited By :10 Export Date: 15 June 2020 PY - 2015 SP - 1261-1269 ST - Clinical Profile and Quality of Life of Adult Patients After the Fontan Procedure T2 - Pediatric Cardiology TI - Clinical Profile and Quality of Life of Adult Patients After the Fontan Procedure UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84937635517&doi=10.1007%2fs00246-015-1156-y&partnerID=40&md5=01b49d5a9e28c719eff10ffc9e5b6b43 VL - 36 ID - 1940 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, expecially 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 - I. Borini, Department of Cardiovascular Surgery, Gaslini Pediatric Institute, Largo G. Gaslini 5, 16148 Genoa, Italy 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. DB - Embase Medline DO - 10.1016/S0967-2109(97)00077-X IS - 6 KW - anastomosis article child clinical article clinical trial congenital heart disease Fontan procedure hemodynamics human infant lung blood flow mortality priority journal pulmonary artery superior cava vein surgical technique LA - English M3 - Article N1 - L27502105 1997-12-06 PY - 1997 SN - 0967-2109 SP - 588-592 ST - Bidirectional cavo-pulmonary anastomosis with an additional flow source to the lungs: Clinical experience in 21 cases T2 - Cardiovascular Surgery TI - Bidirectional cavo-pulmonary anastomosis with an additional flow source to the lungs: Clinical experience in 21 cases UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L27502105 http://dx.doi.org/10.1016/S0967-2109(97)00077-X VL - 5 ID - 1354 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, expecially 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. © 1997, The International Society for Cardiovascular Surgery. All rights reserved. AD - Cardiovascular Surgery, Gaslini Pediatric Institute, Genoa, Italy Department of Cardiology, Gaslini Pediatric Institute, Genoa, Italy Department of Anesthesiology, Gaslini Pediatric Institute, Genoa, Italy 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. DB - Scopus DO - 10.1177/096721099700500608 IS - 6 KW - bidirectional cavopulmonary anastomosis congenital heart disease Fontan operation M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 1997 SP - 588-592 ST - Bidirectional cavo-pulmonary anastomosis with an additional flow source to the lungs: Clinical experience in 21 cases T2 - Vascular TI - Bidirectional cavo-pulmonary anastomosis with an additional flow source to the lungs: Clinical experience in 21 cases UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0031543518&doi=10.1177%2f096721099700500608&partnerID=40&md5=c1cf4ecaf0799f9b7cea9399b0acac4a VL - 5 ID - 2297 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/m2( p<0.001), 2.84±1.95 (ILT) and 8.41±3.77 (ECC) mW/m2 (p<0.001) during dobutamine and 5.21±3.50 (ILT) and 15.28±8.30 (ECC) mW/m2 (p=0.001) with twofold IVC flow. The correlation between cardiac index and Ploss was exponential (ILT: R2=0.811, p<0.001; ECC: R2=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 - W.A. Helbing, Department of Paediatrics, Erasmus Medical Centre, Sophia Children's Hospital, PO Box 2060, Rotterdam, Netherlands AU - Bossers, S. S. M. AU - Cibis, M. AU - Gijsen, F. J. AU - Schokking, M. AU - Strengers, J. L. M. AU - Verhaart, R. F. AU - Moelker, A. AU - Wentzel, J. J. AU - Helbing, W. A. DB - Embase Medline DO - 10.1136/heartjnl-2013-304969 IS - 9 KW - dobutamine article bicycle ergometry cardiovascular magnetic resonance cava vein cavopulmonary connection child clinical article computational fluid dynamics cross-sectional study exercise tolerance female flow rate heart right ventricle double outlet human hypoplastic left heart syndrome male Netherlands nuclear magnetic resonance scanner power loss priority journal pulmonary valve atresia rest school child stress tricuspid valve atresia General Electric Signa Philips Achieva Siemens Avanto LA - English M3 - Article N1 - L53061458 2014-03-24 2014-05-01 PY - 2014 SN - 1468-201X 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L53061458 http://dx.doi.org/10.1136/heartjnl-2013-304969 VL - 100 ID - 952 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 - 104062174. Language: English. Entry Date: 20140613. Revision Date: 20150710. Publication Type: Journal Article AU - Bossers, Sjoerd S. M. AU - Cibis, Merih AU - Gijsen, Frank J. AU - Schokking, Michiel AU - Strengers, Jan L. M. AU - Verhaart, René F. AU - Moelker, Adriaan AU - Wentzel, Jolanda J. AU - Helbing, Willem A. DB - ccm DO - 10.1136/heartjnl-2013-304969 DP - EBSCOhost IS - 9 KW - Bar Coding -- Methods Blood Flow Velocity -- Physiology Exercise Tolerance -- Physiology Cardiopulmonary Bypass -- Methods Heart Defects, Congenital -- Surgery Venae Cavae -- Physiopathology Child Cross Sectional Studies Exercise Test -- Methods Female Prospective Studies Cardiopulmonary Bypass -- Mortality Heart Defects, Congenital -- Mortality Heart Defects, Congenital -- Physiopathology Human Imaging, Three-Dimensional Magnetic Resonance Imaging Male Netherlands Survival -- Trends Treatment Outcomes N1 - research. Journal Subset: Biomedical; Blind Peer Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. NLM UID: 9602087. PMID: NLM24634021. 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104062174&site=ehost-live&scope=site VL - 100 ID - 1548 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/m2( p<0.001), 2.84±1.95 (ILT) and 8.41±3.77 (ECC) mW/m2 (p<0.001) during dobutamine and 5.21±3.50 (ILT) and 15.28±8.30 (ECC) mW/m2 (p=0.001) with twofold IVC flow. The correlation between cardiac index and Ploss was exponential (ILT: R2=0.811, p<0.001; ECC: R2=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, Erasmus Medical Centre, Sophia Children's Hospital, PO Box 2060, Rotterdam, Netherlands Department of Radiology, Erasmus Medical Centre, Rotterdam, Netherlands Department of Biomedical Engineering, Division of Cardiology, Erasmus Medical Centre, Rotterdam, Netherlands Department of Paediatrics, Division of Cardiology, St. Radboud University Medical Center, Nijmegen, Netherlands Department of Paediatrics, University Medical Centre Utrecht, Wilhelmina Children's Hospital, Utrecht, Netherlands AU - Bossers, S. S. M. AU - Cibis, M. AU - Gijsen, F. J. AU - Schokking, M. AU - Strengers, J. L. M. AU - Verhaart, R. F. AU - Moelker, A. AU - Wentzel, J. J. AU - Helbing, W. A. DB - Scopus DO - 10.1136/heartjnl-2013-304969 IS - 9 M3 - Article N1 - Cited By :17 Export Date: 15 June 2020 PY - 2014 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84898464834&doi=10.1136%2fheartjnl-2013-304969&partnerID=40&md5=e40eec911515c9bf3ff29baacaaa8d9c VL - 100 ID - 2015 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. © 2016, The Author(s). AD - Division of Cardiology, Department of Pediatrics, Erasmus Medical Centre, Sophia Children’s Hospital, Sp-2429, PO Box 2060, Rotterdam, 3000 CB, Netherlands Department of Radiology, Erasmus Medical Centre, Rotterdam, Netherlands Division of Cardiology, Department of Biomedical Engineering, Erasmus Medical Centre, Rotterdam, Netherlands Department of Pediatric Cardiology, Radboud University Medical Centre, Nijmegen, Netherlands Department of Pediatrics, Pediatric Cardiology Unit, Tel-Aviv Sourasky Medical Centre, Tel Aviv, Israel Department of Radiology, Amsterdam Academic Medical Center, Amsterdam, Netherlands Department of Radiology, Radboud University Medical Centre, Nijmegen, 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 - Scopus DO - 10.1007/s00246-015-1326-y IS - 4 KW - Cardiac magnetic resonance imaging Fontan Pulmonary arteries Shear stress TCPC M3 - Article N1 - Cited By :6 Export Date: 15 June 2020 PY - 2016 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84954320006&doi=10.1007%2fs00246-015-1326-y&partnerID=40&md5=7a674a11f45db55415a78d038bfa7394 VL - 37 ID - 1913 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. © 2015 Published by Elsevier Ireland Ltd. AD - Department of Pediatrics, Division of Pediatric Cardiology, Erasmus Medical Center, Sophia Children's Hospital, PO Box 2060, CB Rotterdam, 3000, Netherlands Department of Radiology, Erasmus Medical Center, Rotterdam, Netherlands Department of Pediatric Cardiology, Radboud University Medical Centre, Amalia Children's Hospital, Nijmegen, Netherlands Department of Pediatrics, Pediatric Cardiology Unit, Tel-Aviv Sourasky Medical Center, Tel Aviv, Israel Department of Pediatric Cardiology, Academic Medical Center, Amsterdam, Netherlands Department of Pediatric Cardiology, University Medical Center Utrecht, Wilhelmina Children's Hospital, Utrecht, Netherlands Department of Radiology, Leiden University Medical Center, Leiden, Netherlands Department of Radiology, Academic Medical Center, Amsterdam, Netherlands Department of Clinical Chemistry, Erasmus Medical Center, Rotterdam, Netherlands Department of Paediatric Cardiology, Leiden University Medical Center, Leiden, 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 - Scopus DO - 10.1016/j.ijcard.2015.05.181 KW - Cardiac function Congenital heart disease Echocardiography Fontan MRI Total cavopulmonary connection M3 - Article N1 - Cited By :15 Export Date: 15 June 2020 PY - 2015 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84937510796&doi=10.1016%2fj.ijcard.2015.05.181&partnerID=40&md5=85c34355d45cd8453584f6358f478440 VL - 196 ID - 1944 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 μg/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 μg/l (CK-MB 570 ± 280 μg/l). Conversely, survivors showed a lower mean Tn-I value (25.5 ± 28.9 μg/l; CK-MB 76 ± 86 μg/l). Overall, Tn-I peak value was higher than 35 μg/l in 19 patients (19/70; 27.2%); among these, 9 died (median Tn-I was 163 ± 186 μg/l), whereas in survivors it was 73.4 ± 37 μg/l (p = 0.37). The remaining four patients who died had a median Tn-I value of 21 μg/l. When Tn-I exceeded 35 μg/l (>100 μg/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. © 2006 Elsevier B.V. All rights reserved. AD - T. Bottio, Cardiovascular Institute, School of Medicine of Brescia, Brescia, Italy AU - Bottio, T. AU - Vida, V. AU - Padalino, M. AU - Gerosa, G. AU - Stellin, G. DB - Embase Medline DO - 10.1016/j.ejcts.2006.05.001 IS - 2 KW - biological marker creatine kinase MB troponin I arterial trunk article cardiopulmonary bypass child childhood clinical examination congenital heart malformation controlled study data base disease marker disease severity echocardiography Fallot tetralogy female follow up forward heart failure functional assessment great vessels transposition heart atrium septum defect heart disease heart ejection fraction heart function heart performance heart right ventricle double outlet heart surgery heart ventricle function heart ventricle septum defect human human tissue hypoplastic left heart syndrome laboratory test major clinical study male newborn surgery outcome assessment pediatric surgery postoperative period prediction priority journal prognosis quantitative analysis sensitivity and specificity surgical mortality survival vein malformation venous return LA - English M3 - Article N1 - L44051915 2006-07-30 PY - 2006 SN - 1010-7940 SP - 250-255 ST - Early and long-term prognostic value of Troponin-I after cardiac surgery in newborns and children T2 - European Journal of Cardio-thoracic Surgery TI - Early and long-term prognostic value of Troponin-I after cardiac surgery in newborns and children UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L44051915 http://dx.doi.org/10.1016/j.ejcts.2006.05.001 VL - 30 ID - 1258 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 μg/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 μg/l (CK-MB 570 ± 280 μg/l). Conversely, survivors showed a lower mean Tn-I value (25.5 ± 28.9 μg/l; CK-MB 76 ± 86 μg/l). Overall, Tn-I peak value was higher than 35 μg/l in 19 patients (19/70; 27.2%); among these, 9 died (median Tn-I was 163 ± 186 μg/l), whereas in survivors it was 73.4 ± 37 μg/l (p = 0.37). The remaining four patients who died had a median Tn-I value of 21 μg/l. When Tn-I exceeded 35 μg/l (>100 μg/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. © 2006 Elsevier B.V. All rights reserved. AD - Cardiovascular Institute, School of Medicine of Brescia, Brescia, Italy Cardiovascular Institute, School of Medicine of Padova, Padova, Italy AU - Bottio, T. AU - Vida, V. AU - Padalino, M. AU - Gerosa, G. AU - Stellin, G. DB - Scopus DO - 10.1016/j.ejcts.2006.05.001 IS - 2 KW - Congenital heart defects Echocardiographic follow-up Prognostic value Troponin-I M3 - Article N1 - Cited By :19 Export Date: 15 June 2020 PY - 2006 SP - 250-255 ST - Early and long-term prognostic value of Troponin-I after cardiac surgery in newborns and children T2 - European Journal of Cardio-thoracic Surgery TI - Early and long-term prognostic value of Troponin-I after cardiac surgery in newborns and children UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-33745882344&doi=10.1016%2fj.ejcts.2006.05.001&partnerID=40&md5=413c5eacce37994f6b0e6438e08f88e9 VL - 30 ID - 2228 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, United States AU - Boucek Jr, R. J. AU - Chrisant, M. R. DB - Scopus M3 - Article N1 - Cited By :15 Export Date: 15 June 2020 PY - 2004 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-5444253333&partnerID=40&md5=2526c7f8be55d45ef5b1e7925a8b0693 VL - 14 Suppl 1 ID - 2256 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 3.6kg (2.6-5.3kg). Complex lesions (dominant left ventricle): N=12, median age 59 days (10-884 days), median weight 3.4kg (2.4-12kg). 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. Copyright (C) 1999 Elsevier Science Ireland Ltd. AD - W.R. Thies, Department of Pediatric Cardiology, Heart Center NRW, Ruhr Universitat Bochum, Bad Oeynhausen, Germany 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. DB - Embase Medline DO - 10.1016/S1010-7940(99)00155-4 IS - 2 KW - angiography arteriovenous shunt article cardiovascular malformation clinical article Doppler echocardiography Fontan procedure heart right ventricle function heart single ventricle human hypoplastic left heart syndrome infant lung blood flow mortality newborn priority journal pulmonary artery risk factor superior cava vein surgical technique survival LA - English M3 - Article N1 - L29376662 1999-08-22 PY - 1999 SN - 1010-7940 SP - 117-124 ST - Results after Norwood procedure and subsequent cavopulmonary anastomoses for typical hypoplastic left heart syndrome and similar complex cardiovascular malformations T2 - European Journal of Cardio-thoracic Surgery TI - Results after Norwood procedure and subsequent cavopulmonary anastomoses for typical hypoplastic left heart syndrome and similar complex cardiovascular malformations UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L29376662 http://dx.doi.org/10.1016/S1010-7940(99)00155-4 VL - 16 ID - 1342 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 3.6kg (2.6-5.3kg). Complex lesions (dominant left ventricle): N=12, median age 59 days (10-884 days), median weight 3.4kg (2.4-12kg). 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. Copyright (C) 1999 Elsevier Science Ireland Ltd. AD - Dept. Thorac. and Cardiovasc. Surg., Heart Ctr. NRW, Ruhr Univ. B., Bad Oeynhausen, Germany Department of Pediatric Cardiology, Heart Ctr. NRW, Ruhr Univ. B., Bad Oeynhausen, Germany 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. DB - Scopus DO - 10.1016/S1010-7940(99)00155-4 IS - 2 KW - Fontan operation Hypoplastic left heart syndrome Norwood procedure Risk factors Single ventricle Total cavopulmonary anastomosis M3 - Article N1 - Cited By :30 Export Date: 15 June 2020 PY - 1999 SP - 117-124 ST - Results after Norwood procedure and subsequent cavopulmonary anastomoses for typical hypoplastic left heart syndrome and similar complex cardiovascular malformations T2 - European Journal of Cardio-thoracic Surgery TI - Results after Norwood procedure and subsequent cavopulmonary anastomoses for typical hypoplastic left heart syndrome and similar complex cardiovascular malformations UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0032768614&doi=10.1016%2fS1010-7940%2899%2900155-4&partnerID=40&md5=48d234583120b6c298e2151d2c689fad VL - 16 ID - 2282 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 le s ion A 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 cf 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. fix. 1. IKi. AD - R. Brock, Guys Hosp., London AU - Brock, R. DB - Embase Classic IS - 1 KW - ion 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 tricuspid valve atresia LA - English M3 - Article N1 - L281112824 1964-12-01 PY - 1964 SN - 0022-5223 SP - 17-25 ST - Tricuspid atrbsia: a step toward corrective treatment T2 - Journal of Thoracic and Cardiovascular Surgery TI - Tricuspid atrbsia: a step toward corrective treatment UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L281112824 VL - 47 ID - 1414 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 - A.S. John, Division of Pediatric Cardiology, Children’s National Hospital, 111 Michigan Ave, NW, Washington, DC, United States AU - Broda, C. R. AU - Downing, T. E. AU - John, A. S. DB - Embase Medline DO - 10.1007/s10741-020-09932-0 KW - adult child clinical outcome congenital heart disease Fontan procedure heart single ventricle human morbidity mortality palliative therapy phenotype quality of life review survival rate treatment failure LA - English M3 - Article in Press N1 - L2004567955 2020-04-13 PY - 2020 SN - 1573-7322 1382-4147 ST - Diagnosis and management of the adult patient with a failing Fontan circulation T2 - Heart Failure Reviews TI - Diagnosis and management of the adult patient with a failing Fontan circulation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2004567955 http://dx.doi.org/10.1007/s10741-020-09932-0 ID - 571 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, 111 Michigan Ave, NW, 20010, Washington, DC, USA AN - 143633003. Language: English. Entry Date: In Process. Revision Date: 20200611. Publication Type: journal article AU - Broda, Christopher R. AU - Downing, Tacy E. AU - John, Anitha S. DB - ccm DO - 10.1007/s10741-020-09932-0 DP - EBSCOhost IS - 4 N1 - review. Journal Subset: Biomedical; USA. NLM UID: 9612481. PMID: NLM32185561. PY - 2020 SN - 1382-4147 SP - 633-646 ST - Diagnosis and management of the adult patient with a failing Fontan circulation T2 - Heart Failure Reviews TI - Diagnosis and management of the adult patient with a failing Fontan circulation UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=143633003&site=ehost-live&scope=site VL - 25 ID - 1455 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. © 2013 Springer Science+Business Media New York. AD - C. Brosig, Department of Pediatrics, Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI 53201, United States AU - Brosig, C. AU - Mussatto, K. AU - Hoffman, G. AU - Hoffmann, R. G. AU - Dasgupta, M. AU - Tweddell, J. AU - Ghanayem, N. DB - Embase Medline DO - 10.1007/s00246-013-0679-3 IS - 7 KW - article attention child child behavior Child Behavior Checklist female hand movement human hypoplastic left heart syndrome integration intelligence language major clinical study male memory motor performance nerve cell differentiation Norwood procedure occupational therapy pediatric surgery physiotherapy preschool child speech therapy Wechsler intelligence scale LA - English M3 - Article N1 - L52493468 2013-03-20 2013-10-22 PY - 2013 SN - 0172-0643 1432-1971 SP - 1597-1604 ST - Neurodevelopmental outcomes for children with hypoplastic left heart syndrome at the age of 5 years T2 - Pediatric Cardiology TI - Neurodevelopmental outcomes for children with hypoplastic left heart syndrome at the age of 5 years UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52493468 http://dx.doi.org/10.1007/s00246-013-0679-3 VL - 34 ID - 987 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 - C.L. Brosig, Department of Pediatrics, Medical College of Wisconsin, 8701 Watertown Plank Rd, Milwaukee, WI, United States AU - Brosig, C. L. AU - Bear, L. AU - Allen, S. AU - Hoffmann, R. G. AU - Pan, A. AU - Frommelt, M. AU - Mussatto, K. A. DB - Embase Medline DO - 10.1016/j.jpeds.2016.12.044 KW - adaptive behavior article child child development congenital heart disease female human language ability longitudinal study major clinical study male mental development motor performance preschool child priority journal social adaptation spelling LA - English M3 - Article N1 - L614998497 2017-03-29 2017-04-04 PY - 2017 SN - 1097-6833 0022-3476 SP - 80-86.e1 ST - Preschool Neurodevelopmental Outcomes in Children with Congenital Heart Disease T2 - Journal of Pediatrics TI - Preschool Neurodevelopmental Outcomes in Children with Congenital Heart Disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L614998497 http://dx.doi.org/10.1016/j.jpeds.2016.12.044 VL - 183 ID - 761 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 AN - 121910641. Language: English. Entry Date: 20170701. Revision Date: 20181016. Publication Type: journal article. Journal Subset: Biomedical AU - Brosig, Cheryl L. AU - Bear, Laurel AU - Allen, Sydney AU - Hoffmann, Raymond G. AU - Pan, Amy AU - Frommelt, Michele AU - Mussatto, Kathleen A. DB - ccm DO - 10.1016/j.jpeds.2016.12.044 DP - EBSCOhost KW - Developmental Disabilities -- Etiology Heart Defects, Congenital -- Complications Heart Defects, Congenital -- Diagnosis Intellectual Disability -- Etiology Sex Factors Child, Preschool Intellectual Disability -- Diagnosis Age Factors Developmental Disabilities -- Diagnosis Logistic Regression United States Prospective Studies Infant, Newborn Infant Neuropsychological Tests Intellectual Disability -- Epidemiology Male Multivariate Analysis Female Severity of Illness Indices Developmental Disabilities -- Epidemiology Risk Assessment N1 - Peer Reviewed; USA. Grant Information: UL1 TR000055/TR/NCATS NIH HHS/United States. NLM UID: 0375410. PMID: NLM28081891. PY - 2017 SN - 0022-3476 SP - 80-86.e1 ST - Preschool Neurodevelopmental Outcomes in Children with Congenital Heart Disease T2 - Journal of Pediatrics TI - Preschool Neurodevelopmental Outcomes in Children with Congenital Heart Disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=121910641&site=ehost-live&scope=site VL - 183 ID - 1572 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. © 2007 Springer Science+Business Media, LLC. AD - C.L. Brosig, Department of Pediatrics, Medical College of Wisconsin, MS 744, 9000 West Wisconsin Avenue, Milwaukee, WI 53226, United States AU - Brosig, C. L. AU - Mussatto, K. A. AU - Kuhn, E. M. AU - Tweddell, J. S. DB - Embase Medline DO - 10.1007/s00246-006-0013-4 IS - 4 KW - age distribution article cardiopulmonary bypass clinical article congenital heart disease controlled study family functioning female follow up great vessels transposition hospitalization human hypoplastic left heart syndrome length of stay male open heart surgery outcome assessment parental stress preschool child quality of life rating scale scoring system social psychology social status statistical analysis LA - English M3 - Article N1 - L46976646 2007-08-01 PY - 2007 SN - 0172-0643 SP - 255-262 ST - Psychosocial outcomes for preschool children and families after surgery for complex congenital heart disease T2 - Pediatric Cardiology TI - Psychosocial outcomes for preschool children and families after surgery for complex congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L46976646 http://dx.doi.org/10.1007/s00246-006-0013-4 VL - 28 ID - 1230 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 - 106262405. Language: English. Entry Date: 20070406. Revision Date: 20150819. Publication Type: Journal Article AU - Brosig, C. L. AU - Mussatto, K. A. AU - Kuhn, E. M. AU - Tweddell, J. S. DB - ccm DP - EBSCOhost IS - 1 KW - Developmental Disabilities -- Diagnosis Hypoplastic Left Heart Syndrome -- Surgery Transposition of Great Arteries -- Surgery Academic Achievement -- Evaluation Analysis of Variance Aptitude Tests Attention -- Evaluation Behavior Rating Scales Chi Square Test Child Child Behavior Checklist Child, Preschool Cross Sectional Studies Developmental Disabilities -- Risk Factors Female Funding Source Health Screening -- Education Hospitals, Pediatric -- Wisconsin Infant Intelligence Intelligence Tests Language Development Language Tests Male Mann-Whitney U Test Motor Skills -- Evaluation Neuropsychological Tests Parents -- Education Pediatric Nurse Practitioners Record Review Research Instruments Spearman's Rank Correlation Coefficient Surgery, Cardiovascular -- Adverse Effects Treatment Outcomes Wisconsin Human N1 - research; tables/charts. Journal Subset: Core Nursing; Nursing; Peer Reviewed; USA. Instrumentation: McCarthy Scales of Children's Abilities (MSCA); Hollingshead Four Factor Index of Social Status; Child Behavior Checklist (CBCL) (Achenbach and Edelbrock); Expressive One-Word Picture Vocabulary Test-Third Edition (EOWPVT) (Brownell); Woodcock Johnson III Tests of Achievement (WJIII) (Woodcock et al); General Cognitive Index (GCI); Receptive One-Word Picture Vocabulary Test-Second Edition (ROWPVT) (Brownell); Beery-Buktenica Developmental Test of Visual-Motor Integration (VMI). Grant Information: Julie Lathrop Nursing Research Award from thee Children's Hospital Foundation. NLM UID: 8709735. PMID: NLM17198894. PY - 2007 SN - 0891-5245 SP - 3-12 ST - Neurodevelopmental outcome in preschool survivors of complex congenital heart disease: implications for clinical practice T2 - Journal of Pediatric Healthcare TI - Neurodevelopmental outcome in preschool survivors of complex congenital heart disease: implications for clinical practice UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106262405&site=ehost-live&scope=site VL - 21 ID - 1602 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. © 2007 Springer Science+Business Media, LLC. AD - Department of Pediatrics, Medical College of Wisconsin, MS 744, 9000 West Wisconsin Avenue, Milwaukee, WI 53226, United States Children's Hospital of Wisconsin, Herma Heart Center, Milwaukee, WI 53226, United States Children's Hospital of Wisconsin, National Outcomes Center, Inc., Milwaukee, WI 53226, United States Department of Surgery, Medical College of Wisconsin, Milwaukee, WI 53226, United States AU - Brosig, C. L. AU - Mussatto, K. A. AU - Kuhn, E. M. AU - Tweddell, J. S. DB - Scopus DO - 10.1007/s00246-006-0013-4 IS - 4 KW - Congenital heart disease Psychosocial outcomes M3 - Article N1 - Cited By :91 Export Date: 15 June 2020 PY - 2007 SP - 255-262 ST - Psychosocial outcomes for preschool children and families after surgery for complex congenital heart disease T2 - Pediatric Cardiology TI - Psychosocial outcomes for preschool children and families after surgery for complex congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-34250834457&doi=10.1007%2fs00246-006-0013-4&partnerID=40&md5=23169cdb55d794f82a09f9d0d9f71c92 VL - 28 ID - 2207 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 - Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, United States Herma Heart Center, Children's Hospital of Wisconsin, Milwaukee, WI, United States Quality and Outcomes Management, Children's Hospital 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 - Scopus DO - 10.1038/sj.jp.7211807 IS - 11 M3 - Article N1 - Cited By :74 Export Date: 15 June 2020 PY - 2007 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-35648958695&doi=10.1038%2fsj.jp.7211807&partnerID=40&md5=755e94539fbbe9338328924f0d54d73e VL - 27 ID - 2205 ER - TY - JOUR AB - 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 - Cardiac Unit, Great Ormond Street Hospital NHS Trust, London WC1N 3JH, United Kingdom Clinical Operational Research Unit, University College London, London, United Kingdom National Institute Cardiovascular Outcomes Research, Central Cardiac Audit Database, University College London, London, United Kingdom Paediatric Cardiology, Royal Brompton and Harefield Hospitals, London, 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 - Scopus DO - 10.1017/S1047951112001369 IS - 4 KW - congenital heart disease Keywords Outcomes quality assurance M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 2013 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84880207060&doi=10.1017%2fS1047951112001369&partnerID=40&md5=0f37203f13528d40cf57caea5e8ae5db VL - 23 ID - 2041 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. © Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY. Published by BMJ. AD - Cardiorespiratory Division, Great Ormond Street Hospital for Children, London, United Kingdom Clinical Operational Research Unit, UCL, London, United Kingdom Institute of Child Health, UCL, London, United Kingdom Evelina London Children's Hospital, London, United Kingdom Birmingham Women's and Children's NHS Foundation Trust, Birmingham, United Kingdom Paediatric Intensive Care, Bristol Royal Hospital for Children, Bristol, United Kingdom Health Economics, University College London, London, United Kingdom Congenital Heart Surgery, Royal Hospital for Children, Glasgow, United Kingdom Department of Applied Health Research, University College London, London, United Kingdom Royal Hospital for Children, Glasgow, United Kingdom Bristol Royal Hospital for Children, Bristol, United Kingdom University Hospitals Bristol NHS Foundation Trust, Bristol, United Kingdom Paediatric Intensive Care, Evelina London Children's Hospital, 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. C7 - e028533 DB - Scopus DO - 10.1136/bmjopen-2018-028533 IS - 9 KW - cardiac surgery complications morbidity outcome paediatrics quality of life M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2019 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85071974820&doi=10.1136%2fbmjopen-2018-028533&partnerID=40&md5=a8b0064a24c17accf915adf40474f779 VL - 9 ID - 1734 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 - Cardiology Department, Hospital Sant Joan de Déu-Hospital Clínic, University of Barcelona, 08036 Barcelona, Spain Department of Pediatric Cardiology, Leiden University Medical Center and Academical Medical Center Amsterdam, 2300 RC Leiden, Netherlands Cardiology Department, Hospital Sant Joan de Déu, University of Barcelona, 08950 Barcelona, Spain Department of Cardiology, Uppsala University, s-75236 Uppsala, Sweden Cardiothoracic Unit, Great Ormond Street Hospital, Great Ormond Street, WC1N 3JH London, United Kingdom Children's Heart Centre, University Hospital Motol, 15006 Prague, Czech Republic Cardiac Electrophysiology Division, Department of Cardiology, Children's Hospital, Boston, 02115 MA, United States Medizinische Fakultät, Kinderspital Zürich, 8032 Zürich, Switzerland Cardiovascular Genetics Center, Institut d'Investigació Biomèdica Girona-IdIBGi, 17003 Girona, Spain Arrhythmia Unit, Pediatric Cardiology and Heart Surgery Department, Bambino Gesù Pediatric Hospital, Palidoro, 00055 Fiumicino, Italy Department of Cardiology, Erasmus MC, 3015 Rotterdam, Netherlands Department of Pediatric Cardiology, Children's Hospital, University of Helsinki and Helsinki University Central Hospital, 00290 Helsinki, Finland Center for Electrophysiology, 28277 Bremen, Germany Cardiac Morphology Unit, Royal Brompton Hospital and Imperial College, London, SW3 6NP, United Kingdom Paris Cardiovascular Research Center, Inserm U970, European Georges Pompidou Hospital, 75908 Paris, France Department of Pediatric Cardiology and Intensive Care Medicine, Childreńs University Hospital, Georg-August-University, 37099 Göttingen, Germany Pedriatic Cardiology, University of Bern, 3010 Bern, Switzerland Evelina Children's Hospital, Guy's and St Thomas' Hospital, SE1 7EH London, United Kingdom 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 - Scopus DO - 10.1093/europace/eut082 IS - 9 KW - Antiarrhythmic drugs Arrhythmias Electrical devices Paediatrics Radiofrequency ablation M3 - Article N1 - Cited By :131 Export Date: 15 June 2020 PY - 2013 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84883437124&doi=10.1093%2feuropace%2feut082&partnerID=40&md5=be13fffaaf2ca7365ac9a61fa6cf47ad VL - 15 ID - 2038 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 - Division of Cardiovascular Surgery, Mayo Clinic, Mayo Foundation, Rochester, MN, United States Section of Pediatric Cardiology, Mayo Clinic, Mayo Foundation, Rochester, MN, United States Division of Cardiovascular Diseases, Mayo Clinic, Mayo Foundation, Rochester, MN, United States Division of Biostatistics, Mayo Clinic, Mayo Foundation, Rochester, MN, United States 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 - Scopus DO - 10.1016/S0022-5223(03)00117-X IS - 6 M3 - Article N1 - Cited By :61 Export Date: 15 June 2020 PY - 2003 SP - 1252-1258 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0038649817&doi=10.1016%2fS0022-5223%2803%2900117-X&partnerID=40&md5=7e7c059e40b0c23bbcceb1a2a73998c4 VL - 125 ID - 2261 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. © 2018 Wiley Periodicals, Inc. AD - Department of Psychiatry, Boston Children’s Hospital, Boston, MA, United States Department of Cardiology, Boston Children’s Hospital, Boston, MA, United States Developmental Medicine Center, Boston Children’s Hospital, Boston, MA, United States Department of Psychiatry, Harvard Medical School, Boston, MA, United States Department of Pediatrics, Harvard Medical School, Boston, MA, United States Department of Biostatistics, Harvard T.H. Chan School of Public Health, 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 - Scopus DO - 10.1111/chd.12686 IS - 2 KW - assessment cardiac developmental impairments infancy neurodevelopment M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2019 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85054910396&doi=10.1111%2fchd.12686&partnerID=40&md5=bed189179b0dcb9485e23a3bf4cc821d VL - 14 ID - 1759 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. © 2015, Springer Science+Business Media New York. AD - Division of Cardiology, Department of Pediatrics, Medical University of South Carolina, 165 Ashley Avenue, MSC 915, Charleston, SC 29425, 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 - Scopus DO - 10.1007/s00246-015-1262-x IS - 1 KW - Fontan Pressure–volume loop Sildenafil Single-ventricle heart disease M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 2016 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84957439914&doi=10.1007%2fs00246-015-1262-x&partnerID=40&md5=6b6ea297ea9dccd7a3a3ec14d77d864c VL - 37 ID - 1926 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. © 2019 Wiley Periodicals, Inc. AD - Departments of Internal Medicine and Pediatrics, Vanderbilt University Medical Center, Nashville, TN, United States Vanderbilt Heart and Vascular Institute, Vanderbilt University Medical Center, Nashville, TN, United States Department of Biostatistics, Vanderbilt University Medical Center, Nashville, TN, United States Department of Internal Medicine, Division of Gastroenterology, Hepatology and Nutrition, Vanderbilt University Medical Center, Nashville, TN, United States Departments of Pediatrics and Internal Medicine, Division of Cardiology, Indiana University School of Medicine, Riley Hospital for Children, Indianapolis, IN, United States 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. DB - Scopus DO - 10.1111/chd.12820 IS - 5 KW - extra-cardiac Fontan liver MELD renal M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2019 SP - 765-771 ST - More than the heart: Hepatic, renal, and cardiac dysfunction in adult Fontan patients T2 - Congenital Heart Disease TI - More than the heart: Hepatic, renal, and cardiac dysfunction in adult Fontan patients UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85068667796&doi=10.1111%2fchd.12820&partnerID=40&md5=52105a337e937989c40781155244576c VL - 14 ID - 1737 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. AU - Calderon, J. AU - Stopp, C. AU - Wypij, D. AU - DeMaso, D. R. AU - Rivkin, M. AU - Newburger, J. W. AU - Bellinger, D. C. DB - Embase Medline DO - 10.1016/j.jpeds.2016.08.084 KW - adolescent adult article attention deficit disorder behavior rating inventory of executive function birth weight Brief Psychiatric Rating Scale child child psychiatry clinical outcome cohort analysis controlled study cross-sectional study daily life activity demography disease severity executive function female Fontan procedure heart single ventricle human major clinical study male medical record mental development neuroimaging nuclear magnetic resonance imaging prematurity priority journal race retrospective study risk factor self report sex social status term birth LA - English M3 - Article N1 - L613457035 2016-12-05 2016-12-30 PY - 2016 SN - 1097-6833 0022-3476 SP - 96-103 ST - Early-Term Birth in Single-Ventricle Congenital Heart Disease After the Fontan Procedure: Neurodevelopmental and Psychiatric Outcomes T2 - Journal of Pediatrics TI - Early-Term Birth in Single-Ventricle Congenital Heart Disease After the Fontan Procedure: Neurodevelopmental and Psychiatric Outcomes UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L613457035 http://dx.doi.org/10.1016/j.jpeds.2016.08.084 VL - 179 ID - 787 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 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 Neurology, Harvard Medical School, Boston, MA AN - 119482005. Language: English. Entry Date: 20170701. Revision Date: 20180927. Publication Type: journal article AU - Calderon, Johanna AU - Stopp, Christian AU - Wypij, David AU - DeMaso, David R. AU - Rivkin, Michael AU - Newburger, Jane W. AU - Bellinger, David C. DB - ccm DO - 10.1016/j.jpeds.2016.08.084 DP - EBSCOhost KW - Cardiopulmonary Bypass Mental Disorders -- Epidemiology Heart Ventricle -- Abnormalities Heart Defects, Congenital -- Surgery Heart Ventricle -- Surgery Female Executive Function Prospective Studies Attention Deficit Hyperactivity Disorder -- Epidemiology Male Term Birth Cross Sectional Studies Gestational Age Adolescence Clinical Assessment Tools Scales N1 - research. Journal Subset: Biomedical; Peer Reviewed; USA. Instrumentation: Brief Psychiatric Rating Scale (BPRS); Behavior Rating Inventory of Executive Function (BRIEF). Grant Information: U54 HD090255/HD/NICHD NIH HHS/United States. NLM UID: 0375410. PMID: NLM27692462. 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 - Journal of Pediatrics TI - Early-Term Birth in Single-Ventricle Congenital Heart Disease After the Fontan Procedure: Neurodevelopmental and Psychiatric Outcomes UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=119482005&site=ehost-live&scope=site VL - 179 ID - 1480 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 γGT 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 (r2 = 0.34, p = 0.008) and to a lesser extent to reduced heart rate (r2 = 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. © 2008 The Society of Thoracic Surgeons. AD - Department of Pediatric Hepatology, University of Padova, Padova, Italy Department of Pediatric Cardiology, University of Padova, Padova, Italy Department of Pediatric Cardiac Surgery, University of Padova, Padova, Italy AU - Camposilvan, S. AU - Milanesi, O. AU - Stellin, G. AU - Pettenazzo, A. AU - Zancan, L. AU - D'Antiga, L. DB - Scopus DO - 10.1016/j.athoracsur.2008.03.077 IS - 1 M3 - Article N1 - Cited By :107 Export Date: 15 June 2020 PY - 2008 SP - 177-182 ST - Liver and Cardiac Function in the Long Term After Fontan Operation T2 - Annals of Thoracic Surgery TI - Liver and Cardiac Function in the Long Term After Fontan Operation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-47049119689&doi=10.1016%2fj.athoracsur.2008.03.077&partnerID=40&md5=3a3b05a7b2fe0f783e5f9be0d4a3358a VL - 86 ID - 2192 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 month 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 hearth 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 tranplantation 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 tranplantation. AD - C. Canter, Division of Pediatric Cardiology, St Louis Children's Hospital, One Children's Place, Louis, MO 63110, United States 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. DB - Embase Medline DO - 10.1161/01.CIR.96.1.227 IS - 1 KW - article graft failure heart death heart transplantation human infant major clinical study newborn postoperative complication priority journal risk assessment risk factor LA - English M3 - Article N1 - L27298425 1997-08-01 PY - 1997 SN - 0009-7322 SP - 227-231 ST - Survival and risk factors for death after cardiac transplantation in infants: A multi-institutional study T2 - Circulation TI - Survival and risk factors for death after cardiac transplantation in infants: A multi-institutional study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L27298425 http://dx.doi.org/10.1161/01.CIR.96.1.227 VL - 96 ID - 1356 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 month 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 hearth 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 tranplantation 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 tranplantation. AD - Washington University, St Louis, MO, United States University of Alabama, Birmingham, AL, United States Indiana University, Indianapolis, IN, United States Loma Linda University, Loma Linda, CA, United States Northwestern University, Chicago, IL, United States Arkansas Children's Hospital, Little Rock, AR, United States University of Colorado, Denver, CO, United States Children's Hospital of Michigan, Detroit, MI, United States Division of Pediatric Cardiology, St. Louis Children's Hospital, One Children's Place, St Louis, MO 63110, United States 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. DB - Scopus DO - 10.1161/01.CIR.96.1.227 IS - 1 KW - Infants Risk factors Survival Transplantation M3 - Article N1 - Cited By :80 Export Date: 15 June 2020 PY - 1997 SP - 227-231 ST - Survival and risk factors for death after cardiac transplantation in infants: A multi-institutional study T2 - Circulation TI - Survival and risk factors for death after cardiac transplantation in infants: A multi-institutional study UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0030806856&doi=10.1161%2f01.CIR.96.1.227&partnerID=40&md5=a093c0a2f0d5aa3698dbd64f8b121f45 VL - 96 ID - 2296 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. AU - Cantwell-Bartl, A. DB - Medline DO - 10.1080/07481187.2017.1407380 IS - 9 KW - adult child child parent relation classification coping behavior female grief handicapped child human hypoplastic left heart syndrome male nursing psychological model psychology LA - English M3 - Article N1 - L624883276 2019-01-03 PY - 2018 SN - 1091-7683 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 studies 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L624883276 http://dx.doi.org/10.1080/07481187.2017.1407380 VL - 42 ID - 664 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 - Vic, Australia AN - 129370308. Language: English. Entry Date: 20180504. Revision Date: 20200324. Publication Type: Article AU - Cantwell-Bartl, Annie DB - ccm DO - 10.1080/07481187.2017.1407380 DP - EBSCOhost IS - 9 KW - Grief Coping Parental Attitudes Parent-Child Relations Hypoplastic Left Heart Syndrome -- Psychosocial Factors Models, Psychological Human Child Thematic Analysis Secondary Analysis Interviews Hypoplastic Left Heart Syndrome -- Therapy Recovery Hypoplastic Left Heart Syndrome -- Diagnosis N1 - research; tables/charts. Journal Subset: Biomedical; Europe; Peer Reviewed; UK & Ireland. NLM UID: 8506890. 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 Studies 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=129370308&site=ehost-live&scope=site VL - 42 ID - 1435 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. (PsycINFO Database Record (c) 2019 APA, all rights reserved) AD - Cantwell-Bartl, Annie, Private Practice, 24 Market St, Kensington, VIC, Australia, 3031 AN - 2018-20606-004 AU - Cantwell-Bartl, Annie DB - psyh DO - 10.1080/07481187.2017.1407380 DP - EBSCOhost IS - 9 KW - grief coping parents Adaptation, Psychological Adult Child Disabled Children Female Humans Hypoplastic Left Heart Syndrome Male Models, Psychological Coping Behavior Heart Disorders Child Characteristics N1 - Private Practice, Kensington, VIC, Australia. Release Date: 20190826. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Coping Behavior; Heart Disorders; Parents; Child Characteristics. Minor Descriptor: Grief. Classification: Cardiovascular Disorders (3295). Population: Human (10); Male (30); Female (40). Location: Australia. Age Group: Childhood (birth-12 yrs) (100); Adulthood (18 yrs & older) (300). Methodology: Empirical Study; Interview; Qualitative Study. Page Count: 10. Issue Publication Date: Oct, 2018. Copyright Statement: Taylor & Francis. 2018. PY - 2018 SN - 0748-1187 1091-7683 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 Studies 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2018-20606-004&site=ehost-live&scope=site anniecantwellbartl@gmail.com VL - 42 ID - 1660 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. © 2018 Taylor & Francis. AD - VIC, Australia AU - Cantwell-Bartl, A. DB - Scopus DO - 10.1080/07481187.2017.1407380 IS - 9 M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2018 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 Studies 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85045108695&doi=10.1080%2f07481187.2017.1407380&partnerID=40&md5=2c74d74960dd9bc86d2b0953fd863481 VL - 42 ID - 1786 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. © 2008 Centre for Bioethics, IRCM. AD - A.M. Cantwell-Bartl, Pediatric Intensive Care Unit, Royal Children's Hospital, University of Melbourne AU - Cantwell-Bartl, A. M. AU - Tibballs, J. DB - Medline IS - 2 KW - adolescent age distribution article Australia bereavement child counseling decision making heart surgery human human relation hypoplastic left heart syndrome infant mortality newborn parent preschool child psychological aspect retrospective study LA - English M3 - Article N1 - L352022592 2008-10-15 PY - 2008 SN - 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 - Journal of Palliative 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L352022592 VL - 24 ID - 1207 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. (PsycINFO Database Record (c) 2017 APA, all rights reserved) AD - Cantwell-Bartl, Annie M., Pediatric Intensive Care Unit, Royal Children's Hospital, Flemington Road, Parkville, Melbourne, VIC, Australia, 3052 AN - 2008-09313-003 AU - Cantwell-Bartl, Annie M. AU - Tibballs, James DA - Sum 2008 DB - psyh DP - EBSCOhost IS - 2 KW - place age differences infants death mode children hypoplastic left heart syndrome medical counseling psychological counseling palliative care surgery Adolescent Age Distribution Bereavement Cardiac Surgical Procedures Child Child, Preschool Counseling Decision Making Humans Infant Infant, Newborn Parents Professional-Family Relations Retrospective Studies Victoria Death and Dying Heart Disorders Pediatrics Syndromes N1 - Pediatric Intensive Care Unit, Royal Children's Hospital, University of Melbourne, Melbourne, VIC, Australia. Other Publishers: Sage Publications. Release Date: 20090504. Correction Date: 20170206. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Print. Document Type: Journal Article. Language: English. Major Descriptor: Death and Dying; Heart Disorders; Palliative Care; Pediatrics. Minor Descriptor: Counseling; Syndromes. Classification: Medical Treatment of Physical Illness (3363). Population: Human (10). Age Group: Childhood (birth-12 yrs) (100); Infancy (2-23 mo) (140); Preschool Age (2-5 yrs) (160); School Age (6-12 yrs) (180); Adolescence (13-17 yrs) (200). Methodology: Empirical Study; Quantitative Study. References Available: Y. Page Count: 9. Issue Publication Date: Sum 2008. PY - 2008 SN - 0825-8597 2369-5293 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 - Journal of Palliative 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2008-09313-003&site=ehost-live&scope=site VL - 24 ID - 1690 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. Copyright © 2013 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies. AD - A.M. Cantwell-Bartl, Paediatric Intensive Care Unit, Royal Children's Hospital, Melbourne, VIC, Australia AU - Cantwell-Bartl, A. M. AU - Tibballs, J. DB - Embase Medline DO - 10.1097/PCC.0b013e31829b1a88 IS - 9 KW - acute stress disorder adaptation adolescent adult article child child care child death child parent relation clinical article doctor patient relationship family life father female human hypoplastic left heart syndrome intensive care unit male mother narrative parental stress posttraumatic stress disorder prenatal diagnosis preschool child priority journal prognosis psychometry religion retrospective study school child semi structured interview social psychology Structured Clinical Interview for DSM Disorders support group young adult LA - English M3 - Article N1 - L52741053 2013-08-26 2014-02-05 PY - 2013 SN - 1529-7535 1947-3893 SP - 869-875 ST - Psychosocial experiences of parents of infants with hypoplastic left heart syndrome in the PICU T2 - Pediatric Critical Care Medicine TI - Psychosocial experiences of parents of infants with hypoplastic left heart syndrome in the PICU UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52741053 http://dx.doi.org/10.1097/PCC.0b013e31829b1a88 VL - 14 ID - 981 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 - 107918750. Language: English. Entry Date: 20140815. Revision Date: 20150712. Publication Type: Journal Article AU - Cantwell-Bartl, Annie M. AU - Tibballs, James DB - ccm DO - 10.1097/PCC.0b013e31829b1a88 DP - EBSCOhost IS - 9 KW - Fathers -- Psychosocial Factors Hypoplastic Left Heart Syndrome -- Diagnosis Mothers -- Psychosocial Factors Stress Disorders, Post-Traumatic -- Etiology Adaptation, Psychological Adolescence Child Child, Preschool Female Health Facility Environment Human Hypoplastic Left Heart Syndrome -- Surgery Infant Infant, Newborn Intensive Care Units, Pediatric Interviews Male Mother-Child Relations Psychoanalytic Theory Pregnancy Prenatal Diagnosis -- Psychosocial Factors Psychological Tests Retrospective Design Stress Disorders, Post-Traumatic -- Psychosocial Factors Truth Disclosure Young Adult N1 - research. Journal Subset: Biomedical; Peer Reviewed; USA. Special Interest: Critical Care; Pediatric Care. NLM UID: 100954653. PMID: NLM23965635. PY - 2013 SN - 1529-7535 SP - 869-875 ST - Psychosocial Experiences of Parents of Infants With Hypoplastic Left Heart Syndrome in the PICU T2 - Pediatric Critical Care Medicine TI - Psychosocial Experiences of Parents of Infants With Hypoplastic Left Heart Syndrome in the PICU UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=107918750&site=ehost-live&scope=site VL - 14 ID - 1523 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. Copyright © 2013 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies. AD - Paediatric Intensive Care Unit, Royal Children's Hospital, Melbourne, VIC, Australia AU - Cantwell-Bartl, A. M. AU - Tibballs, J. DB - Scopus DO - 10.1097/PCC.0b013e31829b1a88 IS - 9 KW - Acute stress disorder Adaptation Hypoplastic left heart syndrome Intensive care Parents Posttraumatic stress disorder Psychological Stress M3 - Article N1 - Cited By :21 Export Date: 15 June 2020 PY - 2013 SP - 869-875 ST - Psychosocial experiences of parents of infants with hypoplastic left heart syndrome in the PICU T2 - Pediatric Critical Care Medicine TI - Psychosocial experiences of parents of infants with hypoplastic left heart syndrome in the PICU UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84892790332&doi=10.1097%2fPCC.0b013e31829b1a88&partnerID=40&md5=509f83885c9b2b055c2007e98f42bd47 VL - 14 ID - 2040 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 - A.M. Cantwell-Bartl, Honorary Research Fellow, Paediatric Intensive Care Unit, Royal Children's Hospital, Melbourne, VIC, Australia AU - Cantwell-Bartl, A. M. AU - Tibballs, J. DB - Embase Medline DO - 10.1017/S1047951114001590 IS - 6 KW - article controlled study family decision making female human hypoplastic left heart syndrome infant interpersonal communication male maternal behavior mental stress paternal behavior personal experience social psychology LA - English M3 - Article N1 - L600581860 2014-12-02 2015-11-06 PY - 2015 SN - 1467-1107 1047-9511 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 - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L600581860 http://dx.doi.org/10.1017/S1047951114001590 VL - 25 ID - 914 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 - 109594034. Language: English. Entry Date: 20150923. Revision Date: 20161102. Publication Type: journal article. Journal Subset: Biomedical AU - Cantwell-Bartl, Annie M. AU - Tibballs, James DB - ccm DO - 10.1017/S1047951114001590 DP - EBSCOhost IS - 6 N1 - Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Special Interest: Pediatric Care. NLM UID: 9200019. PMID: NLM25215886. PY - 2015 SN - 1047-9511 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 - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=109594034&site=ehost-live&scope=site VL - 25 ID - 1483 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 - A.M. Cantwell-Bartl, 24 Market St, Kensington, Melbourne, VIC, Australia AU - Cantwell-Bartl, A. M. AU - Tibballs, J. DB - Embase Medline DO - 10.1017/S1047951117000270 IS - 7 KW - article child care child parent relation controlled study female grief home care home environment hospital discharge human hypoplastic left heart syndrome interview involuntary commitment love male parental attitude parental stress personal experience psychometry semi structured interview LA - English M3 - Article N1 - L616642916 2017-06-12 2017-08-18 PY - 2017 SN - 1467-1107 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 - Cardiology in the Young TI - Parenting a child at home with hypoplastic left heart syndrome: Experiences of commitment, of stress, and of love UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L616642916 http://dx.doi.org/10.1017/S1047951117000270 VL - 27 ID - 734 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 - 124503271. Language: English. Entry Date: 20180520. Revision Date: 20190315. Publication Type: journal article AU - Cantwell-Bartl, Annie M. AU - Tibballs, James DB - ccm DO - 10.1017/S1047951117000270 DP - EBSCOhost IS - 7 KW - Hypoplastic Left Heart Syndrome -- Surgery Parenting Stress Disorders, Post-Traumatic -- Diagnosis Anxiety -- Diagnosis Parents -- Psychosocial Factors Adaptation, Psychological Child, Preschool Interviews Female Love Psychological Tests Child Male Retrospective Design Infant Adolescence Young Adult Income Human Questionnaires N1 - research; tables/charts. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Instrumentation: Maslach Burnout Inventory; Impact on Family Scale; Psychological Check List-Civilian. NLM UID: 9200019. PMID: NLM28583211. 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 - Cardiology in the Young TI - Parenting a child at home with hypoplastic left heart syndrome: experiences of commitment, of stress, and of love UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=124503271&site=ehost-live&scope=site VL - 27 ID - 1470 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. © 2017 Cambridge University Press. AD - 24 Market St, Kensington, Melbourne, VIC 3031, Australia AU - Cantwell-Bartl, A. M. AU - Tibballs, J. DB - Scopus DO - 10.1017/S1047951117000270 IS - 7 KW - adaptation grief Hypoplastic left heart syndrome parents stress M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2017 SP - 1341-1348 ST - Parenting a child at home with hypoplastic left heart syndrome: Experiences of commitment, of stress, and of love T2 - Cardiology in the Young TI - Parenting a child at home with hypoplastic left heart syndrome: Experiences of commitment, of stress, and of love UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85020234526&doi=10.1017%2fS1047951117000270&partnerID=40&md5=0b63472fbd3d869c5b6cb2d2263237dc VL - 27 ID - 1844 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 - C.L. Cua, Nationwide Children's Hospital, Columbus, OH, United States 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. DB - Embase Medline DO - 10.1111/chd.12387 IS - 6 KW - adult anxiety article caregiver burden caregiver support child child development cohort analysis cross-sectional study demography developmental disorder female health survey human hypoplastic left heart syndrome male parental stress Parenting Stress Index Short Form Pediatric Inventory for Parents Pediatric Quality of Life Inventory priority journal prospective study psychosocial care quality of life quality of life assessment questionnaire stress assessment LA - English M3 - Article N1 - L613108637 2016-11-10 2017-01-03 PY - 2016 SN - 1747-0803 1747-079X SP - 727-732 ST - Anxiety Scores in Caregivers of Children with Hypoplastic Left Heart Syndrome T2 - Congenital Heart Disease TI - Anxiety Scores in Caregivers of Children with Hypoplastic Left Heart Syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L613108637 http://dx.doi.org/10.1111/chd.12387 VL - 11 ID - 790 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. © 2016 Wiley Periodicals, Inc. AD - Nationwide Children's Hospital, Columbus, OH, United States Nicklaus Children's Hospital, Columbus, OH, United States 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. DB - Scopus DO - 10.1111/chd.12387 IS - 6 KW - Congenital Heart Disease Hypoplastic Left Heart Parenting Stress Survey M3 - Article N1 - Cited By :13 Export Date: 15 June 2020 PY - 2016 SP - 727-732 ST - Anxiety Scores in Caregivers of Children with Hypoplastic Left Heart Syndrome T2 - Congenital Heart Disease TI - Anxiety Scores in Caregivers of Children with Hypoplastic Left Heart Syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84994137849&doi=10.1111%2fchd.12387&partnerID=40&md5=110975aa6a39719b13892f0ac021d494 VL - 11 ID - 1884 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 - E.C. Caris, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, United States 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. DB - Embase Medline DO - 10.1111/chd.12619 IS - 4 KW - adolescent adult article caregiver child controlled study cross-sectional study family attitude female human hypoplastic left heart syndrome male Negative Adjustment Composite Score parent pediatrics perception perception test priority journal prospective study quality of life self report sibling Sibling Perception Questionnaire LA - English M3 - Article N1 - L623347258 2018-08-08 2018-09-21 PY - 2018 SN - 1747-0803 1747-079X SP - 528-532 ST - Perception scores of siblings and parents of children with hypoplastic left heart syndrome T2 - Congenital Heart Disease TI - Perception scores of siblings and parents of children with hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L623347258 http://dx.doi.org/10.1111/chd.12619 VL - 13 ID - 675 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. © 2018 Wiley Periodicals, Inc. AD - Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, United States Nationwide Children's Hospital, Columbus, OH, United States Children's National Medical Center, Washington, Washington, DC, United States 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. DB - Scopus DO - 10.1111/chd.12619 IS - 4 KW - congenital heart disease hypoplastic left heart syndrome siblings survey M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2018 SP - 528-532 ST - Perception scores of siblings and parents of children with hypoplastic left heart syndrome T2 - Congenital Heart Disease TI - Perception scores of siblings and parents of children with hypoplastic left heart syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85050948704&doi=10.1111%2fchd.12619&partnerID=40&md5=a5b71b970c065b34275878fc27fdcc26 VL - 13 ID - 1793 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. Copyright © Cambridge University Press 2010. AD - F. A. Casey, Department of Paediatric Cardiology, Royal Belfast Hospital for Sick Children, 180 Falls Road, Belfast, BT12 6BE, United Kingdom 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 - Embase Medline DO - 10.1017/S1047951110000673 IS - 5 KW - article child congenital heart disease consultation controlled study disease classification disease severity exercise tolerance Fontan procedure general practitioner health behavior health care need health status heart catheterization heart surgery human major clinical study maternal behavior medical care parental behavior physical activity social psychology symptom LA - English M3 - Article N1 - L50935986 2010-06-10 2010-12-22 PY - 2010 SN - 1047-9511 1467-1107 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://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50935986 http://dx.doi.org/10.1017/S1047951110000673 VL - 20 ID - 1134 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. Copyright © Cambridge University Press 2010. AD - Department of Paediatric Cardiology, Royal Belfast Hospital for Sick Children, 180 Falls Road, Belfast, BT12 6BE, United Kingdom Queens University of Belfast, University Road, Belfast, United Kingdom 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 - Scopus DO - 10.1017/S1047951110000673 IS - 5 KW - activity outcomes Paediatric cardiology psychological well-being M3 - Article N1 - Cited By :12 Export Date: 15 June 2020 PY - 2010 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-78649959112&doi=10.1017%2fS1047951110000673&partnerID=40&md5=30cb1ff161ddefa2661c158423ec4073 VL - 20 ID - 2144 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. (PsycINFO Database Record (c) 2019 APA, all rights reserved) AD - Cassidy, Adam R., Department of Psychiatry, Boston Children’s Hospital, Harvard Medical School, Boston, MA, US, 02115 AN - 2018-66711-001 AU - Cassidy, Adam R. AU - Bernstein, Jane Holmes AU - Bellinger, David C. AU - Newburger, Jane W. AU - DeMaso, David R. DB - psyh DO - 10.1080/13854046.2018.1503333 DP - EBSCOhost IS - 4 KW - ADHD anxiety cardiac cognitive style pediatric Adolescent Psychopathology Heart Disorders Risk Factors Visuospatial Ability Affective Disorders Anxiety Disorders Attention Deficit Disorder with Hyperactivity N1 - Department of Psychiatry, Boston Children’s Hospital, Harvard Medical School, Boston, MA, US. Release Date: 20181227. Correction Date: 20190613. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Adolescent Psychopathology; Heart Disorders; Risk Factors; Visuospatial Ability. Minor Descriptor: Affective Disorders; Anxiety Disorders; Attention Deficit Disorder with Hyperactivity. Classification: Cardiovascular Disorders (3295). Population: Human (10); Male (30); Female (40). Location: US. Age Group: Adolescence (13-17 yrs) (200). Tests & Measures: Schedule for Affective Disorders and Schizophrenia for School-Aged Children--Present & Lifetime Version; Rey-Osterrieth Complex Figure--Developmental Scoring System; Wechsler Intelligence Scale for Children, Fourth Edition DOI: 10.1037/t15174-000; Wechsler Adult Intelligence Scale--Third Edition DOI: 10.1037/t49755-000. Methodology: Empirical Study; Interview; Quantitative Study. Page Count: 19. Issue Publication Date: May, 2019. Publication History: Accepted Date: Jul 13, 2018; First Submitted Date: Apr 5, 2018. Copyright Statement: Informa UK Limited, trading as Taylor & Francis Group. 2018. Sponsor: National Heart, Lung, and Blood Institute, US. Grant: HL77681; HL74734; HL096825. Recipients: No recipient indicated Sponsor: Farb Family Fund. Recipients: No recipient indicated Sponsor: National Center for Research Resources. Grant: RR02172. Recipients: No recipient indicated PY - 2019 SN - 1385-4046 1744-4144 SP - 760-778 ST - Visual-spatial processing style is associated with psychopathology in adolescents with critical congenital heart disease T2 - The Clinical Neuropsychologist TI - Visual-spatial processing style is associated with psychopathology in adolescents with critical congenital heart disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2018-66711-001&site=ehost-live&scope=site adam.cassidy@childrens.harvard.edu VL - 33 ID - 1657 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. © 2018, © 2018 Informa UK Limited, trading as Taylor & Francis Group. AD - Department of Psychiatry, Boston Children’s Hospital, Harvard Medical School, Boston, MA, United States Department of Neurology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, United States Department of Cardiology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, United States AU - Cassidy, A. R. AU - Bernstein, J. H. AU - Bellinger, D. C. AU - Newburger, J. W. AU - DeMaso, D. R. DB - Scopus DO - 10.1080/13854046.2018.1503333 IS - 4 KW - ADHD anxiety cardiac cognitive style pediatric M3 - Article N1 - Export Date: 15 June 2020 PY - 2019 SP - 760-778 ST - Visual-spatial processing style is associated with psychopathology in adolescents with critical congenital heart disease T2 - Clinical Neuropsychologist TI - Visual-spatial processing style is associated with psychopathology in adolescents with critical congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85059080288&doi=10.1080%2f13854046.2018.1503333&partnerID=40&md5=83dca9f567d850abb42925cd383179d3 VL - 33 ID - 1748 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. AU - Cassidy, A. R. AU - White, M. T. AU - DeMaso, D. R. AU - Newburger, J. W. AU - Bellinger, D. C. DB - Medline DO - 10.1017/S1355617714001027 IS - 1 KW - adolescent analysis of variance child cognitive defect complication congenital heart malformation depth perception executive function female human male neuropsychological test photostimulation physiology questionnaire young adult LA - English M3 - Article N1 - L615304668 2017-04-18 PY - 2015 SN - 1469-7661 SP - 34-49 ST - Executive Function in Children and Adolescents with Critical Cyanotic Congenital Heart Disease T2 - Journal of the International Neuropsychological Society : JINS TI - Executive Function in Children and Adolescents with Critical Cyanotic Congenital Heart Disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L615304668 http://dx.doi.org/10.1017/S1355617714001027 VL - 21 ID - 900 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 - A.M. Cedars, Department of Cardiology, Baylor University Hospital, Dallas, TX, United States 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. DB - Embase Medline DO - 10.1016/j.amjcard.2016.02.024 IS - 9 KW - ambrisentan abdominal pain adult arthralgia article cardiopulmonary exercise test clinical article controlled study coughing crossover procedure diarrhea double blind procedure drug antagonism drug effect drug efficacy drug tolerability drug withdrawal dyspnea edema exercise fatigue female Fontan procedure human kidney function liver function lung gas exchange male nausea oxygen consumption pharyngitis postoperative period priority journal quality of life randomized controlled trial rash rhinitis Short Form 36 thorax pain treatment duration young adult LA - English M3 - Article N1 - L609880209 2016-04-20 2016-05-05 PY - 2016 SN - 1879-1913 0002-9149 SP - 1524-1532 ST - Effect of Ambrisentan on Exercise Capacity in Adult Patients after the Fontan Procedure T2 - American Journal of Cardiology TI - Effect of Ambrisentan on Exercise Capacity in Adult Patients after the Fontan Procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L609880209 http://dx.doi.org/10.1016/j.amjcard.2016.02.024 VL - 117 ID - 811 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 Department of Cardiology, Washington University School of Medicine, St. Louis, Missouri AN - 117407574. Language: English. Entry Date: 20160824. Revision Date: 20190718. Publication Type: journal article AU - Cedars, Ari M. AU - Saef, Joshua AU - Peterson, Linda R. AU - Coggan, Andrew R. AU - Novak, Eric L. AU - Kemp, Debra AU - Ludbrook, Philip A. DB - ccm DO - 10.1016/j.amjcard.2016.02.024 DP - EBSCOhost IS - 9 KW - Cardiopulmonary Bypass Phenylpropionates -- Therapeutic Use Heterocyclic Compounds -- Therapeutic Use Heart Defects, Congenital -- Surgery Heart Defects, Congenital -- Physiopathology Exercise Tolerance -- Physiology Female Adult Double-Blind Studies Treatment Outcomes Heart Defects, Congenital -- Drug Therapy Human Crossover Design Quality of Life Male Oxygen Consumption -- Physiology Young Adult Adolescence Validation Studies Comparative Studies Evaluation Research Multicenter Studies Randomized Controlled Trials Short Form-36 Health Survey (SF-36) Questionnaires N1 - research; randomized controlled trial. Journal Subset: Biomedical; Peer Reviewed; USA. Special Interest: Evidence-Based Practice. Instrumentation: Short Form-36 Health Survey (SF-36). NLM UID: 0207277. PMID: NLM27063478. PY - 2016 SN - 0002-9149 SP - 1524-1532 ST - Effect of Ambrisentan on Exercise Capacity in Adult Patients After the Fontan Procedure T2 - American Journal of Cardiology TI - Effect of Ambrisentan on Exercise Capacity in Adult Patients After the Fontan Procedure UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=117407574&site=ehost-live&scope=site VL - 117 ID - 1508 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. © 2016 Elsevier Inc. All rights reserved. AD - Department of Cardiology, Baylor University Hospital, Dallas, TX, United States Department of Cardiology, Washington University, School of Medicine, St. Louis, MO, United States 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. DB - Scopus DO - 10.1016/j.amjcard.2016.02.024 IS - 9 M3 - Article N1 - Cited By :11 Export Date: 15 June 2020 PY - 2016 SP - 1524-1532 ST - Effect of Ambrisentan on Exercise Capacity in Adult Patients after the Fontan Procedure T2 - American Journal of Cardiology TI - Effect of Ambrisentan on Exercise Capacity in Adult Patients after the Fontan Procedure UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84963611446&doi=10.1016%2fj.amjcard.2016.02.024&partnerID=40&md5=73d5d22314389c1289dc45efafa6cfc2 VL - 117 ID - 1907 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 Department of Anaesthesiology, Faculty of Medicine, University of Malaya, 50603 Lembah Pantai, Kuala Lumpur Malaysia Department of Orthopaedic Surgery, Faculty of Medicine, University of Malaya, 50603, Lembah Pantai, Kuala Lumpur, Malaysia AN - 115775118. Language: English. Entry Date: 20171129. Revision Date: 20180403. Publication Type: journal article AU - Chan, Chris AU - Lim, Chiao AU - Shahnaz Hasan, M. AU - Kwan, Mun AU - Chan, Chris Yin Wei AU - Lim, Chiao Yee AU - Kwan, Mun Keong DB - ccm DO - 10.1007/s00586-016-4538-9 DP - EBSCOhost KW - Scoliosis -- Surgery Spinal Fusion -- Methods Traction -- Methods Cardiopulmonary Bypass -- Adverse Effects Risk Factors Adolescence Pedicle Screws Male Kyphosis -- Surgery Kyphosis -- Pathology Treatment Outcomes Scoliosis -- Pathology Postoperative Period Preoperative Care -- Methods Spinal Fusion -- Adverse Effects N1 - case study; review. Supplement Title: May2016 Supplement. Journal Subset: Biomedical; Continental Europe; Europe; Peer Reviewed. NLM UID: 9301980. PMID: NLM27028615. PY - 2016 SN - 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=115775118&site=ehost-live&scope=site VL - 25 ID - 1604 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. © 2016, Springer-Verlag Berlin Heidelberg. AD - Department of Orthopaedic Surgery, Faculty of Medicine, University of Malaya, Lembah Pantai, Kuala Lumpur, 50603, Malaysia Department of Anaesthesiology, Faculty of Medicine, University of Malaya, Lembah Pantai, Kuala Lumpur, 50603, Malaysia AU - Chan, C. Y. W. AU - Lim, C. Y. AU - Shahnaz Hasan, M. AU - Kwan, M. K. DB - Scopus DO - 10.1007/s00586-016-4538-9 KW - Fontan Circulation Halo Traction Scoliosis M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2016 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84962284202&doi=10.1007%2fs00586-016-4538-9&partnerID=40&md5=fd11044fa2a481830fc9b49ab9494012 VL - 25 ID - 1911 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. © 2019 The Authors AD - Department of Pediatrics and Adolescent Medicine, American University of Beirut Medical Center, 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 - Scopus DO - 10.1016/j.jsha.2019.04.001 IS - 3 KW - Congenital heart disease Fetal echocardiography Prenatal diagnosis M3 - Article N1 - Export Date: 15 June 2020 PY - 2019 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85065104214&doi=10.1016%2fj.jsha.2019.04.001&partnerID=40&md5=2d05b0a3deed8ed426cb3236b1a91abe VL - 31 ID - 1744 ER - TY - JOUR AB - 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. 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. 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 dependant, 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. 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. Copyright © 2005 by The American Association for Thoracic Surgery. AD - Department of Paediatric Cardiology, Freeman Hospital, Newcastle upon Tyne, United Kingdom Dept. of Paediatric Anaesthesiology, Freeman Hospital, Newcastle upon Tyne, United Kingdom Dept. Paediatr. Cardio-Thorac. A., Freeman Hospital, Newcastle upon Tyne, United Kingdom Transplant Coordinator, Dept. of Cardio-Thorac. Transplant., Freeman Hospital, Newcastle upon Tyne, United Kingdom Dept. of Cardiac Transplantation, Freeman Hospital, Newcastle upon Tyne, United Kingdom Dept. of Paediatric Cardiac Surgery, Freeman Hospital, Newcastle upon Tyne, United Kingdom Freeman Hospital, Newcastle upon Tyne, NE 7 7DN, United Kingdom 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. DB - Scopus DO - 10.1016/j.jtcvs.2004.06.030 IS - 2 M3 - Article N1 - Cited By :20 Export Date: 15 June 2020 PY - 2005 SP - 416-422 ST - Rescue cardiac transplantation for early failure of the Fontan-type circulation in children T2 - Journal of Thoracic and Cardiovascular Surgery TI - Rescue cardiac transplantation for early failure of the Fontan-type circulation in children UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-19944431489&doi=10.1016%2fj.jtcvs.2004.06.030&partnerID=40&md5=ae778e4157465c4b07e3e5086de6dedb VL - 129 ID - 2245 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 - S.L. Cheatham, The Heart Center, Nationwide Children’s Hospital, 700 Children’s Drive, Columbus, OH, United States AU - Cheatham, S. L. AU - Carey, H. AU - Chisolm, J. L. AU - Heathcock, J. C. AU - Steward, D. DB - Embase DO - 10.1007/s00246-014-1065-5 IS - 3 KW - tissue inhibitor of metalloproteinase article Bayley Scales of Infant Development clinical article cognitive development controlled study follow up heart arrest heart catheterization human hybrid stage I palliation hypoplastic left heart syndrome infant language mental development assessment motor performance nerve cell differentiation outcome assessment palliative therapy treatment outcome LA - English M3 - Article N1 - L600408592 2014-11-19 2015-06-29 PY - 2015 SN - 1432-1971 0172-0643 SP - 685-691 ST - Early Results of Neurodevelopment Following Hybrid Stage I for Hypoplastic Left Heart Syndrome T2 - Pediatric Cardiology TI - Early Results of Neurodevelopment Following Hybrid Stage I for Hypoplastic Left Heart Syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L600408592 http://dx.doi.org/10.1007/s00246-014-1065-5 VL - 36 ID - 879 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. © 2014, Springer Science+Business Media New York. AD - The Heart Center, Nationwide Children’s Hospital, 700 Children’s Drive, Columbus, OH 43205, United States Clinical Therapies Department, Nationwide Children’s Hospital, 700 Children’s Drive, Columbus, OH 43205, United States Center for Perinatal Research, Nationwide Children’s Hospital, 700 Children’s Drive, Columbus, OH 43205, United States College of Nursing, The Ohio State University, 362 Newton Hall, 1585 Neil Ave, Columbus, OH 43210, United States School of Health and Rehabilitation Services, The Ohio State University, Columbus, OH, United States AU - Cheatham, S. L. AU - Carey, H. AU - Chisolm, J. L. AU - Heathcock, J. C. AU - Steward, D. DB - Scopus DO - 10.1007/s00246-014-1065-5 IS - 3 KW - Congenital heart disease Hybrid Hypoplastic left heart syndrome Neurodevelopment M3 - Article N1 - Cited By :13 Export Date: 15 June 2020 PY - 2015 SP - 685-691 ST - Early Results of Neurodevelopment Following Hybrid Stage I for Hypoplastic Left Heart Syndrome T2 - Pediatric Cardiology TI - Early Results of Neurodevelopment Following Hybrid Stage I for Hypoplastic Left Heart Syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84925481650&doi=10.1007%2fs00246-014-1065-5&partnerID=40&md5=6ae2ede9120d67ed9ef14f40e9878418 VL - 36 ID - 1954 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. Copyright © 2020. Published by Elsevier Inc. AD - Cardiothoracic Surgery, Clinical and Translational Research Program, Stanford University, Palo Alto, CA Health Services Research, Mayo Clinic, MN, Rochester Center for Devices and Radiological Health, United States Food and Drug Administration, MD, Silver Spring Pediatrics - Endocrinology, Stanford University, Palo Alto, CA Knowledge and Evaluation Research Unit, Mayo Clinic, MN, Rochester Pediatric Cardiology, Lucile Packard Children's Hospital Stanford, Palo Alto, CA Pediatric Cardiology, Stanford University, Palo Alto, CA Pediatric Cardiology, Mayo Clinic, MN, Rochester 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 - Scopus DO - 10.1016/j.healun.2020.01.280 IS - 4 M3 - Article N1 - Export Date: 15 June 2020 PY - 2020 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85085634838&doi=10.1016%2fj.healun.2020.01.280&partnerID=40&md5=b227a7bbf2acc98015ba24fc2e62203d VL - 39 ID - 1705 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 - A.N. Redington, Division of Cardiology, Hospital for Sick Children, 555 University Avenue, Toronto, Ont. M5G 1X8, Canada AU - Cheung, M. M. H. AU - Smallhorn, J. F. AU - McCrindle, B. W. AU - Van Arsdell, G. S. AU - Redington, A. N. DB - Embase Medline DO - 10.1136/hrt.2004.048207 IS - 10 KW - article child clinical article congenital heart disease controlled study Doppler echocardiography heart atrium pacing cardiac index heart left ventricle heart muscle contractile force heart muscle contractility heart performance heart rate heart right ventricle heart single ventricle human priority journal LA - English M3 - Article N1 - L41363703 2005-10-25 PY - 2005 SN - 1355-6037 SP - 1338-1342 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L41363703 http://dx.doi.org/10.1136/hrt.2004.048207 VL - 91 ID - 1272 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, 555 University Avenue, Toronto, Ont. M5G 1X8, Canada Division of Cardiology, Hospital for Sick Children, Toronto, Ont., Canada AU - Cheung, M. M. H. AU - Smallhorn, J. F. AU - McCrindle, B. W. AU - Van Arsdell, G. S. AU - Redington, A. N. DB - Scopus DO - 10.1136/hrt.2004.048207 IS - 10 M3 - Article N1 - Cited By :35 Export Date: 15 June 2020 PY - 2005 SP - 1338-1342 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-25144457100&doi=10.1136%2fhrt.2004.048207&partnerID=40&md5=b62b5895a210efd526f33bc2446ebb62 VL - 91 ID - 2236 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 Divisions of Cardiology and Cardiovascular Surgery, Hospital for Sick Children, Toronto, Ontario, Canada AN - 106219933. Language: English. Entry Date: 20070119. Revision Date: 20170413. Publication Type: journal article AU - Cheung, M. M. H. AU - Smallhorn, J. F. AU - McCrindle, B. W. AU - Van Arsdell, G. S. AU - Redington, A. N. AU - Cheung, M. M. H. AU - Smallhorn, J. F. AU - McCrindle, B. W. AU - Van Arsdell, G. S. AU - Redington, A. N. DB - ccm DP - EBSCOhost IS - 10 KW - Echocardiography, Doppler -- Utilization Heart Defects, Congenital -- Physiopathology Adolescence Cardiac Pacing, Artificial Child Data Analysis Software Descriptive Statistics Funding Source Heart Defects, Congenital -- Diagnosis Heart Defects, Congenital -- Therapy Heart Defects, Congenital -- Ultrasonography Heart Rate -- Physiology Heart Ventricle -- Abnormalities Heart Ventricle -- Physiopathology Myocardial Contraction -- Physiology Pacemaker, Artificial Pearson's Correlation Coefficient Post Hoc Analysis Prospective Studies Stroke Volume T-Tests Human N1 - research; tables/charts. Journal Subset: Biomedical; Blind Peer Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Grant Information: Unrestricted educational grant, GE Vingmed Ultrasound, Horten, Norway; Heart and Stroke Foundation of Canada; Research Fellowship, Heart and Stroke Foundation of Ontario. NLM UID: 9602087. PMID: NLM16162630. PY - 2005 SN - 1355-6037 SP - 1338-1342 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106219933&site=ehost-live&scope=site VL - 91 ID - 1626 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 - Department of Pediatrics, Faculty of Medicine, Naresuan University, Phitsanulok, 65000, Thailand Department of Surgery, Faculty of Medicine, Naresuan University, Phitsanulok, 65000, Thailand AU - Cholkraisuwat, E. AU - Buddharaksa, Y. AU - Sayasathid, J. DB - Scopus DO - 10.5372/1905-7415.0702.178 IS - 2 KW - Congenital heart disease screening in children Prevalence Thailand M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2013 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84879953538&doi=10.5372%2f1905-7415.0702.178&partnerID=40&md5=4849d79296000ebe5ee86a0edd71543f VL - 7 ID - 2053 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. © 2005 by The Society of Thoracic Surgeons. AD - U.K. Chowdhury, Department of Cardiothoracic and Vascular Surgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi-110029, India 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. DB - Embase Medline DO - 10.1016/j.athoracsur.2005.02.024 IS - 2 KW - politef radioisotope tracer adolescent adult article child controlled study death echocardiography follow up Fontan procedure growth heart catheterization heart ejection fraction heart supraventricular arrhythmia heart ventricle function heterotaxy syndrome human lung major clinical study mortality pericardium perioperative period postoperative complication postoperative period priority journal risk factor surgical mortality surgical technique survival thromboembolism thrombosis treatment failure LA - English M3 - Article N1 - L41019498 2005-08-15 PY - 2005 SN - 0003-4975 SP - 665-672 ST - Specific issues after extracardiac fontan operation: Ventricular function, growth potential, arrhythmia, and thromboembolism T2 - Annals of Thoracic Surgery TI - Specific issues after extracardiac fontan operation: Ventricular function, growth potential, arrhythmia, and thromboembolism UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L41019498 http://dx.doi.org/10.1016/j.athoracsur.2005.02.024 VL - 80 ID - 1273 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. © 2005 by The Society of Thoracic Surgeons. AD - Depts. of Cardiothoracic and Vasc. Surgery, Cardiology, Biostatistics, and Cardiac-Nuclear Medicine, Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India Department of Cardiothoracic and Vascular Surgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi-110029, India 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. DB - Scopus DO - 10.1016/j.athoracsur.2005.02.024 IS - 2 M3 - Article N1 - Cited By :38 Export Date: 15 June 2020 PY - 2005 SP - 665-672 ST - Specific issues after extracardiac fontan operation: Ventricular function, growth potential, arrhythmia, and thromboembolism T2 - Annals of Thoracic Surgery TI - Specific issues after extracardiac fontan operation: Ventricular function, growth potential, arrhythmia, and thromboembolism UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-22544448940&doi=10.1016%2fj.athoracsur.2005.02.024&partnerID=40&md5=767749fd8aba1c3b51de241ad2620c60 VL - 80 ID - 2237 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. AN - 109646212. Language: English. Entry Date: 20150923. Revision Date: 20160725. Publication Type: journal article AU - Clauss, Sarah B. AU - Anderson, Jeffrey B. AU - Lannon, Carole AU - Lihn, Stacey AU - Beekman, Robert H. AU - Kugler, John D. AU - Martin, Gerard R. DB - ccm DO - 10.1097/MOP.0000000000000263 DP - EBSCOhost IS - 5 N1 - review. Journal Subset: Biomedical; Peer Reviewed; USA. Special Interest: Pediatric Care. NLM UID: 9000850. PMID: NLM26208236. PY - 2015 SN - 1040-8703 SP - 555-562 ST - Quality improvement through collaboration: the National Pediatric Quality improvement Collaborative initiative T2 - Current Opinion in Pediatrics TI - Quality improvement through collaboration: the National Pediatric Quality improvement Collaborative initiative UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=109646212&site=ehost-live&scope=site VL - 27 ID - 1576 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. © 2018, Springer Science+Business Media, LLC, part of Springer Nature. AD - Division of Pediatric Cardiovascular Surgery, University of Alabama at Birmingham, Birmingham, AL, United States Xenotransplantation Program, Department of Surgery, University of Alabama at Birmingham, Birmingham, AL, United States Section of Pediatric Cardiology, 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 - Scopus DO - 10.1007/s00246-018-1998-1 IS - 2 KW - Congenital heart disease Genetically-engineered Immunological tolerance Pig Staged surgical palliation Xenotransplantation M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2019 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85055022632&doi=10.1007%2fs00246-018-1998-1&partnerID=40&md5=72f88cb924063ff324d9038ba6488583 VL - 40 ID - 1760 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 - 126782014. Language: English. Entry Date: 20180802. Revision Date: 20180802. Publication Type: journal article AU - Cohen, Meryl S. DB - ccm DO - 10.1097/HCO.0000000000000466 DP - EBSCOhost IS - 1 KW - Heart Defects, Congenital -- Therapy Heart Ventricle -- Surgery Heart Defects, Congenital -- Physiopathology Aorta, Thoracic -- Abnormalities Heart Valve Diseases Ventricular Outflow Obstruction Ebstein's Anomaly -- Physiopathology Ventricular Outflow Obstruction -- Therapy Pulmonary Atresia -- Therapy Ebstein's Anomaly -- Therapy Pulmonary Atresia Ebstein's Anomaly Ventricular Outflow Obstruction -- Physiopathology Heart Septal Defects -- Physiopathology Aorta, Thoracic Heart Valve Diseases -- Physiopathology Heart Ventricle -- Physiopathology Heart Ventricle -- Abnormalities Pulmonary Atresia -- Physiopathology Aorta, Thoracic -- Physiopathology Infant, Newborn Heart Valve Diseases -- Therapy Mitral Valve Aortic Valve Stenosis -- Therapy Aortic Valve Stenosis Heart Septal Defects -- Therapy Heart Septal Defects Infant Aortic Valve Stenosis -- Physiopathology Heart Ventricle Cardiopulmonary Bypass Heart Defects, Congenital Exercise of Self-Care Agency Scale Ferrans and Powers Quality of Life Index Questionnaires N1 - review. Journal Subset: Biomedical; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. Instrumentation: Exercise of Self-Care Agency Scale (ESCA) (Kearney and Fleischer); Infant Characteristics Questionnaire (ICQ) (Bates et al); Behavioral Inattention Test (BIT); General Health Questionnaire (GHQ); Longitudinal Interval Follow-Up Evaluation (LIFE); Ferrans and Powers Quality of Life Index. NLM UID: 8608087. PMID: NLM29084001. 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 - Current Opinion in Cardiology TI - Assessing the borderline ventricle in a term infant: combining imaging and physiology to establish the right course UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=126782014&site=ehost-live&scope=site VL - 33 ID - 1541 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. © 2010 Mosby, Inc. AD - M. S. Cohen, Division of Cardiology, Children's Hospital of Philadelphia, 34th Street and Civic Center Blvd., Philadelphia, PA 19104, United States 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. DB - Embase Medline DO - 10.1016/j.ahj.2010.07.039 IS - 6 KW - NCT00132782 brain natriuretic peptide adolescent adult article body height body mass child cohort analysis congenital heart disease echocardiography exercise test Fontan procedure functional assessment health status heart valve regurgitation heart ventricle hypertrophy heart ventricle volume hormone blood level human major clinical study nuclear magnetic resonance imaging postoperative period preschool child priority journal questionnaire school child scoring system short stature treatment outcome LA - English M3 - Article N1 - L360154212 2010-12-22 2010-12-30 PY - 2010 SN - 0002-8703 1097-6744 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://www.embase.com/search/results?subaction=viewrecord&from=export&id=L360154212 http://dx.doi.org/10.1016/j.ahj.2010.07.039 VL - 160 ID - 1128 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. © 2010 Mosby, Inc. AD - Division of Cardiology, Children's Hospital of Philadelphia, 34th Street and Civic Center Blvd., Philadelphia, PA 19104, United States New England Research Institutes, Watertown, MA, United States Medical University of South Carolina, Charleston, SC, United States Columbia University College of Physicians and Surgeons, New York, NY, United States University of Utah, Salt Lake City, UT, United States National Heart, Lung and Blood Institute, Bethesda, MD, United States Duke University School of Medicine, Durham, NC, United States Boston Children's Hospital, Boston, MA, United States Hospital for Sick Children, Toronto, ON, Canada 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. DB - Scopus DO - 10.1016/j.ahj.2010.07.039 IS - 6 M3 - Article N1 - Cited By :30 Export Date: 15 June 2020 PY - 2010 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-78650107133&doi=10.1016%2fj.ahj.2010.07.039&partnerID=40&md5=3f0516c2795b77c62933d32980faa71e VL - 160 ID - 2161 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, Greece AU - Cokkinos, D. V. AU - Plessas, S. T. AU - Tolis, G. AU - Voridis, E. M. DB - Scopus IS - 2 M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 1974 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0016169193&partnerID=40&md5=eb03da415cb40f0fbb370dfa172aa5a6 VL - 68 ID - 2336 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. © 2014 Colaneri et al.; licensee BioMed Central Ltd. AD - F.M. De Benedictis, Department of Mother and Child Health, Salesi Children's Hospital, Azienda Ospedaliero-Universitaria Ospedali Riuniti, Ancona, Italy AU - Colaneri, M. AU - Quarti, A. AU - Pozzi, M. AU - Gasparini, S. AU - Carloni, I. AU - De Benedictis, F. M. C1 - actilyse(Boehringer Ingelheim) C2 - Boehringer Ingelheim DB - Embase Medline DO - 10.1186/1824-7288-40-18 IS - 1 KW - acetylcysteine epinephrine alteplase antiasthmatic agent antibiotic agent budesonide deoxyribonuclease dopamine enoximone fibrin salbutamol sildenafil sodium chloride abnormal respiratory sound absence of side effects adolescent article artificial ventilation asplenia atelectasis atrial isomerism atrioventricular block breathing exercise bronchitis bronchus secretion case report chronic lung disease chronic oto sino pulmonary disease ciliary dyskinesia congenital heart disease continuous hemofiltration coughing croupous bronchitis deterioration drug efficacy lack of drug effect dyspnea echocardiography electron microscopy fever fiberoptic bronchoscopy fibrinolytic therapy follow up Fontan procedure forward heart failure Glenn shunt great vessels transposition heart catheterization heart dilatation heart disease heart ejection fraction heart right ventricle double outlet heart ventricle septum defect histopathology hospital admission hospital discharge human human tissue inferior cava vein inflammatory cell intensive care unit lung auscultation lung resistance male mechanical chest compressor medical history multiple organ failure nebulization nebulizer oxygen mask physical examination pleura effusion postoperative complication pulmonary artery stenosis recurrent disease repeated drug dose respiratory distress superior cava vein thorax pain thorax radiography tracheobronchial tree ultrafiltration wheezing actilyse Pari LC Sprint Pari TurboBoy S compressor LA - English M3 - Article N1 - L372798968 2014-04-18 2014-04-28 PY - 2014 SN - 1824-7288 1720-8424 ST - Management of plastic bronchitis with nebulized tissue plasminogen activator: Another brick in the wall T2 - Italian Journal of Pediatrics TI - Management of plastic bronchitis with nebulized tissue plasminogen activator: Another brick in the wall UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L372798968 http://dx.doi.org/10.1186/1824-7288-40-18 VL - 40 ID - 919 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 Cardiovascular Medicine, Azienda Ospedaliero-Universitaria Ospedali Riuniti, Ancona, Italy Department of Internal Medicine, Azienda Ospedaliero-Universitaria Ospedali Riuniti, Ancona, Italy Department of Mother and Child Health, Salesi Children's Hospital, Azienda Ospedaliero-Universitaria Ospedali Riuniti, Ancona, Italy AN - 104030427. Language: English. Entry Date: 20140221. Revision Date: 20150710. Publication Type: Journal Article AU - Colaneri, Massimo AU - Quarti, Andrea AU - Pozzi, Marco AU - Gasparini, Stefano AU - Carloni, Ines AU - de Benedictis, Fernando Maria DB - ccm DO - 10.1186/1824-7288-40-18 DP - EBSCOhost IS - 1 KW - Tissue Plasminogen Activator -- Administration and Dosage Bronchitis -- Drug Therapy Ciliary Motility Disorders -- Prevention and Control Male Adolescence Radiography, Thoracic Bronchoscopy N1 - case study. Journal Subset: Biomedical; Continental Europe; Editorial Board Reviewed; Europe; Expert Peer Reviewed. Special Interest: Pediatric Care. NLM UID: 101510759. PMID: NLM24524376. PY - 2014 SN - 1720-8424 SP - 1-8 ST - Management of plastic bronchitis with nebulized tissue plasminogen activator: another brick in the wall T2 - Italian Journal of Pediatrics TI - Management of plastic bronchitis with nebulized tissue plasminogen activator: another brick in the wall UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104030427&site=ehost-live&scope=site VL - 40 ID - 1536 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 Cardiovascular Medicine, Azienda Ospedaliero-Universitaria Ospedali Riuniti, Ancona, Italy Department of Internal Medicine, Azienda Ospedaliero-Universitaria Ospedali Riuniti, Ancona, Italy Department of Mother and Child Health, Salesi Children's Hospital, Azienda Ospedaliero-Universitaria Ospedali Riuniti, Ancona, Italy AN - 94466133. Language: English. Entry Date: 20140221. Revision Date: 20190313. Publication Type: Article AU - Colaneri, Massimo AU - Quarti, Andrea AU - Pozzi, Marco AU - Gasparini, Stefano AU - Carloni, Ines AU - de Benedictis, Fernando Maria DB - ccm DO - 10.1186/1824-7288-40-18 DP - EBSCOhost IS - 1 KW - Tissue Plasminogen Activator -- Administration and Dosage Bronchitis -- Drug Therapy Ciliary Motility Disorders -- Prevention and Control Male Adolescence Radiography, Thoracic Bronchoscopy N1 - case study. Journal Subset: Biomedical; Continental Europe; Editorial Board Reviewed; Europe; Expert Peer Reviewed. Special Interest: Pediatric Care. NLM UID: 101510759. PY - 2014 SN - 1720-8424 SP - 1-8 ST - Management of plastic bronchitis with nebulized tissue plasminogen activator: another brick in the wall T2 - Italian Journal of Pediatrics TI - Management of plastic bronchitis with nebulized tissue plasminogen activator: another brick in the wall UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=94466133&site=ehost-live&scope=site VL - 40 ID - 1537 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. © 2014 Colaneri et al.; licensee BioMed Central Ltd. AD - Department of Cardiovascular Medicine, Azienda Ospedaliero-Universitaria Ospedali Riuniti, Ancona, Italy Department of Internal Medicine, Azienda Ospedaliero-Universitaria Ospedali Riuniti, Ancona, Italy Department of Mother and Child Health, Salesi Children's Hospital, Azienda Ospedaliero-Universitaria Ospedali Riuniti, Ancona, Italy AU - Colaneri, M. AU - Quarti, A. AU - Pozzi, M. AU - Gasparini, S. AU - Carloni, I. AU - De Benedictis, F. M. C7 - 18 DB - Scopus DO - 10.1186/1824-7288-40-18 IS - 1 KW - Plastic bronchitis Primary ciliary dyskinesia Tissue plasminogen activator M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2014 ST - Management of plastic bronchitis with nebulized tissue plasminogen activator: Another brick in the wall T2 - Italian Journal of Pediatrics TI - Management of plastic bronchitis with nebulized tissue plasminogen activator: Another brick in the wall UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84897997595&doi=10.1186%2f1824-7288-40-18&partnerID=40&md5=7f3c1fb22d697bba3cb60add5fb8a049 VL - 40 ID - 1994 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 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 - Department of Pediatrics, Division of PediatricCardiology, University of Virginia Children's Hospital, University of Virginia, Charlottesville, VA, USA Division of Pediatric Critical Care, University of Virginia Children's Hospital, University of Virginia, Charlottesville, VA, USA Division of Pediatric Cardiology, University of Virginia Children's Hospital, University of Virginia, Charlottesville, VA, USA AN - 133050073. Language: English. Entry Date: 20190318. Revision Date: 20191202. Publication Type: journal article AU - Colombo, Jamie N. AU - Spaeder, Michael C. AU - Hainstock, Michael R. DB - ccm DO - 10.1017/S1047951118001567 DP - EBSCOhost IS - 12 KW - Heart Ventricle -- Abnormalities Heart Catheterization -- Statistics and Numerical Data Readmission -- Statistics and Numerical Data Heart Defects, Congenital -- Surgery Male Resource Databases Treatment Outcomes Retrospective Design Outpatients Female Infant Anastomosis, Surgical Length of Stay Risk Factors Human N1 - research; tables/charts. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. NLM UID: 9200019. PMID: NLM30309401. PY - 2018 SN - 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=133050073&site=ehost-live&scope=site VL - 28 ID - 1457 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. © 2018 Cambridge University Press. AD - Department of Pediatrics, Division of Pediatric Cardiology, University of Virginia Children's Hospital, University of Virginia, Charlottesville, VA, United States Division of Pediatric Critical Care, University of Virginia Children's Hospital, University of Virginia, Charlottesville, VA, United States 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 - Scopus DO - 10.1017/S1047951118001567 IS - 12 KW - cardiac catheterisation CHD hypoplastic left heart syndrome single ventricle M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2018 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85055024272&doi=10.1017%2fS1047951118001567&partnerID=40&md5=1ebb279657d9d162b8c4ae228422a6d6 VL - 28 ID - 1781 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. © 2007 Connor and Thiagarajan; licensee BioMed Central Ltd. AD - Cardiovascular Program, Children's Hospital Boston, United States Department of Cardiology, Division of Cardiovascular Critical Care, Children's Hospital Boston, United States AU - Connor, J. A. AU - Thiagarajan, R. DB - Scopus DO - 10.1186/1750-1172-2-1 IS - 1 M3 - Article N1 - Cited By :17 Export Date: 15 June 2020 PY - 2007 SP - 1-5 ST - Hypoplastic left heart syndrome T2 - Orphanet Journal of Rare Diseases TI - Hypoplastic left heart syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-34249714279&doi=10.1186%2f1750-1172-2-1&partnerID=40&md5=325858ab32847de7ba0016b812d97ffa VL - 2 ID - 2208 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 - T.R. Cooper, Department of Pediatrics, Baylor College of Medicine, Houston, TX 77030, USA. AU - Cooper, T. R. AU - Garcia-Prats, J. A. AU - Brody, B. A. DB - Medline DO - 10.1542/peds.104.4.e48 IS - 4 KW - article cardiology comparative study counseling decision making gastroenterology health personnel attitude heart transplantation human hypoplastic left heart syndrome neonatology newborn palliative therapy prognosis public relations quality of life questionnaire short bowel syndrome total parenteral nutrition LA - English M3 - Article N1 - L129469182 1999-10-13 PY - 1999 SN - 1098-4275 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L129469182 http://dx.doi.org/10.1542/peds.104.4.e48 VL - 104 ID - 1341 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, United States AU - Cooper, T. R. AU - Garcia-Prats, J. A. AU - Brody, B. A. DB - Scopus DO - 10.1542/peds.104.4.e48 IS - 4 M3 - Article N1 - Cited By :10 Export Date: 15 June 2020 PY - 1999 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0033209637&doi=10.1542%2fpeds.104.4.e48&partnerID=40&md5=e80a2b49fa35c18b05e48ed947a8031a VL - 104 ID - 2284 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 - A.F. Corno, Department of Pediatric Cardiology, Ospedale Bambino Gesù, Rome, Italy. AU - Corno, A. F. AU - Parisi, F. AU - Marino, B. AU - Ballerini, L. AU - Marcelletti, C. DB - Medline IS - 3 KW - adolescent article congenital heart malformation evaluation study female follow up human infant male mortality palliative therapy quality of life procedures treatment outcome LA - English M3 - Article N1 - L20817856 1990-04-12 PY - 1987 SN - 1010-7940 SP - 144-147 ST - Palliative Mustard operation: an expanded horizon T2 - European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery TI - Palliative Mustard operation: an expanded horizon UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L20817856 VL - 1 ID - 1388 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%). © 1987. AD - Department of Pediatric Cardiology, Ospedale Bambino Gesù, Rome, Italy AU - Corno, A. F. AU - Parisi, F. AU - Marino, B. AU - Ballerini, L. AU - Marcelletti, C. DB - Scopus DO - 10.1016/1010-7940(87)90030-3 IS - 3 KW - Congenital heart disease Palliative Mustard operation M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 1987 SP - 144-147 ST - Palliative Mustard operation: an expanded horizon T2 - European Journal of Cardio-Thoracic Surgery TI - Palliative Mustard operation: an expanded horizon UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0023459347&doi=10.1016%2f1010-7940%2887%2990030-3&partnerID=40&md5=c5f98895355052958b47ab4b7774ab10 VL - 1 ID - 2325 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. Copyright © 2012 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies. AD - J.M. Costello, Departments of Cardiology, Children's Hospital Boston, Harvard Medical School, Boston, MA, United States 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. DB - Embase Medline DO - 10.1097/PCC.0b013e318238ba21 IS - 4 KW - adolescent adult article cardiac patient child Child Health Questionnaire Parent Form 50 controlled study defibrillator extracorporeal oxygenation female heart disease human intensive care unit length of stay major clinical study male occupational therapy physiotherapy preschool child priority journal psychosocial care quality of life questionnaire school child scoring system special education speech therapy survivor treatment outcome United States LA - English M3 - Article N1 - L51707852 2011-11-11 2012-08-08 PY - 2012 SN - 1529-7535 1947-3893 SP - 428-434 ST - Quality of life of pediatric cardiac patients who previously required extracorporeal membrane oxygenation T2 - Pediatric Critical Care Medicine TI - Quality of life of pediatric cardiac patients who previously required extracorporeal membrane oxygenation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51707852 http://dx.doi.org/10.1097/PCC.0b013e318238ba21 VL - 13 ID - 1053 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 - From the Departments of Cardiology (JMC, MO, DW, JS, JWS, JWN, PCL, RRT), Pediatrics (DW), Anesthesia (JHA), and Cardiac Surgery (FFT), Children's Hospital Boston, Harvard Medical School, Boston, MA; Department of Biostatistics (DW), Harvard School of Public Health, Boston, MA. AN - 108135475. Language: English. Entry Date: 20120907. Revision Date: 20150712. Publication Type: Journal Article AU - Costello, John M. AU - O'Brien, Molly AU - Wypij, David AU - Shubert, Joana AU - Salvin, Joshua W. AU - Newburger, Jane W. AU - Laussen, Peter C. AU - Arnold, John H. AU - Fynn-Thompson, Francis AU - Thiagarajan, Ravi R. DB - ccm DP - EBSCOhost IS - 4 KW - Extracorporeal Membrane Oxygenation -- Evaluation -- In Infancy and Childhood Heart Defects, Congenital -- Therapy -- In Infancy and Childhood Quality of Life -- Evaluation -- In Infancy and Childhood Adolescence Child Child, Preschool Cross Sectional Studies Data Analysis Software Data Analysis, Statistical Descriptive Statistics Fisher's Exact Test Funding Source Human Infant Infant, Newborn Massachusetts P-Value Parents -- Psychosocial Factors Questionnaires T-Tests Wilcoxon Rank Sum Test N1 - research; tables/charts. Commentary: Arrington Amy S, Checchia Paul A. Quality of life in cardiac extracorporeal membrane oxygenation survivors: A leap of faith now gets data. (PEDIATR CRIT CARE MED) 2012 Jul; 13 (4): 488-489. Journal Subset: Biomedical; Peer Reviewed; USA. Special Interest: Critical Care; Pediatric Care. Instrumentation: Child Health Questionnaire Parent Form-50 (CHQ-PF50); Child Health Questionnaire Child Form-87 (CHQ-CF87). Grant Information: Supported, in part, by the Rochelle E. Rose Clinical Research Fund.. NLM UID: 100954653. PMID: NLM22067987. PY - 2012 SN - 1529-7535 SP - 428-434 ST - Quality of life of pediatric cardiac patients who previously required extracorporeal membrane oxygenation T2 - Pediatric Critical Care Medicine TI - Quality of life of pediatric cardiac patients who previously required extracorporeal membrane oxygenation UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=108135475&site=ehost-live&scope=site VL - 13 ID - 1521 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. Copyright © 2012 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies. AD - Departments of Cardiology, Children's Hospital Boston, Harvard Medical School, Boston, MA, United States Departments of Pediatrics, Children's Hospital Boston, Harvard Medical School, Boston, MA, United States Departments of Anesthesia, Children's Hospital Boston, Harvard Medical School, Boston, MA, United States Departments Of, and Cardiac Surgery, Children's Hospital Boston, Harvard Medical School, Boston, MA, United States Department of Biostatistics, Harvard School of Public Health, Boston, MA, United States 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. DB - Scopus DO - 10.1097/PCC.0b013e318238ba21 IS - 4 KW - congenital heart defect extracorporeal membrane oxygenation pediatric quality of life M3 - Article N1 - Cited By :31 Export Date: 15 June 2020 PY - 2012 SP - 428-434 ST - Quality of life of pediatric cardiac patients who previously required extracorporeal membrane oxygenation T2 - Pediatric Critical Care Medicine TI - Quality of life of pediatric cardiac patients who previously required extracorporeal membrane oxygenation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84864198466&doi=10.1097%2fPCC.0b013e318238ba21&partnerID=40&md5=7d29b80ad837a5380ccca66e819dfdfa VL - 13 ID - 2089 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. © 2018 Elsevier B.V. AD - Department of Pediatrics, Division of Pediatric Cardiology, Wayne State University School of Medicine, Children's Hospital of Michigan, 3901 Beaubien St, Detroit, MI 48201, United States Department of Pediatrics, Division of Pediatric Critical Care Medicine, Wayne State University School of Medicine, Children's Hospital of Michigan, 3901 Beaubien St, Detroit, MI 48201, United States Department of Pediatric Cardiac Surgery, Wayne State University School of Medicine, Children's Hospital of Michigan, 3901 Beaubien St, Detroit, MI 48201, United States AU - Covi, S. AU - Clark, J. AU - Delius, R. AU - Chauhan, M. DB - Scopus DO - 10.1016/j.ppedcard.2018.08.001 KW - Acute respiratory distress syndrome Amiodarone Congenital heart disease Pediatrics Toxicity M3 - Article N1 - Export Date: 15 June 2020 PY - 2019 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85051015588&doi=10.1016%2fj.ppedcard.2018.08.001&partnerID=40&md5=3ff0d0058038bc7bd79b6ff6dd2023a7 VL - 52 ID - 1757 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. © 2018 Elsevier Masson SAS AD - Institute of Health & Society, Baddiley-Clark Building, Newcastle UniversityNE2 4AX, United Kingdom School of Geography, Politics and Sociology, 5th Floor Claremont Bridge Building, Newcastle UniversityNE1 7RU, United Kingdom Institute of Cellular Medicine, 3rd Floor William Leech Building, Newcastle UniversityNE2 4HH, United Kingdom AU - Crowe, L. AU - Graham, R. H. AU - Robson, S. C. AU - Rankin, J. DB - Scopus DO - 10.1016/j.ejmg.2018.05.011 IS - 9 KW - Gestational age Health professionals Termination of pregnancy Termination of pregnancy for fetal anomaly M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2018 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85047877795&doi=10.1016%2fj.ejmg.2018.05.011&partnerID=40&md5=c58eae5af712354c610be68356cbb5e0 VL - 61 ID - 1789 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. © The Author(s) 2018. AD - Pediatric Intensive Care, MGH Pediatrics Ethics Committee, Department of Pediatrics, Massachusetts General Hospital, Boston, MA, United States Department of Theology, Boston College, Chestnut Hill, MA, United States AU - Cummings, B. M. AU - Paris, J. J. DB - Scopus DO - 10.1177/0885066618791953 IS - 1 KW - decision-making disagreement end-of-life care ethics consultations pediatric intensive care M3 - Article N1 - Export Date: 15 June 2020 PY - 2019 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85053294230&doi=10.1177%2f0885066618791953&partnerID=40&md5=c2d1d03efa9f616bf894e5e925814a86 VL - 34 ID - 1769 ER - TY - JOUR AB - The authors comment on a study by Goldberg et al that evaluated functional ability behavior and quality of life (QoL) in young school-aged children with hypoplastic left heart syndrome (HLHS). They note that Goldberg showed that HLHS children do have functional impairment that affect QoL and that increased interventions may improve the trajectory of functioning and QoL of these children. Thus they suggest to increase support and prioritize intervention programs to improve QoL in HLHS. AD - Department of Pediatrics, Baylor College of Medicine, Houston, Texas Psychology Service, Texas Children's Hospital, Houston, Texas Pediatric Cardiology, Texas Children's Heart Center, Houston, Texas AN - 139633707. Language: English. Entry Date: 20191115. Revision Date: 20191116. Publication Type: Article AU - Cutitta, Katherine AU - Lantin-Hermoso, M. Regina DB - ccm DO - 10.1542/peds.2019-1664 DP - EBSCOhost IS - 5 KW - Hypoplastic Left Heart Syndrome -- Diagnosis Quality of Life -- Evaluation Child Behavior -- Evaluation Risk Assessment Severity of Illness Caregivers Hypoplastic Left Heart Syndrome -- Therapy Support, Psychosocial Coping Early Diagnosis N1 - commentary. Journal Subset: Biomedical; Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. NLM UID: 0376422. PY - 2019 SN - 0031-4005 SP - 1-2 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=139633707&site=ehost-live&scope=site VL - 144 ID - 1461 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 - R.J. Czosek, Heart Institute, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave MLC, Cincinnati, OH, United States 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. DB - Embase Medline DO - 10.1016/j.hrthm.2015.01.022 IS - 5 KW - adolescent adult article bicuspid aortic valve cardiovascular therapeutic device catheter ablation child childhood disease cohort analysis comparative study complete heart block congenital heart disease cross-sectional study Fallot tetralogy female Fontan procedure health care utilization heart rhythm heart ventricle tachycardia human long QT syndrome major clinical study male multicenter study priority journal quality of life secondary analysis supraventricular tachycardia validation study LA - English M3 - Article N1 - L603241449 2015-03-27 2015-05-22 PY - 2015 SN - 1556-3871 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L603241449 http://dx.doi.org/10.1016/j.hrthm.2015.01.022 VL - 12 ID - 867 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. AN - 109727436. Language: English. Entry Date: 20150923. Revision Date: 20160206. Publication Type: journal article AU - Czosek, Richard J. AU - Cassedy, Amy E. AU - Wray, Jo AU - Wernovsky, Gil AU - Newburger, Jane W. AU - Mussatto, Kathleen A. AU - Mahony, Lynn AU - Tanel, Ronn E. AU - Cohen, Mitchell I. AU - Franklin, Rodney C. AU - Brown, Kate L. AU - Rosenthal, David AU - Drotar, Dennis AU - Marino, Bradley S. DB - ccm DO - 10.1016/j.hrthm.2015.01.022 DP - EBSCOhost IS - 5 N1 - research. Journal Subset: Biomedical; USA. NLM UID: 101200317. PMID: NLM25602174. 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=109727436&site=ehost-live&scope=site VL - 12 ID - 1503 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. © 2015 Heart Rhythm Society. All rights reserved. AD - Heart Institute, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave MLC, Cincinnati, OH 45229, United States Department of Biostatistics, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Critical Care and Cardiorespiratory Division, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, United Kingdom Miami Children's Hospital, Miami, FL, United States Department of Cardiology, Boston Children's Hospital and Harvard Medical School, Boston, MA, United States Herma Heart Center, Children's Hospital of Wisconsin, Milwaukee, WI, United States Department of Pediatrics, University of Texas Southwestern Medical School, Dallas, TX, United States Department of Pediatrics, UCSF Benioff Children's Hospital, UCSF School of Medicine, San Francisco, CA, United States Phoenix Children's Hospital and Arizona Pediatric Cardiology Consultants/Pediatrix, University of Arizona School of Medicine-Phoenix, Phoenix, AZ, United States 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, CA, United States 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. C7 - 6093 DB - Scopus DO - 10.1016/j.hrthm.2015.01.022 IS - 5 KW - Arrhythmia Cardiac rhythm devices Channelopathy Electrophysiology Pediatrics Quality of life M3 - Article N1 - Cited By :15 Export Date: 15 June 2020 PY - 2015 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84928611311&doi=10.1016%2fj.hrthm.2015.01.022&partnerID=40&md5=5ac2e6624e06d4d60e560d2b66fb9ff2 VL - 12 ID - 1947 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 - R.J. Czosek, Division of Pediatric Cardiology, Department of Pediatrics, Heart Institute at Cincinnati, Children's Hospital Medical Center, Cincinnati, OH, United States 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. DB - Embase Medline DO - 10.1016/j.amjcard.2015.11.051 IS - 4 KW - cardiac agent adolescent article bicuspid aortic valve cardiac equipment child congenital heart disease Fallot tetralogy female heart single ventricle human long QT syndrome major clinical study male outcome variable priority journal psychological well-being quality of life social psychology LA - English M3 - Article N1 - L607378337 2015-12-29 2016-03-09 PY - 2016 SN - 1879-1913 0002-9149 SP - 605-610 ST - Quality of Life of Pediatric Patients with Long QT Syndrome T2 - American Journal of Cardiology TI - Quality of Life of Pediatric Patients with Long QT Syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L607378337 http://dx.doi.org/10.1016/j.amjcard.2015.11.051 VL - 117 ID - 822 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 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 - 115727570. Language: English. Entry Date: 20160529. Revision Date: 20190308. Publication Type: journal article AU - Czosek, Richard J. AU - Kaltman, Jonathan R. AU - Cassedy, Amy E. AU - Shah, Maully J. AU - Vetter, Victoria L. AU - Tanel, Ronn E. AU - Wernovksy, Gil AU - Wray, Jo AU - Marino, Bradley S. DB - ccm DO - 10.1016/j.amjcard.2015.11.051 DP - EBSCOhost IS - 4 KW - Quality of Life Self Concept Long QT Syndrome -- Psychosocial Factors Cross Sectional Studies Long QT Syndrome -- Physiopathology Male Electrocardiography Female Prospective Studies Human Adolescence Child Emotions Validation Studies Comparative Studies Evaluation Research Multicenter Studies Scales N1 - research. Journal Subset: Biomedical; Peer Reviewed; USA. Instrumentation: Pediatric Cardiac Quality of Life Inventory (PCQLI); Pediatric QOL Inventory [PedsQL]. NLM UID: 0207277. PMID: NLM26721659. PY - 2016 SN - 0002-9149 SP - 605-610 ST - Quality of Life of Pediatric Patients With Long QT Syndrome T2 - American Journal of Cardiology TI - Quality of Life of Pediatric Patients With Long QT Syndrome UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=115727570&site=ehost-live&scope=site VL - 117 ID - 1477 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. © 2016 Elsevier Inc. AD - Division of Pediatric Cardiology, Department of Pediatrics, Heart Institute at Cincinnati, Children's Hospital Medical Center, Cincinnati, OH, United States Division of Pediatric Cardiology, Department of Pediatrics, Children's National Medical Center, Washington, DC, United States Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Division of Biostatistics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Division of Cardiology, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA, United States Division of Pediatric Cardiology, Department of Pediatrics, UCSF Benioff Children's Hospital, San Francisco, CA, United States Department of Pediatrics, Heart Program at Nicklaus Children's Hospital, Florida International University, Herbert Wertheim College of Medicine, Miami, FL, United States 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 and Robert F. Lurie Children's Hospital of Chicago, Chicago, IL, United States 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. DB - Scopus DO - 10.1016/j.amjcard.2015.11.051 IS - 4 M3 - Article N1 - Cited By :9 Export Date: 15 June 2020 PY - 2016 SP - 605-610 ST - Quality of Life of Pediatric Patients with Long QT Syndrome T2 - American Journal of Cardiology TI - Quality of Life of Pediatric Patients with Long QT Syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84958866217&doi=10.1016%2fj.amjcard.2015.11.051&partnerID=40&md5=633c2c1885d9ab9e2d1f8abf4b24e399 VL - 117 ID - 1922 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 - Department of Cardiac Surgery, Royal Children's Hospital, Department of Pediatrics of the University of Melbourne, Parkville, Vic., Australia Department of Cardiology, Royal Children's Hospital, Department of Pediatrics of the University of Melbourne, Parkville, Vic., Australia Department of Academic Child Psychiatry Unit, Royal Children's Hospital, Department of Pediatrics of the University of Melbourne, Parkville, Vic., Australia Department of Cardiology, Royal Melbourne Hospital, 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 - Scopus DO - 10.1016/j.athoracsur.2009.07.079 IS - 6 M3 - Article N1 - Cited By :47 Export Date: 15 June 2020 PY - 2009 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-71949128398&doi=10.1016%2fj.athoracsur.2009.07.079&partnerID=40&md5=4f6cef31f77adeab361205d5a697b24b VL - 88 ID - 2164 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. AU - Daily, J. AU - FitzGerald, M. AU - Downing, K. AU - King, E. AU - del Rey, J. G. AU - Ittenbach, R. AU - Marino, B. DB - Medline DO - 10.1017/S1047951114002625 IS - 1 KW - adolescent adult attitude to health child congenital heart malformation female health personnel attitude human infant male nurse parent physician preschool child questionnaire LA - English M3 - Article N1 - L615318017 2017-04-17 PY - 2016 SN - 1467-1107 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=L615318017 http://dx.doi.org/10.1017/S1047951114002625 VL - 26 ID - 839 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 - Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, United States of America Institute for Policy Research, University of Cincinnati, Cincinnati, Ohio, United States of America AN - 111829507. Language: English. Entry Date: 20160826. Revision Date: 20180718. Publication Type: journal article AU - Daily, Joshua AU - FitzGerald, Mike AU - Downing, Kimberly AU - King, Eileen AU - del Rey, Javier Gonzalez AU - Ittenbach, Richard AU - Marino, Bradley DB - ccm DO - 10.1017/S1047951114002625 DP - EBSCOhost IS - 1 KW - Attitude to Health Heart Defects, Congenital Nurses Physicians Attitude of Health Personnel Parents Child, Preschool Adolescence Adult Child Male Female Infant Human N1 - research; tables/charts. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. NLM UID: 9200019. PMID: NLM25599703. PY - 2016 SN - 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=111829507&site=ehost-live&scope=site VL - 26 ID - 1514 ER - TY - JOUR AB - Over the past decade, as the majority of patients with single ventricle anatomy who have undergone the Fontan operation reach adulthood, a newly recognized disease process, Fontan-associated liver disease (FALD), has emerged. FALD is an extracardiac complication that may lead to substantial comorbid disease and premature mortality. The risk factors, pathophysiology, longitudinal consequences, and therapeutic options related to FALD remain poorly defined. Although we recognize that Fontan circulatory properties are associated with extracardiac organ dysfunction, numerous gaps in our understanding of the nature of this relationship exist. Such extracardiac manifestations, in addition to other late complications of the circulation, can significantly affect quality of life and healthcare use. Therefore, to initiate a formal evaluation of FALD, the American College of Cardiology (ACC) sponsored a stakeholders meeting on October 1 to 2, 2015, in Washington, DC. The goal of the meeting was to bring together subspecialty experts in the fields of adult and pediatric hepatology, congenital cardiology (adult congenital and pediatric cardiology), heart failure/transplant, epidemiology, and cardiothoracic surgery, as well as patient advocates, patients, parents of children and young adults who have had the Fontan procedure, and research organizations and societies to discuss the current state of FALD. Topics included gaps in knowledge, optimal care, research opportunities and barriers, and sound practices to guide providers, patients, and families. This report summarizes findings from the stakeholders meeting and seeks to establish a platform for understanding and addressing FALD. AN - 127025407. Language: English. Entry Date: 20190531. Revision Date: 20190712. Publication Type: journal article. Journal Subset: Biomedical AU - Daniels, Curt J. AU - Bradley, Elisa A. AU - Landzberg, Mike J. AU - Aboulhosn, Jamil AU - Beekman, Robert H., 3rd AU - Book, Wendy AU - Gurvitz, Michelle AU - John, Anitha AU - John, Binu AU - Marelli, Ariane AU - Marino, Bradley S. AU - Minich, L. LuAnn AU - Poterucha, John J. AU - Rand, Elizabeth B. AU - Veldtman, Gruschen R. DB - ccm DO - 10.1016/j.jacc.2017.10.045 DP - EBSCOhost IS - 25 KW - Liver Diseases -- Etiology Heart Defects, Congenital -- Surgery Cardiopulmonary Bypass -- Adverse Effects Postoperative Complications Survival -- Trends Heart Defects, Congenital -- Mortality Risk Factors World Health Liver Diseases -- Epidemiology Incidence Congresses and Conferences N1 - Peer Reviewed; USA. NLM UID: 8301365. PMID: NLM29268929. PY - 2017 SN - 0735-1097 SP - 3173-3194 ST - Fontan-Associated Liver Disease: Proceedings from the American College of Cardiology Stakeholders Meeting, October 1 to 2, 2015, Washington DC T2 - Journal of the American College of Cardiology (JACC) TI - Fontan-Associated Liver Disease: Proceedings from the American College of Cardiology Stakeholders Meeting, October 1 to 2, 2015, Washington DC UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=127025407&site=ehost-live&scope=site VL - 70 ID - 1463 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. © 2019 Wiley Periodicals, Inc. AD - Connecticut Children’s, Hartford, CT, United States Boston Children’s Hospital, Boston, MA, United States Children’s Hospital at Dartmouth-Hitchcock, Lebanon, NH, United States Yale-New Haven Children’s Hospital, New Haven, CT, United States Child Heart Associates, Worcester, MA, 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 - Scopus DO - 10.1111/chd.12854 IS - 6 KW - congenital heart disease Fontan screening single ventricle survey M3 - Article N1 - Export Date: 15 June 2020 PY - 2019 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85074591621&doi=10.1111%2fchd.12854&partnerID=40&md5=5a0b37b6ca24778595d9fffcc73f9171 VL - 14 ID - 1727 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 - J. Simpson, Department of Congenital Heart Disease, Evelina London Children's Hospital, Guy's and St Thomas' NHS Fiundation Trust, London, United Kingdom AU - Davidson, J. AU - Gringras, P. AU - Fairhurst, C. AU - Simpson, J. DB - Embase Medline DO - 10.1136/archdischild-2014-306449 IS - 5 KW - adolescent article attention deficit disorder autism cardiopulmonary exercise test child controlled study exercise tolerance female heart single ventricle human hypoplastic left heart syndrome major clinical study male observational study outcome assessment pediatric cardiology priority journal retrospective study United Kingdom LA - English M3 - Article N1 - L602247817 2015-02-19 2015-05-13 PY - 2015 SN - 1468-2044 0003-9888 SP - 449-453 ST - Physical and neurodevelopmental outcomes in children with single-ventricle circulation T2 - Archives of Disease in Childhood TI - Physical and neurodevelopmental outcomes in children with single-ventricle circulation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L602247817 http://dx.doi.org/10.1136/archdischild-2014-306449 VL - 100 ID - 883 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 - 109781703. Language: English. Entry Date: 20150717. Revision Date: 20170410. Publication Type: journal article AU - Davidson, Joseph AU - Gringras, Paul AU - Fairhurst, Charlie AU - Simpson, John DB - ccm DO - 10.1136/archdischild-2014-306449 DP - EBSCOhost IS - 5 KW - Child Behavior Disorders -- Physiopathology Child Development Heart Ventricle -- Abnormalities Hypoplastic Left Heart Syndrome -- Physiopathology Psychomotor Performance Adolescence Child Child Behavior Disorders -- Psychosocial Factors Female Great Britain Human Hypoplastic Left Heart Syndrome -- Psychosocial Factors Male Referral and Consultation Retrospective Design Hospitals, Special N1 - research. Journal Subset: Biomedical; Europe; Peer Reviewed; UK & Ireland. Special Interest: Pediatric Care. NLM UID: 0372434. PMID: NLM25480924. PY - 2015 SN - 0003-9888 SP - 449-453 ST - Physical and neurodevelopmental outcomes in children with single-ventricle circulation T2 - Archives of Disease in Childhood TI - Physical and neurodevelopmental outcomes in children with single-ventricle circulation UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=109781703&site=ehost-live&scope=site VL - 100 ID - 1505 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. © 2015, BMJ Publishing Group. All rights reserved. AD - King's College London School of Medicine, London, United Kingdom Department of Paediatric Neurology, Evelina London Children's Hospital, Guy's and St Thomas' NHS Fiundation Trust, London, United Kingdom Department of Congenital Heart Disease, Evelina London Children's Hospital, Guy's and St Thomas' NHS Fiundation Trust, London, SE1 7EH, United Kingdom AU - Davidson, J. AU - Gringras, P. AU - Fairhurst, C. AU - Simpson, J. DB - Scopus DO - 10.1136/archdischild-2014-306449 IS - 5 M3 - Article N1 - Cited By :12 Export Date: 15 June 2020 PY - 2015 SP - 449-453 ST - Physical and neurodevelopmental outcomes in children with single-ventricle circulation T2 - Archives of Disease in Childhood TI - Physical and neurodevelopmental outcomes in children with single-ventricle circulation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84928210936&doi=10.1136%2farchdischild-2014-306449&partnerID=40&md5=94a3839fc5d2d528cfef33c784c59e96 VL - 100 ID - 1956 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 - Division of Pediatrics, Children's Hospital, Cleveland Clinic, 9500 Euclid Avenue, S20, Cleveland, OH 44195, United States Department of Pediatric Critical Care Medicine, Children's Hospital, Cleveland Clinic, 9500 Euclid Avenue, S20, Cleveland, OH 44195, United States Department of Epidemiology and Biostatistics, Children's Hospital, Cleveland Clinic, 9500 Euclid Avenue, S20, Cleveland, OH 44195, United States Pediatric Clinical Trials Office, 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 - Scopus DO - 10.1007/s00246-007-9027-9 IS - 2 KW - d-Transposition of the great arteries Growth failure Hypoplastic left heart syndrome Nasogastric tube M3 - Article N1 - Cited By :102 Export Date: 15 June 2020 PY - 2008 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-40949094044&doi=10.1007%2fs00246-007-9027-9&partnerID=40&md5=7f3382dfeb2f629704f54855fbd9a91f VL - 29 ID - 2198 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 - J.A.M. Davis, Nationwide Children’s Hospital, 700 Children’s Dr, Columbus, OH, United States AU - Davis, J. A. M. AU - Bass, A. AU - Humphrey, L. AU - Texter, K. AU - Garee, A. DB - Embase Medline DO - 10.1007/s00246-019-02231-y IS - 1 KW - age article child collaborative care team congenital heart malformation disease classification family service fetus monitoring Fontan procedure health care quality heart single ventricle hospitalization human major clinical study medical information palliative therapy patient care patient compliance social support standardization terminal care LA - English M3 - Article N1 - L2003531931 2019-11-15 2020-02-10 PY - 2020 SN - 1432-1971 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 - Pediatric Cardiology TI - Early Integration of Palliative Care in Families of Children with Single Ventricle Congenital Heart Defects: A Quality Improvement Project to Enhance Family Support UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2003531931 http://dx.doi.org/10.1007/s00246-019-02231-y VL - 41 ID - 569 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. © 2019, Springer Science+Business Media, LLC, part of Springer Nature. AD - Nationwide Children’s Hospital, 700 Children’s Dr, Columbus, OH 43205, United States Nationwide Children’s Hospital, The Ohio State School of Medicine, 700 Children’s Dr, Columbus, OH 43205, United States AU - Davis, J. A. M. AU - Bass, A. AU - Humphrey, L. AU - Texter, K. AU - Garee, A. DB - Scopus DO - 10.1007/s00246-019-02231-y IS - 1 KW - Cardiac Collaboration End-of-life Palliative Pediatric Single ventricle M3 - Article N1 - Export Date: 15 June 2020 PY - 2020 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 - Pediatric Cardiology TI - Early Integration of Palliative Care in Families of Children with Single Ventricle Congenital Heart Defects: A Quality Improvement Project to Enhance Family Support UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85074692174&doi=10.1007%2fs00246-019-02231-y&partnerID=40&md5=00451e23d58875065b82179d942dcbc0 VL - 41 ID - 1715 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 α-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 - M.A. Gatzoulis, Royal Brompton Hospital, Sydney Street, London SW3 6NP, United Kingdom AU - Davos, C. H. AU - Francis, D. P. AU - Leenarts, M. F. E. AU - Yap, S. C. AU - Li, W. AU - Davlouros, P. A. AU - Wensel, R. AU - Coats, A. J. S. AU - Piepoli, M. AU - Sreeram, N. AU - Gatzoulis, M. A. DB - Embase Medline IS - 10 SUPPL. KW - adolescent adult article autonomic dysfunction cardiovascular disease cholinergic activity clinical article controlled study correlation analysis female Fontan procedure heart atrium enlargement heart rate variability heart right atrium heart single ventricle heart size human male measurement prediction pressoreceptor reflex priority journal prognosis RR interval sinus node disease sudden death supraventricular tachycardia sympathetic tone systolic blood pressure procedures LA - English M3 - Article N1 - L37099951 2003-10-02 PY - 2003 SN - 0009-7322 SP - II180-II185 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L37099951 VL - 108 ID - 1298 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 α-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 Hospital, Sydney Street, London SW3 6NP, United Kingdom AU - Davos, C. H. AU - Francis, D. P. AU - Leenarts, M. F. E. AU - Yap, S. C. AU - Li, W. AU - Davlouros, P. A. AU - Wensel, R. AU - Coats, A. J. S. AU - Piepoli, M. AU - Sreeram, N. AU - Gatzoulis, M. A. DB - Scopus IS - 10 SUPPL. KW - Arrhythmias Baroreceptors Fontan procedure Heart rate/heart rate variability Nervous system autonomic M3 - Article N1 - Cited By :58 Export Date: 15 June 2020 PY - 2003 SP - II180-II185 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0042416965&partnerID=40&md5=340fd27373dcea145753ac387b8e2565 VL - 108 ID - 2257 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. © 2014 Wiley Periodicals, Inc. AD - Division of Cardiology, Children's National Health System, Washington, DC, United States Division of Psychiatry, Children's National Health System, Washington, DC, United States Center for Translational Science, Children's National Health System, 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 - Scopus DO - 10.1111/chd.12221 IS - 2 KW - Adult congenital heart disease Congenital heart disease Sports cardiology Sports participation M3 - Article N1 - Cited By :21 Export Date: 15 June 2020 PY - 2015 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84927761217&doi=10.1111%2fchd.12221&partnerID=40&md5=6ff4c73921dab154f71a90ed3e5624af VL - 10 ID - 1952 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 - Divisions Cardiovasc. Surg., C., Mayo Clinic, Mayo Foundation, Rochester, MN, United States 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 - Scopus DO - 10.1016/S0003-4975(02)04547-2 IS - 2 M3 - Article N1 - Cited By :185 Export Date: 15 June 2020 PY - 2003 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0037312416&doi=10.1016%2fS0003-4975%2802%2904547-2&partnerID=40&md5=cfe986088ce8eff87f5e3365a319a0a0 VL - 75 ID - 2264 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 - D.R. DeMaso, Department of Psychiatry, Boston Children's Hospital, 300 Longwood Ave., Boston, MA, United States 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. DB - Embase Medline DO - 10.1542/peds.2016-2241 IS - 3 KW - adolescent anxiety disorder article attention deficit disorder clinical evaluation cohort analysis comorbidity controlled study deep hypothermic circulatory arrest depression disruptive behavior female Fontan procedure heart single ventricle human intelligence quotient low birth weight major clinical study male mental disease risk assessment risk factor scoring system self report social psychology symptom LA - English M3 - Article N1 - L614992719 2017-04-24 2017-05-12 PY - 2017 SN - 1098-4275 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L614992719 http://dx.doi.org/10.1542/peds.2016-2241 VL - 139 ID - 770 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 displaya 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 - Department of Psychiatry, Boston Children's Hospital, Boston, Massachusetts Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts Department of Psychiatry, Harvard Medical School, Boston, Massachusetts Department of Neurology, Boston Children's Hospital, Boston, Massachusetts Department of Neurology, Harvard Medical School, Boston, Massachusetts Department of Biostatistics, Harvard T.H. Chan School of Public Health, Boston, Massachusetts Department of Pediatrics, Harvard Medical School, Boston, Massachusetts AN - 121669110. Language: English. Entry Date: 20170314. Revision Date: 20190715. Publication Type: Article AU - DeMaso, David R. AU - Calderon, Johanna AU - Taylor, George A. AU - Holland, Jennifer E. AU - Stopp, Christian AU - White, Matthew T. AU - Bellinger, David C. AU - Rivkin, Michael J. AU - Wypij, David AU - Newburger, Jane W. DB - ccm DO - 10.1542/peds.2016-2241 DP - EBSCOhost IS - 3 KW - Heart Defects, Congenital -- Psychosocial Factors -- In Adolescence Heart Ventricle -- Pathology Mental Disorders -- Risk Factors Adolescence Human Female Male Interview Guides Self Report Attention Deficit Hyperactivity Disorder -- Risk Factors Anxiety Disorders -- Risk Factors Scales Early Diagnosis Cross Sectional Studies Fisher's Exact Test Descriptive Statistics Data Analysis Software P-Value Regression Heart Defects, Congenital -- Surgery Wilcoxon Rank Sum Test Semi-Structured Interview Psychological Tests Checklists Brain -- Ultrasonography Magnetic Resonance Imaging Outpatients Hospitals -- Massachusetts Massachusetts Funding Source N1 - glossary; research; tables/charts. Journal Subset: Biomedical; Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. Instrumentation: Children's Global Assessment Scale (CGAS); Revised Children's Manifest Anxiety Scale (R-CMAS); Children's Depression Inventory (CDI) (Kovacs and Beck); Schedule for Affective Disorders and Schizophrenia for School-Age Children-Present and Lifetime version (K-SADS-PL); Conner's ADHD Rating Scales (CADS); Child Stress Disorders Checklist (CSDC); Brief Psychiatric Rating Scale for Children (BPRS-C); Wechsler Intelligence Scale for Children-Fourth Edition (WISC-IV); Wechsler Adult Intelligence Scale-Fourth Edition (WAIS-IV). Grant Information: This study was supported by grants from the National Heart, Lung, and Blood Institute (grant HL096825) and the Farb Family Fund. Funded by the National Institutes of Health (NIH).. NLM UID: 0376422. PY - 2017 SN - 0031-4005 SP - 1-10 ST - Psychiatric Disorders in Adolescents With Single Ventricle Congenital Heart Disease T2 - Pediatrics TI - Psychiatric Disorders in Adolescents 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=121669110&site=ehost-live&scope=site VL - 139 ID - 1469 ER - TY - JOUR AB - Background and objectives: Mental health outcomes for survivors of critical congenital heart abstract 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. (PsycINFO Database Record (c) 2017 APA, all rights reserved) AD - DeMaso, David R., Department of Psychiatry, Boston Children’s Hospital, 300 Longwood Ave, Boston, MA, US, 02115 AN - 2017-22997-013 AU - DeMaso, David R. AU - Calderon, Johanna AU - Taylor, George A. AU - Holland, Jennifer E. AU - Stopp, Christian AU - White, Matthew T. AU - Bellinger, David C. AU - Rivkin, Michael J. AU - Wypij, David AU - Newburger, Jane W. DB - psyh DP - EBSCOhost IS - 3 KW - psychiatric disorders adolescents congenital heart disease Congenital Disorders Heart Disorders Mental Disorders Risk Factors Adolescent Characteristics N1 - Department of Psychiatry, Boston Children’s Hospital, Boston, MA, US. Release Date: 20171002. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Congenital Disorders; Heart Disorders; Mental Disorders. Minor Descriptor: Risk Factors; Adolescent Characteristics. Classification: Cardiovascular Disorders (3295). Population: Human (10); Male (30); Female (40). Location: US. Age Group: Childhood (birth-12 yrs) (100); School Age (6-12 yrs) (180); Adolescence (13-17 yrs) (200); Adulthood (18 yrs & older) (300); Young Adulthood (18-29 yrs) (320). Tests & Measures: Hollingshead Four Factor Index; Schedule for Affective Disorders and Schizophrenia for School-Aged Children—Present and Lifetime Version; Brief Psychiatric Rating Scale for Children; Children's Depression Inventory; Wechsler Adult Intelligence Scale--Fourth Edition DOI: 10.1037/t15169-000; Wechsler Intelligence Scale for Children, Fourth Edition DOI: 10.1037/t15174-000; Revised Children's Manifest Anxiety Scale DOI: 10.1037/t00514-000; Children's Global Assessment Scale; Child Stress Disorders Checklist DOI: 10.1037/t05146-000; Conners' Adult ADHD Rating Scales DOI: 10.1037/t04961-000. Methodology: Brain Imaging; Empirical Study; Quantitative Study. Supplemental Data: Tables and Figures Internet. Page Count: 10. Issue Publication Date: Mar, 2017. Publication History: Accepted Date: Nov 30, 2016. Copyright Statement: The American Academy of Pediatrics. 2017. Sponsor: National Heart, Lung, and Blood Institute, US. Grant: HL096825. Recipients: No recipient indicated Sponsor: Farb Family Fund. Recipients: No recipient indicated Sponsor: National Institutes of Health, US. Recipients: No recipient indicated PY - 2017 SN - 0031-4005 1098-4275 SP - 1-10 ST - Psychiatric disorders in adolescents with single ventricle congenital heart disease T2 - Pediatrics TI - Psychiatric disorders in adolescents with single ventricle congenital heart disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2017-22997-013&site=ehost-live&scope=site david.demaso@childrens.harvard.edu VL - 139 ID - 1666 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. Copyright © 2017 by the American Academy of Pediatrics. AD - Department of Psychiatry, Boston Children's Hospital, 300 Longwood Ave., Boston, MA 02115, United States Department of Cardiology, Boston Children's Hospital, Boston, MA, United States Department of Neurology, Boston Children's Hospital, Boston, MA, United States Department of Psychiatry, Harvard Medical School, Boston, MA, United States Department of Neurology, Harvard Medical School, Boston, MA, United States Department of Pediatrics, Harvard Medical School, Boston, MA, United States Department of Biostatistics, Harvard T.H. Chan School of Public Health, Boston, MA, United States 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. C7 - e20162241 DB - Scopus DO - 10.1542/peds.2016-2241 IS - 3 M3 - Article N1 - Cited By :36 Export Date: 15 June 2020 PY - 2017 ST - Psychiatric disorders in adolescents with single ventricle congenital heart disease T2 - Pediatrics TI - Psychiatric disorders in adolescents with single ventricle congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85016055400&doi=10.1542%2fpeds.2016-2241&partnerID=40&md5=d559d4f62aeab4779e7bb1faeba2f6c3 VL - 139 ID - 1867 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 - N. Dempster, Nationwide Children's Hospital Columbus, 700 Childrens Drive, Columbus, OH, United States AU - Dempster, N. AU - Cua, C. L. AU - Wernovsky, G. AU - Caris, E. AU - Neely, T. AU - Allen, R. AU - Butz, C. DB - Embase Medline DO - 10.1017/S1047951117001159 IS - 1 KW - acute disease adolescent adult article caregiver cerebrovascular accident child chronic disease controlled study demography female functional status human hypoplastic left heart syndrome Internet male Pediatric Quality of Life Inventory quality of life seizure LA - English M3 - Article N1 - L619883408 2017-12-26 2018-07-27 PY - 2018 SN - 1467-1107 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 - Cardiology in the Young TI - Children with hypoplastic left heart syndrome have lower quality of life than healthy controls and children with other illnesses UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L619883408 http://dx.doi.org/10.1017/S1047951117001159 VL - 28 ID - 715 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 - Nationwide Children’s Hospital Columbus, Ohio, United States of America Miami, Florida, United States of America AN - 126608658. Language: English. Entry Date: 20180731. Revision Date: 20190101. Publication Type: journal article AU - Dempster, Nicole AU - Cua, Clifford L. AU - Wernovsky, Gil AU - Caris, Elizabeth AU - Neely, Trent AU - Allen, Robin AU - Butz, Catherine DB - ccm DO - 10.1017/S1047951117001159 DP - EBSCOhost IS - 1 KW - Quality of Life Hypoplastic Left Heart Syndrome -- Physiopathology Psychological Tests Child Caregivers Case Control Studies Female United States Hypoplastic Left Heart Syndrome -- Surgery Adolescence Child, Preschool Severity of Illness Indices Male Questionnaires Human N1 - research; tables/charts. 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: NLM28847316. 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 - Cardiology in the Young TI - Children with hypoplastic left heart syndrome have lower quality of life than healthy controls and children with other illnesses UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=126608658&site=ehost-live&scope=site VL - 28 ID - 1450 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. © Cambridge University Press 2017. AD - Nationwide Children's Hospital Columbus, 700 Childrens Drive, Columbus, OH 43205, United States MiamiFlorida, United States AU - Dempster, N. AU - Cua, C. L. AU - Wernovsky, G. AU - Caris, E. AU - Neely, T. AU - Allen, R. AU - Butz, C. DB - Scopus DO - 10.1017/S1047951117001159 IS - 1 KW - adjustment Hypoplastic left heart syndrome quality of life M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2018 SP - 21-26 ST - Children with hypoplastic left heart syndrome have lower quality of life than healthy controls and children with other illnesses T2 - Cardiology in the Young TI - Children with hypoplastic left heart syndrome have lower quality of life than healthy controls and children with other illnesses UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85038427671&doi=10.1017%2fS1047951117001159&partnerID=40&md5=fdd6cb1dd654b458c1dadf59d112cb78 VL - 28 ID - 1826 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 - N.A. Kasparian, Heart Centre for Children, The Children's Hospital at Westmead, Locked Bag 4001, Westmead, NSW, Australia AU - Denniss, D. L. AU - Sholler, G. F. AU - Costa, D. S. J. AU - Winlaw, D. S. AU - Kasparian, N. A. DB - Embase Medline DO - 10.1016/j.jpeds.2018.09.037 KW - adult article Australia child comorbidity congenital heart disease cross-sectional study family functioning feeding difficulty female heart single ventricle human male mental stress Pediatric Quality of Life Inventory priority journal quality of life social psychology temperament LA - English M3 - Article N1 - L2001462323 2019-01-21 2019-01-24 PY - 2019 SN - 1097-6833 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 - Journal of Pediatrics TI - Need for Routine Screening of Health-Related Quality of Life in Families of Young Children with Complex Congenital Heart Disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2001462323 http://dx.doi.org/10.1016/j.jpeds.2018.09.037 VL - 205 ID - 628 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 AN - 134152654. Language: English. Entry Date: 20191024. Revision Date: 20191029. Publication Type: journal article AU - Denniss, Dominique L. AU - Sholler, Gary F. AU - Costa, Daniel S. J. AU - Winlaw, David S. AU - Kasparian, Nadine A. DB - ccm DO - 10.1016/j.jpeds.2018.09.037 DP - EBSCOhost KW - Heart Defects, Congenital -- Psychosocial Factors Quality of Life -- Psychosocial Factors Mothers -- Psychosocial Factors Case Control Studies Cross Sectional Studies Adult Child, Preschool Female Cognition Heart Defects, Congenital -- Surgery Human Infant Anxiety -- Psychosocial Factors Male Stress, Psychological -- Psychosocial Factors Validation Studies Comparative Studies Evaluation Research Multicenter Studies Scales N1 - research. Journal Subset: Biomedical; Peer Reviewed; USA. Instrumentation: Pediatric Quality of Life Inventory (PEDSQL); Health-Related Hardiness Scale (HRHS); Quality of Life Inventory (QOLI). NLM UID: 0375410. PMID: NLM30366775. PY - 2019 SN - 0022-3476 SP - 21-21 ST - Need for Routine Screening of Health-Related Quality of Life in Families of Young Children with Complex Congenital Heart Disease T2 - Journal of Pediatrics TI - Need for Routine Screening of Health-Related Quality of Life in Families of Young Children with Complex Congenital Heart Disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=134152654&site=ehost-live&scope=site VL - 205 ID - 1494 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. © 2018 Elsevier Inc. 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 Discipline of Child and Adolescent Health, Sydney Medical School, The University of SydneyNSW, Australia Pain Management Research Institute, Royal North Shore Hospital, North Sydney, NSW, Australia Sydney Medical School, The University of SydneyNSW, Australia AU - Denniss, D. L. AU - Sholler, G. F. AU - Costa, D. S. J. AU - Winlaw, D. S. AU - Kasparian, N. A. DB - Scopus DO - 10.1016/j.jpeds.2018.09.037 KW - child development congenital heart disease integrated care mental health psychological stress psychosocial screening quality of life M3 - Article N1 - Cited By :6 Export Date: 15 June 2020 PY - 2019 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 - Journal of Pediatrics TI - Need for Routine Screening of Health-Related Quality of Life in Families of Young Children with Complex Congenital Heart Disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85059634957&doi=10.1016%2fj.jpeds.2018.09.037&partnerID=40&md5=7e889bb27401f92e33606f958f71a907 VL - 205 ID - 1761 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. AD - G.R. Derk, College of Medicine at Champaign-Urbana, 506 S. Mathews Ave, Urbana, IL, United States AU - Derk, G. R. AU - Aboulhosn, J. AU - Reardon, L. C. DB - Embase Medline DO - 10.14503/THIJ-14-4151 IS - 1 KW - acetylsalicylic acid benzodiazepine brain natriuretic peptide clonazepam ibuprofen metoprolol troponin adult akinesia article case report computed tomographic angiography dyspnea echocardiography electrocardiography female heart single ventricle human hyperventilation takotsubo cardiomyopathy thorax pain young adult LA - English M3 - Article N1 - L608142778 2016-02-11 2016-02-15 PY - 2016 SN - 1526-6702 0730-2347 SP - 61-64 ST - Takotsubo cardiomyopathy in a 22-year-old single-ventricle patient T2 - Texas Heart Institute Journal TI - Takotsubo cardiomyopathy in a 22-year-old single-ventricle patient UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L608142778 http://dx.doi.org/10.14503/THIJ-14-4151 VL - 43 ID - 827 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. © 2016 by the Texas Heart® Institute, Houston. AD - Ahmanson/UCLA Adult Congenital Heart Disease Center, Los Angeles, CA 90095, United States AU - Derk, G. R. AU - Aboulhosn, J. AU - Reardon, L. C. DB - Scopus DO - 10.14503/THIJ-14-4151 IS - 1 KW - Apical-ballooning syndrome Cardiomyopathies/diagnosis Congenital Coronary vasospasm/complications Female Fontan procedure Heart disease Psychological/complications Single ventricle Stress Takotsubo cardiomyopathy/diagnosis/epidemiology/therapy Treatment outcome Ventricular dysfunction/etiology/radiography M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2016 SP - 61-64 ST - Takotsubo cardiomyopathy in a 22-year-old single-ventricle patient T2 - Texas Heart Institute Journal TI - Takotsubo cardiomyopathy in a 22-year-old single-ventricle patient UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84956926185&doi=10.14503%2fTHIJ-14-4151&partnerID=40&md5=094b85bcdff1e1fa309601b88e1ee87b VL - 43 ID - 1924 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 - J.W. Gaynor, Pediatric Cardiothoracic Surgery, The Children's Hospital of Philadelphia, Perelman School of Medicine at The University of Pennsylvania, 3400 Civic Center Boulevard, Ninth Floor, Main Building, Philadelphia, PA, United States 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. DB - Embase Medline DO - 10.1016/j.jtcvs.2016.03.095 IS - 2 KW - apolipoprotein E desflurane halothane inhalation anesthetic agent isoflurane sevoflurane aortic arch surgery article child deep hypothermic circulatory arrest extracorporeal oxygenation female heart surgery human hypoplastic left heart syndrome infant intelligence quotient left ventricular assist device major clinical study male medical record review mental disease minimum lung alveolus concentration nerve degeneration priority journal velocity LA - English M3 - Article N1 - L610353321 2016-05-20 2018-08-03 PY - 2016 SN - 1097-685X 0022-5223 SP - 482-489 ST - Increasing cumulative exposure to volatile anesthetic agents is associated with poorer neurodevelopmental outcomes in children with hypoplastic left heart syndrome T2 - Journal of Thoracic and Cardiovascular Surgery TI - Increasing cumulative exposure to volatile anesthetic agents is associated with poorer neurodevelopmental outcomes in children with hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L610353321 http://dx.doi.org/10.1016/j.jtcvs.2016.03.095 VL - 152 ID - 799 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. © 2016 The American Association for Thoracic Surgery 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, United States Department of Pediatrics, The Children's Hospital of Philadelphia, Perelman School of Medicine at The University of Pennsylvania, Philadelphia, Pa, United States Division of Pediatric Cardiothoracic Surgery, The Children's Hospital of Philadelphia, Perelman School of Medicine at The University of Pennsylvania, Philadelphia, Pa, United States Center for Biomedical Informatics, The Children's Hospital of Philadelphia, Perelman School of Medicine at The University of Pennsylvania, Philadelphia, Pa, United States Division of Biostatistics and Epidemiology, Cincinnati Children's Medical Center, University of Cincinnati School of Medicine, Cincinnati, Ohio, United States 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. DB - Scopus DO - 10.1016/j.jtcvs.2016.03.095 IS - 2 KW - anesthesia congenital heart surgery neurodevelopment neurotoxicity M3 - Article N1 - Cited By :22 Export Date: 15 June 2020 PY - 2016 SP - 482-489 ST - Increasing cumulative exposure to volatile anesthetic agents is associated with poorer neurodevelopmental outcomes in children with hypoplastic left heart syndrome T2 - Journal of Thoracic and Cardiovascular Surgery TI - Increasing cumulative exposure to volatile anesthetic agents is associated with poorer neurodevelopmental outcomes in children with hypoplastic left heart syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84966708505&doi=10.1016%2fj.jtcvs.2016.03.095&partnerID=40&md5=08b1570cf8dbe30e0e0b1295c59103ca VL - 152 ID - 1894 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. © Annals of Cardiothoracic Surgery. AD - Labatt Family Heart Centre, Hospital for Sick Children, University of Toronto, Toronto, Canada AU - Dipchand, A. I. DB - Scopus DO - 10.21037/acs.2018.01.07 IS - 1 KW - Heart transplant Mechanical support Outcomes Sensitization Waitlist M3 - Article N1 - Cited By :19 Export Date: 15 June 2020 PY - 2018 SP - 31-55 ST - Current state of pediatric cardiac transplantation T2 - Annals of Cardiothoracic Surgery TI - Current state of pediatric cardiac transplantation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85041344078&doi=10.21037%2facs.2018.01.07&partnerID=40&md5=fb1364dfdd761f716ee11a054d597ea4 VL - 7 ID - 1835 ER - TY - JOUR AB - Plastic bronchitis is an uncommon condition characterized by the production of large pale bronchial casts that obstruct the tracheobronchial tree. The cellular content, cohesiveness, and often rubber-like consistency distinguish bronchial casts from the usual mucus plugs found with such disease states as asthma. Plastic bronchitis can be found secondary to many conditions, and a simplified classification scheme organizes it into two groups: an inflammatory type consisting of casts with an eosinophilic inflammatory infiltrate and an acellular type with a predominance of fibrin distinguished by its relative lack of cellular infiltrate, its mucin predominance, and its appearance only in children with congenital cyanotic heart disease. This report describes a 5-year-old girl who experienced plastic bronchitis 3 months after a Fontan procedure for hypoplastic left heart syndrome that was treated successfully with aerosolized tissue plasminogen activator. © 2008 Springer Science+Business Media, LLC. AD - Children's Hospital of Orange County, 505 South Main Street, Orange, CA 92868, United States AU - Do, T. B. AU - Chu, J. M. AU - Berdjis, F. AU - Anas, N. G. DB - Scopus DO - 10.1007/s00246-008-9312-2 IS - 3 KW - Fontan procedure Plastic bronchitis Tissue plasminogen activator M3 - Article N1 - Cited By :49 Export Date: 15 June 2020 PY - 2009 SP - 352-355 ST - Fontan patient with plastic bronchitis treated successfully using aerosolized tissue plasminogen activator: A case report and review of the literature T2 - Pediatric Cardiology TI - Fontan patient with plastic bronchitis treated successfully using aerosolized tissue plasminogen activator: A case report and review of the literature UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-64249085918&doi=10.1007%2fs00246-008-9312-2&partnerID=40&md5=d4fd28f63382c9477c3d2ba934734d1a VL - 30 ID - 2180 ER - 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 - Clinical Genetics Research Program, Centre for Addiction and Mental Health, Toronto, ON, Canada Division of Cardiology, Department of Medicine, University Health Network, Toronto, ON, Canada Toronto Congenital Cardiac Centre for Adults, Toronto General Hospital, Toronto, ON, Canada Centre for Applied Genomics, Program in Genetics and Genome Biology, Hospital for Sick Children, Toronto, ON, Canada Department of Molecular Genetics and McLaughlin Centre, University of Toronto, Toronto, ON, Canada Cytogenetics Laboratory, Department of Pediatric Laboratory Medicine, Hospital for Sick Children, Toronto, ON, Canada Department of Laboratory Medicine and Pathology, University of Toronto, Toronto, ON, Canada Department of Psychiatry, University of Toronto, 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 - Scopus DO - 10.1038/gim.2012.129 IS - 4 KW - 1q21.1 duplication copy-number variation macrocephaly schizophrenia tetralogy of Fallot M3 - Article N1 - Cited By :49 Export Date: 15 June 2020 PY - 2013 SP - 282-283 ST - 1q21.1 Microduplication expression in adults T2 - Genetics in Medicine TI - 1q21.1 Microduplication expression in adults UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84875933554&doi=10.1038%2fgim.2012.129&partnerID=40&md5=9a6116221f8c9b8f51306cfe4aa0c6b7 VL - 15 ID - 2055 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 - Department of Cardiology, Children's Hospital Boston, Boston, MA, United States Department of Pediatrics, Harvard Medical School, Boston, MA, United States Department of Anesthesia, Children's Hospital Boston, Boston, MA, United States Department of Anesthesia, Harvard Medical School, Boston, MA, United States Department of Radiology, Children's Hospital Boston, Boston, MA, United States Department of Radiology, Harvard Medical School, Boston, MA, United States 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. DB - Scopus DO - 10.1080/10976640701545305 IS - 5 KW - Adverse Events Complications Congenital Heart Disease Magnetic Resonance Imaging Risk Factors M3 - Article N1 - Cited By :37 Export Date: 15 June 2020 PY - 2007 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-34648818886&doi=10.1080%2f10976640701545305&partnerID=40&md5=724156df001b1b6e97a8ec1a4ddf4a3c VL - 9 ID - 2206 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 - D.C. Drinkwater, Division of Cardiothoracic Surgery, University of California, Los Angeles Medical Center 90024. AU - Drinkwater, D. C. AU - Laks, H. DB - Medline IS - 2 KW - article cardiopulmonary bypass child congenital heart malformation heart arrest heart muscle reperfusion human methodology pathophysiology LA - English M3 - Article N1 - L23891725 1993-06-01 PY - 1993 SN - 1043-0679 SP - 168-175 ST - Pediatric cardioplegic techniques T2 - Seminars in thoracic and cardiovascular surgery TI - Pediatric cardioplegic techniques UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L23891725 VL - 5 ID - 1380 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., United States AU - Drinkwater, D. C. AU - Laks, H. DB - Scopus IS - 2 M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 1993 SP - 168-175 ST - Pediatric cardioplegic techniques T2 - Seminars in thoracic and cardiovascular surgery TI - Pediatric cardioplegic techniques UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0027580916&partnerID=40&md5=105d4ce6c545e8f1c23aff779da35ac3 VL - 5 ID - 2313 ER - TY - JOUR AB - 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. To explore adolescents with a Fontan circulation, and their parents', readiness for transition to adult care and pre-implementation of a transition program. Seventeen adolescents (15-18 years) and 15 of their parents completed questionnaires at the start of their first transition clinic. 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. 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 - K. Du Plessis, Heart Research Group, Murdoch Children's Research Institute, Melbourne, Victoria, 50 Flemington Road, Melbourne, VIC, Australia AU - Du Plessis, K. AU - Culnane, E. AU - Peters, R. AU - D'Udekem, Y. DB - Embase Medline DO - 10.1515/ijamh-2017-0021 KW - adolescent adolescent health adult anxiety cardiologist clinical article congenital heart disease female heart single ventricle human juvenile male questionnaire surgical technique transition to adult care wellbeing LA - English M3 - Article in Press N1 - L619617161 2017-12-15 PY - 2017 SN - 2191-0278 0334-0139 ST - Adolescent and parent perspectives prior to involvement in a Fontan transition program T2 - International Journal of Adolescent Medicine and Health TI - Adolescent and parent perspectives prior to involvement in a Fontan transition program UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L619617161 http://dx.doi.org/10.1515/ijamh-2017-0021 ID - 754 ER - TY - JOUR AB - 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. To explore adolescents with a Fontan circulation, and their parents', readiness for transition to adult care and pre-implementation of a transition program. Seventeen adolescents (15-18 years) and 15 of their parents completed questionnaires at the start of their first transition clinic. 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. 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. © 2017 2017 Walter de Gruyter GmbH, Berlin/Boston. AD - Heart Research Group, Murdoch Children's Research Institute, Melbourne, Victoria, 50 Flemington Road, Melbourne, VIC 3052, Australia Transition Support Service, Royal Children's Hospital, Melbourne, VIC, Australia Heart Research Group, Murdoch Children's Research Institute, Melbourne, VIC, Australia Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, VIC, Australia AU - Du Plessis, K. AU - Culnane, E. AU - Peters, R. AU - D'Udekem, Y. C7 - 20170021 DB - Scopus DO - 10.1515/ijamh-2017-0021 KW - Fontan circulation transition from paediatric to adult care transition support programs M3 - Article in Press N1 - Cited By :2 Export Date: 15 June 2020 Article in Press PY - 2017 ST - Adolescent and parent perspectives prior to involvement in a Fontan transition program T2 - International Journal of Adolescent Medicine and Health TI - Adolescent and parent perspectives prior to involvement in a Fontan transition program UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85037619552&doi=10.1515%2fijamh-2017-0021&partnerID=40&md5=610491882f5e15e727f7e2087b3de22a ID - 1862 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 - Y. d'Udekem, The Royal Children's Hospital Melbourne, 3 West Clinical Offices, 50 Flemington Road, Parkville, Victoria, Australia 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. DB - Embase Medline DO - 10.1016/j.ijcard.2018.01.098 KW - adult article confusion (uncertainty) congenital heart disease death ethnicity exercise fear female financial management Fontan procedure health status human life expectancy major clinical study male physical capacity pregnancy priority journal quality of life self report LA - English M3 - Article N1 - L2000621178 2018-04-09 2018-10-31 PY - 2018 SN - 1874-1754 0167-5273 SP - 54-59 ST - “How long will I continue to be normal?” Adults with a Fontan circulation's greatest concerns T2 - International Journal of Cardiology TI - “How long will I continue to be normal?” Adults with a Fontan circulation's greatest concerns UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2000621178 http://dx.doi.org/10.1016/j.ijcard.2018.01.098 VL - 260 ID - 678 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 - Y. d'Udekem, The Royal Children's Hospital Melbourne, 3 West Clinical Offices, 50 Flemington Road, Parkville, Victoria, Australia 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. DB - Embase DO - 10.1016/j.ijcha.2018.02.008 KW - warfarin article bone density child emotional stability exhaustion fatigue Fontan procedure human international normalized ratio life expectancy major clinical study mental health pregnancy priority journal quality of life social psychology LA - English M3 - Article N1 - L621080065 2018-03-15 2018-03-20 PY - 2018 SN - 2352-9067 SP - 65-70 ST - “Will she live a long happy life?” Parents' concerns for their children with Fontan circulation T2 - IJC Heart and Vasculature TI - “Will she live a long happy life?” Parents' concerns for their children with Fontan circulation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L621080065 http://dx.doi.org/10.1016/j.ijcha.2018.02.008 VL - 18 ID - 699 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. © 2018 Elsevier B.V. AD - Murdoch Children's Research Institute, Melbourne, Victoria, Australia Australian and New Zealand Fontan Advisory Committee, Australia Cardiac Surgery Department, Royal Children's Hospital, Melbourne, Victoria, Australia Department of Pediatrics, Faculty of Medicine, The University of Melbourne, Melbourne, Victoria, Australia 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. DB - Scopus DO - 10.1016/j.ijcard.2018.01.098 KW - Adult congenital Congenital heart disease Fontan Qualitative research M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2018 SP - 54-59 ST - “How long will I continue to be normal?” Adults with a Fontan circulation's greatest concerns T2 - International Journal of Cardiology TI - “How long will I continue to be normal?” Adults with a Fontan circulation's greatest concerns UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85044785406&doi=10.1016%2fj.ijcard.2018.01.098&partnerID=40&md5=c7834bc7a239172b12d7495d48194f35 VL - 260 ID - 1798 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. © 2017 AD - Murdoch Children's Research Institute, Melbourne, Victoria, Australia Australian and New Zealand Fontan Advisory Committee, Australia Cardiac Surgery Department, Royal Children's Hospital, Melbourne, Victoria, Australia Department of Pediatrics, Faculty of Medicine, The University of Melbourne, Melbourne, Victoria, Australia 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. DB - Scopus DO - 10.1016/j.ijcha.2018.02.008 KW - Congenital heart disease Fontan Parents of children with a Fontan circulation Qualitative research M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2018 SP - 65-70 ST - “Will she live a long happy life?” Parents' concerns for their children with Fontan circulation T2 - IJC Heart and Vasculature TI - “Will she live a long happy life?” Parents' concerns for their children with Fontan circulation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85043397157&doi=10.1016%2fj.ijcha.2018.02.008&partnerID=40&md5=08b2ddec68f225d2ef67bd90d62dee7b VL - 18 ID - 1814 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 - Department of Child and Adolescent Psychiatry/Psychology, Erasmus Medical Centre-Sophia Children's Hospital Department of Paediatrics, Division of Cardiology, Erasmus Medical Centre - Sophia Children's Hospital, Rotterdam Department of Paediatrics, Division of Cardiology, Academic Medical Centre, Amsterdam Department of Paediatrics, Division of Cardiology, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands Department of Paediatrics, Pediatric Cardiology Unit, Tel-Aviv Sourasky Medical Centre, Tel Aviv, Israel Department of Paediatrics, Division of Cardiology, University Medical Centre Utrecht - Wilhelmina Children's Hospital, Utrecht Department of Paediatrics, Division of Cardiology, Leiden University Medical Centre, Leiden Child Development and Exercise Centre, University Medical Centre Utrecht - Wilhelmina Children's Hospital, Utrecht, the Netherlands AN - 115588329. Language: English. Entry Date: 20161229. Revision Date: 20170301. Publication Type: journal article AU - Dulfer, Karolijn AU - Bossers, Sjoerd S. M. AU - Utens, Elisabeth M. W. J. AU - Duppen, Nienke AU - Kuipers, Irene M. AU - Kapusta, Livia AU - van Iperen, Gabrielle AU - Schokking, Michiel AU - ten Harkel, Arend D. J. AU - Takken, Tim AU - Helbing, Willem A. DB - ccm DO - 10.1017/S1047951115000426 DP - EBSCOhost IS - 3 KW - Heart Ventricle -- Physiopathology Quality of Life Health Status Cardiopulmonary Bypass Heart Defects, Congenital -- Surgery Magnetic Resonance Imaging Parents Prospective Studies Child Human Treatment Outcomes Female Male Exercise Test Adolescence Netherlands Cross Sectional Studies Multivariate Analysis Validation Studies Comparative Studies Evaluation Research Multicenter Studies Scales N1 - research. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Instrumentation: Health-Related Hardiness Scale (HRHS). NLM UID: 9200019. PMID: NLM25906441. PY - 2016 SN - 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=115588329&site=ehost-live&scope=site VL - 26 ID - 1489 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. ©Cambridge University Press 2015. AD - Department of Child and Adolescent Psychiatry/Psychology, Erasmus Medical Centre, Sophia Children's Hospital, Rotterdam, Netherlands Department of Pediatric Cardiology, Division of Cardiology, Erasmus Medical Centre, Sophia Children's Hospital, PO Box 2060, Rotterdam, 3000 CB, Netherlands Department of Paediatrics, Division of Cardiology, Academic Medical Centre, Amsterdam, Netherlands Department of Paediatrics, Division of Cardiology, Radboud University, Nijmegen Medical Centre, Nijmegen, Netherlands Department of Paediatrics, Pediatric Cardiology Unit, Tel-Aviv Sourasky Medical Centre, Tel Aviv, Israel Department of Paediatrics, Division of Cardiology, University Medical Centre Utrecht, Wilhelmina Children's Hospital, Utrecht, Netherlands Department of Paediatrics, Division of Cardiology, Leiden University Medical Centre, Leiden, Netherlands Child Development and Exercise Centre, University Medical Centre Utrecht, Wilhelmina Children's Hospital, Utrecht, 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 - Scopus DO - 10.1017/S1047951115000426 IS - 3 KW - congenital heart disease Fontan circulation quality of life M3 - Article N1 - Cited By :13 Export Date: 15 June 2020 PY - 2016 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84928254328&doi=10.1017%2fS1047951115000426&partnerID=40&md5=acd0501e0e0bba33af5bd36c69858724 VL - 26 ID - 1918 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. © 2013 Wiley Periodicals, Inc. AD - Department of Child and Adolescent Psychiatry/Psychology, Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, Netherlands Department of Pediatrics, Division of Cardiology, Erasmus Medical Center-Sophia Children's hospital, Rotterdam, Netherlands Department of Pediatric Cardiology, Leiden University Medical Center, Leiden, Netherlands Department of Cardiology, Radboud University Nijmegen Medical Center, Nijmegen, 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 - Scopus DO - 10.1111/chd.12154 IS - 5 KW - Adolescents Aerobic Exercise Behavior Children Congenital Heart Disease Randomized Controlled Trial M3 - Article N1 - Cited By :12 Export Date: 15 June 2020 PY - 2014 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84927690309&doi=10.1111%2fchd.12154&partnerID=40&md5=1964968d10268bfd1211dc54717b478d VL - 9 ID - 1984 ER - TY - JOUR AB - Abstract: 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 AN - 103962969. Language: English. Entry Date: 20140626. Revision Date: 20150710. Publication Type: Journal Article AU - Dulfer, Karolijn AU - Duppen, Nienke AU - Kuipers, Irene M. AU - Schokking, Michiel AU - van Domburg, Ron T. AU - Verhulst, Frank C. AU - Helbing, Willem A. AU - Utens, Elisabeth M. W. J. DB - ccm DO - 10.1016/j.jadohealth.2013.12.010 DP - EBSCOhost IS - 1 KW - Heart Defects, Congenital -- Therapy Aerobic Exercises -- Evaluation -- In Adolescence Quality of Life -- Evaluation -- In Adolescence Human Randomized Controlled Trials Child Adolescence Young Adult Netherlands Male Female Secondary Analysis Descriptive Statistics Questionnaires Self Report P-Value Parents -- Psychosocial Factors N1 - research; randomized controlled trial. Journal Subset: Allied Health; Nursing; Peer Reviewed; Public Health; USA. Special Interest: Pediatric Care; Public Health. NLM UID: 9102136. PMID: NLM24518533. 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 - 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=103962969&site=ehost-live&scope=site VL - 55 ID - 1491 ER - TY - JOUR AB - Abstract: 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 AN - 96660775. Language: English. Entry Date: 20140626. Revision Date: 20191120. Publication Type: Article AU - Dulfer, Karolijn AU - Duppen, Nienke AU - Kuipers, Irene M. AU - Schokking, Michiel AU - van Domburg, Ron T. AU - Verhulst, Frank C. AU - Helbing, Willem A. AU - Utens, Elisabeth M. W. J. DB - ccm DO - 10.1016/j.jadohealth.2013.12.010 DP - EBSCOhost IS - 1 KW - Heart Defects, Congenital -- Therapy Aerobic Exercises -- Evaluation -- In Adolescence Quality of Life -- Evaluation -- In Adolescence Human Randomized Controlled Trials Child Adolescence Young Adult Netherlands Male Female Secondary Analysis Descriptive Statistics Questionnaires Self Report P-Value Parents -- Psychosocial Factors N1 - research; randomized controlled trial. Journal Subset: Allied Health; Nursing; Peer Reviewed; Public Health; USA. Special Interest: Pediatric Care. NLM UID: 9102136. 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 - 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=96660775&site=ehost-live&scope=site VL - 55 ID - 1492 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 10e15 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. (PsycINFO Database Record (c) 2017 APA, all rights reserved) AD - Utens, Elisabeth M. W. J., Erasmus MC, Sophia Children’s Hospital, Department of Child & Adolescent Psychiatry, P.O. Box 2060, 3000 CB, Rotterdam, Netherlands AN - 2014-05448-001 AU - Dulfer, Karolijn AU - Duppen, Nienke AU - Kuipers, Irene M. AU - Schokking, Michiel AU - van Domburg, Ron T. AU - Verhulst, Frank C. AU - Helbing, Willem A. AU - Utens, Elisabeth M. W. J. DB - psyh DO - 10.1016/j.jadohealth.2013.12.010 DP - EBSCOhost IS - 1 KW - aerobic exercise quality of life congenital heart disease clinical trials children Adolescent Adult Child Cognition Exercise Exercise Tolerance Female Fontan Procedure Humans Male Netherlands Prospective Studies Social Behavior Tetralogy of Fallot Young Adult Health Promotion Heart Disorders Childhood Development N1 - Department of Child and Adolescent Psychiatry, Erasmus Medical Center, Sophia Children’s Hospital, Rotterdam, Netherlands. Release Date: 20140217. Correction Date: 20170928. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Aerobic Exercise; Clinical Trials; Health Promotion; Heart Disorders; Quality of Life. Minor Descriptor: Childhood Development. Classification: Cardiovascular Disorders (3295); Promotion & Maintenance of Health & Wellness (3365). Population: Human (10); Male (30); Female (40). Location: Netherlands. Age Group: Childhood (birth-12 yrs) (100); School Age (6-12 yrs) (180); Adolescence (13-17 yrs) (200); Adulthood (18 yrs & older) (300); Young Adulthood (18-29 yrs) (320). Tests & Measures: Semistructured Interview; Child Quality of Life Questionnaire-Child Form; Linear Analogue Scale; 36-Item Short Form Health Survey-Dutch Version; Child Quality of Life Questionnaire-Parent Form; Web-Based Questionnaire. Methodology: Clinical Trial; Empirical Study; Followup Study; Interview; Quantitative Study. References Available: Y. Page Count: 8. Issue Publication Date: Jul, 2014. Publication History: Accepted Date: Dec 9, 2013; First Submitted Date: Sep 19, 2013. Copyright Statement: All rights reserved. Society for Adolescent Health and Medicine. 2014. Sponsor: Stichting Rotterdams Kinderrevalidatie Fonds Adriaanstichting. Recipients: No recipient indicated Sponsor: Netherlands Heart Foundation, Netherlands. Grant: 2008B026. Other Details: NHS. Recipients: No recipient indicated PY - 2014 SN - 1054-139X 1879-1972 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2014-05448-001&site=ehost-live&scope=site e.utens@erasmusmc.nl VL - 55 ID - 1668 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 - Department of Child and Adolescent Psychiatry/Psychology, Erasmus Medical Center, Sophia Children's Hospital, P.O. Box 2060, 3000 CB, Rotterdam, Netherlands Department of Paediatrics, Erasmus Medical Center, Sophia Children's Hospital, Rotterdam, Netherlands Department of Pediatric Cardiology, Academic Medical Center, Amsterdam, Netherlands Department of Pediatric Cardiology, Radboud University, Nijmegen Medical Centre, Nijmegen, Netherlands Department of Cardiology-Thoraxcenter, Erasmus Medical Center, Rotterdam, Netherlands Erasmus MC-Sophia Children's Hospital, Department of Child and Adolescent Psychiatry/Psychology, 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 - Scopus DO - 10.1016/j.jadohealth.2013.12.010 IS - 1 KW - Adolescents Aerobic exercise Children Congenital heart disease Quality of life Randomized controlled trial Young adults M3 - Article N1 - Cited By :31 Export Date: 15 June 2020 PY - 2014 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84903198728&doi=10.1016%2fj.jadohealth.2013.12.010&partnerID=40&md5=004399b6387fa3d18260d16f00171ae2 VL - 55 ID - 1998 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). © Springer Science+Business Media New York 2014. AD - Department of Child and Adolescent Psychiatry/Psychology, Erasmus Medical Center - Sophia Children’s Hospital, P.O. Box 2060, Rotterdam, 3000 CB, Netherlands Department of Paediatrics, Division of Cardiology, Erasmus Medical Center - Sophia Children’s Hospital, Rotterdam, Netherlands Department of Cardiology, Radboud University Nijmegen Medical Center, Nijmegen, Netherlands Department of Pediatric Cardiology, Emma Children’s Hospital, Academic Medical Center, Amsterdam, Netherlands Department of Cardiology - Thoraxcenter, Erasmus Medical Center, 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 - Scopus DO - 10.1007/s00246-014-0961-z IS - 1 KW - Adolescents Aerobic exercise Children Congenital heart defects Parents M3 - Article N1 - Cited By :8 Export Date: 15 June 2020 PY - 2015 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84926668510&doi=10.1007%2fs00246-014-0961-z&partnerID=40&md5=0161919f190fc49e380b71a5b1bd12aa VL - 36 ID - 1943 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 day 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, United States 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. DB - Scopus IS - 6 M3 - Article N1 - Cited By :20 Export Date: 15 June 2020 PY - 1987 SP - 334-342 ST - Pediatric heart transplantation at St. Christopher's Hospital for Children T2 - Journal of Heart Transplantation TI - Pediatric heart transplantation at St. Christopher's Hospital for Children UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0023502126&partnerID=40&md5=0284e7a0e82dd1e987f95d54f0ff3d63 VL - 6 ID - 2323 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 - Department of Pediatrics, Division of Cardiology, Erasmus Medical Center–Sophia Children's Hospital, Rotterdam, the Netherlands Department of Radiology, Erasmus Medical Center, Rotterdam, the Netherlands Child Development & Exercise Center, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, the Netherlands Department of Rehabilitation Medicine, Erasmus Medical Center, Rotterdam, the Netherlands Department of Cardiology, Thorax Center, Erasmus Medical Center, Rotterdam, the Netherlands Department of Pediatrics Cardiology, Radboud University Medical Center, Nijmegen, the Netherlands Department of Child and Adolescent Psychiatry/Psychology, Erasmus Medical Center–Sophia Children's Hospital, Rotterdam, the Netherlands Department of Integrative Physiology, Radboud University Medical Center, Nijmegen, the Netherlands AN - 109491919. Language: English. Entry Date: 20151217. Revision Date: 20151217. Publication Type: journal article. Journal Subset: Biomedical AU - Duppen, Nienke AU - Etnel, Jonathan R. AU - Spaans, Laura AU - Takken, Tim AU - van den Berg-Emons, Rita J. AU - Boersma, Eric AU - Schokking, Michiel AU - Dulfer, Karolijn AU - Utens, Elisabeth M. AU - Helbing, Willem AU - Hopman, Maria T. DB - ccm DO - 10.1016/j.ahj.2015.06.018 DP - EBSCOhost IS - 3 KW - Tetralogy of Fallot -- Surgery Postoperative Care -- Methods Activities of Daily Living Physical Fitness Cardiopulmonary Bypass -- Rehabilitation Therapeutic Exercise -- Methods Motor Activity -- Physiology Prospective Studies Male Tetralogy of Fallot -- Physiopathology Female Adult Treatment Outcomes Adolescence Tetralogy of Fallot -- Rehabilitation Child Young Adult Exercise of Self-Care Agency Scale N1 - USA. Instrumentation: Exercise of Self-Care Agency Scale (ESCA) (Kearney and Fleischer). NLM UID: 0370465. PMID: NLM26385046. PY - 2015 SN - 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=109491919&site=ehost-live&scope=site VL - 170 ID - 1511 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. Copyright © 2010 by ASME. AD - K. Pekkan, Department of Biomedical Engineering, Department of Mechanical Engineering, Carnegie Mellon University, Pittsburgh, PA 15219, United States AU - Dur, O. AU - DeGroff, C. G. AU - Keller, B. B. AU - Pekkan, K. DB - Embase Medline DO - 10.1115/1.4000954 IS - 3 KW - article breathing cavopulmonary connection computational fluid dynamics coronary artery blood flow Fontan procedure inferior cava vein nuclear magnetic resonance imaging process optimization real time polymerase chain reaction superior cava vein surgical technique waveform LA - English M3 - Article N1 - L359268883 2010-08-09 2010-08-18 http://asmedl.aip.org/getpdf/servlet/GetPDFServlet?filetype=pdf&id=JBENDY000132000003031012000001&idtype=cvips&prog=normal PY - 2010 SN - 0148-0731 1528-8951 ST - Optimization of inflow waveform phase-difference for minimized total cavopulmonary power loss T2 - Journal of Biomechanical Engineering TI - Optimization of inflow waveform phase-difference for minimized total cavopulmonary power loss UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L359268883 http://dx.doi.org/10.1115/1.4000954 VL - 132 ID - 1151 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. Copyright © 2010 by ASME. AD - Department of Biomedical Engineering, Carnegie Mellon University, Pittsburgh, PA 15219, United States Congenital Heart Center, University of Florida, Gainesville, FL 32610, United States Cardiovascular Innovation Institute, University of Louisville, Louisville, KY 40202, United States Department of Biomedical Engineering, Department of Mechanical Engineering, Carnegie Mellon University, Pittsburgh, PA 15219, United States AU - Dur, O. AU - DeGroff, C. G. AU - Keller, B. B. AU - Pekkan, K. DB - Scopus DO - 10.1115/1.4000954 IS - 3 M3 - Article N1 - Cited By :24 Export Date: 15 June 2020 PY - 2010 ST - Optimization of inflow waveform phase-difference for minimized total cavopulmonary power loss T2 - Journal of Biomechanical Engineering TI - Optimization of inflow waveform phase-difference for minimized total cavopulmonary power loss UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-77955111313&doi=10.1115%2f1.4000954&partnerID=40&md5=b984011c04caa027bd472b64e3b2b06b VL - 132 ID - 2157 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. © 2009, International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc. AD - K. Pekkan, Department of Biomedical and Mechanical (Courtesy) Engineering, School of Biomedical Engineering, Carnegie Mellon University, Pittsburgh, PA 15219, United States AU - Dur, O. AU - Lara, M. AU - Arnold, D. AU - Vandenberghe, S. AU - Keller, B. B. AU - Degroff, C. AU - Pekkan, K. DB - Embase Medline DO - 10.1111/j.1525-1594.2009.00951.x IS - 11 KW - article assisted circulation blood vessel compliance clinical decision making controlled study Fontan procedure heart assist device heart left ventricle pressure heart single ventricle hemodynamics hypertension in vitro study patient care postoperative period priority journal pulmonary hypertension pulsatile flow simulation vascular resistance vein blood flow Medos Pediaflow LA - English M3 - Article N1 - L355704357 2009-12-29 PY - 2009 SN - 0160-564X 1525-1594 SP - 967-976 ST - Pulsatile in vitro simulation of the pediatric univentricular circulation for evaluation of cardiopulmonary assist scenarios T2 - Artificial Organs TI - Pulsatile in vitro simulation of the pediatric univentricular circulation for evaluation of cardiopulmonary assist scenarios UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L355704357 http://dx.doi.org/10.1111/j.1525-1594.2009.00951.x VL - 33 ID - 1170 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. © 2009, International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc. AD - Department of Biomedical and Mechanical (Courtesy) Engineering, School of Biomedical Engineering, Carnegie Mellon University, Pittsburgh, PA 15219, United States ARTORG Cardiovascular Engineering, University of Bern, Bern, Switzerland Cardiovascular Innovation Institute, University of Louisville, Louisville, KY, United States Congenital Heart Center, University of Florida, Gainesville, FL, United States AU - Dur, O. AU - Lara, M. AU - Arnold, D. AU - Vandenberghe, S. AU - Keller, B. B. AU - Degroff, C. AU - Pekkan, K. DB - Scopus DO - 10.1111/j.1525-1594.2009.00951.x IS - 11 KW - Assisted circulation/methods Fontan procedure/methods Hemodynamics/ physiology Pulsatile flow Ventricle-assist devices/utilization M3 - Article N1 - Cited By :12 Export Date: 15 June 2020 PY - 2009 SP - 967-976 ST - Pulsatile in vitro simulation of the pediatric univentricular circulation for evaluation of cardiopulmonary assist scenarios T2 - Artificial Organs TI - Pulsatile in vitro simulation of the pediatric univentricular circulation for evaluation of cardiopulmonary assist scenarios UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-70450272579&doi=10.1111%2fj.1525-1594.2009.00951.x&partnerID=40&md5=e27c652021116343c3f02f9f86fcf6fa VL - 33 ID - 2167 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 - Queensland Paediatric Cardiac Service, Mater Health Services, Brisbane, QLD, Australia School of Population Health, University of Queensland, Brisbane, QLD, Australia School of Paediatric Medicine, University of Queensland, Brisbane, QLD, Australia AU - Eagleson, K. J. AU - Justo, R. N. AU - Ware, R. S. AU - Johnson, S. G. AU - Boyle, F. M. DB - Scopus DO - 10.1111/jpc.12296 IS - 10 KW - congenital heart disease paediatrics quality of life M3 - Article N1 - Cited By :23 Export Date: 15 June 2020 PY - 2013 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84886094379&doi=10.1111%2fjpc.12296&partnerID=40&md5=e53dfa7dc7c5626ad1c72def61200a04 VL - 49 ID - 2036 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 and14 (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 - H. El-Said, 3020 Children's Way, San Diego, CA, United States AU - Ebrahim, M. AU - Hagood, J. AU - Moore, J. AU - El-Said, H. DB - Embase Medline DO - 10.1002/ccd.25772 IS - 5 KW - adult aortic coarctation article balloon catheter balloon dilatation bronchial compression bronchoscopy bronchus obstruction case report child computer assisted tomography flexible bronchoscopy human hypoplastic left heart syndrome male pediatrics percutaneous transluminal angioplasty preschool child pulmonary artery catheterization vascular stent LA - English M3 - Article N1 - L601107439 2015-01-09 2015-04-01 PY - 2015 SN - 1522-726X 1522-1946 SP - 832-836 ST - Bronchoscopic guidance of endovascular stenting limits airway compression T2 - Catheterization and Cardiovascular Interventions TI - Bronchoscopic guidance of endovascular stenting limits airway compression UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L601107439 http://dx.doi.org/10.1002/ccd.25772 VL - 85 ID - 869 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 and14 (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. © 2014 Wiley Periodicals, Inc. © 2014 Wiley Periodicals, Inc. AD - Division of Cardiology, UCSD School of Medicine, Rady Children's Hospital, San Diego, CA, United States Department of Pediatrics, UCSD School of Medicine, Rady Children's Hospital, San Diego, CA, United States AU - Ebrahim, M. AU - Hagood, J. AU - Moore, J. AU - El-Said, H. DB - Scopus DO - 10.1002/ccd.25772 IS - 5 KW - branch pulmonary arteries bronchial compression bronchoscopy LPA stent M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 2015 SP - 832-836 ST - Bronchoscopic guidance of endovascular stenting limits airway compression T2 - Catheterization and Cardiovascular Interventions TI - Bronchoscopic guidance of endovascular stenting limits airway compression UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84925348360&doi=10.1002%2fccd.25772&partnerID=40&md5=7f36275739f4557da611002865b8db7b VL - 85 ID - 1948 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 - K. Baba, Department of Pediatrics, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan 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. DB - Embase Medline DO - 10.1111/ped.13485 IS - 3 KW - NCT01273857 NCT01829750 5F Launcher guiding catheter imaging system INFX 8000V Biplane Kudos adverse event age article balloon occlusion blood pressure body weight bradycardia cardiac muscle cell cardiosphere derived cell cardiovascular function cardiovascular procedure cell therapy cell transfer child clinical article controlled study coronary artery spasm depression female heart rate heart single ventricle human hypoplastic left heart syndrome hypotension male patient safety priority journal ST segment elevation stop flow technique transcoronary cell infusion treatment duration treatment outcome LA - English M3 - Article N1 - L621391714 2018-03-29 2018-08-09 PY - 2018 SN - 1442-200X 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://www.embase.com/search/results?subaction=viewrecord&from=export&id=L621391714 http://dx.doi.org/10.1111/ped.13485 VL - 60 ID - 702 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. © 2017 Japan Pediatric Society 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 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. DB - Scopus DO - 10.1111/ped.13485 IS - 3 KW - congenital heart disease regeneration therapy stop-flow technique temporary occlusion balloon transcoronary infusion M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2018 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85044292275&doi=10.1111%2fped.13485&partnerID=40&md5=54459f536971cc797b467a46c684102e VL - 60 ID - 1816 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 - B.M. Ekman-Joelsson, Pediatric and Adolescent Medical Care, Skaraborg Hospital, Skövde, Sweden. AU - Ekman-Joelsson, B. M. AU - Berntsson, L. AU - Sunnegårdh, J. DB - Medline IS - 6 KW - adolescent adult article child comparative study heart septum defect human infant pulmonary valve atresia preschool child psychological aspect quality of life questionnaire LA - English M3 - Article N1 - L40759728 2005-06-13 PY - 2004 SN - 1047-9511 SP - 615-621 ST - Quality of life in children with pulmonary atresia and intact ventricular septum T2 - Cardiology in the young TI - Quality of life in children with pulmonary atresia and intact ventricular septum UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L40759728 VL - 14 ID - 1287 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. © Cambridge University Press. AD - Paediatric and Adolescent Medical Care, Skaraborg Hospital, 541 85 Skövde, Sweden Nordic School of Public Health, Institute of Nursing, Göteborg University, Sweden Queen Silvia Children's Hospital, Göteborg University, Sweden AU - Ekman-Joelsson, B. M. AU - Berntsson, L. AU - Sunnegårdh, J. DB - Scopus DO - 10.1017/S1047951104006067 IS - 6 KW - Congenital heart defect Fontan circulation Hypoplastic right heart M3 - Article N1 - Cited By :31 Export Date: 15 June 2020 PY - 2004 SP - 615-621 ST - Quality of life in children with pulmonary atresia and intact ventricular septum T2 - Cardiology in the Young TI - Quality of life in children with pulmonary atresia and intact ventricular septum UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-19844370501&doi=10.1017%2fS1047951104006067&partnerID=40&md5=dc102a5874d14e11741c0e69fa3004b8 VL - 14 ID - 2248 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. Copyright © 2010 by the National Association of Neonatal Nurses. AD - M. K. Ellinger, Faculty of Nursing, University of Alberta, Clinical Sciences Bldg, Edmonton, AB T6G 2G3, Canada AU - Ellinger, M. K. AU - Rempel, G. R. DB - Medline DO - 10.1097/ANC.0b013e3181fc7c5d IS - 6 KW - adult aortic valve article child parent relation decision making female heart valve replacement human hypoplastic left heart syndrome male newborn parent pericardium psychological aspect pulmonary artery questionnaire transplantation LA - English M3 - Article N1 - L360124470 2011-04-08 PY - 2010 SN - 1536-0903 SP - 316-322 ST - Parental decision making regarding treatment of hypoplastic left heart syndrome T2 - Advances in Neonatal Care TI - Parental decision making regarding treatment of hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L360124470 http://dx.doi.org/10.1097/ANC.0b013e3181fc7c5d VL - 10 ID - 1127 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 - Harran University School of Medicine, Department of Pediatrics, 63100, Şanliurfa, Turkey Department of Pediatrics, Division of Pediatric Cardiology, Harran University School of Medicine, Şanliurfa, Turkey Department of Clinical Chemistry, Harran University School of Medicine, Şanliurfa, Turkey AU - Ercan, S. AU - Çakmak, A. AU - Kösecik, M. AU - Erel, Ö DB - Scopus IS - 6 KW - Acyanotic Child Congenital heart diseases Cyanotic Oxidative stress M3 - Article N1 - Cited By :26 Export Date: 15 June 2020 PY - 2009 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-74049094419&partnerID=40&md5=c0623af81b98981cc3b5ccc0d9d21a02 VL - 9 ID - 2165 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 - Acibadem Hospital Bakirkoy, Department of Cardiovascular Surgery, Bakirkoy, Istanbul, Turkey 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 - Scopus DO - 10.1055/s-2005-865872 IS - 1 KW - Atriopulmonary anastomosis Fontan conversion Fontan modifications Kawashima procedure M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 2006 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-32544448670&doi=10.1055%2fs-2005-865872&partnerID=40&md5=0f0f4845312ff309699c95f3d93ba0e0 VL - 54 ID - 2234 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. © 2012 Wiley Periodicals, Inc. AD - D.R. Ezmigna, Department of Pediatrics, University of Arizona, 1501 N Cambell Ave., Tucson, AZ 85724, United States AU - Ezmigna, D. R. AU - Morgan, W. J. AU - Witte, M. H. AU - Brown, M. A. DB - Embase Medline DO - 10.1002/ppul.22673 IS - 5 KW - fibrin mucin article bronchitis bronchoscopy case report child child health care congenital heart disease Fontan procedure heart disease human lymph flow lymphangiography lymphoscintigraphy lymphovenous anastomosis male pathogenesis plastic bronchitis pneumonia preschool child priority journal respiratory distress thoracic duct tracheobronchial tree LA - English M3 - Article N1 - L52195989 2012-09-07 2013-05-02 PY - 2013 SN - 8755-6863 1099-0496 SP - 515-518 ST - Lymphoscintigraphy in plastic bronchitis, A pediatric case report T2 - Pediatric Pulmonology TI - Lymphoscintigraphy in plastic bronchitis, A pediatric case report UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52195989 http://dx.doi.org/10.1002/ppul.22673 VL - 48 ID - 1003 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. © 2012 Wiley Periodicals, Inc. AD - Department of Pediatrics, University of Arizona, 1501 N Cambell Ave., Tucson, AZ 85724, United States Department of Surgery, University of Arizona, Tucson, AZ, United States AU - Ezmigna, D. R. AU - Morgan, W. J. AU - Witte, M. H. AU - Brown, M. A. DB - Scopus DO - 10.1002/ppul.22673 IS - 5 KW - congenital heart disease lymph leakage lymphoscintigraphy plastic bronchitis M3 - Article N1 - Cited By :8 Export Date: 15 June 2020 PY - 2013 SP - 515-518 ST - Lymphoscintigraphy in plastic bronchitis, A pediatric case report T2 - Pediatric Pulmonology TI - Lymphoscintigraphy in plastic bronchitis, A pediatric case report UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84876407010&doi=10.1002%2fppul.22673&partnerID=40&md5=39fc006591ccf94a6e00218d5ba8540a VL - 48 ID - 2050 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. AD - S.M. Feliciano AU - Feliciano, S. M. AU - Girard, N. J. AU - Calhoon, J. H. DB - Medline IS - 1 KW - article heart surgery human infant methodology newborn newborn nursing nursing operating room personnel patient care planning LA - English M3 - Article N1 - L23893594 1993-06-09 PY - 1992 SN - 1056-8670 SP - 37-50 ST - Case study: infant transplantation: a change of heart T2 - Seminars in perioperative nursing TI - Case study: infant transplantation: a change of heart UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L23893594 VL - 1 ID - 1385 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. AU - Feliciano, S. M. AU - Girard, N. J. AU - Calhoon, J. H. DB - Scopus IS - 1 M3 - Article N1 - Export Date: 15 June 2020 PY - 1992 SP - 37-50 ST - Case study: infant transplantation: a change of heart T2 - Seminars in perioperative nursing TI - Case study: infant transplantation: a change of heart UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0026617933&partnerID=40&md5=8d9ebcab3f3482e646e82fac7f58bc9a VL - 1 ID - 2314 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. © 2019, Arquivos Brasileiros de Cardiologia. All rights reserved. AD - Instituto do Coração (InCor), Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HCFMUSP), 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 - Scopus DO - 10.5935/abc.20180256 IS - 2 KW - Arrihythmias Cardiac/surgery Fontan procedure Fontan-Kreutzer prodedure Heart defects congenital/surgery Mortality M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2019 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85061866622&doi=10.5935%2fabc.20180256&partnerID=40&md5=9101b172b43a3fb620ba2033f7d409c8 VL - 112 ID - 1765 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. © 2019, Springer Science+Business Media, LLC, part of Springer Nature. AD - Division of Pediatric Cardiology, Department of Pediatrics, Stanford University, Palo Alto, CA, United States Division of Cardiovascular Medicine, Department of Medicine, Stanford University, Palo Alto, CA, United States Adult Congenital Heart Association, Philadelphia, PA, United States Department of Cardiology, Boston Children’s Hospital, Boston, MA, United States Knight Cardiovascular Institute, Oregon Health and Science University, Portland, OR, United States Division of Cardiology, Akron Children’s Hospital, Akron, OH, United States Division of Cardiology, Children’s Hospital of Minnesota, Minneapolis, MN, United States Division of Cardiology, Children’s Hospital of Los Angeles, Los Angeles, CA, United States Division of Cardiology, University of California Los Angeles, Los Angeles, CA, United States Department of Medicine, Stanford University, Quantitative Sciences Unit, Palo Alto, CA, United States Division of Cardiology, Nationwide Children’s Hospital, Columbus, OH, United States Division of Cardiology, Massachusetts General Hospital, Boston, MA, United States Montreal Heart Institute, Montreal, QC, Canada Division of Cardiology, Phoenix Children’s Hospital, Phoenix, AZ, United States Stanford University School of Medicine, 150 Governor’s Lane, T242, Stanford, CA 94305, 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. AU - The Adult Congenital Heart, Association AU - the Adult Congenital Cardiac Care Associate Research, Group DB - Scopus DO - 10.1007/s00246-019-02154-8 IS - 7 KW - Adult congenital heart disease Congenital heart disease Health care transition Pediatric cardiology M3 - Article N1 - Export Date: 15 June 2020 PY - 2019 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85069897714&doi=10.1007%2fs00246-019-02154-8&partnerID=40&md5=e1fb647e80fd668049f170feb6eb73c2 VL - 40 ID - 1731 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 - 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 - Scopus DO - 10.1097/00003072-199708000-00013 IS - 8 KW - Congenital Heart Defects Pulmonary Atresia Technetium Tc-99m Sestamibi M3 - Article N1 - Export Date: 15 June 2020 PY - 1997 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0031430950&doi=10.1097%2f00003072-199708000-00013&partnerID=40&md5=a8a49b22de6118ddb15bc8018a1530f5 VL - 22 ID - 2295 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. © 2014 Wiley Periodicals, Inc. AD - Center for Cardiovascular and Pulmonary Research, The Research Institute at Nationwide Children's Hospital, Columbus, OH, United States Department of Pediatrics, College of Medicine, The Ohio State University, Columbus, OH, United States Center for Innovation in Pediatric Practice, The Research Institute at Nationwide Children's Hospital, Columbus, OH, United States AU - Fitzgerald-Butt, S. M. AU - Klima, J. AU - Kelleher, K. AU - Chisolm, D. AU - McBride, K. L. DB - Scopus DO - 10.1002/ajmg.a.36763 IS - 12 KW - And practice Attitudes Congenital Genetic testing Health knowledge Heart defects Multifactorial inheritance M3 - Article N1 - Cited By :10 Export Date: 15 June 2020 PY - 2014 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84911086206&doi=10.1002%2fajmg.a.36763&partnerID=40&md5=ec072cfaa94c7ee21b9e0c754e274a5e VL - 164 ID - 1980 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. © 2017 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies. AD - Department of Congenital Heart Disease and Pediatric Cardiology, University Heart Centre Freiburg, Freiburg, Germany Department of Cardiovascular Surgery, University Heart Centre Freiburg, Freiburg, Germany Department of Neuropediatric and Muscle Disorders, University Medical Center, Freiburg, Germany 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 - Scopus DO - 10.1097/PCC.0000000000001019 IS - 2 KW - Children congenital heart disease extracorporeal membrane oxygenation health-related quality of life mechanical circulatory support M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2017 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84995450721&doi=10.1097%2fPCC.0000000000001019&partnerID=40&md5=55fac53339f98be707301c1a88ed48ce VL - 18 ID - 1873 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 - Division of Cardiology, Children's Hospital of Philadelphia, 34th St. and Civic Center Blvd., Philadelphia, PA 19104, United States AU - Fogel, M. A. AU - Gupta, K. B. AU - Weinberg, P. M. AU - Hoffman, E. A. DB - Scopus DO - 10.1152/ajpheart.1995.269.3.h1132 IS - 3 38-3 KW - congenital heart disease contraction magnetic resonance imaging twist M3 - Article N1 - Cited By :69 Export Date: 15 June 2020 PY - 1995 SP - H1132-H1152 ST - Regional wall motion and strain analysis across stages of Fontan reconstruction by magnetic resonance tagging T2 - American Journal of Physiology - Heart and Circulatory Physiology TI - Regional wall motion and strain analysis across stages of Fontan reconstruction by magnetic resonance tagging UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0029094595&doi=10.1152%2fajpheart.1995.269.3.h1132&partnerID=40&md5=4165861b353a17f3687685f3657f63cb VL - 269 ID - 2306 ER - TY - JOUR AB - Background: Ventricular-ventricular interaction is known to occur in the 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 - Division of Pediatric Cardiology, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA, United States Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA, United States Children's Hospital of Philadelphia, Division of Cardiology, 34th St and Civic Center Blvd, Philadelphia, PA 19104, United States AU - Fogel, M. A. AU - Weinberg, P. M. AU - Fellows, K. E. AU - Huffman, E. A. DB - Scopus DO - 10.1161/01.CIR.92.2.219 IS - 2 KW - contraction Fontan procedure transposition of the great arteries ventricles M3 - Article N1 - Cited By :116 Export Date: 15 June 2020 PY - 1995 SP - 219-230 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0029053073&doi=10.1161%2f01.CIR.92.2.219&partnerID=40&md5=02edb35b38e1191040a51e36814a7170 VL - 92 ID - 2301 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 - J.M. Forbess, Department of Cardiovascular Surgery, Children's Hospital, 300 Longwood Ave, Boston, MA 02115, United States AU - Forbess, J. M. AU - Visconti, K. J. AU - Bellinger, D. C. AU - Jonas, R. A. DB - Embase Medline IS - SUPPL. 1 KW - article clinical article controlled study developmental disorder follow up Fontan procedure human intelligence quotient neuropsychology postoperative complication preschool child priority journal treatment outcome LA - English M3 - Article N1 - L32884790 2001-10-05 PY - 2001 SN - 0009-7322 SP - i127-i132 ST - Neurodevelopmental outcomes in children after the Fontan operation T2 - Circulation TI - Neurodevelopmental outcomes in children after the Fontan operation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L32884790 VL - 104 ID - 1321 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, 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 - J.M. Forbess, Department of Cardiovascular Surgery, Children's Hospital, 300 Longwood Ave, Boston, MA 02115, United States AU - Forbess, J. M. AU - Visconti, K. J. AU - Hancock-Friesen, C. AU - Howe, R. C. AU - Bellinger, D. C. AU - Jonas, R. A. DB - Embase Medline IS - 13 SUPPL. KW - adolescent article child congenital heart disease female heart arrest heart surgery human intelligence quotient intensive care learning major clinical study male memory motor performance nerve cell differentiation neuropsychological test outcomes research priority journal register risk factor social status velocardiofacial syndrome verbal behavior LA - English M3 - Article N1 - L35101515 2002-10-22 PY - 2002 SN - 0009-7322 SP - I95-I102 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L35101515 VL - 106 ID - 1313 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 - Department of Paediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München an der Technischen Universität München, Munich, Germany Department of Radiology, 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. C7 - 46 DB - Scopus DO - 10.1186/1532-429X-10-46 IS - 1 M3 - Article N1 - Cited By :32 Export Date: 15 June 2020 PY - 2008 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-58749097222&doi=10.1186%2f1532-429X-10-46&partnerID=40&md5=5e53be8c202e1e5b88982c22d57c9467 VL - 10 ID - 2188 ER - TY - JOUR AB - Background Infants who require extracorporeal membrane oxygenation (ECMO) support after a Norwood operation are at increased risk for early and late death compared with patients who do not require ECMO post-Norwood. Little is known about the effect that ECMO post-Norwood has on functional status and quality of life among long-term survivors. Methods We prospectively evaluated functional status and health-related quality of life in 12 surviving patients (cases) and 19 corresponding patients (controls) from a previous retrospective case-control assessment of long-term survival in patients requiring ECMO post-Norwood. Functional status was assessed with the Vineland Adaptive Behavior Scale-II, and health-related quality of life was assessed with the Pediatric Quality of Life Inventory (PedsQL) core and cardiac modules. Results There were no differences in demographics, extracardiac or genetic anomalies, or age at follow-up assessment between ECMO cases and non-ECMO controls. The Vineland Adaptive Behavior Scale-II scores were comparable between groups, with both groups demonstrating function in the normal range in all four domains tested. The only difference in PedsQL scores between cases and controls was perceived physical appearance, which was lower among ECMO survivors by both patient and proxy report. PedsQL scores of both groups were comparable to published scores for patients with single-ventricle congenital heart disease but generally lower than scores for the healthy population. Conclusions The requirement for ECMO support after a Norwood operation does not appear to significantly affect functional status or quality of life among the subset of patients who achieve long-term survival. AD - J.M. Friedland-Little, Heart Center, Seattle Children's Hospital, RC.2.820, PO Box 5371, 4800 Sand Point Way NE, Seattle, WA, United States AU - Friedland-Little, J. M. AU - Uzark, K. AU - Yu, S. AU - Lowery, R. AU - Aiyagari, R. AU - Hirsch-Romano, J. C. DB - Embase Medline DO - 10.1016/j.athoracsur.2016.11.015 IS - 6 KW - adolescent article child clinical article controlled clinical trial controlled study extracorporeal oxygenation female functional assessment functional status human long term survival male Norwood procedure physical appearance priority journal prospective study quality of life quality of life assessment self evaluation social interaction LA - English M3 - Article N1 - L614488845 2017-02-23 2017-05-31 PY - 2017 SN - 1552-6259 0003-4975 SP - 1950-1955 ST - Functional Status and Quality of Life in Survivors of Extracorporeal Membrane Oxygenation After the Norwood Operation T2 - Annals of Thoracic Surgery TI - Functional Status and Quality of Life in Survivors of Extracorporeal Membrane Oxygenation After the Norwood Operation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L614488845 http://dx.doi.org/10.1016/j.athoracsur.2016.11.015 VL - 103 ID - 752 ER - TY - JOUR AB - Background Infants who require extracorporeal membrane oxygenation (ECMO) support after a Norwood operation are at increased risk for early and late death compared with patients who do not require ECMO post-Norwood. Little is known about the effect that ECMO post-Norwood has on functional status and quality of life among long-term survivors. Methods We prospectively evaluated functional status and health-related quality of life in 12 surviving patients (cases) and 19 corresponding patients (controls) from a previous retrospective case-control assessment of long-term survival in patients requiring ECMO post-Norwood. Functional status was assessed with the Vineland Adaptive Behavior Scale-II, and health-related quality of life was assessed with the Pediatric Quality of Life Inventory (PedsQL) core and cardiac modules. Results There were no differences in demographics, extracardiac or genetic anomalies, or age at follow-up assessment between ECMO cases and non-ECMO controls. The Vineland Adaptive Behavior Scale-II scores were comparable between groups, with both groups demonstrating function in the normal range in all four domains tested. The only difference in PedsQL scores between cases and controls was perceived physical appearance, which was lower among ECMO survivors by both patient and proxy report. PedsQL scores of both groups were comparable to published scores for patients with single-ventricle congenital heart disease but generally lower than scores for the healthy population. Conclusions The requirement for ECMO support after a Norwood operation does not appear to significantly affect functional status or quality of life among the subset of patients who achieve long-term survival. © 2017 The Society of Thoracic Surgeons AD - Division of Pediatric Cardiology, University of Washington School of Medicine, Seattle Children's Hospital, Seattle, Washington, United States Division of Pediatric Cardiology, Department of Pediatrics and Communicable Disease, University of Michigan, Ann Arbor, Michigan, United States Section of Pediatric Cardiac Surgery, Department of Cardiac Surgery, University of Michigan, Ann Arbor, Michigan, United States AU - Friedland-Little, J. M. AU - Uzark, K. AU - Yu, S. AU - Lowery, R. AU - Aiyagari, R. AU - Hirsch-Romano, J. C. DB - Scopus DO - 10.1016/j.athoracsur.2016.11.015 IS - 6 M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2017 SP - 1950-1955 ST - Functional Status and Quality of Life in Survivors of Extracorporeal Membrane Oxygenation After the Norwood Operation T2 - Annals of Thoracic Surgery TI - Functional Status and Quality of Life in Survivors of Extracorporeal Membrane Oxygenation After the Norwood Operation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85013076667&doi=10.1016%2fj.athoracsur.2016.11.015&partnerID=40&md5=2d585e3d52fae6fee1a21200ffcf99b4 VL - 103 ID - 1859 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 - K.G. Friedman, Department of Cardiology, Children’s Hospital Boston, Boston, MA, United States AU - Friedman, K. G. AU - Freud, L. AU - Escobar-Diaz, M. AU - Banka, P. AU - Emani, S. AU - Tworetzky, W. DB - Embase Medline DO - 10.1007/s00246-015-1193-6 IS - 7 KW - aortic regurgitation aortic valve replacement aortic valve repair article biventricular circulation cardiovascular risk child clinical article cohort analysis diastolic dysfunction female fetal aortic valvuloplasty fetus follow up heart disease heart left ventricle ejection fraction heart left ventricle enddiastolic volume heart left ventricle function heart left ventricle hypertrophy heart left ventricle mass heart left ventricle volume heart ventricle remodeling human hypoplastic left heart syndrome male mitral valve regurgitation LA - English M3 - Article N1 - L604431549 2015-05-26 2015-09-29 PY - 2015 SN - 1432-1971 0172-0643 SP - 1502-1509 ST - Left Ventricular Remodeling and Function in Children with Biventricular Circulation After Fetal Aortic Valvuloplasty T2 - Pediatric Cardiology TI - Left Ventricular Remodeling and Function in Children with Biventricular Circulation After Fetal Aortic Valvuloplasty UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L604431549 http://dx.doi.org/10.1007/s00246-015-1193-6 VL - 36 ID - 865 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. © 2015, Springer Science+Business Media New York. AD - Department of Cardiology, Children’s Hospital Boston, Boston, MA 02115, United States Department of Pediatrics, Harvard Medical School, Boston, MA, United States AU - Friedman, K. G. AU - Freud, L. AU - Escobar-Diaz, M. AU - Banka, P. AU - Emani, S. AU - Tworetzky, W. DB - Scopus DO - 10.1007/s00246-015-1193-6 IS - 7 KW - Aortic stenosis Diastolic dysfunction Fetal cardiology Left ventricular remodeling M3 - Article N1 - Cited By :11 Export Date: 15 June 2020 PY - 2015 SP - 1502-1509 ST - Left Ventricular Remodeling and Function in Children with Biventricular Circulation After Fetal Aortic Valvuloplasty T2 - Pediatric Cardiology TI - Left Ventricular Remodeling and Function in Children with Biventricular Circulation After Fetal Aortic Valvuloplasty UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84941943228&doi=10.1007%2fs00246-015-1193-6&partnerID=40&md5=71392f78d575e9a1bed26387b6f52ad5 VL - 36 ID - 1938 ER - TY - JOUR AB - 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). 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. 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. 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. Copyright © 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved. AD - S. Fuller, Division of Cardiothoracic Surgery, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA. 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 - Medline IS - 6 KW - article child cognition comparative study congenital heart malformation deep hypothermic circulatory arrest developmental disorder female follow up human incidence infant male motor activity newborn physiology postoperative period preschool child prognosis prospective study risk factor time United States LA - English M3 - Article N1 - L360254535 2011-03-08 PY - 2010 SN - 1552-6259 SP - 1985-1994; discussion 1994-1995 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 - The Annals of thoracic surgery 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L360254535 VL - 90 ID - 1130 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 - The Heart Center, Nationwide Children's Hospital, Columbus, OH, United States AU - Galantowicz, M. DB - Scopus DO - 10.1053/j.pcsu.2013.01.005 IS - 1 M3 - Article N1 - Cited By :13 Export Date: 15 June 2020 PY - 2013 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84875789846&doi=10.1053%2fj.pcsu.2013.01.005&partnerID=40&md5=d5c607604a00738204cb98e9eac80b6d VL - 16 ID - 2052 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. © 2011 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies. AD - N.N. Gandhi, Department of Pediatrics, Duke University Medical Center, Duke Children's Hospital, Durham, NC, United States AU - Gandhi, N. N. AU - Hartman, M. E. AU - Williford, W. L. AU - Peters, M. A. AU - Cheifetz, I. M. AU - Turner, D. A. DB - Embase Medline DO - 10.1097/PCC.0b013e3181fe3189 IS - 6 KW - epinephrine antibiotic agent hydrocortisone nitric oxide oseltamivir vasopressin adult respiratory distress syndrome airway pressure arteriovenous shunt article artificial ventilation case report child extracorporeal oxygenation Fontan procedure hemodialysis human hypoplastic left heart syndrome hypoxemia influenza A (H1N1) lung function lung resistance male oxygen saturation polymerase chain reaction positive end expiratory pressure priority journal school child subdural hematoma LA - English M3 - Article N1 - L51171505 2010-12-02 2011-11-24 PY - 2011 SN - 1529-7535 1947-3893 SP - e398-e401 ST - Successful use of extracorporeal membrane oxygenation for pH1N1-induced refractory hypoxemia in a child with hypoplastic left heart syndrome T2 - Pediatric Critical Care Medicine TI - Successful use of extracorporeal membrane oxygenation for pH1N1-induced refractory hypoxemia in a child with hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51171505 http://dx.doi.org/10.1097/PCC.0b013e3181fe3189 VL - 12 ID - 1086 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 - From the Division of Pediatric Critical Care Medicine (NNG, MEH, IMC, DAT), Department of Pediatrics, Duke Children's Hospital, Duke University Medical Center, Durham, NC; and Respiratory Care Services (WLW, MAP, IMC), Duke University Hospital, Duke University Medical Center, Durham, NC. AN - 108205703. Language: English. Entry Date: 20120203. Revision Date: 20170411. Publication Type: journal article AU - Gandhi, Niyati N. AU - Hartman, Mary E. AU - Williford, Walter L. AU - Peters, Michelle A. AU - Cheifetz, Ira M. AU - Turner, David A. DB - ccm DO - 10.1097/PCC.0b013e3181fe3189 DP - EBSCOhost IS - 6 KW - Extracorporeal Membrane Oxygenation -- Evaluation Hypoplastic Left Heart Syndrome -- Therapy -- In Infancy and Childhood Influenza, Pandemic (H1N1) 2009 -- Complications -- In Infancy and Childhood Child Intensive Care Units, Pediatric Male N1 - case study. Journal Subset: Biomedical; Peer Reviewed; USA. Special Interest: Critical Care; Pediatric Care. NLM UID: 100954653. PMID: NLM21116209. PY - 2011 SN - 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 - Pediatric Critical Care Medicine TI - Successful use of extracorporeal membrane oxygenation for pH1N1-induced refractory hypoxemia in a child with hypoplastic left heart syndrome UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=108205703&site=ehost-live&scope=site VL - 12 ID - 1625 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. © 2011 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies. AD - Department of Pediatrics, Duke University Medical Center, Duke Children's Hospital, Durham, NC, United States Respiratory Care Services, Duke University Hospital, Duke University Medical Center, Durham, NC, United States AU - Gandhi, N. N. AU - Hartman, M. E. AU - Williford, W. L. AU - Peters, M. A. AU - Cheifetz, I. M. AU - Turner, D. A. DB - Scopus DO - 10.1097/PCC.0b013e3181fe3189 IS - 6 KW - acute lung injury acute respiratory distress syndrome congenital heart disease extracorporeal membrane oxygenation Fontan procedure H1N1 influenza hypoplastic left heart syndrome hypoxemia mechanical ventilation pediatric pneumonia M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2011 SP - e398-e401 ST - Successful use of extracorporeal membrane oxygenation for pH1N1-induced refractory hypoxemia in a child with hypoplastic left heart syndrome T2 - Pediatric Critical Care Medicine TI - Successful use of extracorporeal membrane oxygenation for pH1N1-induced refractory hypoxemia in a child with hypoplastic left heart syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-80053344453&doi=10.1097%2fPCC.0b013e3181fe3189&partnerID=40&md5=7f72cb276a042897ad517039a01114bb VL - 12 ID - 2135 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 - Department of Surgery, Washington University School of Medicine, St. Louis Children's Hospital, St. Louis, MO, United States Department of Pediatrics, 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 - Scopus DO - 10.1016/j.athoracsur.2006.08.016 IS - 1 M3 - Article N1 - Cited By :8 Export Date: 15 June 2020 PY - 2007 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-33845477491&doi=10.1016%2fj.athoracsur.2006.08.016&partnerID=40&md5=08d031101507a38947e09bf42cea7271 VL - 83 ID - 2217 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 - Department of Pediatrics, University of Alberta, 3A3.07 Stollery Children's Hospital, 8440-112 St, Edmonton, AB T6G 2B7, Canada Pediatric Rehabilitation Outcomes Evaluation and Research Unit, Glenrose Rehabilitation Hospital, Edmonton, AB, Canada University of Alberta School of Public Health, Edmonton, AB, Canada University of Calgary, Faculty of Social Work, Calgary, AB, Canada Department of Surgery, University of Alberta, Edmonton, AB, 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 - Scopus DO - 10.1016/j.jtcvs.2012.03.050 IS - 2 M3 - Article N1 - Cited By :27 Export Date: 15 June 2020 PY - 2013 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84872351547&doi=10.1016%2fj.jtcvs.2012.03.050&partnerID=40&md5=adb945c7dbc7d78881afebaaf9d0feea VL - 145 ID - 2067 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 - K.L. Gaskin, Department of Nursing, Midwifery and Paramedic Science, Institute of Health and Society, University of Worcester, Henwick Grove, Worcester, United Kingdom AU - Gaskin, K. L. AU - Barron, D. J. AU - Daniels, A. DB - Embase Medline DO - 10.1017/S1047951116001062 IS - 7 KW - adult aged article clinical assessment tool clinical practice Congenital Heart Assessment Tool deductive thematic analysis emotion fear female first stage cardiac surgery general aspects of disease health education heart single ventricle heart surgery home monitoring hospital discharge human infant male medical information middle aged mixed emotion online analysis parental attitude parental education parental preparedness qualitative analysis signs of deterioration social support thematic analysis treatment outcome young adult LA - English M3 - Article N1 - L611320994 2016-07-26 2016-09-30 PY - 2016 SN - 1467-1107 1047-9511 SP - 1414-1424 ST - Parents' preparedness for their infants' discharge following first-stage cardiac surgery: Development of a parental early warning tool T2 - Cardiology in the Young TI - Parents' preparedness for their infants' discharge following first-stage cardiac surgery: Development of a parental early warning tool UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L611320994 http://dx.doi.org/10.1017/S1047951116001062 VL - 26 ID - 793 ER - TY - JOUR AB - Unlabelled: 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 - Department of Nursing, Midwifery and Paramedic Science, Institute of Health and Society, University of Worcester, Henwick Grove, Worcester, United Kingdom Department of Cardiac Surgery, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, United Kingdom Cardiac Unit, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, United Kingdom AN - 118188099. Language: English. Entry Date: 20170228. Revision Date: 20190612. Publication Type: journal article AU - Gaskin, Kerry L. AU - Barron, David J. AU - Daniels, Amanda DB - ccm DO - 10.1017/S1047951116001062 DP - EBSCOhost IS - 7 KW - Heart Surgery Parents -- Education Patient Discharge Hypoplastic Left Heart Syndrome -- Nursing Parents -- Psychosocial Factors Home Health Care -- Standards Child, Preschool Middle Age Adult Infant, Newborn Male Attitude to Health Female Young Adult Hypoplastic Left Heart Syndrome -- Surgery Infant Human N1 - research; tables/charts. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. NLM UID: 9200019. PMID: NLM27431411. PY - 2016 SN - 1047-9511 SP - 1414-1424 ST - Parents' preparedness for their infants' discharge following first-stage cardiac surgery: development of a parental early warning tool T2 - Cardiology in the Young TI - Parents' preparedness for their infants' discharge following first-stage cardiac surgery: development of a parental early warning tool UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=118188099&site=ehost-live&scope=site VL - 26 ID - 1512 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. © Cambridge University Press 2016. AD - Department of Nursing, Midwifery and Paramedic Science, Institute of Health and Society, University of Worcester, Henwick Grove, Worcester, WR2 6AJ, United Kingdom Department of Cardiac Surgery, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, United Kingdom Cardiac Unit, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, United Kingdom AU - Gaskin, K. L. AU - Barron, D. J. AU - Daniels, A. DB - Scopus DO - 10.1017/S1047951116001062 IS - 7 KW - CHD family heart defects Hypoplastic left heart syndrome infants parents patient discharge M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 2016 SP - 1414-1424 ST - Parents' preparedness for their infants' discharge following first-stage cardiac surgery: Development of a parental early warning tool T2 - Cardiology in the Young TI - Parents' preparedness for their infants' discharge following first-stage cardiac surgery: Development of a parental early warning tool UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84978680775&doi=10.1017%2fS1047951116001062&partnerID=40&md5=e1b9dfc82630748ea6a8cb532322f06f VL - 26 ID - 1890 ER - TY - JOUR AD - Cardiologie pédiatrique et congénitale – M3C, CHU de Montpellier, France UMR Cnrs 9214 – Inserm U1046, Université de Montpellier, France Physiologie, CHU de Montpellier, Montpellier, France Cardiologie et réhabilitation pédiatrique, Institut-Saint-Pierre, Palavas-Les-Flots, France Cardiologie adulte, CHU de Montpellier, Montpellier, France 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 - Scopus DO - 10.1016/S1878-6480(16)30570-5 IS - 5 M3 - Article N1 - Export Date: 15 June 2020 PY - 2016 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84994908810&doi=10.1016%2fS1878-6480%2816%2930570-5&partnerID=40&md5=2988d8bef58af139f35f1c6e8b19c913 VL - 8 ID - 1893 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. © 2010 by The American Association for Thoracic Surgery. AD - J. W. Gaynor, Division of Cardiothoracic Surgery, Children's Hospital of Philadelphia, 34th and Civic Center Boulevard, Philadelphia, PA 19104, United States 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. DB - Embase Medline DO - 10.1016/j.jtcvs.2010.07.069 IS - 6 KW - apolipoprotein E article child cognition controlled study deep hypothermic circulatory arrest diagnostic procedure education ethnicity evaluation study Fallot tetralogy female genetics genotype gestational age great vessels transposition heart surgery heart ventricle septum defect human hypoplastic left heart syndrome infancy infant language length of stay major clinical study male memory nerve cell differentiation outcome assessment preschool child priority journal protein polymorphism reading social status speech visuomotor coordination LA - English M3 - Article N1 - L51111529 2010-10-19 2010-12-01 PY - 2010 SN - 0022-5223 SP - 1230-1236 ST - Is cardiac diagnosis a predictor of neurodevelopmental outcome after cardiac surgery in infancy? T2 - Journal of Thoracic and Cardiovascular Surgery TI - Is cardiac diagnosis a predictor of neurodevelopmental outcome after cardiac surgery in infancy? UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51111529 http://dx.doi.org/10.1016/j.jtcvs.2010.07.069 VL - 140 ID - 1132 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. © 2014 The American Association for Thoracic Surgery 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, Children's Hospital of Philadelphia, 34th and Civic Center Boulevard, Philadelphia, PA 19104, United States Department of Medicine (Division of Medical Genetics), University of Washington School of Medicine, Seattle, WA, United States Primary Children's Medical Center, Salt Lake City, UT, United States Division of Pediatric Cardiology, Medical University of South Carolina, Charleston, SC, United States Department of Neurology, Boston Children's Hospital, Boston, MA, United States Division of Medical Genetics, Department of Medicine, University of Washington School of Medicine, Seattle, WA, United States Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, United States Johns Hopkins Children's Heart Institute, All Children's Hospital, Florida Hospital for Children, St Petersburg, FL, United States Department of Pediatrics, Columbia University Medical Center, New York, NY, United States Children's Hospital Los Angeles, Los Angeles, CA, United States Children's Healthcare of Atlanta, Atlanta, GA, United States Ann and Robert F. Lurie Children's Hospital of Chicago, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Division of Pediatric Oncology, Duke University Medical Center, Durham, NC, United States Department of Cardiology, Boston Children's Hospital, Boston, MA, United States Nemours Cardiac Center, Alfred I. Dupont Hospital for Children, Wilmington, DE, United States Division of Pediatric Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA, United States Department of Pathology and Laboratory Medicine, Children's Hospital of Philadelphia, Philadelphia, PA, United States Department of Anesthesia, University of Michigan Medical School, Ann Arbor, MI, United States Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada Department of Pediatrics and Communicable Diseases (Division of Pediatric Cardiology), University of Michigan Medical School, Ann Arbor, MI, United States Department of Neurology, Harvard Medical School, Boston, MA, United States 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. DB - Scopus DO - 10.1016/j.jtcvs.2014.07.052 IS - 6 M3 - Article N1 - Cited By :33 Export Date: 15 June 2020 PY - 2014 SP - 2560-2568 ST - Validation of association of the apolipoprotein e ε2 allele with neurodevelopmental dysfunction after cardiac surgery in neonates and infants T2 - Journal of Thoracic and Cardiovascular Surgery TI - Validation of association of the apolipoprotein e ε2 allele with neurodevelopmental dysfunction after cardiac surgery in neonates and infants UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84920024512&doi=10.1016%2fj.jtcvs.2014.07.052&partnerID=40&md5=d3f70aced5fc5100bf15189418982006 VL - 148 ID - 2029 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 - M. Gerstle, Division of Behavioral Medicine and Clinical Psychology, Cincinnati Children's Hospital Medical Center (CCHMC), 3333 Burnet Ave, MLC 3015, Cincinnati, OH, United States AU - Gerstle, M. AU - Beebe, D. W. AU - Drotar, D. AU - Cassedy, A. AU - Marino, B. S. DB - Embase Medline DO - 10.1016/j.jpeds.2016.01.028 KW - academic achievement adolescent adverse outcome article child competence congenital heart disease controlled study cross-sectional study disease severity executive function female heart injury heart single ventricle human intelligence quotient learning disorder major clinical study male metacognition predictive value prevalence priority journal quality of life survivor LA - English M3 - Article N1 - L610582639 2016-06-08 2016-06-16 PY - 2016 SN - 1097-6833 0022-3476 SP - 154-159 ST - Executive functioning and school performance among pediatric survivors of complex congenital heart disease T2 - Journal of Pediatrics TI - Executive functioning and school performance among pediatric survivors of complex congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L610582639 http://dx.doi.org/10.1016/j.jpeds.2016.01.028 VL - 173 ID - 806 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 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 - 115337519. Language: English. Entry Date: 20170604. Revision Date: 20180626. Publication Type: journal article AU - Gerstle, Melissa AU - Beebe, Dean W. AU - Drotar, Dennis AU - Cassedy, Amy AU - Marino, Bradley S. DB - ccm DO - 10.1016/j.jpeds.2016.01.028 DP - EBSCOhost KW - Heart Defects, Congenital -- Physiopathology Educational Status Executive Function Quality of Life Adolescence Survivors Female Child Cross Sectional Studies Male Funding Source Human N1 - research. Journal Subset: Biomedical; Peer Reviewed; USA. Grant Information: UL1 TR001425/TR/NCATS NIH HHS/United States. NLM UID: 0375410. PMID: NLM26875011. PY - 2016 SN - 0022-3476 SP - 154-159 ST - Executive Functioning and School Performance among Pediatric Survivors of Complex Congenital Heart Disease T2 - Journal of Pediatrics TI - Executive Functioning and School Performance among Pediatric Survivors of Complex Congenital Heart Disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=115337519&site=ehost-live&scope=site VL - 173 ID - 1495 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. (PsycINFO Database Record (c) 2017 APA, all rights reserved) AD - Gerstle, Melissa, Division of Behavioral Medicine and Clinical Psychology, Cincinnati Children’s Hospital Medical Center (CCHMC), 3333 Burnet Ave., MLC 3015, Cincinnati, OH, US, 45229 AN - 2016-26542-044 AU - Gerstle, Melissa AU - Beebe, Dean W. AU - Drotar, Dennis AU - Cassedy, Amy AU - Marino, Bradley S. DB - psyh DO - 10.1016/j.jpeds.2016.01.028 DP - EBSCOhost KW - cardiac cognitive child scholastic neuropsychology Cognitive Ability Heart Disorders Performance Survivors Pediatrics N1 - Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, US. Release Date: 20171109. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Conference Information: Scientific Session and Expo of the American College of Cardiology, Apr, 2011, New Orleans, LA, US. Conference Note: Portions of the study were presented at the aforementioned conference. Major Descriptor: Cognitive Ability; Heart Disorders; Performance; Survivors. Minor Descriptor: Pediatrics. Classification: Cardiovascular Disorders (3295). Population: Human (10); Male (30); Female (40). Age Group: Childhood (birth-12 yrs) (100); School Age (6-12 yrs) (180); Adolescence (13-17 yrs) (200). Tests & Measures: Parent-Report Version of the Behavior Rating Inventory of Executive Function; Child Behavior Checklist; Wechsler Intelligence Scale for Children, Fourth Edition DOI: 10.1037/t15174-000. Methodology: Empirical Study; Followup Study; Quantitative Study. Page Count: 6. Issue Publication Date: Jun, 2016. Publication History: Accepted Date: Jan 8, 2016; Revised Date: Nov 16, 2015; First Submitted Date: Aug 27, 2015. Copyright Statement: All rights reserved. Elsevier Inc. 2016. Sponsor: Cincinnati Children Hospital Medical Center, Heart Institute Research Core, US. Recipients: No recipient indicated Sponsor: National Institutes of Health, Clinical Translational Research Center, US. Grant: UL1 RR026314. Recipients: No recipient indicated PY - 2016 SN - 0022-3476 1097-6833 SP - 154-159 ST - Executive functioning and school performance among pediatric survivors of complex congenital heart disease T2 - The Journal of Pediatrics TI - Executive functioning and school performance among pediatric survivors of complex congenital heart disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2016-26542-044&site=ehost-live&scope=site melissa.gerstle@cchmc.org VL - 173 ID - 1670 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. © 2016 Elsevier Inc. All rights reserved. AD - Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, United States Division of Behavioral Medicine and Clinical Psychology, Cincinnati Children's Hospital Medical Center (CCHMC), 3333 Burnet Ave, MLC 3015, Cincinnati, OH 45229, United States Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Department of Sociology, McMicken College of Arts and Science, University of Cincinnati, Cincinnati, OH, United States Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, IL, United States Divisions of Cardiology and Critical Care Medicine, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, United States AU - Gerstle, M. AU - Beebe, D. W. AU - Drotar, D. AU - Cassedy, A. AU - Marino, B. S. DB - Scopus DO - 10.1016/j.jpeds.2016.01.028 KW - cardiac child cognitive neuropsychology scholastic M3 - Article N1 - Cited By :29 Export Date: 15 June 2020 PY - 2016 SP - 154-159 ST - Executive functioning and school performance among pediatric survivors of complex congenital heart disease T2 - Journal of Pediatrics TI - Executive functioning and school performance among pediatric survivors of complex congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84971413431&doi=10.1016%2fj.jpeds.2016.01.028&partnerID=40&md5=e7727005f16da51eb8890c7bc7f594c1 VL - 173 ID - 1903 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 results 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 - Cardiothoracic Unit, Hospital for Sick Children, London, United Kingdom Division of Paediatric Cardiology, Vanderbilt Medical Center North, Nashville, TN, United States Department of Paediatric Cardiology, Hospital for Sick Children, Great Ormond Street, London, WC1 3JH, United Kingdom AU - Gewillig, M. H. AU - Lundström, U. R. AU - Deanfield, J. E. AU - Bull, C. AU - Franklin, R. C. AU - Graham Jr, T. P. AU - Wyse, R. K. DB - Scopus DO - 10.1161/01.CIR.81.1.118 IS - 1 KW - Contractility Fontan procedure Volume loading M3 - Article N1 - Cited By :78 Export Date: 15 June 2020 PY - 1990 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0025022573&doi=10.1161%2f01.CIR.81.1.118&partnerID=40&md5=19e92e645a773e9e64773246f948961d VL - 81 ID - 2319 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 - Medical Sociology Unit, Hannover Medical School, 30635 Hannover, Germany Department of Paediatric Cardiology and Intensive Care, Hannover Medical School, 30635 Hannover, Germany Department of Paediatric and Adolescent Medicine, Caritas Hospital, Bad Mergentheim, Germany AU - Geyer, S. AU - Norozi, K. AU - Zoege, M. AU - Kempa, A. AU - Buchhorn, R. AU - Wessel, A. DB - Scopus DO - 10.1017/S1047951106001442 IS - 6 KW - Body image Cardiac performance Gender Limitations of activity M3 - Article N1 - Cited By :16 Export Date: 15 June 2020 PY - 2006 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-33751200638&doi=10.1017%2fS1047951106001442&partnerID=40&md5=953aec0b8ce3dd86bdcb0aad3aff5c49 VL - 16 ID - 2218 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/BSA1.3 versus 84 mL/BSA1.3, 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. 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. DB - Medline DO - 10.1161/CIRCIMAGING.117.006738 IS - 7 KW - adolescent child comparative study congenital heart malformation diagnostic imaging exercise test female Fontan procedure heart catheterization heart contraction heart left ventricle function heart right ventricle function heart transplantation heart ventricle heart ventricle remodeling human male mortality nuclear magnetic resonance imaging pathophysiology retrospective study risk factor time factor treatment outcome young adult LA - English M3 - Article N1 - L628966288 2019-08-23 2019-09-02 PY - 2018 SN - 1942-0080 SP - e006738 ST - Impact of Ventricular Morphology on Fiber Stress and Strain in Fontan Patients T2 - Circulation. Cardiovascular imaging TI - Impact of Ventricular Morphology on Fiber Stress and Strain in Fontan Patients UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L628966288 http://dx.doi.org/10.1161/CIRCIMAGING.117.006738 VL - 11 ID - 677 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/BSA1.3 versus 84 mL/BSA1.3, 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. © 2018 American Heart Association, Inc. AD - Department of Cardiology, Boston Children's Hospital, MA. Department of Pediatrics, Harvard Medical School, Boston, MA, United States 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. DB - Scopus DO - 10.1161/CIRCIMAGING.117.006738 IS - 7 KW - blood pressure dilatation heart transplantation prevalence M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2018 SP - e006738 ST - Impact of Ventricular Morphology on Fiber Stress and Strain in Fontan Patients T2 - Circulation. Cardiovascular imaging TI - Impact of Ventricular Morphology on Fiber Stress and Strain in Fontan Patients UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85070820045&doi=10.1161%2fCIRCIMAGING.117.006738&partnerID=40&md5=9a51cf3d24354cb23cc400652907d2ee VL - 11 ID - 1796 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. © 2016, Springer Science+Business Media Dordrecht. AD - Department of Cardiology, Boston Children’s Hospital, 300 Longwood Ave, Boston, MA 02115, United States Department of Pediatrics, Harvard Medical School, Boston, MA, United States AU - Ghelani, S. J. AU - Harrild, D. M. AU - Gauvreau, K. AU - Geva, T. AU - Rathod, R. H. DB - Scopus DO - 10.1007/s10554-016-0882-4 IS - 7 KW - Congenital heart disease Fontan Single ventricle Strain M3 - Article N1 - Cited By :12 Export Date: 15 June 2020 PY - 2016 SP - 1113-1120 ST - Echocardiography and magnetic resonance imaging based strain analysis of functional single ventricles: a study of intra- and inter-modality reproducibility T2 - International Journal of Cardiovascular Imaging TI - Echocardiography and magnetic resonance imaging based strain analysis of functional single ventricles: a study of intra- and inter-modality reproducibility UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84964375066&doi=10.1007%2fs10554-016-0882-4&partnerID=40&md5=266a21dfd234fdc3267c8d02df8158e7 VL - 32 ID - 1898 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 < 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 - S. Giannico, Pediatric Cardiology Department, Bambino Gesu' Hospital, Piazza Sant' Onofrio, 4, I-00165 Rome, Italy AU - Giannico, S. AU - Santoro, G. AU - Marino, B. AU - Cicini, M. P. AU - Marcelletti, C. DB - Embase Medline IS - 4 KW - adolescent adult anastomosis article child congenital heart disease cyanotic heart disease female heart left ventricle enddiastolic volume human infant major clinical study male LA - English M3 - Article N1 - L22285650 1992-09-30 PY - 1992 SN - 0340-9937 SP - 234-241 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L22285650 VL - 17 ID - 1383 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 < 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 - Pediatric Cardiology Department, Bambino Gesu' Hospital, Piazza Sant' Onofrio, 4, I-00165 Rome, Italy AU - Giannico, S. AU - Santoro, G. AU - Marino, B. AU - Cicini, M. P. AU - Marcelletti, C. DB - Scopus IS - 4 M3 - Article N1 - Cited By :9 Export Date: 15 June 2020 PY - 1992 SP - 234-241 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0026640371&partnerID=40&md5=a5804287035efb11867133933dd151c1 VL - 17 ID - 2315 ER - TY - JOUR AD - Nemours Cardiac Center, A. I. DuPont Hospital for Children, Wilmington, Delaware, United States AU - Gidding, S. S. DB - Scopus DO - 10.1016/j.jpeds.2012.03.017 IS - 3 M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2012 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84872011889&doi=10.1016%2fj.jpeds.2012.03.017&partnerID=40&md5=f501bb63b3ceacea14be55e5e9f093ed VL - 161 ID - 2084 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. Copyright © 2013 by The American Association for Thoracic Surgery. AD - Departments of Bioengineering and Surgery, Cardiovascular Innovation Institute, University of Louisville, Louisville, KY, United States School of Mechanical Engineering, Purdue University, Lafayette, IN, United States Department of Surgery, Indiana University School of Medicine, Emerson Hall 215, 545 Barnhill Dr., Indianapolis, IN 46202, United States 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. DB - Scopus DO - 10.1016/j.jtcvs.2012.01.082 IS - 1 M3 - Article N1 - Cited By :45 Export Date: 15 June 2020 PY - 2013 SP - 249-257 ST - Performance evaluation of a pediatric viscous impeller pump for Fontan cavopulmonary assist T2 - Journal of Thoracic and Cardiovascular Surgery TI - Performance evaluation of a pediatric viscous impeller pump for Fontan cavopulmonary assist UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84871210711&doi=10.1016%2fj.jtcvs.2012.01.082&partnerID=40&md5=3ef616b33fb5f968d8e10f85083cd492 VL - 145 ID - 2070 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 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. © 1986, The Society of Thoracic Surgeons. All rights reserved. AD - Departments of Cardiac Surgery and Pediatric Cardiology, Children's Hospital, Departments of Surgery and Pediatrics, Harvard Medical School, Boston, Massachusetts, United States AU - Gold, J. P. AU - Jonas, R. A. AU - Lang, P. AU - Elixson, E. M. AU - Mayer, J. E. AU - Castaneda, A. R. DB - Scopus DO - 10.1016/S0003-4975(10)60515-2 IS - 2 M3 - Article N1 - Cited By :60 Export Date: 15 June 2020 PY - 1986 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0022449944&doi=10.1016%2fS0003-4975%2810%2960515-2&partnerID=40&md5=d6fd4684968118140bcdfa965f338f91 VL - 42 ID - 2329 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 (R² = 0.32). Independent predictors of worse Behavior Assessment System for Children 2 (R² = 0.0700.20) and Pediatric Quality of Life Inventory 4.0 (R² = 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 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 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 AN - 139633696. Language: English. Entry Date: 20191115. Revision Date: 20191116. Publication Type: Article AU - Goldberg, Caren S. AU - Chenwei, Hu AU - Brosig, Cheryl AU - Gaynor, J. William AU - Mahle, William T. AU - Miller, Thomas AU - Mussatto, Kathleen A. AU - Sananes, Renee AU - Uzark, Karen AU - Trachtenberg, Felicia AU - Pizarro, Christian AU - Pemberton, Victoria L. AU - Lewis, Alan B. AU - S. Li, Jennifer AU - Jacobs, Jeffrey P. AU - Cnota, James AU - Atz, Andrew M. AU - Lai, Wyman W. AU - Bellinger, David AU - Newburger, Jane W. DB - ccm DO - 10.1542/peds.2019-1010 DP - EBSCOhost IS - 5 KW - Child Behavior -- Evaluation Quality of Life -- Evaluation Hypoplastic Left Heart Syndrome -- Surgery Functional Assessment Pediatric Care Heart Surgery -- Methods Surgery, Reconstructive -- Methods Human Treatment Outcomes Risk Assessment Scales Questionnaires Wilcoxon Rank Sum Test Multiple Regression Motor Skills Perioperative Care Extracorporeal Membrane Oxygenation -- Evaluation Male Perfusion -- Evaluation Catheterization Vision Disorders -- Diagnosis Seizures -- Diagnosis Morbidity N1 - research; tables/charts. Journal Subset: Biomedical; Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. Instrumentation: Vineland Adaptive Behavior Scale (VABS); Behavior Assessment System for Children, Second Edition (BASC-2); Vineland Adaptive Behavior Scales-II (VABS-II); Pediatric Quality of Life Inventory 4.0 (PedsQL 4.0). NLM UID: 0376422. PY - 2019 SN - 0031-4005 SP - 1-9 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=139633696&site=ehost-live&scope=site VL - 144 ID - 1433 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 6 17 vs 100 6 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 (R2 = 0.32). Independent predictors of worse Behavior Assessment System for Children 2 (R2 = 0.07-0.20) and Pediatric Quality of Life Inventory 4.0 (R2 = 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 - C.S. Goldberg, Division of Pediatric Cardiology, C.S. Mott Children's Hospital, University of Michigan, 1540 E Hospital Dr, Ann Arbor, MI, United States 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. DB - Embase Medline DO - 10.1542/peds.2019-1010 IS - 5 KW - article behavior assessment brain perfusion catheterization child child behavior demography extracorporeal oxygenation female follow up functional status functional status assessment human hypoplastic left heart syndrome major clinical study male morbidity Pediatric Quality of Life Inventory perioperative period prediction preschool child priority journal quality of life quality of life assessment rating scale risk factor scoring system sex social status LA - English M3 - Article N1 - L2004336679 2019-12-30 2020-01-01 PY - 2019 SN - 1098-4275 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2004336679 http://dx.doi.org/10.1542/peds.2019-1010 VL - 144 ID - 590 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 6 17 vs 100 6 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 (R2 = 0.32). Independent predictors of worse Behavior Assessment System for Children 2 (R2 = 0.07-0.20) and Pediatric Quality of Life Inventory 4.0 (R2 = 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. © 2019 by the American Academy of Pediatrics. AD - Department of Pediatrics, Medical School, University of Michigan and Michigan Medicine, Ann Arbor, MI, United States New England Research Institutes, Watertown, MA, United States Department of Pediatrics, Medical College of Wisconsin, Children's Hospital of Wisconsin, Milwaukee, WI, United States School of Medicine, University of Pennsylvania, Children's Hospital of Philadelphia, Philadelphia, PA, United States Department of Pediatrics, Emory University, Children's Healthcare of Atlanta, Atlanta, GA, United States Department of Pediatrics, University of Utah, Primary Children's Hospital, Salt Lake City, UT, United States Labatt Family Heart Center, Hospital for Sick Children, Toronto, Canada Department of Surgery, Nemours Cardiac Center, Nemours/ Alfred I. DuPont Hospital for Children, Wilmington, DE, United States National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, United States Department of Pediatrics, Children's Hospital Los Angeles, Los Angeles, CA, United States Department of Pediatrics, Duke University, Durham, NC, United States Department of Pediatrics, East Carolina University, Greenville, NC, United States Department of Pediatrics, Wake Forest University, Winston-Salem, NC, United States Congenital Heart Institute of Florida, St Petersburg, FL, United States Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Department of Pediatrics, Medical University of South Carolina, Charleston, SC, United States Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States Department of Cardiology, Boston Children's Hospital, Boston, MA, United States Department of Pediatrics, NewYork-Presbyterian Morgan Stanley Children's Hospital, New York, NY, United States 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. C7 - e20191010 DB - Scopus DO - 10.1542/peds.2019-1010 IS - 5 M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2019 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85074445715&doi=10.1542%2fpeds.2019-1010&partnerID=40&md5=9c7c1a98c39badc874d1c0100acda2ae VL - 144 ID - 1725 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. Copyright © 2014 Elsevier Inc. All rights reserved. AD - C.S. Goldberg, Department of Pediatrics, University of Michigan, C.S. Mott Children's Hospital, 1540 E Hospital Drive, Ann Arbor, MI 48109-4204, United States 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. DB - Embase Medline DO - 10.1016/j.jpeds.2014.05.019 IS - 3 KW - article child child growth child nutrition functional status hearing disorder heart right ventricle failure heart transplantation human major clinical study medical history nerve cell differentiation nervous system malformation preschool child priority journal quality of life visual disorder LA - English M3 - Article N1 - L53199674 2014-06-26 2014-09-09 PY - 2014 SN - 1097-6833 0022-3476 SP - 490-496.e8 ST - Factors associated with neurodevelopment for children with single ventricle lesions T2 - Journal of Pediatrics TI - Factors associated with neurodevelopment for children with single ventricle lesions UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L53199674 http://dx.doi.org/10.1016/j.jpeds.2014.05.019 VL - 165 ID - 959 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. AN - 103842322. Corporate Author: Pediatric Heart Network Investigators. Language: English. Entry Date: 20150123. Revision Date: 20190517. Publication Type: journal article AU - Goldberg, Caren S. AU - Lu, Minmin AU - Sleeper, Lynn A. AU - Mahle, William T. AU - Gaynor, J. William AU - Williams, Ismee A. AU - Mussatto, Kathleen A. AU - Ohye, Richard G. AU - Graham, Eric M. AU - Frank, Deborah U. AU - Jacobs, Jeffrey P. AU - Krawczeski, Catherine AU - Lambert, Linda AU - Lewis, Alan AU - Pemberton, Victoria L. AU - Sananes, Renee AU - Sood, Erica AU - Wechsler, Stephanie B. AU - Bellinger, David C. AU - Newburger, Jane W. DB - ccm DO - 10.1016/j.jpeds.2014.05.019 DP - EBSCOhost IS - 3 KW - Developmental Disabilities -- Etiology Heart Defects, Congenital -- Complications Heart Defects, Congenital -- Surgery Heart Ventricle -- Abnormalities Heart Ventricle -- Surgery Nervous System Child, Preschool Scales Female Human Infant Male Prospective Studies Questionnaires Funding Source N1 - research; randomized controlled trial. Journal Subset: Biomedical; Peer Reviewed; USA. Special Interest: Pediatric Care. Instrumentation: Bayley Scales of Infant Development-Second Edition (BSID-II); Ages and Stages Questionnaire (ASQ). Grant Information: U10 HL109737/HL/NHLBI NIH HHS/United States. NLM UID: 0375410. PMID: NLM24952712. PY - 2014 SN - 0022-3476 SP - 490-496.e8 ST - Factors associated with neurodevelopment for children with single ventricle lesions T2 - Journal of Pediatrics TI - Factors associated with neurodevelopment for children with single ventricle lesions UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=103842322&site=ehost-live&scope=site VL - 165 ID - 1624 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. (PsycINFO Database Record (c) 2016 APA, all rights reserved) AD - Goldberg, Caren S., Department of Pediatrics, University of Michigan, C.S. Mott Children’s Hospital, 1540 E Hospital Drive, Ann Arbor, MI, US, 48109-4204 AN - 2014-36070-026 AU - Goldberg, Caren S. AU - Lu, Minmin AU - Sleeper, Lynn A. AU - Mahle, William T. AU - Gaynor, J. William AU - Williams, Ismee A. AU - Mussatto, Kathleen A. AU - Ohye, Richard G. AU - Graham, Eric M. AU - Frank, Deborah U. AU - Jacobs, Jeffrey P. AU - Krawczeski, Catherine AU - Lambert, Linda AU - Lewis, Alan AU - Pemberton, Victoria L. AU - Sananes, Renee AU - Sood, Erica AU - Wechsler, Stephanie B. AU - Bellinger, David C. AU - Newburger, Jane W. DB - psyh DO - 10.1016/j.jpeds.2014.05.019 DP - EBSCOhost IS - 3 KW - neurodevelopment disorders single ventricle lesions childhood development quality of life Child, Preschool Developmental Disabilities Female Heart Defects, Congenital Heart Ventricles Humans Infant Male Nervous System Prospective Studies Lesions Neural Development Neurodevelopmental Disorders Morbidity Sequelae N1 - University of Michigan Medical School, Ann Arbor, MI, US. Institutional Authors: Pediatric Heart Network Investigators. Release Date: 20150202. Correction Date: 20151207. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Childhood Development; Lesions; Neural Development; Quality of Life; Neurodevelopmental Disorders. Minor Descriptor: Morbidity; Sequelae. Classification: Neurological Disorders & Brain Damage (3297). Population: Human (10); Male (30); Female (40). Location: US. Age Group: Childhood (birth-12 yrs) (100); Preschool Age (2-5 yrs) (160). Tests & Measures: Ages and Stages Questionnaire; Bayley Scales of Infant Development, Second Edition; Parent-Report Behavior Assessment System for Children, Second Edition; Pediatric Quality of Life Inventory; Functional Status, 2nd Edition Revised; Behavior Assessment System for Children--Second Edition DOI: 10.1037/t15079-000. Methodology: Empirical Study; Longitudinal Study; Prospective Study; Interview; Quantitative Study. References Available: Y. Page Count: 7. Issue Publication Date: Sep, 2014. Publication History: Accepted Date: May 9, 2014; Revised Date: Mar 19, 2014; First Submitted Date: Nov 25, 2013. Copyright Statement: All rights reserved. Elsevier Inc. 2014. Sponsor: National Heart, Lung, and Blood Institute, US. Grant: HL068269; HL068270; HL068279; HL068281; HL068285; HL068292; HL068290; HL068288; HL085057; HL109781; HL109737. Recipients: No recipient indicated PY - 2014 SN - 0022-3476 SP - 490-496 ST - Factors associated with neurodevelopment for children with single ventricle lesions T2 - The Journal of Pediatrics TI - Factors associated with neurodevelopment for children with single ventricle lesions UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2014-36070-026&site=ehost-live&scope=site cgoldber@umich.edu VL - 165 ID - 1673 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. Copyright © 2014 Elsevier Inc. All rights reserved. AD - University of Michigan Medical School, CS Mott Children's Hospital, Ann Arbor, MI, United States New England Research Institute, Watertown, MA, United States Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, GA, United States Childrens Hospital of Philadelphia, Philadelphia, PA, United States Columbia University, Morgan Stanley Children's Hospital, New York, NY, United States Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI, United States Medical University of South Carolina, Charleston, SC, United States University of Utah, Primary Children's Medical Center, Salt Lake City, UT, United States All Children's Hospital, Johns Hopkins Medicine, St Petersburg, FL, United States Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Children's Hospital of Los Angeles, Los Angeles, CA, United States National Heart, Lung and Blood Institute, Bethesda, MD, United States Hospital for Sick Children, Toronto, ON, Canada Alfred I. DuPont Hospital for Children, Wilmington, DE, United States Duke University Medical Center, Durham, NC, United States Boston Children's Hospital, Boston, MA, United States 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. DB - Scopus DO - 10.1016/j.jpeds.2014.05.019 IS - 3 M3 - Article N1 - Cited By :50 Export Date: 15 June 2020 PY - 2014 SP - 490-496.e8 ST - Factors associated with neurodevelopment for children with single ventricle lesions T2 - Journal of Pediatrics TI - Factors associated with neurodevelopment for children with single ventricle lesions UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84906794554&doi=10.1016%2fj.jpeds.2014.05.019&partnerID=40&md5=b75961a25ab0afe0221c79939861b67d VL - 165 ID - 2022 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 - 104497362. Language: English. Entry Date: 20120831. Revision Date: 20161119. Publication Type: journal article AU - Goldberg, Caren S. AU - Mussatto, Kathleen AU - Licht, Daniel AU - Wernovsky, Gil DB - ccm DO - 10.1017/S104795111100165X DP - EBSCOhost IS - S2 KW - Child Behavior Child Development Hypoplastic Left Heart Syndrome -- Psychosocial Factors Nervous System Quality of Life Child Prospective Studies Human N1 - research. Supplement Title: Dec2011 Supplement 2. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Special Interest: Pediatric Care. Grant Information: R01 NS072338/NS/NINDS NIH HHS/United States. NLM UID: 9200019. PMID: NLM22152534. PY - 2011 SN - 1047-9511 SP - 88-92 ST - Neurodevelopment and quality of life for children with hypoplastic left heart syndrome: current knowns and unknowns T2 - Cardiology in the Young TI - Neurodevelopment and quality of life for children with hypoplastic left heart syndrome: current knowns and unknowns UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104497362&site=ehost-live&scope=site VL - 21 ID - 1552 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 - C.S. Goldberg, Pediatric Cardiology, C.S. Mott Children's Hospital, Box 204, 1500 E. Medical Center Dr, Ann Arbor, MI 48109-0204, United States 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. DB - Embase Medline DO - 10.1067/mpd.2000.108952 IS - 5 KW - article brain development child child behavior Fontan procedure heart arrest heart single ventricle human hypoplastic left heart syndrome intelligence test major clinical study prediction priority journal seizure socioeconomics LA - English M3 - Article N1 - L30840122 2000-12-02 PY - 2000 SN - 0022-3476 SP - 646-652 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 - Journal of Pediatrics TI - Neurodevelopmental outcome of patients after the Fontan operation: A comparison between children with hypoplastic left heart syndrome and other functional single ventricle lesions UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L30840122 http://dx.doi.org/10.1067/mpd.2000.108952 VL - 137 ID - 1328 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 - Cardiac Center, Children's Hospital of Philadelphia, Division of Cardiology, 34th Street and Civic Center Boulevard, Philadelphia, PA 19146, United States Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States AU - Goldberg, D. J. AU - Dodds, K. AU - Rychik, J. DB - Scopus DO - 10.1017/S1047951111001636 IS - SUPPL. 2 KW - follow-up Fontan functionally univentricular heart single ventricle M3 - Article N1 - Cited By :6 Export Date: 15 June 2020 PY - 2011 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-83755163959&doi=10.1017%2fS1047951111001636&partnerID=40&md5=efb443cf7cd3d5d4225eb94bb43fdc76 VL - 21 ID - 2108 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. © 2011 American Heart Association, Inc. AD - J. Rychik, Division of Cardiology, Children's Hospital of Philadelphia, 34th and Civic Center Blvd, Philadelphia, PA 19104, United States 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. C1 - revatio(Pfizer,United Arab Emirates) C2 - Pfizer(United Arab Emirates) DB - Embase Medline DO - 10.1161/CIRCULATIONAHA.110.981746 IS - 11 KW - brain natriuretic peptide carbon dioxide oxygen placebo sildenafil abdominal pain adolescent adult anaerobic threshold article clinical article constipation crossover procedure double blind procedure exercise female flushing Fontan procedure headache human lung capacity male oxygen consumption priority journal protein blood level randomized controlled trial side effect revatio LA - English M3 - Article N1 - L51310070 2011-03-11 2011-04-05 PY - 2011 SN - 0009-7322 1524-4539 SP - 1185-1193 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51310070 http://dx.doi.org/10.1161/CIRCULATIONAHA.110.981746 VL - 123 ID - 1109 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. © 2011 American Heart Association, Inc. AD - Division of Cardiology, Children's Hospital of Philadelphia, 34th and Civic Center Blvd, Philadelphia, PA 19104, United States Departments of Pediatrics, United States Departments of Biostatistics and Epidemiology, University of Pennsylvania School of Medicine, Philadelphia, United States Divisions of Cardiology and Critical Care Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States 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. DB - Scopus DO - 10.1161/CIRCULATIONAHA.110.981746 IS - 11 KW - exercise Fontan procedure physiology trials M3 - Article N1 - Cited By :179 Export Date: 15 June 2020 PY - 2011 SP - 1185-1193 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-79953055063&doi=10.1161%2fCIRCULATIONAHA.110.981746&partnerID=40&md5=7db1e38fbf8999728191cd73c63bba5f VL - 123 ID - 2131 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 Division of Cardiology, The Children's Hospital of Philadelphia, 34th and Civic Center Blvd, Philadelphia, PA 19104. rychik@email.chop.edu. AN - 104853110. Language: English. Entry Date: 20110603. Revision Date: 20161222. Publication Type: journal article 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. AU - Goldberg, David J. AU - French, Benjamin AU - McBride, Michael G. AU - Marino, Bradley S. AU - Mirarchi, Nicole AU - Hanna, Brian D. AU - Wernovsky, Gil AU - Paridon, Stephen M. AU - Rychik, Jack DB - ccm DO - 10.1161/CIRCULATIONAHA.110.981746 DP - EBSCOhost IS - 11 KW - Cardiopulmonary Bypass Exercise Heart Defects, Congenital -- Surgery Heterocyclic Compounds -- Therapeutic Use Phosphodiesterase Inhibitors -- Therapeutic Use Sulfones -- Therapeutic Use Administration, Oral Adolescence Adult Child Crossover Design Double-Blind Studies Female Heart Defects, Congenital -- Physiopathology Heterocyclic Compounds -- Adverse Effects Human Male Natriuretic Peptide, Brain -- Blood Oxygen Consumption -- Drug Effects Purines -- Adverse Effects Purines -- Therapeutic Use Randomized Controlled Trials Sulfones -- Adverse Effects Young Adult N1 - research; randomized controlled trial. Journal Subset: Biomedical; Peer Reviewed; USA. Grant Information: HL07915/HL/NHLBI NIH HHS/United States. NLM UID: 0147763. PMID: NLM21382896. PY - 2011 SN - 0009-7322 SP - 1185-1193 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104853110&site=ehost-live&scope=site VL - 123 ID - 1559 ER - TY - JOUR AD - Division of Cardiology, Department of Pediatrics, C. S. Mott Children's Hospital, University of Michigan Health System, Ann Arbor, Michigan. AN - 104659866. Language: English. Entry Date: 20110923. Revision Date: 20150711. Publication Type: Journal Article 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. DB - ccm DP - EBSCOhost IS - 3 KW - Disability Evaluation Exercise Test Cardiopulmonary Bypass Health Status Manometry Peripheral Vascular Diseases -- Diagnosis Postoperative Complications -- Diagnosis Vascular Resistance -- Physiology Adolescence Adult Blood Pressure -- Physiology Child Child, Preschool Echocardiography, Doppler Endothelium -- Physiopathology Female Fingers -- Blood Supply Hyperemia -- Physiopathology Male Nitric Oxide -- Physiology Oxygen -- Blood Peripheral Vascular Diseases -- Physiopathology Postoperative Complications -- Physiopathology Prognosis Prospective Studies Pulse Quality of Life Reference Values Young Adult N1 - research. Journal Subset: Biomedical; Peer Reviewed; USA. NLM UID: 0207277. PMID: NLM21600541. PY - 2011 SN - 0002-9149 SP - 428-434 ST - Usefulness of peripheral vascular function to predict functional health status in patients with fontan circulation T2 - American Journal of Cardiology TI - Usefulness of peripheral vascular function to predict functional health status in patients with fontan circulation UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104659866&site=ehost-live&scope=site VL - 108 ID - 1589 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. © 2011 Elsevier Inc. AD - Department of Pediatrics, C. S. Mott Children's Hospital, University of Michigan Health System, Ann Arbor, United States 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. DB - Scopus DO - 10.1016/j.amjcard.2011.03.064 IS - 3 M3 - Article N1 - Cited By :28 Export Date: 15 June 2020 PY - 2011 SP - 428-434 ST - Usefulness of peripheral vascular function to predict functional health status in patients with fontan circulation T2 - American Journal of Cardiology TI - Usefulness of peripheral vascular function to predict functional health status in patients with fontan circulation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-79960350170&doi=10.1016%2fj.amjcard.2011.03.064&partnerID=40&md5=34940d3eda5f10b2d5a71c1ba8100a97 VL - 108 ID - 2123 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 (VO2) 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 VO2 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 VO2. 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 - B.H. Goldstein, 3333 Burnet Avenue, MLC 2003, Cincinnati, OH, United States 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. DB - Embase DO - 10.1161/JAHA.116.004258 IS - 9 KW - carbon dioxide adolescent adult aerobic capacity age distribution amplitude modulation anaerobic threshold arterial stiffness article augmentation index brachial artery cardiopulmonary exercise test cardiovascular function controlled study cross-sectional study female flow-mediated dilation test Fontan procedure functional assessment human hyperemia lung minute volume major clinical study male operation duration outcome assessment outcome variable oxygen consumption Pediatric Quality of Life Inventory peripheral arterial tonometry Physical Activity Questionnaire predictor variable priority journal quality of life assessment questionnaire scoring system sex ratio survivor vascular endothelium young adult LA - English M3 - Article N1 - L618953079 2017-11-07 2017-11-09 PY - 2016 SN - 2047-9980 ST - Endothelial Function and Arterial Stiffness Relate to Functional Outcomes in Adolescent and Young Adult Fontan Survivors T2 - Journal of the American Heart Association TI - Endothelial Function and Arterial Stiffness Relate to Functional Outcomes in Adolescent and Young Adult Fontan Survivors UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L618953079 http://dx.doi.org/10.1161/JAHA.116.004258 VL - 5 ID - 796 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 (VO2) 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 VO2 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 VO2. 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. © 2016 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell. AD - The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Division of Cardiology, Northwestern University Feinberg School of Medicine and Lurie Children's Hospital, Chicago, IL, United States 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. C7 - e004258 DB - Scopus DO - 10.1161/JAHA.116.004258 IS - 9 KW - Arterial stiffness Endothelial function Exercise capacity Fontan procedure Quality of life M3 - Article N1 - Cited By :6 Export Date: 15 June 2020 PY - 2016 ST - Endothelial Function and Arterial Stiffness Relate to Functional Outcomes in Adolescent and Young Adult Fontan Survivors T2 - Journal of the American Heart Association TI - Endothelial Function and Arterial Stiffness Relate to Functional Outcomes in Adolescent and Young Adult Fontan Survivors UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85020578350&doi=10.1161%2fJAHA.116.004258&partnerID=40&md5=2afc7f97ab9b01c688a27982d00696c3 VL - 5 ID - 1892 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 - N. Golfenshtein, Department of Nursing, University of Haifa, Haifa, Israel AU - Golfenshtein, N. AU - Hanlon, A. L. AU - Deatrick, J. A. AU - Medoff-Cooper, B. DB - Embase Medline DO - 10.1111/chd.12858 IS - 6 KW - adult article biventricular congenital heart disease child behavior cohort analysis congenital heart disease coronary care unit disease association disease severity emotional attachment female heart single ventricle human infant longitudinal study major clinical study male outpatient department parental stress pediatric hospital postoperative period priority journal prospective study stress assessment temperament LA - English M3 - Article N1 - L2003523923 2019-11-12 PY - 2019 SN - 1747-0803 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 - Congenital Heart Disease TI - Parenting stress trajectories during infancy in infants with congenital heart disease: Comparison of single-ventricle and biventricular heart physiology UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2003523923 http://dx.doi.org/10.1111/chd.12858 VL - 14 ID - 588 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. © 2019 Wiley Periodicals, Inc. AD - Department of Nursing, University of Haifa, Haifa, Israel Center for Biostatistics and Health Data Science, Virginia Tech, Blacksburg, VA, United States School of Nursing, University of Pennsylvania, Philadelphia, PA, United States The Cardiac Center, The Children's Hospital of Philadelphia, Philadelphia, PA, United States AU - Golfenshtein, N. AU - Hanlon, A. L. AU - Deatrick, J. A. AU - Medoff-Cooper, B. DB - Scopus DO - 10.1111/chd.12858 IS - 6 KW - congenital heart disease infancy longitudinal design parenting stress uni-ventricular heart M3 - Article N1 - Export Date: 15 June 2020 PY - 2019 SP - 1113-1122 ST - Parenting stress trajectories during infancy in infants with congenital heart disease: Comparison of single-ventricle and biventricular heart physiology T2 - Congenital Heart Disease TI - Parenting stress trajectories during infancy in infants with congenital heart disease: Comparison of single-ventricle and biventricular heart physiology UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85074556623&doi=10.1111%2fchd.12858&partnerID=40&md5=5a97e882eef34acdc6013246393b6022 VL - 14 ID - 1726 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 2.6kg to 71kg (median 14.1kg), 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 332h (range 11-2030h). 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 2000h was very rare, but it was possible to wean this patient from ECMO using HFO. © 2011, Copyright the Authors. Artificial Organs © 2011, International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc. AD - Y. Suzuki, Department of Thoracic and Cardiovascular Surgery, Hirosaki University School of Medicine, 5 Zaifucho, Hirosaki, Aomori, 036-8562, Japan AU - Goto, T. AU - Suzuki, Y. AU - Suzuki, Y. AU - Osanai, A. AU - Aoki, K. AU - Yamazaki, A. AU - Daitoku, K. AU - Fukuda, I. DB - Embase Medline DO - 10.1111/j.1525-1594.2011.01374.x IS - 11 KW - adolescent adult anticoagulant therapy article Blalock Taussig shunt cardiopulmonary insufficiency child clinical article congenital heart disease continuous hemodiafiltration cystic adenomatoid malformation extracorporeal oxygenation Fontan procedure forward heart failure Glenn shunt human hypoxemia infant lung resection myocarditis palliative therapy preschool child priority journal renal replacement therapy retrospective study school child survival systemic pulmonary shunt LA - English M3 - Article N1 - L362979215 2011-11-30 2011-12-02 PY - 2011 SN - 0160-564X 1525-1594 SP - 1002-1009 ST - The Impact of Extracorporeal Membrane Oxygenation on Survival in Pediatric Patients With Respiratory and Heart Failure: Review of Our Experience T2 - Artificial Organs TI - The Impact of Extracorporeal Membrane Oxygenation on Survival in Pediatric Patients With Respiratory and Heart Failure: Review of Our Experience UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L362979215 http://dx.doi.org/10.1111/j.1525-1594.2011.01374.x VL - 35 ID - 1085 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 2.6kg to 71kg (median 14.1kg), 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 332h (range 11-2030h). 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 2000h was very rare, but it was possible to wean this patient from ECMO using HFO. © 2011, Copyright the Authors. Artificial Organs © 2011, International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc. AD - Department of Clinical Engineering, Hirosaki University School of Medicine and Hospital, Japan Department of Thoracic Cardiovascular Surgery, Hirosaki University School of Medicine, Hirosaki, Aomori, Japan AU - Goto, T. AU - Suzuki, Y. AU - Suzuki, Y. AU - Osanai, A. AU - Aoki, K. AU - Yamazaki, A. AU - Daitoku, K. AU - Fukuda, I. DB - Scopus DO - 10.1111/j.1525-1594.2011.01374.x IS - 11 KW - Acute respiratory distress syndrome Congenital cardiac surgery Extracorporeal membrane oxygenation Heart failure M3 - Article N1 - Cited By :10 Export Date: 15 June 2020 PY - 2011 SP - 1002-1009 ST - The Impact of Extracorporeal Membrane Oxygenation on Survival in Pediatric Patients With Respiratory and Heart Failure: Review of Our Experience T2 - Artificial Organs TI - The Impact of Extracorporeal Membrane Oxygenation on Survival in Pediatric Patients With Respiratory and Heart Failure: Review of Our Experience UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-81855166277&doi=10.1111%2fj.1525-1594.2011.01374.x&partnerID=40&md5=b935a6b2de9f16be622e28bce2dd547b VL - 35 ID - 2112 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 < .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 < .001 and p < .004; group 2, 80 ± 7 ml/m2 and 128 ± 10 g/m2, p < .02 and p < .001), and group 2 patients showed significantly lower values than group 1 patients (p < .001 and p < .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 ± 5 g/cm2, p < .02; group 2, 59 ± 7 g/cm2, p < .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. Fontan repair in patients with tricuspid atresia resulted in less volume overload and hypertrophy than palliation only, but an increased afterload and abnormal contractile function persisted in a significant number of patients. Abnormal function is prevalent in patients older than 5 years with palliation only. Prospective data are needed in an attempt to clarify causes of myocardial dysfunction in patients with tricuspid atresia. AD - Division of Pediatric Cardiology, Vanderbilt Medical Center North, Nashville, TN 37232 AU - Graham Jr, T. P. AU - Franklin, R. C. G. AU - Wyse, R. K. H. DB - Embase Medline IS - 3 II MONOGR. 123 KW - adult child childhood clinical article congenital disorder diagnosis echocardiography Fontan procedure heart human palliative therapy postoperative period priority journal therapy tricuspid valve atresia LA - English M3 - Article N1 - L16050305 1986-10-29 PY - 1986 SN - 0009-7322 SP - 61-69 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L16050305 VL - 74 ID - 1392 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 < .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 < .001 and p < .004; group 2, 80 ± 7 ml/m2 and 128 ± 10 g/m2, p < .02 and p < .001), and group 2 patients showed significantly lower values than group 1 patients (p < .001 and p < .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 ± 5 g/cm2, p < .02; group 2, 59 ± 7 g/cm2, p < .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. Fontan repair in patients with tricuspid atresia resulted in less volume overload and hypertrophy than palliation only, but an increased afterload and abnormal contractile function persisted in a significant number of patients. Abnormal function is prevalent in patients older than 5 years with palliation only. Prospective data are needed in an attempt to clarify causes of myocardial dysfunction in patients with tricuspid atresia. AD - Division of Pediatric Cardiology, Vanderbilt Medical Center North, Nashville, TN 37232, United States AU - Graham Jr, T. P. AU - Franklin, R. C. G. AU - Wyse, R. K. H. AU - Gooch, V. AU - Deanfield, J. E. DB - Scopus IS - 3 II MONOGR. 123 M3 - Article N1 - Cited By :46 Export Date: 15 June 2020 PY - 1986 SP - 61-69 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0022509243&partnerID=40&md5=f39a85e30c0d3f0404a8513df7a0c277 VL - 74 ID - 2326 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 × 10-33), patients only reported reductions in HRQoL concerning physical functioning (P = 4.41 × 10-15) and general health (P = 6.17 × 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 × 10-27) and general health (r = 0.275, P = 3.79 × 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. © The Author 2008. AD - A. Hager, Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Lazarettstr. 36, D-80636 München, Germany AU - Gratz, A. AU - Hess, J. AU - Hager, A. DB - Embase Medline DO - 10.1093/eurheartj/ehn531 IS - 4 KW - adolescent adult aerobic exercise aged aortic coarctation aortic stenosis article cardiopulmonary exercise test congenital heart disease controlled study cyanotic heart disease diagnosis related group Ebstein anomaly Fallot tetralogy female fitness great vessels transposition health survey heart septum defect human major clinical study male motor performance oxygen consumption priority journal pulmonary valve insufficiency pulmonary valve stenosis quality of life self report social psychology LA - English M3 - Article N1 - L354216051 2009-03-16 PY - 2009 SN - 0195-668X 1522-9645 SP - 497-504 ST - Self-estimated physical functioning poorly predicts actual exercise capacity in adolescents and adults with congenital heart disease T2 - European Heart Journal TI - Self-estimated physical functioning poorly predicts actual exercise capacity in adolescents and adults with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L354216051 http://dx.doi.org/10.1093/eurheartj/ehn531 VL - 30 ID - 1190 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 × 10-33), patients only reported reductions in HRQoL concerning physical functioning (P = 4.41 × 10-15) and general health (P = 6.17 × 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 × 10-27) and general health (r = 0.275, P = 3.79 × 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. © The Author 2008. AD - Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Lazarettstr. 36, D-80636 München, Germany AU - Gratz, A. AU - Hess, J. AU - Hager, A. DB - Scopus DO - 10.1093/eurheartj/ehn531 IS - 4 KW - Cardiopulmonary exercise testing Congenital heart disease Quality of life M3 - Article N1 - Cited By :108 Export Date: 15 June 2020 PY - 2009 SP - 497-504 ST - Self-estimated physical functioning poorly predicts actual exercise capacity in adolescents and adults with congenital heart disease T2 - European Heart Journal TI - Self-estimated physical functioning poorly predicts actual exercise capacity in adolescents and adults with congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-60749125533&doi=10.1093%2feurheartj%2fehn531&partnerID=40&md5=1fb7389ba7a6ac8f707a7013c0a8dc0d VL - 30 ID - 2183 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. © Springer Science+Business Media 2013. AD - S.B. Greenberg, Arkansas Children's Hospital, 1 Children's Way, Slot #105, Little Rock, AR 72202, United States AU - Greenberg, S. B. AU - Dyamenahalli, U. DB - Embase Medline DO - 10.1007/s10554-013-0344-1 IS - 2 KW - contrast medium aortic coarctation aortic occlusion article artificial ventilation Blalock Taussig shunt blood pressure bronchomalacia chromosome aberration clinical article computed tomographic angiography congenital blood vessel malformation congenital heart disease coronary artery obstruction Down syndrome dynamic pulmonary computed tomography angiography Ebstein anomaly Fallot tetralogy heart ventricle septum defect human hypoplastic left heart syndrome infant lung angiography lung artery banding lung vein drainage anomaly non invasive procedure patent ductus arteriosus patient care pulmonary artery stenosis pulmonary hypertension pulmonary valve atresia respiratory distress respiratory tract disease right ventricle to pulmonary artery conduit standard surgical technique tracheobronchomalacia tracheomalacia LA - English M3 - Article N1 - L52908325 2013-12-12 2014-05-07 PY - 2014 SN - 1573-0743 1569-5794 SP - 407-414 ST - Dynamic pulmonary computed tomography angiography: A new standard for evaluation of combined airway and vascular abnormalities in infants T2 - International Journal of Cardiovascular Imaging TI - Dynamic pulmonary computed tomography angiography: A new standard for evaluation of combined airway and vascular abnormalities in infants UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52908325 http://dx.doi.org/10.1007/s10554-013-0344-1 VL - 30 ID - 947 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 - 104031734. Language: English. Entry Date: 20141107. Revision Date: 20171023. Publication Type: journal article AU - Greenberg, S. Bruce AU - Dyamenahalli, Umesh DB - ccm DO - 10.1007/s10554-013-0344-1 DP - EBSCOhost IS - 2 KW - Abnormalities, Multiple Cineangiography Pulmonary Artery -- Radiography Tomography, X-Ray Computed -- Methods Tracheobronchomalacia -- Radiography Vascular Malformations -- Radiography Heart Surgery Constriction, Pathologic Contrast Media -- Administration and Dosage Contrast Media -- Diagnostic Use Heart Defects, Congenital -- Complications Heart Defects, Congenital -- Radiography Heart Defects, Congenital -- Surgery Human Infant Infant, Newborn Injections, Intravenous Intubation, Intratracheal Predictive Value of Tests Pulmonary Artery -- Abnormalities Pulmonary Artery -- Surgery Respiration Respiration, Artificial Respiratory Distress Syndrome -- Etiology Respiratory Distress Syndrome -- Radiography Respiratory Distress Syndrome -- Therapy Retrospective Design Tracheobronchomalacia -- Complications Tracheobronchomalacia -- Therapy Vascular Malformations -- Complications Vascular Malformations -- Therapy N1 - research. Journal Subset: Biomedical; Blind Peer Reviewed; Peer Reviewed; USA. Special Interest: Diagnostic Imaging. NLM UID: 100969716. PMID: NLM24322888. PY - 2014 SN - 1569-5794 SP - 407-414 ST - Dynamic pulmonary computed tomography angiography: a new standard for evaluation of combined airway and vascular abnormalities in infants T2 - International Journal of Cardiovascular Imaging TI - Dynamic pulmonary computed tomography angiography: a new standard for evaluation of combined airway and vascular abnormalities in infants UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104031734&site=ehost-live&scope=site VL - 30 ID - 1544 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. © Springer Science+Business Media 2013. AD - Arkansas Children's Hospital, 1 Children's Way, Slot #105, Little Rock, AR 72202, United States AU - Greenberg, S. B. AU - Dyamenahalli, U. DB - Scopus DO - 10.1007/s10554-013-0344-1 IS - 2 KW - Bronchomalacia Computed tomography Congenital heart disease Respiratory distress Tracheomalacia M3 - Article N1 - Cited By :12 Export Date: 15 June 2020 PY - 2014 SP - 407-414 ST - Dynamic pulmonary computed tomography angiography: A new standard for evaluation of combined airway and vascular abnormalities in infants T2 - International Journal of Cardiovascular Imaging TI - Dynamic pulmonary computed tomography angiography: A new standard for evaluation of combined airway and vascular abnormalities in infants UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84899093633&doi=10.1007%2fs10554-013-0344-1&partnerID=40&md5=a7e36c5be1cdc29856a16fa9c7060968 VL - 30 ID - 2019 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. © 2012 Wiley Periodicals, Inc. AD - Division of Cardiology, St. Paul's Hospital, University of British Columbia, Vancouver, BC, Canada Department of Radiology, St. Paul's Hospital, University of British Columbia, Vancouver, BC, Canada AU - Grewal, J. AU - Al Hussein, M. AU - Feldstein, J. AU - Kiess, M. AU - Ellis, J. AU - Human, D. AU - Leipsic, J. DB - Scopus DO - 10.1111/j.1747-0803.2012.00699.x IS - 1 KW - Cardiac CT Embolism Fontan Pulmonary artery Thrombus M3 - Article N1 - Cited By :22 Export Date: 15 June 2020 PY - 2013 SP - 40-47 ST - Evaluation of Silent Thrombus after the Fontan Operation T2 - Congenital Heart Disease TI - Evaluation of Silent Thrombus after the Fontan Operation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84873033727&doi=10.1111%2fj.1747-0803.2012.00699.x&partnerID=40&md5=c9ec334bffefd00d35fb8c13b7fcf856 VL - 8 ID - 2077 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 - L. Fernández-Trujillo, Avenida Simón Bolívar. Cra 98 No. 18-49, Fundación Valle del Lili, Tower 6 Office 446, Cali, Colombia 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. DB - Embase DO - 10.1016/j.rmcr.2019.100876 KW - arterial stent dornase alfa heparin sodium chloride article bronchitis bronchoscopy case report child clinical article clinical evaluation computer assisted tomography congenital heart disease coughing dyspnea edema emergency ward erythema Fontan procedure heart catheterization hospital discharge hospitalization human lung mucosa male medical history oxygen therapy palliative therapy plastic bronchitis priority journal pulmonary artery stenosis respiratory care respiratory distress staining thorax radiography LA - English M3 - Article N1 - L2002101387 2019-06-18 2019-06-25 PY - 2019 SN - 2213-0071 ST - Plastic bronchitis: A case report T2 - Respiratory Medicine Case Reports TI - Plastic bronchitis: A case report UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2002101387 http://dx.doi.org/10.1016/j.rmcr.2019.100876 VL - 28 ID - 634 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. © 2019 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 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. C7 - 100876 DB - Scopus DO - 10.1016/j.rmcr.2019.100876 KW - Bronchial casts Congenital heart disease Fontan Plastic bronchitis M3 - Article N1 - Export Date: 15 June 2020 PY - 2019 ST - Plastic bronchitis: A case report T2 - Respiratory Medicine Case Reports TI - Plastic bronchitis: A case report UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85067017441&doi=10.1016%2fj.rmcr.2019.100876&partnerID=40&md5=6205950316935e4e32c74c8da2f7f3c1 VL - 28 ID - 1775 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 2years 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 2months 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 2years, survivors were assessed using the Bayley Scales of Infant Development-III. Results At 2years, 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 - L.S. Shekerdemian, Department of Pediatric Critical Care, Texas Children's Hospital, Pediatric Critical Care, Baylor College of Medicine, 6621 Fannin Street, Houston, TX, United States 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. DB - Embase Medline DO - 10.1136/archdischild-2015-309449 IS - 11 KW - lactic acid protein S100B anatomical variation article Bayley Scales of Infant Development clinical assessment cognitive defect cohort analysis congenital heart disease controlled study disease severity female gestational age heart single ventricle heart surgery high risk infant human language disability longitudinal study lung circulation major clinical study male mental disease metabolic acidosis newborn newborn death outcome assessment perioperative period prenatal diagnosis priority journal prospective study pulmonary valve atresia risk factor systemic pulmonary shunt LA - English M3 - Article N1 - L610660328 2016-06-14 2016-12-02 PY - 2016 SN - 1468-2044 0003-9888 SP - 1010-1016 ST - Perioperative risk factors for impaired neurodevelopment after cardiac surgery in early infancy T2 - Archives of Disease in Childhood TI - Perioperative risk factors for impaired neurodevelopment after cardiac surgery in early infancy UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L610660328 http://dx.doi.org/10.1136/archdischild-2015-309449 VL - 101 ID - 788 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 - Children's Hospital, Tampa, FL, United States Herzzentrum, Univ. Leipzig, Russenstrasse 19, D., 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 - Scopus DO - 10.1016/S1010-7940(00)00433-4 IS - 6 KW - Bidirectional cavopulmonary anastomosis Fontan operation Univentricular heart M3 - Article N1 - Cited By :49 Export Date: 15 June 2020 PY - 2000 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0034213611&doi=10.1016%2fS1010-7940%2800%2900433-4&partnerID=40&md5=0df860055cc99ef05525f86083d95615 VL - 17 ID - 2274 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 VO2), a higher ventilatory equivalent (VE/VCO2 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 - A. Hager, Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Lazarettstrasse 36, 80636 Munich, Germany AU - Hager, A. AU - Christov, F. AU - Hess, J. DB - Embase Medline DO - 10.1007/s00246-012-0210-2 IS - 5 KW - amino terminal pro brain natriuretic peptide amiodarone anticoagulant agent beta adrenergic receptor blocking agent digoxin dipeptidyl carboxypeptidase inhibitor diuretic agent sotalol adolescent adult article atrioventricular septal defect body mass cardiopulmonary exercise test cavopulmonary connection child controlled study female Fontan procedure heart atrium overload heart function heart right ventricle double outlet heart single ventricle heart ventricle overload human hypoplastic left heart syndrome lung ventilation major clinical study male oxygen consumption preschool child protein secretion rest right heart hypoplasia school child tricuspid valve atresia LA - English M3 - Article N1 - L51886073 2012-03-02 2012-07-31 PY - 2012 SN - 0172-0643 1432-1971 SP - 764-769 ST - Increase in N-Terminus-Pro-B-Type natriuretic peptide during exercise of patients with univentricular heart after a total cavopulmonary connection T2 - Pediatric Cardiology TI - Increase in N-Terminus-Pro-B-Type natriuretic peptide during exercise of patients with univentricular heart after a total cavopulmonary connection UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51886073 http://dx.doi.org/10.1007/s00246-012-0210-2 VL - 33 ID - 1060 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 VO 2), 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 AU - Hager, A. AU - Christov, F. AU - Hess, J. DB - Scopus DO - 10.1007/s00246-012-0210-2 IS - 5 KW - Congenital heart disease Exercise test NT-proBNP Total cavopulmonary connection Univentricular heart M3 - Article N1 - Cited By :8 Export Date: 15 June 2020 PY - 2012 SP - 764-769 ST - Increase in N-Terminus-Pro-B-Type natriuretic peptide during exercise of patients with univentricular heart after a total cavopulmonary connection T2 - Pediatric Cardiology TI - Increase in N-Terminus-Pro-B-Type natriuretic peptide during exercise of patients with univentricular heart after a total cavopulmonary connection UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84863990119&doi=10.1007%2fs00246-012-0210-2&partnerID=40&md5=e216a5eecf85b66cda56cd4840e0b9f4 VL - 33 ID - 2093 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. AU - Hahn, E. AU - Szwast, A. AU - Cnota, J. AU - Levine, J. C. AU - Fifer, C. G. AU - Jaeggi, E. AU - Andrews, H. AU - Williams, I. A. DB - Medline DO - 10.1002/uog.14881 IS - 4 KW - abnormalities brain circulation female fetus fetus development fetus echography gestational age heart ventricle human male middle cerebral artery mental disease pathophysiology physiology pregnancy procedures pulsatile flow retrospective study LA - English M3 - Article N1 - L616487365 2017-06-02 PY - 2016 SN - 1469-0705 SP - 460-465 ST - Association between fetal growth, cerebral blood flow and neurodevelopmental outcome in univentricular fetuses T2 - Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology TI - Association between fetal growth, cerebral blood flow and neurodevelopmental outcome in univentricular fetuses UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L616487365 http://dx.doi.org/10.1002/uog.14881 VL - 47 ID - 814 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 - M.S. Si, Section of Pediatric Cardiovascular Surgery, Department of Cardiac Surgery, University of Michigan, 11-735 C.S. Mott Children's Hospital, 1540 E. Hospital Drive, SPC 4204, Ann Arbor, MI, United States AU - Halaweish, I. AU - Ohye, R. G. AU - Si, M. S. DB - Embase Medline DO - 10.1111/petr.12607 IS - 8 KW - acetylsalicylic acid bortezomib dipyridamole dopamine immunoglobulin milrinone rituximab warfarin adolescent anticoagulant therapy aortic surgery artery catheterization artery reconstruction article blood transfusion case report congestive heart failure coughing desensitization diuresis dyspnea echocardiography extracorporeal oxygenation Fontan procedure heart muscle biopsy heart right ventricle failure heart right ventricle function heart transplantation hospital discharge hospitalization human hypoplastic left heart syndrome immunotherapy international normalized ratio kidney function liver function male postoperative hemorrhage postoperative period pulmonary artery treatment indication treatment outcome ventricular assist device EXCOR VAD LA - English M3 - Article N1 - L606698508 2015-11-06 2016-02-02 PY - 2015 SN - 1399-3046 1397-3142 SP - E193-E195 ST - Berlin heart ventricular assist device as a long-term bridge to transplantation in a Fontan patient with failing single ventricle T2 - Pediatric Transplantation TI - Berlin heart ventricular assist device as a long-term bridge to transplantation in a Fontan patient with failing single ventricle UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L606698508 http://dx.doi.org/10.1111/petr.12607 VL - 19 ID - 842 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. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd. AD - Section of Pediatric Cardiovascular Surgery, Department of Cardiac Surgery, University of Michigan, 11-735 C.S. Mott Children's Hospital, 1540 E. Hospital Drive, SPC 4204, Ann Arbor, MI 48109-4204, United States AU - Halaweish, I. AU - Ohye, R. G. AU - Si, M. S. DB - Scopus DO - 10.1111/petr.12607 IS - 8 KW - Fontan single ventricle transplant ventricular assist device M3 - Article N1 - Cited By :20 Export Date: 15 June 2020 PY - 2015 SP - E193-E195 ST - Berlin heart ventricular assist device as a long-term bridge to transplantation in a Fontan patient with failing single ventricle T2 - Pediatric Transplantation TI - Berlin heart ventricular assist device as a long-term bridge to transplantation in a Fontan patient with failing single ventricle UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84955197434&doi=10.1111%2fpetr.12607&partnerID=40&md5=aa1d67ba0fff5399b2549017f214a841 VL - 19 ID - 1931 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 - M.D. Jarboe, C.S. Mott Children’s Hospital, University of Michigan, 1540 E. Hospital Dr., SPC 4211, Ann Arbor, MI, United States AU - Halaweish, I. AU - Ralls, M. AU - Siddiqui, S. AU - Dickinson, C. AU - Jarboe, M. D. DB - Embase Medline DO - 10.1007/s00383-015-3804-y IS - 12 KW - article case report child cholestasis common bile duct disease congenital diaphragm hernia diaphragm hernia follow up Fontan procedure gallbladder disease heart output heart surgery hepatomegaly human hyperbilirubinemia laparoscopic surgery magnetic resonance cholangiopancreatography obstructive jaundice preschool child priority journal respiratory distress safety vomiting LA - English M3 - Article N1 - L606627055 2015-11-03 2015-11-23 PY - 2015 SN - 1437-9813 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://www.embase.com/search/results?subaction=viewrecord&from=export&id=L606627055 http://dx.doi.org/10.1007/s00383-015-3804-y VL - 31 ID - 841 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. © 2015, Springer-Verlag Berlin Heidelberg. AD - Section of Pediatric Surgery, Department of Surgery, University of Michigan, Ann Arbor, MI, United States Section of Pediatric Gastroenterology, Department of Pediatrics, University of Michigan, Ann Arbor, MI, United States C.S. Mott Children’s Hospital, University of Michigan, 1540 E. Hospital Dr., SPC 4211, Ann Arbor, MI 48109-0344, United States AU - Halaweish, I. AU - Ralls, M. AU - Siddiqui, S. AU - Dickinson, C. AU - Jarboe, M. D. DB - Scopus DO - 10.1007/s00383-015-3804-y IS - 12 KW - Diaphragmatic Fontan Hernia Jaundice Morgagni Pediatric M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2015 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84947025352&doi=10.1007%2fs00383-015-3804-y&partnerID=40&md5=e635eeb3f17396b160d877f9871f3b91 VL - 31 ID - 1933 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 - H.S. Hancock, Ward Family Heart Center, Children's Mercy Kansas City, 2401 Gillham Road, Kansas City, MO, United States 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. DB - Embase Medline DO - 10.1017/S1047951117002761 IS - 4 KW - NCT02462434 adult anxiety article Beck Depression Inventory clinical article cohort analysis controlled study coping behavior depression early intervention family relation female heart single ventricle hospital discharge human infant interpersonal communication male maternal stress palliative therapy perinatal period pilot study prenatal diagnosis psychosocial care quality of life randomized controlled trial spiritual care State Trait Anxiety Inventory LA - English M3 - Article N1 - L621113679 2018-03-13 2018-09-05 PY - 2018 SN - 1467-1107 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 - Cardiology in the Young TI - A randomised trial of early palliative care for maternal stress in infants prenatally diagnosed with single-ventricle heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L621113679 http://dx.doi.org/10.1017/S1047951117002761 VL - 28 ID - 692 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 - Department of Pediatrics, Division of Pediatric Cardiology, Ward Family Heart Center, Children’s Mercy Kansas City, Missouri, United States of America Department of Pediatrics, Palliative Care, University of Michigan, Ann Arbor, Michigan, United States of America Department of Pediatrics, Division of Pediatric Cardiology, University of Michigan, Ann Arbor, Michigan, United States of America Department of Pediatrics, School of Medicine, Divisions of Pediatric Cardiology and Critical Care Medicine, Washington University, St. Louis, Missouri, United States of America Department of Internal Medicine, Division of Geriatric Medicine and Palliative Care, University of Michigan, Ann Arbor, Michigan, United States of America AN - 128340420. Language: English. Entry Date: 20180524. Revision Date: 20190401. Publication Type: journal article AU - Hancock, Hayley S. AU - Pituch, Ken AU - Uzark, Karen AU - Bhat, Priya AU - Fifer, Carly AU - Silveira, Maria AU - Yu, Sunkyung AU - Welch, Suzanne AU - Donohue, Janet AU - Lowery, Ray AU - Aiyagari, Ranjit DB - ccm DO - 10.1017/S1047951117002761 DP - EBSCOhost IS - 4 KW - Mothers -- Psychosocial Factors Heart Ventricle -- Abnormalities Stress, Psychological -- Therapy Palliative Care -- Psychosocial Factors Adaptation, Psychological Heart Defects, Congenital -- Surgery Mother-Child Relations Heart Ventricle -- Surgery Pregnancy Female Palliative Care -- Methods Heart Defects, Congenital -- Psychosocial Factors Stress, Psychological -- Diagnosis Heart Defects, Congenital -- Diagnosis Heart Ventricle Infant, Newborn Prospective Studies Human Stress, Psychological -- Psychosocial Factors Time Factors Psychological Tests Adult Validation Studies Comparative Studies Evaluation Research Multicenter Studies Randomized Controlled Trials N1 - research; randomized controlled trial. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Special Interest: Evidence-Based Practice. Instrumentation: Beck Depression Inventory II (BDI-II); Beck Depression Inventory (BDI); Cope (Carver et al); Brief Cope. NLM UID: 9200019. PMID: NLM29316996. 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 - Cardiology in the Young TI - A randomised trial of early palliative care for maternal stress in infants prenatally diagnosed with single-ventricle heart disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=128340420&site=ehost-live&scope=site VL - 28 ID - 1445 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. © Cambridge University Press 2018. AD - Ward Family Heart Center, Children's Mercy Kansas City, 2401 Gillham Road, Kansas City, MO 64108-4619, United States Department of Pediatrics, Palliative Care, University of Michigan, Ann Arbor, MI, United States Department of Pediatrics, Division of Pediatric Cardiology, University of Michigan, Ann Arbor, MI, United States Department of Pediatrics, School of Medicine, Divisions of Pediatric Cardiology and Critical Care Medicine, Washington University, St. Louis, MO, United States Department of Internal Medicine, Division of Geriatric Medicine and Palliative Care, University of Michigan, Ann Arbor, MI, United States 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. DB - Scopus DO - 10.1017/S1047951117002761 IS - 4 KW - CHD hypoplastic left heart syndrome paediatric palliative care parental stress M3 - Article N1 - Cited By :15 Export Date: 15 June 2020 PY - 2018 SP - 561-570 ST - A randomised trial of early palliative care for maternal stress in infants prenatally diagnosed with single-ventricle heart disease T2 - Cardiology in the Young TI - A randomised trial of early palliative care for maternal stress in infants prenatally diagnosed with single-ventricle heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85043237290&doi=10.1017%2fS1047951117002761&partnerID=40&md5=995a19fafda225e5c768020ddcbaebd8 VL - 28 ID - 1809 ER - TY - JOUR AB - Our programmatic approach to the Fontan operation has evolved to include using an extracardiac conduit with aggressive presumptive treatment of associated lesions either in the catheterization laboratory or the operating room. Fenestration is used selectively based on hemodynamics, anatomy, and presence of associated lesions. We reviewed our experience to determine the effectiveness and outcome of this strategy and to assess the cumulative trauma to the patients. The records of 137 consecutive patients who underwent Fontan at Miami Children's Hospital from 1995 to 2008 were reviewed. At mean follow up of 5.76 years, freedom from death or transplantation is 94.2% (129/137). Median age at operation was 4.6 years. Longer length of stay correlated with older operative age (P = 0.0056). Pacemakers were implanted in 11.7% (16/137). Additional (not pre-Glenn or pre-Fontan) interventional catheterizations were performed in 51.8% (71/137). Additional operations were done in 10.2% (14/137). No patient has required replacement or revision of the extracardiac conduit. Our current approach to the Fontan operation provides acceptable midterm results. The pursuit of residual lesions results in a significant number of additional interventional catheterizations and operative procedures but might have an important influence on long-term survival after the Fontan procedure. © 2011 The Author(s). AD - R.L. Hannan, Department of Cardiovascular Surgery, Congenital Heart Institute, Miami Children's Hospital, 3100 SW 62nd Avenue, Miami, FL 33155, United States AU - Hannan, R. L. AU - Zabinsky, J. A. AU - Salvaggio, J. L. AU - Rossi, A. F. AU - Khan, D. M. AU - Alonso, F. A. AU - Ojito, J. W. AU - Nykanen, D. G. AU - Zahn, E. M. AU - Burke, R. P. DB - Embase Medline DO - 10.1007/s00246-011-9973-0 IS - 6 KW - angioplasty aortic stenosis article artificial heart pacemaker atrioventricular canal child Ebstein anomaly Fallot tetralogy follow up Fontan procedure great vessels transposition heart catheterization heart right ventricle double outlet heart ventricle septum defect hospitalization human hypoplastic left heart syndrome length of stay major clinical study medical record review mitral valve atresia preschool child pulmonary valve atresia retrospective study school child stent survival thoracotomy thorax drainage treatment outcome tricuspid valve atresia tricuspid valve disease LA - English M3 - Article N1 - L51368138 2011-04-15 2011-08-18 PY - 2011 SN - 0172-0643 1432-1971 SP - 778-784 ST - The fontan operation: The pursuit of associated lesions and cumulative trauma T2 - Pediatric Cardiology TI - The fontan operation: The pursuit of associated lesions and cumulative trauma UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51368138 http://dx.doi.org/10.1007/s00246-011-9973-0 VL - 32 ID - 1098 ER - TY - JOUR AB - Our programmatic approach to the Fontan operation has evolved to include using an extracardiac conduit with aggressive presumptive treatment of associated lesions either in the catheterization laboratory or the operating room. Fenestration is used selectively based on hemodynamics, anatomy, and presence of associated lesions. We reviewed our experience to determine the effectiveness and outcome of this strategy and to assess the cumulative trauma to the patients. The records of 137 consecutive patients who underwent Fontan at Miami Children's Hospital from 1995 to 2008 were reviewed. At mean follow up of 5.76 years, freedom from death or transplantation is 94.2% (129/137). Median age at operation was 4.6 years. Longer length of stay correlated with older operative age (P = 0.0056). Pacemakers were implanted in 11.7% (16/137). Additional (not pre-Glenn or pre-Fontan) interventional catheterizations were performed in 51.8% (71/137). Additional operations were done in 10.2% (14/137). No patient has required replacement or revision of the extracardiac conduit. Our current approach to the Fontan operation provides acceptable midterm results. The pursuit of residual lesions results in a significant number of additional interventional catheterizations and operative procedures but might have an important influence on long-term survival after the Fontan procedure. © 2011 The Author(s). AD - Department of Cardiovascular Surgery, Congenital Heart Institute, Miami Children's Hospital, 3100 SW 62nd Avenue, Miami, FL 33155, United States Department of Cardiology, Congenital Heart Institute, Miami Children's Hospital, 3100 SW 62nd Avenue, Miami, FL 33155, United States Department of Pediatric Cardiology, Arnold Palmer Hospital for Children, Orlando, FL, United States AU - Hannan, R. L. AU - Zabinsky, J. A. AU - Salvaggio, J. L. AU - Rossi, A. F. AU - Khan, D. M. AU - Alonso, F. A. AU - Ojito, J. W. AU - Nykanen, D. G. AU - Zahn, E. M. AU - Burke, R. P. DB - Scopus DO - 10.1007/s00246-011-9973-0 IS - 6 KW - Cardiac catheterization/intervention Cardiac surgery Congenital heart disease Pediatric M3 - Article N1 - Cited By :6 Export Date: 15 June 2020 PY - 2011 SP - 778-784 ST - The fontan operation: The pursuit of associated lesions and cumulative trauma T2 - Pediatric Cardiology TI - The fontan operation: The pursuit of associated lesions and cumulative trauma UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-80051475048&doi=10.1007%2fs00246-011-9973-0&partnerID=40&md5=de8b443b781a5f7d83b56b563b03104e VL - 32 ID - 2121 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. © 2012 The Authors. AD - E. Hansen, Buehlstrasse 25, 8125 Zollikerberg, Switzerland 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. DB - Embase Medline DO - 10.1111/j.1442-200X.2012.03711.x IS - 6 KW - adolescent article attention deficit disorder child congenital heart disease controlled study cyanosis female human hyperactivity impulsiveness major clinical study male parent prevalence priority journal rating scale school child Swanson, Nolan and Pelham teacher and parent rating scale IV LA - English M3 - Article N1 - L368172022 2013-01-31 2013-02-12 PY - 2012 SN - 1328-8067 1442-200X SP - 838-843 ST - Prevalence of ADHD symptoms in patients with congenital heart disease T2 - Pediatrics International TI - Prevalence of ADHD symptoms in patients with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L368172022 http://dx.doi.org/10.1111/j.1442-200X.2012.03711.x VL - 54 ID - 1033 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 UC Irvine Child Development Center Department of Pediatrics, UC Irvine Department of Pediatrics, UCLA AN - 84484023. Language: English. Entry Date: 20121228. Revision Date: 20131202. Publication Type: Article AU - Hansen, Erlend AU - Poole, Tiffany A. AU - Nguyen, Van AU - Lerner, Marc AU - Wigal, Tim AU - Shannon, Kevin AU - Wigal, Sharon B. AU - Batra, Anjan S. DB - ccm DO - 10.1111/j.1442-200X.2012.03711.x DP - EBSCOhost IS - 6 KW - Heart Defects, Congenital Attention Deficit Hyperactivity Disorder -- Epidemiology Human Prevalence Cross Sectional Studies Child Adolescence Descriptive Statistics Data Analysis Software T-Tests Scales Chi Square Test Pilot Studies N1 - research; tables/charts. Journal Subset: Asia; Biomedical; Peer Reviewed. Special Interest: Pediatric Care. Instrumentation: Swanson, Nolan and Pelham teacher and parent rating scales, version 4 (SNAP-IV). NLM UID: 100886002. PY - 2012 SN - 1328-8067 SP - 838-843 ST - Prevalence of ADHD symptoms in patients with congenital heart disease T2 - Pediatrics International TI - Prevalence of ADHD symptoms in patients with congenital heart disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=84484023&site=ehost-live&scope=site VL - 54 ID - 1571 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. © 2016 The American Association for Thoracic Surgery. AD - Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Campus Kiel, Arnold-Heller Strasse 3, House 9, Kiel, 24105, Germany Department of Anesthesiology and Intensive Care Medicine, University Hospital Schleswig-Holstein, Kiel, Germany Department of Cardiovascular Surgery, University Hospital Schleswig-Holstein, Kiel, Germany AU - Hansen, J. H. AU - Rotermann, I. AU - Logoteta, J. AU - Jung, O. AU - Dütschke, P. AU - Scheewe, J. AU - Kramer, H. H. DB - Scopus DO - 10.1016/j.jtcvs.2016.02.035 IS - 5 KW - hypoplastic left heart syndrome, developmental outcome, near-infrared spectroscopy M3 - Article N1 - Cited By :21 Export Date: 15 June 2020 PY - 2016 SP - 1358-1366 ST - Neurodevelopmental outcome in hypoplastic left heart syndrome: Impact of perioperative cerebral tissue oxygenation of the Norwood procedure T2 - Journal of Thoracic and Cardiovascular Surgery TI - Neurodevelopmental outcome in hypoplastic left heart syndrome: Impact of perioperative cerebral tissue oxygenation of the Norwood procedure UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84963682428&doi=10.1016%2fj.jtcvs.2016.02.035&partnerID=40&md5=6477546866ac6a04bb01f95c095ce5e2 VL - 151 ID - 1908 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. © 2011 Elsevier Inc. AD - I.S. Harris, 505 Parnassus Avenue, M314, San Francisco, CA 94143-0124, United States AU - Harris, I. S. DB - Embase Medline DO - 10.1016/j.pcad.2010.08.001 IS - 4 KW - antibiotic agent heparin warfarin antibiotic prophylaxis anticoagulant therapy aortic coarctation aortic disease aortic stenosis article cardiovascular response clinical assessment congenital heart disease cyanotic heart disease drug efficacy drug safety drug substitution drug withdrawal embryotoxicity endocarditis Fallot tetralogy fetus echography fetus risk first trimester pregnancy Fontan procedure heart atrium septum defect heart hemodynamics heart ventricle septum defect high risk pregnancy human labor low drug dose maternal care maternal mortality patient counseling pregnancy pregnancy complication pregnancy disorder puerperium pulmonary artery stenosis risk assessment second trimester pregnancy third trimester pregnancy thrombosis tricuspid valve atresia LA - English M3 - Article N1 - L361196495 2011-02-08 2011-02-15 PY - 2011 SN - 0033-0620 SP - 305-311 ST - Management of pregnancy in patients with congenital heart disease T2 - Progress in Cardiovascular Diseases TI - Management of pregnancy in patients with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L361196495 http://dx.doi.org/10.1016/j.pcad.2010.08.001 VL - 53 ID - 1121 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. © 2017 Elsevier Inc. AD - Department of Pediatrics and Child Health, Aga Khan Medical University, Pakistan Department of Cardiology Boston Children's Hospital, Harvard Medical School, Boston, MA, United States Department of Pediatrics, Harvard Medical School, Boston, MA, United States AU - Hasan, B. S. AU - Lunze, F. I. AU - Alvi, N. AU - Shafer, K. M. AU - Rhodes, J. DB - Scopus DO - 10.1016/j.ahj.2017.02.029 M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2017 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85015394381&doi=10.1016%2fj.ahj.2017.02.029&partnerID=40&md5=72b696480f1f28d8a9e096a5f8bfc82c VL - 188 ID - 1861 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 - M. Hauser, Deutsches Herzzentrum München, Pediatric Cardiology, Lazarettstrasse 36, 80636 Munich, Germany 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. DB - Embase Medline DO - 10.1007/s00246-002-0355-5 IS - 4 KW - adolescent adult angiocardiography article child clinical article coronary artery blood flow coronary artery disease coronary vascular resistance diagnostic imaging female Fontan procedure heart catheterization heart left ventricle function heart left ventricle mass heart muscle perfusion heart ventricle performance human ischemic heart disease male microcirculation muscle mass positron emission tomography risk assessment transesophageal echocardiography LA - English M3 - Article N1 - L37021440 2003-09-07 PY - 2003 SN - 0172-0643 SP - 386-392 ST - Myocardial perfusion and coronary flow reserve assessed by positron emission tomography in patients after Fontan-like operations T2 - Pediatric Cardiology TI - Myocardial perfusion and coronary flow reserve assessed by positron emission tomography in patients after Fontan-like operations UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L37021440 http://dx.doi.org/10.1007/s00246-002-0355-5 VL - 24 ID - 1301 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 Nuklearmedizinische Klinik, Technischen Universität, Ismaningerstraße 22, 81675 Munich, Germany 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. DB - Scopus DO - 10.1007/s00246-002-0355-5 IS - 4 KW - Coronary disease Fontan procedure Microcirculation Positron emission tomography imaging M3 - Article N1 - Cited By :26 Export Date: 15 June 2020 PY - 2003 SP - 386-392 ST - Myocardial perfusion and coronary flow reserve assessed by positron emission tomography in patients after Fontan-like operations T2 - Pediatric Cardiology TI - Myocardial perfusion and coronary flow reserve assessed by positron emission tomography in patients after Fontan-like operations UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0042470912&doi=10.1007%2fs00246-002-0355-5&partnerID=40&md5=5456c0ea6cc74a5348446749ac9d3ab4 VL - 24 ID - 2260 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. © 2014 Hayashi Y. AD - Department of Neurosurgery, Kanazawa University, Ishikawa, 13-1, Takara-machi, Kanazawa, 920-8641, Japan AU - Hayashi, Y. AU - Kita, D. AU - Furuta, T. AU - Oishi, M. AU - Hamada, J. I. C7 - 134911 DB - Scopus DO - 10.4103/2152-7806.134911 IS - Supplement KW - Anticoagulant agent Endoscope Removal Subgaleal hematoma M3 - Article N1 - Export Date: 15 June 2020 PY - 2014 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84916223224&doi=10.4103%2f2152-7806.134911&partnerID=40&md5=5410d41a467736475ee7bd66e7a4d4a2 VL - 5 ID - 2025 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. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. AD - Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Cardiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Division of Pathology, Cincinnati Children's Hospital Medical Centre, Cincinnati, OH, United States University of Colorado at Boulder, Boulder, CO, United States Pediatrics, Children's Hospital, Boston, MA, United States Adolescent and Adult Congenital Heart Disease Program, Cincinnati Children's Hospital Medical Centre, Cincinnati, OH 45229, 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. C7 - 311838 DB - Scopus DO - 10.1136/heartjnl-2017-311838 IS - 4 KW - Congenital Heart Disease Fontan Physiology Vascular Biology M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2018 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85041699824&doi=10.1136%2fheartjnl-2017-311838&partnerID=40&md5=a8b943d6abb21ce99096d717a0c9226e VL - 104 ID - 1822 ER - TY - JOUR AB - Summary: 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 - 104434990. Language: English. Entry Date: 20121116. Revision Date: 20171129. Publication Type: Journal Article. Journal Subset: Biomedical AU - Healy, F. AU - Hanna, B. D. AU - Zinman, R. DB - ccm DP - EBSCOhost IS - 1 KW - Heart Defects, Congenital -- Complications Heart Defects, Congenital -- Surgery Postoperative Complications Respiratory Tract Diseases -- Etiology Respiratory Tract Diseases -- Physiopathology Adult Child N1 - Europe; UK & Ireland. Special Interest: Pediatric Care. NLM UID: 100898941. PMID: NLM22208788. PY - 2012 SN - 1526-0542 SP - 10-15 ST - Pulmonary complications of congenital heart disease T2 - Paediatric Respiratory Reviews TI - Pulmonary complications of congenital heart disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104434990&site=ehost-live&scope=site VL - 13 ID - 1563 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 - F. Muscara, Murdoch Children's Research Institute, Flemington Road, Parkville, VIC, Australia 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 Medline DO - 10.1017/S1047951113000760 IS - 4 KW - adaptive behavior adult article caregiver child parent relation congenital heart disease correlation analysis crying disease severity educational status emotional stress family life female heart surgery human infant feeding male mother normal human parental behavior perinatal period prediction prenatal diagnosis psychologic test Psychosocial Assessment Tool quality of life risk assessment risk factor sleep social bonding social psychology LA - English M3 - Article N1 - L600358149 2014-11-13 2014-11-19 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=L600358149 http://dx.doi.org/10.1017/S1047951113000760 VL - 24 ID - 964 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 - 103831197. Language: English. Entry Date: 20150227. Revision Date: 20150803. Publication Type: Journal Article AU - Hearps, Simone J. AU - McCarthy, Maria C. AU - Muscara, Frank AU - Hearps, Stephen J. C. AU - Burke, Kylie AU - Jones, Bryn AU - Anderson, Vicki A. DB - ccm DO - 10.1017/S1047951113000760 DP - EBSCOhost IS - 4 KW - Heart Defects, Congenital -- Surgery Parenting Parents -- Psychosocial Factors Quality of Life -- Psychosocial Factors Stress, Psychological -- Psychosocial Factors Adult Female Human Infant Infant, Newborn Linear Regression Male Parent-Child Relations Prenatal Diagnosis Questionnaires Risk Assessment Young Adult N1 - research. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Special Interest: Pediatric Care. NLM UID: 9200019. PMID: NLM23803336. PY - 2014 SN - 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=103831197&site=ehost-live&scope=site VL - 24 ID - 1504 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 - Critical Care and Neurosciences, Murdoch Children's Research Institute, Melbourne, Australia Children's Cancer Centre, Royal Children's Hospital, Melbourne, Australia Department of Paediatrics, University of Melbourne, Melbourne, Australia Psychology Service, Royal Children's Hospital, Melbourne, Australia Parenting Research Centre, Melbourne, Australia Department of Cardiology, Royal Children's Hospital, Melbourne, Australia 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 - Scopus DO - 10.1017/S1047951113000760 IS - 4 KW - acute stress. child congenital heart disease Psychosocial risk M3 - Article N1 - Cited By :40 Export Date: 15 June 2020 PY - 2014 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84903636976&doi=10.1017%2fS1047951113000760&partnerID=40&md5=8e6dbfcacc1ea5aa076e0321c49b2ef0 VL - 24 ID - 2002 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.Trial registration: clinicaltrials.gov NCT01292551. © 2013 Hebert et al.; licensee BioMed Central Ltd. AD - A. Hebert, Department of Cardiology, dep. 2014, Copenhagen University Hospital, Rigshospitalet, Blegdamsvej 9, 2100, Copenhagen, Denmark AU - Hebert, A. AU - Jensen, A. S. AU - Idorn, L. AU - Sørensen, K. E. AU - Søndergaard, L. DB - Embase Medline DO - 10.1186/1471-2261-13-36 KW - NCT01292551 aspartate aminotransferase bilirubin bosentan brain natriuretic peptide endothelin receptor antagonist hemoglobin placebo adolescent aminotransferase blood level article bicycle ergometry bilirubin blood level child controlled study double blind procedure drug safety drug withdrawal exercise female Fontan procedure heart single ventricle hematocrit hemoglobin blood level human lung blood flow major clinical study male multicenter study oxygen consumption oxygen saturation priority journal protein analysis quality of life randomized controlled trial school child side effect surgical patient systolic blood pressure platelet count total cavopulmonary connection treatment outcome LA - English M3 - Article N1 - L52580603 2013-05-15 2013-06-04 http://www.biomedcentral.com/1471-2261/13/36 PY - 2013 SN - 1471-2261 ST - The effect of Bosentan on exercise capacity in Fontan patients; rationale and design for the TEMPO study T2 - BMC Cardiovascular Disorders TI - The effect of Bosentan on exercise capacity in Fontan patients; rationale and design for the TEMPO study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52580603 http://dx.doi.org/10.1186/1471-2261-13-36 VL - 13 ID - 1001 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.Trial registration: clinicaltrials.gov NCT01292551. © 2013 Hebert et al.; licensee BioMed Central Ltd. AD - Department of Cardiology, dep. 2014, Copenhagen University Hospital, Rigshospitalet, Blegdamsvej 9, 2100, Copenhagen, Denmark Department of Cardiology, Aarhus University Hospital, Skejby, Brendstrupgaardsvej 100, 8200, Aarhus N, Denmark AU - Hebert, A. AU - Jensen, A. S. AU - Idorn, L. AU - Sørensen, K. E. AU - Søndergaard, L. C7 - 36 DB - Scopus DO - 10.1186/1471-2261-13-36 KW - Endothelin receptor antagonist Exercise capacity Fontan Randomized placebo-controlled trial Univentricular heart M3 - Article N1 - Cited By :12 Export Date: 15 June 2020 PY - 2013 ST - The effect of Bosentan on exercise capacity in Fontan patients; rationale and design for the TEMPO study T2 - BMC Cardiovascular Disorders TI - The effect of Bosentan on exercise capacity in Fontan patients; rationale and design for the TEMPO study UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84877260982&doi=10.1186%2f1471-2261-13-36&partnerID=40&md5=a6f47cdc2e71fcc1c1cb5710a3a1b7b6 VL - 13 ID - 2047 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/m2) 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/m2) 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). © 2014 Elsevier Ireland Ltd. All rights reserved. AD - Department of Cardiology, Rigshospitalet, Blegdamsvej 9, 2100 Copenhagen, Denmark Department of Orthopedic Surgery, Institute of Sports Medicine, 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 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. DB - Scopus DO - 10.1016/j.ijcard.2014.06.015 IS - 3 KW - Cardio-pulmonary exercise test Chronotropic impairment Fontan Stroke volume M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2014 SP - 478-483 ST - Hemodynamic causes of exercise intolerance in Fontan patients T2 - International Journal of Cardiology TI - Hemodynamic causes of exercise intolerance in Fontan patients UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84905116861&doi=10.1016%2fj.ijcard.2014.06.015&partnerID=40&md5=ea8dd3a56c551d6e6ae185b9f829f158 VL - 175 ID - 1987 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. AD - A. Hebert, Department of Cardiology, Copenhagen University Hospital, Rigshospitalet Blegdamsvej 9, Copenhagen, Denmark 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. DB - Embase Medline DO - 10.1161/CIRCULATIONAHA.113.008441 IS - 23 KW - NCT01292551 aminotransferase bosentan brain natriuretic peptide oxygen placebo adult aminotransferase blood level anaerobic threshold arthralgia article backache cardiopulmonary exercise test controlled study depressed blood pressure double blind procedure drug effect drug efficacy drug safety drug withdrawal dyspepsia edema exercise tolerance faintness fatigue female flushing Fontan procedure functional assessment heart muscle oxygen consumption heart palpitation human major clinical study male nausea New York Heart Association class outcome assessment protein blood level protein losing gastroenteropathy pruritus quality of life randomized controlled trial recurrent disease Short Form 36 side effect systolic blood pressure thorax pain LA - English M3 - Article N1 - L600756671 2014-12-19 2014-12-24 PY - 2014 SN - 1524-4539 0009-7322 SP - 2021-2030 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L600756671 http://dx.doi.org/10.1161/CIRCULATIONAHA.113.008441 VL - 130 ID - 969 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 - 103856292. Language: English. Entry Date: 20150320. Revision Date: 20150710. Publication Type: Journal Article AU - Hebert, Anders AU - Mikkelsen, Ulla R. AU - Thilen, Ulf AU - Idorn, Lars AU - Jensen, Annette S. AU - Nagy, Edit AU - Hanseus, Katarina AU - Sørensen, Keld E. AU - Søndergaard, Lars DB - ccm DO - 10.1161/CIRCULATIONAHA.113.008441 DP - EBSCOhost IS - 23 KW - Cardiopulmonary Bypass -- Adverse Effects Endothelins -- Antagonists and Inhibitors Exercise Tolerance -- Drug Effects Oxygen Consumption -- Drug Effects Postoperative Complications -- Drug Therapy Sulfonamides -- Administration and Dosage Adolescence Adult Child Child, Preschool Double-Blind Studies Female Hemodynamics Human Infant Male Nonparametric Statistics Placebos Receptors, Cell Surface -- Blood Sulfonamides -- Adverse Effects Treatment Outcomes Young Adult N1 - research; randomized controlled trial. Journal Subset: Biomedical; Peer Reviewed; USA. NLM UID: 0147763. PMID: NLM25446057. PY - 2014 SN - 0009-7322 SP - 2021-2030 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=103856292&site=ehost-live&scope=site VL - 130 ID - 1507 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. © 2014 American Heart Association, Inc. AD - Department of Cardiology, Copenhagen University Hospital, Rigshospitalet Blegdamsvej 9, Copenhagen, 2100, Denmark Institute of Sports Medicine, Department of Orthopedic Surgery, University of Copenhagen, Copenhagen, Denmark Departments of Cardiology, Lund University Hospital, Lund, Sweden Departments of Pediatric Cardiology, Lund University Hospital, Lund, Sweden Department of Cardiology/Department of Medicine, Karolinska University Hospital, Karolinska Institutet, Stockholm, Sweden Department of Cardiology, Skejby, Aarhus University Hospital, Aarhus, Denmark 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. DB - Scopus DO - 10.1161/CIRCULATIONAHA.113.008441 IS - 23 KW - Bosentan Congenital Exercise test Fontan procedure Heart defects Vasodilation M3 - Article N1 - Cited By :98 Export Date: 15 June 2020 PY - 2014 SP - 2021-2030 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84916631939&doi=10.1161%2fCIRCULATIONAHA.113.008441&partnerID=40&md5=6015476f8487fe213eb7c1fa4c3602b0 VL - 130 ID - 2008 ER - TY - JOUR AB - “Frontiers in Fontan Failure” was the title of a 2015 conference sponsored by Children's Healthcare of Atlanta and Emory University School of Medicine. In what is hoped to be the first of many such gatherings, speakers and attendees gathered to discuss the problem of long-term clinical deterioration in these patients. Specific focuses included properly defining the problem and then discussing different treatment strategies, both medical and surgical. The health of the liver after Fontan palliation was a particular point of emphasis, as were quality of life and future directions. AD - C. Hebson, Division of Cardiology, Department of Medicine, Emory University, Atlanta, GA, United States AU - Hebson, C. AU - Book, W. AU - Elder, R. W. AU - Ford, R. AU - Jokhadar, M. AU - Kanter, K. AU - Kogon, B. AU - Kovacs, A. H. AU - Levit, R. D. AU - Lloyd, M. AU - Maher, K. AU - Reshamwala, P. AU - Rodriguez, F. AU - Romero, R. AU - Tejada, T. AU - Marie Valente, A. AU - Veldtman, G. AU - McConnell, M. DB - Embase Medline DO - 10.1111/chd.12407 IS - 1 KW - acetylsalicylic acid bosentan budesonide diuretic agent enalapril iloprost sildenafil warfarin article bleeding cavopulmonary connection clinical research compression stocking diastolic heart failure drug efficacy Fontan procedure heart pacing human ischemic cardiomyopathy kidney dysfunction leg varicosis liver disease liver transplantation medical society nonhuman obesity pacemaker implantation patient attitude physician attitude portal hypertension protein losing gastroenteropathy quality of life resistance training stem cell transplantation surgical approach surgical technique systolic heart failure therapy effect thromboembolism transitional care treatment failure treatment outcome United States LA - English M3 - Article N1 - L612961884 2016-11-03 2017-03-01 PY - 2017 SN - 1747-0803 1747-079X SP - 6-16 ST - “Frontiers in Fontan failure: A summary of conference proceedings” T2 - Congenital Heart Disease TI - “Frontiers in Fontan failure: A summary of conference proceedings” UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L612961884 http://dx.doi.org/10.1111/chd.12407 VL - 12 ID - 777 ER - TY - JOUR AB - “Frontiers in Fontan Failure” was the title of a 2015 conference sponsored by Children's Healthcare of Atlanta and Emory University School of Medicine. In what is hoped to be the first of many such gatherings, speakers and attendees gathered to discuss the problem of long-term clinical deterioration in these patients. Specific focuses included properly defining the problem and then discussing different treatment strategies, both medical and surgical. The health of the liver after Fontan palliation was a particular point of emphasis, as were quality of life and future directions. © 2016 Wiley Periodicals, Inc. AD - Division of Cardiology, Department of Medicine, Emory University, Atlanta, GA, United States Division of Pediatric Cardiology, Department of Pediatrics, Emory University, Atlanta, GA, United States Division of Cardiology, Department of Medicine, Yale University, New Haven, CT, United States Division of Gastroenterology, Department of Medicine, Emory University, Atlanta, GA, United States Division of Cardiothoracic Surgery, Department of Surgery, Emory University, Atlanta, GA, United States Division of Cardiology, Department of Medicine, Oregon Health and Science University, Portland, OR, United States Division of Pediatric Gastroenterology, Department of Pediatrics, Emory University, Atlanta, GA, United States Division of Cardiology, Department of Medicine, Harvard University, Boston, MA, United States Division of Pediatric Cardiology, Department of Pediatrics, University of Cincinnati, Cincinnati, OH, United States AU - Hebson, C. AU - Book, W. AU - Elder, R. W. AU - Ford, R. AU - Jokhadar, M. AU - Kanter, K. AU - Kogon, B. AU - Kovacs, A. H. AU - Levit, R. D. AU - Lloyd, M. AU - Maher, K. AU - Reshamwala, P. AU - Rodriguez, F. AU - Romero, R. AU - Tejada, T. AU - Marie Valente, A. AU - Veldtman, G. AU - McConnell, M. DB - Scopus DO - 10.1111/chd.12407 IS - 1 KW - cardiac surgery circulatory failure cirrhosis congenital heart disease Fontan hepatology M3 - Article N1 - Cited By :6 Export Date: 15 June 2020 PY - 2017 SP - 6-16 ST - “Frontiers in Fontan failure: A summary of conference proceedings” T2 - Congenital Heart Disease TI - “Frontiers in Fontan failure: A summary of conference proceedings” UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84992497191&doi=10.1111%2fchd.12407&partnerID=40&md5=3fdfac7ca4c84070ce4455bf56af13cf VL - 12 ID - 1880 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 - E.R. Hedlund, Department of Women's and Children's Health, Karolinska Institutet, Astrid Lindgren Children's Hospital Solna, Eugeniavägen 23, Stockholm, Sweden AU - Hedlund, E. R. AU - Lundell, B. AU - Soderstrom, L. AU - Sjoberg, G. DB - Embase Medline DO - 10.1017/S1047951117002360 IS - 3 KW - bicycle ergometer carbon dioxide oxygen adolescent article cardiopulmonary exercise test child controlled study endurance training exercise female human major clinical study male muscle weakness oxygen consumption Pediatric Quality of Life Inventory physical capacity quality of life questionnaire scoring system self concept six minute walk test social psychology LA - English M3 - Article N1 - L620783570 2018-02-27 2018-10-15 PY - 2018 SN - 1467-1107 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://www.embase.com/search/results?subaction=viewrecord&from=export&id=L620783570 http://dx.doi.org/10.1017/S1047951117002360 VL - 28 ID - 698 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. © Cambridge University Press 2017A. AD - Department of Women's and Children's Health, Karolinska Institutet, Astrid Lindgren Children's Hospital Solna, Eugeniavägen 23, Stockholm, S-171 76, Sweden AU - Hedlund, E. R. AU - Lundell, B. AU - Soderstrom, L. AU - Sjoberg, G. DB - Scopus DO - 10.1017/S1047951117002360 IS - 3 KW - Endurance training Fontan palliation Physical capacity Quality of life M3 - Article N1 - Cited By :9 Export Date: 15 June 2020 PY - 2018 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85042227462&doi=10.1017%2fS1047951117002360&partnerID=40&md5=d38b7c51f36a639de86971c6f5b9c372 VL - 28 ID - 1815 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 - E.R. Hedlund, Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden AU - Hedlund, E. R. AU - Lundell, B. AU - Villard, L. AU - Sjöberg, G. DB - Embase Medline DO - 10.1111/apa.13544 IS - 11 KW - acetylsalicylic acid captopril enalapril accelerometer adolescent adult age distribution anticoagulation article child circulation clinical article controlled clinical trial controlled study exercise exercise intensity female Fontan procedure human male cardiac rhythm management device palliative therapy physical performance priority journal quality of life questionnaire self report time LA - English M3 - Article N1 - L612950720 2016-11-02 2016-11-08 PY - 2016 SN - 1651-2227 0803-5253 SP - 1322-1328 ST - Reduced physical exercise and health-related quality of life after Fontan palliation T2 - Acta Paediatrica, International Journal of Paediatrics TI - Reduced physical exercise and health-related quality of life after Fontan palliation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L612950720 http://dx.doi.org/10.1111/apa.13544 VL - 105 ID - 789 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 Department of Physiotherapy, Karolinska University Hospital, Stockholm Sweden Division of Physiotherapy, Department of Neurobiology Care Sciences and Society, Karolinska Institutet, Stockholm Sweden AN - 118687821. Language: English. Entry Date: 20170322. Revision Date: 20190619. Publication Type: journal article AU - Hedlund, Eva R. AU - Lundell, Bo AU - Villard, Li AU - Sjöberg, Gunnar DB - ccm DO - 10.1111/apa.13544 DP - EBSCOhost IS - 11 KW - Cardiopulmonary Bypass -- Rehabilitation Quality of Life Tricuspid Atresia -- Surgery Exercise Exercise -- Psychosocial Factors Exercise Tolerance -- Physiology Sweden Female Adolescence Cardiopulmonary Bypass -- Adverse Effects Case Control Studies Accelerometry -- Equipment and Supplies Self Report Survival Analysis Accelerometry -- Methods Young Adult Cardiopulmonary Bypass -- Psychosocial Factors Child Male Linear Regression Human N1 - research; tables/charts. Journal Subset: Biomedical; Continental Europe; Europe; Peer Reviewed. NLM UID: 9205968. PMID: NLM27515293. PY - 2016 SN - 0803-5253 SP - 1322-1328 ST - Reduced physical exercise and health-related quality of life after Fontan palliation T2 - Acta Paediatrica TI - Reduced physical exercise and health-related quality of life after Fontan palliation UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=118687821&site=ehost-live&scope=site VL - 105 ID - 1498 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. ©2016 Foundation Acta Pædiatrica. Published by John Wiley & Sons Ltd AD - 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 AU - Hedlund, E. R. AU - Lundell, B. AU - Villard, L. AU - Sjöberg, G. DB - Scopus DO - 10.1111/apa.13544 IS - 11 KW - Accelerometer Fontan circulation Fontan palliation Physical exercise Quality of life M3 - Article N1 - Cited By :11 Export Date: 15 June 2020 PY - 2016 SP - 1322-1328 ST - Reduced physical exercise and health-related quality of life after Fontan palliation T2 - Acta Paediatrica, International Journal of Paediatrics TI - Reduced physical exercise and health-related quality of life after Fontan palliation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85015578810&doi=10.1111%2fapa.13544&partnerID=40&md5=4809dbcf5fe7ba505fffe1811dfac596 VL - 105 ID - 1886 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 - D.A. Hehir, Nemours Cardiac Center, AI Dupont Hospital for Children, 1600 Rockland Road, Wilmington, DE, United States AU - Hehir, D. A. AU - Easley, R. B. AU - Byrnes, J. DB - Embase Medline DO - 10.1177/2150135115615847 IS - 2 KW - analgesic agent blood clotting factor heparin sedative agent analgesia anesthesia level anticoagulation article blood clotting coronary care unit cardiac patient chronic pain congenital heart disease feeding difficulty growth disorder heart single ventricle heart surgery heparin induced thrombocytopenia human hypoplastic left heart syndrome inflammation intensive care unit multiple organ failure outcome assessment partial thromboplastin time perioperative period priority journal quality of life scoring system body weight gain LA - English M3 - Article N1 - L608976914 2016-03-21 2020-03-03 PY - 2016 SN - 2150-136X 2150-1351 SP - 199-209 ST - Noncardiac Challenges in the Cardiac ICU: Feeding, Growth and Gastrointestinal Complications, Anticoagulation, and Analgesia T2 - World Journal for Pediatric and Congenital Heart Surgery TI - Noncardiac Challenges in the Cardiac ICU: Feeding, Growth and Gastrointestinal Complications, Anticoagulation, and Analgesia UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L608976914 http://dx.doi.org/10.1177/2150135115615847 VL - 7 ID - 820 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. © Translational Pediatrics. All rights reserved. AD - Department of Biomedical Engineering, School of Engineering, Virginia Commonwealth University, Richmond, VA, United States BioCirc Research Laboratory, School of Biomedical Engineering, Science and Health Systems, Drexel University, Philadelphia, PA, United States Division of Pediatric Cardiology, Children’s Hospital of Richmond, School of Medicine, Virginia Commonwealth University, Richmond, VA, United States AU - Hernandez, J. AU - Chopski, S. G. AU - Lee, S. AU - Moskowitz, W. B. AU - Throckmorton, A. L. DB - Scopus DO - 10.21037/tp.2017.08.01 IS - 1 KW - Congenital heart disease (CHD) Enhanced external counterpulsation (EECP) Single ventricle physiology M3 - Article N1 - Export Date: 15 June 2020 PY - 2018 SP - 14-22 ST - Externally applied compression therapy for Fontan patients T2 - Translational Pediatrics TI - Externally applied compression therapy for Fontan patients UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85042373685&doi=10.21037%2ftp.2017.08.01&partnerID=40&md5=737b1cf3b042f1834c5b7522c435e4bc VL - 7 ID - 1832 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 developed 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. Parents 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. © Georg Thieme Verlag KG Stuttgart New York. AD - A. Heusch, Abteilung Paediatrische Kardiologie/Pulmonologie, Helios-Klinikum Wuppertal, Heusnerstrasse 40, 42238 Wuppertal, Germany AU - Heusch, A. AU - Calaminus, G. AU - Kahl, J. AU - Schmidt, K. DB - Embase Medline DO - 10.1055/s-0034-1382019 IS - 5 KW - adolescent adult age distribution article body image child clinical evaluation congenital heart disease controlled study emotionality Fallot tetralogy family interaction female human major clinical study male mental capacity outpatient patient autonomy physical capacity plastic surgery quality of life questionnaire rating scale school child sex ratio treatment duration young adult LA - English M3 - Article N1 - L53263890 2014-08-01 2014-09-09 PY - 2014 SN - 1439-3824 0300-8630 SP - 281-286 ST - Health related quality of life after corrective surgery for congenital heart disease T2 - Klinische Padiatrie TI - Health related quality of life after corrective surgery for congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L53263890 http://dx.doi.org/10.1055/s-0034-1382019 VL - 226 ID - 960 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 developed 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. Parents 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. © Georg Thieme Verlag KG Stuttgart New York. AD - Abteilung Paediatrische Kardiologie/Pulmonologie, Helios-Klinikum Wuppertal, Heusnerstrasse 40, 42238 Wuppertal, Germany Pediatric Oncology, University Muenster, Muenster, Germany Kinderkardiologische Praxis, Uhlandstrasse, Duesseldorf, Germany Pediatric Cardiology, University Duesseldorf, Duesseldorf, Germany AU - Heusch, A. AU - Calaminus, G. AU - Kahl, J. AU - Schmidt, K. DB - Scopus DO - 10.1055/s-0034-1382019 IS - 5 KW - children congenital health related quality heart defect parents M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2014 SP - 281-286 ST - Health related quality of life after corrective surgery for congenital heart disease T2 - Klinische Padiatrie TI - Health related quality of life after corrective surgery for congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84906937764&doi=10.1055%2fs-0034-1382019&partnerID=40&md5=225c0e093be82f805386a55bd6fbd9b3 VL - 226 ID - 2009 ER - TY - JOUR AB - Summary: 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 - A. Heusch, Wuppertal, Germany AU - Heusch, A. AU - Kahl, H. J. AU - Hensel, K. O. AU - Calaminus, G. DB - Embase Medline DO - 10.1007/s11136-017-1653-y IS - 11 KW - arterial switch operation article body image child clinical assessment controlled study Fallot tetralogy female great vessels transposition heart single ventricle heart ventricle septum defect human major clinical study male patient-reported outcome physical capacity plastic surgery priority journal quality of life school child social status surgical technique LA - English M3 - Article N1 - L617431399 2017-07-26 2017-11-07 PY - 2017 SN - 1573-2649 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://www.embase.com/search/results?subaction=viewrecord&from=export&id=L617431399 http://dx.doi.org/10.1007/s11136-017-1653-y VL - 26 ID - 726 ER - TY - JOUR AB - Summary: 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. (PsycINFO Database Record (c) 2019 APA, all rights reserved) AD - Heusch, A. AN - 2017-32054-001 AU - Heusch, A. AU - Kahl, H. J. AU - Hensel, K. O. AU - Calaminus, G. DB - psyh DO - 10.1007/s11136-017-1653-y DP - EBSCOhost IS - 11 KW - Congenital heart defect Quality of life Heart surgery Child Female Heart Defects, Congenital Humans Male Sickness Impact Profile Surveys and Questionnaires Congenital Disorders Pediatrics Health Related Quality of Life Test Construction N1 - Heart Surgery; Pediatrics; Quality of Life; Health Related Quality of Life. Minor Descriptor: Test Construction. Classification: Physical & Somatoform & Psychogenic Disorders (3290). Population: Human (10); Male (30); Female (40). Location: Germany. Age Group: Childhood (birth-12 yrs) (100); School Age (6-12 yrs) (180); Adolescence (13-17 yrs) (200); Adulthood (18 yrs & older) (300); Young Adulthood (18-29 yrs) (320). Tests & Measures: HRQoL and Relevant Patient Characteristics Questionnaire. Methodology: Empirical Study; Quantitative Study. Page Count: 7. Issue Publication Date: Nov, 2017. Publication History: First Posted Date: Jul 20, 2017; Accepted Date: Jul 13, 2017. Copyright Statement: Springer International Publishing AG. 2017. Sponsor: ‘‘Stiftung Kind und Jugend’’. Recipients: No recipient indicated PY - 2017 SN - 0962-9343 1573-2649 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: An International Journal of Quality of Life Aspects of Treatment, Care & Rehabilitation 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=psyh&AN=2017-32054-001&site=ehost-live&scope=site ORCID: 0000-0003-2805-1670 andreas.heusch@helios-kliniken.de VL - 26 ID - 1659 ER - TY - JOUR AB - Summary: 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. © 2017, Springer International Publishing AG. AD - Wuppertal, Germany Düsseldorf, Germany Bonn, Germany AU - Heusch, A. AU - Kahl, H. J. AU - Hensel, K. O. AU - Calaminus, G. DB - Scopus DO - 10.1007/s11136-017-1653-y IS - 11 KW - Congenital heart defect Heart surgery Quality of life M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2017 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85025082819&doi=10.1007%2fs11136-017-1653-y&partnerID=40&md5=e475d897452165f3818a8863d72da30b VL - 26 ID - 1841 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. Interpretation: Despite 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 - K.N. Heye, Child Research Centre, University Children's Hospital, Steinwiesstrasse 75, Zurich, Switzerland 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. DB - Embase Medline DO - 10.1017/S1047951118001993 IS - 2 KW - article brain child clinical article cohort analysis controlled study female follow up heart single ventricle human male multiple linear regression analysis nuclear magnetic resonance imaging prematurity preschool child quality of life risk factor LA - English M3 - Article N1 - L626484853 2019-02-27 PY - 2019 SN - 1467-1107 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://www.embase.com/search/results?subaction=viewrecord&from=export&id=L626484853 http://dx.doi.org/10.1017/S1047951118001993 VL - 29 ID - 631 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. Interpretation: Despite 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. © 2018 Cambridge University Press. AD - Child Research Centre, University Children's Hospital, Steinwiesstrasse 75, Zurich, 8032, 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 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. DB - Scopus DO - 10.1017/S1047951118001993 IS - 2 KW - CHD Health-related quality of life risk factors single-ventricle M3 - Article N1 - Export Date: 15 June 2020 PY - 2019 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85061857012&doi=10.1017%2fS1047951118001993&partnerID=40&md5=785d41bafc2b319d88a234adcaa2a1ca VL - 29 ID - 1762 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, noenatologists, 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 - S.S. Higgins, Division of Pediatric Cardiology, Children's Hospital Oakland, 747 52nd St., Oakland, CA 94609, United States AU - Higgins, S. S. AU - Paul, S. M. AU - Hardy, C. E. AU - Ternullo-Retta, C. AU - Affonso, D. DB - Embase Medline IS - 1 I KW - article fatality heart disease heart transplantation human medical decision making medical practice newborn surgery physician attitude priority journal LA - English M3 - Article N1 - L24068940 1994-03-16 PY - 1994 SN - 1053-2498 SP - 59-65 ST - Infant heart transplantation: A survey of physician beliefs T2 - Journal of Heart and Lung Transplantation TI - Infant heart transplantation: A survey of physician beliefs UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L24068940 VL - 13 ID - 1375 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 - S.S. Higgins, Division of Pediatric Cardiology, Children's Hospital, Oakland, CA 94609. AU - Higgins, S. S. AU - Paul, S. M. AU - Hardy, C. E. AU - Ternullo-Retta, C. AU - Affonso, D. DB - Medline IS - 1 Pt 1 KW - article cardiology congenital heart malformation counseling female health maintenance organization health personnel attitude heart transplantation human human relation male middle aged neonatology newborn parent patient attitude patient participation pediatrics physician private practice university hospital LA - English M3 - Article N1 - L24971308 1994-01-01 PY - 1994 SN - 1053-2498 SP - 59-65 ST - Infant heart transplantation: a survey of physician beliefs T2 - The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation TI - Infant heart transplantation: a survey of physician beliefs UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L24971308 VL - 13 ID - 1377 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, noenatologists, 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, 747 52nd St., Oakland, CA 94609, United States AU - Higgins, S. S. AU - Paul, S. M. AU - Hardy, C. E. AU - Ternullo-Retta, C. AU - Affonso, D. DB - Scopus IS - 1 I M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 1994 SP - 59-65 ST - Infant heart transplantation: A survey of physician beliefs T2 - Journal of Heart and Lung Transplantation TI - Infant heart transplantation: A survey of physician beliefs UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0028118876&partnerID=40&md5=ca106d17e61628c81ffaa4c296ba56a5 VL - 13 ID - 2310 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. © 2013 Wiley Periodicals, Inc. AD - G. Hill, Children's Hospital of Wisconsin, Department of Pediatric Cardiology, 9000 W Wisconsin Ave., Milwaukee, WI 53226, United States AU - Hill, G. AU - Silverman, A. AU - Noel, R. AU - Bartz, P. J. DB - Embase Medline DO - 10.1111/chd.12071 IS - 1 KW - article behavior body weight caregiver child clinical article controlled study distress syndrome eating feeding feeding behavior feeding disorder female heart single ventricle human male parent parental stress priority journal questionnaire social interaction LA - English M3 - Article N1 - L52579289 2013-05-14 2014-02-04 PY - 2014 SN - 1747-079X 1747-0803 SP - 26-29 ST - Feeding dysfunction in single ventricle patients with feeding disorder T2 - Congenital Heart Disease TI - Feeding dysfunction in single ventricle patients with feeding disorder UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52579289 http://dx.doi.org/10.1111/chd.12071 VL - 9 ID - 939 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. © 2013 Wiley Periodicals, Inc. AD - Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, Milwaukee, WI, United States Department of Pediatrics, Division of Gastroenterology, Medical College of Wisconsin, Milwaukee, WI, United States Department of Internal Medicine, Division of Adult Cardiovascular Medicine, Medical College of Wisconsin, Milwaukee, WI, United States AU - Hill, G. AU - Silverman, A. AU - Noel, R. AU - Bartz, P. J. DB - Scopus DO - 10.1111/chd.12071 IS - 1 KW - Feeding Growth Nutrition Single Ventricle M3 - Article N1 - Cited By :8 Export Date: 15 June 2020 PY - 2014 SP - 26-29 ST - Feeding dysfunction in single ventricle patients with feeding disorder T2 - Congenital Heart Disease TI - Feeding dysfunction in single ventricle patients with feeding disorder UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84892884329&doi=10.1111%2fchd.12071&partnerID=40&md5=b71d58de14e9addd1d2709bc951382a0 VL - 9 ID - 2016 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 - G.D. Hill, Children's Hospital of Wisconsin, 9000 W Wisconsin Ave., Milwaukee, WI 53226, United States 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. DB - Embase Medline DO - 10.1016/j.jpeds.2013.09.030 IS - 2 KW - airway obstruction article child childhood disease controlled study disease association feeding disorder female follow up food aversion gastrointestinal symptom heart single ventricle height household human major clinical study male medical record review palliative therapy preschool child prevalence priority journal prospective study risk factor single parent stomach tube vomiting weight LA - English M3 - Article N1 - L52827638 2013-10-28 2014-01-31 PY - 2014 SN - 0022-3476 1097-6833 SP - 243-246.e1 ST - Feeding dysfunction in children with single ventricle following staged palliation T2 - Journal of Pediatrics TI - Feeding dysfunction in children with single ventricle following staged palliation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52827638 http://dx.doi.org/10.1016/j.jpeds.2013.09.030 VL - 164 ID - 921 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 - G.D. Hill, Children's Hospital of Wisconsin, 9000W Wisconsin Ave, Milwaukee, WI, United States 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. DB - Embase DO - 10.1016/j.jpeds.2013.09.030 IS - 2 KW - article body height body weight child childhood disease clinical article cohort analysis comparative study controlled study feeding behavior feeding disorder female follow up gastrointestinal symptom heart single ventricle human hypoplastic left heart syndrome larynx injury male medical record review palliative therapy prospective study retrospective study stomach tube vomiting LA - English M3 - Article N1 - L600478324 2014-11-25 2014-12-05 PY - 2014 SN - 1097-6833 0022-3476 SP - 243-246.e1 ST - Feeding dysfunction in children with single ventricle following staged palliation T2 - Journal of Pediatrics TI - Feeding dysfunction in children with single ventricle following staged palliation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L600478324 http://dx.doi.org/10.1016/j.jpeds.2013.09.030 VL - 164 ID - 965 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 - 104011876. Language: English. Entry Date: 20140404. Revision Date: 20161117. Publication Type: journal article AU - Hill, Garick D. AU - Silverman, Alan H. AU - Noel, Richard J. AU - Simpson, Pippa M. AU - Slicker, Julie AU - Scott, Ann E. AU - Bartz, Peter J. DB - ccm DO - 10.1016/j.jpeds.2013.09.030 DP - EBSCOhost IS - 2 KW - Eating Disorders -- Etiology Enteral Nutrition -- Methods Eating Behavior Heart Defects, Congenital -- Surgery Heart Ventricle -- Abnormalities Palliative Care -- Methods Body Weight Child Child, Preschool Eating Disorders -- Epidemiology Eating Disorders -- Physiopathology Female Prospective Studies Heart Ventricle -- Surgery Human Male Postoperative Period Prevalence Questionnaires Retrospective Design Treatment Outcomes Wisconsin N1 - research; randomized controlled trial. Journal Subset: Biomedical; Peer Reviewed; USA. Special Interest: Pediatric Care. Grant Information: UL1RR031973/RR/NCRR NIH HHS/United States. NLM UID: 0375410. PMID: NLM24161218. PY - 2014 SN - 0022-3476 SP - 243-246.e1 ST - Feeding dysfunction in children with single ventricle following staged palliation T2 - Journal of Pediatrics TI - Feeding dysfunction in children with single ventricle following staged palliation UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104011876&site=ehost-live&scope=site VL - 164 ID - 1632 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. © 2012 Springer Science+Business Media, LLC. AD - D. Hilton-Kamm, California Heart Connection, Irvine CA, United States AU - Hilton-Kamm, D. AU - Chang, R. K. AU - Sklansky, M. DB - Embase Medline DO - 10.1007/s00246-012-0366-9 IS - 8 KW - adult article cardiologist empathy family decision making female human hypoplastic left heart syndrome life expectancy major clinical study male optimism parent counseling parental attitude perception pregnancy termination prenatal diagnosis prognosis support group LA - English M3 - Article N1 - L52024436 2012-05-25 2013-10-18 PY - 2012 SN - 0172-0643 1432-1971 SP - 1402-1410 ST - Prenatal diagnosis of hypoplastic left heart syndrome: Impact of counseling patterns on parental perceptions and decisions regarding termination of pregnancy T2 - Pediatric Cardiology TI - Prenatal diagnosis of hypoplastic left heart syndrome: Impact of counseling patterns on parental perceptions and decisions regarding termination of pregnancy UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52024436 http://dx.doi.org/10.1007/s00246-012-0366-9 VL - 33 ID - 1035 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. © 2013 Springer Science+Business Media New York. AD - D. Hilton-Kamm, California Heart Connection, Irvine, CA, United States AU - Hilton-Kamm, D. AU - Sklansky, M. AU - Chang, R. K. DB - Embase Medline DO - 10.1007/s00246-013-0765-6 IS - 2 KW - adult age distribution article birth child death childhood disease congenital heart disease doctor patient relationship educational status empathy female follow up health survey human human experiment hypoplastic left heart syndrome Internet life expectancy long term care male medical information medical terminology optimism parent parent counseling pediatric cardiology perception perinatal period personal experience physician attitude pregnancy termination prenatal diagnosis support group survivor United States LA - English M3 - Article N1 - L52720372 2013-08-12 2014-02-11 PY - 2014 SN - 0172-0643 1432-1971 SP - 239-252 ST - How not to tell parents about their child's new diagnosis of congenital heart disease: An internet survey of 841 parents T2 - Pediatric Cardiology TI - How not to tell parents about their child's new diagnosis of congenital heart disease: An internet survey of 841 parents UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52720372 http://dx.doi.org/10.1007/s00246-013-0765-6 VL - 35 ID - 920 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. © 2013 Springer Science+Business Media New York. AD - California Heart Connection, Irvine, CA, United States David Geffen School of Medicine at UCLA, Los Angeles, CA, United States Harbor-UCLA Medical Center, Torrance, CA, United States AU - Hilton-Kamm, D. AU - Sklansky, M. AU - Chang, R. K. DB - Scopus DO - 10.1007/s00246-013-0765-6 IS - 2 KW - Congenital heart disease Counseling Hypoplastic left heart syndrome Internet survey Parent perceptions Psychosocial needs Support groups M3 - Article N1 - Cited By :16 Export Date: 15 June 2020 PY - 2014 SP - 239-252 ST - How not to tell parents about their child's new diagnosis of congenital heart disease: An internet survey of 841 parents T2 - Pediatric Cardiology TI - How not to tell parents about their child's new diagnosis of congenital heart disease: An internet survey of 841 parents UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84893644145&doi=10.1007%2fs00246-013-0765-6&partnerID=40&md5=2fadc3f3b7b6408c26707697aa075a57 VL - 35 ID - 1996 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 49.0kg, 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. © 2011 European Association for Cardio-Thoracic Surgery. AD - Department of Cardiovascular Surgery, Tokyo Women's Medical University, Tokyo, Japan AU - Hiramatsu, T. AU - Iwata, Y. AU - Matsumura, G. AU - Konuma, T. AU - Yamazaki, K. DB - Scopus DO - 10.1016/j.ejcts.2011.01.022 IS - 4 KW - Arrhythmia surgery Fontan conversion Pacemaker therapy M3 - Article N1 - Cited By :23 Export Date: 15 June 2020 PY - 2011 SP - 1007-1010 ST - Impact of Fontan conversion with arrhythmia surgery and pacemaker therapy T2 - European Journal of Cardio-thoracic Surgery TI - Impact of Fontan conversion with arrhythmia surgery and pacemaker therapy UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-80052966318&doi=10.1016%2fj.ejcts.2011.01.022&partnerID=40&md5=9f127e36c4f784def0d23ad931d91fd5 VL - 40 ID - 2115 ER - TY - JOUR AB - Objective: Children and adolescents with an univentricular heart after total cavopulmonary connection (TCPC) have functional impairments. This study assesses health-related physical fitness (HRPF) and exercise capacity, as well as their relation to health-related quality of life (HRQoL) in patients with an univentricular heart after total-pulmonary connection (TCPC). Patients and methods: Between July 2014 and October 2016 a total of 78 children and adolescents with TCPC (12.0 ± 3.2 years, 21 female) performed a motor test including five tasks for strength and flexibility during their routine follow-up appointment. They also underwent a symptom limited cardio-pulmonary exercise test and filled in a HRQoL questionnaire (KINDL-R). Patients' data were compared to a recent sample of healthy children (n = 1650, 12.6 ± 2.4 years, 49% female). Results: Multivariable regressions corrected for sex, age and BMI showed that TCPC patients achieved 12.4 repetitions of curl-ups (p < 0.001) and 2.6 push-ups (p = 0.010) less than healthy counterparts. They had impairments in trunk (− 8.5 cm; p < 0.001), shoulder (− 7.5 cm; p < 0.001) and lower limb flexibility (− 4.7 cm; p < 0.001). Peak oxygen uptake was reduced to 34.8 ± 7.5 ml/min/kg and 77.7% respectively, compared to peers (p < 0.001). Ventilatory efficiency was also impaired (healthy: 27.5 ± 2.9 vs. TCPC: 31.6 ± 3.3; p < 0.001). HRQoL did not differ significantly (p = 0.233). Conclusions: Children and adolescents with TCPC still present impaired HRPF and exercise capacity whereas HRQoL is similar to healthy peers. Since low HRPF may yield to worse motor competence and exercise capacity, early screening for HRPF and early treatment, if indicated, is recommended. © 2017 Elsevier Ireland Ltd AD - Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Germany Institute of Preventive Pediatrics, Technische Universität München, Germany AU - Hock, J. AU - Reiner, B. AU - Neidenbach, R. C. AU - Oberhoffer, R. AU - Hager, A. AU - Ewert, P. AU - Müller, J. DB - Scopus DO - 10.1016/j.ijcard.2017.11.092 KW - Congenital heart disease Fontan Motor competence Peak oxygen uptake Univentricular heart M3 - Article N1 - Cited By :12 Export Date: 15 June 2020 PY - 2018 SP - 50-54 ST - Functional outcome in contemporary children with total cavopulmonary connection – Health-related physical fitness, exercise capacity and health-related quality of life T2 - International Journal of Cardiology TI - Functional outcome in contemporary children with total cavopulmonary connection – Health-related physical fitness, exercise capacity and health-related quality of life UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85041651770&doi=10.1016%2fj.ijcard.2017.11.092&partnerID=40&md5=551256570b9d21915b65cdd63e814656 VL - 255 ID - 1811 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). Successfull 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. © 2008, the Authors Journal compilation © 2008, Blackwell Publishing, Inc. AD - A. Hoffmann, Lange Gasse 78, CH 4052 Basel, Switzerland AU - Hoffmann, A. AU - Wyler, F. AU - Günthard, J. AU - Grädel, E. DB - Embase Medline DO - 10.1111/j.1747-0803.2007.00166.x IS - 2 KW - adult article artificial heart pacemaker bacterial endocarditis cerebrovascular accident child health clinical article congenital heart disease female follow up heart arrhythmia heart surgery heart transplantation human hypoxia long term care male mortality palliative therapy pregnancy outcome priority journal prognosis survival rate survival time LA - English M3 - Article N1 - L351467921 2008-04-15 PY - 2008 SN - 1747-079X 1747-0803 SP - 155-158 ST - Late follow-up of patients who underwent palliation for complex congenital heart disease in childhood T2 - Congenital Heart Disease TI - Late follow-up of patients who underwent palliation for complex congenital heart disease in childhood UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L351467921 http://dx.doi.org/10.1111/j.1747-0803.2007.00166.x VL - 3 ID - 1219 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). Successfull 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. © 2008, the Authors Journal compilation © 2008, Blackwell Publishing, Inc. AD - University Hospital, Division of Cardiology, Basel, Switzerland University Hospital, Division of Cardiac Surgery, Basel, Switzerland University Children's Hospital, Department of Pediatrics, Basel, Switzerland Lange Gasse 78, CH 4052 Basel, Switzerland AU - Hoffmann, A. AU - Wyler, F. AU - Günthard, J. AU - Grädel, E. DB - Scopus DO - 10.1111/j.1747-0803.2007.00166.x IS - 2 KW - Congenital Heart Disease Long-Term Palliation M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2008 SP - 155-158 ST - Late follow-up of patients who underwent palliation for complex congenital heart disease in childhood T2 - Congenital Heart Disease TI - Late follow-up of patients who underwent palliation for complex congenital heart disease in childhood UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-41549165684&doi=10.1111%2fj.1747-0803.2007.00166.x&partnerID=40&md5=f82f7a0251cdcd798db2579c14f2ac46 VL - 3 ID - 2199 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. © 2018 Wiley Periodicals, Inc. AD - Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States University of Louisville, Louisville, KY, United States KU Leuven, Leuven, Belgium University of Gothenburg, Gothenburg, Sweden Amsterdam Medical Center, Amsterdam, 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, OH, United States University Hospitals Leuven and Department of Cardiovascular Sciences, Leuven, Belgium Oregon Health & Science University, Portland, OR, United States 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, CA, United States Mater Dei Hospital, Msida, Malta Monash Medical Center, Melbourne, Australia Helen DeVos Children's Hospital, Grand Rapids, MI, United States University of Alberta, Edmonton, Canada Washington University and Barnes Jewish Heart & Vascular Center, University of Missouri, Saint Louis, MO, United States Montreal Heart Institute, Montreal, Canada Children's Hospital & Medical Center, Omaha, NE, United States 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. AU - consortium, Approach-Is AU - the International Society for Adult Congenital Heart, Disease DB - Scopus DO - 10.1111/chd.12583 IS - 3 KW - congenital heart disease Fontan circulation illness perceptions quality of life M3 - Article N1 - (ISACHD) Cited By :10 Export Date: 15 June 2020 PY - 2018 SP - 392-400 ST - A multinational observational investigation of illness perceptions and quality of life among patients with a Fontan circulation T2 - Congenital Heart Disease TI - A multinational observational investigation of illness perceptions and quality of life among patients with a Fontan circulation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85048272375&doi=10.1111%2fchd.12583&partnerID=40&md5=064006295903e183ec447f97422c0497 VL - 13 ID - 1807 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 (Mage = 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. © 2019 Elsevier Inc. AD - Department of Child and Adolescent Psychiatry and Behavioral Sciences, Children's Hospital of Philadelphia, Philadelphia, PA, United States 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, OR, United States School Psychology and Development in Context, KU Leuven - University of Leuven, Leuven, Belgium UNIBS, University of the Free State Bloemfontein, Bloemfontein, South Africa 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, OH, United States 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, CA, United States 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 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, NE, United States 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, MO, United States Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands Clinical Psychology Service, IRCCS Policlinico San Donato, Milan, Italy Adult Congenital Heart Disease Center, Helen DeVos Children's Hospital, Grand Rapids, MI, United States Adult Congenital Heart Center, Montreal Heart Institute, Université de Montréal, Montreal, Canada The University of Texas Southwestern Medical Center, Dallas, TX, United States 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. AU - consortium, Approach-Is AU - the International Society for Adult Congenital Heart, Disease DB - Scopus DO - 10.1016/j.amjcard.2019.03.039 IS - 1 M3 - Article N1 - (ISACHD) Cited By :1 Export Date: 15 June 2020 PY - 2019 SP - 144-150 ST - Perceived Health Mediates Effects of Physical Activity on Quality of Life in Patients With a Fontan Circulation T2 - American Journal of Cardiology TI - Perceived Health Mediates Effects of Physical Activity on Quality of Life in Patients With a Fontan Circulation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85064899686&doi=10.1016%2fj.amjcard.2019.03.039&partnerID=40&md5=d987d59668ffc2d2b6320ff6e160d017 VL - 124 ID - 1745 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. AD - Stamford University, Birmingham, United States AU - Holston, J. T. DB - Scopus DO - 10.1097/CNJ.0000000000000118 IS - 1 M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2015 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84929502494&doi=10.1097%2fCNJ.0000000000000118&partnerID=40&md5=28d1588134f0ca66867f594235841dc2 VL - 32 ID - 1962 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 height2.7 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 - G.D. Hill, Children’s Hospital of Wisconsin, 9000 W. Wisconsin Ave., Milwaukee, WI, United States AU - Horriat, N. L. AU - Deatsman, S. L. AU - Stelter, J. AU - Frommelt, P. C. AU - Hill, G. D. DB - Embase Medline DO - 10.1007/s00246-016-1471-y IS - 8 KW - article body surface clinical article cohort analysis controlled study early intervention echocardiography female follow up Fontan procedure Glenn shunt heart ejection fraction heart function heart left ventricle enddiastolic volume heart left ventricle mass heart single left ventricle heart single ventricle heart surgery heart ventricle remodeling heart volume height human infant infant disease male newborn Norwood procedure palliative therapy tricuspid valve atresia LA - English M3 - Article N1 - L611841178 2016-08-31 2016-12-14 PY - 2016 SN - 1432-1971 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 - Pediatric Cardiology TI - Variable Myocardial Response to Load Stresses in Infants with Single Left Ventricular Anatomy: Influence of Initial Physiology and Surgical Palliative Strategy UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L611841178 http://dx.doi.org/10.1007/s00246-016-1471-y VL - 37 ID - 784 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 height2.7 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. © 2016, Springer Science+Business Media New York. AD - Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI 53226, United States Department of Obstetrics and Gynecology, University of Florida, PO Box 100294, Gainesville, FL 32610, United States Children’s Hospital of Wisconsin, 9000 W. Wisconsin Ave., Milwaukee, WI 53226, United States AU - Horriat, N. L. AU - Deatsman, S. L. AU - Stelter, J. AU - Frommelt, P. C. AU - Hill, G. D. DB - Scopus DO - 10.1007/s00246-016-1471-y IS - 8 KW - Echocardiography Fontan Glenn Single ventricle M3 - Article N1 - Export Date: 15 June 2020 PY - 2016 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 - Pediatric Cardiology TI - Variable Myocardial Response to Load Stresses in Infants with Single Left Ventricular Anatomy: Influence of Initial Physiology and Surgical Palliative Strategy UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84983384918&doi=10.1007%2fs00246-016-1471-y&partnerID=40&md5=82686eac1f195a8d1488e76564571bb1 VL - 37 ID - 1883 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 - P.R. Houin, Pediatric Pulmonary Division, Department of Pediatrics, 12700 E. 19th Ave. Mail Stop 8615, Aurora, CO, United States 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 - Sigma DB - Embase Medline DO - 10.1002/ppul.23043 IS - 2 KW - 2 chloroethyl ethyl sulfide blood clotting factor 10a heparin mustard gas mustard gas derivative phosphate buffered saline unclassified drug aerosol animal experiment animal model animal tissue article blood analysis bronchitis controlled study cost effectiveness analysis drug absorption erythrocyte hypoxemia lung lavage male microdissection morbidity mortality nonhuman oxygen saturation partial thromboplastin time plastic bronchitis priority journal rat Sprague Dawley rat survival thrombin time LA - English M3 - Article N1 - L53087042 2014-04-11 2015-01-27 PY - 2015 SN - 1099-0496 8755-6863 SP - 118-126 ST - Intratracheal heparin improves plastic bronchitis due to sulfur mustard analog T2 - Pediatric Pulmonology TI - Intratracheal heparin improves plastic bronchitis due to sulfur mustard analog UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L53087042 http://dx.doi.org/10.1002/ppul.23043 VL - 50 ID - 877 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. © 2014 Wiley Periodicals, Inc. AD - Department of Pediatrics, University of Colorado Health Sciences Center, Aurora, CO, United States 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. DB - Scopus DO - 10.1002/ppul.23043 IS - 2 KW - CEES Heparin Inhalation injury Plastic bronchitis Pulmonology Sulfur mustard M3 - Article N1 - Cited By :11 Export Date: 15 June 2020 PY - 2015 SP - 118-126 ST - Intratracheal heparin improves plastic bronchitis due to sulfur mustard analog T2 - Pediatric Pulmonology TI - Intratracheal heparin improves plastic bronchitis due to sulfur mustard analog UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84920945187&doi=10.1002%2fppul.23043&partnerID=40&md5=f44b8e372afd589461d6a910816abede VL - 50 ID - 1978 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. © 2020 Georg Thieme Verlag KG Stuttgart New York. AD - Department of Pediatric Cardio-Thoracic Surgery, Shanghai Children's Medical Center, Dongfang Road 1678, Shanghai, 200127, China Department of Radiology, Shanghai Children's Medical Center, Shanghai, China Institute of Pediatric Translational Medicine, Shanghai Children's Medical Center, Shanghai, China AU - Hu, J. AU - Wang, Q. AU - Tong, Z. AU - Shen, J. AU - Liu, J. AU - Sun, Y. AU - Zhang, H. DB - Scopus DO - 10.1055/s-0038-1677490 IS - 1 KW - bioengineering cardiac CHD congenital heart disease pulmonary arteries/veins M3 - Article N1 - Export Date: 15 June 2020 PY - 2020 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85078711990&doi=10.1055%2fs-0038-1677490&partnerID=40&md5=0e3e4ca177dc680ebbebfe8d2c678dce VL - 68 ID - 1718 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. © 2009 Pulsus Group Inc. All rights reserved. AD - D.G. Human, BC Children's Hospital, BC Women's Hospital and Health Centre, Faculty of Medicine, 4480 Oak Street, Vancouver, BC V6H 3V4, Canada AU - Human, D. G. DB - Embase IS - 3 KW - article body growth career planning child growth clinical feature cognition congenital heart disease exercise tolerance Fontan procedure health status heart rhythm heart single ventricle heart ventricle function human quality of life surgical approach surgical risk survival rate survival time thromboembolism valvular heart disease LA - English M3 - Article N1 - L355234639 2009-10-30 http://www.pulsus.com/journals/pdf_frameset.jsp?jnlKy=5&atlKy=8642&isArt=t&jnlAdvert=Paeds&adverifHCTp=&sTitle=Living%20with%20complex%20congenital%20heart%20disease,%20Pulsus%20Group%20Inc&HCtype=Physician PY - 2009 SN - 1205-7088 SP - 161-166 ST - Living with complex congenital heart disease T2 - Paediatrics and Child Health TI - Living with complex congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L355234639 VL - 14 ID - 1173 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. © 2009 Pulsus Group Inc. All rights reserved. AD - BC Children's Hospital, BC Women's Hospital and Health Centre, Faculty of Medicine, 4480 Oak Street, Vancouver, BC V6H 3V4, Canada AU - Human, D. G. DB - Scopus IS - 3 KW - Congenital heart disease Fontan circulation Quality of life M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2009 SP - 161-166 ST - Living with complex congenital heart disease T2 - Paediatrics and Child Health TI - Living with complex congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-70049085870&partnerID=40&md5=fe0a3edfa1c2a9bf32564e96b5db70c4 VL - 14 ID - 2172 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 < 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. © 1986, American College of Cardiology Foundation. All rights reserved. AD - Indianapolis, Indiana, United States AU - Hurwitz, R. A. AU - Caldwell, R. L. AU - Girod, D. A. AU - Wellman, H. DB - Scopus DO - 10.1016/S0735-1097(86)80435-1 IS - 4 M3 - Article N1 - Cited By :12 Export Date: 15 June 2020 PY - 1986 SP - 916-921 ST - Left ventricular function in tricuspid atresia: A radionuclide study T2 - Journal of the American College of Cardiology TI - Left ventricular function in tricuspid atresia: A radionuclide study UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0022556335&doi=10.1016%2fS0735-1097%2886%2980435-1&partnerID=40&md5=bd11c57b87c86e716a4d041dd139e070 VL - 8 ID - 2328 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. © 2013 Elsevier Ireland Ltd. AD - L. Idorn, Blegdamsvej 9, DK-2100, Copenhagen East, Denmark 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. DB - Embase Medline DO - 10.1016/j.ijcard.2013.04.008 IS - 4 KW - adolescent article child child behavior cognition controlled study Denmark emotion female Fontan procedure functional assessment human hypoplastic left heart syndrome major clinical study male mental function mental health physical capacity priority journal quality of life questionnaire rating scale school child scoring system Short Form 36 social interaction LA - English M3 - Article N1 - L52556245 2013-05-01 2013-11-26 PY - 2013 SN - 0167-5273 1874-1754 SP - 3230-3235 ST - Quality of life and cognitive function in Fontan patients, a population-based study T2 - International Journal of Cardiology TI - Quality of life and cognitive function in Fontan patients, a population-based study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52556245 http://dx.doi.org/10.1016/j.ijcard.2013.04.008 VL - 168 ID - 985 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. © 2013 Elsevier Ireland Ltd. AD - Department of Cardiology, Rigshospitalet, Copenhagen, Denmark Department of Paediatrics, Section of Paediatric Cardiology, Rigshospitalet, Copenhagen, Denmark Department of Pulmonary and Infectious Diseases, Hilleroed Hospital, Hillerød, Denmark Department of Psychiatry, Hvidovre Hospital, Copenhagen, Denmark Department of Cardiology, Aarhus University Hospital, Skejby, Aarhus, Denmark 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. DB - Scopus DO - 10.1016/j.ijcard.2013.04.008 IS - 4 KW - Cognitive function Congenital heart disease Fontan patients Quality of life M3 - Article N1 - Cited By :37 Export Date: 15 June 2020 PY - 2013 SP - 3230-3235 ST - Quality of life and cognitive function in Fontan patients, a population-based study T2 - International Journal of Cardiology TI - Quality of life and cognitive function in Fontan patients, a population-based study UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84886243543&doi=10.1016%2fj.ijcard.2013.04.008&partnerID=40&md5=9a595317c69032dc51d10e13d0de9be3 VL - 168 ID - 2034 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 - A.A. Rosenberg, Univ. of Colorado Hlth. Sci. Center, Box B195, 4200 E 9th Ave, Denver, CO 80262, United States AU - Ikle, L. AU - Hale, K. AU - Fashaw, L. AU - Boucek, M. AU - Rosenberg, A. A. DB - Embase Medline DO - 10.1067/mpd.2003.mpd0340 IS - 1 KW - adaptive behavior article behavior disorder clinical article cognitive defect controlled study female heart transplantation human hypoplastic left heart syndrome infant intelligence quotient interpersonal communication male mental development mental performance outcomes research population priority journal socialization treatment outcome LA - English M3 - Article N1 - L36106382 2003-01-28 PY - 2003 SN - 0022-3476 SP - 20-25 ST - Developmental outcome of patients with hypoplastic left heart syndrome treated with heart transplantation T2 - Journal of Pediatrics TI - Developmental outcome of patients with hypoplastic left heart syndrome treated with heart transplantation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L36106382 http://dx.doi.org/10.1067/mpd.2003.mpd0340 VL - 142 ID - 1308 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 - D. Ilardi, Department of Neuropsychology, Children's Healthcare of Atlanta, Atlanta, GA, United States AU - Ilardi, D. AU - Ono, K. E. AU - McCartney, R. AU - Book, W. AU - Stringer, A. Y. DB - Embase Medline DO - 10.1111/chd.12434 IS - 2 KW - adult aging article cerebrovascular accident clinical article cognition comorbidity congenital heart disease controlled study cyanotic heart disease depth perception disability disease severity education employment executive function family female health care quality heart disease heart single ventricle human male mental disease neurologic disease neuropsychological test patient quality of life resource allocation risk factor screening seizure transitional care unemployment working memory young adult LA - English M3 - Article N1 - L613825907 2016-12-27 2017-04-18 PY - 2017 SN - 1747-0803 1747-079X SP - 166-173 ST - Neurocognitive functioning in adults with congenital heart disease T2 - Congenital Heart Disease TI - Neurocognitive functioning in adults with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L613825907 http://dx.doi.org/10.1111/chd.12434 VL - 12 ID - 766 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. © 2016 Wiley Periodicals, Inc. AD - Department of Neuropsychology, Children's Healthcare of Atlanta, Atlanta, GA, United States Department of Rehabilitation Medicine, Emory University, Atlanta, GA, United States Behavioral Health, Southeast Permanente Medical Group, Tucker, GA, United States Department of Internal Medicine, Division of Cardiology, Emory University, Atlanta, GA, United States AU - Ilardi, D. AU - Ono, K. E. AU - McCartney, R. AU - Book, W. AU - Stringer, A. Y. DB - Scopus DO - 10.1111/chd.12434 IS - 2 KW - adult congenital heart disease adult transition cognitive functioning executive functioning neurodevelopmental outcomes neuropsychological outcomes M3 - Article N1 - Cited By :16 Export Date: 15 June 2020 PY - 2017 SP - 166-173 ST - Neurocognitive functioning in adults with congenital heart disease T2 - Congenital Heart Disease TI - Neurocognitive functioning in adults with congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85006836443&doi=10.1111%2fchd.12434&partnerID=40&md5=6cc391be53512bd54973a417e04339bb VL - 12 ID - 1870 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. Copyright © 2011 by the American Academy of Pediatrics. AD - M.V. Ilina, Cardiothoracic Services, Freeman Hospital, Freeman Road, High Heaton, Newcastle Upon Tyne, NE7 7DN, United Kingdom AU - Ilina, M. V. AU - Kepron, C. A. AU - Taylor, G. P. AU - Perrin, D. G. AU - Kantor, P. F. AU - Somers, G. R. DB - Embase Medline DO - 10.1542/peds.2010-2307 IS - 3 KW - adolescent age distribution aortic stenosis article autopsy Canada child childhood congestive cardiomyopathy coronary artery anomaly demography female heart disease human hypoplastic left heart syndrome infant major clinical study male medical record review myocarditis newborn preschool child priority journal retrospective study school child sudden death symptom LA - English M3 - Article N1 - L362480621 2011-09-12 2011-09-16 http://pediatrics.aappublications.org/content/128/3/e513.full.pdf+html PY - 2011 SN - 0031-4005 1098-4275 SP - e513-e520 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L362480621 http://dx.doi.org/10.1542/peds.2010-2307 VL - 128 ID - 1092 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. Copyright © 2011 by the American Academy of Pediatrics. AD - Division of Pediatric Cardiology, Hospital for Sick Children, Toronto, ON, Canada Department of Laboratory Medicine and Pathobiology, Faculty of Medicine, University of Toronto, Toronto, ON, Canada Ontario Forensic Pathology Service, Toronto, ON, Canada Department of Pediatric Laboratory Medicine, Hospital for Sick Children, Toronto, ON, Canada AU - Ilina, M. V. AU - Kepron, C. A. AU - Taylor, G. P. AU - Perrin, D. G. AU - Kantor, P. F. AU - Somers, G. R. DB - Scopus DO - 10.1542/peds.2010-2307 IS - 3 KW - Cardiomyopathy Congenital heart disease Hypoplastic left heart syndrome Myocarditis Sudden cardiac death M3 - Article N1 - Cited By :11 Export Date: 15 June 2020 PY - 2011 SP - e513-e520 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-80052366173&doi=10.1542%2fpeds.2010-2307&partnerID=40&md5=05b94c8db5717755ce48f4b77c400938 VL - 128 ID - 2119 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. © 2011 Elsevier Ireland Ltd. AD - R. Inuzuka, Departments of Pediatric Cardiology, Chiba Children's Hospital, 579-1 Heta-cho, Midori-ku, Chiba-city, Chiba, Japan AU - Inuzuka, R. AU - Tatebe, S. AU - Wakiguchi, S. AU - Nakajima, H. AU - Ohtsu, H. AU - Dimopoulos, K. AU - Aotsuka, H. DB - Embase Medline DO - 10.1016/j.ijcard.2011.02.023 IS - 2 KW - brain natriuretic peptide article cardiovascular parameters cavopulmonary connection child child death circulation clinical article end diastolic wall stress female follow up Fontan procedure heart catheterization heart failure heart hemodynamics heart single ventricle heart transplantation heart valve regurgitation heart volume overload heart work high risk patient human infant male observational study patient identification preschool child priority journal proportional hazards model pulmonary to systemic flow ratio univentricular circulation ventricular end systolic wall stress ventricular mass to end diastolic volume ratio wall stress LA - English M3 - Article N1 - L51315556 2011-03-17 2012-09-04 PY - 2012 SN - 0167-5273 1874-1754 SP - 88-93 ST - B-type natriuretic peptide at the early stage of univentricular circulation reflects inadequate adaptation to volume overload T2 - International Journal of Cardiology TI - B-type natriuretic peptide at the early stage of univentricular circulation reflects inadequate adaptation to volume overload UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51315556 http://dx.doi.org/10.1016/j.ijcard.2011.02.023 VL - 159 ID - 1048 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. © 2011 Elsevier Ireland Ltd. AD - Departments of Pediatric Cardiology, Chiba Children's Hospital, 579-1 Heta-cho, Midori-ku, Chiba-city, Chiba, Japan Department of Clinical Trial Data Management, Graduate School of Medicine, University of Tokyo, Japan National Heart and Lung Institute, Imperial College School of Medicine, London, United Kingdom AU - Inuzuka, R. AU - Tatebe, S. AU - Wakiguchi, S. AU - Nakajima, H. AU - Ohtsu, H. AU - Dimopoulos, K. AU - Aotsuka, H. DB - Scopus DO - 10.1016/j.ijcard.2011.02.023 IS - 2 KW - Congenital Heart defect Heart failure Hemodynamics Natriuretic peptide Stress M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 2012 SP - 88-93 ST - B-type natriuretic peptide at the early stage of univentricular circulation reflects inadequate adaptation to volume overload T2 - International Journal of Cardiology TI - B-type natriuretic peptide at the early stage of univentricular circulation reflects inadequate adaptation to volume overload UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84865032073&doi=10.1016%2fj.ijcard.2011.02.023&partnerID=40&md5=0f04dc5c5f8eb8c8e25965884a2ab559 VL - 159 ID - 2087 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 - Department of Paediatric Cardiology, Freeman Hospital, Freeman Road, Newcastle upon Tyne NE7 7DN, UK; claire.irving@nuth.nhs.uk. AN - 104540153. Language: English. Entry Date: 20120601. Revision Date: 20150711. Publication Type: Journal Article AU - Irving, C. A. AU - Chaudhari, M. P. DB - ccm DP - EBSCOhost IS - 4 KW - Down Syndrome -- Complications Heart Defects, Congenital -- Epidemiology Adolescence Child Child, Preschool Down Syndrome -- Epidemiology Down Syndrome -- Surgery Eisenmenger Complex -- Epidemiology England Heart Defects, Congenital -- Surgery Heart Transplantation -- Utilization Human Incidence Infant Infant, Newborn Prognosis Survival Analysis Treatment Outcomes N1 - research. Journal Subset: Biomedical; Europe; Peer Reviewed; UK & Ireland. Special Interest: Pediatric Care. NLM UID: 0372434. PMID: NLM21835834. PY - 2012 SN - 0003-9888 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104540153&site=ehost-live&scope=site VL - 97 ID - 1566 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 - H. Oh, Department of Regenerative Medicine, Center for Innovative Clinical Medicine, Okayama University Hospital, 2-5-1 Shikata-cho, Kita-ku, Okayama, Japan 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. DB - Embase Medline DO - 10.1161/CIRCRESAHA.116.310253 IS - 7 KW - NCT01829750 amiodarone scatter factor somatomedin C article body growth cardiac stem cell cell therapy child clinical article clinical outcome controlled study female heart ejection fraction heart failure heart function heart muscle compliance heart muscle fibrosis heart performance heart proarrhythmia heart single ventricle heart ventricle function human human cell male open study phase 2 clinical trial priority journal quality of life quantitative analysis randomized controlled trial LA - English M3 - Article N1 - L614057292 2017-01-18 2017-04-11 PY - 2017 SN - 1524-4571 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 - Circulation Research TI - Intracoronary cardiac progenitor cells in single ventricle physiology: the perseus (cardiac progenitor cell infusion to treat univentricular heart disease) randomized phase 2 trial UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L614057292 http://dx.doi.org/10.1161/CIRCRESAHA.116.310253 VL - 120 ID - 764 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. © 2017 American Heart Association, Inc. AD - Departments of Cardiovascular Surgery, Okayama University, Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Japan Departments of Pediatrics, Okayama University, Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Japan Departments of Anesthesiology and Resuscitology, Okayama University, Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Japan Departments of Radiology, Okayama University, Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Japan Department of Regenerative Medicine, Center for Innovative Clinical Medicine, Okayama University Hospital, 2-5-1 Shikata-cho, Kita-ku, Okayama, 700-8558, Japan 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. DB - Scopus DO - 10.1161/CIRCRESAHA.116.310253 IS - 7 KW - cell therapy heart disease heart failure hypoplastic left heart syndrome stem cell M3 - Article N1 - Cited By :50 Export Date: 15 June 2020 PY - 2017 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 - Circulation Research TI - Intracoronary cardiac progenitor cells in single ventricle physiology: the perseus (cardiac progenitor cell infusion to treat univentricular heart disease) randomized phase 2 trial UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85009178481&doi=10.1161%2fCIRCRESAHA.116.310253&partnerID=40&md5=4df428561ec15810493f353cf4e8466b VL - 120 ID - 1866 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. © 2003 Elsevier Science Ireland Ltd. All rights reserved. AD - J.E. Kerschner, Division of Pediatric Otolaryngology, Dept. of Otolaryngology/Commun. Sci., Children's Hospital of Wisconsin, 9000 W. Wisconsin Avenue, Milwaukee, WI 53226, United States AU - Ishman, S. AU - Book, D. T. AU - Conley, S. F. AU - Kerschner, J. E. DB - Embase Medline DO - 10.1016/S0165-5876(03)00004-1 IS - 5 KW - article bronchitis bronchoscopy case report clinical trial congenital heart malformation controlled clinical trial controlled study croupous bronchitis disease association disease course endoscopy human male medical literature preschool child priority journal respiratory distress surgical technique LA - English M3 - Article N1 - L36407731 2003-04-23 PY - 2003 SN - 0165-5876 SP - 543-548 ST - Plastic bronchitis: An unusual bronchoscopic challenge associated with congenital heart disease repair T2 - International Journal of Pediatric Otorhinolaryngology TI - Plastic bronchitis: An unusual bronchoscopic challenge associated with congenital heart disease repair UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L36407731 http://dx.doi.org/10.1016/S0165-5876(03)00004-1 VL - 67 ID - 1304 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. © 2003 Elsevier Science Ireland Ltd. All rights reserved. AD - Dept. of Otolaryngology/Commun. Sci., Medical College of Wisconsin, 9200 West Wisconsin Avenue, Milwaukee, WI 53226, United States Division of Pediatric Otolaryngology, Dept. of Otolaryngology/Commun. Sci., Children's Hospital of Wisconsin, 9000 W. Wisconsin Avenue, Milwaukee, WI 53226, United States AU - Ishman, S. AU - Book, D. T. AU - Conley, S. F. AU - Kerschner, J. E. DB - Scopus DO - 10.1016/S0165-5876(03)00004-1 IS - 5 KW - Bronchial casts Fontan procedure Plastic bronchitis M3 - Article N1 - Cited By :22 Export Date: 15 June 2020 PY - 2003 SP - 543-548 ST - Plastic bronchitis: An unusual bronchoscopic challenge associated with congenital heart disease repair T2 - International Journal of Pediatric Otorhinolaryngology TI - Plastic bronchitis: An unusual bronchoscopic challenge associated with congenital heart disease repair UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0037402464&doi=10.1016%2fS0165-5876%2803%2900004-1&partnerID=40&md5=74797acf710cdd8ad5da2e3421bcb475 VL - 67 ID - 2265 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 - King Abdulaziz Medical City, King Saud University for Health Sciences, Department of Cardiac Sciences, National Guard Hospital Health Affairs, Riyadh, 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 - Scopus DO - 10.1016/j.jsha.2014.01.001 IS - 2 KW - Cardiac surgery Chylothorax Pediatric Post operative M3 - Article N1 - Cited By :9 Export Date: 15 June 2020 PY - 2014 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84896318755&doi=10.1016%2fj.jsha.2014.01.001&partnerID=40&md5=4b4fce094bf23a6a727d2c381c64a3c3 VL - 26 ID - 2018 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. Copyright © 2012 by The American Association for Thoracic Surgery. AD - K. Miyaji, 1-15-1 Kitasato, Sagamihara, Kanagawa 228-8555, Japan 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. DB - Embase Medline DO - 10.1016/j.jtcvs.2011.08.013 IS - 1 KW - aorta atresia aortic coarctation aortic flow aortic stenosis aortic arch surgery arterial stiffness article atresia blood flow velocity blood vessel graft body surface cardiac workload child clinical article echocardiography heart hemodynamics heart single ventricle heart work human Norwood procedure preschool child priority journal pulsatile flow shear stress LA - English M3 - Article N1 - L51612127 2011-09-12 2012-07-04 PY - 2012 SN - 0022-5223 1097-685X SP - 130-138 ST - Influence of surgical arch reconstruction methods on single ventricle workload in the Norwood procedure T2 - Journal of Thoracic and Cardiovascular Surgery TI - Influence of surgical arch reconstruction methods on single ventricle workload in the Norwood procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51612127 http://dx.doi.org/10.1016/j.jtcvs.2011.08.013 VL - 144 ID - 1052 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. Copyright © 2012 by The American Association for Thoracic Surgery. AD - Department of the Cardiovascular Surgery, Kitasato University School of Medicine, Kanagawa, Japan Center for Advanced Biomedical Science, TWIns, Waseda University, Tokyo, Japan Department of Cardiac Surgery, Graduate School of Medicine, University of Tokyo, Japan Australian School of Advanced Medicine, Macquarie University, Sydney, NSW, Australia 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. DB - Scopus DO - 10.1016/j.jtcvs.2011.08.013 IS - 1 M3 - Article N1 - Cited By :38 Export Date: 15 June 2020 PY - 2012 SP - 130-138 ST - Influence of surgical arch reconstruction methods on single ventricle workload in the Norwood procedure T2 - Journal of Thoracic and Cardiovascular Surgery TI - Influence of surgical arch reconstruction methods on single ventricle workload in the Norwood procedure UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84862622527&doi=10.1016%2fj.jtcvs.2011.08.013&partnerID=40&md5=0c078e4e808ec294a4facd2e34a34d7d VL - 144 ID - 2092 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. © 2018 Elsevier B.V. AD - Department of Pediatrics, Division of Pediatric Cardiology, Medical College of Wisconsin, 9000 W. Wisconsin Avenue, MS 713, Milwaukee, WI 53226, United States Department of Pediatrics, Division of Pediatric Cardiology, Children's Hospital of Wisconsin, 9000 W. Wisconsin Avenue, MS 713, Milwaukee, WI 53226, United States College of Health Sciences, Program in Exercise Science, Marquette University, PO Box 1881, Milwaukee, WI 53201-1881, United States Department of Internal Medicine, Division of Cardiovascular Medicine, Medical College of Wisconsin, 9000 W. Wisconsin Avenue, Milwaukee, WI 53226, United States Department of Pediatrics, Division of Pediatric Cardiology, University of Colorado School of Medicine, 13123 E 16th Street, B100, Aurora, CO 80045, United States Department of Internal Medicine, Division of Cardiology, University of Colorado School of Medicine, 12605 E 16th Avenue, Aurora, CO 80045, United States Department of Pediatrics, Division of Pediatric Cardiology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229, United States AU - Jacobsen, R. AU - Danduran, M. AU - Mussatto, K. AU - Hill, G. D. AU - Ginde, S. DB - Scopus DO - 10.1016/j.ppedcard.2018.06.003 KW - Congenital Heart Disease, CHD Exercise Fontan Physical activity Quality of life Rehabilitation M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2018 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85050866534&doi=10.1016%2fj.ppedcard.2018.06.003&partnerID=40&md5=2bdbfc6d96e17e157bb90d75746ddd9b VL - 50 ID - 1788 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 VO2max, 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 - R.M. Jacobsen, 9000 W. Wisconsin Avenue, MS 713, Milwaukee, WI, United States AU - Jacobsen, R. M. AU - Ginde, S. AU - Mussatto, K. AU - Neubauer, J. AU - Earing, M. AU - Danduran, M. DB - Embase Medline DO - 10.1111/chd.12330 IS - 2 KW - article child clinical article clinical assessment tool exercise intensity feasibility study female Fitbit Flex Activity Monitor follow up Fontan procedure functional status health program home care human male outcome assessment patient safety physical activity physical capacity physical performance pilot study preschool child priority journal program effectiveness quality of life questionnaire school child social psychology The Shuttle Test Run treatment duration venous oxygen tension LA - English M3 - Article N1 - L608635077 2016-03-02 2016-04-26 PY - 2016 SN - 1747-0803 1747-079X SP - 175-182 ST - Can a Home-based Cardiac Physical Activity Program Improve the Physical Function Quality of Life in Children with Fontan Circulation? T2 - Congenital Heart Disease TI - Can a Home-based Cardiac Physical Activity Program Improve the Physical Function Quality of Life in Children with Fontan Circulation? UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L608635077 http://dx.doi.org/10.1111/chd.12330 VL - 11 ID - 818 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 VO2max, 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. © 2016 Wiley Periodicals, Inc. AD - Division of Pediatric Cardiology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, United States Division of Cardiovascular Medicine, Department of Internal Medicine, Medical College of Wisconsin, Milwaukee, WI, United States Division of Pediatric Cardiology, Department of Pediatrics, Children's Hospital of Wisconsin, Milwaukee, WI, United States College of Health Sciences, Marquette University, Milwaukee, WI, United States AU - Jacobsen, R. M. AU - Ginde, S. AU - Mussatto, K. AU - Neubauer, J. AU - Earing, M. AU - Danduran, M. DB - Scopus DO - 10.1111/chd.12330 IS - 2 KW - Congenital Heart Disease Exercise Fontan Physical Activity Quality of Life Rehabilitation M3 - Article N1 - Cited By :17 Export Date: 15 June 2020 PY - 2016 SP - 175-182 ST - Can a Home-based Cardiac Physical Activity Program Improve the Physical Function Quality of Life in Children with Fontan Circulation? T2 - Congenital Heart Disease TI - Can a Home-based Cardiac Physical Activity Program Improve the Physical Function Quality of Life in Children with Fontan Circulation? UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84959105851&doi=10.1111%2fchd.12330&partnerID=40&md5=b3952a4e17a7cba5bf97b226d60bbc7d VL - 11 ID - 1920 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. © 2010 European Association for Cardio-Thoracic Surgery. AD - K. Januszewska, Department of Cardiac Surgery, Klinikum Grosshadern, Ludwig Maximilians University, Marchioninistr. 15, 81377 Munich, Germany AU - Januszewska, K. AU - Kozlik-Feldmann, R. AU - Kordon, Z. AU - Urschel, S. AU - Netz, H. AU - Reichart, B. AU - Malec, E. DB - Embase Medline DO - 10.1016/j.ejcts.2010.12.023 IS - 2 KW - aortic occlusion aortoplasty article child disease severity female follow up heart ventricle function hemodynamics human hypoplastic left heart syndrome infant major clinical study male medical record review mitral valve regurgitation Norwood procedure preschool child priority journal right ventricle to pulmonary artery conduit school child survival treatment outcome LA - English M3 - Article N1 - L51261947 2011-07-20 2011-07-25 PY - 2011 SN - 1010-7940 1873-734X SP - 508-513 ST - Significance of the residual aortic obstruction in multistage repair of hypoplastic left heart syndrome T2 - European Journal of Cardio-thoracic Surgery TI - Significance of the residual aortic obstruction in multistage repair of hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51261947 http://dx.doi.org/10.1016/j.ejcts.2010.12.023 VL - 40 ID - 1097 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. © 2010 European Association for Cardio-Thoracic Surgery. AD - Department of Cardiac Surgery, Klinikum Grosshadern, Ludwig Maximilians University, Munich, Germany Department of Pediatric Cardiology, Klinikum Grosshadern, Ludwig Maximilians University, Munich, Germany Department of Pediatric Cardiology, Polish-American Children's Hospital, Jagiellonian University, Cracow, Poland AU - Januszewska, K. AU - Kozlik-Feldmann, R. AU - Kordon, Z. AU - Urschel, S. AU - Netz, H. AU - Reichart, B. AU - Malec, E. DB - Scopus DO - 10.1016/j.ejcts.2010.12.023 IS - 2 KW - Aortic obstruction Hypoplastic left heart syndrome Norwood procedure Right ventricle-to-pulmonary artery shunt M3 - Article N1 - Cited By :10 Export Date: 15 June 2020 PY - 2011 SP - 508-513 ST - Significance of the residual aortic obstruction in multistage repair of hypoplastic left heart syndrome T2 - European Journal of Cardio-thoracic Surgery TI - Significance of the residual aortic obstruction in multistage repair of hypoplastic left heart syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-79960270865&doi=10.1016%2fj.ejcts.2010.12.023&partnerID=40&md5=e1b24de4cab13ca1be271a317893491c VL - 40 ID - 2122 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, Mayo Clinic, Rochester, MN Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, MN Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN Department of Cardiovascular Surgery, Mayo Clinic, Rochester, MN AN - 137279325. Language: English. Entry Date: 20200104. Revision Date: 20200104. Publication Type: journal article AU - Javed, Rabia AU - Cetta, Frank AU - Said, Sameh M. AU - Olson, Timothy M. AU - O'Leary, Patrick W. AU - Qureshi, Muhammad Yasir DB - ccm DO - 10.1542/pir.2018-0005 DP - EBSCOhost IS - 7 KW - Hypoplastic Left Heart Syndrome -- Surgery Heart Surgery -- Methods Hypoplastic Left Heart Syndrome -- Complications Hypoplastic Left Heart Syndrome -- Physiopathology Hypoplastic Left Heart Syndrome -- Diagnosis Dehydration -- Etiology Developmental Disabilities -- Etiology Respiratory Tract Infections -- Etiology Primary Health Care Palliative Care -- Methods Infant Cardiopulmonary Bypass N1 - review. Journal Subset: Biomedical; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. NLM UID: 8103046. PMID: NLM31263042. PY - 2019 SN - 0191-9601 SP - 344-351 ST - Hypoplastic Left Heart Syndrome: An Overview for Primary Care Providers T2 - Pediatrics in Review TI - Hypoplastic Left Heart Syndrome: An Overview for Primary Care Providers UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=137279325&site=ehost-live&scope=site VL - 40 ID - 1476 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 ≥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 × 103 versus 195 × 103, 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. © 2008 Mosby, Inc. All rights reserved. AD - P.C. Jenkins, Department of Pediatrics, Dartmouth Medical School, Hanover, NH, United States 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. DB - Embase Medline DO - 10.1016/j.jpeds.2007.09.050 IS - 4 KW - adolescent article bicycle ergometry cardiovascular function child clinical article comparative study controlled study electrocardiography exercise test female heart disease heart rate heart transplantation human hypoplastic left heart syndrome male oxygen consumption physical activity priority journal quality of life statistical significance treadmill exercise vascular capacity LA - English M3 - Article N1 - L351381037 2008-04-02 PY - 2008 SN - 0022-3476 SP - 507-512 ST - Decreased Exercise Performance with Age in Children with Hypoplastic Left Heart Syndrome T2 - Journal of Pediatrics TI - Decreased Exercise Performance with Age in Children with Hypoplastic Left Heart Syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L351381037 http://dx.doi.org/10.1016/j.jpeds.2007.09.050 VL - 152 ID - 1215 ER - TY - JOUR AD - Department of Pediatrics, Dartmouth Medical School, Hanover, NH 03756, USA. pcj@hitchcock.org AN - 105905790. Language: English. Entry Date: 20080502. Revision Date: 20150711. Publication Type: Journal Article 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. DB - ccm DP - EBSCOhost IS - 4 KW - Cardiopulmonary Bypass Exercise Tolerance Heart Transplantation Hypoplastic Left Heart Syndrome -- Physiopathology Adolescence Analysis of Variance Blood Pressure Case Control Studies Child Electrocardiography Exercise Test Female Heart Rate Hypoplastic Left Heart Syndrome -- Metabolism Hypoplastic Left Heart Syndrome -- Surgery Male Oxygen Consumption Quality of Life Reference Values Human N1 - research. Commentary: Goldberg CS, Goldstein B. [Commentary on] Decreased exercise performance with age in children with hypoplastic left heart syndrome. (ACC CARDIOSOURCE REV J) 2008 May; 17 (5): 17-18. Journal Subset: Biomedical; Peer Reviewed; USA. NLM UID: 0375410. PMID: NLM18346505. PY - 2008 SN - 0022-3476 SP - 507-512 ST - Decreased exercise performance with age in children with hypoplastic left heart syndrome T2 - Journal of Pediatrics TI - Decreased exercise performance with age in 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=105905790&site=ehost-live&scope=site VL - 152 ID - 1614 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 ≥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 × 103 versus 195 × 103, 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. © 2008 Mosby, Inc. All rights reserved. AD - Department of Pediatrics, Dartmouth Medical School, Hanover, NH, United States Department of Pediatrics, Loma Linda University Children's Hospital, Loma Linda, CA, United States Department of Cardiology, Children's Hospital, Boston, MA, United States Department of Cardiology, Children's Healthcare of Atlanta, GA, United States Division of Pediatric Cardiology, Washington University, St. Louis Children's Hospital, St. Louis, MO, United States 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. DB - Scopus DO - 10.1016/j.jpeds.2007.09.050 IS - 4 M3 - Article N1 - Cited By :27 Export Date: 15 June 2020 PY - 2008 SP - 507-512 ST - Decreased Exercise Performance with Age in Children with Hypoplastic Left Heart Syndrome T2 - Journal of Pediatrics TI - Decreased Exercise Performance with Age in Children with Hypoplastic Left Heart Syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-40749146708&doi=10.1016%2fj.jpeds.2007.09.050&partnerID=40&md5=352a024800e943249682954dc8de0f21 VL - 152 ID - 2197 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. © 2013 by the American College of Cardiology Foundation. AD - A.T. Yetman, Adult Congenital Cardiology Program, Primary Children's Medical Center, 100 North Mario Capecchi Drive, Salt Lake City, UT 84113, United States 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. DB - Embase Medline DO - 10.1016/j.jacc.2013.01.008 IS - 12 KW - adolescent adult altitude article child cohort analysis female Fontan procedure heart failure heart single ventricle heart transplantation human liver dysfunction lung artery pressure lung vascular resistance major clinical study male mortality outcome assessment priority journal residential altitude retrospective study risk factor survival thrombosis treatment failure LA - English M3 - Article N1 - L52442201 2013-02-20 2014-02-19 PY - 2013 SN - 0735-1097 1558-3597 SP - 1283-1289 ST - Living at altitude adversely affects survival among patients with a fontan procedure T2 - Journal of the American College of Cardiology TI - Living at altitude adversely affects survival among patients with a fontan procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52442201 http://dx.doi.org/10.1016/j.jacc.2013.01.008 VL - 61 ID - 1009 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. © 2013 by the American College of Cardiology Foundation. AD - Adult Congenital Cardiology Program, Primary Children's Medical Center, 100 North Mario Capecchi Drive, Salt Lake City, UT 84113, United States 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. DB - Scopus DO - 10.1016/j.jacc.2013.01.008 IS - 12 KW - Altitude Fontan Protein-losing enteropathy Survival M3 - Article N1 - Cited By :14 Export Date: 15 June 2020 PY - 2013 SP - 1283-1289 ST - Living at altitude adversely affects survival among patients with a fontan procedure T2 - Journal of the American College of Cardiology TI - Living at altitude adversely affects survival among patients with a fontan procedure UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84877272802&doi=10.1016%2fj.jacc.2013.01.008&partnerID=40&md5=89c80e998d4a1da1434f96a3bc2ea6f8 VL - 61 ID - 2056 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. © 2004 Elsevier B.V. All rights reserved. AD - Dept. Neonatology Pediat. I., Univ. Children's Hospital Zurich, Steinwiesstrasse 75, CH-8032 Z. AU - Joho-Arreola, A. L. AU - Bauersfeld, U. AU - Stauffer, U. G. AU - Baenziger, O. AU - Bernet, V. DB - Embase Medline DO - 10.1016/j.ejcts.2004.10.002 IS - 1 KW - algorithm analytic method anastomosis article assisted ventilation cause of death diaphragm paralysis follow up Fontan procedure heart surgery human incidence priority journal respiratory distress shunting surgical technique treatment failure treatment indication LA - English M3 - Article N1 - L40038528 2005-01-12 PY - 2005 SN - 1010-7940 SP - 53-57 ST - Incidence and treatment of diaphragmatic paralysis after cardiac surgery in children T2 - European Journal of Cardio-thoracic Surgery TI - Incidence and treatment of diaphragmatic paralysis after cardiac surgery in children UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L40038528 http://dx.doi.org/10.1016/j.ejcts.2004.10.002 VL - 27 ID - 1285 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. © 2004 Elsevier B.V. All rights reserved. AD - Dept. Neonatology Pediat. I., Univ. Children's Hospital Zurich, Steinwiesstrasse 75, CH-8032 Z., Switzerland Division of Pediatric Cardiology, Univ. Children's Hospital Zurich, Steinwiesstrasse 75, CH-8032 Z., Switzerland Department of Pediatric Surgery, Univ. Children's Hospital Zurich, Steinwiesstrasse 75, CH-8032 Z., Switzerland AU - Joho-Arreola, A. L. AU - Bauersfeld, U. AU - Stauffer, U. G. AU - Baenziger, O. AU - Bernet, V. DB - Scopus DO - 10.1016/j.ejcts.2004.10.002 IS - 1 KW - Diaphragmatic plication Infant Newborn Phrenic nerve injury M3 - Article N1 - Cited By :76 Export Date: 15 June 2020 PY - 2005 SP - 53-57 ST - Incidence and treatment of diaphragmatic paralysis after cardiac surgery in children T2 - European Journal of Cardio-thoracic Surgery TI - Incidence and treatment of diaphragmatic paralysis after cardiac surgery in children UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-11144337403&doi=10.1016%2fj.ejcts.2004.10.002&partnerID=40&md5=077adc34a6559df280136f3acabeeead VL - 27 ID - 2246 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 - Clinical Sciences Theme, Murdoch Childrens Research Institute, Melbourne, VIC, Australia Department of Paediatrics, University of Melbourne, Melbourne, VIC, Australia Social Work Department, Royal Children's Hospital, Melbourne, VIC, Australia School of Behavioural Science, University of Melbourne, Melbourne, VIC, Australia Monash Heart, Monash Medical Centre, Southern Health, Clayton, VIC, Australia School of Psychology and Psychiatry, Monash University, Clayton, VIC, Australia AU - Jordan, B. AU - Franich-Ray, C. AU - Albert, N. AU - Anderson, V. AU - Northam, E. AU - Cochrane, A. AU - Menahem, S. DB - Scopus DO - 10.1136/archdischild-2012-303488 IS - 7 M3 - Article N1 - Cited By :18 Export Date: 15 June 2020 PY - 2014 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84902358705&doi=10.1136%2farchdischild-2012-303488&partnerID=40&md5=bacab36aafd3ef5092f9b0382a1e0165 VL - 99 ID - 2013 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 2-38mmHg for flow rates of 1-4L/min at 4000-7000RPM, 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 7×10-5g/100L. 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. © 2015 International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc. AD - 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 - 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. DB - Scopus DO - 10.1111/aor.12443 IS - 1 KW - Blood pump Computational fluid dynamics Fontan physiology Intravascular blood pump Mechanical cavopulmonary assist Pediatric circulatory support Single ventricle physiology Total cavopulmonary connection M3 - Article N1 - Cited By :10 Export Date: 15 June 2020 PY - 2015 SP - 34-42 ST - Design of axial blood pumps for patients with dysfunctional fontan physiology: Computational studies and performance testing T2 - Artificial Organs TI - Design of axial blood pumps for patients with dysfunctional fontan physiology: Computational studies and performance testing UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84922566743&doi=10.1111%2faor.12443&partnerID=40&md5=ed77f55a130c5e1a7658d5eddb3971ad VL - 39 ID - 1961 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_volavg cycle, KEavg cycle, ELavg cycle). The relation to maximum oxygen uptake (VO2 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 (KEavg cycle: 1.8 ± 0.5 vs 3.3 ± 0.9 mJ, P < 0.001), a significant 108 ± 49% increase in EL (ELavg cycle: 0.9 ± 0.4 vs 1.9 ± 0.9 mW, P < 0.001) and a significant 27 ± 19% increase in vorticity (vorticity_volavg cycle: 3441 ± 899 vs 4394 ± 1322 mL/s, P = 0.002). All rest-stress differences (%) were negatively correlated to VO2 max (KEavg cycle: r = - 0.83, P = 0.003; ELavg cycle: r = - 0.80, P = 0.006; vorticity_volavg 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 VO2 max. 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. DB - Medline DO - 10.1186/s12968-019-0553-4 IS - 1 KW - beta 1 adrenergic receptor stimulating agent dobutamine adolescent cine magnetic resonance imaging comparative study computer assisted diagnosis congenital heart malformation coronary artery blood flow diagnostic imaging drug effect exercise test exercise tolerance female Fontan procedure hemodynamics human male myocardial perfusion imaging oxygen consumption pathophysiology predictive value procedures prospective study time factor treatment outcome young adult LA - English M3 - Article N1 - L628897236 2019-08-19 2020-02-04 PY - 2019 SN - 1532-429X SP - 43 ST - Stress increases intracardiac 4D flow cardiovascular magnetic resonance -derived energetics and vorticity and relates to VO2max in Fontan patients T2 - Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance TI - Stress increases intracardiac 4D flow cardiovascular magnetic resonance -derived energetics and vorticity and relates to VO2max in Fontan patients UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L628897236 http://dx.doi.org/10.1186/s12968-019-0553-4 VL - 21 ID - 607 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_volavg cycle, KEavg cycle, ELavg cycle). The relation to maximum oxygen uptake (VO2 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 (KEavg cycle: 1.8 ± 0.5 vs 3.3 ± 0.9 mJ, P < 0.001), a significant 108 ± 49% increase in EL (ELavg cycle: 0.9 ± 0.4 vs 1.9 ± 0.9 mW, P < 0.001) and a significant 27 ± 19% increase in vorticity (vorticity_volavg cycle: 3441 ± 899 vs 4394 ± 1322 mL/s, P = 0.002). All rest-stress differences (%) were negatively correlated to VO2 max (KEavg cycle: r = - 0.83, P = 0.003; ELavg cycle: r = - 0.80, P = 0.006; vorticity_volavg 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 VO2 max. AD - Department of Pediatrics division of Pediatric Cardiology, Leiden University Medical Center, Leiden, Netherlands Netherlands Heart InstituteUtrecht, Netherlands Northwestern University, Department of Radiology Feinberg School of Medicine, Chicago, United States Department of Radiology, Leiden University Medical Center, Leiden, Netherlands Department of Cardiothoracic Surgery, Leiden University Medical Center, Leiden, Netherlands Department of Cardiology, Leiden University Medical Center, Leiden, Netherlands Department of Pediatrics division of Pediatric Cardiology, Academic Medical Center, Amsterdam, Netherlands Department of Pediatrics, division of Pediatric Cardiology, Erasmus Medical Center, Rotterdam, Netherlands Department of Pediatrics division of Pediatric Cardiology, Radboud university Medical Center, Nijmegen, Netherlands 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. DB - Scopus DO - 10.1186/s12968-019-0553-4 IS - 1 KW - 4D flow CMR Flow Fontan Kinetic energy: energy loss Vorticity M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2019 SP - 43 ST - Stress increases intracardiac 4D flow cardiovascular magnetic resonance -derived energetics and vorticity and relates to VO2max in Fontan patients T2 - Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance TI - Stress increases intracardiac 4D flow cardiovascular magnetic resonance -derived energetics and vorticity and relates to VO2max in Fontan patients UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85070520735&doi=10.1186%2fs12968-019-0553-4&partnerID=40&md5=1556cbf04a6e6cf098290c5db59ba545 VL - 21 ID - 1740 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. © 2020 Elsevier B.V. AD - Children's Hospital Of Illinois, Peoria, IL, United States Functional Fluidics, Wayne State University, Detroit, MI, United States Wayne State University, Detroit, MI, United States Children's Hospital of Michigan, Detroit, MI, 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., III AU - Hines, P. C. C7 - 101191 DB - Scopus DO - 10.1016/j.ppedcard.2019.101191 M3 - Article N1 - Export Date: 15 June 2020 PY - 2020 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85079879516&doi=10.1016%2fj.ppedcard.2019.101191&partnerID=40&md5=572afd6d576daeecf5d5e8cba58501a8 VL - 56 ID - 1710 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 - A. Karowicz-Bilińska, High Risk Pregnancy Clinic Medical University Lodz, 1-st Department of Obstetrics and Gynecology Wileńska 37. 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 - Medline IS - 6 KW - adult article differential diagnosis female high risk pregnancy human hypoplastic left heart syndrome methodology Poland pregnancy pregnancy outcome prenatal care prenatal diagnosis prognosis retrospective study second trimester pregnancy LA - English M3 - Article N1 - L352116516 2008-08-13 PY - 2008 SN - 0017-0011 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 - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L352116516 VL - 79 ID - 1208 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 - M. Kasem, Department of Congenital Cardiology, Leeds General Infirmary, Great George Street, Leeds, United Kingdom AU - Kasem, M. AU - Bentham, J. AU - Thomson, J. DB - Embase Medline DO - 10.1017/S1047951118001464 IS - 12 KW - arterial stent biliary stent coronary stent everolimus eluting coronary stent Genesis politef hemoglobin adolescent adult aortopulmonary shunt arterial embolization artery occlusion article Blalock Taussig shunt clinical article clinical outcome congenital heart disease cyanosis disease severity exercise tolerance female heart single ventricle heart valve regurgitation hemodynamics hemoglobin blood level human lung blood flow male middle aged New York Heart Association class oxygen saturation retrospective study surgical approach transthoracic echocardiography Gore-Tex Multi-Link Ultra Visi-pro Xience LA - English M3 - Article N1 - L624435857 2018-10-24 2019-03-22 PY - 2018 SN - 1467-1107 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 - Cardiology in the 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L624435857 http://dx.doi.org/10.1017/S1047951118001464 VL - 28 ID - 650 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. © 2018 Cambridge University Press. AD - Department of Congenital Cardiology, Leeds General Infirmary, Great George Street, Leeds, LS13EX, United Kingdom AU - Kasem, M. AU - Bentham, J. AU - Thomson, J. DB - Scopus DO - 10.1017/S1047951118001464 IS - 12 KW - Arterial shunt Blalock-Taussig shunt central shunt stenting M3 - Article N1 - Export Date: 15 June 2020 PY - 2018 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 - Cardiology in the 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85055048024&doi=10.1017%2fS1047951118001464&partnerID=40&md5=16e39537e7afb0a575e54453bbc271d2 VL - 28 ID - 1782 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 (Mage = 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 - A. Kaugars, Department of Psychology, Marquette University, Milwaukee, WI, United States AU - Kaugars, A. AU - Shields, C. AU - Brosig, C. DB - Embase Medline DO - 10.1111/chd.12547 IS - 1 KW - adolescent article attention deficit disorder behavior disorder child congenital heart disease controlled study female gestational age heart single ventricle human infant major clinical study male mental health service parental stress patient referral pediatrics priority journal quality of life retrospective study social psychology LA - English M3 - Article N1 - L618954344 2017-10-31 2018-02-09 PY - 2018 SN - 1747-0803 1747-079X SP - 72-78 ST - Stress and quality of life among parents of children with congenital heart disease referred for psychological services T2 - Congenital Heart Disease TI - Stress and quality of life among parents of children with congenital heart disease referred for psychological services UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L618954344 http://dx.doi.org/10.1111/chd.12547 VL - 13 ID - 714 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 (Mage = 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. © 2017 Wiley Periodicals, Inc. AD - Department of Psychology, Marquette University, Milwaukee, WI, United States Department of Psychological Sciences, Kent State University, Kent, OH, United States Department of Pediatrics, Medical College of Wisconsin, Herma Heart Center, Children's Hospital of Wisconsin, Milwaukee, WI, United States AU - Kaugars, A. AU - Shields, C. AU - Brosig, C. DB - Scopus DO - 10.1111/chd.12547 IS - 1 KW - children congenital heart disease health-related quality of life parent stress parents M3 - Article N1 - Cited By :10 Export Date: 15 June 2020 PY - 2018 SP - 72-78 ST - Stress and quality of life among parents of children with congenital heart disease referred for psychological services T2 - Congenital Heart Disease TI - Stress and quality of life among parents of children with congenital heart disease referred for psychological services UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85032223364&doi=10.1111%2fchd.12547&partnerID=40&md5=f4a3bc41c7eddbf563080e393451d467 VL - 13 ID - 1827 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 - 137845157. Language: English. Entry Date: 19970801. Revision Date: 20190803. Publication Type: journal article AU - Kelley, J. R. AU - Mack, G. W. AU - Fahey, J. T. DB - ccm DP - EBSCOhost IS - 3 KW - Vascular Resistance Cardiopulmonary Bypass -- Statistics and Numerical Data Heart -- Physiopathology Heart Ventricle -- Physiopathology Relaxation -- Physiology Hemodynamics Adolescence Adult Indicator Dilution Techniques Analysis of Variance Exercise Test -- Statistics and Numerical Data Human Time Factors Decompression Sickness -- Prevention and Control Postoperative Period Exercise Test -- Methods Validation Studies Comparative Studies Evaluation Research Multicenter Studies Exercise of Self-Care Agency Scale N1 - research. Journal Subset: Biomedical; Peer Reviewed; USA. Instrumentation: Exercise of Self-Care Agency Scale (ESCA) (Kearney and Fleischer); Defining Issues Test (DIT) (Rest). Grant Information: HL07272/HL/NHLBI NIH HHS/United States. NLM UID: 0207277. PMID: NLM7611151. PY - 1995 SN - 0002-9149 SP - 158-163 ST - Diminished venous vascular capacitance in patients with univentricular hearts after the Fontan operation T2 - American Journal of Cardiology TI - Diminished venous vascular capacitance in patients with univentricular hearts after the Fontan operation UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=137845157&site=ehost-live&scope=site VL - 76 ID - 1627 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 orthostaric stress indicated higher venous tone in Fontan than control subjects. We propose that increased resting venous tone in Fontan subjects may limit their ability to mobilize blood from capacitance vessels during exercise and may contribute to impaired cardiac output response. © 1995 Excerpta Medica, Inc. All rights reserved under the United States, International, and Pan-American Copyright Conventions. AD - Department of Pediatrics, Yale University School of Medicine, Yale-New Haven Hospital, United States John B. Pierce Laboratory, New Haven, CT, United States AU - Kelley, J. R. AU - Mack, G. W. AU - Fahey, J. T. DB - Scopus DO - 10.1016/S0002-9149(99)80049-6 IS - 3 M3 - Article N1 - Cited By :55 Export Date: 15 June 2020 PY - 1995 SP - 158-163 ST - Diminished venous vascular capacitance in patients with univentricular hearts after the Fontan operation T2 - The American Journal of Cardiology TI - Diminished venous vascular capacitance in patients with univentricular hearts after the Fontan operation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0029078050&doi=10.1016%2fS0002-9149%2899%2980049-6&partnerID=40&md5=59340c37686eee37dba795460f583d2d VL - 76 ID - 2300 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 - J.H. Kern, Division of Pediatric Cardiology, New York Hospital, Cornell Medical Center, 525 E 68th St, New York, NY 10021, United States AU - Kern, J. H. AU - Hinton, V. J. AU - Nereo, N. E. AU - Hayes, C. J. AU - Gersony, W. M. DB - Embase Medline DO - 10.1542/peds.102.5.1148 IS - 5 KW - adaptation article clinical article controlled study early diagnosis female heart arrest human human cell human tissue hypoplastic left heart syndrome intelligence quotient intelligence test male mental development mental retardation malformation syndrome outcomes research preschool child priority journal risk factor LA - English M3 - Article N1 - L28511723 1998-11-23 PY - 1998 SN - 0031-4005 SP - 1148-1152 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L28511723 http://dx.doi.org/10.1542/peds.102.5.1148 VL - 102 ID - 1346 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 - J. Keski-Nisula, New Children's Hospital, Stenbäckinkatu 9, 00029 HUS, Helsinki, Finland AU - Keski-Nisula, J. AU - Arvola, O. AU - Jahnukainen, T. AU - Andersson, S. AU - Pesonen, E. DB - Embase Medline DO - 10.1053/j.jvca.2019.10.015 IS - 6 KW - EudraCT2008-007413-76 8 hydroxydeoxyguanosine biological marker free radical interleukin 10 interleukin 6 interleukin 8 methylprednisolone placebo sodium chloride antiinflammatory activity aortic arch surgery artery reconstruction article blood level clinical article clinical outcome controlled study cytokine production double blind procedure drug effect drug mechanism drug megadose female Glenn shunt heart single ventricle human infant inflammation male oxidative stress palliative therapy pediatric intensive care unit postoperative period preoperative period priority journal pulmonary artery repair randomized controlled trial university hospital LA - English M3 - Article N1 - L2004868838 2020-02-13 PY - 2020 SN - 1532-8422 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 - Journal of Cardiothoracic and Vascular Anesthesia 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2004868838 http://dx.doi.org/10.1053/j.jvca.2019.10.015 VL - 34 ID - 548 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. © 2019 Elsevier Inc. AD - Division of Anaesthesiology, Department of Anaesthesiology, Intensive Care and Pain Medicine, Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland Department of Anesthesiology, Perioperative and Pain Medicine, Stanford University School of Medicine, Stanford, CA, United States 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 AU - Keski-Nisula, J. AU - Arvola, O. AU - Jahnukainen, T. AU - Andersson, S. AU - Pesonen, E. DB - Scopus DO - 10.1053/j.jvca.2019.10.015 IS - 6 KW - bidirectional Glenn procedure congenital heart defect infant inflammation methylprednisolone radical stress M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2020 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 - Journal of Cardiothoracic and Vascular Anesthesia 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85079195196&doi=10.1053%2fj.jvca.2019.10.015&partnerID=40&md5=bb55feed2a087def06baa155bc68869e VL - 34 ID - 1699 ER - TY - JOUR AB - As early as 1699, Chemineau described a heart composed of 2 auricles but only 1 ventricle.(1) 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 - Canada Research Chair, Electrophysiology and Adult Congenital Heart Disease, Adult Congenital Heart Center, Montreal Heart Institute, 5000 Bélanger St, Montreal, Quebec, H1T 1C8, Canada. paul.khairy@umontreal.ca. AN - 106162363. Language: English. Entry Date: 20071005. Revision Date: 20150711. Publication Type: Journal Article. Journal Subset: Biomedical AU - Khairy, P. AU - Poirier, N. AU - Mercier, L. A. DB - ccm DP - EBSCOhost IS - 6 KW - Heart Defects, Congenital -- Classification Heart Defects, Congenital -- Diagnosis Heart Defects, Congenital -- Pathology Heart Defects, Congenital -- Surgery Adolescence Child Child, Preschool Cyanosis -- Therapy Echocardiography Electrocardiography Exercise Cardiopulmonary Bypass Heart Catheterization Infant Infant, Newborn Quality of Life Nomenclature N1 - Peer Reviewed; USA. NLM UID: 0147763. PMID: NLM17296869. PY - 2007 SN - 0009-7322 SP - 800-812 ST - Univentricular heart T2 - Circulation TI - Univentricular heart UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106162363&site=ehost-live&scope=site VL - 115 ID - 1609 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 - O.M. Khalid, The Heart Center, Nationwide Children’s Hospital, 700 Children’s Dr, Columbus, OH, United States AU - Khalid, O. M. AU - Harrison, T. M. DB - Embase Medline DO - 10.1007/s00246-019-02191-3 IS - 8 KW - lactic acid article artificial ventilation body weight clinical article cognitive development developmental delay heart single ventricle heart surgery human hybrid procedure hypoplastic left heart syndrome infant language development length of stay longitudinal study motor development neonatal intensive care unit Norwood procedure retrospective study risk factor surgical technique LA - English M3 - Article N1 - L2002770202 2019-09-20 PY - 2019 SN - 1432-1971 0172-0643 SP - 1591-1598 ST - Early Neurodevelopmental Outcomes in Children with Hypoplastic Left Heart Syndrome and Related Anomalies After Hybrid Procedure T2 - Pediatric Cardiology TI - Early Neurodevelopmental Outcomes in Children with Hypoplastic Left Heart Syndrome and Related Anomalies After Hybrid Procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2002770202 http://dx.doi.org/10.1007/s00246-019-02191-3 VL - 40 ID - 582 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. © 2019, Springer Science+Business Media, LLC, part of Springer Nature. AD - The Heart Center, Nationwide Children’s Hospital, 700 Children’s Dr, Columbus, OH 43205, United States The Ohio State University College of Nursing, 1585 Neil Ave., Columbus, OH 43210, United States AU - Khalid, O. M. AU - Harrison, T. M. DB - Scopus DO - 10.1007/s00246-019-02191-3 IS - 8 KW - Congenital heart disease Hybrid procedure Hypoplastic left heart syndrome Neurodevelopmental outcome M3 - Article N1 - Export Date: 15 June 2020 PY - 2019 SP - 1591-1598 ST - Early Neurodevelopmental Outcomes in Children with Hypoplastic Left Heart Syndrome and Related Anomalies After Hybrid Procedure T2 - Pediatric Cardiology TI - Early Neurodevelopmental Outcomes in Children with Hypoplastic Left Heart Syndrome and Related Anomalies After Hybrid Procedure UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85072037165&doi=10.1007%2fs00246-019-02191-3&partnerID=40&md5=42a265a044b837cb4eccae83c3df9015 VL - 40 ID - 1720 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 - S. Khambadkone, Cardiology Department, Great Ormond Street Hospital, Great Ormond St., London WC1N 3JH, United Kingdom AU - Khambadkone, S. AU - Li, J. AU - De Leval, M. R. AU - Cullen, S. AU - Deanfield, J. E. AU - Redington, A. N. DB - Embase Medline DO - 10.1161/01.CIR.0000074210.49434.40 IS - 25 KW - nitric oxide oxygen adolescent article calculation clinical article clinical protocol endothelium female Fontan procedure human lung blood flow lung circulation lung vascular resistance male mass spectrometry oxygen consumption priority journal school child LA - English M3 - Article N1 - L36793092 2003-07-21 PY - 2003 SN - 0009-7322 SP - 3204-3208 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L36793092 http://dx.doi.org/10.1161/01.CIR.0000074210.49434.40 VL - 107 ID - 1300 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 - Cardiology Department, Great Ormond Street Hospital, Great Ormond St, London WC1N 3JH, UK; s.khambadkone@ich.ucl.ac.uk AN - 106785623. Language: English. Entry Date: 20031205. Revision Date: 20150711. Publication Type: Journal Article AU - Khambadkone, S. AU - Li, J. AU - de Leval, M. R. AU - Cullen, S. AU - Deanfield, J. E. AU - Redington, A. N. DB - ccm DP - EBSCOhost IS - 25 KW - Endothelium -- Physiology Nitric Oxide -- Pharmacodynamics Pulmonary Artery -- Analysis Surgery, Cardiovascular -- Adverse Effects Vascular Resistance Adolescence Angiography Child Descriptive Statistics Female Hemodynamics -- Evaluation Male Oxygen Consumption Paired T-Tests Prospective Studies Time Factors Two-Tailed Test United Kingdom Unpaired T-Tests Funding Source Human N1 - research; tables/charts. Journal Subset: Biomedical; Peer Reviewed; USA. Grant Information: Supported by a British Heart Foundation Research Grant and by the NHS Executive. NLM UID: 0147763. PMID: NLM12821557. PY - 2003 SN - 0009-7322 SP - 3204-3208 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106785623&site=ehost-live&scope=site VL - 107 ID - 1618 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, Gt. Ormond St. Hosp. Children NHS T., Grown Up Congenital Heart Unit, London, United Kingdom Cardiology Department, Great Ormond Street Hospital, Great Ormond St., London WC1N 3JH, United Kingdom AU - Khambadkone, S. AU - Li, J. AU - De Leval, M. R. AU - Cullen, S. AU - Deanfield, J. E. AU - Redington, A. N. DB - Scopus DO - 10.1161/01.CIR.0000074210.49434.40 IS - 25 KW - Endothelial dysfunction Fontan procedure Pulmonary vascular resistance M3 - Article N1 - Cited By :147 Export Date: 15 June 2020 PY - 2003 SP - 3204-3208 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0038078913&doi=10.1161%2f01.CIR.0000074210.49434.40&partnerID=40&md5=d4146f57c08937ff401996e9e9b13116 VL - 107 ID - 2259 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 - 103874614. Language: English. Entry Date: 20150327. Revision Date: 20150710. Publication Type: Journal Article AU - Khiabani, Reza H. AU - Whitehead, Kevin K. AU - Han, David AU - Restrepo, Maria AU - Tang, Elaine AU - Bethel, James AU - Paridon, Stephen M. AU - Fogel, Mark A. AU - Yoganathan, Ajit P. DB - ccm DO - 10.1136/heartjnl-2014-306337 DP - EBSCOhost IS - 2 KW - Exercise Tolerance Cardiopulmonary Bypass -- Methods Cardiopulmonary Bypass -- Psychosocial Factors Cardiopulmonary Bypass -- Rehabilitation Heart Defects, Congenital -- Diagnosis Heart Defects, Congenital -- Metabolism Heart Defects, Congenital -- Physiopathology Heart Defects, Congenital -- Surgery Heart Ventricle -- Abnormalities Exertion Postoperative Complications -- Diagnosis Postoperative Complications -- Physiopathology Postoperative Complications -- Prevention and Control Quality of Life Adolescence Adult Blood Flow Velocity Cardiac Output Disease Management Exercise Test -- Methods Therapeutic Exercise -- Methods Female Hemodynamics Human Male Pennsylvania N1 - research. Journal Subset: Biomedical; Blind Peer Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. NLM UID: 9602087. PMID: NLM25184826. PY - 2015 SN - 1355-6037 SP - 139-143 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=103874614&site=ehost-live&scope=site VL - 101 ID - 1513 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 singleventricle 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 phaseencoded 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=0.9671x3.0263, 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. © 2014, BMJ Publishing Group. All rights reserved. AD - Wallace H. Coulter Department of Biomedical Engineering, Georgia Institute of Technology, Emory University, 387 Technology Circle, Suite 232, Atlanta, GA 30313, United States Division of Cardiology, Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA, United States Westat, Rockville, MD, United States 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. DB - Scopus DO - 10.1136/heartjnl-2014-306337 IS - 2 M3 - Article N1 - Cited By :53 Export Date: 15 June 2020 PY - 2015 SP - 139-143 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84920530595&doi=10.1136%2fheartjnl-2014-306337&partnerID=40&md5=00de27cd4eff41c4d4dcca70503e459d VL - 101 ID - 1967 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. © 2013 Canadian Cardiovascular Society. AD - Stollery Children's Hospital, University of Alberta, Edmonton, AB, Canada AU - Khoo, N. S. AU - Tham, E. B. AU - Kantor, P. F. DB - Scopus DO - 10.1016/j.cjca.2013.03.025 IS - 7 M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2013 SP - 886-889 ST - Newer imaging modalities in the assessment of heart function in single ventricle hearts T2 - Canadian Journal of Cardiology TI - Newer imaging modalities in the assessment of heart function in single ventricle hearts UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84879507884&doi=10.1016%2fj.cjca.2013.03.025&partnerID=40&md5=65a14844c1d1dd10b1ae7c10e52af545 VL - 29 ID - 2043 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 and flow configurations and as such, provides an additional step toward understanding the detailed fluid mechanical behavior of the more complex physiological configurations seen clinically. AD - Cardiovasc. Flow Research Laboratory, Children's Hospital, CU Health Sciences Center, 1056 E. 19th Avenue, Denver, CO 80218, United States AU - Khunatorn, Y. AU - Mahalingam, S. AU - DeGroff, C. G. AU - Shandas, R. DB - Scopus DO - 10.1115/1.1487880 IS - 4 M3 - Article N1 - Cited By :47 Export Date: 15 June 2020 PY - 2002 SP - 364-377 ST - Influence of connection geometry and SVC-IVC flow rate ratio on flow structures within the total cavopulmonary connection: A numerical study T2 - Journal of Biomechanical Engineering TI - Influence of connection geometry and SVC-IVC flow rate ratio on flow structures within the total cavopulmonary connection: A numerical study UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0036667708&doi=10.1115%2f1.1487880&partnerID=40&md5=52a361ba43692c0d88ab066a7c8873eb VL - 124 ID - 2266 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 - A. Khushnood, Department of Cardiopulmonary Transplantation, Freeman Hospital, Newcastle Upon Tyne, United Kingdom 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. DB - Embase Medline DO - 10.1111/petr.13536 IS - 6 KW - azathioprine cyclosporine dipeptidyl carboxypeptidase inhibitor methylprednisolone sildenafil thymocyte antibody aortic valve disease article case report child clinical article donation after circulatory determined death follow up graft recipient heart donor heart function heart perfusion heart transplantation human human tissue hypoplastic left heart syndrome immunosuppressive treatment length of stay male mitral valve atresia operation duration pediatrics school child surgical technique treatment outcome treatment response LA - English M3 - Article N1 - L628430794 2019-07-12 2019-09-04 PY - 2019 SN - 1399-3046 1397-3142 ST - Paediatric donation after circulatory determined death heart transplantation using donor normothermic regional perfusion and ex situ heart perfusion: A case report T2 - Pediatric Transplantation TI - Paediatric donation after circulatory determined death heart transplantation using donor normothermic regional perfusion and ex situ heart perfusion: A case report UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L628430794 http://dx.doi.org/10.1111/petr.13536 VL - 23 ID - 644 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. © 2019 Wiley Periodicals, Inc. AD - Department of Cardiopulmonary Transplantation, Freeman Hospital, Newcastle Upon Tyne, United Kingdom Department of Cardiothoracic Surgery and Transplantation, Freeman Hospital, Newcastle Upon Tyne, United Kingdom Department of Cardiac Anaesthesia and Intensive Care, Freeman Hospital, Newcastle Upon Tyne, United Kingdom Department of Cardiothoracic Transplantation, Royal Papworth Hospital, Cambridgeshire, United Kingdom Department of Cardiothoracic Surgery, Institute of Cellular Medicine, Newcastle University, Newcastle Upon Tyne, United Kingdom 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. C7 - e13536 DB - Scopus DO - 10.1111/petr.13536 IS - 6 KW - donation after circulatory determined death heart transplantation normothermic regional perfusion paediatric M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2019 ST - Paediatric donation after circulatory determined death heart transplantation using donor normothermic regional perfusion and ex situ heart perfusion: A case report T2 - Pediatric Transplantation TI - Paediatric donation after circulatory determined death heart transplantation using donor normothermic regional perfusion and ex situ heart perfusion: A case report UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85068536827&doi=10.1111%2fpetr.13536&partnerID=40&md5=cd7210d94399b60197272c6f6f76fb48 VL - 23 ID - 1768 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 - Department of Anesthesiology and Pain Medicine, School of Medicine, Pusan National University, Beomeo-ri, Mulgeum-eup, Yangsan 626-770, South Korea Medical Research Institute, Pusan National University Hospital, Busan, South Korea AU - Kim, H. J. AU - Kim, J. Y. AU - Baek, S. H. AU - Kim, H. K. DB - Scopus DO - 10.4097/kjae.2010.58.5.480 IS - 5 KW - Congenital heart surgery Intravascular ultrasound catheter Transesophageal echocardiography M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2010 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-77953334164&doi=10.4097%2fkjae.2010.58.5.480&partnerID=40&md5=9b616378889a99ef150af3b05fdb67ca VL - 58 ID - 2152 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 - Cardiovascular Center, Showa University, Northern Yokohama Hospital, 35-1 Chigasaki-chuo, Tsuzuki-ku, Yokohama 224-8503, Japan Renal Center, Japanese Red Cross Kumamoto Hospital, Kumamoto, Japan Children's Medical Center, Showa University, Northern Yokohama Hospital, Yokohama, Japan Department of Pediatrics, University of Yamanashi Hospital, Yamanashi, 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 - Scopus DO - 10.1111/joic.12122 IS - 4 M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2014 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84904858284&doi=10.1111%2fjoic.12122&partnerID=40&md5=dd2d1dd500c60785863eda5ddec16d1f VL - 27 ID - 2027 ER - TY - JOUR AU - Knirsch, W. AU - Liamlahi, R. AU - Dave, H. AU - Kretschmar, O. AU - Latal, B. DB - Medline DO - 10.5761/atcs.lte.16-00106 IS - 6 KW - adverse event age child behavior child development cognition comparative study female growth, development and aging human hypoplastic left heart syndrome infant male motor activity nervous system Norwood procedure pathophysiology preschool child psychology time factor treatment outcome LA - English M3 - Article N1 - L616659085 2017-06-12 PY - 2016 SN - 2186-1005 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 - Annals of thoracic and cardiovascular surgery : official journal of the Association of Thoracic and Cardiovascular Surgeons of Asia TI - Neurodevelopmental Outcome of Children with Hypoplastic Left Heart Syndrome at One and Four Years of Age Comparing Hybrid and Norwood Procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L616659085 http://dx.doi.org/10.5761/atcs.lte.16-00106 VL - 22 ID - 783 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. © The Author 2012. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. AD - W. Knirsch, Division of Pediatric Cardiology, University Children's Hospital Zurich, Steinwiesstrasse 75, 8032 Zurich, Switzerland 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. DB - Embase Medline DO - 10.1093/ejcts/ezr286 IS - 1 KW - adult article artificial ventilation ascending aorta birth weight child clinical article cognitive defect female heart single ventricle heart surgery hospitalization human hybrid procedure hypoplastic left heart syndrome intensive care unit intermethod comparison length of stay male mental development nerve cell differentiation Norwood procedure preschool child priority journal psychomotor development school child LA - English M3 - Article N1 - L368247614 2013-02-08 2013-02-20 PY - 2012 SN - 1010-7940 1873-734X SP - 33-39 ST - Mortality and neurodevelopmental outcome at 1 year of age comparing hybrid and Norwood procedures T2 - European Journal of Cardio-thoracic Surgery TI - Mortality and neurodevelopmental outcome at 1 year of age comparing hybrid and Norwood procedures UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L368247614 http://dx.doi.org/10.1093/ejcts/ezr286 VL - 42 ID - 1051 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. © The Author 2012. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. AD - Division of Paediatric Cardiology, University Children's Hospital Zurich, Zurich, Switzerland Neonatology and Paediatric Intensive Care Unit, University Children's Hospital Zurich, Zurich, Switzerland Child Development Center, University Children's Hospital Zurich, Zurich, Switzerland Division of Congenital Cardiac Surgery, University Children's Hospital Zurich, Zurich, Switzerland 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. C7 - ezr286 DB - Scopus DO - 10.1093/ejcts/ezr286 IS - 1 KW - Cardiopulmonary bypass Congenital heart defects Hybrid procedure Hypoplastic left heart syndrome Neurocognitive deficits Norwood operation M3 - Article N1 - Cited By :41 Export Date: 15 June 2020 PY - 2012 SP - 33-39 ST - Mortality and neurodevelopmental outcome at 1 year of age comparing hybrid and Norwood procedures T2 - European Journal of Cardio-thoracic Surgery TI - Mortality and neurodevelopmental outcome at 1 year of age comparing hybrid and Norwood procedures UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84871674573&doi=10.1093%2fejcts%2fezr286&partnerID=40&md5=d075551d6b40872eb1d91027eaacc4a5 VL - 42 ID - 2090 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. © 2019 Heart Rhythm Society AD - Department of Pediatric Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan Ohori Children's Clinic, 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 - Scopus DO - 10.1016/j.hrthm.2019.06.019 IS - 12 KW - Apical pacing Epicardial lead Fontan procedure Functional single ventricle Pacemaker implantation M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2019 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85074409782&doi=10.1016%2fj.hrthm.2019.06.019&partnerID=40&md5=c9d44adb8d41a4b62c4d78bbaa0809ae VL - 16 ID - 1722 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. AD - Section of Pediatric Critical Care Medicine and the Program in Bioethics, University of California, Davis, Ticon II, Room 228, 2516 Stockton Blvd., Sacramento, CA 95817 AN - 106621516. Language: English. Entry Date: 20050429. Revision Date: 20170831. Publication Type: Journal Article AU - Kon, A. A. DB - ccm DP - EBSCOhost IS - 8 KW - Professional-Family Relations Communication -- Methods Hypoplastic Left Heart Syndrome -- Surgery Specialties, Medical Confidence Intervals Coefficient Alpha Multivariate Analysis Data Analysis Software Linear Regression Funding Source Human N1 - research; tables/charts. Journal Subset: Biomedical; Peer Reviewed; USA. Grant Information: Kadima Foundation, Mill Valley, CA and by the University of California, San Francisco, and the University of California, Davis. NLM UID: 8501884. PMID: NLM15129229. 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106621516&site=ehost-live&scope=site VL - 24 ID - 1506 ER - TY - JOUR AD - A.A. Kon, University of California, Davis, Department of Pediatrics, Ticon II, 2516 Stockton Boulevard, Sacramento, CA 95817, United States AU - Kon, A. A. AU - Ackerson, L. AU - Lo, B. DB - Embase Medline DO - 10.1016/S0002-9149(03)00412-0 IS - 12 KW - article controlled study diagnostic value heart volume human hypoplastic left heart syndrome intensive care male mother child relation neonatology patient care physician postoperative period pregnancy termination priority journal statistical analysis survival rate treatment outcome LA - English M3 - Article N1 - L36694738 2003-06-24 PY - 2003 SN - 0002-9149 SP - 1506-1509 ST - Choices physicians would make if they were the parents of a child with hypoplastic left heart syndrome T2 - American Journal of Cardiology TI - Choices physicians would make if they were the parents of a child with hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L36694738 http://dx.doi.org/10.1016/S0002-9149(03)00412-0 VL - 91 ID - 1302 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 Department of Pediatrics, Children's Mercy Kansas City, Kansas City, Missouri AN - 119318933. Language: English. Entry Date: 20161118. Revision Date: 20190222. Publication Type: Article AU - Kon, Alexander A. AU - Patel, Angira AU - Leuthner, Steven AU - Lantos, John D. DB - ccm DO - 10.1542/peds.2016-1730 DP - EBSCOhost IS - 5 KW - Tricuspid Atresia -- Surgery Treatment Refusal Parental Attitudes Tricuspid Atresia -- Diagnosis -- In Utero Decision Making, Ethical Ethics, Medical Palliative Care -- Ethical Issues -- In Infancy and Childhood Pregnancy Female Life Support Care Fetus Survival Quality of Life Infant, Newborn N1 - case study. Journal Subset: Biomedical; Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. NLM UID: 0376422. PY - 2016 SN - 0031-4005 SP - 1-7 ST - Parental Refusal of Surgery in an Infant With Tricuspid Atresia T2 - Pediatrics TI - Parental Refusal of Surgery in an Infant With Tricuspid Atresia UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=119318933&site=ehost-live&scope=site VL - 138 ID - 1484 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. Copyright © 2016 by the American Academy of Pediatrics. AD - Naval Medical Center San Diego, University of California San Diego, School of Medicine, San Diego, CA, United States Lurie Children's Hospital, Chicago, IL, United States Medical College of Wisconsin, Milwaukee, WI, United States Department of Pediatrics, Children's Mercy Hospital, 2401 Gillham Rd., Kansas City, MO 64108, United States AU - Kon, A. A. AU - Patel, A. AU - Leuthner, S. AU - Lantos, J. D. C7 - e20161730 DB - Scopus DO - 10.1542/peds.2016-1730 IS - 5 M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2016 ST - Parental refusal of surgery in an infant with tricuspid atresia T2 - Pediatrics TI - Parental refusal of surgery in an infant with tricuspid atresia UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84994885741&doi=10.1542%2fpeds.2016-1730&partnerID=40&md5=ee9a3caccf656c914e507035599af5a1 VL - 138 ID - 1885 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 - Depts. Pediat. Cardiol. and Radiol., Heart Institute of Japan, Tokyo Women's Medical College, Japan Department of Pediatric Cardiology, Heart Institute of Japan, Tokyo Women's Medical College, 8-1, Kawada-cho, Shinjuku-ku, Tokyo, 162, Japan AU - Kondo, C. AU - Nakazawa, M. AU - Kusakabe, K. AU - Momma, K. DB - Scopus DO - 10.1016/S1071-3581(96)90021-5 IS - 1 KW - Congenital Heart defects Myocardial metabolism M3 - Article N1 - Cited By :6 Export Date: 15 June 2020 PY - 1996 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0030066072&doi=10.1016%2fS1071-3581%2896%2990021-5&partnerID=40&md5=4f6753abdcf4c7f478089d53a3e16885 VL - 3 ID - 2299 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/m2 in preserved Fontans versus 30.4±6.2 mL/m2 in impaired Fontans (P=0.002) and 51.1±6.9 mL/m2 in controls (P=0.107). The cutoff value for differentiation of Fontan groups was SVi (AAo, end-expiratory) of 32.1 mL/m2. After hyperoxygenation, the mean SVi (AAo) increased to 48.7±12.7 mL/m2 in preserved Fontans (P=0.045) but remained unchanged in impaired Fontans (31.1±5.8 mL/m2, 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 SV i 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 SV i represents a valuable measure for classifying the severity of Fontan hemodynamics impairment. AD - H. Körperich, Herz-und Diabeteszentrum NRW, Institut für Radiologie, Nuklearmedizin und Molekulare Bildgebung, Georgstraße 11, Bad Oeynhausen, Germany 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. DB - Embase Medline DO - 10.1097/RTI.0000000000000261 IS - 3 KW - adolescent adult article blood flow velocity breathing cardiac imaging cardiovascular magnetic resonance child circulation clinical article controlled clinical trial controlled study diagnostic accuracy diagnostic test accuracy study echo planar imaging Fontan procedure cardiac index heart rate heart stroke volume hemodynamics human hyperoxia inferior cava vein lung blood flow prospective study receiver operating characteristic superior cava vein vasodilatation LA - English M3 - Article N1 - L615055857 2017-04-03 2017-05-05 PY - 2017 SN - 1536-0237 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 - Journal of Thoracic Imaging TI - Differentiation of Impaired from Preserved Hemodynamics in Patients with Fontan Circulation Using Real-time Phase-velocity Cardiovascular Magnetic Resonance UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L615055857 http://dx.doi.org/10.1097/RTI.0000000000000261 VL - 32 ID - 780 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 - 122601523. Language: English. Entry Date: 20180731. Revision Date: 20171113. Publication Type: journal article. Journal Subset: Biomedical AU - Körperich, Hermann AU - Müller, Katja AU - Barth, Peter AU - Gieseke, Jürgen AU - Haas, Nikolaus AU - Schulze-Neick, Ingram AU - Burchert, Wolfgang AU - Kececioglu, Deniz AU - Laser, Kai T. DB - ccm DO - 10.1097/RTI.0000000000000261 DP - EBSCOhost IS - 3 KW - Magnetic Resonance Imaging -- Methods Hemodynamics -- Physiology Vena Cava, Inferior -- Physiopathology Aorta -- Physiopathology Vena Cava, Superior -- Physiopathology Cardiopulmonary Bypass Aorta Adult Prospective Studies Heart Rate -- Physiology Adolescence Female Vena Cava, Inferior Vena Cava, Superior Male Young Adult Diagnosis, Differential Child Ferrans and Powers Quality of Life Index Impact of Events Scale N1 - Peer Reviewed; USA. Instrumentation: Ferrans and Powers Quality of Life Index; Impact of Events Scale (IES); Home Observation for Measurement of the Environment (HOME) (Bradley and Caldwell); Longitudinal Interval Follow-Up Evaluation (LIFE). NLM UID: 8606160. PMID: NLM28346330. PY - 2017 SN - 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 - Journal of Thoracic Imaging TI - Differentiation of Impaired From Preserved Hemodynamics in Patients With Fontan Circulation Using Real-time Phase-velocity Cardiovascular Magnetic Resonance UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=122601523&site=ehost-live&scope=site VL - 32 ID - 1490 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/m2 in preserved Fontans versus 30.4±6.2 mL/m2 in impaired Fontans (P=0.002) and 51.1±6.9 mL/m2 in controls (P=0.107). The cutoff value for differentiation of Fontan groups was SVi (AAo, end-expiratory) of 32.1 mL/m2. After hyperoxygenation, the mean SVi (AAo) increased to 48.7±12.7 mL/m2 in preserved Fontans (P=0.045) but remained unchanged in impaired Fontans (31.1±5.8 mL/m2, 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 SV i 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 SV i represents a valuable measure for classifying the severity of Fontan hemodynamics impairment. Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved. AD - Herz-und Diabeteszentrum NRW, Institut für Radiologie, Nuklearmedizin und Molekulare Bildgebung, Georgstraße 11, Bad Oeynhausen, D-32545, Germany Department for Congenital Heart Defects, Heart and Diabetes Centre North Rhine Westphalia Ruhr University Bochum, Bad Oeynhausen, Germany Department of Radiology, University of Bonn, Bonn, Germany Department for Pediatric Cardiology and Pediatric Intensive Care, Ludwig Maximilians University, Medical Hospital of the University of Munich, Munich, Germany 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. DB - Scopus DO - 10.1097/RTI.0000000000000261 IS - 3 KW - congenital heart Defects hemodynamics magnetic resonance imaging respiration vasodilation M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2017 SP - 159-168 ST - Differentiation of Impaired from Preserved Hemodynamics in Patients with Fontan Circulation Using Real-time Phase-velocity Cardiovascular Magnetic Resonance T2 - Journal of Thoracic Imaging TI - Differentiation of Impaired from Preserved Hemodynamics in Patients with Fontan Circulation Using Real-time Phase-velocity Cardiovascular Magnetic Resonance UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85016091903&doi=10.1097%2fRTI.0000000000000261&partnerID=40&md5=48f36242e3ee91774ebcfbdd95b7897d VL - 32 ID - 1882 ER - TY - JOUR AD - Department of Pediatric Cardiology and Pulmonology, Heinrich Heine University, Duesseldorf, Moorenstrasse 540225, Germany AU - Kovacevic, A. AU - Schmidt, K. G. AU - Schaper, J. AU - Messing-Juenger, M. AU - Hoehn, T. DB - Scopus DO - 10.1007/s00381-012-1726-8 IS - 10 M3 - Article N1 - Export Date: 15 June 2020 PY - 2012 SP - 1815-1817 ST - Severely impaired wound healing in a Fontan patient after neurosurgery: a novel topic in univentricular heart physiology? T2 - Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery TI - Severely impaired wound healing in a Fontan patient after neurosurgery: a novel topic in univentricular heart physiology? UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85028123910&doi=10.1007%2fs00381-012-1726-8&partnerID=40&md5=5ee60d8e32383d63efa5ac61d0ce0fc1 VL - 28 ID - 2101 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 - Intensive Care Unit, Pediatric Cardiac Center, Limbova 1, 833 51 Bratislava, Slovakia AU - Kovacikova, L. AU - Dobos, D. AU - Zahorec, M. DB - Scopus DO - 10.1510/icvts.2008.187096 IS - 1 KW - Complications Congenital heart disease Diaphragm Ventilation M3 - Article N1 - Cited By :12 Export Date: 15 June 2020 PY - 2009 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-58149387830&doi=10.1510%2ficvts.2008.187096&partnerID=40&md5=4feeaf29fdaaa2f6bc91ac83eaa59475 VL - 8 ID - 2185 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 - Division of Cardiovascular Surgery, Heart Institute for Children, Hope Children's Hospital, Oak-Lawn, IL, United States Division of Cardiothoracic Surgery, University of Illinois of Chicago, Chicago, IL, United States 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. DB - Scopus IS - 1 M3 - Article N1 - Cited By :10 Export Date: 15 June 2020 PY - 2002 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0036025405&partnerID=40&md5=a20213572e7948b9c690020c54a953ae VL - 123 ID - 2267 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 - UNMC/CUMC Joint Division of Pediatric Cardiology, Children's Hospital and Medical Center, 8200 Dodge Street, Omaha, NE 68114, United States Division of Pediatric Cardiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Pediatrics Institute, Cleveland Clinic, Cleveland, OH, United States Department of Cardiology, Boston Children's Hospital, Boston, MA, United States Division of Pediatric Cardiology, Mattel Children's Hospital, University of California Loas Angeles, Los Angeles, CA, United States Department of Pediatric Cardiology, Children's National Medical Center, Washington, DC, United States Pediatric Cardiology Section, Texas Children's Hospital, Houston, TX, United States Center for Health Care Quality, Division of Health Policy and Clinical Effectiveness, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 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 - Scopus DO - 10.1111/j.1747-0803.2009.00328.x IS - 5 KW - Cardiac Pediatric Quality improvement M3 - Article N1 - Cited By :89 Export Date: 15 June 2020 PY - 2009 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-70149110655&doi=10.1111%2fj.1747-0803.2009.00328.x&partnerID=40&md5=4d2881d43f4f5508d211fa5de6b80b8c VL - 4 ID - 2171 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. Copyright © 2019 by the American Academy of Pediatrics. AD - Center for Bioethics and Social Sciences in Medicine, University of Michigan, Ann Arbor, MI, United States Division of Neonatal-Perinatal Medicine, Department of Pediatrics, University of Michigan, Ann Arbor, MI, United States Division of Professional Education, Department of Learning Health Sciences, University of Michigan, Ann Arbor, MI, United States Department of Surgery, University of Michigan, Ann Arbor, MI, United States Children’s Mercy Bioethics Center, Children’s Mercy Hospital, Kansas City, MO, United States AU - Kukora, S. AU - Firn, J. AU - Laventhal, N. AU - Vercler, C. AU - Moore, B. AU - Lantos, J. D. C7 - Y DB - Scopus DO - 10.1542/peds.2018-3779 IS - 5 M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2019 ST - Infant with trisomy 18 and hypoplastic left heart syndrome T2 - Pediatrics TI - Infant with trisomy 18 and hypoplastic left heart syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85065509392&doi=10.1542%2fpeds.2018-3779&partnerID=40&md5=e8a1ab581c9da16728c41d613714b74a VL - 143 ID - 1749 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 - J.K. Kirklin, Division of Cardiothoracic Surgery, Department of Surgery, University of Alabama, Birmingham, Alabama, United States AU - Kukreja, M. AU - Bryant, A. S. AU - Cleveland, D. C. AU - Dabal, R. AU - Hingorani, N. AU - Kirklin, J. K. DB - Embase Medline DO - 10.1053/j.semtcvs.2015.08.007 IS - 3 KW - adolescent adult article child comparative study controlled study cross-sectional study energy female Fontan procedure graft recipient heart graft heart single ventricle human life satisfaction major clinical study male mental function pain palliative therapy pediatric surgery physical activity preschool child quality of life school child Short Form 36 social interaction survivor United States LA - English M3 - Article N1 - L607389189 2015-12-30 2016-09-16 PY - 2015 SN - 1532-9488 1043-0679 SP - 299-306 ST - Health-Related Quality of Life in Adult Survivors After the Fontan Operation T2 - Seminars in Thoracic and Cardiovascular Surgery TI - Health-Related Quality of Life in Adult Survivors After the Fontan Operation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L607389189 http://dx.doi.org/10.1053/j.semtcvs.2015.08.007 VL - 27 ID - 848 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 AN - 111876890. Language: English. Entry Date: 20160826. Revision Date: 20190515. Publication Type: journal article AU - Kukreja, Manisha AU - Bryant, Ayesha S. AU - Cleveland, David C. AU - Dabal, Robert AU - Hingorani, Neha AU - Kirklin, James K. DA - Fall2015 DB - ccm DO - 10.1053/j.semtcvs.2015.08.007 DP - EBSCOhost IS - 3 KW - Heart Defects, Congenital -- Surgery Quality of Life Cardiopulmonary Bypass Health Status Postoperative Period Young Adult Prospective Studies Female Cross Sectional Studies Adolescence Human Child Retrospective Design Adult Heart Defects, Congenital -- Mortality Time Factors Male Alabama Survival -- Trends Child, Preschool Validation Studies Comparative Studies Evaluation Research Multicenter Studies Questionnaires Short Form-36 Health Survey (SF-36) N1 - research. Journal Subset: Biomedical; USA. Instrumentation: Short Form-36 Health Survey (SF-36). NLM UID: 8917640. PMID: NLM26708372. PY - 2015 SN - 1043-0679 SP - 299-306 ST - Health-Related Quality of Life in Adult Survivors After the Fontan Operation T2 - Seminars in Thoracic & Cardiovascular Surgery TI - Health-Related Quality of Life in Adult Survivors After the Fontan Operation UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=111876890&site=ehost-live&scope=site VL - 27 ID - 1487 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. © 2015 Elsevier Inc. AD - Department of Surgery, University of Alabama at Birmingham, Birmingham, Alabama, United States AU - Kukreja, M. AU - Bryant, A. S. AU - Cleveland, D. C. AU - Dabal, R. AU - Hingorani, N. AU - Kirklin, J. K. DB - Scopus DO - 10.1053/j.semtcvs.2015.08.007 IS - 3 KW - Fontan procedures health-related quality of life single-ventricle conditions M3 - Article N1 - Cited By :8 Export Date: 15 June 2020 PY - 2015 SP - 299-306 ST - Health-Related Quality of Life in Adult Survivors After the Fontan Operation T2 - Seminars in Thoracic and Cardiovascular Surgery TI - Health-Related Quality of Life in Adult Survivors After the Fontan Operation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84951309694&doi=10.1053%2fj.semtcvs.2015.08.007&partnerID=40&md5=3d291779112218b6a997ef4478544beb VL - 27 ID - 1935 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, Japan 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. DB - Scopus IS - 3 II SUPPl. M3 - Article N1 - Cited By :24 Export Date: 15 June 2020 PY - 1987 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0023176945&partnerID=40&md5=383664f57105e3c794a7e1b4c7a85f70 VL - 76 ID - 2324 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. ClinicalTrials.gov Identifier: NCT00861848. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2011. AD - S. Kutty, Joint Division of Pediatric Cardiology, University of Nebraska College of Medicine/Creighton University, Children's Hospital and Medical Center, Omaha, NE, United States 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. DB - Embase Medline DO - 10.1093/ejechocard/jer287 IS - 6 KW - NCT00861848 echo contrast medium adolescent adult aortic valve disease article bicycle blood pressure measurement cardiac imaging cardiovascular magnetic resonance clinical trial congenital heart disease contrast enhancement Doppler flowmetry Fallot tetralogy female Fontan procedure great vessels transposition heart arrhythmia heart hemodynamics heart left ventricle heart left ventricle ejection fraction heart right ventricle heart ventricle contractility heart ventricle function heart ventricle pressure human image quality low back pain low mechanical index real time perfusion major clinical study male mucocutaneous lymph node syndrome cardiac rhythm management device priority journal prospective study rest stress echocardiography supine bicycle stress echocardiography supine position systolic blood pressure LA - English M3 - Article N1 - L365242774 2012-07-23 2012-07-30 PY - 2012 SN - 2047-2404 SP - 500-509 ST - Ultrasound contrast and real-time perfusion in conjunction with supine bicycle stress echocardiography for comprehensive evaluation of surgically corrected congenital heart disease T2 - European Heart Journal Cardiovascular Imaging TI - Ultrasound contrast and real-time perfusion in conjunction with supine bicycle stress echocardiography for comprehensive evaluation of surgically corrected congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L365242774 http://dx.doi.org/10.1093/ejechocard/jer287 VL - 13 ID - 1057 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 Department of Internal Medicine, Section of Cardiology , University of Nebraska Medical Center , Omaha, NE , USA AN - 82563650. Language: English. Entry Date: 20121025. Revision Date: 20190226. Publication Type: Article AU - Kutty, Shelby AU - Olson, Joan AU - Danford, Christopher J. AU - Sandene, Erin K. AU - Xie, Feng AU - Fletcher, Scott E. AU - Erickson, Christopher C. AU - Kugler, John D. AU - Danford, David A. AU - Porter, Thomas R. DB - ccm DO - 10.1093/ejechocard/jer287 DP - EBSCOhost IS - 6 KW - Contrast Media -- Diagnostic Use Echocardiography -- Methods Fluorocarbons -- Diagnostic Use Heart Defects, Congenital -- Physiopathology Heart Defects, Congenital -- Surgery Heart Defects, Congenital -- Ultrasonography Image Interpretation, Computer Assisted -- Methods Human Adolescence Adult Female Male Hemodynamics Linear Regression Magnetic Resonance Imaging -- Methods Nebraska Prospective Studies Descriptive Statistics Young Adult Supine Position Clinical Trials Wilcoxon Signed Rank Test Data Analysis Software Funding Source N1 - clinical trial; diagnostic images; research; tables/charts; tracings. Journal Subset: Biomedical; Continental Europe; Europe; Peer Reviewed. Grant Information: This study was supported by the Children’s Hospital and Medical Centre Foundation Grant (2009–2011).. PY - 2012 SN - 2047-2404 SP - 500-509 ST - Ultrasound contrast and real-time perfusion in conjunction with supine bicycle stress echocardiography for comprehensive evaluation of surgically corrected congenital heart disease T2 - European Heart Journal - Cardiovascular Imaging TI - Ultrasound contrast and real-time perfusion in conjunction with supine bicycle stress echocardiography for comprehensive evaluation of surgically corrected congenital heart disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=82563650&site=ehost-live&scope=site VL - 13 ID - 1565 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. ClinicalTrials.gov Identifier: NCT00861848. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2011. AD - Joint Division of Pediatric Cardiology, University of Nebraska College of Medicine/Creighton University, Children's Hospital and Medical Center, Omaha, NE, United States Department of Internal Medicine, Section of Cardiology, University of Nebraska Medical Center, Omaha, NE, United States 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. DB - Scopus DO - 10.1093/ejechocard/jer287 IS - 6 KW - Adult congenital heart disease Paediatric cardiology Real-time perfusion Stress testing Ultrasound contrast Wall motion M3 - Article N1 - Cited By :18 Export Date: 15 June 2020 PY - 2012 SP - 500-509 ST - Ultrasound contrast and real-time perfusion in conjunction with supine bicycle stress echocardiography for comprehensive evaluation of surgically corrected congenital heart disease T2 - European Heart Journal Cardiovascular Imaging TI - Ultrasound contrast and real-time perfusion in conjunction with supine bicycle stress echocardiography for comprehensive evaluation of surgically corrected congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84863853801&doi=10.1093%2fejechocard%2fjer287&partnerID=40&md5=dcb1e8666a4843460c2358935ecdc9d3 VL - 13 ID - 2095 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 - K.N. Lopez, Division of Cardiology, Department of Pediatrics, Texas Children’s Hospital, Baylor College of Medicine, 6621 Fannin, MC# 19345-C, Houston, TX, United States AU - Kyle, W. B. AU - Denfield, S. W. AU - Valdes, S. O. AU - Penny, D. J. AU - Bolin, E. H. AU - Lopez, K. N. DB - Embase Medline DO - 10.1007/s00246-015-1312-4 IS - 3 KW - methoxy isobutyl isonitrile technetium tc 99m metoprolol adolescent angiocardiography article Blalock Taussig shunt cardiopulmonary exercise test child clinical article echocardiography electrocardiogram female fenestration follow up Fontan procedure heart arrhythmia heart catheterization heart muscle ischemia heart right bundle branch block heart surgery heart ventricle function heart ventricle hypertrophy heart ventricle tachycardia Holter monitoring human hypoplastic left heart syndrome male medical record review myocardial perfusion imaging Norwood procedure palliative therapy Sano modification school child ST segment ST segment depression supraventricular tachycardia treadmill exercise LA - English M3 - Article N1 - L607490289 2016-01-07 2016-04-25 PY - 2016 SN - 1432-1971 0172-0643 SP - 545-551 ST - Assessing ST Segment Changes and Ischemia During Exercise Stress Testing in Patients with Hypoplastic Left Heart Syndrome and Fontan Palliation T2 - Pediatric Cardiology TI - Assessing ST Segment Changes and Ischemia During Exercise Stress Testing in Patients with Hypoplastic Left Heart Syndrome and Fontan Palliation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L607490289 http://dx.doi.org/10.1007/s00246-015-1312-4 VL - 37 ID - 816 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. © 2016, Springer Science+Business Media New York. AD - Division of Cardiology, Department of Pediatrics, Texas Children’s Hospital, Baylor College of Medicine, 6621 Fannin, MC# 19345-C, Houston, TX 77030, United States Department of Pediatrics, Arkansas Children’s Hospital, University of Arkansas Medical School, Little Rock, AR, United States AU - Kyle, W. B. AU - Denfield, S. W. AU - Valdes, S. O. AU - Penny, D. J. AU - Bolin, E. H. AU - Lopez, K. N. DB - Scopus DO - 10.1007/s00246-015-1312-4 IS - 3 KW - Congenital heart disease Exercise stress testing Fontan Hypoplastic left heart syndrome Ischemia ST segment abnormality M3 - Article N1 - Export Date: 15 June 2020 PY - 2016 SP - 545-551 ST - Assessing ST Segment Changes and Ischemia During Exercise Stress Testing in Patients with Hypoplastic Left Heart Syndrome and Fontan Palliation T2 - Pediatric Cardiology TI - Assessing ST Segment Changes and Ischemia During Exercise Stress Testing in Patients with Hypoplastic Left Heart Syndrome and Fontan Palliation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84952683181&doi=10.1007%2fs00246-015-1312-4&partnerID=40&md5=243494498c15c299032a17640b2faf97 VL - 37 ID - 1917 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. AU - Lafranchi, T. AU - Lincoln, P. DB - Medline DO - 10.4037/ccn2015247 IS - 5 KW - counseling female fetus heart disease hospital human intensive care unit obstetric delivery pregnancy prenatal care prenatal diagnosis procedures prognosis LA - English M3 - Article N1 - L616488397 2017-06-02 PY - 2015 SN - 1940-8250 SP - 53-61 ST - Prenatal Counseling and Care for Single-Ventricle Heart Disease: One Center's Model for Care T2 - Critical care nurse TI - Prenatal Counseling and Care for Single-Ventricle Heart Disease: One Center's Model for Care UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L616488397 http://dx.doi.org/10.4037/ccn2015247 VL - 35 ID - 849 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. © 2015 American Association of Critical-Care Nurses. AD - Department of Cardiology, Boston Children's Hospital, United States Cardiovascular Intensive Care Unit, Boston Children's Hospital, United States AU - Lafranchi, T. AU - Lincoln, P. DB - Scopus DO - 10.4037/ccn2015247 IS - 5 M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2015 SP - 53-61 ST - Prenatal counseling and care for single-ventricle heart disease: One center's model for care T2 - Critical Care Nurse TI - Prenatal counseling and care for single-ventricle heart disease: One center's model for care UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84945969020&doi=10.4037%2fccn2015247&partnerID=40&md5=eb376e12a75ce065aa4d73676769f94d VL - 35 ID - 1973 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 - L.M. Lambert, Administrative Director Research-PCH Heart Center, University of Utah, 81 N Mario Capecchi Dr, Salt Lake City, UT, 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. DB - Embase Medline DO - 10.1016/j.ahj.2020.03.022 KW - anthropometry article body height body mass body weight child controlled study exercise female heart right ventricle human longitudinal study major clinical study male mortality multicenter study oxygen consumption Pediatric Quality of Life Inventory quality of life survivor LA - English M3 - Article N1 - L2005884738 2020-05-20 PY - 2020 SN - 1097-6744 0002-8703 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 - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2005884738 http://dx.doi.org/10.1016/j.ahj.2020.03.022 VL - 224 ID - 549 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 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 AN - 143681071. Corporate Author: Pediatric Heart Network Investigators. Language: English. Entry Date: In Process. Revision Date: 20200613. Publication Type: journal article. Journal Subset: Biomedical AU - Lambert, Linda M. AU - McCrindle, Brian W. AU - Pemberton, Victoria L. AU - Hollenbeck-Pringle, Danielle AU - Atz, Andrew M. AU - Ravishankar, Chitra AU - Campbell, M. Jay AU - Dunbar-Masterson, Carolyn AU - Uzark, Karen AU - Rolland, Martha AU - Trachtenberg, Felicia L. AU - Menon, Shaji C. DB - ccm DO - 10.1016/j.ahj.2020.03.022 DP - EBSCOhost N1 - USA. Grant Information: U10 HL109781/HL/NHLBI NIH HHS/United States. NLM UID: 0370465. PMID: NLM32428726. PY - 2020 SN - 0002-8703 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 - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=143681071&site=ehost-live&scope=site VL - 224 ID - 1430 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. Copyright © 2009 by the American Academy of Pediatrics. AD - L. M. Lambert, Department of Pediatrics, Primary Children's Medical Center, 100 N. Mario Capecchi Dr, Salt Lake City, UT 84113, United States 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 - Mingfen, X. AU - Radojewski, E. AU - Servedio, D. AU - McCrindle, B. W. DB - Embase Medline DO - 10.1542/peds.2008-1697 IS - 5 KW - dipeptidyl carboxypeptidase inhibitor adult article attitude to health behavior disorder child child health controlled study emotional disorder female fenestration Fontan procedure health health status heart catheterization human male mental health pain parental attitude patient attitude priority journal pulmonary artery malformation questionnaire school child self esteem LA - English M3 - Article N1 - L355524627 2009-11-11 http://pediatrics.aappublications.org/cgi/reprint/124/5/e942 PY - 2009 SN - 0031-4005 1098-4275 SP - e942-e949 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L355524627 http://dx.doi.org/10.1542/peds.2008-1697 VL - 124 ID - 1168 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. Copyright © 2009 by the American Academy of Pediatrics. AD - Department of Cardiothoracic, Primary Children's Medical Center, University of Utah, Salt Lake City, UT, United States Department of Pediatrics, Primary Children's Medical Center, University of Utah, Salt Lake City, UT, United States Department of Pediatrics, Children's Hospital Boston, Boston, MA, United States New England Research Institutes, Watertown, MA, United States National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, United States Department of Pediatrics, Medical University of South Carolina, Charleston, SC, United States Department of Pediatrics, Duke University Medical Center, Durham, NC, United States Department of Pediatrics, University of Toronto, Hospital for Sick Children, Toronto, ON, Canada Department of Pediatrics, Columbia University Medical Center, New York, NY, United States 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 - Mingfen, X. AU - Radojewski, E. AU - Servedio, D. AU - McCrindle, B. W. DB - Scopus DO - 10.1542/peds.2008-1697 IS - 5 KW - Congenital heart disease Fontan procedure Functional health status Quality of life Single ventricle M3 - Article N1 - Cited By :40 Export Date: 15 June 2020 PY - 2009 SP - e942-e949 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-70350497723&doi=10.1542%2fpeds.2008-1697&partnerID=40&md5=8573b5741b41c355fc84d8915a94f4f0 VL - 124 ID - 2166 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 - 100 N. Mario Capecchi Dr, Suite 2800, Salt Lake City, UT 84113; linda.lambert@imail.org AN - 105242257. Corporate Author: Pediatric Heart Network Investigators. Language: English. Entry Date: 20100101. Revision Date: 20150711. Publication Type: Journal Article 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. DB - ccm DO - 10.1542/peds.2008-1697 DP - EBSCOhost IS - 5 KW - Child Health Functional Status Health Status Heart Defects, Congenital -- Surgery Adolescence Child Data Analysis Software Descriptive Statistics Female Funding Source Human Male Questionnaires Self Report T-Tests Wilcoxon Signed Rank Test N1 - research; tables/charts. Journal Subset: Biomedical; Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. Instrumentation: Child Health Questionnaire (CHQ). Grant Information: National Heart, Lung, and Blood Institute. NLM UID: 0376422. PMID: NLM19841109. PY - 2009 SN - 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105242257&site=ehost-live&scope=site VL - 124 ID - 1642 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. (PsycINFO Database Record (c) 2016 APA, all rights reserved) AD - Lambert, Linda M., Department of Pediatrics, Primary Children’s Medical Center, 100 N. Mario Capecchi Dr, Suite 2800, Salt Lake City, UT, US, 84113 AN - 2010-01268-006 AU - Lambert, Linda M. AU - Minich, L. LuAnn AU - Newburger, Jane W. AU - Lu, Minmin AU - Pemberton, Victoria L. AU - McGrath, Ellen A. AU - Atz, Andrew M. AU - Xu, Mingfen AU - Radojewski, Elizabeth AU - Servedio, Darlene AU - McCrindle, Brian W. DB - psyh DO - 10.1542/peds.2008-1697 DP - EBSCOhost IS - 5 KW - parents child reported functional health status Fontan procedure Activities of Daily Living Adolescent Attitude to Health Child Female Health Status Heart Defects, Congenital Humans Male Quality of Life Surveys and Questionnaires Health Attitudes Parental Attitudes Surgery N1 - Department of Cardiothoracic, Primary Children’s Medical Center, Salt Lake City, UT, US. Institutional Authors: Pediatric Heart Network Investigators. Release Date: 20100531. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Health Attitudes; Parental Attitudes; Surgery. Classification: Medical Treatment of Physical Illness (3363). Population: Human (10); Male (30); Female (40). Location: Canada; US. Age Group: Childhood (birth-12 yrs) (100); School Age (6-12 yrs) (180); Adolescence (13-17 yrs) (200); Adulthood (18 yrs & older) (300). Tests & Measures: Child Health Questionnaire. Methodology: Empirical Study; Quantitative Study. References Available: Y. Page Count: 8. Issue Publication Date: Nov, 2009. Publication History: Accepted Date: Jun 5, 2009. Copyright Statement: American Academy of Pediatrics. 2009. Sponsor: National Heart, Lung, and Blood Institute, US. Grant: HL068269; HL068270; HL068279; HL068281; HL068285; HL068292; HL068290; HL068288. Recipients: No recipient indicated PY - 2009 SN - 0031-4005 1098-4275 SP - e942-e949 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2010-01268-006&site=ehost-live&scope=site linda.lambert@imail.org VL - 124 ID - 1689 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. © 2013 - IOS Press and the authors. All rights reserved. AD - K. Lang, Department of Pediatric and Congenital Cardiothoracic Surgery, Levine Children's Hospital, 1000 Blythe Blvd, Charlotte, NC 28203, United States AU - Lang, K. AU - Champion, E. AU - Cosper, G. AU - Peeler, B. AU - Maxey, T. DB - Embase DO - 10.3233/PIC-13062 IS - 3 KW - heparin warfarin adolescent anticoagulation arterial trunk article bronchitis bronchoscopy case report DiGeorge syndrome extracorporeal oxygenation female follow up human lethality nasal cannula priority journal right ventricle to pulmonary artery conduit treatment outcome truncus vagotomy LA - English M3 - Article N1 - L372345075 2014-02-19 2014-02-25 PY - 2013 SN - 2146-4618 2146-4626 SP - 131-135 ST - A near lethal case of plastic bronchitis following truncal valve replacement T2 - Journal of Pediatric Intensive Care TI - A near lethal case of plastic bronchitis following truncal valve replacement UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L372345075 http://dx.doi.org/10.3233/PIC-13062 VL - 2 ID - 979 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. © 2013 - IOS Press and the authors. © 2012 - IOS Press and the authors. AD - Department of Pediatric and Congenital Cardiothoracic Surgery, Levine Children’s Hospital, Charlotte, NC, United States Department of Pediatric Pulmonology, Levine Children’s Hospital, Charlotte, NC, United States Department of Pediatric General Surgery, Levine Children’s Hospital, Charlotte, NC, United States AU - Lang, K. AU - Champion, E. AU - Cosper, G. AU - Peeler, B. AU - Maxey, T. DB - Scopus DO - 10.3233/PIC-13062 IS - 3 KW - Bronchial casts Congenital heart disease Plastic bronchitis Truncal valve replacement Truncus arteriosus M3 - Article N1 - Export Date: 15 June 2020 PY - 2013 SP - 131-135 ST - A near lethal case of plastic bronchitis following truncal valve replacement T2 - Journal of Pediatric Intensive Care TI - A near lethal case of plastic bronchitis following truncal valve replacement UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85013607711&doi=10.3233%2fPIC-13062&partnerID=40&md5=ffd9fe28c0105f0a0f9c53ff4f6588fc VL - 2 ID - 2076 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 H2O (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 - K. Laohachai, The Heart Centre for Children, The Children's Hospital at Westmead, 212 Hawkesbury Road, Locked Bag 4001, Westmead, NSW, Australia AU - Laohachai, K. AU - Winlaw, D. AU - Selvadurai, H. AU - Gnanappa, G. K. AU - d'Udekem, Y. AU - Celermajer, D. AU - Ayer, J. DB - Embase Medline DO - 10.1161/JAHA.117.005750 IS - 8 KW - Ergoselect 200 Phillips Intera adolescent adult article cardiopulmonary exercise test cardiovascular magnetic resonance clinical article ergometer exercise female Fontan procedure forced expiratory volume forced vital capacity heart ejection fraction heart output human inspiratory capacity lung blood flow lung function male maximal expiratory pressure maximal inspiratory pressure morbidity mortality muscle strength muscle training nuclear magnetic resonance scanner priority journal program evaluation respiratory function therapy effect LA - English M3 - Article N1 - L618551741 2017-10-13 2017-10-18 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 - Journal of the American Heart Association 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L618551741 http://dx.doi.org/10.1161/JAHA.117.005750 VL - 6 ID - 738 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 H2O (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. © 2017 The Authors and Medtronic. AD - From the The Children's Hospital at Westmead, Sydney, NSW, Australia The University of Sydney, 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 AU - Laohachai, K. AU - Winlaw, D. AU - Selvadurai, H. AU - Gnanappa, G. K. AU - d'Udekem, Y. AU - Celermajer, D. AU - Ayer, J. C7 - e005750 DB - Scopus DO - 10.1161/JAHA.117.005750 IS - 8 KW - Exercise Exercise capacity Fontan procedure Inspiratory muscle strength M3 - Article N1 - Cited By :24 Export Date: 15 June 2020 PY - 2017 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 - Journal of the American Heart Association 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85030682292&doi=10.1161%2fJAHA.117.005750&partnerID=40&md5=6d34b1510ab41abd30db5904486b30f2 VL - 6 ID - 1849 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. 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. DB - Embase Medline DO - 10.1016/j.jpeds.2017.01.034 KW - adaptive behavior aortic stenosis aortic valve repair article behavior assessment Behavior Assessment System for Children behavior rating inventory of executive function child cohort analysis cross-sectional study female General Adaptive Composite score heart single ventricle human hypoplastic left heart syndrome length of stay major clinical study male nervous system development outcome assessment palliative therapy Pediatric Quality of Life Inventory priority journal quality of life assessment retrospective study LA - English M3 - Article N1 - L614502501 2017-02-24 2017-05-02 PY - 2017 SN - 1097-6833 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 - Journal of Pediatrics TI - Neurodevelopmental Outcome in Children after Fetal Cardiac Intervention for Aortic Stenosis with Evolving Hypoplastic Left Heart Syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L614502501 http://dx.doi.org/10.1016/j.jpeds.2017.01.034 VL - 184 ID - 757 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 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 - 122435158. Language: English. Entry Date: 20170817. Revision Date: 20190127. Publication Type: journal article AU - Laraja, Kristin AU - Sadhwani, Anjali AU - Tworetzky, Wayne AU - Marshall, Audrey C. AU - Gauvreau, Kimberlee AU - Freud, Lindsay AU - Hass, Cara AU - Dunbar-Masterson, Carolyn AU - Ware, Janice AU - Lafranchi, Terra AU - Wilkins-Haug, Louise AU - Newburger, Jane W. DB - ccm DO - 10.1016/j.jpeds.2017.01.034 DP - EBSCOhost KW - Postoperative Complications -- Epidemiology Aortic Valve Stenosis -- Surgery Aortic Valve Stenosis -- Complications Hypoplastic Left Heart Syndrome -- Complications Prospective Studies Child Risk Factors Pregnancy Infant Female Retrospective Design Balloon Dilatation Heart Surgery -- Methods Child, Preschool Human Clinical Assessment Tools Questionnaires Fetus N1 - research. Journal Subset: Biomedical; Peer Reviewed; USA. Instrumentation: Behavior Assessment System for Children (BASC); Pediatric Quality of Life Inventory (PEDSQL); Behavior Rating Inventory of Executive Function (BRIEF); Adaptive Behavior Assessment System Questionnaire-Second Edition. Grant Information: P30 HD018655/HD/NICHD NIH HHS/United States. NLM UID: 0375410. PMID: NLM28233547. PY - 2017 SN - 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 - Journal of Pediatrics TI - Neurodevelopmental Outcome in Children after Fetal Cardiac Intervention for Aortic Stenosis with Evolving Hypoplastic Left Heart Syndrome UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=122435158&site=ehost-live&scope=site VL - 184 ID - 1549 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. © 2017 Elsevier Inc. AD - Department of Cardiology, Boston Children's Hospital, Boston, MA, United States Department of Pediatrics, Harvard Medical School, Boston, MA, United States Department of Psychiatry, Boston Children's Hospital, Boston, MA, United States Department of Psychiatry, Harvard Medical School, Boston, MA, United States Developmental Medicine Center, Boston Children's Hospital, Boston, MA, United States Department of Obstetrics and Gynecology, Brigham and Women's Hospital, Boston, MA, United States Department of Obstetrics and Gynecology, Harvard Medical School, Boston, MA, United States 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. DB - Scopus DO - 10.1016/j.jpeds.2017.01.034 KW - child development congenital heart disease fetal cardiac intervention neurodevelopmental outcomes M3 - Article N1 - Cited By :14 Export Date: 15 June 2020 PY - 2017 SP - 130-136.e4 ST - Neurodevelopmental Outcome in Children after Fetal Cardiac Intervention for Aortic Stenosis with Evolving Hypoplastic Left Heart Syndrome T2 - Journal of Pediatrics TI - Neurodevelopmental Outcome in Children after Fetal Cardiac Intervention for Aortic Stenosis with Evolving Hypoplastic Left Heart Syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85013157637&doi=10.1016%2fj.jpeds.2017.01.034&partnerID=40&md5=6c2c900be5e0c38d646b41199893bc85 VL - 184 ID - 1863 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. © 2011, Wiley Periodicals, Inc. AD - G.R. Rempel, Faculty of Nursing, University of Alberta and Population Health Investigator, Alberta Heritage Foundation for Medical Research, Edmontony AB, Canada AU - Lee, A. AU - Rempel, G. R. DB - Medline DO - 10.1111/j.1744-6155.2011.00289.x IS - 3 KW - adaptive behavior adult age article Canada caregiver child parent relation cohort analysis female follow up handicapped child heart surgery human hypoplastic left heart syndrome infant long term care male methodology middle aged patient care preschool child psychological aspect risk factor treatment outcome vulnerable population LA - English M3 - Article N1 - L51458525 2011-11-08 PY - 2011 SN - 1539-0136 1744-6155 SP - 179-189 ST - Parenting children with hypoplastic left heart syndrome: Finding a balance T2 - Journal for Specialists in Pediatric Nursing TI - Parenting children with hypoplastic left heart syndrome: Finding a balance UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51458525 http://dx.doi.org/10.1111/j.1744-6155.2011.00289.x VL - 16 ID - 1102 ER - TY - JOUR AB - This study described the role normalization played for parents related to their young children with hypoplastic left heart syndrome. The data source for this secondary analysis was 30 interviews with 16 parents. 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. Nurses can collaborate with parents to determine strategies to restore a sense of normalcy to simultaneously promote child independence and parent well-being. AD - Anra Lee, BScN Honors, RN, is a Staff Nurse, Alberta Children's Hospital, Calgary; and Gwen R. Rempel, PhD, RN, is Assistant Professor, Faculty of Nursing, University of Alberta, and Population Health Investigator, Alberta Heritage Foundation for Medical Research, Edmonton, Alberta, Canada AN - 104676624. Language: English. Entry Date: 20110823. Revision Date: 20150711. Publication Type: Journal Article AU - Lee, Anra AU - Rempel, Gwen R. DB - ccm DO - 10.1111/j.1744-6155.2011.00289.x DP - EBSCOhost IS - 3 KW - Parenting Hypoplastic Left Heart Syndrome Parental Role Family Coping Child, Medically Fragile Parents Secondary Analysis Thematic Analysis Parental Attitudes Disease Susceptibility Grounded Theory Child Development Adult Middle Age Educational Status Employment Status Infant Child, Preschool Chronic Disease Semi-Structured Interview Human Constant Comparative Method Attitude to Illness Attitude to Health Survival Fear Discipline Professional-Family Relations Pediatric Nursing Nursing Practice Descriptive Statistics N1 - research; tables/charts. Journal Subset: Core Nursing; Nursing; Peer Reviewed; USA. Special Interest: Pediatric Care. NLM UID: 101142025. PMID: NLM21702878. PY - 2011 SN - 1539-0136 SP - 179-189 ST - Parenting children with hypoplastic left heart syndrome: Finding a balance T2 - Journal for Specialists in Pediatric Nursing TI - Parenting children with hypoplastic left heart syndrome: Finding a balance UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104676624&site=ehost-live&scope=site VL - 16 ID - 1557 ER - TY - JOUR AB - This study described the role normalization played for parents related to their young children with hypoplastic left heart syndrome. The data source for this secondary analysis was 30 interviews with 16 parents. 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. Nurses can collaborate with parents to determine strategies to restore a sense of normalcy to simultaneously promote child independence and parent well-being. AD - Staff Nurse, Alberta Children's Hospital, Calgary, Alberta, Canada Assistant Professor, Faculty of Nursing, University of Alberta, and Population Health Investigator, Alberta Heritage Foundation for Medical Research, Edmonton, Alberta, Canada AN - 61873537. Language: English. Entry Date: 20110823. Revision Date: 20170203. Publication Type: Article AU - Lee, Anra AU - Rempel, Gwen R. DB - ccm DO - 10.1111/j.1744-6155.2011.00289.x DP - EBSCOhost IS - 3 KW - Parenting Hypoplastic Left Heart Syndrome Parental Role Family Coping Child, Medically Fragile Parents Secondary Analysis Thematic Analysis Parental Attitudes Disease Susceptibility Grounded Theory Child Development Adult Middle Age Educational Status Employment Status Infant Child, Preschool Chronic Disease Semi-Structured Interview Human Constant Comparative Method Attitude to Illness Attitude to Health Survival Fear Discipline Professional-Family Relations Pediatric Nursing Nursing Practice Descriptive Statistics N1 - research; tables/charts. Journal Subset: Core Nursing; Nursing; Peer Reviewed; USA. Special Interest: Pediatric Care. NLM UID: 101142025. PY - 2011 SN - 1539-0136 SP - 179-189 ST - Parenting children with hypoplastic left heart syndrome: Finding a balance T2 - Journal for Specialists in Pediatric Nursing TI - Parenting children with hypoplastic left heart syndrome: Finding a balance UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=61873537&site=ehost-live&scope=site VL - 16 ID - 1558 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. (PsycINFO Database Record (c) 2016 APA, all rights reserved) AD - Rempel, Gwen R. AN - 2011-13491-003 AU - Lee, Anra AU - Rempel, Gwen R. DB - psyh DO - 10.1111/j.1744-6155.2011.00289.x DP - EBSCOhost IS - 3 KW - parenting hypoplastic left heart syndrome parental perception vulnerability well being Adaptation, Psychological Adult Age Factors Alberta Cardiac Surgical Procedures Caregivers Child, Preschool Cohort Studies Continuity of Patient Care Disabled Children Female Follow-Up Studies Humans Infant Long-Term Care Male Middle Aged Parent-Child Relations Risk Factors Treatment Outcome Vulnerable Populations Young Adult Heart Disorders Parental Attitudes Parental Characteristics Parents N1 - Maternal-Child Nursing Journal. Partial author list: First Author & Affiliation: Lee, Anra; Alberta Children’s Hospital, Calgary, AB, Canada. Other Publishers: Blackwell Publishing. Release Date: 20111024. Correction Date: 20120618. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Grant Information: Lee, Anra. Major Descriptor: Heart Disorders; Parental Attitudes; Parental Characteristics; Well Being. Minor Descriptor: Parents. Classification: Cardiovascular Disorders (3295). Population: Human (10); Male (30); Female (40). Location: Canada. 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); Middle Age (40-64 yrs) (360). Methodology: Empirical Study; Interview; Qualitative Study. References Available: Y. Page Count: 11. Issue Publication Date: Jul, 2011. Publication History: Accepted Date: Mar 8, 2011; Revised Date: Mar 8, 2011; First Submitted Date: May 4, 2010. Copyright Statement: Wiley Periodicals, Inc. 2011. Sponsor: Canadian Institutes of Health Research, Canada. Other Details: Summer studentships. Recipients: Lee, Anra Sponsor: Alberta Heritage Foundation for Medical Research, Honor’s Program, Canada. Recipients: No recipient indicated Sponsor: Mazankowski Alberta Heart Institute, Canada. Other Details: TD Clinical Fellowship. Recipients: Rempel, Gwen R. PY - 2011 SN - 1539-0136 1744-6155 SP - 179-189 ST - Parenting children with hypoplastic left heart syndrome: Finding a balance T2 - Journal for Specialists in Pediatric Nursing TI - Parenting children with hypoplastic left heart syndrome: Finding a balance UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2011-13491-003&site=ehost-live&scope=site gwen.rempel@ualberta.ca VL - 16 ID - 1686 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. © 2011, Wiley Periodicals, Inc. AD - Alberta Children's Hospital, Calgary, AB, Canada Faculty of Nursing, University of Alberta and Population Health Investigator, Alberta Heritage Foundation for Medical Research, Edmontony, AB, Canada AU - Lee, A. AU - Rempel, G. R. DB - Scopus DO - 10.1111/j.1744-6155.2011.00289.x IS - 3 KW - Congenital heart disease Hypoplastic left heart syndrome Normalization Parenting Qualitative research Secondary analysis M3 - Article N1 - Cited By :23 Export Date: 15 June 2020 PY - 2011 SP - 179-189 ST - Parenting children with hypoplastic left heart syndrome: Finding a balance T2 - Journal for Specialists in Pediatric Nursing TI - Parenting children with hypoplastic left heart syndrome: Finding a balance UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-79959744334&doi=10.1111%2fj.1744-6155.2011.00289.x&partnerID=40&md5=7013606265f230c849757d952cbd6222 VL - 16 ID - 2125 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 - Pediatric Cardiology, Changhua Christian Hospital, Changhua, Taiwan Cardiovascular Surgery, Coll. Med., Natl. Taiwan Univ. H., 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 - Scopus DO - 10.1016/S0167-5273(97)00325-2 IS - 3 KW - Absent pulmonary valve syndrome Bicuspid pulmonary valve DiGeorge syndrome Double aortic arch Tetralogy of Fallot Tricuspid atresia Ventricular septal defect M3 - Article N1 - Cited By :13 Export Date: 15 June 2020 PY - 1998 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0032574240&doi=10.1016%2fS0167-5273%2897%2900325-2&partnerID=40&md5=84d74343f47809878e3aa12e55da6f65 VL - 63 ID - 2290 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, Univ. of California, Davis Med. Ctr., Sacramento, CA, United States AU - Leung, W. K. AU - Jahr, J. S. AU - Hotz, J. AU - Pollock, M. DB - Scopus DO - 10.1016/S0952-8180(98)00059-2 IS - 5 KW - Anesthetic technique: general Complications: nonmaalignant hyperthermia Glenn procedue Pediatrics M3 - Article N1 - Cited By :9 Export Date: 15 June 2020 PY - 1998 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0032146960&doi=10.1016%2fS0952-8180%2898%2900059-2&partnerID=40&md5=31b92d5aea03b58936848ac5f5133a31 VL - 10 ID - 2287 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 - W. Li, Jane Somerville GUCH Unit and Department of Echocardiography, Royal Brompton Hospital and Imperial College, London University, United Kingdom. AU - Li, W. AU - Sarubbi, B. AU - Sutton, R. AU - Somerville, J. AU - Gibson, D. AU - Henein, M. Y. DB - Medline IS - 3 KW - adolescent adult article chi square distribution congenital malformation Doppler echocardiography echography electrocardiography female Fontan procedure heart atrium flutter heart muscle conduction system heart ventricle human male methodology middle aged palliative therapy pathophysiology prospective study LA - English M3 - Article N1 - L33442336 2001-03-12 PY - 2001 SN - 0894-7317 SP - 186-193 ST - Atrial and ventricular electromechanical function in 1-ventricle hearts: influence of atrial flutter and Fontan procedure T2 - Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography TI - Atrial and ventricular electromechanical function in 1-ventricle hearts: influence of atrial flutter and Fontan procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L33442336 VL - 14 ID - 1327 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 - 138693837. Language: English. Entry Date: 20010601. Revision Date: 20190920. Publication Type: journal article AU - Li, W. AU - Sarubbi, B. AU - Sutton, R. AU - Somerville, J. AU - Gibson, D. AU - Henein, M. Y. DB - ccm DP - EBSCOhost IS - 3 KW - Heart Ventricle -- Abnormalities Atrial Flutter -- Etiology Atrial Flutter Cardiopulmonary Bypass Echocardiography, Doppler -- Methods Chi Square Test Male Palliative Care Heart Ventricle -- Surgery Middle Age Female Prospective Studies Electrocardiography Atrial Flutter -- Physiopathology Heart Conduction System -- Physiopathology Adult Adolescence Human Validation Studies Comparative Studies Evaluation Research Multicenter Studies Clinical Assessment Tools N1 - research. Journal Subset: Allied Health; Biomedical; Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. Instrumentation: Basic Knowledge Assessment Tool (BKAT). NLM UID: 8801388. PMID: NLM11241014. PY - 2001 SN - 0894-7317 SP - 186-193 ST - Atrial and ventricular electromechanical function in 1-ventricle hearts: influence of atrial flutter and Fontan procedure T2 - Journal of the American Society of Echocardiography TI - Atrial and ventricular electromechanical function in 1-ventricle hearts: influence of atrial flutter and Fontan procedure UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=138693837&site=ehost-live&scope=site VL - 14 ID - 1620 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. Condition: 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. Copyright © 2001 by the American Society of Echocardiography. AD - Jane Somerville GUCH Unit, Royal Brompton Hospital, Sydney Street, London, SW3, 6NP, United Kingdom AU - Li, W. AU - Sarubbi, B. AU - Sutton, R. AU - Somerville, J. AU - Gibson, D. AU - Henein, M. Y. DB - Scopus DO - 10.1067/mje.2001.110141 IS - 3 M3 - Article N1 - Cited By :11 Export Date: 15 June 2020 PY - 2001 SP - 186-193 ST - Atrial and ventricular electromechanical function in 1-ventricle hearts: Influence of atrial flutter and fontan procedure T2 - Journal of the American Society of Echocardiography TI - Atrial and ventricular electromechanical function in 1-ventricle hearts: Influence of atrial flutter and fontan procedure UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0035290682&doi=10.1067%2fmje.2001.110141&partnerID=40&md5=4e3fe716755bd1245daa4094bdb027ea VL - 14 ID - 2273 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. © 2008 Lippincott Williams & Wilkins, Inc. AD - S.W. Lichtman, Helen Hayes Hospital, Route 9W, West Haverstraw, NY 10993, United States AU - Lichtman, S. W. AU - Caravano, M. AU - Schneyman, M. AU - Howell, B. AU - King, M. L. DB - Embase Medline DO - 10.1097/01.HCR.0000311509.16226.b8 IS - 1 KW - adult article case report dietary intake echocardiography exercise test female health survey heart rehabilitation heart surgery human hypoplastic left heart syndrome outpatient priority journal tricuspid valve atresia LA - English M3 - Article N1 - L351271745 2008-03-07 PY - 2008 SN - 1932-7501 1932-751X 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 - Journal of Cardiopulmonary Rehabilitation and Prevention TI - Successful outpatient cardiac rehabilitation in an adult patient post-surgical repair for tricuspid valve atresia and hypoplastic right ventricle: A case study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L351271745 http://dx.doi.org/10.1097/01.HCR.0000311509.16226.b8 VL - 28 ID - 1221 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 - J.D. Kugler, Division of Cardiology, Children's Hospital and Medical Center, 8200 Dodge Street, Omaha, NE, United States AU - Lihn, S. L. AU - Kugler, J. D. AU - Peterson, L. E. AU - Lannon, C. M. AU - Pickles, D. AU - Beekman, R. H. DB - Embase Medline DO - 10.1111/chd.12314 IS - 6 KW - article cardiologist congenital heart disease doctor patient relationship health care organization health care quality heart single ventricle human hypoplastic left heart syndrome information processing Internet parent physician priority journal total quality management LA - English M3 - Article N1 - L607158746 2015-12-11 2016-01-07 PY - 2015 SN - 1747-0803 1747-079X SP - 572-580 ST - Transparency in a Pediatric Quality Improvement Collaborative: A Passionate Journey by NPC-QIC Clinicians and Parents T2 - Congenital Heart Disease TI - Transparency in a Pediatric Quality Improvement Collaborative: A Passionate Journey by NPC-QIC Clinicians and Parents UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L607158746 http://dx.doi.org/10.1111/chd.12314 VL - 10 ID - 847 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. © 2015 Wiley Periodicals, Inc. AD - Division of Cardiology, Children's Hospital and Medical Center, Omaha, NE, United States Boston, MA, United States James M. Anderson Center for Health Systems Excellence, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Department of Cardiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States AU - Lihn, S. L. AU - Kugler, J. D. AU - Peterson, L. E. AU - Lannon, C. M. AU - Pickles, D. AU - Beekman, R. H. DB - Scopus DO - 10.1111/chd.12314 IS - 6 KW - Congenital heart disease Hypoplastic left heart syndrome Public reporting Quality improvement collaborative Transparency M3 - Article N1 - Cited By :8 Export Date: 15 June 2020 PY - 2015 SP - 572-580 ST - Transparency in a Pediatric Quality Improvement Collaborative: A Passionate Journey by NPC-QIC Clinicians and Parents T2 - Congenital Heart Disease TI - Transparency in a Pediatric Quality Improvement Collaborative: A Passionate Journey by NPC-QIC Clinicians and Parents UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84952871360&doi=10.1111%2fchd.12314&partnerID=40&md5=fd8f9b6730e503eec88b3ee7e2cda81c VL - 10 ID - 1936 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. © 2019 The American Association for Thoracic Surgery AD - Department of Mechanical and Industrial Engineering, University of Toronto, Toronto, Ontario, Canada Division of Vascular Surgery, Department of Surgery, Peter Munk Cardiac Centre, University Health Network, University of Toronto, Toronto, Ontario, Canada Division of Cardiology, Department of Medicine, Peter Munk Cardiac Centre, University Health Network, University of Toronto, Toronto, Ontario, Canada Division of Cardiovascular Surgery, Department of Surgery, Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada Institute of Biomaterials and Biomedical Engineering, University of Toronto, Toronto, Ontario, 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 - Scopus DO - 10.1016/j.jtcvs.2019.03.008 IS - 5 KW - cavopulmonary assist computational fluid dynamics Fontan circulation hemolysis multilumen cannula M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2019 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85064311723&doi=10.1016%2fj.jtcvs.2019.03.008&partnerID=40&md5=e2f61b12e9de2ab429b0b53ae58e1e66 VL - 158 ID - 1729 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 - D.R. Liptzin, Pediatrics, Children's Hospital Colorado and University of Colorado Denver School of Medicine, 13123 East 16th Ave, Box 395, Aurora, CO, United States 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. DB - Embase Medline DO - 10.1016/j.jpeds.2018.03.050 KW - adolescent adult article bronchitis child female fenestration Fontan procedure human institutional review lung function test major clinical study male medical record review outpatient department physical examination priority journal retrospective study screening test six minute walk test LA - English M3 - Article N1 - L2000727292 2018-05-17 2018-07-19 PY - 2018 SN - 1097-6833 0022-3476 SP - 140-143 ST - Pulmonary Screening in Subjects after the Fontan Procedure T2 - Journal of Pediatrics TI - Pulmonary Screening in Subjects after the Fontan Procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2000727292 http://dx.doi.org/10.1016/j.jpeds.2018.03.050 VL - 199 ID - 671 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 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 AN - 130688886. Language: English. Entry Date: 20190220. Revision Date: 20190517. Publication Type: journal article AU - Liptzin, Deborah R. AU - Di Maria, Michael V. AU - Younoszai, Adel AU - Narkewicz, Michael R. AU - Kelly, Sarah L. AU - Wolfe, Kelly R. AU - Veress, Livia A. DB - ccm DO - 10.1016/j.jpeds.2018.03.050 DP - EBSCOhost KW - Postoperative Care -- Methods Postoperative Complications -- Diagnosis Lung Diseases -- Diagnosis Cardiopulmonary Bypass Male Young Adult Exercise Test Retrospective Design Lung Diseases -- Epidemiology Child, Preschool Female Postoperative Complications -- Epidemiology Adolescence Child Lung Diseases -- Etiology Patient History Taking Incidence Respiratory Function Tests Physical Examination Human N1 - research. Journal Subset: Biomedical; Peer Reviewed; USA. NLM UID: 0375410. PMID: NLM29747936. PY - 2018 SN - 0022-3476 SP - 140-143 ST - Pulmonary Screening in Subjects after the Fontan Procedure T2 - Journal of Pediatrics TI - Pulmonary Screening in Subjects after the Fontan Procedure UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=130688886&site=ehost-live&scope=site VL - 199 ID - 1464 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. © 2018 Elsevier Inc. AD - Pediatric Pulmonary Medicine, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, CO, United States Heart Institute, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, CO, United States Digestive Health Institute, Children's Hospital Colorado and Section of Pediatric Gastroenterology, Hepatology and Nutrition, University of Colorado School of Medicine, Aurora, CO, United States Pediatric Neurology, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, CO, United States 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. DB - Scopus DO - 10.1016/j.jpeds.2018.03.050 KW - altitude congenital executive function heart defects hypoplastic left heart syndrome neuropsychology sleep apnea syndromes sternotomy M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2018 SP - 140-143 ST - Pulmonary Screening in Subjects after the Fontan Procedure T2 - Journal of Pediatrics TI - Pulmonary Screening in Subjects after the Fontan Procedure UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85046762459&doi=10.1016%2fj.jpeds.2018.03.050&partnerID=40&md5=17b8c4a7f483bd57d0af1288dcf54670 VL - 199 ID - 1791 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 - Department of Thoracic and Cardiovascular Surgery, Shanghai Children's Medical Center, Shanghai, China AU - Liu, J. DB - Scopus DO - 10.1053/j.pcsu.2009.01.020 IS - 1 KW - collaboration Congenital heart disease developing country non-profit organization M3 - Article N1 - Cited By :12 Export Date: 15 June 2020 PY - 2009 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-63449135336&doi=10.1053%2fj.pcsu.2009.01.020&partnerID=40&md5=ddf527f74aa191ed348c1d4f8e9ba941 VL - 12 ID - 2178 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. © 2013 Jinlong Liu et al. 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, Macquarie University, 2 Technology Place, Sydney, NSW 2109, Australia ASMeW Lab, Centre for Advanced Biomedical Sciences, TWIns, Waseda University, 2-2 Wakamatsucho, Shinjuku, Tokyo 162-8480, Japan AU - Liu, J. AU - Qian, Y. AU - Sun, Q. AU - Liu, J. AU - Umezu, M. C7 - 131597 DB - Scopus DO - 10.1155/2013/131597 M3 - Article N1 - Cited By :11 Export Date: 15 June 2020 PY - 2013 ST - Use of computational fluid dynamics to estimate hemodynamic effects of respiration on hypoplastic left heart syndrome surgery: Total cavopulmonary connection treatments T2 - The Scientific World Journal TI - Use of computational fluid dynamics to estimate hemodynamic effects of respiration on hypoplastic left heart syndrome surgery: Total cavopulmonary connection treatments UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84893840676&doi=10.1155%2f2013%2f131597&partnerID=40&md5=1a874abd95d0ea88e00710e99c2d2344 VL - 2013 ID - 2030 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. AU - Long, C. C. AU - Hsu, M. C. AU - Bazilevs, Y. AU - Feinstein, J. A. AU - Marsden, A. L. DB - Medline DO - 10.1002/cnm.1485 IS - 5 KW - biological model biomechanics child computer simulation female finite element analysis Fontan procedure congenital heart malformation hemodynamics human male mechanical stress pathology pathophysiology physiology preschool child pressure LA - English M3 - Article N1 - L603230456 2015-03-26 PY - 2012 SN - 2040-7947 SP - 513-527 ST - Fluid-structure interaction simulations of the Fontan procedure using variable wall properties T2 - International journal for numerical methods in biomedical engineering TI - Fluid-structure interaction simulations of the Fontan procedure using variable wall properties UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L603230456 http://dx.doi.org/10.1002/cnm.1485 VL - 28 ID - 1061 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. © 2012 John Wiley & Sons, Ltd. AD - University of California San Diego, 9500 Gilman Drive, Mail Code 0411, La Jolla, CA 92093-0411, United States University of California San Diego, 9500 Gilman Drive, Mail Code 0085, La Jolla, CA 92093-0085, United States Stanford University, 750 Welch Rd., #305, Stanford, CA 94305-5731, United States AU - Long, C. C. AU - Hsu, M. C. AU - Bazilevs, Y. AU - Feinstein, J. A. AU - Marsden, A. L. DB - Scopus DO - 10.1002/cnm.1485 IS - 5 KW - Congenital heart disease Finite element method Fluid-structure interaction Fontan procedure M3 - Article N1 - Cited By :47 Export Date: 15 June 2020 PY - 2012 SP - 513-527 ST - Fluid-structure interaction simulations of the Fontan procedure using variable wall properties T2 - International Journal for Numerical Methods in Biomedical Engineering TI - Fluid-structure interaction simulations of the Fontan procedure using variable wall properties UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84860430688&doi=10.1002%2fcnm.1485&partnerID=40&md5=3803ecb3735c00e306bbe762059c0160 VL - 28 ID - 2096 ER - TY - JOUR AU - Long, S. S. DB - Scopus DO - 10.1016/j.jpeds.2008.07.023 IS - 3 M3 - Article N1 - Export Date: 15 June 2020 PY - 2008 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-49349085755&doi=10.1016%2fj.jpeds.2008.07.023&partnerID=40&md5=62dc70fb1cd70055bead3fcb83c766a4 VL - 153 ID - 2190 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 - 1 Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Canada. AN - 104361872. Language: English. Entry Date: 20130208. Revision Date: 20150711. Publication Type: Journal Article AU - Longmuir, P. E. AU - Banks, L. AU - McCrindle, B. W. DB - ccm DO - 10.1017/S104795111100206X DP - EBSCOhost IS - 4 KW - Child Development Developmental Disabilities -- Physiopathology Cardiopulmonary Bypass Heart Defects, Congenital -- Surgery Motor Skills Child Child, Preschool Cross Sectional Studies Female Human Locomotion Male Motor Activity Life Style, Sedentary N1 - research. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Special Interest: Pediatric Care. NLM UID: 9200019. PMID: NLM22269013. PY - 2012 SN - 1047-9511 SP - 443-450 ST - Cross-sectional study of motor development among children after the Fontan procedure T2 - Cardiology in the Young TI - Cross-sectional study of motor development among children after the Fontan procedure UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104361872&site=ehost-live&scope=site VL - 22 ID - 1543 ER - TY - JOUR AD - Hospital for Sick Children and Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada. AN - 105508136. Language: English. Entry Date: 20090529. Revision Date: 20150711. Publication Type: Journal Article AU - Longmuir, P. E. AU - McCrindle, B. W. DB - ccm DO - 10.1016/j.ahj.2009.02.014 DP - EBSCOhost IS - 5 KW - Cardiopulmonary Bypass -- Adverse Effects Dissent and Disputes Heart Defects, Congenital -- Physiopathology Medical Records Motor Activity -- Physiology Movement Disorders -- Epidemiology Child Cross Sectional Studies Female Heart Defects, Congenital -- Surgery Male Movement Disorders -- Diagnosis Movement Disorders -- Etiology Ontario Physician-Patient Relations Postoperative Complications Prevalence Prospective Studies Questionnaires Human N1 - research. Journal Subset: Biomedical; USA. NLM UID: 0370465. PMID: NLM19376311. PY - 2009 SN - 0002-8703 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105508136&site=ehost-live&scope=site VL - 157 ID - 1610 ER - TY - JOUR AD - Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, Canada; Department of Physical Therapy, Faculty of Medicine, University of Toronto, Toronto, Canada. AN - 104817635. Language: English. Entry Date: 20110415. Revision Date: 20150711. Publication Type: Journal Article AU - Longmuir, P. E. AU - Russell, J. L. AU - Corey, M. AU - Faulkner, G. AU - McCrindle, B. W. DB - ccm DO - 10.1016/j.ahj.2010.11.019 DP - EBSCOhost IS - 2 KW - Exercise Cardiopulmonary Bypass Motor Activity Child Child, Preschool Cross Sectional Studies Female Human Male N1 - research. Journal Subset: Biomedical; USA. NLM UID: 0370465. PMID: NLM21315227. PY - 2011 SN - 0002-8703 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104817635&site=ehost-live&scope=site VL - 161 ID - 1590 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. © 2014 John Wiley & Sons, Ltd. AD - Slone Epidemiology Center, Boston University, Boston, MA, United States AU - Louik, C. AU - Kerr, S. AU - Mitchell, A. A. DB - Scopus DO - 10.1002/pds.3661 IS - 10 KW - Antidepressants Bupropion Cardiac malformations Pharmacoepidemiology M3 - Article N1 - Cited By :24 Export Date: 15 June 2020 PY - 2014 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84911807135&doi=10.1002%2fpds.3661&partnerID=40&md5=f16a2e03a7b8cfbce98ddc5d673739fc VL - 23 ID - 1983 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 - C. Ronai, School of Medicine, Oregon Health and Science University, Portland, OR, United States AU - Lowenstein, S. AU - Macauley, R. AU - Perko, K. AU - Ronai, C. DB - Embase Medline DO - 10.1017/S1047951120000128 KW - child controlled study family stress health care quality heart single ventricle human hypoplastic left heart syndrome palliative therapy patient education patient referral perinatal period physician prenatal diagnosis prenatal period registered nurse review LA - English M3 - Article in Press N1 - L631262498 2020-03-25 PY - 2020 SN - 1467-1107 1047-9511 ST - Provider perspective on the role of palliative care in hypoplastic left heart syndrome T2 - Cardiology in the Young TI - Provider perspective on the role of palliative care in hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L631262498 http://dx.doi.org/10.1017/S1047951120000128 ID - 579 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. © 1967-2013 Harvey Whitney Books Co. All rights reserved. AD - N. L. Lubcke, Department of Pharmacy, American Family Children's Hospital, University of Wisconsin Hospital and Clinics, Madison, United States AU - Lubcke, N. L. AU - Nussbaum, V. M. AU - Schroth, M. C2 - Genentech DB - Embase Medline DO - 10.1345/aph.1R690 IS - 3 KW - azithromycin ceftriaxone dornase alfa fibrin oxygen salbutamol tissue plasminogen activator adolescent article atelectasis bronchitis bronchoscopy case report cyanosis emergency ward Fontan procedure hemidiaphragm human inflammatory infiltrate loading drug dose lung extravascular fluid male mediastinum disease nebulization oxygen saturation oxygen therapy plastic bronchitis priority journal respiratory distress spirometry thorax pain thorax radiography treatment outcome LA - English M3 - Article N1 - L368561383 2013-03-28 2013-04-02 http://www.theannals.com/content/47/3/e13.full.pdf+html PY - 2013 SN - 1060-0280 SP - e13 ST - Use of aerosolized tissue plasminogen activator in the treatment of plastic bronchitis T2 - Annals of Pharmacotherapy TI - Use of aerosolized tissue plasminogen activator in the treatment of plastic bronchitis UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L368561383 http://dx.doi.org/10.1345/aph.1R690 VL - 47 ID - 1012 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. AN - 107993333. Language: English. Entry Date: 20130906. Revision Date: 20191209. Publication Type: journal article AU - Lubcke, Nicole L. AU - Nussbaum, Vicki M. AU - Schroth, Mary DB - ccm DO - 10.1345/aph.1R690 DP - EBSCOhost IS - 3 KW - Bronchitis -- Drug Therapy Tissue Plasminogen Activator -- Administration and Dosage Administration, Inhalation Adolescence Aerosols Bronchitis -- Radiography Human Male N1 - case study; research. Journal Subset: Biomedical; Blind Peer Reviewed; Double Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. NLM UID: 9203131. PMID: NLM23424228. PY - 2013 SN - 1060-0280 SP - e13-e13 ST - Use of aerosolized tissue plasminogen activator in the treatment of plastic bronchitis T2 - Annals of Pharmacotherapy TI - Use of aerosolized tissue plasminogen activator in the treatment of plastic bronchitis UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=107993333&site=ehost-live&scope=site VL - 47 ID - 1631 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. © 1967-2013 Harvey Whitney Books Co. All rights reserved. AD - Department of Pharmacy, American Family Children's Hospital, University of Wisconsin Hospital and Clinics, Madison, United States University of Wisconsin, School of Medicine and Public Health, United States AU - Lubcke, N. L. AU - Nussbaum, V. M. AU - Schroth, M. DB - Scopus DO - 10.1345/aph.1R690 IS - 3 M3 - Article N1 - Cited By :8 Export Date: 15 June 2020 PY - 2013 ST - Use of aerosolized tissue plasminogen activator in the treatment of plastic bronchitis T2 - Annals of Pharmacotherapy TI - Use of aerosolized tissue plasminogen activator in the treatment of plastic bronchitis UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84875156198&doi=10.1345%2faph.1R690&partnerID=40&md5=2a68c520c789cff673e423a6c05bba88 VL - 47 ID - 2060 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 - 135751609. Language: English. Entry Date: 20200220. Revision Date: 20200220. Publication Type: journal article AU - Luo, Shuhua AU - Honjo, Osami DB - ccm DO - 10.1097/HCO.0000000000000603 DP - EBSCOhost IS - 2 KW - Heart Defects, Congenital -- Surgery Cardiopulmonary Bypass -- Mortality Time Factors Adult Quality of Life Pulmonary Circulation Adolescence Ferrans and Powers Quality of Life Index N1 - review. Journal Subset: Biomedical; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. Instrumentation: Longitudinal Interval Follow-Up Evaluation (LIFE); Ferrans and Powers Quality of Life Index. NLM UID: 8608087. PMID: NLM30575650. PY - 2019 SN - 0268-4705 SP - 156-163 ST - Late deaths after Fontan procedure: the next frontier T2 - Current Opinion in Cardiology TI - Late deaths after Fontan procedure: the next frontier UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=135751609&site=ehost-live&scope=site VL - 34 ID - 1510 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. © 2014 The Society of Thoracic Surgeons. AD - Department of Congenital Heart Disease, Marie Lannelongue Hospital/M3C, Paris-Sud University, Le Plessis-Robinson; and Faculty of Medicine, Paris-Sud University, Orsay, France 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. DB - Scopus DO - 10.1016/j.athoracsur.2014.07.043 IS - 6 M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2014 SP - 2181-2186 ST - The modified Fontan procedure with use of extracardiac conduit in adults: Analysis of 32 consecutive patients T2 - Annals of Thoracic Surgery TI - The modified Fontan procedure with use of extracardiac conduit in adults: Analysis of 32 consecutive patients UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84919464503&doi=10.1016%2fj.athoracsur.2014.07.043&partnerID=40&md5=f0e9cdc8750858bbedc75d75f1d17818 VL - 98 ID - 2021 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 - I. D. Lytrivi, Dept. of Pediatric Cardiology, Mitera Children's Hospital, 6, Erythrou Stavrou St, 151 23 Maroussi Athens, Greece AU - Lytrivi, I. D. AU - Sfyridis, P. G. AU - Papagiannis, J. AU - Kirvassilis, G. AU - Zavaropoulos, P. AU - Sarris, G. E. DB - Embase Medline IS - 2 KW - adolescent adult age article body height body weight cavopulmonary connection child clinical article controlled study developmental disorder diastolic blood pressure echocardiography electrocardiogram female follow up Fontan procedure functional status human learning disorder male medical record review physical development preschool child questionnaire school child systolic blood pressure tissue Doppler imaging LA - English M3 - Article N1 - L361655532 2011-05-03 2011-05-09 http://hellenicjcardiol.com/archive/full_text/2011/2/2011_2_118.pdf PY - 2011 SN - 1011-7970 SP - 118-122 ST - Impact of age at fontan completion on functional status at mid-term follow up T2 - Hellenic Journal of Cardiology TI - Impact of age at fontan completion on functional status at mid-term follow up UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L361655532 VL - 52 ID - 1110 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. © 2011 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies. AD - K. Madden, Department of Pediatrics and Anesthesiology Critical Care Medicine, Children's Hospital Los Angeles, Los Angeles, CA, United States AU - Madden, K. AU - Turkel, S. AU - Jacobson, J. AU - Epstein, D. AU - Moromisato, D. Y. DB - Embase Medline DO - 10.1097/PCC.0b013e31820ac2bf IS - 6 KW - benzodiazepine derivative codeine ibuprofen lorazepam morphine olanzapine opiate derivative paracetamol article artificial ventilation bedtime dosage case report congestive heart failure delirium extracorporeal oxygenation heart surgery hospitalization human hypoplastic left heart syndrome infant insomnia intensive care unit male priority journal restlessness treatment response LA - English M3 - Article N1 - L51283310 2011-02-22 2011-11-24 PY - 2011 SN - 1529-7535 1947-3893 SP - e413-e415 ST - Recurrent delirium after surgery for congenital heart disease in an infant T2 - Pediatric Critical Care Medicine TI - Recurrent delirium after surgery for congenital heart disease in an infant UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51283310 http://dx.doi.org/10.1097/PCC.0b013e31820ac2bf VL - 12 ID - 1087 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 - From Departments of Pediatrics and Anesthesiology Critical Care Medicine (KM, DE, DM) and Psychiatry (ST, JJ), Children's Hospital Los Angeles, Los Angeles, CA. AN - 108205717. Language: English. Entry Date: 20120203. Revision Date: 20150712. Publication Type: Journal Article AU - Madden, Kevin AU - Turkel, Susan AU - Jacobson, Julienne AU - Epstein, David AU - Moromisato, David Y. DB - ccm DP - EBSCOhost IS - 6 KW - Delirium -- Epidemiology -- In Infancy and Childhood Heart Defects, Congenital -- Surgery -- In Infancy and Childhood Postoperative Complications -- Epidemiology -- In Infancy and Childhood Delirium -- Drug Therapy Infant Male Olanzapine -- Therapeutic Use N1 - case study. Journal Subset: Biomedical; Peer Reviewed; USA. Special Interest: Critical Care; Pediatric Care; Perioperative Care. NLM UID: 100954653. PMID: NLM21336229. PY - 2011 SN - 1529-7535 SP - e413-5 ST - Recurrent delirium after surgery for congenital heart disease in an infant T2 - Pediatric Critical Care Medicine TI - Recurrent delirium after surgery for congenital heart disease in an infant UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=108205717&site=ehost-live&scope=site VL - 12 ID - 1639 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 - H.S. Magdo, University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, 1540 East Hospital Dr, Ann Arbor, MI, United States 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. DB - Embase Medline DO - 10.1016/j.jpeds.2015.04.061 IS - 2 KW - biological marker immunoglobulin A immunoglobulin G immunoglobulin G1 immunoglobulin G2 immunoglobulin G3 immunoglobulin M article autoimmune disease B lymphocyte case control study CD4 lymphocyte count CD8+ T lymphocyte child clinical article controlled study demography female Fontan procedure human immunoglobulin blood level immunological procedures interview intestine lymphangiectasia lymphocyte subpopulation lymphocytopenia male measles mumps natural killer cell opportunistic infection postoperative period preschool child priority journal prospective study protein losing gastroenteropathy rubella vaccination LA - English M3 - Article N1 - L604469341 2015-05-27 2015-08-03 PY - 2015 SN - 1097-6833 0022-3476 SP - 331-337 ST - Immune Abnormalities in Fontan Protein-Losing Enteropathy: A Case-Control Study T2 - Journal of Pediatrics TI - Immune Abnormalities in Fontan Protein-Losing Enteropathy: A Case-Control Study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L604469341 http://dx.doi.org/10.1016/j.jpeds.2015.04.061 VL - 167 ID - 887 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 depressedCD4 T cell counts (median 58 cells/mL) 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. (PsycINFO Database Record (c) 2016 APA, all rights reserved) AD - Magdo, H. Sonali, University of Michigan, Congenital Heart Center, C.S. Mott Children’s Hospital, 1540 East Hospital Dr., Ann Arbor, MI, US, 48109 AN - 2015-35580-075 AU - Magdo, H. Sonali AU - Stillwell, Terri L. AU - Greenhawt, Matthew J. AU - Stringer, Kathleen A. AU - Yu, Sunkyung AU - Fifer, Carlen G. AU - Russell, Mark W. AU - Schumacher, Kurt R. DB - psyh DO - 10.1016/j.jpeds.2015.04.061 DP - EBSCOhost IS - 2 KW - immune abnormalities protein-losing enteropathy immunologic characteristics lymphocytes CD4 Lymphocyte Count CD4-Positive T-Lymphocytes CD8-Positive T-Lymphocytes Case-Control Studies Child Child, Preschool Female Fontan Procedure Heart Defects, Congenital Humans Immunoglobulin Isotypes Infant Lymphopenia Male Prospective Studies Protein-Losing Enteropathies Immune System Immunologic Disorders Proteins N1 - University of Michigan, Congenital Heart Center, C.S. Mott Children’s Hospital, Ann Arbor, MI, US. Release Date: 20150928. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Grant Information: Magdo, H. Sonali. Major Descriptor: Immune System; Immunologic Disorders; Lymphocytes; Proteins. Classification: Immunological Disorders (3291). Population: Human (10); Male (30); Female (40). Age Group: Adulthood (18 yrs & older) (300). Tests & Measures: Patient Interview. Methodology: Empirical Study; Longitudinal Study; Prospective Study; Interview; Quantitative Study. References Available: Y. Page Count: 7. Issue Publication Date: Aug, 2015. Publication History: Accepted Date: Apr 22, 2015; Revised Date: Mar 10, 2015; First Submitted Date: Nov 24, 2014. Copyright Statement: All rights reserved. Elsevier Inc. 2015. Sponsor: University of Michigan, Congenital Heart Center, US. Grant: G012107. Recipients: Magdo, H. Sonali PY - 2015 SN - 0022-3476 SP - 331-337 ST - Immune abnormalities in fontan protein-losing enteropathy: A case-control study T2 - The Journal of Pediatrics TI - Immune abnormalities in fontan protein-losing enteropathy: A case-control study UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2015-35580-075&site=ehost-live&scope=site ORCID: 0000-0003-0238-7774 ORCID: 0000-0002-2365-9372 hmagdo@med.umich.edu VL - 167 ID - 1677 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. © 2015 Elsevier Inc. AD - University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, 1540 East Hospital Dr, Ann Arbor, MI 48109, United States University of Michigan Pediatric Infectious Disease, Ann Arbor, MI, United States University of Michigan Allergy and Immunology, Ann Arbor, MI, United States University of Michigan College of Pharmacy, Ann Arbor, MI, United States University of Michigan, Michigan Congenital Heart Outcomes Research and Discovery Ann ArborMI, United States 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. DB - Scopus DO - 10.1016/j.jpeds.2015.04.061 IS - 2 M3 - Article N1 - Cited By :19 Export Date: 15 June 2020 PY - 2015 SP - 331-337 ST - Immune Abnormalities in Fontan Protein-Losing Enteropathy: A Case-Control Study T2 - Journal of Pediatrics TI - Immune Abnormalities in Fontan Protein-Losing Enteropathy: A Case-Control Study UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84937728913&doi=10.1016%2fj.jpeds.2015.04.061&partnerID=40&md5=9350a5c1088dd8d74c1e9e969602be99 VL - 167 ID - 1942 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 - G. Wemovsky, Cardiac Intensive Care Unit, Division of Cardiology, Children's Hospital of Philadelphia, 34th and Civic Center Blvd, Philadelphia, PA, United States AU - Mahle, W. T. AU - Clancy, R. R. AU - Moss, E. M. AU - Gerdes, M. AU - Jobes, D. R. AU - Wernovsky, G. DB - Embase Medline DO - 10.1542/peds.105.5.1082 IS - 5 KW - academic achievement adolescent article attention deficit disorder behavior disorder cognitive defect cognitive development female health status human hypoplastic left heart syndrome intelligence quotient language development lifestyle major clinical study male neurologic disease priority journal quality of life school child seizure LA - English M3 - Article N1 - L30243787 2000-05-17 PY - 2000 SN - 0031-4005 SP - 1082-1089 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L30243787 http://dx.doi.org/10.1542/peds.105.5.1082 VL - 105 ID - 1337 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 AN - 107117558. Language: English. Entry Date: 20000701. Revision Date: 20150711. Publication Type: Journal Article AU - Mahle, W. T. AU - Clancy, R. R. AU - Moss, E. M. AU - Gerdes, M. AU - Jobes, D. R. AU - Wernovsky, G. DB - ccm DP - EBSCOhost IS - 5 KW - Hypoplastic Left Heart Syndrome Outcomes (Health Care) Cross Sectional Studies Convenience Sample Questionnaires Mail Intelligence Tests Child Behavior Checklist Data Analysis Software Data Analysis, Statistical Child Male Female Human N1 - research; tables/charts. Journal Subset: Biomedical; Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. Instrumentation: Developmental Test of Visual Motor Integration (VMI); Wechsler Intelligence Scale for Children (WISC); Child Behavior Checklist (CBCL) (Achenbach and Edelbrock); Clinical Evaluation of Language Fundamentals-Revised (CELF-R); Woodcock-Johnson Psychoeducational Battery (WJPB). NLM UID: 0376422. PMID: NLM10790466. PY - 2000 SN - 0031-4005 SP - 1082-1089 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=107117558&site=ehost-live&scope=site VL - 105 ID - 1592 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, PA, United States Division of Neurology, Children's Hospital of Philadelphia, Philadelphia, PA, United States Division of Neonatology, Children's Hospital of Philadelphia, Philadelphia, PA, United States Department of Pediatrics, Univ. of Pennsylvania Sch. of Med., Philadelphia, PA, United States Department of Neurology, Univ. of Pennsylvania Sch. of Med., Philadelphia, PA, United States Department of Psychology, Children's Seashore House, Philadelphia, PA, United States Dept. of Anesth. at the Hospital, University of Pennsylvania, Philadelphia, PA, United States Cardiac Intensive Care Unit, Division of Cardiology, Children's Hospital of Philadelphia, 34th and Civic Center Blvd, Philadelphia, PA, United States AU - Mahle, W. T. AU - Clancy, R. R. AU - Moss, E. M. AU - Gerdes, M. AU - Jobes, D. R. AU - Wernovsky, G. DB - Scopus DO - 10.1542/peds.105.5.1082 IS - 5 KW - Hypoplastic left heart syndrome Neurodevelopmental outcome Quality of life M3 - Article N1 - Cited By :335 Export Date: 15 June 2020 PY - 2000 SP - 1082-1089 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0034099787&doi=10.1542%2fpeds.105.5.1082&partnerID=40&md5=c50e3e54cc929216538d3fab3c27828f VL - 105 ID - 2277 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. © 2012 Springer Science+Business Media, LLC. AD - W.T. Mahle, Children's Healthcare of Atlanta, Emory University School of Medicine, 1405 Clifton Road NE, Atlanta, GA 30322, United States 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. DB - Embase Medline DO - 10.1007/s00246-012-0450-1 IS - 2 KW - article child cognition cognitive defect female genetic disorder human infant intensive care unit low birth weight major clinical study male Norwood procedure patient monitoring postoperative period preschool child risk factor LA - English M3 - Article N1 - L52147234 2012-08-07 2013-07-11 PY - 2013 SN - 0172-0643 1432-1971 SP - 327-333 ST - A predictive model for neurodevelopmental outcome after the norwood procedure T2 - Pediatric Cardiology TI - A predictive model for neurodevelopmental outcome after the norwood procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52147234 http://dx.doi.org/10.1007/s00246-012-0450-1 VL - 34 ID - 1022 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, Emory University School of Medicine, 1405 Clifton Rd, NE, Atlanta, GA 30322-1062; mahlew@kidsheart.com AN - 106428243. Language: English. Entry Date: 20060421. Revision Date: 20150711. Publication Type: Journal Article 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. DB - ccm DP - EBSCOhost IS - 1 KW - Developmental Disabilities -- Diagnosis Hypoplastic Left Heart Syndrome -- Surgery Neuropsychological Tests Adolescence Chi Square Test Child Cross Sectional Studies Developmental Disabilities -- Etiology Female Fisher's Exact Test Funding Source Heart Surgery -- Adverse Effects Heart Transplantation -- Adverse Effects Male Multiple Linear Regression Palliative Care Psychometrics T-Tests Human N1 - research; tables/charts. Journal Subset: Biomedical; Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. Instrumentation: Clinical Evaluation of Language Fundamentals (CELF) (Semel et al); Behavior Assessment System for Children (BASC); Beery-Buktenica Developmental Test of Visual-Motor Integration; Behavior Rating Inventory of Executive Function (BRIEF); Wechsler Abbreviated Scale of Intelligence; Wechsler Individual Achievement Test. Grant Information: Doris Duke Charitable Foundation and the Agency for Healthcare Research and Quality. NLM UID: 0376422. PMID: NLM16361221. 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106428243&site=ehost-live&scope=site VL - 117 ID - 1654 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. © 2004 Elsevier Inc. All rights reserved. AD - W.T. Mahle, Children's Healthcare, Atlanta Pediatric Cardiology, Emory University School of Medicine, 1405 Clifton Road NE, Atlanta, GA 30322-1062, United States AU - Mahle, W. T. AU - Wernovsky, G. DB - Embase Medline DO - 10.1053/j.pcsu.2004.02.019 KW - adolescent article brain perfusion cognition heart hemodynamics heart surgery heart transplantation heredity human hypoplastic left heart syndrome long term care motoneuron neurologic examination patient monitoring postoperative care risk factor school child social psychology surgical technique survival treatment outcome LA - English M3 - Article N1 - L38678723 2004-06-08 PY - 2004 SN - 1092-9126 SP - 39-47 ST - Neurodevelopmental outcomes in hypoplastic left heart syndrome T2 - Pediatric Cardiac Surgery Annual TI - Neurodevelopmental outcomes in hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L38678723 http://dx.doi.org/10.1053/j.pcsu.2004.02.019 VL - 7 ID - 1293 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. © 2004 Elsevier Inc. All rights reserved. AD - Department of Pediatrics, Children's Healthcare of Atlanta, Emory University School of Medicine, 1405 Clifton Road NE, Atlanta, GA 30322-1062, United States Children's Hospital of Philadelphia, Univ. of Pennsylvania School of Med., Philadelphia, PA, United States AU - Mahle, W. T. AU - Wernovsky, G. DB - Scopus DO - 10.1053/j.pcsu.2004.02.019 IS - 1 KW - Hypoplastic left heart syndrome Neurologic Outcomes M3 - Article N1 - Cited By :67 Export Date: 15 June 2020 PY - 2004 SP - 39-47 ST - Neurodevelopmental outcomes in hypoplastic left heart syndrome T2 - Pediatric Cardiac Surgery Annual TI - Neurodevelopmental outcomes in hypoplastic left heart syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-2542422017&doi=10.1053%2fj.pcsu.2004.02.019&partnerID=40&md5=1ef53f82b075c0688a2860c68c1dde98 VL - 7 ID - 2255 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 unloading was 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 (V̇O2(max)) 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 V̇O2(max) 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 - S.M. Paridon, Children's Hospital of Philadelphia, 34th and Civic Center Boulevard, Philadelphia, PA 19104, United States AU - Mahle, W. T. AU - Wernovsky, G. AU - Bridges, N. D. AU - Linton, A. B. AU - Paridon, S. M. DB - Embase Medline DO - 10.1016/S0735-1097(99)00392-7 IS - 5 KW - aerobic capacity article clinical article exercise Fontan procedure forced expiratory volume heart single ventricle heart stroke volume human human cell human tissue infant oxygen consumption preschool child priority journal school child surgical technique LA - English M3 - Article N1 - L29528682 1999-11-23 PY - 1999 SN - 0735-1097 SP - 1637-1643 ST - Impact of early ventricular unloading on exercise performance in preadolescents with single ventricle Fontan physiology T2 - Journal of the American College of Cardiology TI - Impact of early ventricular unloading on exercise performance in preadolescents with single ventricle Fontan physiology UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L29528682 http://dx.doi.org/10.1016/S0735-1097(99)00392-7 VL - 34 ID - 1340 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 unloading was 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 (V̇O2(max)) 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 V̇O2(max) 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 - Children's Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania, Philadelphia, PA, United States Children's Hospital of Philadelphia, 34th and Civic Center Boulevard, Philadelphia, PA 19104, United States AU - Mahle, W. T. AU - Wernovsky, G. AU - Bridges, N. D. AU - Linton, A. B. AU - Paridon, S. M. DB - Scopus DO - 10.1016/S0735-1097(99)00392-7 IS - 5 M3 - Article N1 - Cited By :73 Export Date: 15 June 2020 PY - 1999 SP - 1637-1643 ST - Impact of early ventricular unloading on exercise performance in preadolescents with single ventricle Fontan physiology T2 - Journal of the American College of Cardiology TI - Impact of early ventricular unloading on exercise performance in preadolescents with single ventricle Fontan physiology UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0033230603&doi=10.1016%2fS0735-1097%2899%2900392-7&partnerID=40&md5=905957d1f0cd2220e3f0eb64e6bc713c VL - 34 ID - 2281 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. © 2007 Springer Science+Business Media, LLC. AD - Department of Pediatrics, Indiana University School of Medicine, Riley Research, 702, Barnhill Drive, Indianapolis, IN 46202, United States AU - Maiers, J. AU - Hurwitz, R. DB - Scopus DO - 10.1007/s00246-007-9038-6 IS - 1 M3 - Article N1 - Cited By :11 Export Date: 15 June 2020 PY - 2008 SP - 19-23 ST - Identification of coronary artery disease in the pediatric cardiac transplant patient T2 - Pediatric Cardiology TI - Identification of coronary artery disease in the pediatric cardiac transplant patient UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-38349101836&doi=10.1007%2fs00246-007-9038-6&partnerID=40&md5=a47ce5ce330a2849e2602f5d4d94f4a2 VL - 29 ID - 2201 ER - TY - JOUR AB - OBJECTIVES: We assessed the operative and late mortality and the present clinical status of 216 patients with tricuspid atresia who had a nonfenestrated Fontan procedure performed at the Mayo Clinic in the 25-year period 1973 to 1998. BACKGROUND: The Fontan operation eliminates the systemic hypoxemia and ventricular volume overload characteristic of prior forms of palliation. However, it originally did so at the cost of systemic venous and right atrial hypertension, and the long-term effects of this "price" were unknown when the procedure was initially proposed. METHODS: We reviewed the clinical records of the 216 patients retrospectively. These were arbitrarily grouped into early (1973 through 1980), middle (1981 through 1987) and late (1988 through 1997) surgical eras. Patient outcome was also analyzed according to age at surgery. Operative and late mortality rates were determined and present clinical status was ascertained in 167 of 171 surviving patients. RESULTS: Overall survival was 79%. Operative mortality steadily declined and was 2% (one of 58 patients) during the most recent decade. Late survival also continues to improve. Age at operation had no effect on operative mortality, and late mortality was significantly increased only in patients who were operated on at age 18 years or older. Eighty-nine percent of surviving patients are currently in New York Heart Association class I or II. CONCLUSIONS: The initial 25-year experience with the nonfenestrated Fontan procedure for tricuspid atresia has been gratifying, with most survivors now leading lives of good quality into adulthood. These results justify continued application of this procedure for children born with tricuspid atresia. © 2001 by the American College of Cardiology. AD - Section of Pediatric Cardiology, Mayo Clinic, Rochester, MN, United States Division of Cardiovascular Surgery, Mayo Clinic, Rochester, MN, United States Mayo Clinic, 200 First Street SW, Rochester, MN 55905, United States AU - Mair, D. D. AU - Puga, F. J. AU - Danielson, G. K. DB - Scopus DO - 10.1016/S0735-1097(00)01164-5 IS - 3 M3 - Article N1 - Cited By :119 Export Date: 15 June 2020 PY - 2001 SP - 933-939 ST - The Fontan procedure for tricuspid atresia: Early and late results of a 25-year experience with 216 patients T2 - Journal of the American College of Cardiology TI - The Fontan procedure for tricuspid atresia: Early and late results of a 25-year experience with 216 patients UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0035281528&doi=10.1016%2fS0735-1097%2800%2901164-5&partnerID=40&md5=350406396c6bfbc186bd6d9a40df5755 VL - 37 ID - 2271 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. © 2019 Wiley Periodicals, Inc. AD - Interventional Radiology, University Hospitals Leuven, Leuven, Belgium Department of Paediatric and Congenital Cardiology, University Hospitals Leuven, Leuven, Belgium Paediatric Cardiology, University Hospital Maastricht, Maastricht, Netherlands Paediatric Cardiology MC3, Necker Hospital for Sick Children, Paris, France Paediatric Cardiology, University of the Free State, Bloemfontein, South Africa 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 - Scopus DO - 10.1002/ccd.28501 IS - 7 KW - congenital heart disease lymphangiography lymphatic embolization n-butyl cyanoacrylate plastic bronchitis protein losing enteropathy M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2019 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85074566148&doi=10.1002%2fccd.28501&partnerID=40&md5=09ac251215306e8f84a84841f8c01e6e VL - 94 ID - 1721 ER - TY - JOUR AB - Background: Studies have suggested that patients who have undergone the Fontan procedure experience lower functional health status and diminished exercise capacity compared with other children. Objectives: To compare the functional health status of Fontan patients with and without siblings, assess whether there are any differences between Fontan patients and their siblings, and determine associated factors. Methods: A cross-sectional, single-centre, observational study was performed on Fontan patients 10 to 20 years of age, and their sibling closest in age, followed in a tertiary pediatric hospital. Functional health status was measured by the Child Health Questionnaire Child Form and the Pediatric Quality of Life, Inventory. Results: A total of 68 patients and 38 siblings were enrolled. Patients with siblings scored significantly lower on numerous domains of physical functional status than those without siblings. Compared with their matched siblings, Fontan patients reported significantly lower scores in all domains of the Pediatric Quality of Life Inventory and on physical (but not psychosocial) domains of the Child Health Questionnaire Child Form. Factors associated with increased patient-sibling differences included younger patient age, female sex, intracardiac lateral tunnel Fontan connection and lower ejection fraction at the time of study enrollment. Conclusions: Adolescents with Fontan physiology reported a lower functional health status in physical domains than their siblings, but had similar status in psychosocial domains. Having a sibling was associated with lower reported functional health status, suggesting an important effect of self-perceived physical limitations over true limitations. © 2009 Pulsus Group Inc. All rights reserved. AD - Division of Cardiology, Labatt Family Heart Centre, The Hospital for Sick Children, 555 University Avenue, Toronto, ON M5G 1X8, Canada Department of Pediatrics, University of Toronto, The Hospital for Sick Children, 555 University Avenue, Toronto, ON M5G 1X8, Canada Division of Cardiovascular Surgery, Department of Surgery, The Hospital for Sick Children, 555 University Avenue, Toronto, ON M5G 1X8, Canada AU - Manlhiot, C. AU - Knezevich, S. AU - Radojewski, E. AU - Cullen-Dean, G. AU - Williams, W. G. AU - McCrindle, B. W. DB - Scopus IS - 9 KW - Congenital heart disease Fontan procedure Functional health status Quality of life Single ventricle M3 - Article N1 - Cited By :33 Export Date: 15 June 2020 PY - 2009 SP - S294-S300 ST - Functional health status of adolescents after the Fontan procedure - Comparison with their siblings T2 - Canadian Journal of Cardiology TI - Functional health status of adolescents after the Fontan procedure - Comparison with their siblings UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-70349561833&partnerID=40&md5=790472c726031d8cd756731a4517fbb0 VL - 25 ID - 2184 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. Copyright 2018 Mantakaki et al. AD - Surgical Division, Teleflex Incorporated, Bristol, United Kingdom Department of Anatomical Sciences, University of Medicine and Health Sciences, Basseterre, Saint Kitts and Nevis Department of Physiology, Faculty of Medicine, Justus Liebig University, Giessen, Germany AU - Mantakaki, A. AU - Fakoya, A. O. J. AU - Sharifpanah, F. C7 - e5805 DB - Scopus DO - 10.7717/peerj.5805 IS - 10 KW - Biomaterials Bioprosthetics Cardiac progenitor cells Congenital heart disease Scaffolds Stem cells Tissue engineering M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2018 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85055719821&doi=10.7717%2fpeerj.5805&partnerID=40&md5=dba488ccef4ef3179c1a935e55072c26 VL - 2018 ID - 1833 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 - 124061922. Language: English. Entry Date: 20170714. Revision Date: 20180702. Publication Type: Article AU - March, Sarita DB - ccm DO - 10.1111/jspn.12185 DP - EBSCOhost IS - 3 KW - Parents Caregivers Caregiver Support Home Nursing Hypoplastic Left Heart Syndrome -- Nursing Patient Discharge Patient Attitudes Child, Medically Fragile Systematic Review Human Medline CINAHL Database PubMed Parent-Child Relations Caregiver Burden Health Knowledge Models, Theoretical Thematic Analysis Patient Discharge Education Discharge Planning Family Coping Child Inpatients N1 - research; systematic review; tables/charts. Journal Subset: Core Nursing; Nursing; Peer Reviewed; USA. Special Interest: Evidence-Based Practice. NLM UID: 101142025. PY - 2017 SN - 1539-0136 SP - n/a-N.PAG 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 - Journal for Specialists in Pediatric Nursing 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? UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=124061922&site=ehost-live&scope=site VL - 22 ID - 1468 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. © 2017 Wiley Periodicals, Inc. AD - University of Virginia School of Nursing, Charlottesville, VA, United States AU - March, S. C7 - e12185 DB - Scopus DO - 10.1111/jspn.12185 IS - 3 KW - caregiver congenital heart discharge home care hospital care hypoplastic left heart syndrome parents transition M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2017 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 - Journal for Specialists in Pediatric Nursing 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? UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85021871419&doi=10.1111%2fjspn.12185&partnerID=40&md5=4741cce46c3b74d2541997b93c624b64 VL - 22 ID - 1852 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. AU - March, S. AU - Keim-Malpass, J. DB - Medline DO - 10.1111/jspn.12225 IS - 3 KW - adult blogging child parent relation coping behavior female home care hospital discharge human hypoplastic left heart syndrome infant male nursing organization and management preschool child prognosis psychology qualitative research risk assessment transitional care verbal communication LA - English M3 - Article N1 - L624598271 2018-10-31 2019-11-19 PY - 2018 SN - 1744-6155 SP - e12225 ST - Transition experiences between hospital- and home-care for parents of children with hypoplastic left heart syndrome T2 - Journal for specialists in pediatric nursing : JSPN TI - Transition experiences between hospital- and home-care for parents of children with hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L624598271 http://dx.doi.org/10.1111/jspn.12225 VL - 23 ID - 676 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. © 2018 Wiley Periodicals, Inc. AD - Levine Children's Hospital, Carolinas Medical Center, Charlotte, NC, United States Department of Acute and Specialty Care, School of Nursing, University of Virginia, Charlottesville, VA, United States Department of Pediatrics, University of Virginia School of Medicine, Charlottesville, VA, United States AU - March, S. AU - Keim-Malpass, J. C7 - e12225 DB - Scopus DO - 10.1111/jspn.12225 IS - 3 KW - blog analysis congenital heart defect hypoplastic left heart syndrome interstage transitions in care M3 - Article N1 - Export Date: 15 June 2020 PY - 2018 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85052812086&doi=10.1111%2fjspn.12225&partnerID=40&md5=67605e7beb28867960ecf4d8f1465c4b VL - 23 ID - 1795 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 - B.S. Marino, Department of Pediatrics and Medical Social Sciences, Northwestern University Feinberg School of Medicine, Heart Center, Research and Academic Affairs, Divisions of Cardiology and Critical Care Medicine, Ann and Robert H. Lurie Children's Hospital of Chicago, Box 21, 225 East Chicago Avenue, Chicago, IL, United States 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. DB - Embase Medline DO - 10.1017/S1047951116000093 IS - 1 KW - alkaline phosphatase biological marker adolescent adult article cardiovascular magnetic resonance case study child congenital heart disease creatinine clearance female Fontan procedure cardiac index human hypoplastic left heart syndrome major clinical study male mean corpuscular volume receiver operating characteristic retrospective study sensitivity and specificity LA - English M3 - Article N1 - L614784524 2017-03-17 2017-04-11 PY - 2017 SN - 1467-1107 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://www.embase.com/search/results?subaction=viewrecord&from=export&id=L614784524 http://dx.doi.org/10.1017/S1047951116000093 VL - 27 ID - 778 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. © Cambridge University Press 2015Â. AD - Department of Pediatrics, Division of Cardiology, Division of Critical Care Medicine, Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Department of Pediatrics, Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Department of Pediatrics, Division of General and Community Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Department of Pediatrics, Division of Hematology/Oncology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Department of Pediatrics, Division of Nephrology and Hypertension, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Department of Pediatrics, Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA, United States Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Philadelphia, Philadelphia, PA, United States Department of Pediatrics, Division of Pediatric Cardiology, University of Michigan C.S. Mott Children's Hospital, Ann Arbor, MI, United States Department of Pediatrics, Division of Pediatric Cardiology, Children's Hospital of Wisconsin, Milwaukee, WI, United States Department of Pediatrics, Division of Cardiology, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, United States Department of Pediatrics, Division of Cardiology, Mayo Clinic, Rochester, MN, United States Department of Pediatrics, Division of Cardiology, Nationwide Children's Hospital, Columbus, OH, United States Department of Pediatric and Congenital Heart Surgery, Cleveland Clinic, Cleveland, OH, United States Department of Pediatrics, Division of Pediatric Cardiology, University of Minnesota Amplatz Children's Hospital, Minneapolis, MN, United States Department of Pediatrics and Medical Social Sciences, Northwestern University Feinberg School of Medicine, Heart Center, Research and Academic Affairs, Divisions of Cardiology and Critical Care Medicine, Ann and Robert H. Lurie Children's Hospital of Chicago, Box 21, 225 East Chicago Avenue, Chicago, IL 60611-2991, United States 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. DB - Scopus DO - 10.1017/S1047951116000093 IS - 1 KW - biomarkers cardiac index cardiac MRI Fontan M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2017 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85014915185&doi=10.1017%2fS1047951116000093&partnerID=40&md5=60914571733a7eb2e6fbc499799ab090 VL - 27 ID - 1879 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. © 2008 Springer Science+Business Media B.V. AD - B. S. Marino, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, 3333 Burnet Avenue, Cincinnati, OH 45229, United States 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. DB - Embase Medline DO - 10.1007/s11136-008-9323-8 IS - 4 KW - adolescent adult article child emotionality female heart disease heart single ventricle heart ventricle human internal consistency major clinical study male pediatric cardiac quality of life inventory pilot study principal component analysis priority journal psychosocial care quality of life LA - English M3 - Article N1 - L50095574 2008-05-21 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://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50095574 http://dx.doi.org/10.1007/s11136-008-9323-8 VL - 17 ID - 1214 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. (PsycINFO Database Record (c) 2019 APA, all rights reserved) AD - Marino, Bradley S., Department of Pediatrics, Divisions of Cardiology and Critical Care Medicine, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, MLC 2003, 3333 Burnet Avenue, Cincinnati, OH, US, 45229 AN - 2008-06767-012 AU - Marino, Bradley S. AU - Shera, David AU - Wernovsky, Gil AU - Tomlinson, Ryan S. AU - Aguirre, Abigail AU - Gallagher, Maureen AU - Lee, Angela AU - Cho, Catherine J. AU - Stern, Whitney AU - Davis, Lauren AU - Tong, Elizabeth AU - Teitel, David AU - Mussatto, Kathleen AU - Ghanayem, Nancy AU - Gleason, Marie AU - Gaynor, J. William AU - Wray, Jo AU - Helfaer, Mark A. AU - Shea, Judy A. DB - psyh DO - 10.1007/s11136-008-9323-8 DP - EBSCOhost IS - 4 KW - Pediatric Cardiac Quality of Life Inventory quality of life heart disease survivors mortality surgery psychometrics test development Adaptation, Psychological Adolescent Cardiac Care Facilities Child Child Welfare Databases as Topic Feasibility Studies Female Heart Diseases Humans Male Pilot Projects Heart Disorders Inventories Pediatrics Death and Dying Test Construction Quality of Life Measures N1 - Department of Anesthesiology and Critical Care, Division of Critical Care Medicine, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, US. Release Date: 20080609. Correction Date: 20190211. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Conference Information: Scientific Session of the American College of Cardiology, 54th, 2005, Orlando, FL, US. Major Descriptor: Heart Disorders; Inventories; Pediatrics; Psychometrics; Quality of Life. Minor Descriptor: Death and Dying; Surgery; Survivors; Test Construction; Quality of Life Measures. Classification: Health Psychology Testing (2226); Cardiovascular Disorders (3295). Population: Human (10); Male (30); Female (40). Location: US. Age Group: Childhood (birth-12 yrs) (100); School Age (6-12 yrs) (180); Adolescence (13-17 yrs) (200); Adulthood (18 yrs & older) (300); Young Adulthood (18-29 yrs) (320). Tests & Measures: Pediatric Cardiac Quality of Life Inventory DOI: 10.1037/t69719-000. Methodology: Empirical Study; Quantitative Study. References Available: Y. Page Count: 14. Issue Publication Date: May, 2008. Sponsor: Children's Hospital of Philadelphia, Critical Care Medicine Endowed Chair, US. Recipients: No recipient indicated PY - 2008 SN - 0962-9343 1573-2649 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: An International Journal of Quality of Life Aspects of Treatment, Care & Rehabilitation 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=psyh&AN=2008-06767-012&site=ehost-live&scope=site ORCID: 0000-0002-4769-1211 bradley.marino@cchmc.org VL - 17 ID - 1680 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. © 2008 Springer Science+Business Media B.V. AD - Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, 3333 Burnet Avenue, Cincinnati, OH 45229, United States Department of Anesthesiology and Critical Care, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, United States Department of Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, United States Division of Biostatistics, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, United States Department of Medicine, Division of General Internal Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA, United States Department of Pediatrics, University of California San Francisco Children's Hospital, University of California, San Francisco, San Francisco, CA, United States Herma Heart Center, Children's Hospital of Wisconsin, Milwaukee, WI, United States Department of Pediatrics, Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI, United States Department of Surgery, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, United States Royal Brompton and Harefield NHS Trust, Harefield Hospital, Harefield, Middlesex UB9 6JH, United Kingdom Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London WC1N 3JH, United Kingdom 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. DB - Scopus DO - 10.1007/s11136-008-9323-8 IS - 4 KW - Children/adolescents Congenital heart disease Quality of life M3 - Article N1 - Cited By :76 Export Date: 15 June 2020 PY - 2008 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-43049179995&doi=10.1007%2fs11136-008-9323-8&partnerID=40&md5=1137dc45a1b13b9024c25582e7baeedb VL - 17 ID - 2195 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 - Division Critical Care Medicine, Department of Anesthesiology and Critical Care Medicine, Children's Hospital of Philadelphia, Philadelphia, PA, United States Division of Cardiology, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA, United States Division of Behavioral Medicine and Clinical Psychology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Division of Cardiology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Division of General Internal Medicine, Department of Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA, United States Division of Biostatistics and Epidemiology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States 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 - Scopus DO - 10.1542/peds.2008-2572 IS - 4 KW - Adolescents Children Congenital heart disease Focus groups Medical providers Quality of life M3 - Article N1 - Cited By :57 Export Date: 15 June 2020 PY - 2009 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-65349177093&doi=10.1542%2fpeds.2008-2572&partnerID=40&md5=e8b3cafb103c5eb93cfaa0c8846783f6 VL - 123 ID - 2179 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. Copyright © 2010 by the American Academy of Pediatrics. AD - B. S. Marino, Cincinnati Children's Hospital Medical Center, Department of Pediatrics, MLC 5050, 3333 Burnet Ave, Cincinnati, OH 45229, United States 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. DB - Embase Medline DO - 10.1542/peds.2009-2973 IS - 3 KW - adolescent adult aortic stenosis aortic regurgitation aortic valve stenosis article child Child Behavior Checklist clinical trial competence complete heart block congenital heart disease construct validity coronary artery anomaly correlation analysis cross-sectional study Diagnostic and Statistical Manual of Mental Disorders disease severity Ebstein anomaly Fallot tetralogy female Fontan procedure great vessels transposition health care utilization heart septum defect heart surgery hospital admission human hypoplastic left heart syndrome major clinical study male medical research mitral valve atresia mitral valve prolapse mitral valve regurgitation mitral valve stenosis multicenter study assessment of humans parent patent ductus arteriosus Pediatric Cardiac Quality of Life Inventory physician priority journal prospective study quality of life rating scale reliability school child scoring system Self Perception Profile for Adolescents Self Perception Profile for Children self report sinus node disease supraventricular tachycardia test retest reliability tricuspid valve stenosis validation study Youth Self Report LA - English M3 - Article N1 - L359472145 2010-09-13 2010-09-16 PY - 2010 SN - 1098-4275 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L359472145 http://dx.doi.org/10.1542/peds.2009-2973 VL - 126 ID - 1156 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; bradley.marino@cchmc.org AN - 105092400. Corporate Author: Pediatric Cardiac Quality of Life Inventory Testing Study Consortium. Language: English. Entry Date: 20101203. Revision Date: 20150711. Publication Type: Journal Article 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. DB - ccm DO - 10.1542/peds.2009-2973 DP - EBSCOhost IS - 3 KW - Cardiac Patients -- Psychosocial Factors -- In Infancy and Childhood Instrument Validation Quality of Life -- In Infancy and Childhood Adolescence Checklists Child Child Behavior Checklist Clinical Assessment Tools Construct Validity Cross Sectional Studies Descriptive Statistics Female Funding Source Human Kruskal-Wallis Test Male One-Way Analysis of Variance Post Hoc Analysis Prospective Studies Questionnaires Spearman's Rank Correlation Coefficient Test-Retest Reliability United States N1 - research; tables/charts. Journal Subset: Biomedical; Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. Special Interest: Pediatric Care. Instrumentation: Child Behavior Checklist (CBCL) (Achenbach and Edelbrock); Pediatric Quality of Life Inventory (PEDSQL); Youth Self-Report (YSR); Self Perception Profile for Adolescents (SPPA); Self Perception Profile for Children (SPPC); Pediatric Cardiac Quality of Life Inventory (PCQLI). Grant Information: National Institute of Child Health and Human Development, American Heart Association, Pennsylvania/Delaware Affiliate, Cincinnati Children's Hospital Research Foundation, and Children's Hospital of Philadelphia Institutional Development Fund.. NLM UID: 0376422. PMID: NLM20805147. PY - 2010 SN - 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105092400&site=ehost-live&scope=site VL - 126 ID - 1525 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. (PsycINFO Database Record (c) 2017 APA, all rights reserved) AN - 2011-14385-013 AU - Marino, Bradley S. AU - Tomlinson, Ryan S. AU - Wernovsky, Gil AU - Drotar, Dennis AU - Newburger, Jane W. AU - Mahony, Lynn AU - Mussatto, Kathleen AU - Tong, Elizabeth AU - Cohen, Mitchell AU - Andersen, Charlotte AU - Shera, David AU - Khoury, Philip R. AU - Wray, Jo AU - Gaynor, J. William AU - Helfaer, Mark A. AU - Kazak, Anne E. AU - Shea, Judy A. DB - psyh DO - 10.1542/peds.2009-2973 DP - EBSCOhost IS - 3 KW - Pediatric Cardiac Quality of Life Inventory test validity test reliability psychometrics pediatric patients heart disease Adolescent Adult Child Cross-Sectional Studies Female Heart Diseases Humans Male Middle Aged Prospective Studies Quality of Life Severity of Illness Index Surveys and Questionnaires Heart Disorders Pediatrics N1 - Divisions of Cardiology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, US. Institutional Authors: Pediatric Cardiac Quality of Life Inventory Testing Study Consortium. Release Date: 20111031. Correction Date: 20170914. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Heart Disorders; Pediatrics; Psychometrics; Quality of Life; Test Validity. Minor Descriptor: Test Reliability. Classification: Clinical Psychological Testing (2224); Cardiovascular Disorders (3295). Population: Human (10); Male (30); Female (40); Inpatient (50); Outpatient (60). Location: US. Age Group: Childhood (birth-12 yrs) (100); School Age (6-12 yrs) (180); Adolescence (13-17 yrs) (200); Adulthood (18 yrs & older) (300); Young Adulthood (18-29 yrs) (320). Tests & Measures: Pediatric Quality of Life Inventory; Cardiac Health Related Quality of Life Inventory; ConQoL Index; Child Behavior Checklist; Self-Perception Profile for Adolescents DOI: 10.1037/t05703-000; Youth Self-Report; Self-Perception Profile for Children DOI: 10.1037/t05338-000. Methodology: Empirical Study; Longitudinal Study; Prospective Study; Quantitative Study. References Available: Y. Page Count: 11. Issue Publication Date: Sep, 2010. Copyright Statement: American Academy of Pediatrics. 2010. Sponsor: National Institute of Child Health and Human Development, US. Grant: 5-K23-HD048637. Recipients: No recipient indicated Sponsor: American Heart Association, US. Grant: 0465467U. Other Details: Beginning Grant-in-Aid. Recipients: No recipient indicated Sponsor: Cincinnati Children’s Hospital Research Foundation. Recipients: No recipient indicated Sponsor: Children’s Hospital of Philadelphia, US. Other Details: Institutional Development Fund. Recipients: No recipient indicated PY - 2010 SN - 0031-4005 1098-4275 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2011-14385-013&site=ehost-live&scope=site ORCID: 0000-0002-4769-1211 ORCID: 0000-0002-6090-2394 VL - 126 ID - 1679 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. Copyright © 2010 by the American Academy of Pediatrics. AD - Division of Cardiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Division of Critical Care Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Division of Behavioral and Clinical Psychology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA, United States Division of Epidemiology and Biostatistics, Children's Hospital of Philadelphia, Philadelphia, PA, United States Division of Psychology, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA, United States Division of Critical Care Medicine, Department of Anesthesiology and Critical Care, Children's Hospital of Philadelphia, Philadelphia, PA, United States Division of Cardiothoracic Surgery, Department of Surgery, Children's Hospital of Philadelphia, Philadelphia, PA, United States Department of Cardiology, Children's Hospital Boston, Boston, MA, United States Department of Pediatrics, School of Medicine, Harvard University, Boston, MA, United States Department of Pediatrics, University of Texas, Southwestern Medical Center at Dallas, Dallas, TX, United States Division of Cardiology, Department of Pediatrics, Children's Hospital of Wisconsin, Milwaukee, WI, United States Department of Pediatrics, University of California, San Francisco, Children's Hospital, San Francisco, CA, United States Division of Cardiology, Department of Pediatrics, Phoenix Children's Hospital, Phoenix, AZ, United States Department of Paediatric Cardiology, Royal Brompton and Harefield National Health Service Trust, London, United Kingdom Department of Medicine, School of Medicine, University of Pennsylvania, Philadelphia, PA, United States 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. DB - Scopus DO - 10.1542/peds.2009-2973 IS - 3 KW - Acquired heart disease Congenital heart disease Health-related quality of life Reliability Validity M3 - Article N1 - Cited By :76 Export Date: 15 June 2020 PY - 2010 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-77956272570&doi=10.1542%2fpeds.2009-2973&partnerID=40&md5=9c828ec6d00d34ab4de65530c8256120 VL - 126 ID - 2159 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. © 2010 Copyright the Authors. Journal Compilation © 2010 Wiley Periodicals, Inc. AD - A.L. Marsden, Mechanical and Aerosapce Engineering, University of California San Diego, 9500 Gilman Dr., La Jolla, CA 92093-0411, United States AU - Marsden, A. L. AU - Reddy, V. M. AU - Shadden, S. C. AU - Chan, F. P. AU - Taylor, C. A. AU - Feinstein, J. A. DB - Embase Medline DO - 10.1111/j.1747-0803.2010.00383.x IS - 2 KW - adolescent adult article audiovisual equipment blood flow breathing child clinical article computational fluid dynamics contrast enhancement energy exercise female Fontan procedure heart catheterization hemodynamics human inferior cava vein male multidetector computed tomography nuclear magnetic resonance imaging parameters performance preschool child priority journal rest shear stress simulation superior cava vein venous pressure Sensation 16 Signa Twin Speed LA - English M3 - Article N1 - L359134706 2010-07-15 2010-07-21 PY - 2010 SN - 1747-079X 1747-0803 SP - 104-117 ST - A New Multiparameter Approach to Computational Simulation for Fontan Assessment and Redesign T2 - Congenital Heart Disease TI - A New Multiparameter Approach to Computational Simulation for Fontan Assessment and Redesign UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L359134706 http://dx.doi.org/10.1111/j.1747-0803.2010.00383.x VL - 5 ID - 1150 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. © 2010 Copyright the Authors. Journal Compilation © 2010 Wiley Periodicals, Inc. AD - Mechanical and Aerospace Engineering Department, University of California, San Diego, United States Department of Cardiothoracic Surgery, Stanford University, Stanford, Calif, United States Department of Mechanical, Materials and Aerospace Engineering, Illinois Institute of Technology, Chicago, IL, United States Department of Radiology, Stanford University, Stanford, Calif, United States Department of Pediatrics, Stanford University, Stanford, Calif, United States Department of Bioengineering, Stanford University, Stanford, Calif, United States AU - Marsden, A. L. AU - Reddy, V. M. AU - Shadden, S. C. AU - Chan, F. P. AU - Taylor, C. A. AU - Feinstein, J. A. DB - Scopus DO - 10.1111/j.1747-0803.2010.00383.x IS - 2 KW - Computational Fluid Dynamics Exercise Fontan Surgery Hemodynamics Patient-Specific Modeling M3 - Article N1 - Cited By :35 Export Date: 15 June 2020 PY - 2010 SP - 104-117 ST - A New Multiparameter Approach to Computational Simulation for Fontan Assessment and Redesign T2 - Congenital Heart Disease TI - A New Multiparameter Approach to Computational Simulation for Fontan Assessment and Redesign UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-77953616927&doi=10.1111%2fj.1747-0803.2010.00383.x&partnerID=40&md5=6051ee9f6dc2fad58b1fd8694a401e63 VL - 5 ID - 2156 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. 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. DB - Medline DO - 10.1161/JAHA.119.014172 IS - 6 KW - adult aged article child chronic disease controlled study English (language) female Fontan procedure human hypoplastic left heart syndrome male mental health mental stress meta analysis outcome assessment quality of life sensitivity analysis Short Form 36 social interaction systematic review LA - English M3 - Article N1 - L631270985 2020-03-26 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 - Journal of the American Heart Association TI - Health-Related Quality of Life in Children, Adolescents, and Adults With a Fontan Circulation: A Meta-Analysis UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L631270985 http://dx.doi.org/10.1161/JAHA.119.014172 VL - 9 ID - 561 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 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 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 AN - 142359293. Language: English. Entry Date: In Process. Revision Date: 20200324. Publication Type: journal article. Journal Subset: Biomedical AU - Marshall, Kate H. AU - D'Udekem, Yves AU - Sholler, Gary F. AU - Opotowsky, Alexander R. AU - Costa, Daniel S. J. AU - Sharpe, Louise AU - Celermajer, David S. AU - Winlaw, David S. AU - Newburger, Jane W. AU - Kasparian, Nadine A. DB - ccm DO - 10.1161/JAHA.119.014172 DP - EBSCOhost IS - 6 N1 - Europe; UK & Ireland. NLM UID: 101580524. PMID: NLM32172648. PY - 2020 SN - 2047-9980 SP - 1-23 ST - Health-Related Quality of Life in Children, Adolescents, and Adults With a Fontan Circulation: A Meta-Analysis T2 - Journal of the American Heart Association TI - Health-Related Quality of Life in Children, Adolescents, and Adults With a Fontan Circulation: A Meta-Analysis UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=142359293&site=ehost-live&scope=site VL - 9 ID - 1434 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 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. DB - Scopus DO - 10.1161/JAHA.119.014172 IS - 6 KW - chronic illness congenital heart disease Fontan circulation health‐related quality of life mental health psychological stress M3 - Article N1 - Export Date: 15 June 2020 PY - 2020 SP - e014172 ST - Health-Related Quality of Life in Children, Adolescents, and Adults With a Fontan Circulation: A Meta-Analysis T2 - Journal of the American Heart Association TI - Health-Related Quality of Life in Children, Adolescents, and Adults With a Fontan Circulation: A Meta-Analysis UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85081994644&doi=10.1161%2fJAHA.119.014172&partnerID=40&md5=e343ac83e87245d118029b7d21235f91 VL - 9 ID - 1708 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 - G.R. Martin, Division of Cardiology, Children’s National Heart Institute, George Washington University School of Medicine, Washington, DC, United States AU - Martin, G. R. AU - Anderson, J. B. AU - Vincent, R. N. DB - Embase Medline DO - 10.1177/2150135118815059 IS - 1 KW - atrial septal occluder aortic valve repair article blood clotting disorder carotid artery stenting congenital heart disease electrophysiology Glenn shunt health care system heart atrium septum defect heart catheterization human hypoplastic left heart syndrome mortality patent ductus arteriosus pediatric cardiology percutaneous transluminal angioplasty priority journal pulmonary valve replacement pulmonary valvuloplasty quality of life radiofrequency ablation total quality management treatment outcome LA - English M3 - Article N1 - L625899145 2019-01-17 2020-03-03 PY - 2019 SN - 2150-136X 2150-1351 SP - 72-80 ST - IMPACT Registry and National Pediatric Cardiology Quality Improvement Collaborative: Contributions to Quality in Congenital Heart Disease T2 - World Journal for Pediatric and Congenital Heart Surgery TI - IMPACT Registry and National Pediatric Cardiology Quality Improvement Collaborative: Contributions to Quality in Congenital Heart Disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L625899145 http://dx.doi.org/10.1177/2150135118815059 VL - 10 ID - 639 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 - Division of Cardiology, Children's National Heart Institute and the George Washington University School of MedicineWA, United States 2 Heart Institute at Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Division of Pediatric Cardiology, Children's Healthcare of Atlanta-Emory University, Atlanta, GA, United States AU - Martin, G. R. AU - Anderson, J. B. AU - Vincent, R. N. DB - Scopus DO - 10.1177/2150135118815059 IS - 1 KW - cardiac catheterization/intervention congenital heart disease databases outcomes M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2019 SP - 72-80 ST - IMPACT Registry and National Pediatric Cardiology Quality Improvement Collaborative: Contributions to Quality in Congenital Heart Disease T2 - World journal for pediatric & congenital heart surgery TI - IMPACT Registry and National Pediatric Cardiology Quality Improvement Collaborative: Contributions to Quality in Congenital Heart Disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85062049628&doi=10.1177%2f2150135118815059&partnerID=40&md5=14e1ddf5c02ccd6d897f8fcc7c8db0ed VL - 10 ID - 1773 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. AN - 105980280. Language: English. Entry Date: 20080215. Revision Date: 20150819. Publication Type: Journal Article AU - McBride, M. G. AU - Binder, T. J. AU - Paridon, S. M. DB - ccm DP - EBSCOhost IS - 4 KW - Exercise Tolerance -- Physiology Heart Failure -- Rehabilitation Inpatients Therapeutic Exercise -- Methods Adolescence Adult Blood Pressure -- Physiology Child Child, Preschool Female Heart Failure -- Physiopathology Heart Rate -- Physiology Infant Infant, Newborn Male Patient Compliance Pilot Studies Program Evaluation Prospective Studies Retrospective Design Treatment Outcomes Human N1 - research. Journal Subset: Allied Health; Core Nursing; Double Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Nursing; Peer Reviewed; USA. NLM UID: 101291247. PMID: NLM17667017. PY - 2007 SN - 1932-7501 SP - 219-222 ST - Safety and feasibility of inpatient exercise training in pediatric heart failure: a preliminary report T2 - Journal of Cardiopulmonary Rehabilitation & Prevention TI - Safety and feasibility of inpatient exercise training in pediatric heart failure: a preliminary report UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105980280&site=ehost-live&scope=site VL - 27 ID - 1591 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 - B.W. McCrindle, Hospital for Sick Children, 555 University Avenue, Toronto, Ont. M5G 1X8, Canada 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. DB - Embase Medline DO - 10.1136/adc.2006.105239 IS - 6 KW - accelerometry adolescent adult anaerobic capacity article cardiopulmonary exercise test controlled study exercise female Fontan procedure functional status human major clinical study male physical activity preschool child priority journal questionnaire school child scoring system self esteem self report social psychology LA - English M3 - Article N1 - L46867270 2007-06-01 PY - 2007 SN - 0003-9888 1468-2044 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://www.embase.com/search/results?subaction=viewrecord&from=export&id=L46867270 http://dx.doi.org/10.1136/adc.2006.105239 VL - 92 ID - 1235 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 - Hospital for Sick Children, 555 University Avenue, Toronto, Ont. M5G 1X8, Canada University of Toronto, Hospital for Sick Children, Toronto, Ont., Canada University of Utah, Salt Lake City, UT, United States Columbia University Medical Center, New York, NY, United States Children's Hospital of Philadelphia, Philadelphia, PA, United States New England Research Institutes, Watertown, MA, United States Iowa State University, Ames, IA, United States 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. DB - Scopus DO - 10.1136/adc.2006.105239 IS - 6 M3 - Article N1 - Cited By :109 Export Date: 15 June 2020 PY - 2007 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-34249910666&doi=10.1136%2fadc.2006.105239&partnerID=40&md5=962166bd0873b16a24078ec1257a94c9 VL - 92 ID - 2209 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. © 2006 American Heart Association, Inc. AD - B.W. McCrindle, Hospital for Sick Children, 555 University Ave, Toronto, Ont. M5G 1X8, Canada 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. DB - Embase Medline DO - 10.1161/CIRCULATIONAHA.105.576660 IS - 8 KW - adult allergy anxiety article asthma attention disturbance behavior disorder child depression developmental disorder follow up Fontan procedure health status hearing disorder human income learning disorder major clinical study parent priority journal prognosis social psychology speech disorder United States visual disorder LA - English M3 - Article N1 - L43754280 2006-06-13 PY - 2006 SN - 0009-7322 SP - 1123-1129 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L43754280 http://dx.doi.org/10.1161/CIRCULATIONAHA.105.576660 VL - 113 ID - 1267 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 - 106144992. Corporate Author: Pediatric Heart Network Investigators. Language: English. Entry Date: 20070831. Revision Date: 20150711. Publication Type: Journal Article 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. DB - ccm DP - EBSCOhost IS - 8 KW - Cardiopulmonary Bypass Health Status Adolescence Checklists Child Child, Preschool Cross Sectional Studies Female Health Status Indicators Heart Defects, Congenital -- Complications Heart Defects, Congenital -- Epidemiology Heart Defects, Congenital -- Surgery Infant Male Morbidity Psychological Tests Quality of Life Questionnaires Human N1 - research; tables/charts. Journal Subset: Biomedical; Peer Reviewed; USA. NLM UID: 0147763. PMID: NLM16490823. PY - 2006 SN - 0009-7322 SP - 1123-1129 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106144992&site=ehost-live&scope=site VL - 113 ID - 1539 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. © 2006 American Heart Association, Inc. AD - University of Toronto, Hospital for Sick Children, Toronto, Ont., Canada University of Utah, Salt Lake City, UT, United States New England Research Institutes, Watertown, MA, United States Columbia University Medical Center, New York, NY, United States Children's Hospital of Philadelphia, Philadelphia, PA, United States Medical University of South Carolina, Charleston, SC, United States Duke University Medical Center, Durham, NC, United States Children's Hospital Boston, Boston, MA, United States Hospital for Sick Children, 555 University Ave, Toronto, Ont. M5G 1X8, Canada 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. DB - Scopus DO - 10.1161/CIRCULATIONAHA.105.576660 IS - 8 KW - Follow-up studies Fontan procedure Health status Heart defects, congenital Quality of life M3 - Article N1 - Cited By :127 Export Date: 15 June 2020 PY - 2006 SP - 1123-1129 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-33645314424&doi=10.1161%2fCIRCULATIONAHA.105.576660&partnerID=40&md5=ee4770b6bcfee85ee51535c3ba72d2a4 VL - 113 ID - 2232 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 (R2 = 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. © 2013 Springer Science+Business Media. AD - B.W. McCrindle, Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, ON M5G 1X8, Canada 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. DB - Embase Medline DO - 10.1007/s00246-013-0831-0 IS - 4 KW - adolescent adolescent health adult article breathing cavopulmonary connection child child health cross-sectional study echocardiography exercise exercise test female follow up Fontan procedure health status heart function human longitudinal study major clinical study male morbidity nuclear magnetic resonance imaging physical performance preschool child protein losing gastroenteropathy quality of life questionnaire school child social psychology social status young adult LA - English M3 - Article N1 - L372686918 2014-04-02 2014-04-09 PY - 2014 SN - 1432-1971 0172-0643 SP - 632-640 ST - The relationship of patient medical and laboratory characteristics to changes in functional health status in children and adolescents after the Fontan procedure T2 - Pediatric Cardiology TI - The relationship of patient medical and laboratory characteristics to changes in functional health status in children and adolescents after the Fontan procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L372686918 http://dx.doi.org/10.1007/s00246-013-0831-0 VL - 35 ID - 924 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 (R2 = 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. © 2013 Springer Science+Business Media. AD - Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, ON M5G 1X8, Canada New England Research Institutes, Watertown, MA, United States Boston Children's Hospital, Boston, MA, United States University of Texas Southwestern Medical Center, Dallas, TX, United States Columbia University Medical Center, New York, NY, United States National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, United States University of Utah, Salt Lake City, UT, United States Children's Hospital of Philadelphia, Phladelphia, PA, United States Duke University Medical Center, Durham, NC, United States Medical University of South Carolina, Charleston, SC, United States 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. DB - Scopus DO - 10.1007/s00246-013-0831-0 IS - 4 KW - Congenital Fontan procedure Functional health status Heart defects Pediatrics M3 - Article N1 - Cited By :6 Export Date: 15 June 2020 PY - 2014 SP - 632-640 ST - The relationship of patient medical and laboratory characteristics to changes in functional health status in children and adolescents after the Fontan procedure T2 - Pediatric Cardiology TI - The relationship of patient medical and laboratory characteristics to changes in functional health status in children and adolescents after the Fontan procedure UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84896978987&doi=10.1007%2fs00246-013-0831-0&partnerID=40&md5=96e070270614fa827d2136210be2bfe2 VL - 35 ID - 2005 ER - TY - JOUR AB - Purpose: The aim of this study was to compare associations between generic versus disease-specific functional health status assessments and patient and clinical characteristics for patients with severe congenital heart disease. Methods: This was a cross-sectional observational study involving 325 single ventricle patients, aged 10-18 years, after Fontan procedure. Enrolled patients underwent a medical history review, laboratory testing, and assessment of the functional health status by completion of the generic Child Report Child Health Questionnaire and the disease-specific Congenital Heart Adolescent and Teenage questionnaire. Correlated conceptually equivalent domains from both questionnaires were identified and their associations with patient and clinical variables were compared. Results: From the generic assessment, patients perceived marginally lower physical functioning (p = 0.05) but greater freedom from bodily pain compared with a normal population (p < 0.001). The equivalent physical functioning/limitations domain of the generic instrument, compared with the disease-specific instrument, had similar associations (higher multi-variable model R2) with medical history variables (R 2 = 0.14 versus R2 = 0.12, respectively) and stronger associations with exercise testing variables (R2 = 0.22 versus R 2 = 0.06). Similarly, the corresponding freedom from bodily pain/symptoms domains from both questionnaires showed a greater association for the generic instrument with medical history variables (R2 = 0.15 versus R2 = 0.09, respectively) and non-cardiac conditions (R 2 = 0.13 versus R2 = 0.06). The associations of each questionnaire with echocardiographic results, cardiac magnetic resonance imaging results, and serum brain natriuretic peptide levels were uniformly weak (R 2 range <0.01 to 0.04). Conclusions: Assessment of the physical functional health status using generic and disease-specific instruments yields few differences with regard to associations between conceptually similar domains and patient and clinical characteristics for adolescents after Fontan procedure. © Cambridge University Press 2013. AD - Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, ON M5G 1X8, Canada New England Research Institutes, Watertown, MA, United States National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, United States Primary Children's Medical Center, University of Utah, Salt Lake City, UT, United States Children's Hospital of Philadelphia, Philadelphia, PA, United States Columbia University Medical Center, New York, NY, United States Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Children's Hospital Boston, Boston, MA, United States Medical University of South Carolina, Charleston, SC, United States Wake Forest University, Baptist Medical Center, Winston-Salem, NC, United States AU - McCrindle, B. W. AU - Zak, V. AU - Pemberton, V. L. AU - Lambert, L. M. AU - Vetter, V. L. AU - Lai, W. W. AU - Uzark, K. AU - Margossian, R. AU - Atz, A. M. AU - Cook, A. AU - Newburger, J. W. DB - Scopus DO - 10.1017/S1047951113000632 IS - 3 KW - cardiac defects congenital Fontan procedure paediatrics M3 - Article N1 - Cited By :10 Export Date: 15 June 2020 PY - 2014 SP - 469-477 ST - Functional health status in children and adolescents after Fontan: Comparison of generic and disease-specific assessments T2 - Cardiology in the Young TI - Functional health status in children and adolescents after Fontan: Comparison of generic and disease-specific assessments UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84899443507&doi=10.1017%2fS1047951113000632&partnerID=40&md5=381930ace0a7eb3efba7052d306b69e2 VL - 24 ID - 2004 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. © 2010 American Heart Association. All rights reserved. AD - B. W. McCrindle, Hospital for Sick Children, 555 University Ave, Toronto, ON M5G 1X8, Canada 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. DB - Embase Medline DO - 10.1161/CIRCULATIONAHA.109.869396 IS - 1 KW - brain natriuretic peptide adolescent adult article cardiopulmonary exercise test child controlled study echocardiography exercise female Fontan procedure health status heart hemodynamics heart ventricle function human male nuclear magnetic resonance imaging preschool child priority journal school child scoring system structured questionnaire tricuspid valve regurgitation LA - English M3 - Article N1 - L358141666 2010-02-10 PY - 2010 SN - 0009-7322 1524-4539 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L358141666 http://dx.doi.org/10.1161/CIRCULATIONAHA.109.869396 VL - 121 ID - 1152 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. © 2010 American Heart Association. All rights reserved. AD - Hospital for Sick Children, University of Toronto, Toronto, Canada New England Research Institutes, Watertown, MA, United States Children's Hospital of Philadelphia, Philadelphia, PA, United States Children's Hospital Boston, Boston, MA, United States University of Texas Southwestern Medical Center, Dallas, United States Duke University Medical Center, Durham, NC, United States University of Utah, Salt Lake City, United States Columbia University Medical Center, New York, NY, United States Medical University of South Carolina, Charleston, United States 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. DB - Scopus DO - 10.1161/CIRCULATIONAHA.109.869396 IS - 1 KW - Fontan procedure Heart defects, congenital Pediatrics M3 - Article N1 - Cited By :30 Export Date: 15 June 2020 PY - 2010 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-74549211308&doi=10.1161%2fCIRCULATIONAHA.109.869396&partnerID=40&md5=884cd78b8c430c63152d8cccd758820f VL - 121 ID - 2160 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 AN - 105285054. Corporate Author: Pediatric Heart Network Investigators. Language: English. Entry Date: 20100212. Revision Date: 20161125. Publication Type: journal article 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. AU - McCrindle, Brian W. AU - Zak, Victor AU - Sleeper, Lynn A. AU - Paridon, Stephen M. AU - Colan, Steven D. AU - Geva, Tal AU - Mahony, Lynn DB - ccm DO - 10.1161/CIRCULATIONAHA.109.869396 DP - EBSCOhost IS - 1 KW - Exercise Tolerance -- Physiology Cardiopulmonary Bypass Health Status Heart Defects, Congenital -- Surgery Postoperative Complications -- Physiopathology Ventricular Dysfunction -- Physiopathology Adolescence Cardiovascular System Physiology Child Cross Sectional Studies Echocardiography Exercise Test Female Human Magnetic Resonance Imaging Male Natriuretic Peptide, Brain -- Blood Postoperative Complications -- Pathology Postoperative Complications -- Ultrasonography Questionnaires Ventricular Dysfunction -- Pathology Ventricular Dysfunction -- Ultrasonography N1 - research. Journal Subset: Biomedical; Peer Reviewed; USA. Instrumentation: Child Health Questionnaire (CHQ). Grant Information: HL068292/HL/NHLBI NIH HHS/United States. NLM UID: 0147763. PMID: NLM20026781. PY - 2010 SN - 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105285054&site=ehost-live&scope=site VL - 121 ID - 1587 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. © 2017 Wiley Periodicals, Inc. AD - Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, ON, Canada Department of Paediatrics, University of Toronto, Toronto, ON, Canada University Health Network, Toronto, ON, Canada AU - McKillop, A. AU - McCrindle, B. W. AU - Dimitropoulos, G. AU - Kovacs, A. H. DB - Scopus DO - 10.1111/chd.12553 IS - 2 KW - congenital heart disease exercise physical activity qualitative M3 - Article N1 - Cited By :6 Export Date: 15 June 2020 PY - 2018 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85034976031&doi=10.1111%2fchd.12553&partnerID=40&md5=ed3133c2ea59f1e379c3110f0d629c46 VL - 13 ID - 1817 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 - 89682302. Language: English. Entry Date: 20130819. Revision Date: 20191227. Publication Type: Article AU - McRae, Marion E. DB - ccm DO - 10.4037/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. NLM UID: 101269322. 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=89682302&site=ehost-live&scope=site VL - 24 ID - 1534 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 - 1Division of Pediatric Cardiology, University of California, San Francisco, California, United States of America. AN - 104573535. Language: English. Entry Date: 20120323. Revision Date: 20150711. Publication Type: Journal Article AU - Meadows, J. AU - Jenkins, K. DB - ccm DP - EBSCOhost IS - 4 KW - Cardiopulmonary Bypass -- Adverse Effects Heart Defects, Congenital -- Surgery Intestinal Diseases -- Prevention and Control Intestinal Diseases -- Therapy Academic Medical Centers Adolescence Heart Surgery -- Methods Child Child, Preschool Prospective Studies Combined Modality Therapy Drug Therapy, Combination Female Cardiopulmonary Bypass -- Methods Heart Catheterization Heart Defects, Congenital -- Diagnosis Infant Infant, Newborn Kaplan-Meier Estimator Male Prognosis Intestinal Diseases -- Etiology Intestinal Diseases -- Mortality Retrospective Design Risk Assessment Severity of Illness Indices Survival Analysis Treatment Outcomes Young Adult N1 - research. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Special Interest: Pediatric Care. NLM UID: 9200019. PMID: NLM21349233. PY - 2011 SN - 1047-9511 SP - 363-377 ST - Protein-losing enteropathy: integrating a new disease paradigm into recommendations for prevention and treatment T2 - Cardiology in the Young TI - Protein-losing enteropathy: integrating a new disease paradigm into recommendations for prevention and treatment UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104573535&site=ehost-live&scope=site VL - 21 ID - 1595 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 O2 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 (VO2) 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)/CO2 elimination slope (VE/VCO2), a low end-tidal CO2, and high end-tidal O2 at the ventilatory anaerobic threshold. Although arterial saturation improved significantly after fenestration closure, there was no change in peak VO2 (70.9 ± 18.6% to 74.0 ± 18.6%, p = NS), heart rate, or O2 pulse at peak exercise. In contrast, ventilatory abnormalities (VE/VCO2) improved considerably (44.4 ± 10.9 to 33.3 ± 5.5, p ≤ 0.001). Conclusions: Fontan fenestration closure does not significantly improve peak VO2. However, ventilatory abnormalities improve considerably. © 2008 American College of Cardiology Foundation. AD - J. Meadows, Department of Cardiology, Children's Hospital-Boston, Boston, MA, United States AU - Meadows, J. AU - Lang, P. AU - Marx, G. AU - Rhodes, J. DB - Embase Medline DO - 10.1016/j.jacc.2007.12.063 IS - 2 KW - carbon dioxide adolescent adult article cardiovascular risk child clinical article controlled study exercise fenestration Fontan procedure heart catheterization heart ventricle failure human lung minute volume morbidity oxygen consumption priority journal LA - English M3 - Article N1 - L351885447 2008-08-13 PY - 2008 SN - 0735-1097 SP - 108-113 ST - Fontan Fenestration Closure Has No Acute Effect on Exercise Capacity but Improves Ventilatory Response to Exercise T2 - Journal of the American College of Cardiology TI - Fontan Fenestration Closure Has No Acute Effect on Exercise Capacity but Improves Ventilatory Response to Exercise UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L351885447 http://dx.doi.org/10.1016/j.jacc.2007.12.063 VL - 52 ID - 1203 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 O2 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 (VO2) 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)/CO2 elimination slope (VE/VCO2), a low end-tidal CO2, and high end-tidal O2 at the ventilatory anaerobic threshold. Although arterial saturation improved significantly after fenestration closure, there was no change in peak VO2 (70.9 ± 18.6% to 74.0 ± 18.6%, p = NS), heart rate, or O2 pulse at peak exercise. In contrast, ventilatory abnormalities (VE/VCO2) improved considerably (44.4 ± 10.9 to 33.3 ± 5.5, p ≤ 0.001). Conclusions: Fontan fenestration closure does not significantly improve peak VO2. However, ventilatory abnormalities improve considerably. © 2008 American College of Cardiology Foundation. AD - Department of Cardiology, Children's Hospital-Boston, Boston, MA, United States Department of Pediatrics, Harvard Medical School, Boston, MA, United States AU - Meadows, J. AU - Lang, P. AU - Marx, G. AU - Rhodes, J. DB - Scopus DO - 10.1016/j.jacc.2007.12.063 IS - 2 KW - congenital heart disease exercise testing fenestrated Fontan M3 - Article N1 - Cited By :39 Export Date: 15 June 2020 PY - 2008 SP - 108-113 ST - Fontan Fenestration Closure Has No Acute Effect on Exercise Capacity but Improves Ventilatory Response to Exercise T2 - Journal of the American College of Cardiology TI - Fontan Fenestration Closure Has No Acute Effect on Exercise Capacity but Improves Ventilatory Response to Exercise UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-45849150392&doi=10.1016%2fj.jacc.2007.12.063&partnerID=40&md5=19ab75a7d5150541e71dec76fd3d5a3e VL - 52 ID - 2191 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. AU - Meakins, L. AU - Ray, L. AU - Hegadoren, K. AU - Rogers, L. G. AU - Rempel, G. R. DB - Medline IS - 1 KW - adaptive behavior adult attention caregiver female human hypoplastic left heart syndrome interview male middle aged nursing parent psychology qualitative research social support uncertainty LA - English M3 - Article N1 - L605944738 2015-09-14 PY - 2015 SN - 0097-9805 SP - 31-41, 50 ST - Parental Vigilance in Caring for Their Children with Hypoplastic Left Heart Syndrome T2 - Pediatric nursing TI - Parental Vigilance in Caring for Their Children with Hypoplastic Left Heart Syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L605944738 VL - 41 ID - 894 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 AD - Pediatric Nurse Practitioner, Stollery Children’s Hospital, Edmonton, Alberta, Canada Associate Professor, University of Alberta, Faculty of Nursing, Edmonton, Alberta, Canada Professor, University of Alberta, Faculty of Nursing, Edmonton, Alberta, Canada Research Associate, University of Athabasca, Faculty of Health Discipline, Athabasca, Alberta, Canada Associate Professor, University of Athabasca, Faculty of Health Discipline, Athabasca, Alberta, Canada AN - 103756360. Language: English. Entry Date: 20150212. Revision Date: 20150818. Publication Type: Journal Article AU - Meakins, Leanne AU - Ray, Lynne AU - Hegadoren, Kathleen AU - Rogers, Laura G. AU - Rempel, Gwen R. DB - ccm DP - EBSCOhost IS - 1 KW - Hypoplastic Left Heart Syndrome Parenting Parents -- Psychosocial Factors Human Qualitative Studies Grounded Theory Secondary Analysis Thematic Analysis Content Analysis Exploratory Research Uncertainty Child Care -- Psychosocial Factors Support, Psychosocial Interviews Infant Child, Preschool Interview Guides Constant Comparative Method Data Analysis Software Female Male Adult Alberta Demography Coding Home Nursing -- Psychosocial Factors Social Isolation Parental Behavior N1 - research; tables/charts. Journal Subset: Core Nursing; Nursing; Peer Reviewed; USA. Special Interest: Pediatric Care. NLM UID: 7505804. PY - 2015 SN - 0097-9805 SP - 31-50 ST - Parental Vigilance in Caring for Their Children with Hypoplastic Left Heart Syndrome T2 - Pediatric Nursing TI - Parental Vigilance in Caring for Their 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=103756360&site=ehost-live&scope=site VL - 41 ID - 1451 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 AD - Pediatric Nurse Practitioner, Stollery Children’s Hospital, Edmonton, Alberta, Canada Associate Professor, University of Alberta, Faculty of Nursing, Edmonton, Alberta, Canada Professor, University of Alberta, Faculty of Nursing, Edmonton, Alberta, Canada Research Associate, University of Athabasca, Faculty of Health Discipline, Athabasca, Alberta, Canada Associate Professor, University of Athabasca, Faculty of Health Discipline, Athabasca, Alberta, Canada AN - 100924647. Language: English. Entry Date: 20150212. Revision Date: 20190617. Publication Type: Article AU - Meakins, Leanne AU - Ray, Lynne AU - Hegadoren, Kathleen AU - Rogers, Laura G. AU - Rempel, Gwen R. DB - ccm DP - EBSCOhost IS - 1 KW - Hypoplastic Left Heart Syndrome Parenting Parents -- Psychosocial Factors Human Qualitative Studies Grounded Theory Secondary Analysis Thematic Analysis Content Analysis Exploratory Research Uncertainty Child Care -- Psychosocial Factors Support, Psychosocial Interviews Infant Child, Preschool Interview Guides Constant Comparative Method Data Analysis Software Female Male Adult Alberta Demography Coding Home Nursing -- Psychosocial Factors Social Isolation Parental Behavior N1 - research; tables/charts. Journal Subset: Core Nursing; Nursing; Peer Reviewed; USA. NLM UID: 7505804. PY - 2015 SN - 0097-9805 SP - 31-50 ST - Parental Vigilance in Caring for Their Children with Hypoplastic Left Heart Syndrome T2 - Pediatric Nursing TI - Parental Vigilance in Caring for Their 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=100924647&site=ehost-live&scope=site VL - 41 ID - 1452 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. © 2015, Anthony J. Jannetti Publications, Inc. All rights reserved. AD - Stollery Children’s Hospital, Edmonton, AB, Canada University of Alberta, Faculty of Nursing, Edmonton, AB, Canada University of Athabasca, Faculty of Health Discipline, Athabasca, AB, Canada AU - Meakins, L. AU - Ray, L. AU - Hegadoren, K. AU - Rogers, L. G. AU - Rempel, G. R. DB - Scopus IS - 1 M3 - Article N1 - Cited By :16 Export Date: 15 June 2020 PY - 2015 SP - 31-41 and 50 ST - Parental vigilance in caring for their children with hypoplastic left heart syndrome T2 - Pediatric Nursing TI - Parental vigilance in caring for their children with hypoplastic left heart syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84941004794&partnerID=40&md5=af658252f25284e85894fab658858ef5 VL - 41 ID - 1957 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. (PsycINFO Database Record (c) 2016 APA, all rights reserved) AD - Medoff-Cooper, Barbara, University of Pennsylvania School of Nursing, 418 Curie Blvd, Claire Fagin Hall, Philadelphia, PA, US, 19104 AN - 2016-05316-028 AU - Medoff-Cooper, Barbara AU - Irving, Sharon Y. AU - Hanlon, Alexandra L. AU - Golfenshtein, Nadya AU - Radcliffe, Jerilynn AU - Stallings, Virginia A. AU - Marino, Bradley S. AU - Ravishankar, Chitra DB - psyh DO - 10.1016/j.jpeds.2015.10.017 DP - EBSCOhost KW - feeding mode growth outcomes developmental outcomes infants complex congenital heart disease length of stay Assistive Technology Congenital Disorders Eating Behavior Heart Disorders Infant Development Treatment Duration N1 - University of Pennsylvania School of Nursing, Philadelphia, PA, US. Release Date: 20160505. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Assistive Technology; Congenital Disorders; Eating Behavior; Heart Disorders. Minor Descriptor: Infant Development; Treatment Duration. Classification: Medical Treatment of Physical Illness (3363). Population: Human (10); Male (30); Female (40). Location: US. Age Group: Childhood (birth-12 yrs) (100); Infancy (2-23 mo) (140). Tests & Measures: Bayley Scales of Infant Development II; Psychomotor Developmental Index; Mental Developmental Index. Methodology: Empirical Study; Longitudinal Study; Prospective Study; Quantitative Study. References Available: Y. Page Count: 6. Issue Publication Date: Feb, 2016. Publication History: Accepted Date: Oct 6, 2015; Revised Date: Aug 6, 2015; First Submitted Date: Apr 6, 2015. Copyright Statement: All rights reserved. Elsevier Inc. 2016. Sponsor: National Institutes of Health, National Institute of Nursing Research, US. Grant: R01 NR002093; MO1-RR00240; UL1-RR-024134. Recipients: No recipient indicated PY - 2016 SN - 0022-3476 SP - 154-159 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 - The Journal of Pediatrics TI - The association among feeding mode, growth, and developmental outcomes in infants with complex congenital heart disease at 6 and 12 months of age UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2016-05316-028&site=ehost-live&scope=site medoff@nursing.upenn.edu VL - 169 ID - 1681 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 - aSchool of Nursing, University of Pennsylvania bDepartment of Pediatrics, The Children's Hospital of Philadelphia, USA. AN - 108028343. Language: English. Entry Date: 20130802. Revision Date: 20150712. Publication Type: Journal Article. Journal Subset: Biomedical AU - Medoff-Cooper, B. AU - Ravishankar, C. DB - ccm DO - 10.1097/HCO.0b013e32835dd005 DP - EBSCOhost IS - 2 KW - Failure to Thrive -- Etiology Heart Defects, Congenital -- Complications Heart Defects, Congenital -- Physiopathology Malnutrition -- Complications Nutritional Support -- Methods Nutritional Support -- Standards Child Energy Intake Enteral Nutrition Failure to Thrive -- Physiopathology Heart Defects, Congenital -- Surgery Parents -- Psychosocial Factors Preoperative Care Risk Factors Stress, Psychological N1 - Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. NLM UID: 8608087. PMID: NLM23370229. PY - 2013 SN - 0268-4705 SP - 122-129 ST - Nutrition and growth in congenital heart disease: a challenge in children T2 - Current Opinion in Cardiology TI - Nutrition and growth in congenital heart disease: a challenge in children UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=108028343&site=ehost-live&scope=site VL - 28 ID - 1529 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 < 0.001) 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. © 2006 The Author(s). AD - M. Mellander, Department of Paediatric Cardiology, Queen Silva Children's Hospital, S-416 85, Göteborg, Sweden AU - Mellander, M. AU - Berntsson, L. AU - Nilsson, B. DB - Embase Medline DO - 10.1111/j.1651-2227.2007.00039.x IS - 1 KW - age distribution article child controlled study divorce family female health survey human hypoplastic left heart syndrome major clinical study male Norwood procedure patient satisfaction priority journal psychological well-being quality of life questionnaire self esteem sex ratio social status statistical significance Sweden treatment outcome LA - English M3 - Article N1 - L46307401 2007-03-21 PY - 2007 SN - 0803-5253 1651-2227 SP - 53-57 ST - Quality of life in children with hypoplastic left heart syndrome T2 - Acta Paediatrica, International Journal of Paediatrics TI - Quality of life in children with hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L46307401 http://dx.doi.org/10.1111/j.1651-2227.2007.00039.x VL - 96 ID - 1244 ER - TY - JOUR AB - [Correction Notice: An erratum for this article was reported in Vol 96(2) of Acta Paediatrica (see record [rid]2008-07782-014[/rid]). 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.] 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) AD - Mellander, M., Department of Paediatric Cardiology, Queen Silva Children's Hospital, S-416 85, Goteborg, Sweden AN - 2008-07779-002 AU - Mellander, M. AU - Berntsson, L. AU - Nilsson, B. DB - psyh DO - 10.1111/j.1651-2227.2007.00039.x DP - EBSCOhost IS - 1 KW - quality of life hypoplastic left heart syndrome children Cardiac Surgical Procedures Child Child, Preschool Developmental Disabilities Female Humans Male Postoperative Complications Socioeconomic Factors Surveys and Questionnaires Sweden 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: 20090504. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Heart Disorders; Pediatrics; Quality of Life; Syndromes. Classification: Cardiovascular Disorders (3295). Population: Human (10); Male (30); Female (40). Location: Sweden. Age Group: Childhood (birth-12 yrs) (100); Preschool Age (2-5 yrs) (160); School Age (6-12 yrs) (180). Methodology: Empirical Study; Quantitative Study. Supplemental Data: Tables and Figures Internet. References Available: Y. Page Count: 5. Issue Publication Date: Jan, 2007. Sponsor: Gothenburg Medical Society, Sweden. Recipients: No recipient indicated Sponsor: Joint Committee of the Nordic Social Science Research Councils. Recipients: No recipient indicated Sponsor: Nordic School of the Public Health. Recipients: No recipient indicated PY - 2007 SN - 0803-5253 1651-2227 SP - 53-57 ST - Quality of life in children with hypoplastic left heart syndrome T2 - Acta Paediatrica TI - Quality of life in children with hypoplastic left heart syndrome UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2008-07779-002&site=ehost-live&scope=site mats.mellander@vgregion.se VL - 96 ID - 1684 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 < 0.001) 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. © 2006 The Author(s). AD - Department of Pediatric Cardiology, Queen Silvia Childreńs Hospital, Göteborg University, Göteborg, Sweden Institute of Health and Care Sciences, Queen Silvia Childreńs Hospital, Göteborg University, Göteborg, Sweden Pediatric Cardiac Surgery, Queen Silvia Childreńs Hospital, Göteborg University, Göteborg, Sweden Department of Paediatric Cardiology, Queen Silva Children's Hospital, S-416 85, Göteborg, Sweden AU - Mellander, M. AU - Berntsson, L. AU - Nilsson, B. DB - Scopus DO - 10.1111/j.1651-2227.2007.00039.x IS - 1 KW - Congenital heart defects HLHS Quality of life M3 - Article N1 - Cited By :24 Export Date: 15 June 2020 PY - 2007 SP - 53-57 ST - Quality of life in children with hypoplastic left heart syndrome T2 - Acta Paediatrica, International Journal of Paediatrics TI - Quality of life in children with hypoplastic left heart syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-33847205754&doi=10.1111%2fj.1651-2227.2007.00039.x&partnerID=40&md5=e7ca008c3aff76e362dd912100d70b04 VL - 96 ID - 2215 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. © Cambridge University Press, 2014. AD - Department of Paediatric Cardiology, Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Göteborg, Sweden Texas Fetal Center, University of Texas, Houston, TX, United States AU - Mellander, M. AU - Gardiner, H. DB - Scopus DO - 10.1017/S1047951114001231 KW - Evidence based medicine Foetal heart Foetal therapies M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2014 SP - 36-40 ST - Foetal therapy, what works? An overview T2 - Cardiology in the Young TI - Foetal therapy, what works? An overview UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84913532335&doi=10.1017%2fS1047951114001231&partnerID=40&md5=a03564cda8d0e8fe3ae78a1e61e0b2f0 VL - 24 ID - 1986 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. © 2014 Mosby Inc. All rights reserved. AD - K. Mellion, University of Toledo College of Medicine, 4366 Niagara Lane, Perrysburg, OH 43551, United States 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. DB - Embase Medline DO - 10.1016/j.jpeds.2013.11.066 IS - 4 KW - adolescent article asthma child chronic disease congenital heart disease controlled study disease severity end stage renal disease female heart single ventricle human major clinical study male obesity Pediatric Quality of Life Inventory 4.0 Generic Core Scale priority journal quality of life school child scoring system Student t test LA - English M3 - Article N1 - L52950116 2014-01-15 2014-04-01 PY - 2014 SN - 0022-3476 1097-6833 SP - 781-788.e1 ST - Health-related quality of life outcomes in children and adolescents with congenital heart disease T2 - Journal of Pediatrics TI - Health-related quality of life outcomes in children and adolescents with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52950116 http://dx.doi.org/10.1016/j.jpeds.2013.11.066 VL - 164 ID - 917 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 - 104048426. Corporate Author: Pediatric Cardiac Quality of Life Inventory Testing Study Consortium. Language: English. Entry Date: 20140523. Revision Date: 20161117. Publication Type: journal article AU - Mellion, Katelyn AU - Uzark, Karen AU - Cassedy, Amy AU - Drotar, Dennis AU - Wernovsky, Gil AU - Newburger, Jane W. AU - Mahony, Lynn AU - Mussatto, Kathy AU - Cohen, Mitchell AU - Limbers, Christine AU - Marino, Bradley S. DB - ccm DO - 10.1016/j.jpeds.2013.11.066 DP - EBSCOhost IS - 4 KW - Heart Defects, Congenital -- Diagnosis Heart Diseases Quality of Life Adolescence Child Chronic Disease Clinical Assessment Tools Cross Sectional Studies Female Heart Diseases -- Diagnosis Human Male Retrospective Design N1 - research. Journal Subset: Biomedical; Peer Reviewed; USA. Special Interest: Pediatric Care. Instrumentation: Pediatric Quality of Life Inventory 4.0 Generic Core Scales (PedsQL). Grant Information: 5-K23-HD048637/HD/NICHD NIH HHS/United States. NLM UID: 0375410. PMID: NLM24412135. 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 - Journal of Pediatrics TI - Health-related quality of life outcomes in children and adolescents with congenital heart disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104048426&site=ehost-live&scope=site VL - 164 ID - 1596 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. (PsycINFO Database Record (c) 2019 APA, all rights reserved) AD - Mellion, Katelyn, University of Toledo, College of Medicine, M3, 4366 Niagara Lane, Perrysburg, OH, US, 43551 AN - 2014-10099-036 AU - Mellion, Katelyn AU - Uzark, Karen AU - Cassedy, Amy AU - Drotar, Dennis AU - Wernovsky, Gil AU - Newburger, Jane W. AU - Mahony, Lynn AU - Mussatto, Kathy AU - Cohen, Mitchell AU - Limbers, Christine AU - Marino, Bradley S. DB - psyh DO - 10.1016/j.jpeds.2013.11.066 DP - EBSCOhost IS - 4 KW - quality of life congenital heart disease Adolescent Child Chronic Disease Cross-Sectional Studies Female Heart Defects, Congenital Heart Diseases Humans Male Retrospective Studies Chronicity (Disorders) Heart Disorders Pediatrics Health Related Quality of Life N1 - Division of Cardiology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, US. Institutional Authors: Pediatric Cardiac Quality of Life Inventory Testing Study Consortium. Release Date: 20140721. Correction Date: 20190211. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Conference Information: American Academy of Pediatrics' National Conference and Exhibition, Oct, 2010, San Francisco, CA, US. Conference Note: Portions of the study were presented as a poster at the aforementioned conference. Major Descriptor: Chronicity (Disorders); Heart Disorders; Pediatrics; Quality of Life; Health Related Quality of Life. Classification: Cardiovascular Disorders (3295). Population: Human (10); Male (30); Female (40); Outpatient (60). Location: US. Age Group: Childhood (birth-12 yrs) (100); School Age (6-12 yrs) (180); Adolescence (13-17 yrs) (200); Adulthood (18 yrs & older) (300); Young Adulthood (18-29 yrs) (320). Tests & Measures: Generic Core Scales; Pediatric Cardiac Quality of Life Inventory DOI: 10.1037/t69719-000. Methodology: Empirical Study; Quantitative Study. References Available: Y. Page Count: 8. Issue Publication Date: Apr, 2014. Publication History: Accepted Date: Nov 26, 2013; Revised Date: Oct 18, 2013; First Submitted Date: May 28, 2013. Copyright Statement: All rights reserved. Mosby Inc. 2014. Sponsor: National Institute of Child Health and Human Development, US. Grant: 5-K23-HD048637. Recipients: No recipient indicated Sponsor: American Heart Association, US. Recipients: No recipient indicated Sponsor: Great Rivers Affiliate, US. Grant: Aid 0465467U. Other Details: Pennsylvania/Delaware Affiliate; beginning Grant-in-Aid. Recipients: No recipient indicated Sponsor: Cincinnati Children’s Hospital Research Foundation, US. Recipients: No recipient indicated Sponsor: Children’s Hospital of Philadelphia, US. Other Details: Institutional Development Fund. Recipients: No recipient indicated PY - 2014 SN - 0022-3476 1097-6833 SP - 781-788 ST - Health-related quality of life outcomes in children and adolescents with congenital heart disease T2 - The Journal of Pediatrics TI - Health-related quality of life outcomes in children and adolescents with congenital heart disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2014-10099-036&site=ehost-live&scope=site ORCID: 0000-0002-6090-2394 katelyn.mellion@rockets.utoledo.edu VL - 164 ID - 1669 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. © 2014 Mosby Inc. All rights reserved. AD - Division of Cardiology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Department of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Division of Behavioral and Clinical Psychology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Division of Cardiology, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA, United States Division of Cardiology, Department of Pediatrics, Children's Hospital Boston, Boston, MA, United States Department of Pediatrics, University of Texas, Southwestern Medical Center Dallas, Dallas, TX, United States Division of Cardiology, Department of Pediatrics, Children's Hospital of Wisconsin, Milwaukee, WI, United States Department of Cardiology, Phoenix Children's Hospital, Phoenix, AZ, United States Department of Psychology and Neuroscience, Baylor University, Waco, TX, United States Division of Critical Care Medicine, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States 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. DB - Scopus DO - 10.1016/j.jpeds.2013.11.066 IS - 4 M3 - Article N1 - Cited By :70 Export Date: 15 June 2020 PY - 2014 SP - 781-788.e1 ST - Health-related quality of life outcomes in children and adolescents with congenital heart disease T2 - Journal of Pediatrics TI - Health-related quality of life outcomes in children and adolescents with congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84896544743&doi=10.1016%2fj.jpeds.2013.11.066&partnerID=40&md5=eae014f5be542339e685f0660a69b9ad VL - 164 ID - 2000 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 - S.C. Menon, University of Utah and Primary Children's Hospital, Salt Lake City, UT, United States 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. DB - Embase Medline DO - 10.1111/chd.12597 IS - 3 KW - adolescent anthropometry article body mass child controlled study cross-sectional study delayed puberty disease severity exercise family income female Fontan procedure human hypoplastic left heart syndrome major clinical study male multicenter study New York Heart Association class oxygen consumption Pediatric Quality of Life Inventory physical appearance quality of life short stature social status survivor United States LA - English M3 - Article N1 - L622527485 2018-06-15 2018-07-20 PY - 2018 SN - 1747-0803 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 - Congenital Heart Disease TI - Delayed puberty and abnormal anthropometry and its associations with quality of life in young Fontan survivors: A multicenter cross-sectional study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L622527485 http://dx.doi.org/10.1111/chd.12597 VL - 13 ID - 689 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. © 2018 Wiley Periodicals, Inc. AD - University of Utah and Primary Children's Hospital, Salt Lake City, UT, United States The Hospital for Sick Children, Toronto, ON, Canada Children's Hospital of Philadelphia, Philadelphia, PA, United States Emory University School of Med, Atlanta, GA, United States Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Texas Children's Hospital, Houston, TX, United States University of Michigan, Ann Arbor, MI, United States Columbia University Medical Center, New York, NY, United States Medical University of South Carolina, Charleston, SC, United States Duke University School of Medicine, Durham, NC, United States Riley Hospital for Children, Indianapolis, IN, United States 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. DB - Scopus DO - 10.1111/chd.12597 IS - 3 KW - abnormal growth delayed puberty Fontan quality of life M3 - Article N1 - Cited By :6 Export Date: 15 June 2020 PY - 2018 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 - Congenital Heart Disease TI - Delayed puberty and abnormal anthropometry and its associations with quality of life in young Fontan survivors: A multicenter cross-sectional study UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85048273222&doi=10.1111%2fchd.12597&partnerID=40&md5=337221fd04e7b63e01d19b5e2f057940 VL - 13 ID - 1806 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 - 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 - Scopus IS - 1 KW - Amplatzer Catheterization Fontan procedure M3 - Article N1 - Export Date: 15 June 2020 PY - 2008 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-77953387395&partnerID=40&md5=809a8f812f2a7c121aea25932bf31a01 VL - 3 ID - 2187 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. © 2020 Elsevier Ltd AD - Department of Histology and Embryology, Jessenius Faculty of Medicine in Martin, Comenius University in Bratislava, Malá Hora 4, Martin, 036 01, Slovakia Department of Histology and Embryology, Faculty of Medicine, Comenius University in Bratislava, Sasinkova 4, Bratislava, 811 08, Slovakia AU - Mestanova, V. AU - Varga, I. AU - Adamkov, M. C7 - 109599 DB - Scopus DO - 10.1016/j.mehy.2020.109599 KW - Apoptosis Congenital heart defects Histomorphology Immunohistochemistry Proliferation Thymus M3 - Article N1 - Export Date: 15 June 2020 PY - 2020 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85078909496&doi=10.1016%2fj.mehy.2020.109599&partnerID=40&md5=e739c2095fa442ed82ffc7c221ac9538 VL - 138 ID - 1701 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), 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 - Division of Pediatric Cardiology, Johns Hopkins University School of Medicine, Baltimore, Maryland AN - 107145922. Language: English. Entry Date: 20001101. Revision Date: 20150711. Publication Type: Journal Article AU - Miller, M. R. AU - Forrest, C. B. AU - Kan, J. S. DB - ccm DP - EBSCOhost IS - 2 KW - Heart Defects, Congenital Heart Defects, Congenital -- In Adolescence Parental Attitudes Physicians Adolescence Child Convenience Sample Data Analysis Software Data Analysis, Statistical Descriptive Statistics Funding Source Human Parental Attitudes -- Evaluation Questionnaires Survey Research N1 - research; tables/charts. Journal Subset: Biomedical; Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. Grant Information: Hayden Whitney Smith Research Scholarship. NLM UID: 0376422. PMID: NLM10920149. 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=107145922&site=ehost-live&scope=site VL - 106 ID - 1597 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. Copyright © 2019 by the American Academy of Pediatrics. AD - Department of Pediatrics, Texas Children’s Hospital, Baylor College of Medicine, Houston, TX, United States Department of Cardiology, Boston Children’s Hospital, Department of Pediatrics, Harvard Medical School, Harvard University, Boston, MA, United States Department of Pediatrics, University of Toronto, Labatt Family Heart Centre, Hospital for Sick Children, Toronto, Canada New England Research Institute, Watertown, MA, United States Department of Pediatrics, Children’s Hospital of Philadelphia, Philadelphia, PA, United States Heart Institute, Cincinnati Children’s Hospital, Cincinnati, OH, United States National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, United States Department of Pediatrics, School of Medicine, Emory University, Atlanta, GA, United States Department of Pediatrics, Children’s Hospital of Los Angeles, Los Angeles, CA, United States Department of Pediatrics, University of Michigan, Ann Arbor, MI, United States Department of Pediatrics, Columbia University, New York, NY, United States Department of Pediatrics, Medical University of South Carolina, Charleston, SC, United States Departments of Surgery and Pediatrics, Thomas Jefferson University, Wilmington, DE, United States Congenital Heart Institute of Florida, St. Petersburg, FL, United States Department of Pediatrics, Duke University Medical Center, Durham, NC, United States Department of Pediatrics, University of Utah, 81 N Mario Capecchi Dr, Salt Lake City, UT 84113, 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. C7 - Y DB - Scopus DO - 10.1542/peds.2018-2577 IS - 5 M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2019 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85065508049&doi=10.1542%2fpeds.2018-2577&partnerID=40&md5=5a642be0772cb4ad928dba5e433175cc VL - 143 ID - 1750 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. (PsycINFO Database Record (c) 2016 APA, all rights reserved) AD - Miller, Stephen G., 81 N Mario Capecchi Dr, Salt Lake City, UT, US, 84113 AN - 2015-58644-045 AU - Miller, Thomas A. AU - Zak, Victor AU - Shrader, Peter AU - Ravishankar, Chitra AU - Pemberton, Victoria L. AU - Newburger, Jane W. AU - Shillingford, Amanda J. AU - Dagincourt, Nicholas AU - Cnota, James F. AU - Lambert, Linda M. AU - Sananes, Renee AU - Richmond, Marc E. AU - Hsu, Daphne T. AU - Miller, Stephen G. AU - Zyblewski, Sinai C. AU - Williams, Richard V. DB - psyh DO - 10.1016/j.jpeds.2015.09.041 DP - EBSCOhost KW - asymmetric growth neurodevelopmental outcomes bivariate analyses high-risk infants single ventricle Angiotensin-Converting Enzyme Inhibitors Cardiovascular Abnormalities Cephalometry Double-Blind Method Enalapril Female Growth Disorders Heart Defects, Congenital Heart Ventricles Humans Infant Infant, Newborn Male Neurodevelopmental Disorders Prospective Studies At Risk Populations Cardiovascular Disorders Head (Anatomy) Neural Development N1 - Department of Pediatrics, University of Utah, Salt Lake City, UT, US. Institutional Authors: Pediatric Heart Network Investigators. Release Date: 20160225. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: At Risk Populations; Cardiovascular Disorders; Head (Anatomy); Neural Development. Classification: Physical & Somatoform & Psychogenic Disorders (3290). Population: Human (10); Male (30); Female (40). Location: US. Age Group: Adulthood (18 yrs & older) (300). Tests & Measures: Bayley Scales of Infant Development-II; Psychomotor Developmental Index. Methodology: Empirical Study; Longitudinal Study; Prospective Study; Quantitative Study; Treatment Outcome. References Available: Y. Page Count: 6. Issue Publication Date: Jan, 2016. Publication History: Accepted Date: Sep 10, 2015; Revised Date: Jul 10, 2015; First Submitted Date: Mar 30, 2015. Copyright Statement: All rights reserved. Elsevier Inc. 2016. Sponsor: National Heart, Lung, and Blood Institute, US. Grant: HL068269; HL068270; HL068279; HL068281; HL068285; HL068292; HL068290; HL068288; HL085057; HL109781; HL109737. Recipients: No recipient indicated Sponsor: US Food and Drug Administration’s, Office of Orphan Products Development, US. Recipients: No recipient indicated PY - 2016 SN - 0022-3476 SP - 220-225 ST - Growth asymmetry, head circumference, and neurodevelopmental outcomes in infants with single ventricles T2 - The Journal of Pediatrics TI - Growth asymmetry, head circumference, and neurodevelopmental outcomes in infants with single ventricles UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2015-58644-045&site=ehost-live&scope=site thomas.a.miller@hsc.utah.edu VL - 168 ID - 1675 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 - 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 - Scopus DO - 10.1007/s00246-007-9154-3 IS - 3 KW - Fenestration closure Fontan procedure Transcatheter closure M3 - Article N1 - Cited By :9 Export Date: 15 June 2020 PY - 2008 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-43549120035&doi=10.1007%2fs00246-007-9154-3&partnerID=40&md5=928c5e150b5f378163ebcbfc9a9c0c6a VL - 29 ID - 2196 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. © 2019 The Society of Thoracic Surgeons AD - The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA, United States Department of Cardiology, Children's Hospital Boston, Boston, MA, United States Kirklin Institute for Research in Surgical Outcomes, Birmingham, AL, United States Division of Cardiac Surgery, Children's Mercy Hospital, Kansas City, MO, United States Division of Cardiology, Children's Health Dallas, Dallas, TX, United States Division of Cardiac Surgery, Texas Children's Hospital, Houston, TX, United States Division of Cardiology, Stanford University, Palo Alto, CA, 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. AU - Pedimacs, Investigators DB - Scopus DO - 10.1016/j.athoracsur.2019.01.038 IS - 4 M3 - Article N1 - Cited By :22 Export Date: 15 June 2020 PY - 2019 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85063108154&doi=10.1016%2fj.athoracsur.2019.01.038&partnerID=40&md5=53d2246ca84bc28589472b00ea6dcdd4 VL - 107 ID - 1755 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 - C.H. Lorenz, Ctr. for Cardiovasc. Magnetic Reson., Barnes-Jewish Hospital, Washington University Medical Center, 216 South Kingshighway Blvd., St. Louis, MO 63110, United States AU - Morgan, V. L. AU - Graham, T. P., Jr. AU - Roselli, R. J. AU - Lorenz, C. H. DB - Embase Medline DO - 10.1016/S0022-5223(98)70130-8 IS - 2 KW - adolescent adult article blood vessel wall child clinical article congenital heart disease controlled study female Fontan procedure heart single ventricle human lung blood flow male nuclear magnetic resonance imaging priority journal shear stress surgical technique three-dimensional imaging tricuspid valve atresia Siemens Magneton SP 4000 scanner LA - English M3 - Article N1 - L28367097 1998-09-11 PY - 1998 SN - 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 - Journal of Thoracic and Cardiovascular Surgery TI - Alterations in pulmonary artery flow patterns and shear stress determined with three-dimensional phase-contrast magnetic resonance imaging in Fontan patients UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L28367097 http://dx.doi.org/10.1016/S0022-5223(98)70130-8 VL - 116 ID - 1347 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. © 1998 Mosby, Inc. AD - Department of Biomedical Engineering, United States Department of Radiology and Radiological Sciences, United States Department of Pediatric Cardiology, United States Vanderbilt University, Nashville, TN, United States Cardiovascular Division, Barnes- Jewish Hospital, Washington University Medical Center, 216 South Kingshighway Blvd., St. Louis, MO 63110, United States AU - Morgan, V. L. AU - Graham, T. P., Jr. AU - Roselli, R. J. AU - Lorenz, C. H. DB - Scopus DO - 10.1016/S0022-5223(98)70130-8 IS - 2 M3 - Article N1 - Cited By :44 Export Date: 15 June 2020 PY - 1998 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 - Journal of Thoracic and Cardiovascular Surgery TI - Alterations in pulmonary artery flow patterns and shear stress determined with three-dimensional phase-contrast magnetic resonance imaging in fontan patients UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0031869747&doi=10.1016%2fS0022-5223%2898%2970130-8&partnerID=40&md5=12e51afa7c119c38326790625151d341 VL - 116 ID - 2291 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. (C) 2000 Elsevier Science Ireland Ltd. AD - Division of Pediatric Cardiology, Dept. Pediat., Univ. Arkansas M., Little Rock, AR, United States Division of Cardiovascular Surgery, Dept. Surg., Univ. Alabama B., Birmingham, AL, United States AU - Morrow, W. R. AU - Frazier, E. AU - Naftel, D. C. DB - Scopus DO - 10.1016/S1058-9813(00)00041-2 IS - 2 KW - Cardiac transplantation Competing outcomes Infancy and childhood Survival analysis M3 - Article N1 - Cited By :35 Export Date: 15 June 2020 PY - 2000 SP - 99-105 ST - Survival after listing for cardiac transplantation in children T2 - Progress in Pediatric Cardiology TI - Survival after listing for cardiac transplantation in children UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0034214308&doi=10.1016%2fS1058-9813%2800%2900041-2&partnerID=40&md5=6b52e2fbfe9a01494175e8193269eda5 VL - 11 ID - 2275 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 - J.R. Heimall, Division of Allergy and Immunology, Children's Hospital of Philadelphia, 3550 Market St, 3rd Fl, Philadelphia, PA, United States AU - Morsheimer, M. M. AU - Rychik, J. AU - Forbes, L. AU - Dodds, K. AU - Goldberg, D. J. AU - Sullivan, K. AU - Heimall, J. R. DB - Embase Medline DO - 10.1016/j.jaip.2015.11.034 IS - 3 KW - antibiotic agent immunoglobulin immunoglobulin G adolescent adult antibiotic prophylaxis article asplenia atopy bacteremia cellulitis child common variable immunodeficiency Epstein Barr virus infection female Fontan procedure heart single ventricle Hodgkin disease human lymphocyte count lymphocytopenia major clinical study male molluscum contagiosum protein losing gastroenteropathy retrospective study verruca vulgaris viral clearance viral skin disease LA - English M3 - Article N1 - L608450527 2016-02-23 2016-05-25 PY - 2016 SN - 2213-2198 SP - 491-496 ST - Risk Factors and Clinical Significance of Lymphopenia in Survivors of the Fontan Procedure for Single-Ventricle Congenital Cardiac Disease T2 - Journal of Allergy and Clinical Immunology: In Practice TI - Risk Factors and Clinical Significance of Lymphopenia in Survivors of the Fontan Procedure for Single-Ventricle Congenital Cardiac Disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L608450527 http://dx.doi.org/10.1016/j.jaip.2015.11.034 VL - 4 ID - 809 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. © 2016 Elsevier Inc. AD - Division of Allergy and Immunology, The Children's Hospital of PhiladelphiaPA, United States Division of Cardiology, The Children's Hospital of PhiladelphiaPA, United States Section of Immunology, Allergy, Rheumatology, Baylor College of Medicine, Tex Children's Hospital, Houston, TX, United States AU - Morsheimer, M. M. AU - Rychik, J. AU - Forbes, L. AU - Dodds, K. AU - Goldberg, D. J. AU - Sullivan, K. AU - Heimall, J. R. DB - Scopus DO - 10.1016/j.jaip.2015.11.034 IS - 3 KW - Fontan Hypogammaglobulinemia Protein-losing enteropathy (PLE) Secondary immunodeficiency Single ventricle T-cell lymphopenia Warts M3 - Article N1 - Cited By :9 Export Date: 15 June 2020 PY - 2016 SP - 491-496 ST - Risk Factors and Clinical Significance of Lymphopenia in Survivors of the Fontan Procedure for Single-Ventricle Congenital Cardiac Disease T2 - Journal of Allergy and Clinical Immunology: In Practice TI - Risk Factors and Clinical Significance of Lymphopenia in Survivors of the Fontan Procedure for Single-Ventricle Congenital Cardiac Disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84958191630&doi=10.1016%2fj.jaip.2015.11.034&partnerID=40&md5=dfa8770dda3c20cd69bb4dfc2ec33f4c VL - 4 ID - 1906 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 - H. Ohuchi, Department of Pediatrics, National Cardiovascular Center, 5-7-1 Fujishirodai, Suita 565-8565, Japan AU - Motoki, N. AU - Ohuchi, H. AU - Miyazaki, A. AU - Yamada, O. DB - Embase Medline DO - 10.1253/circj.CJ-08-1155 IS - 9 KW - angiotensin receptor antagonist antiarrhythmic agent anticoagulant agent anticonvulsive agent beta adrenergic receptor blocking agent brain natriuretic peptide carvedilol creatinine digoxin dipeptidyl carboxypeptidase inhibitor diuretic agent hemoglobin metoprolol nitrogen propranolol urea uric acid adult age distribution arterial oxygen saturation article bacterial endocarditis bradycardia brain abscess cardiovascular disease central venous pressure controlled study creatinine blood level female follow up Fontan procedure functional status glomerulus filtration gout health status heart arrhythmia heart ejection fraction heart failure cardiac index heart single ventricle heart ventricle enddiastolic pressure heart ventricle pressure heart ventricle tachycardia hemoglobin blood level hemoptysis human kidney function long term care major clinical study male marriage medical record review occupation oxygen consumption pathophysiology protein blood level protein losing gastroenteropathy retrospective study social behavior supraventricular tachycardia thrombus urea nitrogen blood level uric acid blood level LA - English M3 - Article N1 - L355187506 2009-10-20 http://www.jstage.jst.go.jp/article/circj/73/9/1711/_pdf PY - 2009 SN - 1346-9843 1347-4820 SP - 1711-1716 ST - Clinical profiles of adult patients with single ventricular physiology T2 - Circulation Journal TI - Clinical profiles of adult patients with single ventricular physiology UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L355187506 http://dx.doi.org/10.1253/circj.CJ-08-1155 VL - 73 ID - 1176 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 Department of Pediatrics, National Cardiovascular Center, 5-7-1 Fujishirodai, Suita 565-8565, Japan AU - Motoki, N. AU - Ohuchi, H. AU - Miyazaki, A. AU - Yamada, O. DB - Scopus DO - 10.1253/circj.CJ-08-1155 IS - 9 KW - Adult congenital heart disease Fontan Quality of life Single ventricle Survival M3 - Article N1 - Cited By :17 Export Date: 15 June 2020 PY - 2009 SP - 1711-1716 ST - Clinical profiles of adult patients with single ventricular physiology T2 - Circulation Journal TI - Clinical profiles of adult patients with single ventricular physiology UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-69549137860&doi=10.1253%2fcircj.CJ-08-1155&partnerID=40&md5=62e91befd2d1049317fac4b8f97b6e12 VL - 73 ID - 2173 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 - Department of Mechanical Engineering, University of Auckland, Private Bag 92019, Auckland, New Zealand Centre for Advanced MRI, University of Auckland, Private Bag 92019, Auckland, New Zealand Green Lane Paediatric and Congenital Cardiac Service, Starship Children's Hospital, Auckland, New Zealand AU - Moyle, K. R. AU - Mallinson, G. D. AU - Occleshaw, C. J. AU - Cowan, B. R. AU - Gentles, T. L. DB - Scopus DO - 10.1007/s00246-005-0918-3 IS - 3 KW - Atriopulmonary Cavopulmonary Fontan connection Hemodynamic energy losses M3 - Article N1 - Cited By :16 Export Date: 15 June 2020 PY - 2006 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-33744781550&doi=10.1007%2fs00246-005-0918-3&partnerID=40&md5=606f13267068d0796ef5d9429ae1aee9 VL - 27 ID - 2230 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 (αs), alpha long (αL), root mean square short and long (RMSs and RMSL), 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 αS, RMSL, 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, αS 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 - S.B. Mulkey, Division of Fetal and Transitional Medicine, Children’s National Health System, Washington, DC, United States 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. DB - Embase Medline DO - 10.1007/s10286-019-00616-w IS - 2 KW - hypertensive agent neuromuscular blocking agent prostaglandin E1 sedative agent Apgar score article artificial ventilation case control study controlled study coronary care unit electrocardiogram gestational age great vessels transposition head circumference heart cycle heart rate variability human hypoplastic left heart syndrome infant major clinical study newborn perinatal period preoperative period prospective study QRS complex sympathetic tone LA - English M3 - Article N1 - L628343445 2019-07-05 2020-04-20 PY - 2020 SN - 1619-1560 0959-9851 SP - 165-172 ST - Heart rate variability is depressed in the early transitional period for newborns with complex congenital heart disease T2 - Clinical Autonomic Research TI - Heart rate variability is depressed in the early transitional period for newborns with complex congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L628343445 http://dx.doi.org/10.1007/s10286-019-00616-w VL - 30 ID - 558 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 (αs), alpha long (αL), root mean square short and long (RMSs and RMSL), 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 αS, RMSL, 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, αS 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. © 2019, Springer-Verlag GmbH Germany, part of Springer Nature. AD - Division of Fetal and Transitional Medicine, Children’s National Health System, Washington, DC, United States Biostatistics and Study Methodology, The George Washington University School of Medicine and Health Sciences, Washington, DC, United States Department of Cardiology, Children’s National Health System, Washington, DC, United States Department of Pediatrics, The George Washington University School of Medicine and Health Sciences, Washington, DC, United States Department of Neurology, The George Washington University School of Medicine and Health Sciences, Washington, DC, United States Department of Pediatrics, Inova Children’s Hospital, Fairfax, VA, United States Fairfax Neonatal Associates, Fairfax, VA, United States Department of Obstetrics and Gynecology, Inova Fairfax Hospital, Fairfax, VA, United States 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. DB - Scopus DO - 10.1007/s10286-019-00616-w IS - 2 KW - Autonomic nervous system Congenital heart disease Heart rate variability Newborn M3 - Article N1 - Export Date: 15 June 2020 PY - 2020 SP - 165-172 ST - Heart rate variability is depressed in the early transitional period for newborns with complex congenital heart disease T2 - Clinical Autonomic Research TI - Heart rate variability is depressed in the early transitional period for newborns with complex congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85068146893&doi=10.1007%2fs10286-019-00616-w&partnerID=40&md5=25561e8aab7bf727a7e210db9d155f7b VL - 30 ID - 1706 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 resultsFifty-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 (≥60 min, ≥3 metabolic equivalent, ≥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.ConclusionDespite 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 - J. Müller, Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Lazarettstr. 36, D-80636 München, Germany AU - Müller, J. AU - Christov, F. AU - Schreiber, C. AU - Hess, J. AU - Hager, A. DB - Embase Medline DO - 10.1093/eurheartj/ehp305 IS - 23 KW - oxygen accelerometer adolescent adult age distribution aged article cardiopulmonary exercise test cavopulmonary connection child consensus daily life activity exercise female follow up heart single ventricle human major clinical study male mental health oxygen consumption priority journal quality of life school child sex difference Short Form 36 United Kingdom LA - English M3 - Article N1 - L355759499 2009-12-25 PY - 2009 SN - 0195-668X 1522-9645 SP - 2915-2920 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 - European Heart Journal TI - Exercise capacity, quality of life, and daily activity in the long-term follow-up of patients with univentricular heart and total cavopulmonary connection UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L355759499 http://dx.doi.org/10.1093/eurheartj/ehp305 VL - 30 ID - 1162 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 resultsFifty-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 (≥60 min, ≥3 metabolic equivalent, ≥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.ConclusionDespite 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 Department of Cardiovascular Surgery, Deutsches Herzzentrum München, Technische Universität München, München, Germany AU - Müller, J. AU - Christov, F. AU - Schreiber, C. AU - Hess, J. AU - Hager, A. DB - Scopus DO - 10.1093/eurheartj/ehp305 IS - 23 KW - Congenital heart disease Daily activity Exercise capacity Long-term follow-up Quality of life Total cavopulmonary connection M3 - Article N1 - Cited By :81 Export Date: 15 June 2020 PY - 2009 SP - 2915-2920 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 - European Heart Journal TI - Exercise capacity, quality of life, and daily activity in the long-term follow-up of patients with univentricular heart and total cavopulmonary connection UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-71549164641&doi=10.1093%2feurheartj%2fehp305&partnerID=40&md5=7c05acb9086663d7cfb025b4c3eb5bb1 VL - 30 ID - 2163 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. © 2011 Published by Elsevier Ireland Ltd. AD - J. Müller, Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Lazarettstr. 36, D-80636 München, Germany AU - Müller, J. AU - Hess, J. AU - Hager, A. DB - Embase Medline DO - 10.1016/j.ijcard.2010.09.029 IS - 3 KW - adolescent adult aged aortic coarctation aortic stenosis article clinical assessment clinical feature cohort analysis congenital heart disease controlled study cyanotic heart disease depression disease association Ebstein anomaly exercise Fallot tetralogy female great vessels transposition heart septum defect human major clinical study male oxygen consumption population research prevalence priority journal pulmonary valve insufficiency pulmonary valve stenosis quality of life scoring system LA - English M3 - Article N1 - L51096324 2010-10-08 2012-01-30 PY - 2012 SN - 0167-5273 1874-1754 SP - 265-269 ST - Minor symptoms of depression in patients with congenital heart disease have a larger impact on quality of life than limited exercise capacity T2 - International Journal of Cardiology TI - Minor symptoms of depression in patients with congenital heart disease have a larger impact on quality of life than limited exercise capacity UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51096324 http://dx.doi.org/10.1016/j.ijcard.2010.09.029 VL - 154 ID - 1068 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. © 2011 Published by Elsevier Ireland Ltd. AD - Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Lazarettstr. 36, D-80636 München, Germany AU - Müller, J. AU - Hess, J. AU - Hager, A. DB - Scopus DO - 10.1016/j.ijcard.2010.09.029 IS - 3 KW - Congenital heart disease Depression Exercise capacity Quality of life M3 - Article N1 - Cited By :48 Export Date: 15 June 2020 PY - 2012 SP - 265-269 ST - Minor symptoms of depression in patients with congenital heart disease have a larger impact on quality of life than limited exercise capacity T2 - International Journal of Cardiology TI - Minor symptoms of depression in patients with congenital heart disease have a larger impact on quality of life than limited exercise capacity UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84855950631&doi=10.1016%2fj.ijcard.2010.09.029&partnerID=40&md5=4c24828c5b350c296247190a164808b7 VL - 154 ID - 2100 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. © 2011 Cambridge University Press. AD - M.J. Elliott, Department of Cardiothoracic Surgery, Great Ormond Street Hospital for Children, London WC1N 3JH, United Kingdom AU - Murtuza, B. AU - Elliott, M. J. DB - Embase Medline DO - 10.1017/S1047951111001739 IS - SUPPL. 2 KW - article controlled study exercise family counseling functional assessment heart performance human hypoplastic left heart syndrome mental development Norwood procedure patient care patient information physician attitude pregnancy termination prenatal diagnosis professional practice prognosis psychosocial care quality of life surgical mortality survival rate survival time LA - English M3 - Article N1 - L363111240 2011-12-26 2012-01-06 PY - 2011 SN - 1047-9511 1467-1107 SP - 148-158 ST - Changing attitudes to the management of hypoplastic left heart syndrome: A European perspective T2 - Cardiology in the Young TI - Changing attitudes to the management of hypoplastic left heart syndrome: A European perspective UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L363111240 http://dx.doi.org/10.1017/S1047951111001739 VL - 21 ID - 1078 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 - 104497370. Language: English. Entry Date: 20120831. Revision Date: 20150711. Publication Type: Journal Article. Supplement Title: Dec2011 Supplement 2. Journal Subset: Biomedical AU - Murtuza, Bari AU - Elliott, Martin J. DB - ccm DP - EBSCOhost IS - S2 KW - Attitude of Health Personnel Heart Surgery -- Methods Disease Management Hypoplastic Left Heart Syndrome -- Diagnosis Hypoplastic Left Heart Syndrome -- Embryology Hypoplastic Left Heart Syndrome -- Surgery Pregnancy Complications, Cardiovascular Europe Female Infant, Newborn Pregnancy Prenatal Diagnosis N1 - Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Special Interest: Pediatric Care. NLM UID: 9200019. PMID: NLM22152542. PY - 2011 SN - 1047-9511 SP - 148-158 ST - Changing attitudes to the management of hypoplastic left heart syndrome: a European perspective T2 - Cardiology in the Young TI - Changing attitudes to the management of hypoplastic left heart syndrome: a European perspective UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104497370&site=ehost-live&scope=site VL - 21 ID - 1564 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. © 2011 Cambridge University Press. AD - Department of Paediatric Cardiothoracic Surgery, Great Ormond Street Hospital for Children, National Health Service Trust, London, United Kingdom AU - Murtuza, B. AU - Elliott, M. J. DB - Scopus DO - 10.1017/S1047951111001739 IS - SUPPL. 2 KW - antenatal diagnosis comfort care counselling European foetal diagnosis hypoplasia of the left heart hypoplastic left heart syndrome Norwood pregnancy termination single-ventricle repair surgical palliation M3 - Article N1 - Cited By :11 Export Date: 15 June 2020 PY - 2011 SP - 148-158 ST - Changing attitudes to the management of hypoplastic left heart syndrome: A European perspective T2 - Cardiology in the Young TI - Changing attitudes to the management of hypoplastic left heart syndrome: A European perspective UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-83755163904&doi=10.1017%2fS1047951111001739&partnerID=40&md5=65e5deb04ac1e898ca5fc88f3c15bb0d VL - 21 ID - 2109 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. Copyright © 2014 by the American Academy of Pediatrics. AD - K.A. Mussatto, Herma Heart Center, Children's Hospital of Wisconsin, MS B550A, 9000 West Wisconsin Ave, Milwaukee, WI 53201, United States 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. DB - Embase Medline DO - 10.1542/peds.2013-2309 IS - 3 KW - age article Bayley Scales of Infant Development cardiopulmonary bypass child cognition comorbidity congenital heart disease developmental disorder enteric feeding female genetic disorder heart single ventricle hospitalization human language major clinical study male motor performance outcome assessment practice guideline preschool child prevalence priority journal risk assessment risk factor scoring system LA - English M3 - Article N1 - L372509765 2014-03-11 2014-03-20 PY - 2014 SN - 1098-4275 0031-4005 SP - e570-e577 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L372509765 http://dx.doi.org/10.1542/peds.2013-2309 VL - 133 ID - 923 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 - K.A. Mussatto, Children's Hospital of Wisconsin, Herma Heart Center, 9000 Wisconsin Avenue, Milwaukee, WI, United States 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. DB - Embase Medline DO - 10.1017/S104795111700169X IS - 1 KW - article biogeographic region brain development child cognitive therapy cohort analysis developmental delay early childhood intervention follow up health care utilization human hypoplastic left heart syndrome medical history mental disease occupational therapy physiotherapy speech therapy survival rate LA - English M3 - Article N1 - L619883557 2017-12-26 2018-07-27 PY - 2018 SN - 1467-1107 1047-9511 SP - 126-133 ST - Utilisation of early intervention services in young children with hypoplastic left heart syndrome T2 - Cardiology in the Young TI - Utilisation of early intervention services in young children with hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L619883557 http://dx.doi.org/10.1017/S104795111700169X VL - 28 ID - 716 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. © Cambridge University Press 2017. AD - Children's Hospital of Wisconsin, Herma Heart Center, 9000 Wisconsin Avenue, Milwaukee, WI 53201, United States New England Research Institutes, Inc., Watertown, Massachusetts, United States Nemours/Alfred I. DuPont Hospital for Children, Department of Pediatrics, Wilmington, Delaware, United States Hospital for Sick Children, Department of Pediatrics, Toronto, Ontario, Canada Children's Hospital Los Angeles, Department of Pediatrics, Los Angeles, California, United States Primary Children's Hospital, Department of Pediatrics, Salt Lake City, Utah, United States Children's Healthcare of Atlanta, Department of Pediatrics, Atlanta, Georgia, United States Children's Hospital of Philadelphia, Department of Pediatrics, Philadelphia, Pennsylvania, United States University of Michigan Health System, Department of Pediatrics, Ann Arbor, Michigan, United States Boston Children's Hospital, Department of Pediatrics, Boston, Massachusetts, United States Medical College of Wisconsin, Department of Pediatrics, Milwaukee, Wisconsin, United States Ann and Robert H. Lurie Children's Hospital, Department of Pediatrics, Chicago, Illinois, United States Duke University Hospital, Department of Pediatrics, Durham, North Carolina, United States Department 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 Johns Hopkins All Children's Hospital, Johns Hopkins University, Department of Pediatrics, Saint Petersburg, Florida and Baltimore, Pediatric Heart NetworkMaryland, United States Medical University of South Carolina, Department of Pediatrics, Charleston, South Carolina, United States National Heart, Lung, and Blood Institute, Bethesda, Pediatric Heart NetworkMaryland, United States 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. DB - Scopus DO - 10.1017/S104795111700169X IS - 1 KW - CHD early intervention neurodevelopmental disorders M3 - Article N1 - Cited By :9 Export Date: 15 June 2020 PY - 2018 SP - 126-133 ST - Utilisation of early intervention services in young children with hypoplastic left heart syndrome T2 - Cardiology in the Young TI - Utilisation of early intervention services in young children with hypoplastic left heart syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85038413629&doi=10.1017%2fS104795111700169X&partnerID=40&md5=13f28c097ec5d65baa7304f4ed704e45 VL - 28 ID - 1828 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 children 3 . 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. © 2018 Lahore Medical And Dental College. All Rights Reserved. AD - Department of Child Health, College of Medicine, King Khalid University, Saudi Arabia Department of Pediatrics, Faculty of Medicine, University of Kordofan, Sudan Department of Family and Community Medicine, Faculty of Medicine, King Khalid University, Saudi Arabia Department of Anatomy, Faculty of Medicine, King Khalid University, Saudi Arabia Faculty of Medicine and Health Sciences, University of Kordofan, Al-Ubayyid, Sudan AU - Mustafa, A. E. M. AU - Tahir Ali, N. M. AU - Elrahim, S. Y. A. AU - Bharti, R. K. R. AU - Chaudhary, S. DB - Scopus IS - 4 KW - Children Echocardiography Management Rheumatic Cardiac Disease Sudan M3 - Article N1 - Export Date: 15 June 2020 PY - 2018 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85062681877&partnerID=40&md5=0724ecca72935ca9f7fe0f8bec4458c9 VL - 12 ID - 1834 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 - S.P. Namachivayam, Intensive Care Unit, The Royal Children's Hospital, Flemington Rd, Parkville, Australia AU - Namachivayam, S. P. AU - d'Udekem, Y. AU - Millar, J. AU - Cheung, M. M. AU - Butt, W. DB - Embase Medline DO - 10.1016/j.jtcvs.2016.05.006 IS - 4 KW - acute kidney failure article child disability disease severity female functional status heart surgery hospital discharge human hypoplastic left heart syndrome incidence infant intensive care length of stay long term survival major clinical study male mortality outcome assessment pediatric intensive care unit priority journal quality of life reoperation risk factor survival time survivor LA - English M3 - Article N1 - L611037589 2016-07-06 2018-08-07 PY - 2016 SN - 1097-685X 0022-5223 SP - 1104-1112.e3 ST - Survival status and functional outcome of children who required prolonged intensive care after cardiac surgery T2 - Journal of Thoracic and Cardiovascular Surgery TI - Survival status and functional outcome of children who required prolonged intensive care after cardiac surgery UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L611037589 http://dx.doi.org/10.1016/j.jtcvs.2016.05.006 VL - 152 ID - 792 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. © 2016 The American Association for Thoracic Surgery AD - Intensive Care Unit, Royal Children's Hospital, Melbourne, Australia Cardiac Surgery, Royal Children's Hospital, 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 AU - Namachivayam, S. P. AU - d'Udekem, Y. AU - Millar, J. AU - Cheung, M. M. AU - Butt, W. DB - Scopus DO - 10.1016/j.jtcvs.2016.05.006 IS - 4 KW - functional outcome pediatric cardiac surgery prolonged intensive care survival status M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 2016 SP - 1104-1112.e3 ST - Survival status and functional outcome of children who required prolonged intensive care after cardiac surgery T2 - Journal of Thoracic and Cardiovascular Surgery TI - Survival status and functional outcome of children who required prolonged intensive care after cardiac surgery UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84976476618&doi=10.1016%2fj.jtcvs.2016.05.006&partnerID=40&md5=8d3526f1feda6f1b8ed2210a0a6fa9f7 VL - 152 ID - 1888 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. © 2017 The Author. AD - Department of Pediatric Cardiac Surgery, Regina Margherita Children's Hospital, Piazza Polonia 94, Torino, 10126, Italy AU - Napoleone, C. P. AU - Cascarano, M. T. AU - Deorsola, L. AU - Valori, A. DB - Scopus DO - 10.1093/icvts/ivx288 IS - 2 KW - Extracorporeal membrane oxygenation Fontan procedureFontan procedure Ventricular assist device M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2018 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85041524205&doi=10.1093%2ficvts%2fivx288&partnerID=40&md5=fbd7fee2ee98fb99b438928e0185f0c7 VL - 26 ID - 1824 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. © 2014 by The American Association for Thoracic Surgery. AD - M. Nathan, Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School, 300 Longwood Ave, Bader 273, Boston, MA 02215, United States AU - Nathan, M. AU - Sadhwani, A. AU - Gauvreau, K. AU - Agus, M. AU - Ware, J. AU - Newburger, J. W. AU - Pigula, F. DB - Embase Medline DO - 10.1016/j.jtcvs.2013.08.032 IS - 1 KW - article Bayley Scales of Infant Development childhood disease clinical assessment tool cognition disease association disease classification disease severity echocardiography female heart surgery human infant major clinical study male morbidity motor activity nerve cell differentiation palliative therapy priority journal retrospective study technical performance score trisomy 21 LA - English M3 - Article N1 - L52795296 2013-10-02 2014-07-02 PY - 2014 SN - 1097-685X 0022-5223 SP - 232-237.e3 ST - Association between Technical Performance Scores and neurodevelopmental outcomes after congenital cardiac surgery T2 - Journal of Thoracic and Cardiovascular Surgery TI - Association between Technical Performance Scores and neurodevelopmental outcomes after congenital cardiac surgery UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52795296 http://dx.doi.org/10.1016/j.jtcvs.2013.08.032 VL - 148 ID - 945 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 - C. Neri, Department of Women's and Children's Health, Sacro Cuore Catholic University, Agostino Gemelli University Hospital Foundation, Largo Francesco Vito 1, Rome, Italy 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. DB - Embase Medline DO - 10.23736/S0026-4784.18.04190-4 IS - 3 KW - lithium adult article autism bipolar disorder birth weight cesarean section congenital malformation echocardiography female fetus malformation gestational age human intrauterine growth retardation language delay major clinical study neonatal respiratory distress syndrome newborn pregnancy prepregnancy care retrospective study teratology vaginal delivery LA - English M3 - Article N1 - L622657491 2018-06-26 2018-11-07 PY - 2018 SN - 0026-4784 SP - 261-267 ST - Managing fertile women under lithium treatment: The challenge of a Teratology Information Service T2 - Minerva Ginecologica TI - Managing fertile women under lithium treatment: The challenge of a Teratology Information Service UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L622657491 http://dx.doi.org/10.23736/S0026-4784.18.04190-4 VL - 70 ID - 681 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. © 2018 EDIZIONI MINERVA MEDICA. AD - Department of Women's and Children's Health, Sacro Cuore Catholic University, Agostino Gemelli University Hospital Foundation, Largo Francesco Vito 1, Rome, 00168, Italy 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. DB - Scopus DO - 10.23736/S0026-4784.18.04190-4 IS - 3 KW - Lithium Preconception care Pregnancy M3 - Article N1 - Export Date: 15 June 2020 PY - 2018 SP - 261-267 ST - Managing fertile women under lithium treatment: The challenge of a Teratology Information Service T2 - Minerva Ginecologica TI - Managing fertile women under lithium treatment: The challenge of a Teratology Information Service UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85048783938&doi=10.23736%2fS0026-4784.18.04190-4&partnerID=40&md5=e7a95fe434d46b976d40060a6dfc97f5 VL - 70 ID - 1803 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 - F. Neunhoeffer, Department of Pediatric Cardiology, Pulmology and Pediatric Intensive Care Medicine, University Children’s Hospital Tübingen, Hoppe-Seyler-Str. 1, Tuebingen, Germany AU - Neunhoeffer, F. AU - Hofbeck, M. AU - Schlensak, C. AU - Schuhmann, M. U. AU - Michel, J. DB - Embase Medline DO - 10.1007/s00246-018-1952-2 IS - 8 KW - arterial switch operation arterio cerebral difference in oxygen content article brain injury brain oxygen consumption cardiopulmonary bypass cerebral metabolic rate of oxygen cerebral oxygen saturation clinical article cohort analysis controlled study Doppler flowmetry female great vessels transposition hemodynamics human hypoplastic left heart syndrome male nervous system parameters newborn Norwood procedure observational study oxygen extraction fraction oxygen saturation perioperative period prospective study risk factor spectroscopy LA - English M3 - Article N1 - L623774983 2018-09-11 2019-01-18 PY - 2018 SN - 1432-1971 0172-0643 SP - 1681-1687 ST - Perioperative Cerebral Oxygenation Metabolism in Neonates with Hypoplastic Left Heart Syndrome or Transposition of the Great Arteries T2 - Pediatric Cardiology TI - Perioperative Cerebral Oxygenation Metabolism 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=L623774983 http://dx.doi.org/10.1007/s00246-018-1952-2 VL - 39 ID - 648 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. © 2018, Springer Science+Business Media, LLC, part of Springer Nature. AD - Department of Pediatric Cardiology, Pulmology and Pediatric Intensive Care Medicine, University Children’s Hospital Tübingen, Hoppe-Seyler-Str. 1, Tuebingen, 72076, Germany Department of Thoracic and Cardiovascular Surgery, University Hospital Tübingen, Hoppe-Seyler-Str. 1, Tuebingen, 72076, Germany Department of Pediatric Neurosurgery, University Hospital Tübingen, Hoppe-Seyler-Str. 1, Tuebingen, 72076, Germany AU - Neunhoeffer, F. AU - Hofbeck, M. AU - Schlensak, C. AU - Schuhmann, M. U. AU - Michel, J. DB - Scopus DO - 10.1007/s00246-018-1952-2 IS - 8 KW - Arterial switch operation Cerebral autoregulation Congenital heart defect Norwood M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2018 SP - 1681-1687 ST - Perioperative Cerebral Oxygenation Metabolism in Neonates with Hypoplastic Left Heart Syndrome or Transposition of the Great Arteries T2 - Pediatric Cardiology TI - Perioperative Cerebral Oxygenation Metabolism in Neonates with Hypoplastic Left Heart Syndrome or Transposition of the Great Arteries UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85052800125&doi=10.1007%2fs00246-018-1952-2&partnerID=40&md5=3178173a5e9d915ef88292d725c8066f VL - 39 ID - 1779 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. AN - 108121495. Corporate Author: Pediatric Heart Network Investigators. Language: English. Entry Date: 20120727. Revision Date: 20170504. Publication Type: journal article 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. DB - ccm DO - 10.1161/CIRCULATIONAHA.111.064113 DP - EBSCOhost IS - 17 KW - Anastomosis, Surgical Developmental Disabilities -- Epidemiology Heart Surgery -- Methods Heart Ventricle -- Surgery Hypoplastic Left Heart Syndrome -- Surgery Postoperative Complications -- Epidemiology Psychomotor Disorders -- Epidemiology Pulmonary Artery -- Surgery Vascular Surgery -- Methods Abnormalities, Multiple -- Epidemiology Brain Damage, Chronic -- Epidemiology Brain Damage, Chronic -- Etiology Brain Damage, Chronic -- Prevention and Control Brain Damage, Chronic -- Rehabilitation Child Child, Preschool Clinical Assessment Tools Developmental Disabilities -- Etiology Developmental Disabilities -- Rehabilitation Early Childhood Intervention Female Human Hypoplastic Left Heart Syndrome -- Complications Hypoxia, Brain -- Etiology Hypoxia, Brain -- Psychosocial Factors Infant Infant, Newborn Male Neuropsychological Tests Postoperative Complications -- Etiology Postoperative Complications -- Rehabilitation Prospective Studies Psychological Tests Psychomotor Disorders -- Etiology Psychomotor Disorders -- Rehabilitation Randomized Controlled Trials Risk Factors Scales Socioeconomic Factors N1 - research; randomized controlled trial. Journal Subset: Biomedical; Peer Reviewed; USA. Instrumentation: Mental Developmental Index (MDI); Psychomotor Development Index (PDI); Bayley Scales of Infant Development-Second Edition (BSID-II). Grant Information: K23 HD061601/HD/NICHD NIH HHS/United States. NLM UID: 0147763. PMID: NLM22456475. PY - 2012 SN - 0009-7322 SP - 2081-2091 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=108121495&site=ehost-live&scope=site VL - 125 ID - 1554 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 - Department of Paediatric Cardiology and Intensive Care Medicine, Medical School Hannover, OE 6730 Carl-Neuberg-Str. 1, 3063 Hannover, Germany Medical Sociology Unit, Medical School Hannover, OE 6730 Carl-Neuberg-Str. 1, 3063 Hannover, Germany Department of Pediatric Cardiology and Intensive Care Medicine, University of Göttingen, Göttingen, Germany AU - Norozi, K. AU - Zoege, M. AU - Buchhorn, R. AU - Wessel, A. AU - Geyer, S. DB - Scopus DO - 10.1111/j.1747-0803.2006.00048.x IS - 6 KW - Congenital heart disease Psychological characteristic Spiroergometry M3 - Article N1 - Cited By :16 Export Date: 15 June 2020 PY - 2006 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-33751022358&doi=10.1111%2fj.1747-0803.2006.00048.x&partnerID=40&md5=9839c19718537761ee4f95067f1b8497 VL - 1 ID - 2220 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. Copyright © 2001 by W.B. Saunders Company AD - Cardiovascular Program, Children's Hospital, 300 Longwood Ave, Boston, MA; patricia.obrien@tch.harvard.edu AN - 106894779. Language: English. Entry Date: 20020125. Revision Date: 20150819. Publication Type: Journal Article AU - O'Brien, P. AU - Boisvert, J. T. DB - ccm DP - EBSCOhost IS - 5 KW - Heart Defects, Congenital -- Surgery -- In Infancy and Childhood Heart Ventricle -- Abnormalities Heart Defects, Congenital -- Classification Heart Defects, Congenital -- Nursing Hypoplastic Left Heart Syndrome -- Complications Hypoplastic Left Heart Syndrome -- Physiopathology Hypoplastic Left Heart Syndrome -- Surgery Treatment Outcomes -- In Infancy and Childhood Postoperative Care -- In Infancy and Childhood Pediatric Nursing Parents -- Psychosocial Factors Anastomosis, Surgical -- Methods -- In Infancy and Childhood Heart Surgery -- Methods -- In Infancy and Childhood Heart Surgery -- Adverse Effects -- In Infancy and Childhood Postoperative Complications -- In Infancy and Childhood Arrhythmia -- Etiology -- In Infancy and Childhood Metabolic Diseases -- Etiology -- In Infancy and Childhood Child Development Child Development Disorders -- Etiology Infant, Newborn Infant N1 - pictorial; tables/charts. Journal Subset: Core Nursing; Double Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Nursing; Peer Reviewed; USA. NLM UID: 8607529. PMID: NLM11598866. PY - 2001 SN - 0882-5963 SP - 338-350 ST - Current management of infants and children with single ventricle anatomy T2 - Journal of Pediatric Nursing TI - Current management of infants and children with single ventricle anatomy UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106894779&site=ehost-live&scope=site VL - 16 ID - 1584 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 - Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA, United States Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States Center for Pediatric Clinical Effectiveness, Children's Hospital of Philadelphia, Philadelphia, PA, United States Leonard Davis Institute, University of Pennsylvania, Philadelphia, PA, United States AU - O'Byrne, M. L. AU - McBride, M. G. AU - Paridon, S. AU - Goldmuntz, E. DB - Scopus DO - 10.1177/2150135117752122 IS - 2 KW - exercise physiology hypoplastic left heart syndrome outcomes research pediatric cardiology transposition of the great arteries M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2018 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 journal for pediatric & congenital heart surgery 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85052593454&doi=10.1177%2f2150135117752122&partnerID=40&md5=14519e5415e5a520d0d9c02d5f372fe6 VL - 9 ID - 1813 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. 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. DB - Medline DO - 10.1161/JAHA.114.001234 IS - 7 KW - adolescent child congenital heart malformation factual database female health status heart surgery human male quality of life survivor therapy United Kingdom United States LA - English M3 - Article N1 - L620102477 2018-01-16 PY - 2016 SN - 2047-9980 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://www.embase.com/search/results?subaction=viewrecord&from=export&id=L620102477 http://dx.doi.org/10.1161/JAHA.114.001234 VL - 5 ID - 801 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. © 2016 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell. AD - Cardiorespiratory Department, Great Ormond Street Hospital for Children NHS Foundation Trust, UK Department of Pediatric Cardiology, Royal Brompton and Harefield NHS Foundation Trust, London, London, United Kingdom Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States 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. DB - Scopus DO - 10.1161/JAHA.114.001234 IS - 7 KW - Aristotle Basic Complexity congenital heart disease surgery quality of life Risk Adjustment in Congenital Heart Surgery M3 - Article N1 - Cited By :14 Export Date: 15 June 2020 PY - 2016 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85017413799&doi=10.1161%2fJAHA.114.001234&partnerID=40&md5=b44a35448083fce58896a03d3e760450 VL - 5 ID - 1895 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 - R.D. Oberhuber, Children's Heart Center Linz, Kepler University Hospital, Krankenhausstrasse 26, Linz, Austria AU - Oberhuber, R. D. AU - Huemer, S. AU - Mair, R. AU - Sames-Dolzer, E. AU - Kreuzer, M. AU - Tulzer, G. DB - Embase Medline DO - 10.1017/S1047951120000554 KW - adolescence adolescent article child controlled study education female follow up heart center human hypoplastic left heart syndrome juvenile major clinical study male Pediatric Quality of Life Inventory preschool child psychologist psychosocial development quality of life social interaction LA - English M3 - Article in Press N1 - L631369456 2020-04-07 PY - 2020 SN - 1467-1107 1047-9511 ST - Health-related quality of life for children and adolescents in school age with hypoplastic left heart syndrome: A single-centre study T2 - Cardiology in the Young TI - Health-related quality of life for children and adolescents in school age with hypoplastic left heart syndrome: A single-centre study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L631369456 http://dx.doi.org/10.1017/S1047951120000554 ID - 580 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. © The Author(s) 2020. Published by Cambridge University Press. AD - Children's Heart Center Linz, Kepler University Hospital, Krankenhausstrasse 26, Linz, 4020, Austria Department of Inclusive Education, University of Education of Upper Austria, Linz, 4020, Austria AU - Oberhuber, R. D. AU - Huemer, S. AU - Mair, R. AU - Sames-Dolzer, E. AU - Kreuzer, M. AU - Tulzer, G. DB - Scopus DO - 10.1017/S1047951120000554 KW - Adolescence children health-related quality of life hypoplastic left heart syndrome long-term follow-up M3 - Article N1 - Export Date: 15 June 2020 PY - 2020 ST - Health-related quality of life for children and adolescents in school age with hypoplastic left heart syndrome: A single-centre study T2 - Cardiology in the Young TI - Health-related quality of life for children and adolescents in school age with hypoplastic left heart syndrome: A single-centre study UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85082516294&doi=10.1017%2fS1047951120000554&partnerID=40&md5=5454b513fa5820460d074557f3db9483 ID - 1713 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 - Department of Paediatric Cardiology, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden Department of Paediatric Cardiology, Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Sweden 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 - Scopus DO - 10.1017/S1047951112000352 IS - 1 KW - home monitoring hypoplastic left heart syndrome oxygen saturation psychosocial aspects Single ventricle surgical shunt M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 2013 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84873327429&doi=10.1017%2fS1047951112000352&partnerID=40&md5=d39c6be59c851e3c6dc9579f984bb4ec VL - 23 ID - 2062 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 (PVO2: % of normal value) from CPX1 to CPX2 (1-dPVO2) and from CPX2 to CPX3 (2-dPVO2) were calculated, and then the patients were divided into four subgroups according the 1-dPVO2 and 2-dPVO2. Results: In their adulthood, when compared with groups with negative 1-dPVO2, the central venous pressure, plasma brain natriuretic peptide level, and renal resistive index were lower, whereas liver synthetic function, body fat-free percentage, and PVO2 were higher in those with positive 1-dPVO2 (p < 0.05–0.0001). However, these favorable associations of 2-d-PVO2 with adult Fontan pathophysiology were not observed, except for the PVO2. After CPX3, 13 unexpected events occurred, and the risk was 76% lower in the groups having positive 1-dPVO2 than in those with negative 1-dPVO2 (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. © 2018 Elsevier B.V. AD - Departments of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan Adult Congenital Heart Disease, 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 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. DB - Scopus DO - 10.1016/j.ijcard.2018.06.067 KW - Adulthood Childhood Exercise capacity Fontan operation Morbidity M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 2019 SP - 80-87 ST - Positive pediatric exercise capacity trajectory predicts better adult Fontan physiology rationale for early establishment of exercise habits T2 - International Journal of Cardiology TI - Positive pediatric exercise capacity trajectory predicts better adult Fontan physiology rationale for early establishment of exercise habits UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85049885701&doi=10.1016%2fj.ijcard.2018.06.067&partnerID=40&md5=b2f752bc12f9a7e491b2963229962bfb VL - 274 ID - 1770 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 - R.G. Ohye, University of Michigan, C. S. Mott Children's Hospital, 1540 E Hospital Dr/SPC 4204, Ann Arbor, MI, United States AU - Ohye, R. G. AU - Schranz, D. AU - D'Udekem, Y. DB - Embase Medline DO - 10.1161/CIRCULATIONAHA.116.022816 IS - 17 KW - dipeptidyl carboxypeptidase inhibitor adolescence adulthood article Blalock Taussig shunt bradycardia catheterization clinical outcome exercise follow up Fontan procedure functional status heart single ventricle heart transplantation human hybrid procedure hypoplastic left heart syndrome kidney failure liver failure long term survival lung blood flow lung disease nerve cell differentiation newborn period Norwood procedure palliative therapy plastic bronchitis pregnancy priority journal protein losing gastroenteropathy psychosocial development quality of life randomized controlled trial (topic) right ventricle to pulmonary artery conduit short term survival surgical approach surgical technique tachycardia thromboembolism LA - English M3 - Article N1 - L612979724 2016-11-04 2016-11-17 PY - 2016 SN - 1524-4539 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L612979724 http://dx.doi.org/10.1161/CIRCULATIONAHA.116.022816 VL - 134 ID - 791 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 - University of Michigan C. S. Mott Children's Hospital, Ann Arbor Pediatric Heart Center, Justus Liebig University Giessen, Germany Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia AN - 119046466. Language: English. Entry Date: 20170430. Revision Date: 20190628. Publication Type: journal article AU - Ohye, Richard G. AU - Schranz, Dietmar AU - D'Udekem, Yves DB - ccm DO - 10.1161/CIRCULATIONAHA.116.022816 DP - EBSCOhost IS - 17 KW - Hypoplastic Left Heart Syndrome -- Pathology Heart Ventricle -- Physiopathology Heart Ventricle -- Surgery Hypoplastic Left Heart Syndrome -- Physiopathology Cardiopulmonary Bypass Hypoplastic Left Heart Syndrome -- Mortality Hypoplastic Left Heart Syndrome -- Surgery Male Survival Multicenter Studies Clinical Trials Prognosis Female N1 - diagnostic images; pictorial; review; tables/charts. Journal Subset: Biomedical; Peer Reviewed; USA. NLM UID: 0147763. PMID: NLM27777296. PY - 2016 SN - 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=119046466&site=ehost-live&scope=site VL - 134 ID - 1562 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. © 2016 American Heart Association, Inc. AD - University of Michigan, C. S. Mott Children's Hospital, 1540 E Hospital Dr/SPC 4204, Ann Arbor, MI 48109-4204, United States Pediatric Heart Center, Justus Liebig University Giessen, Germany Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia AU - Ohye, R. G. AU - Schranz, D. AU - D'Udekem, Y. DB - Scopus DO - 10.1161/CIRCULATIONAHA.116.022816 IS - 17 KW - congenital heart defects heart ventricles hybrid imaging hypoplastic left heart syndrome surgery M3 - Article N1 - Cited By :39 Export Date: 15 June 2020 PY - 2016 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84992533414&doi=10.1161%2fCIRCULATIONAHA.116.022816&partnerID=40&md5=dd96e7bbbdfa78186edbd280cd509dac VL - 134 ID - 1887 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 - Department of Pathology, Faculty of Medicine of Ribeirão Preto, University of São Paulo, Av. Bandeirantes 3900, 14049-900 Ribeirão Preto, SP, Brazil Department of Statistics, Faculty of Medicine of Ribeirão Preto, University of São Paulo, 14049-900 Ribeirão Preto, SP, Brazil Department of Surgery and Anatomy, Faculty of Medicine of Ribeirão Preto, University of São Paulo, 14049-900 Ribeirão Preto, SP, Brazil Department of Histopathology, Sheffield Children's NHS Foundation Trust, Sheffield, United Kingdom 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 - Scopus DO - 10.1016/j.carpath.2010.01.012 IS - 1 KW - Congenital heart disease Infants Ischemia Lipid peroxidation Myocardial injury Oxidative stress M3 - Article N1 - Cited By :13 Export Date: 15 June 2020 PY - 2011 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-78650418119&doi=10.1016%2fj.carpath.2010.01.012&partnerID=40&md5=0f5bd534f2a279b3a38589517c8e582e VL - 20 ID - 2140 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 - Y. Ono, Department of Cardiology, Shizuoka Children’s Hospital, 860 Urushiyama, Aoi-ku, Shizuoka, Japan AU - Ono, Y. AU - Mitsushita, N. DB - Embase Medline DO - 10.1536/ihj.14-353 KW - beraprost bosentan sildenafil angiography aortopulmonary septal defect artery reconstruction article asplenia atrioventricular septal defect case report coronary artery collateral circulation cyanosis Down syndrome Fontan procedure Glenn shunt heart catheterization heart single ventricle home oxygen therapy human infant lung vein drainage anomaly oxygen saturation priority journal protein losing gastroenteropathy pulmonary hypertension pulmonary valve atresia LA - English M3 - Article N1 - L605821660 2015-09-03 2015-09-08 PY - 2015 SN - 1349-3299 1349-2365 SP - S31-S34 ST - Case studies of patients successfully and unsuccessfully managed pre- and post-fontan procedure T2 - International Heart Journal TI - Case studies of patients successfully and unsuccessfully managed pre- and post-fontan procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L605821660 http://dx.doi.org/10.1536/ihj.14-353 VL - 56 ID - 871 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. © 2015, International Heart Journal Association. All rights reserved. AD - Department of Cardiology, Shizuoka Children’s Hospital, Shizuoka, Japan AU - Ono, Y. AU - Mitsushita, N. DB - Scopus DO - 10.1536/ihj.14-353 KW - Beraprost Bosentan Cardiac malformation Fenestration Pulmonary hypertension Sildenafil M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2015 SP - S31-S34 ST - Case studies of patients successfully and unsuccessfully managed pre- and post-fontan procedure T2 - International Heart Journal TI - Case studies of patients successfully and unsuccessfully managed pre- and post-fontan procedure UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84929941021&doi=10.1536%2fihj.14-353&partnerID=40&md5=b164e8b63fc00d009633bd9b3f615512 VL - 56 ID - 1949 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. © 2015 Wiley Periodicals, Inc. AD - Department of Medical Neurobiology, Laboratory of Teratology, Hebrew University Hadassah Medical School, Jerusalem, Israel Departments of Obstetrics, Gynecology and Reproductive Sciences and Biochemistry, Molecular Biology, and Office of the Dean, University of Maryland School of Medicine, United States Laboratory of Molecular Pathogenetics Institute of Biotechnology AS CR, v.v.i., Prague, Czech Republic Department of Developmental Biology, Pennington Biomedical Research Center, Louisiana State University System, Baton Rouge, LA, United States Department of Obstetrics and Gynecology, University of Rochester School of Medicine and Dental Medicine, Rochester, United States AU - Ornoy, A. AU - Reece, E. A. AU - Pavlinkova, G. AU - Kappen, C. AU - Miller, R. K. DB - Scopus DO - 10.1002/bdrc.21090 IS - 1 KW - Congenital anomalies Developmental disorders Diabetic embryopathy Epigenetics GDM PGDM M3 - Article N1 - Cited By :87 Export Date: 15 June 2020 PY - 2015 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84925400339&doi=10.1002%2fbdrc.21090&partnerID=40&md5=516180cccf654efb242aa43cd572df79 VL - 105 ID - 1953 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. © 2015 American Heart Association, Inc. AD - Children's Healthcare, Atlanta, GA, United States Emory University, School of Medicine, 2835 Brandywine Rd, Atlanta, GA 30341, United States Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 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 - Scopus DO - 10.1161/CIRCULATIONAHA.114.014107 IS - 6 KW - heart defects, congenital home care services hypoxia mortality patient readmission M3 - Article N1 - Cited By :27 Export Date: 15 June 2020 PY - 2015 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84939449053&doi=10.1161%2fCIRCULATIONAHA.114.014107&partnerID=40&md5=e76045730a9a5ffc6257663aaba09fe4 VL - 132 ID - 1974 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. © 2009 Copyright © Cambridge University Press 2009. AD - L. Mertens, The Hospital for Sick Children, The Labatt Family Heart Centre, 555 University Avenue, Toronto, ON M5G 1X8, Canada AU - Ovaert, C. AU - Thijs, D. AU - Dewolf, D. AU - Ottenkamp, J. AU - Dessy, H. AU - Moons, P. AU - Gewillig, M. AU - Mertens, L. DB - Embase Medline DO - 10.1017/S1047951109990023 IS - 4 KW - aciclovir amoxicillin anticoagulant agent antithrombocytic agent bosentan dipeptidyl carboxypeptidase inhibitor diuretic agent meprednisone vasodilator agent adolescent adult age distribution article blood chemistry child clinical article clinical trial cyanosis dose response drug tolerability drug withdrawal edema exercise fatigue female fever Fontan procedure heart hemodynamics herpes labialis human ischemia liver function male multicenter study oxygen consumption oxygen saturation protein losing gastroenteropathy quality of life questionnaire side effect tertiary health care treatment failure upper respiratory tract infection venous congestion virus reactivation walking body weight gain LA - English M3 - Article N1 - L50546238 2009-11-04 PY - 2009 SN - 1047-9511 1467-1107 SP - 331-339 ST - The effect of bosentan in patients with a failing Fontan circulation T2 - Cardiology in the Young TI - The effect of bosentan in patients with a failing Fontan circulation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50546238 http://dx.doi.org/10.1017/S1047951109990023 VL - 19 ID - 1167 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. © 2009 Copyright © Cambridge University Press 2009. AD - Department of Pediatric Cardiology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium Department of Pediatric Cardiology, University Hospitals Leuven, Leuven, Belgium Department of Pediatric Cardiology, Universitair Ziekenhuis Gent, Gent, Belgium Department of Pediatric Cardiology, University Medical Centre, Leiden, Netherlands Hôpital Universitaire des Enfants Reine Fabiola, Université Libre de Bruxelles, Brussels, Belgium Center for Health Services and Nursing Research, University Hospitals Leuven, Leuven, Belgium The Hospital for Sick Children, The Labatt Family Heart Centre, 555 University Avenue, Toronto, ON M5G 1X8, Canada AU - Ovaert, C. AU - Thijs, D. AU - Dewolf, D. AU - Ottenkamp, J. AU - Dessy, H. AU - Moons, P. AU - Gewillig, M. AU - Mertens, L. DB - Scopus DO - 10.1017/S1047951109990023 IS - 4 KW - Congenital heart defect Endothelin receptor antagonist Functionally single ventricle Pulmonary vascular resistance M3 - Article N1 - Cited By :66 Export Date: 15 June 2020 PY - 2009 SP - 331-339 ST - The effect of bosentan in patients with a failing Fontan circulation T2 - Cardiology in the Young TI - The effect of bosentan in patients with a failing Fontan circulation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-70349221143&doi=10.1017%2fS1047951109990023&partnerID=40&md5=4b12ae8f4ede6669a35880a145a743b7 VL - 19 ID - 2176 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 - D. Overgaard, Heart Centre, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark. AU - Overgaard, D. AU - King, C. AU - Christensen, R. F. AU - Schrader, A. M. AU - Adamsen, L. DB - Medline IS - 2 KW - adaptive behavior adolescent adult article congenital malformation daily life activity Denmark female Fontan procedure heart ventricle human male middle aged psychological aspect quality of life survivor LA - English M3 - Article N1 - L369465551 2013-08-09 PY - 2013 SN - 1550-5049 SP - 187-196 ST - Living with half a heart--experiences of young adults with single ventricle physiology: a qualitative study T2 - The Journal of cardiovascular nursing TI - Living with half a heart--experiences of young adults with single ventricle physiology: a qualitative study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L369465551 VL - 28 ID - 1014 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. © 2011 S. Karger AG, Basel. AD - Heart Centre, Copenhagen University Hospital, Denmark University College Metropol, Copenhagen, Denmark Department of Cardiology, Aarhus University, Skejby, Aarhus, Denmark Centre of Health Services and Nursing Research, Katholieke Universiteit Leuven, Leuven, Belgium Copenhagen University Hospital, Rigshospitalet, Department 2151, Blegdamsvej 9, DK-2100 Copenhagen O, Denmark 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 - Scopus DO - 10.1159/000333112 IS - 1 KW - Congenital heart disease Patient-reported outcomes Perceived health Quality of life Single-ventricle physiology M3 - Article N1 - Cited By :25 Export Date: 15 June 2020 PY - 2011 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-81155148735&doi=10.1159%2f000333112&partnerID=40&md5=320a6dde50ce477af9dbc204b9fde9ef VL - 120 ID - 2107 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. © 2013 Wiley Periodicals, Inc. AD - D. Overgaard, Nordsjaellands Hospital, Lung-and Infection Medical Section, Dyrehavevej 29, 3400 Hillerod, Denmark 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 - Embase Medline DO - 10.1111/chd.12086 IS - 1 KW - cannabis adult alcohol consumption article binge drinking clinical article congenital heart malformation controlled study female human male mouth hygiene physical activity priority journal questionnaire single ventricle physiology smoking substance use young adult LA - English M3 - Article N1 - L52585699 2013-05-17 2014-02-04 PY - 2014 SN - 1747-079X 1747-0803 SP - 75-82 ST - Substance use, dental hygiene, and physical activity in adult patients with single ventricle physiology T2 - Congenital Heart Disease TI - Substance use, dental hygiene, and physical activity in adult patients with single ventricle physiology UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52585699 http://dx.doi.org/10.1111/chd.12086 VL - 9 ID - 940 ER - TY - JOUR AN - 60836230. Language: English. Entry Date: 20130312. Revision Date: 20170203. Publication Type: Abstract 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=60836230&site=ehost-live&scope=site VL - 10 ID - 1651 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 - Medical College of Wisconsin, Pediatrics, Critical Care, Milwaukee, WI, United States Medical College of Wisconsin, Pediatrics, Critical Care, 9000 W. Wisconsin Ave, Milwaukee, WI 53226, United States AU - Owens, J. L. AU - Musa, N. DB - Scopus DO - 10.1177/0884533609332086 IS - 2 KW - Cardiac surgical procedures Enteral nutrition Infant Newborn Nutritional support Parenteral nutrition M3 - Article N1 - Cited By :50 Export Date: 15 June 2020 PY - 2009 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-63849310171&doi=10.1177%2f0884533609332086&partnerID=40&md5=3c9771383d59d55eec9c690e8a4d15ca VL - 24 ID - 2181 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 - School of Nursing, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States of America School of Nursing, Duke University, Durham, North Carolina, United States of America School of Nursing, Boston College, Chestnut Hill, Massachusetts, United States of America AN - 121745189. Language: English. Entry Date: 20170324. Revision Date: 20190308. Publication Type: journal article AU - Pados, Britt F. AU - Thoyre, Suzanne M. AU - Estrem, Hayley H. AU - Park, Jinhee AU - Knafl, George J. AU - Nix, Brant DB - ccm DO - 10.1017/S1047951116000251 DP - EBSCOhost IS - 1 KW - Hypoplastic Left Heart Syndrome -- Rehabilitation Milk, Human Bottle Feeding -- Methods Heart Rate -- Physiology Infant Behavior Hypoplastic Left Heart Syndrome -- Physiopathology Female Infant, Newborn Male Infant Hypoplastic Left Heart Syndrome -- Psychosocial Factors Human N1 - research; tables/charts. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. NLM UID: 9200019. PMID: NLM26982280. PY - 2017 SN - 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=121745189&site=ehost-live&scope=site VL - 27 ID - 1497 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. © Cambridge University Press 2016. AD - School of Nursing, University of North Carolina at Chapel Hill, Carrington Hall, CB#7460, Chapel Hill, NC 27599-7460, United States School of Nursing, Duke University, Durham, NC, United States School of Nursing, Boston College, Chestnut Hill, MA, 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 - Scopus DO - 10.1017/S1047951116000251 IS - 1 KW - bottle feeding feeding methods Hypoplastic left heart syndrome M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 2017 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84961204175&doi=10.1017%2fS1047951116000251&partnerID=40&md5=5e1ded3c0760b3f62141f8c37824a9a2 VL - 27 ID - 1877 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 - C. Panchangam, University of Missouri-Columbia, 500 N Keene St, Suite 207, Columbia, MO, United States 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. DB - Embase Medline DO - 10.1007/s00246-020-02354-7 KW - adolescent adverse outcome aged article body composition body mass cardiomyopathy child congenital heart disease controlled study exhaustion female frailty gender grip strength heart single ventricle human longitudinal study major clinical study male outcome assessment physical activity prospective study pulmonary hypertension quantitative analysis questionnaire school child six minute walk test skinfold thickness slowness triceps brachii muscle weakness LA - English M3 - Article in Press N1 - L2004853080 2020-05-26 PY - 2020 SN - 1432-1971 0172-0643 ST - Translation of the Frailty Paradigm from Older Adults to Children with Cardiac Disease T2 - Pediatric Cardiology TI - Translation of the Frailty Paradigm from Older Adults to Children with Cardiac Disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2004853080 http://dx.doi.org/10.1007/s00246-020-02354-7 ID - 573 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. © 2020, Springer Science+Business Media, LLC, part of Springer Nature. AD - Department of Child Health, University of Missouri Health Care, Columbia, MO, United States The Ward Family Heart Center, Children’s Mercy Hospital, Kansas City, MO, United States UMKC School of Medicine, Kansas City, MO, United States University of Missouri-Columbia, 500 N Keene St, Suite 207, Columbia, MO 65201, United States 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. DB - Scopus DO - 10.1007/s00246-020-02354-7 KW - Cardiomyopathy Children with heart disease Congenital heart disease Fontan Frailty Heart failure Pulmonary hypertension Quality and outcomes M3 - Article N1 - Export Date: 15 June 2020 PY - 2020 ST - Translation of the Frailty Paradigm from Older Adults to Children with Cardiac Disease T2 - Pediatric Cardiology TI - Translation of the Frailty Paradigm from Older Adults to Children with Cardiac Disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85085105071&doi=10.1007%2fs00246-020-02354-7&partnerID=40&md5=2653a5be009d03f2a847608dbc57ff89 ID - 1716 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. © 2017, Springer Science+Business Media New York. AD - Division of Pediatric Cardiology, Indiana University School of Medicine – Riley Hospital, Indiana University Health, 705 Riley Hospital Drive, RR 127, Indianapolis, IN 46202, United States Division of Pediatric Cardiology, Ann and Robert H Lurie Children’s Hospital of Chicago, Chicago, United States The Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, United States The Heart Institute, Le Bonheur Children’s Hospital, Memphis, 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 - Scopus DO - 10.1007/s00246-017-1568-y IS - 5 KW - Fontan Pediatric heart transplantation Plastic bronchitis Single ventricle M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2017 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85009727739&doi=10.1007%2fs00246-017-1568-y&partnerID=40&md5=e8eafb6570341bf527f3c13bdd7d6d9b VL - 38 ID - 1858 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. © 2012 Nature America, Inc. All rights reserved. AD - J.J. Paris, S.J. Walsh Department of Bioethics, Boston College, Chestnut Hill, MA 02467, United States AU - Paris, J. J. AU - Moore, M. P. AU - Schreiber, M. D. DB - Embase Medline DO - 10.1038/jp.2012.72 IS - 10 KW - article cardiopulmonary bypass child care childhood mortality counseling decision making health care quality heart transplantation human hypoplastic left heart syndrome infant informed consent morbidity motor performance Norwood procedure outcome assessment parental decision making physician counseling quality of life survival rate LA - English M3 - Article N1 - L52049141 2012-06-15 2012-10-15 PY - 2012 SN - 0743-8346 1476-5543 SP - 748-751 ST - Physician counseling, informed consent and parental decision making for infants with hypoplastic left-heart syndrome T2 - Journal of Perinatology TI - Physician counseling, informed consent and parental decision making for infants with hypoplastic left-heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52049141 http://dx.doi.org/10.1038/jp.2012.72 VL - 32 ID - 1040 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. © 2012 Nature America, Inc. All rights reserved. AD - S.J. Walsh Department of Bioethics, Boston College, Chestnut Hill, MA 02467, United States Center for Clinical Ethics, Stanford University School of Medicine, Stanford, CA, United States J. D. Boston College, School of Law, Newton, MA, United States Department of Pediatrics, University of Chicago, Chicago, IL, United States AU - Paris, J. J. AU - Moore, M. P. AU - Schreiber, M. D. DB - Scopus DO - 10.1038/jp.2012.72 IS - 10 KW - hypoplastic left heart syndrome informed consent parental decision making physician counseling M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2012 SP - 748-751 ST - Physician counseling, informed consent and parental decision making for infants with hypoplastic left-heart syndrome T2 - Journal of Perinatology TI - Physician counseling, informed consent and parental decision making for infants with hypoplastic left-heart syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84867058418&doi=10.1038%2fjp.2012.72&partnerID=40&md5=3f7e6e46a79a7d85249462433e99349b VL - 32 ID - 2083 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 - H.J. Shin, Department of Thoracic and Cardiovascular Surgery, College of Medicine, Chungbuk National University, Cheongju, South Korea AU - Park, H. K. AU - Shin, H. J. AU - Park, Y. H. DB - Embase Medline DO - 10.1093/icvts/ivw062 IS - 1 KW - adolescent adult article atrioventricular valve regurgitation cardiopulmonary bypass cavopulmonary connection child clinical article female follow up Fontan procedure great vessels transposition heart atrium arrhythmia heart atrium enlargement heart atrium thrombosis heart right atrium human male New York Heart Association class outcome assessment pleura effusion postoperative period priority journal protein losing gastroenteropathy recurrent disease reoperation retrospective study survival rate treatment failure tricuspid valve atresia valvular heart disease LA - English M3 - Article N1 - L611320837 2016-07-26 2016-08-05 PY - 2016 SN - 1569-9285 1569-9293 SP - 14-17 ST - Outcomes of Fontan conversion for failing Fontan circulation: Mid-term results T2 - Interactive Cardiovascular and Thoracic Surgery TI - Outcomes of Fontan conversion for failing Fontan circulation: Mid-term results UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L611320837 http://dx.doi.org/10.1093/icvts/ivw062 VL - 23 ID - 803 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. © 2016 The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. AD - Division of Cardiovascular Surgery, Department of Thoracic and Cardiovascular Surgery, Severance Cardiovascular Hospital, Yonsei University Health System, Seoul, South Korea Department of Thoracic and Cardiovascular Surgery, College of Medicine, Chungbuk National University, Cheongju, South Korea AU - Park, H. K. AU - Shin, H. J. AU - Park, Y. H. DB - Scopus DO - 10.1093/icvts/ivw062 IS - 1 KW - Fontan operation M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 2016 SP - 14-17 ST - Outcomes of Fontan conversion for failing Fontan circulation: Mid-term results T2 - Interactive Cardiovascular and Thoracic Surgery TI - Outcomes of Fontan conversion for failing Fontan circulation: Mid-term results UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84978698978&doi=10.1093%2ficvts%2fivw062&partnerID=40&md5=d0d530a5d805a5760602e0ecc20bd3fc VL - 23 ID - 1897 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 - M. Nakazawa, Department of Pediatric Cardiology, Heart Institute of Japan, Tokyo Women's Medical College, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162, Japan AU - Park, I. AU - Nakazawa, M. AU - Imai, Y. AU - Sawatari, K. AU - Momma, K. DB - Embase Medline IS - 8 KW - adolescent adult article child clinical article clinical trial follow up Fontan procedure great vessels transposition heart atrium septum defect heart right ventricle double outlet heart single ventricle human hypoplastic left heart syndrome quality of life tricuspid valve atresia LA - English M3 - Article N1 - L24274251 1994-09-19 PY - 1994 SN - 0047-1828 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 - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L24274251 VL - 58 ID - 1372 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. © 2018, Springer Science+Business Media, LLC, part of Springer Nature. 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, United States Feinberg School of Medicine, Northwestern University, Chicago, IL, United States University of California San Francisco Benioff Children’s Hospitals, San Francisco, CA, United States AU - Patel, A. AU - Michelson, K. AU - Andrei, A. C. AU - Pahl, E. AU - Gossett, J. G. DB - Scopus DO - 10.1007/s00246-018-1965-x IS - 1 KW - Ethics Heart transplantation Pediatric M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2019 SP - 101-109 ST - Variations in Criteria and Practices for Heart Transplantation Listing Among Pediatric Transplant Cardiologists T2 - Pediatric Cardiology TI - Variations in Criteria and Practices for Heart Transplantation Listing Among Pediatric Transplant Cardiologists UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85052227087&doi=10.1007%2fs00246-018-1965-x&partnerID=40&md5=185bccbb9f1371df96b22753c90f36c3 VL - 40 ID - 1767 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 - M.H. Patil, University at Buffalo, Department of Pulmonary Critical Care Medicine, Buffalo, NY, United States AU - Patil, M. H. AU - Siddiqi, A. AU - Jeffrey Mador, M. DB - Embase DO - 10.1016/j.rmcr.2016.03.003 KW - propofol adult article bronchitis bronchoscopy case report coughing dyspnea female human hypoxia medical history plastic bronchitis pregnant woman priority journal respiratory distress risk benefit analysis young adult LA - English M3 - Article N1 - L609079139 2016-03-28 2016-04-01 PY - 2016 SN - 2213-0071 SP - 8-9 ST - Successful bronchoscopy in a pregnant patient with plastic bronchitis T2 - Respiratory Medicine Case Reports TI - Successful bronchoscopy in a pregnant patient with plastic bronchitis UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L609079139 http://dx.doi.org/10.1016/j.rmcr.2016.03.003 VL - 18 ID - 836 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. © 2016 The Authors. AD - University at Buffalo, Department of Pulmonary Critical Care Medicine, Buffalo, NY, United States Buffalo General Medical Center, Department of Critical Care Medicine, Buffalo, NY, United States Veteran Affairs Western New York Health Care System-Buffalo Division, Department of Pulmonary and Critical Care Medicine, Buffalo, NY, United States AU - Patil, M. H. AU - Siddiqi, A. AU - Jeffrey Mador, M. DB - Scopus DO - 10.1016/j.rmcr.2016.03.003 KW - Bronchoscope Plastic bronchitis Pregnancy Propofol M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2016 SP - 8-9 ST - Successful bronchoscopy in a pregnant patient with plastic bronchitis T2 - Respiratory Medicine Case Reports TI - Successful bronchoscopy in a pregnant patient with plastic bronchitis UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84961204858&doi=10.1016%2fj.rmcr.2016.03.003&partnerID=40&md5=242e07562394a62f2f0b90fe76e40bbb VL - 18 ID - 1929 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 - Department of Cardiac Surgery, Heart Transplantation and Mechanical Circulatory Support for Children, Silesian Centre for Heart Diseases, Zabrze, Poland. Department of Cardiac Surgery and Cardiac Intensive Care, University Children’s Hospital, Krakow, Poland. Department of Cardiac Surgery, Children’s Memorial Health Institute, Warsaw, Poland. Department of Congenital Heart Diseases and Paediatric Cardiology, Silesian Centre for Heart Diseases, Medical University of Silesia, Zabrze, Poland. Chair and Clinical Department of Cardiac Surgery, Transplantation, Vascular, and Endovascular Surgery, Silesian Centre for Heart Diseases, Silesian Medical University, Zabrze, Poland. AN - 127807962. Language: English. Entry Date: 20180210. Revision Date: 20180210. Publication Type: Article AU - Pawlak, Szymon AU - Przybylski, Roman AU - Skalski, Janusz AU - Śliwka, Joanna AU - Kansy, Andrzej AU - Grzybowski, Adam AU - Wierzyk, Arkadiusz AU - Białkowski, Jacek AU - Maruszewski, Bohdan AU - Zembala, Marian DB - ccm DO - 10.5603/KP.a2017.0201 DP - EBSCOhost IS - 1 KW - Heart Failure -- Surgery -- In Infancy and Childhood Heart Assist Devices -- Trends -- Poland Human Poland Infant Child, Preschool Child Adolescence Myocardial Diseases Cardiopulmonary Bypass Treatment Duration Heart Transplantation Regeneration Thromboembolism -- Complications Heart Failure -- Mortality Patient Compliance N1 - pictorial; research; tables/charts. Journal Subset: Biomedical; Continental Europe; Europe. NLM UID: 0376352. PY - 2018 SN - 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 - Polish Heart Journal / 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=127807962&site=ehost-live&scope=site VL - 76 ID - 1466 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 - Department of Pediatrics, University of Montral, Montreal, QC, Canada Clinical Ethics Unit, Sainte Justine University, Health Center, Montreal, QC, Canada Department of Neonatology, Sainte Justine University, Health Center, Montreal, QC, Canada AU - Payot, A. AU - Barrington, K. J. DB - Scopus DO - 10.1016/j.cppeds.2010.10.008 IS - 4 M3 - Article N1 - Cited By :53 Export Date: 15 June 2020 PY - 2011 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-79953082890&doi=10.1016%2fj.cppeds.2010.10.008&partnerID=40&md5=8b897e681ca440c3e1a221d57f05fb7f VL - 41 ID - 2128 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. AU - Peddy, S. B. DB - Medline DO - 10.15766/mep_2374-8265.10706 KW - anatomy and histology complication congenital heart malformation curriculum cyanosis education female heart human hypoxia infant male medical education newborn pathophysiology patient simulation pediatrics procedures resuscitation LA - English M3 - Article N1 - L628782060 2019-08-07 2019-11-07 PY - 2018 SN - 2374-8265 SP - 10706 ST - Acute Hypoxemia in Infants With Cyanotic Complex Cardiac Anatomy: Simulation Cases for Pediatric Fellows T2 - MedEdPORTAL : the journal of teaching and learning resources TI - Acute Hypoxemia in Infants With Cyanotic Complex Cardiac Anatomy: Simulation Cases for Pediatric Fellows UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L628782060 http://dx.doi.org/10.15766/mep_2374-8265.10706 VL - 14 ID - 690 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 - Department of Anesthesiology and Critical Care, Perelman School of Medicine at the University of Pennsylvania Division of Cardiac Critical Care Medicine, Children's Hospital of Philadelphia AU - Peddy, S. B. DB - Scopus DO - 10.15766/mep_2374-8265.10706 KW - Hypoplastic Left Heart Syndrome Hypoxemia Hypoxia Tetralogy of Fallot Transposition of Great Arteries Transposition of Great Vessels M3 - Article N1 - Export Date: 15 June 2020 PY - 2018 SP - 10706 ST - Acute Hypoxemia in Infants With Cyanotic Complex Cardiac Anatomy: Simulation Cases for Pediatric Fellows T2 - MedEdPORTAL : the journal of teaching and learning resources TI - Acute Hypoxemia in Infants With Cyanotic Complex Cardiac Anatomy: Simulation Cases for Pediatric Fellows UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85074378424&doi=10.15766%2fmep_2374-8265.10706&partnerID=40&md5=983ec1acf75369a48697aa90bd288126 VL - 14 ID - 1808 ER - TY - JOUR AD - Dept. Otolaryngol./Head/Neck Surgery, Shaare Zedek Medical Center, P.O. Box 3235, Jerusalem 91031, Israel Department of Pediatrics, Shaare Zedek Medical Center, P.O. Box 3235, Jerusalem 91031, Israel Division of Pediatric Pulmonology, Shaare Zedek Medical Center, P.O. Box 3235, Jerusalem 91031, Israel Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheva, Israel AU - Peleg, U. AU - Schwartz, S. AU - Sirota, G. AU - Hochman, I. AU - Cohen, D. AU - Picard, E. DB - Scopus IS - 2 KW - Bronchoscopy Children Fontan procedure Plastic bronchitis M3 - Article N1 - Cited By :6 Export Date: 15 June 2020 PY - 2005 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-13844314051&partnerID=40&md5=4cced9bb713729622c1b250cb8fa9b34 VL - 7 ID - 2240 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 - O. Stumper, Heart Unit, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, United Kingdom AU - Penford, G. AU - Quandt, D. AU - Stumper, O. DB - Embase Medline DO - 10.1017/S1047951115001262 IS - 3 KW - dobutamine article case report child clinical feature disease association female fenestration follow up Fontan procedure heart disease heart left atrium pressure heart right atrium pressure heart surgery human hypoplastic left heart syndrome male medical history patient assessment pharmacologic stress testing pleura effusion postoperative complication postoperative period preoperative period preschool child protein losing gastroenteropathy restrictive atrial septum school child transthoracic echocardiography treatment response Genesis LA - English M3 - Article N1 - L610769282 2016-06-16 2016-06-22 PY - 2016 SN - 1467-1107 1047-9511 SP - 574-578 ST - Restrictive atrial septum after the Fontan procedure T2 - Cardiology in the Young TI - Restrictive atrial septum after the Fontan procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L610769282 http://dx.doi.org/10.1017/S1047951115001262 VL - 26 ID - 821 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 - 115588343. Language: English. Entry Date: 20161229. Revision Date: 20190517. Publication Type: journal article AU - Penford, Gemma AU - Quandt, Daniel AU - Stumper, Oliver DB - ccm DO - 10.1017/S1047951115001262 DP - EBSCOhost IS - 3 KW - Postoperative Complications -- Surgery Hypoplastic Left Heart Syndrome -- Surgery Cardiopulmonary Bypass -- Adverse Effects Heart Septum -- Physiopathology Venous Pressure Intestinal Diseases -- Therapy Child Reoperation Child, Preschool Treatment Outcomes Intestinal Diseases -- Etiology Human Heart Septum -- Surgery Female Male Pulmonary Veins -- Physiopathology Exercise Test Pulmonary Veins -- Surgery Hypoplastic Left Heart Syndrome -- Etiology Validation Studies Comparative Studies Evaluation Research Multicenter Studies N1 - case study; research. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. NLM UID: 9200019. PMID: NLM26175163. PY - 2016 SN - 1047-9511 SP - 574-578 ST - Restrictive atrial septum after the Fontan procedure T2 - Cardiology in the Young TI - Restrictive atrial septum after the Fontan procedure UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=115588343&site=ehost-live&scope=site VL - 26 ID - 1501 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. ©Cambridge University Press 2015. AD - Heart Unit, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, B4 6NH, United Kingdom AU - Penford, G. AU - Quandt, D. AU - Stumper, O. DB - Scopus DO - 10.1017/S1047951115001262 IS - 3 KW - Fontan procedure hypoplastic left heart syndrome protein-losing enteropathy stent M3 - Article N1 - Export Date: 15 June 2020 PY - 2016 SP - 574-578 ST - Restrictive atrial septum after the Fontan procedure T2 - Cardiology in the Young TI - Restrictive atrial septum after the Fontan procedure UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84973549916&doi=10.1017%2fS1047951115001262&partnerID=40&md5=7b31329ce004a729d4c5913b33f9e6b4 VL - 26 ID - 1919 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. © Copyright 2016 Physicians Postgraduate Press, Inc. AD - Department of Primary Care and Population Health, University College London, Rowland Hill St, London, NW3 2PF, United Kingdom Institute of Child Health, University College London, United Kingdom Department of Medical Statistics, London School of Hygiene and Medical Statistics, United Kingdom AU - Petersen, I. AU - Evans, S. J. AU - Gilbert, R. AU - Marston, L. AU - Nazareth, I. DB - Scopus DO - 10.4088/JCP.14m09241 IS - 1 M3 - Article N1 - Cited By :20 Export Date: 15 June 2020 PY - 2016 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84957801282&doi=10.4088%2fJCP.14m09241&partnerID=40&md5=630990d19677634a9ef463e66791e374 VL - 77 ID - 1930 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. © 2012 The Author and 2012 Royal Australasian College of Physicians. AD - Royal Children’s Hospital Melbourne, Melbourne, VIC, Australia University of Melbourne, Melbourne, VIC, Australia AU - Pflaumer, A. DB - Scopus DO - 10.1111/j.1445-5994.2012.02899.x KW - Ablation technique Arrhythmia Cardiac Child Congenital Electrophysiology Heart defect M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2012 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84880882721&doi=10.1111%2fj.1445-5994.2012.02899.x&partnerID=40&md5=89ea7303d38593010890c0db46dd94a3 VL - 42 ID - 2103 ER - TY - JOUR AB - 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. 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. 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. 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. 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 - N.A. Pike, School of Nursing, University of California, Los Angeles, CA 90095-6919, USA. AU - Pike, N. A. AU - Evangelista, L. S. AU - Doering, L. V. AU - Eastwood, J. A. AU - Lewis, A. B. AU - Child, J. S. DB - Medline IS - 6 KW - adolescent adult article comparative study cross-sectional study depression female Fontan procedure health status human male quality of life questionnaire retrospective study LA - English M3 - Article N1 - L366395487 2013-04-10 PY - 2012 SN - 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 - The 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://www.embase.com/search/results?subaction=viewrecord&from=export&id=L366395487 VL - 27 ID - 1036 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, selfesteem, 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 selfesteem between the two groups, female patients with SVCHD\21 years of age reported lower perceived bodyimage. 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. © Springer Science+Business Media, LLC 2012. AD - N.A. Pike, School of Nursing, University of California, Factor Building Room 3-938, Los Angeles, CA 90095-6919, United States AU - Pike, N. A. AU - Evangelista, L. S. AU - Doering, L. V. AU - Eastwood, J. A. AU - Lewis, A. B. AU - Child, J. S. DB - Embase Medline DO - 10.1007/s00246-012-0198-7 IS - 5 KW - adolescent adult age article body height body image body mass body weight controlled study face female heart single ventricle heart surgery human major clinical study male medical record review muscle tone questionnaire self esteem sex difference thorax trunk LA - English M3 - Article N1 - L51851342 2012-02-09 2012-07-31 PY - 2012 SN - 0172-0643 1432-1971 SP - 705-712 ST - Sex and age differences in body-image, Self-Esteem, and Body mass index in adolescents and adults after single-ventricle palliation T2 - Pediatric Cardiology TI - Sex and age differences in body-image, Self-Esteem, and Body mass index in adolescents and adults after single-ventricle palliation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51851342 http://dx.doi.org/10.1007/s00246-012-0198-7 VL - 33 ID - 1059 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 - Nancy A. Pike, PhD, RN, CPNP-AC Assistant Professor, School of Nursing, University of California, Los Angeles. Lorraine S. Evangelista, PhD, RN Associate Professor, Program of Nursing Science, University of California, Irvine. Lynn V. Doering, PhD, RN Professor, School of Nursing, University of California, Los Angeles. Jo-Ann Eastwood, PhD, RN Assistant Professor, School of Nursing, University of California, Los Angeles. Alan B. Lewis, MD Pediatric Cardiologist, Division of Pediatric Cardiology, Children's Hospital Los Angeles. John S. Child, MD Cardiologist and Director, Adult Congenital Heart Disease Center, Ahmanson-University of California, Los Angeles. AN - 108106913. Language: English. Entry Date: 20121123. Revision Date: 20150818. Publication Type: Journal Article AU - Pike, N. A. AU - Evangelista, L. S. AU - Doering, L. V. AU - Eastwood, J. A. AU - Lewis, A. B. AU - Child, J. S. DB - ccm DP - EBSCOhost IS - 6 KW - Depression Health Status Heart Defects, Congenital -- Surgery Quality of Life Treatment Outcomes Adolescence Adult Analysis of Variance California Comparative Studies Control Group Convenience Sample Cross Sectional Studies Depression -- Epidemiology Descriptive Research Descriptive Statistics Female Health Status -- Evaluation Heart Defects, Congenital -- Classification Hospitals -- California Human Male Medical Records Mental Health -- Evaluation Multiple Linear Regression Quality of Life -- Evaluation Questionnaires Record Review Scales Severity of Illness Short Form-36 Health Survey (SF-36) Support, Psychosocial -- Evaluation Treatment Outcomes -- Evaluation N1 - research; tables/charts. Journal Subset: Core Nursing; Nursing; Peer Reviewed; USA. Instrumentation: Short Form-36 Health Survey (SF-36); Multidimensional Scale of Perceived Social Support (MSPSS); Satisfaction with Life Scale (SWLS); Patient Health Questionnaire (PHQ). NLM UID: 8703516. PMID: NLM21912272. PY - 2012 SN - 0889-4655 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=ccm&AN=108106913&site=ehost-live&scope=site VL - 27 ID - 1502 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. Copyright © 2012 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. AD - School of Nursing, University of California, Los Angeles, Factor Bldg, Los Angeles, CA 90095-6919, United States Program of Nursing Science, University of California, Irvine, United States Division of Pediatric Cardiology, Children's Hospital Los Angeles, United States Adult Congenital Heart Disease Center, Ahmanson-University of California, Los Angeles, United States AU - Pike, N. A. AU - Evangelista, L. S. AU - Doering, L. V. AU - Eastwood, J. A. AU - Lewis, A. B. AU - Child, J. S. DB - Scopus DO - 10.1097/JCN.0b013e31822ce5f6 IS - 6 KW - depression Fontan health status quality of life single ventricle congenital heart disease M3 - Article N1 - Cited By :36 Export Date: 15 June 2020 PY - 2012 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84867880707&doi=10.1097%2fJCN.0b013e31822ce5f6&partnerID=40&md5=2081a942e6346404198956b74e7f8cbd VL - 27 ID - 2080 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, selfesteem, 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 selfesteem between the two groups, female patients with SVCHD\21 years of age reported lower perceived bodyimage. 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. © Springer Science+Business Media, LLC 2012. AD - School of Nursing, University of California, Factor Building Room 3-938, Los Angeles, CA 90095-6919, United States Program of Nursing Science, University of California, Irvine, CA, United States Division of Pediatric Cardiology, Children's Hospital Los Angeles, Los Angeles, CA, United States Ahmanson/UCLA Adult Congenital Heart Disease Center, Los Angeles, CA, United States AU - Pike, N. A. AU - Evangelista, L. S. AU - Doering, L. V. AU - Eastwood, J. A. AU - Lewis, A. B. AU - Child, J. S. DB - Scopus DO - 10.1007/s00246-012-0198-7 IS - 5 KW - Body mass index Body-image Self-esteem Single-ventricle congenital heart disease. M3 - Article N1 - Cited By :9 Export Date: 15 June 2020 PY - 2012 SP - 705-712 ST - Sex and age differences in body-image, Self-Esteem, and Body mass index in adolescents and adults after single-ventricle palliation T2 - Pediatric Cardiology TI - Sex and age differences in body-image, Self-Esteem, and Body mass index in adolescents and adults after single-ventricle palliation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84863981232&doi=10.1007%2fs00246-012-0198-7&partnerID=40&md5=a38d471dd178bccab3f934b337957491 VL - 33 ID - 2094 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. © 2007 Mosby, Inc. All rights reserved. AD - N.A. Pike, Division of Cardiothoracic Surgery, Childrens Hospital Los Angeles, Los Angeles, United States AU - Pike, N. A. AU - Evangelista, L. S. AU - Doering, L. V. AU - Koniak-Griffin, D. AU - Lewis, A. B. AU - Child, J. S. DB - Embase Medline DO - 10.1016/j.hrtlng.2006.06.002 IS - 1 KW - article cognitive development congenital heart disease Fontan procedure functional status heart single ventricle human measurement nerve cell differentiation outcome assessment priority journal quality of life self report LA - English M3 - Article N1 - L46096592 2007-02-16 PY - 2007 SN - 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 and Lung: Journal of Acute and Critical Care TI - Health-related quality of life: A closer look at related research in patients who have undergone the Fontan operation over the last decade UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L46096592 http://dx.doi.org/10.1016/j.hrtlng.2006.06.002 VL - 36 ID - 1243 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, 4650 Sunset Blvd, Mail Stop #66, Los Angeles, CA 90027 AN - 106262415. Language: English. Entry Date: 20070406. Revision Date: 20150818. Publication Type: Journal Article AU - Pike, N. A. AU - Evangelista, L. S. AU - Doering, L. V. AU - Koniak-Griffin, D. AU - Lewis, A. B. AU - Child, J. S. DB - ccm DP - EBSCOhost IS - 1 KW - Heart Defects, Congenital -- Surgery Quality of Life -- In Adolescence Quality of Life -- In Adulthood Treatment Outcomes Adolescence Adult Child Child, Preschool Clinical Research Heart Surgery -- Methods Infant Infant, Newborn N1 - review; tables/charts. Journal Subset: Core Nursing; Nursing; Peer Reviewed; USA. NLM UID: 0330057. PMID: NLM17234472. PY - 2007 SN - 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106262415&site=ehost-live&scope=site VL - 36 ID - 1637 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. © 2007 Mosby, Inc. All rights reserved. AD - Division of Cardiothoracic Surgery, Childrens Hospital Los Angeles, Los Angeles, United States University of California, Los Angeles, School of Nursing, Los Angeles, United States Division of Pediatric Cardiology, Childrens Hospital Los Angeles, Los Angeles, United States Division of Cardiology, University of California, Los Angeles, United States AU - Pike, N. A. AU - Evangelista, L. S. AU - Doering, L. V. AU - Koniak-Griffin, D. AU - Lewis, A. B. AU - Child, J. S. DB - Scopus DO - 10.1016/j.hrtlng.2006.06.002 IS - 1 M3 - Article N1 - Cited By :17 Export Date: 15 June 2020 PY - 2007 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 and Lung: Journal of Acute and Critical Care TI - Health-related quality of life: A closer look at related research in patients who have undergone the Fontan operation over the last decade UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-33846208380&doi=10.1016%2fj.hrtlng.2006.06.002&partnerID=40&md5=96d162866a9e72723842672bd526a5bd VL - 36 ID - 2216 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 © 2011 Copyright the Authors. Congenital Heart Disease © 2011 Wiley Periodicals, Inc. AD - N.A. Pike, UCLA School of Nursing, Factor Building, Los Angeles, CA 90095-6919, United States AU - Pike, N. A. AU - Evangelista, L. S. AU - Doering, L. V. AU - Koniak-Griffin, D. AU - Lewis, A. B. AU - Child, J. S. DB - Embase Medline DO - 10.1111/j.1747-0803.2010.00475.x IS - 1 KW - adolescent adult article ascites cross-sectional study female Fontan procedure headache heart arrhythmia heart left ventricle ejection fraction heart single ventricle human liver cirrhosis major clinical study male outpatient oxygen saturation priority journal psychosocial care quality of life questionnaire scoliosis survivor symptomatology tricuspid valve atresia varicosis LA - English M3 - Article N1 - L361175748 2011-02-02 2011-02-15 PY - 2011 SN - 1747-079X 1747-0803 SP - 9-17 ST - Clinical profile of the adolescent/adult fontan survivor T2 - Congenital Heart Disease TI - Clinical profile of the adolescent/adult fontan survivor UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L361175748 http://dx.doi.org/10.1111/j.1747-0803.2010.00475.x VL - 6 ID - 1120 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 © 2011 Copyright the Authors. Congenital Heart Disease © 2011 Wiley Periodicals, Inc. AD - School of Nursing, University of California, Los Angeles, CA, United States Division of Pediatric Cardiology, Childrens Hospital Los Angeles, Los Angeles, CA, United States Ahmanson-UCLA Adult Congenital Heart Disease Center, Los Angeles, CA, United States AU - Pike, N. A. AU - Evangelista, L. S. AU - Doering, L. V. AU - Koniak-Griffin, D. AU - Lewis, A. B. AU - Child, J. S. DB - Scopus DO - 10.1111/j.1747-0803.2010.00475.x IS - 1 KW - Congenital Heart Disease Fontan Health-related Quality of Life Single Ventricle M3 - Article N1 - Cited By :34 Export Date: 15 June 2020 PY - 2011 SP - 9-17 ST - Clinical profile of the adolescent/adult fontan survivor T2 - Congenital Heart Disease TI - Clinical profile of the adolescent/adult fontan survivor UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-79251491028&doi=10.1111%2fj.1747-0803.2010.00475.x&partnerID=40&md5=9c1407e3649bb4679085f53eff1db2b6 VL - 6 ID - 2136 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 - N.A. Pike, UCLA School of Nursing, University of California, Los Angeles, CA, United States 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. DB - Embase Medline DO - 10.1002/jnr.24215 IS - 6 KW - adolescent amygdala anxiety disorder article attention Beck Anxiety Inventory brain region brain stem brain tissue cerebellum clinical article cognitive defect comparative study controlled study corpus callosum cross-sectional study depression female heart single ventricle hippocampus human hypothalamus learning male memory Montreal cognitive assessment parahippocampal gyrus Patient Health Questionnaire 9 prefrontal cortex priority journal sex difference thalamus vision visual memory white matter working memory LA - English M3 - Article N1 - L621675010 2018-04-19 2018-04-24 PY - 2018 SN - 1097-4547 0360-4012 SP - 1104-1118 ST - Brain abnormalities in cognition, anxiety, and depression regulatory regions in adolescents with single ventricle heart disease T2 - Journal of Neuroscience Research TI - Brain abnormalities in cognition, anxiety, and depression regulatory regions in adolescents with single ventricle heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L621675010 http://dx.doi.org/10.1002/jnr.24215 VL - 96 ID - 680 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. © 2018 Wiley Periodicals, Inc. AD - UCLA School of Nursing, University of California, Los Angeles, CA 90095, United States Department of Anesthesiology, University of California, Los Angeles, CA, United States Division of Pediatric Cardiology, University of California, Los Angeles, CA 90095, United States Division of Pediatric Cardiology, Children's Hospital Los AngelesCA 90027, United States Department of Radiological Sciences, University of California, Los Angeles, CA, United States Department of Bioengineering, University of California, Los Angeles, CA, United States Brain Research Institute, University of California, Los Angeles, CA, United States 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. DB - Scopus DO - 10.1002/jnr.24215 IS - 6 KW - cingulate congenital heart disease hippocampus insula magnetic resonance imaging T2-relaxometry M3 - Article N1 - Cited By :6 Export Date: 15 June 2020 PY - 2018 SP - 1104-1118 ST - Brain abnormalities in cognition, anxiety, and depression regulatory regions in adolescents with single ventricle heart disease T2 - Journal of Neuroscience Research TI - Brain abnormalities in cognition, anxiety, and depression regulatory regions in adolescents with single ventricle heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85045340273&doi=10.1002%2fjnr.24215&partnerID=40&md5=f010d0a0878d78e69acb819d307b558e VL - 96 ID - 1801 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 EF was 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. AU - Prachasilchai, P. AU - Promphan, W. AU - Katanyuwong, P. AU - Layangool, T. AU - Sangtawesin, C. AU - Kirawittaya, T. AU - Mokarapong, P. DB - Embase Medline KW - acid phosphatase prostate isoenzyme article cavopulmonary connection child clinical article cross-sectional study diastolic dysfunction echocardiography female Fontan procedure heart arrhythmia heart ejection fraction heart palpitation heart performance heart single ventricle human hypotension male myocardial perfusion imaging outcome assessment postvoid residual urine volume septicemia systolic dysfunction tissue Doppler imaging treadmill exercise LA - English M3 - Article N1 - L605899834 2015-09-09 2015-09-14 PY - 2014 SN - 0125-2208 SP - S205-S212 ST - Outcomes of straightforward extracardiac Fontan operation in advanced-age group T2 - Journal of the Medical Association of Thailand TI - Outcomes of straightforward extracardiac Fontan operation in advanced-age group UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L605899834 VL - 97 ID - 976 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 EF was 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. © 2014, Medical Association of Thailand. All rights reserved. AD - Pediatric Cardiology Unit, Department of Pediatrics, College of Medicine, Rangsit University, Queen Sirikit National Institute of Child Health (QSNICH), Bangkok, Thailand Pediatric Cardiologist Consultant, Bangkok Hospital Medical Center, Bangkok, Thailand Cardiovascular Thoracic Surgery Unit, Institute of Cardiovascular Disease, Rajvithi Hospital, Ministry of Public Health, Bangkok, Thailand AU - Prachasilchai, P. AU - Promphan, W. AU - Katanyuwong, P. AU - Layangool, T. AU - Sangtawesin, C. AU - Kirawittaya, T. AU - Mokarapong, P. DB - Scopus KW - Fontan operation Outcome Post M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2014 SP - S205-S212 ST - Outcomes of straightforward extracardiac Fontan operation in advanced-age group T2 - Journal of the Medical Association of Thailand TI - Outcomes of straightforward extracardiac Fontan operation in advanced-age group UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84929030412&partnerID=40&md5=8170395cad27f07b14bcd38df95f8e76 VL - 97 ID - 2017 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. © 2013 Wiley Periodicals, Inc. AD - E. Prifti, Division of Cardiac Surgery, University Hospital Center of Tirana, Medical University of Tirana, Rr. Dibres, 370, Tirana, Albania AU - Prifti, E. AU - Baboci, A. AU - Esposito, G. AU - Kajo, E. AU - Dado, E. AU - Vanini, V. DB - Embase Medline DO - 10.1111/jocs.12321 IS - 3 KW - adolescent article azygos vein cavopulmonary connection child complete heart block cyanosis disease severity dyspnea Ebstein anomaly echocardiography epicardial pacemaker female follow up Fontan procedure heart arrhythmia heart right ventricle failure heart ventricle septum defect hemiazygos vein human major clinical study male mortality New York Heart Association class outcome assessment peacock tail technique postoperative period pulmonary valve stenosis reimplantation tricuspid valve prosthesis tricuspid valve repair vein Epic LA - English M3 - Article N1 - L372969469 2014-05-09 2014-05-15 PY - 2014 SN - 1540-8191 0886-0440 SP - 383-389 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 - Journal of Cardiac Surgery 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L372969469 http://dx.doi.org/10.1111/jocs.12321 VL - 29 ID - 928 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. © 2013 Wiley Periodicals, Inc. AD - Division of Cardiac Surgery, University Hospital Center of Tirana, Medical University of Tirana, Rr. Dibres, 370, Tirana, Albania Heart of Children Foundation, Bergamo, Italy AU - Prifti, E. AU - Baboci, A. AU - Esposito, G. AU - Kajo, E. AU - Dado, E. AU - Vanini, V. DB - Scopus DO - 10.1111/jocs.12321 IS - 3 M3 - Article N1 - Export Date: 15 June 2020 PY - 2014 SP - 383-389 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 - Journal of Cardiac Surgery 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84899575650&doi=10.1111%2fjocs.12321&partnerID=40&md5=81925c8dc99c3e9f28ffa31adf6fd390 VL - 29 ID - 2028 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. © 2012 Springer Science+Business Media New York. AD - A. Saxena, Department of Cardiology, All India Institute of Medical Sciences, New Delhi 110029, India AU - Priyadarshini, A. AU - Saxena, A. AU - Patel, C. AU - Paul, V. K. AU - Lodha, R. AU - Airan, B. DB - Embase Medline DO - 10.1007/s00246-012-0531-1 IS - 4 KW - tetrofosmin tc 99m absence of side effects adolescent adult article cardiac imaging child clinical article controlled study cross-sectional study echocardiography electrocardiography Fallot tetralogy female Fontan procedure heart ejection fraction heart muscle perfusion heart single ventricle human male myocardial perfusion imaging palliative therapy PET scanner prospective study school child single photon emission computed tomography Infinia Hawkeye LA - English M3 - Article N1 - L369195945 2013-07-05 2013-07-09 PY - 2013 SN - 0172-0643 1432-1971 SP - 786-794 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 - Pediatric Cardiology 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) UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L369195945 http://dx.doi.org/10.1007/s00246-012-0531-1 VL - 34 ID - 1006 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. © 2012 Springer Science+Business Media New York. AD - Department of Pediatrics, All India Institute of Medical Sciences, New Delhi 110029, India Department of Cardiology, All India Institute of Medical Sciences, New Delhi 110029, India Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi 110029, India Department of Cardiac Surgery, All India Institute of Medical Sciences, New Delhi 110029, India AU - Priyadarshini, A. AU - Saxena, A. AU - Patel, C. AU - Paul, V. K. AU - Lodha, R. AU - Airan, B. DB - Scopus DO - 10.1007/s00246-012-0531-1 IS - 4 KW - Fontan procedure Myocardial perfusion defects Tetralogy of Fallot M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2013 SP - 786-794 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 - Pediatric Cardiology 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) UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84879482181&doi=10.1007%2fs00246-012-0531-1&partnerID=40&md5=08adfafc796466aa34029a14d31ed506 VL - 34 ID - 2054 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. AD - Montreal Children's Hospital, Division of Cardiology, 2300 Tupper St, Montreal, Quebec, H3H 1P3, Canada; charles.rohlicek@mcgill.ca. AN - 105133796. Language: English. Entry Date: 20100430. Revision Date: 20150711. Publication Type: Journal Article AU - Prsa, M. AU - Holly, C. D. AU - Carnevale, F. A. AU - Justino, H. AU - Rohlicek, C. V. DB - ccm DO - 10.1542/peds.2009-1678 DP - EBSCOhost IS - 3 KW - Cardiology Counseling Expectant Parents -- Psychosocial Factors Hypoplastic Left Heart Syndrome -- Surgery Physician Attitudes Practice Patterns Surgeons Confidence Intervals Data Analysis Software Female Heart Transplantation Human Linear Regression North America Odds Ratio Pregnancy Prenatal Diagnosis Questionnaires Thematic Analysis N1 - research; tables/charts. Journal Subset: Biomedical; Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. NLM UID: 0376422. PMID: NLM20156891. PY - 2010 SN - 0031-4005 SP - e625-30 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105133796&site=ehost-live&scope=site VL - 125 ID - 1643 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 - R. Przybylski, Department of Cardiology, Boston Children’s Hospital, Boston, MA, United States 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. DB - Embase Medline DO - 10.1007/s00246-019-02169-1 IS - 7 KW - acute kidney tubule necrosis adolescent age article bacterial infection cellulitis child circulation Cobb angle cohort analysis congenital heart disease cyanosis delirium device removal disease association drug eruption drug use female fluid shift group B streptococcal infection heart surgery heart ventricle tachycardia high risk patient Horner syndrome human hypervolemia length of stay lower extremity numbness lower extremity weakness major clinical study male meralgia paresthetica motor evoked potential neurologic disease operative blood loss paresthesia peroperative complication pneumonia postoperative complication postoperative hemorrhage Propionibacterium acnes infection reoperation retrospective study risk factor scoliosis septic shock single ventricle circulation somatosensory evoked potential spinal cord infarction spine fusion spine surgery stomach perforation supraventricular tachycardia thrombocytopenia urinary tract infection weakness wound infection LA - English M3 - Article N1 - L2002327988 2019-07-31 2019-10-31 PY - 2019 SN - 1432-1971 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 - Pediatric Cardiology TI - Adverse Perioperative Events in Children with Complex Congenital Heart Disease Undergoing Operative Scoliosis Repair in the Contemporary Era UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2002327988 http://dx.doi.org/10.1007/s00246-019-02169-1 VL - 40 ID - 593 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. © 2019, Springer Science+Business Media, LLC, part of Springer Nature. AD - Department of Cardiology, Boston Children’s Hospital, Boston, MA, United States Department of Orthopedic Surgery, Boston Children’s Hospital, Boston, MA, United States Division of Cardiac Anesthesia, Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children’s Hospital, Boston, MA, United States Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children’s Hospital, Boston, MA, United States 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. DB - Scopus DO - 10.1007/s00246-019-02169-1 IS - 7 KW - Congenital heart disease Scoliosis Spinal fusion M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2019 SP - 1468-1475 ST - Adverse Perioperative Events in Children with Complex Congenital Heart Disease Undergoing Operative Scoliosis Repair in the Contemporary Era T2 - Pediatric Cardiology TI - Adverse Perioperative Events in Children with Complex Congenital Heart Disease Undergoing Operative Scoliosis Repair in the Contemporary Era UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85069689707&doi=10.1007%2fs00246-019-02169-1&partnerID=40&md5=d2af1ce566d75a1c4c681547cef7081a VL - 40 ID - 1732 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. © Copyright The International Neuropsychological Society 2011. AD - R. Puosi, Division of Child Neurology, Helsinki University Central Hospital, P.O. Box 280, 00029 HUS, Finland AU - Puosi, R. AU - Korkman, M. AU - Sarajuuri, A. AU - Jokinen, E. AU - Mildh, L. AU - Mattila, I. AU - Lönnqvist, T. DB - Medline DO - 10.1017/S135561771100110X IS - 6 KW - article behavior disorder chi square distribution cognitive defect congenital heart malformation developmental disorder female heart ventricle human infant language disability male neurologic examination neuropsychological test pathology pathophysiology preschool child severity of illness index LA - English M3 - Article N1 - L362867266 2013-02-05 PY - 2011 SN - 1355-6177 1469-7661 SP - 1094-1103 ST - Neurocognitive development and behavioral outcome of 2-year-old children with univentricular heart T2 - Journal of the International Neuropsychological Society TI - Neurocognitive development and behavioral outcome of 2-year-old children with univentricular heart UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L362867266 http://dx.doi.org/10.1017/S135561771100110X VL - 17 ID - 1084 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). © 2017 by the American Academy of Pediatrics. All rights reserved. AD - Department of Pediatrics, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, United States CE Mullins Cardiac Catheterization Laboratories, Lillie Frank Abercombie Section of Cardiology, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, United States AU - Puri, K. AU - Allen, H. D. AU - Qureshi, A. M. DB - Scopus DO - 10.1542/pir.2017-0032 IS - 10 M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2017 SP - 471-484 ST - Congenital heart disease T2 - Pediatrics in Review TI - Congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85030867988&doi=10.1542%2fpir.2017-0032&partnerID=40&md5=ec5c806da4ac4a6f624d905b0e98e8ac VL - 38 ID - 1843 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 mg/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±0.59WU m2 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 - R. Razavi, Division of Imaging Sciences, King's College London BHF Centre, NIHR Biomedical Research Centre at Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom 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. DB - Embase Medline DO - 10.1093/ehjci/jev178 IS - 6 KW - dobutamine nitric oxide oxygen article catheter cavopulmonary connection child clinical article clinical evaluation collateral circulation controlled study diagnostic catheter elevated blood pressure female Fontan procedure heart catheterization cardiac index heart left ventricle enddiastolic volume heart output heart ventricle enddiastolic pressure hemodynamic stress hemodynamics human hypoplastic left heart syndrome lung vascular resistance male nuclear magnetic resonance imaging nuclear magnetic resonance scanner oxygen saturation priority journal prospective study radiation dose 1.5T Philips Achieva Wedge catheter LA - English M3 - Article N1 - L611358169 2016-07-28 2016-08-08 PY - 2016 SN - 2047-2412 2047-2404 SP - 644-651 ST - Magnetic resonance imaging catheter stress haemodynamics post-Fontan in hypoplastic left heart syndrome T2 - European Heart Journal Cardiovascular Imaging TI - Magnetic resonance imaging catheter stress haemodynamics post-Fontan in hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L611358169 http://dx.doi.org/10.1093/ehjci/jev178 VL - 17 ID - 808 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 mg/kg/min (Stage 2), and dobutamine at 20 mg/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+0.59WU m2 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 University of Nebraska/Creighton University Joint Division of Pediatric Cardiology, Omaha, NE, USA Paediatric Cardiovascular Sciences, Rayne Institute, King's College London and Evelina London Children's Hospital,Westminster Bridge Road, London SE1 7EH, UK AN - 115654531. Language: English. Entry Date: 20180117. Revision Date: 20170601. Publication Type: Article AU - Pushparajah, Kuberan AU - Wong, James K. AU - Bellsham-Revell, Hannah R. AU - Hussain, Tarique AU - Valverde, Israel AU - Bell, Aaron AU - Tzifa, Aphrodite AU - Greil, Gerald AU - Simpson, John M. AU - Kutty, Shelby AU - Razavi, Reza DB - ccm DO - 10.1093/ehjci/jev178 DP - EBSCOhost IS - 6 N1 - equations & formulas; research; tables/charts. Journal Subset: Biomedical; Continental Europe; Europe; Peer Reviewed. PY - 2016 SN - 2047-2404 SP - 644-651 ST - Magnetic resonance imaging catheter stress haemodynamics post-Fontan in hypoplastic left heart syndrome T2 - European Heart Journal - Cardiovascular Imaging TI - Magnetic resonance imaging catheter stress haemodynamics post-Fontan in hypoplastic left heart syndrome UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=115654531&site=ehost-live&scope=site VL - 17 ID - 1481 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 mg/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±0.59WU m2 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. © 2016 The Author. 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, United Kingdom Department of Congenital Heart Disease, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom University of Nebraska, Creighton University Joint Division of Pediatric Cardiology, Omaha, NE, United States Paediatric Cardiovascular Sciences, Rayne Institute, King's College London and Evelina London Children's Hospital, Westminster Bridge Road, London, SE1 7EH, United Kingdom 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. DB - Scopus DO - 10.1093/ehjci/jev178 IS - 6 KW - dobutamine Fontan hypoplastic left heart syndrome MRI pulmonary vascular resistance M3 - Article N1 - Cited By :16 Export Date: 15 June 2020 PY - 2016 SP - 644-651 ST - Magnetic resonance imaging catheter stress haemodynamics post-Fontan in hypoplastic left heart syndrome T2 - European Heart Journal Cardiovascular Imaging TI - Magnetic resonance imaging catheter stress haemodynamics post-Fontan in hypoplastic left heart syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84978933087&doi=10.1093%2fehjci%2fjev178&partnerID=40&md5=76d0ba623fabd84dd6927d794f8e9293 VL - 17 ID - 1901 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 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 - L563072955 2015-02-18 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=L563072955 ID - 1073 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 - Department of Pediatric Cardiology, Saarland University Hospital, Kirrberger Str., D-66421 Homburg/Saar, Germany 2nd Department of Internal Medicine, Saarland University Hospital, Homburg/Saar, Germany Institute of Biostatistics and Mathematical Modelling, University of Frankfurt/Main, Frankfurt/Main, Germany AU - Raedle-Hurst, T. M. AU - Koenigstein, K. AU - Gruenhage, F. AU - Raedle, J. AU - Herrmann, E. AU - Abdul-Khaliq, H. DB - Scopus DO - 10.1016/j.ahj.2010.08.033 IS - 6 M3 - Article N1 - Cited By :11 Export Date: 15 June 2020 PY - 2010 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-78650111041&doi=10.1016%2fj.ahj.2010.08.033&partnerID=40&md5=bf1ae7f35f6ef16a24615f07b3d67d13 VL - 160 ID - 2141 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 - H.S. Bernstein, Merck and Co., Kenilworth, NJ, United States AU - Ramachandran, S. AU - Lowenthal, A. AU - Ritner, C. AU - Lowenthal, S. AU - Bernstein, H. S. DB - Embase Medline DO - 10.1371/journal.pone.0183624 IS - 8 KW - bone morphogenetic protein receptor 2 microRNA microRNA 129 5p unclassified drug animal cell article cardiac muscle cell child cohort analysis controlled study cross-sectional study down regulation embryo embryonic stem cell female heart disease heart failure human human cell infant informed consent major clinical study male membrane microparticle mouse nonhuman observational study oxidative stress pathophysiology protein RNA binding pulmonary vascular disease Ross score scoring system univentricular heart disease LA - English M3 - Article N1 - L618215982 2017-09-18 2017-09-19 PY - 2017 SN - 1932-6203 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L618215982 http://dx.doi.org/10.1371/journal.pone.0183624 VL - 12 ID - 737 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. © 2017 Ramachandran et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. AD - Department of Pediatrics, Cardiovascular Research Institute, University of California San Francisco, San Francisco, CA, United States Department of Pediatrics, Mindich Child Health and Development Institute, Icahn School of Medicine at Mount Sinai, New York, NY, United States Schneider Children’s Medical Center of Israel, Petah Tikva, Israel Merck and Co., Kenilworth, NJ, United States AU - Ramachandran, S. AU - Lowenthal, A. AU - Ritner, C. AU - Lowenthal, S. AU - Bernstein, H. S. C7 - e0183624 DB - Scopus DO - 10.1371/journal.pone.0183624 IS - 8 M3 - Article N1 - Cited By :10 Export Date: 15 June 2020 PY - 2017 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85029185613&doi=10.1371%2fjournal.pone.0183624&partnerID=40&md5=e10a4278f0b15e35ec8d581565688b15 VL - 12 ID - 1848 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. © 2010 The Authors. Journal of Advanced Nursing © 2010 Blackwell Publishing Ltd. AD - G.R. Rempel, Faculty of Nursing, University of Alberta, TD Clinical Fellow Mazankowski Alberta Heart Institute, Edmonton, AB, Canada AU - Ravindran, V. P. AU - Rempel, G. R. DB - Medline DO - 10.1111/j.1365-2648.2010.05482.x IS - 1 KW - adolescent adult aged article child child care child parent relation family health family size female home care human human relation hypoplastic left heart syndrome infant male middle aged organization and management pregnancy preschool child psychological aspect qualitative research role playing sibling LA - English M3 - Article N1 - L360159519 2011-05-12 PY - 2011 SN - 0309-2402 1365-2648 SP - 169-175 ST - Grandparents and siblings of children with congenital heart disease T2 - Journal of Advanced Nursing TI - Grandparents and siblings of children with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L360159519 http://dx.doi.org/10.1111/j.1365-2648.2010.05482.x VL - 67 ID - 1118 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, CanadaCollege of Nursing, Christian Medical College, Vellore, Tamilnadu, India AN - 104971738. Language: English. Entry Date: 20110203. Revision Date: 20190412. Publication Type: Journal Article AU - Ravindran, V. P. AU - Rempel, G. R. DB - ccm DO - 10.1111/j.1365-2648.2010.05482.x DP - EBSCOhost IS - 1 KW - Grandparents Siblings Grandchildren Child, Hospitalized Role Hypoplastic Left Heart Syndrome Human Interviews Child, Preschool Family Centered Care Grounded Theory Qualitative Studies Middle Age Aged Parent-Child Relations Funding Source N1 - research. Special Interest: Advanced Nursing Practice. Grant Information: This study was funded by a University Hospital Foundation Medical Research Award and a University of Alberta Endowment Fund for the Future Support for the Advancement of Scholarship.. PMID: NLM21039778. PY - 2011 SN - 0309-2402 SP - 169-175 ST - Grandparents and siblings of children with congenital heart disease T2 - Journal of Advanced Nursing (John Wiley & Sons, Inc.) TI - Grandparents and siblings of children with congenital heart disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104971738&site=ehost-live&scope=site VL - 67 ID - 1582 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 College of Nursing, Christian Medical College, Vellore, Tamilnadu, India Assistant Professor, Faculty of Nursing, University of Alberta, Edmonton, Alberta, Canada TD Clinical Fellow, Mazankowski Alberta Heart Institute, Edmonton, Alberta, Canada AN - 55742804. Language: English. Entry Date: 20110203. Revision Date: 20190412. Publication Type: Article AU - Ravindran, Vinitha Paul AU - Rempel, Gwen R. DB - ccm DO - 10.1111/j.1365-2648.2010.05482.x DP - EBSCOhost IS - 1 KW - Grandparents Siblings Grandchildren Child, Hospitalized Role Hypoplastic Left Heart Syndrome Human Interviews Child, Preschool Family Centered Care Grounded Theory Qualitative Studies Middle Age Aged Parent-Child Relations Funding Source N1 - research. Grant Information: This study was funded by a University Hospital Foundation Medical Research Award and a University of Alberta Endowment Fund for the Future Support for the Advancement of Scholarship.. PY - 2011 SN - 0309-2402 SP - 169-175 ST - Grandparents and siblings of children with congenital heart disease T2 - Journal of Advanced Nursing (John Wiley & Sons, Inc.) TI - Grandparents and siblings of children with congenital heart disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=55742804&site=ehost-live&scope=site VL - 67 ID - 1583 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. (PsycINFO Database Record (c) 2016 APA, all rights reserved) AD - Rempel, Gwen R. AN - 2011-00899-019 AU - Ravindran, Vinitha Paul AU - Rempel, Gwen R. DB - psyh DO - 10.1111/j.1365-2648.2010.05482.x DP - EBSCOhost IS - 1 KW - grandparents siblings children with congenital heart disease Adolescent Adult Aged Child Child Care Child, Preschool Family Characteristics Family Health Female Home Nursing Humans Hypoplastic Left Heart Syndrome Infant Intergenerational Relations Male Middle Aged Parenting Pregnancy Qualitative Research Role Congenital Disorders Grandchildren Heart Disorders N1 - Faculty of Nursing, University of Alberta, Edmonton, AB, Canada. Other Publishers: Blackwell Publishing. Release Date: 20110725. Correction Date: 20120618. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Grant Information: Ravindran, Vinitha Paul. Major Descriptor: Congenital Disorders; Grandchildren; Grandparents; Heart Disorders; Siblings. Classification: Cardiovascular Disorders (3295). Population: Human (10); Male (30); Female (40). Age Group: Adulthood (18 yrs & older) (300); Middle Age (40-64 yrs) (360); Aged (65 yrs & older) (380). Methodology: Empirical Study; Interview; Qualitative Study. References Available: Y. Page Count: 7. Issue Publication Date: Jan, 2011. Publication History: Accepted Date: Sep 3, 2010. Copyright Statement: The Authors. Journal of Advanced Nursing—Blackwell Publishing Ltd. 2010. Sponsor: University Hospital Foundation. Other Details: Medical Research Award. Recipients: No recipient indicated Sponsor: University of Alberta, Canada. Other Details: Endowment Fund for the Future Support for the Advancement of Scholarship. Recipients: No recipient indicated Sponsor: Enhancing Qualitative Understanding of Illness Processes and Prevention. Recipients: No recipient indicated Sponsor: Canadian Institutes of Health Research, Institute of Gender and Health, Canada. Other Details: Strategic Training Initiative in Health Research award. Recipients: Ravindran, Vinitha Paul Sponsor: Sponsor name not included. Other Details: TD Financial Group Clinical Fellowship. Recipients: Rempel, Gwen R. PY - 2011 SN - 0309-2402 1365-2648 SP - 169-175 ST - Grandparents and siblings of children with congenital heart disease T2 - Journal of Advanced Nursing TI - Grandparents and siblings of children with congenital heart disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2011-00899-019&site=ehost-live&scope=site gwen.rempel@ualberta.ca VL - 67 ID - 1691 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. © 2010 The Authors. Journal of Advanced Nursing © 2010 Blackwell Publishing Ltd. AD - Faculty of Nursing, University of Alberta, Edmonton, AB, Canada College of Nursing, Christian Medical College, Vellore, Tamilnadu, India Faculty of Nursing, University of Alberta, TD Clinical Fellow Mazankowski Alberta Heart Institute, Edmonton, AB, Canada AU - Ravindran, V. P. AU - Rempel, G. R. DB - Scopus DO - 10.1111/j.1365-2648.2010.05482.x IS - 1 KW - Congenital heart defect Family-centred care Grandparents Grounded theory Nursing Siblings Sick children M3 - Article N1 - Cited By :14 Export Date: 15 June 2020 PY - 2011 SP - 169-175 ST - Grandparents and siblings of children with congenital heart disease T2 - Journal of Advanced Nursing TI - Grandparents and siblings of children with congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-78650122918&doi=10.1111%2fj.1365-2648.2010.05482.x&partnerID=40&md5=e053277401954df87f608afc1c9d1baa VL - 67 ID - 2138 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. Copyright © 2006 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies. AD - C. Ravishankar, Division of Cardiology, Department of Pediatrics, Children's Hospital of Philadelphia, 34th St. and Civic Center Boulevard, Philadelphia, PA 19104-4399, United States 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. DB - Embase Medline DO - 10.1097/01.PCC.0000227109.82323.CE IS - 4 KW - acute disease article cardiopulmonary bypass clinical article controlled study extracorporeal oxygenation female follow up heart surgery hospital discharge human hypoplastic left heart syndrome infant male postoperative care postoperative period priority journal retrospective study shunt thrombosis surgical technique treatment duration treatment indication treatment outcome LA - English M3 - Article N1 - L44048273 2006-07-27 PY - 2006 SN - 1529-7535 SP - 319-323 ST - Extracorporeal membrane oxygenation after stage I reconstruction for hypoplastic left heart syndrome T2 - Pediatric Critical Care Medicine TI - Extracorporeal membrane oxygenation after stage I reconstruction for hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L44048273 http://dx.doi.org/10.1097/01.PCC.0000227109.82323.CE VL - 7 ID - 1259 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, Department of Pediatrics, The Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphie, PA 19104-4399; Ravishankar@email.chop.edu AN - 106366171. Language: English. Entry Date: 20061124. Revision Date: 20150711. Publication Type: Journal Article 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. DB - ccm DP - EBSCOhost IS - 4 KW - Extracorporeal Membrane Oxygenation -- Adverse Effects -- In Infancy and Childhood Hypoplastic Left Heart Syndrome -- Surgery Postoperative Care Data Analysis Software Female Fisher's Exact Test Heart Arrest -- Therapy Infant Infant, Newborn Male Multiple Logistic Regression Multivariate Analysis Retrospective Design Risk Factors Step-Wise Multiple Regression Survival Analysis Treatment Outcomes Wilcoxon Rank Sum Test Human N1 - research; tables/charts. Commentary: McMullan DM, Dalton HJ. Extracorporeal support in hypoplastic left heart syndrome: who and why? (PEDIATR CRIT CARE MED) Jul2006; 7 (4): 390-391. Journal Subset: Biomedical; Peer Reviewed; USA. NLM UID: 100954653. PMID: NLM16738497. PY - 2006 SN - 1529-7535 SP - 319-323 ST - Extracorporeal membrane oxygenation after stage I reconstruction for hypoplastic left heart syndrome T2 - Pediatric Critical Care Medicine TI - Extracorporeal membrane oxygenation after stage I reconstruction for hypoplastic left heart syndrome UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106366171&site=ehost-live&scope=site VL - 7 ID - 1588 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. Copyright © 2006 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies. AD - Division of Cardiology, Department of Pediatrics, United States Division of Cardiothoracic Surgery, Department of Surgery Department of Anesthesiology and Critical Care Medicine, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, United States Division of Cardiology, Department of Pediatrics, Children's Hospital of Pittsburgh, United States Division of Cardiology, Department of Pediatrics, Children's Hospital of Philadelphia, 34th St. and Civic Center Boulevard, Philadelphia, PA 19104-4399, United States 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. DB - Scopus DO - 10.1097/01.PCC.0000227109.82323.CE IS - 4 KW - Arrest Cardiac Extracorporeal membrane oxygenation Hypoplastic left heart syndrome Infant Outcome M3 - Article N1 - Cited By :77 Export Date: 15 June 2020 PY - 2006 SP - 319-323 ST - Extracorporeal membrane oxygenation after stage I reconstruction for hypoplastic left heart syndrome T2 - Pediatric Critical Care Medicine TI - Extracorporeal membrane oxygenation after stage I reconstruction for hypoplastic left heart syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-33745894986&doi=10.1097%2f01.PCC.0000227109.82323.CE&partnerID=40&md5=19f39424bd12900eab53e4c52f5c5e89 VL - 7 ID - 2229 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. AD - S. Menahem, MonashHeart, Monash Medical Centre, 246 Clayton Road, Clayton, VIC, Australia AU - Re, J. AU - Dean, S. AU - Menahem, S. DB - Embase DO - 10.1177/2150135113481480 IS - 3 KW - adolescent adult altruism anticipatory anxiety article congenital heart disease content analysis female heart surgery heart ventricle septum defect human hypoplastic left heart syndrome infant intensive care unit mother child relation posttraumatic stress disorder priority journal qualitative research quality of life resuscitation semi structured interview treatment outcome LA - English M3 - Article N1 - L604335202 2015-05-18 2015-05-25 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 Heart Surgery TI - Infant Cardiac Surgery: Mothers Tell Their Story: A Therapeutic Experience UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L604335202 http://dx.doi.org/10.1177/2150135113481480 VL - 4 ID - 1031 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 - B. Reich, Pediatric Heart Center Giessen, Department of Pediatric Cardiology, Justus-Liebig-University, Feulgenstrasse 10-12, Giessen, Germany 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. DB - Embase DO - 10.1053/j.semtcvs.2017.09.014 IS - 4 KW - article artificial ventilation Bayley Scales of Infant Development cavopulmonary connection child child development clinical article cohort analysis female Fontan procedure heart single ventricle hospitalization human hypoplastic left heart syndrome male nerve cell differentiation Norwood procedure patient assessment postoperative period preschool child quality of life quality of life assessment questionnaire repeat procedure risk factor scoring system LA - English M3 - Article N1 - L619562133 2017-12-12 2018-02-20 PY - 2017 SN - 1532-9488 1043-0679 SP - 504-513 ST - Neurodevelopmental Outcome and Health-related Quality of Life in Children With Single-ventricle Heart Disease Before Fontan Procedure T2 - Seminars in Thoracic and Cardiovascular Surgery TI - Neurodevelopmental Outcome and Health-related Quality of Life in Children With Single-ventricle Heart Disease Before Fontan Procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L619562133 http://dx.doi.org/10.1053/j.semtcvs.2017.09.014 VL - 29 ID - 772 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 Pediatric Cardiology, 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 Child Development Center, University Children's Hospital, Zurich, Switzerland Child Development Center, SPZ Frankfurt Mitte, Frankfurt/Main, Germany Pediatric Neurology, University Hospital, Giessen, Germany AN - 127919852. Language: English. Entry Date: 20180619. Revision Date: 20180213. Publication Type: journal article. Journal Subset: Biomedical AU - Reich, Bettina AU - Heye, Kristina AU - Tuura, Ruth AU - Beck, Ingrid AU - Wetterling, Kristina AU - Hahn, Andreas AU - Hofmann, Karoline AU - Schranz, Dietmar AU - Akintürk, Hakan AU - Latal, Beatrice AU - Knirsch, Walter DA - Winter2017 DB - ccm DO - 10.1053/j.semtcvs.2017.09.014 DP - EBSCOhost IS - 4 N1 - USA. NLM UID: 8917640. PMID: NLM29104017. PY - 2017 SN - 1043-0679 SP - 504-513 ST - Neurodevelopmental Outcome and Health-related Quality of Life in Children With Single-ventricle Heart Disease Before Fontan Procedure T2 - Seminars in Thoracic & Cardiovascular Surgery TI - Neurodevelopmental Outcome and Health-related Quality of Life in Children With Single-ventricle Heart Disease Before Fontan Procedure UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=127919852&site=ehost-live&scope=site VL - 29 ID - 1454 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. © 2017 Elsevier Inc. AD - Pediatric Heart Center, University Hospital, Giessen, Germany Pediatric Cardiology, 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 Child Development Center, University Children's Hospital, Zurich, Switzerland Child Development Center, SPZ Frankfurt Mitte, Frankfurt/Main, Germany Pediatric Neurology, University Hospital, Giessen, Germany 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. DB - Scopus DO - 10.1053/j.semtcvs.2017.09.014 IS - 4 KW - congenital heart disease neurodevelopmental outcome risk factors M3 - Article N1 - Cited By :11 Export Date: 15 June 2020 PY - 2017 SP - 504-513 ST - Neurodevelopmental Outcome and Health-related Quality of Life in Children With Single-ventricle Heart Disease Before Fontan Procedure T2 - Seminars in Thoracic and Cardiovascular Surgery TI - Neurodevelopmental Outcome and Health-related Quality of Life in Children With Single-ventricle Heart Disease Before Fontan Procedure UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85037051348&doi=10.1053%2fj.semtcvs.2017.09.014&partnerID=40&md5=60f4de10d05778928d580921f8c8dce5 VL - 29 ID - 1872 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 - B. Reich, Pediatric Heart Center, University Hospital Giessen, Feulgenstrasse 12, Giessen, Germany 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. DB - Embase DO - 10.21037/tp.2019.04.05 IS - 2 KW - Magnetom Verio B17 nuclear magnetic resonance scanner article Bayley Scales of Infant Development cerebrospinal fluid cerebrovascular accident child clinical article clinical assessment cognition controlled study female human hypoplastic left heart syndrome language male middle cerebral artery motor activity nerve cell differentiation nuclear magnetic resonance imaging pediatric cardiac quality of life inventory preschool children quality of life questionnaire quality of life questionnaire surgical approach volumetry LA - English M3 - Article N1 - L2002000194 2019-05-28 2019-05-29 PY - 2019 SN - 2224-4344 2224-4336 SP - 94-106 ST - Neurodevelopmental outcome in hypoplastic left heart syndrome after hybrid procedure T2 - Translational Pediatrics TI - Neurodevelopmental outcome in hypoplastic left heart syndrome after hybrid procedure UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2002000194 http://dx.doi.org/10.21037/tp.2019.04.05 VL - 8 ID - 619 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. © Translational Pediatrics. All rights reserved. AD - Pediatric Heart Center, University Hospital Giessen, Justus-Liebig-University, Giessen, Germany Division of Neurology, Children’s Hospital of Philadelphia, Philadelphia, PA, United States 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 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. DB - Scopus DO - 10.21037/tp.2019.04.05 IS - 2 KW - Brain development Congenital heart disease (CHD) Hybrid procedure Hypoplastic left heart syndrome (HLHS) Neurodevelopmental outcome M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2019 SP - 94-106 ST - Neurodevelopmental outcome in hypoplastic left heart syndrome after hybrid procedure T2 - Translational Pediatrics TI - Neurodevelopmental outcome in hypoplastic left heart syndrome after hybrid procedure UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85065864286&doi=10.21037%2ftp.2019.04.05&partnerID=40&md5=8b938123377da6f45013b6e02c53f9d3 VL - 8 ID - 1753 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. AU - Reid, A. AU - Gaskin, K. DB - Medline DO - 10.7748/ncyp.2018.e1078 IS - 6 KW - adult child parent relation congenital heart malformation echography female human infant life event male middle aged newborn pregnancy prenatal diagnosis preschool child procedures psychology qualitative research questionnaire time factor United Kingdom LA - English M3 - Article N1 - L624921678 2018-11-20 2019-02-04 PY - 2018 SN - 2046-2344 SP - 19-25 ST - Parents' experiences of receiving an antenatal versus postnatal diagnosis of complex congenital heart disease T2 - Nursing children and young people TI - Parents' experiences of receiving an antenatal versus postnatal diagnosis of complex congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L624921678 http://dx.doi.org/10.7748/ncyp.2018.e1078 VL - 30 ID - 654 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 - Paediatric staff nurse, children's ward, Gloucestershire Hospitals NHS Foundation Trust, Gloucestershire, England Principal lecturer in advanced clinical practice, Department of Nursing and Midwifery, Institute of Health and Society, University of Worcester, Worcester, England AN - 132862660. Language: English. Entry Date: 20181110. Revision Date: 20181126. Publication Type: Article AU - Reid, Annette AU - Gaskin, Kerry DB - ccm DO - 10.7748/ncyp.2018.e1078 DP - EBSCOhost IS - 6 KW - Parental Attitudes Heart Defects, Congenital -- Diagnosis Postnatal Period Prenatal Diagnosis Human Thematic Analysis Surveys Parents -- Psychosocial Factors Emotions -- Evaluation Infant Heart Surgery -- In Infancy and Childhood Truth Disclosure N1 - research. Journal Subset: Core Nursing; Double Blind Peer Reviewed; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Nursing; Peer Reviewed; UK & Ireland. NLM UID: 101554473. PY - 2018 SN - 2046-2336 SP - 19-25 ST - Parents' experiences of receiving an antenatal versus postnatal diagnosis of complex congenital heart disease T2 - Nursing Children & Young People TI - Parents' experiences of receiving an antenatal versus postnatal diagnosis of complex congenital heart disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=132862660&site=ehost-live&scope=site VL - 30 ID - 1472 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. ©2018 RCN Publishing Company Ltd. All rights reserved. Not to be copied, transmitted or recorded in any way, in whole or part, without prior permission of the publishers. AD - Children's ward, Gloucestershire Hospitals NHS Foundation TrustGloucestershire, United Kingdom Advanced clinical practice, Department of Nursing and Midwifery, Institute of Health and Society, University of Worcester, Worcester, United Kingdom AU - Reid, A. AU - Gaskin, K. DB - Scopus DO - 10.7748/ncyp.2018.e1078 IS - 6 KW - child health congenital abnormalities heart diseases research M3 - Article N1 - Export Date: 15 June 2020 PY - 2018 SP - 19-25 ST - Parents' experiences of receiving an antenatal versus postnatal diagnosis of complex congenital heart disease T2 - Nursing children and young people TI - Parents' experiences of receiving an antenatal versus postnatal diagnosis of complex congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85056374939&doi=10.7748%2fncyp.2018.e1078&partnerID=40&md5=b30a1530ba69ced2a1a415de44ed4ef3 VL - 30 ID - 1783 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. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2019. All rights reserved. No commercial use is permitted unless otherwise expressly granted. AD - Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Munich, Germany Institute of Preventive Pediatrics, Technische Universität München, Munich, Germany AU - Reiner, B. AU - Oberhoffer, R. AU - Ewert, P. AU - Müller, J. DB - Scopus DO - 10.1136/archdischild-2017-314211 IS - 2 KW - Health-related quality of life severity of heart defects M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2019 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85049166944&doi=10.1136%2farchdischild-2017-314211&partnerID=40&md5=76cf0a90b777a1f9671f8649fdb96fc7 VL - 104 ID - 1764 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. © 2006 The Society of Thoracic Surgeons. AD - O. Reinhartz, Department of Cardiothoracic Surgery, Division of Pediatric Cardiac Surgery, Stanford University, Stanford, CA, United States 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. DB - Embase Medline DO - 10.1016/j.athoracsur.2006.03.063 IS - 3 KW - article blood vessel child clinical article collateral circulation coronary artery blood flow data analysis female follow up Fontan procedure Glenn shunt heart right ventricle heart single ventricle heart surgery heart ventricle heart volume heart work human infant life expectancy lung artery lung artery pressure lung blood vessel major aortopulmonary collateral artery male newborn postoperative period priority journal quality of life sepsis surgical technique survival rate time series analysis unifocalization vascular surgery LA - English M3 - Article N1 - L44246685 2006-08-30 PY - 2006 SN - 0003-4975 SP - 934-939 ST - Unifocalization of Major Aortopulmonary Collaterals in Single-Ventricle Patients T2 - Annals of Thoracic Surgery TI - Unifocalization of Major Aortopulmonary Collaterals in Single-Ventricle Patients UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L44246685 http://dx.doi.org/10.1016/j.athoracsur.2006.03.063 VL - 82 ID - 1254 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. © 2006 The Society of Thoracic Surgeons. AD - Department of Cardiothoracic Surgery, Division of Pediatric Cardiac Surgery, Stanford University, Stanford, CA, United States Department of Pediatrics, Division of Pediatric Cardiology, Stanford University, Stanford, CA, United States 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. DB - Scopus DO - 10.1016/j.athoracsur.2006.03.063 IS - 3 M3 - Article N1 - Cited By :19 Export Date: 15 June 2020 PY - 2006 SP - 934-939 ST - Unifocalization of Major Aortopulmonary Collaterals in Single-Ventricle Patients T2 - Annals of Thoracic Surgery TI - Unifocalization of Major Aortopulmonary Collaterals in Single-Ventricle Patients UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-33747364709&doi=10.1016%2fj.athoracsur.2006.03.063&partnerID=40&md5=ecbf2668d15cd8f555503665181affc2 VL - 82 ID - 2225 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. Copyright © American Society of Artificial Internal Organs. AD - N. Reiss, Service de Chirurgie Thoracique et Cardiovasculaire, Groupe Hospitalier Pitie-Salpetriere, 47-83, Boulevard de l'Hopital, 75651 Paris Cedex 13, France AU - Reiss, N. AU - Blanz, U. AU - Breymann, T. AU - Kind, K. AU - Bairaktaris, A. AU - Körfer, R. DB - Embase Medline DO - 10.1097/01.mat.0000242598.30744.24 IS - 5 KW - anticoagulant agent phenprocoumon adolescent anticoagulation article child clinical article female follow up heart valve replacement human infant international normalized ratio male mortality outcome assessment quality of life school child sinus rhythm tricuspid valve disease LA - English M3 - Article N1 - L44394269 2006-10-01 PY - 2006 SN - 1058-2916 SP - 559-561 ST - Mechanical valve replacement of the systemic atrioventricular valve in children T2 - ASAIO Journal TI - Mechanical valve replacement of the systemic atrioventricular valve in children UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L44394269 http://dx.doi.org/10.1097/01.mat.0000242598.30744.24 VL - 52 ID - 1256 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. Copyright © American Society of Artificial Internal Organs. AD - Clinic for Thoracic and Cardiovascular Surgery, Heart Center North Rhine-Westphalia, Ruhr-University of Bochum, Bad Oeynhausen, Germany Service de Chirurgie Thoracique et Cardiovasculaire, Groupe Hospitalier Pitie-Salpetriere, 47-83, Boulevard de l'Hopital, 75651 Paris Cedex 13, France AU - Reiss, N. AU - Blanz, U. AU - Breymann, T. AU - Kind, K. AU - Bairaktaris, A. AU - Körfer, R. DB - Scopus DO - 10.1097/01.mat.0000242598.30744.24 IS - 5 M3 - Article N1 - Cited By :11 Export Date: 15 June 2020 PY - 2006 SP - 559-561 ST - Mechanical valve replacement of the systemic atrioventricular valve in children T2 - ASAIO Journal TI - Mechanical valve replacement of the systemic atrioventricular valve in children UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-33748699606&doi=10.1097%2f01.mat.0000242598.30744.24&partnerID=40&md5=8b228e2783e82cf3cfd6f852bae1ac7c VL - 52 ID - 2224 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 - G.R. Rempel, University of Alberta, Edmonton, Alberta, Canada. AU - Rempel, G. R. AU - Blythe, C. AU - Rogers, L. G. AU - Ravindran, V. DB - Medline IS - 1 KW - adaptive behavior adult article Canada caregiver comparative study decision making female heart surgery human hypoplastic left heart syndrome interview male middle aged newborn Norwood procedure palliative therapy parent psychological aspect psychological model LA - English M3 - Article N1 - L365184445 2012-07-16 PY - 2012 SN - 1552-549X SP - 35-64 ST - The process of family management when a baby is diagnosed with a lethal congenital condition T2 - Journal of family nursing TI - The process of family management when a baby is diagnosed with a lethal congenital condition UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L365184445 VL - 18 ID - 1069 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 University of Alberta, Edmonton, Alberta, Canada, Lloydminster Hospital, Lloydminster, Saskatchewan, Canada University of Alberta, Edmonton, Alberta, Canada, Christian Medical College Vellore, Tamilnadu, India AN - 71765348. Language: English. Entry Date: 20120301. Revision Date: 20190322. Publication Type: Article AU - Rempel, Gwen R. AU - Blythe, Catriona AU - Rogers, Laura G. AU - Ravindran, Vinitha DB - ccm DO - 10.1177/1074840711427143 DP - EBSCOhost IS - 1 KW - Family Nursing Hypoplastic Left Heart Syndrome -- Diagnosis Professional-Family Relations Decision Making, Family Attitude to Illness Human Alberta Conceptual Framework Secondary Analysis Thematic Analysis Interviews Grounded Theory Purposive Sample Male Female Young Adult Adult Middle Age Severity of Illness Funding Source Family Centered Care N1 - research; tables/charts. Journal Subset: Core Nursing; Nursing; Peer Reviewed; USA. Special Interest: Advanced Nursing Practice; Pediatric Care. Grant Information: Heart and Stroke Foundation of Canada (HSFC) Nursing Research Fellowship and aCanadian Institutes of Health Research Doctoral Fellowship; University of Alberta Hospital Foundation Medical Research Award; TD Financial Group/Mazankowski Alberta Heart Institute Clinical Fellowship, and a HSFC Research Scholarship.. NLM UID: 9503761. 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 - Journal of Family Nursing TI - The Process of Family Management When a Baby Is Diagnosed With a Lethal Congenital Condition UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=71765348&site=ehost-live&scope=site VL - 18 ID - 1594 ER - TY - JOUR AD - University of Alberta, Edmonton, Alberta, Canada University of Alberta, Edmonton, Alberta, Canada, Lloydminster Hospital, Lloydminster, Saskatchewan, Canada University of Alberta, Edmonton, Alberta, Canada, Christian Medical College Vellore, Tamilnadu, India AN - 104516732. Language: English. Entry Date: 20120301. Revision Date: 20150820. Publication Type: Journal Article AU - Rempel, Gwen R. AU - Blythe, Catriona AU - Rogers, Laura G. AU - Ravindran, Vinitha DB - ccm DO - 10.1177/1074840711427143 DP - EBSCOhost IS - 1 KW - Family Nursing Hypoplastic Left Heart Syndrome -- Diagnosis Professional-Family Relations Decision Making, Family Attitude to Illness Human Alberta Conceptual Framework Secondary Analysis Thematic Analysis Interviews Grounded Theory Purposive Sample Male Female Young Adult Adult Middle Age Severity of Illness Funding Source Family Centered Care N1 - research; tables/charts. Journal Subset: Core Nursing; Nursing; Peer Reviewed; USA. Special Interest: Advanced Nursing Practice; Pediatric Care. Grant Information: Heart and Stroke Foundation of Canada (HSFC) Nursing Research Fellowship and a Canadian Institutes of Health Research Doctoral Fellowship; University of Alberta Hospital Foundation Medical Research Award; TD Financial Group/Mazankowski Alberta Heart Institute Clinical Fellowship, and a HSFC Research Scholarship.. NLM UID: 9503761. PMID: NLM22223497. 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 - Journal of Family Nursing TI - The Process of Family Management When a Baby Is Diagnosed With a Lethal Congenital Condition UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104516732&site=ehost-live&scope=site VL - 18 ID - 1600 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 - G.R. Rempel, Faculty of Nursing, University of Alberta, Edmonton, AB, Canada AU - Rempel, G. R. AU - Harrison, M. J. DB - Embase Medline DO - 10.1177/1049732307303164 IS - 6 KW - adult article child care child parent relation congenital heart disease female grounded theory health care personnel health promotion human hypoplastic left heart syndrome interview male medical technology parental attitude priority journal surgical approach survival uncertainty LA - English M3 - Article N1 - L46951859 2007-07-01 PY - 2007 SN - 1049-7323 1552-7557 SP - 824-837 ST - Safeguarding precarious survival: Parenting children who have life-threatening heart disease T2 - Qualitative Health Research TI - Safeguarding precarious survival: Parenting children who have life-threatening heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L46951859 http://dx.doi.org/10.1177/1049732307303164 VL - 17 ID - 1232 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. Copyright © Cambridge University Press 2009. AD - G.R. Rempel, University of Alberta, Faculty of Nursing, Clinical Sciences Building, Edmonton, AB T6G 2G3, Canada AU - Rempel, G. R. AU - Harrison, M. J. AU - Williamson, D. L. DB - Embase Medline DO - 10.1017/S1047951109003485 IS - 2 KW - article attitude to death child child care child growth child parent relation clinical article developmental disorder female growth curve heart surgery human hypoplastic left heart syndrome infant interview male parental attitude parenting education preschool child qualitative research surgical technology survivor underweight LA - English M3 - Article N1 - L355275345 2009-11-04 PY - 2009 SN - 1047-9511 1467-1107 SP - 135-144 ST - Is "Treat your child normally" helpful advice for parents of survivors of treatment of hypoplastic left heart syndrome? T2 - Cardiology in the Young TI - Is "Treat your child normally" helpful advice for parents of survivors of treatment of hypoplastic left heart syndrome? UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L355275345 http://dx.doi.org/10.1017/S1047951109003485 VL - 19 ID - 1166 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 - 105485073. Language: English. Entry Date: 20090612. Revision Date: 20150711. Publication Type: Journal Article AU - Rempel, G. R. AU - Harrison, M. J. AU - Williamson, D. L. DB - ccm DO - 10.1017/S1047951109003485 DP - EBSCOhost IS - 2 KW - Consent -- Ethical Issues Heart Surgery -- Methods Hypoplastic Left Heart Syndrome -- Surgery Parent-Child Relations Parents -- Education Adult Alberta Child Development Child, Preschool Consent -- Psychosocial Factors Female Hypoplastic Left Heart Syndrome -- Mortality Infant Male Middle Age Prospective Studies Quality of Life Questionnaires Retrospective Design Survival -- Trends Human N1 - research. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. NLM UID: 9200019. PMID: NLM19272201. PY - 2009 SN - 1047-9511 SP - 135-144 ST - Is 'treat your child normally' helpful advice for parents of survivors of treatment of hypoplastic left heart syndrome? T2 - Cardiology in the Young TI - Is 'treat your child normally' helpful advice for parents of survivors of treatment of hypoplastic left heart syndrome? UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105485073&site=ehost-live&scope=site VL - 19 ID - 1575 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. Copyright © Cambridge University Press 2009. AD - University of Alberta, Faculty of Nursing, Clinical Sciences Building, Edmonton, AB T6G 2G3, Canada Faculty of Medicine and Dentistry, Department of Pediatrics, University of Alberta, Edmonton, AB, Canada Department of Human Ecology, University of Alberta, Edmonton, AB, Canada Mazankowski Alberta Heart Institute, Edmonton, AB, Canada AU - Rempel, G. R. AU - Harrison, M. J. AU - Williamson, D. L. DB - Scopus DO - 10.1017/S1047951109003485 IS - 2 KW - Cardio-thoracic nursing Child development Child nursing Congenital heart disease Early intervention Family care M3 - Article N1 - Cited By :24 Export Date: 15 June 2020 PY - 2009 SP - 135-144 ST - Is "Treat your child normally" helpful advice for parents of survivors of treatment of hypoplastic left heart syndrome? T2 - Cardiology in the Young TI - Is "Treat your child normally" helpful advice for parents of survivors of treatment of hypoplastic left heart syndrome? UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-66149156244&doi=10.1017%2fS1047951109003485&partnerID=40&md5=b8ba891ccf525e2cb37ba764e833d9c8 VL - 19 ID - 2170 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 (6months-4·5years) 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. © 2012 Blackwell Publishing Ltd. AD - G.R. Rempel, Faculty of Nursing, University of Alberta, Edmonton, AL, Canada AU - Rempel, G. R. AU - Ravindran, V. AU - Rogers, L. G. AU - Magill-Evans, J. DB - Medline DO - 10.1111/j.1365-2648.2012.06044.x IS - 3 KW - adaptive behavior adult aged article caregiver child parent relation family female human hypoplastic left heart syndrome infant male mental stress middle aged parent preschool child psychological aspect qualitative research LA - English M3 - Article N1 - L52028862 2013-08-09 PY - 2013 SN - 0309-2402 1365-2648 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 TI - Parenting under Pressure: A grounded theory of parenting young children with life-threatening congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52028862 http://dx.doi.org/10.1111/j.1365-2648.2012.06044.x VL - 69 ID - 1016 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 - 104315960. Language: English. Entry Date: 20130215. Revision Date: 20190412. Publication Type: Journal 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: Advanced Nursing Practice; Pediatric Care. Grant Information: University of Alberta Hospital Foundation and the University of Alberta.. PMID: NLM22616855. 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=104315960&site=ehost-live&scope=site VL - 69 ID - 1531 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 (6months-4·5years) 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. © 2012 Blackwell Publishing Ltd. AD - Faculty of Nursing, University of Alberta, Edmonton, AL, Canada Department of Pediatrics, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, AL, Canada College of Nursing, Christian Medical College, Vellore, TN, India Faculty of Rehabilitation Medicine, University of Alberta, Edmonton, AL, Canada AU - Rempel, G. R. AU - Ravindran, V. AU - Rogers, L. G. AU - Magill-Evans, J. DB - Scopus DO - 10.1111/j.1365-2648.2012.06044.x IS - 3 KW - Congenital heart disease Grounded theory Hypoplastic left heart syndrome Nursing Parenting Qualitative research M3 - Article N1 - Cited By :49 Export Date: 15 June 2020 PY - 2013 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 TI - Parenting under Pressure: A grounded theory of parenting young children with life-threatening congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84873994018&doi=10.1111%2fj.1365-2648.2012.06044.x&partnerID=40&md5=1f6b5028543ebbc2cc3a8088f00e2ba6 VL - 69 ID - 2058 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 - Division of Pediatric Cardiology, Mattel Children's Hospital, University of California Los Angeles (UCLA) Medical Center, 10833 Le Conte Avenue, Los Angeles, CA 90095, United States Division of Pediatric Cardiology, Harbor-UCLA Medical Center, 1000 W. Carson Street, Torrance, CA 90502, United States Division of Pediatric Cardiology, Cedars-Sinai Medical Center, 8700 Beverly Blvd., Los Angeles, CA 90048, United States Division of Critical Care Medicine, Childrens Hospital Los Angeles, Mailstop #12, 4650 Sunset Blvd, Los Angeles, CA 90027, United States Division of Cardiology, Childrens Hospital Los Angeles, Mailstop #34, 4650 Sunset Blvd, Los Angeles, CA 90027, United States 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 - Scopus DO - 10.1002/pd.1835 IS - 11 KW - Comfort care Ethics Pregnancy M3 - Article N1 - Cited By :11 Export Date: 15 June 2020 PY - 2007 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-36148972427&doi=10.1002%2fpd.1835&partnerID=40&md5=10509ab0db4eb6153e705c1abd69670c VL - 27 ID - 2204 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, 300 Longwood Ave, Boston, MA 02115; jonathan-rhodes@cardio.chboston.org AN - 106417050. Language: English. Entry Date: 20060331. Revision Date: 20200316. Publication Type: Journal Article 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. DB - ccm DO - 10.1542/peds.2004-2697 DP - EBSCOhost IS - 6 KW - Exercise Tolerance Heart Defects, Congenital -- Rehabilitation Rehabilitation, Cardiac -- Methods Adolescence Child Funding Source Hemodynamics Hospitals, Pediatric McNemar's Test Oxygen Consumption Paired T-Tests Pearson's Correlation Coefficient Physical Activity Pilot Studies Human N1 - research; tables/charts. Journal Subset: Biomedical; Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. Grant Information: Deborah Munroe Noonan Memorial Fund and by the Pediatric Health Network (5U01 HL068285). NLM UID: 0376422. PMID: NLM16322156. PY - 2005 SN - 0031-4005 SP - 1339-1345 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106417050&site=ehost-live&scope=site VL - 116 ID - 1607 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. Copyright © 2005 by the American Academy of Pediatrics. AD - Department of Pediatric Cardiology, Children's Hospital, Boston, MA, United States Department of Cardiology, 300 Longwood Ave, Boston, MA 02115, United States 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. DB - Scopus DO - 10.1542/peds.2004-2697 IS - 6 KW - Cardiac rehabilitation Congenital heart defects Exercise M3 - Article N1 - Cited By :91 Export Date: 15 June 2020 PY - 2005 SP - 1339-1345 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-33644698113&doi=10.1542%2fpeds.2004-2697&partnerID=40&md5=26833e167417a8ca0fdc5c5ee0be2faa VL - 116 ID - 2235 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. They 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 - Depts. of Pediat., Surg. and Anesth., Columbia University, Presbyterian Hospital, New York, NY, United States Division of Pediatric Cardiology, SUNY Hlth. Sci. Center at Brooklyn, Box 1200, 450 Clarkson Avenue, Brooklyn, NY 11203, United States AU - Rhodes, J. AU - Garofano, R. P. AU - Bowman Jr, F. O. AU - Grant, G. P. AU - Bierman, F. Z. AU - Gersony, W. M. DB - Scopus DO - 10.1161/01.CIR.81.6.1811 IS - 6 KW - Single ventricle Tetralogy of fallot Tricuspid atresia M3 - Article N1 - Cited By :50 Export Date: 15 June 2020 PY - 1990 SP - 1811-1817 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0025336437&doi=10.1161%2f01.CIR.81.6.1811&partnerID=40&md5=0fea439db72e570797370a9e1c31e006 VL - 81 ID - 2318 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. © 2013 Elsevier Ireland Ltd. All rights reserved. AD - J. Rhodes, 300 Longwood Ave, Boston, MA 02115, United States 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. DB - Embase Medline DO - 10.1016/j.ijcard.2013.03.014 IS - 3 KW - iloprost oxygen placebo adolescent adult article cardiopulmonary exercise test child clinical article controlled study crossover procedure double blind procedure drug effect exercise female Fontan procedure heart atrium flutter heart single ventricle human hypoplastic left heart syndrome male nebulizer oxygen tension postoperative period priority journal prospective study randomized controlled trial school child sore throat thorax pain tricuspid valve atresia LA - English M3 - Article N1 - L52516106 2013-04-02 2013-11-13 PY - 2013 SN - 0167-5273 1874-1754 SP - 2435-2440 ST - Effect of inhaled iloprost on the exercise function of Fontan patients: A demonstration of concept T2 - International Journal of Cardiology TI - Effect of inhaled iloprost on the exercise function of Fontan patients: A demonstration of concept UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52516106 http://dx.doi.org/10.1016/j.ijcard.2013.03.014 VL - 168 ID - 986 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. © 2013 Elsevier Ireland Ltd. All rights reserved. AD - Department of Cardiology, Boston Children's Hospital, Boston, MA, United States 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. DB - Scopus DO - 10.1016/j.ijcard.2013.03.014 IS - 3 KW - Exercise testing Fontan procedure Heart defects, congenital Vasodilation M3 - Article N1 - Cited By :39 Export Date: 15 June 2020 PY - 2013 SP - 2435-2440 ST - Effect of inhaled iloprost on the exercise function of Fontan patients: A demonstration of concept T2 - International Journal of Cardiology TI - Effect of inhaled iloprost on the exercise function of Fontan patients: A demonstration of concept UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84885640563&doi=10.1016%2fj.ijcard.2013.03.014&partnerID=40&md5=f35a74b5d79aeee241ead8563b36901e VL - 168 ID - 2035 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-β (TGF-β)/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-β/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-β/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. © 2010 Elsevier Inc. All rights reserved. AD - M. Ricci, Pediatric Cardiac Surgery, University of Miami, Jackson Memorial Hospital, 1611 NW 12th Avenue (R-114), Miami, FL 33136, United States 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 - Embase Medline DO - 10.1016/j.cardfail.2010.03.007 IS - 8 KW - activin receptor 2 activin receptor like kinase 1 bone morphogenetic protein bone morphogenetic protein 5 cyclin dependent kinase growth differentiation factor 3 osteogenin transforming growth factor beta article cell growth cell survival clinical article controlled study female gene expression genetic variability gestational age heart development heart left ventricle cardiac muscle heart right ventricle human hypoplastic left heart syndrome male newborn priority journal real time polymerase chain reaction signal transduction LA - English M3 - Article N1 - L50896914 2010-05-05 2010-08-27 PY - 2010 SN - 1071-9164 1532-8414 SP - 628-634 ST - Differential changes in TGF-β/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-β/BMP signaling pathway in the right ventricular myocardium of newborns with hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50896914 http://dx.doi.org/10.1016/j.cardfail.2010.03.007 VL - 16 ID - 1139 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-β (TGF-β)/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-β/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-β/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. © 2010 Elsevier Inc. All rights reserved. AD - Division of Cardiothoracic Surgery, University of Miami, Holtz Children's Hospital, Miami, FL, United States Cytogenetics Laboratory, Department of Zoology, BHU, Varanasi, India Department of Pathology, University of Miami, Holtz Children's Hospital, Miami, FL, United States Division of Pediatric Cardiology, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, United States Department of Molecular Physiology and Biophysics, Baylor College of Medicine, Houston, TX, United States Department of Molecular and Cellular Pharmacology, University of Miami, Miller School of Medicine, Miami, FL, United States 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 - Scopus DO - 10.1016/j.cardfail.2010.03.007 IS - 8 KW - gene expression heart failure Hypoplastic left heart M3 - Article N1 - Cited By :22 Export Date: 15 June 2020 PY - 2010 SP - 628-634 ST - Differential changes in TGF-β/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-β/BMP signaling pathway in the right ventricular myocardium of newborns with hypoplastic left heart syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-77955512394&doi=10.1016%2fj.cardfail.2010.03.007&partnerID=40&md5=4354f35d4ef5472cc3102f76d0a57492 VL - 16 ID - 2147 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 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 AN - 112240097. Corporate Author: Western Canadian Complex Pediatric Therapies Follow-up Group. Language: English. Entry Date: 20160603. Revision Date: 20190620. Publication Type: journal article AU - Ricci, M. Florencia AU - Alton, Gwen Y. AU - Ross, David B. AU - Dicken, Bryan J. AU - Moddemann, Diane M. AU - Robertson, Charlene M. T. DB - ccm DO - 10.1016/j.jpeds.2015.10.087 DP - EBSCOhost KW - Postoperative Care Enteral Nutrition Gastrostomy Heart Surgery Developmental Disabilities -- Prevention and Control Early Intervention Prospective Studies Female Infant, Newborn Human Male Validation Studies Comparative Studies Evaluation Research Multicenter Studies Scales Clinical Assessment Tools N1 - research. Journal Subset: Biomedical; Peer Reviewed; USA. Instrumentation: Bayley Scales of Infant and Toddler Development-Third Edition; Adaptive Behavior Assessment System-Second Edition. NLM UID: 0375410. PMID: NLM26651431. PY - 2016 SN - 0022-3476 SP - 160-165.e1 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=112240097&site=ehost-live&scope=site VL - 169 ID - 1630 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 X² 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. (PsycINFO Database Record (c) 2016 APA, all rights reserved) AD - Robertson, Charlene M. T., Glenrose Rehabilitation Hospital, 10230 111 Ave, Edmonton, AB, Canada, T5G 0B7 AN - 2016-05316-029 AU - Ricci, M. Florencia AU - Alton, Gwen Y. AU - Ross, David B. AU - Dicken, Bryan J. AU - Moddemann, Diane M. AU - Robertson, Charlene M. T. DB - psyh DO - 10.1016/j.jpeds.2015.10.087 DP - EBSCOhost KW - gastrostomy tube feeding neonatal complex cardiac surgery early developmental intervention cardiopulmonary bypass Delayed Development Early Intervention Eating Behavior Heart Surgery Early Childhood Development N1 - Division of Developmental Pediatrics, Department of Pediatrics, University of Alberta, Edmonton, AB, Canada. Institutional Authors: Western Canadian Complex Pediatric Therapies Follow-up Group. Release Date: 20160505. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Delayed Development; Early Intervention; Eating Behavior; Heart Surgery. Minor Descriptor: Early Childhood Development. Classification: Medical Treatment of Physical Illness (3363). Population: Human (10); Male (30); Female (40). Location: Canada. Age Group: Childhood (birth-12 yrs) (100). Tests & Measures: Adaptive Behavior Assessment System-Second Edition; Bayley Scales of Infant Development-Second Edition; Bayley Scales of Infant and Toddler Development, Third Edition DOI: 10.1037/t14978-000. Methodology: Empirical Study; Followup Study; Quantitative Study. References Available: Y. Page Count: 6. Issue Publication Date: Feb, 2016. Publication History: Accepted Date: Oct 28, 2015; Revised Date: Sep 10, 2015; First Submitted Date: Jul 7, 2015. Copyright Statement: All rights reserved. Elsevier Inc. 2016. Sponsor: Government of Alberta, Alberta Health and Wellness, Canada. Grant: 1507. Date: from 2000 to 2006. Recipients: No recipient indicated Sponsor: Western Canadian Complex Pediatric Therapies Followup Group, Canada. Recipients: No recipient indicated PY - 2016 SN - 0022-3476 SP - 160-165 ST - Gastrostomy tube feeding after neonatal complex cardiac surgery identifies the need for early developmental intervention T2 - The Journal of Pediatrics TI - Gastrostomy tube feeding after neonatal complex cardiac surgery identifies the need for early developmental intervention UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2016-05316-029&site=ehost-live&scope=site Charlene.Robertson@albertahealthservices.ca VL - 169 ID - 1674 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 - A.F. Riley, Section of Pediatric Cardiology, Department of Pediatrics, Texas Children’s Hospital, Baylor College of Medicine, Houston, TX, United States AU - Riley, A. F. AU - Ocampo, E. C. AU - Hagan, J. AU - Lantin-Hermoso, M. R. DB - Embase Medline DO - 10.1111/chd.12774 IS - 5 KW - echocardiograph portable ultrasound scanner age distribution article body weight cardiac imaging cardiologist child clinical article controlled study correlation coefficient diagnostic accuracy diagnostic test accuracy study disease severity echocardiography female Glenn shunt heart right ventricle function human hypoplastic left heart syndrome infant male morbidity mortality newborn Norwood procedure pediatrician pilot study priority journal prospective study risk factor sensitivity and specificity single blind procedure systolic dysfunction tricuspid valve regurgitation Vscan LA - English M3 - Article N1 - L627274836 2019-05-14 2019-10-31 PY - 2019 SN - 1747-0803 1747-079X SP - 706-712 ST - Hand-held echocardiography in children with hypoplastic left heart syndrome T2 - Congenital Heart Disease TI - Hand-held echocardiography in children with hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L627274836 http://dx.doi.org/10.1111/chd.12774 VL - 14 ID - 598 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. © 2019 Wiley Periodicals, Inc. AD - Section of Pediatric Cardiology, Department of Pediatrics, Texas Children’s Hospital, Baylor College of Medicine, Houston, TX, United States Newborn Center, Texas Children's Hospital, Houston, TX, United States AU - Riley, A. F. AU - Ocampo, E. C. AU - Hagan, J. AU - Lantin-Hermoso, M. R. DB - Scopus DO - 10.1111/chd.12774 IS - 5 KW - congenital heart disease focused cardiac ultrasound hypoplastic left heart syndrome M3 - Article N1 - Export Date: 15 June 2020 PY - 2019 SP - 706-712 ST - Hand-held echocardiography in children with hypoplastic left heart syndrome T2 - Congenital Heart Disease TI - Hand-held echocardiography in children with hypoplastic left heart syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85064515382&doi=10.1111%2fchd.12774&partnerID=40&md5=55eacc22ddac283d0214ba7b692e1dad VL - 14 ID - 1736 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 μg/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. © 2008 Wiley-Liss, Inc. AD - W. A. Helbing, Erasmus MC-Sophia Children's Hospital, Department of Paediatric Cardiology, Sp-2429, PO Box 2060, 3000 CB Rotterdam, Netherlands 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. DB - Embase Medline DO - 10.1002/jmri.21544 IS - 5 KW - adolescent adult artery blood flow artery compliance artery diameter article child clinical article contrast enhancement controlled study female Fontan procedure heart stroke volume human male priority journal pulmonary artery shear stress whole body MRI LA - English M3 - Article N1 - L352643283 2008-12-03 http://www3.interscience.wiley.com/cgi-bin/fulltext/121490724/PDFSTART PY - 2008 SN - 1053-1807 1522-2586 SP - 1101-1107 ST - Pulmonary artery size and function after fontan operation at a young age T2 - Journal of Magnetic Resonance Imaging TI - Pulmonary artery size and function after fontan operation at a young age UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L352643283 http://dx.doi.org/10.1002/jmri.21544 VL - 28 ID - 1195 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 μg/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. © 2008 Wiley-Liss, Inc. AD - Department of Pediatrics, Division of Cardiology, Erasmus MC - Sophia Children's Hospital, Rotterdam, Netherlands Department of Radiology, Erasmus MC, Rotterdam, Netherlands Department of Cardiology, Erasmus MC, Rotterdam, Netherlands Department of Pediatric Cardiology, University MC Utrecht-Wilhelmina Children's Hospital, Utrecht, Netherlands Children's Heart Center, UMC St. Radboud, Nijmegen, Netherlands Department of Cardiothoracic Surgery, Erasmus MC, Rotterdam, Netherlands Erasmus MC-Sophia Children's Hospital, Department of Paediatric Cardiology, Sp-2429, PO Box 2060, 3000 CB Rotterdam, Netherlands 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. DB - Scopus DO - 10.1002/jmri.21544 IS - 5 KW - Endothelial function Fontan operation Pulmonary artery size Shear stress M3 - Article N1 - Cited By :19 Export Date: 15 June 2020 PY - 2008 SP - 1101-1107 ST - Pulmonary artery size and function after fontan operation at a young age T2 - Journal of Magnetic Resonance Imaging TI - Pulmonary artery size and function after fontan operation at a young age UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-55749111186&doi=10.1002%2fjmri.21544&partnerID=40&md5=4182a9ab7ba2ed5f8b249b0463990b68 VL - 28 ID - 2189 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 μg/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 β-adrenergic stimulation. However, as a result of impaired preload with stress, cardiac output can be increased only by increasing heart rate. © 2008 Elsevier Inc. All rights reserved. AD - W.A. Helbing, Department of Pediatric Cardiology, Erasmus Medical Center, Sophia Children's Hospital, Rotterdam, Netherlands 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. C2 - Centrafarm(Netherlands) DB - Embase Medline DO - 10.1016/j.amjcard.2008.01.050 IS - 11 KW - dobutamine adolescent adrenergic system adult article atriopulmonary connection blood flow cavopulmonary connection child clinical article continuous infusion diagnostic value exercise test female follow up Fontan procedure groups by age heart afterload heart ejection fraction cardiac index heart muscle contractility heart output heart preload heart rate heart stress heart ventricle enddiastolic pressure heart ventricle function human low drug dose male nuclear magnetic resonance imaging postoperative complication priority journal statistical significance cerebrovascular accident venous return LA - English M3 - Article N1 - L50096648 2008-06-23 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://www.embase.com/search/results?subaction=viewrecord&from=export&id=L50096648 http://dx.doi.org/10.1016/j.amjcard.2008.01.050 VL - 101 ID - 1209 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 μg/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 β-adrenergic stimulation. However, as a result of impaired preload with stress, cardiac output can be increased only by increasing heart rate. © 2008 Elsevier Inc. All rights reserved. AD - Department of Pediatric Cardiology, Erasmus Medical Center, Sophia Children's Hospital, Rotterdam, Netherlands Department of Radiology, Erasmus Medical Center, Rotterdam, Netherlands Department of Cardiology, Erasmus Medical Center, Rotterdam, Netherlands Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, Netherlands Children's Heart Center, University Medical Center St. Radboud, Nijmegen, Netherlands Department of Pediatric Cardiology, University Medical Center Utrecht, Wilhelmina Children's Hospital, Utrecht, Netherlands 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. DB - Scopus DO - 10.1016/j.amjcard.2008.01.050 IS - 11 M3 - Article N1 - Cited By :35 Export Date: 15 June 2020 PY - 2008 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-43549099261&doi=10.1016%2fj.amjcard.2008.01.050&partnerID=40&md5=31c3cab503a080bc3b0318fe1cfaecb9 VL - 101 ID - 2193 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. © 2019, Springer Science+Business Media, LLC, part of Springer Nature. AD - Section of Endocrinology, Department of Pediatrics, Children’s Hospital Colorado, University of Colorado Anschutz Medical Campus, 13123 E 16th Ave, Box 265, Aurora, CO 80045, United States Section of Cardiology, Department of Pediatrics, Children’s Hospital Colorado, The Heart Institute, University of Colorado Anschutz Medical Campus, Aurora, CO, United States Section of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Children’s Hospital Colorado, The Digestive Health Institute, University of Colorado Anschutz Medical Campus, Aurora, CO, United States AU - Roberts, R. O., III AU - Di Maria, M. V. AU - Brigham, D. AU - Hsu, S. DB - Scopus DO - 10.1007/s00246-019-02248-3 IS - 2 KW - Adrenal suppression Budesonide Fontan Protein-losing enteropathy M3 - Article N1 - Export Date: 15 June 2020 PY - 2020 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85074827812&doi=10.1007%2fs00246-019-02248-3&partnerID=40&md5=80f5ca029c8d9de15dc7dab0986ccd75 VL - 41 ID - 1712 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. (J PEDIATR 1995;126:496-8). © 1995 Mosby, Inc. All rights reserved. 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 - Scopus DO - 10.1016/S0022-3476(95)70478-7 IS - 3 M3 - Article N1 - Cited By :124 Export Date: 15 June 2020 PY - 1995 SP - 496-498 ST - Neurodevelopmental outcome of infants with hypoplastic left heart syndrome T2 - The Journal of Pediatrics TI - Neurodevelopmental outcome of infants with hypoplastic left heart syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0028930436&doi=10.1016%2fS0022-3476%2895%2970478-7&partnerID=40&md5=702c04117177ee97204597feeb69fe4b VL - 126 ID - 2304 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 - A. Redington, R. Brompton Natl. Heart/Lung Hosp., Sydney Street, London SW3 6NP, United Kingdom AU - Rosenthal, M. AU - Bush, A. AU - Deanfield, J. AU - Redington, A. DB - Embase Medline DO - 10.1161/01.CIR.91.2.372 IS - 2 KW - adolescent article cardiopulmonary hemodynamics child clinical article controlled study exercise female Fontan procedure heart stroke volume human lung blood flow lung hemodynamics lung minute volume male oxygen consumption preschool child priority journal school child surgical technique LA - English M3 - Article N1 - L25018016 1995-02-01 PY - 1995 SN - 0009-7322 SP - 372-378 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L25018016 http://dx.doi.org/10.1161/01.CIR.91.2.372 VL - 91 ID - 1370 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 - Dept. of Paediatr. Resp. Medicine, Roy. Brompton Natl. Heart Lung Hosp., London, United Kingdom Department of Cardiology, Roy. Brompton Natl. Heart Lung Hosp., London, United Kingdom Cardiothoracic Unit, Hospital for Sick Children, London, United Kingdom Roy. Brompton Natl. Heart Lung Hosp., Sydney Street, London SW3 6NP, United Kingdom AU - Rosenthal, M. AU - Bush, A. AU - Deanfield, J. AU - Redington, A. DB - Scopus DO - 10.1161/01.CIR.91.2.372 IS - 2 KW - exercise pediatrics spectrometry surgery M3 - Article N1 - Cited By :95 Export Date: 15 June 2020 PY - 1995 SP - 372-378 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0028883241&doi=10.1161%2f01.CIR.91.2.372&partnerID=40&md5=b275c6a2ebb096d773ae73a8b258ff0b VL - 91 ID - 2303 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 (R2 = 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 - A. Rösner, Department of Cardiology, Division of Cardiothoracic and Respiratory Medicine, University Hospital of North Norway, Tromsø, Norway AU - Rösner, A. AU - Khalapyan, T. AU - Pedrosa, J. AU - Dalen, H. AU - McElhinney, D. B. AU - Friedberg, M. K. AU - Lui, G. K. DB - Embase Medline DO - 10.1111/echo.14169 IS - 12 KW - adolescent adult akinesia article coronary artery blood flow correlational study cross-sectional study female Fontan procedure geometry heart ejection fraction heart ventricle contractility heart ventricle function heart ventricle volume human major clinical study male palliative therapy priority journal retrospective study speckle tracking echocardiography systolic blood pressure LA - English M3 - Article N1 - L624532288 2018-10-29 2018-12-11 PY - 2018 SN - 1540-8175 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L624532288 http://dx.doi.org/10.1111/echo.14169 VL - 35 ID - 652 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 (R2 = 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 - 133318758. Language: English. Entry Date: 20181205. Revision Date: 20191202. Publication Type: Article AU - Rösner, Assami AU - Khalapyan, Tigran AU - Pedrosa, João AU - Dalen, Håvard AU - McElhinney, Doff B. AU - Friedberg, Mark K. AU - Lui, George K. DB - ccm DO - 10.1111/echo.14169 DP - EBSCOhost IS - 12 KW - Cardiopulmonary Bypass Blood Circulation Heart Ventricle -- Anatomy and Histology Ventricular Function, Left Mechanics Stress Human Adolescence Retrospective Design Cross Sectional Studies Ventricular Ejection Fraction Aged, 80 and Over Sex Factors Young Adult Adult Middle Age Movement Disorders N1 - diagnostic images; research; tables/charts. Journal Subset: Allied Health; Biomedical; Double Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. NLM UID: 8511187. 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=133318758&site=ehost-live&scope=site VL - 35 ID - 1432 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. © 2018 Wiley Periodicals, Inc. 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, CA, United States 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, CA, United States Division of Pediatric Cardiology, Hospital for Sick Children, Toronto, ON, Canada Division of Cardiovascular Medicine, Department of Medicine, Stanford University School of Medicine, Stanford, CA, United States AU - Rösner, A. AU - Khalapyan, T. AU - Pedrosa, J. AU - Dalen, H. AU - McElhinney, D. B. AU - Friedberg, M. K. AU - Lui, G. K. DB - Scopus DO - 10.1111/echo.14169 IS - 12 KW - Fontan circulation strain rate echocardiography ventricular function ventricular geometry M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2018 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85055289949&doi=10.1111%2fecho.14169&partnerID=40&md5=aaa02ab557d9c6e04793b5558086e185 VL - 35 ID - 1780 ER - TY - JOUR AD - L.F. Ross, Departments of Medicine, Pediatrics, Surgery and the College, MacLean Center for Clinical Medical Ethics, University of Chicago, Chicago, IL, United States AU - Ross, L. F. AU - Frader, J. DB - Embase Medline DO - 10.1016/j.jpeds.2009.03.008 IS - 1 KW - article child health care genetic disorder heart transplantation human hypoplastic left heart syndrome life extension medical decision making medical ethics medical practice palliative therapy priority journal quality of life risk factor survival rate LA - English M3 - Article N1 - L354864620 2009-07-31 PY - 2009 SN - 0022-3476 SP - 12-15 ST - Hypoplastic Left Heart Syndrome: A Paradigm Case for Examining Conscientious Objection in Pediatric Practice T2 - Journal of Pediatrics TI - Hypoplastic Left Heart Syndrome: A Paradigm Case for Examining Conscientious Objection in Pediatric Practice UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L354864620 http://dx.doi.org/10.1016/j.jpeds.2009.03.008 VL - 155 ID - 1178 ER - TY - JOUR AD - Department of Medicine, University of Chicago, Chicago, IL 60637, USA. Lross@uchicago.edu AN - 105374474. Language: English. Entry Date: 20090807. Revision Date: 20150711. Publication Type: Journal Article. Journal Subset: Biomedical AU - Ross, L. F. AU - Frader, J. DB - ccm DO - 10.1016/j.jpeds.2009.03.008 DP - EBSCOhost IS - 1 KW - Decision Making Hypoplastic Left Heart Syndrome -- Therapy Parents -- Psychosocial Factors Attitude of Health Personnel Counseling Infant Outcome Assessment Palliative Care -- Ethical Issues Professional-Family Relations N1 - Peer Reviewed; USA. NLM UID: 0375410. PMID: NLM19559290. PY - 2009 SN - 0022-3476 SP - 12-15 ST - Hypoplastic left heart syndrome: a paradigm case for examining conscientious objection in pediatric practice T2 - Journal of Pediatrics TI - Hypoplastic left heart syndrome: a paradigm case for examining conscientious objection in pediatric practice UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105374474&site=ehost-live&scope=site VL - 155 ID - 1599 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 - R.D. Ross, Division of Pediatric Cardiology, Children's Hospital of Michigan, Wayne State University School of Medicine, Detroit, Michigan 48201, USA. AU - Ross, R. D. DB - Medline IS - 1 KW - agents interacting with transmitter, hormone or drug receptors beta adrenergic receptor blocking agent cardiotonic agent digoxin dipeptidyl carboxypeptidase inhibitor diuretic agent article blood child clinical trial congestive heart failure diet therapy human infant pathophysiology LA - English M3 - Article N1 - L38431552 2004-04-15 PY - 2001 SN - 1175-3277 SP - 37-44 ST - Medical management of chronic heart failure in children T2 - American journal of cardiovascular drugs : drugs, devices, and other interventions TI - Medical management of chronic heart failure in children UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L38431552 VL - 1 ID - 1318 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 - Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, TX, United States Michael E. DeBakey Department of Surgery, Baylor College of Medicine, 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 - Scopus DO - 10.1016/j.athoracsur.2006.08.058 IS - 2 M3 - Article N1 - Cited By :21 Export Date: 15 June 2020 PY - 2007 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-33846366648&doi=10.1016%2fj.athoracsur.2006.08.058&partnerID=40&md5=888fdae7380bed6886ad15b4e1e030de VL - 83 ID - 2214 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 - 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 Department of Surgery, The Cardiac Center, The Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, United States Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, United States Department of Surgery, The Herma Heart Center, Children's Hospital of Wisconsin, Milwaukee, WI, United States Department of Pediatrics, The Herma Heart Center, Children's Hospital of Wisconsin, Milwaukee, WI, United States Department of Medicine, Medical College of Wisconsin, Milwaukee, WI, United States Department of Surgery, The Heart Institute, Cincinnati Children's Hospital, University of Cincinnati, Cincinnati, OH, United States Department of Surgery, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, United States Sacred Heart Medical Center and Children's Hospital, Spokane, WA, United States Department of Surgery, Duke Children's Hospital, Duke University School of Medicine, Durham, NC, 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 - Scopus DO - 10.1111/chd.12053 IS - 3 KW - Registry Single Ventricle Ventricular Assist Device M3 - Article N1 - Cited By :36 Export Date: 15 June 2020 PY - 2013 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84878732295&doi=10.1111%2fchd.12053&partnerID=40&md5=3395bb4bbd82d763710b8526798d44d1 VL - 8 ID - 2049 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. 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. DB - Medline DO - 10.1093/ejcts/ezx119 IS - 3 KW - child cognition cognitive defect developmental disorder female Germany human hypoplastic left heart syndrome incidence male neuropsychological test Norwood procedure pathophysiology physiology preschool child risk assessment risk factor treatment outcome LA - English M3 - Article N1 - L622205507 2018-05-23 PY - 2017 SN - 1873-734X SP - 558-564 ST - Neuro-developmental outcome in single-ventricle patients: is the Norwood procedure a risk factor? T2 - European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery TI - Neuro-developmental outcome in single-ventricle patients: is the Norwood procedure a risk factor? UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L622205507 http://dx.doi.org/10.1093/ejcts/ezx119 VL - 52 ID - 735 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 - R. Chabiniok, School of Biomedical Engineering and Imaging Sciences, St Thomas’ Hospital, King’s College London, London, United Kingdom 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. DB - Embase Medline DO - 10.1371/journal.pone.0229015 IS - 2 KW - dobutamine adolescent article biomechanics cardiac muscle child clinical article conceptual framework controlled study disease classification exercise test Fontan procedure heart catheterization heart cycle heart failure heart muscle contractility human muscle rigidity nuclear magnetic resonance imaging pathophysiology preschool child school child vascular resistance venous return workflow LA - English M3 - Article N1 - L2005026107 2020-02-27 2020-03-05 PY - 2020 SN - 1932-6203 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2005026107 http://dx.doi.org/10.1371/journal.pone.0229015 VL - 15 ID - 575 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. © 2020 Ruijsink et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. AD - School of Biomedical Engineering and Imaging Sciences, St Thomas’ Hospital, King’s College London, London, United Kingdom Department of Pediatrics, UT Southwestern Medical Center, Dallas, TX, United States Inria Centre de Recherche Saclay-Ile-de-France, Palaiseau, France LMS, Ecole Polytechnique, CNRS, Institut Polytechnique de Paris, Paris, France 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. C7 - e0229015 DB - Scopus DO - 10.1371/journal.pone.0229015 IS - 2 M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2020 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85079786304&doi=10.1371%2fjournal.pone.0229015&partnerID=40&md5=a492e4a5a23e73a8d59c8d64042a6d80 VL - 15 ID - 1717 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. © 2017 AD - Division of Cardiology, The Heart Institute, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave-MLC 2003, Cincinnati, Ohio 45229, United States AU - Ryan, T. D. AU - Chin, C. DB - Scopus DO - 10.1053/j.sempedsurg.2017.07.012 IS - 4 KW - Heart Pediatric Transplantation M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2017 SP - 206-212 ST - Pediatric cardiac transplantation T2 - Seminars in Pediatric Surgery TI - Pediatric cardiac transplantation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85028366316&doi=10.1053%2fj.sempedsurg.2017.07.012&partnerID=40&md5=9249f2e99692199119a97a6518d09e33 VL - 26 ID - 1847 ER - TY - JOUR AD - Division of Cardiology, Cardiac Center, Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, United States Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States AU - Rychik, J. AU - Goldberg, D. J. DB - Scopus DO - 10.1161/CIRCULATIONAHA.114.005341 IS - 17 M3 - Article N1 - Cited By :28 Export Date: 15 June 2020 PY - 2014 SP - 1525-1528 ST - Late consequences of the fontan operation T2 - Circulation TI - Late consequences of the fontan operation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84922042484&doi=10.1161%2fCIRCULATIONAHA.114.005341&partnerID=40&md5=dfbf82178731f33b2714cff357885c28 VL - 130 ID - 2010 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 - A. Rydberg, Section of Pediatric Cardiology, Department of Clinical Sciences, Umeå Hospital, S-901 85 Umeå, Sweden AU - Rydberg, A. AU - Rask, P. AU - Teien, D. E. AU - Hörnsten, R. DB - Embase Medline DO - 10.1007/s00246-002-0374-2 IS - 5 KW - digoxin adolescent ambulatory monitoring article child clinical article comparative study congenital heart disease controlled study correlation analysis daily life activity diagnostic procedure diagnostic value disease classification electrocardiography exercise test female Fontan procedure heart ventricle function human male ST segment depression statistical significance Danica Holter Replay Unit Sherpa recorder Tracker II LA - English M3 - Article N1 - L36993514 2003-08-28 PY - 2003 SN - 0172-0643 SP - 468-472 ST - Electrocardiographic ST segment depression and clinical function in children with Fontan circulation T2 - Pediatric Cardiology TI - Electrocardiographic ST segment depression and clinical function in children with Fontan circulation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L36993514 http://dx.doi.org/10.1007/s00246-002-0374-2 VL - 24 ID - 1299 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 Dept. of Surg./Perioperative Science, Umeå University Hospital, S-901 85 Umeå, Sweden AU - Rydberg, A. AU - Rask, P. AU - Teien, D. E. AU - Hörnsten, R. DB - Scopus DO - 10.1007/s00246-002-0374-2 IS - 5 KW - Electrocardiographic ST depression Fontan circulation NYHA classification M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2003 SP - 468-472 ST - Electrocardiographic ST segment depression and clinical function in children with Fontan circulation T2 - Pediatric Cardiology TI - Electrocardiographic ST segment depression and clinical function in children with Fontan circulation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0043023659&doi=10.1007%2fs00246-002-0374-2&partnerID=40&md5=a07cd47f39b1a3c2052a033f01d0e992 VL - 24 ID - 2258 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 Umeå, Umeå, Sweden Department of Clinical Physiology, University Hospital of Umeå, Umeå, Sweden AU - Rydberg, A. AU - Teien, D. E. AU - Rask, P. AU - Hörnsten, R. DB - Scopus DO - 10.1046/j.1365-2281.2000.00227.x IS - 1 KW - Children Electrocardiographic ST depression Fontan Univentricular heart M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2000 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0033972673&doi=10.1046%2fj.1365-2281.2000.00227.x&partnerID=40&md5=2626e5437938f1f6f50b978b25a9451c VL - 20 ID - 2279 ER - TY - JOUR AU - Saavedra, M. J. AU - Eymann, A. AU - Pérez, L. AU - Busaniche, J. AU - Nápoli, N. AU - Marantz, P. AU - Llera, J. DB - Medline DO - 10.5546/aap.2020.eng.166 IS - 3 KW - article child chronic disease cohort analysis controlled study female general hospital heart single ventricle heart surgery human major clinical study male newborn period observational study preschool child quality of life surgery LA - English Spanish M3 - Article N1 - L631938634 2020-06-05 PY - 2020 SN - 1668-3501 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 - Archivos argentinos de pediatria TI - Health related quality of life in children with congenital heart disease that undergo cardiac surgery during their first year of life UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L631938634 http://dx.doi.org/10.5546/aap.2020.eng.166 VL - 118 ID - 550 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. Sociedad Argentina de Pediatría. Introducción. En las últimas décadas, el desarrollo de métodos diagnósticos, técnicas quirúrgicas y cuidados intensivos, aumentó la sobrevida de los niños con cardiopatía congénita. En este contexto, las secuelas a largo plazo adquirieron mayor relevancia. La calidad de vida relacionada con la salud mide el impacto de las enfermedades crónicas. Nuestro objetivo fue describir la calidad de vida relacionada con la salud en pacientes con cardiopatía congénita operados durante el primer año de vida. Población y métodos. Estudio observacional, transversal, realizado entre agosto de 2017 y diciembre de 2018 en un hospital general universitario. Se utilizó el cuestionario PedsQL 4.0, versión genérica. Se incluyeron pacientes de entre 2 y 4 años con cardiopatía congénita operados durante el primer año de vida y niños sanos. Se compararon los resultados con T-Test o Wilcoxon según la distribución observada. Se consideró significativo el valor de p < 0,05. Resultados. Se incluyeron 31 pacientes. El 26 % tenían ventrículo único. La primera cirugía fue, en un 61,3 %, en el período neonatal. No hubo diferencia (p = 0,10) entre la calidad de vida relacionada con la salud global de la población con cardiopatía congénita y de los niños sanos. Sin embargo, se observaron puntajes más bajos con diferencia estadísticamente significativa en las esferas social (p = 0,0092) y escolar (p = 0,0001). Conclusiones. Nuestra cohorte de niños con cardiopatía congénita tiene una calidad de vida global comparable con la de los niños sanos, excepto en las escalas correspondientes a funcionamiento social y escolar. AD - Servicio de Clínica Pediátrica, Departamento de Pediatría, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires Servicio de Cardiología Pediátrica, Departamento de Pediatría, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires AU - Saavedra, M. J. AU - Eymann, A. AU - Pérez, L. AU - Busaniche, J. AU - Nápoli, N. AU - Marantz, P. AU - Llera, J. DB - Scopus DO - 10.5546/aap.2020.eng.166 IS - 3 KW - cardiac surgery chronic disease congenital heart disease health related quality of life M3 - Article N1 - Export Date: 15 June 2020 PY - 2020 SP - 166-172 ST - Calidad de vida relacionada con la salud en niños con cardiopatía congénita operados durante el primer año de vida T2 - Archivos argentinos de pediatria TI - Health related quality of life in children with congenital heart disease that undergo cardiac surgery during their first year of life UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85085714412&doi=10.5546%2faap.2020.eng.166&partnerID=40&md5=3ae31aa8a29e1079f6658fea5b1e9559 VL - 118 ID - 1700 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 - A.A. Sabati, Division of Cardiology, Children's Hospital Los Angeles, Mail Stop 34, 4650 Sunset Boulevard, Los Angeles, CA, United States 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. DB - Embase Medline DO - 10.1016/j.healun.2015.01.006 IS - 7 KW - inotropic agent adolescent adult article artificial ventilation body weight cardiovascular mortality child childhood mortality congenital heart disease death end stage heart failure female follow up growth disorder heart failure heart single ventricle heart surgery heart transplantation human infant inotropism major clinical study male priority journal quality of life retrospective study survival LA - English M3 - Article N1 - L602902794 2015-03-17 2015-07-01 PY - 2015 SN - 1557-3117 1053-2498 SP - 906-911 ST - Survival and quality of life for children with end-stage heart failure who are not candidates for cardiac transplant T2 - Journal of Heart and Lung Transplantation TI - Survival and quality of life for children with end-stage heart failure who are not candidates for cardiac transplant UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L602902794 http://dx.doi.org/10.1016/j.healun.2015.01.006 VL - 34 ID - 861 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. © 2015 International Society for Heart and Lung Transplantation. AD - Division of Cardiology, Children's Hospital Los Angeles, Mail Stop 34, 4650 Sunset Boulevard, Los Angeles, CA 90027, United States Department of Cardiothoracic Surgery, Children's Hospital Los Angeles, University of Southern California, Los Angeles, CA, United States Clinical Investigation Center, Children's Hospital Los Angeles, University of Southern California, Los Angeles, CA, United States 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. DB - Scopus DO - 10.1016/j.healun.2015.01.006 IS - 7 KW - alloimmunization congenital heart disease congenital heart surgery panel reactive antibodies Pediatric heart failure pediatric heart transplant M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2015 SP - 906-911 ST - Survival and quality of life for children with end-stage heart failure who are not candidates for cardiac transplant T2 - Journal of Heart and Lung Transplantation TI - Survival and quality of life for children with end-stage heart failure who are not candidates for cardiac transplant UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84931561176&doi=10.1016%2fj.healun.2015.01.006&partnerID=40&md5=cb95e4dbfd98b5c92c1c71b32a219913 VL - 34 ID - 1960 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 - A. Zolfi-Gol, Isfahan University of Medical Sciences, Isfahan, Iran AU - Sabri, M. R. AU - Zolfi-Gol, A. AU - Ahmadi, A. AU - Haghjooy-Javanmard, S. DB - Embase Medline DO - 10.1007/s00246-015-1238-x IS - 1 KW - biochemical marker intercellular adhesion molecule lymphocyte antigen nitric oxide tadalafil unclassified drug vascular cell adhesion molecule adolescent arterial wall thickness article blood vessel parameters cell function clinical article demography endothelial function exercise female flow mediated dilatation follow up Fontan procedure heart function heart ventricle function human male modified fontan operation morbidity mortality New York Heart Association class single drug dose LA - English M3 - Article N1 - L605390722 2015-08-03 2016-02-22 PY - 2016 SN - 1432-1971 0172-0643 SP - 55-61 ST - Effect of Tadalafil on Myocardial and Endothelial Function and Exercise Performance After Modified Fontan Operation T2 - Pediatric Cardiology TI - Effect of Tadalafil on Myocardial and Endothelial Function and Exercise Performance After Modified Fontan Operation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L605390722 http://dx.doi.org/10.1007/s00246-015-1238-x VL - 37 ID - 830 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. © 2015, Springer Science+Business Media New York. AD - Isfahan University of Medical Sciences, Isfahan, Iran AU - Sabri, M. R. AU - Zolfi-Gol, A. AU - Ahmadi, A. AU - Haghjooy-Javanmard, S. DB - Scopus DO - 10.1007/s00246-015-1238-x IS - 1 KW - Endothelial function Exercise Fontan procedure Single ventricle Tadalafil M3 - Article N1 - Cited By :8 Export Date: 15 June 2020 PY - 2016 SP - 55-61 ST - Effect of Tadalafil on Myocardial and Endothelial Function and Exercise Performance After Modified Fontan Operation T2 - Pediatric Cardiology TI - Effect of Tadalafil on Myocardial and Endothelial Function and Exercise Performance After Modified Fontan Operation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84957440995&doi=10.1007%2fs00246-015-1238-x&partnerID=40&md5=2964f033a3be6bf91db806d50a7c6bc9 VL - 37 ID - 1925 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 - Department of Pediatrics, Children's Hospital of New York, 3959 Broadway, 10-24, New York, NY 10032, United States Department of Pediatrics, Columbia University, New York, United States AU - Sachdeva, T. AU - Morris, M. C. DB - Scopus DO - 10.1542/peds.2013-1700 IS - 5 KW - Best-interest standard Research ethics higher-hazard no-benefit research M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2013 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84887081863&doi=10.1542%2fpeds.2013-1700&partnerID=40&md5=d8c194552d830a8c00f4c09ffc01fdbe VL - 132 ID - 2033 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/m2/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 - H. Senzaki, Division of Pediatric Cardiology, Saitama Medical Center, Saitama Medical University, Saitama, Japan AU - Saiki, H. AU - Kuwata, S. AU - Kurishima, C. AU - Iwamoto, Y. AU - Ishido, H. AU - Masutani, S. AU - Senzaki, H. DB - Embase Medline DO - 10.1016/j.amjcard.2014.08.030 IS - 10 KW - aldosterone creatinine hydrocortisone adrenal insufficiency aldosterone cortisol imbalance aldosterone urine level article ascites cardiopulmonary bypass central venous pressure child circadian rhythm clinical article controlled study correlation analysis creatinine clearance elevated blood pressure fluid balance Fontan procedure forward heart failure heart hemodynamics cardiac index heart output human hydrocortisone urine level intensive care unit length of stay mean arterial pressure metabolic disorder pleura effusion postoperative complication postoperative period systemic vascular resistance urine volume water retention LA - English M3 - Article N1 - L600307559 2014-11-07 2014-11-13 PY - 2014 SN - 1879-1913 0002-9149 SP - 1578-1583 ST - Aldosterone-cortisol imbalance immediately after Fontan operation with implications for abnormal fluid homeostasis T2 - American Journal of Cardiology TI - Aldosterone-cortisol imbalance immediately after Fontan operation with implications for abnormal fluid homeostasis UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L600307559 http://dx.doi.org/10.1016/j.amjcard.2014.08.030 VL - 114 ID - 906 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/m2/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. © 2014 Elsevier Inc. All rights reserved. AD - Division of Pediatric Cardiology, Saitama Medical Center, Saitama Medical University, Saitama, Japan AU - Saiki, H. AU - Kuwata, S. AU - Kurishima, C. AU - Iwamoto, Y. AU - Ishido, H. AU - Masutani, S. AU - Senzaki, H. DB - Scopus DO - 10.1016/j.amjcard.2014.08.030 IS - 10 M3 - Article N1 - Cited By :9 Export Date: 15 June 2020 PY - 2014 SP - 1578-1583 ST - Aldosterone-cortisol imbalance immediately after Fontan operation with implications for abnormal fluid homeostasis T2 - American Journal of Cardiology TI - Aldosterone-cortisol imbalance immediately after Fontan operation with implications for abnormal fluid homeostasis UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84908341334&doi=10.1016%2fj.amjcard.2014.08.030&partnerID=40&md5=fe1708a0c4e7579050039619dcdc6a30 VL - 114 ID - 2026 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. © 2019 The Authors. Journal of Cellular and Molecular Medicine published by John Wiley & Sons Ltd and Foundation for Cellular and Molecular Medicine. AD - Department of Physiology and Pharmacology, Schulich School of Medicine and Dentistry, University of Western Ontario, Children's Health Research Institute, London, ON, Canada Department of Medicine, 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 - Scopus DO - 10.1111/jcmm.14439 IS - 8 KW - congenital heart defects exercise heart development oxidative stress pre-gestational diabetes M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2019 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85069640768&doi=10.1111%2fjcmm.14439&partnerID=40&md5=d72d34e29e98f554e2bf6eb02cc76587 VL - 23 ID - 1739 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 - D. Bonnet, Service de Cardiologie Pediatrique, Inst. Cardiol. Congenitale Adultes, Hôpital Necker-Enfants Malades, 149 rue de Sèvres, 75743 Paris Cedex 15, France AU - Saliba, Z. AU - Butera, G. AU - Bonnet, D. AU - Bonhoeffer, P. AU - Villain, E. AU - Kachaner, J. AU - Sidi, D. AU - Iserin, L. DB - Embase Medline IS - 1 KW - adolescent adult age article clinical feature controlled study cyanosis demography health status heart disease heart single ventricle human major clinical study mental health mitral valve atresia orthopedics prediction priority journal psychosocial disorder quality of life retrospective study scoring system social aspect stress LA - English M3 - Article N1 - L32977784 2001-10-30 PY - 2001 SN - 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L32977784 VL - 86 ID - 1319 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 Cong&eacue;nitale des Adultes, Hôpital Necker -- Enfants Malades, 149 rue de Sèvres, 75743 Paris Cedexl5, France. AN - 12940846. Language: English. Entry Date: 20020118. Revision Date: 20190515. Publication Type: journal article AU - Saliba, Z. AU - Butera, G. AU - Bonnet, D. AU - Bonhoeffer, P. AU - Villain, E. AU - Kachaner, J. AU - Sidi, D. AU - Iserin, L. DB - ccm DO - 10.1136/heart.86.1.69 DP - EBSCOhost IS - 1 KW - Heart Defects, Congenital -- Psychosocial Factors Health Status Quality of Life Middle Age Retrospective Design Heart Ventricle -- Abnormalities Cross Sectional Studies Adult Human Adolescence Male Female Validation Studies Comparative Studies Evaluation Research Multicenter Studies Questionnaires N1 - research. Journal Subset: Biomedical; Blind Peer Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Instrumentation: General Health Questionnaire (GHQ). NLM UID: 9602087. PMID: NLM11410565. PY - 2001 SN - 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=12940846&site=ehost-live&scope=site VL - 86 ID - 1586 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, Institut de Cardiologie Congénitale des Adultes, Hôpital Necker-Enfants Malades, 149 rue de Sèvres, 75743 Paris Cedex 15, France AU - Saliba, Z. AU - Butera, G. AU - Bonnet, D. AU - Bonhoeffer, P. AU - Villain, E. AU - Kachaner, J. AU - Sidi, D. AU - Iserin, L. DB - Scopus DO - 10.1136/heart.86.1.69 IS - 1 KW - Congenital heart defect Psychosocial problems Quality of life Univentricular heart M3 - Article N1 - Cited By :82 Export Date: 15 June 2020 PY - 2001 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0034758383&doi=10.1136%2fheart.86.1.69&partnerID=40&md5=39515e03c029d3c10a2398c7bca36195 VL - 86 ID - 2272 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 - Kardiocentrum, Teaching Hospital Motol, V uvalu 84, 150 18 Praha 5, Czech Republic AU - Samanek, M. DB - Scopus IS - 1 KW - Boys:girls ratio Cardiac malformation Children Critical heart disease Prevalence Quality of life Stillborns Survival M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 1994 SP - 11-14 ST - Congenital heart diseases in Bohemia. Epidemiologic data T2 - Cor et Vasa TI - Congenital heart diseases in Bohemia. Epidemiologic data UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0028345892&partnerID=40&md5=9d1568ea8540bdca081092cb9bcffe6f VL - 36 ID - 2311 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 - Cardiac Intensive Care Unit, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Passeig de la Vall d'Hebron 119-129, 08035 Barcelona, Spain Department of Pediatric Intensive Care, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain Department of Pediatric Cardiac Surgery, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain Department of Cardiology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain AU - Sanchez-de-Toledo, J. AU - Vega, L. AU - Gil, L. AU - Balcells, J. AU - Abella, F. AU - Gran, F. DB - Scopus IS - 8 KW - Acute respiratory distress syndrome Cavopulmonary anastomosis Congenital heart disease Ventilation M3 - Article N1 - Export Date: 15 June 2020 PY - 2014 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84905843108&partnerID=40&md5=05a4d2bd1551bfa6607ddda21a226054 VL - 20 ID - 2023 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. © 2011 Wiley-Liss, Inc. AD - L. Potocki, Department of Molecular and Human Genetics, Baylor College of Medicine, Texas Children's Hospital, 6701 Fannin; Mail Code CC1560, Houston, TX 77030, United States AU - Sanchez-Valle, A. AU - Pierpont, M. E. AU - Potocki, L. DB - Embase Medline DO - 10.1002/ajmg.a.33844 IS - 2 KW - article borderline hypertension case report child chromosome 17p chromosome analysis chromosome duplication delayed diagnosis developmental disorder disease severity echocardiography face dysmorphia genetic analysis genetic disorder heart transplantation human hypoplastic left heart syndrome language disability male muscle hypotonia periodic limb movement disorder Potocki Lupski syndrome priority journal school child LA - English M3 - Article N1 - L361175669 2011-02-02 2011-02-04 PY - 2011 SN - 1552-4825 1552-4833 SP - 363-366 ST - The severe end of the spectrum: Hypoplastic left heart in Potocki-Lupski syndrome T2 - American Journal of Medical Genetics, Part A TI - The severe end of the spectrum: Hypoplastic left heart in Potocki-Lupski syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L361175669 http://dx.doi.org/10.1002/ajmg.a.33844 VL - 155 ID - 1113 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. © 2011 Wiley-Liss, Inc. AD - Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, United States Texas Children's Hospital, Houston, TX, United States Department of Medical Genetics, Children's Hospitals and Clinics of Minnesota, Minneapolis, MN, United States Department of Pediatrics, University of Minnesota, Minneapolis, MN, United States AU - Sanchez-Valle, A. AU - Pierpont, M. E. AU - Potocki, L. DB - Scopus DO - 10.1002/ajmg.a.33844 IS - 2 KW - Autism Chromosome 17p duplication Failure to thrive Hypoplastic left heart syndrome Left ventricular outflow track RAI1 M3 - Article N1 - Cited By :19 Export Date: 15 June 2020 PY - 2011 SP - 363-366 ST - The severe end of the spectrum: Hypoplastic left heart in Potocki-Lupski syndrome T2 - American Journal of Medical Genetics, Part A TI - The severe end of the spectrum: Hypoplastic left heart in Potocki-Lupski syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-79251525210&doi=10.1002%2fajmg.a.33844&partnerID=40&md5=abfd523514cfd0e94a65536455d18686 VL - 155 ID - 2134 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 tower 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. © 1994. AD - Division of Cardiology, Department of Paediatrics and Cardiovascular Surgery, University of British Columbia, Vancouver, BC, Canada AU - Sandor, G. G. S. AU - Patterson, M. W. H. AU - LeBlanc, J. G. DB - Scopus DO - 10.1016/0002-9149(94)90236-4 IS - 4 M3 - Article N1 - Cited By :8 Export Date: 15 June 2020 PY - 1994 SP - 292-297 ST - Systolic and diastolic function in tricuspid valve atresia before the fontan operation T2 - The American Journal of Cardiology TI - Systolic and diastolic function in tricuspid valve atresia before the fontan operation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0028045716&doi=10.1016%2f0002-9149%2894%2990236-4&partnerID=40&md5=70eb416d9edd9625435328a8ca997dba VL - 73 ID - 2308 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). Althought 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 < 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 < 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 < 0.001) greater in the RV type than in the LV type patients. There was a significant inverse correlation (p < 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-sysolic stress-MNSERc relation. The end-systolic stress:end-systolic volume ratio was also significantly depressed in both patient types of univentricular heart volume ratio (3.49 ± 1.77 for LV type, 40.7 ± 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. Thus, despite the absence of significant difference either in preload or myocardial contractile function between LV and RV type patients, afterload was significantly increased in RV type patients compared with LV type patients. Therefore, impaired ejection performance in RV type compared with that in LV type patients may be responsible for afterload mismatch caused by inadequate ventricular hypertrophy rather than by myocardial contractile dysfunction. AD - Department of Pediatrics, Osaka University Medical School, Osaka 553, Japan 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. DB - Scopus DO - 10.1161/01.CIR.79.6.1247 IS - 6 M3 - Article N1 - Cited By :39 Export Date: 15 June 2020 PY - 1989 SP - 1247-1256 ST - Assessement of ventricular contractile state and function in patients with univentricular heart T2 - Circulation TI - Assessement of ventricular contractile state and function in patients with univentricular heart UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0024474544&doi=10.1161%2f01.CIR.79.6.1247&partnerID=40&md5=8559424af4c211bae6f1c36b99ec40b9 VL - 79 ID - 2320 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 - J.H. Sanz, Division of Neuropsychology, Children's National Health System, Washington, DC, United States AU - Sanz, J. H. AU - Wang, J. AU - Berl, M. M. AU - Armour, A. C. AU - Cheng, Y. I. AU - Donofrio, M. T. DB - Embase Medline DO - 10.1016/j.jpeds.2018.07.018 KW - adolescent aortic occlusion article child congenital heart disease controlled study executive function Fallot tetralogy female great vessels transposition heart single ventricle heart surgery heart ventricle septum defect human hypoplastic left heart syndrome latent class analysis major clinical study male prematurity priority journal pulmonary valve atresia quality of life questionnaire social psychology structural equation modeling LA - English M3 - Article N1 - L2001110798 2018-09-27 2018-10-22 PY - 2018 SN - 1097-6833 0022-3476 SP - 63-69 ST - Executive Function and Psychosocial Quality of Life in School Age Children with Congenital Heart Disease T2 - Journal of Pediatrics TI - Executive Function and Psychosocial Quality of Life in School Age Children with Congenital Heart Disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2001110798 http://dx.doi.org/10.1016/j.jpeds.2018.07.018 VL - 202 ID - 656 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 AN - 132346834. Language: English. Entry Date: 20190220. Revision Date: 20190423. Publication Type: journal article AU - Sanz, Jacqueline H. AU - Wang, Jichuan AU - Berl, Madison M. AU - Armour, Anna C. AU - Cheng, Yao I. AU - Donofrio, Mary T. DB - ccm DO - 10.1016/j.jpeds.2018.07.018 DP - EBSCOhost KW - Executive Function Heart Defects, Congenital -- Physiopathology Heart Defects, Congenital -- Psychosocial Factors Quality of Life Adolescence Heart Defects, Congenital -- Classification Male Female Sex Factors Childbirth, Premature Parents -- Psychosocial Factors Models, Statistical Arterial Occlusive Diseases -- Physiopathology Stress, Psychological Child Pregnancy Human Validation Studies Comparative Studies Evaluation Research Multicenter Studies Funding Source N1 - research; tables/charts. Journal Subset: Biomedical; Peer Reviewed; USA. Grant Information: U54 HD090257/HD/NICHD NIH HHS/United States. NLM UID: 0375410. PMID: NLM30243535. 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 - Journal of Pediatrics TI - Executive Function and Psychosocial Quality of Life in School Age Children with Congenital Heart Disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=132346834&site=ehost-live&scope=site VL - 202 ID - 1449 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. © 2018 Elsevier Inc. AD - Division of Neuropsychology, Children's National Health System, Washington, DC, United States Department of Psychiatry and Behavioral Sciences at The George Washington University School of Medicine, Washington, DC, United States Department of Pediatrics at The George Washington University School of Medicine, Washington, DC, United States Biostatistics, Children's National Health System, Washington, DC, United States Research Professor of Epidemiology and Biostatistics, George Washington University, Washington, DC, United States Division of Cardiology, Children's National Health System, Washington, DC, United States AU - Sanz, J. H. AU - Wang, J. AU - Berl, M. M. AU - Armour, A. C. AU - Cheng, Y. I. AU - Donofrio, M. T. DB - Scopus DO - 10.1016/j.jpeds.2018.07.018 KW - congenital heart defects executive function neurodevelopmental outcome quality of life M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2018 SP - 63-69 ST - Executive Function and Psychosocial Quality of Life in School Age Children with Congenital Heart Disease T2 - Journal of Pediatrics TI - Executive Function and Psychosocial Quality of Life in School Age Children with Congenital Heart Disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85053660353&doi=10.1016%2fj.jpeds.2018.07.018&partnerID=40&md5=5b4feb70e711960e64514c50bf4ba332 VL - 202 ID - 1784 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. (PsycINFO Database Record (c) 2018 APA, all rights reserved) AD - Sarajuuri, Anne, University Central Hospital, Children’s Hospital, PL 280, 00029 HUS, Helsinki, Finland AN - 2013-20605-012 AU - Sarajuuri, Anne AU - Jokinen, Eero AU - Mildh, Leena AU - Tujulin, Anna-Mari AU - Mattila, Ilkka AU - Valanne, Leena AU - Lönnqvist, Tuula DB - psyh DO - 10.1542/peds.2012-0486 DP - EBSCOhost IS - 6 KW - neurodevelopmental burden univentricular heart patients brain MRI risk factors patient history Congenital Disorders Heart Disorders Neurodevelopmental Disorders Pediatrics Functional Magnetic Resonance Imaging N1 - Division of Child Neurology, Helsinki University Central Hospital, Helsinki, Finland. Release Date: 20131216. Correction Date: 20181213. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Congenital Disorders; Heart Disorders; Patient History; Risk Factors; Neurodevelopmental Disorders. Minor Descriptor: Pediatrics; Functional Magnetic Resonance Imaging. Classification: Physical & Somatoform & Psychogenic Disorders (3290). Population: Human (10). Location: Finland. Age Group: Childhood (birth-12 yrs) (100); Neonatal (birth-1 mo) (120); Infancy (2-23 mo) (140); Preschool Age (2-5 yrs) (160). Tests & Measures: Beery-Buktenica Developmental Test of Visual-Motor Integration; Mental Developmental Index; Movement Assessment Battery for Children; Vineland Adaptive Behavior Scales; Wechsler Preschool and Primary Scale of Intelligence--Revised DOI: 10.1037/t48859-000. Methodology: Brain Imaging; Empirical Study; Followup Study; Longitudinal Study; Prospective Study; Quantitative Study. References Available: Y. Page Count: 11. Issue Publication Date: Dec 1, 2012. Publication History: Accepted Date: Jul 24, 2012. Copyright Statement: The American Academy of Pediatrics. 2012. Sponsor: Arvo and Lea Ylppö Foundation. Recipients: No recipient indicated Sponsor: Lastenlinna Foundation. Recipients: No recipient indicated Sponsor: Foundation for Pediatric Research. Recipients: No recipient indicated Sponsor: Märta Donner Foundation. Recipients: No recipient indicated Sponsor: Finnish Foundation for Cardiovascular Research, Finland. Recipients: No recipient indicated PY - 2012 SN - 0031-4005 1098-4275 SP - 1636-1646 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2013-20605-012&site=ehost-live&scope=site ORCID: 0000-0002-8572-829X anne.sarajuuri@hus.fi VL - 130 ID - 1682 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. Copyright © 2012 by the American Academy of Pediatrics. AD - A. Sarajuuri, Helsinki University Central Hospital, Children's Hospital, PL 280, 00029 HUS, Helsinki, Finland AU - Sarajuuri, A. AU - Jokinen, E. AU - Mildh, L. AU - Tujulin, A. M. AU - Mattila, I. AU - Valanne, L. AU - Lönnqvist, T. DB - Embase Medline DO - 10.1542/peds.2012-0486 IS - 6 KW - adaptive behavior article ascending aorta child clinical article controlled study developmental disorder disease severity Glenn shunt heart muscle ischemia heart single ventricle heart surgery human hypoplastic left heart syndrome intelligence quotient motor performance nervous system development neurologic disease neurologic examination neuropsychological test nuclear magnetic resonance imaging perioperative period postoperative period preoperative period priority journal prospective study risk factor seizure surgical technique LA - English M3 - Article N1 - L366192887 2012-12-12 2012-12-19 http://pediatrics.aappublications.org/content/130/6/e1636.full.pdf+html PY - 2012 SN - 0031-4005 1098-4275 SP - e1636-e1646 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L366192887 http://dx.doi.org/10.1542/peds.2012-0486 VL - 130 ID - 1032 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. © 2007 The American Association for Thoracic Surgery. AD - A. Sarajuuri, Department of Neurology, Helsinki University Central Hospital, Helsinki, Finland AU - Sarajuuri, A. AU - Jokinen, E. AU - Puosi, R. AU - Eronen, M. AU - Mildh, L. AU - Mattila, I. AU - Valanne, L. AU - Lönnqvist, T. DB - Embase Medline DO - 10.1016/j.jtcvs.2006.12.022 IS - 6 KW - article brain atrophy brain infarction brain ischemia cardiopulmonary bypass cerebral palsy child clinical article cognitive defect computer assisted tomography controlled study diagnostic imaging diuresis heart single ventricle human hypoplastic left heart syndrome intelligence quotient motor dysfunction nerve cell differentiation neurologic disease neuroradiology Norwood procedure nuclear magnetic resonance imaging priority journal risk assessment surgical technique survival LA - English M3 - Article N1 - L46802572 2007-06-01 PY - 2007 SN - 0022-5223 SP - 1524-1532 ST - Neurodevelopmental and neuroradiologic outcomes in patients with univentricular heart aged 5 to 7 years: Related risk factor analysis T2 - Journal of Thoracic and Cardiovascular Surgery TI - Neurodevelopmental and neuroradiologic outcomes in patients with univentricular heart aged 5 to 7 years: Related risk factor analysis UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L46802572 http://dx.doi.org/10.1016/j.jtcvs.2006.12.022 VL - 133 ID - 1234 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. © 2011 The Author(s)/Acta Pædiatrica © 2011 Foundation Acta Pædiatrica. AD - A. Sarajuuri, Division of Child Neurology, Children's Hospital, Helsinki University, PL 280, 00029 HUS, Helsinki, Finland AU - Sarajuuri, A. AU - Lönnqvist, T. AU - Schmitt, F. AU - Almqvist, F. AU - Jokinen, E. DB - Embase Medline DO - 10.1111/j.1651-2227.2011.02509.x IS - 3 KW - article child child behavior Child Behavior Checklist clinical article comparative study controlled study emotional disorder female follow up heart single ventricle human hypoplastic left heart syndrome male outcome assessment parental stress preschool child priority journal prospective study scoring system self concept LA - English M3 - Article N1 - L51745568 2011-12-02 2012-02-08 PY - 2012 SN - 0803-5253 1651-2227 SP - 252-257 ST - Patients with univentricular heart in early childhood: Parenting stress and child behaviour T2 - Acta Paediatrica, International Journal of Paediatrics TI - Patients with univentricular heart in early childhood: Parenting stress and child behaviour UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51745568 http://dx.doi.org/10.1111/j.1651-2227.2011.02509.x VL - 101 ID - 1067 ER - TY - JOUR AD - Children's Hospital, University of Helsinki and Helsinki University Central Hospital, Helsinki, Finland Child Psychiatry Clinic, Turku University and Turku University Hospital, Turku, Finland. AN - 104507717. Language: English. Entry Date: 20120824. Revision Date: 20150711. Publication Type: Journal Article AU - Sarajuuri, A. AU - Lönnqvist, T. AU - Schmitt, F. AU - Almqvist, F. AU - Jokinen, E. DB - ccm DO - 10.1111/j.1651-2227.2011.02509.x DP - EBSCOhost IS - 3 KW - Child Behavior Disorders -- Etiology Heart Ventricle -- Abnormalities Hypoplastic Left Heart Syndrome -- Psychosocial Factors Infant Behavior Parenting Stress, Psychological -- Etiology Case Control Studies Female Prospective Studies Human Hypoplastic Left Heart Syndrome -- Complications Infant Male Perception Questionnaires Child Behavior Checklist N1 - research. Journal Subset: Biomedical; Continental Europe; Europe; Peer Reviewed. Special Interest: Pediatric Care. Instrumentation: Child Behavior Checklist (CBCL) (Achenbach and Edelbrock); Parenting Stress Index (PSI). NLM UID: 9205968. PMID: NLM22040350. PY - 2012 SN - 0803-5253 SP - 252-257 ST - Patients with univentricular heart in early childhood: parenting stress and child behaviour T2 - Acta Paediatrica TI - Patients with univentricular heart in early childhood: parenting stress and child behaviour UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104507717&site=ehost-live&scope=site VL - 101 ID - 1556 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. (PsycINFO Database Record (c) 2016 APA, all rights reserved) AD - Sarajuuri, A., Division of Child Neurology, Children’s Hospital, Helsinki University Central Hospital, PL 280, 00029 HUS, Helsinki, Finland AN - 2012-02574-008 AU - Sarajuuri, A. AU - Lönnqvist, T. AU - Schmitt, F. AU - Almqvist, F. AU - Jokinen, E. DB - psyh DO - 10.1111/j.1651-2227.2011.02509.x DP - EBSCOhost IS - 3 KW - univentricular heart defects early childhood parenting stress child behavior Case-Control Studies Child Behavior Disorders Female Follow-Up Studies Heart Ventricles Humans Hypoplastic Left Heart Syndrome Infant Infant Behavior Male Parenting Perception Prospective Studies Stress, Psychological Surveys and Questionnaires Child Attitudes Heart Disorders Stress N1 - Acta Paediatrica Scandinavica (Sweden). Partial author list: First Author & Affiliation: Sarajuuri, A.; Children’s Hospital, University of Helsinki, Helsinki, Finland. Other Publishers: Almqvist & Wiksell Periodical Co.; Scandinavian University Press; Taylor & Francis. Release Date: 20120521. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Child Attitudes; Heart Disorders; Stress; Parenting. Classification: Cardiovascular Disorders (3295). Population: Human (10). Location: Finland. Age Group: Childhood (birth-12 yrs) (100); Infancy (2-23 mo) (140). Tests & Measures: Child Behavior Checklist; Achenbach Child Behavior Checklist; Parenting Stress Index DOI: 10.1037/t02445-000. Methodology: Empirical Study; Longitudinal Study; Prospective Study; Quantitative Study. Supplemental Data: Tables and Figures Internet. References Available: Y. Page Count: 6. Issue Publication Date: Mar, 2012. Publication History: Accepted Date: Oct 28, 2011; Revised Date: Oct 25, 2011; First Submitted Date: Jun 7, 2011. Copyright Statement: The Author(s)/Acta Pædiatrica—Foundation Acta Pædiatrica. 2011. Sponsor: Arvo and Lea Ylppö Foundation. Recipients: No recipient indicated Sponsor: Lastenlinna Foundation. Recipients: No recipient indicated Sponsor: Foundation for Pediatric Research. Recipients: No recipient indicated Sponsor: Finnish Foundation for Cardiovascular Research, Finland. Recipients: No recipient indicated PY - 2012 SN - 0803-5253 1651-2227 SP - 252-257 ST - Patients with univentricular heart in early childhood: Parenting stress and child behaviour T2 - Acta Paediatrica TI - Patients with univentricular heart in early childhood: Parenting stress and child behaviour UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2012-02574-008&site=ehost-live&scope=site anne.sarajuuri@hus.fi VL - 101 ID - 1683 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. © 2011 The Author(s)/Acta Pædiatrica © 2011 Foundation Acta Pædiatrica. AD - Division of Child Neurology, Children's Hospital, Helsinki University, PL 280, 00029 HUS, Helsinki, Finland Child Psychiatry Clinic, Turku University and Turku University Hospital, Turku, Finland AU - Sarajuuri, A. AU - Lönnqvist, T. AU - Schmitt, F. AU - Almqvist, F. AU - Jokinen, E. DB - Scopus DO - 10.1111/j.1651-2227.2011.02509.x IS - 3 KW - Child behaviour Congenital heart defect Hypoplastic left heart syndrome Parents Psychological stress M3 - Article N1 - Cited By :29 Export Date: 15 June 2020 PY - 2012 SP - 252-257 ST - Patients with univentricular heart in early childhood: Parenting stress and child behaviour T2 - Acta Paediatrica, International Journal of Paediatrics TI - Patients with univentricular heart in early childhood: Parenting stress and child behaviour UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84856320429&doi=10.1111%2fj.1651-2227.2011.02509.x&partnerID=40&md5=e1879a6214bf21b75c98dbd57f0037af VL - 101 ID - 2099 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 - I. Sarrechia, Department of Experimental Psychology, Clinical Developmental Psychology, Ghent University, Henri Dunantlaan 2, Ghent, Belgium AU - Sarrechia, I. AU - Miatton, M. AU - De Wolf, D. AU - François, K. AU - Gewillig, M. AU - Meyns, B. AU - Vingerhoets, G. DB - Embase Medline DO - 10.1093/ejcts/ezv029 IS - 1 KW - article artificial ventilation brain development child child behavior Child Behavior Checklist cognition congenital heart disease controlled study cross-sectional study extracorporeal circulation female heart single ventricle hospitalization human information processing major clinical study male memory motor performance neuropsychological test performance priority journal school child special education LA - English M3 - Article N1 - L608459553 2016-02-23 2016-03-02 PY - 2016 SN - 1873-734X 1010-7940 SP - 167-174 ST - Neurocognitive development and behaviour in school-aged children after surgery for univentricular or biventricular congenital heart disease T2 - European Journal of Cardio-thoracic Surgery TI - Neurocognitive development and behaviour in school-aged children after surgery for univentricular or biventricular congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L608459553 http://dx.doi.org/10.1093/ejcts/ezv029 VL - 49 ID - 835 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. © The Author 2015. AD - Department of Experimental Psychology, Ghent University, Ghent, Belgium 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 AU - Sarrechia, I. AU - Miatton, M. AU - De Wolf, D. AU - François, K. AU - Gewillig, M. AU - Meyns, B. AU - Vingerhoets, G. DB - Scopus DO - 10.1093/ejcts/ezv029 IS - 1 KW - Congenital heart defect Hypoplastic left heart Neurodevelopmental outcome Neuropsychology Tricuspid atresia M3 - Article N1 - Cited By :23 Export Date: 15 June 2020 PY - 2016 SP - 167-174 ST - Neurocognitive development and behaviour in school-aged children after surgery for univentricular or biventricular congenital heart disease T2 - European Journal of Cardio-thoracic Surgery TI - Neurocognitive development and behaviour in school-aged children after surgery for univentricular or biventricular congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84958622572&doi=10.1093%2fejcts%2fezv029&partnerID=40&md5=f6c35c42153dc4ed3c5d82aef7592323 VL - 49 ID - 1927 ER - TY - JOUR AB - Objective: To evaluate the physical activity level and functional capacity of children and adolescents with congenital heart disease and to describe correlations between functionality, surgical and echocardiographic findings, metabolic and inflammatory profile and differences between acyanotic and cyanotic heart defects. Methods: A cross-sectional study including children and adolescents with congenital heart disease between six and 18 years old that were evaluated with the 6-minute walk test (6MWT) to assess functional capacity. The short version form of the International Physical Activity Questionnaire (IPAQ) was performed to evaluate physical activity levels. Also, echocardiography and blood collection, to evaluate the metabolic (blood glucose, lipids, insulin) and inflammatory markers (C-reactive protein), were assessed. Results: Twenty-five individuals were evaluated. Of them, 14 had acyanotic heart defects and 11 cyanotic heart defects. Mean age was 12.0±3.7 years, and 20 (80%) were male. IPAQ showed that six (24%) individuals were very active, eight (32%) were active, nine (36%) had irregular physical activity, and two (8%) were sedentary. The mean distance walked in the 6MWT, considering all studied individuals, was 464.7±100.4 m, which was 181.4±42.0 m less than the predicted (p=0.005). There was a positive correlation between Z score 6MWT and the number of surgical procedures (r=-0.455; p=0.022). Conclusions: Children and adolescents with congenital heart disease have low functional capacity, but they are not completely sedentary. © 2019 Sociedade de Pediatria de São Paulo. Published by Zeppelini Publishers. AD - Instituto de Cardiologia do Rio Grande do Sul, Fundação Universitária de Cardiologia, Porto Alegre, RS, Brazil Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil Universidade Federal de Ciências da Saúde de Porto Alegre, RS, Porto Alegre, Brazil AU - Schaan, C. W. AU - Feltez, G. AU - D’Agord Schaan, B. AU - Pellanda, L. C. DB - Scopus DO - 10.1590/1984-0462/;2019;37;1;00016 IS - 1 KW - Adolescent Child Congenital Exercise tolerance Heart defects M3 - Article N1 - Export Date: 15 June 2020 PY - 2019 SP - 65-72 ST - Functional capacity in children and adolescents with congenital heart disease T2 - Revista Paulista de Pediatria TI - Functional capacity in children and adolescents with congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85061158339&doi=10.1590%2f1984-0462%2f%3b2019%3b37%3b1%3b00016&partnerID=40&md5=9beb7fb54b98f05293973af42d2e31ee VL - 37 ID - 1774 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. © 2014 American Heart Association, Inc. AD - Department for Congenital Heart Disease and Paediatric Cardiology, University Hospital of Schleswig-Holstein, Campus Kiel, Arnold-Heller-Str.3, Haus 9, Kiel, 24105, Germany Department of Pediatric, Fetal and Congenital Cardiology, Hawaii Permanente Medical Group, Kaiser Permanente Moanalua Medical Center, Honolulu, HI, United States Adult Congenital Heart Disease Centre, National Centre for Pulmonary Hypertension, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom 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. DB - Scopus DO - 10.1161/CIRCIMAGING.114.002110 IS - 6 KW - Hypoplastic left heart syndrome Ventricular function M3 - Article N1 - Cited By :36 Export Date: 15 June 2020 PY - 2014 SP - 880-886 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 - Circulation: Cardiovascular 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84922393948&doi=10.1161%2fCIRCIMAGING.114.002110&partnerID=40&md5=f3a9da46ca580ff0ab3ecef66e2f3b1f VL - 7 ID - 2003 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. © 2017, Springer Science+Business Media, LLC. AD - Department of Pathology and Laboratory Medicine, University of Rochester Medical Center, 601 Elmwood Ave, Box 608, Rochester, NY 14642, United States Department of Pediatrics, University of Rochester Medical Center, Rochester, NY, United States Aytu Biosciences, Englewood, CO, 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 - Scopus DO - 10.1007/s00246-017-1755-x IS - 2 KW - Blood Cardiac surgery Complications Oxidation reduction potential Pediatrics Red blood cells M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2018 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85032683707&doi=10.1007%2fs00246-017-1755-x&partnerID=40&md5=e6a0753e56fddd0a534e4cfcde424511 VL - 39 ID - 1823 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. © 2010 American Heart Association, Inc. AD - T. Kuehne, Unit of Cardiovascular Imaging, Department of Congenital Heart Disease and Pediatric Cardiology, Deutsches Herzzentrum Berlin, Augustenburger Platz 1, 13353 Berlin, Germany 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. DB - Embase Medline DO - 10.1161/CIRCIMAGING.109.931592 IS - 5 KW - dobutamine adolescent adult aortic flow article catheterization circulation clinical article collateral circulation controlled study Fontan circulation heart left ventricle enddiastolic pressure heart left ventricle endsystolic volume heart muscle contractility heart stroke volume heart ventricle function heart ventricle pressure human lung blood flow lung pressure lung vascular resistance nuclear magnetic resonance imaging priority journal stress LA - English M3 - Article N1 - L360148419 2010-12-22 2010-12-30 PY - 2010 SN - 1941-9651 1942-0080 SP - 623-631 ST - Pulmonary vascular resistance, collateral flow, and ventricular function in patients with a fontan circulation at rest and during dobutamine stress T2 - Circulation: Cardiovascular Imaging TI - Pulmonary vascular resistance, collateral flow, and ventricular function in patients with a fontan circulation at rest and during dobutamine stress UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L360148419 http://dx.doi.org/10.1161/CIRCIMAGING.109.931592 VL - 3 ID - 1136 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. © 2010 American Heart Association, Inc. AD - Department of Congenital Heart Disease and Pediatric Cardiology, Deutsches Herzzentrum Berlin, Medical University Berlin, Berlin, Germany Departments of Cardiology and Cardiothoracic Surgery, Leiden University Medical Center, Leiden, Netherlands Unit of Cardiovascular Imaging, Department of Congenital Heart Disease and Pediatric Cardiology, Deutsches Herzzentrum Berlin, Augustenburger Platz 1, 13353 Berlin, Germany 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. DB - Scopus DO - 10.1161/CIRCIMAGING.109.931592 IS - 5 KW - Congenital Heart defects Heart failure Magnetic resonance imaging Pulmonary heart disease M3 - Article N1 - Cited By :36 Export Date: 15 June 2020 PY - 2010 SP - 623-631 ST - Pulmonary vascular resistance, collateral flow, and ventricular function in patients with a fontan circulation at rest and during dobutamine stress T2 - Circulation: Cardiovascular Imaging TI - Pulmonary vascular resistance, collateral flow, and ventricular function in patients with a fontan circulation at rest and during dobutamine stress UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-77957334125&doi=10.1161%2fCIRCIMAGING.109.931592&partnerID=40&md5=d97022cb96b78786b05b36f99433343f VL - 3 ID - 2146 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 - The Cardiac Center, Children's Hospital of Philadelphia, Univ. of Pennsylvania School of Med., 34th and Civic Center Blvd., Philadelphia, PA 18104, United States AU - Schultz, A. H. AU - Wernovsky, G. DB - Scopus DO - 10.1053/j.pcsu.2005.01.015 IS - 1 KW - Congenital Follow-up studies Heart defects Hypoplastic left heart syndrome Tetralogy of Fallot Transposition of great vessels M3 - Article N1 - Cited By :21 Export Date: 15 June 2020 PY - 2005 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-16344384958&doi=10.1053%2fj.pcsu.2005.01.015&partnerID=40&md5=38836035c045a455ee8c2fe425bd9dcf VL - 8 ID - 2238 ER - TY - JOUR AB - AimsAn 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 resultsThis 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.ConclusionAn increased NT-proBNP level was present in the majority of Fontan patients. Six months of bosentan treatment was not beneficial.Trial registrationNTR1557All rights reserved. © 2013 The Author. AD - Department of Cardiology, Academic Medical Centre, Meibergdreef 9, 1105 AZ Amsterdam, Netherlands Interuniversity Cardiology Institute of the Netherlands, Utrecht, Netherlands Department of Cardiology, University Nijmegen Medical Centre, Nijmegen, Netherlands Department of Cardiology, University Medical Centre Groningen, Groningen, Netherlands Department of Cardiology, Leiden University Medical Centre, Leiden, Netherlands Department of Cardiology, University Medical Centre Utrecht, Utrecht, Netherlands AU - Schuuring, M. J. AU - Vis, J. C. AU - Van Dijk, A. P. J. AU - Van Melle, J. P. AU - Vliegen, H. W. AU - Pieper, P. G. AU - Sieswerda, G. T. AU - De Bruin-Bon, R. H. A. C. M. AU - Mulder, B. J. M. AU - Bouma, B. J. DB - Scopus DO - 10.1093/eurjhf/hft017 IS - 6 KW - Bosentan Exercise capacity Fontan M3 - Article N1 - Cited By :75 Export Date: 15 June 2020 PY - 2013 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84878017417&doi=10.1093%2feurjhf%2fhft017&partnerID=40&md5=8098fa6f66b3f76afaf52c82a698705d VL - 15 ID - 2045 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 - Department for Congenital Cardiovascular Surgery, University Children's Hospital, Zurich, Switzerland Divisions of Pediatric Cardiology and Children's Research Centre, University Children's Hospital, Zurich, Switzerland Department for Anesthesiology, University Children's Hospital, Zurich, Switzerland Department of Intensive Care and Neonatology, 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 - Scopus DO - 10.3978/j.issn.2072-1439.2015.01.38 IS - 3 KW - Heart defects congenital Heart transplantation (HTx) Transplant recipients M3 - Article N1 - Cited By :15 Export Date: 15 June 2020 PY - 2015 SP - 552-559 ST - Pediatric heart transplantation T2 - Journal of Thoracic Disease TI - Pediatric heart transplantation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84928622546&doi=10.3978%2fj.issn.2072-1439.2015.01.38&partnerID=40&md5=19101b84bb6a389520a3e737376feb0c VL - 7 ID - 1959 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. © 2015 Elsevier Inc. AD - Department of Pediatrics (Cardiology), University of Arizona, Tucson, AZ, United States Department of Pediatrics, University of Arizona, Tucson, AZ, United States Arizona Pediatric Cardiology, Phoenix Children's Hospital, Phoenix, AZ, United States University of Arizona, College of Medicine, Tucson, AZ, United States Sarver Heart Center, University of Arizona, Tucson, AZ, United States 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. DB - Scopus DO - 10.1016/j.amjcard.2015.09.008 IS - 11 M3 - Article N1 - Cited By :9 Export Date: 15 June 2020 PY - 2015 SP - 1756-1761 ST - Hospital Resource Utilization for Common Noncardiac Diagnoses in Adult Survivors of Single Cardiac Ventricle T2 - American Journal of Cardiology TI - Hospital Resource Utilization for Common Noncardiac Diagnoses in Adult Survivors of Single Cardiac Ventricle UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84947322188&doi=10.1016%2fj.amjcard.2015.09.008&partnerID=40&md5=c2a64e9e41e6c56529f1c23631a560a4 VL - 116 ID - 1971 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, 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 - Scopus IS - 2 M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2013 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84876849598&partnerID=40&md5=bdec31dad5050285b13363be6cbdecda VL - 25 ID - 2063 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 - Department of Pediatrics, Stollery Childrens Hospital, University of Alberta, Edmonton, AB, Canada Department of Surgery, Stollery Childrens Hospital, University of Alberta AB, Edmonton, AB, Canada Department of Immunology, University of Alberta, Edmonton, AB, Canada Department of Pediatrics, Cardiac Transplant Research, 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 - Scopus DO - 10.1111/tri.12294 IS - 7 KW - ABO incompatibility allograft dysfunction failed Fontan circulation heart transplantation mechanical circulatory support protein-losing enteropathy M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2014 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84902536267&doi=10.1111%2ftri.12294&partnerID=40&md5=5b4b292c36324c3677a4547a68a15a18 VL - 27 ID - 2007 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 trunco-atrioventricular 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. © 1978, International Heart Journal Association. All rights reserved. AD - Departments of Pediatric Cardiology, Queen Pahlavi Cardiovascular Center, Teheran, Iran AU - Shakibi, J. G. AU - Aryanpur, I. AU - Nazarian, I. AU - Siassi, B. DB - Scopus DO - 10.1536/ihj.19.346 IS - 3 KW - Common ventricle Congenetal heart disease Mitral atresia M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 1978 SP - 346-357 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 - Japanese Heart Journal 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0018118477&doi=10.1536%2fihj.19.346&partnerID=40&md5=09c394c021d49b335bb7fc5f82ffa23f VL - 19 ID - 2335 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. AU - Shamsuddin, A. K. AU - Biswas, S. K. AU - Rahman, M. Z. AU - Biswas, S. AU - Hasan, N. A. AU - Sharifuzzaman, M. DB - Medline IS - 3 KW - case report Fontan procedure human infant male diaphragm paralysis LA - English M3 - Article N1 - L601775167 2015-02-02 PY - 2014 SN - 1022-4742 SP - 595-598 ST - A young child with bilateral diaphragmatic palsy after bilateral bidirectional Glenn shunt T2 - Mymensingh medical journal : MMJ TI - A young child with bilateral diaphragmatic palsy after bilateral bidirectional Glenn shunt UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L601775167 VL - 23 ID - 916 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 - Senior Consultant, Sher-E-Bangladesh, Pediatric Cardiac Surgery, National Heart Foundation Hospital and Research Institute (NHFH & RI), Associate Professor &, Dr AK Shamsuddin, Bangladesh AU - Shamsuddin, A. K. AU - Biswas, S. K. AU - Rahman, M. Z. AU - Biswas, S. AU - Hasan, N. A. AU - Sharifuzzaman, M. DB - Scopus IS - 3 M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2014 SP - 595-598 ST - A young child with bilateral diaphragmatic palsy after bilateral bidirectional Glenn shunt T2 - Mymensingh medical journal : MMJ TI - A young child with bilateral diaphragmatic palsy after bilateral bidirectional Glenn shunt UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84921691941&partnerID=40&md5=66ed5d3f17e9e73f6012039c8716054f VL - 23 ID - 1990 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 3800964 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. © 2013 Wiley Periodicals, Inc. AD - P. Shamszad, 3333 Burnet Ave., MLC 2003, Cincinnati, OH 45229, United States AU - Shamszad, P. AU - Barnes, J. N. AU - Morris, S. A. DB - Embase Medline DO - 10.1111/chd.12090 IS - 1 KW - adolescence adolescent aortic arch anomaly aortic dissection aortic valve disease article cardiovascular procedure child child care child hospitalization childhood mortality congenital heart disease connective tissue disease diagnosis related group Ehlers Danlos syndrome female great vessels transposition hospital patient human hypertension hypoplastic left heart syndrome incidence infancy injury isolated hypertension major clinical study male Marfan syndrome medical information system outcome assessment preschool child priority journal school child LA - English M3 - Article N1 - L52600286 2013-05-28 2014-02-04 PY - 2014 SN - 1747-079X 1747-0803 SP - 54-62 ST - Aortic dissection in hospitalized children and young adults: A multiinstitutional study T2 - Congenital Heart Disease TI - Aortic dissection in hospitalized children and young adults: A multiinstitutional study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52600286 http://dx.doi.org/10.1111/chd.12090 VL - 9 ID - 941 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 3800964 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. © 2013 Wiley Periodicals, Inc. AD - Lillie Frank Abercrombie Section of Cardiology, Department of Pediatrics, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, United States Children's Hospital Association, Overland Park, KS, United States AU - Shamszad, P. AU - Barnes, J. N. AU - Morris, S. A. DB - Scopus DO - 10.1111/chd.12090 IS - 1 KW - Aortic Disease Hospital Utilization Mortality Pediatrics Risk Factors M3 - Article N1 - Cited By :11 Export Date: 15 June 2020 PY - 2014 SP - 54-62 ST - Aortic dissection in hospitalized children and young adults: A multiinstitutional study T2 - Congenital Heart Disease TI - Aortic dissection in hospitalized children and young adults: A multiinstitutional study UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84892844507&doi=10.1111%2fchd.12090&partnerID=40&md5=7964d37980e317e789e87f58a412ccf7 VL - 9 ID - 1999 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. © 2015 Elsevier Inc. AD - Texas Children's Fetal Center, Texas Children's Hospital, Houston, TX, United States Department of Surgery, Baylor College of Medicine, Houston, TX, United States Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, TX, United States Department of Pediatrics, Baylor College of Medicine, Houston, TX, United States Department of Radiology, Baylor College of Medicine, 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 - Scopus DO - 10.1016/j.jpedsurg.2015.02.035 IS - 7 KW - Cervical lymphatic malformation Cervical teratoma Cervical thyroid cyst Cervical vascular malformation Fetal neck mass M3 - Article N1 - Cited By :17 Export Date: 15 June 2020 PY - 2015 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84952631552&doi=10.1016%2fj.jpedsurg.2015.02.035&partnerID=40&md5=7791b5e9baaad9de226fb2d7a6e30db9 VL - 50 ID - 1966 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 left 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 systemic flow ratio (r = +0.89, p < 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 < 0.05). A-V valve regurgitation was also a factor that increased ventricular cavity volume, although the ventricular function became more depressed. © 1980. AD - First Department of Surgery, Osaka University Medical School, Fukushima-ku, Osaka, Japan First Department of Radiology, Osaka University Medical School, Fukushima-ku, Osaka, Japan AU - Shimazaki, Y. AU - Kawashima, Y. AU - Mori, T. AU - Kitamura, S. AU - Matsuda, H. AU - Yokota, K. DB - Scopus DO - 10.1016/0002-9149(80)90125-3 IS - 4 M3 - Article N1 - Cited By :21 Export Date: 15 June 2020 PY - 1980 SP - 806-810 ST - Ventricular volume characteristics of single ventricle before corrective surgery T2 - The American Journal of Cardiology TI - Ventricular volume characteristics of single ventricle before corrective surgery UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0018831049&doi=10.1016%2f0002-9149%2880%2990125-3&partnerID=40&md5=7f710de27278c49182b44a4f96755a13 VL - 45 ID - 2334 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. © 1983 Raven Press, New York. AD - Department of Diagnostic Radiology, School of Medicine, University of Alabama in Birmingham, Veterans Administration Hospital, Birmingham, AL, United States Department of Pathology, School of Medicine, University of Alabama in Birmingham, Veterans Administration Hospital, Birmingham, AL, United States Department of Pediatrics, School of Medicine, University of Alabama in Birmingham, Veterans Administration Hospital, Birmingham, AL, United States AU - Shin, M. S. AU - Ceballos, R. AU - Bini, R. M. AU - Ho, K. J. DB - Scopus DO - 10.1097/00004728-198306000-00028 IS - 3 KW - Computed tomography Pseudoaneurysm, pulmonary artery Pulmonary arteries M3 - Article N1 - Cited By :8 Export Date: 15 June 2020 PY - 1983 SP - 524-526 ST - CT diagnosis of false aneurysm of the pulmonary artery not demonstrated by angiography T2 - Journal of Computer Assisted Tomography TI - CT diagnosis of false aneurysm of the pulmonary artery not demonstrated by angiography UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0020511074&doi=10.1097%2f00004728-198306000-00028&partnerID=40&md5=3c0023cbe2927cf6ed7649b647678f2c VL - 7 ID - 2332 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 - Ward Family Heart Center, Children's Mercy Kansas City Health Services and Outcomes Research, Children's Mercy Kansas City, MO Saint Luke's Mid America Heart Institute, Kansas City, MO AN - 115450331. Language: English. Entry Date: 20171105. Revision Date: 20180702. Publication Type: journal article AU - Shirali, Girish AU - Erickson, Lori AU - Apperson, Jonathan AU - Goggin, Kathy AU - Williams, David AU - Reid, Kimberly AU - Bradley-Ewing, Andrea AU - Tucker, Dawn AU - Bingler, Michael AU - Spertus, John AU - Rabbitt, Leslie AU - Stroup, Richard DB - ccm DO - 10.1161/CIRCOUTCOMES.115.002452 DP - EBSCOhost IS - 3 KW - Process Assessment (Health Care) -- Administration Heart Ventricle -- Surgery Heart Defects, Congenital -- Surgery Multidisciplinary Care Team -- Administration Heart Surgery -- Mortality Telemedicine -- Administration Health Facility Departments -- Administration Health Care Delivery, Integrated -- Administration Heart Surgery -- Adverse Effects Time Factors Diffusion of Innovation Caregivers -- Psychosocial Factors Attitude to Computers Treatment Outcomes Child, Preschool Program Evaluation Attitude to Health Heart Defects, Congenital -- Mortality Infant Telemetry Predictive Value of Tests Heart Defects, Congenital -- Diagnosis Management Computers, Hand-Held Caregiver Strain Index N1 - research. Journal Subset: Biomedical; USA. Instrumentation: Home Observation for Measurement of the Environment (HOME) (Bradley and Caldwell); Caregiver Strain Index. NLM UID: 101489148. PMID: NLM27166202. PY - 2016 SN - 1941-7713 SP - 303-311 ST - Harnessing Teams and Technology to Improve Outcomes in Infants With Single Ventricle T2 - Circulation: Cardiovascular Quality & Outcomes TI - Harnessing Teams and Technology to Improve Outcomes in Infants With Single Ventricle UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=115450331&site=ehost-live&scope=site VL - 9 ID - 1518 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. © 2016 American Heart Association, Inc. AD - Ward Family Heart Center, Children's Mercy Kansas City, 2401 Gillham Rd, Kansas City, MO 64108, United States Health Services and Outcomes Research, Children's Mercy Kansas City, Kansas City, MO, United States Saint Luke's Mid America Heart Institute, Kansas City, MO, United States 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. DB - Scopus DO - 10.1161/CIRCOUTCOMES.115.002452 IS - 3 KW - caregivers congenital mortality survival telemedicine M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2016 SP - 303-311 ST - Harnessing teams and technology to improve outcomes in infants with single ventricle T2 - Circulation: Cardiovascular Quality and Outcomes TI - Harnessing teams and technology to improve outcomes in infants with single ventricle UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84969143055&doi=10.1161%2fCIRCOUTCOMES.115.002452&partnerID=40&md5=f534bf9a6f560515e22dc8e9580c0f01 VL - 9 ID - 1912 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. © 2011 The Authors. AD - G.F. Sholler, Heart Centre for Children, Children's Hospital at Westmead, Locked Bag 4001, Westmead, NSW 2145, Australia AU - Sholler, G. F. AU - Kasparian, N. A. AU - Pye, V. E. AU - Cole, A. D. AU - Winlaw, D. S. DB - Embase Medline DO - 10.1111/j.1440-1754.2011.02039.x IS - 10 KW - arterial trunk article clinical feature congenital heart disease female health care delivery heart surgery heart ventricle septum defect hemodynamic monitoring human infant major clinical study male medical care mental health mortality postnatal care prenatal diagnosis prenatal period priority journal pulmonary valve atresia LA - English M3 - Article N1 - L51361600 2011-04-08 2011-11-01 PY - 2011 SN - 1034-4810 1440-1754 SP - 717-722 ST - Fetal and post-natal diagnosis of major congenital heart disease: Implications for medical and psychological care in the current era T2 - Journal of Paediatrics and Child Health TI - Fetal and post-natal diagnosis of major congenital heart disease: Implications for medical and psychological care in the current era UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L51361600 http://dx.doi.org/10.1111/j.1440-1754.2011.02039.x VL - 47 ID - 1090 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. © 2011 The Authors. AD - Heart Centre for Children, Children's Hospital at Westmead, Locked Bag 4001, Westmead, NSW 2145, Australia Sydney Medical School, University of Sydney, Sydney, Australia School of Women's and Children's Health, Faculty of Medicine, University of New South Wales, Kensington, NSW, Australia AU - Sholler, G. F. AU - Kasparian, N. A. AU - Pye, V. E. AU - Cole, A. D. AU - Winlaw, D. S. DB - Scopus DO - 10.1111/j.1440-1754.2011.02039.x IS - 10 KW - cardiac surgery congenital heart disease fetal psychological care M3 - Article N1 - Cited By :20 Export Date: 15 June 2020 PY - 2011 SP - 717-722 ST - Fetal and post-natal diagnosis of major congenital heart disease: Implications for medical and psychological care in the current era T2 - Journal of Paediatrics and Child Health TI - Fetal and post-natal diagnosis of major congenital heart disease: Implications for medical and psychological care in the current era UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-80054795790&doi=10.1111%2fj.1440-1754.2011.02039.x&partnerID=40&md5=de580a02fcd120fbddc98b690e74988e VL - 47 ID - 2117 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. © 2017 Nature America, Inc., part of Springer Nature. All rights reserved. AD - Department of Pediatrics, Columbia University Medical Center, 3959 Broadway CHN 2-253, New York, NY 10032, United States Department of Psychiatry, Columbia University Medical Center, New York, NY, United States Division of Developmental Neuroscience, New York State Psychiatric Institute, New York, NY, United States AU - Siddiqui, S. AU - Fifer, W. P. AU - Ordonez-Retamar, M. AU - Nugent, J. D. AU - Williams, I. A. DB - Scopus DO - 10.1038/jp.2017.59 IS - 8 M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2017 SP - 953-957 ST - An antenatal marker of neurodevelopmental outcomes in infants with congenital heart disease T2 - Journal of Perinatology TI - An antenatal marker of neurodevelopmental outcomes in infants with congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85028618761&doi=10.1038%2fjp.2017.59&partnerID=40&md5=6c67d609279db143fd2e7254acce5464 VL - 37 ID - 1850 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. AU - Sikora, K. AU - Janusz, B. DB - Medline IS - 4 KW - adaptive behavior adult child care female congenital heart malformation heart surgery human literature mother mother child relation newborn object relation psychology qualitative research verbal communication young adult LA - English M3 - Article N1 - L604326697 2015-05-15 PY - 2014 SN - 1428-345X SP - 439-446 ST - Maternal bond with cardiosurgically treated infant. Qualitative analysis of mothers' narratives T2 - Developmental period medicine TI - Maternal bond with cardiosurgically treated infant. Qualitative analysis of mothers' narratives UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L604326697 VL - 18 ID - 910 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 - Katedra Psychiatrii Uniwersytet Jagielloński, Zakład Psychologii Lekarskiej, Collegium Medicum, ul. Kopernika 21a, Kraków, tel. 602-469-928, Poland AU - Sikora, K. AU - Janusz, B. DB - Scopus IS - 4 M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2014 SP - 439-446 ST - Maternal bond with cardiosurgically treated infant. Qualitative analysis of mothers' narratives T2 - Developmental period medicine TI - Maternal bond with cardiosurgically treated infant. Qualitative analysis of mothers' narratives UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84929047481&partnerID=40&md5=85256d62dec250a9bc3d0e52c18a5240 VL - 18 ID - 1982 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. © 2019 Wiley Periodicals, Inc. AD - Division of Pediatric Cardiology, University of California San Diego and Rady Children's Hospital, United States Division of Pediatric ICU, University of California San Diego and Rady Children's Hospital, United States Division of Gastroenterology, University of California San Diego, United States Division of Pediatric Pathology, University of California San Diego and Rady Children's Hospital, United States Division of Pathology, University of California San Diego, United States Department of Radiology, Rady Children's Hospital, United States Department of medicine, Division of digestive diseases & transplant, University of Emory, United States Department of Pathology, Sharp Memorial hospital, United States Department of Interventional Radiology, Sharp Memorial hospital, United States University of California, United States University of Zagazig, Faculty of Medicine, Egypt, Egypt Division of Cardiology, University of California San Diego, United States 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. DB - Scopus DO - 10.1111/chd.12770 IS - 4 KW - cirrhosis exercise stress test fontan liver biopsy magnetic resonance elastography transient elastography M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2019 SP - 600-608 ST - Evaluation of Fontan liver disease: Correlation of transjugular liver biopsy with magnetic resonance and hemodynamics T2 - Congenital Heart Disease TI - Evaluation of Fontan liver disease: Correlation of transjugular liver biopsy with magnetic resonance and hemodynamics UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85065195966&doi=10.1111%2fchd.12770&partnerID=40&md5=4059afa1a1fe109dfd062f3f1c3acb9e VL - 14 ID - 1772 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 - L.C. Simko, School of Nursing, Duquesne University, Pittsburgh, PA 15282, USA. AU - Simko, L. C. AU - McGinnis, K. A. DB - Medline DO - 10.1097/00005082-200505000-00013 IS - 3 KW - adult analysis of variance article attitude to health classification comparative study congenital heart malformation cross-sectional study cyanosis daily life activity disabled person female health status hospitalization human male needs assessment nurse attitude nursing assessment patient education psychological aspect quality of life questionnaire Sickness Impact Profile United States LA - English M3 - Article N1 - L40832490 2005-06-27 PY - 2005 SN - 0889-4655 SP - 206-214 ST - What is the perceived quality of life of adults with congenital heart disease and does it differ by anomaly? T2 - The Journal of cardiovascular nursing TI - What is the perceived quality of life of adults with congenital heart disease and does it differ by anomaly? UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L40832490 http://dx.doi.org/10.1097/00005082-200505000-00013 VL - 20 ID - 1278 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, 600 Forbes Ave, Pittsburgh, PA 15282; Simko@duq.edu AN - 106472972. Language: English. Entry Date: 20050624. Revision Date: 20150818. Publication Type: Journal Article AU - Simko, L. C. AU - McGinnis, K. A. DB - ccm DP - EBSCOhost IS - 3 KW - Heart Defects, Congenital -- Classification Heart Defects, Congenital -- Psychosocial Factors -- In Adulthood Quality of Life -- Evaluation Academic Medical Centers Adult Analysis of Variance Aorta -- Abnormalities Aortic Coarctation Convenience Sample Cross Sectional Studies Cyanosis Data Analysis Software Descriptive Research Descriptive Statistics Employment Female Fisher's Exact Test Funding Source Health Status Indicators Heart Defects, Congenital -- Surgery Heart Septal Defects Heart -- Pathology Mail Male Middle Age Pennsylvania Pulmonary Artery -- Abnormalities Questionnaires Sickness Impact Profile Sleep T-Tests Tetralogy of Fallot Transposition of Great Arteries Tricuspid Atresia Human N1 - pictorial; research; tables/charts. Journal Subset: Core Nursing; Nursing; Peer Reviewed; USA. Instrumentation: Sickness Impact Profile (SIP). Grant Information: Sigma Theta Tau, Epsilon Phi Chapter, and a Dean's Dissertation Seed Grant, School of Nursing, Duquesne University. NLM UID: 8703516. PMID: NLM15870592. PY - 2005 SN - 0889-4655 SP - 206-214 ST - What is the perceived quality of life of adults with congenital heart disease and does it differ by anomaly? T2 - Journal of Cardiovascular Nursing TI - What is the perceived quality of life of adults with congenital heart disease and does it differ by anomaly? UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106472972&site=ehost-live&scope=site VL - 20 ID - 1522 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. (PsycINFO Database Record (c) 2020 APA, all rights reserved) AD - Simko, Lynn Coletta, School of Nursing, Duquesne University, 600 Forbes Ave, Pittsburgh, PA, US, 15282 AN - 2007-00865-004 AU - Simko, Lynn Coletta AU - McGinnis, Kathleen A. DB - psyh DO - 10.1097/00005082-200505000-00013 DP - EBSCOhost IS - 3 KW - perceived quality of life congenital heart disease acyanotic anomalies cyanotic lesions Activities of Daily Living Adult Analysis of Variance Attitude to Health Cross-Sectional Studies Cyanosis Disabled Persons Female Health Knowledge, Attitudes, Practice Health Status Heart Defects, Congenital Humans Male Needs Assessment New England Nurse's Role Nursing Assessment Patient Education as Topic Quality of Life Severity of Illness Index Sickness Impact Profile Surveys and Questionnaires Client Attitudes Heart Disorders N1 - School of Nursing, Duquesne University, Pittsburgh, PA, US. Release Date: 20070430. Correction Date: 20200312. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Client Attitudes; Heart Disorders; Quality of Life. Classification: Cardiovascular Disorders (3295); Health Psychology & Medicine (3360). Population: Human (10); Male (30); Female (40). Location: US. Age Group: Adulthood (18 yrs & older) (300). Tests & Measures: Sickness Impact Profile DOI: 10.1037/t04351-000. Methodology: Empirical Study; Quantitative Study. References Available: Y. Page Count: 9. Issue Publication Date: May-Jun, 2005. Sponsor: Sigma Theta Tau. Recipients: No recipient indicated Sponsor: Epsilon Phi Chapter. Recipients: No recipient indicated Sponsor: Duquesne University, School of Nursing. Other Details: Dean's Dissertation Seed Grant. Recipients: No recipient indicated PY - 2005 SN - 0889-4655 1550-5049 SP - 206-214 ST - What is the perceived quality of life of adults with congenital heart disease and does it differ by anomaly? T2 - Journal of Cardiovascular Nursing TI - What is the perceived quality of life of adults with congenital heart disease and does it differ by anomaly? UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2007-00865-004&site=ehost-live&scope=site Simko@duq.edu VL - 20 ID - 1693 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. © 2005 Lippincott Williams & Wilkins, Inc. AD - CCRN Associate Professor, School of Nursing, Duquesne University, Pittsburgh, United States AU - Simko, L. C. AU - McGinnis, K. A. DB - Scopus DO - 10.1097/00005082-200505000-00013 IS - 3 KW - Adults with congenital heart disease Quality of life Sickness Impact Profile (SIP) M3 - Article N1 - Cited By :20 Export Date: 15 June 2020 PY - 2005 SP - 206-214 ST - What is the perceived quality of life of adults with congenital heart disease and does it differ by anomaly? T2 - Journal of Cardiovascular Nursing TI - What is the perceived quality of life of adults with congenital heart disease and does it differ by anomaly? UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-20644452864&doi=10.1097%2f00005082-200505000-00013&partnerID=40&md5=cf953c4b2dd67d5486615042782dccb9 VL - 20 ID - 2247 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 ± 1.1 years; male, 10) and controls (n = 31; age, 16.0 ± 1.1 years; 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 - R. Kumar, Department of Anesthesiology, University of California, Los Angeles, CA, United States AU - Singh, S. AU - Kumar, R. AU - Roy, B. AU - Woo, M. A. AU - Lewis, A. AU - Halnon, N. AU - Pike, N. DB - Embase Medline DO - 10.1016/j.neulet.2017.12.011 KW - Manetom Tim Trio and Prisma nuclear magnetic resonance scanner adolescent article brain region caudate nucleus cerebellum clinical article cognition controlled study executive function female gray matter heart single ventricle human insula male mood change occipital gyrus parahippocampal gyrus prefrontal cortex primary motor cortex priority journal putamen temporal gyrus temporal lobe thalamus voxel based morphometry LA - English M3 - Article N1 - L619541254 2017-12-13 2018-11-21 PY - 2018 SN - 1872-7972 0304-3940 SP - 156-162 ST - Regional brain gray matter changes in adolescents with single ventricle heart disease T2 - Neuroscience Letters TI - Regional brain gray matter changes in adolescents with single ventricle heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L619541254 http://dx.doi.org/10.1016/j.neulet.2017.12.011 VL - 665 ID - 704 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 ± 1.1 years; male, 10) and controls (n = 31; age, 16.0 ± 1.1 years; 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. © 2017 Elsevier B.V. AD - Department of Anesthesiology, University of California, Los Angeles, CA, United States Department of Radiological Sciences, University of California, Los Angeles, CA, United States Department of Bioengineering, University of California, Los Angeles, CA, United States Brain Research Institute, University of California, Los Angeles, CA, United States UCLA School of Nursing, University of California, Los Angeles, CA, United States Division of Pediatric Cardiology, Children's Hospital, Los Angeles, CA, United States Division of Pediatric Cardiology, University of California, Los Angeles, CA, United States AU - Singh, S. AU - Kumar, R. AU - Roy, B. AU - Woo, M. A. AU - Lewis, A. AU - Halnon, N. AU - Pike, N. DB - Scopus DO - 10.1016/j.neulet.2017.12.011 KW - Cognition Congenital heart disease Gray matter Magnetic resonance imaging Voxel based morphometry M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2018 SP - 156-162 ST - Regional brain gray matter changes in adolescents with single ventricle heart disease T2 - Neuroscience Letters TI - Regional brain gray matter changes in adolescents with single ventricle heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85037353531&doi=10.1016%2fj.neulet.2017.12.011&partnerID=40&md5=12d468dfbcdf1a9ca2f4c7fd34f0fd4b VL - 665 ID - 1821 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 - R. Kumar, 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, United States 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. DB - Embase Medline DO - 10.1007/s00234-019-02208-x IS - 7 KW - adolescent adult amygdala anterior cingulate anterior insula article axial diffusivity basal forebrain brain fornix brain region brain tissue capsula interna caudate nucleus cerebellar peduncle cerebellum cerebellum cortex cingulate gyrus cognition controlled study corpus callosum diffusion tensor imaging female Fontan procedure fractional anisotropy frontal cortex heart single ventricle hippocampus human hypothalamus major clinical study male mammillary body mean diffusivity medial prefrontal cortex mesencephalon mood neuroimaging occipital cortex palliative therapy parahippocampal gyrus parietal cortex posterior cingulate posterior insula prefrontal cortex primary motor cortex priority journal radial diffusivity temporal cortex thalamus LA - English M3 - Article N1 - L627556547 2019-05-14 2019-06-17 PY - 2019 SN - 1432-1920 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L627556547 http://dx.doi.org/10.1007/s00234-019-02208-x VL - 61 ID - 610 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, 90095-1763, Los Angeles, CA, 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 Radiological Sciences, University of California Los Angeles, Los Angeles, CA, USA AN - 136828397. Language: English. Entry Date: 20190613. Revision Date: 20190613. Publication Type: Article AU - Singh, Sadhana AU - Roy, Bhaswati AU - Pike, Nancy AU - Daniel, Ebenezer AU - Ehlert, Luke AU - Lewis, Alan B. AU - Halnon, Nancy AU - Woo, Mary A. AU - Kumar, Rajesh DB - ccm DO - 10.1007/s00234-019-02208-x DP - EBSCOhost IS - 7 KW - Heart Ventricle -- Surgery -- In Adolescence Heart Defects, Congenital -- Surgery -- In Adolescence Magnetic Resonance Imaging -- Methods Brain Injuries -- In Adolescence Human Neuroradiography Adolescence Affect Cognition Analysis of Covariance Telencephalon Neural Pathways Temporal Lobe Frontal Lobe Hypothalamus Thalamus Cerebellum Amygdala N1 - diagnostic images; research; tables/charts. Journal Subset: Biomedical; Continental Europe; Europe; Peer Reviewed. NLM UID: 1302751. PY - 2019 SN - 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=136828397&site=ehost-live&scope=site VL - 61 ID - 1439 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. © 2019, Springer-Verlag GmbH Germany, part of Springer Nature. 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, United States UCLA School of Nursing, University of California Los Angeles, Los Angeles, CA, United States Division of Cardiology, Children’s Hospital, Los Angeles, CA, United States Division of Pediatric Cardiology, University of California Los Angeles, Los Angeles, CA, United States Department of Radiological Sciences, University of California Los Angeles, Los Angeles, CA, United States 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. DB - Scopus DO - 10.1007/s00234-019-02208-x IS - 7 KW - Brain injury Cognition Diffusion tensor imaging Single ventricle heart disease M3 - Article N1 - Export Date: 15 June 2020 PY - 2019 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85065247336&doi=10.1007%2fs00234-019-02208-x&partnerID=40&md5=5ac769ca5429715b93f1938224a20e82 VL - 61 ID - 1741 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 we size of the atrial septal opening. If the atrial septal defect is small it causes raised right atrial pressure, and this in turn results in raised systemic venous pressure and severe congestive failure. Jn such cases 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. AD - S.P. Singh, Univ. Birmingham Hosps, Birmingham AU - Singh, S. P. AU - Astley, R. AU - Parsons, C. G. DB - Embase Classic Medline IS - 5586 KW - 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 - L288098840 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=L288098840 VL - 1 ID - 1411 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 - Children's Hospital Colorado, University of Colorado, Denver, Aurora, CO, 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 - Scopus DO - 10.1177/1089253213475879 IS - 1 KW - angiotensin-converting enzyme inhibitors cardiac anesthesia cardiovascular risk children circulatory arrest pediatric congenital heart disease critical care heart failure intensive care unit near-infrared spectroscopy M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2013 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84874410481&doi=10.1177%2f1089253213475879&partnerID=40&md5=b73c27078964abf8285decf42b7624c0 VL - 17 ID - 2057 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 - J.J. Sistino, Medical University of South Carolina, MSC 962, 151B Rutledge Ave, Charleston, SC, United States AU - Sistino, J. J. AU - Atz, A. M. AU - Simpson, K. N. AU - Ellis, C. AU - Ikonomidis, J. S. AU - Bradley, S. M. DB - Embase Medline DO - 10.1017/S1047951114000547 IS - 4 KW - adolescent aortic arch interruption aortic coarctation aortic arch surgery article attention deficit disorder child cross-sectional study female heart ventricle septum defect human hypoplastic left heart syndrome major clinical study male newborn surgery Norwood procedure observational study prevalence quality of life risk assessment risk factor LA - English M3 - Article N1 - L53155123 2014-06-09 2015-03-18 PY - 2015 SN - 1467-1107 1047-9511 SP - 663-669 ST - The prevalence of attention-deficit/hyperactivity disorder following neonatal aortic arch repair T2 - Cardiology in the Young TI - The prevalence of attention-deficit/hyperactivity disorder following neonatal aortic arch repair UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L53155123 http://dx.doi.org/10.1017/S1047951114000547 VL - 25 ID - 862 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. AN - 109705332. Language: English. Entry Date: 20150923. Revision Date: 20160331. Publication Type: journal article. Journal Subset: Biomedical AU - Sistino, Joseph J. AU - Atz, Andrew M. AU - Simpson, Kit N. AU - Ellis, Charles AU - Ikonomidis, John S. AU - Bradley, Scott M. DB - ccm DO - 10.1017/S1047951114000547 DP - EBSCOhost IS - 4 N1 - Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Special Interest: Pediatric Care. NLM UID: 9200019. PMID: NLM24775274. PY - 2015 SN - 1047-9511 SP - 663-669 ST - The prevalence of attention-deficit/hyperactivity disorder following neonatal aortic arch repair T2 - Cardiology in the Young TI - The prevalence of attention-deficit/hyperactivity disorder following neonatal aortic arch repair UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=109705332&site=ehost-live&scope=site VL - 25 ID - 1528 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. © Cambridge University Press, 2014. AD - Medical University of South Carolina, College of Health Professions, Charleston, United States Medical University of South Carolina, College of Medicine, Charleston, United States Medical University of South Carolina, Division of Cardiothoracic Surgery, Charleston, United States AU - Sistino, J. J. AU - Atz, A. M. AU - Simpson, K. N. AU - Ellis, C., Jr. AU - Ikonomidis, J. S. AU - Bradley, S. M. DB - Scopus DO - 10.1017/S1047951114000547 IS - 4 KW - aortic arch repair Attention-deficit/hyperactivity disorder hypoplastic left-heart syndrome neonatal cardiac surgery M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2015 SP - 663-669 ST - The prevalence of attention-deficit/hyperactivity disorder following neonatal aortic arch repair T2 - Cardiology in the Young TI - The prevalence of attention-deficit/hyperactivity disorder following neonatal aortic arch repair UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84924499518&doi=10.1017%2fS1047951114000547&partnerID=40&md5=b083de521116c0231d81eff24603bc26 VL - 25 ID - 1945 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 - New England Research Institutes, Watertown, MA, United States Duke University Medical Center, Durham, NC, United States Columbia University Medical Center, New York, NY, United States University of Texas Southwestern Medical Center, Dallas, TX, United States The Hospital for Sick Children, Toronto, Ont., Canada Children's Hospital Boston, Boston, MA, United States Medical University of South Carolina, Charleston, SC, United States Primary Children's Medical Center, Salt Lake City, UT, United States Children's Hospital of Philadelphia, Philadelphia, PA, United States AU - Sleeper, L. A. AU - Anderson, P. AU - T. Hsu, D. 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 - Scopus DO - 10.1016/j.ahj.2006.02.009 IS - 3 M3 - Article N1 - Cited By :44 Export Date: 15 June 2020 PY - 2006 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-33747177409&doi=10.1016%2fj.ahj.2006.02.009&partnerID=40&md5=7006f0aa6e9ab1c316298cf4bbb37151 VL - 152 ID - 2223 ER - TY - JOUR AB - Background: Despite the low early mortality of Fontan procedures, Fontan patients are prone to various 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 complications 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. © 2018, Via Medica. All rights reserved. AD - Department of Cardiac and Vascular Diseases, Institute of Cardiology, Jagiellonian University Medical College, Krakow, Poland Department of Pediatric Cardiology, Polish-American Institute of Pediatrics (PAIP), Jagiellonian University, Medical College, Krakow, Poland Heart Surgery and General Pediatrics Surgery Department, Medical University of Warsaw, Poland 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 - Scopus DO - 10.5603/CJ.a2017.0078 IS - 1 KW - Adult Fontan patients Exercise tolerance Multiorgan complications Pulmonary function test Quality of life SF-36 M3 - Article N1 - Cited By :6 Export Date: 15 June 2020 PY - 2018 SP - 72-80 ST - What determines the quality of life of adult patients after fontan procedure? T2 - Cardiology Journal TI - What determines the quality of life of adult patients after fontan procedure? UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85042674115&doi=10.5603%2fCJ.a2017.0078&partnerID=40&md5=50ad3d978581f5cd387a016ff531c899 VL - 25 ID - 1820 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-D, 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 (0/16 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 - J.R. Sherbotie, Pediatric Nephrology, Children's Hospital of Philadelphia, 34th Street/Civic Center Boulevard, Philadelphia, PA 19104, United States AU - Smoyer, W. E. AU - McAdams, C. AU - Kaplan, B. S. AU - Sherbotie, J. R. DB - Embase Medline IS - 5 KW - adolescent adult anticoagulation article child female hemodialysis hemofiltration human infant major clinical study male newborn priority journal survival LA - English M3 - Article N1 - L25371144 1996-01-17 PY - 1995 SN - 1046-6673 SP - 1401-1409 ST - Determinants of survival in pediatric continuous hemofiltration T2 - Journal of the American Society of Nephrology TI - Determinants of survival in pediatric continuous hemofiltration UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L25371144 VL - 6 ID - 1366 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-D, 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 (0/16 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 - Pediatric Nephrology, Children's Hospital of Philadelphia, 34th Street/Civic Center Boulevard, Philadelphia, PA 19104, United States AU - Smoyer, W. E. AU - McAdams, C. AU - Kaplan, B. S. AU - Sherbotie, J. R. DB - Scopus IS - 5 KW - Anticoagulation Dialysis Filter life span Ultrafiltration M3 - Article N1 - Cited By :59 Export Date: 15 June 2020 PY - 1995 SP - 1401-1409 ST - Determinants of survival in pediatric continuous hemofiltration T2 - Journal of the American Society of Nephrology TI - Determinants of survival in pediatric continuous hemofiltration UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0028824246&partnerID=40&md5=6eb34d8fe62bc8314dd19a276abd3e77 VL - 6 ID - 2305 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 - Divisions of Cardiology, Pediatric Cardiology, University of Alberta, WMC 4C2.23, 8440 112th Street, Edmonton, AB T6G 2B7, Canada Department of Pediatrics, Inje University, Paik Hospital, Busan, South Korea Clinical and Metabolic Genetics, Department of Pediatrics, Canada Prenatal Diagnosis and Medical Genetics Program, Department of Laboratory Medicine and Pathobiology, Canada Fetal Medicine Unit, Department of Obstetrics and Gynecology, Mount Sinai Hospital, United States Department of Obstetrics and Gynecology, Women's College Hospital, University of Toronto, Toronto, ON, Canada Department of Medical Imaging, The Hospital for Sick Children, 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 - Scopus DO - 10.1002/uog.6309 IS - 5 KW - Chromosomal abnormalities Congenital heart defects Extracardiac lesions Fetal echocardiography Fetal ultrasound M3 - Article N1 - Cited By :58 Export Date: 15 June 2020 PY - 2009 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-66249105352&doi=10.1002%2fuog.6309&partnerID=40&md5=62f6599b9ff5271d1b7d7ac0ff9268ba VL - 33 ID - 2177 ER - TY - JOUR AD - University of Texas, Houston, United States AU - Spike, J. P. DB - Scopus DO - 10.1080/15265161.2017.1314707 IS - 7 M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2017 SP - 65-66 ST - The Ethics of Treatment for Hypoplastic Left Heart Syndrome (HLHS) T2 - American Journal of Bioethics TI - The Ethics of Treatment for Hypoplastic Left Heart Syndrome (HLHS) UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85021740041&doi=10.1080%2f15265161.2017.1314707&partnerID=40&md5=ed1d53779e853f7ce632284c800714fd VL - 17 ID - 1851 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 - G.B. Pettersson, Department of Thoracic and Cardiovascular Surgery, Cleveland Clinic, 9500 Euclid Ave/Desk J4-1, Cleveland, OH, 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. DB - Embase Medline DO - 10.1016/j.jtcvs.2019.08.104 IS - 4 KW - adolescent aorta valve atresia aortic valve disease article chronic disease controlled study cross-sectional study female follow up Fontan procedure heart atrium septum defect heart transplantation heart ventricle septum defect human major clinical study male mitral valve atresia newborn Norwood procedure palliative therapy Pediatric Quality of Life Inventory priority journal pulmonary valve insufficiency quality of life survival tricuspid valve regurgitation LA - English M3 - Article N1 - L2003800197 2019-11-28 2020-03-26 PY - 2020 SN - 1097-685X 0022-5223 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 - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2003800197 http://dx.doi.org/10.1016/j.jtcvs.2019.08.104 VL - 159 ID - 553 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 - Department of Psychosomatics and Health Behavior, Division of Mental Health, Norwegian Institute of Public Health, PO Box 4404 Nydalen, 0403 Oslo, Norway Institute of Psychology, University of Oslo, Oslo, Norway Department of Pediatrics, Oslo University Hospital, Oslo, Norway Department of Psychosomatics and Psychiatry, University Children's Hospital Zurich, Zurich, Switzerland Department of Pediatrics, Sørlandet Hospital HF, Kristiandsand, 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 - Scopus DO - 10.1111/j.1651-2227.2010.02015.x IS - 2 KW - Child internalizing problems Congenital heart defects (CHD) M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2011 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-78651376376&doi=10.1111%2fj.1651-2227.2010.02015.x&partnerID=40&md5=bcc073b212b310b76a6c78d9cc0dc640 VL - 100 ID - 2132 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 - C.L. Cua, Heart Center, Nationwide Children's Hospital, Columbus, OH, United States 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. DB - Embase Medline DO - 10.1111/chd.12257 IS - 2 KW - antidepressant agent anxiolytic agent adult age anxiety anxiety disorder article birth order bottle feeding caregiver child nutrition cross-sectional study depression educational status enteric feeding family size father feeding female food intake health care survey heart single ventricle heart surgery hospital discharge human interstage period major clinical study male mother nasogastric tube nose feeding postoperative period priority journal prospective study sex difference stomach tube time body weight gain LA - English M3 - Article N1 - L603849870 2015-04-23 2015-05-05 PY - 2015 SN - 1747-0803 1747-079X SP - E98-E106 ST - Caregiver anxiety due to interstage feeding concerns T2 - Congenital Heart Disease TI - Caregiver anxiety due to interstage feeding concerns UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L603849870 http://dx.doi.org/10.1111/chd.12257 VL - 10 ID - 875 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. © 2015 Wiley Periodicals, Inc. AD - Heart Center, Nationwide Children's Hospital, Columbus, OH, United States Section of Psychology, Nationwide Children's Hospital, Columbus, OH, United States 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. DB - Scopus DO - 10.1111/chd.12257 IS - 2 KW - Anxiety Hypoplastic left heart Interstage Nutrition Single ventricle M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2015 SP - E98-E106 ST - Caregiver anxiety due to interstage feeding concerns T2 - Congenital Heart Disease TI - Caregiver anxiety due to interstage feeding concerns UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84927725587&doi=10.1111%2fchd.12257&partnerID=40&md5=e0dfb64245cc6e4b0f74eb195462fef4 VL - 10 ID - 1951 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 - F. Stöcker, Department for Sport and Health Sciences, Teaching and Educational Center, Technical University Munich, Munich, Germany AU - Stöcker, F. AU - Neidenbach, R. AU - Fritz, C. AU - Oberhoffer, R. M. AU - Ewert, P. AU - Hager, A. AU - Nagdyman, N. DB - Embase DO - 10.3389/fped.2019.00096 IS - MAR KW - oxygen adolescent adult article body surface breathing muscle cardiopulmonary exercise test child clinical article deoxygenation exercise exercise intensity exhaustion female Fontan procedure heart rate human male muscle perfusion near infrared spectroscopy oxygen consumption oxygenation pediatric surgery post hoc analysis skinfold thickness LA - English M3 - Article N1 - L627528567 2019-07-18 PY - 2019 SN - 2296-2360 ST - Oxygen availability in respiratory muscles during exercise in children following fontan operation T2 - Frontiers in Pediatrics TI - Oxygen availability in respiratory muscles during exercise in children following fontan operation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L627528567 http://dx.doi.org/10.3389/fped.2019.00096 VL - 7 ID - 642 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. © 2019 Stöcker, Neidenbach, Fritz, Oberhoffer, Ewert, Hager and Nagdyman. 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 AU - Stöcker, F. AU - Neidenbach, R. AU - Fritz, C. AU - Oberhoffer, R. M. AU - Ewert, P. AU - Hager, A. AU - Nagdyman, N. C7 - 96 DB - Scopus DO - 10.3389/fped.2019.00096 IS - MAR KW - CHD Fontan Muscle oxygenation NIRS Pediatrics Respiratory muscles M3 - Article N1 - Export Date: 15 June 2020 PY - 2019 ST - Oxygen availability in respiratory muscles during exercise in children following fontan operation T2 - Frontiers in Pediatrics TI - Oxygen availability in respiratory muscles during exercise in children following fontan operation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85065399798&doi=10.3389%2ffped.2019.00096&partnerID=40&md5=c8ecbd065426496c60d55144ca0818b4 VL - 7 ID - 1771 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. AU - Stoffel, G. AU - Spirig, R. AU - Stiasny, B. AU - Bernet, V. AU - Dave, H. AU - Knirsch, W. DB - Medline DO - 10.1111/jocn.13694 IS - 21-22 KW - adult coping behavior female human hypoplastic left heart syndrome infant male newborn parent pathophysiology physiologic monitoring prospective study psychology qualitative research quality of life questionnaire young adult LA - English M3 - Article N1 - L620872450 2018-03-02 PY - 2017 SN - 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://www.embase.com/search/results?subaction=viewrecord&from=export&id=L620872450 http://dx.doi.org/10.1111/jocn.13694 VL - 26 ID - 729 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 - 125591383. Language: English. Entry Date: 20171017. Revision Date: 20190412. Publication Type: Article AU - Stoffel, Gaby AU - Spirig, Rebecca AU - Stiasny, Brian AU - Bernet, Vera AU - Dave, Hitendu AU - Knirsch, Walter DB - ccm DO - 10.1111/jocn.13694 DP - EBSCOhost IS - 21-22 KW - Hypoplastic Left Heart Syndrome Parents -- Psychosocial Factors Home Health Care -- In Infancy and Childhood Monitoring, Physiologic -- In Infancy and Childhood Parental Attitudes -- Evaluation Human Prospective Studies Multimethod Studies Infant Coping -- Classification Quality of Life Semi-Structured Interview Switzerland Quality of Life -- Evaluation Convenience Sample Short Form-36 Health Survey (SF-36) Questionnaires Scales Audiorecording Content Analysis Descriptive Statistics Friedman Test Wilcoxon Signed Rank Test Male Female Adult Middle Age Sex Factors Health Status Funding Source N1 - research; tables/charts. Journal Subset: Peer Reviewed. Special Interest: Home Health Care; Pediatric Care. Instrumentation: Short Form-36 Health Survey (SF-36); Impact on Family Scale (IOF-G)German version(Stein & Riessman). Grant Information: Anna M€uller GrocholskiFoundation (Reachout II). 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 - Journal of Clinical Nursing (John Wiley & Sons, Inc.) 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=ccm&AN=125591383&site=ehost-live&scope=site VL - 26 ID - 1462 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. © 2016 John Wiley & Sons Ltd 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 AU - Stoffel, G. AU - Spirig, R. AU - Stiasny, B. AU - Bernet, V. AU - Dave, H. AU - Knirsch, W. DB - Scopus DO - 10.1111/jocn.13694 IS - 21-22 KW - family hypoplastic left heart syndrome infant interstage monitoring psychosocial impact quality of life M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2017 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85013158313&doi=10.1111%2fjocn.13694&partnerID=40&md5=22b10f4fbdcce4130c613359b54ab6b4 VL - 26 ID - 1840 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 - J.M. Stulak, Division of Cardiovascular Surgery, Mayo Clinic and Foundation, Rochester, MN 55905, USA. AU - Stulak, J. M. AU - Dearani, J. A. AU - Danielson, G. K. DB - Medline DO - 10.1053/j.pcsu.2007.01.007 KW - adolescent adult age aged article bioprosthesis child Ebstein anomaly heart left ventricle function heart right ventricle function heart surgery heart valve replacement human infant methodology middle aged pathophysiology preschool child retrospective study risk factor supraventricular tachycardia survival suture technique treatment outcome United States LA - English M3 - Article N1 - L47451970 2007-09-28 PY - 2007 SN - 1092-9126 SP - 105-111 ST - Surgical management of Ebstein's anomaly T2 - Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual TI - Surgical management of Ebstein's anomaly UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L47451970 http://dx.doi.org/10.1053/j.pcsu.2007.01.007 ID - 1228 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. © 2007 Elsevier Inc. All rights reserved. AD - J.A. Dearani, Division of Cardiovascular Surgery, Mayo Clinic and Foundation, Rochester, MN, United States AU - Stulak, J. M. AU - Dearani, J. A. AU - Danielson, G. K. C1 - aspirin DB - Embase DO - 10.1053/j.pcsu.2007.01.007 IS - 1 KW - acetylsalicylic acid warfarin article cardiopulmonary bypass cryoablation Ebstein anomaly exercise tolerance Fontan procedure heart right left shunt heart right ventricle failure heart surgery heart valve replacement human intermethod comparison pediatric surgery postoperative period postoperative thrombosis quality of life reoperation supraventricular tachycardia surgical anatomy surgical mortality surgical risk surgical technique survival rate treatment indication treatment outcome tricuspid valve vein anastomosis aspirin LA - English M3 - Article N1 - L46547287 2007-05-04 PY - 2007 SN - 1092-9126 SP - 105-111 ST - Surgical Management of Ebstein's Anomaly T2 - Pediatric Cardiac Surgery Annual TI - Surgical Management of Ebstein's Anomaly UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L46547287 http://dx.doi.org/10.1053/j.pcsu.2007.01.007 VL - 10 ID - 1236 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. © 2007 Elsevier Inc. All rights reserved. AD - Division of Cardiovascular Surgery, Mayo Clinic and Foundation, Rochester, MN, United States AU - Stulak, J. M. AU - Dearani, J. A. AU - Danielson, G. K. DB - Scopus DO - 10.1053/j.pcsu.2007.01.007 IS - 1 KW - congenital tricuspid valve Ebstein's anomaly heart defect M3 - Article N1 - Cited By :16 Export Date: 15 June 2020 PY - 2007 SP - 105-111 ST - Surgical Management of Ebstein's Anomaly T2 - Pediatric Cardiac Surgery Annual TI - Surgical Management of Ebstein's Anomaly UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-34047276654&doi=10.1053%2fj.pcsu.2007.01.007&partnerID=40&md5=f84609915422b9734d00ec75b5bf12ae VL - 10 ID - 2211 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. © 2006 The Society of Thoracic Surgeons. AD - J.A. Dearani, Division of Cardiovascular Surgery, Mayo Clinic and Foundation, Rochester, MN, United States AU - Stulak, J. M. AU - Dearani, J. A. AU - Puga, F. J. AU - Zehr, K. J. AU - Schaff, H. V. AU - Danielson, G. K. DB - Embase Medline DO - 10.1016/j.athoracsur.2005.10.046 IS - 5 KW - adult age distribution aged article congenital heart disease controlled study disease duration Ebstein anomaly Fallot tetralogy female follow up atrial fibrillation heart atrium flutter heart atrium septum defect heart single ventricle heart surgery heart valve replacement heart valve surgery hospital discharge human major clinical study male patient coding priority journal pulmonary valve recurrent disease right sided maze procedure school child sinus rhythm supraventricular tachycardia survival time tricuspid valve regurgitation LA - English M3 - Article N1 - L350029997 2006-05-01 PY - 2006 SN - 0003-4975 SP - 1780-1785 ST - Right-Sided Maze Procedure for Atrial Tachyarrhythmias in Congenital Heart Disease T2 - Annals of Thoracic Surgery TI - Right-Sided Maze Procedure for Atrial Tachyarrhythmias in Congenital Heart Disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L350029997 http://dx.doi.org/10.1016/j.athoracsur.2005.10.046 VL - 81 ID - 1263 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. © 2006 The Society of Thoracic Surgeons. AD - Division of Cardiovascular Surgery, Mayo Clinic and Foundation, Rochester, MN, United States AU - Stulak, J. M. AU - Dearani, J. A. AU - Puga, F. J. AU - Zehr, K. J. AU - Schaff, H. V. AU - Danielson, G. K. DB - Scopus DO - 10.1016/j.athoracsur.2005.10.046 IS - 5 M3 - Article N1 - Cited By :54 Export Date: 15 June 2020 PY - 2006 SP - 1780-1785 ST - Right-Sided Maze Procedure for Atrial Tachyarrhythmias in Congenital Heart Disease T2 - Annals of Thoracic Surgery TI - Right-Sided Maze Procedure for Atrial Tachyarrhythmias in Congenital Heart Disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-33646847803&doi=10.1016%2fj.athoracsur.2005.10.046&partnerID=40&md5=772a4a3e6cc689ef1cf685327fb6dfc0 VL - 81 ID - 2231 ER - TY - JOUR AD - Birmingham Children's Hospital NHS Trust, Birmingham, UK. oliver.stumper@bch.nhs.uk AN - 20133423 AU - Stumper, O. DA - Feb DB - PubMed DO - 10.1136/hrt.2008.159889 DP - NLM ET - 2010/02/06 IS - 3 KW - Cardiac Surgical Procedures/*methods/mortality Child Child, Preschool Counseling Developmental Disabilities/etiology Humans Hypoplastic Left Heart Syndrome/diagnosis/genetics/*surgery Infant Medical Illustration Postoperative Care/methods Prenatal Diagnosis LA - eng N1 - 1468-201x Stumper, Oliver Journal Article England Heart. 2010 Feb;96(3):231-6. doi: 10.1136/hrt.2008.159889. PY - 2010 SN - 1355-6037 SP - 231-6 ST - Hypoplastic left heart syndrome T2 - Heart TI - Hypoplastic left heart syndrome VL - 96 ID - 473 ER - TY - JOUR AD - Birmingham Children's Hospital NHS Trust, Birmingham, UK; oliver.stumper@bch.nhs.uk. AN - 105316138. Language: English. Entry Date: 20100409. Revision Date: 20160106. Publication Type: Journal Article. Journal Subset: Biomedical AU - Stumper, O. DB - ccm DO - 10.1136/hrt.2008.159889 DP - EBSCOhost IS - 3 KW - Heart Surgery -- Methods Hypoplastic Left Heart Syndrome -- Surgery Heart Surgery -- Mortality Child Child, Preschool Counseling Developmental Disabilities -- Etiology Hypoplastic Left Heart Syndrome -- Diagnosis Hypoplastic Left Heart Syndrome Infant Medical Illustration Postoperative Care -- Methods Prenatal Diagnosis N1 - Blind Peer Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. NLM UID: 9602087. PMID: NLM20133423. PY - 2010 SN - 1355-6037 SP - 231-236 ST - Hypoplastic left heart syndrome T2 - Heart TI - Hypoplastic left heart syndrome UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105316138&site=ehost-live&scope=site VL - 96 ID - 1608 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. © 2017 Cambridge University Press. AD - Department of Cardiovascular Surgery, Kitasato University, School of Medicine, 1-15-1, Kitasato, Minami-ward, Sagamihara, 252-0375, Japan Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia Graduate School of Engineering, Chiba University, 1-33, Yayoi-cho, Inage-ku, Chiba, 263-8522, Japan Department of Paediatrics, University of Melbourne, Melbourne, Australia School of Naval Architecture, Ocean and Civil Engineering, Shanghai Jiao Tong University, Shanghai, China Shanghai Jiao Tong University, Chiba University International Cooperative Research Centre (SJTU-CUICRC), Shanghai, China AU - Sughimoto, K. AU - Asakura, Y. AU - Brizard, C. P. AU - Liang, F. AU - Fujiwara, T. AU - Miyaji, K. AU - Liu, H. DB - Scopus DO - 10.1017/S1047951117000099 IS - 7 KW - computer fluid dynamics fenestration Fontan haemodynamics M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2017 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85017149209&doi=10.1017%2fS1047951117000099&partnerID=40&md5=5463c7ad3ef7a462326316b7c15ead80 VL - 27 ID - 1845 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 antiarrhythmics, 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. © 2001 Elsevier Science Ireland Ltd. AD - Department of Thoracic and Cardiovascular Surgery, Sapporo Medical University School of Medicine, South-1, West-16, Cho-ku, Sapporo 060-8556, Japan AU - Sugimoto, S. AU - Takagi, N. AU - Hachiro, Y. AU - Abe, T. DB - Scopus DO - 10.1016/S0167-5273(00)00460-5 IS - 1 KW - Anticoagulant Arrhythmias Atrio-pulmonary connection Intracardiac thrombus Late sequelae Modified Fontan operation M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 2001 SP - 33-39 ST - High frequency of arrhythmias after Fontan operation indicates earlier anticoagulant therapy T2 - International Journal of Cardiology TI - High frequency of arrhythmias after Fontan operation indicates earlier anticoagulant therapy UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0035079646&doi=10.1016%2fS0167-5273%2800%2900460-5&partnerID=40&md5=e8856489e324491de81a1bcf37edfb98 VL - 78 ID - 2270 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. © 2018 Elsevier Inc. AD - Tissue Engineering Program and Surgical Research, Nationwide Children's Hospital, Columbus, Ohio, United States Department of Cardiovascular Surgery, Tokyo Women's Medical University, Tokyo, Japan Department of Cardiothoracic Surgery, The Heart Center, Nationwide Children's Hospital, Columbus, Ohio, United States AU - Sugiura, T. AU - Matsumura, G. AU - Miyamoto, S. AU - Miyachi, H. AU - Breuer, C. K. AU - Shinoka, T. DB - Scopus DO - 10.1053/j.semtcvs.2018.02.002 IS - 2 KW - congenital heart disease Fontan surgery pediatric cardiac surgery tissue-engineering univentricular physiology M3 - Article N1 - Cited By :21 Export Date: 15 June 2020 PY - 2018 SP - 175-179 ST - Tissue-engineered Vascular Grafts in Children With Congenital Heart Disease: Intermediate Term Follow-up T2 - Seminars in Thoracic and Cardiovascular Surgery TI - Tissue-engineered Vascular Grafts in Children With Congenital Heart Disease: Intermediate Term Follow-up UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85044273633&doi=10.1053%2fj.semtcvs.2018.02.002&partnerID=40&md5=b8bd342026dab7285eba0ff314537b18 VL - 30 ID - 1799 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 - Y. D'Udekem, Cardiac Surgery Department, Royal Children's Hospital, 50 Flemington Road, Melbourne, VIC, Australia 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. DB - Embase Medline DO - 10.1017/S1047951118001166 IS - 11 KW - adolescent adult anaerobic exercise article cardiopulmonary exercise test child clinical article controlled study exercise female Fontan procedure home care home visit hospital care human male oxygen consumption Pediatric Quality of Life Inventory physiotherapist postoperative period prospective study pulse rate quality of life social psychology teleconsultation LA - English M3 - Article N1 - L623816550 2018-09-13 2019-01-11 PY - 2018 SN - 1467-1107 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://www.embase.com/search/results?subaction=viewrecord&from=export&id=L623816550 http://dx.doi.org/10.1017/S1047951118001166 VL - 28 ID - 657 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. 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. DB - Medline DO - 10.1017/S1047951118001166 IS - 11 KW - adolescent child clinical trial congenital heart malformation exercise tolerance female Fontan procedure home care hospital patient human kinesiotherapy male multicenter study pathophysiology physiology postoperative care procedures quality of life questionnaire treatment outcome young adult LA - English M3 - Article N1 - L624490649 2018-12-03 PY - 2018 SN - 1467-1107 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://www.embase.com/search/results?subaction=viewrecord&from=export&id=L624490649 http://dx.doi.org/10.1017/S1047951118001166 VL - 28 ID - 659 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. © Cambridge University Press 2018. AD - Cardiac Surgery Department, Royal Children's Hospital, 50 Flemington Road, Melbourne, VIC 3052, Australia Murdoch Childrens Research Institute, Melbourne, VIC, Australia Department of Paediatrics, Faculty of Medicine, University of Melbourne, Melbourne, VIC, Australia Department of Cardiology, Royal Children's Hospital, Melbourne, VIC, Australia Department of Physiotherapy, Royal Children's Hospital, Melbourne, VIC, Australia 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. DB - Scopus DO - 10.1017/S1047951118001166 IS - 11 KW - CHD exercise therapy Fontan procedure rehabilitation resistance training M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2018 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85052937377&doi=10.1017%2fS1047951118001166&partnerID=40&md5=d8ebb135c15bd4285517fe3cd1dfb4d4 VL - 28 ID - 1785 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 - B. Svensson, Department of Cardiology, Pediatric Heart Center, Skane University Hospital, Lund, Sweden AU - Svensson, B. AU - Idvall, E. AU - Nilsson, F. AU - Liuba, P. DB - Embase Medline DO - 10.1017/S1047951116000585 IS - 2 KW - cardiovascular agent adolescent article biventricular heart child chronic disease congenital heart disease controlled study disease registry female health survey heart single ventricle heart surgery human major clinical study male mental capacity patient coding quality of life retrospective study school child school health service scoring system Sweden LA - English M3 - Article N1 - L610530457 2016-06-03 2017-04-25 PY - 2017 SN - 1467-1107 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 - Cardiology in the Young TI - Health-related quality of life in children with surgery for CHD: A study from the Swedish National Registry for Congenital Heart Disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L610530457 http://dx.doi.org/10.1017/S1047951116000585 VL - 27 ID - 765 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 - Department of Cardiology, Pediatric Heart Center, Skåne University Hospital, Lund, Sweden Department of Care and Science, Faculty of Health and Society, Malmö University, Malmö, Sweden Research and Development Centre, Medical Statistics and Epidemiology, Skåne University Hospital, Lund, Sweden AN - 122400116. Language: English. Entry Date: 20170701. Revision Date: 20180301. Publication Type: journal article. Journal Subset: Biomedical AU - Svensson, Birgitta AU - Idvall, Ewa AU - Nilsson, Fredrik AU - Liuba, Petru DB - ccm DO - 10.1017/S1047951116000585 DP - EBSCOhost IS - 2 KW - Data Collection Quality of Life Heart Surgery Heart Defects, Congenital -- Surgery Health Status Risk Factors Incidence Sweden Heart Defects, Congenital -- Psychosocial Factors Male Retrospective Design Child Female Heart Defects, Congenital -- Epidemiology Adolescence Scales N1 - Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Instrumentation: Health-Related Hardiness Scale (HRHS). NLM UID: 9200019. PMID: NLM27225489. 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 - Cardiology in the Young TI - Health-related quality of life in children with surgery for CHD: a study from the Swedish National Registry for Congenital Heart Disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=122400116&site=ehost-live&scope=site VL - 27 ID - 1486 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. © Cambridge University Press 2016. AD - Department of Cardiology, Pediatric Heart Center, Skane University Hospital, Lund, 22185, Sweden Department of Care and Science, Faculty of Health and Society, Malmö University, Malmö, Sweden Research and Development Centre, Medical Statistics and Epidemiology, Skane University Hospital, Lund, Sweden Lund University, Box 117, Lund, 22100, Sweden AU - Svensson, B. AU - Idvall, E. AU - Nilsson, F. AU - Liuba, P. DB - Scopus DO - 10.1017/S1047951116000585 IS - 2 KW - CHD children Health-related quality of life registry study M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2017 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 - Cardiology in the Young TI - Health-related quality of life in children with surgery for CHD: A study from the Swedish National Registry for Congenital Heart Disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84970005767&doi=10.1017%2fS1047951116000585&partnerID=40&md5=978e01dc4c371c89f088114940bc3a68 VL - 27 ID - 1868 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. AD - L.T. Swanson AU - Swanson, L. T. DB - Medline IS - 3 KW - article case report decision making human hypoplastic left heart syndrome male mother newborn palliative therapy psychological aspect LA - English M3 - Article N1 - L125074558 1995-07-12 PY - 1995 SN - 0279-5442 SP - 70-72, 76-79 ST - Treatment options for hypoplastic left heart syndrome: a mother's perspective T2 - Critical care nurse TI - Treatment options for hypoplastic left heart syndrome: a mother's perspective UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L125074558 VL - 15 ID - 1367 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. AU - Swanson, L. T. DB - Scopus IS - 3 M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 1995 SP - 70-72, 76 ST - Treatment options for hypoplastic left heart syndrome: a mother's perspective T2 - Critical care nurse TI - Treatment options for hypoplastic left heart syndrome: a mother's perspective UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0029321430&partnerID=40&md5=d969421541b3fd4476c175e0cbbb4eeb VL - 15 ID - 2302 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 I 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 - S. Tabbutt, Gardiac Intensive Care Unit, Cardiac Center, Children Hospital of Philadelphia, 4th Street and Civic Center Boulevard, Philadelphia, PA 19104 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. DB - Embase Medline DO - 10.1542/peds.2007-1282 IS - 3 KW - article Bayley Scales of Infant Development II birth weight cardiopulmonary bypass chromosome aberration deep hypothermic circulatory arrest developmental disorder developmental outcome developmental screening endotracheal intubation ethnicity extracorporeal oxygenation female genetic disorder gestational age head circumference human hypoplastic left heart syndrome infant Kabuki makeup syndrome length of stay major clinical study male mental development metabolic acidosis microcephaly Norwood procedure outcome assessment priority journal psychomotor development risk factor shock statistical analysis survival rate Turner syndrome LA - English M3 - Article N1 - L354318000 2009-04-02 http://pediatrics.aappublications.org/cgi/reprint/121/3/476 PY - 2008 SN - 0031-4005 1098-4275 SP - 476-483 ST - Neurodevelopmental outcomes after staged palliation for hypoplastic left heart syndrome T2 - Pediatrics TI - Neurodevelopmental outcomes after staged palliation for hypoplastic left heart syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L354318000 http://dx.doi.org/10.1542/peds.2007-1282 VL - 121 ID - 1220 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 90â€̂485 admissions (rate 30 per 10â€̂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 - C.M. Takemoto, Division of Pediatric Hematology, Johns Hopkins Hospital, 720 Rutland Ave/Ross 1125, Baltimore, MD 21205, United States 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. DB - Embase Medline DO - 10.1016/j.jpeds.2013.10.025 IS - 2 KW - adolescent adult age distribution article central venous catheter child childhood injury comorbidity disease association female heart disease hospital associated venous thromboembolism hospitalized child human major clinical study male medical record review patient history of surgery preschool child priority journal retrospective study school child venous thromboembolism young adult LA - English M3 - Article N1 - L52913754 2013-12-18 2014-02-04 PY - 2014 SN - 0022-3476 1097-6833 SP - 332-338 ST - Hospital-associated venous thromboembolism in children: Incidence and clinical characteristics T2 - Journal of Pediatrics TI - Hospital-associated venous thromboembolism in children: Incidence and clinical characteristics UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52913754 http://dx.doi.org/10.1016/j.jpeds.2013.10.025 VL - 164 ID - 922 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 90â€̂485 admissions (rate 30 per 10â€̂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 Hospital, 720 Rutland Ave/Ross 1125, Baltimore, MD 21205, United States Division of Quality and Safety, Department of Pediatrics, Johns Hopkins University, Baltimore, MD, United States Pediatric Thrombosis Program, All Children's Hospital - Johns Hopkins Medicine, St. Petersburg, FL, United States Division of Adult Hematology, Johns Hopkins Hospital, Baltimore, MD, United States 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. DB - Scopus DO - 10.1016/j.jpeds.2013.10.025 IS - 2 KW - CCC Central venous catheter Complex chronic condition CVC Deep venous thrombosis DVT HLHS Hypoplastic left heart syndrome ICD-9 Incidence rate ratio International Classification of Diseases, Ninth Revision IRR PE Pulmonary embolism Venous thromboembolism VTE M3 - Article N1 - Cited By :53 Export Date: 15 June 2020 PY - 2014 SP - 332-338 ST - Hospital-associated venous thromboembolism in children: Incidence and clinical characteristics T2 - Journal of Pediatrics TI - Hospital-associated venous thromboembolism in children: Incidence and clinical characteristics UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84892782518&doi=10.1016%2fj.jpeds.2013.10.025&partnerID=40&md5=782d6a169873dab735bf0866db418970 VL - 164 ID - 1995 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 - Department of Cardiothoracic and Vascular Surgery, Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, 110 029, India Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India Department of Biostatistics, 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 - Scopus DO - 10.4103/apc.APC_43_17 IS - 1 KW - Bidirectional Glenn exercise performance fontan operation single ventricle M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2018 SP - 40-47 ST - Exercise performance after univentricular palliation T2 - Annals of Pediatric Cardiology TI - Exercise performance after univentricular palliation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85041097471&doi=10.4103%2fapc.APC_43_17&partnerID=40&md5=fcab417e7ac5230abf820ff7d5774f2c VL - 11 ID - 1836 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 - A.P. Yoganathan, School of Chemical and Biomolecular Engineering, Georgia Institute of Technology, Emory University, Technology Enterprise Park, Suite 200, 387 Technology Circle, Atlanta, GA, United States AU - Tang, E. AU - Wei, Z. 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. DB - Embase Medline DO - 10.1136/heartjnl-2016-310855 IS - 22 KW - adolescent adult article blood flow velocity body weight cardiovascular magnetic resonance child clinical article controlled study correlation analysis exercise exercise test exercise tolerance female Fontan procedure hemodynamics human lower limb lung ventilation male oxygen consumption priority journal school child simulation supine position ventilatory anaerobic threshold LA - English M3 - Article N1 - L619048572 2017-11-09 2017-11-14 PY - 2017 SN - 1468-201X 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 UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L619048572 http://dx.doi.org/10.1136/heartjnl-2016-310855 VL - 103 ID - 727 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. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. AD - School of Chemical and Biomolecular Engineering, Georgia Institute of Technology, Emory University, Technology Enterprise Park, Suite 200, 387 Technology Circle, Atlanta, GA 30313-2412, United States Wallace H. Coulter Department of Biomedical Engineering, Georgia Institute of Technology, Emory University, Atlanta, GA, United States Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA, United States Westat Inc, Rockville, MD, United States Division of Cardiology, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, United States AU - Tang, E. AU - Wei, Z. 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. DB - Scopus DO - 10.1136/heartjnl-2016-310855 IS - 22 KW - congenital heart disease Fontan procedure exercise hemodynamics computational fluid dynamics M3 - Article N1 - Cited By :16 Export Date: 15 June 2020 PY - 2017 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85032735731&doi=10.1136%2fheartjnl-2016-310855&partnerID=40&md5=1bf5c069d045196029fbccb32c134364 VL - 103 ID - 1842 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. © 2015 The American Association for Thoracic Surgery. AD - Department of Cardiovascular Surgery, Okayama University, Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan Department of Pediatrics, Okayama University, Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan Department of Regenerative Medicine, Center for Innovative Clinical Medicine, Okayama University Hospital, 2-5-1 Shikata-cho, Kita-ku, Okayama, 700-8558, Japan AU - Tarui, S. AU - Ishigami, S. AU - Ousaka, D. AU - Kasahara, S. AU - Ohtsuki, S. AU - Sano, S. AU - Oh, H. DB - Scopus DO - 10.1016/j.jtcvs.2015.06.076 IS - 5 KW - cardiosphere congenital heart disease heart failure hypoplastic left heart syndrome stem cell therapy M3 - Article N1 - Cited By :55 Export Date: 15 June 2020 PY - 2015 SP - 1198-1208.e2 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84948714563&doi=10.1016%2fj.jtcvs.2015.06.076&partnerID=40&md5=ddc71fe126f13e655cd9e073d369f32c VL - 150 ID - 1937 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 - Department of Pathology, Faculty of Medicine, Chulalongkorn University, 1873 King Rama IV Street, Pathumwan, Bangkok, 10330, Thailand Department of Pathology and Laboratory Medicine, Hospital for Sick Children, University of Toronto, ON M5G1X8, Canada AU - Taweevisit, M. AU - Atikankul, T. AU - Thorner, P. S. DB - Scopus DO - 10.2350/13-07-1354-OA.1 IS - 3 KW - Adipophilin immunohistochemistry Adrenal gland Definitive zone Fetal zone Hemoglobin Bart Hydrops fetalis M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2014 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84905030307&doi=10.2350%2f13-07-1354-OA.1&partnerID=40&md5=0d80491b21a4b12b37a766d159716f5e VL - 17 ID - 2024 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 - K. Texter, Division of Cardiology, Nationwide Children's Hospital, Columbus, OH, United States AU - Texter, K. AU - Davis, J. A. M. AU - Phelps, C. AU - Cheatham, S. AU - Cheatham, J. AU - Galantowicz, M. AU - Feltes, T. F. DB - Embase Medline DO - 10.1111/chd.12459 IS - 4 KW - article heart center heart single ventricle human longitudinal care major clinical study medical care mortality outcome assessment patient care priority journal protocol compliance quality of life standardization treatment outcome LA - English M3 - Article N1 - L617721262 2017-08-17 2017-08-22 PY - 2017 SN - 1747-0803 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 - Congenital Heart Disease TI - Building a comprehensive team for the longitudinal care of single ventricle heart defects: Building blocks and initial results UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L617721262 http://dx.doi.org/10.1111/chd.12459 VL - 12 ID - 745 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. © 2017 Wiley Periodicals, Inc. AD - Division of Cardiology, Nationwide Children's Hospital, Columbus, OH, United States Department of Pediatrics, The Ohio State University, Columbus, OH, United States Division of Cardiothoracic Surgery, Nationwide Children's Hospital, Columbus, OH, United States AU - Texter, K. AU - Davis, J. A. M. AU - Phelps, C. AU - Cheatham, S. AU - Cheatham, J. AU - Galantowicz, M. AU - Feltes, T. F. DB - Scopus DO - 10.1111/chd.12459 IS - 4 KW - care coordination hypoplastic left heart quality improvement M3 - Article N1 - Cited By :6 Export Date: 15 June 2020 PY - 2017 SP - 403-410 ST - Building a comprehensive team for the longitudinal care of single ventricle heart defects: Building blocks and initial results T2 - Congenital Heart Disease TI - Building a comprehensive team for the longitudinal care of single ventricle heart defects: Building blocks and initial results UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85026914882&doi=10.1111%2fchd.12459&partnerID=40&md5=3860964984c1891824605680de6f58f3 VL - 12 ID - 1853 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. Copyright © 2012 by The American Association for Thoracic Surgery. AD - Department of Mechanical Engineering, Virginia Commonwealth University School of Engineering, 401 West Main Street, Richmond, VA 23284, United States Division of Pediatric Cardiology, Virginia Commonwealth University, Medical College of Virginia, Richmond, VA, United States Division of Thoracic and Cardiovascular Surgery, University of Virginia School of Medicine, Charlottesville, VA, United States Wallace H. Coulter School of Biomedical Engineering, Georgia Institute of Technology, Emory University, Atlanta, GA, United States AU - Throckmorton, A. L. AU - Carr, J. P. AU - Moskowitz, W. B. AU - Gangemi, J. J. AU - Haggerty, C. M. AU - Yoganathan, A. P. DB - Scopus DO - 10.1016/j.jtcvs.2011.12.061 IS - 3 M3 - Article N1 - Cited By :9 Export Date: 15 June 2020 PY - 2012 SP - 704-709 ST - Uniquely shaped cardiovascular stents enhance the pressure generation of intravascular blood pumps T2 - Journal of Thoracic and Cardiovascular Surgery TI - Uniquely shaped cardiovascular stents enhance the pressure generation of intravascular blood pumps UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84865697251&doi=10.1016%2fj.jtcvs.2011.12.061&partnerID=40&md5=0e63553870e475e733e0d06d506e2c70 VL - 144 ID - 2086 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 mm Hg 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. © 2012 Wichtig Editore. AD - A. L. Throckmorton, BioCirc Research Laboratory, Department of Mechanical and Nuclear Engineering, School of Engineering, Virginia Commonwealth University, 401 West Main Street, P.O. Box 843015, Richmond, VA 23284, United States AU - Throckmorton, A. L. AU - Downs, E. A. AU - Hazelwood, J. A. AU - Monroe, J. O. AU - Chopski, S. G. DB - Embase Medline DO - 10.5301/ijao.5000105 IS - 5 KW - article blood flow velocity blood pump computational fluid dynamics energy transfer Fontan procedure geometry heart assist device hydraulic conductivity intravascular axial flow blood pump pressure simulation stress validation process LA - English M3 - Article N1 - L365145449 2012-07-09 2012-07-17 http://www.artificial-organs.com/public/IJAO/Article/Attach.action?cmd=Download&uid=98BD7545-65CB-455D-9DC6-26B24FA10694 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://www.embase.com/search/results?subaction=viewrecord&from=export&id=L365145449 http://dx.doi.org/10.5301/ijao.5000105 VL - 35 ID - 1050 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 mm Hg 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. © 2012 Wichtig Editore. AD - BioCirc Research Laboratory, Department of Mechanical and Nuclear Engineering, School of Engineering, Virginia Commonwealth University, 401 West Main Street, P.O. Box 843015, Richmond, VA 23284, United States AU - Throckmorton, A. L. AU - Downs, E. A. AU - Hazelwood, J. A. AU - Monroe, J. O. AU - Chopski, S. G. DB - Scopus DO - 10.5301/ijao.5000105 IS - 5 KW - Artificial heart pump Axial flow blood pump Cardiovascular stent Mechanical circulatory support Rotary blood pump M3 - Article N1 - Cited By :7 Export Date: 15 June 2020 PY - 2012 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84863203002&doi=10.5301%2fijao.5000105&partnerID=40&md5=fb14b3945d08bd30a41743bf3183e58e VL - 35 ID - 2088 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. © Wichtig Editore, 2008. AD - Department of Mechanical Engineering, Virginia Commonwealth University, 601 West Main Street, Richmond, VA 23284, United States AU - Throckmorton, A. L. AU - Kapadia, J. AU - Madduri, D. DB - Scopus DO - 10.1177/039139880803101107 IS - 11 KW - Artificial right ventricle Blood pump Cavopulmonary assist device Fontan conversion Heart pump Pediatric circulatory support Pediatric mechanical circulatory support Pediatric ventricular assist device Single ventricle physiology Ventricular assist device M3 - Article N1 - Cited By :16 Export Date: 15 June 2020 PY - 2008 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-59549095868&doi=10.1177%2f039139880803101107&partnerID=40&md5=25d46aceadbc94e53fedce0464d6faae VL - 31 ID - 2202 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 16 mm Hg of pressure rise for flow rates of 1 to 4 L/min at 4000 to 7000 rpm. The difference between the CFD predictions and experimental results was found to be approximately 9.8%. Scalar stress levels remained below 570 Pa with exposure times on the order of 1.5 s. 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. © 2010, the Authors. Artificial Organs © 2010, International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc. AD - A.L. Throckmorton, Department of Mechanical Engineering, Virginia Commonwealth University, 401 West Main Street, P.O. Box 843015, Richmond, VA 23284, United States AU - Throckmorton, A. L. AU - Kapadia, J. Y. AU - Wittenschlaeger, T. M. AU - Medina, T. J. AU - Hoang, H. Q. AU - Bhavsar, S. S. DB - Embase Medline DO - 10.1111/j.1525-1594.2010.01139.x IS - 11 KW - article blood pump cavopulmonary connection computational fluid dynamics flow rate Fontan procedure heart assist device hydraulic conductivity mechanical stress performance measurement system prediction priority journal pump design LA - English M3 - Article N1 - L360067714 2010-12-08 2010-12-15 PY - 2010 SN - 0160-564X 1525-1594 SP - 1039-1044 ST - Filament support spindle for an intravascular cavopulmonary assist device T2 - Artificial Organs TI - Filament support spindle for an intravascular cavopulmonary assist device UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L360067714 http://dx.doi.org/10.1111/j.1525-1594.2010.01139.x VL - 34 ID - 1133 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 16 mm Hg of pressure rise for flow rates of 1 to 4 L/min at 4000 to 7000 rpm. The difference between the CFD predictions and experimental results was found to be approximately 9.8%. Scalar stress levels remained below 570 Pa with exposure times on the order of 1.5 s. 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. © 2010, the Authors. Artificial Organs © 2010, International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc. AD - BioCirc Research Laboratory, Department of Mechanical Engineering, Virginia Commonwealth University, Richmond, VA, United States AU - Throckmorton, A. L. AU - Kapadia, J. Y. AU - Wittenschlaeger, T. M. AU - Medina, T. J. AU - Hoang, H. Q. AU - Bhavsar, S. S. DB - Scopus DO - 10.1111/j.1525-1594.2010.01139.x IS - 11 KW - Artificial right ventricle Cavopulmonary assist device Fontan completion Heart pump Intravascular blood pump Mechanical cavopulmonary assist Pediatric circulatory support Single ventricle physiology Ventricular assist device M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2010 SP - 1039-1044 ST - Filament support spindle for an intravascular cavopulmonary assist device T2 - Artificial Organs TI - Filament support spindle for an intravascular cavopulmonary assist device UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-78649557585&doi=10.1111%2fj.1525-1594.2010.01139.x&partnerID=40&md5=d424a507816202ccf357ec200e7f423b VL - 34 ID - 2143 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. © 2009 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies. AD - N. Tokuhira, Intensive Care Unit, Osaka City General Hospital, Miyakojima-ku, Osaka, Japan AU - Tokuhira, N. AU - Atagi, K. AU - Shimaoka, H. AU - Ujiro, A. AU - Otsuka, Y. AU - Ramsay, M. DB - Embase Medline DO - 10.1097/PCC.0b013e31819a3a3e IS - 2 KW - dexmedetomidine propofol arterial carbon dioxide tension article artificial heart pacemaker bradycardia child clinical article congenital heart malformation controlled study drug efficacy female Fontan procedure heart output hemodynamics hospitalized child human intensive care unit lung vascular resistance male medical record review pediatric anesthesia postoperative analgesia postoperative pain postoperative period preschool child priority journal respiration depression retrospective study school child sedation treatment outcome LA - English M3 - Article N1 - L355297650 2009-11-04 PY - 2009 SN - 1529-7535 SP - 207-212 ST - Dexmedetomidine sedation for pediatric post-Fontan procedure patients T2 - Pediatric Critical Care Medicine TI - Dexmedetomidine sedation for pediatric post-Fontan procedure patients UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L355297650 http://dx.doi.org/10.1097/PCC.0b013e31819a3a3e VL - 10 ID - 1188 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 - 105475563. Language: English. Entry Date: 20090515. Revision Date: 20150711. Publication Type: Journal Article AU - Tokuhira, N. AU - Atagi, K. AU - Shimaoka, H. AU - Ujiro, A. AU - Otsuka, Y. AU - Ramsay, M. DB - ccm DO - 10.1097/PCC.0b013e31819a3a3e DP - EBSCOhost IS - 2 KW - Cardiopulmonary Bypass -- In Infancy and Childhood Heart Defects, Congenital -- Surgery Hypnotics and Sedatives -- Therapeutic Use -- In Infancy and Childhood Imidazoles -- Therapeutic Use -- In Infancy and Childhood Postoperative Care -- In Infancy and Childhood Bradycardia Child Child, Preschool Descriptive Statistics Infant Pacemaker, Artificial -- Utilization Respiration Disorders Retrospective Design Surgery, Cardiovascular T-Tests Treatment Outcomes Vascular Resistance Human N1 - research; tables/charts. Journal Subset: Biomedical; Peer Reviewed; USA. NLM UID: 100954653. PMID: NLM19188869. PY - 2009 SN - 1529-7535 SP - 207-212 ST - Dexmedetomidine sedation for pediatric post-Fontan procedure patients T2 - Pediatric Critical Care Medicine TI - Dexmedetomidine sedation for pediatric post-Fontan procedure patients UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105475563&site=ehost-live&scope=site VL - 10 ID - 1567 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. © 2009 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies. AD - Intensive Care Unit, Osaka City General Hospital, Miyakojima-ku, Osaka, Japan Department of Intensive Care Medicine, Hyogo Medical University, Wakayama, Japan Department of Anesthesiology and Pain Management, Medical Center, Baylor University, Dallas, TX, United States AU - Tokuhira, N. AU - Atagi, K. AU - Shimaoka, H. AU - Ujiro, A. AU - Otsuka, Y. AU - Ramsay, M. DB - Scopus DO - 10.1097/PCC.0b013e31819a3a3e IS - 2 KW - Alpha-2 agonists Congenital heart surgery Dexmedetomidine Fontan procedure Hemodynamics Pediatric critical care Postoperative sedation Pulmonary vascular resistance M3 - Article N1 - Cited By :32 Export Date: 15 June 2020 PY - 2009 SP - 207-212 ST - Dexmedetomidine sedation for pediatric post-Fontan procedure patients T2 - Pediatric Critical Care Medicine TI - Dexmedetomidine sedation for pediatric post-Fontan procedure patients UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-68849118812&doi=10.1097%2fPCC.0b013e31819a3a3e&partnerID=40&md5=cf4ab4aa4ef4afb19d80e668ddafa921 VL - 10 ID - 2182 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. © 2020 The Author(s). AD - 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, 06720, Mexico Facultad de Psicología, Universidad Autónoma de Nuevo León, Col. Mitras Centro, Dr. Carlos Canseco, 110, Esq. Dr. Aguirre Pequeño, Monterrey, 64460, Mexico College of Education and Human Development, University of Louisville, 1905S1st St, Louisville, KY 40208, United States Comisión Nacional de Arbitraje Médico, Mitla No. 250, esq. Axis 5 South (Eugenia). Col. Narvarte, Benito Juárez, Mexico City, 03020, Mexico AU - Toledano-Toledano, F. AU - Moral De La Rubia, J. AU - McCubbin, L. D. AU - Cauley, B. AU - Luna, D. C7 - 104 DB - Scopus DO - 10.1186/s12955-020-01357-5 IS - 1 KW - Anxiety Coping Depression Family caregivers Mexico Psychometrics M3 - Article N1 - Export Date: 15 June 2020 PY - 2020 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85083755214&doi=10.1186%2fs12955-020-01357-5&partnerID=40&md5=9b7fe9ca1a7e156d7ec41681aa3209d1 VL - 18 ID - 1702 ER - TY - JOUR 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 - Department of Pharmacy Practice, Arnold and Marie Schwartz College of Pharmacy and Health Sciences, Long Island University, Brooklyn, NY, United States Pediatrics Children's Hospital at Montefiore, Bronx, NY, United States AU - Tom-Revzon, C. DB - Scopus IS - 3 M3 - Article N1 - Export Date: 15 June 2020 PY - 2007 SP - 52-65 ST - Cardiovascular diseases in children T2 - U.S. Pharmacist TI - Cardiovascular diseases in children UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-47749123469&partnerID=40&md5=4a87c4389aa07cf373c67fc6e0b31cdb VL - 32 ID - 2213 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. © 2015 Wiley Periodicals, Inc. © 2015 Wiley Periodicals, Inc. AD - Department of Pediatrics, Columbia University Medical Center, New York, NY, United States Department of Cardiology, Boston Children's Hospital, Boston, MA, United States Department of Pediatrics, University of California, San Diego, CA, United States Department of Pediatrics, University of Louisville, Louisville, KY, United States Department of Pathology, Brigham and Women's Hospital, Boston, MA, United States Department of Cardiothoracic Surgery, Lucille Packard Children's Hospital Stanford, 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 - Scopus DO - 10.1002/ccd.25897 IS - 3 KW - balloon angioplasty complications congenital heart disease pediatric catheterization/intervention pediatrics percutaneous intervention pulmonary valve disease stent bare metal M3 - Article N1 - Cited By :13 Export Date: 15 June 2020 PY - 2015 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84939428668&doi=10.1002%2fccd.25897&partnerID=40&md5=45b19a1ce2509d6f7a57e89be54391b9 VL - 86 ID - 1975 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. AU - Tran, N. N. AU - Kumar, S. R. AU - Hodge, F. S. AU - Macey, P. M. DB - Medline DO - 10.4037/ajcc2018672 IS - 5 KW - oxygen brain case control study complication congenital heart malformation cross-sectional study developmental disorder female homeostasis human hypoxia male metabolism near infrared spectroscopy neurologic examination newborn pathophysiology physiology prospective study LA - English M3 - Article N1 - L628330123 2019-07-05 2019-10-14 PY - 2018 SN - 1937-710X SP - 410-416 ST - Cerebral Autoregulation in Neonates With and Without Congenital Heart Disease T2 - American journal of critical care : an official publication, American Association of Critical-Care Nurses TI - Cerebral Autoregulation in Neonates With and Without Congenital Heart Disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L628330123 http://dx.doi.org/10.4037/ajcc2018672 VL - 27 ID - 669 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. AD - an associate professor, School of Nursing, University of California, Los Angeles. AN - 131514630. Language: English. Entry Date: 20180904. Revision Date: 20181127. Publication Type: Article AU - Tran, Nhu N. AU - Kumar, S. Ram AU - Hodge, Felicia S. AU - Macey, Paul M. DB - ccm DO - 10.4037/ajcc2018672 DP - EBSCOhost IS - 5 KW - Cognition Infant Development Heart Defects, Congenital Brain -- Physiology Human Infant, Newborn Scales Regression Prospective Studies Cross Sectional Studies California Male Female Chi Square Test Multiple Linear Regression Oxygenation N1 - research; tables/charts. Journal Subset: Blind Peer Reviewed; Core Nursing; Expert Peer Reviewed; Nursing; Peer Reviewed; USA. Instrumentation: Einstein Neonatal Neurobehavioral Assessment Scale (ENNAS). NLM UID: 9211547. PY - 2018 SN - 1062-3264 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 - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=131514630&site=ehost-live&scope=site VL - 27 ID - 1441 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. © 2011 American Society of Mechanical Engineers. AD - G. Troianowski, Institute for Computational and Mathematical Engineering, Stanford University, Stanford, CA 94305, United States AU - Troianowski, G. AU - Taylor, C. A. AU - Feinstein, J. A. AU - Vignon-Clementel, I. E. DB - Embase Medline DO - 10.1115/1.4005377 IS - 11 KW - anastomosis artery blood flow article catheterization child computational fluid dynamics Fontan procedure Glenn shunt cardiac index hemodynamics human laminar flow lung blood flow nuclear magnetic resonance imaging oscillatory potential outcome assessment power analysis preschool child pressure pulmonary artery sensitivity analysis shear stress simulation superior cava vein three-dimensional imaging LA - English M3 - Article N1 - L363045883 2011-12-14 2011-12-16 PY - 2011 SN - 0148-0731 1528-8951 ST - Three-dimensional simulations in glenn patients: Clinically based boundary conditions, hemodynamic results and sensitivity to input data T2 - Journal of Biomechanical Engineering TI - Three-dimensional simulations in glenn patients: Clinically based boundary conditions, hemodynamic results and sensitivity to input data UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L363045883 http://dx.doi.org/10.1115/1.4005377 VL - 133 ID - 1075 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. © 2011 American Society of Mechanical Engineers. AD - Institute for Computational and Mathematical Engineering, Stanford University, Stanford, CA 94305, United States Bioengineering Department, Stanford University, Stanford, CA 94305, United States Surgery Department, Stanford University, Stanford, CA 94305, United States Pediatrics Department, Stanford University, Stanford, CA 94305, United States INRIA Paris-Rocquencourt, BP 105, 78153 Le Chesnay Cedex, France AU - Troianowski, G. AU - Taylor, C. A. AU - Feinstein, J. A. AU - Vignon-Clementel, I. E. C7 - 111006 DB - Scopus DO - 10.1115/1.4005377 IS - 11 KW - boundary conditions clinical data congenital heart disease numerical simulations sensitivity analysis M3 - Article N1 - Cited By :35 Export Date: 15 June 2020 PY - 2011 ST - Three-dimensional simulations in glenn patients: Clinically based boundary conditions, hemodynamic results and sensitivity to input data T2 - Journal of Biomechanical Engineering TI - Three-dimensional simulations in glenn patients: Clinically based boundary conditions, hemodynamic results and sensitivity to input data UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-82955195078&doi=10.1115%2f1.4005377&partnerID=40&md5=253090aeb3bd7f26a9a298ad73f535c4 VL - 133 ID - 2105 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 - Division of Pediatric Cardiology, Dept. of Pediat., Sch. of Med., U., Westwood, CA, United States Div. of Resp. and Critical Care, Dept. of Pediat., Harbor-UCLA M., Torrance, CA, United States AU - Troutman, W. B. AU - Barstow, T. J. AU - Galindo, A. J. AU - Cooper, D. M. DB - Scopus DO - 10.1016/S0735-1097(97)00545-7 IS - 3 M3 - Article N1 - Cited By :70 Export Date: 15 June 2020 PY - 1998 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0032030905&doi=10.1016%2fS0735-1097%2897%2900545-7&partnerID=40&md5=26b6a4d201e8e5f2ef9af9a69b1c60fa VL - 31 ID - 2289 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 - D. Balaguru, University of Texas Health Science Center at Houston, 6410 Fannin St, Houston, TX, United States AU - Turiy, Y. AU - Douglas, W. AU - Balaguru, D. DB - Embase Medline DO - 10.1016/j.athoracsur.2015.01.077 IS - 6 KW - Blalock Taussig shunt prostaglandin anastomosis aortic valve disease aortic valve atresia artery surgery article assisted circulation balloon dilatation balloon valvuloplasty catheter Blalock Taussig shunt stenosis brachiocephalic trunk cardioplegia cardiopulmonary bypass case report cause of death coronary artery anomaly coronary artery circumflex branch coronary stent Damus Kaye Stansel anastomosis echocardiography extracorporeal oxygenation heart catheterization heart failure heart murmur heart right ventricle function human hypoplastic left heart syndrome infant intensive care unit left anterior descending coronary artery low birth weight metabolic acidosis mitral valve atresia mixed infection Norwood procedure oxygen desaturation politef implant postoperative period priority journal pulmonary artery right coronary artery sepsis shunt occlusion ST segment elevation weaning Tyshak Mini balloon Veriflex MR stent LA - English M3 - Article N1 - L611183656 2016-07-21 2016-08-05 PY - 2015 SN - 1552-6259 0003-4975 SP - 2346-2348 ST - Anomalous Origin of Coronary Artery From Main Pulmonary Artery in Hypoplastic Left Heart Syndrome T2 - Annals of Thoracic Surgery TI - Anomalous Origin of Coronary Artery From Main Pulmonary Artery in Hypoplastic Left Heart Syndrome UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L611183656 http://dx.doi.org/10.1016/j.athoracsur.2015.01.077 VL - 100 ID - 898 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. © 2015 The Society of Thoracic Surgeons AD - Divisions of Pediatric Cardiology and Pediatric Cardiovascular Surgery, University of Texas Health Science Center, Houston, Texas, United States AU - Turiy, Y. AU - Douglas, W. AU - Balaguru, D. DB - Scopus DO - 10.1016/j.athoracsur.2015.01.077 IS - 6 M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2015 SP - 2346-2348 ST - Anomalous Origin of Coronary Artery From Main Pulmonary Artery in Hypoplastic Left Heart Syndrome T2 - Annals of Thoracic Surgery TI - Anomalous Origin of Coronary Artery From Main Pulmonary Artery in Hypoplastic Left Heart Syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84961960856&doi=10.1016%2fj.athoracsur.2015.01.077&partnerID=40&md5=f53d85024c020b49df399ea72fbd1313 VL - 100 ID - 1968 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 - Division of Cardiothoracic Surgery, The Children's Hospital of Wisconsin, 9000 W. Wisconsin Ave., Milwaukee, WI 53226, United States Department of Surgery, The Medical College of Winconsin, Milwaukee, WI, United States AU - Tweddell, J. S. AU - Hoffman, G. M. DB - Scopus DO - 10.1053/pcsu.2002.31499 IS - 1 KW - Cardiac output Congenital Heart defects Oxygen delivery Postoperative management Systematic vascular resistance M3 - Article N1 - Cited By :49 Export Date: 15 June 2020 PY - 2002 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0742296534&doi=10.1053%2fpcsu.2002.31499&partnerID=40&md5=183413e8d9dc21a9e757a0e58015e64f VL - 5 ID - 2268 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 - S.P. Newman, Centre for Health Services Research, School of Health Sciences, City University London, Northampton Square, London, United Kingdom 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. DB - Embase Medline DO - 10.1017/S1047951116001396 IS - 5 KW - adolescent adult age aged anxiety disorder article attention Center for Edpidemiological Studies Short Depression Scale clinical decision making cognition cognitive defect congestive cardiomyopathy controlled study cross-sectional study depression disease association disease classification education executive function Fallot tetralogy female great vessels transposition heart single ventricle human intelligence quotient major clinical study male memory mood motor performance neuropsychological test Positive and Negative Affect Schedule problem solving questionnaire Rey auditory verbal learning test scoring system State Trait Anxiety Inventory verbalization Wechsler adult intelligence scale working memory LA - English M3 - Article N1 - L612827731 2016-10-25 2017-06-12 PY - 2017 SN - 1467-1107 1047-9511 SP - 851-859 ST - Cognitive dysfunction in adult CHD with different structural complexity T2 - Cardiology in the Young TI - Cognitive dysfunction in adult CHD with different structural complexity UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L612827731 http://dx.doi.org/10.1017/S1047951116001396 VL - 27 ID - 739 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 - Centre for Health Services Research, School of Health Sciences, City University London, London, United Kingdom GUCH Unit, The Heart Hospital, University College of London Hospitals NHS Foundation Trust, London, United Kingdom Division of Cardiovascular Sciences, University College London (UCL), London, United Kingdom AN - 123303350. Language: English. Entry Date: 20180123. Revision Date: 20190516. Publication Type: journal article AU - Tyagi, Manavi AU - Fteropoulli, Theodora AU - Hurt, Catherine S. AU - Hirani, Shashivadan P. AU - Rixon, Lorna AU - Davies, Anna AU - Picaut, Nathalie AU - Kennedy, Fiona AU - Deanfield, John AU - Cullen, Shay AU - Newman, Stanton P. DB - ccm DO - 10.1017/S1047951116001396 DP - EBSCOhost IS - 5 KW - Heart Defects, Congenital -- Complications Heart Defects, Congenital -- Classification Anxiety -- Psychosocial Factors Depression -- Psychosocial Factors Young Adult Female Adolescence Male Psychological Tests Adult Aged Cross Sectional Studies Executive Function Attention Neuropsychological Tests Middle Age Cognition Self Report England Funding Source Human N1 - research; tables/charts. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Grant Information: MR/K006584/1//Medical Research Council/United Kingdom. NLM UID: 9200019. PMID: NLM27751192. PY - 2017 SN - 1047-9511 SP - 851-859 ST - Cognitive dysfunction in adult CHD with different structural complexity T2 - Cardiology in the Young TI - Cognitive dysfunction in adult CHD with different structural complexity UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=123303350&site=ehost-live&scope=site VL - 27 ID - 1499 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. © Cambridge University Press 2016. AD - Centre for Health Services Research, School of Health Sciences, City University London, Northampton Square, London, ECIV 0HB, United Kingdom GUCH Unit, Heart Hospital, University College of London Hospitals NHS Foundation Trust, London, United Kingdom Division of Cardiovascular Sciences, University College London (UCL), London, United Kingdom 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. DB - Scopus DO - 10.1017/S1047951116001396 IS - 5 KW - Adult CHD cognition mood neuropsychological assessment M3 - Article N1 - Cited By :10 Export Date: 15 June 2020 PY - 2017 SP - 851-859 ST - Cognitive dysfunction in adult CHD with different structural complexity T2 - Cardiology in the Young TI - Cognitive dysfunction in adult CHD with different structural complexity UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84991658453&doi=10.1017%2fS1047951116001396&partnerID=40&md5=deef9ecdbff5ae54c8ee681bc84a645a VL - 27 ID - 1854 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 - Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital of Schleswig-Holstein, Campus Kiel, Arnold-Heller-Strasse 3, Haus 9, 24105 Kiel, Germany Department of Thoracic and Cardiovascular Surgery, 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 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 - Scopus DO - 10.1016/j.jtcvs.2011.01.036 IS - 3 M3 - Article N1 - Cited By :27 Export Date: 15 June 2020 PY - 2011 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-80051796007&doi=10.1016%2fj.jtcvs.2011.01.036&partnerID=40&md5=3f5850d87d5e5ab47ed7dc1017da9fb7 VL - 142 ID - 2120 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. © 2017 The Japanese Orthopaedic Association AD - Department of Orthopaedic Surgery, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano, 390-8621, Japan Department of Pediatrics, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano, 390-8621, 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 - Scopus DO - 10.1016/j.jos.2017.11.004 IS - 2 M3 - Article N1 - Export Date: 15 June 2020 PY - 2018 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85043364016&doi=10.1016%2fj.jos.2017.11.004&partnerID=40&md5=019daade61ab069cb6ac59f9ef975f9e VL - 23 ID - 1818 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 - Departments of Pediatrics, Surgery and Bioengineering, Penn State Hershey College of Medicine, 500 University Drive, Hershey, PA 17033, United States Department of Pharmacology, Pennsylvania State University, College of Medicine, Hershey, PA 17033, United States Department of Surgery, Pennsylvania State University, College of Medicine, Hershey, PA 17033, United States Department of Public Health Sciences, Pennsylvania State University, College of Medicine, Hershey, PA 17033, United States Department of Bioengineering, Pennsylvania State University, College of Medicine, 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 - Scopus DO - 10.1203/PDR.0b013e3181dceef5 IS - 6 M3 - Article N1 - Cited By :15 Export Date: 15 June 2020 PY - 2010 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-77952548696&doi=10.1203%2fPDR.0b013e3181dceef5&partnerID=40&md5=829d4b92dd5481fc49bcf085e398d583 VL - 67 ID - 2151 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, United States AU - Uzark, K. DB - Scopus IS - 3 M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 1984 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0021629640&partnerID=40&md5=8e3f8c032ae03e2a4b77511966b98293 VL - 19 ID - 2331 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. © 2019 Elsevier Inc. AD - Department of Pediatrics, University of Michigan Mott Children's Hospital, Ann Arbor, MI, United States Department of Cardiac Surgery, University of Michigan Mott Children's Hospital, 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 - Scopus DO - 10.1016/j.jpeds.2019.04.060 KW - adult congenital heart disease healthcare transition patient education quality of life self-management M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2019 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85066849635&doi=10.1016%2fj.jpeds.2019.04.060&partnerID=40&md5=0d515d783e8ad3f01c5ec93212df54fd VL - 212 ID - 1735 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 - K. Uzark, University of Michigan Mott Children's Hospital, 1500 E Medical Center Dr, Ann Arbor, MI, 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. DB - Embase Medline DO - 10.1016/j.jpeds.2015.11.016 KW - NCT00132782 Fontan procedure adolescent adult article Child Health Questionnaire cohort analysis comparative study controlled clinical trial controlled study cross-sectional study emotion female follow up heart single ventricle human major clinical study male multicenter study Pediatric Quality of Life Inventory physical activity physical performance priority journal quality of life quality of life assessment self report Short Form 36 social interaction social psychology survival young adult LA - English M3 - Article N1 - L608874113 2016-06-16 2019-10-31 PY - 2016 SN - 1097-6833 0022-3476 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 - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L608874113 http://dx.doi.org/10.1016/j.jpeds.2015.11.016 VL - 170 ID - 819 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 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 - 113214059. Corporate Author: Pediatric Heart Network Investigators. Language: English. Entry Date: 20160715. Revision Date: 20190308. Publication Type: journal article AU - Uzark, Karen AU - Zak, Victor AU - Shrader, Peter AU - McCrindle, Brian W. AU - Radojewski, Elizabeth AU - Varni, James W. AU - Daniels, Kaitlyn AU - Handisides, Jill AU - Hill, Kevin D. AU - Lambert, Linda M. AU - Margossian, Renee AU - Pemberton, Victoria L. AU - Lai, Wyman W. AU - Atz, Andrew M. DB - ccm DO - 10.1016/j.jpeds.2015.11.016 DP - EBSCOhost KW - Quality of Life Cardiopulmonary Bypass -- Psychosocial Factors Young Adult Male Prospective Studies Adult Human Adolescence United States Survivors Cross Sectional Studies Female Age Factors Validation Studies Comparative Studies Evaluation Research Multicenter Studies Funding Source Questionnaires Clinical Assessment Tools N1 - research. Journal Subset: Biomedical; Peer Reviewed; USA. Instrumentation: Child Health Questionnaire (CHQ); Pediatric Quality of Life Inventory (PEDSQL); Short Form Health Survey. Grant Information: HL068269/HL/NHLBI NIH HHS/United States. NLM UID: 0375410. PMID: NLM26685073. PY - 2016 SN - 0022-3476 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 - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=113214059&site=ehost-live&scope=site VL - 170 ID - 1547 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. (PsycINFO Database Record (c) 2016 APA, all rights reserved) AD - Uzark, Karen, University of Michigan Mott Children’s Hospital, L2110 Women’s, 1500 E Medical Center Dr, Ann Arbor, MI, US, 48109-5202 AN - 2016-11525-036 AU - Uzark, Karen AU - Zak, Victor AU - Shrader, Peter AU - McCrindle, Brian W. AU - Radojewski, Elizabeth AU - Varni, James W. AU - Daniels, Kaitlyn AU - Handisides, Jill AU - Hill, Kevin D. AU - Lambert, Linda M. AU - Margossian, Renee AU - Pemberton, Victoria L. AU - Lai, Wyman W. AU - Atz, Andrew M. DB - psyh DO - 10.1016/j.jpeds.2015.11.016 DP - EBSCOhost KW - quality of life frontal operation single ventricle congenital disorders surgical procedure Heart Ventricles Pediatrics Surgery Surgical Patients Measurement Treatment Outcomes N1 - University of Michigan Mott Children’s Hospital, Ann Arbor, MI, US. Institutional Authors: Pediatric Heart Network Investigators. Release Date: 20160623. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Conference Information: American Heart Association Scientific Sessions, Nov, 2014, Chicago, IL, US. Conference Note: Portions of the study were presented as an abstract and poster at the aforementioned conference. Major Descriptor: Heart Ventricles; Pediatrics; Quality of Life; Surgery; Surgical Patients. Minor Descriptor: Congenital Disorders; Measurement; Treatment Outcomes. Classification: Medical Treatment of Physical Illness (3363). Population: Human (10); Male (30); Female (40). Age Group: Childhood (birth-12 yrs) (100); School Age (6-12 yrs) (180); Adolescence (13-17 yrs) (200); Adulthood (18 yrs & older) (300); Young Adulthood (18-29 yrs) (320). Tests & Measures: Pediatric Quality of Life Inventory; Child Health Questionnaire; 36-Item Short Form Health Survey DOI: 10.1037/t07023-000. Methodology: Empirical Study; Followup Study; Quantitative Study. References Available: Y. Page Count: 7. Issue Publication Date: Mar, 2016. Publication History: Accepted Date: Nov 5, 2015; Revised Date: Oct 12, 2015; First Submitted Date: Jul 2, 2015. Copyright Statement: All rights reserved. Elsevier Inc. 2016. Sponsor: National Heart, Lung, and Blood Institute, US. Grant: HL068269; HL068270; HL068279; HL068281; HL068285; HL068292; HL068290; HL068288. Recipients: No recipient indicated PY - 2016 SN - 0022-3476 SP - 166-172 ST - Assessment of quality of life in young patients with single ventricle after the Fontan operation T2 - The Journal of Pediatrics TI - Assessment of quality of life in young patients with single ventricle after the Fontan operation UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2016-11525-036&site=ehost-live&scope=site ORCID: 0000-0002-4334-700X karenu@med.umich.edu VL - 170 ID - 1663 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. © 2018 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies. AD - Department of Pediatrics, Section of Pediatric Cardiology, University of Michigan C.S. Mott Children's Hospital, Ann Arbor, MI, United States Department of Pediatrics, Section of Pediatric Cardiology, Northwestern University Feinberg School of Medicine, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, United States Division of Pediatric Cardiology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States Department of Cardiology, Section of Pediatric Cardiac Intensive Care, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States 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 - Scopus DO - 10.1097/PCC.0000000000001523 IS - 6 KW - pediatric cardiac intensive care pediatric cardiac surgery pediatric cardiology pediatric intensive care M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2018 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85053865867&doi=10.1097%2fPCC.0000000000001523&partnerID=40&md5=eabb861d6bcf96595c8db3cff918525c VL - 19 ID - 1802 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 - N. Vahsen, Paediatric Cardiology, German Paediatric Heart Centre Sankt Augustin, Arnold-Janssen-Strasse 29, Sankt Augustin, Germany AU - Vahsen, N. AU - Bröder, A. AU - Hraska, V. AU - Schneider, M. DB - Embase Medline DO - 10.1055/s-0043-120526 IS - 1 KW - adolescent adult article behavior brain perfusion cavopulmonary connection child deep hypothermic circulatory arrest depth perception developmental delay early diagnosis early intervention female follow up heart single ventricle human intelligence major clinical study male mental development motor performance nervous system development neuropsychological test quality of life risk factor LA - English M3 - Article N1 - L619906594 2017-12-27 2018-01-30 PY - 2018 SN - 1439-3824 0300-8630 SP - 24-30 ST - Neurodevelopmental Outcome in Children with Single Ventricle after Total Cavopulmonary Connection T2 - Klinische Padiatrie TI - Neurodevelopmental Outcome in Children with Single Ventricle after Total Cavopulmonary Connection UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L619906594 http://dx.doi.org/10.1055/s-0043-120526 VL - 230 ID - 717 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 - Paediatric Cardiology, German Paediatric Heart Centre Sankt Augustin, Arnold-Janssen-Strasse 29, Sankt Augustin, 53757, Germany University of Mannheim, School of Social Sciences, Mannheim, Germany Children's Hospital of Wisconsin, Cardiothoracic Surgery, Milwaukee, United States AU - Vahsen, N. AU - Bröder, A. AU - Hraska, V. AU - Schneider, M. DB - Scopus DO - 10.1055/s-0043-120526 IS - 1 KW - follow-up studies neurodevelopmental outcome quality of life risk factors Total cavopulmonary connection M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2018 SP - 24-30 ST - Neurodevelopmental Outcome in Children with Single Ventricle after Total Cavopulmonary Connection T2 - Klinische Padiatrie TI - Neurodevelopmental Outcome in Children with Single Ventricle after Total Cavopulmonary Connection UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85038638790&doi=10.1055%2fs-0043-120526&partnerID=40&md5=1d95259ad76a2f77ed8e88781b145980 VL - 230 ID - 1830 ER - TY - JOUR AD - Department of Cardiology, Thoraxcenter, Erasmus MC, Rotterdamn, The Netherlands AN - 106571186. Language: English. Entry Date: 20050128. Revision Date: 20150711. Publication Type: Journal Article AU - van den Bosch, A. E. AU - Roos-Hesselink, J. W. AU - van Domburg, R. AU - Bogers, A. J. J. AU - Simoons, M. L. AU - Meijboom, F. J. DB - ccm DP - EBSCOhost IS - 9 KW - Heart Defects, Congenital -- Prognosis Heart Defects, Congenital -- Surgery Heart Surgery -- Standards Adolescence Adult Arrhythmia -- Epidemiology Child Child, Preschool Clinical Assessment Tools Descriptive Statistics Female Health Status Indicators Male Middle Age Mortality -- Epidemiology Quality of Life Reoperation Short Form-36 Health Survey (SF-36) Thromboembolism -- Epidemiology Treatment Outcomes Human N1 - research; tables/charts. Journal Subset: Biomedical; Peer Reviewed; USA. Instrumentation: Short Form-36 Health Survey (SF-36). NLM UID: 0207277. PMID: NLM15110207. 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106571186&site=ehost-live&scope=site VL - 93 ID - 1621 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 - Dept. Cardiol. Cardiothorac. Surg., Thoraxcenter, Erasmus MC, Rotterdam, Netherlands 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 - Scopus DO - 10.1016/j.amjcard.2004.01.041 IS - 9 M3 - Article N1 - Cited By :126 Export Date: 15 June 2020 PY - 2004 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-1942467998&doi=10.1016%2fj.amjcard.2004.01.041&partnerID=40&md5=6e6701a09a688125b34bf88b67922811 VL - 93 ID - 2254 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 - A. Vereczkey, Versys Clinics, Human Reproduction Institute, Budapest, Hungary AU - Vereczkey, A. AU - Gerencsér, B. AU - Czeizel, A. E. AU - Szabó, I. DB - Embase Medline DO - 10.1016/j.ejogrb.2014.08.022 KW - carbamazepine insulin phenytoin valproic acid adult aortic coarctation aortic valve stenosis article congenital malformation cardiovascular risk chronic disease congenital heart malformation controlled study disease association Ebstein anomaly endocardial cushion defect environmental factor epilepsy essential hypertension Fallot tetralogy female great vessels transposition heart arrhythmia heart atrium septum defect heart right ventricle double outlet heart single ventricle heart ventricle septum defect human human tissue hypoplastic left heart syndrome insulin dependent diabetes mellitus major clinical study maternal diabetes mellitus maternal disease maternal hypertension migraine newborn non insulin dependent diabetes mellitus panic paroxysmal supraventricular tachycardia patent ductus arteriosus population based case control study priority journal pulmonary artery malformation pulmonary valve atresia pulmonary valve stenosis risk assessment tricuspid valve atresia tricuspid valve stenosis LA - English M3 - Article N1 - L601179942 2015-01-16 2019-05-14 PY - 2014 SN - 1872-7654 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 - European Journal of Obstetrics and Gynecology and Reproductive Biology TI - Association of certain chronic maternal diseases with the risk of specific congenital heart defects: A population-based study UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L601179942 http://dx.doi.org/10.1016/j.ejogrb.2014.08.022 VL - 182 ID - 908 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 - Intensive Care, Erasmus MC-Sophia Children's Hospital, P.O. Box 2060, Rotterdam 3000 CB, Netherlands Department of Pediatrics, Maasstad Hospital, Rotterdam, Netherlands Department of Pediatrics, Erasmus MC, Sophia Children's Hospital, Rotterdam, Netherlands Department of Pediatrics, Ghent University Hospital, Ghent, Belgium Department of Epidemiology and Biostatistics, Erasmus MC, Rotterdam, Netherlands Department of Cardiothoracic Surgery, Erasmus MC, Rotterdam, Netherlands Department of Pediatrics, Division of Pediatric Cardiology, Erasmus MC-Sophia Children's Hospital, Rotterdam, 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 - Scopus DO - 10.1007/s00246-010-9829-z IS - 2 KW - Cardiac surgery Child Critical illness Glucocorticoids Hyperglycemia Insulin M3 - Article N1 - Cited By :20 Export Date: 15 June 2020 PY - 2011 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-79952311424&doi=10.1007%2fs00246-010-9829-z&partnerID=40&md5=37926412cc610ce71d97f3add1a83f9d VL - 32 ID - 2133 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 - V.L. Vida, Pediatric and Congenital Cardiac Surgery Unit, Department of Thoracic, Cardiac and Vascular Sciences, University of Padua, Via Giustiniani, 2, Padua, Italy AU - Vida, V. L. AU - Guariento, A. AU - Zucchetta, F. AU - Padalino, M. A. AU - Milanesi, O. AU - Maschietto, N. AU - Stellin, G. DB - Embase Medline DO - 10.1111/jocs.12595 IS - 9 KW - article balloon dilatation child combining surgical and interventional technique congenital heart disease Fallot tetralogy follow up heart single ventricle heart surgery heart ventricle septum defect human infant interventional cardiovascular procedure major clinical study operating room preschool child pulmonary artery pulmonary artery stenosis pulmonary valve respiratory distress septal occluder transatrial balloon dilation transinfundibular balloon dilation LA - English M3 - Article N1 - L605888069 2015-09-09 2015-09-10 PY - 2015 SN - 1540-8191 0886-0440 SP - 719-723 ST - Combined surgical and interventional approaches for treating patients with congenital heart disease T2 - Journal of Cardiac Surgery TI - Combined surgical and interventional approaches for treating patients with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L605888069 http://dx.doi.org/10.1111/jocs.12595 VL - 30 ID - 892 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. AN - 109640656. Language: English. Entry Date: 20150923. Revision Date: 20160831. Publication Type: journal article. Journal Subset: Biomedical AU - Vida, Vladimiro L. AU - Guariento, Alvise AU - Zucchetta, Fabio AU - Padalino, Massimo A. AU - Milanesi, Ornella AU - Maschietto, Nicola AU - Stellin, Giovanni DB - ccm DO - 10.1111/jocs.12595 DP - EBSCOhost IS - 9 N1 - Peer Reviewed; USA. Special Interest: Perioperative Care. NLM UID: 8908809. PMID: NLM26174169. PY - 2015 SN - 0886-0440 SP - 719-723 ST - Combined Surgical and Interventional Approaches for Treating Patients with Congenital Heart Disease T2 - Journal of Cardiac Surgery TI - Combined Surgical and Interventional Approaches for Treating Patients with Congenital Heart Disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=109640656&site=ehost-live&scope=site VL - 30 ID - 1516 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. © 2015 Wiley Periodicals, Inc. AD - Pediatric and Congenital Cardiac Surgery Unit, Department of Thoracic, Cardiac and Vascular Sciences, University of Padua, Via Giustiniani, 2, Padua, 35100, Italy Pediatric Cardiology Unit, Department of Paediatrics, University of Padua, Padua, Italy AU - Vida, V. L. AU - Guariento, A. AU - Zucchetta, F. AU - Padalino, M. A. AU - Milanesi, O. AU - Maschietto, N. AU - Stellin, G. DB - Scopus DO - 10.1111/jocs.12595 IS - 9 M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2015 SP - 719-723 ST - Combined surgical and interventional approaches for treating patients with congenital heart disease T2 - Journal of Cardiac Surgery TI - Combined surgical and interventional approaches for treating patients with congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84940772225&doi=10.1111%2fjocs.12595&partnerID=40&md5=9b9d43bfa158ae3bd72115fd9e71da3d VL - 30 ID - 1976 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 - Child Development Center, University Children's Hospital of Zurich, Steinwiesstrasse 75, Zurich CH-8032, Switzerland Department of Pediatric Neurology, University Children's Hospital, Mainz, Germany Department of Pediatric Cardiology, University Children's Hospital, Zurich, Switzerland Department of Psychosomatics and Psychiatry, University Children's Hospital, Zurich, Switzerland Center for Integrative Human Physiology, University of Zurich, Zurich, Switzerland AU - Von Rhein, M. AU - Dimitropoulos, A. AU - Valsangiacomo Buechel, E. R. AU - Landolt, M. A. AU - Latal, B. DB - Scopus DO - 10.1016/j.jtcvs.2012.02.005 IS - 3 M3 - Article N1 - Cited By :24 Export Date: 15 June 2020 PY - 2012 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84865699411&doi=10.1016%2fj.jtcvs.2012.02.005&partnerID=40&md5=ed1337efa16668d15406d93e8994cef3 VL - 144 ID - 2085 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 - Department of Pediatric Cardiac Surgery, Sick Children's Hospital and University Paris Descartes, Paris, France AU - Vouhé, P. R. DB - Scopus DO - 10.1053/j.semtcvs.2011.09.003 IS - 3 KW - Adult cardiac surgery Congenital heart disease M3 - Article N1 - Cited By :8 Export Date: 15 June 2020 PY - 2011 SP - 209-215 ST - Adult congenital surgery: Current management T2 - Seminars in Thoracic and Cardiovascular Surgery TI - Adult congenital surgery: Current management UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-83455200844&doi=10.1053%2fj.semtcvs.2011.09.003&partnerID=40&md5=34e3099321c4bf80804097902097d39b VL - 23 ID - 2104 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, P.O. Box 9600, 2300 RC Leiden, The Netherlands. AN - 105254967. Language: English. Entry Date: 20100312. Revision Date: 20150711. Publication Type: Journal Article AU - Vrijmoet-Wiersma, C. M. AU - Ottenkamp, J. AU - van Roozendaal, M. AU - Grootenhuis, M. A. AU - Koopman, H. M. DB - ccm DO - 10.1017/S1047951109991831 DP - EBSCOhost IS - 6 KW - Heart Defects, Congenital -- Psychosocial Factors Parents -- Psychosocial Factors Stress, Psychological -- Etiology Adolescence Adult Analysis of Variance Child Child, Preschool Cross Sectional Studies Disease Susceptibility Female Heart Defects, Congenital -- Surgery Human Infant Male Middle Age Questionnaires Risk Factors N1 - research. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Instrumentation: General Health Questionnaire (GHQ). NLM UID: 9200019. PMID: NLM19825253. PY - 2009 SN - 1047-9511 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105254967&site=ehost-live&scope=site VL - 19 ID - 1553 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 - Pediatric Department, Leiden University Medical Center, P.O. Box 9600, 2300 RC Leiden, Netherlands CAHAL, Center for Congenital Anomalies Heart Amsterdam/Leiden, P.O. Box 9600, 2300 RC Leiden, Netherlands Medical Psychology, Leiden University Medical Center, P.O. Box 9600, 2300 RC Leiden, Netherlands Psychosocial Department, Academic Medical Center, Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, Netherlands AU - Vrijmoet-Wiersma, C. M. J. AU - Ottenkamp, J. AU - Van Roozendaal, M. AU - Grootenhuis, M. A. AU - Koopman, H. M. DB - Scopus DO - 10.1017/S1047951109991831 IS - 6 KW - Children Congenitally malformed hearts Overprotection Psychological problems M3 - Article N1 - Cited By :44 Export Date: 15 June 2020 PY - 2009 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-76349087141&doi=10.1017%2fS1047951109991831&partnerID=40&md5=82542d39c433ed8e7f2b194978bd77cb VL - 19 ID - 2169 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 - 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 - Scopus DO - 10.1016/S0031-3955(05)70125-5 IS - 2 M3 - Article N1 - Cited By :25 Export Date: 15 June 2020 PY - 1999 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0032946516&doi=10.1016%2fS0031-3955%2805%2970125-5&partnerID=40&md5=32da489f77685dda9a70523fa3a304bf VL - 46 ID - 2285 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 Cardiology, Labatt Family Heart Centre, Hospital for Sick Children, Toronto, ON, Canada Department of Paediatrics, Hospital for Sick Children, University of Toronto School of Medicine, Toronto, ON, Canada Cardiovascular Surgery, Labatt Family Heart Centre, Hospital for Sick Children, Toronto, ON, Canada Department of Surgery, Hospital for Sick Children, University of Toronto School of Medicine, Toronto, ON, Canada Cardiac Critical Care Medicine, Labatt Family Heart Centre, Hospital for Sick Children, Toronto, ON, Canada Department of Critical Care Medicine, Hospital for Sick Children, University of Toronto School of Medicine, Toronto, ON, Canada Division of Cardiac Critical Care Medicine, Hospital for Sick Children, 555 University Ave, Toronto, ON M5G 1X8, Canada 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 - Scopus DO - 10.1136/hrt.2008.156612 IS - 15 M3 - Article N1 - Cited By :29 Export Date: 15 June 2020 PY - 2009 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-67650717433&doi=10.1136%2fhrt.2008.156612&partnerID=40&md5=41d8ec5d8c3166fd5c986ce3ac65e8c4 VL - 95 ID - 2174 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. ©2006Amercian Society of Artificial Internal Organs. AD - Department of Mechanical Engineering, University of Colorado, Boulder, CO, United States Department of Pediatric Cardiology, University of Colorado Health Sciences Center, Denver, CO, United States Cardiothoracic Surgery, Children's Hospital, Denver, CO, United States Cardiovascular Flow Dynamics Research Laboratory, Division of Cardiology, Children's Hospital/University of Colorado Health Sciences Center, Denver, CO 80218, United States 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. DB - Scopus DO - 10.1097/01.mat.0000249038.69048.3c IS - 6 M3 - Article N1 - Cited By :17 Export Date: 15 June 2020 PY - 2006 SP - 682-692 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 Journal TI - Initial experience with the development and numerical and in vitro studies of a novel low-pressure artificial right ventricle for pediatric fontan patients UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-33751212454&doi=10.1097%2f01.mat.0000249038.69048.3c&partnerID=40&md5=6d56b75f4656c2b8d4e1ef44a80b8f88 VL - 52 ID - 2221 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. © 2013 Mosby Inc. All rights reserved. AD - Y. Wang, Congenital Malformations Registry, Bureau of Environmental and Occupational Epidemiology, Center for Environmental Health, New York State Department of Health, Empire State Plaza, Albany, NY 12237, United States AU - Wang, Y. AU - Liu, G. AU - Druschel, C. M. AU - Kirby, R. S. DB - Embase Medline DO - 10.1016/j.jpeds.2013.06.084 IS - 5 KW - aortic coarctation article congenital heart disease ethnic difference ethnicity Fallot tetralogy female great vessels transposition human hypoplastic left heart syndrome infant major clinical study male maternal age mortality overall survival phenotype priority journal race difference risk factor sex difference survival rate LA - English M3 - Article N1 - L52717446 2013-08-09 2013-11-13 PY - 2013 SN - 0022-3476 1097-6833 SP - 1437-1442.e2 ST - Maternal race/ethnicity and survival experience of children with congenital heart disease T2 - Journal of Pediatrics TI - Maternal race/ethnicity and survival experience of children with congenital heart disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L52717446 http://dx.doi.org/10.1016/j.jpeds.2013.06.084 VL - 163 ID - 984 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. © 2013 Mosby Inc. All rights reserved. AD - Congenital Malformations Registry, Bureau of Environmental and Occupational Epidemiology, Center for Environmental Health, New York State Department of Health, Empire State Plaza, Albany, NY 12237, United States School of Public Health, State University of New York, Albany, NY, United States Department of Community and Family Health, College of Public Health, University of South Florida, Tampa, FL, United States AU - Wang, Y. AU - Liu, G. AU - Druschel, C. M. AU - Kirby, R. S. DB - Scopus DO - 10.1016/j.jpeds.2013.06.084 IS - 5 M3 - Article N1 - Cited By :24 Export Date: 15 June 2020 PY - 2013 SP - 1437-1442.e2 ST - Maternal race/ethnicity and survival experience of children with congenital heart disease T2 - Journal of Pediatrics TI - Maternal race/ethnicity and survival experience of children with congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84886722718&doi=10.1016%2fj.jpeds.2013.06.084&partnerID=40&md5=ba37f0631bd173f675c3035ba2d5ef3d VL - 163 ID - 2072 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 - Z. Chen, Department of Cardiology, Hunan Children's Hospital, Changsha, Hunan, China AU - Wang, Y. AU - Liu, Q. AU - Deng, X. AU - Xiao, Y. AU - Chen, Z. DB - Embase Medline DO - 10.1097/MD.0000000000014320 IS - 6 KW - ablation catheter electrode catheter adenosine amiodarone angiocardiography article atrioventricular nodal reentry tachycardia case report catheter ablation child clinical article cyanosis digital clubbing electrocardiogram electrophysiological procedures follow up Fontan procedure Glenn shunt heart atrium septum defect heart catheterization heart electrophysiology heart outflow tract obstruction heart palpitation heart ventricle septum defect His bundle electrogram hospitalization human male New York Heart Association class oxygen saturation paroxysmal supraventricular tachycardia pediatric patient physical examination priority journal QRS complex school child thorax pain tricuspid valve atresia LA - English M3 - Article N1 - L629144172 2019-09-04 2019-09-12 PY - 2019 SN - 1536-5964 0025-7974 ST - Atrioventricular reentrant tachycardia in a child with tricuspid atresia: A case report of catheter ablation T2 - Medicine (United States) TI - Atrioventricular reentrant tachycardia in a child with tricuspid atresia: A case report of catheter ablation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L629144172 http://dx.doi.org/10.1097/MD.0000000000014320 VL - 98 ID - 632 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. © 2019 the Author(s). Published by Wolters Kluwer Health, Inc.. AD - Department of Cardiology, Hunan Children's Hospital, Changsha, Hunan, 410007, China Department of Cardiology, Second Xiangya Hospital, Central South University, Changsha, Hunan, China Department of Cardiothoracic Surgery, Hunan Children's Hospital, Changsha, Hunan, China AU - Wang, Y. AU - Liu, Q. AU - Deng, X. AU - Xiao, Y. AU - Chen, Z. C7 - e14320 DB - Scopus DO - 10.1097/MD.0000000000014320 IS - 6 KW - Atrioventricular reentrant tachycardia catheter ablation children tricuspid atresia M3 - Article N1 - Export Date: 15 June 2020 PY - 2019 ST - Atrioventricular reentrant tachycardia in a child with tricuspid atresia: A case report of catheter ablation T2 - Medicine (United States) TI - Atrioventricular reentrant tachycardia in a child with tricuspid atresia: A case report of catheter ablation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85061244918&doi=10.1097%2fMD.0000000000014320&partnerID=40&md5=5e2dd87b3a06936a6f5838673ee1a269 VL - 98 ID - 1763 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. AD - Paediatric and Adolescent Unit, National Heart Hospital, Westmoreland Street, London W1M 8BA, United Kingdom AU - Warnes, C. A. AU - Somerville, J. DB - Scopus DO - 10.1136/hrt.56.6.535 IS - 6 M3 - Article N1 - Cited By :69 Export Date: 15 June 2020 PY - 1986 SP - 535-543 ST - Tricuspid atresia in adolescents and adults: Current state and late complications T2 - Heart TI - Tricuspid atresia in adolescents and adults: Current state and late complications UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0023013918&doi=10.1136%2fhrt.56.6.535&partnerID=40&md5=9d0929e79e0c655e2f7c44986fbdc5f1 VL - 56 ID - 2327 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. © 2018 AD - Graduate Program for Neuroscience, Boston University, Boston, MA, United States Department of Neurology, Boston Children's Hospital, Boston, MA, United States Department of Cardiology, Boston Children's Hospital, Boston, MA, United States Department of Biostatistics, Harvard T.H. Chan School of Public Health, Boston, MA, United States Department of Pediatrics, Harvard Medical School, Boston, MA, United States Department of Psychiatry, Boston Children's Hospital, Boston, MA, United States Department of Psychiatry, Harvard Medical School, Boston, MA, United States Department of Neurology, Harvard Medical School, Boston, MA, United States Department of Radiology, Boston Children's Hospital, Boston, MA, United States AU - Watson, C. G. AU - Stopp, C. AU - Wypij, D. AU - Bellinger, D. C. AU - Newburger, J. W. AU - Rivkin, M. J. DB - Scopus DO - 10.1016/j.jpeds.2018.04.022 KW - brain cognition congenital heart disease DTI Fontan MRI single ventricle M3 - Article N1 - Cited By :8 Export Date: 15 June 2020 PY - 2018 SP - 140-149.e4 ST - Altered White Matter Microstructure Correlates with IQ and Processing Speed in Children and Adolescents Post-Fontan T2 - Journal of Pediatrics TI - Altered White Matter Microstructure Correlates with IQ and Processing Speed in Children and Adolescents Post-Fontan UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85048716878&doi=10.1016%2fj.jpeds.2018.04.022&partnerID=40&md5=17114c625d98ff8d15369938bfefa98c VL - 200 ID - 1787 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. © 2019, Biomedical Engineering Society. AD - Wallace H. Coulter School of Biomedical Engineering, Georgia Institute of Technology, 387 Technology Circle, Suite 232, Atlanta, GA 30313-2412, United States School of Chemistry and Biochemistry, Georgia Institute of Technology, Atlanta, GA, United States Department of Cardiology, Children’s Hospital of Philadelphia, Philadelphia, PA, United States Department of Mathematics, Department of Computer Science, Emory University, Atlanta, GA, United States 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. DB - Scopus DO - 10.1007/s10439-019-02307-z IS - 11 KW - Computational fluid dynamics Fontan hemodynamics Inlet velocity profiles M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2019 SP - 2258-2270 ST - Analysis of Inlet Velocity Profiles in Numerical Assessment of Fontan Hemodynamics T2 - Annals of Biomedical Engineering TI - Analysis of Inlet Velocity Profiles in Numerical Assessment of Fontan Hemodynamics UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85068112331&doi=10.1007%2fs10439-019-02307-z&partnerID=40&md5=49c1742db61a7057eab280689e34e72f VL - 47 ID - 1728 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 AN - 137866234. Language: English. Entry Date: 20001201. Revision Date: 20190803. Publication Type: journal article 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. DB - ccm DP - EBSCOhost IS - 8 KW - Cognition Disorders -- Etiology Cardiopulmonary Bypass -- Adverse Effects Heart Defects, Congenital -- Complications Child Human Intelligence Tests Learning Disorders -- Etiology Educational Measurement Prospective Studies Treatment Outcomes Heart Defects, Congenital -- Surgery Child, Preschool Heart Ventricle -- Abnormalities Multivariate Analysis Adult Adolescence Heart Ventricle -- Surgery Validation Studies Comparative Studies Evaluation Research Multicenter Studies Questionnaires N1 - research. Journal Subset: Biomedical; Peer Reviewed; USA. Instrumentation: General Health Questionnaire (GHQ); Craig Handicap Assessment and Reporting Technique (CHART). NLM UID: 0147763. PMID: NLM10952957. PY - 2000 SN - 0009-7322 SP - 883-889 ST - Cognitive development after the Fontan operation T2 - Circulation TI - Cognitive development after the Fontan operation UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=137866234&site=ehost-live&scope=site VL - 102 ID - 1615 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. © 2007 The Authors. AD - J.D. Tobias, Department of Anesthesiology, Division of Pediatric Anesthesiology, University of Missouri, One Hospital Drive, Columbia, MO 65212, United States AU - Wheeler, A. D. AU - Tobias, J. D. DB - Embase Medline DO - 10.1111/j.1460-9592.2006.02153.x IS - 5 KW - article artificial ventilation case report child chyle chylothorax female Fontan procedure gastroschisis human lung hemodynamics newborn oliguria pediatrics pneumothorax priority journal respiratory distress respiratory function sepsis shock symptom tachycardia tension chylothorax tube LA - English M3 - Article N1 - L46633396 2007-05-01 PY - 2007 SN - 1155-5645 1460-9592 SP - 488-491 ST - Tension chylothorax in two pediatric patients T2 - Paediatric Anaesthesia TI - Tension chylothorax in two pediatric patients UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L46633396 http://dx.doi.org/10.1111/j.1460-9592.2006.02153.x VL - 17 ID - 1238 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. © 2007 The Authors. AD - Department of Anesthesiology, University of Missouri, Columbia, MO, United States Department of Pediatrics, University of Missouri, Columbia, MO, United States Department of Anesthesiology, Division of Pediatric Anesthesiology, University of Missouri, One Hospital Drive, Columbia, MO 65212, United States AU - Wheeler, A. D. AU - Tobias, J. D. DB - Scopus DO - 10.1111/j.1460-9592.2006.02153.x IS - 5 KW - Chyle Chylothorax Pleural effusion M3 - Article N1 - Cited By :6 Export Date: 15 June 2020 PY - 2007 SP - 488-491 ST - Tension chylothorax in two pediatric patients T2 - Paediatric Anaesthesia TI - Tension chylothorax in two pediatric patients UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-34247375531&doi=10.1111%2fj.1460-9592.2006.02153.x&partnerID=40&md5=1dcd8b2483d1886d98822c4c79000f42 VL - 17 ID - 2210 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 - A.C. Gelijns, Intl. Ctr. Hlth. Outcomes/Inno. Res., Columbia University, Harkness Pavillion, 180 Fort Washington Ave, New York, NY 10032, United States 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. DB - Embase Medline IS - 4 I KW - inotropic agent article child child health child hospitalization health care cost heart surgery heart transplantation hospital cost human hypoplastic left heart syndrome infant major clinical study newborn outpatient care priority journal quality of life survival LA - English M3 - Article N1 - L30395469 2000-07-06 PY - 2000 SN - 0022-5223 SP - 720-731 ST - Hypoplastic left heart syndrome: Valuing the survival T2 - Journal of Thoracic and Cardiovascular Surgery TI - Hypoplastic left heart syndrome: Valuing the survival UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L30395469 VL - 119 ID - 1333 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, Departments of Surgery, Medicine Pediatrics, Columbia University, New York, NY, United States 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. DB - Scopus DO - 10.1016/S0022-5223(00)70007-9 IS - 4 M3 - Article N1 - Cited By :87 Export Date: 15 June 2020 PY - 2000 SP - 720-731 ST - Hypoplastic left heart syndrome: Valuing the survival T2 - Journal of Thoracic and Cardiovascular Surgery TI - Hypoplastic left heart syndrome: Valuing the survival UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-0034041268&doi=10.1016%2fS0022-5223%2800%2970007-9&partnerID=40&md5=04e325ded06370fc50c9165f30520619 VL - 119 ID - 2278 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, R2 = 0.13) and FL/BPD at F2 predicted Language score (B = 127, P = 0.03, R2 = 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 - I.A. Williams, 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, United States AU - Williams, I. A. AU - Fifer, W. P. AU - Andrews, H. DB - Embase Medline DO - 10.1007/s00246-015-1132-6 IS - 6 KW - abdominal circumference article Bayley Scales of Infant Development biparietal distance cognition assessment cohort analysis congenital heart disease controlled study disease association Fallot tetralogy female femur fetus fetus growth fetus weight great vessels transposition growth rate head circumference human hypoplastic left heart syndrome language low birth weight major clinical study male nerve cell differentiation newborn pregnancy prospective study LA - English M3 - Article N1 - L602897061 2015-03-17 2015-07-28 PY - 2015 SN - 1432-1971 0172-0643 SP - 1135-1144 ST - Fetal Growth and Neurodevelopmental Outcome in Congenital Heart Disease T2 - Pediatric Cardiology TI - Fetal Growth and Neurodevelopmental Outcome in Congenital Heart Disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L602897061 http://dx.doi.org/10.1007/s00246-015-1132-6 VL - 36 ID - 854 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 - S. Wittekind, Cincinnati Children’s Hospital Medical Center, Heart Institute, 3333 Burnet Avenue, MLC 2003, Cincinnati, OH, United States AU - Wittekind, S. AU - Mays, W. AU - Gerdes, Y. AU - Knecht, S. AU - Hambrook, J. AU - Border, W. AU - Jefferies, J. L. DB - Embase Medline DO - 10.1007/s00246-018-1854-3 IS - 5 KW - carbon dioxide adolescent adult article cardiopulmonary exercise test child clinical article controlled study exercise female Fontan procedure heart rehabilitation heart stroke volume human hypoplastic left heart syndrome lung gas exchange lung ventilation male oxygen consumption prospective study stress echocardiography tricuspid valve atresia LA - English M3 - Article N1 - L621189013 2018-03-15 2018-09-17 PY - 2018 SN - 1432-1971 0172-0643 SP - 1023-1030 ST - A Novel Mechanism for Improved Exercise Performance in Pediatric Fontan Patients After Cardiac Rehabilitation T2 - Pediatric Cardiology TI - A Novel Mechanism for Improved Exercise Performance in Pediatric Fontan Patients After Cardiac Rehabilitation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L621189013 http://dx.doi.org/10.1007/s00246-018-1854-3 VL - 39 ID - 679 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. © 2018, Springer Science+Business Media, LLC, part of Springer Nature. AD - Cincinnati Children’s Hospital Medical Center, Heart Institute, 3333 Burnet Avenue, MLC 2003, Cincinnati, OH 45229-3026, United States Children’s Healthcare of Atlanta, Emory University School of Medicine, Atlanta, GA, United States AU - Wittekind, S. AU - Mays, W. AU - Gerdes, Y. AU - Knecht, S. AU - Hambrook, J. AU - Border, W. AU - Jefferies, J. L. DB - Scopus DO - 10.1007/s00246-018-1854-3 IS - 5 KW - Cardiac rehabilitation Congenital heart disease Exercise testing Exercise therapy Fontan circulation M3 - Article N1 - Cited By :8 Export Date: 15 June 2020 PY - 2018 SP - 1023-1030 ST - A Novel Mechanism for Improved Exercise Performance in Pediatric Fontan Patients After Cardiac Rehabilitation T2 - Pediatric Cardiology TI - A Novel Mechanism for Improved Exercise Performance in Pediatric Fontan Patients After Cardiac Rehabilitation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85043380468&doi=10.1007%2fs00246-018-1854-3&partnerID=40&md5=06b866f661e2656d8c02efbebef15cd0 VL - 39 ID - 1797 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. © 2009 Elsevier Inc. All rights reserved. AD - E.P. Walsh, Department of Pediatrics, Harvard Medical School, Boston, MA, United States 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. DB - Embase Medline DO - 10.1016/j.amjcard.2009.07.061 IS - 12 KW - adolescent adult aging article child clinical article controlled study female Fontan procedure heart atrium arrhythmia heart atrium muscle heart atrium remodeling heart hemodynamics heart left atrium heart muscle fibrosis heart right atrium heart single ventricle histopathology human human tissue infant male morphometry priority journal risk factor surgical technique thickness tissue structure LA - English M3 - Article N1 - L355720226 2009-12-30 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://www.embase.com/search/results?subaction=viewrecord&from=export&id=L355720226 http://dx.doi.org/10.1016/j.amjcard.2009.07.061 VL - 104 ID - 1161 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. © 2009 Elsevier Inc. All rights reserved. AD - Department of Pediatrics, Harvard Medical School, Boston, MA, United States Department of Cardiology, Children's Hospital Boston, Boston, MA, United States Department of Pathology, Children's Hospital Boston, Boston, MA, United States Department of Cardiac Anesthesia, Children's Hospital Boston, Boston, MA, United States Department of Cardiothoracic Surgery, Children's Hospital Boston, Boston, MA, United States 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. DB - Scopus DO - 10.1016/j.amjcard.2009.07.061 IS - 12 M3 - Article N1 - Cited By :21 Export Date: 15 June 2020 PY - 2009 SP - 1737-1742 ST - Atrial Remodeling After the Fontan Operation T2 - American Journal of Cardiology TI - Atrial Remodeling After the Fontan Operation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-70749114425&doi=10.1016%2fj.amjcard.2009.07.061&partnerID=40&md5=d62dd28b1b8281d6a484737392a37bd1 VL - 104 ID - 2162 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 - D. Wolff, Department of Pediatric Cardiology, Center for Congenital Heart Diseases, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, Netherlands 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. DB - Embase Medline DO - 10.1111/chd.12576 IS - 2 KW - adult article bullying clinical article cross-sectional study dyspareunia erectile dysfunction female Fontan procedure human male Netherlands pilot study pregnancy priority journal quality of life self esteem semi structured interview sexual function sexual intercourse sexual satisfaction sexual wellbeing social isolation wellbeing LA - English M3 - Article N1 - L621242306 2018-03-20 2018-07-02 PY - 2018 SN - 1747-0803 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 - Congenital Heart Disease TI - Quality of life and sexual well-being in patients with a Fontan circulation: An explorative pilot study with a mixed method design UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L621242306 http://dx.doi.org/10.1111/chd.12576 VL - 13 ID - 701 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. © 2018 The Authors. Congenital Heart Disease published by Wiley Periodicals, Inc. AD - Department of Pediatric Cardiology, Center for Congenital Heart Diseases, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, Netherlands Wenckebach Institute, University Medical Center Groningen, University of Groningen, Groningen, Netherlands Center for Congenital Heart Diseases, Department of Cardiology, University Medical Center Groningen, University of Groningen, Groningen, Netherlands Center for Congenital Heart Diseases, Department of Cardiothoracic Surgery, University Medical Center Groningen, University of Groningen, Groningen, Netherlands Department of Obstetrics and Gynecology, University Medical Center Groningen, University of Groningen, Groningen, Netherlands 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. DB - Scopus DO - 10.1111/chd.12576 IS - 2 KW - congenital heart disease Fontan physiology quality of life sexual functioning special populations M3 - Article N1 - Export Date: 15 June 2020 PY - 2018 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 - Congenital Heart Disease TI - Quality of life and sexual well-being in patients with a Fontan circulation: An explorative pilot study with a mixed method design UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85043585066&doi=10.1111%2fchd.12576&partnerID=40&md5=94e0e2753a2326085825efdf7e974c83 VL - 13 ID - 1812 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. © 2016 AD - Division of Imaging Sciences and Biomedical Engineering, King's College London, St. Thomas’ Hospital, London, SE1 7EH, 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 - Scopus DO - 10.1016/j.ijcard.2016.12.087 KW - Catheterisation Congenital heart disease Fontan procedure Hypoplastic left heart syndrome Magnetic resonance imaging M3 - Article N1 - Cited By :10 Export Date: 15 June 2020 PY - 2017 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85009264090&doi=10.1016%2fj.ijcard.2016.12.087&partnerID=40&md5=1c2a64a73271117466da96f7714711d7 VL - 230 ID - 1869 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. AU - Wray, J. AU - Cassedy, A. AU - Ernst, M. M. AU - Franklin, R. C. AU - Brown, K. AU - Marino, B. S. DB - Medline DO - 10.1007/s00431-018-3250-7 IS - 12 KW - adolescent adult child child parent relation clinical trial coping behavior cross-sectional study female heart disease human male mental disease mental stress middle aged multicenter study preschool child psychology questionnaire United Kingdom United States LA - English M3 - Article N1 - L624882306 2018-11-27 PY - 2018 SN - 1432-1076 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=L624882306 http://dx.doi.org/10.1007/s00431-018-3250-7 VL - 177 ID - 653 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, WC1N 3JH, London, 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 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 - 132974888. Language: English. Entry Date: 20181122. Revision Date: 20191202. Publication Type: journal article AU - Wray, Jo AU - Cassedy, Amy AU - Ernst, Michelle M. AU - Franklin, Rodney C. AU - Brown, Katherine AU - Marino, Bradley S. DB - ccm DO - 10.1007/s00431-018-3250-7 DP - EBSCOhost IS - 12 KW - Adaptation, Psychological Heart Diseases -- Psychosocial Factors Parents -- Psychosocial Factors Mental Disorders -- Epidemiology Parenting Cross Sectional Studies Mental Disorders -- Etiology Child Female United States Male Adult Middle Age Stress, Psychological -- Psychosocial Factors Adolescence Human Child, Preschool Validation Studies Comparative Studies Evaluation Research Multicenter Studies Funding Source N1 - research; tables/charts. Journal Subset: Biomedical; Continental Europe; Europe. Grant Information: 0465467//American Heart Association/. NLM UID: 7603873. PMID: NLM30232593. PY - 2018 SN - 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=132974888&site=ehost-live&scope=site VL - 177 ID - 1431 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. © 2018, Springer-Verlag GmbH Germany, part of Springer Nature. AD - Critical Care and Cardiorespiratory Department, Great Ormond Street Hospital for Children NHS Foundation Trust, Great Ormond Street, London, WC1N 3JH, United Kingdom Division of Biostatistics and Epidemiology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, United States Department of Pediatrics, Division of Behavioral Medicine and Clinical Psychology, Cincinnati Children’s Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH, United States Department of Pediatric Cardiology, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom 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, United States AU - Wray, J. AU - Cassedy, A. AU - Ernst, M. M. AU - Franklin, R. C. AU - Brown, K. AU - Marino, B. S. DB - Scopus DO - 10.1007/s00431-018-3250-7 IS - 12 KW - Heart disease Parents Psychological functioning M3 - Article N1 - Cited By :3 Export Date: 15 June 2020 PY - 2018 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85053679520&doi=10.1007%2fs00431-018-3250-7&partnerID=40&md5=7667034fdcedf64dba6da75dbd46122d VL - 177 ID - 1778 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 - C. Limperopoulos, Center for the Developing Brain, Children's National Health System, 111 Michigan Ave NW, Washington, DC, United States 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. DB - Embase Medline DO - 10.1001/jamapediatrics.2019.5316 IS - 3 KW - adult anxiety disorder article brain development brain size brain stem case control study cerebellum congenital heart disease controlled study depression distress syndrome Edinburgh Postnatal Depression Scale female fetus heart single ventricle hippocampus human image reconstruction in utero brain development longitudinal study maternal stress neuroimaging nuclear magnetic resonance imaging outcome assessment Perceived Stress Scale pregnant woman priority journal prospective study right hippocampus State Trait Anxiety Inventory LA - English M3 - Article N1 - L630618684 2020-01-21 2020-03-24 PY - 2020 SN - 2168-6211 2168-6203 ST - Association of Maternal Psychological Distress with in Utero Brain Development in Fetuses with Congenital Heart Disease T2 - JAMA Pediatrics TI - Association of Maternal Psychological Distress with in Utero Brain Development in Fetuses with Congenital Heart Disease UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L630618684 http://dx.doi.org/10.1001/jamapediatrics.2019.5316 VL - 174 ID - 564 ER - TY - JOUR AB - Key Points: Question: Is there an association of prenatal maternal stress, anxiety, and depression with brain growth in fetuses with congenital heart disease? Findings: This longitudinal case-control study of 140 fetuses, including 48 fetuses with congenital heart disease having 74 magnetic resonance imaging scans and 92 healthy fetuses with 149 magnetic resonance imaging scans, showed that psychological distress in women with fetal congenital heart disease appears to be prevalent and associated with impaired fetal cerebellar and hippocampal development during the second half of gestation. Meaning: This study's findings suggest that universal screening for prenatal psychological distress and integrated cognitive-behavioral interventions are needed to better support pregnant women and optimize neurodevelopment in fetuses with congenital heart disease. 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. This case-control study assesses whether an association exists between maternal psychological distress and brain development in fetuses with congenital heart disease among pregnant women carrying fetuses with the disease vs healthy volunteers w th low-risk pregnancies recruited from the US Children's National Health System. 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 - 142106361. Language: English. Entry Date: 20200310. Revision Date: 20200331. Publication Type: Article AU - Wu, Yao AU - Kapse, Kushal AU - Jacobs, Marni AU - Niforatos-Andescavage, Nickie AU - Donofrio, Mary T. AU - Krishnan, Anita AU - Vezina, Gilbert AU - Wessel, David AU - du Plessis, Adré AU - Limperopoulos, Catherine DB - ccm DO - 10.1001/jamapediatrics.2019.5316 DP - EBSCOhost IS - 3 KW - Maternal Attitudes -- In Pregnancy Psychological Distress Fetal Development Brain -- Physiology Heart Defects, Congenital -- Physiopathology -- In Utero Human Pregnancy Female Fetus Prospective Studies Case Control Studies Anxiety Depression Edinburgh Postnatal Depression Scale Scales Hippocampus Cerebellum Brain Stem Magnetic Resonance Imaging Pregnancy Trimester, Third Descriptive Statistics Confidence Intervals N1 - diagnostic images; research; tables/charts. Journal Subset: Biomedical; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. Instrumentation: Perceived Stress Scale (PSS) (Cohen et al); Edinburgh Postnatal Depression Scale (EPDS); Spielberger State-Trait Anxiety Inventory. NLM UID: 101589544. PY - 2020 SN - 2168-6203 SP - e195316-e195316 ST - Association of Maternal Psychological Distress With In Utero Brain Development in Fetuses With Congenital Heart Disease T2 - JAMA Pediatrics TI - Association of Maternal Psychological Distress With In Utero Brain Development in Fetuses With Congenital Heart Disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=142106361&site=ehost-live&scope=site VL - 174 ID - 1459 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.. © 2020 American Medical Association. All rights reserved. AD - Center for the Developing Brain, Children's National Health System, 111 Michigan Ave NW, Washington, DC 20010, United States Division of Biostatistics and Study Methodology, Children's Research Institute, Children's National Health System, Washington, DC, United States Division of Neonatology, Children's National Health System, Washington, DC, United States Division of Cardiology, Children's National Health System, Washington, DC, United States Department of Diagnostic Imaging and Radiology, Children's National Health System, Washington, DC, United States Hospital and Specialty Services, Children's National Health System, Washington, DC, United States Fetal Medicine Institute, Children's National Health System, Washington, DC, United States 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. DB - Scopus DO - 10.1001/jamapediatrics.2019.5316 IS - 3 M3 - Article N1 - Export Date: 15 June 2020 PY - 2020 ST - Association of Maternal Psychological Distress with in Utero Brain Development in Fetuses with Congenital Heart Disease T2 - JAMA Pediatrics TI - Association of Maternal Psychological Distress with in Utero Brain Development in Fetuses with Congenital Heart Disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85077859218&doi=10.1001%2fjamapediatrics.2019.5316&partnerID=40&md5=e961f226ea815fb1c512e5c6eac8e6d0 VL - 174 ID - 1709 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 - 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 - Scopus DO - 10.3969/j.issn.1674-8115.2011.09.003 IS - 9 KW - Complete transposition of the great arteries Congenital corrected transposition of the great arteries Congenital heart disease Hypoplastic left heart syndrome Tetralogy of Fallot Total abnormal pulmonary venous connection M3 - Article N1 - Export Date: 15 June 2020 PY - 2011 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-80053191115&doi=10.3969%2fj.issn.1674-8115.2011.09.003&partnerID=40&md5=f2f6d2f17254ec3522e148009d8312a2 VL - 31 ID - 2118 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. AU - Yaman, A. AU - Kendirli, T. AU - Ödek, C. AU - Karadeniz, C. AU - Uçar, T. AU - Göllü, G. AU - Eyileten, Z. AU - Ince, E. DB - Embase Medline IS - 1 KW - epinephrine argon dopamine fresh frozen plasma heparin milrinone protamine sulfate acute respiratory failure article artificial ventilation assisted ventilation bleeding tendency blood analysis bronchoscopy case report child drug megadose edema endotracheal intubation endotracheal tube female Fontan procedure human hyperemia hypotension hypoxia intubation liver function test lung hemorrhage mortality noninvasive ventilation peak expiratory flow peak inspiratory flow positive end expiratory pressure preschool child prothrombin time thorax radiography tracheal hemorrhage weaning LA - English M3 - Article N1 - L372713958 2014-04-05 2014-04-14 PY - 2014 SN - 0041-4301 SP - 111-114 ST - Successful noninvasive mechanical ventilation in a child on prolonged and life-threatening invasive mechanical ventilation after Fontan operation T2 - Turkish Journal of Pediatrics TI - Successful noninvasive mechanical ventilation in a child on prolonged and life-threatening invasive mechanical ventilation after Fontan operation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L372713958 VL - 56 ID - 925 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 - Divisions of Pediatric Intensive Care Unit, Ankara University School of Medicine, Ankara, Turkey Divisions of Pediatric Cardiology, Department of Pediatrics, Ankara University School of Medicine, Ankara, Turkey Department of Pediatric Surgery, Ankara University School of Medicine, Ankara, Turkey Department of Cardiovascular Surgery, Ankara University School of Medicine, Ankara, Turkey AU - Yaman, A. AU - Kendirli, T. AU - Ödek, C. AU - Karadeniz, C. AU - Uçar, T. AU - Göllü, G. AU - Eyileten, Z. AU - Ince, E. DB - Scopus IS - 1 KW - Children Fontan operation Noninvasive mechanical ventilation Prolonged invasive mechanical ventilation M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2014 SP - 111-114 ST - Successful noninvasive mechanical ventilation in a child on prolonged and life-threatening invasive mechanical ventilation after Fontan operation T2 - Turkish Journal of Pediatrics TI - Successful noninvasive mechanical ventilation in a child on prolonged and life-threatening invasive mechanical ventilation after Fontan operation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84897031440&partnerID=40&md5=dbb2f3d6bfe84c9df686895b0a6e7498 VL - 56 ID - 2020 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 - H. Yamauchi, Department of Surgery II, Division of Cardiovascular Surgery, Nippon Medical School, Tokyo, Japan. 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. DB - Medline DO - 10.1272/jnms.67.455 IS - 6 KW - aorta aortic coarctation article cardiovascular surgery congenital heart malformation female follow up human infant male methodology newborn plastic surgery retrospective study treatment outcome LA - English M3 - Article N1 - L33416037 2001-01-19 PY - 2000 SN - 1345-4676 SP - 455-458 ST - Surgical treatment of coarctation complex in neonates and infants T2 - Journal of Nippon Medical School = Nihon Ika Daigaku zasshi TI - Surgical treatment of coarctation complex in neonates and infants UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L33416037 http://dx.doi.org/10.1272/jnms.67.455 VL - 67 ID - 1330 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 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. DB - Scopus DO - 10.1272/jnms.67.455 IS - 6 M3 - Article N1 - Cited By :2 Export Date: 15 June 2020 PY - 2000 SP - 455-458 ST - Surgical treatment of coarctation complex in neonates and infants T2 - Journal of Nippon Medical School = Nihon Ika Daigaku zasshi TI - Surgical treatment of coarctation complex in neonates and infants UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-18044373226&doi=10.1272%2fjnms.67.455&partnerID=40&md5=2846d2cb6986b62b098010dc81a5cb5c VL - 67 ID - 2280 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. Copyright © 2012 by The American Association for Thoracic Surgery. AD - Department of Mechanical and Aerospace Engineering, University of California San Diego, 9500 Gilman Dr, San Diego, CA 92093-0411, United States INRIA Paris-Rocquencourt, Le Chesnay Cedex, France Institute for Computational and Mathematical Engineering, Stanford University, Stanford, CA, United States Department of Cardiothoracic Surgery, Stanford University, Stanford, CA, United States Departments of Pediatrics and Bioengineering, Stanford University, Stanford, CA, United States AU - Yang, W. AU - Vignon-Clementel, I. E. AU - Troianowski, G. AU - Reddy, V. M. AU - Feinstein, J. A. AU - Marsden, A. L. DB - Scopus DO - 10.1016/j.jtcvs.2011.06.042 IS - 5 M3 - Article N1 - Cited By :41 Export Date: 15 June 2020 PY - 2012 SP - 1086-1097 ST - Hepatic blood flow distribution and performance in conventional and novel Y-graft Fontan geometries: A case series computational fluid dynamics study T2 - Journal of Thoracic and Cardiovascular Surgery TI - Hepatic blood flow distribution and performance in conventional and novel Y-graft Fontan geometries: A case series computational fluid dynamics study UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84859772737&doi=10.1016%2fj.jtcvs.2011.06.042&partnerID=40&md5=0d5bd9a7830a64f9a9c7d669d1df773a VL - 143 ID - 2097 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 - A.R. Yates, Nationwide Children's Hospital and Research Institute, Columbus, OH, USA. AU - Yates, A. R. AU - Hoffman, T. M. AU - Shepherd, E. AU - Boettner, B. AU - McBride, K. L. DB - Medline IS - 5 KW - article chromosome 13 chromosome 18 cohort analysis congenital heart malformation female genetics human male workforce pediatrics physician psychological aspect trisomy LA - English M3 - Article N1 - L560000290 2012-01-03 PY - 2011 SN - 1573-3599 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://www.embase.com/search/results?subaction=viewrecord&from=export&id=L560000290 VL - 20 ID - 1091 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 - Istanbul Üniversitesi Kardiyoloji Enstitüsü, Kalp ve Damar Cerrahisi Anabilim Dali, Istanbul, Turkey Yeditepe Üniversitesi Tip Fakültesi, Ergen ve Çocuk Psikiyatrisi Anabilim Dali, Istanbul, Turkey Istanbul Üniversitesi, Cerrahpaşa Tip Fakültesi, Çocuk Kardiyolojisi Bilim Dali, Istanbul, Turkey Istanbul Üniversitesi, Kardiyoloji Enstitüsü, Kardiyoloji 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 - Scopus DO - 10.4274/tpa.46.78 IS - 3 KW - Adolescent Congenital heart disease Cyanosis Quality of life Self-perception profile M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2011 SP - 220-227 ST - Bebeklik döneminde siyanotik kalp hastalıǧı nedeniyle açık kalp cerrahisi uygulanmış ergenlerde kendilik algısı ve yaşam niteliǧi 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-80054688964&doi=10.4274%2ftpa.46.78&partnerID=40&md5=a1bfc6545df5bf10ba9d2838a67c45b4 VL - 46 ID - 2137 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 followup. 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. © 2011 Published by European Association for Cardio-Thoracic Surgery. All rights reserved. AD - S. B. Zaets, 1 Wall Street 5B, Fort Lee, NJ 07024, United States 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. DB - Embase Medline DO - 10.1510/icvts.2010.253567 IS - 4 KW - adolescent adult article atrioventricular block bidirectional cavopulmonary anastomosis cause of death cavopulmonary connection child congestive heart failure controlled study follow up Fontan procedure heart arrhythmia heart single ventricle heart valve regurgitation heterotaxy syndrome human intermethod comparison kidney failure liver failure major clinical study modified Fontan operation mortality pleura effusion pneumonia postoperative complication preschool child priority journal quality of life risk factor sepsis supraventricular tachycardia surgical infection surgical risk surgical technique LA - English M3 - Article N1 - L361537635 2011-04-08 2011-05-03 http://icvts.ctsnetjournals.org/cgi/reprint/12/4/563?ck=nck PY - 2011 SN - 1569-9293 1569-9285 SP - 563-568 ST - Experience with bidirectional cavopulmonary anastomosis and modified Fontan operation in patients with single ventricle and concomitant visceral heterotaxy T2 - Interactive Cardiovascular and Thoracic Surgery TI - Experience with bidirectional cavopulmonary anastomosis and modified Fontan operation in patients with single ventricle and concomitant visceral heterotaxy UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L361537635 http://dx.doi.org/10.1510/icvts.2010.253567 VL - 12 ID - 1106 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 followup. 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. © 2011 Published by European Association for Cardio-Thoracic Surgery. All rights reserved. AD - Department of Congenital Heart Defects, Bakoulev Center for Cardiovascular Surgery, Moscow, Russian Federation 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. DB - Scopus DO - 10.1510/icvts.2010.253567 IS - 4 KW - Bidirectional cavopulmonary anastomosis Heterotaxy syndrome Left isomerism Modified Fontan operation Right isomerism Single ventricle M3 - Article N1 - Cited By :5 Export Date: 15 June 2020 PY - 2011 SP - 563-568 ST - Experience with bidirectional cavopulmonary anastomosis and modified Fontan operation in patients with single ventricle and concomitant visceral heterotaxy T2 - Interactive Cardiovascular and Thoracic Surgery TI - Experience with bidirectional cavopulmonary anastomosis and modified Fontan operation in patients with single ventricle and concomitant visceral heterotaxy UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-79953285901&doi=10.1510%2ficvts.2010.253567&partnerID=40&md5=d1a098bc36c1c876a55e1d415e6e21ee VL - 12 ID - 2130 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 - O. Zahmacioglu, Department of Child and Adolescent Psychiatry, Yeditepe University Medical Faculty, Istanbul, Turkey. 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. DB - Medline DO - 10.1186/1749-8090-6-155 KW - adaptive behavior adolescent article comparative study congenital heart malformation congenital malformation female heart surgery heart ventricle human infant male newborn preschool child psychological aspect qualitative research questionnaire LA - English M3 - Article N1 - L364681917 2012-05-07 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 - 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 - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L364681917 http://dx.doi.org/10.1186/1749-8090-6-155 VL - 6 ID - 1080 ER - TY - JOUR AD - Department of Child and Adolescent Psychiatry, Yeditepe University Medical Faculty, Istanbul, Turkey. AN - 104497942. Language: English. Entry Date: 20130607. Revision Date: 20200316. Publication Type: Journal Article AU - Zahmacioglu, Oguzhan AU - Yildiz, Cenk Eray AU - Koca, Bulent AU - Ugurlucan, Murat AU - Gokalp, Selman AU - Cetin, Gurkan AU - Eroglu, Ayse Guler AU - Oztunc, Funda DB - ccm DO - 10.1186/1749-8090-6-155 DP - EBSCOhost IS - 1 KW - Adaptation, Psychological Heart Surgery -- Psychosocial Factors Heart Defects, Congenital -- Surgery Heart Ventricle -- Abnormalities Qualitative Studies Adolescence Child, Preschool Female Heart Defects, Congenital -- Psychosocial Factors Heart Ventricle -- Surgery Human Infant Infant, Newborn Male Questionnaires N1 - research. Journal Subset: Biomedical; Europe; UK & Ireland. Special Interest: Perioperative Care. NLM UID: 101265113. PMID: NLM22112589. PY - 2011 SN - 1749-8090 SP - 155-155 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 - 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 - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104497942&site=ehost-live&scope=site VL - 6 ID - 1645 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 - M. Karimi, Yale University School of Medicine, Department of Surgery, Section of Cardiac Surgery, Pediatric Cardiothoracic Surgery, Boardman Bldg 204, 330 Cedar St, New Haven, CT, United States 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. DB - Embase Medline DO - 10.1016/j.athoracsur.2015.08.018 IS - 1 KW - aortopulmonary shunt article artificial heart pacemaker Blalock Taussig shunt child cohort analysis coil embolization electronic medical record follow up Fontan procedure Glenn shunt heart arrhythmia heart single ventricle human long term survival major clinical study medical record review overall survival palliative therapy postoperative complication postoperative period priority journal pulmonary arteriovenous fistula pulmonary valve prosthesis retrospective study sinus node disease survival rate tachycardia treatment outcome tricuspid valve atresia LA - English M3 - Article N1 - L607376013 2015-12-29 2016-02-09 PY - 2016 SN - 1552-6259 0003-4975 SP - 177-182 ST - Half a century's experience with the superior cavopulmonary (classic glenn) shunt T2 - Annals of Thoracic Surgery TI - Half a century's experience with the superior cavopulmonary (classic glenn) shunt UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L607376013 http://dx.doi.org/10.1016/j.athoracsur.2015.08.018 VL - 101 ID - 833 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. © 2016 The Society of Thoracic Surgeons. AD - Department of Pediatrics, Section of Pediatric Cardiology, Department of Surgery, Section of Pediatric Cardiothoracic Surgery, Yale University School of Medicine, New Haven, CT, United States 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. DB - Scopus DO - 10.1016/j.athoracsur.2015.08.018 IS - 1 M3 - Article N1 - Cited By :6 Export Date: 15 June 2020 PY - 2016 SP - 177-182 ST - Half a century's experience with the superior cavopulmonary (classic glenn) shunt T2 - Annals of Thoracic Surgery TI - Half a century's experience with the superior cavopulmonary (classic glenn) shunt UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84955720635&doi=10.1016%2fj.athoracsur.2015.08.018&partnerID=40&md5=7376892c06bbea34606e5c15d67ff3ba VL - 101 ID - 1928 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. © 2020 The Author(s). Published by Elsevier Ltd. This is an Open Access article under the CC BY 4.0 license 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 - Scopus DO - 10.1016/S2352-4642(19)30402-X IS - 3 M3 - Article N1 - Cited By :4 Export Date: 15 June 2020 PY - 2020 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85079540364&doi=10.1016%2fS2352-4642%2819%2930402-X&partnerID=40&md5=cc5928f1544aa59124931bf7bad0e22d VL - 4 ID - 1711 ER -