TY - JOUR AB - Williams syndrome (WS) is an arteriopathic derangement associated with supravalvular aortic stenosis and branch pulmonary stenosis. We describe double-outlet right ventricle with mitral atresia and aortic arch hypoplasia in an infant with WS. This case demonstrates the difficulty in managing patients with WS with complex cardiac defects. To our knowledge, this is the first reported single-ventricle physiology in a patient with WS. (Level of Difficulty: Advanced.) AD - J.A. Robinson, Pediatric Cardiology, Dr. C.C. and Mabel L. Criss Heart Center, Children's Hospital & Medical Center, 8200 Dodge Street, Omaha, NE, United States AU - Katt, T. E. AU - Spicer, R. L. AU - Yetman, A. T. AU - Ibrahimiye, A. N. AU - Hammel, J. M. AU - Robinson, J. A. DB - UEmbase DO - 10.1016/j.jaccas.2020.05.098 IS - 11 KW - Ommaya reservoir prostaglandin E1 anasarca aorta anomaly aortic arch hypoplasia aortic arch surgery artery reconstruction article ascites atrioventricular septal defect brain hemorrhage cardiomegaly cardiopulmonary bypass cardiovascular system examination case report clinical article clinical effectiveness congenital heart disease diastolic blood pressure diastolic heart murmur differential diagnosis disease severity Ebstein anomaly echocardiography electrocardiogram endotracheal intubation Fallot tetralogy female fetus echography great vessels transposition heart catheterization heart output heart single ventricle heart surgery heart valve commissurotomy heart ventricle hypertrophy human hypotension hypoxemia image analysis infant intracranial pressure lung vascular resistance lung vein drainage anomaly medical history metabolic acidosis mitral valve atresia neonatal intensive care unit newborn surgery oxygen therapy physical examination priority journal pulmonary artery stenosis respiratory acidosis supravalvular aortic stenosis systolic blood pressure thorax radiography transthoracic echocardiography tricuspid valve regurgitation Williams Beuren syndrome LA - English M3 - Article N1 - L2007661769 2020-09-07 2020-09-22 Williams syndrome (WS) is an arteriopathic derangement associated with supravalvular aortic stenosis and branch pulmonary stenosis. We describe… PY - 2020 SN - 2666-0849 SP - 1716-1719 ST - Williams Syndrome and Neonatal Cardiac Surgery for Congenital Single Ventricle T2 - JACC: Case Reports TI - Williams Syndrome and Neonatal Cardiac Surgery for Congenital Single Ventricle UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2007661769&from=export http://dx.doi.org/10.1016/j.jaccas.2020.05.098 VL - 2 ID - 4397 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. AU - Cassidy, A. R. AU - Bernstein, J. H. AU - Bellinger, D. C. AU - Newburger, J. W. AU - DeMaso, D. R. DB - UEmbase DO - 10.1080/13854046.2018.1503333 IS - 4 KW - adolescent congenital heart malformation female human male mental disease physiology procedures psychology spatial behavior vision LA - English M3 - Article N1 - L628023666 2020-12-21 PY - 2019 SN - 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 - https://www.embase.com/search/results?subaction=viewrecord&id=L628023666&from=export http://dx.doi.org/10.1080/13854046.2018.1503333 VL - 33 ID - 4491 ER - TY - JOUR AB - Background: The standard first stage palliation for univentricular heart with unrestricted pulmonary blood flow (PBF) is surgical pulmonary artery (PA) banding for which the ideal age is within the first 8 weeks of life. This study aimed to look for the utility of PA band done beyond 3 months of age for patients presenting beyond the stipulated period. Materials and Methods: This is a retrospective analysis of the outcome of twenty patients with single ventricle (SV) physiology with unrestricted PBF who presented late and were selected on the basis of clinical, radiological, and echocardiographic parameters for PA banding. Results: The median age of the patients was 5.5 months (3.5-96 months), and the median body weight was 4.7 kg (3.2-22.0 kg). The patients were divided into three groups as follows: Ten patients between 3 and 6 months of age (Group A), seven patients between 6 months to 1 year of age (Group B), and three patients > 1 year of age with additional features of pulmonary venous hypertension (Group C). The mean reduction of PA pressures following PA band was 60.9%, 48.8%, and 58.3% and the mean fall in oxygen saturation was 10.4%, 8.0%, and 6.6% in the three groups, respectively. The postoperative mortality rate was 10%. The mean follow up duration was 13.5 months (7-23 months). There was a statistically significant improvement in weight for age Z scores following PA band (P = 0.0001). On follow up cardiac catheterization, the mean PA pressures were 16.6 (±3.6), 22.7 (±5.7), and 33.3 (±12.4) mmHg, respectively, in the three groups, and the mean pulmonary vascular resistance index was 1.86 (±0.5), 2.45 (±0.7), and 3.5 (±1.6) WU.m2, respectively. Subsequently, seven patients in Group A, three patients in Group B, and one patient from Group C underwent successful bidirectional Glenn (BDG) surgery. Conclusions: Late PA band in selected patients with SV physiology can have definite benefit in terms of correction of heart failure symptoms and subsequent conversion to BDG and can potentially change the natural history of disease both in terms of survival and quality of life. AD - S. Ghosh, Department of Pediatric Cardiology, Narayana Superspeciality Hospital, 120/1 Andul Road, Near Nabanna, Shibpur, Howrah, West Bengal, India AU - Mukherji, A. AU - Ghosh, S. AU - Pathak, N. AU - Das, J. AU - Dutta, N. AU - Das, D. AU - Chattopadhyay, A. DB - UEmbase DO - 10.4103/apc.APC_128_20 IS - 1 KW - article body weight clinical article controlled study female follow up heart catheterization heart failure heart single ventricle history human hypertension infant lung artery banding lung artery pressure lung blood flow lung vascular resistance male oxygen saturation pulmonary vascular disease pulmonary vein quality of life retrospective study surgical mortality survival LA - English M3 - Article N1 - L634042426 2021-02-03 PY - 2021 SN - 0974-5149 0974-2069 SP - 26-34 ST - Utility of late pulmonary artery banding in single-ventricle physiology: A mid-term follow-up T2 - Annals of Pediatric Cardiology TI - Utility of late pulmonary artery banding in single-ventricle physiology: A mid-term follow-up UR - https://www.embase.com/search/results?subaction=viewrecord&id=L634042426&from=export http://dx.doi.org/10.4103/apc.APC_128_20 VL - 14 ID - 4372 ER - TY - JOUR AB - Background: Exercise limitation is almost universal among Fontan patients. Identifying unique clinical features in the small fraction of Fontan patients with normal exercise capacity (high-capacity Fontan [HCF]) provides potential to inform clinical strategies for those with low exercise capacity (usual Fontan). Methods: We performed a retrospective chart review of all patients with single-ventricle physiology palliated with a Fontan operation who underwent incremental cardiopulmonary exercise testing at Cincinnati Children's Hospital Medical Center from 2013 to 2018. Comparison was between patients with peak oxygen uptake < vs ≥ 80% predicted. Results: A total of 22 of 112 patients were classified as HCF (68% were female; aged 18 ± 7 years). During incremental exercise, peak oxygen uptake (86.1% ± 6.1% vs 62% ± 12.2% predicted; P < 0.001) was greater in HCF vs usual Fontan despite similar chronotropic impairment, resulting in a greater oxygen pulse in HCF. Pulmonary function, breathing reserve, and ventilatory equivalent for CO2 output slope were not different between groups. Those in the HCF group were more likely to self-report exercise ≥ 4 days/week for at least 30 minutes (77% vs 10%, P < 0.001), have normal systolic function (95% vs 74%, P = 0.003), have fewer postoperative complications (8% vs 36%, P = 0.04), and have shorter post-Fontan length of stay (8 ± 2.8 vs 12.4 ± 0.9 days, P = 0.04). Conclusions: Approximately 1 in 5 Fontan patients who undergo cardiopulmonary exercise testing have normal exercise capacity despite chronotropic impairment. This implies a better preserved stroke volume, perhaps due to greater muscle pump-mediated preload. Additionally, a complicated perioperative Fontan course is associated with eventual impaired functional capacity. AD - A.W. Powell, Cincinnati, 3333 Burnett Ave, MLC 2003, Ohio, United States AU - Powell, A. W. AU - Chin, C. AU - Alsaied, T. AU - Rossiter, H. B. AU - Wittekind, S. AU - Mays, W. A. AU - Lubert, A. AU - Veldtman, G. DB - OEmbase DO - 10.1016/j.cjca.2019.11.006 KW - adult aerobic capacity article breathing child chronotropism complication controlled study exercise test female Fontan procedure functional status heart single ventricle heart stroke volume human length of stay lung function major clinical study male medical record review muscle phenotype postoperative complication retrospective study self report young adult carbon dioxide oxygen LA - English M3 - Article in Press N1 - L2005547819 2020-04-20 PY - 2020 SN - 0828-282X ST - The Unique Clinical Phenotype and Exercise Adaptation of Fontan Patients With Normal Exercise Capacity T2 - Canadian Journal of Cardiology TI - The Unique Clinical Phenotype and Exercise Adaptation of Fontan Patients With Normal Exercise Capacity UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2005547819 http://dx.doi.org/10.1016/j.cjca.2019.11.006 ID - 3489 ER - TY - JOUR AB - BACKGROUND: Trisomy 21 (T21) patients often have elevated pulmonary vascular resistance, which may result in suboptimal cavopulmonary connection (CPC) following Glenn or Fontan operations. The purpose of this study was to assess in a nationwide, multi-institution cohort of CPC patients the impact of T21 on patient morbidity, mortality, and resource utilization. METHODS: A total of 23,271 CPC pediatric patients (2004 - 2019) at 50 U.S. hospitals were evaluated using the Pediatric Health Information System (PHIS) database. Univariable and multivariable regression analyses were used to assess risk-adjusted associations between Down syndrome and other risk factors and postoperative measures of morbidity, mortality, lengths of stay (LOS) and cost of hospitalization. RESULTS: The overall prevalence of T21 among Glenn and Fontan patients was 1.5% (199/13,268) and 0.8% (78/1,003), respectively. Among both CPC cohorts, T21 status significantly increased unadjusted mortality, hospital lengths of stay and total costs of hospitalization compared to non-T21 CPC patients (all P < 0.001). T21 patients also incurred a higher incidence of prolonged mechanical ventilation compared to non-T21 patient in both Glenn and Fontan patients (P < 0.001). Multivariable regression analysis further estimated that T21 patients are associated with a 5.5-fold increase in mortality (P < 0.001) compared to non-T21 patients. Lastly, T21 patients had increased long-term mortality compared to their peers. CONCLUSIONS: Trisomy 21 significantly increases risk-adjusted morbidity, inpatient mortality, long-term mortality and resource utilization following cavopulmonary connections. Further investigation is needed to clarify modifiable patient-level and center-specific risk factors to improve outcomes for T21 patients. AU - Allen, P. AU - Anderson, B. R. AU - Bacha, E. AU - LaPar, D. J. DB - UEmbase DO - 10.1016/j.athoracsur.2020.10.014 KW - article artificial ventilation cavopulmonary connection child cohort analysis controlled study Down syndrome female hospital mortality hospitalization human length of stay major clinical study male medical information system morbidity pediatric patient postoperative care prevalence risk assessment risk factor LA - English M3 - Article in Press N1 - L633375762 2020-11-16 PY - 2020 SN - 1552-6259 ST - Trisomy 21 Patients Undergoing Cavopulmonary Connections Need Improved Pre- and Postoperative Care T2 - The Annals of thoracic surgery TI - Trisomy 21 Patients Undergoing Cavopulmonary Connections Need Improved Pre- and Postoperative Care UR - https://www.embase.com/search/results?subaction=viewrecord&id=L633375762&from=export http://dx.doi.org/10.1016/j.athoracsur.2020.10.014 ID - 4384 ER - TY - JOUR AB - OBJECTIVE: Prolonged admission to a paediatric intensive care unit (PICU) consumes significant healthcare resource. An increase in the number of long-stay admissions and bed utilisation has been reported elsewhere in the world but not in the UK. If an increasing trend of long-stay admissions is evident, this may have significant implications for provision of paediatric intensive care in the future. DESIGN/SETTING/PATIENTS: We retrospectively analysed prospectively collected data from Birmingham Children's Hospital, UK, over a 20-year period from 1998 to 2017. PICU admissions, bed-days, length of stay and mortality trends were analysed and reported over four different epochs (1998-2002, 2003-2007, 2008-2012 and 2013-2017) for long-stay admissions (PICU length of stay ≥28 days) and others. Differences in patient demographics, diagnostic categorisation and hospital utilisation were also analysed. RESULTS: In total, 24 203 admissions accounted for 131 553 bed-days over the 20-year period. 705 (2.9%) long-stay admissions accounted for 42 312 (32%) bed-days. Proportion of long-stay admissions and corresponding bed-days increased from 1.6% and 20.5% in 1998-2002 to 4.5% and 42.6%, respectively, in 2013-2017 (p<0.001). Long-stay patients had a significantly higher number of hospital admissions (median: 4 vs 2, p<0.001) per patient and overall hospital length of stay (median: 98 vs 15, p<0.001) bed-days compared with other patients. Long-stay admissions were associated with significantly higher crude mortality (23% vs 6%, p<0.001) compared with other admissions. CONCLUSIONS: A significant increase in the proportion of prolonged PICU admissions with disproportionately high resource utilisation and mortality is evident over two decades. AD - Paediatric Intensive Care Unit, Birmingham Children's Hospital, Birmingham Women's and Children's NHS Foundation Trust, Birmingham, UK hari.krishnan2@nhs.net. Birmingham Clinical Trials Unit, University of Birmingham, Birmingham, UK. Paediatric Intensive Care Unit, Birmingham Children's Hospital, Birmingham Women's and Children's NHS Foundation Trust, Birmingham, UK. Birmingham Acute Care Research Group, Institute of Inflammation and Ageing, University of Birmingham, Birmingham, UK. Institute of Applied Health Research, University of Birmingham, Birmingham, UK. AN - 31848145 AU - Kanthimathinathan, H. K. AU - Plunkett, A. AU - Scholefield, B. R. AU - Pearson, G. A. AU - Morris, K. P. DA - Jun DB - UPubmed DO - 10.1136/archdischild-2019-317797 DP - NLM ET - 2019/12/19 IS - 6 KW - Bone Marrow Transplantation/statistics & numerical data Cerebral Palsy/epidemiology Child, Preschool Developmental Disabilities/epidemiology Female Hospital Mortality Hospitals, Pediatric Humans Hypoplastic Left Heart Syndrome/epidemiology Infant Infant, Newborn *Intensive Care Units, Pediatric Length of Stay/*trends Liver Transplantation/statistics & numerical data Male Patient Admission/*statistics & numerical data Retrospective Studies Tracheostomy/statistics & numerical data United Kingdom/epidemiology *capacity *intensive care *length of stay *paediatric practice LA - eng N1 - 1468-2044 Kanthimathinathan, Hari Krishnan Orcid: 0000-0001-5170-1534 Plunkett, Adrian Scholefield, Barnaby R Pearson, Gale A Orcid: 0000-0001-5329-5680 Morris, Kevin P Journal Article England Arch Dis Child. 2020 Jun;105(6):558-562. doi: 10.1136/archdischild-2019-317797. Epub 2019 Dec 17. PY - 2020 SN - 0003-9888 SP - 558-562 ST - Trends in long-stay admissions to a UK paediatric intensive care unit T2 - Arch Dis Child TI - Trends in long-stay admissions to a UK paediatric intensive care unit VL - 105 ID - 2388 ER - TY - JOUR AB - Successfully transitioning to adult care is important for congenital heart disease (CHD) patients, particularly those at the worst end of the spectrum with a Fontan circulation, as their ongoing health engagement affects their health outcomes. Yet, there is a lack of literature exploring patient perspectives about their transition experiences, and what helps or hinders successful transition to adult care. Young adults with a Fontan circulation (n = 18) from the Australian and New Zealand Fontan Registry participated in in-depth phone interviews. Thematic analyses were used to analyze the qualitative interview data. We identified six key themes which stood out from patient experiences, including differences between pediatric and adult congenital cardiac care, the need for preparation through formal transition programs, and the important role of support systems. To become active managers of their healthcare and remain engaged with the healthcare system, young people would benefit from coordinated efforts between pediatric and adult care to formally prepare them for transition to adult care. This should include involvement from their support network and ongoing education about their health condition. The study highlights the importance of studying patient perspectives through qualitative research to better inform health service provision in the CHD space. AD - Y. D'udekem, Murdoch Children's Research Institute, Melbourne, Victoria, Australia AU - Du Plessis, K. AU - Peters, R. AU - Culnane, E. AU - D'Udekem, Y. DB - UEmbase DO - 10.1515/ijamh-2018-0020 IS - 6 KW - adult article Australia clinical article congenital heart disease female follow up Fontan procedure health behavior health care health care system human interview male New Zealand patient education qualitative research thematic analysis transition to adult care young adult LA - English M3 - Article N1 - L624201245 2018-10-11 2021-03-05 PY - 2020 SN - 2191-0278 0334-0139 ST - Traversing the liminal: What can Fontan adults' transition experiences and perspectives teach us about optimizing healthcare? T2 - International Journal of Adolescent Medicine and Health TI - Traversing the liminal: What can Fontan adults' transition experiences and perspectives teach us about optimizing healthcare? UR - https://www.embase.com/search/results?subaction=viewrecord&id=L624201245&from=export http://dx.doi.org/10.1515/ijamh-2018-0020 VL - 32 ID - 4381 ER - TY - JOUR AB - Children and adolescents with cardiac disease (CCD) have significant morbidity and lower quality of life. However, there are no broadly applicable tools similar to the frailty score as described in the elderly, to define functional phenotype in terms of physical capability and psychosocial wellbeing in CCD. The purpose of this study is to investigate the domains of the frailty in CCD. We prospectively recruited CCD (8-17.5 years old, 70% single ventricle, 27% heart failure, 12% pulmonary hypertension; NYHA classes I, II and III) and age and gender matched healthy controls (total n = 56; CCD n = 34, controls n = 22; age 12.6 ± 2.6 years; 39.3% female). We measured the five domains of frailty: slowness, weakness, exhaustion, body composition and physical activity using developmentally appropriate methods. Age and gender-based population norms were used to obtain Z scores and percentiles for each measurement. Two-tailed t-tests were used to compare the two groups. The CCD group performed significantly worse in all five domains of frailty compared to healthy controls. Slowness: 6-min walk test with Z score -3.9 ± 1.3 vs -1.4 ± 1.3, p < 0.001; weakness: handgrip strength percentile 18.9 ± 20.9 vs 57.9 ± 26.0, p < 0.001; exhaustion: multidimensional fatigue scale percentile 63.7 ± 13.5 vs 83.3 ± 14.4, p < 0.001; body composition: height percentile 43.4 ± 29.5 vs 71.4 ± 25.2, p < 0.001, weight percentile 46.0 ± 36.0 vs 70.9 ± 24.3, p = 0.006, BMI percentile 48.4 ± 35.5 vs 66.9 ± 24.2, p = 0.04, triceps skinfold thickness 41.0 ± 24.0 vs 54.4 ± 22.1, p = 0.04; physical activity: pediatric activity questionnaire score 2 ± 0.6 vs 2.7 ± 0.6, p < 0.001. The domains of frailty can be quantified in children using developmentally appropriate methods. CCD differ significantly from controls in all five domains, supporting the concept of quantifying the domains of frailty. Larger longitudinal studies are needed to study frailty in CCD and examine if it predicts adverse health outcomes.Clinical Trial Registration: The ClinicalTrials.gov identification number is NCT02999438. https://clinicaltrials.gov/ct2/show/NCT02999438. AD - Department of Child Health, University of Missouri Health Care, Columbia, MO, USA. panchangams@health.missouri.edu. University of Missouri-Columbia, 500 N Keene St, Suite 207, Columbia, MO, 65201, USA. panchangams@health.missouri.edu. The Ward Family Heart Center, Children's Mercy Hospital, Kansas City, MO, USA. UMKC School of Medicine, Kansas City, MO, USA. AN - 32377892 AU - Panchangam, C. AU - White, D. A. AU - Goudar, S. AU - Birnbaum, B. AU - Malloy-Walton, L. AU - Gross-Toalson, J. AU - Reid, K. J. AU - Shirali, G. AU - Parthiban, A. C2 - PMC7223568 DA - Jun DB - UPubmed DO - 10.1007/s00246-020-02354-7 DP - NLM ET - 2020/05/08 IS - 5 KW - Adolescent Case-Control Studies Child Female Frailty/*diagnosis/etiology/physiopathology Heart Defects, Congenital/*complications/physiopathology Humans Male Phenotype Physical Functional Performance Prospective Studies Quality of Life Cardiomyopathy Children with heart disease Congenital heart disease Fontan Frailty Heart failure Pulmonary hypertension Quality and outcomes LA - eng N1 - 1432-1971 Panchangam, Chaitanya Orcid: 0000-0001-6515-0526 White, David A Goudar, Suma Birnbaum, Brian Malloy-Walton, Lindsey Gross-Toalson, Jami Reid, Kimberly J Shirali, Girish Parthiban, Anitha Journal Article Pediatr Cardiol. 2020 Jun;41(5):1031-1041. doi: 10.1007/s00246-020-02354-7. Epub 2020 May 6. PY - 2020 SN - 0172-0643 (Print) 0172-0643 SP - 1031-1041 ST - Translation of the Frailty Paradigm from Older Adults to Children with Cardiac Disease T2 - Pediatr Cardiol TI - Translation of the Frailty Paradigm from Older Adults to Children with Cardiac Disease VL - 41 ID - 2372 ER - TY - JOUR AB - Background: Children with congenital heart disease (CHD) require lifelong cardiology follow-up. Many experience gaps in care around the age of transition to adult-oriented care with associated comorbidity. We describe the impact of a clinic-based intervention on follow-up rates in this high-risk population.Methods: Patients ≥11 years seen in a paediatric outpatient CHD Transition Clinic completed self-assessment questionnaires, underwent focused teaching, and were followed on a clinic registry with assessment of care continuation. The cohort lost to follow-up rate, defined as absence from care at least 6 months beyond the recommended timeframe, was compared with a control group. Secondary outcomes included questionnaire scores and adult cardiology referral trends.Results: Over 26 months, 53 participants completed an initial Transition Clinic visit; 43% (23/53) underwent a second visit. Median participant age was 18.0 years (interquartile range 16.0, 22.0). The cohort's lost to follow-up rate was 7.3%, which was significantly lower than the control rate (25.9%, p < 0.01). Multivariable regression analyses demonstrated clinic participation as the only factor independently associated with follow-up rates (p = 0.048). Transition readiness was associated with older age (p = 0.01) but not sex, univentricular heart, interventional history, or surgical complexity. One-third of adult participants transferred to adult care.Conclusions: A CHD Transition Clinic intervention can improve follow-up rates in adolescents and young adults. Age is an important factor in transition readiness, and retention of adults in paediatric care appears multi-factorial. We postulate that serial assessments of self-management, focused education, and registry utilisation may improve patient outcomes by reducing lapses in care. AD - S.S. Gaydos, Division of Pediatric Cardiology, Department of Pediatrics, Medical University of South Carolina, Shawn Jenkins Children's Hospital, 10 McClellan Banks Drive, Charleston, SC, United States AU - Gaydos, S. S. AU - Chowdhury, S. M. AU - Judd, R. N. AU - McHugh, K. E. DB - UEmbase DO - 10.1017/S1047951120000682 IS - 5 KW - adolescent adult age distribution article case control study child cohort analysis congenital heart disease controlled study female follow up heart single ventricle high risk population human major clinical study male medical history outcome assessment outpatient care patient education patient referral quality of life register retention in care retrospective study self evaluation sex difference transition to adult care transitional care trend study young adult LA - English M3 - Article N1 - L631474307 2020-04-20 PY - 2020 SN - 1467-1107 1047-9511 SP - 633-640 ST - A transition clinic intervention to improve follow-up rates in adolescents and young adults with congenital heart disease T2 - Cardiology in the Young TI - A transition clinic intervention to improve follow-up rates in adolescents and young adults with congenital heart disease UR - https://www.embase.com/search/results?subaction=viewrecord&id=L631474307&from=export http://dx.doi.org/10.1017/S1047951120000682 VL - 30 ID - 4418 ER - TY - JOUR AB - We report on two patients who received a transcatheter cavopulmonary connection by a needle puncture under deep conscious sedation. In both patients, the vessel-to-vessel connection was achieved by a venous access into the superior caval vein and direct needle puncture of the pulmonary artery. The two cavopulmonary anastomoses were held open by a covered stent and a bare-metal stent, respectively. AD - D. Tanase, Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Center Munich, Technische Universität München, Munich, Germany AU - Tanase, D. AU - Georgiev, S. AU - Eicken, A. AU - Ewert, P. DB - UEmbase DO - 10.1002/ccd.28771 IS - 7 KW - Amplatzer Vascular Amplatzer vascular plug Andra XXL balloon valvuloplasty catheter bare metal stent catheter sheath Chiba needle goose neck snare vascular guide wire microcatheter sizing balloon catheter snare wire Somatex article ascites atrioventricular septal defect cardiac imaging case report cavopulmonary connection child clinical article comorbidity computer assisted tomography decision making disease severity Fontan procedure heart atrium enlargement heart atrium flutter heart failure hospital discharge human informed consent lung vein drainage anomaly needle biopsy outpatient department preschool child pulmonary valve atresia treatment refusal wound closure Amplatzer Check Flo Performer CP Stent HI Torque Floppy II Progreat Z MED II LA - English M3 - Article N1 - L2004243325 2020-02-19 2020-07-07 PY - 2020 SN - 1522-726X 1522-1946 SP - 1305-1309 ST - Transcatheter creation of bidirectional cavopulmonary connections by needle punctures in two patients T2 - Catheterization and Cardiovascular Interventions TI - Transcatheter creation of bidirectional cavopulmonary connections by needle punctures in two patients UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2004243325&from=export http://dx.doi.org/10.1002/ccd.28771 VL - 95 ID - 4408 ER - TY - JOUR AB - Trajectories of neurodevelopment and quality of life were analyzed in children with hypoplastic left heart syndrome according to socioeconomic status (SES) and maternal education. Lower SES and less maternal education were associated with greater early delays in communication and problem-solving and progressive delays in problem-solving and fine motor skills over time. AD - Department of Cardiology, Boston Children's Hospital, Boston, MA; Department of Pediatrics, Harvard Medical School, Boston, MA. Department of Psychology, The Hospital for Sick Children, Toronto, Ontario, Canada. Department of Pediatrics, Medical College of Wisconsin and Herma Heart Institute, Children's Wisconsin, Milwaukee, WI. Department of Pediatrics, University of Michigan CS Mott Children's Hospital, Ann Arbor, MI. Department of Cardiology, Boston Children's Hospital, Boston, MA; Department of Pediatrics, Harvard Medical School, Boston, MA. Electronic address: jane.newburger@cardio.chboston.org. AN - 33031800 AU - Bucholz, E. M. AU - Sleeper, L. A. AU - Sananes, R. AU - Brosig, C. L. AU - Goldberg, C. S. AU - Pasquali, S. K. AU - Newburger, J. W. C2 - PMC7855010 C6 - NIHMS1647566 DA - Feb DB - UPubmed DO - 10.1016/j.jpeds.2020.09.066 DP - NLM ET - 2020/10/09 KW - Child Child, Preschool *Educational Status Female *Functional Status Humans Male Mothers/*education Nervous System/*growth & development *Quality of Life *Social Class *Univentricular Heart *congenital heart disease *hypoplastic left heart syndrome LA - eng N1 - 1097-6833 Bucholz, Emily M Sleeper, Lynn A Sananes, Renee Brosig, Cheryl L Goldberg, Caren S Pasquali, Sara K Newburger, Jane W T32 HL007572/HL/NHLBI NIH HHS/United States U10 HL068270/HL/NHLBI NIH HHS/United States U24 HL135691/HL/NHLBI NIH HHS/United States UG1 HL135685/HL/NHLBI NIH HHS/United States Journal Article Research Support, N.I.H., Extramural J Pediatr. 2021 Feb;229:289-293.e3. doi: 10.1016/j.jpeds.2020.09.066. Epub 2020 Oct 6. PY - 2021 SN - 0022-3476 (Print) 0022-3476 SP - 289-293.e3 ST - Trajectories in Neurodevelopmental, Health-Related Quality of Life, and Functional Status Outcomes by Socioeconomic Status and Maternal Education in Children with Single Ventricle Heart Disease T2 - J Pediatr TI - Trajectories in Neurodevelopmental, Health-Related Quality of Life, and Functional Status Outcomes by Socioeconomic Status and Maternal Education in Children with Single Ventricle Heart Disease VL - 229 ID - 2353 ER - TY - JOUR AB - Topsy‐turvy heart is a rare congenital anomaly; it has a specific characteristic of cardiac malrotation and superior–inferior arrangement of right/left ventricles. A five‐day‐old patient was diagnosed antenatally with hypoplastic left heart and right hydronephrosis and had been admitted to the hospital with symptoms of respiratory distress. The postnatal imaging studies revealed an abnormal ventricular arrangement with a large aortopulmonary window, ventricular septal defect (VSD), and the upside‐down orientation resulting in the posterior–inferior displacement of the common arterial confluence and, in turn, the left main bronchial stenosis. For the first time, the diagnosis of a topsy‐turvy heart with the intracardiac anomaly (VSD) was confirmed. AD - Cardiology Division, Department of Internal Medicine, South Valley University, Qena, Egypt Congenital Heart Disease Unit, Department of Cardiology, Ain Shams University, Cairo, Egypt Department of Pediatric Cardiology, Basaksehir Cam and Sakura Hospital, Istanbul, Turkey AN - 148430028. Language: English. Entry Date: 20210205. Revision Date: 20210208. Publication Type: Article AU - Alkhateeb, Areej AU - Mansour, Amr AU - Tanidir, Ibrahim Cansaran DB - Ucinahl DO - 10.1111/echo.14950 DP - EBSCOhost IS - 1 KW - Heart Defects, Congenital -- Diagnosis Rare Diseases Aortopulmonary Septal Defect -- Diagnosis Heart Septal Defects, Ventricular -- Diagnosis Heart Ventricle -- Abnormalities Heart Ventricle, Right -- Abnormalities Infant, Newborn Hypoplastic Left Heart Syndrome Hydronephrosis Respiratory Distress Syndrome N1 - case study; diagnostic images; tables/charts. Journal Subset: Allied Health; Biomedical; Double Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. NLM UID: 8511187. PY - 2021 SN - 0742-2822 SP - 114-117 ST - Topsy‐turvy heart: A first recorded case report with intracardiac anomaly T2 - Echocardiography TI - Topsy‐turvy heart: A first recorded case report with intracardiac anomaly UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=148430028&site=ehost-live&scope=site VL - 38 ID - 5313 ER - TY - JOUR AB - To examine whether neurodevelopment at preschool age predicts IQ levels and needs for special education services in school-age children with single ventricle (SV) physiology. Thirty-five patients with SV physiology were assessed using the Bayley Scale of Infant and Toddler Development (BSID) II or III at 3 years and the Wechsler Intelligence Scale for Children-Fourth Edition (WISC) at 8 years. Whether the children were receiving special education services was also determined. Factors associated with the mental developmental index (MDI) of the BSID, the full-scale IQ (FSIQ) of the WISC, and special education services were also analyzed. The MDI and FSIQ of children with SV physiology were significantly lower than the values in healthy children (P < 0.01). The MDI at 3 years was moderately correlated with FSIQ at 8 years (P < 0.01, R(2) = 0.41). Ten patients (28.6%) received special education services in their school. Children with MDI <85 were more likely than those with MDI ≥85 to require special education services at school age (53% and 10%, respectively, P < 0.01). Weight at birth and stage II were correlated with the MDI, extracorporeal circulation time at stage II was correlated with FSIQ, and age at Fontan operation was correlated with FSIQ and special education services. The toddler neurodevelopment index may predict not only IQ levels but also the need for special education services in school-age children. Children with low neurodevelopmental scores need to be followed closely for a long time. AD - Department of Pediatrics, Faculty of Medicine, University of Toyama, Toyama, Japan. Electronic address: sakiko-tym@umin.ac.jp. Department of Pediatrics, Faculty of Medicine, University of Toyama, Toyama, Japan. Faculty of Human Development, Department of Educational Sciences, University of Toyama, Toyama, Japan. The 1(st) Department of Surgery, Faculty of Medicine, University of Toyama, Toyama, Japan. AN - 31698030 AU - Hiraiwa, A. AU - Ibuki, K. AU - Tanaka, T. AU - Hirono, K. AU - Miya, K. AU - Yoshimura, N. AU - Ichida, F. DA - Summer DB - UPubmed DO - 10.1053/j.semtcvs.2019.10.017 DP - NLM ET - 2019/11/08 IS - 2 KW - Age Factors Brain/*growth & development *Cardiac Surgical Procedures/adverse effects Case-Control Studies Child *Child Behavior *Child Development Child, Preschool Developmental Disabilities/diagnosis/etiology/*physiopathology/psychology Education, Special Female Heart Defects, Congenital/diagnosis/physiopathology/*surgery Heart Ventricles/abnormalities/physiopathology/*surgery Humans *Intelligence Longitudinal Studies Male Prospective Studies Treatment Outcome Ventricular Function Wechsler Scales IQ levels Neurodevelopment School age Single ventricle physiology Special education services LA - eng N1 - 1532-9488 Hiraiwa, Akiko Ibuki, Keijiro Tanaka, Tomomi Hirono, Keiichi Miya, Kazushi Yoshimura, Naoki Ichida, Fukiko Journal Article Video-Audio Media United States Semin Thorac Cardiovasc Surg. 2020 Summer;32(2):302-310. doi: 10.1053/j.semtcvs.2019.10.017. Epub 2019 Nov 4. PY - 2020 SN - 1043-0679 SP - 302-310 ST - Toddler Neurodevelopmental Outcomes Are Associated With School-Age IQ in Children With Single Ventricle Physiology T2 - Semin Thorac Cardiovasc Surg TI - Toddler Neurodevelopmental Outcomes Are Associated With School-Age IQ in Children With Single Ventricle Physiology VL - 32 ID - 2391 ER - TY - JOUR AB - Growing interest lies in the assessment of the metabolic status of patients with a univentricular circulation after Fontan operation, especially in changes of amino acid metabolism. Using targeted metabolomic examinations, we investigated amino acid metabolism in a homogeneous adult Fontan-patient group with a dominant left ventricle, seeking biomarker patterns that might permit better understanding of Fontan pathophysiology and early detection of subtle ventricular or circulatory dysfunction. We compared serum amino acid levels (42 analytes; AbsoluteIDQ p180 kit, Biocrates Life Sciences, Innsbruck, Austria) in 20 adult Fontan patients with a dominant left ventricle and those in age- and sex-matched biventricular controls. Serum concentrations of asymmetric dimethylarginine, methionine sulfoxide, glutamic acid, and trans-4-hydroxyproline and the methionine sulfoxide/methionine ratio (Met-SO/Met) were significantly higher and serum concentrations of asparagine, histidine, taurine, and threonine were significantly lower in patients than in controls. Met-SO/Met values exhibited a significant negative correlation with oxygen uptake during exercise. The alterations in amino acid metabolome that we found in Fontan patients suggest links between Fontan pathophysiology, altered cell energy metabolism, oxidative stress, and endothelial dysfunction like those found in biventricular patients with congestive heart failure. Studies of extended amino acid metabolism may allow better understanding of Fontan pathophysiology that will permit early detection of subtle ventricular or circulatory dysfunction in Fontan patients. AD - Department of Pediatrics III, Division of Pediatric Cardiology, Medical University of Innsbruck, Anichstraße 35, 6020, Innsbruck, Austria. miriam.michel@i-med.ac.at. Center of Pediatric Cardiology and Congenital Heart Disease, Heart and Diabetes Center North Rhine-Westphalia, Ruhr-University of Bochum, Georgstraße 11, 32545, Bad Oeynhausen, Germany. miriam.michel@i-med.ac.at. Center of Pediatric Cardiology and Congenital Heart Disease, Heart and Diabetes Center North Rhine-Westphalia, Ruhr-University of Bochum, Georgstraße 11, 32545, Bad Oeynhausen, Germany. Department of Pediatrics I, Division of Gastroenterology and Hepatology, Medical University of Innsbruck, Anichstraße 35, 6020, Innsbruck, Austria. Department of Pediatrics I, Division of Inherited Metabolic Disorders, Medical University of Innsbruck, Anichstraße 35, 6020, Innsbruck, Austria. Biocrates Life Sciences AG, Eduard-Bodem-Gasse 8, 6020, Innsbruck, Austria. Department of Pediatrics III, Division of Pediatric Cardiology, Medical University of Innsbruck, Anichstraße 35, 6020, Innsbruck, Austria. AN - 32488174 AU - Michel, M. AU - Dubowy, K. O. AU - Entenmann, A. AU - Karall, D. AU - Adam, M. G. AU - Zlamy, M. AU - Odri Komazec, I. AU - Geiger, R. AU - Niederwanger, C. AU - Salvador, C. AU - Müller, U. AU - Laser, K. T. AU - Scholl-Bürgi, S. C2 - PMC7265548 by a financial grant given to Dr. Michel from the “Tiroler Wissenschaftsförderung” (grant UNI-0404-2126). Dr. Michel has no further financial and no non-financial competing interests, or other interests that might be perceived to influence the results and/or discussion reported in this manuscript. All other authors declare no financial and no non-financial competing interests, or other interests that might be perceived to influence the results and/or discussion reported in this manuscript. DA - Jun 2 DB - UPubmed DO - 10.1038/s41598-020-65852-x DP - NLM ET - 2020/06/04 IS - 1 KW - Amino Acids/*blood/metabolism Case-Control Studies Coronary Circulation/physiology Female *Fontan Procedure/adverse effects Heart Defects, Congenital/blood/metabolism/surgery Humans Male Metabolomics Oxidative Stress Ventricular Dysfunction, Left/*blood/metabolism Young Adult LA - eng N1 - 2045-2322 Michel, Miriam Orcid: 0000-0002-3626-3270 Dubowy, Karl-Otto Entenmann, Andreas Karall, Daniela Adam, Mark Gordian Zlamy, Manuela Orcid: 0000-0003-3822-933x Odri Komazec, Irena Geiger, Ralf Niederwanger, Christian Salvador, Christina Müller, Udo Laser, Kai Thorsten Scholl-Bürgi, Sabine Journal Article Research Support, Non-U.S. Gov't Sci Rep. 2020 Jun 2;10(1):8930. doi: 10.1038/s41598-020-65852-x. PY - 2020 SN - 2045-2322 SP - 8930 ST - Targeted metabolomic analysis of serum amino acids in the adult Fontan patient with a dominant left ventricle T2 - Sci Rep TI - Targeted metabolomic analysis of serum amino acids in the adult Fontan patient with a dominant left ventricle VL - 10 ID - 2368 ER - TY - JOUR AB - Background Management of patients with hypoplastic left heart syndrome has benefited from advancements in medical and surgical care. Outcomes have improved, although survival and long-term functional and cognitive deficits remain a concern. Methods and Results This is a cohort study of all consecutive patients with hypoplastic left heart syndrome undergoing surgical palliation at a single center. We aimed to examine demographic and perioperative factors from each surgical stage for their association with survival and neurocognitive outcomes. A total of 117 consecutive patients from 1996 to 2010 underwent surgical palliation. Seventy patients (60%) survived to the Fontan stage and 68 patients (58%) survived to undergo neurocognitive assessment at a mean (SD) age of 56.6 months (6.4 months). Full-scale, performance, and verbal intelligence quotient, as well as visual-motor integration mean (SD) scores were 86.7 (16.1), 86.3 (15.8), 88.8 (17.2), and 83.2 (14.8), respectively. On multivariable analysis, older age at Fontan, sepsis peri-Norwood, lowest arterial partial pressure of oxygen postbidirectional cavopulmonary anastomosis, and presence of neuromotor disability pre-Fontan were strongly associated with lower scores for all intelligence quotient domains. Older age at Fontan and sepsis peri-Norwood remained associated with lower scores for all intelligence quotient domains in a subgroup analysis excluding patients with disability pre-Fontan or with chromosomal abnormalities. Conclusions Older age at Fontan and sepsis are among independent predictors of poor neurocognitive outcomes for patients with hypoplastic left heart syndrome. Further studies are required to identify the appropriate age range for Fontan completion, balancing a lower risk of acute and long-term hemodynamic complications while optimizing long-term neurocognitive outcomes. AD - Department of Pediatrics University of Alberta Edmonton Alberta Canada. Glenrose Rehabilitation Hospital Edmonton Alberta Canada. Department of Medicine University of Alberta Edmonton Alberta Canada. Department of Pediatrics and Child Health University of Manitoba Winnipeg Manitoba Canada. Department of Surgery University of Alberta Edmonton Alberta Canada. AN - 32067591 AU - Atallah, J. AU - Garcia Guerra, G. AU - Joffe, A. R. AU - Bond, G. Y. AU - Islam, S. AU - Ricci, M. F. AU - AlAklabi, M. AU - Rebeyka, I. M. AU - Robertson, C. M. T. C2 - PMC7070198 DA - Feb 18 DB - UPubmed DO - 10.1161/jaha.119.013632 DP - NLM ET - 2020/02/19 IS - 4 KW - Age Factors *Blalock-Taussig Procedure/adverse effects/mortality Child *Child Behavior *Child Development Child, Preschool *Cognition Databases, Factual *Fontan Procedure/adverse effects/mortality Humans Hypoplastic Left Heart Syndrome/mortality/physiopathology/psychology/*surgery Infant Infant, Newborn Intelligence Nervous System/*growth & development *Norwood Procedures/adverse effects/mortality *Palliative Care Risk Assessment Risk Factors Time Factors Treatment Outcome *Fontan procedure *Glenn procedure *Norwood procedure *cognitive *mortality *neurodevelopment *single ventricle LA - eng N1 - 2047-9980 Atallah, Joseph Garcia Guerra, Gonzalo Joffe, Ari R Bond, Gwen Y Islam, Sunjidatul Ricci, M Florencia AlAklabi, Mohammed Rebeyka, Ivan M Robertson, Charlene M T Western Canadian Complex Pediatric Therapies Follow‐up Program* Journal Article Observational Study Research Support, Non-U.S. Gov't J Am Heart Assoc. 2020 Feb 18;9(4):e013632. doi: 10.1161/JAHA.119.013632. Epub 2020 Feb 11. PY - 2020 SN - 2047-9980 SP - e013632 ST - Survival, Neurocognitive, and Functional Outcomes After Completion of Staged Surgical Palliation in a Cohort of Patients With Hypoplastic Left Heart Syndrome T2 - J Am Heart Assoc TI - Survival, Neurocognitive, and Functional Outcomes After Completion of Staged Surgical Palliation in a Cohort of Patients With Hypoplastic Left Heart Syndrome VL - 9 ID - 2381 ER - TY - JOUR AB - INTRODUCTION: Surveillance and management guidelines for Fontan patients are lacking due to the paucity of evidence in the literature of screening efficacy on outcome measures. METHODS: The Fontan Working Group within the New England Congenital Cardiology Association designed an electronic survey to assess surveillance practices for patients with Fontan procedures among New England congenital cardiologists and to explore variability in screening low-risk vs high-risk Fontan patients across regional programs. RESULTS: Fifty-six cardiologists representing 12 regional programs responded to the survey, comprising ~40% of the total New England congenital cardiac physicians. The majority of desired testing and consultation was available within 50 miles of the patient's home institution with some limitations of cardiac catheterization and cardiac magnetic resonance imaging availability. Surveillance and screening were less frequent in low-risk Fontan patients compared to high-risk Fontan patients. Counseling practices were similar for both low-risk and high-risk Fontan patients. Aspirin monotherapy was recommended by 82% of providers for low-risk Fontan patients, while anticoagulation regimens were more varied for the high-risk population. Practitioners with ≤15 years of experience were more likely to provide quality of life testing in both low-risk and high-risk Fontan patients. There were no other major differences in testing frequencies by years of practice, quaternary vs nonquaternary care facility, or the number of Fontan patients in a practice. CONCLUSION: This survey provides insight into regional practices of screening and surveillance of Fontan patients. These data may be used to design future research studies and evidence-based guidelines to streamline the approach to manage these complex patients. AD - Connecticut Children's, Hartford, Connecticut. Boston Children's Hospital, Boston, Massachusetts. Children's Hospital at Dartmouth-Hitchcock, Lebanon, New Hampshire. Yale-New Haven Children's Hospital, New Haven, Connecticut. Child Heart Associates, Worcester, Massachusetts. AN - 31642600 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. DA - Nov DB - UPubmed DO - 10.1111/chd.12854 DP - NLM ET - 2019/10/24 IS - 6 KW - Adolescent Adult Cardiologists/*trends Child Child, Preschool *Fontan Procedure/adverse effects Health Care Surveys Health Services Accessibility/trends Healthcare Disparities/*trends Heart Defects, Congenital/diagnosis/physiopathology/*surgery Heart Function Tests/*trends Humans New England Practice Patterns, Physicians'/*trends Predictive Value of Tests Referral and Consultation/trends Risk Factors Treatment Outcome Young Adult Fontan congenital heart disease screening single ventricle survey LA - eng N1 - 1747-0803 Davey, Brooke T Toro-Salazar, Olga H Gauthier, Naomi Valente, Anne Marie Elder, Robert W Wu, Fred M Berman, Norman Pollack, Phyllis Lee, Ji Hyun Rathod, Rahul H 2017-2/New England Congenital Cardiology Research Foundation/ Journal Article United States Congenit Heart Dis. 2019 Nov;14(6):1013-1023. doi: 10.1111/chd.12854. Epub 2019 Oct 23. PY - 2019 SN - 1747-079x SP - 1013-1023 ST - Surveillance and screening practices of New England congenital cardiologists for patients after the Fontan operation T2 - Congenit Heart Dis TI - Surveillance and screening practices of New England congenital cardiologists for patients after the Fontan operation VL - 14 ID - 2395 ER - TY - JOUR AB - Peripheral pulmonary artery stenosis is a relatively rare form of congenital heart disease typically associated with genetic syndromes, such as Williams or Alagille syndromes. However, some patients present with severe stenosis without associated syndromes. The purpose of the study was to review our surgical experience in such patients. This was a retrospective review of 30 patients who underwent surgical repair for peripheral pulmonary artery stenosis. Concomitant anatomical diagnoses in 20 patients (67%) included supravalvar aortic stenosis (n = 8), tetralogy of Fallot (n = 4), d-transposition of the great arteries (n = 2), truncus arteriosus (n = 2), hypoplastic left heart syndrome (n = 2), ventricular septal defect (n = 1), and patent ductus arteriosus (n = 1). Additional medical diagnoses in 15 patients (50%) included elastin arteriopathy (n = 9), pulmonary artery calcinosis (n = 1), arterial tortuosity syndrome (n = 1), DiGeorge syndrome (n = 1), and Noonan syndrome (n = 1). Median age at surgery was 3.6 years (interquartile range 1.6–7.4 years). Seventeen patients (57%) had prior cardiac operations, and 16 patients (53%) previously underwent percutaneous intervention. With surgery, mean right ventricle-to-aortic systolic pressure ratio decreased from 0.95 ± 0.2 to 0.28 ± 0.08 (P< 0.0001). Median duration of cardiopulmonary bypass was 369 minutes. There was 100% survival to hospital discharge, with no mortality at mean follow-up of 2.3 years. No patient required reoperation, while 4 underwent balloon dilation. Freedom from pulmonary artery catheter-based reintervention was 95% and 80% at 12 and 36 months, respectively. Patients with peripheral pulmonary artery stenosis without either Williams or Alagille syndrome can successfully undergo surgical repair with a significant reduction in right ventricle-to-aortic pressure ratios. AD - R.D. Mainwaring, Stanford University School of Medicine, 300 Pasteur Drive, Falk CVRC, Stanford, CA, United States AU - Martin, E. AU - Mainwaring, R. D. AU - Collins, R. T. AU - MacMillen, K. L. AU - Hanley, F. L. DB - OEmbase DO - 10.1053/j.semtcvs.2020.01.003 KW - Alagille syndrome aortic pressure artery disease balloon dilatation calcinosis cardiopulmonary bypass child clinical article conference paper congenital heart disease controlled study DiGeorge syndrome Fallot tetralogy female follow up great vessels transposition heart right ventricle heart ventricle septum defect hospital discharge human hypoplastic left heart syndrome male mortality Noonan syndrome patent ductus arteriosus preschool child pulmonary artery catheter pulmonary artery stenosis pulmonary valve reoperation retrospective study supravalvular aortic stenosis surgery survival systolic blood pressure Williams Beuren syndrome elastin endogenous compound LA - English M3 - Article in Press N1 - L2004926229 2020-02-24 PY - 2020 SN - 1532-9488 1043-0679 ST - Surgical Repair of Peripheral Pulmonary Artery Stenosis in Patients Without Williams or Alagille Syndromes T2 - Seminars in Thoracic and Cardiovascular Surgery TI - Surgical Repair of Peripheral Pulmonary Artery Stenosis in Patients Without Williams or Alagille Syndromes UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2004926229 http://dx.doi.org/10.1053/j.semtcvs.2020.01.003 ID - 3487 ER - TY - JOUR AB - We present a 31 gestational weeks' premature baby whose fetal echocardiogram showed ectopia cordis, single ventricle and severe pulmonary stenosis. At 31 gestational weeks, an emergency caesarean section was performed, and his birth weight was 1756 g, SpO2 was 80% on 100% O2. Epicardial echocardiogram showed double inlet right ventricle, severe valvular pulmonary stenosis and no ductus arteriosus. The risk of surgery was very high, so we decided to perform balloon valvuloplasty by direct puncture of the heart. We punctured the apex of the ventricle using a 16-gauge needle under echoguidance, advanced the guidewire to the pulmonary artery and performed balloon valvuloplasty. Soon after the procedure, the cyanosis improved dramatically. This is the first report of a transcatheter procedure performed by direct puncture of the heart for ectopia cordis with complex congenital heart disease. © 2020 The Author(s). Published by Cambridge University Press. AD - Department of Cardiology, Kobe Children's Hospital, 1-6-7, Minatojima-minamimachi, Chuo-ku, Kobe, Hyogo 650-0047, Japan AU - Tanaka, T. AU - Kubo, S. AU - Kamei, N. DB - UScopus DO - 10.1017/S1047951120002863 IS - 12 KW - balloon valvuloplasty direct puncture of the heart ectopia cordis Pentalogy of Cantrell pulmonary stenosis M3 - Article N1 - Export Date: 10 May 2021 PY - 2020 SP - 1951-1953 ST - Successful balloon valvuloplasty using direct puncture of the heart for pentalogy of Cantrell with complete ectopia cordis, low birth weight, single ventricle and severe pulmonary stenosis T2 - Cardiology in the Young TI - Successful balloon valvuloplasty using direct puncture of the heart for pentalogy of Cantrell with complete ectopia cordis, low birth weight, single ventricle and severe pulmonary stenosis UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85093981969&doi=10.1017%2fS1047951120002863&partnerID=40&md5=c2ccc098e5be293909f7801af4287858 VL - 30 ID - 5881 ER - TY - JOUR AB - Background: Stress hyperglycemia (SH) is a common finding in patients in pediatric emergency departments (PED) and has been related to increased morbidity and mortality. Objectives: To assess the incidence of SH among children visiting the PED. To identify which diseases predispose patients to SH and whether they indicate a worse outcome. Methods: Data were collected retrospectively from the medical records of all children aged 0-18 years who visited the PED during the years 2010-20 14 and who had a glucose level of > 150 mg/dl. Data collected included age, gender, weight, blood glucose level, presence or absence of a pre-existing or a new diagnosis of diabetes mellitus, and previous treatment with medications affecting blood glucose levels or with intravenous fluids containing dextrose. Data were collected regarding hospitalization, duration of hospitalization, discharge diagnosis, and survival status. Results: The study population included 1245 children with SH, which comprised 2.6% of all patients whose blood glucose level was measured in the PED during the study period. The mean age of children with SH was 49 months; 709 (56.9%) were male. The mean blood glucose level was 184 mg/dl. The rate of hospitalization was 57.8%. The mean duration of hospital stay was 5.6 days and mortality rate was 0.96%. The majority were diagnosed with a respiratory illness. Conclusions: SH is a common phenomenon among children evaluated in the PED and is associated with a high incidence of hospitalization. It may serve as an additional clinical indicator of disease severity. AD - F. Levy-Khademi, Dept. of Pediatrics, Shaare Zedek Medical Center, Jerusalem, Israel AU - Levmore-Tamir, M. AU - Weiser, G. AU - Berliner, E. AU - Erlichman, M. AU - Ziv, C. A. AU - Levy-Khademi, F. DB - UEmbase IS - 7 KW - Vitros 5.1 beta adrenergic receptor stimulating agent corticosteroid epinephrine glucose oxidase abdominal pain allergic reaction article asthma autoimmune disease bronchiolitis burn child child hospitalization dehydration diabetes mellitus disease severity diseases emergency ward faintness female gastroenteritis gastrointestinal disease glucose blood level heart arrhythmia heart disease hospital discharge human hyperglycemia hypoplastic left heart syndrome hypovolemic shock infection injury kidney disease larynx disorder length of stay major clinical study male mortality rate neurologic disease outcome assessment pediatric intensive care unit pediatric ward pneumonia preschool child respiratory tract disease resuscitation retrospective study seizure thorax pain urinary tract infection wheezing LA - English M3 - Article N1 - L2007535536 2020-10-08 2020-10-19 PY - 2020 SN - 1565-1088 SP - 365-368 ST - Stress hyperglycemia in a pediatric emergency department setting and its relationship to the underlying disease and outcome T2 - Israel Medical Association Journal TI - Stress hyperglycemia in a pediatric emergency department setting and its relationship to the underlying disease and outcome UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2007535536&from=export VL - 22 ID - 4405 ER - TY - JOUR AB - and purpose Static balloon atrial septostomy is a widely accepted intervention for children with CHD. Successful surgical palliation is creating increasing numbers of adult CHD patients who need subsequent left heart intervention requiring transseptal access. In these patients, the interatrial septum is usually thick and fibrotic because of a previous open heart surgery or catheter intervention, and conventional transseptal puncture may be unsuccessful. Static balloon atrial septostomy to access the left atrium may facilitate intervention via the interatrial septum in such situations. The purpose of this study was to investigate the usefulness and the safety of static balloon atrial septostomy, and the evolution of an iatrogenic atrial septal defect post procedure in adult CHD.Methods We retrospectively reviewed six procedures in five adults with CHD and collected demographic characteristics, details of the procedures, clinical outcome, and size changes of the iatrogenic atrial septal defect.Results The mean age at the time of the procedure was 35 years. The intended primary interventions were pulmonary vein isolation, stenting for pulmonary vein obstruction, and catheter ablation for focal atrial tachycardia. All static balloon atrial septostomies were effective, and the left heart interventions were successfully achieved via transseptal sheaths. There were no major complications associated with the static balloon atrial septostomy. There were no adverse clinical outcomes related to iatrogenic atrial septal defect, and the size of the defects regressed over time in all cases.Conclusions Static balloon atrial septostomy can be a safe and useful technique in adult CHD patients needing left heart procedures. The thick interatrial septum found in postoperative patients may reduce the risk of persistent iatrogenic atrial septal defect. AD - T. Fujii, Cardiovascular Center, Showa University Northern Yokohama Hospital, 35-1 Chigasakichuo, Tuzuki-ku, Yokohama, Japan AU - Fujii, T. AU - Tomita, H. AU - Hata, Y. AU - Sasaki, T. AU - Asada, D. AU - Tarui, S. AU - Miyahara, Y. AU - Ishino, K. AU - Soga, T. DB - OEmbase DO - 10.1017/S1047951118001063 IS - 10 KW - balloon catheter Conquest balloon Jackal balloon adult age distribution article cardiovascular procedure catheter ablation clinical article clinical effectiveness clinical outcome congenital heart disease demography disease severity human pulmonary vein isolation pulmonary vein obstruction retrospective study risk reduction safety static balloon atrial septostomy supraventricular tachycardia Sterling balloon LA - English M3 - Article N1 - L623048724 2018-07-20 2019-04-17 PY - 2018 SN - 1467-1107 1047-9511 SP - 1116-1121 ST - Static balloon atrial septostomy for the purpose of left heart intervention in postoperative adult CHD T2 - Cardiology in the Young TI - Static balloon atrial septostomy for the purpose of left heart intervention in postoperative adult CHD UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L623048724 http://dx.doi.org/10.1017/S1047951118001063 VL - 28 ID - 3574 ER - TY - JOUR AB - Objectives: In-hospital complications after the Norwood operation for single ventricle heart defects account for the majority of morbidity and mortality. Inpatient care variation occurs within and across centers. This multidisciplinary quality improvement project standardized perioperative management in a large referral center. Design: Quality improvement project. Setting: High volume cardiac center, tertiary care children's hospital. Patients: Neonates undergoing Norwood operation. Interventions: The quality improvement team developed and implemented a clinical guideline (preoperative admission to 48 hr after surgery). The composite process metric, Guideline Adherence Score, contained 13 recommendations in the guideline that reflected consistent care for all patients. Measurements and Main Results: One-hundred two consecutive neonates who underwent Norwood operation (January 1, 2013, to July 12, 2016) before guideline implementation were compared with 50 consecutive neonates after guideline implementation (July 13, 2016, to May 4, 2018). No preguideline operations met the goal Guideline Adherence Score. In the first 6 months after guideline implementation, 10 of 12 operations achieved goal Guideline Adherence Score and continued through implementation, reaching 100% for the last 10 operations. Statistical process control analysis demonstrated less variability and decreased hours of postoperative mechanical ventilation and cardiac ICU length of stay during implementation. There were no statistically significant differences in major hospital complications or in 30-day mortality. A higher percentage of patients were extubated by postoperative day 2 after guideline implementation (67% [30/47] vs 41% [41/99], respectively; p = 0.01). Of these patients, reintubation within 72 hours of extubation significantly decreased after guideline implementation (0% [0/30] vs 17% [7/41] patients, respectively; p = 0.02). Conclusions: This initiative successfully implemented a standardized perioperative care guideline for neonates undergoing the Norwood operation at a large center. Positive statistical process control centerline shifts in Guideline Adherence Score, length of postoperative mechanical ventilation, and cardiac ICU length of stay were demonstrated. A higher percentage were successfully extubated by postoperative day 2. Establishment of standard processes can lead to best practices to decrease major adverse events. AD - S.S. Natarajan, Department of Pediatrics, University of Pennsylvania, Children's Hospital of Philadelphia, Philadelphia, PA, United States AU - Natarajan, S. S. AU - Stagg, A. AU - Taylor, A. M. AU - Griffis, H. M. AU - Bosler, C. K. AU - Cates, M. AU - Dewitt, A. G. AU - Giglia, T. M. AU - Mascio, C. E. AU - Ravishankar, C. AU - Rossano, J. W. AU - Taylor, L. C. AU - Ware, E. P. AU - Nicolson, S. C. AU - Rome, J. J. DB - UEmbase DO - 10.1097/PCC.0000000000002478 KW - article artificial ventilation child complication controlled study coronary care unit extubation female heart center human hypoplastic left heart syndrome length of stay major clinical study male mortality newborn Norwood procedure perioperative period practice guideline process control protocol compliance standardization tertiary health care total quality management LA - English M3 - Article in Press N1 - L633004609 2020-10-07 PY - 2020 SN - 1947-3893 1529-7535 SP - E848-E857 ST - Standardization of the perioperative management for neonates undergoing the norwood operation for hypoplastic left heart syndrome and related heart defects T2 - Pediatric Critical Care Medicine TI - Standardization of the perioperative management for neonates undergoing the norwood operation for hypoplastic left heart syndrome and related heart defects UR - https://www.embase.com/search/results?subaction=viewrecord&id=L633004609&from=export http://dx.doi.org/10.1097/PCC.0000000000002478 ID - 4451 ER - TY - JOUR AB - The aim of the study was to identify and correlate the anatomical variants of cardiac structures among patients with heterotaxy. In this retrospective cross-sectional analysis of 13 years duration, 302 patients of congenital heart diseases associated with heterotaxy were studied. All these patients had undergone a meticulous clinical evaluation, echocardiography, and cardiac computed tomography. The mean age of the cohort was 38.4 months, with 180 males and 122 females. The cohort had 184 patients of right isomerism and 118 of left isomerism. More than half of the cohort had abnormal pulmonary veins. Over 75% of the cohort had low pulmonary blood flow. Abnormal relationship of great arteries was seen in nearly 69% of the cohort. Atrio-venticular canal defect was the commonest anatomical variant. Overall, 43% of the cohort had single ventricle physiology, predominantly associated with right isomerism. Heterotaxy forms a difficult anatomical subset to comprehend due to the plethora of possible abnormalities. However, unless the cardiac and visceral anatomy is delineated well, the surgical plan becomes suboptimal and often elusive, leaving tough choices for cardiac surgeon. It is essential to obtain all the possible anatomical information using additional imaging modalities to devise a basis for a comprehensive plan for medical and surgical management. A better understanding of the genetic and molecular factors in the etiology, coupled with the application of state-of-the-art imaging techniques, is likely to add to our knowledge of heterotaxy to bring about improved surgical outcomes and a better quality of life for patients suffering from this complex entity. AD - Departments of Paediatric Cardiology, Narayana Institute Of Cardiovascular Sciences, Narayana Health City, 258/A, Bommasandra, Hosur Road, Bangalore, 560099, India. drkiranvs@gmail.com. Departments of Paediatric Cardiology, Narayana Institute Of Cardiovascular Sciences, Narayana Health City, 258/A, Bommasandra, Hosur Road, Bangalore, 560099, India. Departments of Radiodiagnosis, Narayana Institute of Cardiovascular Sciences, Narayana Health City, 258/A, Bommasandra, Hosur Road, Bangalore, 560099, India. AN - 32556488 AU - Kiran, V. S. AU - Choudhary, S. AU - Shaik, A. AU - Gadabanahalli, K. AU - Raj, V. AU - Bhat, V. DA - Oct DB - UPubmed DO - 10.1007/s00246-020-02402-2 DP - NLM ET - 2020/06/20 IS - 7 KW - Child, Preschool Cross-Sectional Studies Female Heterotaxy Syndrome/diagnostic imaging/*pathology Humans Male Pulmonary Veins/abnormalities Quality of Life Retrospective Studies Tomography, X-Ray Computed/*methods Asplenia Heterotaxy Isomerism Polysplenia Situs ambiguus LA - eng N1 - 1432-1971 Kiran, Viralam S Orcid: 0000-0002-9576-8076 Choudhary, Sandeep Shaik, Amira Gadabanahalli, Karthik Raj, Vimal Bhat, Venkatraman Journal Article United States Pediatr Cardiol. 2020 Oct;41(7):1414-1424. doi: 10.1007/s00246-020-02402-2. Epub 2020 Jun 18. PY - 2020 SN - 0172-0643 SP - 1414-1424 ST - The Spectrum of Cardiac Anomalies Associated with Heterotaxy: A Single-Center Study of a Large Series Based on Computed Tomography T2 - Pediatr Cardiol TI - The Spectrum of Cardiac Anomalies Associated with Heterotaxy: A Single-Center Study of a Large Series Based on Computed Tomography VL - 41 ID - 2366 ER - TY - JOUR AB - BACKGROUND: Low socioeconomic status (SES) has emerged as an important risk factor for higher short-term mortality and neurodevelopmental outcomes in children with hypoplastic left heart syndrome and related anomalies; yet little is known about how SES affects these outcomes over the long-term. METHODS: We linked data from the Single Ventricle Reconstruction trial to US Census Bureau data to analyze the relationship of neighborhood SES tertiles with mortality and transplantation, neurodevelopment, quality of life, and functional status at 5 and 6 years post-Norwood procedure (N = 525). Cox proportional hazards regression and linear regression were used to assess the association of SES with mortality and neurodevelopmental outcomes, respectively. RESULTS: Patients in the lowest SES tertile were more likely to be racial minorities, older at stage 2 and Fontan procedures, and to have more complications and fewer cardiac catheterizations over follow-up (all P < .05) compared with patients in higher SES tertiles. Unadjusted mortality was highest for patients in the lowest SES tertile and lowest in the highest tertile (41% vs 29%, respectively; log-rank P = .027). Adjustment for patient birth and Norwood factors attenuated these differences slightly (P = .055). Patients in the lowest SES tertile reported lower functional status and lower fine motor, problem-solving, adaptive behavior, and communication skills at 6 years (all P < .05). These differences persisted after adjustment for baseline and post-Norwood factors. Quality of life did not differ by SES. CONCLUSIONS: Among patients with hypoplastic left heart syndrome, those with low SES have worse neurodevelopmental and functional status outcomes at 6 years. These differences were not explained by other patient or clinical characteristics. AD - Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts. Department of Pediatrics, Harvard Medical School, Harvard University, Boston, Massachusetts. Department of Pediatrics, University of Michigan and C.S. Mott Children's Hospital, Ann Arbor, Michigan. Division of Pediatric Cardiology, Department of Pediatrics, Columbia University Irving Medical Center and NewYork-Presbyterian Morgan Stanley Children's Hospital, New York City, New York. Division of Pediatric Cardiac Surgery, Cardiac Center, Children's Hospital of Pennsylvania, Philadelphia, Pennsylvania; and. Department of Cardiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts; jane.newburger@cardio.chboston.org. AN - 32973120 AU - Bucholz, E. M. AU - Sleeper, L. A. AU - Goldberg, C. S. AU - Pasquali, S. K. AU - Anderson, B. R. AU - Gaynor, J. W. AU - Cnota, J. F. AU - Newburger, J. W. C2 - PMC7546087 conflicts of interest to disclose. DA - Oct DB - UPubmed DO - 10.1542/peds.2020-1240 DP - NLM ET - 2020/09/26 IS - 4 KW - Cardiac Catheterization/statistics & numerical data Child Child Development Child, Preschool Developmental Disabilities/epidemiology Educational Status Female Fontan Procedure/*methods/mortality/statistics & numerical data Heart Transplantation/statistics & numerical data Humans Hypoplastic Left Heart Syndrome/complications/ethnology/mortality/*surgery Income Infant, Newborn Male Occupations Postoperative Complications/epidemiology Proportional Hazards Models Quality of Life Residence Characteristics *Social Class Treatment Outcome Univentricular Heart/mortality/surgery LA - eng N1 - 1098-4275 Bucholz, Emily M Sleeper, Lynn A Goldberg, Caren S Pasquali, Sara K Anderson, Brett R Gaynor, J William Cnota, James F Newburger, Jane W U10 HL068270/HL/NHLBI NIH HHS/United States Journal Article Multicenter Study Randomized Controlled Trial Research Support, N.I.H., Extramural Pediatrics. 2020 Oct;146(4):e20201240. doi: 10.1542/peds.2020-1240. PY - 2020 SN - 0031-4005 (Print) 0031-4005 ST - Socioeconomic Status and Long-term Outcomes in Single Ventricle Heart Disease T2 - Pediatrics TI - Socioeconomic Status and Long-term Outcomes in Single Ventricle Heart Disease VL - 146 ID - 2354 ER - TY - JOUR AB - OBJECTIVES: To determine if neurodevelopmental deficits in children with single-ventricle abstract physiology change with age and early developmental scores predict 6-year outcomes. METHODS: In the Single Ventricle Reconstruction Trial, Bayley Scales of Infant Development, Second Edition, were administered at 14 months of age, and parents completed the Behavior Assessment System for Children, Second Edition (BASC-2) annually from the ages of 2 to 6 years. Scores were classified as average, at risk, or impaired. We calculated sensitivities, specificities, and positive and negative predictive values of earlier tests on 6-year outcomes. RESULTS: Of 291 eligible participants, 244 (84%) completed the BASC-2 at 6 years; more Single Ventricle Reconstruction participants than expected on the basis of normative data scored at risk or impaired on the BASC-2 Adaptive Skills Index at that evaluation (28.7% vs 15.9%; P,.001). Children with Adaptive Skills Composite scores,2 SD below the mean at the age of 6 were more likely to have had delayed development at 14 months, particularly on the Psychomotor Development Index (sensitivity of 79%). However, the positive predictive value of the 14-month Mental Development Index and Psychomotor Development Index for 6-year BASC-2 Adaptive Scores was low (44% and 36%, respectively). Adaptive Skills Composite score impairments at the age of 6 were poorly predicted by using earlier BASC-2 assessments, with low sensitivities at the ages of 3 (37%), 4 (48%), and 5 years (55%). CONCLUSIONS: Many children with hypoplastic left heart syndrome who have low adaptive skills at the age of 6 years will not be identified by screening at earlier ages. With our findings, we highlight the importance of serial evaluations for children with critical congenital heart disease throughout development. AD - R. Sananes, Department of Psychology, Hospital for Sick Children, 555 University Ave, Toronto, ON, Canada AU - Sananes, R. AU - Goldberg, C. S. AU - Newburger, J. W. AU - Hu, C. AU - Trachtenberg, F. AU - Gaynor, J. W. AU - Mahle, W. T. AU - Miller, T. AU - Uzark, K. AU - Mussatto, K. A. AU - Pizarro, C. AU - Jacobs, J. P. AU - Cnota, J. AU - Atz, A. M. AU - Lai, W. W. AU - Burns, K. M. AU - Milazzo, A. AU - Votava-Smith, J. AU - Brosig, C. L. DB - UEmbase DO - 10.1542/peds.2020-014589 IS - 2 KW - article Bayley Scales of Infant Development behavior assessment child controlled study diagnostic test accuracy study female heart single ventricle human hypoplastic left heart syndrome infant major clinical study male predictive value preschool child psychomotor development sensitivity and specificity skill surgery LA - English M3 - Article N1 - L2011108092 2021-03-02 PY - 2021 SN - 1098-4275 0031-4005 ST - Six-year neurodevelopmental outcomes for children with single-ventricle physiology T2 - Pediatrics TI - Six-year neurodevelopmental outcomes for children with single-ventricle physiology UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2011108092&from=export http://dx.doi.org/10.1542/peds.2020-014589 VL - 147 ID - 4356 ER - TY - JOUR AB - Objective: We employed echocardiographic tissue characterization for the assessment of single ventricle systolic function and examined its relationship with cardiopulmonary exercise testing (CPET) performance in the Fontan circulation. Methods: 40 consecutive adult Fontan patients (16 female, 24 male; mean age 26.3 ± 5.9) were enrolled in our prospective cross-sectional study. All patients underwent echocardiographic assessment including tissue characterization and CPET within 3 days from each other. Results: LAPSE (lateral annular plane systolic excursion) was higher in patients with a dominant left ventricle compared to a dominant right ventricle (12.3 + −2.6 vs 9.8 + −1.1 mm, p =.008) and in patients with AP (right atrium-pulmonary artery) connection than with lateral tunnel or extracardiac conduits (13.9 ± 1.7 vs 11.3 ± 2.6 and 10.9 ± 2.2 mm, p =.014 and p =.014, respectively). Maximal oxygen pulse (O2 pulse max) was significantly higher in patients with a dominant left or common ventricle compared to patients with a dominant right ventricle (10.3 and 12.0 vs 6.6 ml/beat, p =.002 and p =.032, respectively). There was a positive correlation between O2 pulse max and LAPSE (r = 0.442, p =.009), atrioventricular valve annulus systolic velocity (S′) (r = 0.606, p <.0001), lateral wall basal segment systolic velocity (bas vel s) (r = 0.469, p =.009) and lateral wall mid segment systolic velocity (mid vel s) (r = 0.452, p =.012). In the multivariable regression model, S′ (r = 0.606, p <.0001) remained predictive of O2 pulse max. Conclusions: LAPSE and S′ are suitable parameters for the assessment of systolic function in Fontan patients. Subclinical systolic dysfunction of longitudinal fibers is associated with worse CPET performance. O2 pulse max seems to be the best parameter reflecting Fontan patients' physical performance. AD - P. Dobrowolski, Adult Congenital Heart Disease Department, Institute of Cardiology, Alpejska 42 Street, Warsaw, Poland AU - Rybicka, J. AU - Dobrowolski, P. AU - Marczak, M. AU - Niewiadomska, J. AU - Kowalczyk, M. AU - Hoffman, P. AU - Kowalski, M. DB - UEmbase DO - 10.1016/j.ijcard.2020.06.024 KW - EchoPac BT09 version mask Oxycon Pro real time ultrasound scanner treadmill ultrasound transducer Vivid 9 ZAN Ergo 600 adolescent adult article atriopulmonary connection atrioventricular valve annulus systolic velocity cardiopulmonary exercise test cardiovascular parameters child clinical article common ventricle congenital heart malformation controlled study cross-sectional study dominant left ventricle dominant right ventricle extracardiac conduit female Fontan procedure heart single ventricle heart ventricle contraction human human tissue lateral annular plane systolic excursion lateral tunnel lateral wall basal segment systolic velocity lateral wall mid segment systolic velocity male maximal oxygen pulse physical performance priority journal prospective study speckle tracking echocardiography systolic dysfunction task performance tissue characterization transthoracic echocardiography LA - English M3 - Article N1 - L2007063781 2020-07-23 2020-11-17 PY - 2020 SN - 1874-1754 0167-5273 SP - 75-80 ST - Single ventricle systolic function and cardiopulmonary exercise performance in adult patients with Fontan circulation T2 - International Journal of Cardiology TI - Single ventricle systolic function and cardiopulmonary exercise performance in adult patients with Fontan circulation UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2007063781&from=export http://dx.doi.org/10.1016/j.ijcard.2020.06.024 VL - 321 ID - 4374 ER - TY - JOUR AB - This is a newborn male prenatally diagnosed with severe right ventricular (RV) hypertrophy and depressed function, aneurysmal dilation of the main pulmonary artery and tachyarrhythmia. Postnatally, he required immediate intuba-tion and inotropic support. Echocardiogram revealed a large dysplastic RV, absent pulmonary valve syndrome (APVS), markedly dilated pulmonary arteries and tri-cuspid atresia (TA). The trabecular portion of the RV was excessively trabeculated and severely dilated. Inflow and infundibular walls were thin, with multiple infun-dibular aneurysms. There was APVS with free regurgitation and massively dilated pulmonary arteries. The RV bulged into the LV, though there was no outflow tract obstruction. The LV had mildly depressed systolic function. Computed tomogra-phy angiography showed marked dilation of the main and branch pulmonary arteries, with compression of the airway. This, along with profound anasarca, pro-hibited weaning of ventilatory support. Ventricular tachycardia contributed to low cardiac output. Genetic testing revealed a heterozygous variant in the desmopla-kin (DSP) gene, which is associated with familial arrhythmogenic RV dysplasia and dilated cardiomyopathy. The parents opted to withdraw care. Severe RV dys-plasia associated with APVS and TA has previously been reported, however the degree of RV dilation with primitive myocardium in this case is profound. Further, presence of both fetal and postnatal ventricular tachycardia contributing to low cardiac is a novel presentation. This demonstrates that the overall poor prognosis was multifactorial. AD - K. Parikh, Nicklaus Children’s Hospital, Department of Cardiology 2ndFloor, Miami, FL, United States K. Parikh, Herbert Wertheim School of Medicine, Florida International University, Miami, FL, United States AU - Parikh, K. AU - Muniz, J. C. AU - Welch, E. AU - Aldousany, A. AU - Sasaki, N. DB - UEmbase DO - 10.32604/CHD.2020.012910 IS - 5 KW - airway anasarca aneurysm angiography article assisted ventilation canine tooth compression congestive cardiomyopathy echocardiography fetus forward heart failure genetic association genetic screening genetic susceptibility heart right ventricle dysplasia heart right ventricle hypertrophy heart ventricle tachycardia heterozygosity human inotropism male newborn obstruction prognosis pulmonary artery pulmonary valve tricuspid valve atresia weaning LA - English M3 - Article N1 - L2006154594 2021-03-10 PY - 2020 SN - 1747-0803 1747-079X SP - 339-346 ST - Severe right ventricular dysplasia with absent pulmonary valve syndrome and tricuspid atresia: A literature review T2 - Congenital Heart Disease TI - Severe right ventricular dysplasia with absent pulmonary valve syndrome and tricuspid atresia: A literature review UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2006154594&from=export http://dx.doi.org/10.32604/CHD.2020.012910 VL - 15 ID - 4443 ER - TY - JOUR AB - Fontan patients with protein-losing enteropathy (PLE) represent poor candidates for cardiac transplantation due to end-organ injury and severely impaired clinical condition. Ventricular assist device (VAD) therapy has evolved as a promising bridge to transplant strategy improving quality of life and survival on the waiting list. However, VAD therapy for the Fontan circulation remains challenging. For Fontan patients with preserved ventricular function implantation of a right ventricular assist device (RVAD) has been described by Prêtre et al as bridge to transplant. We present the second case of RVAD support in a Fontan patient with PLE. AD - Department of Pediatric Cardiology, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany. Department of Pediatric Cardiac Surgery, Friedrich-AlexanderUniversity Erlangen-Nürnberg, Erlangen, Germany. AN - 32557829 AU - Moosmann, J. AU - Dittrich, S. AU - Purbojo, A. AU - Cesnjevar, R. DA - Jul DB - UPubmed DO - 10.1111/jocs.14658 DP - NLM ET - 2020/06/20 IS - 7 KW - Female *Fontan Procedure Heart Defects, Congenital/complications Heart Failure/etiology/*surgery Heart Transplantation Heart Ventricles *Heart-Assist Devices Humans Prosthesis Implantation/*methods Protein-Losing Enteropathies/*etiology Quality of Life Waiting Lists Young Adult Fontan circulation assist in Fontan congenital heart disease protein-losing enteropathy right ventricular assist device LA - eng N1 - 1540-8191 Moosmann, Julia Orcid: 0000-0002-8843-7084 Dittrich, Sven Purbojo, Ariawan Cesnjevar, Robert Case Reports United States J Card Surg. 2020 Jul;35(7):1721-1724. doi: 10.1111/jocs.14658. Epub 2020 Jun 19. PY - 2020 SN - 0886-0440 SP - 1721-1724 ST - RVAD implantation in a Fontan patient with protein-losing enteropathy as a bridge to transplant: Prêtre modification T2 - J Card Surg TI - RVAD implantation in a Fontan patient with protein-losing enteropathy as a bridge to transplant: Prêtre modification VL - 35 ID - 2365 ER - TY - JOUR AB - An arterial switch operation (ASO) is the standard treatment for infants and children born with D-loop transposition of the great arteries. During the ASO, the great vessels are transected from the native roots, switched and anastomosed with the opposite roots. This is accompanied by the relocation of the pulmonary artery anterior to the aorta by using the LeCompte maneuver and the translocation of coronary arteries to the neo-aorta. ASO has led to improved overall survival, and postoperative mortality is rare. Despite the improved outcomes, several postoperative sequelae may occur, and therefore patients require longterm follow-up. Computed tomography (CT) has emerged as a robust imaging modality in pre and postoperative evaluation of a variety of congenital heart disorders including ASO. Unlike echocardiography and cardiovascular magnetic resonance, CT is not hindered by a poor acoustic window, metallic devices or the need for sedation or general anesthesia. CT with advanced three-dimensional postprocessing techniques, high pitch scanning, wider detector system, electrocardiogram-dependent modulation and dose-reduction strategies is invaluable in assessing the postoperative complications after ASO. © 2021 Korean Society of Echocardiography. AD - Department of Radiodiagnosis & Imaging, Fortis Escort Heart Institute, New Delhi, India Cardiac Imaging, Cardiological Society of India, Kolkata, India International Regional Committee, India Chapter, Society of Cardiovascular Computed Tomography, Arlington, VA, United States AU - Kumar, P. AU - Bhatia, M. DB - UScopus DO - 10.4250/jcvi.2020.0106 IS - 1 KW - Arterial switch procedure Atrial switch operation Atrial switch repair Jatene procedure Transposition of great vessels M3 - Article N1 - Export Date: 10 May 2021 PY - 2021 SP - 1-19 ST - Role of computed tomography in postoperative follow-up of arterial switch operation T2 - Journal of Cardiovascular Imaging TI - Role of computed tomography in postoperative follow-up of arterial switch operation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85100211303&doi=10.4250%2fjcvi.2020.0106&partnerID=40&md5=3a77489b710f457837ccd912d91880fc VL - 29 ID - 5875 ER - TY - JOUR AB - Purpose Of Review: We aim to review select literature pertaining to congenital heart disease (CHD)-induced right ventricular (RV) function and failure.Recent Findings: We review recent findings pertaining to children and adults with repaired tetralogy of Fallot (rTOF), systemic RV and hypoplastic left heart syndrome (HLHS). We emphasize pathophysiological mechanisms contributing to RV dysfunction in these conditions, the risk factors for adverse outcomes and the continuing challenges in treating these patients. We discuss how recent pathology findings, as well as developments in imaging and computer modeling have broadened our understanding of the pathophysiology of these conditions. We further review developments in the molecular and cellular basis of RV failure; and in particular, the RV molecular response to stress in repaired tetralogy of Fallot (rTOF). We highlight some of the genetic complexities in HLHS and how these may influence the long-term outcomes in these patients.Summary: Recent literature has led to new understandings in the pathology, pathophysiology, risk factors for adverse outcomes, molecular and genetic basis for RV dysfunction and failure in CHD. Although these findings provide new therapeutic targets, the treatment of RV failure at this time remains limited. AD - Division of Cardiology, Labatt Family Heart Centre, Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada Department of Pediatrics (Cardiology), Stanford University, Stanford, California, USA AN - 144232776. Language: English. Entry Date: 20200627. Revision Date: 20200925. Publication Type: journal article AU - Friedberg, Mark K. AU - Reddy, Sushma DB - Ucinahl DO - 10.1097/MOP.0000000000000804 DP - EBSCOhost IS - 5 KW - Heart Failure -- Physiopathology Heart Defects, Congenital -- Physiopathology Ventricular Dysfunction, Right -- Physiopathology N1 - review. Journal Subset: Biomedical; Peer Reviewed; USA. Grant Information: K08 HL127277/HL/NHLBI NIH HHS/United States. NLM UID: 9000850. PMID: NLM31356354. PY - 2019 SN - 1040-8703 SP - 604-610 ST - Right ventricular failure in congenital heart disease T2 - Current Opinion in Pediatrics TI - Right ventricular failure in congenital heart disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=144232776&site=ehost-live&scope=site VL - 31 ID - 5415 ER - TY - JOUR AB - OBJECTIVE: To investigate potential relationships between neuropsychologic functioning and cardiac, gastroenterologic/hepatologic, and pulmonary complications in the single ventricle heart disease (SVHD) post-Fontan population. STUDY DESIGN: Following the initiation of a Fontan Multidisciplinary Clinic, patients with SVHD were evaluated systematically according to a clinical care pathway, and data from multiple subspecialty evaluations were collected prospectively from 2016 to 2019. Biomarkers of cardiology, pulmonary, and hepatology/gastroenterology functioning were abstracted, along with neuropsychologic testing results. Bivariate correlations and regression analyses examined cross-sectional relationships between physiologic predictors and neuropsychologic outcomes. RESULTS: The sample included a cohort of 68 youth with SVHD age 3-19 years, after Fontan palliation. Sleep-disordered breathing was related to poorer visual-motor integration skills (r = -0.33; P < .05) and marginally related to poorer executive functioning (r = -0.33; P = .05). Lower arterial blood oxygen content was related to poorer executive functioning (r = .45; P < .05). Greater atrioventricular valve regurgitation was related to lower parent-rated adaptive functioning (ρ = -0.34; P < .01). These results were maintained in regression analyses controlling for history of stroke and/or seizures. CONCLUSIONS: We demonstrated associations between neuropsychologic functioning and potentially modifiable aspects of physiologic functioning in a prospectively evaluated cohort of patients with SVHD with Fontan physiology. Our findings emphasize the importance of multidisciplinary screening and care after a Fontan procedure and suggest avenues for intervention that may improve patient outcomes and quality of life. AD - Section of Neurology, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO. Electronic address: kelly.wolfe@childrenscolorado.org. Section of Pulmonology, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO. Section of Gastroenterology, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO. Section of Cardiology, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO. Section of Anesthesiology, University of Colorado School of Medicine, Aurora, CO. AN - 32687915 AU - Wolfe, K. R. AU - Liptzin, D. R. AU - Brigham, D. AU - Kelly, S. L. AU - Rafferty, C. AU - Albertz, M. AU - Younoszai, A. K. AU - Di Maria, M. V. DA - Dec DB - UPubmed DO - 10.1016/j.jpeds.2020.07.043 DP - NLM ET - 2020/07/21 KW - Adolescent Child Child, Preschool Cross-Sectional Studies Female *Fontan Procedure Humans Male Neuropsychological Tests Postoperative Complications/epidemiology/*physiopathology/*psychology Prospective Studies Univentricular Heart/*surgery Young Adult *cognition *hypoplastic left heart *multiorgan comorbidities *neurodevelopment *pediatric neuropsychology *single ventricle LA - eng N1 - 1097-6833 Wolfe, Kelly R Liptzin, Deborah R Brigham, Dania Kelly, Sarah L Rafferty, Carey Albertz, Megan Younoszai, Adel K Di Maria, Michael V Journal Article United States J Pediatr. 2020 Dec;227:239-246. doi: 10.1016/j.jpeds.2020.07.043. Epub 2020 Jul 18. PY - 2020 SN - 0022-3476 SP - 239-246 ST - Relationships between Physiologic and Neuropsychologic Functioning after Fontan T2 - J Pediatr TI - Relationships between Physiologic and Neuropsychologic Functioning after Fontan VL - 227 ID - 2360 ER - TY - JOUR AB - INTRODUCTION: Adolescents with single ventricle congenital heart disease (SVHD) show functional deficits, particularly in memory and mood regulation. Hippocampi are key brain structures that regulate mood and memory; however, their tissue integrity in SVHD is unclear. Our study aim is to evaluate hippocampal volumes and their associations with memory, anxiety, and mood scores in adolescents with SVHD compared to healthy controls. METHODS: We collected brain magnetic resonance imaging data from 25 SVHD (age 15.9 ± 1.2 years; 15 male) and 38 controls (16.0 ± 1.1 years; 19 male) and assessed memory (Wide Range Assessment of Memory and Learning 2, WRAML2), anxiety (Beck Anxiety Inventory, BAI), and mood (Patient Health Questionnaire 9, PHQ-9) functions. Both left and right hippocampi were outlined and global volumes, as well as three-dimensional surfaces were compared between groups using ANCOVA and associations with cognitive and behavioral scores with partial correlations (covariates: age and total brain volume). RESULTS: The SVHD group showed significantly higher BAI (p = .001) and PHQ-9 (p < .001) scores, indicating anxiety and depression symptoms and significantly reduced WRAML2 scores (p < .001), suggesting memory deficits compared with controls. SVHD group had significantly reduced right global hippocampal volumes (p = .036) compared with controls, but not the left (p = .114). Right hippocampal volume reductions were localized in the CA1, CA4, subiculum, and dentate gyrus. Positive correlations emerged between WRAML2 scores and left (r = 0.32, p = .01) and right (r = 0.28, p = .03) hippocampal volumes, but BAI and PHQ-9 did not show significant correlations. CONCLUSION: Adolescents with SVHD show reduced hippocampal volumes, localized in several sites (CA1, CA4, subiculum, and dentate gyrus), which are associated with memory deficits. The findings indicate the need to explore ways to improve memory to optimize academic achievement and ability for self-care in the condition. AD - UCLA School of Nursing, University of California Los Angeles, Los Angeles, CA, USA. Division of Pediatric Cardiology, University of California Los Angeles, Los Angeles, CA, USA. Division of Pediatric Cardiology, Children's Hospital Los Angeles, Los Angeles, CA, USA. Departments of Anesthesiology, University of California Los Angeles, Los Angeles, CA, USA. Radiological Sciences, University of California Los Angeles, Los Angeles, CA, USA. Bioengineering, University of California Los Angeles, Los Angeles, CA, USA. Brain Research Institute, University of California Los Angeles, Los Angeles, CA, USA. AN - 33410605 AU - Pike, N. A. AU - Roy, B. AU - Moye, S. AU - Cabrera-Mino, C. AU - Woo, M. A. AU - Halnon, N. J. AU - Lewis, A. B. AU - Kumar, R. C2 - PMC7882179 DA - Feb DB - UPubmed DO - 10.1002/brb3.1977 DP - NLM ET - 2021/01/08 IS - 2 KW - cognition congenital heart disease gray matter magnetic resonance imaging LA - eng N1 - 2162-3279 Pike, Nancy A Orcid: 0000-0001-6198-9957 Roy, Bhaswati Moye, Stefanie Cabrera-Mino, Cristina Woo, Mary A Halnon, Nancy J Lewis, Alan B Kumar, Rajesh Orcid: 0000-0001-5355-9586 R01NR 013930/NR/NINR NIH HHS/United States R01NR016463/NR/NINR NIH HHS/United States Journal Article Brain Behav. 2021 Feb;11(2):e01977. doi: 10.1002/brb3.1977. Epub 2021 Jan 7. PY - 2021 SP - e01977 ST - Reduced hippocampal volumes and memory deficits in adolescents with single ventricle heart disease T2 - Brain Behav TI - Reduced hippocampal volumes and memory deficits in adolescents with single ventricle heart disease VL - 11 ID - 2348 ER - TY - JOUR AB - BACKGROUND: Adolescents with single ventricle heart disease (SVHD) who have undergone the Fontan procedure show cognitive/memory deficits. Mammillary bodies are key brain sites that regulate memory; however, their integrity in SVHD is unclear. We evaluated mammillary body (MB) volumes and their associations with cognitive/memory scores in SVHD and controls. METHODS: Brain MRI data were collected from 63 adolescents (25 SVHD; 38 controls) using a 3.0-Tesla MRI scanner. Cognition and memory were assessed using Montreal Cognitive Assessment (MoCA) and Wide Range Assessment of Memory and Learning 2. MB volumes were calculated and compared between groups (ANCOVA, covariates: age, sex, and total brain volume [TBV]). Partial correlations and linear regression were performed to examine associations between volumes and cognitive scores (covariates: age, sex, and TBV). RESULTS: SVHD group showed significantly lower MoCA and WRAML2 scores over controls. MB volumes were significantly reduced in SVHD over controls. After controlling for age, sex, and TBV, MB volumes correlated with MoCA and delayed memory recall scores in SVHD and controls. CONCLUSION: Adolescents with SVHD show reduced MB volumes associated with cognitive/memory deficits. Potential mechanisms of volume losses may include developmental and/or hypoxic/ischemic-induced processes. Providers should screen for cognitive deficits and explore possible interventions to improve memory. AD - UCLA School of Nursing, University of California Los Angeles, Los Angeles, CA, USA. Department of Anesthesiology, University of California Los Angeles, Los Angeles, CA, USA. Division of Pediatric Cardiology, University of California Los Angeles, Los Angeles, CA, USA. Division of Pediatric Cardiology, Children's Hospital Los Angeles, Los Angeles, CA, USA. Department of Radiological Sciences, University of California Los Angeles, Los Angeles, CA, USA. Department of Bioengineering, University of California Los Angeles, Los Angeles, CA, USA. Brain Research Institute, University of California Los Angeles, Los Angeles, CA, USA. UCLA School of Nursing, University of California Los Angeles, Los Angeles, CA, USA. npike@sonnet.ucla.edu. AN - 31499515 AU - Cabrera-Mino, C. AU - Roy, B. AU - Woo, M. A. AU - Singh, S. AU - Moye, S. AU - Halnon, N. J. AU - Lewis, A. B. AU - Kumar, R. AU - Pike, N. A. C2 - PMC6962527 C6 - NIHMS1538752 DA - Jan DB - UPubmed DO - 10.1038/s41390-019-0569-3 DP - NLM ET - 2019/09/10 IS - 1 KW - Adolescent Case-Control Studies *Cognition Cognitive Dysfunction/*diagnostic imaging/physiopathology/psychology Female Fontan Procedure/*adverse effects Humans *Magnetic Resonance Imaging Male Mammillary Bodies/*diagnostic imaging/physiopathology *Memory Memory Disorders/*diagnostic imaging/physiopathology/psychology Neuropsychological Tests Predictive Value of Tests Treatment Outcome Univentricular Heart/*surgery LA - eng N1 - 1530-0447 Cabrera-Mino, Cristina Roy, Bhaswati Woo, Mary A Singh, Sadhana Moye, Stefanie Halnon, Nancy J Lewis, Alan B Kumar, Rajesh Pike, Nancy A R01 NR013930/NR/NINR NIH HHS/United States R01 NR016463/NR/NINR NIH HHS/United States Journal Article Research Support, N.I.H., Extramural Pediatr Res. 2020 Jan;87(1):169-175. doi: 10.1038/s41390-019-0569-3. Epub 2019 Sep 9. PY - 2020 SN - 0031-3998 (Print) 0031-3998 SP - 169-175 ST - Reduced brain mammillary body volumes and memory deficits in adolescents who have undergone the Fontan procedure T2 - Pediatr Res TI - Reduced brain mammillary body volumes and memory deficits in adolescents who have undergone the Fontan procedure VL - 87 ID - 2399 ER - TY - JOUR AB - RATIONALE: Renal vein pseudoaneurysm after blunt trauma is an extremely rare clinical disease. Different interventions, such as conservative, surgical, and endovascular treatments, can be considered. However, previous studies have not described the optimal treatment strategies for this condition. Furthermore, there is a significant lack of prior case reports and of standardized treatment guidelines for trauma-induced renal vein pseudoaneurysm patients who previously maintained antithrombotic agent. PATIENT CONCERNS: A 23-year-old female patient visited the emergency department after sustaining blunt injury caused by falling. The patient was diagnosed with multiple limb and rib fractures. A right renal vein pseudoaneurysm was found on abdominal computed tomography scan. Initially, there was no other organ damage, and the patient was hemodynamically stable. Thus, nonsurgical, conservative management was considered. However, the patient's hematocrit and hemoglobin levels decreased, and there was no hemodynamic improvement. The patient required lifelong treatment with aspirin because she previously underwent Fontan surgery, and orthopedic surgery for multiple fractures was planned. Thus, considering these factors, the treatment method was changed from conservative management to endovascular stent insertion. DIAGNOSES: Abdominal computed tomography and renal venography revealed a right renal vein pseudoaneurysm. INTERVENTIONS: On the basis of the abdominal computed tomography scan and renal venography findings, the endovascular stent graft was inserted across the pseudoaneurysm area. OUTCOMES: Upon placement of the endovascular stent, hemoglobin and hematocrit levels gradually returned to normal. The patient's vital signs and general condition had improved. The patient recovered without any complications and was discharged 29 days after hospitalization. LESSONS: Some patients with traumatic renal vein pseudoaneurysm do not experience hemodynamic improvement despite conservative treatment. Hence, endovascular procedure may be considered for these patients, particularly those who require antithrombotic treatment for a previous disease. AD - Department of Trauma Surgery. Department of Emergency Medicine, Eulji University Hospital, Daejeon, Republic of Korea. AN - 33466219 AU - Mun, Y. S. AU - Sung, W. Y. C2 - PMC7808473 research, authorship, and/or publication of this article. DA - Jan 15 DB - UPubmed DO - 10.1097/md.0000000000024299 DP - NLM ET - 2021/01/21 IS - 2 KW - Aneurysm, False/*etiology Female Humans *Renal Veins Wounds, Nonpenetrating/*complications Young Adult LA - eng N1 - 1536-5964 Mun, Yun Su Sung, Won Young Orcid: 0000-0003-1224-9236 Case Reports Journal Article Medicine (Baltimore). 2021 Jan 15;100(2):e24299. doi: 10.1097/MD.0000000000024299. PY - 2021 SN - 0025-7974 (Print) 0025-7974 SP - e24299 ST - A rare case report of renal vein pseudoaneurysm after blunt trauma T2 - Medicine (Baltimore) TI - A rare case report of renal vein pseudoaneurysm after blunt trauma VL - 100 ID - 2345 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 - 6557 ER - TY - JOUR AB - Objective: Hypoplastic left heart syndrome is a single ventricle defect. While staged surgical palliative treatments have revolutionised care, patients with hypoplastic left heart syndrome continue to have significant morbidity and mortality. In 2017, the National Pediatric Cardiology Quality Improvement Collaborative recommended all single ventricle patients to receive a prenatal palliative care consult. This study aimed to elucidate provider perspectives on the implementation of prenatal palliative care consults for families expecting a child with hypoplastic left heart syndrome.Methods: An online survey was administered to obstetric and paediatric providers of relevant disciplines to assess their experience with palliative care involvement in hypoplastic left heart syndrome cases.Results: Nearly, all physicians (97%) and most registered nurses (79%) agreed that the initial palliative care consult for patients with hypoplastic left heart syndrome should occur during the prenatal period. Respondents also indicated that prenatal palliative care consults should also be offered in a variety of other CHD conditions. Participants believed positive aspects of this new referral protocol included an expanded support network for families, decreased family stress during the postnatal period, increased patient education about what to expect during the postnatal period, and continuity of care.Conclusion: Multidisciplinary healthcare professionals believe that prenatal palliative care consults provide a variety of benefits for patients and families with hypoplastic left heart syndrome. Additional, multi-centre research is necessary to evaluate whether prenatal palliative care consults should become standard of care for families expecting a child with a single ventricle defect. AD - School of Medicine, Oregon Health & Science University, Portland, OR, USA Bridges Palliative Care Program, Doernbecher Children's Hospital, Portland, OR, USA Pediatric Cardiology, Doernbecher Children's Hospital, Portland, OR, USA AN - 144805975. Language: English. Entry Date: 20210107. Revision Date: 20210301. Publication Type: journal article AU - Lowenstein, Sarah AU - Macauley, Robert AU - Perko, Kathleen AU - Ronai, Christina DB - Ucinahl DO - 10.1017/S1047951120000128 DP - EBSCOhost IS - 3 KW - Palliative Care -- Methods Parents -- Psychosocial Factors Palliative Care -- Psychosocial Factors Hypoplastic Left Heart Syndrome -- Therapy Hypoplastic Left Heart Syndrome -- Diagnosis Professional-Family Relations Oregon Multidisciplinary Care Team -- Administration Referral and Consultation -- Statistics and Numerical Data Attitude of Health Personnel Prenatal Diagnosis Female Human Pregnancy Quality Improvement Infant, Newborn Validation Studies Comparative Studies Evaluation Research Multicenter Studies Ferrans and Powers Quality of Life Index N1 - research. Journal Subset: Biomedical; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Instrumentation: Ferrans and Powers Quality of Life Index. NLM UID: 9200019. PMID: NLM32146916. PY - 2020 SN - 1047-9511 SP - 377-382 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=144805975&site=ehost-live&scope=site VL - 30 ID - 5318 ER - TY - JOUR AB - Background: Down syndrome is the most common chromosomal disorder at birth and is often accompanied by structural birth defects. Current data on major structural defects in this population are limited. Methods: States and territorial population-based surveillance programs submitted data on identified cases of Down syndrome and identified structural birth defects during 2013–2017. We estimated prevalence by program type and maternal and infant characteristics. Among programs with active case ascertainment, we estimated the prevalence of birth defects by organ system and for specific defects by maternal age (<35, ≥35) and infant sex. Results: We identified 13,376 cases of Down syndrome. Prevalence among all programs was 12.7 per 10,000 live births. Among these children, 75% had at least one reported co-occurring birth defect diagnosis code. Among 6,210 cases identified by active programs, 66% had a cardiovascular defect with septal defects being the most common: atrial (32.5%), ventricular (20.6%), and atrioventricular (17.4%). Defect prevalence differed by infant sex more frequently than by maternal age. For example, atrioventricular septal defects were more common in female children (20.1% vs. 15.1%) while limb deficiencies were more prevalent in male children (0.4% vs. 0.1%). Conclusions: Our study provides updated prevalence estimates for structural defects, including rare defects, among children with Down syndrome using one of the largest and most recent cohorts to date. These data may aid clinical care and surveillance. AD - D. Heinke, Center for Birth Defects Research and Prevention, Massachusetts Department of Public Health, Boston, MA, United States AU - Heinke, D. AU - Isenburg, J. L. AU - Stallings, E. B. AU - Short, T. D. AU - Le, M. AU - Fisher, S. AU - Shan, X. AU - Kirby, R. S. AU - Nguyen, H. H. AU - Nestoridi, E. AU - Nembhard, W. N. AU - Romitti, P. A. AU - Salemi, J. L. AU - Lupo, P. J. DB - UEmbase DO - 10.1002/bdr2.1854 IS - 2 KW - anophthalmia aortic arch interruption aortic coarctation aortic valve stenosis arterial trunk article atrioventricular septal defect bile duct atresia bladder exstrophy child choana atresia cleft lip cleft lip palate cleft palate congenital cataract congenital malformation craniofacial synostosis diaphragm hernia Down syndrome Ebstein anomaly encephalocele esophagus atresia Fallot tetralogy gastroschisis gestational age great vessels transposition heart atrium septum defect heart right ventricle double outlet heart septum defect holoprosencephaly human hypoplastic left heart syndrome hypospadias ICD-10-CM ICD-9-CM intestine atresia kidney agenesis limb defect lung vein drainage anomaly maternal age microphthalmia microtia omphalocele pregnancy outcome prevalence pulmonary valve atresia risk factor spinal dysraphism stenosis tracheoesophageal fistula tricuspid valve atresia trisomy 13 trisomy 18 trisomy 21 Turner syndrome LA - English M3 - Article N1 - L2007642740 2020-12-23 PY - 2021 SN - 2472-1727 SP - 189-202 ST - Prevalence of structural birth defects among infants with Down syndrome, 2013–2017: A US population-based study T2 - Birth Defects Research TI - Prevalence of structural birth defects among infants with Down syndrome, 2013–2017: A US population-based study UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2007642740&from=export http://dx.doi.org/10.1002/bdr2.1854 VL - 113 ID - 4360 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 - Ocinahl 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 - 5687 ER - TY - JOUR AD - Hospital Israelita Albert Einstein, São Paulo, SP, Brazil Cardioeco – Centro de Diagnóstico Cardiovascular, Curitiba, PR, Brazil Quanta Diagnóstico e Terapia, Curitiba, PR, Brazil Instituto da Criança e do Adolescente do Hospital das Clínicas da Universidade de São Paulo (HC-FMUSP), São Paulo, SP, Brazil Hospital do Coração, São Paulo, SP, Brazil Hospital Evangélico de Cachoeiro de Itapemirim e Clínica CORImagem, Cachoeiro de Itapemirim, ES, Brazil Hospital e Maternidade São Luiz Itaim, São Paulo, SP, Brazil Santa Casa de Misericórdia de MaceióAL, Brazil The Hospital for Sick Children, Toronto, Canada Hospital Português, Salvador, BA, Brazil Hospital Aliança, Salvador, BA, Brazil Texas Children’s Hospital, Baylor College of Medicine, Houston, TX, United States Instituto do Coração da Universidade de São Paulo (InCor, FMUSP), São Paulo, SP, Brazil AU - Morhy, S. S. AU - Barberato, S. H. AU - Lianza, A. C. AU - Soares, A. M. AU - Leal, G. N. AU - Rivera, I. R. AU - Barberato, M. F. A. AU - Guerra, V. AU - De Sá Ribeiro, Z. V. AU - Pignatelli, R. AU - Rochitte, C. E. AU - Vieira, M. L. C. DB - UScopus DO - 10.36660/abc.20201122 IS - 5 M3 - Article N1 - Cited By :1 Export Date: 10 May 2021 PY - 2020 SP - 987-1005 ST - Position statement on indications for echocardiography in fetal and pediatric cardiology and congenital heart disease of the adult – 2020 T2 - Arquivos Brasileiros de Cardiologia TI - Position statement on indications for echocardiography in fetal and pediatric cardiology and congenital heart disease of the adult – 2020 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85097517763&doi=10.36660%2fabc.20201122&partnerID=40&md5=1f024cf2153d35fa5679bf6aee1dc606 VL - 115 ID - 5926 ER - TY - JOUR AB - Background: Patients with a Fontan circulation have a reduced exercise capacity, which is an important prognostic predictor of morbidity and mortality. A way to increase exercise capacity in Fontan patients might be exercise training. This systematic review assesses the effects of exercise training investigated in Fontan patients in order to provide an overview of current insights. Design and methods: Studies evaluating an exercise training intervention in Fontan patients published up to February 2020 were included in this systematic review. Results: From 3000 potential studies, 16 studies reported in 22 publications met the inclusion criteria. In total, 264 Fontan patients with mean age range 8.7–31 years, were included. Different training types including inspiratory muscle training, resistance training and aerobic training were investigated. Main outcome measures reported were peak oxygen uptake, cardiac function, lung function, physical activity levels and quality of life. Peak oxygen uptake increased significantly in 56% of the studies after training with an overall mean increase of +1.72 ml/kg/min (+6.3%). None of the studies reported negative outcome measures related to the exercise programme. In four studies an adverse event was reported, most likely unrelated to the training intervention. Conclusions: Exercise training in Fontan patients is most likely safe and has positive effects on exercise capacity, cardiac function and quality of life. Therefore exercise training in Fontan patients should be encouraged. Further studies are required to assess the optimal training type, intensity, duration and long-term effects. AD - W.A. Helbing, Department of Pediatric Cardiology, Erasmus MC-Sophia Children’s Hospital, Netherlands AU - Scheffers, L. E. AU - Berg, L. E. M. V. AU - Ismailova, G. AU - Dulfer, K. AU - Takkenberg, J. J. M. AU - Helbing, W. A. DB - UEmbase DO - 10.1177/2047487320942869 KW - adolescent adult aerobic capacity article child congenital heart malformation endurance female Fontan procedure heart function human lung function male muscle training outcome assessment physical activity quality of life resistance training school child systematic review LA - English M3 - Article in Press N1 - L2005711196 PY - 2020 SN - 2047-4881 2047-4873 ST - Physical exercise training in patients with a Fontan circulation: A systematic review T2 - European Journal of Preventive Cardiology TI - Physical exercise training in patients with a Fontan circulation: A systematic review UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2005711196&from=export http://dx.doi.org/10.1177/2047487320942869 ID - 4442 ER - TY - JOUR AB - Purpose: To quantify the differences in daily physical activity (PA) patterns, intensity-specific volumes, and PA bouts in youth with and without heart disease (HD). Methods: Seven-day PA was measured on children/adolescents with HD (n = 34; median age 12.4 y; 61.8% male; 70.6% single ventricle, 17.7% heart failure, and 11.8% pulmonary hypertension) and controls without HD (n = 22; median age 12.3 y; 59.1% male). Mean counts per minute were classified as sedentary, light, and moderate to vigorous PA (MVPA), and bouts of MVPA were calculated. PA was calculated separately for each hour of wear time from 8:00 to 22:00. Multilevel linear mixed modeling compared the outcomes, stratifying by group, time of day, and day part (presented as median percentage of valid wear time [interquartile range]). Results: Compared with the controls, the HD group had more light PA (33.9% [15%] vs 29.6% [9.5%]), less MVPA (1.7% [2.5%] vs 3.2% [3.3%]), and more sporadic bouts (97.4% [5.7%] vs 89.9% [9.2%]), but fewer short (2.0% [3.9%] vs 7.1% [5.7%]) and medium-to-long bouts (0.0% [1.9%] vs 1.6% [4.6%]) of MVPA. The HD group was less active in the late afternoon, between 15:00 and 17:00 (P <.03). There were no differences between groups in sedentary time. Conclusion: Children/adolescents with HD exhibit differences in intensity-specific volumes, PA bouts, and daily PA patterns compared with controls. AD - Children's Mercy Hospital University of Missouri-Kansas City The University of North Carolina at Chapel Hill AN - 146363699. Language: English. Entry Date: 20201021. Revision Date: 20201021. Publication Type: Article AU - White, David A. AU - Willis, Erik A. AU - Panchangam, Chaitanya AU - Teson, Kelli M. AU - Watson, Jessica S. AU - Birnbaum, Brian F. AU - Shirali, Girish AU - Parthiban, Anitha DB - Ucinahl DO - 10.1123/pes.2020-0073 DP - EBSCOhost IS - 4 KW - Heart Defects, Congenital Cardiac Patients -- In Infancy and Childhood Cardiac Patients -- In Adolescence Physical Activity -- Evaluation Human Male Female Child Adolescence Life Style, Sedentary Exercise Intensity Descriptive Statistics Time Factors Accelerometers Health Behavior N1 - research; tables/charts. Journal Subset: Allied Health; Biomedical; Peer Reviewed; USA. NLM UID: 8909729. PY - 2020 SN - 0899-8493 SP - 233-240 ST - Physical Activity Patterns in Children and Adolescents With Heart Disease T2 - Pediatric Exercise Science TI - Physical Activity Patterns in Children and Adolescents With Heart Disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=146363699&site=ehost-live&scope=site VL - 32 ID - 5312 ER - TY - JOUR AB - Objective: To explore if there is association between vitamin D supplementation through cod liver oil ingestion around the periconceptional period and the risk of developing severe CHD in offspring. Furthermore, we would examine the interaction between vitamin D and folic acid supplementation in the association. Methods: A case-control study was conducted in Shanghai Children's Medical Center, in which, a total of 262 severe CHD cases versus 262 controls were recruited through June 2016 to December 2017. All children were younger than 2 years. To reduce potential selection bias and to minimise confounding effects, propensity score matching was applied. Results: After propensity score matching, vitamin D supplementation seemed to be associated with decreased odds ratio of severe CHD (odds ratio = 0.666; 95% confidence intervals: 0.449-0.990) in the multivariable conditional logistic analysis. Furthermore, we found an additive interaction between vitamin D and folic acid supplementation (relative excess risk due to interaction = 0.810, 95% confidence intervals: 0.386-1.235) in the association. Conclusion: The results suggested that maternal vitamin D supplementation could decrease the risk of offspring severe CHD; moreover, it could strengthen the protective effect of folic acid. The significance of this study lies in providing epidemiological evidence that vitamin D supplementation around the periconceptional period could be a potential nutritional intervention strategy to meet the challenge of increasing CHD. AD - S. Li, Department of Maternal, Child and Adolescent Health, School of Public Health, Shanghai Jiao Tong University, School of Medicine, Shanghai, China AU - Zhao, A. AU - Zhao, K. AU - Xia, Y. AU - Yin, Y. AU - Zhu, J. AU - Hong, H. AU - Li, S. DB - UEmbase DO - 10.1017/S1047951120002280 IS - 10 KW - cod liver oil folic acid vitamin D aortic arch interruption article atrioventricular septal defect case control study child China congenital heart disease congenital mitral stenosis controlled study disease severity Fallot tetralogy female great vessels transposition heart right ventricle double outlet heart single ventricle hospital based case control study human hypoplastic left heart syndrome infant lung vein drainage anomaly major clinical study male propensity score pulmonary valve atresia risk reduction single atrium tricuspid valve atresia vitamin supplementation LA - English M3 - Article N1 - L632800085 2020-09-15 2021-03-05 PY - 2020 SN - 1467-1107 1047-9511 SP - 1445-1451 ST - Periconceptional use of cod liver oil, a vitamin D source, could decrease the risk of CHD in offspring T2 - Cardiology in the Young TI - Periconceptional use of cod liver oil, a vitamin D source, could decrease the risk of CHD in offspring UR - https://www.embase.com/search/results?subaction=viewrecord&id=L632800085&from=export http://dx.doi.org/10.1017/S1047951120002280 VL - 30 ID - 4392 ER - TY - JOUR AB - Background: There is a paucity of data on palliative or total percutaneous pulmonary artery debanding (p-debanding), particularly with use of a stent. Methods: Twelve p-debandings in eight patients were included in this study. Age at pulmonary artery banding (PAB) ranged from 3 days to 1 year (median, 13 days), while p-debanding was performed at 2–157 (7) months. The body weight at the p-debanding ranged from 3.2 to 22.2 (7.3) kg. We chose the balloon diameter of 30–50% to the circumference of the band for palliative, and larger than 50% for total p-debanding, respectively. In either way, the balloon diameter did not exceed 1.5 times the reference vessel diameter. Stent was implanted for palliative p-debanding in 2 patients. Results: 1. The circumference of the band ranged from 16 to 23 (20) mm, while the balloon diameter ranged from 20–60 (40)% to that, where larger than 50% was used for 2 procedures intended total p-debanding. 2. PAB diameter increased from 2.5–4.7 (3.0) mm to 2.8–9.5 (4.5) mm (p < 0.01), however, there was no significant change in the diameter in 2 procedures. In one patient, p-debanding was the definitive treatment associated with spontaneous near closure of muscular ventricular septal defect, in another patient of congenitally corrected transposition of the great arteries, severely depressed left ventricular ejection fraction was recovered following p-debanding. 3. Arterial oxygen saturation (SaO2) increased from 64–97 (80)% to 66–95 (90)% (p < 0.01), while in 10 procedures of 6 patients where the indication of p-debanding was hypoxia, SaO2 increased in 8 procedures. There was no significant pulmonary hypertension following p-debanding. Conclusion: Palliative or total p-debanding using balloon and/or stenting is generally feasible and effective. A balloon diameter 35–50% to the band circumference in palliative, and more than 50% in total p-debanding, while in either way less than 1.5 times the reference vessel diameter, is safe. AD - H. Tomita, Pediatric Heart Disease & Adult Congenital Heart Disease Center, Showa University, 1-5-8 Hatanodai, Sinagawa-ku, Tokyo, Japan AU - Tomita, H. AU - Fujii, T. AU - Kise, H. AU - Oyama, N. AU - Shimizu, T. AU - Nagaoka, K. AU - Tarui, S. AU - Miyahara, Y. AU - Ishino, K. DB - UEmbase DO - 10.1016/j.jjcc.2020.10.021 IS - 3 KW - balloon catheter polytetrafluoroethylene arterial oxygen saturation article balloon dilatation body weight child chromosome aberration clinical article congenitally corrected transposition of the great arteries controlled study female heart left ventricle ejection fraction heart left ventricle failure heart single ventricle heart ventricle septum defect human infant lung artery banding lung artery pressure male newborn observational study percutaneous pulmonary artery debanding pulmonary hypertension pulmonary valve stenosis retrospective study LA - English M3 - Article N1 - L2010228030 2020-12-14 2021-02-16 PY - 2021 SN - 1876-4738 0914-5087 SP - 307-312 ST - Percutaneous pulmonary artery debanding T2 - Journal of Cardiology TI - Percutaneous pulmonary artery debanding UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2010228030&from=export http://dx.doi.org/10.1016/j.jjcc.2020.10.021 VL - 77 ID - 4348 ER - TY - JOUR AB - BACKGROUND: To determine the feasibility and clinical result of selective embolization of hepatoduodenal or paratracheal lymphatics in Fontan patients with protein-losing enteropathy (PLE) or plastic bronchitis (PB). METHODS: Dilated lymph vessels in periportal (PLE) or paratracheal (PB) position were percutaneously punctured with a 22G Chiba needle. Intralymphatic position was confirmed by water soluble contrast injection with drainage to hepatoduodenal or tracheal fistulae. After flushing with 10% glucose solution, occlusion of hepatoduodenal or paratreacheal lymphatics was effected by injection of 1-4 cc mixture 4/1 of Lipiodol/n-butyl cyanoacrylate (n-BCA; Histoacryl). RESULTS: Seven patients with proven PLE were treated with periportal lymphatic embolization 10.7 (range: 6.6-13.5) years after the Fontan operation. The Fontan operation was performed at a median age of 3.7 (range: 2.9-5.7) years and PLE started a median of 3.1 (range: 0.9-4.7) years later. Five patients required a second procedure 2-8 months later. Complications were limited (spillage of glue in portal branch, transient cholangitis, and caustic duodenal bleeding). Six of seven patients reported significant improvement in quality of life and normalization of albumin levels after limited follow-up (p < .01). One patient (Fontan at 2.9 years; age 16.4 years) had PB for 2 years. Selective transthoracic cone-beam-directed puncture of left and right paratracheal lymphatics with n-BCA embolization of distal lymphatic fistulae resulted in lasting absence of tracheal casts (11 months). CONCLUSIONS: Embolization of periportal/peritracheal lymphatics is a promising technique in Fontan patients with PLE/PB. Larger series are required to determine incidence and reasons of success/failure, with long-term results and effects on liver function. AD - Interventional Radiology, University Hospitals Leuven, Leuven, Belgium. Department of Paediatric and Congenital Cardiology, University Hospitals Leuven, Leuven, Belgium. Paediatric Cardiology, University Hospital Maastricht, Maastricht, the Netherlands. Paediatric Cardiology MC3, Necker Hospital for Sick Children, Paris, France. Paediatric Cardiology, University of the Free State, Bloemfontein, South Africa. AN - 31596530 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. DA - Dec 1 DB - UPubmed DO - 10.1002/ccd.28501 DP - NLM ET - 2019/10/10 IS - 7 KW - Adolescent Bronchitis/diagnosis/etiology/*therapy Child *Embolization, Therapeutic/adverse effects Enbucrilate/*administration & dosage/adverse effects Feasibility Studies Fistula/diagnostic imaging/etiology/*therapy Fontan Procedure/*adverse effects Heart Defects, Congenital/*surgery Humans Lymphatic Diseases/diagnostic imaging/etiology/*therapy Protein-Losing Enteropathies/diagnosis/etiology/*therapy Retrospective Studies Treatment Failure Treatment Outcome congenital heart disease lymphangiography lymphatic embolization n-butyl cyanoacrylate plastic bronchitis protein losing enteropathy LA - eng N1 - 1522-726x Maleux, Geert Storme, Emma Cools, Bjorn Heying, Ruth Boshoff, Derize Louw, Jacoba J Frerich, Stefan Malekzadeh-Milanii, Sofie Hubrechts, Jelena Brown, Stephen C Orcid: 0000-0002-8508-8667 Gewillig, Marc Orcid: 0000-0002-4595-5922 de Kleine Hartjes/ Eddy Merckx Research Foundation/ Journal Article United States Catheter Cardiovasc Interv. 2019 Dec 1;94(7):996-1002. doi: 10.1002/ccd.28501. Epub 2019 Oct 9. PY - 2019 SN - 1522-1946 SP - 996-1002 ST - Percutaneous embolization of lymphatic fistulae as treatment for protein-losing enteropathy and plastic bronchitis in patients with failing Fontan circulation T2 - Catheter Cardiovasc Interv TI - Percutaneous embolization of lymphatic fistulae as treatment for protein-losing enteropathy and plastic bronchitis in patients with failing Fontan circulation VL - 94 ID - 2398 ER - TY - JOUR AB - Trisomy 13 and 18 are associated with congenital heart disease. Cardiac palliation has been reported in the literature, but is not usually done in this population. Thus, a multi-disciplinary team may experience controversy in formulating a care plan that includes cardiac intervention. Our objective was to determine differences in recommendations for cardiac intervention in this population between physicians specializing in pediatric cardiac critical care, neonatology, and genetics. A web-based survey was performed between April 2007 and August 2008. This survey evaluated surgical and transcatheter cardiac palliations that had been performed for individuals with trisomy 13 or 18 at the respondent's institution, the respondent's recommendations for cardiac intervention in hypothetical symptomatic patients with trisomy 13 or trisomy 18 and the influence of parental preference on these recommendations. Eight hundred fifty-nine responses were obtained from a primarily academic practice setting (59%). Cardiologists were most likely to recommend intervention; low risk interventions were recommended by 32% of cardiologists, 7% of neonatologists and 20% of geneticists. Parental request to intervene resulted in a 3 fold increased in the likelihood of all specialist recommending intervention. Counseling of families frequently occurred by multiple sub specialists (50%) and there was frequently (71%) a difference in opinion. Individuals with trisomy 13 or 18 are receiving cardiac intervention at many institutions. Cardiologists were more likely than geneticists or neonatologists to recommend intervention on all heart lesions other than single ventricle palliation which no specialists recommended. Parental wishes that 'everything be done' significantly influenced all specialists' recommendations. AD - Nationwide Children's Hospital and Research Institute, Columbus USA AN - 64590404. Language: English. Entry Date: 20111021. Revision Date: 20191120. Publication Type: Article AU - Yates, Andrew AU - Hoffman, Timothy AU - Shepherd, Edward AU - Boettner, Bethany AU - McBride, Kim DB - Ocinahl DO - 10.1007/s10897-011-9373-x DP - EBSCOhost IS - 5 KW - Trisomy 13 -- Complications Trisomy 18 -- Complications Heart Defects, Congenital -- Etiology Heart Defects, Congenital -- Therapy Attitude of Health Personnel Human Questionnaires Pediatrics Specialties, Medical Parental Attitudes Data Analysis Software Analysis of Variance Post Hoc Analysis Male Female Descriptive Statistics Infant, Newborn Ethics, Medical N1 - questionnaire/scale; research; tables/charts. Journal Subset: Biomedical; Continental Europe; Europe; Peer Reviewed. Special Interest: Pediatric Care; Psychiatry/Psychology. NLM UID: 9206865. PY - 2011 SN - 1059-7700 SP - 495-509 ST - Pediatric Sub-specialist Controversies in the Treatment of Congenital Heart Disease in Trisomy 13 or 18 T2 - Journal of Genetic Counseling TI - Pediatric Sub-specialist Controversies in the Treatment of Congenital Heart Disease in Trisomy 13 or 18 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=64590404&site=ehost-live&scope=site VL - 20 ID - 5601 ER - TY - JOUR AB - This 2020 International Consensus on Cardiopulmonary Resuscitation and Emergency Cardiovascular Care Science With Treatment Recommendations (CoSTR) for pediatric life support is based on the most extensive evidence evaluation ever performed by the Pediatric Life Support Task Force. Three types of evidence evaluation were used in this review: systematic reviews, scoping reviews, and evidence updates. Per agreement with the evidence evaluation recommendations of the International Liaison Committee on Resuscitation, only systematic reviews could result in a new or revised treatment recommendation. Systematic reviews performed for this 2020 CoSTR for pediatric life support included the topics of sequencing of airway-breaths-compressions versus compressions-airway-breaths in the delivery of pediatric basic life support, the initial timing and dose intervals for epinephrine administration during resuscitation, and the targets for oxygen and carbon dioxide levels in pediatric patients after return of spontaneous circulation. The most controversial topics included the initial timing and dose intervals of epinephrine administration (new treatment recommendations were made) and the administration of fluid for infants and children with septic shock (this latter topic was evaluated by evidence update). All evidence reviews identified the paucity of pediatric data and the need for more research involving resuscitation of infants and children. © 2020 AU - Maconochie, I. K. AU - Aickin, R. AU - Hazinski, M. F. AU - Atkins, D. L. AU - Bingham, R. AU - Couto, T. B. AU - Guerguerian, A. M. AU - Nadkarni, V. M. AU - Ng, K. C. AU - Nuthall, G. A. AU - Ong, G. Y. K. AU - Reis, A. G. AU - Schexnayder, S. M. AU - Scholefield, B. R. AU - Tijssen, J. A. AU - Nolan, J. P. AU - Morley, P. T. AU - Van de Voorde, P. AU - Zaritsky, A. L. AU - de Caen, A. R. AU - Moylan, A. AU - Topjian, A. AU - Nation, K. AU - Ohshimo, S. AU - Bronicki, R. A. AU - Kadlec, K. D. AU - Knight, L. J. AU - McCormick, T. N. AU - Morgan, R. W. AU - Roberts, J. S. AU - Tabbutt, S. AU - Thiagarajan, R. AU - Walsh, B. AU - Raymond, T. AU - Chan, M. AU - Duff, J. P. AU - Joyner, B. L., Jr. AU - Lasa, J. J. AU - Levy, A. AU - Roberts, K. E. AU - Sutton, R. M. AU - de Lucas, N. AU - Hoffmann, F. AU - Turner, N. AU - Biarent, D. AU - Lauritsen, T. AU - Brissaud, O. AU - Johannesdottir, G. AU - Djakow, J. AU - Mejias, A. M. AU - Meckler, G. DB - UScopus DO - 10.1016/j.resuscitation.2020.09.013 KW - AHA Scientific Statements arrhythmia cardiopulmonary resuscitation child congenital heart disease ECMO pediatrics M3 - Article N1 - Cited By :8 Export Date: 10 May 2021 PY - 2020 SP - A120-A155 ST - Pediatric Life Support: 2020 International Consensus on Cardiopulmonary Resuscitation and Emergency Cardiovascular Care Science With Treatment Recommendations T2 - Resuscitation TI - Pediatric Life Support: 2020 International Consensus on Cardiopulmonary Resuscitation and Emergency Cardiovascular Care Science With Treatment Recommendations UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85093701656&doi=10.1016%2fj.resuscitation.2020.09.013&partnerID=40&md5=6c7ba80fd5e9b9f7410d209f3ccd4f19 VL - 156 ID - 5882 ER - TY - JOUR AB - In order to evaluate physicians' willingness to seek legal action to mandate surgery when parents refuse surgery for various congenital heart lesions, we surveyed pediatric cardiologists and cardiovascular surgeons at 4 children's hospitals. We asked whether physicians would support parental refusal of surgery for specific heart defects and, if not, whether they would seek legal action to mandate surgery. We then analyzed associations between physicians' willingness to mandate surgery and national operative mortality rates for each lesion. We surveyed 126 cardiologists and 9 cardiac surgeons at four tertiary referral centers. Overall response rate was 77%. Greater than 70% of physicians would seek legal action and mandate surgery for the following lesions: ventricular septal defect, coarctation of the aorta, complete atrioventricular canal, transposition of the great arteries, tetralogy of Fallot, and unobstructed total anomalous pulmonary venous return. Surgery for all of these lesions has reported mortality rates of < 5%. Physicians were less likely to seek legal action when parents refused surgery for Shone complex, any single ventricle lesion, or any congenital heart disease accompanied by Trisomy 13 or Trisomy 18. Among experts in pediatric cardiology, there is widespread agreement about the appropriate response to parental refusal of surgery for most congenital heart lesions, and these lesions tended to be heart defects with lower surgical mortality rates. Lesions for which there was greater consensus among experts were those with the best outcomes. There was less consensus for lesions with higher mortality rates. Such surveys, revealing disagreement among expert professionals, can provide an operational definition of the current professional "gray zone" in which parental preferences should determine treatment. AD - Section of Cardiology, Children's Mercy Hospital, University of Missouri-Kansas City School of Medicine, Kansas City, MO, USA. Section of Cardiology, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, 225 E. Chicago Ave., Chicago, IL, 60611, USA. anpatel@luriechildrens.org. Center for Ethics, Children's Mercy Hospital, University of Missouri-Kansas City School of Medicine, Kansas City, MO, USA. Section of Cardiology, Baylor College of Medicine, Texas Children's Hospital, Houston, TX, USA. Section of Cardiology, Stanford University School of Medicine, Lucile Packard Children's Hospital, Stanford, CA, USA. AN - 32419096 AU - Swanson, T. M. AU - Patel, A. AU - Baxter, A. J. AU - Morris, S. A. AU - Maskatia, S. A. AU - Lantos, J. D. DA - Aug DB - UPubmed DO - 10.1007/s00246-020-02367-2 DP - NLM ET - 2020/05/19 IS - 6 KW - *Attitude of Health Personnel Cardiology/legislation & jurisprudence/*statistics & numerical data Child Heart Defects, Congenital/psychology/*surgery Humans Palliative Care/legislation & jurisprudence/*psychology Parents/psychology Surveys and Questionnaires Treatment Refusal/legislation & jurisprudence/*psychology Comfort care Congenital heart disease Ethics LA - eng N1 - 1432-1971 Swanson, Tara M Patel, Angira Orcid: 0000-0001-6760-0340 Baxter, Austin J Morris, Shaine A Maskatia, Shiraz A Lantos, John D Journal Article United States Pediatr Cardiol. 2020 Aug;41(6):1160-1165. doi: 10.1007/s00246-020-02367-2. Epub 2020 May 18. PY - 2020 SN - 0172-0643 SP - 1160-1165 ST - Pediatric Cardiology Specialist's Opinions Toward the Acceptability of Comfort Care for Congenital Heart Disease T2 - Pediatr Cardiol TI - Pediatric Cardiology Specialist's Opinions Toward the Acceptability of Comfort Care for Congenital Heart Disease VL - 41 ID - 2371 ER - TY - JOUR AB - PURPOSE: The purpose of this study was to explore parents' experiences of one specific timepoint in their infant's journey: the transition from hospital to home, following the first stage of their infant's cardiac surgery for complex congenital heart disease. DESIGN AND METHODS: A prospective longitudinal mixed methods study, underpinned with Middle Range Transition Theory (Meleis, Sawyer, Im, Hilfinger Messias, & Schumacher, 2000). Face to face and telephone interviews were conducted and self-report forms completed by parents at four-time points: before discharge (T0), 2weeks after discharge (T1), 8weeks after discharge (T2) and after stage two surgery (T3). Interviews were transcribed verbatim before inductive thematic analysis. RESULTS: Parents were recruited over a 15-month period from 2013 to 2015. Twelve mothers and 4 fathers took part. The infants had functionally univentricular heart (left n=10, right n=1) and a systemic shunt dependent lesion, tetralogy of Fallot (n=1). Dynamic constructivist and constructionist social processes occurred for all parents, involving physical, physiological, psychological and cognitive elements within four 'patterns of experience', two of which 'safety and security' and 'love and support' are presented in this paper. IMPLICATIONS: Parental support is essential; parents need to be engaged in discharge planning process and given the opportunity to express their needs to ensure that discharge care is truly patient and family centered. CONCLUSIONS: Transition from hospital to home was complex and multi-faceted, with unanticipated physical and emotional transitions superimposed upon those that were expected. AU - Gaskin, K. L. DB - UEmbase DO - 10.1016/j.pedn.2017.11.013 KW - article clinical article controlled study Fallot tetralogy father female genetic transcription heart single ventricle heart surgery hospital discharge human infant male mother prospective study security self report surgery telephone interview thematic analysis theoretical study LA - English M3 - Article N1 - L634142520 2021-02-16 PY - 2018 SN - 1532-8449 SP - e23-e32 ST - Patterns of Transition Experience for Parents Going Home from Hospital with their Infant after First Stage Surgery for Complex Congenital Heart Disease T2 - Journal of pediatric nursing TI - Patterns of Transition Experience for Parents Going Home from Hospital with their Infant after First Stage Surgery for Complex Congenital Heart Disease UR - https://www.embase.com/search/results?subaction=viewrecord&id=L634142520&from=export http://dx.doi.org/10.1016/j.pedn.2017.11.013 VL - 41 ID - 4552 ER - TY - JOUR AB - Children with single-ventricle heart disease (SVHD) are at risk for morbidity across multiple organ systems. A single-ventricle multidisciplinary clinic (SVMDC) may address complex health-care needs by providing access to, and coordination among, pediatric subspecialties. However, the patient and family experience of multidisciplinary care for SVHD remains unexplored. We e-mailed a 26-question survey to families after an SVMDC visit, which included evaluation with subspecialists from cardiology, pulmonology, gastroenterology, neuropsychology, and pediatric psychology, as well as social activities during clinic. Responses were anonymized to protect privacy, and data were analyzed quantitatively and qualitatively. Over 3 years, 22% (27/122) of families completed the survey. Overall, families' experiences were positive, with 100% reporting that they would recommend the SVMDC to others. Qualitative themes emerged regarding logistics, multidisciplinary care, key takeaways from clinic, and connection-making with other families. A multidisciplinary clinic demonstrated overall acceptability and perceived benefit to families of children with SVHD. Considerations for mixed experiences regarding financial commitment and connection-making among parents are discussed, as are the benefits of the synergy achieved through multidisciplinary care. AD - Neuroscience Institute, Section of Pediatric Neurology, Department of Pediatrics, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO, USA. RINGGOLD: 12225 Heart Institute, Section of Pediatric Cardiology, Department of Pediatrics, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO, USA. RINGGOLD: 12225 Breathing Institute, Section of Pulmonary Medicine, Department of Pediatrics, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO, USA. RINGGOLD: 12225 Digestive Health Institute, Section of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, University of Colorado School of Medicine, Children's Hospital Colorado Aurora, CO, USA. RINGGOLD: 12225 AN - 33457591 AU - Wolfe, K. R. AU - Kelly, S. L. AU - Liptzin, D. R. AU - Brigham, D. AU - Rafferty, C. AU - Younoszai, A. K. AU - Di Maria, M. V. C2 - PMC7786718 DA - Dec DB - UPubmed DO - 10.1177/2374373520904214 DP - NLM ET - 2021/01/19 IS - 6 KW - Fontan family experience survey hypoplastic left heart syndrome multidisciplinary clinic single ventricle of interest with respect to the research, authorship, and/or publication of this article. LA - eng N1 - 2374-3743 Wolfe, Kelly R Orcid: 0000-0003-4754-4577 Kelly, Sarah L Liptzin, Deborah R Brigham, Dania Rafferty, Carey Younoszai, Adel K Di Maria, Michael V Orcid: 0000-0003-0346-8517 Journal Article Review J Patient Exp. 2020 Dec;7(6):1384-1390. doi: 10.1177/2374373520904214. Epub 2020 Mar 2. PY - 2020 SN - 2374-3735 (Print) 2374-3735 SP - 1384-1390 ST - Patient and Family Experience in a Multidisciplinary Clinic for Children With Single-Ventricle Heart Disease T2 - J Patient Exp TI - Patient and Family Experience in a Multidisciplinary Clinic for Children With Single-Ventricle Heart Disease VL - 7 ID - 2346 ER - TY - JOUR AB - Aim: To describe leisure participation in adolescents with congenital heart defects (CHD) and identify factors associated with intensity of participation. Method: Eighty adolescents with CHD were recruited (39 males, 41 females; mean age [SD] 15y 8mo [1y 8mo] range 11y 5mo–19y 11mo) of whom 78 completed the Children’s Assessment of Participation and Enjoyment (CAPE) outcome measure of leisure participation. The measure has five subscales: recreational, active-physical, social, skill-based, and self-improvement. Associations between the CAPE and age, sex, and development were examined. Motor ability (Movement Assessment Battery for Children, Second Edition), cognition (Leiter International Performance Scale-Revised), behavior (Strengths and Difficulties Questionnaire), and motivation (Dimensions of Mastery Questionnaire) were assessed. Results: Participants exhibited impaired motor (43.5%), behavioral (23.7%), and cognitive (29.9%) development. The most intense participation was in social (mean [SD] 3.3 [0.99]) and recreational (2.9 [0.80]) activity types on the CAPE. Male sex (p<0.05) and younger age were associated with greater physical activity (<15y: 1.87; ≥15y: 1.31, p<0.05). Greater engagement in social activities was related to better cognition (r=0.28, p<0.05), higher motor function (r=0.30–0.36, p<0.01), and fewer behavioral difficulties (r=−0.32 to −0.47, p<0.01). Cognitive ability (r=0.27, p<0.05), dexterity and aiming/catching (r=0.27–0.33, p<0.05), and behavior problems (r=0.38–0.49, p=0.001) were correlated with physical activity participation. Persistence in tasks, an aspect of motivation, correlated with physical (r=0.45, p<0.001) and social activity involvement (r=0.28, p<0.05). Interpretation: Ongoing developmental impairments in adolescents with CHD are associated with decreased active-physical and social engagement, putting them at risk of poor physical and mental health. Health promotion strategies should be considered. What this paper adds: Adolescents with congenital heart defects (CHD) have limited engagement in active-physical leisure activities. Cognitive, motor, and behavioral impairments are associated with decreased participation in leisure in children with CHD. Female sex and older age are associated with less engagement in leisure. Mastery motivation correlates with participation, suggesting an avenue for intervention. AD - A. Majnemer, Research Institute of the McGill University Health Centre, Montreal Children’s Hospital, Montreal, QC, Canada A. Majnemer, School of Physical and Occupational Therapy, Faculty of Medicine, McGill University, Montreal, QC, Canada A. Majnemer, Centre for Interdisciplinary Research in Rehabilitation of Greater Montreal, Montreal, QC, Canada AU - Majnemer, A. AU - Rohlicek, C. AU - Dahan-Oliel, N. AU - Sahakian, S. AU - Mazer, B. AU - Maltais, D. B. AU - Schmitz, N. DB - UEmbase DO - 10.1111/dmcn.14422 IS - 8 KW - adolescent age article attention deficit disorder child cognitive defect cognitive development congenital heart malformation educational status Fallot tetralogy female hearing impairment heart single ventricle heart ventricle septum defect human learning disorder leisure major clinical study male motivation motor dysfunction motor performance open heart surgery outcome assessment physical activity priority journal problem behavior rehabilitation care sex difference social acceptance social behavior social participation LA - English M3 - Article N1 - L2003824005 2019-12-17 2020-07-16 PY - 2020 SN - 1469-8749 0012-1622 SP - 946-953 ST - Participation in leisure activities in adolescents with congenital heart defects T2 - Developmental Medicine and Child Neurology TI - Participation in leisure activities in adolescents with congenital heart defects UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2003824005&from=export http://dx.doi.org/10.1111/dmcn.14422 VL - 62 ID - 4399 ER - TY - JOUR AB - Purpose: Single ventricle heart disease is fraught with risk for infant mortality and morbidity. During the interstage period, or the time between palliative cardiac surgeries, mobile health (mHealth) technology improves the ability of registered nurse coordinators to monitor infant symptoms through parental monitoring and reporting. The concept of parental mHealth adherence to symptom home monitoring of infants with single ventricle congenital heart disease has not been defined, despite increasing use of mHealth technology. Methods: Rodger's concept analysis method was used to derive a unified definition of parental mHealth adherence to symptom home monitoring of infants with congenital heart disease during the single ventricle interstage period. A literature review included a search of databases for studies that addressed interstage home mHealth monitoring. Thematic analysis was applied to selected articles to derive a unified definition based on attributes, antecedents, consequences, related terms, and an illustrative case example. Sixteen publications were selected. Attributes, antecedents, and consequences of the concept were derived from the literature leading to a definition of parental mHealth adherence for infants with congenital heart disease during the single ventricle interstage period. The definition is the degree of adherence to which parents' transfer mHealth data for their infant meet healthcare providers' recommendations for symptom home monitoring. Consequences were improved infant symptom home monitoring through parental mHealth adherence measured by initiation, implementation, and discontinuation. Conclusion: The unified definition of the concept will provide a firmer ground for research in mHealth and interstage pediatric care and a guide for clinicians in developing new mHealth interventions for symptom home monitoring. Practice Implications: This concept analysis hypothesizes that infants with a single ventricle during the interstage period, whose parents are mHealth symptom home monitoring adherent, will have timelier symptom identification with resultant superior outcomes compared with infants with a single ventricle during the interstage period whose parents are not mHealth adherent. AD - L.A. Erickson, Ward Family Heart Center, Children's Mercy Hospital, Kansas City, MO, United States AU - Erickson, L. A. AU - Emerson, A. AU - Russell, C. L. DB - UEmbase DO - 10.1111/jspn.12303 IS - 4 KW - article child growth congenital heart disease family functioning field hospital health care delivery health care personnel health care system hospital readmission hospitalization human infant infant mortality medication compliance mobile application morbidity mortality oxygen saturation prenatal diagnosis priority journal randomized controlled trial (topic) risk factor social determinants of health telemedicine thematic analysis three dimensional echocardiography LA - English M3 - Article N1 - L2005558074 2020-07-22 PY - 2020 SN - 1744-6155 1539-0136 ST - Parental mobile health adherence to symptom home monitoring for infants with congenital heart disease during the single ventricle interstage period: A concept analysis T2 - Journal for Specialists in Pediatric Nursing TI - Parental mobile health adherence to symptom home monitoring for infants with congenital heart disease during the single ventricle interstage period: A concept analysis UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2005558074&from=export http://dx.doi.org/10.1111/jspn.12303 VL - 25 ID - 4390 ER - TY - JOUR AB - Background The unpredictable trajectory of pediatric advanced heart disease makes prognostication difficult for physicians and informed decision-making challenging for families. This study evaluated parent and physician understanding of disease burden and prognosis in hospitalized children with advanced heart disease. Methods and Results A longitudinal survey study of parents and physicians caring for patients with advanced heart disease age 30 days to 19 years admitted for ≥7 days was performed over a 1-year period (n=160 pairs). Percentage agreement and weighted kappa statistics were used to assess agreement. Median patient age was 1 year (interquartile range, 1-5), 39% had single-ventricle lesions, and 37% were in the cardiac intensive care unit. Although 92% of parents reported understanding their child's prognosis "extremely well" or "well," 28% of physicians thought parents understood the prognosis only "a little," "somewhat," or "not at all." Better parent-reported prognostic understanding was associated with greater preparedness for their child's medical problems (odds ratio, 4.7; 95% CI, 1.4-21.7, P=0.02). There was poor parent-physician agreement in assessing functional class, symptom burden, and likelihood of limitations in physical activity and learning/behavior; on average, parents were more optimistic. Many parents (47%) but few physicians (6%) expected the child to have normal life expectancy. Conclusions Parents and physicians caring for children with advanced heart disease differed in their perspectives regarding prognosis and disease burden. Physicians tended to underestimate the degree of parent-reported symptom burden. Parents were less likely to expect limitations in physical activity, learning/behavior, and life expectancy. Combined interventions involving patient-reported outcomes, parent education, and physician communication tools may be beneficial. AD - Division of Cardiology Department of Pediatrics Children's Hospital Los Angeles Los Angeles CA. Department of Cardiology Boston Children's Hospital Boston MA. AN - 33442989 AU - Morell, E. AU - Miller, M. K. AU - Lu, M. AU - Friedman, K. G. AU - Breitbart, R. E. AU - Reichman, J. R. AU - McDermott, J. AU - Sleeper, L. A. AU - Blume, E. D. C2 - PMC7955315 DA - Jan 19 DB - UPubmed DO - 10.1161/jaha.120.018488 DP - NLM ET - 2021/01/15 IS - 2 KW - communication congenital heart disease heart failure pediatrics prognosis quality of life LA - eng N1 - 2047-9980 Morell, Emily Miller, Mary Katherine Lu, Minmin Friedman, Kevin G Breitbart, Roger E Reichman, Jeffrey R McDermott, Julie Sleeper, Lynn A Blume, Elizabeth D Journal Article J Am Heart Assoc. 2021 Jan 19;10(2):e018488. doi: 10.1161/JAHA.120.018488. Epub 2021 Jan 14. PY - 2021 SN - 2047-9980 SP - e018488 ST - Parent and Physician Understanding of Prognosis in Hospitalized Children With Advanced Heart Disease T2 - J Am Heart Assoc TI - Parent and Physician Understanding of Prognosis in Hospitalized Children With Advanced Heart Disease VL - 10 ID - 2347 ER - TY - JOUR AB - Obstructed total anomalous pulmonary venous connection (TAPVC) involves anomalous drainage of all pulmonary veins to a location other than the left atrium and typically causes significant respiratory symptoms requiring urgent surgical repair. Premature infants born with obstructed TAPVC are often not considered to be suitable surgical candidates due to their size and therefore pose a significant treatment dilemma. Catheter-based interventions provide a temporizing option for infants with obstructed TAPVC, allowing for hemodynamic stabilization and growth until surgical intervention is feasible. We describe the course of two premature infants with obstructed TAPVC who were palliated with stenting of their anomalous pulmonary veins. Both infants required repeat catheter-based intervention for recurrent stenosis and ultimately underwent successful surgical repair at an acceptable weight and corrected gestational age. We propose that a novel catheter-based approach to initial management may be a beneficial option in the premature and low-weight patient population. AD - R.C. Chamberlain, Division of Pediatric Cardiology, Department of Pediatrics, Duke University Medical Center, Durham, NC, United States AU - Chamberlain, R. C. AU - Hill, K. D. AU - Fleming, G. A. DB - UEmbase DO - 10.1177/2150135118782191 IS - 4 KW - bare metal coronary stent enoxaparin heparin sildenafil article brain hemorrhage case report clinical article ductus venosus echocardiography follow up Fontan procedure heart catheterization hemodynamics hospitalization human hydrocephalus infant length of stay lung artery pressure lung vein drainage anomaly male oxygen saturation percutaneous transluminal angioplasty prematurity priority journal quality of life venous pressure LA - English M3 - Article N1 - L623910034 2018-09-21 PY - 2020 SN - 2150-136X 2150-1351 SP - NP164-NP167 ST - Palliating Premature Infants With Obstructed Total Anomalous Pulmonary Venous Connection via Catheterization T2 - World Journal for Pediatric and Congenital Heart Surgery TI - Palliating Premature Infants With Obstructed Total Anomalous Pulmonary Venous Connection via Catheterization UR - https://www.embase.com/search/results?subaction=viewrecord&id=L623910034&from=export http://dx.doi.org/10.1177/2150135118782191 VL - 11 ID - 4406 ER - TY - JOUR AB - Background: Compared with those born at term gestation, infants with complex congenital heart defects (CCHD) who were delivered before 37 weeks gestational age and received neonatal open-heart surgery (OHS) have poorer neurodevelopmental outcomes in early childhood. We aimed to describe the growth, disability, functional, and neurodevelopmental outcomes in early childhood of preterm infants with CCHD after neonatal OHS. Prediction models were evaluated at various timepoints during hospitalization which could be useful in the management of these infants. Study Design: We studied all preterm infants with CCHD who received OHS within 6 weeks of corrected age between 1996 and 2016. The Western Canadian Complex Pediatric Therapies Follow-up Program completed multidisciplinary comprehensive neurodevelopmental assessments at 2-year corrected age at the referral-site follow-up clinics. We collected demographic and acute-care clinical data, standardized age-appropriate outcome measures including physical growth with calculated z-scores; disabilities including cerebral palsy, visual impairment, permanent hearing loss; adaptive function (Adaptive Behavior Assessment System-II); and cognitive, language, and motor skills (Bayley Scales of Infant and Toddler Development-III). Multiple variable logistic or linear regressions determined predictors displayed as Odds Ratio (OR) or Effect Size (ES) with 95% confidence intervals. Results: Of 115 preterm infants (34 ± 2 weeks gestation, 2,339 ± 637 g, 64% males) with CCHD and OHS, there were 11(10%) deaths before first discharge and 21(18%) deaths by 2-years. Seven (6%) neonates had cerebral injuries, 7 had necrotizing enterocolitis; none had retinopathy of prematurity. Among 94 survivors, 9% had cerebral palsy and 6% had permanent hearing loss, with worse outcomes in those with syndromic diagnoses. Significant predictors of mortality included birth weight z-score [OR 0.28(0.11,0.72), P = 0.008], single-ventricle anatomy [OR 5.92(1.31,26.80), P = 0.021], post-operative ventilation days [OR 1.06(1.02,1.09), P = 0.007], and cardiopulmonary resuscitation [OR 11.58 (1.97,68.24), P = 0.007]; for adverse functional outcome in those without syndromic diagnoses, birth weight 2,000–2,499 g [ES −11.60(−18.67, −4.53), P = 0.002], post-conceptual age [ES −0.11(−0.22,0.00), P = 0.044], post-operative lowest pH [ES 6.75(1.25,12.25), P = 0.017], and sepsis [ES −9.70(−17.74, −1.66), P = 0.050]. Conclusions: Our findings suggest preterm neonates with CCHD and early OHS had significant mortality and morbidity at 2-years and were at risk for cerebral palsy and adverse neurodevelopment. This information may be important for management, parental counseling and the decision-making process. AD - P.-Y. Cheung, Department of Pediatrics, University of Alberta, Edmonton, AB, Canada P.-Y. Cheung, NICU, Northern Alberta Neonatal Program of Alberta Health Services, Edmonton, AB, Canada AU - Cheung, P. Y. AU - Hajihosseini, M. AU - Dinu, I. A. AU - Switzer, H. AU - Joffe, A. R. AU - Bond, G. Y. AU - Robertson, C. M. T. DB - UEmbase DO - 10.3389/fped.2020.616659 KW - adaptive behavior article artificial ventilation Bayley Scales of Infant Development birth weight brain injury cerebral palsy child childhood controlled study counseling decision making demography effect size emergency care female follow up hearing impairment heart single ventricle hospitalization human infant language linear regression analysis major clinical study male morbidity mortality motor performance necrotizing enterocolitis nervous system development newborn open heart surgery patient referral prediction pregnancy resuscitation retrolental fibroplasia risk factor sepsis surgery survivor visual impairment LA - English M3 - Article N1 - L634149605 2021-02-24 PY - 2020 SN - 2296-2360 ST - Outcomes of Preterm Infants With Congenital Heart Defects After Early Surgery: Defining Risk Factors at Different Time Points During Hospitalization T2 - Frontiers in Pediatrics TI - Outcomes of Preterm Infants With Congenital Heart Defects After Early Surgery: Defining Risk Factors at Different Time Points During Hospitalization UR - https://www.embase.com/search/results?subaction=viewrecord&id=L634149605&from=export http://dx.doi.org/10.3389/fped.2020.616659 VL - 8 ID - 4358 ER - TY - JOUR AB - OBJECTIVES: The modified extracardiac Fontan of direct total cavopulmonary connection (dTCPC) with entirely autologous vessels is a better solution in selected patients with functionally univentricular hearts because it combines the advantages of a tubular connection and of full growth potential. We investigated the mid-term to long-term outcomes of the physical condition of patients who had the dTCPC and assessed the growth potential of the autologous dTCPC pathway. METHODS: From July 2005 to June 2014, 31 patients, aged 1.8-14.0 years, underwent a modified extracardiac Fontan with dTCPC at our institution. Twenty-two patients underwent a 1-stage dTCPC and 9 patients underwent a 2-stage dTCPC. The preoperative and postoperative data were reviewed retrospectively. RESULTS: There were 17 (54.8%) boys and 14 (45.2%) girls, with a median age of 6.4 years (range 1.8-14.0 years) and a mean weight of 17.8 kg (range 9-41 kg). The mean follow-up period was 6.0 years (range 2.1-10.2 years). There was 1 early death and 2 late deaths. The event-free survival rate for the 31 patients was 88.9% at 10 years during the follow-up period. Late protein-losing enteropathy, thromboembolism, arrhythmia or heart failure were not observed. There was a significant difference between the preoperative and postoperative data for body mass index and the diameters of the autologous dTCPC pathway and pulmonary artery branches, confirming that the growth potential of the autologous dTCPC pathway was realized. CONCLUSIONS: The dTCPC procedure could be performed with satisfactory midterm to long-term outcomes in selected patients. It has the potential of retaining the advantage of the extracardiac Fontan operation together with the potential for growth and the avoidance of prosthetic materials. AU - Liu, X. AU - Yuan, H. AU - Chen, J. AU - Cen, J. AU - Nie, Z. AU - Xu, G. AU - Wen, S. AU - Zhuang, J. DB - OEmbase DO - 10.1093/ejcts/ezz310 IS - 4 KW - adolescent article avoidance behavior body mass case report cavopulmonary connection child clinical article event free survival female follow up Fontan procedure heart arrhythmia heart single ventricle human infant male preoperative evaluation preschool child prosthesis material protein losing gastroenteropathy pulmonary artery retrospective study thromboembolism treatment failure LA - English M3 - Article N1 - L629889238 2019-11-22 PY - 2020 SN - 1873-734X SP - 628-634 ST - Outcomes following modified extracardiac Fontan procedure of direct total cavopulmonary connection with autologous vessels: a single-centre 10-year experience T2 - European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery TI - Outcomes following modified extracardiac Fontan procedure of direct total cavopulmonary connection with autologous vessels: a single-centre 10-year experience UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L629889238 http://dx.doi.org/10.1093/ejcts/ezz310 VL - 57 ID - 3473 ER - TY - JOUR AB - BACKGROUND: Survivors of single ventricle heart disease must cope with the physical, neurodevelopmental, and psychosocial sequelae of their cardiac disease, which may also affect academic achievement and social relationships. The purpose of this study was to qualitatively examine the experiences of school and social relationships in adolescents with single ventricle heart disease. METHODS: A descriptive phenomenological methodology was employed, utilising semi-structured interviews. Demographic and clinical characteristics were obtained via chart review. RESULTS: Fourteen adolescents (aged 14 to 19 years) with single ventricle heart disease participated. Interviews ranged from 25 to 80 minutes in duration. Four themes emerged from the interviews, including "Don't assume": Pervasive ableism; "The elephant in the room": Uncertain future; "Everyone finds something to pick on": Bullying at school; "They know what I have been through": Social support. The overall essence generated from the data was "optimism despite profound uncertainty." CONCLUSIONS: Adolescents with single ventricle heart disease identified physical limitations and school challenges in the face of an uncertain health-related future. Despite physical and psychosocial limitations, most remained optimistic for the future and found activities that were congruent with their abilities. These experiences reflect "optimism despite profound uncertainty." AD - Johns Hopkins University School of Nursing, Baltimore, MD, USA. Sue and Bill Gross School of Nursing, University of California, Irvine, CA, USA. University of Texas Medical Branch School of Nursing, Galveston, TX, USA. School of Nursing, University of California, Los Angeles, CA, USA. Cardiothoracic Surgery, Children's Hospital, Los Angeles, CA, USA. AN - 33176897 AU - Peterson, J. K. AU - Olshansky, E. F. AU - Guo, Y. AU - Evangelista, L. S. AU - Pike, N. A. C2 - PMC7897217 C6 - NIHMS1637170 DA - Feb DB - UPubmed DO - 10.1017/s1047951120003790 DP - NLM ET - 2020/11/13 IS - 2 KW - Fontan procedure academic achievement peer relationships qualitative research LA - eng N1 - 1467-1107 Peterson, Jennifer K Orcid: 0000-0001-7628-567x Olshansky, Ellen F Guo, Yuqing Evangelista, Lorraine S Pike, Nancy A 15PRE25300024/AHA/American Heart Association-American Stroke Association/United States R01 NR013930/NR/NINR NIH HHS/United States R01 NR016463/NR/NINR NIH HHS/United States Journal Article Cardiol Young. 2021 Feb;31(2):252-259. doi: 10.1017/S1047951120003790. Epub 2020 Nov 12. PY - 2021 SN - 1047-9511 (Print) 1047-9511 SP - 252-259 ST - Optimism despite profound uncertainty: school and social relationships in adolescents with single ventricle heart disease T2 - Cardiol Young TI - Optimism despite profound uncertainty: school and social relationships in adolescents with single ventricle heart disease VL - 31 ID - 2352 ER - TY - JOUR AB - Growth failure is a widespread health problem in infants with congenital heart disease, specifically infants with single ventricle heart lesions, including hypoplastic left heart syndrome. Optimizing nutrition delivery for neonates undergoing cardiac surgery is challenging given environmental and physiologic barriers. Concomitant with nutrition delivery challenges, wide practice variations exist among clinicians. Malnutrition and poor growth are associated with poor wound healing, increased infection risk, prolonged hospitalizations, and long-term neurodevelopmental disability, including worse school performance. The cause of early postnatal growth failure is multifactorial and related to the delay in onset of enteral feedings, increased metabolic stress of cardiac surgery, cyanosis, low cardiac output, increased energy expenditure, altered splanchnic perfusion, and gastrointestinal morbidities. In addition, cardiac infants are susceptible to unique complications, including chylothorax, vocal cord dysmotility, and swallowing dysfunction. © 2015 by the American Academy of Pediatrics. All rights reserved. AD - Department of Pediatrics, Medical University of South Carolina, Charleston, SC, United States AU - Fogg, K. L. AU - Zyblewski, S. C. DB - Scopus DO - 10.1542/neo.16-3-e144 IS - 3 M3 - Article N1 - Cited By :1 Export Date: 15 June 2020 PY - 2015 SP - e144-e151 ST - Nutrition and growth in neonates undergoing cardiac surgery T2 - NeoReviews TI - Nutrition and growth in neonates undergoing cardiac surgery UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84923932271&doi=10.1542%2fneo.16-3-e144&partnerID=40&md5=a7bcb84f57b5e070480c7f27c742455c VL - 16 ID - 6815 ER - TY - JOUR AB - We describe the use of liposomal bupivacaine (Exparel) in erector spinae plane blocks for two patients undergoing pediatric cardiac surgery with cardiopulmonary bypass and one undergoing division of the compressive vascular ring. The perioperative course of all patients was remarkable for low pain and sedation scores, especially after chest tube removal. Erector spinae plane blocks are an expanding pain-control technique in both adult and pediatric cardiac surgery for postoperative analgesia. Liposomal bupivacaine offers prolonged analgesia and may be an attractive option for this indication. AD - S. Voulgarelis, Department of Anesthesiology, Division of Pediatric Anesthesiology, Children's of Wisconsin, Medical College of Wisconsin, Milwaukee, WI, United States AU - Voulgarelis, S. AU - Halenda, G. M. AU - Tanem, J. M. C1 - exparel DB - UEmbase DO - 10.1155/2021/5521136 KW - chest tube mediastinal drainage catheter bupivacaine dexmedetomidine epinephrine milrinone morphine aortic stenosis article autism cardiopulmonary bypass case report child choana atresia chromosome 13 chromosome deletion clinical article congenital heart disease controlled study Dandy Walker syndrome developmental delay Ebstein anomaly erector spinae muscle erector spinae plane block female Glenn shunt heart right ventricle double outlet heart surgery heterotaxy syndrome horseshoe kidney human hypertension hypoplastic left heart syndrome infant liposomal delivery male nerve block pediatric surgery postoperative analgesia pulmonary valve stenosis school child sedation syndrome CHARGE transluminal valvuloplasty vascular ring exparel LA - English M3 - Article N1 - L2011493410 2021-04-02 2021-04-19 PY - 2021 SN - 2090-6390 2090-6382 ST - A Novel Use of Liposomal Bupivacaine in Erector Spinae Plane Block for Pediatric Congenital Cardiac Surgery T2 - Case Reports in Anesthesiology TI - A Novel Use of Liposomal Bupivacaine in Erector Spinae Plane Block for Pediatric Congenital Cardiac Surgery UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2011493410&from=export http://dx.doi.org/10.1155/2021/5521136 VL - 2021 ID - 4369 ER - TY - JOUR AB - The Fontan procedure is a common palliative surgery for congenital single ventricle patients. In silico and in vitro patient-specific modeling approaches are widely utilized to investigate potential improvements of Fontan hemodynamics that are related to long-term complications. However, there is a lack of consensus regarding the use of non-Newtonian rheology, warranting a systematic investigation. This study conducted in silico patient-specific modeling for twelve Fontan patients, using a Newtonian and a non-Newtonian model for each patient. Differences were quantified by examining clinically relevant metrics: indexed power loss (iPL), indexed viscous dissipation rate (iVDR), hepatic flow distribution (HFD), and regions of low wall shear stress (AWSS). Four sets of “non-Newtonian importance factors” were calculated to explore their effectiveness in identifying the non-Newtonian effect. No statistical differences were observed in iPL, iVDR, and HFD between the two models at the population-level, but large inter-patient variations exist. Significant differences were detected regarding AWSS, and its correlations with non-Newtonian importance factors were discussed. Additionally, simulations using the non-Newtonian model were computationally faster than those using the Newtonian model. These findings distinguish good importance factors for identifying non-Newtonian rheology and encourage the use of a non-Newtonian model to assess Fontan hemodynamics. AD - A.P. Yoganathan, Wallace H. Coulter School of Biomedical Engineering, Georgia Institute of Technology, 313 Ferst Drive NW, Atlanta, GA, United States AU - Wei, Z. AU - Singh-Gryzbon, S. AU - Trusty, P. M. AU - Huddleston, C. AU - Zhang, Y. AU - Fogel, M. A. AU - Veneziani, A. AU - Yoganathan, A. P. DB - UEmbase DO - 10.1007/s10439-020-02527-8 IS - 8 KW - adolescent article clinical article computational fluid dynamics computer model female Fontan procedure hemodynamic parameters hepatic flow distribution human indexed power loss indexed viscous dissipation rate male Newtonian model nuclear magnetic resonance imaging priority journal regions of low wall shear stress LA - English M3 - Article N1 - L2004844871 2020-05-26 2020-08-18 PY - 2020 SN - 1573-9686 0090-6964 SP - 2204-2217 ST - Non-Newtonian Effects on Patient-Specific Modeling of Fontan Hemodynamics T2 - Annals of Biomedical Engineering TI - Non-Newtonian Effects on Patient-Specific Modeling of Fontan Hemodynamics UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2004844871&from=export http://dx.doi.org/10.1007/s10439-020-02527-8 VL - 48 ID - 4400 ER - TY - JOUR AB - BACKGROUND: Both congenital heart disease (CHD) and very low birth weight (VLBW) infants are very high risk for neurodevelopmental delay. We investigated neurological development at 3 years among pediatric patients with CHD after surgical intervention, very low birth weight (VLBW), and healthy controls. METHODS: We enrolled pediatric patients with CHD (n = 67), VLBW (n = 67), and healthy controls (n = 81). Infants with CHD were grouped into those with single ventricle and two ventricles, and infants with VLBW were grouped into those with birth weights of <1000 and 1000-1499 g. Neurodevelopmental outcomes at 3 years were evaluated using the Bayley Scales of Infant and Toddler Development, Third Edition. RESULTS: Compared with healthy controls, a significant deficit in the language, cognition, and motor skills scores were observed in infants with CHD and VLBW. Infants with a single ventricle exhibited significantly low scores in language and gross motor skills. No statistically significant difference was observed between the birth weight groups of <1000 and 1000-1499 g. CONCLUSION: Neurodevelopmental outcomes for infants with both CHD and VLBW showed impairment. Notably, neurodevelopmental delays in infants with a single ventricle were remarkable. Thus, because infants with both CHD and VLBW are at high risk for neurodevelopmental disorders, periodic developmental screenings and support are warranted for these children. (215 words). AU - Yoshida, T. AU - Hiraiwa, A. AU - Ibuki, K. AU - Makimoto, M. AU - Inomata, S. AU - Tamura, K. AU - Kawasaki, Y. AU - Ozawa, S. AU - Hirono, K. AU - Ichida, F. DB - OEmbase DO - 10.1111/ped.14160 KW - article Bayley Scales of Infant Development child cognition controlled study developmental screening female heart single ventricle human infant language major clinical study male motor performance nervous system malformation pediatric patient surgery very low birth weight LA - English M3 - Article in Press N1 - L630663476 2020-01-24 PY - 2020 SN - 1442-200X ST - Neurodevelopmental outcomes at three years for infants with CHD and VLBW T2 - Pediatrics international : official journal of the Japan Pediatric Society TI - Neurodevelopmental outcomes at three years for infants with CHD and VLBW UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L630663476 http://dx.doi.org/10.1111/ped.14160 ID - 3480 ER - TY - JOUR AB - Background: Both congenital heart disease (CHD) and very‐low birthweight (VLBW) infants are at a very high risk of neurodevelopmental delay. We investigated neurological development at 3 years in pediatric patients with CHD after surgical intervention, those of VLBW, and healthy controls. Methods: We enrolled pediatric patients with CHD (n = 67), VLBW (n = 67), and healthy controls (n = 81). Infants with CHD were grouped into those with single ventricle and two ventricles, and infants with VLBW were grouped into those with birthweights of <1000 and 1000–1499 g. Neurodevelopmental outcomes at 3 years were evaluated using the Bayley Scales of Infant and Toddler Development, Third Edition. Results: Compared with healthy controls, a significant deficit in the language, cognition, and motor skills scores were observed in infants with CHD and VLBW. Infants with a single ventricle exhibited significantly low scores in language and gross motor skills. No statistically significant difference was observed between the birthweight groups of <1000 and 1000–1499 g. Conclusion: Neurodevelopmental outcomes for infants with both CHD and VLBW showed impairment. Notably, neurodevelopmental delays in infants with a single ventricle were remarkable. Thus, because infants with both CHD and VLBW are at high risk of neurodevelopmental disorders, periodic developmental screenings and support are warranted for these children. AD - Division of Neonatology, Maternal and Perinatal Center, Toyama University Hospital, Toyama, Japan Department of Pediatrics, Toyama University, Toyama, Japan AN - 144749354. Language: English. Entry Date: 20200801. Revision Date: 20200801. Publication Type: Article AU - Yoshida, Taketoshi AU - Hiraiwa, Akiko AU - Ibuki, Keijiro AU - Makimoto, Masami AU - Inomata, Satomi AU - Tamura, Kentaro AU - Kawasaki, Yukako AU - Ozawa, Sayaka AU - Hirono, Keiichi AU - Ichida, Fukiko DB - Ucinahl DO - 10.1111/ped.14160 DP - EBSCOhost IS - 7 KW - Heart Defects, Congenital -- Complications Postoperative Complications Infant, Very Low Birth Weight Mental Disorders -- Risk Factors Risk Assessment Human Infant Child, Preschool Heart Ventricle -- Pathology Scales Language Disorders -- Diagnosis Cognition Disorders -- Diagnosis Motor Skills Disorders -- Diagnosis Diagnosis, Developmental N1 - research; tables/charts. Journal Subset: Asia; Biomedical; Peer Reviewed. Instrumentation: Bayley Scales of Infant and Toddler Development, Third edition (Bayley-III). NLM UID: 100886002. PY - 2020 SN - 1328-8067 SP - 797-803 ST - Neurodevelopmental outcomes at 3 years for infants with congenital heart disease and very‐low birthweight T2 - Pediatrics International TI - Neurodevelopmental outcomes at 3 years for infants with congenital heart disease and very‐low birthweight UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=144749354&site=ehost-live&scope=site VL - 62 ID - 5323 ER - TY - JOUR AB - BACKGROUND: Both congenital heart disease (CHD) and very-low birthweight (VLBW) infants are at a very high risk of neurodevelopmental delay. We investigated neurological development at 3 years in pediatric patients with CHD after surgical intervention, those of VLBW, and healthy controls. METHODS: We enrolled pediatric patients with CHD (n = 67), VLBW (n = 67), and healthy controls (n = 81). Infants with CHD were grouped into those with single ventricle and two ventricles, and infants with VLBW were grouped into those with birthweights of <1000 and 1000-1499 g. Neurodevelopmental outcomes at 3 years were evaluated using the Bayley Scales of Infant and Toddler Development, Third Edition. RESULTS: Compared with healthy controls, a significant deficit in the language, cognition, and motor skills scores were observed in infants with CHD and VLBW. Infants with a single ventricle exhibited significantly low scores in language and gross motor skills. No statistically significant difference was observed between the birthweight groups of <1000 and 1000-1499 g. CONCLUSION: Neurodevelopmental outcomes for infants with both CHD and VLBW showed impairment. Notably, neurodevelopmental delays in infants with a single ventricle were remarkable. Thus, because infants with both CHD and VLBW are at high risk of neurodevelopmental disorders, periodic developmental screenings and support are warranted for these children. AD - Division of Neonatology, Maternal and Perinatal Center, Toyama University Hospital, Toyama, Japan. Department of Pediatrics, Toyama University, Toyama, Japan. AN - 31957091 AU - Yoshida, T. AU - Hiraiwa, A. AU - Ibuki, K. AU - Makimoto, M. AU - Inomata, S. AU - Tamura, K. AU - Kawasaki, Y. AU - Ozawa, S. AU - Hirono, K. AU - Ichida, F. DA - Jul DB - UPubmed DO - 10.1111/ped.14160 DP - NLM ET - 2020/01/21 IS - 7 KW - Cardiac Surgical Procedures/methods Child Development Child, Preschool Cognition Developmental Disabilities/*epidemiology Female Follow-Up Studies Heart Defects, Congenital/*epidemiology/surgery Humans Infant Infant, Newborn *Infant, Very Low Birth Weight Male Motor Skills Neurodevelopmental Disorders/epidemiology Neuropsychological Tests Risk Factors Bayley Scales congenital heart disease neurodevelopmental outcomes very-low-birthweight infant LA - eng N1 - 1442-200x Yoshida, Taketoshi Orcid: 0000-0002-9937-1703 Hiraiwa, Akiko Ibuki, Keijiro Makimoto, Masami Inomata, Satomi Tamura, Kentaro Kawasaki, Yukako Ozawa, Sayaka Hirono, Keiichi Ichida, Fukiko Journal Article Australia Pediatr Int. 2020 Jul;62(7):797-803. doi: 10.1111/ped.14160. Epub 2020 Jun 22. PY - 2020 SN - 1328-8067 SP - 797-803 ST - Neurodevelopmental outcomes at 3 years for infants with congenital heart disease and very-low birthweight T2 - Pediatr Int TI - Neurodevelopmental outcomes at 3 years for infants with congenital heart disease and very-low birthweight VL - 62 ID - 2384 ER - TY - JOUR AB - Purpose: To evaluate the role of the ophthalmologist in the management of children with arterial stroke, at presentation and during follow-up. Methods: This retrospective case series comprised children with arterial stroke who were followed for at least 12 months in a tertiary pediatric medical center in 2005-2016. Demographic data and findings on radiological neuroimaging and ophthalmological and neurological examination were retrieved from the medical files. Results: The cohort included 26 children with stroke. Underlying disorders included metabolic syndrome (n = 5, 19.2%), cardiac anomaly or Fontan repair (n = 3 each, 11.5%), vascular anomaly (n = 3, 11.5%), head trauma with traumatic dissection (n = 3, 11.5%), and hypercoagulability (n = 1, 3.8%); in eight patients (30.8%), no apparent cause was found. Eleven patients (42.3%) had a non-ophthalmological neurological deficit as a result of the stroke. Eye examination was performed in nine patients (34.6%) during follow-up. Ophthalmological manifestations included hemianopic visual field defect in seven patients (7.7%) and complete blindness and poor visual acuity in one patient each (3.8%). At the last visit, no change in visual function was detected. Conclusion: The variable etiology and presentation of pediatric stroke may mask specific visual signs. Children with arterial stroke should be referred for early ophthalmological evaluation and visual rehabilitation. AD - Department of Radiology, Rabin Medical Center - Beilinson Hospital, Petach Tikva, Israel. Responsable de l'Unité Fonctionnelle Vision et Cognition Service de Neurologie Fondation Ophtalmologique Rothschild, Paris, France. Department of Neurosurgery, Hadassah Hebrew University, Jerusalem, Israel. Pediatric Oncology, Schneider Children's Medical Center of Israel, Petach Tikva, Israel. Department of Ophthalmology, Rabin Medical Center - Beilinson Hospital, Petach Tikva, Israel. Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. Krieger Eye Research Laboratory, Felsenstein Medical Research Center, Rabin Medical Center, Petach Tikva, Israel. Department of Ophthalmology, Bnai Zion Medical Center, Haifa, Israel. Faculty of Medicine, Technion - Israel Institute of Technology, Haifa, Israel. AN - 32714272 AU - Luckman, J. AU - Chokron, S. AU - Michowiz, S. AU - Belenky, E. AU - Toledano, H. AU - Zahavi, A. AU - Goldenberg-Cohen, N. C2 - PMC7343911 DB - UPubmed DO - 10.3389/fneur.2020.00617 DP - NLM ET - 2020/07/28 KW - arterial stroke children cortical visual impairment eye exam ophthalmological complication LA - eng N1 - 1664-2295 Luckman, Judith Chokron, Sylvie Michowiz, Shalom Belenky, Eugenia Toledano, Helen Zahavi, Alon Goldenberg-Cohen, Nitza Journal Article Front Neurol. 2020 Jul 2;11:617. doi: 10.3389/fneur.2020.00617. eCollection 2020. PY - 2020 SN - 1664-2295 (Print) 1664-2295 SP - 617 ST - The Need to Look for Visual Deficit After Stroke in Children T2 - Front Neurol TI - The Need to Look for Visual Deficit After Stroke in Children VL - 11 ID - 2358 ER - TY - JOUR AB - Objective: N-terminal pro-brain natriuretic peptide has an established role in the diagnosis and prognosis of heart failure. In Fontan patients, this peptide is often increased, but its diagnostic value in this particular non-physiologic, univentricular circulation is unclear. We investigated whether N-terminal pro-brain natriuretic peptide represents ventricular function or other key variables in Fontan patients.Methods and results: Ninety-five consecutive Fontan patients ≥10 years old who attended the outpatient clinic of the Center for Congenital Heart Diseases in 2012-2013 were included. Time since Fontan completion was 16 ± 9 years. Median N-terminal pro-brain natriuretic peptide was 114 (61-264) ng/l and was higher than gender-and age-dependent normal values in 54% of the patients. Peptide Z-scores were higher in patients in NYHA class III/IV compared to those in class I/II, but did not correlate with ventricular function assessed by MRI and echocardiography, nor with peak exercise capacity. Instead, peptide Z-scores significantly correlated with follow-up duration after Fontan completion (p < 0.001), right ventricular morphology (p = 0.004), indexed ventricular mass (p = 0.001), and inferior caval vein diameter (p < 0.001) (adjusted R= 0.615).Conclusions: N-terminal pro-brain natriuretic peptide levels in Fontan patients correlate with functional class, but do not necessarily indicate ventricular dysfunction. Increased peptide levels were associated with a longer existence of the Fontan circulation, morphologic ventricular characteristics, and signs of increased systemic venous congestion. Since the latter are known to be key determinants of the performance of the Fontan circulation, these findings suggest increase in N-terminal pro-brain natriuretic peptide levels to indicate attrition of the Fontan circulation, independent of ventricular function. AD - D. Wolff, Center for Congenital Heart Diseases, Department of Pediatric Cardiology/Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, Netherlands AU - Wolff, D. AU - Van Melle, J. P. AU - Willems, T. P. AU - Bartelds, B. AU - Ploegstra, M. J. AU - Hillege, H. AU - Ebels, T. AU - Berger, R. M. F. DB - UEmbase DO - 10.1017/S1047951120000657 IS - 6 KW - nuclear magnetic resonance scanner amino terminal pro brain natriuretic peptide adolescent adult aging article child comparative study cross-sectional study echocardiography exercise female follow up Fontan procedure heart left ventricle mass heart right ventricle heart ventricle function human inferior cava vein major clinical study male New York Heart Association class nuclear magnetic resonance imaging outpatient department patient attendance Peptide Z score protein analysis protein blood level school child scoring system sex ratio vein diameter venous congestion LA - English M3 - Article N1 - L632172461 2020-07-09 2020-09-25 PY - 2020 SN - 1467-1107 1047-9511 SP - 753-760 ST - N-terminal pro-brain natriuretic peptide serum levels reflect attrition of the Fontan circulation T2 - Cardiology in the Young TI - N-terminal pro-brain natriuretic peptide serum levels reflect attrition of the Fontan circulation UR - https://www.embase.com/search/results?subaction=viewrecord&id=L632172461&from=export http://dx.doi.org/10.1017/S1047951120000657 VL - 30 ID - 4416 ER - TY - JOUR AB - Objective: To evaluate how outcomes changed in newborns undergoing surgery for congenital heart disease after implementation of a standardized preoperative and postoperative nutrition program. Study design: We performed a single-center cohort study of newborns who underwent cardiac surgery between September 2008 and July 2015. We evaluated growth and feeding outcomes in the 2 years of preprogram time (phase 0), in the 2 years after initiation of a postoperative feeding algorithm (phase 1), and in the 2 years following introduction of a preoperative feeding program (phase 2) using traditional statistics and quality improvement methods. Results: The study included 570 newborns with congenital heart disease. Weight-for-age z-score change from birth to hospital discharge significantly improved from phase 0 (−1.02 [IQR, −1.45 to −0.63]) to phase 1 (−0.83 [IQR, −1.25 to −0.54]; P = .006), with this improvement maintained in phase 2 (−0.89 [IQR, −1.30 to −0.56]; P = .017 across phases). Gastrostomy tube use decreased significantly (25% in phase 0 vs 12% and 14% in phases 1 and 2; P < .001) and preoperative enteral feeding increased significantly (47% and 46% in phases 0 and 1 vs 76% in phase 2; P < .001) without increases in necrotizing enterocolitis, hospital stay, or mortality. Conclusions: Introduction of a multi-interventional nutrition program was associated with improved weight gain, fewer gastrostomy tubes at hospital discharge, and increased preoperative enteral feeding without increases in necrotizing enterocolitis, hospital stay, or mortality. AD - S.A.S. Killen, Monroe Carell Jr. Children's Hospital at Vanderbilt, 2200 Children's Way, Suite 5230, Nashville, TN, United States AU - O'Neal Maynord, P. AU - Johnson, M. AU - Xu, M. AU - Slaughter, J. C. AU - Killen, S. A. S. DB - UEmbase DO - 10.1016/j.jpeds.2020.08.039 KW - feeding tube nasogastric tube stomach tube age article body weight child growth child nutrition clinical outcome cohort analysis congenital heart disease controlled study donor milk enteric feeding feeding behavior feeding difficulty female follow up heart single ventricle heart surgery high risk infant hospital discharge hospital readmission hospitalization human length of stay lung blood flow major clinical study male multidisciplinary team necrotizing enterocolitis newborn newborn intensive care newborn mortality newborn surgery patent ductus arteriosus postoperative care postoperative period priority journal stakeholder engagement standardization surgical mortality total quality management LA - English M3 - Article N1 - L2008042130 2020-10-19 2021-04-02 PY - 2021 SN - 1097-6833 0022-3476 SP - 66-73.e2 ST - A Multi-Interventional Nutrition Program for Newborns with Congenital Heart Disease T2 - Journal of Pediatrics TI - A Multi-Interventional Nutrition Program for Newborns with Congenital Heart Disease UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2008042130&from=export http://dx.doi.org/10.1016/j.jpeds.2020.08.039 VL - 228 ID - 4364 ER - TY - JOUR AB - Background and Objectives: This study aimed to provide morbidity and mortality information on very low birth weight (VLBW) infants with congenital heart disease (CHD-VLBWs). Methods: The study used a 10-year cohort of VLBW infants from a single institution. CHD was classified according to International Classification of Diseases, Version 9, Clinical Modification. Mortality and neonatal outcomes were assessed by comparing the CHD-VLBWs with gestational age- and birth weight-matched controls. Results: The prevalence of CHD-VLBWs was 7.5% (79/1,050), mean gestational age was 31.1±3.2 weeks, and mean birth weight was 1,126.2±268.3 g; 50.6% of the infants were small for the gestational age. The CHD-VLBWs more commonly had bronchopulmonary dysplasia (BPD), and the longer they were exposed to oxygen, the more frequently they developed BPD. Those with cyanotic heart disease developed severe BPD more frequently. Necrotizing enterocolitis (NEC) occurred frequently in the CHD-VLBWs and was not associated with their feeding patterns. CHD-VLBWs had a higher mortality rate; prematurity-related diseases were the leading cause of death before surgery, while heart-related problems were the leading cause of death after surgery. We found no significant difference in mortality from prematurity-related disease between the CHD-VLBWs and controls. In the subgroup analysis of CHD, the cyanotic CHD group had a higher incidence of BPD and higher mortality rate than the acyanotic CHD group. Conclusions: CHD-VLBWs showed higher BPD, NEC, and mortality rates than those without CHD. There was also a higher incidence of BPD and mortality in VLBW infants with cyanotic CHD than in those with acyanotic CHD. AD - E. Jung, Division of Neonatology, Department of Pediatrics, Asan Medical Center Children's Hospital, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, South Korea AU - Song, W. S. AU - Kim, C. Y. AU - Lee, B. S. AU - Kim, E. A. R. AU - Kim, K. S. AU - Jung, E. DB - UEmbase DO - 10.4070/KCJ.2020.0135 KW - information processing device mechanical ventilator SPSS 22.0 prostaglandin E1 steroid surfactant adult aortic coarctation aortopulmonary shunt article artificial ventilation atrioventricular septal defect birth weight cause of death clinical study cohort analysis congenital heart disease cyanotic heart disease data analysis software Ebstein anomaly Fallot tetralogy feeding behavior female gestational age great vessels transposition heart right ventricle double outlet heart single ventricle heart ventricle septum defect human hypoplastic left heart syndrome ICD-9-CM low level laser therapy lung artery banding lung dysplasia major clinical study male mortality rate necrotizing enterocolitis newborn newborn morbidity newborn mortality oxygen therapy patent ductus arteriosus prematurity prevalence pulmonary valve atresia pulmonary valve stenosis pulmonary valvuloplasty retrolental fibroplasia retrospective study risk factor small for date infant surgical mortality very low birth weight LA - English M3 - Article N1 - L2008604940 2020-11-20 2021-03-23 PY - 2020 SN - 1738-5555 1738-5520 ST - Morbidity and mortality of very low birth weight infants with congenital heart disease T2 - Korean Circulation Journal TI - Morbidity and mortality of very low birth weight infants with congenital heart disease UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2008604940&from=export http://dx.doi.org/10.4070/KCJ.2020.0135 VL - 51 ID - 4446 ER - TY - JOUR AB - BACKGROUND: Data on anxiety, depression, and attention-deficit/hyperactivity disorder (ADHD) are lacking for youth with congenital heart disease (CHD), particularly those with simple CHD. This study aims to characterize these disorders in youth with CHD compared to those without CHD. METHODS: A comparative cross-sectional study was conducted by using the electronic medical records of a large tertiary care hospital between 2011 and 2016. Inclusion criteria were youth aged 4 to 17 years with >1 hospitalization or emergency department visits. Exclusion criteria were patients with arrhythmias or treatment with clonidine and/or benzodiazepines. The primary predictor variable was CHD type: simple, complex nonsingle ventricle, and complex single ventricle. The primary outcome variable was a diagnosis and/or medication for anxiety and/or depression or ADHD. Data were analyzed by using logistic regression (Stata v15; Stata Corp, College Station, TX). RESULTS: We identified 118 785 patients, 1164 with CHD. Overall, 18.2% (n = 212) of patients with CHD had a diagnosis or medication for anxiety or depression, compared with 5.2% (n = 6088) of those without CHD. All youth with CHD had significantly higher odds of anxiety and/or depression or ADHD. Children aged 4 to 9 years with simple CHD had ∼5 times higher odds (odds ratio: 5.23; 95% confidence interval: 3.87-7.07) and those with complex single ventricle CHD had ∼7 times higher odds (odds ratio: 7.46; 95% confidence interval: 3.70-15.07) of diagnosis or treatment for anxiety and/or depression. Minority and uninsured youth were significantly less likely to be diagnosed or treated for anxiety and/or depression or ADHD, regardless of disease severity. CONCLUSIONS: Youth with CHD of all severities have significantly higher odds of anxiety and/or depression and ADHD compared to those without CHD. Screening for these conditions should be considered in all patients with CHD. AD - Department of Pediatrics, Section of Pediatric Cardiology, Texas Children's Hospital and Baylor College of Medicine, Houston, Texas; and vincent.gonzalez@bcm.edu. Department of Sociology, Rice University, Houston, Texas. Department of Pediatrics, Section of Pediatric Cardiology, Texas Children's Hospital and Baylor College of Medicine, Houston, Texas; and. AN - 33397689 AU - Gonzalez, V. J. AU - Kimbro, R. T. AU - Cutitta, K. E. AU - Shabosky, J. C. AU - Bilal, M. F. AU - Penny, D. J. AU - Lopez, K. N. C2 - PMC7849200 conflicts of interest to disclose. DA - Feb DB - UPubmed DO - 10.1542/peds.2020-1693 DP - NLM ET - 2021/01/06 IS - 2 LA - eng N1 - 1098-4275 Gonzalez, Vincent J Kimbro, Rachel T Cutitta, Katherine E Shabosky, John C Bilal, Mohammad F Penny, Daniel J Lopez, Keila N K23 HL127164/HL/NHLBI NIH HHS/United States Journal Article Pediatrics. 2021 Feb;147(2):e20201693. doi: 10.1542/peds.2020-1693. Epub 2021 Jan 4. PY - 2021 SN - 0031-4005 (Print) 0031-4005 ST - Mental Health Disorders in Children With Congenital Heart Disease T2 - Pediatrics TI - Mental Health Disorders in Children With Congenital Heart Disease VL - 147 ID - 2349 ER - TY - JOUR AB - Objectives Congenital heart defects (CHD) are the most prevalent congenital anomalies. This study aims to examine the association between maternal occupational exposures to organic and mineral dust, solvents, pesti-cides, and metal dust and fumes and CHD in the offspring, assessing several subgroups of CHD. Methods For this case–control study, we examined 1174 cases with CHD from EUROCAT Northern Netherlands and 5602 controls without congenital anomalies from the Lifelines cohort study. Information on maternal jobs held early in pregnancy was collected via self-administered questionnaires, and job titles were linked to occupational exposures using a job exposure matrix. Results An association was found between organic dust exposure and coarctation of aorta [adjusted odds ratio (ORadj) 1.90, 95% confidence interval (CI) 1.01–3.59] and pulmonary (valve) stenosis in combination with ventricular septal defect (ORadj 2.68, 95% CI 1.07–6.73). Mineral dust exposure was associated with increased risk of coarctation of aorta (ORadj 2.94, 95% CI 1.21–7.13) and pulmonary valve stenosis (ORadj 1.99, 95% CI 1.10–3.62). Exposure to metal dust and fumes was infrequent but was associated with CHD in general (ORadj 2.40, 95% CI 1.09–5.30). Exposure to both mineral dust and metal dust and fumes was associated with septal defects (ORadj 3.23, 95% CI 1.14–9.11). Any maternal occupational exposure was associated with a lower risk of aortic stenosis (ORadj 0.32, 95% CI 0.11–0.94). Conclusions Women should take preventive measures or avoid exposure to mineral and organic dust as well as metal dust and fumes early in pregnancy as this could possibly affect foetal heart development. AD - N. Spinder, University of Groningen, University Medical Center Groningen, Department of Epidemiology, Groningen, Netherlands N. Spinder, University of Groningen, University Medical Center Groningen, Department of Genetics, Groningen, Netherlands AU - Spinder, N. AU - Bergman, J. E. H. AU - Kromhout, H. AU - Vermeulen, R. AU - Corsten-Janssen, N. AU - Boezen, H. M. AU - du Marchie Sarvaas, G. J. AU - de Walle, H. E. K. DB - UEmbase DO - 10.5271/sjweh.3912 IS - 6 KW - folic acid pesticide solvent adolescent adult alcohol consumption aortic coarctation aortic stenosis article atrioventricular septal defect bicuspid aortic valve case control study cohort analysis congenital heart malformation controlled study dust Fallot tetralogy female fume heart atrium septum defect heart left ventricle outflow tract obstruction heart right ventricle outflow tract obstruction heart septum defect heart ventricle septum defect human hypoplastic left heart syndrome infant major clinical study male maternal exposure maternal smoking mineral dust Netherlands obesity occupational exposure organic dust pregnancy priority journal progeny pulmonary valve atresia pulmonary valve stenosis questionnaire underweight young adult LA - English M3 - Article N1 - L2005379869 2020-11-05 2020-12-10 PY - 2020 SN - 1795-990X 0355-3140 SP - 599-608 ST - Maternal occupational exposure and congenital heart defects in offspring T2 - Scandinavian Journal of Work, Environment and Health TI - Maternal occupational exposure and congenital heart defects in offspring UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2005379869&from=export http://dx.doi.org/10.5271/sjweh.3912 VL - 46 ID - 4441 ER - TY - JOUR AB - Adults with congenital heart disease who underwent the Fontan operation to palliate single ventricle heart defects-by direct connection of caval venous return to the pulmonary circulation-have improved survival due to advancements in surgical and interventional techniques. However, cardiac and non-cardiac comorbidities often coexist, complicating management, and contributing to premature morbidity and mortality. Cardiac issues include heart failure symptoms related to systolic and diastolic dysfunction, atrial and ventricular arrhythmias and systemic atrioventricular valve regurgitation. Structural issues may be related to obstruction of the Fontan pathway, or to branch pulmonary artery stenosis, both of which exacerbate symptoms. Non-cardiac complications in adults involve hepatic congestion, fibrosis and cirrhosis, hepatocellular carcinoma, chronic kidney disease, stroke, venous stasis, lymphatic issues and involvement of other organ systems. 'Fontan failure' refers to circulatory dysfunction, either cardiac, non-cardiac, or both, diagnosed after exclusion or treatment of reversible contributors such as structural issues and arrhythmias. Counselling about reproductive health, mental health, perioperative management and overall wellness are paramount for patients' well-being. Fontan patients are typically managed in highly specialised adult congenital heart centres, but may present to cardiologists or other practitioners with cardiac and non-cardiac emergencies or urgencies, sometimes after being out of care. In this review, we discuss the management of the adult Fontan patient, including surveillance, cardiac and non-cardiac complications, reproductive health, and advanced therapies. AD - Internal Medicine and Pediatrics, Emory University, Atlanta, Georgia, USA. Pediatric Cardiology, Children's Healthcare of Atlanta Inc, Atlanta, Georgia, USA. Internal Medicine, Division of Cardiology, Emory University, Atlanta, Georgia, USA wbook@emory.edu. AN - 31662332 AU - Rodriguez, F. H. AU - Book, W. M. DA - Jan DB - UPubmed DO - 10.1136/heartjnl-2019-314937 DP - NLM ET - 2019/10/31 IS - 2 KW - Adult Age Factors *Delivery of Health Care, Integrated Health Status Heart Defects, Congenital/mortality/physiopathology/psychology/*therapy Humans Patient Care Team *Patient Transfer Prognosis Quality of Life Risk Factors *Survivors/psychology Time Factors *Transition to Adult Care Young Adult *Fontan physiology *complex congenital heart disease *congenital heart disease *heart failure with preserved ejection fraction *heart failure with reduced ejection fraction LA - eng N1 - 1468-201x Rodriguez, Fred H Book, Wendy M Orcid: 0000-0002-3599-0329 Journal Article Research Support, U.S. Gov't, P.H.S. Review England Heart. 2020 Jan;106(2):105-110. doi: 10.1136/heartjnl-2019-314937. Epub 2019 Oct 29. PY - 2020 SN - 1355-6037 SP - 105-110 ST - Management of the adult Fontan patient T2 - Heart TI - Management of the adult Fontan patient VL - 106 ID - 2393 ER - TY - JOUR AB - The Fontan circulation describes the circulatory state resulting from an operation in congenital heart disease where systemic venous return is directed to the lungs without an intervening active pumping chamber. As survival increases, so too does recognition of the potential health challenges. This document aims to allow clinicians, people with a Fontan circulation, and their families to benefit from consensus agreement about management of the person with a Fontan circulation. The document was crafted with input from a multidisciplinary group of health care providers as well as individuals with a Fontan circulation and families. It is hoped that the shared common vision of long-term wellbeing will continue to drive improvements in care and quality of life in this patient population and eventually translate into improved survival. Keypoints: • Lifelong quality medical care with access to multidisciplinary services, is of prime importance. Care includes regular tests for surveillance of health status. • Transition from paediatric to adult care is an active process that should commence during early adolescence and continue until successful engagement with adult congenital cardiology care. • Children and adults with a Fontan circulation often have reduced peak exercise capacity (on average, 60–65% of predicted values). Increasingly, evidence suggests exercise training may improve exercise capacity and cardiovascular function. • People with a Fontan circulation have higher rates of anxiety and behavioural disorders, and there needs to be a low threshold for the provision of mental health care. • Pregnancy has increased maternal and fetal risks, and pre-conception multidisciplinary assessment and counselling is essential. • Atrial arrhythmias are common, often late after Fontan surgical repair and due to intra-atrial re-entry or “flutter” mediated by atrial stretch and scarring. Some anti-arrhythmic agents, most classically the type IC drugs, may allow haemodynamically unstable, life-threatening 1:1 AV conduction. • Anticoagulation with warfarin is routine care in patients with atrial arrhythmias. • In patients with recurrent atrial arrhythmias, catheter ablation or surgical conversion may be considered. • The Fontan circulation is an ideal substrate for thrombus formation and may result in intracardiac or intravascular thrombosis, ischaemic stroke, or other embolic phenomena. Antiplatelet and anticoagulant agents are commonly prescribed for thromboprophylaxis in patients with a Fontan circulation. Evidence suggests that treatment with one of these agents is advantageous, but there is no consensus on which is optimal. Despite treatment, symptomatic thromboembolic events are associated with significant mortality. • Heart failure is the leading cause of morbidity and mortality. Diuretics provide symptomatic relief, however standard heart failure medical therapy is not of proven benefit. • Though not well understood, there is increasing concern regarding progressive liver disease with a long-term risk of hepatocellular carcinoma. • Despite early higher mortality post heart transplant, these individuals have better long-term survival outcomes compared with many other heart transplant recipients. AD - D. Zentner, Department of Cardiology, Royal Melbourne Hospital, Melbourne, Vic, Australia AU - Zentner, D. AU - Celermajer, D. S. AU - Gentles, T. AU - d'Udekem, Y. AU - Ayer, J. AU - Blue, G. M. AU - Bridgman, C. AU - Burchill, L. AU - Cheung, M. AU - Cordina, R. AU - Culnane, E. AU - Davis, A. AU - du Plessis, K. AU - Eagleson, K. AU - Finucane, K. AU - Frank, B. AU - Greenway, S. AU - Grigg, L. AU - Hardikar, W. AU - Hornung, T. AU - Hynson, J. AU - Iyengar, A. J. AU - James, P. AU - Justo, R. AU - Kalman, J. AU - Kasparian, N. AU - Le, B. AU - Marshall, K. AU - Mathew, J. AU - McGiffin, D. AU - McGuire, M. AU - Monagle, P. AU - Moore, B. AU - Neilsen, J. AU - O'Connor, B. AU - O'Donnell, C. AU - Pflaumer, A. AU - Rice, K. AU - Sholler, G. AU - Skinner, J. R. AU - Sood, S. AU - Ward, J. AU - Weintraub, R. AU - Wilson, T. AU - Wilson, W. AU - Winlaw, D. AU - Wood, A. DB - UEmbase DO - 10.1016/j.hlc.2019.09.010 IS - 1 KW - arterial stent artificial heart biventricular assist device implantable cardioverter defibrillator right ventricular assist device ventricular assist device angiotensin receptor antagonist beta adrenergic receptor blocking agent dipeptidyl carboxypeptidase inhibitor diuretic agent endothelin receptor antagonist phosphodiesterase V inhibitor spironolactone vasodilator agent warfarin adolescence antibiotic prophylaxis anticoagulation Australia blood clotting bronchitis cardiovascular function cardiovascular magnetic resonance cardiovascular mortality catheter ablation clinical practice congenital heart disease contraception echocardiography electrocardiography exercise exercise test fertility Fontan procedure gastroscopy genetic disorder genetic screening health care access health status heart atrium arrhythmia heart catheterization heart failure heart pacing heart single ventricle heart transplantation heart valve regurgitation heart ventricle arrhythmia human incidence intracardiac thrombosis kidney dysfunction leisure letter liver cell carcinoma liver disease lung vascular resistance medical society mental health care morbidity New Zealand palliative therapy patient care patient counseling patient monitoring patient referral patient safety plastic bronchitis pregnancy prescription priority journal protein losing gastroenteropathy pulmonary hypertension quality of life sudden cardiac death surgical technique survival rate systemic vascular resistance thromboembolism time to treatment transitional care treatment contraindication treatment indication wellbeing LA - English M3 - Article N1 - L2003779409 2019-12-20 2020-07-07 PY - 2020 SN - 1444-2892 1443-9506 SP - 5-39 ST - Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement T2 - Heart Lung and Circulation TI - Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2003779409&from=export http://dx.doi.org/10.1016/j.hlc.2019.09.010 VL - 29 ID - 4437 ER - TY - JOUR AB - Children in the intensive care unit (ICU) are at high risk of developing delirium, given their underlying disease processes, the adverse effects of treatments and medications, and the stressful, abnormal environment. If prevention and nonpharmacologic measures to treat delirium are unsuccessful, atypical antipsychotics are considered, although they are not approved by Food and Drug Administration for the treatment of pediatric delirium and could have significant adverse side effects. This case report presents three pediatric patients with hyperactive ICU delirium that risked life-Threating complications who were successfully treated with short courses of atypical antipsychotic medications. AD - A.O. Jesus, Division of Neurodevelopmental and Behavioral Pediatrics, University of Virginia, Stacey Hall, PO Box 800828, Charlottesville, VA, United States AU - Jesus, A. O. AU - Jones, L. AU - Linares, R. AU - Buck, M. L. AU - Frank, D. U. DB - UEmbase DO - 10.1055/s-0039-3400458 IS - 2 KW - wrist restraint atypical antipsychotic agent cisatracurium clonidine dexmedetomidine fentanyl gabapentin haloperidol hydromorphone ketamine lorazepam melatonin methadone midazolam morphine neuromuscular blocking agent pentobarbital risperidone steroid apathy article case report child clinical article continuous infusion delirium drug substitution drug withdrawal evening dosage female heart catheterization human hypoplastic left heart syndrome infant low drug dose male pediatric intensive care unit priority journal QT prolongation somnolence syndrome CHARGE tachyphylaxis LA - English M3 - Article N1 - L631671963 2020-05-08 2020-06-30 PY - 2020 SN - 2146-4626 2146-4618 SP - 119-123 ST - Management of Hyperactive Delirium in the Pediatric Intensive Care Unit: Case Series of Three Young Children T2 - Journal of Pediatric Intensive Care TI - Management of Hyperactive Delirium in the Pediatric Intensive Care Unit: Case Series of Three Young Children UR - https://www.embase.com/search/results?subaction=viewrecord&id=L631671963&from=export http://dx.doi.org/10.1055/s-0039-3400458 VL - 9 ID - 4415 ER - TY - JOUR AB - Background and aim: To investigate whether neonates with prenatally detected congenital heart defects (CHD) demonstrate cerebral abnormalities on early preoperative cranial ultrasound (CUS), compared to healthy neonates, and to measure brain structures to assess brain growth and development in both groups. Study design, subjects and outcome measures: Prospective cohort study with controls. Between September 2013 and May 2016 consecutive cases of prenatally detected severe isolated CHD were included. Neonatal CUS was performed shortly after birth, before surgery and in a healthy control group. Blinded images were reviewed for brain abnormalities and various measurements of intracranial structures were compared. Results: CUS was performed in 59 healthy controls and 50 CHD cases. Physiological CUS variants were present in 54% of controls and in 52% of CHD cases. Abnormalities requiring additional monitoring (both significant and minor) were identified in four controls (7%) and five CHD neonates (10%). Significant abnormalities were only identified in four CHD neonates (8%) and never in controls. A separate analysis of an additional 8 CHD neonates after endovascular intervention demonstrated arterial stroke in two cases that underwent balloon atrioseptostomy (BAS). Cerebral measurements were smaller in CHD neonates, except for the cerebrospinal fluid measurements, which were similar to the controls. Conclusions: The prevalence of significant preoperative CUS abnormalities in CHD cases was lower than previously reported, which may be partially caused by a guarding effect of a prenatal diagnosis. Arterial stroke occurred only in cases after BAS. As expected, neonates with CHD display slightly smaller head size and cerebral growth. AD - F.A.R. Jansen, Leiden University Medical Centre, K6-P, Postbus 9600, Leiden, Netherlands AU - Jansen, F. A. R. AU - Haak, M. C. AU - van Wesemael, M. S. AU - ten Harkel, A. D. J. D. J. AU - van Lith, J. M. M. AU - Blom, N. A. AU - Steggerda, S. J. DB - UEmbase DO - 10.1016/j.earlhumdev.2020.105097 KW - article atrioventricular septal defect balloon atrioseptostomy brain damage brain development brain growth brain hemorrhage cerebrovascular accident clinical article cohort analysis congenital heart malformation controlled study Ebstein anomaly echography Fallot tetralogy female heart right ventricle double outlet heart surgery human hypoplastic left heart syndrome leukomalacia male nerve cell differentiation newborn preoperative evaluation prospective study pulmonary valve atresia pulmonary valve stenosis smoking LA - English M3 - Article N1 - L2006163351 2020-07-03 2020-07-07 PY - 2020 SN - 1872-6232 0378-3782 ST - A low incidence of preoperative neurosonographic abnormalities in neonates with heart defects T2 - Early Human Development TI - A low incidence of preoperative neurosonographic abnormalities in neonates with heart defects UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2006163351&from=export http://dx.doi.org/10.1016/j.earlhumdev.2020.105097 VL - 148 ID - 4395 ER - TY - JOUR AB - BACKGROUND: Growth abnormalities in single-ventricle survivors may reduce quality of life (QoL) and exercise capacity. METHODS: This multicenter, longitudinal analysis evaluated changes in height and body mass index (BMI) compared to population norms and their relationship to mortality, ventricular morphology, QoL, and exercise capacity in the Pediatric Heart Network Fontan studies. RESULTS: Fontan 1 (F1) included 546 participants (12 ± 3.4 years); Fontan 2 (F2), 427 (19 ± 3.4 years); and Fontan 3 (F3), 362 (21 ± 3.5 years), with ~60% male at each time point. Height z-score was -0.67 ± -1.27, -0.60 ± 1.34, and- 0.43 ± 1.14 at F1-F3, lower compared to norms at all time points (P ≤ .001). BMI z-score was similar to population norms. Compared to survivors, participants who died had lower height z-score (P ≤ .001). Participants with dominant right ventricle (n = 112) had lower height z-score (P ≤ .004) compared to dominant left (n = 186) or mixed (n = 64) ventricular morphologies. Higher height z-score was associated with higher Pediatric Quality of Life Inventory for the total score (slope = 2.82 ± 0.52; P ≤ .001). Increase in height z-score (F1 to F3) was associated with increased oxygen consumption (slope = 2.61 ± 1.08; P = .02), whereas, for participants >20 years old, an increase in BMI (F1 to F3) was associated with a decrease in oxygen consumption (slope = -1.25 ± 0.33; P ≤ .001). CONCLUSIONS: Fontan survivors, especially those with right ventricular morphology, are shorter when compared to the normal population but have similar BMI. Shorter stature was associated with worse survival. An increase in height z-score over the course of the study was associated with better QoL and exercise capacity; an increase in BMI was associated with worse exercise capacity. AD - University of Utah/Primary Children's Hospital, Salt Lake City, UT. Electronic address: linda.lambert@hsc.utah.edu. The Hospital for Sick Children, Toronto, Ontario, Canada. National Heart, Lung, and Blood Institute, NIH, Bethesda, MD. New England Research Institutes, Watertown, MA. Medical University of South Carolina, Charleston, SC. Children's Hospital of Philadelphia, Philadelphia, PA. Duke University Hospital, Durham, NC. Boston Children's Hospital, Boston, MA. University of Michigan/CS Mott Children's Hospital, Ann Arbor, MI. University of Utah/Primary Children's Hospital, Salt Lake City, UT. AN - 32428726 AU - Lambert, L. M. AU - McCrindle, B. W. AU - Pemberton, V. L. AU - Hollenbeck-Pringle, D. AU - Atz, A. M. AU - Ravishankar, C. AU - Campbell, M. J. AU - Dunbar-Masterson, C. AU - Uzark, K. AU - Rolland, M. AU - Trachtenberg, F. L. AU - Menon, S. C. C2 - PMC7293556 C6 - NIHMS1581927 DA - Jun DB - UPubmed DO - 10.1016/j.ahj.2020.03.022 DP - NLM ET - 2020/05/20 KW - Adolescent Anthropometry/*methods Body Mass Index Canada/epidemiology Child Exercise Exercise Test Female Follow-Up Studies Fontan Procedure/*methods Heart Defects, Congenital/mortality/physiopathology/*surgery Heart Ventricles/diagnostic imaging/*physiopathology Humans Male Postoperative Period Prognosis *Quality of Life Retrospective Studies Survival Rate/trends United States/epidemiology Young Adult LA - eng N1 - 1097-6744 Lambert, Linda M McCrindle, Brian W Pemberton, Victoria L Hollenbeck-Pringle, Danielle Atz, Andrew M Ravishankar, Chitra Campbell, M Jay Dunbar-Masterson, Carolyn Uzark, Karen Rolland, Martha Trachtenberg, Felicia L Menon, Shaji C Pediatric Heart Network Investigators UG1 HL135666/HL/NHLBI NIH HHS/United States U10 HL109818/HL/NHLBI NIH HHS/United States U10 HL109741/HL/NHLBI NIH HHS/United States UG1 HL135680/HL/NHLBI NIH HHS/United States UG1 HL135646/HL/NHLBI NIH HHS/United States U10 HL109781/HL/NHLBI NIH HHS/United States UG1 HL135685/HL/NHLBI NIH HHS/United States U10 HL109743/HL/NHLBI NIH HHS/United States U01 HL068270/HL/NHLBI NIH HHS/United States U10 HL109737/HL/NHLBI NIH HHS/United States U10 HL109777/HL/NHLBI NIH HHS/United States U10 HL109778/HL/NHLBI NIH HHS/United States U10 HL109673/HL/NHLBI NIH HHS/United States U01 HL068290/HL/NHLBI NIH HHS/United States UG1 HL135682/HL/NHLBI NIH HHS/United States Journal Article Multicenter Study Am Heart J. 2020 Jun;224:192-200. doi: 10.1016/j.ahj.2020.03.022. Epub 2020 Apr 4. PY - 2020 SN - 0002-8703 (Print) 0002-8703 SP - 192-200 ST - Longitudinal study of anthropometry in Fontan survivors: Pediatric Heart Network Fontan study T2 - Am Heart J TI - Longitudinal study of anthropometry in Fontan survivors: Pediatric Heart Network Fontan study VL - 224 ID - 2370 ER - TY - JOUR AB - OBJECTIVES: Because of the nature of the Fontan physiology, patients are at an increased risk of thromboembolic complications. As such, warfarin or aspirin is generally prescribed lifelong for thromboprophylaxis. This study aimed to compare long-term rates of cerebrovascular injury, thrombosis, bleeding, bone mineral density, and quality of life in people living with Fontan circulation receiving warfarin compared with aspirin. METHODS: This was a multicenter study of a selected cohort from the Australia and New Zealand Fontan population. Participants underwent cerebral magnetic resonance imaging to detect the presence of cerebrovascular injury (n = 84) and dual-energy X-ray absorptiometry to assess bone mineral density (n = 120). Bleeding (n = 100) and quality of life (n = 90) were assessed using validated questionnaires: Warfarin and Aspirin Bleeding assessment tool and Pediatric Quality of Life Inventory, respectively. RESULTS: Stroke was detected in 33 participants (39%), with only 7 (6%) being clinically symptomatic. There was no association between stroke and Fontan type or thromboprophylaxis type. Microhemorrhage and white matter injury were detected in most participants (96% and 86%, respectively), regardless of thromboprophylaxis type. Bleeding rates were high in both groups; however, bleeding was more frequent in the warfarin group. Bone mineral density was reduced in our cohort compared with the general population; however, this was further attenuated in the warfarin group. Quality of life was similar between the warfarin and aspirin groups. Home international normalized ratio monitoring was associated with better quality of life scores in the warfarin group. CONCLUSIONS: Cerebrovascular injury is a frequent occurrence in the Australia and New Zealand Fontan population regardless of thromboprophylaxis type. No benefit of long-term warfarin prophylaxis could be demonstrated over aspirin; however, consideration must be given to important clinical features such as cardiac function and lung function. Furthermore, the association of reduced bone health in children receiving warfarin warrants further mechanistic studies. AD - Murdoch Children's Research Institute, Parkville, Australia; The University of Melbourne, Parkville, Australia. Electronic address: Chantal.attard@mcri.edu.au. Murdoch Children's Research Institute, Parkville, Australia; The University of Melbourne, Parkville, Australia; The Royal Children's Hospital, Parkville, Australia. Nuclear Medicine, The Children's Hospital at Westmead, Westmead, Australia; The University of Sydney Children's Hospital, Westmead Clinical School, Westmead, Australia. Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia; Faculty of Medicine and Health Sciences, University of Sydney, Sydney, Australia. Australian Institute for Musculoskeletal Science, The University of Melbourne and Western Health, Melbourne, Australia; Department of Medicine-Western Health, The University of Melbourne, Melbourne, Australia. Murdoch Children's Research Institute, Parkville, Australia; The Royal Children's Hospital, Parkville, Australia; Department of Paediatric Endocrinology and Diabetes, Monash Children's Hospital, Clayton, Australia. Paediatric and Congenital Cardiac Service, Starship Children's Hospital, Auckland, New Zealand. Murdoch Children's Research Institute, Parkville, Australia; The University of Melbourne, Parkville, Australia. AN - 33563422 AU - Attard, C. AU - Monagle, P. T. AU - d'Udekem, Y. AU - Mackay, M. T. AU - Briody, J. AU - Cordina, R. AU - Hassan, E. B. AU - Simm, P. AU - Rice, K. AU - Ignjatovic, V. DA - Jan 5 DB - UPubmed DO - 10.1016/j.jtcvs.2020.12.102 DP - NLM ET - 2021/02/11 KW - Fontan aspirin bone density single ventricle stroke warfarin LA - eng N1 - 1097-685x Attard, Chantal Monagle, Paul T d'Udekem, Yves Mackay, Mark T Briody, Julie Cordina, Rachael Hassan, Ebrahim Bani Simm, Peter Rice, Kathryn Ignjatovic, Vera ANZ Fontan Registry Research group Journal Article United States J Thorac Cardiovasc Surg. 2021 Jan 5:S0022-5223(20)33457-7. doi: 10.1016/j.jtcvs.2020.12.102. PY - 2021 SN - 0022-5223 ST - Long-term outcomes of warfarin versus aspirin after Fontan surgery T2 - J Thorac Cardiovasc Surg TI - Long-term outcomes of warfarin versus aspirin after Fontan surgery ID - 2343 ER - TY - JOUR AB - Objectives: The one-and-a-half ventricle repair (1.5VR) is a surgical alternative to Fontan circulation or high-risk biventricular repair in patients with complex congenital heart disease (CHD) with a hypoplastic right ventricle (RV). We report our 25 years of experience to evaluate whether the degree of anatomical complexity of the CHD can affect long-term outcomes. Methods: This is a retrospective study including patients undergoing 1.5VR between 1994 and 2018. Clinical records and operative reports were reviewed. Follow-up data were collected from all survivors at the last evaluation or by phone contact. Results: Twenty-nine patients underwent 1.5VR [median age: 3.5 years, interquartile range (IQR) 0.8-7.8]. Fifteen patients had 'simple' (i.e. confined to right heart lesions) anatomical characteristics (48.3%); the median tricuspid valve annulus z-score was -3.5 (IQR -6.2 to +3.6). There were no operative deaths. The median hospital stay was 21 days (IQR 10-33), with postoperative complications in 21 patients (75.8%). At a median follow-up of 13.2 years (IQR 3.2-25.6 years; completeness 96.6%), there were 3 late deaths. There was no significant difference in survival and in freedom from adverse events between simple and complex anatomy groups. However, stress test findings showed a tendency towards a higher maximum oxygen uptake in the simple group (P = 0.055). Conclusions: The 1.5VR strategy is an effective low-risk surgical option for patients with CHD with hypoplastic RV. When it is planned from birth, late outcomes can be satisfactory. In our experience, the presence of associated major cardiac anomalies was not associated with either worse early or long-term outcomes and functional status. AD - M.A. Padalino, UOC Cardiochirurgia Pediatrica e Cardiopatie Congenite, Department of Cardio-thoracic-vascular Sciences and Public Health, University of Padova, Via Giustiniani 2, Padova, Italy AU - Cabrelle, G. AU - Castaldi, B. AU - Vedovelli, L. AU - Gregori, D. AU - Vida, V. L. AU - Padalino, M. A. DB - UEmbase DO - 10.1093/ejcts/ezaa289 IS - 1 KW - article child clinical article complication congenital heart malformation controlled study female follow up functional status heart injury heart right ventricle hospitalization human male maximal oxygen uptake physiological stress postoperative complication preschool child retrospective study risk assessment survivor tricuspid valve LA - English M3 - Article N1 - L2010881370 2021-02-09 PY - 2021 SN - 1873-734X 1010-7940 SP - 244-252 ST - Long-term experience with the one-and-a-half ventricle repair for simple and complex congenital heart defects T2 - European Journal of Cardio-thoracic Surgery TI - Long-term experience with the one-and-a-half ventricle repair for simple and complex congenital heart defects UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2010881370&from=export http://dx.doi.org/10.1093/ejcts/ezaa289 VL - 59 ID - 4366 ER - TY - JOUR AB - Background: There is increasing concern that moderate preterm (32–33 weeks’ gestation) and late preterm (34–36 weeks’ gestation) birth may be associated with minor neurodevelopmental problems affecting poor school performance. Purpose: We explored the cognitive function, cognitive visual function, executive function, and behavioral problems at school age in moderate to late preterm infants. Methods: Children aged 7–10 years who were born at 32+0 to 36+6 weeks of gestation and admitted to the neonatal intensive care unit from August 2006 to July 2011 at the National Health Insurance Service Ilsan Hospital were included. We excluded children with severe neurologic impairments, congenital malfor-mations, or chromosomal abnormalities. Neuropsychological assessments consisted of 5 neuropsychological tests and 3 questionnaires. Results: A total of 37 children (mean age, 9.1±1.2 years) participated. The mean gestational age at birth was 34.6±7.5 weeks, while the mean birth weight was 2,229.2±472.8 g. The mean full-scale intelligence quotient was 92.89±11.90; 24.3% scored between 70 and 85 (borderline intelligence functioning). An abnormal score was noted for at least one of the variables on the attention deficit hyperactivity disorder diagnostic system for 65% of the children. Scores below borderline function for executive quotient and memory quotient were 32.4% and 24.3%, respectively. Borderline or clinically relevant internalizing problems were noted in 13.5% on the Child Behavior Check List. There were no significant associations between perinatal factors or socioeconomic status and cognitive, visual perception, executive function, or behavior outcomes. Conclusion: Moderate to late preterm infants are at risk of developing borderline intelligence functioning and attention problems at early school age. Cognitive and executive functions that are important for academic performance must be carefully monitored and continuously followed up in moderate to late preterm infants. AD - H.J. Chung, Department of Pediatrics, National Health Insurance Service Ilsan Hospital, Goyang, South Korea AU - Jin, J. H. AU - Yoon, S. W. AU - Son, J. AU - Kim, S. W. AU - Chung, H. J. DB - UEmbase DO - 10.3345/kjp.2019.00647 IS - 6 KW - academic achievement Apgar score article attention attention deficit disorder behavior birth weight child Child Behavior Checklist clinical article cognition cognitive defect cognitive proficiency index Down syndrome emotionality executive function full scale intelligence quotient gastrointestinal malformation general abilities index gestational age human hypoplastic left heart syndrome hypoxic ischemic encephalopathy intelligence quotient male memory mental disease neonatal intensive care unit neuropsychological test perceptual reasoning index polydactyly prematurity processing speed index questionnaire social status tracheoesophageal fistula verbal comprehension index vision Wechsler intelligence scale for children working memory index LA - English M3 - Article N1 - L2004557456 2020-07-24 2020-09-01 PY - 2020 SN - 2092-7258 1738-1061 SP - 219-225 ST - Long-term cognitive, executive, and behavioral outcomes of moderate and late preterm at school age T2 - Korean Journal of Pediatrics TI - Long-term cognitive, executive, and behavioral outcomes of moderate and late preterm at school age UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2004557456&from=export http://dx.doi.org/10.3345/kjp.2019.00647 VL - 63 ID - 4409 ER - TY - JOUR AB - In this month's patient story, Linda McStay describes her early life as an individual born with a univentricular heart and how it felt to undergo Fontan surgery at 10 years of age. AD - North Lanarkshire, Scotland AN - 148974259. Language: English. Entry Date: 20210305. Revision Date: 20210308. Publication Type: Article AU - McStay, Linda DB - Ucinahl DO - 10.12968/bjca.2021.0003 DP - EBSCOhost IS - 2 KW - Univentricular Heart -- Diagnosis -- In Infancy and Childhood Univentricular Heart -- Surgery -- In Infancy and Childhood Patient Attitudes Attitude to Illness Life Experiences Cardiac Patients -- Psychosocial Factors Office Visits Hematologic Tests Electrocardiography Echocardiography Attitude to Medical Treatment Color Infant, Newborn Infant Child, Preschool Child N1 - anecdote. Journal Subset: Double Blind Peer Reviewed; Editorial Board Reviewed; Europe; Expert Peer Reviewed; Nursing; Peer Reviewed; UK & Ireland. PY - 2021 SN - 1749-6403 SP - 1-2 ST - Life with half a heart: the early years T2 - British Journal of Cardiac Nursing TI - Life with half a heart: the early years UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=148974259&site=ehost-live&scope=site VL - 16 ID - 5341 ER - TY - JOUR AB - Tricuspid valve atresia with severe pulmonary stenosis is one of the common cyanotic diseases in neonate. Child can succumb due to profound cyanosis and arterial hypoxaemia after closure of patent ductus arteriosus. Evolving procedure of right ventricular outflow tract stenting may be considered as a palliative procedure in such vulnerable group, destined for a later definitive management. The right ventricular outflow tract stenting is described essentially for tetralogy of Fallot physiology with a catheter course across tricuspid valve. We describe a case of successful right ventricular outflow tract stenting in a 5-day-old symptomatic neonate. We discuss the possible routes and the tips to facilitate right ventricular outflow tract stenting in such a case. This happens to be the first reported case description with successful stenting of neonate with tricuspid atresia with critical pulmonic stenosis. © The Author(s) 2020. AD - Department of Pediatric Cardiology, Max Superspeciality Hospital, Saket, Delhi, India AU - Awasthy, N. AU - Chimoriya, R. AU - Kumar, G. DB - UScopus DO - 10.1017/S1047951120002620 IS - 10 KW - pulmonic stenosis Right ventricular outflow tract stenting tricuspid atresia M3 - Article N1 - Export Date: 10 May 2021 PY - 2020 SP - 1541-1543 ST - Learning from a case of right ventricular outflow tract stenting in tricuspid atresia with critical pulmonary stenosis T2 - Cardiology in the Young TI - Learning from a case of right ventricular outflow tract stenting in tricuspid atresia with critical pulmonary stenosis UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85090456709&doi=10.1017%2fS1047951120002620&partnerID=40&md5=9d71932c446eabe854a4d41b2f3132db VL - 30 ID - 5886 ER - TY - JOUR AB - In the article, the authors discuss the sufficiency of developmental screening in children with hypoplastic left heart syndrome and associated single right ventricle conditions. Also cited are a study which examined the effectiveness of a diagnostic developmental assessment and screening in predicting developmental functioning outcome in said children. The children's screening is recommended by the American Academy of Pediatrics (AAP). AD - Division of Developmental and Behavioral Pediatrics, University of Cincinnati College of Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio AN - 148485032. Language: English. Entry Date: 20210208. Revision Date: 20210211. Publication Type: Article AU - Anixt, Julia S. AU - Wiley, Susan DB - Ucinahl DO - 10.1542/peds.2020-033043 DP - EBSCOhost IS - 2 KW - Heart Ventricle -- Abnormalities -- In Infancy and Childhood Developmental Disabilities Clinical Assessment Tools -- Utilization Age Factors Child Development Child N1 - commentary. Journal Subset: Biomedical; Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. NLM UID: 0376422. PY - 2021 SN - 0031-4005 SP - 1-2 ST - Is Developmental Screening Enough in High-Risk Populations? T2 - Pediatrics TI - Is Developmental Screening Enough in High-Risk Populations? UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=148485032&site=ehost-live&scope=site VL - 147 ID - 5332 ER - TY - JOUR AB - Donohue syndrome (leprechaunism; OMIM ∗246200) is a rare and often lethal autosomal recessive disease caused by mutations in the INSR gene. We report the case of a 29-year-old pregnant woman, primigravida, who was referred at 33 weeks of gestation for severe intrauterine growth restriction (IUGR). Ultrasound examination found severe IUGR associated with an obstructive hypertrophic cardiomyopathy (HCM), confirmed postnatally. The newborn's blood glucose level fluctuated from fasting hypoglycemia to postprandial hyperglycemia. The infant was found to be homozygous for a novel missense pathogenic variant, c.632C>T (p.T211l), in exon 2 of the INSR gene, predicted to result in an abnormal insulin receptor. To our knowledge, this is the first report of leprechaunism being revealed by IUGR and HCM during the prenatal period. Clinicians should keep in mind that the association of these prenatal signs could indicate leprechaunism and specific early neonatal management could be proposed, in particular with recombinant human insulin-like growth factor-I. AD - K. Perge, Service d'Endocrinologie Pediatrique, Hopital Femme Mere Enfant, Hospices Civils de Lyon 59, boulevard Pinel, Bron cedex, France AU - Perge, K. AU - Massoud, M. AU - Gauthier-Moulinier, H. AU - Lascols, O. AU - Pangaud, N. AU - Villanueva, C. AU - Pons, L. DB - UEmbase DO - 10.1159/000509837 IS - 4 KW - blood clotting factor 7 C peptide chorionic gonadotropin insulin pregnancy associated plasma protein A propranolol recombinant somatomedin C adult anemia aortic occlusion article carbohydrate intake case report clinical article clinical assessment clinical feature continuous infusion crown rump length Doppler flowmetry drug megadose ear malformation echocardiography exophthalmos fasting female fetus echography fetus weight genetic association heart right ventricle outflow tract obstruction human hyperglycemia hypertrichosis hypertrophic cardiomyopathy hypoalbuminemia hypoglycemia hypoplastic left heart syndrome intrauterine growth retardation leprechaunism male missense mutation necrotizing enterocolitis newborn noninvasive ventilation nuchal translucency measurement pediatric patient pericardial effusion pregnancy pregnant woman prenatal diagnosis prenatal period primigravida priority journal prothrombin time respiratory distress Sanger sequencing thrombocytopenia ulcerative colitis LA - English M3 - Article N1 - L632868803 2020-09-22 2021-03-29 PY - 2020 SN - 1661-8777 1661-8769 SP - 223-227 ST - Intrauterine Growth Restriction and Hypertrophic Cardiomyopathy as Prenatal Ultrasound Findings in a Case of Leprechaunism T2 - Molecular Syndromology TI - Intrauterine Growth Restriction and Hypertrophic Cardiomyopathy as Prenatal Ultrasound Findings in a Case of Leprechaunism UR - https://www.embase.com/search/results?subaction=viewrecord&id=L632868803&from=export http://dx.doi.org/10.1159/000509837 VL - 11 ID - 4385 ER - TY - JOUR AB - Individuals with single-ventricle congenital heart disease who are palliated to a Fontan circulation are at risk for heart failure and liver disease, with recurrent ascites being a potentially debilitating cause of late morbidity. Although ascites associated with heart failure or liver failure is usually characterized by a high serum-ascites albumin gradient (SAAG), we have observed multiple instances of ascites in Fontan patients with low SAAG, suggesting an inflammatory process. We present three cases in which recalcitrant ascites severely and adversely impacted the quality of life and describe our initial experience with intraperitoneal corticosteroids in this setting. AD - F.M. Wu, Department of Medicine, Division of Cardiology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, United States F.M. Wu, Department of Cardiology, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States AU - Wu, F. M. AU - Valente, A. M. AU - Nigrovic, P. A. AU - Rutherford, A. E. AU - Singh, M. N. DB - UEmbase DO - 10.1111/jocs.15244 IS - 2 KW - C reactive protein diuretic agent fresh frozen plasma hydrocortisone methotrexate prednisone triamcinolone acetonide triamcinolone hexacetonide adolescent adrenal insufficiency adult article ascites atriopulmonary connection cardiac index case report child clinical article computer assisted tomography corticotropin test drug dose escalation erythrocyte transfusion female Fontan procedure heart arrhythmia heart atrium arrhythmia heart catheterization heart right ventricle double outlet hemoperitoneum human human tissue hyperglycemia liver biopsy liver cirrhosis liver fibrosis low drug dose middle aged mitral valve atresia paracentesis peritoneal biopsy peritonitis plasma transfusion preschool child protein blood level pulmonary valve atresia quality of life radiofrequency ablation recurrent ascites recurrent disease sudden death traffic accident young adult LA - English M3 - Article N1 - L2007577260 2020-12-15 PY - 2021 SN - 1540-8191 0886-0440 SP - 735-738 ST - Intraperitoneal corticosteroids for recurrent ascites in patients with Fontan circulation: Initial clinical experience T2 - Journal of Cardiac Surgery TI - Intraperitoneal corticosteroids for recurrent ascites in patients with Fontan circulation: Initial clinical experience UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2007577260&from=export http://dx.doi.org/10.1111/jocs.15244 VL - 36 ID - 4352 ER - TY - JOUR AB - Objective: To evaluate the current interventions and outcomes in neonates born with hypoplastic left heart syndrome (HLHS) and congenital diaphragmatic hernias (CDHs). Methods: A comprehensive search of the literature using relevant search words was conducted from the date of each database inception to August 2018 to identify interventions and outcomes in patients with CDH and HLHS. Conference papers, review articles, case reports, and animal studies were not eligible for this review. Articles were also excluded if left heart hypoplasia, rather than HLHS, was studied. Neither language nor sample size was used for exclusion. A preliminary screening of article titles was performed to rule out irrelevant articles and article types by a single reviewer. A second screening circle was performed by two independent reviewers to assess remaining abstracts. Data were presented as a systematic review and were descriptively summarized. Results: Of the 162 retrieved articles, five studies were eligible for inclusion. The total number of neonates included in this systematic review was 5063. One hundred and eighteen (2.3%) presented specifically with CDH and HLHS. Overall, a total of 62 of the 118 patients underwent a surgical procedure (51%) with 36 (59%) surviving to discharge. Conclusions: Available data suggest current surgical interventions still result in a high degree of mortality. Age at which surgery occurred, pulmonary function and the use of extra-corporeal membrane oxygenation (ECMO) are potential variables that may determine and improve outcomes in these patients in the future. However, long-term outcomes and analyses of more variables are needed to determine effective treatment for this rare but often fatal comorbid congenital diagnosis. AD - R. Ruano, 200 First Street SW, Rochester, MN, United States AU - Kanade, R. AU - Shazly, S. AU - Ruano, R. DB - UEmbase DO - 10.1080/14767058.2020.1849091 KW - congenital diaphragm hernia extracorporeal oxygenation female fetal therapy fetus fetus echography fetus malformation human hypoplastic left heart syndrome language lung function male mortality newborn review sample size surgical technique systematic review LA - English M3 - Article in Press N1 - L2007327342 2020-11-24 PY - 2020 SN - 1476-4954 1476-7058 ST - Interventions and neonatal outcomes of fetuses with hypoplastic left heart syndrome and congenital diaphragmatic hernia: a systematic review T2 - Journal of Maternal-Fetal and Neonatal Medicine TI - Interventions and neonatal outcomes of fetuses with hypoplastic left heart syndrome and congenital diaphragmatic hernia: a systematic review UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2007327342&from=export http://dx.doi.org/10.1080/14767058.2020.1849091 ID - 4444 ER - TY - JOUR AB - BACKGROUND: The desirable distance, defined as offset, between the central line of the superior vena cava (SVC) and the intra-atrial conduit after an intra-atrial conduit (IAC) Fontan's procedure remained unclear. We compared the hemodynamic features using virtual surgery with different offset designs in our study. METHODS: Three-dimensional models of IAC Fontan's procedure were reconstructed according to the magnetic resonance imagings (MRIs) of three patients, then four models for each patient with different offsets equaling 100, 67, 33, and 0% of the diameter of the IVC were reconstructed. Computational fluid dynamics (CFD) were performed in each model to predict the best hemodynamic features, including streamlines of blood flow, wall shear stress (WSS), energy loss (EL), and the hepatic flow distribution (HFD) ratio. RESULTS: Comprehensive evaluation of WSS, EL, and HFD revealed than an offset of 33% presents the best hemodynamic performance among the three patients modeled. In patient A, an offset of 33% resulted in the best HFD (left pulmonary artery/right pulmonary artery [LPA/RPA] = 35/65%). In patient B, the best trade-off between HFD (35/65%), and WSS was achieved with an offset of 33%. In patient C, EL peaked at an offset of 0% and significantly dropped at an offset of 33% with a desirable HFD (60/40%). CONCLUSIONS: We verified that the offset distance influences hemodynamic performance in IAC Fontan's procedure. Considering several hemodynamic parameters, the best trade-offs between hemorheology, pulmonary perfusion, and energy efficiency were achieved at an offset of 33%. This distance should be taken into consideration and optimized during the surgical planning for the IAC Fontan's procedure. AD - Department of Pediatric Cardio-Thoracic Surgery, Shanghai Children's Medical Center, Shanghai, China. Department of Radiology, Shanghai Children's Medical Center, Shanghai, China. Institute of Pediatric Translational Medicine, Shanghai Children's Medical Center, Shanghai, China. AN - 30669173 AU - Hu, J. AU - Wang, Q. AU - Tong, Z. AU - Shen, J. AU - Liu, J. AU - Sun, Y. AU - Zhang, H. DA - Jan DB - UPubmed DO - 10.1055/s-0038-1677490 DP - NLM ET - 2019/01/23 IS - 1 KW - Adolescent Blood Flow Velocity Female *Fontan Procedure/adverse effects Heart Defects, Congenital/diagnostic imaging/physiopathology/*surgery *Hemodynamics Humans Magnetic Resonance Imaging Male Models, Cardiovascular Patient-Specific Modeling Pulmonary Artery/abnormalities/diagnostic imaging/physiopathology/*surgery *Pulmonary Circulation Treatment Outcome Vena Cava, Inferior/abnormalities/diagnostic imaging/physiopathology/*surgery Vena Cava, Superior/abnormalities/diagnostic imaging/physiopathology/*surgery LA - eng N1 - 1439-1902 Hu, Jie Wang, Qian Tong, Zhirong Shen, Juanya Liu, Jinlong Sun, Yanjun Zhang, Haibo Case Reports Comparative Study Journal Article Research Support, Non-U.S. Gov't Germany Thorac Cardiovasc Surg. 2020 Jan;68(1):38-44. doi: 10.1055/s-0038-1677490. Epub 2019 Jan 22. PY - 2020 SN - 0171-6425 SP - 38-44 ST - Influence of Offset on Hemodynamics of Intra-atrial Conduit Fontan's Procedure and Its Clinical Implications T2 - Thorac Cardiovasc Surg TI - Influence of Offset on Hemodynamics of Intra-atrial Conduit Fontan's Procedure and Its Clinical Implications VL - 68 ID - 2429 ER - TY - JOUR AB - Previous reports have identified risk factors associated with development of post-Fontan protein-losing enteropathy. Less is known about the economic impact and resource utilisation required for post-Fontan protein-losing enteropathy in the current era. We conducted a single-centre retrospective study to assess the impact of post-Fontan protein-losing enteropathy on transplant-free survival. We also described resource utilisation and treatment variations among post-Fontan protein-losing enteropathy patients. Children who received care at our centre between 2009 and 2017 after the Fontan surgery were eligible. Initial admissions for the Fontan operative procedure were excluded. Demographics, hospital admissions, resource utilisation, medications and charges were reviewed. Patients were divided into two groups based on the presence of post-Fontan protein-losing enteropathy. Of the 343 patients screened, 147 met the eligibility criteria. Of these, 28 (19%) developed protein-losing enteropathy. After adjusting for follow-up duration, the protein-losing enteropathy group had higher number of encounters (2.15 ± 2.16 versus 1.47 ± 2.56, p 0.002), hospital length of stay (days) (25 ± 51.3 versus 11.4 ± 41.7, p < 0.0001) and total charges (2018US$) (388,489 ± 759,859 versus 202,725 ± 1,076,625, p < 0.0001). Encounters for patients with protein-losing enteropathy utilised more therapies. Among those with protein-losing enteropathy, use of digoxin was associated with slightly decreased odds for mortality and/or transplant (0.95, confidence interval 0.90-0.99, p 0.021). The 10-year transplant-free survival for patients with/without protein-losing enteropathy was 65.7/97.3% (p 0.002), respectively. Post-Fontan protein-losing enteropathy is associated with reduced 10-year transplant-free survival, higher resource utilisation, charges and medication use compared with the non-protein-losing enteropathy group. Practice variation among post-Fontan protein-losing-enteropathy patients is common. Further larger studies are needed to assess the impact of standardisation on the well-being of children with post-Fontan protein-losing enteropathy. AD - Department of Pediatrics, George Washington University School of Medicine, Washington, DC, USA. Division of Biostatistics and Study Methodology, Children's National Hospital, Washington, DC, USA. Division of Cardiology, Children's National Hospital, Washington, DC, USA. AN - 32684182 AU - Alkofair, B. AU - Alruwaili, A. AU - Gai, J. AU - Harahsheh, A. S. DA - Sep DB - UPubmed DO - 10.1017/s1047951120001985 DP - NLM ET - 2020/07/21 IS - 9 KW - Post-Fontan protein-losing enteropathy congenital heart surgery functionally single ventricle anatomy health economics resource utilisation LA - eng N1 - 1467-1107 Alkofair, Baneen Alruwaili, Abdulmajeed Gai, Jiaxiang Harahsheh, Ashraf S Orcid: 0000-0002-2622-573x Journal Article England Cardiol Young. 2020 Sep;30(9):1273-1280. doi: 10.1017/S1047951120001985. Epub 2020 Jul 20. PY - 2020 SN - 1047-9511 SP - 1273-1280 ST - Impact of protein-losing enteropathy in children who underwent the Fontan operation T2 - Cardiol Young TI - Impact of protein-losing enteropathy in children who underwent the Fontan operation VL - 30 ID - 2361 ER - TY - JOUR AB - OBJECTIVE: To explore the role of antenatal counselling in how parents make treatment decisions following an antenatal diagnosis of Hypoplastic Left Heart Syndrome (HLHS). BACKGROUND: Antenatal counselling is a critical part of patient management following a diagnosis of fetal congenital heart disease; however, there is a very limited evidence base examining how parents actually experience antenatal counselling and make decisions in this context. METHODS: Semi-structured interviews were conducted with women who had received an antenatal diagnosis of HLHS. Interviews were digitally recorded, anonymised and transcribed verbatim. A thematic content analysis was performed using a constant comparative approach. RESULTS: Eight mothers of surviving children with HLHS were interviewed. Eight key themes emerged including new perspectives on how women receive antenatal counselling and how it affects their decision making. Three themes in particular are new to the literature: (a) Mothers of children with HLHS reported feelings of intense guilt that arose in the antenatal period around potentially causing the condition in their child. (b) For this group of women, recollections of perceived pessimism during antenatal counselling had a lasting impact. (c) Despite support from partners or extended family, women nevertheless experienced a strong sense that antenatal decision making was largely a 'maternal' responsibility. CONCLUSIONS: When recounting their experiences of antenatal counselling, mothers of surviving children with HLHS offer new perspectives that can guide fetal cardiologists in how best to support their individual patients. Further research is needed to comprehensively understand the experience of prospective parents counselled for severe forms of fetal congenital heart disease. AU - Bertaud, S. AU - Lloyd, D. F. A. AU - Sharland, G. AU - Razavi, R. AU - Bluebond-Langner, M. DB - UEmbase DO - 10.1111/hex.13103 IS - 5 KW - article cardiologist child clinical article content analysis controlled study counseling decision making extended family female fetus genetic transcription guilt human hypoplastic left heart syndrome interview mother pessimism prenatal diagnosis prenatal period responsibility LA - English M3 - Article N1 - L632369714 2020-07-24 PY - 2020 SN - 1369-7625 SP - 1224-1230 ST - The impact of prenatal counselling on mothers of surviving children with hypoplastic left heart syndrome: A qualitative interview study T2 - Health expectations : an international journal of public participation in health care and health policy TI - The impact of prenatal counselling on mothers of surviving children with hypoplastic left heart syndrome: A qualitative interview study UR - https://www.embase.com/search/results?subaction=viewrecord&id=L632369714&from=export http://dx.doi.org/10.1111/hex.13103 VL - 23 ID - 4391 ER - TY - JOUR AB - Objective: This study aimed to provide an insight into the impact of the early outbreak of the novel Coronavirus Disease 2019 on the care management for patients with congenital heart disease. Methods: This study respectively enrolled a cohort of surgical patients who underwent surgery in 2018 (group I), 2019 (group II), and 2020 (group III) and a cohort of follow-up patients who had follow-up in 2017 (group A), 2018 (group B), and 2019 (group C) in 13 children hospitals. Results: During the Coronavirus Disease 2019 era, there was a significant decrease in total surgical volume and a change in case mix in terms of an increase in the proportion of emergency operations. Decrease in migration scale index was correlated to the decrease in both surgical volume (r = 0.64, P = .02) and outpatient visit volume (r = 0.61, P = .03). There was a significantly higher proportion of patients who had follow-up through the internet or phone in group C (26.4% vs 9.6% in group B and 8.9% in group A; P < .0001). There was no statistical difference in death or rehospitalization among the 3 follow-up groups (P =. 49). There was higher parents' anxiety score (P < .0001) and more use of telemedicine (P = .004) in group C compared with groups A and B. Conclusions: The Coronavirus Disease 2019 pandemic has resulted in a considerable decrease in total surgical volume and a change of case mix, which seems to be related to the strict traffic ban. Follow-up through the online medical service appears to be an effective alternative to the conventional method. AD - H. Zhang, Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, National Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China Y. Ding, Department of Cardiothoracic Surgery Shenzhen Children Hospital Shenzhen, Guangdong, China AU - Shi, G. AU - Huang, J. AU - Pi, M. AU - Chen, X. AU - Li, X. AU - Ding, Y. AU - Zhang, H. AU - Xiao, D. AU - Huang, G. AU - Ming, T. AU - Huang, P. AU - Liu, C. AU - Zhao, Q. AU - Tian, H. AU - Chen, R. AU - Guo, J. AU - Tang, J. AU - Chen, W. AU - Chen, H. DB - UEmbase DO - 10.1016/j.jtcvs.2020.11.074 IS - 5 KW - information processing device anxiety aortic arch interruption aortic coarctation article atrioventricular septal defect case mix child childhood mortality China clinical article clinical outcome cohort analysis congenital heart disease controlled study coronavirus disease 2019 COVID-19 nucleic acid testing COVID-19 serological testing data analysis software elective surgery Fallot tetralogy female follow up great vessels transposition heart atrium septum defect heart right ventricle double outlet heart single ventricle heart surgery heart ventricle septum defect hospital readmission human infant internet access lung vein drainage anomaly male migration observational study pandemic parent patent atrial duct pediatric surgery priority journal pulmonary valve atresia pulmonary vein malformation scoring system surgical patient surgical volume telemedicine LA - English M3 - Article N1 - L2010573172 2021-01-12 PY - 2021 SN - 1097-685X 0022-5223 SP - 1605-1614.e4 ST - Impact of early Coronavirus Disease 2019 pandemic on pediatric cardiac surgery in China T2 - Journal of Thoracic and Cardiovascular Surgery TI - Impact of early Coronavirus Disease 2019 pandemic on pediatric cardiac surgery in China UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2010573172&from=export http://dx.doi.org/10.1016/j.jtcvs.2020.11.074 VL - 161 ID - 4341 ER - TY - JOUR AB - The objective of this analysis was to update trends in LOS and costs by survivorship and ECMO use among neonates with hypoplastic left heart syndrome (HLHS) undergoing stage 1 palliation surgery using 2016 data from the Healthcare Cost and Utilization Project Kids’ Inpatient Database. We identified neonates ≤ 28 days old with HLHS undergoing Stage 1 surgery, defined as a Norwood procedure with modified Blalock–Taussig (BT) shunt, Sano modification, or both. Multivariable regression with year random effects was used to compare LOS and costs by hospital region, case volume, survivorship, and ECMO vs. no ECMO. An E-value analysis, an approach for conducting sensitivity analysis for unmeasured confounding, was performed to determine if unmeasured confounding contributed to the observed effects. Significant differences in total costs, LOS, and mortality were noted by hospital region, ECMO use, and sub-analyses of case volume. However, other than ECMO use and mortality, the maximum E-value confidence interval bound was 1.71, suggesting that these differences would disappear with an unmeasured confounder 1.71 times more associated with both the outcome and exposure (e.g., socioeconomic factors, environment, etc.) Our findings confirm previous literature demonstrating significant resource utilization among Norwood patients, particularly those undergoing ECMO use. Based on our E-value analysis, differences by hospital region and case volume can be explained by moderate unobserved confounding, rather than a reflection of the quality of care provided. Future analyses on surgical quality must account for unobserved factors to provide meaningful information for quality improvement. AD - C.L. Gong, Division of Neonatology, Department of Pediatrics, Fetal & Neonatal Institute, Children’s Hospital Los Angeles, 4650 Sunset Boulevard, MS #31, Los Angeles, CA, United States AU - Gong, C. L. AU - Song, A. Y. AU - Horak, R. AU - Friedlich, P. S. AU - Lakshmanan, A. AU - Pruetz, J. D. AU - Yieh, L. AU - Ram Kumar, S. AU - Williams, R. G. DB - UEmbase DO - 10.1007/s00246-020-02348-5 IS - 5 KW - article environment extracorporeal oxygenation female health care cost health care quality health care utilization heart surgery human hypoplastic left heart syndrome length of stay major clinical study male mortality newborn palliative therapy social status survival survivorship treatment outcome LA - English M3 - Article N1 - L2004783819 2020-05-08 2020-07-14 PY - 2020 SN - 1432-1971 0172-0643 SP - 996-1011 ST - Impact of Confounding on Cost, Survival, and Length-of-Stay Outcomes for Neonates with Hypoplastic Left Heart Syndrome Undergoing Stage 1 Palliation Surgery T2 - Pediatric Cardiology TI - Impact of Confounding on Cost, Survival, and Length-of-Stay Outcomes for Neonates with Hypoplastic Left Heart Syndrome Undergoing Stage 1 Palliation Surgery UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2004783819&from=export http://dx.doi.org/10.1007/s00246-020-02348-5 VL - 41 ID - 4410 ER - TY - JOUR AB - PURPOSE: The purpose of this study is to identify gaps in support for parents of children with Hypoplastic Left Heart Syndrome. DESIGN AND METHODS: Using a mixed-methods approach, the researchers first studied the parental and care team experience through interviews of Hypoplastic Left Heart Syndrome mothers and members of the inter-professional care team and then conducted an international survey of 690 Hypoplastic Left Heart Syndrome primary caregivers to validate the qualitative findings. RESULTS: Parental and care team interviews revealed three main gaps in parental support, including lack of open communication, unrealistic parental expectations, and unclear inter-professional team roles. Survey results found that parents whose children were diagnosed with Hypoplastic Left Heart Syndrome after birth indicated significant dissatisfaction with the care team's open communication and welcoming of feedback (p = 0.008). As parents progress through the stages of surgical intervention, they also indicate significant dissatisfaction with the care team's anticipation of parental emotional needs and provision of coping resources (p = 0.003). CONCLUSIONS: Parental support interventions should focus on providing resources to help parents cope, helping the care team model open communication, and welcoming feedback on the parental experience. PRACTICE IMPLICATIONS: Interventions should be piloted with parents who are in the later stages of the surgical intervention timeline or whose children were diagnosed after birth as they are the populations who perceived the least support within this study. AD - Nursing Department, St. Catherine University, St. Paul, MN, USA. Wanek Program for Hypoplastic Left Heart Syndrome, Mayo Clinic, Rochester, MN, USA. Center for Regenerative Medicine, Mayo Clinic, Rochester, MN, USA. Division of General Internal Medicine, Mayo Clinic, Rochester, MN, USA. Department of Molecular Pharmacology and Experimental Therapeutics, Mayo Clinic, Rochester, MN, USA. Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA. Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA. Department of Interprofessional Education, St. Catherine University, St. Paul, MN, USA. Women's Health Integrative Research Center, St. Catherine University, St. Paul, MN, USA. AN - 33966677 AU - Landry, M. AU - Katers, M. AU - Olson, E. AU - Cern, L. AU - Nelson, T. J. AU - Campbell, K. A. DA - May 10 DB - UPubmed DO - 10.1017/s1047951121001736 DP - NLM ET - 2021/05/11 KW - Hypoplastic left heart syndrome (HLHS) congenital heart disease (CHD) family-centred care inter-professional care team parental support LA - eng N1 - 1467-1107 Landry, Meghan Katers, Meghan Olson, Erica Cern, Lynn Nelson, Timothy J Campbell, Katherine A Orcid: 0000-0002-4571-3149 Journal Article England Cardiol Young. 2021 May 10:1-8. doi: 10.1017/S1047951121001736. PY - 2021 SN - 1047-9511 SP - 1-8 ST - Identifying gaps in parental support for families of children with hypoplastic left heart syndrome T2 - Cardiol Young TI - Identifying gaps in parental support for families of children with hypoplastic left heart syndrome ID - 2340 ER - TY - JOUR AB - Hypoplastic left heart syndrome (HLHS) accounts for 2% to 3% of all congenital heart disease but is responsible for 25% to 40% of all neonatal cardiac deaths. Although the exact genetic origins of HLHS have not been clearly defined, various genetic and chromosomal associations have been identified. Advancements in fetal echocardiography have resulted in accurate diagnosis of congenital heart disease. On the basis of physical examination findings, fetuses may be candidates for prenatal intervention. In general, after prenatal diagnosis of HLHS, parents are faced with 2 choices: termination or continuation of pregnancy. If pregnancy is continued to delivery, patients may choose comfort care, surgical palliation with the Fontan procedure, or transplantation. A once lethal congenital anomaly, HLHS has undergone a marked evolution in management and prognosis during the last several decades.With advancements in prenatal diagnosis, neonatal management, and surgical palliation, patient survival has drastically improved: at an experienced center, current survival rates are very high after the Norwood procedure, with high rates of overall freedom from death or transplantation through 20 years. With survival becoming more promising, the issues that now take precedence are neurodevelopmental outcomes, Fontan procedure complications, and quality of life. Although much progress has been made in caring for this patient population, HLHS remains a high-risk condition that requires lifelong medical follow-up and has significant long-term morbidity, affecting overall quality of life for patients and their families. © 2015 by the American Academy of Pediatrics. All rights reserved. AD - British Columbia Children’s Hospital, Vancouver, BC, Canada Lucile Packard Children’s Hospital at Stanford University Medical Center, Palo Alto, CA, United States AU - Moodley, S. AU - Tacy, T. A. DB - Scopus DO - 10.1542/neo.16-2-e109 IS - 2 M3 - Article N1 - Export Date: 15 June 2020 PY - 2015 SP - e109-e119 ST - Hypoplastic left heart syndrome: Diagnosis, care and management from fetal life and beyond T2 - NeoReviews TI - Hypoplastic left heart syndrome: Diagnosis, care and management from fetal life and beyond UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-84922329193&doi=10.1542%2fneo.16-2-e109&partnerID=40&md5=559aec5877e2542812b0122994261205 VL - 16 ID - 6810 ER - TY - JOUR AD - J.K. Potter, Department of Emergency Medicine, Carolinas Medical Center, Blvd Charlotte, Blythe, NC, United States AU - Potter, J. K. AU - Clemente, J. D. AU - Asimos, A. W. DB - UEmbase DO - 10.1016/j.ajem.2020.11.055 KW - warfarin artery occlusion article ataxia attention deficit disorder basilar artery brain ischemia case report child clinical article computed tomographic angiography confusion cranial nerve paralysis craniectomy diagnostic imaging dizziness endotracheal intubation fever Fontan procedure headache hemiparesis human incidence lethargy male mechanical thrombectomy mental health nausea nuclear magnetic resonance imaging pediatric basilar artery occlusion physical examination priority journal pulmonary valve atresia symptomatology thrombectomy vomiting x-ray computed tomography LA - English M3 - Article N1 - L2010318063 2020-12-22 PY - 2021 SN - 1532-8171 0735-6757 SP - 221-224 ST - Hyperdense basilar artery identified on unenhanced head CT in three cases of pediatric basilar artery occlusion T2 - American Journal of Emergency Medicine TI - Hyperdense basilar artery identified on unenhanced head CT in three cases of pediatric basilar artery occlusion UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2010318063&from=export http://dx.doi.org/10.1016/j.ajem.2020.11.055 VL - 42 ID - 4345 ER - TY - JOUR AB - We treated three pediatric cardiac transplant patients with chronic parvovirus viremia with high-dose intravenous immunoglobulin (HD-IVIG). One patient with severe T-cell lymphopenia suffered recurrent viremia and aseptic meningitis, which resolved remarkably when he was switched to highdose hyaluronidase-facilitated subcutaneous immunoglobulin (HD-SCIG-Hy). We discuss the advantages of HD-SCIG-Hy vs HD-IVIG treatment for similar cases. AD - J.E. Walter, 501 6th Ave South, St. Petersburg, FL, United States AU - Cruz, R. J. AU - Dasso, J. F. AU - Duff, C. AU - Krasnopero, D. AU - Long, Z. AU - Ellison, M. AU - Nieves, D. AU - Sriaroon, P. AU - Asante-Korang, A. AU - Walter, J. E. DB - UEmbase DO - 10.1093/OFID/OFAA076 IS - 5 KW - hemoglobin hyaluronidase immunoglobulin G mycophenolic acid prednisone rapamycin tacrolimus thymocyte antibody adult antiretroviral therapy article case report CD4 lymphocyte count CD8+ T lymphocyte child chronic kidney failure clinical article drug administration route drug dose increase drug megadose drug substitution drug withdrawal erythema infectiosum human hypoplastic left heart syndrome immunomodulation immunosuppressive treatment lymphocytopenia male meningitis patient history of heart transplantation posttransplant lymphoproliferative disease proteinuria pulmonary valve atresia quality of life school child T lymphocyte virus load young adult LA - English M3 - Article N1 - L2011117863 2021-03-02 2021-03-19 PY - 2020 SN - 2328-8957 ST - Hyaluronidase-facilitated high-dose subcutaneous igg effectively controls parvovirus b19 infection in a pediatric cardiac transplant patient with severe t-cell lymphopenia T2 - Open Forum Infectious Diseases TI - Hyaluronidase-facilitated high-dose subcutaneous igg effectively controls parvovirus b19 infection in a pediatric cardiac transplant patient with severe t-cell lymphopenia UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2011117863&from=export http://dx.doi.org/10.1093/OFID/OFAA076 VL - 7 ID - 4447 ER - TY - JOUR AB - We report the case of a 5-year-old boy who developed chronic plastic bronchitis after Fontan surgery for a complex congenital heart disease. During a new admission for acute exacerbation of plastic bronchitis, he started on a mucolytic treatment with inhaled rhDNAse instead of inhaled fibrinolytics because of the potential bleeding risk in a patient on combined coumarin and aspirin treatment. Respiratory symptoms resolved promptly, and the patient was discharged home on rhDNAse treatment. He remained clinically stable on rhDNAse treatment without further hospitalization until definitive treatment with dynamic lymphangiography and percutaneous embolization. © 2020, Springer Science+Business Media, LLC, part of Springer Nature. AD - Division of Neonatal and Pediatric Intensive Care, Department of Pediatrics, Obstetrics and Gynecology, Geneva University Hospitals, Geneva, Switzerland Division of Clinical Pathology, Geneva University Hospitals, Geneva, Switzerland Pediatric Pulmonology Unit, Department of Pediatrics, Obstetrics and Gynecology, Geneva University Hospitals, Geneva, Switzerland AU - Grazioli, S. AU - Rougemont, A. L. AU - Ruchonnet-Métrailler, I. DB - UScopus DO - 10.1007/s00246-020-02368-1 IS - 5 KW - Bronchial casts Children Congenital heart disease Fontan physiology Plastic bronchitis Pulmonary lymphatics M3 - Article N1 - Export Date: 10 May 2021 PY - 2020 SP - 1071-1073 ST - Human Deoxyribonuclease (rhDNase) Nebulization as an Alternative Treatment for Refractory Plastic Bronchitis After Fontan Surgery T2 - Pediatric Cardiology TI - Human Deoxyribonuclease (rhDNase) Nebulization as an Alternative Treatment for Refractory Plastic Bronchitis After Fontan Surgery UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85084580780&doi=10.1007%2fs00246-020-02368-1&partnerID=40&md5=5164a18dec4986a31026b4587dcd35b5 VL - 41 ID - 5902 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 - OEmbase 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 - 3834 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 - OEmbase 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 - 3474 ER - TY - JOUR AB - Data from neurological and radiological research show an abnormal neurological development in patients treated for hypoplastic left heart syndrome. Thus, the aim of this study was to survey the quality of life scores in comparison with healthy children and children with other heart diseases (mild, moderate, and severe heart defects, heart defects in total). Children with hypoplastic left heart syndrome (aged 6.3-16.9 years) under compulsory education requirements, who were treated at the Children's Heart Center Linz between 1997 and 2009 (n = 74), were surveyed. Totally, 41 children and 44 parents were examined prospectively by psychologists according to Pediatric Quality of Life Inventory, a health-related quality of life measurement. The results of the self-assessments of health-related quality of life on a scale of 1-100 showed a wide range, from a minimum of 5.00 (social functioning) to a maximum of 100 (physical health-related summary scores, emotional functioning, school functioning), with a total score of 98.44. The parents' assessments (proxy) were quite similar, showing a range from 10 (social functioning) up to 100. Adolescent hypoplastic left heart syndrome patients rated themselves on the same level as healthy youths and youths with different heart diseases. The results show that patients with hypoplastic left heart syndrome aged 6-16 years can be successfully supported and assisted in their psychosocial development even if they show low varying physical and psychosocial parameters. The finding that adolescent hypoplastic left heart syndrome patients estimated themselves similar to healthy individuals suggests that they learnt to cope with a severe heart defect. AD - Children's Heart Center Linz, Kepler University Hospital, 4020 Linz, Austria Department of Inclusive Education, University of Education of Upper Austria, 4020 Linz, Austria AN - 144813687. Language: English. Entry Date: 20210115. Revision Date: 20210401. Publication Type: journal article. Journal Subset: Biomedical AU - Oberhuber, Raphael D. AU - Huemer, Sonja AU - Mair, Rudolf AU - Sames-Dolzer, Eva AU - Kreuzer, Michaela AU - Tulzer, Gerald DB - Ucinahl DO - 10.1017/S1047951120000554 DP - EBSCOhost IS - 4 KW - Adaptation, Psychological Hypoplastic Left Heart Syndrome -- Psychosocial Factors Quality of Life -- Psychosocial Factors Schools Health Status Prospective Studies Adolescence Retrospective Design Female Child Male Scales N1 - Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. Instrumentation: Pediatric Quality of Life Inventory (PEDSQL); Wide Range Achievement Test (WRAT); Health-Related Hardiness Scale (HRHS); Quality of Life Inventory (QOLI). NLM UID: 9200019. PMID: NLM32216849. PY - 2020 SN - 1047-9511 SP - 539-548 ST - Health-related quality of life for children and adolescents in school age with hypoplastic left heart syndrome: a single-centre study T2 - 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=144813687&site=ehost-live&scope=site VL - 30 ID - 5306 ER - TY - JOUR AB - Background: Managing oral anticoagulant therapy with vitamin K antagonists remains challenging in paediatric medicine. Aims: This study aimed to assess the correlation between time in therapeutic range and quality of life in children participating in a non-selective International Normalised Ratio self-monitoring and vitamin K antagonist education programme. Methods: Children aged from 2 to 18 years and receiving vitamin K antagonist therapy were eligible for this prospective multicentre study. Clinical and demographic data were collected. Health-related quality of life was assessed using the PedsQL™ 4.0 questionnaire. Correlations between quality of life scores and time in therapeutic range were measured. Results: A total of 121 children were included in the study (mean age 9.6 ± 4.9 years). Cardiac conditions were the predominant indication for vitamin K antagonists. The mean time in therapeutic range was 0.78 ± 0.15 overall, and 0.76 ± 0.24 over the 3-month period before quality of life assessment. The mean total quality of life score was 76.2 ± 18 in self reports, 71.4 ± 22 in mother reports and 73.5 ± 19 in father reports. The time in therapeutic range correlated with the total quality of life scores in self reports (r = 0.22; P = 0.04), mother reports (r = 0.23; P = 0.02) and father reports (r = 0.28; P = 0.02). The time in therapeutic range predominantly correlated with school functioning in self reports (r = 0.38; P = 0.002) and mother reports (r = 0.40; P < 0.001), and with physical functioning in father reports (r = 0.28; P = 0.03). Conclusions: Time in therapeutic range correlated with quality of life in children participating in a non-selective International Normalised Ratio self-monitoring and vitamin K antagonist education programme. Regular assessment of quality of life in patient education programmes contributes towards understanding the concerns and needs of patients. AD - P. Amedro, Paediatric and congenital cardiology department, Arnaud-De-Villeneuve University Hospital, 371, avenue du Doyen-Giraud, Montpellier, France AU - Abassi, H. AU - Bajolle, F. AU - Werner, O. AU - Auer, A. AU - Marquina, A. AU - Mura, T. AU - Lavastre, K. AU - Guillaumont, S. AU - Manna, F. AU - Auquier, P. AU - Bonnet, D. AU - Amedro, P. DB - UEmbase DO - 10.1016/j.acvd.2020.05.022 IS - 12 KW - CoaguChek XS CoaguChek XS Connect software coagulometer antivitamin K adolescent adult anticoagulant therapy article child controlled study correlation analysis cross-sectional study diagnosis education program emotionality female Fontan procedure human international normalized ratio Likert scale major clinical study male mother multicenter study patient education Pediatric Quality of Life Inventory prospective study quality of life quality of life assessment self monitoring self report social interaction social psychology LA - English French M3 - Article N1 - L2008335129 2020-11-10 2021-01-20 PY - 2020 SN - 1875-2128 1875-2136 SP - 811-820 ST - Health-related quality of life correlates with time in therapeutic range in children on anticoagulants with International Normalised Ratio self-monitoring T2 - Archives of Cardiovascular Diseases TI - Health-related quality of life correlates with time in therapeutic range in children on anticoagulants with International Normalised Ratio self-monitoring UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2008335129&from=export http://dx.doi.org/10.1016/j.acvd.2020.05.022 VL - 113 ID - 4379 ER - TY - JOUR AB - INTRODUCTION: Advances in diagnostic methods, surgical techniques and postoperative intensive care have significantly increased the survival rates for children with congenital heart disease. In this context, long term sequelae acquired greater relevance. Health-related quality of life (HRQOL) is a concept that helps to measure the impact of chronic diseases. The purpose of the current study was to describe HRQOL in children with congenital heart disease that undergo cardiac surgery during their first year of life. METHODS: A cross-sectional observational study was conducted between August 2017 and December 2018 at a University General Hospital. PedsQL 4.0 Generic Core Scales were used. Children with congenital heart disease between 2 and 4 years old who had cardiac surgery during their first year of life and healthy children were included. Scores were compared with T-test or Wilcoxon according to the observed distribution. p value < 0.05 was considered significant. RESULTS: A total of 31 children with congenital heart disease (26 % with a single ventricle) and 62 healthy children were enrolled. The first surgery was in the neonatal period in 61.3 %. Our study showed no statistical differences (p = 0.10) between HRQOL Total Scale Score of children with congenital heart disease compared to healthy children. However, lower scores were observed with statistically significant differences in social (p = 0.0092) and school (p = 0.0001) scales. CONCLUSIONS: Our cohort of children diagnosed with congenital heart disease has a global quality of life comparable with healthy children except in social and school functioning scales. AD - Servicio de Clínica Pediátrica, Departamento de Pediatría, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires. melina.saavedra@hiba.org.ar. Servicio de Cardiología Pediátrica, Departamento de Pediatría, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires. Servicio de Clínica Pediátrica, Departamento de Pediatría, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires. AN - 32470251 AU - Saavedra, M. J. AU - Eymann, A. AU - Pérez, L. AU - Busaniche, J. AU - Nápoli, N. AU - Marantz, P. AU - Llera, J. DA - Jun DB - UPubmed DO - 10.5546/aap.2020.eng.166 DP - NLM ET - 2020/05/30 IS - 3 KW - Case-Control Studies Child, Preschool Cross-Sectional Studies Female Health Status Indicators Heart Defects, Congenital/*surgery Humans Infant Infant, Newborn Male *Quality of Life *cardiac surgery *chronic disease *congenital heart disease *health related quality of life LA - eng spa N1 - 1668-3501 Saavedra, Melina J Eymann, Alfredo Pérez, Lucía Busaniche, Julio Nápoli, Natalia Marantz, Pablo Llera, Julián Journal Article Observational Study Argentina Arch Argent Pediatr. 2020 Jun;118(3):166-172. doi: 10.5546/aap.2020.eng.166. OP - Calidad de vida relacionada con la salud en niños con cardiopatía congénita operados durante el primer año de vida. PY - 2020 SN - 0325-0075 SP - 166-172 ST - Health related quality of life in children with congenital heart disease that undergo cardiac surgery during their first year of life T2 - Arch Argent Pediatr TI - Health related quality of life in children with congenital heart disease that undergo cardiac surgery during their first year of life VL - 118 ID - 2369 ER - TY - JOUR AB - BACKGROUND: Despite the advances in medical and surgical care have improved the survival rates of children with congenital heart disease (CHD), they still remain risky for nutritional, cognitive problems, and quality of life. Those impacts vary according to the severity of heart lesions and still manifested years after surgery. AIM: The objective of this study was to compare growth, development, and quality of life between cyanotic and acyanotic CHD. METHODS: The study was performed on 52 patients aged 24–69 months old from June to January 2018 in Sanglah Pediatric Cardiology clinic used WHO Anthro software, The Mullen Scales of Early Learning and PedsQL Cardiac module. RESULTS: We found significant different proportion of underweight 11.5% in acyanotic children, 42.3% in cyanotic by weight/age z-score <−2SD (p = 0.033). Height/ age z-score <−3SD 38.5% in cyanotic versus 11.5% in acyanotic (p = 0.025). The cyanotic showed a significant difference in cognitive function, presented by early learning composite score (p = 0.044) particularly in gross motor (p = 0.034) and receptive language (0.047). Quality of life differs significantly between both groups in heart problem and therapy (p = 0.042), treatment anxiety (p = 0.016), cognitive problems (p = 0.038), and communication (p = 0.022). CONCLUSION: Development, growth problems, and lower quality of life are common in cyanotic children, thus highlight the need for longitudinal surveillance. AD - S. Maya, Department of Child Health, Sondosia General Hospital, Bima, West Nusa Tenggara, Indonesia AU - Maya, S. AU - Gunawijaya, E. AU - Yantie, N. P. V. K. AU - Windiani, I. G. A. T. DB - UEmbase DO - 10.3889/oamjms.2020.4047 IS - B KW - oxyhemoglobin acyanotic heart disease anthropometry anxiety article atrioventricular septal defect child cognition cognitive defect communication skill congenital heart disease cross-sectional study cyanotic heart disease development echocardiography female growth heart catheterization heart injury heart surgery human infant language disability lung vascular resistance major clinical study male malnutrition mental deficiency motor function test nutritional assessment oxygen saturation Pediatric Quality of Life Inventory physical appearance pulmonary valve atresia quality of life questionnaire tricuspid valve atresia underweight LA - English M3 - Article N1 - L2005564412 2020-12-14 2020-12-18 PY - 2020 SN - 1857-9655 SP - 613-618 ST - Growth, development, and quality of life in children with congenital heart disease T2 - Open Access Macedonian Journal of Medical Sciences TI - Growth, development, and quality of life in children with congenital heart disease UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2005564412&from=export http://dx.doi.org/10.3889/oamjms.2020.4047 VL - 8 ID - 4434 ER - TY - JOUR AB - Dr Rodney Franklin is the focus of our third in a planned series of interviews in Cardiology in the Young entitled, "Global Leadership in Paediatric and Congenital Cardiac Care."Dr Franklin was born in London, England, spending the early part of his childhood in the United States of America before coming back to England. He then attended University College London Medical School and University College Hospital in London, England, graduating in 1979. Dr Franklin would then go on to complete his general and neonatal paediatrics training in 1983 at Northwick Park Hospital and University College Hospital in London, England, followed by completing his paediatric cardiology training in 1989 at Great Ormond Street Hospital for Children in London, England. During this training, he additionally would hold the position of British Heart Foundation Junior Research Fellow from 1987 to 1989. Dr Franklin would then complete his training in 1990 as a Senior Registrar and subsequent Consultant in Paediatric and Fetal Cardiology at Wilhelmina Sick Children's Hospital in Utrecht, the Netherlands. He subsequently obtained his research doctorate at University of London in 1997, consisting of a retrospective audit of 428 infants with functionally univentricular hearts. Dr Franklin has spent his entire career as a Consultant Paediatric Cardiologist at the Royal Brompton & Harefield Hospital NHS Foundation Trust, being appointed in 1991. He additionally holds honorary Consultant Paediatric Cardiology positions at Hillingdon Hospital, Northwick Park Hospital, and Lister Hospital in the United Kingdom, and Honorary Senior Lecturer at Imperial College, London. He has been the Clinical Lead of the National Congenital Heart Disease Audit (2013-2020), which promotes data collection within specialist paediatric centres. Dr Franklin has been a leading figure in the efforts towards creating international, pan European, and national coding systems within the multidisciplinary field of congenital cardiac care. These initiatives include but are not limited to the development and maintenance of The International Paediatric & Congenital Cardiac Code and the related International Classification of Diseases 11th Revision for CHD and related acquired terms and definitions. This article presents our interview with Dr Franklin, an interview that covers his experience in developing these important coding systems and consensus nomenclature to both improve communication and the outcomes of patients. We additionally discuss his experience in the development and implementation of strategies to assess the quality of paediatric and congenital cardiac care and publicly report outcomes. AD - J.T. Tretter, Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States AU - Tretter, J. T. AU - Jacobs, J. P. DB - UEmbase DO - 10.1017/S104795112000476X KW - cardiologist career child congenital heart disease consensus consultation England fetus heart single ventricle human ICD-11 infant interview leadership major clinical study male Netherlands nomenclature outcome assessment pediatric cardiology pediatric hospital review trust LA - English M3 - Article in Press N1 - L634119515 2021-02-12 PY - 2021 SN - 1467-1107 1047-9511 ST - Global Leadership in Paediatric and Congenital Cardiac Care: "coding our way to improved care: An interview with Rodney C. G. Franklin, MBBS, MD, FRCP, FRCPCH" T2 - Cardiology in the Young TI - Global Leadership in Paediatric and Congenital Cardiac Care: "coding our way to improved care: An interview with Rodney C. G. Franklin, MBBS, MD, FRCP, FRCPCH" UR - https://www.embase.com/search/results?subaction=viewrecord&id=L634119515&from=export http://dx.doi.org/10.1017/S104795112000476X ID - 4373 ER - TY - JOUR AB - Background: This study describes 35 years of experience in a tertiary care level hospital that treats cardiac patients with univentricular heart physiology who underwent Glenn surgery. Methods: The study consisted of a retrospective analysis of patients who underwent Glenn surgery, including variables related to pre-operative, intra-operative, and post-operative morbidity and mortality. Results: From 1980 to 2015, 204 Glenn surgeries were performed. The most common heart disease was tricuspid atresia IB (19.2%). In 48.1% of the cases, the procedure was performed with antegrade flow. A bilateral Glenn procedure was performed in 12.5% of the cases and 10.3% were carried out without using a cardiopulmonary bypass pump. Reported complications included infections, bleeding, arrhythmias, chylothorax, neurological alterations, and pleural effusion. The mortality rate was 2.9%. Conclusions: Glenn surgery is a palliative surgery with good results. It significantly improves patient quality of life over a long period until a total cavopulmonary shunt is performed. The complications observed are few, and the mortality rate is low. Therefore, it is a safe surgery that should be used for univentricular congenital heart disease. AD - G. Hernández-Morales, Departamento de Cirugía Cardiaca en Pediatría, Hospital Militar de Especialidades de la Mujer y Neonatología, Mexico AU - Hernández-Morales, G. AU - Bolio-Cerdán, A. AU - Ruiz-González, S. AU - Romero-Cárdenas, P. AU - Villasís-Keever, M. A. DB - UEmbase DO - 10.24875/BMHIM.20000094 IS - 2 KW - cardiopulmonary bypass cannula catheter adult article bleeding cardiac patient cardiopulmonary bypass cardiovascular infection carotid angioplasty catheterization cavopulmonary connection cerclage child chylothorax congenital hypoplastic anemia female follow up Glenn shunt heart arrhythmia heart single ventricle heterotaxy syndrome human hypoplasia intraoperative morbidity major clinical study male middle aged morbidity mortality rate neurologic disease oximetry palliative therapy perception disorder pleura effusion postoperative morbidity postoperative period preoperative morbidity pulmonary valve atresia quality of life retrospective study stenosis surgical mortality survival rate tertiary care center tricuspid valve atresia urinary tract infection ventilator associated pneumonia LA - English M3 - Article N1 - L2006935115 2021-04-23 2021-05-07 PY - 2021 SN - 1665-1146 0539-6115 SP - 123-129 ST - Glenn surgery: A safe procedure in the path of univentricular correction T2 - Boletin Medico del Hospital Infantil de Mexico TI - Glenn surgery: A safe procedure in the path of univentricular correction UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2006935115&from=export http://dx.doi.org/10.24875/BMHIM.20000094 VL - 78 ID - 4363 ER - TY - JOUR AB - Surgical innovation and multidisciplinary management have allowed children born with univentricular physiology congenital heart disease to survive into adulthood. An estimated global population of 70 000 patients have undergone the Fontan procedure and are alive today, most of whom are <25 years of age. Several unexpected consequences of the Fontan circulation include Fontan-associated liver disease. Surveillance biopsies have demonstrated that virtually 100% of these patients develop clinically silent fibrosis by adolescence. As they mature, there are increasing reports of combined heart-liver transplantation resulting from advanced liver disease, including bridging fibrosis, cirrhosis, and hepatocellular carcinoma, in this population. In the absence of a transplantation option, these young patients face a poor quality of life and overall survival. Acknowledging that there are no consensus guidelines for diagnosing and monitoring Fontan-associated liver disease or when to consider heart transplantation versus combined heart-liver transplantation in these patients, a multidisciplinary working group reviewed the literature surrounding Fontan-associated liver disease, with a specific focus on considerations for transplantation. AD - Liver Transplant Center, Children's Hospital-Los Angeles, CA (J.E.). Department of Surgery (J.E.), Keck School of Medicine, University of Southern California, Los Angeles. Mount Sinai Cardiovascular Institute (A.N.Z.), Recanati/Miller Transplantation Institute, Icahn School of Medicine at Mount Sinai, New York, NY. The Children's Heart Center (A.N.Z.), Recanati/Miller Transplantation Institute, Icahn School of Medicine at Mount Sinai, New York, NY. Divisions of Hepatology (T.S.), Recanati/Miller Transplantation Institute, Icahn School of Medicine at Mount Sinai, New York, NY. Division of Gastrointestinal and Liver Diseases (J.K.), Keck School of Medicine, University of Southern California, Los Angeles. Section of Gastroenterology and Hepatology, Department of Pediatrics, Yale University School of Medicine, New Haven, CT (P.L.V.). Departments of Anesthesiology and Perioperative Medicine (R.E.H.), Mayo Clinic, Rochester, MN. Surgery (T.T.), Mayo Clinic, Rochester, MN. Immunology (T.T.), Mayo Clinic, Rochester, MN. Division of Cardiology, Department of Medicine (J.W.W.), University of Pennsylvania, Philadelphia, PA. Department of Surgery (K.M.O.), University of Pennsylvania, Philadelphia, PA. Pediatric Hepatology (J.B.), Recanati/Miller Transplantation Institute, Icahn School of Medicine at Mount Sinai, New York, NY. Department of Gastroenterology, Liver Care Center, Children's Mercy Kansas City, MO (R.F.). AN - 32776846 AU - Emamaullee, J. AU - Zaidi, A. N. AU - Schiano, T. AU - Kahn, J. AU - Valentino, P. L. AU - Hofer, R. E. AU - Taner, T. AU - Wald, J. W. AU - Olthoff, K. M. AU - Bucuvalas, J. AU - Fischer, R. C2 - PMC7422927 C6 - NIHMS1610326 DA - Aug 11 DB - UPubmed DO - 10.1161/circulationaha.120.045597 DP - NLM ET - 2020/08/11 IS - 6 KW - Fontan procedure heart transplantation liver diseases liver transplantation LA - eng N1 - 1524-4539 Emamaullee, Juliet Zaidi, Ali N Schiano, Thomas Kahn, Jeffrey Valentino, Pamela L Hofer, Ryan E Taner, Timucin Wald, Joyce W Olthoff, Kim M Bucuvalas, John Fischer, Ryan R01 HL141857/HL/NHLBI NIH HHS/United States Journal Article Circulation. 2020 Aug 11;142(6):591-604. doi: 10.1161/CIRCULATIONAHA.120.045597. Epub 2020 Aug 10. PY - 2020 SN - 0009-7322 (Print) 0009-7322 SP - 591-604 ST - Fontan-Associated Liver Disease: Screening, Management, and Transplant Considerations T2 - Circulation TI - Fontan-Associated Liver Disease: Screening, Management, and Transplant Considerations VL - 142 ID - 2357 ER - TY - JOUR AB - The Fontan Outcomes Network was created to improve outcomes for children and adults with single ventricle CHD living with Fontan circulation. The network mission is to optimise longevity and quality of life by improving physical health, neurodevelopmental outcomes, resilience, and emotional health for these individuals and their families. This manuscript describes the systematic design of this new learning health network, including the initial steps in development of a national, lifespan registry, and pilot testing of data collection forms at 10 congenital heart centres. AD - Heart Institute, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH, USA. Division of Cardiology, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA. Department of Pediatrics, Feinberg Northwestern University School of Medicine, Chicago, IL, USA. Division of Developmental and Behavioral Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA. Department of Pediatrics, University of Cincinnati, Cincinnati, OH, USA. Department of Cardiology, Boston Children's Hospital, Boston, MA, USA. Department of Pediatrics, Harvard Medical School, Boston, MA, USA. Department of Pediatric Cardiology, The Mayo Clinic, Rochester, MN, USA. Royal Prince Alfred Hospital, Sydney, Australia. Children's National Cardiac Surgery and Heart Institute, Washington, DC, USA. Sisters by Heart Organization, El Segundo, CA, USA. The National Pediatric Cardiology Quality Improvent Collaborative, Cincinnati, OH, USA. Department of Cardiology, The Children's Hospital of Wisconsin, Milwaukee, WI, USA. Heart Institute, Children's Hospital Colorado, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO, USA. The Anderson Center for Health Systems Excellence, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA. Department of Pediatric Cardiology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA. Heart Institute, UPMC Children's Hospital of Pittsburgh, Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA. Additional Ventures, Palo Alto, CA, USA. Knight Cardiovascular Institute, Oregon Health & Science University, Portland, OR, USA. American Board of Pediatrics, Chapel Hill, NC, USA. Data Management and Analysis Center, Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA. One Brave Idea at American Heart Association, Boston, MA, USA. Department of Cardiothoracic Surgery, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH, USA. Department of Pediatric Cardiology, Stanford University, Palo Alto, CA, USA. Rady Children's Hospital, Department of Pediatric Cardiology, San Diego, California. Congenital Heart Center, C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI, USA. AN - 32635947 AU - Alsaied, T. AU - Allen, K. Y. AU - Anderson, J. B. AU - Anixt, J. S. AU - Brown, D. W. AU - Cetta, F. AU - Cordina, R. AU - D'Udekem, Y. AU - Didier, M. AU - Ginde, S. AU - Di Maria, M. V. AU - Eversole, M. AU - Goldberg, D. AU - Goldstein, B. H. AU - Hoffmann, E. AU - Kovacs, A. H. AU - Lannon, C. AU - Lihn, S. AU - Lubert, A. M. AU - Marino, B. S. AU - Mullen, E. AU - Pickles, D. AU - Rathod, R. H. AU - Rychik, J. AU - Tweddell, J. S. AU - Wooton, S. AU - Wright, G. AU - Younoszai, A. AU - Glenn, T. AU - Wilmoth, A. AU - Schumacher, K. DA - Aug DB - UPubmed DO - 10.1017/s1047951120001869 DP - NLM ET - 2020/07/09 IS - 8 KW - Fontan improving outcomes registry single ventricle LA - eng N1 - 1467-1107 Alsaied, Tarek Orcid: 0000-0002-3777-4822 Allen, Kiona Y Anderson, Jeffrey B Anixt, Julia S Brown, David W Cetta, Frank Cordina, Rachael D'udekem, Yves Didier, Meghan Ginde, Salil Di Maria, Michael V Eversole, Michelle Goldberg, David Goldstein, Bryan H Hoffmann, Erin Kovacs, Adrienne H Lannon, Carole Lihn, Stacey Lubert, Adam M Marino, Bradley S Mullen, Emily Pickles, Diane Rathod, Rahul H Rychik, Jack Tweddell, James S Wooton, Sharyl Wright, Gail Younoszai, Adel Glenn, Tom Wilmoth, Alicia Schumacher, Kurt Journal Article England Cardiol Young. 2020 Aug;30(8):1070-1075. doi: 10.1017/S1047951120001869. Epub 2020 Jul 8. PY - 2020 SN - 1047-9511 SP - 1070-1075 ST - The Fontan outcomes network: first steps towards building a lifespan registry for individuals with Fontan circulation in the United States T2 - Cardiol Young TI - The Fontan outcomes network: first steps towards building a lifespan registry for individuals with Fontan circulation in the United States VL - 30 ID - 2363 ER - TY - JOUR AB - Background: Children with congenital heart disease (CHD) are at risk of adverse long-term neurodevelopmental outcomes, believed to be, in part, secondary to prenatal insults. Placental pathology and altered fetal middle cerebral arterial (MCA) flow suggestive of brain sparing have been documented in fetal CHD. In the present study we investigated the relationship between MCA and umbilical arterial (UA) flow patterns in fetal transposition of the great arteries (d-TGA) and hypoplastic left heart syndrome (HLHS) and growth and 2-year neurodevelopmental outcomes. Methods: We included children with d-TGA and HLHS who had third-trimester fetal echocardiograms between 2004 and 2014, at which time umbilical artery (UA) and MCA pulsatility indices (PIs) were measured, and who underwent 2-year growth and neurodevelopmental assessments. Results: We identified 24 children with d-TGA and 36 with HLHS. Mean age at fetal echocardiography was 33.8 ± 3.5 weeks. At 2-year follow-up, head circumference z score (standard deviation [SD]) was –0.09 (1.07) and 0.17 (1.7) for the d-TGA and HLHS groups, respectively. Bayley III mean (SD) cognitive, language, and motor scores were 97.7 (10.8), 94.7 (13.4), and 98.6 (8.6) for the d-TGA group and 90.3 (13.9), 87.2 (17.5), and 85.3 (16.2) for the HLHS group. On multivariate linear regression analysis, UA-PI was associated (effect sizes [95% CI]) with length (–1.45 [–2.7, –0.17], P = 0.027), weight (–1.46 [–2.6 to –0.30], P = 0.015) and cognitive scores (–14.86 [–29.95 to 0.23], P = 0.05) at 2 years of age. MCA PI showed no statistically significant correlation. Conclusions: In fetal d-TGA and HLHS, a higher UA-PI in the third trimester, suggestive of placental insufficiency—but not MCA-PI—is associated with worse 2-year growth and neurodevelopment. AD - L.K. Hornberger, Pediatric Cardiology, Stollery Children's Hospital 4C2, 8440-112 Street, Edmonton, AB, Canada AU - Abeysekera, J. B. AU - Gyenes, D. L. AU - Atallah, J. AU - Robertson, C. M. T. AU - Bond, G. Y. AU - Rebeyka, I. M. AU - Moez, E. K. AU - Dinu, I. A. AU - Switzer, H. N. AU - Hornberger, L. K. DB - UEmbase DO - 10.1016/j.cjca.2020.06.024 IS - 3 KW - adult artery blood flow article Bayley Scales of Infant Development child clinical article controlled study echocardiography effect size female fetus follow up great vessels transposition head circumference human hypoplastic left heart syndrome language linear regression analysis nervous system development placenta insufficiency preschool child pulse wave third trimester pregnancy umbilical artery LA - English M3 - Article N1 - L2011015043 2021-02-18 PY - 2021 SN - 0828-282X SP - 425-432 ST - Fetal Umbilical Arterial Pulsatility Correlates With 2-Year Growth and Neurodevelopmental Outcomes in Congenital Heart Disease T2 - Canadian Journal of Cardiology TI - Fetal Umbilical Arterial Pulsatility Correlates With 2-Year Growth and Neurodevelopmental Outcomes in Congenital Heart Disease UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2011015043&from=export http://dx.doi.org/10.1016/j.cjca.2020.06.024 VL - 37 ID - 4349 ER - TY - JOUR AB - BACKGROUND: Fenestration in the Fontan circulation potentially liberates patients from factors leading to cardiovascular remodelling, through stable haemodynamics with attenuated venous congestion. We hypothesised that a fenestrated Fontan procedure would possess chronic haemodynamic advantages beyond the preload preservation. METHODS: We enrolled 35 patients with fenestrated Fontan with a constructed pressure-volume relationship under dobutamine (DOB) infusion and/or transient fenestration occlusion (TFO). Despite the use of antiplatelets and anticoagulants, natural closure of fenestration was confirmed in 11 patients. Cardiovascular properties in patients with patent fenestration (P-F) were compared with those in patients with naturally closed fenestration (NC-F). To further delineate the roles of fenestration, paired analysis in patients with P-F was performed under DOB or rapid atrial pacing with/without TFO. RESULTS: As compared with P-F, patients with NC-F had a higher heart rate (HR), smaller ventricular end-diastolic area, better ejection fraction and higher central venous pressure, with higher pulmonary resistance. While this was similarly observed after DOB infusion, DOB markedly augmented diastolic and systolic ventricular stiffness in patients with NC-F compared with patients with P-F. As a mirror image of the relationship between patients with P-F and NC-F, TFO markedly reduced preload, suppressed cardiac output, and augmented afterload and diastolic stiffness. Importantly, rapid atrial pacing compromised these haemodynamic advantages of fenestration. CONCLUSIONS: As compared with patients with NC-F, patients with P-F had robust haemodynamics with secured preload reserve, reduced afterload and a suppressed beta-adrenergic response, along with a lower HR at baseline, although these advantages had been overshadowed, or worsened, by an increased HR. AD - Department of Pediatrics and Pediatric Cardiology, Kitasato University School of Medicine, Sagamihara, Japan. Department of Pediatric Cardiology, Saitama Medical University, Kawagoe, Japan. Department of Pediatrics, Hokkaido Ryoiku-en, Asahikawa, Japan. AN - 30826770 AU - Saiki, H. AU - Kuwata, S. AU - Iwamoto, Y. AU - Ishido, H. AU - Taketazu, M. AU - Masutani, S. AU - Nishida, T. AU - Senzaki, H. DA - Aug DB - UPubmed DO - 10.1136/heartjnl-2018-314183 DP - NLM ET - 2019/03/04 IS - 16 KW - Adrenergic beta-1 Receptor Agonists Child Child, Preschool Dobutamine Echocardiography Echocardiography, Stress Female Fontan Procedure/*methods Heart Defects, Congenital/physiopathology/*surgery Heart Failure/prevention & control Heart Rate/*physiology Hemodynamics/*physiology Humans Hyperemia Male Myocardial Contraction/*physiology *Fontan physiology *congenital heart disease *congenital heart surgery *heart failure LA - eng N1 - 1468-201x Saiki, Hirofumi Kuwata, Seiko Iwamoto, Yoichi Ishido, Hirotaka Taketazu, Mio Masutani, Satoshi Nishida, Takashi Senzaki, Hideaki Orcid: 0000-0002-8289-0953 Journal Article Research Support, Non-U.S. Gov't England Heart. 2019 Aug;105(16):1266-1272. doi: 10.1136/heartjnl-2018-314183. Epub 2019 Mar 2. PY - 2019 SN - 1355-6037 SP - 1266-1272 ST - Fenestration in the Fontan circulation as a strategy for chronic cardioprotection T2 - Heart TI - Fenestration in the Fontan circulation as a strategy for chronic cardioprotection VL - 105 ID - 2424 ER - TY - JOUR AB - Survival for hypoplastic left heart syndrome (HLHS) has improved dramatically. Little is known about early family function, quality of life (QOL), or well-being/adjustment for parents of infants with HLHS. Parent/family outcomes over time, predictors, and differences in 143 mothers and 72 fathers were examined. Parents reported better family function compared with published norms, but 26% experienced family dysfunction. QOL and well-being were significantly lower than adult norms. QOL scores generally declined over time, whereas self-reported well-being improved. Responses from mothers and fathers showed different trends, with mothers having worse scores on most measures and at most time points. Being a single parent was a risk factor for poorer family function, but not for lower individual QOL or well-being. Family characteristics, stress, and coping skills were predictive of outcomes. Parents' psychosocial responses to the challenges of life with infants with HLHS change over time. Individually tailored psychosocial support is needed. AU - Mussatto, K. A. AU - Van Rompay, M. I. AU - Trachtenberg, F. L. AU - Pemberton, V. AU - Young-Borkowski, L. AU - Uzark, K. AU - Hollenbeck-Pringle, D. AU - Dunbar-Masterson, C. AU - Infinger, P. AU - Walter, P. AU - Sawin, K. DB - UEmbase DO - 10.1177/1074840720987309 KW - adult article controlled study coping behavior family size father female human hypoplastic left heart syndrome infant male physiological stress psychosocial care quality of life risk factor single parent wellbeing LA - English M3 - Article in Press N1 - L634195641 2021-02-26 PY - 2021 SN - 1552-549X SP - 1074840720987309 ST - Family Function, Quality of Life, and Well-Being in Parents of Infants With Hypoplastic Left Heart Syndrome T2 - Journal of family nursing TI - Family Function, Quality of Life, and Well-Being in Parents of Infants With Hypoplastic Left Heart Syndrome UR - https://www.embase.com/search/results?subaction=viewrecord&id=L634195641&from=export http://dx.doi.org/10.1177/1074840720987309 ID - 4350 ER - TY - JOUR AB - A five-month-old male with a hypoplastic left heart syndrome developed severe respiratory insufficiency due to influenza type B viral pneumonia following bidirectional Glenn. He was treated with extracorporeal membrane oxygenation and successfully weaned without neurological consequences. AD - Department of Pediatrics, University Hospital Centre Zagreb, Kispaticeva, Zagreb, Croatia. Department of Cardiac Surgery, University Hospital Centre Zagreb, Kispaticeva, Zagreb, Croatia. Department of Surgery, Special Hospital for Cardiovascular Disease Magdalena, Ljudevita Gaja, Krapinske Toplice, Croatia. AN - 32294017 AU - Matic, T. AU - Bakos, M. AU - Belina, D. AU - Duric, Z. AU - Bartonicek, D. AU - Malcic, I. DA - May DB - UPubmed DO - 10.1177/2150135119899619 DP - NLM ET - 2020/04/16 IS - 3 KW - Cardiac Surgical Procedures/*adverse effects *Extracorporeal Membrane Oxygenation Heart Bypass, Right Humans Hypoplastic Left Heart Syndrome/*complications/physiopathology Infant Influenza B virus Influenza, Human/*complications/physiopathology Male Pneumonia, Viral/*complications/physiopathology Respiratory Distress Syndrome ECMO (extracorporeal membrane oxygenation) cavopulmonary anastomosis hypoplastic left heart syndrome lung infection LA - eng N1 - 2150-136x Matic, Toni Bakos, Matija Belina, Drazen Duric, Zeljko Bartonicek, Dorotea Malcic, Ivan Case Reports Journal Article United States World J Pediatr Congenit Heart Surg. 2020 May;11(3):370-371. doi: 10.1177/2150135119899619. PY - 2020 SN - 2150-1351 SP - 370-371 ST - Extracorporeal Membrane Oxygenation After Glenn Procedure in a Child With Influenza B Pneumonia T2 - World J Pediatr Congenit Heart Surg TI - Extracorporeal Membrane Oxygenation After Glenn Procedure in a Child With Influenza B Pneumonia VL - 11 ID - 2373 ER - TY - JOUR AB - Objective This study explored the lived experiences of women with congenital heart disease (CHD) during pregnancy and early motherhood. Design Qualitative study using semistructured interviews. Data were analysed according to interpretative phenomenological analysis. Setting San Donato Milanese, Italy. Participants 12 adult women during pregnancy or early motherhood. Results Three main themes emerged from the analysis that were labelled as follows: 'Being a woman with CHD'; 'Being a mother with CHD'; and 'Don't be alone'. Mothers described both positive and negative feelings about their pregnancies and transitions from childless women to mothers with CHD. They needed supportive care to improve the management of their health during pregnancy and early motherhood. Conclusion This study explored the lived experiences of women with CHD during pregnancy and early motherhood. The emerged themes represent an initial framework for implementing theory-grounded educational and supportive strategies that improve self-care, engagement and quality of life for women with CHD. Furthermore, the study's results provide guidance for operationalising the described experiences into items and domains for future cross-national surveys. © © Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. AD - Health Professions Research and Development Unit, IRCCS Policlinico San Danato, San Donato Milanese, Lombardia, Italy Department of Psychology, EngageMinds HUB-Consumer and Health Research Center, Università Cattolica Del Sacro Cuore, Milan, Italy Department of Biomedicine and Prevention, University of Rome Tor Vergata, Roma, Lazio, Italy AU - Flocco, S. F. AU - Caruso, R. AU - Barello, S. AU - Nania, T. AU - Simeone, S. AU - Dellafiore, F. C7 - e034588 DB - UScopus DO - 10.1136/bmjopen-2019-034588 IS - 1 KW - cardiology congenital heart disease qualitative research M3 - Article N1 - Cited By :1 Export Date: 10 May 2021 PY - 2020 ST - Exploring the lived experiences of pregnancy and early motherhood in Italian women with congenital heart disease: An interpretative phenomenological analysis T2 - BMJ Open TI - Exploring the lived experiences of pregnancy and early motherhood in Italian women with congenital heart disease: An interpretative phenomenological analysis UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85078297712&doi=10.1136%2fbmjopen-2019-034588&partnerID=40&md5=7baa0a7310916c99c6a9157a4cc82ba8 VL - 10 ID - 5916 ER - TY - JOUR AB - Background: Most children who have congenital heart disease in low-and middle-income countries (LMICs), including Uzbekistan, do not receive adequate and timely pediatric cardiac surgical care. To strengthen the surgical capacity of a local pediatric cardiac surgery team in Tashkent, Uzbekistan, the JW LEE Center for Global Medicine at Seoul National University College of Medicine has developed a team-based training program and has been collaboratively conducting surgeries and care in order to transfer on-site knowledge and skills from 2009 to 2019. Objectives: To evaluate the long-term effects of the collaborative program on the cardiac surgical capacity of medical staff (teamwork, surgical complexity, and patients’ pre-surgical weights) as well as changes in the lives of the patients and their families. To derive lessons and challenges for other pediatric cardiac surgical programs in LMICs. Methods: To assess the effects of this ten-year long program, a mixed-methods design was developed to examine the trend of surgical complexity measured by Risk Adjustment for Congenital Heart Surgery 1 score (RACHS-1) and patients’ pre-surgical weights via medical record review (surgical cases: n = 107) during the decade. Qualitative data was analyzed from in-depth interviews (n = 31) with Uzbek and Korean medical staff (n = 10; n = 4) and caregivers (n = 17). Findings: During the decade, the average RACHS-1 of the cases increased from 1.9 in 2010 to 2.78 in 2019. The average weight of patients decreased by 2.8 kg from 13 kg to 10.2 kg during the decade. Qualitative findings show that the surgical capacity, as well as attitudes toward patients and colleagues of the Uzbek medical staff, improved through the effective collaboration between the Uzbek and Korean teams. Changes in the lives of patients and their families were also found following successful surgery. Conclusions: Team-based training of the workforce in Uzbekistan was effective in improving the surgical skills, teamwork, and attitudes of medical staff, in addition, a positive impact on the life of patients and their families was demonstrated. It can be an effective solution to facilitate improvements in pediatric cardiovascular disease in LMICs if training is sustained over a long period. AD - W.-H. Kim, Program in Global Surgery and Implementation Science, JW LEE Center for Global Medicine, Seoul National University College of Medicine, Seoul, South Korea W.-H. Kim, Department of Thoracic and Cardiovascular Surgery, Seoul National University College of Medicine, Seoul, South Korea W.-H. Kim, Department of Thoracic and Cardiovascular Surgery, Seoul National University Children’s Hospital, Seoul, South Korea AU - Han, S. AU - Choi, S. AU - Heo, J. AU - Park, J. AU - Kim, W. H. DB - UEmbase DO - 10.5334/AOGH.2883 IS - 1 KW - administrative personnel anesthesiologist aortic coarctation article atrioventricular septal defect capacity building cardiac surgeon cardiologist caregiver child collaborative care team congenital heart disease Ebstein anomaly Fallot tetralogy family female great vessels transposition health personnel attitude health program heart atrium septum defect heart right ventricle double outlet heart single ventricle heart surgery heart ventricle septum defect human low income country lung vein drainage anomaly major clinical study male medical record review medical staff middle income country nurse operating room personnel pediatric surgery perfusionist preschool child professional competence program evaluation pulmonary valve atresia qualitative research quantitative study risk assessment scientist teamwork tricuspid valve atresia Uzbekistan LA - English M3 - Article N1 - L2005112643 2020-10-12 2020-10-16 PY - 2020 SN - 2214-9996 SP - 1-11 ST - Evaluation of a ten-year team-based collaborative capacity-building program for pediatric cardiac surgery in uzbekistan: Lessons and implications T2 - Annals of Global Health TI - Evaluation of a ten-year team-based collaborative capacity-building program for pediatric cardiac surgery in uzbekistan: Lessons and implications UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2005112643&from=export http://dx.doi.org/10.5334/AOGH.2883 VL - 86 ID - 4440 ER - TY - JOUR AU - Mellander, M. AU - Berntsson, L. AU - Nilsson, B. DB - UEmbase DO - 10.1111/j.1651-2227.2007.00221.x IS - 2 KW - erratum error priority journal LA - English M3 - Erratum N1 - L46307374 2007-03-21 PY - 2007 SN - 0803-5253 1651-2227 SP - 324 ST - Erratum: Quality of life in children with hypoplastic left heart syndrome (Acta Paediatrica, International Journal of Paediatrics (2007) 96, (53-57)) T2 - Acta Paediatrica, International Journal of Paediatrics TI - Erratum: Quality of life in children with hypoplastic left heart syndrome (Acta Paediatrica, International Journal of Paediatrics (2007) 96, (53-57)) UR - https://www.embase.com/search/results?subaction=viewrecord&id=L46307374&from=export http://dx.doi.org/10.1111/j.1651-2227.2007.00221.x VL - 96 ID - 5113 ER - TY - JOUR AB - Rationale: Anesthetic management of pregnant women with Fontan circulation remains challenging. There are few reports that describe the anesthetic management of cesarean section after Fontan surgery. Here, we present a case of successful epidural anesthesia in a woman with Fontan circulation who required emergency cesarean section.Patient Concerns: A 29-year-old woman at gestational week 28 was scheduled for emergency cesarean section because of fetal distress. Her past medical history was significant for congenital transposition of the great arteries that had been treated by Fontan surgery 26 years earlier. Her postoperative course had been uneventful and she had reached a near normal level of activity with no arrhythmias or thrombotic complications. On presentation, her oxygen saturation was approximately 84% and she had digital clubbing. Arterial blood gas analysis showed a PCO2 of 35 mmHg, PO2 of 55.5 mmHg, and hemoglobin of 16.3 g/dL. Her blood coagulation parameters were within normal limits except for a high fibrinogen concentration (4.55 g/L).Diagnosis: The diagnosis was pregnancy requiring emergency cesarean section because of fetal distress.Interventions: Before anesthesia, a radial artery line was established for continuous measurement of blood pressure. An air pressure pump was placed on the patient's lower limbs and a low-dose dobutamine infusion was started. Next, epidural anesthesia was successfully performed at L2-3. Five milliliters of 2% lidocaine followed by 10 mL of 0.75% ropivacaine were injected. Dobutamine was infused to maintain a target blood pressure of 100-120/60-70 mmHg.Outcomes: The procedure was uneventful with the patient maintaining a stable heart rate of 80 to 90 beats/min and an oxygen saturation of 90% to 94%. A male infant weighing 840 g was delivered. The Apgar score was 9 at 1 and 5 minutes. The patient was transferred to the intensive care unit for 20 hours of monitoring and discharged 9 days later. The neonate was discharged after 2 months of specialist neonatal treatment.Lessons: Epidural anesthesia may be used in women with Fontan circulation undergoing emergency cesarean section. Knowledge of the physiology of the heart lesion and that of pregnancy are critical to the outcome. AD - Department of Anesthesia, First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, PR China AN - 144197129. Language: English. Entry Date: 20200625. Revision Date: 20210112. Publication Type: journal article AU - Pin, Wu AU - Sheng-Mei, Zhu AU - Yong-Xing, Yao AU - Wu, Pin AU - Zhu, Sheng-Mei AU - Yao, Yong-Xing DB - Ucinahl DO - 10.1097/MD.0000000000018986 DP - EBSCOhost IS - 4 KW - Anesthesia, Obstetrical -- Methods Cardiopulmonary Bypass -- Adverse Effects Anesthesia, Epidural -- Methods Pregnancy Complications, Cardiovascular -- Therapy Infant, Newborn Pregnancy Cesarean Section -- Methods Fetal Distress Adult Infant, Premature Female Apgar Score N1 - case study. Journal Subset: Biomedical; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. Instrumentation: Apgar Score. NLM UID: 2985248R. PMID: NLM31977915. PY - 2020 SN - 0025-7974 SP - 1-3 ST - Epidural anesthesia for emergency cesarean section in a woman with Fontan circulation: A case report T2 - Medicine TI - Epidural anesthesia for emergency cesarean section in a woman with Fontan circulation: A case report UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=144197129&site=ehost-live&scope=site VL - 99 ID - 5334 ER - TY - JOUR AB - Background: The aim of the study was to evaluate the educational achievement of patients diagnosed with univentricular heart physiology (UVHP) or transposition of the great arteries (TGA) after neonatal cardiac surgery. Methods: An exploratory online survey was performed with patients registered with the National Register for Congenital Heart Defects in Germany. For this publication, a subgroup analysis was conducted among patients diagnosed with TGA (n = 173; 36.3%) and UVHP (n = 304; 63.7%). Results: Median age of the sample at school enrollment was 6 years (range, 5-8 years). The majority were enrolled at a standard elementary school (n = 368 of 477; 77.1%), although patients with UVHP were enrolled significantly more often at a special needs school (n = 52 of 304; 17.1%, TGA patients n = 11/ of 173; 6.4%, P < .001). A total of 45.8% (n = 66 of 144) of the patients graduated with a high school diploma. A substantial number of patients had been diagnosed with behavioral or learning disorders (TGA patients n = 63 of 173 [36.4%], UVHP patients n = 148 of 304 [48.7%]) and received early supportive therapy or remedial teaching before (TGA patients n = 89 of 173 [51.4%], UVHP patients n = 209 of 304 [68.8%]) and/or during their school careers (TGA patients n = 54 of 173 [31.2%], UVHP patients n = 120 of 304 [39.5%]). Conclusions: A large proportion of patients who underwent neonatal cardiac surgery graduated with a high school diploma. These results are of great importance to congenital heart defect patients, affected families, and treating physicians. Nevertheless, study participants, especially patients with UVHP, face some academic challenges. We conclude that long-term follow-up examinations and regular developmental assessments may be beneficial. AD - C. Pfitzer, Department of Congenital Heart Disease / Pediatric Cardiology, Deutsches Herzzentrum Berlin, Augustenburger Platz 1, Berlin, Germany AU - Pfitzer, C. AU - Buchdunger, L. A. AU - Helm, P. C. AU - Blickle, M. J. AU - Rosenthal, L. M. AU - Ferentzi, H. AU - Berger, F. AU - Bauer, U. M. M. AU - Schmitt, K. R. L. DB - UEmbase DO - 10.1016/j.athoracsur.2020.07.072 KW - achievement article behavior disorder career child controlled study cyanotic heart disease female follow up Germany great vessels transposition heart single ventricle heart surgery high school human learning disorder major clinical study male newborn physician preschool child primary school surgery teaching LA - English M3 - Article in Press N1 - L2011698894 2021-04-23 PY - 2021 SN - 1552-6259 0003-4975 ST - Education of Children With Cyanotic Congenital Heart Disease After Neonatal Cardiac Surgery T2 - Annals of Thoracic Surgery TI - Education of Children With Cyanotic Congenital Heart Disease After Neonatal Cardiac Surgery UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2011698894&from=export http://dx.doi.org/10.1016/j.athoracsur.2020.07.072 ID - 4370 ER - TY - JOUR AB - Children with single ventricle congenital heart defects (SVCHD) experience a significant risk of early mortality throughout their lifespan, particularly during their first year of life. Due to the intense care needed for these children and families, pediatric palliative care (PPC) team consults should be routine; however, medical staff are often reluctant to broach the idea of PPC to families. The involvement of PPC for many carries with it an association to end-of-life (EOL) care. Setting the standard of PPC involvement from the time of admission for the first palliative surgery led to increased family support, decreased days to consult, improved acceptance and communication. The purpose of this article is to describe a quality improvement project of early integration of PPC with families of children with SVCHD. Lessons learned will be presented, including the resources needed and the barriers encountered in assimilating PPC into the standard of care for all patients with SVCHD. The single ventricle (SV) and PPC teams collaborated to enhance the support given to SV families. Education was initiated with cardiology and PPC providers to understand the goal of consistent PPC consults beginning after birth for patients with SVCHD. Parents were educated during fetal consultation regarding the involvement of the PPC team. The SV team ensured compliance with the PPC initiative by identifying eligible patients and requesting consult orders from the primary providers. PPC consultation increased significantly over the 40 month study period to nearly 100% compliance for children with SVCHD who are undergoing pre-Fontan surgery. In addition, mean days to consult decreased dramatically during the study to a current average of 3 days into the patient's hospitalization; the data likely suggest that more PPC consults were routinely ordered versus urgently placed for unexpected complications. Data indicate that patients are being followed by the PPC team at an earlier age and stage in their SV journey which allows for more opportunity to provide meaningful support to these patients and families. The early involvement of the PPC team for children with SV physiology was operationally feasible and was accepted by families, thus allowing PPC providers to establish a therapeutic relationship early in the disease trajectory with the family. It allowed more continuity throughout the SV journey in a proactive fashion rather than a reactive manner. AD - Nationwide Children's Hospital, 700 Children's Dr, Columbus, OH, 43205, USA. joann.davis@nationwidechildrens.org. Nationwide Children's Hospital, 700 Children's Dr, Columbus, OH, 43205, USA. Nationwide Children's Hospital, The Ohio State School of Medicine, 700 Children's Dr, Columbus, OH, 43205, USA. AN - 31676955 AU - Davis, J. A. M. AU - Bass, A. AU - Humphrey, L. AU - Texter, K. AU - Garee, A. DA - Jan DB - UPubmed DO - 10.1007/s00246-019-02231-y DP - NLM ET - 2019/11/05 IS - 1 KW - Female Humans Infant Infant, Newborn Male Palliative Care/*methods/psychology Parents/*psychology Patient Care Team/organization & administration *Professional-Family Relations Quality Improvement Referral and Consultation/statistics & numerical data Retrospective Studies Time Factors Univentricular Heart/mortality/*therapy Cardiac Collaboration End-of-life Palliative Pediatric Single ventricle LA - eng N1 - 1432-1971 Davis, Jo Ann M Orcid: 0000-0002-9609-8262 Bass, Alice Humphrey, Lisa Texter, Karen Garee, Amy Journal Article United States Pediatr Cardiol. 2020 Jan;41(1):114-122. doi: 10.1007/s00246-019-02231-y. Epub 2019 Nov 1. PY - 2020 SN - 0172-0643 SP - 114-122 ST - Early Integration of Palliative Care in Families of Children with Single Ventricle Congenital Heart Defects: A Quality Improvement Project to Enhance Family Support T2 - Pediatr Cardiol TI - Early Integration of Palliative Care in Families of Children with Single Ventricle Congenital Heart Defects: A Quality Improvement Project to Enhance Family Support VL - 41 ID - 2392 ER - TY - JOUR AB - Background The reported incidence of necrotising enterocolitis in neonates with complex CHD with ductus-dependent systemic circulation ranges from 6.8 to 13% despite surgical treatment; the overall mortality is between 25 and 97%. The incidence of gastrointestinal complications after hybrid palliation for neonates with ductus-dependent systemic circulation still has to be defined, but seems comparable with that following the Norwood procedure. Methods We reviewed the incidence of gastrointestinal complications in a series of 42 consecutive neonates with ductus-dependent systemic circulation, who received early hybrid palliation associated with a standardised feeding protocol. Results The median age and birth weight at the time of surgery were 3 days (with a range from 1 to 10 days) and 3.07 kg (with a range from 1.5 to 4.5 kg), respectively. The median ICU length of stay was 7 days (1-70 days), and the median hospital length of stay was 16 days (6-70 days). The median duration of mechanical ventilation was 3 days. Hospital mortality was 16% (7/42). In the postoperative period, 26% of patients were subjected to early extubation, and all of them received treatment with systemic vasodilatory agents. Feeding was started 6 hours after extubation according to a dedicated feeding protocol. After treatment, none of our patients experienced any grade of necrotising enterocolitis or major gastrointestinal adverse events. Conclusions Our experience indicates that the combination of an early hybrid approach, systemic vasodilator therapy, and dedicated feeding protocol adherence could reduce the incidence of gastrointestinal complications in this group of neonates. Fast weaning from ventilatory support, which represents a part of our treatment strategy, could be associated with low incidence of necrotising enterocolitis. AD - L. Manuri, Cardiovascular Department, Mediterranean Pediatric Cardiology Center, Bambino Gesù Children's Hospital, Contrada Sirina, Taormina, Italy AU - Manuri, L. AU - Morelli, S. AU - Agati, S. AU - Saitta, M. B. AU - Oreto, L. AU - Mandraffino, G. AU - Iannace, E. AU - Iorio, F. S. AU - Guccione, P. DB - OEmbase DO - 10.1017/S1047951116000275 IS - 1 KW - amrinone dobutamine dopamine epinephrine milrinone noradrenalin prostaglandin algorithm article artificial ventilation birth weight clinical article disease severity drug dose reduction ductus dependent systemic circulation early hybrid approach enteric feeding extubation female hospital mortality human incidence length of stay male necrotizing enterocolitis neonatal intensive care unit newborn Norwood procedure palliative therapy patent ductus arteriosus postoperative period protocol compliance risk reduction standardization systemic circulation systemic therapy ventilator weaning LA - English M3 - Article N1 - L614784771 2017-03-17 2017-04-11 PY - 2017 SN - 1467-1107 1047-9511 SP - 154-160 ST - Early hybrid approach and enteral feeding algorithm could reduce the incidence of necrotising enterocolitis in neonates with ductus-dependent systemic circulation T2 - Cardiology in the Young TI - Early hybrid approach and enteral feeding algorithm could reduce the incidence of necrotising enterocolitis in neonates with ductus-dependent systemic circulation UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L614784771 http://dx.doi.org/10.1017/S1047951116000275 VL - 27 ID - 3691 ER - TY - JOUR AB - Objective To assess the atrial and ventricular diastolic function response to dobutamine stress in Fontan patients, and to relate these measurements to exercise capacity and events during the follow-up. Methods We performed a secondary analysis of a cross-sectional multicentre study of Fontan patients with intra-atrial lateral tunnel (ILT) or extracardiac conduit (ECC) modification. Subjects underwent cardiac MRI during rest and low-dose dobutamine stress, and cardiopulmonary exercise testing. Atrial and diastolic ventricular function parameters were derived from volume-time curves. Medical records were abstracted for a composite end-point of death, listing for transplant, arrhythmia and reintervention. Spearman's r correlation tests and Cox proportional hazards models were used to assess the relation between the dobutamine response for atrial and diastolic ventricular function and outcomes, including exercise capacity. Results We included 57 patients (26 ECC; 31 ILT) aged 12.8 (IQR (10.3-15.5)) years. During dobutamine stress atrial cyclic volume change increased (3.0 (0.4-5.9) mL/m 2, p<0.001), as did early (1.9 (-1.6 to 3.6) mL/m 2, p=0.001) and late emptying volume (2.2 (0.2-4.4) mL/m 2, p<0.001). Ventricular early filling decreased (-1.6 (-5.7 to 0.7) mL/m 2,p=0.046) and ventricular late filling increased (1.0 (-0.4 to 3.4) mL/m 2,p<0.001) while stroke volume remained similar. Only for patients with the ECC modification, atrial early emptying volume increase correlated with peak oxygen uptake (ρ=0.66,p=0.002). No other parameter related to exercise capacity. During a median 7.1-year follow-up, 22 patients reached the composite endpoint. No parameter predicted events during the follow-up. Conclusions Dobutamine stress augmented atrial reservoir and pump function for Fontan patients. Atrial early emptying reserve related to exercise capacity in ECC patients. No other atrial or diastolic ventricular function parameter related to outcomes. AD - W.A. Helbing, Paediatric Cardiology and Radiology, Erasmus MC Sophia Children Hospital, Rotterdam, Zuid-Holland, Netherlands AU - Van Der Ven, J. P. G. AU - Bossers, S. S. M. AU - Van Den Bosch, E. AU - Dam, N. AU - Kuipers, I. M. AU - Van Iperen, G. G. AU - Kroft, L. J. M. AU - Kapusta, L. AU - Ten Harkel, A. D. J. AU - Helbing, W. A. DB - UEmbase DO - 10.1136/openhrt-2020-001487 IS - 1 KW - article cardiovascular magnetic resonance child congenital heart malformation controlled study exercise test female follow up Fontan procedure heart arrhythmia heart atrium heart stroke volume heart ventricle function human low drug dose major clinical study male medical record multicenter study peak oxygen uptake school child secondary analysis surgery transplantation dobutamine LA - English M3 - Article N1 - L634475745 2021-03-23 PY - 2021 SN - 2053-3624 2398-595X ST - Dobutamine stress testing for the evaluation of atrial and diastolic ventricular function in Fontan patients T2 - Open Heart TI - Dobutamine stress testing for the evaluation of atrial and diastolic ventricular function in Fontan patients UR - https://www.embase.com/search/results?subaction=viewrecord&id=L634475745&from=export http://dx.doi.org/10.1136/openhrt-2020-001487 VL - 8 ID - 4346 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 - OEmbase 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 - 3485 ER - TY - JOUR AB - Objective: To develop a risk score to predict mortality or transplant in the interstage period. Background: The “interstage” period between the stage 1 and stage 2 palliation is a time of high morbidity and mortality for infants with single-ventricle congenital heart disease. Methods: This was an analysis of patients with single-ventricle congenital heart disease requiring arch reconstruction who were enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry from 2008 to 2015. The primary composite endpoint was interstage mortality or transplant. Multivariable logistic regression and classification and regression tree analysis were performed on two-thirds of the patients (“learning cohort”) to build a risk score for the composite endpoint, that was validated in the remaining patients (“validation cohort”). Results: In the 2128 patients analyzed in the registry, the overall event rate was 9% (153 [7%] deaths, 42 [2%] transplants). In the learning cohort, factors independently associated with the composite endpoint were (1) type of Norwood; (2) postoperative ECMO; (3) discharge with Opiates; (4) No Digoxin at discharge; (5) postoperative Arch obstruction, (6) moderate-to-severe Tricuspid regurgitation without an oxygen requirement, and (7) Extra Oxygen required at discharge in patients with moderate-to-severe tricuspid regurgitation. This model was used to create a weighted risk score (“NEONATE” score; 0-76 points), with >75% accuracy in the learning and validation cohorts. In the validation cohort, the event rate in patients with a score >17 was nearly three times those with a score ≤17. Conclusions: We introduce a risk score that can be used post-stage 1 palliation to predict freedom from interstage mortality or transplant. AD - D.W. Brown, Department of Cardiology, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, United States AU - Ahmed, H. AU - Anderson, J. B. AU - Bates, K. E. AU - Fleishman, C. E. AU - Natarajan, S. AU - Ghanayem, N. S. AU - Sleeper, L. A. AU - Lannon, C. M. AU - Brown, D. W. DB - UEmbase DO - 10.1016/j.jtcvs.2019.11.001 IS - 4 KW - acetylsalicylic acid antiarrhythmic agent benzodiazepine derivative beta adrenergic receptor blocking agent clonidine clopidogrel digoxin dipeptidyl carboxypeptidase inhibitor diuretic agent enoxaparin opiate spironolactone aortic arch surgery article birth weight cohort analysis controlled study disease registry disease severity echocardiography extracorporeal oxygenation female follow up gestational age heart right ventricle double outlet heart single ventricle human hypoplastic left heart syndrome major clinical study male morbidity mortality rate oxygen consumption palliative therapy personal experience priority journal prospective study quality of life right ventricle to pulmonary artery conduit scoring system systolic dysfunction tricuspid valve regurgitation LA - English M3 - Article N1 - L2004522069 2020-01-14 PY - 2020 SN - 1097-685X 0022-5223 SP - 1021-1030 ST - Development of a validated risk score for interstage death or transplant after stage I palliation for single-ventricle heart disease T2 - Journal of Thoracic and Cardiovascular Surgery TI - Development of a validated risk score for interstage death or transplant after stage I palliation for single-ventricle heart disease UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2004522069&from=export http://dx.doi.org/10.1016/j.jtcvs.2019.11.001 VL - 160 ID - 4387 ER - TY - JOUR AB - AIM: To explore parents' experience of transition in the period between the palliative cardiac surgeries (i.e., the interstage period) of an infant with single ventricle congenital heart disease. DESIGN: We conducted an exploratory naturalistic inquiry using a qualitative descriptive approach. METHODS: A purposive sample of 11 parents of children with single ventricle disease was selected among families that participated in an interstage-period parental home monitoring program during the past 2 years. Interviews and field observations were conducted September-October 2019, and data were analyzed for themes. Analysis of data was inductive, although study questions and the interpretation of results were informed by Meleis' transition theory. RESULTS: Parents described the experience of transition in interstage as a striving for normality, a theme that was clustered in subthemes of home, self, and infant. CONCLUSION: Parents' experiences of striving for normality indicated a need for more targeted efforts to address parents' psychosocial needs during the highly stressful interstage transition. IMPACT: This research underscored the complexity of parents' psychosocial support needs on returning home after their child's first palliative surgery. The findings also suggest need for examination of the transition following the second palliative heart surgery, when the home monitoring program is withdrawn. Understanding parent needs will help guide healthcare teams in developing ways to support parents as they adjust to home, self, and child. AD - University of Missouri-Kansas City, Kansas City, Missouri, USA. Children's Mercy Hospital, Kansas City, Missouri, USA. AN - 33591609 AU - Elliott, M. AU - Erickson, L. AU - Russell, C. L. AU - Chrisman, M. AU - Gross Toalson, J. AU - Emerson, A. DA - May DB - UPubmed DO - 10.1111/jan.14785 DP - NLM ET - 2021/02/17 IS - 5 KW - adherence congenital heart disease home monitoring program infant mHealth nursing stress parent experience single ventricle interstage transition to home LA - eng N1 - 1365-2648 Elliott, Melissa Orcid: 0000-0002-9125-1395 Erickson, Lori Russell, Cynthia L Chrisman, Matthew Gross Toalson, Jami Emerson, Amanda American Nurse Credentialing Center/ Journal Article England J Adv Nurs. 2021 May;77(5):2437-2446. doi: 10.1111/jan.14785. Epub 2021 Feb 16. PY - 2021 SN - 0309-2402 SP - 2437-2446 ST - Defining a new normal: A qualitative exploration of the parent experience during the single ventricle congenital heart disease interstage period T2 - J Adv Nurs TI - Defining a new normal: A qualitative exploration of the parent experience during the single ventricle congenital heart disease interstage period VL - 77 ID - 2342 ER - TY - JOUR AB - Background: Congenital anomalies (CAs) are a major cause of infant morbidity and mortality in Canada. Reliably identifying CAs is essential for CA surveillance and research. The main objective of this study was to assess the agreement of eight sentinel anomalies including: neural tube defects (NTD), orofacial clefts, limb deficiency defects (LDD), Down syndrome (DS), tetralogy of Fallot (TOF), gastroschisis (GS), hypoplastic left heart syndrome (HLHS) and transposition of great vessels (TGA) captured in the BORN Information System (BIS) database and the Canadian Institute for Health Information (CIHI) Discharge Abstract Database (DAD). Methods: Live birth and stillbirth records between the BIS and CIHI-DAD in the fiscal years of 2012–2013 to 2015–2016 were linked using 10 digit infant Ontario Health Insurance Plan (OHIP) numbers. Percent agreement and Kappa statistics were performed to assess the reliability (agreement) of CAs identified in the linked BIS and CIHI-DAD birth records. Then, further investigations were conducted on those CA cases identified in the CIHI-DAD only. Results: Kappa coefficients of the eight selected CAs between BIS (“Confirmed” or “Suspected” cases) and CIHI-DAD were 0.96 (95% CI: 0.93–0.98) for GS; 0.81 (95% CI: 0.78–0.83) for Orofacial clefts; 0.75 (95% CI: 0.72–0.77) for DS; 0.71 (95% CI: 0.65–0.77) for TOF; 0.62 (95% CI: 0.55–0.68) for TGA; 0.59 (95% CI: 0.49–0.68) for HLHS, 0.53 (95% CI: 0.46–0.60) for NTD-all; and 0.30 (95% CI: 0.23–0.37) for LDD. Conclusions: The degree of agreement varied among sentinel CAs identified between the BIS and CIHI. The potential reasons for discrepancies include incompleteness of capturing CAs using existing picklist values, especially for certain sub-types, incomplete neonatal special care data in the BIS, and differences between clinical diagnosis in the BIS and ICD-10-CA classification in the DAD. A future data abstraction study will be conducted to investigate the potential reasons for discrepancies of CA capture between two databases. This project helps quantify the quality of CA data collection in the BIS, enhances understanding of CA prevalence in Ontario and provides direction for future data quality improvement activities. AD - Q. Miao, The Better Outcomes Registry Network (BORN) Ontario, Ottawa, ON, Canada Q. Miao, Children's Hospital of Eastern Ontario (CHEO) Research Institute, Ottawa, ON, Canada Q. Miao, School of Epidemiology and Public Health, University of Ottawa, Ottawa, ON, Canada AU - Miao, Q. AU - Moore, A. M. AU - Dougan, S. D. DB - UEmbase DO - 10.3389/fped.2020.573090 KW - article cleft face clinical assessment congenital malformation data quality assessment Down syndrome Fallot tetralogy fetus gastroschisis gestational age great vessels transposition human hypoplastic left heart syndrome ICD-10 infant kappa statistics limb defect live birth neural tube defect Ontario prevalence stillbirth LA - English M3 - Article N1 - L633566202 2020-12-11 2021-04-13 PY - 2020 SN - 2296-2360 ST - Data Quality Assessment on Congenital Anomalies in Ontario, Canada T2 - Frontiers in Pediatrics TI - Data Quality Assessment on Congenital Anomalies in Ontario, Canada UR - https://www.embase.com/search/results?subaction=viewrecord&id=L633566202&from=export http://dx.doi.org/10.3389/fped.2020.573090 VL - 8 ID - 4382 ER - TY - JOUR AB - We report a case of two-month old with a functionally univentricular heart and parallel circulation who presented to the emergency department with Covid-19 and subsequently developed acute respiratory distress syndrome. The course of illness, clinical values, and laboratory markers are characterized in this report. AU - Loomba, R. S. AU - Lee, B. AU - Phillips, M. AU - Vricella, L. AU - Wong, J. DB - UEmbase DO - 10.1017/S1047951120004837 KW - adult respiratory distress syndrome article case report clinical article coronavirus disease 2019 emergency ward female heart single ventricle human infant male systemic pulmonary shunt LA - English M3 - Article in Press N1 - L633764659 2020-12-31 PY - 2020 SN - 1467-1107 1047-9511 ST - Covid-19 in an infant with systemic to pulmonary artery shunt dependent functionally univentricular physiology T2 - Cardiology in the Young TI - Covid-19 in an infant with systemic to pulmonary artery shunt dependent functionally univentricular physiology UR - https://www.embase.com/search/results?subaction=viewrecord&id=L633764659&from=export http://dx.doi.org/10.1017/S1047951120004837 ID - 4454 ER - TY - JOUR AD - Division of Congenital Heart Surgery, Hospital Beneficência Portuguesa de São Paulo, São Paulo, Brazil Heart Institute (InCor), Hospital das Clinicas da Faculdade de Medicina da Universidade de São Paulo, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, Brazil Division of Cardiothoracic Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pa, United States AU - Bezerra, R. F. AU - Franchi, S. M. AU - Khader, H. AU - Castro, R. M. AU - Liguori, G. R. AU - da Fonseca da Silva, L. AU - Pedro da Silva, J. DB - UScopus DO - 10.1016/j.jtcvs.2020.05.081 IS - 2 M3 - Article N1 - Cited By :3 Export Date: 10 May 2021 PY - 2021 SP - e97-e101 ST - COVID-19 as a confounding factor in a child submitted to staged surgical palliation of hypoplastic left heart syndrome: One of the first reports of SARS-CoV-2 infection in patients with congenital heart disease T2 - Journal of Thoracic and Cardiovascular Surgery TI - COVID-19 as a confounding factor in a child submitted to staged surgical palliation of hypoplastic left heart syndrome: One of the first reports of SARS-CoV-2 infection in patients with congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85087421701&doi=10.1016%2fj.jtcvs.2020.05.081&partnerID=40&md5=a2b73e9afb7987b1175753da98838a3f VL - 161 ID - 5867 ER - TY - JOUR AB - Contrast-induced encephalopathy (CIE) is a well-known but rare complication following contrast media administration. Its nonspecific clinical manifestations hinder diagnosis, particularly in the pediatric population. The majority of cases are reversible, with clinical improvement and resolution of signs noted on diagnostic imaging. Here, we report the case of a 2-month-old patient with a history of complex cardiovascular disease who presented with a single episode of seizure after undergoing cardiac catheterization with nonionic iodinated contrast media. CIE is diagnosed based on the signs and symptoms exhibited by the patient and the findings on plain head computed tomography (CT) scan. Subsequently, the absence of neurological symptoms and disappearance of the imaging alterations on a control CT are documented. AD - O. Gonzalez-Pardo, Fundación Clínica Shaio, Dg 115 A #70C-75, Bogota, Colombia, Colombia AU - Gonzalez-Pardo, O. AU - Ordoñez, A. AU - Roa, C. DB - UEmbase DO - 10.1016/j.radcr.2021.02.028 IS - 5 KW - furosemide iopromide spironolactone article body weight brain disease cardiopulmonary insufficiency case report cava vein clinical article clonic seizure computer assisted tomography congenital heart disease contrast induced encephalopathy cyanosis differential diagnosis drug induced disease female frontal lobe good general condition great vessels transposition heart atrium septum defect heart catheterization heart massage heart right ventricle double outlet heart single ventricle hemodynamics human infant lung artery banding occipital lobe parietal lobe pediatric cardiology physical examination priority journal sucking surgical technique symptom LA - English M3 - Article N1 - L2011125729 2021-03-02 2021-05-04 PY - 2021 SN - 1930-0433 SP - 1065-1067 ST - Contrast-induced encephalopathy in an infant T2 - Radiology Case Reports TI - Contrast-induced encephalopathy in an infant UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2011125729&from=export http://dx.doi.org/10.1016/j.radcr.2021.02.028 VL - 16 ID - 4342 ER - TY - JOUR AD - J.Y. Zheng, Department of Cardiology, Sixth Medical Center of Pla General Hospital, Beijing, China AU - Qiu, Y. G. AU - Zheng, J. Y. AU - Han, L. AU - Ding, W. H. AU - Li, T. C. AU - Zhao, J. H. DB - UEmbase DO - 10.14744/AnatolJCardiol.2020.03162 IS - 2 KW - beta adrenergic receptor blocking agent adult artery occlusion article atresia cardiovascular magnetic resonance case report child claustrophobia clinical article collateral artery computed tomographic angiography congenital heart disease congestive cardiomyopathy coronary angiography dyspnea echocardiography electrocardiogram female heart failure heart left ventricle ejection fraction heart muscle fibrosis human hypoplastic left heart syndrome male mitral valve regurgitation Q wave ST segment depression systolic heart murmur tissue Doppler imaging ventricular noncompaction LA - English M3 - Article N1 - L2011507802 2021-04-01 2021-05-03 PY - 2021 SN - 2149-2271 2149-2263 SP - 143-147 ST - Congenital atresia of the left main coronary artery with left ventricular noncompaction: From infancy to adulthood T2 - Anatolian Journal of Cardiology TI - Congenital atresia of the left main coronary artery with left ventricular noncompaction: From infancy to adulthood UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2011507802&from=export http://dx.doi.org/10.14744/AnatolJCardiol.2020.03162 VL - 25 ID - 4357 ER - TY - JOUR AB - The use of mechanical circulatory support for failing Fontan patients is an area of growing interest, as the increased life expectancy of these patients continues to be accompanied by numerous end-organ complications. In vitro work has shown positive results using the CentriMag device for right-sided Fontan support, however the generalizability across various patient anatomies and cannulations is unknown. Computational simulations are first validated against in vitro modeling, then used to assess generalizability and further explore hemodynamic metrics including relative pressure changes, hepatic flow distribution, wall shear stress and power added. Computational modeling matched previous in vitro work very well, with vessel flow rates and relative average pressure change each within 1%. Positive results were seen across all patient anatomies and cannulations. On average, pressure from the vena cava to pulmonary arteries increased by 5.4 mmHg corresponding to 32 mW of power added. Hepatic flow distribution and wall shear stress were within acceptable ranges, with an average hepatic flow distribution of 47% and all patients showing ≤ 1% of the total Fontan connection surface area at a wall shear stress above 150 Pa. The positive results previously seen using CentriMag as a right-sided Fontan support device were found to be repeatable across multiple patient anatomies and cannulations. While animal models and eventual patient studies will provide further insight into the efficacy of this support strategy, our findings here suggest this method may reproduce right heart function. AD - A.P. Yoganathan, 387 Technology Cir NW Suite 200, Atlanta, GA, United States AU - Trusty, P. M. AU - Alan Wei, Z. AU - Fogel, M. A. AU - Maher, K. AU - Deshpande, S. R. AU - Yoganathan, A. P. DB - UEmbase DO - 10.1016/j.jbiomech.2020.109917 KW - centrifugal blood pump ventricular assist device adolescent adult anatomical variation article blood flow velocity blood pressure cannulation cava vein child clinical article computer model female Fontan procedure hemodynamics human in vitro study liver blood flow male preschool child priority journal pulmonary artery right sided Fontan assist device shear stress young adult CentriMag LA - English M3 - Article N1 - L2007042855 2020-07-23 2020-07-24 PY - 2020 SN - 1873-2380 0021-9290 ST - Computational modeling of a right-sided Fontan assist device: Effectiveness across patient anatomies and cannulations T2 - Journal of Biomechanics TI - Computational modeling of a right-sided Fontan assist device: Effectiveness across patient anatomies and cannulations UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2007042855&from=export http://dx.doi.org/10.1016/j.jbiomech.2020.109917 VL - 109 ID - 4398 ER - TY - JOUR AB - In univentricular (Fontan) physiology, peripheral and splanchnic vascular tone may be raised to counteract reduced cardiac output (CO) and elevated central venous pressure and thus maintain vital organ perfusion. This could negatively affect the normal cardiovascular response to food ingestion, where mesenteric vasodilation and a concurrent rise in CO are central. We sought to elucidate this using rapid cardiovascular MRI. Thirty fasting subjects (50% controls, 40% women and 60% men) ingested a standardized meal. Responses over ~50 min in mean arterial pressure (MAP), CO, and blood flow in all major aortic branches were measured, and regional vascular impedance (Z(0)) was calculated. Differences from baseline and between groups were assessed by repeated-measures mixed models. Compared with the control group, the Fontan patient group had greater fasting Z(0) of the legs and kidneys, resulting in greater systemic Z(0) and similar MAP. They further had similar blood flow to the digestive organs at baseline, despite larger variation in mesenteric resistance. Postprandially, blood flow to the legs decreased in the control group but not in the Fontan patient group. Increases in CO and superior mesenteric blood flow were similar in both groups, but the celiac response was blunted in the Fontan patient group. No significant differences in MAP responses were observed. In conclusion, alterations in vascular tone to counteract adverse hemodynamics and raised hepatic afterload may blunt vasoreactivity in the legs and the celiac axis in Fontan physiology. Further study is needed to determine whether blunted celiac or mesenteric vasoreactivity is linked to deteriorating hemodynamics and poor prognosis in Fontan patients.NEW & NOTEWORTHY Novel data on cardiovascular physiology in response to a meal in Fontan patients are presented. Using a previously validated dynamic MRI protocol, we demonstrated that the usual increase in cardiac output and the dilation of the superior mesenteric artery are preserved in clinically well Fontan patients. In contrast, vasoconstriction of the legs may have prevented redistribution of blood flow from this region in response to the meal. This may also affect responses to other types of stress. Celiac vasodilation was also absent in Fontan patients. This may be due to abnormal hepatic circulation. The proposed protocol may be used to study Fontan complications secondary to abnormal regional hemodynamics. AD - Centre for Translational Cardiovascular Imaging, University College London, London, United Kingdom. Division of Pediatric Cardiology, Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria. Department of Pediatrics, University of Oxford, Oxford, United Kingdom. Barts Heart Centre, London, United Kingdom. Great Ormond Street Hospital for Children National Health Service Foundation Trust, London, United Kingdom. AN - 32857602 AU - Hauser, J. A. AU - Jones, A. AU - Pandya, B. AU - Taylor, A. M. AU - Muthurangu, V. C2 - PMC7654659 DA - Oct 1 DB - UPubmed DO - 10.1152/ajpheart.00500.2020 DP - NLM ET - 2020/08/29 IS - 4 KW - Adolescent Adult Case-Control Studies *Eating Female *Fontan Procedure Heart Defects, Congenital/diagnostic imaging/physiopathology/*surgery *Hemodynamics Humans *Magnetic Resonance Imaging Male Postprandial Period Predictive Value of Tests *Splanchnic Circulation Time Factors Treatment Outcome Young Adult *Fontan *blood flow *magnetic resonance imagine *postprandial *vascular physiology LA - eng N1 - 1522-1539 Hauser, Jakob A Orcid: 0000-0002-9613-7284 Jones, Alexander Pandya, Bejal Taylor, Andrew M Muthurangu, Vivek NH/18/1/33511/BHF_/British Heart Foundation/United Kingdom PG/17/47/32963/BHF_/British Heart Foundation/United Kingdom FS/18/22/33479/British Heart Foundation (BHF)/International Journal Article Research Support, Non-U.S. Gov't Am J Physiol Heart Circ Physiol. 2020 Oct 1;319(4):H808-H813. doi: 10.1152/ajpheart.00500.2020. Epub 2020 Aug 28. PY - 2020 SN - 0363-6135 (Print) 0363-6135 SP - H808-h813 ST - Comprehensive MRI assessment of the cardiovascular responses to food ingestion in Fontan physiology T2 - Am J Physiol Heart Circ Physiol TI - Comprehensive MRI assessment of the cardiovascular responses to food ingestion in Fontan physiology VL - 319 ID - 2355 ER - TY - JOUR AB - The chromosomal region critical in Down syndrome has long been analyzed through genotype–phenotype correlation studies using data from many patients with partial trisomy 21. Owing to that, a relatively small region of human chromosome 21 (35.9 ~ 38.0 Mb) has been considered as Down syndrome critical region (DSCR). In this study, microarray-based comparative genomic hybridization analysis identified complex rearrangements of chromosome 21 in a patient manifesting clinical features partially overlapped with that of Down syndrome. Although the patient did not show up-slanting palpebral fissures and single transverse palmar creases, other symptoms were consistent with Down syndrome. Rearrangements were analyzed by whole-genome sequencing using Nanopore long-read sequencing. The analysis revealed that chromosome 21 was fragmented into seven segments and reassembled by six connected points. Among 12 breakpoints, 5 are located within the short region and overlapped with repeated segments. The rearrangement resulted in a maximum gain of five copies, but no region showed loss of genomic copy numbers. Breakpoint-junctions showed no homologous region. Based on these findings, chromoanasynthesis was considered as the mechanism. Although the distal 21q22.13 region was not included in the aberrant regions, some of the genes located on the duplicated regions, SOD1, SON, ITSN1, RCAN1, and RUNX1, were considered as possible candidate genes for clinical features of the patient. We discussed the critical region for Down syndrome, with the literature review. AD - T. Yamamoto, Institute of Medical Genetics, Tokyo Women’s Medical University, 8-1 Kawada-cho, Shinjuku-ward, Tokyo, Japan AU - Imaizumi, T. AU - Yamamoto-Shimojima, K. AU - Yanagishita, T. AU - Ondo, Y. AU - Nishi, E. AU - Okamoto, N. AU - Yamamoto, T. DB - UEmbase DO - 10.1007/s00439-020-02196-6 IS - 12 KW - carbonyl reductase copper zinc superoxide dismutase DNA binding protein DNA binding protein SON genomic DNA intersectin intersectin 1 peptides and proteins regulator of calcineurin 1 transcription factor RUNX1 unclassified drug article case report child chromosome 21 chromosome 21q chromosome analysis chromosome duplication chromosome rearrangement clinical article clinical feature comparative genomic hybridization Down syndrome droplet digital polymerase chain reaction echocardiography face malformation female finger malformation gene location genotype phenotype correlation heart atrium septum defect high arched palate human human cell hypertelorism hypoplastic left heart syndrome leg malformation microarray analysis nanopore sequencing nose malformation partial trisomy persistent left superior vena cava preschool child priority journal pulmonary hypertension Sanger sequencing whole genome sequencing LA - English M3 - Article N1 - L2005219128 2020-06-30 2020-11-24 PY - 2020 SN - 1432-1203 0340-6717 SP - 1555-1563 ST - Complex chromosomal rearrangements of human chromosome 21 in a patient manifesting clinical features partially overlapped with that of Down syndrome T2 - Human Genetics TI - Complex chromosomal rearrangements of human chromosome 21 in a patient manifesting clinical features partially overlapped with that of Down syndrome UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2005219128&from=export http://dx.doi.org/10.1007/s00439-020-02196-6 VL - 139 ID - 4376 ER - TY - JOUR AB - Multiple studies have shown that quantitative evaluation of right ventricular (RV) systolic function in children with hypoplastic left heart syndrome (HLHS) is associated with outcomes. However, the most widely used method is qualitative, or subjective echocardiographic evaluation. Tricuspid annular displacement (TMAD) is a quantitative method and has been shown to be associated with transplant/death in a cohort of pediatric patients with HLHS. In this study, the same echocardiograms used in the quantitative TMAD study were qualitatively evaluated to see if the assessment correlated with midterm outcomes. We hypothesized that TMAD measures would outperform qualitative measurements. A previously published retrospective study of patients with systemic right ventricle demonstrated that TMAD measurements of function prior to the Glenn procedure were associated with midterm mortality/transplant (mean TMAD 12.4% for survivors and 10.0% for non-survivors/transplant, p = 0.03). Echocardiographic images used in that study were re-evaluated using qualitative assessment of function. A score between severely depressed (0) and supra-normal (7) was assigned independently by three cardiologists. A chart review included short-term clinical outcome measures in addition to midterm mortality/transplant. Spearman correlations and logistic regression were used to estimate the associations between function scores and clinical outcomes. Function scores were measured with acceptable inter- and intra-rater reliabilities in 47 patients who had echocardiograms used in the prior analysis before their bidirectional Glenn. The mean functional score was 5.3 ± 1.32 in survivors and 5.0 ± 1.02 in non-survivors/transplant. Subjective echocardiographic scores of function were not predictive of mortality (p = 0.23). Scores were correlated with length of cardiac intensive care unit stay (Spearman's rho =  - 0.31, p = 0.04), but not post-Glenn total length of hospital stay (p = 0.4). A sub-analysis was performed for each individual operator. Only one of three operators produced scores with a significant association with mortality/transplant (p = 0.01, p = 0.25, p = 0.22, respectively). Averaged subjective measurement of function by three pediatric cardiologists in children with single right ventricle prior to the Glenn procedure was not associated with midterm outcomes. Previous work based on the same echocardiograms showed that quantitative analysis was associated with midterm outcomes. This suggests quantitative analysis may be more useful in prognostication. Future studies could confirm these results and identify which quantitative methods are most helpful. AD - Department of Pediatric Cardiology, Advocate Children's Hospital, Oaklawn, IL, USA. Department of Pediatric Cardiology, Carle BroMenn Medical Center, Normal, IL, USA. Department of Pediatric Cardiology, Advocate Children's Hospital, Oaklawn, IL, USA. eleanor.ross@aah.org. Advocate Aurora Research Institute, Chicago, IL, USA. Department of Pediatric Cardiology, Lurie Children's Hospital, Chicago, IL, USA. AN - 33517466 AU - Zaidi, S. J. AU - Ross, E. AU - Gandhi, R. AU - Li, Y. AU - Penk, J. DA - Jan 31 DB - UPubmed DO - 10.1007/s00246-021-02539-8 DP - NLM ET - 2021/02/01 KW - Bidirectional Glenn Congenital heart disease Fontan Hypoplastic left heart Norwood Qualitative echo Stage II LA - eng N1 - 1432-1971 Zaidi, S Javed Orcid: 0000-0003-4939-0073 Ross, Eleanor Gandhi, Rupali Li, Yi Penk, Jamie Journal Article United States Pediatr Cardiol. 2021 Jan 31. doi: 10.1007/s00246-021-02539-8. PY - 2021 SN - 0172-0643 ST - Comparison of Quantitative Versus Qualitative Assessment of Single Right Ventricular Function for the Prediction of Midterm Outcomes T2 - Pediatr Cardiol TI - Comparison of Quantitative Versus Qualitative Assessment of Single Right Ventricular Function for the Prediction of Midterm Outcomes ID - 2344 ER - TY - JOUR AB - Commentary on the original paper entitled “Can a home-based cardiac physical activity program improve and sustain quality of life and exercise capacity in children with Fontan circulation?” AD - A. Callegari, University Children's Hospital, Pediatric Heart Center, Department of Surgery, Steinwiesstrasse 75, Zurich, Switzerland AU - Callegari, A. AU - Christmann, M. DB - UEmbase DO - 10.1016/j.ppedcard.2018.11.005 KW - article child exercise heart human physical activity quality of life respiratory function LA - English M3 - Note N1 - L2001460142 2019-01-23 PY - 2019 SN - 1558-1519 1058-9813 SP - 1-2 ST - Comment on the original paper entitled “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 - Comment on the original paper entitled “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.embase.com/search/results?subaction=viewrecord&id=L2001460142&from=export http://dx.doi.org/10.1016/j.ppedcard.2018.11.005 VL - 52 ID - 4499 ER - TY - JOUR AB - Neurodevelopmental disabilities are common sequelae of congenital heart disease, particularly cognitive impairments that impede academic achievement. Cognitive training has been shown to be effective in improving working memory in other patient groups. Computerized cognitive training programs may hold potential for children and adolescents with congenital heart disease, but are untested in this population. In this single-center, single-blinded, randomized controlled trial, the feasibility and efficacy of Cogmed Working Memory Training (Cogmed) was compared with observation in children with hypoplastic left heart syndrome 8 to 16 years of age. Participants were randomized to either Cogmed at home for 5 weeks with coaching by a study team member or an observation control group. Baseline, 6–8 weeks later (post-intervention), and 6-month assessments of working memory were completed. Participants randomized to the Cogmed intervention (n = 10) demonstrated greater improvement than controls (n = 10) on measures of working memory, the Wechsler Intelligence Scale for Children-V Working Memory Index and the National Institutes of Health Toolbox Cognitive Battery post-training that were statistically significant (Cohen's d = 0.76, p < .001 and d = 0.43, p < .01) compared to controls over the same time period. Improvements were not maintained at 6-month follow-up. Three children withdrew from cognitive training, and one additional child did not complete at least 20 of 25 cognitive training sessions despite coaching. In conclusion, study findings show computerized cognitive training is feasible for most children with hypoplastic left heart syndrome, but shorter sessions may improve adherence. Training-related improvements in working memory occur, though continued reinforcement may be necessary for these gains to be maintained. AD - L.C. Jordan, Department of Pediatrics, Division of Pediatric Neurology, Vanderbilt University Medical Center, 2200 Children's Way DOT 11212, Nashville, TN, United States AU - Jordan, L. C. AU - Siciliano, R. E. AU - Cole, D. A. AU - Lee, C. A. AU - Patel, N. J. AU - Murphy, L. K. AU - Markham, L. W. AU - Prussien, K. V. AU - Gindville, M. C. AU - Compas, B. E. DB - OEmbase DO - 10.1016/j.ppedcard.2019.101185 KW - adolescent article child clinical article cognitive defect comparative effectiveness complication controlled study feasibility study female follow up human hypoplastic left heart syndrome male national health organization nervous system malformation randomized controlled trial reinforcement single blind procedure Wechsler intelligence scale for children working memory LA - English M3 - Article in Press N1 - L2004191225 2019-12-20 PY - 2019 SN - 1558-1519 1058-9813 ST - Cognitive training in children with hypoplastic left heart syndrome: A pilot randomized trial T2 - Progress in Pediatric Cardiology TI - Cognitive training in children with hypoplastic left heart syndrome: A pilot randomized trial UR - http://www.embase.com/search/results?subaction=viewrecord&from=export&id=L2004191225 http://dx.doi.org/10.1016/j.ppedcard.2019.101185 ID - 3548 ER - TY - JOUR AB - OBJECTIVE: Despite surgical palliation, children with hypoplastic left heart syndrome (HLHS) have compromised cardiac functioning and increased risk for cognitive deficits. We quantitatively reviewed the empirical data from this literature. METHODS: The present meta-analysis included 13 studies reporting cognitive function for children with HLHS between the ages of 2 years and 6 months and 17 years that used standardized assessments of Full Scale IQ (FSIQ), Verbal IQ (VIQ), and Performance IQ (PIQ). Differences in cognitive function were assessed relative to normative data, and we examined sample mean age and publication year as moderators. RESULTS: Large effects were found for FSIQ (g = -.87, 95% CI [-1.10, -.65], M = 86.88) and PIQ (g = -.89, 95% CI [-1.11, -.68], M = 86.56), and a medium effect was found for VIQ (g = -.61, 95% CI [-.84, -.38], M = 90.82). All models demonstrated significant heterogeneity. Meta-regression analyses of effect size via Hedges' g on child age revealed a significant effect on FSIQ (coefficient = -.07, 95% CI [-.12, -.01], p < .01, R2 = .40) indicating a loss of 1.1 FSIQ points across studies with each increased year of mean sample age. CONCLUSIONS: Deficits in FSIQ may reflect chronic brain injury or failure to make expected gains as children age. This review highlights the importance of early intervention in this population, and the need for longitudinal studies analyzing more specific domains of cognitive function and potential moderators. AD - Department of Psychology and Human Development, Vanderbilt University. Division of Pediatric Neurology, Department of Pediatrics, Vanderbilt University Medical Center. AN - 31069392 AU - Siciliano, R. E. AU - Prussien, K. V. AU - Lee, C. A. AU - Patel, N. J. AU - Murphy, L. K. AU - Compas, B. E. AU - Jordan, L. C. C2 - PMC6705716 DA - Sep 1 DB - UPubmed DO - 10.1093/jpepsy/jsz021 DP - NLM ET - 2019/05/10 IS - 8 KW - Adolescent Child Child, Preschool Cognitive Dysfunction/etiology/*physiopathology Humans Hypoplastic Left Heart Syndrome/complications/*physiopathology *hlhs *age *cognitive deficit *congenital heart disease *meta-analysis LA - eng N1 - 1465-735x Siciliano, Rachel E Prussien, Kemar V Lee, Chelsea A Patel, Niral J Murphy, Lexa K Compas, Bruce E Jordan, Lori C T32 MH018921/MH/NIMH NIH HHS/United States U54 HD083211/HD/NICHD NIH HHS/United States Journal Article Meta-Analysis Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't Systematic Review J Pediatr Psychol. 2019 Sep 1;44(8):937-947. doi: 10.1093/jpepsy/jsz021. PY - 2019 SN - 0146-8693 (Print) 0146-8693 SP - 937-947 ST - Cognitive Function in Pediatric Hypoplastic Left Heart Syndrome: Systematic Review and Meta-Analysis T2 - J Pediatr Psychol TI - Cognitive Function in Pediatric Hypoplastic Left Heart Syndrome: Systematic Review and Meta-Analysis VL - 44 ID - 2414 ER - TY - JOUR AB - Objectives: Congenital heart defects (CHD) can be detected during routine antenatal scans or go undiagnosed until birth. Parents of children with Single Ventricle CHD (SVCHD) have a complex experience. This study aimed to explore the lived experiences of parents of children with SVCHD from diagnosis through to adolescence. Design: Interviews were conducted with 12 parents whose children had completed the Fontan procedure. Interpretative Phenomenological Analysis (IPA) identified themes across parents' accounts. Results: Data were analysed into three superordinate themes of super parents, striving for normality and accepting SVCHD and their role. Through accepting SVCHD, becoming experts, and taking responsibility for the management of their child's physical healthcare, parents developed their own sense of normality. Following distress at the point of diagnosis, parents found their own way of accepting and integrating SVCHD into family life and regained a sense of control and normality within the family. Conclusions: Transition points such as starting nursery, final surgery, puberty, and reaching adulthood are key time points when parents may struggle with their changing roles and the gradual shift of responsibility and independence onto their children. There are implications for supporting parents to educate their children in terms of the management and long-term prognosis of their condition, especially when the child with SVCHD enters puberty or moves into adult services where they gain agency of their condition and its management. The role of healthcare professionals promoting emotional wellbeing and providing support to families during significant periods is discussed. AD - A. Wittkowski, Division of Psychology & Mental Health, School of Health Sciences, Faculty of Biology, Medicine and Health, The University of Manchester and Manchester Academic Health Science Centre, Oxford Road, Manchester, United Kingdom AU - Lumsden, M. R. AU - Smith, D. M. AU - Twigg, E. AU - Guerrero, R. AU - Wittkowski, A. DB - UEmbase DO - 10.1016/j.ppedcard.2020.101297 KW - adolescent adult article child child care clinical article congenital heart disease data analysis female Fontan procedure heart single ventricle human interpretative phenomenological analysis male parent peer group personal experience phenomenology priority journal responsibility school child sibling time to treatment young adult LA - English M3 - Article N1 - L2007988129 2020-10-12 2020-11-10 PY - 2020 SN - 1558-1519 1058-9813 ST - Children with single ventricle congenital heart defects: An interpretative phenomenological analysis of the lived parent experience T2 - Progress in Pediatric Cardiology TI - Children with single ventricle congenital heart defects: An interpretative phenomenological analysis of the lived parent experience UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2007988129&from=export http://dx.doi.org/10.1016/j.ppedcard.2020.101297 VL - 59 ID - 4378 ER - TY - JOUR AB - Adolescents with single ventricle heart disease (SVHD) exhibit mood and cognitive deficits, which may result from injury to the basal ganglia structures, including the caudate nuclei. However, the integrity of the caudate in SVHD adolescents is unclear. Our aim was to examine the global and regional caudate volumes, and evaluate the relationships between caudate volumes and cognitive and mood scores in SVHD and healthy adolescents. We acquired two high-resolution T1-weighted images from 23 SVHD and 37 controls using a 3.0-Tesla MRI scanner, as well as assessed mood (Patient Health Questionnaire-9 [PHQ-9]; Beck Anxiety Inventory [BAI]) and cognition (Montreal Cognitive Assessment [MoCA]; Wide Range Assessment of Memory and Learning-2; General Memory Index [GMI]) functions. Both left and right caudate nuclei were outlined, which were then used to calculate and compare volumes between groups using ANCOVA (covariates: age, gender, and head-size), as well as perform 3D surface morphometry. Partial correlations (covariates: age, gender, and head-size) were used to examine associations between caudate volumes, cognition, and mood scores in SVHD and controls. SVHD subjects showed significantly higher PHQ-9 and BAI scores, indicating more depressive and anxiety symptoms, as well as reduced GMI scores, suggesting impaired cognition, compared to controls. SVHD patients showed significantly reduced caudate volumes (left, 3,198.8 ± 490.1 vs. 3,605.0 ± 480.4 mm(3) , p < 0.004; right, 3,162.1 ± 475.4 vs. 3,504.8 ± 465.9 mm(3) , p < 0.011) over controls, and changes were localized in the rostral, mid-dorsolateral, and caudal areas. Significant negative correlations emerged between caudate volumes with PHQ-9 and BAI scores and positive correlations with GMI and MoCA scores in SVHD and controls. SVHD adolescents show significantly reduced caudate volumes, especially in sites that have projections to regulate mood and cognition, which may result from developmental and/or hypoxia-/ischemia-induced processes. AD - Department of Anesthesiology, David Geffen School of Medicine at UCLA, University of California Los Angeles, Los Angeles, CA, USA. UCLA School of Nursing, University of California Los Angeles, Los Angeles, CA, USA. Division of Pediatric Cardiology, University of California Los Angeles, Los Angeles, CA, USA. Division of Pediatric Cardiology, Children's Hospital Los Angeles, Los Angeles, CA, USA. Department of Radiological Sciences, University of California Los Angeles, Los Angeles, CA, USA. Department of Bioengineering, University of California Los Angeles, Los Angeles, CA, USA. Brain Research Institute, University of California Los Angeles, Los Angeles, CA, USA. AN - 32530059 AU - Noorani, S. AU - Roy, B. AU - Sahib, A. K. AU - Cabrera-Mino, C. AU - Halnon, N. J. AU - Woo, M. A. AU - Lewis, A. B. AU - Pike, N. A. AU - Kumar, R. DA - Oct DB - UPubmed DO - 10.1002/jnr.24667 DP - NLM ET - 2020/06/13 IS - 10 KW - *3D surface morphometry *anxiety *basal ganglia *congenital heart disease *depression *magnetic resonance imaging LA - eng N1 - 1097-4547 Noorani, Sarah Orcid: 0000-0002-3289-4120 Roy, Bhaswati Sahib, Ashish K Cabrera-Mino, Cristina Halnon, Nancy J Woo, Mary A Lewis, Alan B Pike, Nancy A Kumar, Rajesh Orcid: 0000-0001-5355-9586 R01 NR-016463/NR/NINR NIH HHS/United States Journal Article Research Support, N.I.H., Extramural United States J Neurosci Res. 2020 Oct;98(10):1877-1888. doi: 10.1002/jnr.24667. Epub 2020 Jun 12. PY - 2020 SN - 0360-4012 SP - 1877-1888 ST - Caudate nuclei volume alterations and cognition and mood dysfunctions in adolescents with single ventricle heart disease T2 - J Neurosci Res TI - Caudate nuclei volume alterations and cognition and mood dysfunctions in adolescents with single ventricle heart disease VL - 98 ID - 2367 ER - TY - JOUR AB - Lipiodol-based lymphangiography is not only a diagnostic tool for visualization of lymphatic disorders such as plastic bronchitis (PB), but also aims a therapeutic effect by embolizing lymph leakages. We performed such percutaneous lymphatic embolization for PB in a Fontan patient with proven absence of right-to-left shunt, and demonstrated important lymphatic abnormalities in the mediastinum. Shortly after the procedure, the patient developed severe convulsive seizures, revealing multiple cerebral embolisms of Lipiodol. Radiological images were impressive, yet the clinical neurological outcome was favorable. Lipiodol-based lymphography in Fontan patients with plastic bronchitis should be avoided as this subgroup is more likely to have developed lympho-pulmonary venous connections which allow systemic emboli. AD - J. Hubrechts, Department of Pediatric and Congenital Cardiology, Leuven University Hospital, Leuven, Belgium AU - Hubrechts, J. AU - Wåhlander, H. AU - Kjellberg-Olofsson, C. AU - Maleux, G. AU - Gewillig, M. C1 - lipiodol ultrafluid(Guerbet,France) C2 - Guerbet(France) DB - UEmbase DO - 10.3389/fped.2020.584185 KW - spinal needle ultrasound transducer clonidine diazepam hypertensive factor inotropic agent iodinated poppyseed oil levetiracetam midazolam prednisone acute kidney failure aortic coarctation article artificial embolization brain embolism bronchitis case report cavopulmonary connection child chylothorax clinical article clinical outcome cone beam computed tomography dysthymia fluoroscopy Fontan procedure heart atrium septum defect heart right left shunt heart ventricle septum defect human liver dysfunction lymphangiography male mediastinum multiple organ failure neuroimaging oliguria palliative therapy plastic bronchitis pulmonary artery catheterization school child seizure spasticity susceptibility weighted imaging systemic disease unconsciousness lipiodol ultrafluid LA - English M3 - Article N1 - L633352919 2020-11-16 2020-12-29 PY - 2020 SN - 2296-2360 ST - Case Report: Disseminated Systemic Embolism of Lipiodol After Lymphography for Plastic Bronchitis After Fontan Repair T2 - Frontiers in Pediatrics TI - Case Report: Disseminated Systemic Embolism of Lipiodol After Lymphography for Plastic Bronchitis After Fontan Repair UR - https://www.embase.com/search/results?subaction=viewrecord&id=L633352919&from=export http://dx.doi.org/10.3389/fped.2020.584185 VL - 8 ID - 4386 ER - TY - JOUR AB - Background: The NONO gene is located on chromosome Xq13.1 and encodes a nuclear protein involved in RNA synthesis, transcriptional regulation, and DNA repair. Hemizygous variants in NONO have been reported to cause mental retardation, X-linked, syndromic 34 (MRXS34) in males. Due to the scarcity of clinical reports, the clinical characteristics and mutation spectrum of NONO-related disorder have not been entirely determined. Methods: We reported a fetus with hypoplastic left heart syndrome, performed a comprehensive genotyping examination, including copy-number variation sequencing and whole-exome sequencing, and screened for the genetic abnormality. We also conducted an in vitro mini-gene splicing assay to demonstrate the predicted deleterious effects of an intronic variant of NONO. Results: Exome sequencing identified a novel intronic variant (c.154 + 9A > G) in intron 4 of the NONO gene (NM_001145408.1). It was predicted to insert 4 bp of intron 4 into the mature mRNA. Minigene assay revealed that the c.154 + 9A > G variant caused the activation of the intronic cryptic splice site and 4 bp insertion (c.154_155ins GTGT) in mature mRNA. Literature review shows that cardiac phenotype, including left ventricular non-compaction cardiomyopathy and congenital heart disease, are consistent features of MRXS34. Conclusion: This study enlarges the mutation spectrum of NONO, further expands hypoplastic left heart syndrome to the phenotype of MRXS34 and points out the importance of intronic sequence analysis and the need for integrative functional studies in the interpretation of sequence variants. AD - Y. He, Beijing Anzhen Hospital, Capital Medical University, Beijing, China H. Zhang, Key Laboratory of Medical Engineering for Cardiovascular Disease, Ministry of Education, Beijing, China Y. He, Key Laboratory of Medical Engineering for Cardiovascular Disease, Ministry of Education, Beijing, China Y. He, Beijing Key Laboratory of Maternal-Fetal Medicine and Fetal Heart Disease, Beijing Anzhen Hospital, Capital Medical University, Beijing, China H. Zhang, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China AU - Sun, H. AU - Han, L. AU - Zhang, X. AU - Hao, X. AU - Zhou, X. AU - Pan, R. AU - Zhang, H. AU - He, Y. DB - UEmbase DO - 10.3389/fgene.2020.593688 KW - fetal ultrasound monitor messenger RNA adult agar gel electrophoresis aorta hypoplasia article autopsy cardiomyopathy case report chromosome aberration chromosome Xq chromosome Xq 13.1 clinical article color Doppler echocardiography computer model congenital heart disease copy number variation DNA repair DNA vector dysplasia eukaryotic cell female fetus gene gene frequency gene mutation gene sequence genetic disorder genetic transfection genetic variability genotype hemizygous variant human human tissue hypoplasia hypoplastic left heart syndrome hypoplastic left ventricle dysplasia in silico splicing analysis intron intronic variant M mode echocardiography mental deficiency mini gene splicing assay mitral valve dysplasia NONO p54nrb phenotype pulsed Doppler echocardiography real time polymerase chain reaction RNA splicing RNA synthesis sequence analysis splicing vector pEGFP C1 transcription regulation two dimensional echocardiography ultraviolet spectrophotometry ventricular noncompaction whole exome sequencing X linked syndromic 34 young adult LA - English M3 - Article N1 - L633511743 2020-12-07 2020-12-10 PY - 2020 SN - 1664-8021 ST - Case Report: Characterization of a Novel NONO Intronic Mutation in a Fetus With X-Linked Syndromic Mental Retardation-34 T2 - Frontiers in Genetics TI - Case Report: Characterization of a Novel NONO Intronic Mutation in a Fetus With X-Linked Syndromic Mental Retardation-34 UR - https://www.embase.com/search/results?subaction=viewrecord&id=L633511743&from=export http://dx.doi.org/10.3389/fgene.2020.593688 VL - 11 ID - 4383 ER - TY - CASE A4 - Abe, Jiro A2 - Takeda, Atsuhito A2 - Saitoh, Shinji AB - The article discusses the case study of male infant was diagnosed with tricuspid atresia type 1c (TA1c) and intrauterine growth restriction during the prenatal period after his mother had undergone in vitro fertilization and embryo-transfer (IVF-ET) 16 times. It mentions He needed pulmonary artery banding and patent ductus arteriosus ligation one week after his birth. Five months after his first palliative operation, he successfully underwent a bidirectional Glenn procedure. AD - Department of Pediatrics, Graduate School of Medicine, Hokkaido University, Sapporo Japan Department of Pediatrics, Graduate School of Medical Sciences and Medical School, Nagoya City University, Nagoya, Japan DB - Ucinahl DO - 10.1111/ped.14240 DP - EBSCOhost J2 - Pediatrics International KW - Tricuspid Atresia -- Diagnosis Prader-Willi Syndrome -- Diagnosis Male Infant N1 - Accession Number: 146168641. Language: English. Entry Date: 20201012. Revision Date: 20201028. Publication Type: Case Study; case study; pictorial; tables/charts. Journal Subset: Asia; Biomedical; Peer Reviewed. NLM UID: 100886002. NV - Malden, Massachusetts PB - Wiley-Blackwell PY - 2020 SP - 1105-1106 ST - A case of tricuspid atresia with Prader‐Willi syndrome TI - A case of tricuspid atresia with Prader‐Willi syndrome UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=146168641&site=ehost-live&scope=site VL - 62 ID - 5353 ER - TY - JOUR AB - Background: The Coping Health Inventory for Parents (CHIP) has demonstrated good psychometric properties in several language forms and has been used to assess the coping behaviors of families facing disease. However, the CHIP has not been validated in Mexico among families of children with chronic conditions, where it could be useful for research and intervention. The objectives of this instrumental study were to obtain a version of the CHIP for the Spanish language in Mexico, establish the factor structure of the Mexican version of the CHIP, probe its internal consistency reliability, and assess its concurrent construct validity. Methods: A nonprobability sample of 405 family caregivers of children with chronic diseases responded to a battery of measurement instruments that included the CHIP, the Beck Anxiety Inventory, and the Beck Depression Inventory. The sample was randomly divided into two parts. In one subsample (190 participants), an exploratory factor analysis was performed using a principal component analysis and oblique rotation. In the second subsample (215 participants), a confirmatory factor analysis was performed using maximum likelihood estimation. Results: The scale was reduced to 16 items (CHIP-16) with factorial loads greater than.50. The empirical criteria used to determine the number of factors converged on the following five factors: belief and trust (McDonald ω =.85), spouse/partner relationship (ω =.79), home care (ω =.77), family involvement (ω =.75), and security/stability (ω =.79). The overall internal consistency was good (ω =.88). The five-factor model showed acceptable fit indices and high parsimony. The mean CHIP-16 scores and the Spouse/partner relationship scores among the caregivers with anxiety were greater than those among the caregivers without anxiety. The mean home-care scores among the women were greater than those among men. Conclusions: The 16-item version of the CHIP showed good internal consistency and construct validity; thus, the CHIP-16 is a useful instrument for measuring and assessing coping in family caregivers of children with chronic diseases. AD - F. Toledano-Toledano, Unidad de Investigación en Medicina Basada en Evidencias, Hospital Infantil de México Federico Gómez, National Institute of Health, Dr. Márquez 162, Doctores, Cuauhtémoc, México City, Mexico AU - Toledano-Toledano, F. AU - Moral De La Rubia, J. AU - McCubbin, L. D. AU - Cauley, B. AU - Luna, D. DB - UEmbase DO - 10.1186/s12955-020-01357-5 IS - 1 KW - adult article asthma Beck Anxiety Inventory Beck Depression Inventory caregiver child chronic disease concurrent validity confirmatory factor analysis congenital heart malformation construct validity controlled study Coping Health Inventory for Parents cystic fibrosis Down syndrome end stage renal disease exploratory factor analysis female human Human immunodeficiency virus infection internal consistency major clinical study male malignant neoplasm maximum likelihood method nephrotic syndrome oblique rotation pediatric patient principal component analysis psychometry reliability school child Spanish (language) tricuspid valve atresia LA - English M3 - Article N1 - L631627209 2020-05-13 2020-05-22 PY - 2020 SN - 1477-7525 ST - Brief version of the coping health inventory for parents (CHIP) among family caregivers of children with chronic diseases T2 - Health and Quality of Life Outcomes TI - Brief version of the coping health inventory for parents (CHIP) among family caregivers of children with chronic diseases UR - https://www.embase.com/search/results?subaction=viewrecord&id=L631627209&from=export http://dx.doi.org/10.1186/s12955-020-01357-5 VL - 18 ID - 4420 ER - TY - JOUR AB - To define the correlation between neuroanatomic and developmental outcomes of children with single ventricle (SV) or transposition of the great arteries (TGA), a prospective longitudinal study was performed in preschool and school-age children. Twenty-seven children with congenital heart disease (9, TGA; 18, SV) were included. Participants underwent 3-dimensional magnetic resonance imaging (MRI) and neurodevelopmental assessment at around 3 years (preschool age) and at 9 years (school age), and 48 healthy controls underwent MRI, and their data were used to derive best-fit models for normal brain volumes for comparisons with congenital heart disease patients. Total brain volume (TBV) and regional brain volumes remained significantly smaller in SV children than in TGA children at both time points, though the growth slope of TBV was not significantly different between the SV and TGA groups. Although the psychomotor developmental index at preschool was significantly lower in SV patients, the full-scale IQ at school age was not significantly lower in SV patients. There was a strong correlation between full-scale IQ and TBV (r = 0.49, P = 0.005). Despite the current best practices, persistently lower TBV was seen in SV patients until 9 years of age. For both the SV and TGA groups, TBV at 3 years was a strong predictor of TBV at 9 years. Since there was a correlation between TBV and IQ at 9 years, identification of factors that affect brain growth until 3 years will be imperative to improve patients’ cognitive function at school age. AD - A. Hiraiwa, Department of Pediatrics, Graduate School of Medicine, University of Toyama, 2630 Sugitani, Toyama, Toyama, Japan AU - Hiraiwa, A. AU - Kawasaki, Y. AU - Ibuki, K. AU - Hirono, K. AU - Matsui, M. AU - Yoshimura, N. AU - Origasa, H. AU - Oishi, K. AU - Ichida, F. DB - UEmbase DO - 10.1053/j.semtcvs.2019.06.013 IS - 4 KW - article basal ganglion body weight brain cortex brain development brain size cerebellum child clinical article cognition congenital heart disease controlled study corpus callosum extracorporeal oxygenation female Fontan procedure gestational age great vessels transposition head circumference heart right ventricle heart single ventricle human image processing infant longitudinal study male mental disease neuroanatomy nuclear magnetic resonance imaging observational study preschool child prospective study psychomotor development pulmonary artery risk factor school child temporal cortex thalamus three-dimensional imaging white matter LA - English M3 - Article N1 - L2002546526 2019-08-14 2021-02-19 PY - 2020 SN - 1532-9488 1043-0679 SP - 936-944 ST - Brain Development of Children With Single Ventricle Physiology or Transposition of the Great Arteries: A Longitudinal Observation Study T2 - Seminars in Thoracic and Cardiovascular Surgery TI - Brain Development of Children With Single Ventricle Physiology or Transposition of the Great Arteries: A Longitudinal Observation Study UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2002546526&from=export http://dx.doi.org/10.1053/j.semtcvs.2019.06.013 VL - 32 ID - 4375 ER - TY - JOUR AB - Unconventional natural gas developments (UNGD) may release air and water pollutants into the environment, potentially increasing the risk of birth defects. We conducted a case-control study evaluating 52,955 cases with birth defects and 642,399 controls born between 1999 and 2011 to investigate the relationship between UNGD exposure and the risk of gastroschisis, congenital heart defects (CHD), neural tube defects (NTDs), and orofacial clefts in Texas. We calculated UNGD densities (number of UNGDs per area) within 1, 3, and 7.5 km of maternal address at birth and categorized exposure by density tertiles. For CHD subtypes with large case numbers, we also performed time-stratified analyses to examine temporal trends. We calculated adjusted odds ratios (aOR) and 95% confidence intervals (CI) for the association with UNGD exposure, accounting for maternal characteristics and neighborhood factors. We also included a bivariable smooth of geocoded maternal location in an additive model to account for unmeasured spatially varying risk factors. Positive associations were observed between the highest tertile of UNGD density within 1 km of maternal address and risk of anencephaly (aOR: 2.44, 95% CI: 1.55, 3.86), spina bifida (aOR: 2.09, 95% CI: 1.47, 2.99), gastroschisis among older mothers (aOR: 3.19, 95% CI: 1.77, 5.73), aortic valve stenosis (aOR: 1.90, 95% CI: 1.33, 2.71), hypoplastic left heart syndrome (aOR: 2.00, 95% CI: 1.39, 2.86), and pulmonary valve atresia or stenosis (aOR: 1.36, 95% CI: 1.10, 1.66). For CHD subtypes, results did not differ substantially by distance from maternal address or when residual confounding was considered, except for atrial septal defects. We did not observe associations with orofacial clefts. Our results suggest that UNGDs were associated with some CHDs and possibly NTDs. In addition, we identified temporal trends and observed presence of spatial residual confounding for some CHDs. AD - I.W. Tang, 653 E. Peltason Drive, Irvine, CA, United States AU - Tang, I. W. AU - Langlois, P. H. AU - Vieira, V. M. DB - UEmbase DO - 10.1016/j.envres.2020.110511 KW - natural gas adolescent adult anencephalus aortic valve stenosis case control study child cleft lip cleft lip with or without cleft palate cleft palate congenital heart malformation congenital malformation controlled study endocardial cushion defect Fallot tetralogy female gastroschisis great vessels transposition heart atrium septum defect heart ventricle septum defect Hispanic household income human hypoplastic left heart syndrome major clinical study male maternal age maternal exposure maternal smoking maternal stress maternal welfare neural tube defect newborn pregnancy outcome prenatal exposure priority journal pulmonary valve atresia pulmonary valve stenosis rural area rural population social status spinal dysraphism Texas tricuspid valve atresia urban population water pollutant LA - English M3 - Article N1 - L2010164959 2020-12-08 2021-03-16 PY - 2021 SN - 1096-0953 0013-9351 ST - Birth defects and unconventional natural gas developments in Texas, 1999–2011 T2 - Environmental Research TI - Birth defects and unconventional natural gas developments in Texas, 1999–2011 UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2010164959&from=export http://dx.doi.org/10.1016/j.envres.2020.110511 VL - 194 ID - 4347 ER - TY - JOUR AB - Neurodevelopmental (ND) impairment is common in children with congenital heart disease (CHD). While routine ND surveillance and evaluation of high-risk patients has become the standard-of-care, capture rate, barriers to referral, and potential patient benefits remain incompletely understood. Electronic data warehouse records from a single center were reviewed to identify all eligible and evaluated patients between July 2015 and December 2017 based on current guidelines for ND screening in CHD. Diagnoses, referring provider, and payor were considered. Potential benefit of the evaluation was defined as receipt of new diagnosis, referral for additional evaluation, or referral for a new service. Contingencies were assessed with Fisher's exact test. In this retrospective, cohort study, of 3434 children identified as eligible for ND evaluation, 135 were evaluated (4%). Appropriate evaluation was affected by diagnostic bias against coarctation of the aorta (CoArc) and favoring hypoplastic left heart syndrome (HLHS) (1.8 vs. 11.9%, p<0.01). Referrals were disproportionally made by a select group of cardiologists, and the rate of ND appointment non-compliance was higher in self-pay compared to insured patients (78% vs 27%, p<0.01). Potential benefit rate was 70-80% amongst individuals with the three most common diagnoses requiring neonatal surgery (CoArc, transposition of the great arteries, and HLHS). Appropriate ND evaluation in CHD is impacted by diagnosis, provider, and insurance status. Potential benefit of ND evaluation is high regardless of diagnosis. Strategies to improve access to ND evaluations and provider understanding of the at-risk population will likely improve longitudinal ND surveillance and clinical benefit. AD - Department of Pediatrics, Division of Critical Care Medicine, University of Utah, Salt Lake City, UT, USA. Kristi.Glotzbach@hsc.utah.edu. University of Utah School of Medicine, Salt Lake City, UT, USA. Department of Pediatrics, Division of Cardiology, University of Utah, Salt Lake City, UT, USA. Department of Surgery, Division of Cardiothoracic Surgery, University of Utah, Salt Lake City, UT, USA. Department of Pediatrics, Division of General Pediatrics, University of Utah, Salt Lake City, UT, USA. AN - 31865442 AU - Glotzbach, K. L. AU - Ward, J. J. AU - Marietta, J. AU - Eckhauser, A. W. AU - Winter, S. AU - Puchalski, M. D. AU - Miller, T. A. DA - Feb DB - UPubmed DO - 10.1007/s00246-019-02260-7 DP - NLM ET - 2019/12/23 IS - 2 KW - Adolescent Bias Child Child, Preschool Cohort Studies Developmental Disabilities/*diagnosis/etiology Female Heart Defects, Congenital/*complications Humans Infant Male Process Assessment, Health Care/standards Referral and Consultation/statistics & numerical data Retrospective Studies Risk Factors Chd Developmental delay ND outcomes Neurodevelopmental evaluation LA - eng N1 - 1432-1971 Glotzbach, Kristi L Orcid: 0000-0002-4459-0159 Ward, John J Marietta, Jennifer Eckhauser, Aaron W Winter, Sarah Puchalski, Michael D Miller, Thomas A Journal Article United States Pediatr Cardiol. 2020 Feb;41(2):327-333. doi: 10.1007/s00246-019-02260-7. Epub 2019 Dec 21. PY - 2020 SN - 0172-0643 SP - 327-333 ST - The Benefits and Bias in Neurodevelopmental Evaluation for Children with Congenital Heart Disease T2 - Pediatr Cardiol TI - The Benefits and Bias in Neurodevelopmental Evaluation for Children with Congenital Heart Disease VL - 41 ID - 2387 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 = 0.07–0.20) 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. (PsycInfo Database Record (c) 2020 APA, all rights reserved) AD - Goldberg, Caren S., Division of Pediatric Cardiology, C.S. Mott Children’s Hospital, University of Michigan, 1540 E Hospital Dr, Ann Arbor, MI, US, 48109 AN - 2019-77309-001 AU - Goldberg, Caren S. AU - Hu, Chenwei 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 - Li, Jennifer S. AU - Jacobs, Jeffrey P. AU - Cnota, James AU - Atz, Andrew M. AU - Lai, Wyman W. AU - Bellinger, David AU - Newburger, Jane W. DB - Upsyc DO - 10.1542/peds.2019-1010 DP - EBSCOhost IS - 5 KW - child behavior quality of life hypoplastic left heart syndrome adverse outcomes Child Child Behavior Disorders Female Humans Male Prospective Studies Surveys and Questionnaires Adaptive Behavior Congenital Disorders Functional Status Heart Disorders Pediatrics Risk Factors Syndromes N1 - Department of Pediatrics, Medical School, University of Michigan, Ann Arbor, MI, US. Institutional Authors: PHN Investigators. Release Date: 20201012. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Print. Document Type: Journal Article. Language: English. Major Descriptor: Adaptive Behavior; Congenital Disorders; Functional Status; Heart Disorders; Quality of Life. Minor Descriptor: Pediatrics; Risk Factors; Syndromes. 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). Tests & Measures: Pediatric Quality of Life Inventory 4.0; Child Health Questionnaire Parent Report Form 50; Functional Status Revised Second Edition; Behavior Assessment System for Children--Second Edition DOI: 10.1037/t15079-000; Vineland Adaptive Behavior Scales, Second Edition DOI: 10.1037/t15164-000. Methodology: Empirical Study; Quantitative Study. ArtID: e20191010. Issue Publication Date: Nov, 2019. Publication History: Accepted Date: Aug 5, 2019. Copyright Statement: The American Academy of Pediatrics. 2019. Sponsor: National Institutes of Health, National Heart, Lung, and Blood Institute, US. Grant: HL068270; HL068290; HL109673; HL109737; HL109741; HL109741; HL109743; HL109777; HL109778; HL109781; HL109816; HL109818. Recipients: No recipient indicated Sponsor: National Institutes of Health, US. Recipients: No recipient indicated PY - 2019 SN - 0031-4005 1098-4275 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=psyh&AN=2019-77309-001&site=ehost-live&scope=site cgoldber@med.umich.edu VL - 144 ID - 5815 ER - TY - JOUR AB - Background and objectives. The high recurrence rate of congenital heart disease (CHD) in siblings was the rationale for recommending fetal echocardiography. However, in a developing country like Egypt, there are limited fetal echocardiographic examinations under this category. The study was conducted to assess knowledge about fetal echocardiography amongst parents of children with CHD. Method. A questionnaire survey was conducted in a tertiary pediatric hospital from June to December 2018. The study included parents having children with CHD follow up in the outpatient clinic or admitted in the pediatric cardiology ward. The questionnaire included demographic data of parents regarding sex, age, education, and residence. It also includes a detailed inquiry about parents’ knowledge regarding the availability, safety, and value of fetal echocardiography. Results. Participants were 200 parents, mostly mothers 159 (79.5%). The median age of parents included in the study was 33.5 (29-40) years. Regarding awareness, 134 (67%) did not know any prenatal diagnostic investigation for CHD, 46 (23%) knew fetal echocardiography, and 20 (10%) named other tools. Nevertheless, 34% of parents thought they would need fetal echocardiography in a subsequent pregnancy. Although 178 (89%) of parents thought it might be a safe investigation, 33% did not think it will have additional benefits over postnatal echocardiography. The age of the patient and age of the participating parent were the only statistically significant predictors for parents knowledge on fetal echocardiography existence with p= 0.008, 95% CI=1.0391.282 and p= 0.015, 95% CI = 0.864-0.984, respectively. Conclusion. Parents of children with CHD have significant knowledge gaps regarding Fetal Echocardiography. Our findings suggest that the current parent counseling is inadequate and needs further focus, especially in developing countries, to promote parents’ understanding of the prenatal cardiac diagnosis. Data on fetal echocardiography should be clarified at the initial diagnosis of pediatric CHD. AD - S. Rakha, Department of Pediatric Cardiology, Mansoura University, Faculty of Medicine, El Gomhouria St, Mansoura, Egypt AU - Rakha, S. DB - UEmbase DO - 10.24953/turkjped.2020.04.006 IS - 4 KW - adult article child cohort analysis congenital heart disease cyanotic heart disease Down syndrome echocardiography education female follow up heart arrhythmia heart atrium septum defect heart injury hospitalization human hypoplastic left heart syndrome infant LEOPARD syndrome major clinical study male Noonan syndrome parental attitude postnatal care pregnancy pregnancy diabetes mellitus questionnaire recurrence risk LA - English M3 - Article N1 - L2004887102 2020-08-18 2020-10-15 PY - 2020 SN - 0041-4301 SP - 569-577 ST - Awareness assessment for parents of children with congenital heart diseases regarding fetal echocardiography T2 - Turkish Journal of Pediatrics TI - Awareness assessment for parents of children with congenital heart diseases regarding fetal echocardiography UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2004887102&from=export http://dx.doi.org/10.24953/turkjped.2020.04.006 VL - 62 ID - 4439 ER - TY - JOUR AB - BACKGROUND: Cardiac transplantation in early childhood is limited by scarcity of organ donors. Advances in cardiac xenotransplantation (XTx) research suggest that xenografts may one day represent an alternative to allografts. We sought to determine the attitudes among surgeons and cardiologists in the field of pediatric cardiac transplantation toward the potential use of XTx if this clinical option were to become a reality. METHODS: A Likert-scale anonymous survey addressing the use of XTx in pediatric patients was sent to members of the Congenital Heart Surgeons (CHS) Society and the Pediatric Heart Transplant Society. Results were described and compared between the two surgeon/physician groups. RESULTS: Ninety-two CHS and 42 pediatric transplant cardiologists (PTC) responded (N = 134). The potential acceptance of XTx was high in both groups, assuming risks and results were similar to those of cardiac allotransplantation (88% CHS vs 81% PTC; P = .07). When asked if they would recommend a xenograft, if the results were anticipated to be inferior to those of cardiac allotransplantation, as a bridge to a human heart, potential acceptance fell dramatically but remained higher among CHS than PTC (41% vs 17%, p 0.02). Approximately only one-third of CHS and half of PTC preferred primary cardiac XTx for hypoplastic left heart syndrome if there was no waitlist time and had similar outcomes to allotransplantation. CONCLUSIONS: Our findings suggest that potential acceptance of XTx by CHS and PTC would not be a major barrier if XTx demonstrated similar outcomes to allotransplantation. Acceptance by other congenital heart stakeholders remains to be investigated. AD - Department of Surgery, School of Medicine, University of Alabama, Birmingham, AL, USA. Division of Pediatric Cardiology, School of Medicine, University of Alabama, Birmingham, AL, USA. Department of Social Work, Abilene Christian University, Abilene, TX, USA. AN - 32645781 AU - Padilla, L. A. AU - Sorabella, R. A. AU - Carlo, W. F. AU - Dabal, R. J. AU - Rhodes, L. AU - Cleveland, D. C. AU - Cooper, D. K. AU - Paris, W. DA - Jul DB - UPubmed DO - 10.1177/2150135120916744 DP - NLM ET - 2020/07/11 IS - 4 KW - Adult *Attitude *Attitude of Health Personnel Child Cross-Sectional Studies Female Heart Transplantation/*methods Humans Hypoplastic Left Heart Syndrome/*surgery Male Middle Aged Physicians/*psychology Surgeons/*psychology Surveys and Questionnaires *Tissue Donors Transplantation, Heterologous *attitudes *congenital heart disease *physicians *surgeons *xenotransplantation LA - eng N1 - 2150-136x Padilla, Luz A Orcid: 0000-0003-4032-044x Sorabella, Robert A Carlo, Waldemar F Dabal, Robert J Rhodes, Leslie Cleveland, David C Cooper, David K Paris, Wayne U19 AI090959/AI/NIAID NIH HHS/United States Journal Article Multicenter Study Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't United States World J Pediatr Congenit Heart Surg. 2020 Jul;11(4):426-430. doi: 10.1177/2150135120916744. PY - 2020 SN - 2150-1351 SP - 426-430 ST - Attitudes to Cardiac Xenotransplantation by Pediatric Heart Surgeons and Physicians T2 - World J Pediatr Congenit Heart Surg TI - Attitudes to Cardiac Xenotransplantation by Pediatric Heart Surgeons and Physicians VL - 11 ID - 2362 ER - TY - JOUR AB - BACKGROUND: Patients who have undergone the Fontan procedure are at high risk of circulatory failure. In an exploratory analysis we aimed to determine the prognostic value of blood biomarkers in a young cohort who have undergone the Fontan procedure. METHODS AND RESULTS: In multicenter prospective studies patients who have undergone the Fontan procedure underwent blood sampling, cardiopulmonary exercise testing, and stress cardiac magnetic resonance imaging. Several biomarkers including NT-proBNP (N-terminal pro-B-type natriuretic peptide), GDF-15 (growth differentiation factor 15), Gal-3 (galectin-3), ST2 (suppression of tumorigenicity 2), DLK-1 (protein delta homolog 1), FABP-4 (fatty acid-binding protein 4), IGFBP-1 (insulin-like growth factor-binding protein 1), IGFBP-7, MMP-2 (matrix metalloproteinase 2), and vWF (von Willebrand factor) were assessed in blood at 9.6 (7.1–12.1) years after Fontan completion. After this baseline study measurement, follow-up information was collected on the incidence of adverse cardiac events, including cardiac death, out of hospital cardiac arrest, heart transplantation (listing), cardiac reintervention (severe events), hospitalization, and cardioversion/ablation for arrhythmias was collected and the relation with blood biomarkers was assessed by Cox proportional hazard analyses. The correlation between biomarkers and other clinical parameters was evaluated. We included 133 patients who have undergone the Fontan procedure, median age 13.2 (25th, 75th percentile 10.4–15.9) years, median age at Fontan 3.2 (2.5–3.9) years. After a median follow-up of 6.2 (4.9–6.9) years, 36 (27.1%) patients experienced an event of whom 13 (9.8%) had a severe event. NT-proBNP was associated with (all) events during follow-up and remained predictive after correction for age, sex, and dominant ventricle (hazard ratio, 1.89; CI, 1.32–2.68). The severe event-free survival was better in patients with low levels of GDF-15 (P=0.005) and vWF (P=0.008) and high levels of DLK-1 (P=0.041). There was a positive correlation (β=0.33, P=0.003) between DLK-1 and stress cardiac magnetic resonance imaging functional reserve. CONCLUSIONS: NT-proBNP, GDF-15, vWF, DLK-1, ST-2 FABP-4, and IGFBP-7 levels relate to long-term outcome in young patients who have undergone the Fontan procedure. AD - W.A. Helbing, Department of Pediatric Cardiology, Academic Centre for Congenital Heart Disease, Erasmus MC-Sophia Children’s Hospital, PO Box 2060, Rotterdam, Netherlands AU - van den Bosch, E. AU - Bossers, S. S. M. AU - Kamphuis, V. P. AU - Boersma, E. AU - Roos-Hesselink, J. W. AU - Breur, J. M. P. J. AU - Ten Harkel, A. D. J. AU - Kapusta, L. AU - Bartelds, B. AU - Roest, A. A. W. AU - Kuipers, I. M. AU - Blom, N. A. AU - Koopman, L. P. AU - Helbing, W. A. DB - UEmbase DO - 10.1161/JAHA.119.015022 IS - 5 KW - adolescent adverse outcome article blood sampling carcinogenicity cardiovascular magnetic resonance cardioversion clinical assessment congenital heart disease controlled study event free survival exercise test exploratory research female follow up Fontan procedure gene expression heart arrhythmia heart death heart function heart single ventricle heart transplantation hospitalization human incidence major clinical study male multicenter study out of hospital cardiac arrest prospective study protein expression surgery amino terminal pro brain natriuretic peptide biological marker endogenous compound fatty acid binding protein 4 galectin 3 gelatinase A growth differentiation factor 15 somatomedin binding protein 1 von Willebrand factor LA - English M3 - Article N1 - L2006727156 2021-03-23 PY - 2021 SN - 2047-9980 SP - 1-12 ST - Associations between blood biomarkers, cardiac function, and adverse outcome in a young fontan cohort T2 - Journal of the American Heart Association TI - Associations between blood biomarkers, cardiac function, and adverse outcome in a young fontan cohort UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2006727156&from=export http://dx.doi.org/10.1161/JAHA.119.015022 VL - 10 ID - 4362 ER - TY - JOUR AB - Background: As almost all mental disorders are associated with increased suicidal‐related behavior, anhedonia might be a trans‐diagnostic dimension to target for suicide prevention. Methods: For this 3‐year‐long prospective study, 2,839 outpatients with mood disorders were recruited. They were divided in: (a) two groups according to the occurrence or not of suicidal ideation during the follow‐up, and (b) two groups according to the occurrence or not of suicide attempts during the follow‐up. Anhedonia was assessed using a composite score (the French version of the 14‐item Snaith‐Hamilton Pleasure Scale and item 13 of the Quick Inventory of Depressive Symptomatology scale) at inclusion and at 6, 12, 24, and 36 months after inclusion. Results: Patients with mood disorders and anhedonia at least at one follow‐up visit had a 1.4‐fold higher risk of suicidal ideation (adjusted odds ratio = 1.35; 95% confidence interval [1.07, 1.70]), even after adjustment for confounding factors of suicide risk (i.e., bipolar or unipolar disorder, sex, age, marital status, education level, antidepressant intake, personal history of suicide attempt, at least one childhood trauma, and mean of the maximum depression score during the follow‐up). Conversely, association between anhedonia and suicide attempt did not remain significant after adjustment. Conclusions: The significant association between anhedonia and suicide ideation in patients with mood disorders stresses the need of targeting hedonia in mood disorders, and of research focusing on the position to pleasure in life through eudaimonia. (PsycInfo Database Record (c) 2021 APA, all rights reserved) AD - Ducasse, Déborah, Department of Psychiatric Emergency and Acute Care, Lapeyronie Hospital, 371 Avenue Gaston Giraud, 34295, Montpellier, France AN - 2020-51572-001 AU - Ducasse, Déborah AU - Dubois, Jonathan AU - Jaussent, Isabelle AU - Azorin, Jean‐Michel AU - Etain, Bruno AU - Gard, Sébastien AU - Henry, Chantal AU - Bougerol, Thierry AU - Kahn, Jean‐Pierre AU - Aubin, Valérie AU - Bellivier, Frank AU - Belzeaux, Raoul AU - Dubertret, Caroline AU - Dubreucq, Julien AU - Llorca, Pierre‐Michel AU - Loftus, Josephine AU - Passerieux, Christine AU - Polosan, Mircea AU - Samalin, Ludovic AU - Leboyer, Marion AU - Yrondi, Antoine AU - Bennabi, Djamila AU - Haffen, Emmanuel AU - Maruani, Julia AU - Allauze, Etienne AU - Camus, Vincent AU - D'Amato, Thierry AU - Doumy, Olivier AU - Holtzmann, Jérôme AU - Lançon, Christophe AU - Moliere, Fanny AU - Moirand, Rémi AU - Richieri, Raphaëlle Marie AU - Horn, Mathilde AU - Schmitt, Laurent AU - Stephan, Florian AU - Genty, Jean‐Baptiste AU - Vaiva, Guillaume AU - Walter, Michel AU - El‐Hage, Wissam AU - Aouizerate, Bruno AU - Olié, Emilie AU - Courtet, Philippe DB - Upsyc DO - 10.1002/da.23072 DP - EBSCOhost IS - 1 KW - anhedonia mood disorder prospective study suicidal ideation suicide attempt Affective Disorders Attempted Suicide Severity (Disorders) Pleasure Prospective Studies Risk Factors N1 - CHU Montpellier, Hopital Lapeyronie, Department of Emergency Psychiatry and Post Acute Care, CHRU, Montpellier, France. Release Date: 20200716. Correction Date: 20210208. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Affective Disorders; Anhedonia; Attempted Suicide; Severity (Disorders); Suicidal Ideation. Minor Descriptor: Pleasure; Prospective Studies; Risk Factors. Classification: Affective Disorders (3211). Population: Human (10); Male (30); Female (40). Age Group: Adulthood (18 yrs & older) (300). Tests & Measures: Quick Inventory of Depressive Symptomatology; Snaith‐Hamilton Pleasure Scale-French Version; Montgomery‐Åsberg Depression Rating Scale; Childhood Trauma Questionnaire DOI: 10.1037/t02080-000; Pittsburgh Sleep Quality Index DOI: 10.1037/t05178-000. Methodology: Empirical Study; Longitudinal Study; Prospective Study; Quantitative Study. Supplemental Data: Experimental Materials Internet. Page Count: 11. Issue Publication Date: Jan, 2021. Publication History: Accepted Date: Jun 13, 2020; Revised Date: May 30, 2020; First Submitted Date: Mar 19, 2020. Copyright Statement: Wiley Periodicals LLC. 2020. Sponsor: Fondation FondaMental. Other Details: RTRS Santé Mentale. Recipients: No recipient indicated Sponsor: Agence Nationale de la Recherche. Grant: ANR‐11‐IDEX‐0004‐02; ANR‐10‐COHO‐10‐01. Other Details: Investissements d'Avenir program. Recipients: No recipient indicated Sponsor: INSERM (Institut National de la Santé et de la Recherche Médicale). Recipients: No recipient indicated PY - 2021 SN - 1091-4269 1520-6394 SP - 17-27 ST - Association between anhedonia and suicidal events in patients with mood disorders: A 3‐year prospective study T2 - Depression and Anxiety TI - Association between anhedonia and suicidal events in patients with mood disorders: A 3‐year prospective study UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2020-51572-001&site=ehost-live&scope=site ORCID: 0000-0002-5377-1488 ORCID: 0000-0002-8205-7590 ORCID: 0000-0003-3874-5748 d-ducasse@chu-montpellier.fr VL - 38 ID - 5814 ER - TY - JOUR AB - CASE: Phillip is a young man born with hypoplastic left heart syndrome referred to your practice for a range of mental health concerns. He underwent palliation to an extracardiac Fontan in infancy and experienced multiple complications over the next decade including valvular regurgitation and arrhythmias necessitating a pacemaker. Phillip continued to have systolic heart failure with New York Heart Association class II symptoms, managed with 4 medications and anticoagulation.Despite this complex history, Phillip had intact cognitive abilities, achieved typical milestones, and performed well academically in secondary school. His first year of college proved to be more challenging, and Phillip presented to the outpatient psychiatry service with an acute depressive episode. His family history included depression, without known attention-deficit/hyperactivity disorder (ADHD). Treatment, including a selective serotonin reuptake inhibitor, cognitive behavioral therapy, and family support, led to near resolution of his symptoms of depression.In subsequent appointments, Phillip described a long history of inattention and disorganization with onset in childhood. This contributed to the decision to leave college, despite remission of symptoms of depression. Phillip was unable to study for any extended period without "perfect conditions," described as the absence of potential distractions except for background music. Despite attempts to maintain "perfect conditions," Phillip was often off task and "hyperfocusing" on irrelevant topics. Phillip struggled with planning and time management and would misplace items daily. Moreover, although the importance of self-care was well understood, Phillip often forgot to take his cardiac medication or to exercise, and he admitted to inconsistent sleep habits because of losing track of time.Based on a comprehensive psychiatric evaluation including retrospective report from a parent, Phillip was diagnosed with ADHD, coexisting with major depressive disorder, in remission. Significant ADHD symptoms were documented by interview, self-report, and administration of an abbreviated neuropsychological battery.Considering concerns regarding use of stimulants in a patient with congenital heart disease, including death, stroke, and myocardial infarction,1,2 how would you assess the risks-benefits of use of stimulants with Phillip? REFERENCES: 1. Wilens TE, Prince JB, Spencer TJ, et al. Stimulants and sudden death: what is a physician to do? Pediatrics. 2006;118:1215-1219.2. Zito JM, Burcu M. Stimulants and pediatric cardiovascular risk. J Child Adolesc Psychopharmacol. 2017;27:538-545. AD - Department of Psychiatry, Boston Children's & Harvard Medical School, Boston, MA. Department of Psychiatry and Behavioral Sciences, Boston Children's Hospital, Harvard Medical School, Boston, MA. Department of Psychiatry and Psychology, Mayo Clinic, Rochester, MN. Outpatient Psychiatry Service, Boston Children's Hospital, Harvard Medical School, Boston, MA. Department of Psychiatry, Boston Children's Hospital, Boston, MA. ADHD Program, Division of Developmental Medicine, Boston Children's Hospital, Boston, MA. Pediatrics & Psychology, Harvard Medical School, Boston, MA. Department of Pediatrics, Harvard Medical School, Advanced Cardiac Therapies, Boston Children's Hospital, Boston, MA. Division of Developmental-Behavioral Pediatrics, Boston Medical Center, Boston University School of Medicine, Boston, MA. AN - 33859122 AU - Hammerness, P. G. AU - Cassidy, A. R. AU - Potts, H. AU - Richardson, A. AU - Fogler, J. AU - Daly, K. P. AU - Augustyn, M. DA - May 1 DB - UPubmed DO - 10.1097/dbp.0000000000000961 DP - NLM ET - 2021/04/17 IS - 4 LA - eng N1 - 1536-7312 Hammerness, Paul G Cassidy, Adam R Potts, Heather Richardson, Alanna Fogler, Jason Daly, Kevin P Augustyn, Marilyn Journal Article United States J Dev Behav Pediatr. 2021 May 1;42(4):340-342. doi: 10.1097/DBP.0000000000000961. PY - 2021 SN - 0196-206x SP - 340-342 ST - Assessment and Treatment of a Young Adult with Congenital Heart Disease and ADHD T2 - J Dev Behav Pediatr TI - Assessment and Treatment of a Young Adult with Congenital Heart Disease and ADHD VL - 42 ID - 2341 ER - TY - JOUR AB - Objective: To evaluate heart rate against workload and oxygen consumption during exercise in Fontan patients.Method: Fontan patients (n = 27) and healthy controls (n = 25) underwent cardiopulmonary exercise testing with linear increase of load. Heart rate and oxygen uptake were measured during tests. Heart rate recovery was recorded for 10 minutes.Results: Heart rate at midpoint (140 ± 14 versus 153 ± 11, p < 0.001) and at maximal effort (171 ± 14 versus 191 ± 10 beats per minute, p < 0.001) of test was lower for patients than controls. Heart rate recovery was similar between groups. Heart rate in relation to workload was higher for patients than controls both at midpoint and maximal effort. Heart rate in relation to oxygen uptake was similar between groups throughout test. Oxygen pulse, an indirect surrogate measure of stroke volume, was reduced at maximal effort in patients compared to controls (6.6 ± 1.1 versus 7.5 ± 1.4 ml·beat-1·m-2, p < 0.05) and increased significantly less from midpoint to maximal effort for patients than controls (p < 0.05).Conclusions: Heart rate is increased in relation to workload in Fontan patients compared with controls. At higher loads, Fontan patients seem to have reduced heart rate and smaller increase in oxygen pulse, which may be explained by inability to further increase stroke volume and cardiac output. Reduced ability to increase or maintain stroke volume at higher heart rates may be an important limiting factor for maximal cardiac output, oxygen uptake, and physical performance. AD - E.R. Hedlund, Karolinska University Hospital, Astrid Lindgren Children's Hospital Solna, Eugeniav gen 23, C8:34, Stockholm, Sweden AU - Hedlund, E. R. AU - Söderström, L. AU - Lundell, B. DB - UEmbase DO - 10.1017/S1047951120000761 IS - 5 KW - bicycle ergometer Ergomedic 839E Exercise AB GE CASE captopril enalapril adolescent article cardiopulmonary exercise test clinical article controlled study female Fontan procedure heart output heart rate heart stroke volume human lung gas exchange male oxygen consumption prescription pulse oximetry self report LA - English M3 - Article N1 - L631604433 2020-05-04 2020-07-23 PY - 2020 SN - 1467-1107 1047-9511 SP - 674-680 ST - Appropriate heart rate during exercise in Fontan patients T2 - Cardiology in the Young TI - Appropriate heart rate during exercise in Fontan patients UR - https://www.embase.com/search/results?subaction=viewrecord&id=L631604433&from=export http://dx.doi.org/10.1017/S1047951120000761 VL - 30 ID - 4419 ER - TY - JOUR AB - Congenital aortic stenosis ranges from newborns with critical obstruction at valvar level and shock to less symptomatic neonates with gradual development of symptoms of heart failure. A team-based approach may provide the best results. Echocardiography gives essential information about the aortic valve, left-sided heart structures and associated lesions. Decision making between biventricular repair and single ventricle palliation is complex. In the case of borderline left ventricular size and function the hybrid procedure should be considered, allowing patients to recover and “declare themselves” as 1- or 2-ventricle candidates. Treatment strategies should prioritize quality of life and preservation of left ventricular function. Minimizing reintervention is an important secondary priority. Both balloon and open interventions are established and effective treatments for neonates. Reintervention rates depend on original morphology of the valve and method used. In comparison with blind ballooning, where the balloon can only tear the valve tissue, open valvuloplasty is done under direct vision and is individually tailored according to the anatomy, addressing not only commissural fusion but mixoid thickening of the cusps as well, can produce a better valve with a maximum valve orifice, without causing regurgitation. Such repair preserving function of the native valve without using artificial material allows for growth of the child, minimizing the need for intervention with excellent outcome if tricuspid geometry could be achieved. AD - V. Hraska, Division of Congenital Heart Surgery; Department of Surgery, Herma Heart Institute, Medical College of Wisconsin, 9000 W. Wisconsin Avenue, B730, Milwaukee, WI, United States AU - Hraska, V. DB - UEmbase DO - 10.1053/j.optechstcvs.2020.08.001 KW - aortic stenosis aortic valve repair article balloon dilatation child congenital disorder decision making echocardiography geometry heart failure heart left ventricle function heart left ventricle size heart single ventricle human newborn obstruction palliative therapy quality of life tricuspid valve vision LA - English M3 - Article in Press N1 - L2011110393 2021-03-02 PY - 2021 SN - 1532-8627 1522-2942 ST - Aortic Valve Repair in Neonates T2 - Operative Techniques in Thoracic and Cardiovascular Surgery TI - Aortic Valve Repair in Neonates UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2011110393&from=export http://dx.doi.org/10.1053/j.optechstcvs.2020.08.001 ID - 4368 ER - TY - JOUR AB - Congenital heart defects (CHD) is one of the most common types of birth defects. Thanks to advances in surgical techniques and intensive care, the majority of children with severe forms of CHD survive into adulthood. However, this increase in survival comes with a cost. CHD survivors have neurological functioning at the bottom of the normal range. A large spectrum of central nervous system dysmaturation leads to the deficits seen in critical CHD. The heart develops early during gestation, and CHD has a profound effect on fetal brain development for the remainder of gestation. Term infants with critical CHD are born with an immature brain, which is highly susceptible to hypoxic-ischemic injuries. Perioperative blood flow disturbances due to the CHD and the use of cardiopulmonary bypass or circulatory arrest during surgery cause additional neurological injuries. Innate patient factors, such as genetic syndromes and preterm birth, and postoperative complications play a larger role in neurological injury than perioperative factors. Strategies to reduce the disability burden in critical CHD survivors are urgently needed. AD - Department of Pediatrics and Neonatology, Maastricht University Medical Center, Maastricht, The Netherlands. School for Mental Health and Neuroscience, Maastricht University, Maastricht, The Netherlands. Department of Pediatric Cardiac Surgery, University of Barcelona, Vall d'Hebron, Spain. Department of Fetal, Maternal and Neonatal Health, C. Arrigo Children's Hospital, Alessandria, Italy. Department of Pediatrics and Neonatology, Maastricht University Medical Center, Maastricht, The Netherlands. danilo.gavilanes@mumc.nl. School for Mental Health and Neuroscience, Maastricht University, Maastricht, The Netherlands. danilo.gavilanes@mumc.nl. Instituto de Investigación e Innovación de Salud Integral, Facultad de Ciencias Médicas, Universidad Católica de Guayaquil, Guayaquil, Ecuador. danilo.gavilanes@mumc.nl. Department of Pediatrics, Maastricht University Medical Center, P. Debyelaan 25, 6229 HX, Maastricht, The Netherlands. danilo.gavilanes@mumc.nl. AN - 33373013 AU - Barkhuizen, M. AU - Abella, R. AU - Vles, J. S. H. AU - Zimmermann, L. J. I. AU - Gazzolo, D. AU - Gavilanes, A. W. D. C2 - PMC7864813 DA - Jan DB - UPubmed DO - 10.1007/s00246-020-02440-w DP - NLM ET - 2020/12/30 IS - 1 KW - Adult Brain/growth & development Brain Diseases/*epidemiology Brain Injuries/epidemiology Cardiopulmonary Bypass/methods Child Female Heart Defects, Congenital/*epidemiology/surgery Humans Hypoxia-Ischemia, Brain/epidemiology Infant Infant, Newborn Neurodevelopmental Disorders/epidemiology Postoperative Complications/*epidemiology Pregnancy Premature Birth/epidemiology Survivors Brain injury Congenital heart disease Hypoplastic left heart syndrome Immaturity Surgery LA - eng N1 - 1432-1971 Barkhuizen, Melinda Orcid: 0000-0002-9952-7085 Abella, Raul Orcid: 0000-0002-9209-9970 Vles, J S Hans Orcid: 0000-0002-9707-1782 Zimmermann, Luc J I Orcid: 0000-0002-1409-3821 Gazzolo, Diego Orcid: 0000-0002-0643-2555 Gavilanes, Antonio W D Orcid: 0000-0002-6670-3927 Journal Article Review Pediatr Cardiol. 2021 Jan;42(1):1-18. doi: 10.1007/s00246-020-02440-w. Epub 2020 Dec 29. PY - 2021 SN - 0172-0643 (Print) 0172-0643 SP - 1-18 ST - Antenatal and Perioperative Mechanisms of Global Neurological Injury in Congenital Heart Disease T2 - Pediatr Cardiol TI - Antenatal and Perioperative Mechanisms of Global Neurological Injury in Congenital Heart Disease VL - 42 ID - 2351 ER - TY - JOUR AB - BACKGROUND: Although advances in congenital heart disease (CHD) management have allowed survival of children with even highly complex CHD lesions well into adult life, the burden of disease (medical, psychological and social) has not been well characterised, for those living to middle age and beyond. METHODS: We assessed 121 consecutive patients from our adult CHD centre, who survived to age 50 years and who had had moderate or complex CHD lesions. Pre-specified groups included those with repaired tetralogy of Fallot (TOF) (n=56), coarctation of the aorta (CoA) (n=34), systemic right ventricle (RV) (n=9), Fontan surgery for "single ventricle" hearts (n=5), those with repaired Ebstein's Anomaly (n=9) and other complex CHD (n=8). RESULTS: The overall burden of disease was very substantial. Of the TOF patients, almost half (46%) had required at least one open-heart reoperation and 41% had had a pacemaker or implantable defibrillator; 20% had had a radiofrequency ablation and 32% were on anti-arrhythmic therapy. Over 40% had ≥1 admission for heart failure and 9% had had endocarditis. Only 64% were still employed. Of the CoA survivors, 50% had had a second operation (aortic valve and/or descending aortic surgery), 88% were on medications for hypertension and 62% were still employed. In the more complex groups, approximately half had been diagnosed with depression/anxiety and cerebrovascular event, heart failure and/or significant arrhythmias were common. CONCLUSIONS: Despite considerable advances, adults with CHD who survive to age 50 years have experienced high levels of physical and mental health complications. AD - Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia. Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia; Sydney Medical School, University of Sydney, Sydney, NSW, Australia. Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia; Sydney Medical School, University of Sydney, Sydney, NSW, Australia; Heart Research Institute, Sydney, NSW, Australia. Electronic address: david.celermajer@health.nsw.edu.au. AN - 32713766 AU - Rehan, R. AU - Kotchetkova, I. AU - Cordina, R. AU - Celermajer, D. DA - Feb DB - UPubmed DO - 10.1016/j.hlc.2020.05.114 DP - NLM ET - 2020/07/28 IS - 2 KW - Adult congenital heart disease Arrhythmia Depression Survival LA - eng N1 - 1444-2892 Rehan, Rajan Kotchetkova, Irina Cordina, Rachael Celermajer, David Journal Article Australia Heart Lung Circ. 2021 Feb;30(2):261-266. doi: 10.1016/j.hlc.2020.05.114. Epub 2020 Jul 6. PY - 2021 SN - 1443-9506 SP - 261-266 ST - Adult Congenital Heart Disease Survivors at Age 50 Years: Medical and Psychosocial Status T2 - Heart Lung Circ TI - Adult Congenital Heart Disease Survivors at Age 50 Years: Medical and Psychosocial Status VL - 30 ID - 2359 ER - TY - JOUR AB - Background: Successful transition to adult healthcare is particularly important for congenital heart disease (CHD) patients who have undergone palliative surgery, as they risk adverse events if not followed closely. This study examines young people at the worst end of the CHD spectrum who are born with a single ventricle (pumping heart chamber), and who undergo a series of operations that culminate in the Fontan surgical procedure. Purpose: To explore adolescents with a Fontan circulation, and their parents’, readiness for transition to adult care and pre-implementation of a transition program. Methods: Seventeen adolescents (15–18 years) and 15 of their parents completed questionnaires at the start of their first transition clinic. Results: Adolescents reported poor knowledge about their Fontan circulation, and 41% had a poor understanding of the purpose of their medications/treatments. Over half of the adolescents had poor knowledge around medical help-seeking (when, who, how). Most reported feeling comfortable with discussing their medical issues with their cardiologist, but considerably less so about sensitive adolescent issues, in particular, emotional wellbeing. Parents reported high levels of anxiety around transition to adult care services. Conclusion: Findings pre-program indicate poor adolescent health knowledge, a lack of focus in health services on emotional wellbeing and high parental anxiety. These findings highlight the need for dedicated programs that focus on early preparation, parental involvement and acknowledgment, transition as a process, strong integration and prioritisation in the health system with a youth-friendly and holistic focus, in particular, around emotional wellbeing. (PsycInfo Database Record (c) 2021 APA, all rights reserved) AD - Du Plessis, Karin, Heart Research Group, Murdoch Children’s Research Institute, 50 Flemington Road, Parkville, Melbourne, VIC, Australia, 3052 AN - 2019-56920-001 AU - Du Plessis, Karin AU - Culnane, Evelyn AU - Peters, Rebecca AU - d'Udekem, Yves DB - Upsyc DP - EBSCOhost IS - 4 KW - Fontan circulation transition from paediatric to adult care transition support programs Congenital Disorders Continuum of Care Health Knowledge Heart Disorders Surgery Adolescent Attitudes Parental Attitudes Parental Involvement Test Construction Health Anxiety N1 - Heart Research Group, Murdoch Children’s Research Institute, Melbourne, VIC, Australia. Other Publishers: Freund Publishing House. Release Date: 20210429. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: English. Major Descriptor: Congenital Disorders; Continuum of Care; Health Knowledge; Heart Disorders; Surgery. Minor Descriptor: Adolescent Attitudes; Parental Attitudes; Parental Involvement; Test Construction; Health Anxiety. Classification: Medical Treatment of Physical Illness (3363). Population: Human (10); Male (30); Female (40). Location: Australia. Age Group: Adolescence (13-17 yrs) (200); Adulthood (18 yrs & older) (300); Young Adulthood (18-29 yrs) (320). Tests & Measures: Readiness for Transition Questionnaires Surveyed Health Knowledge. Methodology: Empirical Study; Quantitative Study. Page Count: 3. Issue Publication Date: Aug, 2019. Publication History: Accepted Date: Feb 24, 2017; First Submitted Date: Feb 7, 2017. Copyright Statement: Walter de Gruyter GmbH, Berlin/Boston. 2019. Sponsor: National Health and Medical Research Council. Grant: 1076849. Recipients: No recipient indicated Sponsor: Victorian Government, Operational Infrastructure Support Program, Australia. Recipients: No recipient indicated PY - 2019 SN - 0334-0139 2191-0278 SP - 1-3 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://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=psyh&AN=2019-56920-001&site=ehost-live&scope=site karin.duplessis@mcri.edu.au VL - 31 ID - 5816 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 - OEmbase 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 - 3602 ER - TY - JOUR AB - Nail bed avulsion injuries often require reconstruction, particularly in cases where the avulsed fragment is lost. We describe a simple way to reconstruct a large nail bed defect, with no donor site. A 13-year-old boy with a hypoplastic left heart and autism accidentally sustained a left little distal phalanx injury with an avulsion of 60% of the nail bed, exposing the distal phalanx. This boy had a history of poor compliance, qualifying the need to find a technique that would minimise operative time and dressing changes. As such, it was elected to use an acellular dermal matrix (ADM) (Matriderm) as a one-step reconstruction. Signs of vascularisation of the ADM were noted at 2 weeks, and 3-month follow-up demonstrated integration, with normal nail growth. We found that Matriderm was able to support the regeneration of a full thickness wound in a simple one-step procedure. AD - A.L. Kiely, Department of Plastic and Reconstructive Surgery, Queen Elizabeth Hospital, Birmingham, United Kingdom AU - Kiely, A. L. AU - Cooper, L. R. L. AU - Greig, A. DB - UEmbase DO - 10.1136/bcr-2020-236253 IS - 9 KW - acellular dermal matrix Matriderm polyglactin suture surgical implant template wound dressing antibiotic agent acellular dermal matrix reconstruction adolescent article autism case report clinical article finger tip injury follow up human hypoplastic left heart syndrome male nail bed nail infection outcome assessment priority journal skin surgery tetanus Vicryl Rapide LA - English M3 - Article N1 - L632902146 2020-10-08 2020-11-17 PY - 2020 SN - 1757-790X ST - Acellular dermal matrix reconstruction of a nail bed avulsion in a 13-year-old child T2 - BMJ Case Reports TI - Acellular dermal matrix reconstruction of a nail bed avulsion in a 13-year-old child UR - https://www.embase.com/search/results?subaction=viewrecord&id=L632902146&from=export http://dx.doi.org/10.1136/bcr-2020-236253 VL - 13 ID - 4394 ER - TY - JOUR AB - Neurodevelopmental abnormalities are the most common noncardiac complications in patients with congenital heart disease (CHD). Prenatal brain abnormalities may be due to reduced oxygenation, genetic factors, or less commonly, teratogens. Understanding the contribution of these factors is essential to improve outcomes. Because primary sulcal patterns are prenatally determined and under strong genetic control, we hypothesized that they are influenced by genetic variants in CHD. In this study, we reveal significant alterations in sulcal patterns among subjects with single ventricle CHD (n = 115, 14.7 ± 2.9 years [mean ± standard deviation]) compared with controls (n = 45, 15.5 ± 2.4 years) using a graph-based pattern-analysis technique. Among patients with CHD, the left hemisphere demonstrated decreased sulcal pattern similarity to controls in the left temporal and parietal lobes, as well as the bilateral frontal lobes. Temporal and parietal lobes demonstrated an abnormally asymmetric left-right pattern of sulcal basin area in CHD subjects. Sulcal pattern similarity to control was positively correlated with working memory, processing speed, and executive function. Exome analysis identified damaging de novo variants only in CHD subjects with more atypical sulcal patterns. Together, these findings suggest that sulcal pattern analysis may be useful in characterizing genetically influenced, atypical early brain development and neurodevelopmental risk in subjects with CHD. AD - S.U. Morton, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, United States K. Im, Boston Children's Hospital, 401 Park Dr, Boston, MA, United States AU - Morton, S. U. AU - Maleyeff, L. AU - Wypij, D. AU - Yun, H. J. AU - Newburger, J. W. AU - Bellinger, D. C. AU - Roberts, A. E. AU - Rivkin, M. J. AU - Seidman, J. G. AU - Seidman, C. E. AU - Grant, P. E. AU - Im, K. DB - UEmbase DO - 10.1093/cercor/bhz101 IS - 2 KW - adolescent adult article brain development child cohort analysis congenital heart disease controlled study executive function exome female frontal lobe genetic variability heart single ventricle human major clinical study male mental disease nuclear magnetic resonance imaging parietal lobe priority journal processing speed risk factor whole exome sequencing working memory LA - English M3 - Article N1 - L633117547 2020-10-21 2020-11-30 PY - 2020 SN - 1460-2199 1047-3211 SP - 476-487 ST - Abnormal Left-Hemispheric Sulcal Patterns Correlate with Neurodevelopmental Outcomes in Subjects with Single Ventricular Congenital Heart Disease T2 - Cerebral Cortex TI - Abnormal Left-Hemispheric Sulcal Patterns Correlate with Neurodevelopmental Outcomes in Subjects with Single Ventricular Congenital Heart Disease UR - https://www.embase.com/search/results?subaction=viewrecord&id=L633117547&from=export http://dx.doi.org/10.1093/cercor/bhz101 VL - 30 ID - 4427 ER - TY - JOUR AU - Paul, E. A. AU - Hsu, D. T. DB - UEmbase DO - 10.1016/j.jpeds.2020.02.041 KW - prostaglandin E1 arterial switch operation article cardiovascular risk congenital heart disease disease severity heart catheterization heart surgery human hypoplastic left heart syndrome long term care lung vein drainage anomaly overall survival priority journal quality of life surgical approach transthoracic echocardiography LA - English M3 - Article N1 - L2007492913 2020-08-21 2020-08-28 PY - 2020 SN - 1097-6833 0022-3476 SP - 50 ST - 50 Years Ago in THE JOURNAL OF PEDIATRICS: Impact of Technology and Innovation on Congenital Heart Disease Survival T2 - Journal of Pediatrics TI - 50 Years Ago in THE JOURNAL OF PEDIATRICS: Impact of Technology and Innovation on Congenital Heart Disease Survival UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2007492913&from=export http://dx.doi.org/10.1016/j.jpeds.2020.02.041 VL - 224 ID - 4396 ER - TY - JOUR AD - B. Eidem AU - Eidem, B. AU - Mertens, L. DB - UEmbase DO - 10.1016/j.echo.2013.01.005 IS - 3 KW - childhood disease clinical assessment clinical assessment tool clinical decision making congenital heart disease diastolic dysfunction health program heart atrium septum defect heart right ventricle double outlet heart single ventricle human hypoplastic left heart syndrome interpersonal communication life event lung circulation palliative therapy pathologist protein losing gastroenteropathy short survey surgeon three dimensional echocardiography LA - English M3 - Editorial N1 - L368455664 2013-03-13 2013-03-21 PY - 2013 SN - 1097-6795 0894-7317 SP - 40A ST - The 24th annual ASE scientific sessions - The pediatric & congenital heart disease program is a "can't miss" event for 2013! T2 - Journal of the American Society of Echocardiography TI - The 24th annual ASE scientific sessions - The pediatric & congenital heart disease program is a "can't miss" event for 2013! UR - https://www.embase.com/search/results?subaction=viewrecord&id=L368455664&from=export http://dx.doi.org/10.1016/j.echo.2013.01.005 VL - 26 ID - 4898 ER -