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for examination of the transition following the second palliative heart surgery, when the home monitoring program is withdrawn.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. AU - Elliott, M. AU - Erickson, L. AU - Russell, C. L. AU - Chrisman, M. AU - Gross Toalson, J. AU - Emerson, A. DB - UEmbase DO - 10.1111/jan.14785 IS - 5 KW - article child exploratory research heart single ventricle heart surgery home monitoring human human tissue infant interview naturalistic inquiry nursing palliative therapy physiological stress psychosocial care purposive sample surgery theoretical study LA - English M3 - Article N1 - L634295522 2021-03-02 PY - 2021 SN - 1365-2648 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 - Journal of advanced nursing TI - Defining a new normal: A qualitative exploration of the parent experience during the single ventricle congenital heart disease interstage period UR - https://www.embase.com/search/results?subaction=viewrecord&id=L634295522&from=export http://dx.doi.org/10.1111/jan.14785 VL - 77 ID - 4343 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. AU - Hammerness, P. G. AU - Cassidy, A. R. AU - Potts, H. AU - Richardson, A. AU - Fogler, J. AU - Daly, K. P. AU - Augustyn, M. DB - UEmbase DO - 10.1097/DBP.0000000000000961 IS - 4 KW - adult anticoagulation article attention deficit disorder cardiac rhythm management device cerebrovascular accident child cognition cognitive behavioral therapy complication disorientation exercise family history habit heart arrhythmia heart infarction high school human hypoplastic left heart syndrome infancy interview major depression male mental health music New York Heart Association class outpatient palliative therapy psychiatry remission risk assessment self care self report sleep systolic heart failure time management young adult serotonin uptake inhibitor LA - English M3 - Article N1 - L634816376 2021-04-27 PY - 2021 SN - 1536-7312 SP - 340-342 ST - Assessment and Treatment of a Young Adult with Congenital Heart Disease and ADHD T2 - Journal of developmental and behavioral pediatrics : JDBP TI - Assessment and Treatment of a Young Adult with Congenital Heart Disease and ADHD UR - https://www.embase.com/search/results?subaction=viewrecord&id=L634816376&from=export http://dx.doi.org/10.1097/DBP.0000000000000961 VL - 42 ID - 4344 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 - D. Celermajer, Cardiology Department, Royal Prince Alfred Hospital, 50 Missenden Road, Camperdown, NSW, Australia AU - Rehan, R. AU - Kotchetkova, I. AU - Cordina, R. AU - Celermajer, D. DB - UEmbase DO - 10.1016/j.hlc.2020.05.114 IS - 2 KW - adult anxiety aortic coarctation aortic valve article cerebrovascular disease complication congenital heart disease controlled study depression descending aortic surgery disease burden drug therapy Ebstein anomaly endocarditis Fallot tetralogy female heart arrhythmia heart right ventricle heart single ventricle human hypertension implantable cardioverter defibrillator major clinical study male mental health radiofrequency ablation reoperation surgery survivor antiarrhythmic agent LA - English M3 - Article N1 - L2007164137 2020-08-04 PY - 2021 SN - 1444-2892 1443-9506 SP - 261-266 ST - Adult Congenital Heart Disease Survivors at Age 50 Years: Medical and Psychosocial Status T2 - Heart Lung and Circulation TI - Adult Congenital Heart Disease Survivors at Age 50 Years: Medical and Psychosocial Status UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2007164137&from=export http://dx.doi.org/10.1016/j.hlc.2020.05.114 VL - 30 ID - 4351 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 - J.W. Newburger, Department of Cardiology, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, United States 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. DB - UEmbase DO - 10.1016/j.jpeds.2020.09.066 KW - article behavior Blalock Taussig shunt child controlled study educational status female follow up functional status functional status assessment head circumference heart single ventricle human hypoplastic left heart syndrome interpersonal communication major clinical study male maternal attitude nerve cell differentiation nervous system development newborn Norwood procedure patient-reported outcome pediatric patient Pediatric Quality of Life Inventory priority journal problem solving quality of life right ventricle to pulmonary artery conduit social interaction social psychology social status LA - English M3 - Article N1 - L2010040260 2020-11-25 2021-02-12 PY - 2021 SN - 1097-6833 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 - Journal of Pediatrics 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 UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2010040260&from=export http://dx.doi.org/10.1016/j.jpeds.2020.09.066 VL - 229 ID - 4353 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 - N.A. Pike, UCLA School of Nursing, University of California Los Angeles, Los Angeles, CA, United States 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. DB - UEmbase DO - 10.1002/brb3.1977 IS - 2 KW - nuclear magnetic resonance scanner adolescent adult amygdala anxiety article Beck Anxiety Inventory brain size cerebrospinal fluid cognition assessment congenital heart disease controlled study dentate hilus depression female Fourier transform heart single ventricle heart ventricle hippocampus human image analysis image segmentation learning major clinical study male memory assessment Montreal cognitive assessment nuclear magnetic resonance imaging oxygen saturation Patient Health Questionnaire 9 pterygoid muscle visual memory working memory LA - English M3 - Article N1 - L2010149317 2021-01-13 PY - 2021 SN - 2162-3279 ST - Reduced hippocampal volumes and memory deficits in adolescents with single ventricle heart disease T2 - Brain and Behavior TI - Reduced hippocampal volumes and memory deficits in adolescents with single ventricle heart disease UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2010149317&from=export http://dx.doi.org/10.1002/brb3.1977 VL - 11 ID - 4354 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 - V.J. Gonzalez, Department of Pediatrics, Section of Pediatric Cardiology, Texas Children's Hospital, 6651 Main St, Legacy Tower, E1920, Houston, TX, United States 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. DB - UEmbase DO - 10.1542/peds.2020-1693 IS - 2 KW - adolescent anxiety article attention deficit disorder child controlled study cross-sectional study depression drug combination drug therapy electronic medical record emergency ward female heart arrhythmia heart single ventricle hospitalization human juvenile major clinical study male medically uninsured outcome assessment outcome variable predictor variable tertiary care center benzodiazepine derivative clonidine LA - English M3 - Article N1 - L2011108091 2021-03-02 PY - 2021 SN - 1098-4275 0031-4005 ST - Mental health disorders in children with congenital heart disease T2 - Pediatrics TI - Mental health disorders in children with congenital heart disease UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2011108091&from=export http://dx.doi.org/10.1542/peds.2020-1693 VL - 147 ID - 4355 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 childs 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 childs 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 - E.D. Blume, Department of Cardiology, Boston Childrens Hospital, Boston, MA, United States 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. DB - UEmbase DO - 10.1161/JAHA.120.018488 IS - 2 KW - adult article care behavior child congenital heart disease controlled study coronary care unit disease burden female heart single ventricle hospitalized child human infant kappa statistics learning life expectancy major clinical study male outcome assessment patient-reported outcome pediatrics physical activity prognosis young adult LA - English M3 - Article N1 - L2005975020 2021-02-19 PY - 2021 SN - 2047-9980 SP - 1-11 ST - Parent and physician understanding of prognosis in hospitalized children with advanced heart disease T2 - Journal of the American Heart Association TI - Parent and physician understanding of prognosis in hospitalized children with advanced heart disease UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2005975020&from=export http://dx.doi.org/10.1161/JAHA.120.018488 VL - 10 ID - 4359 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. AU - Mun, Y. S. AU - Sung, W. Y. DB - UEmbase DO - 10.1097/MD.0000000000024299 IS - 2 KW - blunt trauma case report complication false aneurysm female human kidney vein young adult LA - English M3 - Article N1 - L634073890 2021-02-08 2021-02-10 PY - 2021 SN - 1536-5964 SP - e24299 ST - A rare case report of renal vein pseudoaneurysm after blunt trauma T2 - Medicine TI - A rare case report of renal vein pseudoaneurysm after blunt trauma UR - https://www.embase.com/search/results?subaction=viewrecord&id=L634073890&from=export http://dx.doi.org/10.1097/MD.0000000000024299 VL - 100 ID - 4361 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 - E. Ross, Department of Pediatric Cardiology, Advocate Children’s Hospital, Oaklawn, IL, United States AU - Zaidi, S. J. AU - Ross, E. AU - Gandhi, R. AU - Li, Y. AU - Penk, J. DB - UEmbase DO - 10.1007/s00246-021-02539-8 KW - article cardiologist child clinical article clinical assessment clinical outcome cohort analysis controlled study coronary care unit echocardiography female Glenn shunt heart right ventricle heart single ventricle heart ventricle function hospitalization human hypoplastic left heart syndrome intrarater reliability male medical record review mortality prediction quantitative analysis retrospective study surgery survivor transplantation diamide LA - English M3 - Article in Press N1 - L2010313716 2021-02-03 PY - 2021 SN - 1432-1971 0172-0643 ST - Comparison of Quantitative Versus Qualitative Assessment of Single Right Ventricular Function for the Prediction of Midterm Outcomes T2 - Pediatric Cardiology TI - Comparison of Quantitative Versus Qualitative Assessment of Single Right Ventricular Function for the Prediction of Midterm Outcomes UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2010313716&from=export http://dx.doi.org/10.1007/s00246-021-02539-8 ID - 4365 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 - C. Attard, Murdoch Children's Research Institute, Royal Children's Hospital, Flemington Rd, Parkville, Australia 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. AU - Mandelstam, S. AU - Munns, C. AU - Gentles, T. AU - Grigg, L. AU - Winlaw, D. AU - Yang, J. Y. M. DB - UEmbase DO - 10.1016/j.jtcvs.2020.12.102 KW - article Australia and New Zealand bleeding bone density cerebrovascular accident child clinical feature cohort analysis controlled study dual energy X ray absorptiometry female heart function heart single ventricle human international normalized ratio lung function major clinical study male multicenter study nuclear magnetic resonance imaging Pediatric Quality of Life Inventory quality of life questionnaire thrombosis prevention white matter injury acetylsalicylic acid warfarin LA - English M3 - Article in Press N1 - L2010942680 2021-02-15 PY - 2021 SN - 1097-685X 0022-5223 ST - Long-term outcomes of warfarin versus aspirin after Fontan surgery T2 - Journal of Thoracic and Cardiovascular Surgery TI - Long-term outcomes of warfarin versus aspirin after Fontan surgery UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2010942680&from=export http://dx.doi.org/10.1016/j.jtcvs.2020.12.102 ID - 4367 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 - K.R. Wolfe, 13123 E 16th Ave, B155, Aurora, CO, United States 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. DB - UEmbase DO - 10.1016/j.jpeds.2020.07.043 KW - adaptive behavior adolescent adult arterial oxygen tension article attention cerebrovascular accident child cohort analysis comparative study controlled study cross-sectional study demography echocardiography elastography executive function female follow up Fontan procedure heart atrioventricular valve heart single ventricle human intelligence quotient length of stay lung disease major clinical study male neuropsychological test outcome assessment priority journal prospective study seizure self monitoring sleep disordered breathing visuomotor coordination Wechsler intelligence scale for children LA - English M3 - Article N1 - L2007636906 2020-09-07 2021-04-26 PY - 2020 SN - 1097-6833 0022-3476 SP - 239-246 ST - Relationships between Physiologic and Neuropsychologic Functioning after Fontan T2 - Journal of Pediatrics TI - Relationships between Physiologic and Neuropsychologic Functioning after Fontan UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2007636906&from=export http://dx.doi.org/10.1016/j.jpeds.2020.07.043 VL - 227 ID - 4377 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 mm3, p < 0.004; right, 3,162.1 ± 475.4 vs. 3,504.8 ± 465.9 mm3, 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 - R. Kumar, Department of Anesthesiology, David Geffen School of Medicine at UCLA, University of California Los Angeles, Los Angeles, CA, United States R. Kumar, Department of Radiological Sciences, University of California Los Angeles, Los Angeles, CA, United States R. Kumar, Department of Bioengineering, University of California Los Angeles, Los Angeles, CA, United States R. Kumar, Brain Research Institute, University of California Los Angeles, Los Angeles, CA, United States 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. DB - UEmbase DO - 10.1002/jnr.24667 IS - 10 KW - imaging software nuclear magnetic resonance scanner adolescent anxiety disorder article attention Beck Anxiety Inventory brain region brain size caudate nucleus cerebrospinal fluid clinical article cognition comparative study conceptual thinking controlled study depression dorsolateral striatum executive function female General Memory Index gray matter heart single ventricle human language left caudate nucleus male memory memory assessment Montreal cognitive assessment mood disorder morphometry neuroimaging nuclear magnetic resonance imaging orientation Patient Health Questionnaire 9 priority journal right caudate nucleus verbal memory visual memory white matter Wide Range Assessment of Memory and Learning 2 working memory Magnetom Prisma statistical parametric mapping LA - English M3 - Article N1 - L2005206023 2020-07-01 PY - 2020 SN - 1097-4547 0360-4012 SP - 1877-1888 ST - Caudate nuclei volume alterations and cognition and mood dysfunctions in adolescents with single ventricle heart disease T2 - Journal of Neuroscience Research TI - Caudate nuclei volume alterations and cognition and mood dysfunctions in adolescents with single ventricle heart disease UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2005206023&from=export http://dx.doi.org/10.1002/jnr.24667 VL - 98 ID - 4388 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 - V.S. Kiran, Departments of Paediatric Cardiology, Narayana Institute Of Cardiovascular Sciences, Narayana Health City, 258/A, Bommasandra, Hosur Road, Bangalore, India AU - Kiran, V. S. AU - Choudhary, S. AU - Shaik, A. AU - Gadabanahalli, K. AU - Raj, V. AU - Bhat, V. DB - UEmbase DO - 10.1007/s00246-020-02402-2 IS - 7 KW - anatomical variation article atrioventricular canal child clinical evaluation cohort analysis computer assisted tomography congenital heart malformation controlled study cross-sectional study disease association disease duration echocardiography female heterotaxy syndrome human image analysis infant major clinical study male preschool child quality of life retrospective study treatment outcome LA - English M3 - Article N1 - L2005253425 2020-07-06 PY - 2020 SN - 1432-1971 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 - Pediatric Cardiology TI - The Spectrum of Cardiac Anomalies Associated with Heterotaxy: A Single-Center Study of a Large Series Based on Computed Tomography UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2005253425&from=export http://dx.doi.org/10.1007/s00246-020-02402-2 VL - 41 ID - 4389 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 - A. Patel, Section of Cardiology, Ann & Robert H. Lurie Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, 225 E. Chicago Ave., Chicago, IL, United States AU - Swanson, T. M. AU - Patel, A. AU - Baxter, A. J. AU - Morris, S. A. AU - Maskatia, S. A. AU - Lantos, J. D. DB - UEmbase DO - 10.1007/s00246-020-02367-2 IS - 6 KW - aortic coarctation article atrioventricular canal cardiac surgeon cardiologist congenital heart disease consensus controlled study Fallot tetralogy great vessels transposition health survey heart single ventricle heart surgery heart ventricle septum defect human lung vein drainage anomaly medical care medical expert medicolegal aspect mortality rate parental attitude patient care pediatric cardiology pediatric hospital pediatric surgeon pediatrician physician attitude surgical mortality tertiary care center treatment refusal trisomy 13 trisomy 18 LA - English M3 - Article N1 - L2004975971 2020-05-28 2020-10-29 PY - 2020 SN - 1432-1971 0172-0643 SP - 1160-1165 ST - Pediatric Cardiology Specialist’s Opinions Toward the Acceptability of Comfort Care for Congenital Heart Disease T2 - Pediatric Cardiology TI - Pediatric Cardiology Specialist’s Opinions Toward the Acceptability of Comfort Care for Congenital Heart Disease UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2004975971&from=export http://dx.doi.org/10.1007/s00246-020-02367-2 VL - 41 ID - 4401 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 - N. Goldenberg-Cohen, Krieger Eye Research Laboratory, Felsenstein Medical Research Center, Rabin Medical Center, Petach Tikva, Israel N. Goldenberg-Cohen, Department of Ophthalmology, Bnai Zion Medical Center, Haifa, Israel N. Goldenberg-Cohen, Faculty of Medicine, Technion – Israel Institute of Technology, Haifa, Israel AU - Luckman, J. AU - Chokron, S. AU - Michowiz, S. AU - Belenky, E. AU - Toledano, H. AU - Zahavi, A. AU - Goldenberg-Cohen, N. DB - UEmbase DO - 10.3389/fneur.2020.00617 KW - adolescent article blindness case study cerebrovascular accident child child health care childhood disease clinical article cohort analysis congenital blood vessel malformation female Fontan procedure head injury heart disease hemianopia human hypercoagulability male metabolic syndrome X ophthalmologist retrospective study role playing visual disorder visual field defect LA - English M3 - Article N1 - L632401923 2020-07-30 2020-08-12 PY - 2020 SN - 1664-2295 ST - The Need to Look for Visual Deficit After Stroke in Children T2 - Frontiers in Neurology TI - The Need to Look for Visual Deficit After Stroke in Children UR - https://www.embase.com/search/results?subaction=viewrecord&id=L632401923&from=export http://dx.doi.org/10.3389/fneur.2020.00617 VL - 11 ID - 4402 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 - J. Moosmann, Department of Pediatric Cardiology, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany AU - Moosmann, J. AU - Dittrich, S. AU - Purbojo, A. AU - Cesnjevar, R. DB - UEmbase DO - 10.1111/jocs.14658 IS - 7 KW - dacron implant ExCor-System right ventricular assist device adult article cardiovascular magnetic resonance case report clinical article diastolic dysfunction end to end anastomosis female Fontan procedure general condition deterioration Glenn shunt heart catheterization heart transplantation heart valve replacement hospitalization human palliative therapy pleura effusion protein losing gastroenteropathy quality of life young adult LA - English M3 - Article N1 - L2005426004 2020-07-14 2020-07-22 PY - 2020 SN - 1540-8191 0886-0440 SP - 1721-1724 ST - RVAD implantation in a Fontan patient with protein-losing enteropathy as a bridge to transplant: Prêtre modification T2 - Journal of Cardiac Surgery TI - RVAD implantation in a Fontan patient with protein-losing enteropathy as a bridge to transplant: Prêtre modification UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2005426004&from=export http://dx.doi.org/10.1111/jocs.14658 VL - 35 ID - 4403 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 - T. Yoshida, Division of Neonatology, Maternal and Perinatal Center, Toyama University Hospital, Toyama, Japan 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 - UEmbase DO - 10.1111/ped.14160 IS - 7 KW - article Bayley Scales of Infant Development congenital heart disease controlled study developmental delay disease association female gestational age human infant language ability major clinical study male motor performance nervous system development priority journal risk factor very low birth weight LA - English M3 - Article N1 - L2005430244 2020-07-14 2020-08-11 PY - 2020 SN - 1442-200X 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 - https://www.embase.com/search/results?subaction=viewrecord&id=L2005430244&from=export http://dx.doi.org/10.1111/ped.14160 VL - 62 ID - 4404 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. 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. DB - UEmbase DO - 10.1038/s41598-020-65852-x IS - 1 KW - amino acid adverse event blood case control study congenital heart malformation coronary artery blood flow female Fontan procedure heart left ventricle function human male metabolism metabolomics oxidative stress physiology young adult LA - English M3 - Article N1 - L631976053 2020-06-10 2021-01-01 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 - Scientific reports TI - Targeted metabolomic analysis of serum amino acids in the adult Fontan patient with a dominant left ventricle UR - https://www.embase.com/search/results?subaction=viewrecord&id=L631976053&from=export http://dx.doi.org/10.1038/s41598-020-65852-x VL - 10 ID - 4407 ER - TY - JOUR AB - Children and adolescents with cardiac disease (CCD) have significant morbidity and lower quality of life. However, there are no broadly applicable tools similar to the frailty score as described in the elderly, to define functional phenotype in terms of physical capability and psychosocial wellbeing in CCD. The purpose of this study is to investigate the domains of the frailty in CCD. We prospectively recruited CCD (8–17.5 years old, 70% single ventricle, 27% heart failure, 12% pulmonary hypertension; NYHA classes I, II and III) and age and gender matched healthy controls (total n = 56; CCD n = 34, controls n = 22; age 12.6 ± 2.6 years; 39.3% female). We measured the five domains of frailty: slowness, weakness, exhaustion, body composition and physical activity using developmentally appropriate methods. Age and gender-based population norms were used to obtain Z scores and percentiles for each measurement. Two-tailed t-tests were used to compare the two groups. The CCD group performed significantly worse in all five domains of frailty compared to healthy controls. Slowness: 6-min walk test with Z score −3.9 ± 1.3 vs −1.4 ± 1.3, p < 0.001; weakness: handgrip strength percentile 18.9 ± 20.9 vs 57.9 ± 26.0, p < 0.001; exhaustion: multidimensional fatigue scale percentile 63.7 ± 13.5 vs 83.3 ± 14.4, p < 0.001; body composition: height percentile 43.4 ± 29.5 vs 71.4 ± 25.2, p < 0.001, weight percentile 46.0 ± 36.0 vs 70.9 ± 24.3, p = 0.006, BMI percentile 48.4 ± 35.5 vs 66.9 ± 24.2, p = 0.04, triceps skinfold thickness 41.0 ± 24.0 vs 54.4 ± 22.1, p = 0.04; physical activity: pediatric activity questionnaire score 2 ± 0.6 vs 2.7 ± 0.6, p < 0.001. The domains of frailty can be quantified in children using developmentally appropriate methods. CCD differ significantly from controls in all five domains, supporting the concept of quantifying the domains of frailty. Larger longitudinal studies are needed to study frailty in CCD and examine if it predicts adverse health outcomes. Clinical Trial Registration: The ClinicalTrials.gov identification number is NCT02999438. https://clinicaltrials.gov/ct2/show/NCT02999438. AD - C. Panchangam, University of Missouri-Columbia, 500 N Keene St, Suite 207, Columbia, MO, United States AU - Panchangam, C. AU - White, D. A. AU - Goudar, S. AU - Birnbaum, B. AU - Malloy-Walton, L. AU - Gross-Toalson, J. AU - Reid, K. J. AU - Shirali, G. AU - Parthiban, A. DB - UEmbase DO - 10.1007/s00246-020-02354-7 IS - 5 KW - NCT02999438 accelerometer ActiGraph wGT3X-BT personal digital assistant adolescent article body composition child controlled study female frailty grip strength hand grip health insurance heart disease heart failure household human major clinical study male multidimensional fatigue scale New York Heart Association class physical activity prediction prospective study psychosocial care pulmonary hypertension quality of life rating scale six minute walk test translational research LA - English M3 - Article N1 - L2004853080 2020-05-26 2020-07-14 PY - 2020 SN - 1432-1971 0172-0643 SP - 1031-1041 ST - Translation of the Frailty Paradigm from Older Adults to Children with Cardiac Disease T2 - Pediatric Cardiology TI - Translation of the Frailty Paradigm from Older Adults to Children with Cardiac Disease UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2004853080&from=export http://dx.doi.org/10.1007/s00246-020-02354-7 VL - 41 ID - 4411 ER - TY - JOUR AB - Background: Growth abnormalities in single-ventricle survivors may reduce quality of life (QoL) and exercise capacity. Methods: This multicenter, longitudinal analysis evaluated changes in height and body mass index (BMI) compared to population norms and their relationship to mortality, ventricular morphology, QoL, and exercise capacity in the Pediatric Heart Network Fontan studies. Results: Fontan 1 (F1) included 546 participants (12 ± 3.4 years); Fontan 2 (F2), 427 (19 ± 3.4 years); and Fontan 3 (F3), 362 (21 ± 3.5 years), with ~60% male at each time point. Height z-score was −0.67 ± −1.27, −0.60 ± 1.34, and− 0.43 ± 1.14 at F1-F3, lower compared to norms at all time points (P ≤ .001). BMI z-score was similar to population norms. Compared to survivors, participants who died had lower height z-score (P ≤ .001). Participants with dominant right ventricle (n = 112) had lower height z-score (P ≤ .004) compared to dominant left (n = 186) or mixed (n = 64) ventricular morphologies. Higher height z-score was associated with higher Pediatric Quality of Life Inventory for the total score (slope = 2.82 ± 0.52; P ≤ .001). Increase in height z-score (F1 to F3) was associated with increased oxygen consumption (slope = 2.61 ± 1.08; P = .02), whereas, for participants >20 years old, an increase in BMI (F1 to F3) was associated with a decrease in oxygen consumption (slope = −1.25 ± 0.33; P ≤ .001). Conclusions: Fontan survivors, especially those with right ventricular morphology, are shorter when compared to the normal population but have similar BMI. Shorter stature was associated with worse survival. An increase in height z-score over the course of the study was associated with better QoL and exercise capacity; an increase in BMI was associated with worse exercise capacity. AD - L.M. Lambert, Administrative Director Research-PCH Heart Center, University of Utah, 81 N Mario Capecchi Dr, Salt Lake City, UT, United States AU - Lambert, L. M. AU - McCrindle, B. W. AU - Pemberton, V. L. AU - Hollenbeck-Pringle, D. AU - Atz, A. M. AU - Ravishankar, C. AU - Campbell, M. J. AU - Dunbar-Masterson, C. AU - Uzark, K. AU - Rolland, M. AU - Trachtenberg, F. L. AU - Menon, S. C. DB - UEmbase DO - 10.1016/j.ahj.2020.03.022 KW - anthropometry article body height body mass body weight child controlled study exercise female heart right ventricle human longitudinal study major clinical study male mortality multicenter study oxygen consumption Pediatric Quality of Life Inventory quality of life survivor LA - English M3 - Article N1 - L2005884738 2020-05-20 PY - 2020 SN - 1097-6744 0002-8703 SP - 192-200 ST - Longitudinal study of anthropometry in Fontan survivors: Pediatric Heart Network Fontan study T2 - American Heart Journal TI - Longitudinal study of anthropometry in Fontan survivors: Pediatric Heart Network Fontan study UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2005884738&from=export http://dx.doi.org/10.1016/j.ahj.2020.03.022 VL - 224 ID - 4413 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 - M.J. Saavedra, Department of Pediatrics, Division of General Pediatrics, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina M.J. Saavedra, Department of Pediatrics, Division of Pediatric Cardiology, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina AU - Saavedra, M. J. AU - Eymann, A. AU - Pérez, L. AU - Busaniche, J. AU - Nápoli, N. AU - Marantz, P. AU - Llera, J. DB - UEmbase DO - 10.5546/aap.2020.eng.166 IS - 3 KW - article child clinical article congenital heart disease controlled study cross-sectional study heart surgery human newborn period observational study pediatric patient preschool child quality of life school scoring system social aspect LA - English M3 - Article N1 - L2006767639 2020-10-12 2020-10-19 PY - 2020 SN - 1668-3501 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 - Archivos Argentinos de Pediatria TI - Health related quality of life in children with congenital heart disease that undergo cardiac surgery during their first year of life UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2006767639&from=export http://dx.doi.org/10.5546/aap.2020.eng.166 VL - 118 ID - 4414 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 (Z0) 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 Z0 of the legs and kidneys, resulting in greater systemic Z0 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 - J.A. Hauser, Centre for Translational Cardiovascular Imaging, University College London, London, United Kingdom AU - Hauser, J. A. AU - Jones, A. AU - Pandya, B. AU - Taylor, A. M. AU - Muthurangu, V. DB - UEmbase DO - 10.1152/AJPHEART.00500.2020 IS - 4 KW - adolescent adult artery resistance article blood flow cardiovascular response clinical assessment controlled study fasting female fontanel food intake heart afterload heart output hemodynamics human human experiment male mean arterial pressure mesenteric artery nuclear magnetic resonance imaging postprandial state priority journal LA - English M3 - Article N1 - L2008426565 2020-11-06 2020-12-15 PY - 2020 SN - 1522-1539 0363-6135 SP - H808-H813 ST - Comprehensive MRI assessment of the cardiovascular responses to food ingestion in Fontan physiology T2 - American Journal of Physiology - Heart and Circulatory Physiology TI - Comprehensive MRI assessment of the cardiovascular responses to food ingestion in Fontan physiology UR - https://www.embase.com/search/results?subaction=viewrecord&id=L2008426565&from=export http://dx.doi.org/10.1152/AJPHEART.00500.2020 VL - 319 ID - 4445 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 - A.S. Harahsheh, Department of Pediatrics, George Washington University School of Medicine, Washington, DC, United States AU - Alkofair, B. AU - Alruwaili, A. AU - Gai, J. AU - Harahsheh, A. S. DB - UEmbase DO - 10.1017/S1047951120001985 KW - article child controlled study demography female follow up Fontan procedure health economics heart single ventricle heart surgery hospital admission human length of stay major clinical study male mortality protein losing gastroenteropathy retrospective study standardization surgery transplant free survival wellbeing digoxin LA - English M3 - Article in Press N1 - L633208327 2020-10-29 PY - 2020 SN - 1467-1107 1047-9511 ST - Impact of protein-losing enteropathy in children who underwent the Fontan operation T2 - Cardiology in the Young TI - Impact of protein-losing enteropathy in children who underwent the Fontan operation UR - https://www.embase.com/search/results?subaction=viewrecord&id=L633208327&from=export http://dx.doi.org/10.1017/S1047951120001985 ID - 4452 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 - J.K. Peterson, Johns Hopkins University School of Nursing, Baltimore, MD, United States AU - Peterson, J. K. AU - Olshansky, E. F. AU - Guo, Y. AU - Evangelista, L. S. AU - Pike, N. A. DB - UEmbase DO - 10.1017/S1047951120003790 KW - academic achievement adolescent adult article bullying clinical article clinical feature demography elephant female Fontan procedure heart single ventricle human male medical record review nonhuman optimism peer group qualitative research semi structured interview social interaction social support uncertainty LA - English M3 - Article in Press N1 - L633389702 2020-11-18 PY - 2020 SN - 1467-1107 1047-9511 ST - Optimism despite profound uncertainty: School and social relationships in adolescents with single ventricle heart disease T2 - Cardiology in the Young TI - Optimism despite profound uncertainty: School and social relationships in adolescents with single ventricle heart disease UR - https://www.embase.com/search/results?subaction=viewrecord&id=L633389702&from=export http://dx.doi.org/10.1017/S1047951120003790 ID - 4453 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 - 149651721. Language: English. Entry Date: 20210411. Revision Date: 20210419. Publication Type: Article AU - Elliott, Melissa AU - Erickson, Lori AU - Russell, Cynthia L. AU - Chrisman, Matthew AU - Gross Toalson, Jami AU - Emerson, Amanda DB - Ucinahl DO - 10.1111/jan.14785 DP - EBSCOhost IS - 5 KW - Parental Attitudes Heart Ventricle -- Abnormalities Heart Defects, Congenital Heart Surgery -- In Infancy and Childhood Human Infant Female Male Exploratory Research Naturalistic Inquiry Qualitative Studies Descriptive Research Purposive Sample Interviews Field Studies Telehealth Eligibility Determination Interview Guides Field Notes Data Analysis Software Thematic Analysis N1 - research; tables/charts. 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 - Journal of Advanced Nursing (John Wiley & Sons, Inc.) TI - Defining a new normal: A qualitative exploration of the parent experience during the single ventricle congenital heart disease interstage period UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=149651721&site=ehost-live&scope=site VL - 77 ID - 5302 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 Department of Sociology, Rice University Houston, Texas AN - 148485000. Language: English. Entry Date: 20210208. Revision Date: 20210211. Publication Type: Article AU - Gonzalez, Vincent J. AU - Kimbro, Rachel T. AU - Cutitta, Katherine E. AU - Shabosky, John C. AU - Bilal, Mohammad F. AU - Penny, Daniel J. AU - Lopez, Keila N. DB - Ucinahl DO - 10.1542/peds.2020-1693 DP - EBSCOhost IS - 2 KW - Mental Disorders -- In Infancy and Childhood Heart Defects, Congenital -- Complications Human Cross Sectional Studies Comparative Studies Odds Ratio Confidence Intervals Child, Preschool Child Adolescence N1 - research; tables/charts. Journal Subset: Biomedical; Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. NLM UID: 0376422. PY - 2021 SN - 0031-4005 SP - 1-10 ST - Mental Health Disorders in Children With Congenital Heart Disease T2 - Pediatrics TI - Mental Health Disorders in Children With Congenital Heart Disease UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=148485000&site=ehost-live&scope=site VL - 147 ID - 5303 ER - TY - JOUR AB - OBJECTIVES: To determine if neurodevelopmental deficits in children with single-ventricle 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 - Department of Pediatrics and Labatt Family Heart Centre, Hospital for Sick Children, University of Toronto, Toronto, Canada Department of Pediatrics, Michigan Medicine, Medical School, University of Michigan, Ann Arbor Michigan Department of Cardiology, Boston Children's Hospital and Department of Pediatrics, Harvard Medical School, Harvard University, Boston, Massachusetts New England Research Institutes, Watertown, Massachusetts Division of Pediatric Cardiothoracic Surgery Children's Hospital of Philadelphia, Perelman School of Medicine, Universty of Pennsylvania, Philadelphia, Pennsylvania Department of Pediatrics, Children's Healthcare of Atlanta, Emory University Atlanta, Georgia Department of Pediatrics, Primary Children's Hospital, University of Utah, Salt Lake City Utah Division of Pediatric Cardiology Maine Medical Center Portland, Maine Department of Surgery, Herma Heart Institute, Children's Wisconsin and Medical College of Wisconsin, Milwaukee, Wisconsin Department of Surgery Nemours Cardiac Center Alfred I du Pont Hospital for Children, Wilmington, Delaware The Heart Institute of Florida, St Petersburg, Florida Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio Department of Pediatrics, Medical University of South Carolina, Charleston, South Carolina Children's Heart Institute, Children's Hospital of Orange County Orange, California National Heart, Lung, and Blood Institute, Bethesda, Maryland Department of Pediatrics, School of Medicine, Duke University, Durham, North Carolina Department of Pediatrics, East Carolina University, Greenville, North Carolina Department of Pediatrics, Wake Forest University Winston-Salem, North Carolina Department of Pediatrics, Children 's Hospital Los Angeles, Los Angeles, California Department of Pediatrics, Herma Heart Institute, Children's Wisconsin and Medical College of Wisconsin, Milwaukee, Wisconsin AN - 148485012. Language: English. Entry Date: 20210208. Revision Date: 20210211. Publication Type: Article AU - Sananes, Renee AU - Goldberg, Garen S. AU - Newburger, Jane W. AU - Chenwei, Hu AU - Trachtenberg, Felicia AU - Gaynor, J. William AU - Mahle, William T. AU - Miller, Thomas AU - Uzark, Karen AU - Mussatto, Kathleen A. AU - Pizarro, Christian AU - Jacobs, Jeffrey P. AU - Cnota, James AU - Atz, Andrew M. AU - Lai, Wyman W. AU - Burns, Kristin M. AU - Milazzo, Angelo AU - Votava-Smith, Jodie AU - Brosig, Gheryl L. DB - Ucinahl DO - 10.1542/peds.2020-014589 DP - EBSCOhost IS - 2 KW - Developmental Disabilities Nervous System -- Physiopathology Heart Defects, Congenital Heart Ventricle -- Physiopathology Child Development Human Descriptive Statistics Data Analysis Software Chi Square Test Male Female Child, Preschool Child Scales Questionnaires N1 - research; tables/charts. Journal Subset: Biomedical; Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. Instrumentation: Behavior Assessment System for Children, Second Edition (BASC-2); Bayley Scales of Infant Development, Second Edition (BSID-II); Ages and Stages Questionnaires [ASQ]. NLM UID: 0376422. PY - 2021 SN - 0031-4005 SP - 1-8 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 - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=148485012&site=ehost-live&scope=site VL - 147 ID - 5305 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 Sydney Medical School, University of Sydney, Sydney, NSW, Australia Heart Research Institute, Sydney, NSW, Australia AN - 148335797. Language: English. Entry Date: 20210305. Revision Date: 20210204. Publication Type: Article. Journal Subset: Biomedical AU - Rehan, Rajan AU - Kotchetkova, Irina AU - Cordina, Rachael AU - Celermajer, David DB - Ucinahl DO - 10.1016/j.hlc.2020.05.114 DP - EBSCOhost IS - 2 N1 - Continental Europe; Europe. NLM UID: 100963739. PMID: NLM32713766. 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 & Circulation TI - Adult Congenital Heart Disease Survivors at Age 50 Years: Medical and Psychosocial Status UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=148335797&site=ehost-live&scope=site VL - 30 ID - 5307 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 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 - 147047051. Language: English. Entry Date: 20210115. Revision Date: 20210130. Publication Type: journal article. Journal Subset: Biomedical AU - Wolfe, Kelly R. AU - Liptzin, Deborah R. AU - Brigham, Dania AU - Kelly, Sarah L. AU - Rafferty, Carey AU - Albertz, Megan AU - Younoszai, Adel K. AU - Di Maria, Michael V. DB - Ucinahl DO - 10.1016/j.jpeds.2020.07.043 DP - EBSCOhost KW - Postoperative Complications -- Psychosocial Factors Cardiopulmonary Bypass Postoperative Complications -- Physiopathology Postoperative Complications -- Epidemiology Prospective Studies Child Young Adult Child, Preschool Adolescence Male Female Cross Sectional Studies Neuropsychological Tests Ferrans and Powers Quality of Life Index Scales N1 - Peer Reviewed; USA. Instrumentation: Clinical Decision Making in Nursing Scale (CDMNS) (Jenkins); Longitudinal Interval Follow-Up Evaluation (LIFE); Ferrans and Powers Quality of Life Index. NLM UID: 0375410. PMID: NLM32687915. PY - 2020 SN - 0022-3476 SP - 239-246 ST - Relationships between Physiologic and Neuropsychologic Functioning after Fontan T2 - Journal of Pediatrics TI - Relationships between Physiologic and Neuropsychologic Functioning after Fontan UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=147047051&site=ehost-live&scope=site VL - 227 ID - 5308 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 - Department of Pediatric Cardiology, Narayana Superspeciality Hospital, Howrah, West Bengal Department of Pediatric Cardiac Surgery, Narayana Superspeciality Hospital, Howrah, West Bengal AN - 148228387. Language: English. Entry Date: 20210126. Revision Date: 20210126. Publication Type: Article AU - Mukherji, Aritra AU - Ghosh, Sanjiban AU - Pathak, Nihar AU - Das, Jayita AU - Dutta, Nilanjan AU - Das, Debasis AU - Chattopadhyay, Amitabha DB - Ucinahl DO - 10.4103/apc.APC_128_20 DP - EBSCOhost IS - 1 KW - Pulmonary Artery -- Surgery Heart Ventricle -- Physiopathology Human Pulmonary Circulation Retrospective Design Infant Child, Preschool Child Heart Catheterization Vascular Resistance Oxygen Saturation N1 - research; tables/charts. Journal Subset: Asia; Biomedical. NLM UID: 101495459. PY - 2021 SN - 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 - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=148228387&site=ehost-live&scope=site VL - 14 ID - 5310 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 Department of Cardiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio AN - 146307654. Language: English. Entry Date: 20201012. Revision Date: 20201012. Publication Type: Article AU - Bucholz, Emily M. AU - Sleeper, Lynn A. AU - Goldberg, Caren S. AU - Pasquali, Sara K. AU - Anderson, Brett R. AU - Gaynor, J. William AU - Cnota, James F. AU - Newburger, Jane W. DB - Ucinahl DO - 10.1542/peds.2020-1240 DP - EBSCOhost IS - 4 KW - Hypoplastic Left Heart Syndrome -- Surgery Socioeconomic Factors Treatment Outcomes -- Evaluation Time Factors Neurodevelopment Quality of Life -- Evaluation Human Child Hypoplastic Left Heart Syndrome -- Mortality Heart Transplantation Functional Status Cox Proportional Hazards Model Regression Race Factors Minority Groups Age Factors Descriptive Statistics Log-Rank Test Motor Skills Problem Solving Adaptation, Psychological Communication Skills N1 - research; tables/charts. Journal Subset: Biomedical; Blind Peer Reviewed; Editorial Board Reviewed; Expert Peer Reviewed; Peer Reviewed; USA. NLM UID: 0376422. PY - 2020 SN - 0031-4005 SP - 1-10 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=146307654&site=ehost-live&scope=site VL - 146 ID - 5314 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, R2 = 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 Faculty of Human Development, Department of Educational Sciences, University of Toyama, Toyama, Japan The 1st Department of Surgery, Faculty of Medicine, University of Toyama, Toyama, Japan AN - 143551792. Language: English. Entry Date: 20200625. Revision Date: 20200926. Publication Type: journal article. Journal Subset: Biomedical AU - Hiraiwa, Akiko AU - Ibuki, Keijiro AU - Tanaka, Tomomi AU - Hirono, Keiichi AU - Miya, Kazushi AU - Yoshimura, Naoki AU - Ichida, Fukiko DA - Summer 2020 DB - Ucinahl DO - 10.1053/j.semtcvs.2019.10.017 DP - EBSCOhost IS - 2 KW - Child Development Child Behavior Developmental Disabilities -- Physiopathology Heart Ventricle -- Surgery Heart Surgery -- Adverse Effects Brain Heart Defects, Congenital -- Surgery Intelligence Intelligence Tests Child Prospective Studies Case Control Studies Developmental Disabilities -- Etiology Child, Preschool Heart Defects, Congenital -- Physiopathology Ventricular Function Education, Special Heart Ventricle -- Physiopathology Developmental Disabilities -- Diagnosis Female Age Factors Male Developmental Disabilities -- Psychosocial Factors Heart Defects, Congenital -- Diagnosis Treatment Outcomes Heart Ventricle -- Abnormalities N1 - USA. Instrumentation: Mental Developmental Index (MDI). NLM UID: 8917640. PMID: NLM31698030. 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 - Seminars in Thoracic & Cardiovascular Surgery TI - Toddler Neurodevelopmental Outcomes Are Associated With School-Age IQ in Children With Single Ventricle Physiology UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=143551792&site=ehost-live&scope=site VL - 32 ID - 5317 ER - TY - CASE A4 - Moosmann, Julia A2 - Dittrich, Sven A2 - Purbojo, Ariawan A2 - Cesnjevar, Robert 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 DB - Ucinahl DO - 10.1111/jocs.14658 DP - EBSCOhost J2 - Journal of Cardiac Surgery KW - Intestinal Diseases -- Etiology Heart Failure -- Surgery Cardiopulmonary Bypass Heart Assist Devices Prostheses and Implants -- Methods Heart Transplantation Heart Defects, Congenital -- Complications Heart Ventricle Heart Failure -- Etiology Female Waiting Lists Young Adult Quality of Life Ferrans and Powers Quality of Life Index Scales N1 - Accession Number: 144522203. Language: English. Entry Date: 20201024. Revision Date: 20201024. Publication Type: case study. Journal Subset: Biomedical; Peer Reviewed; USA. Instrumentation: Clinical Decision Making in Nursing Scale (CDMNS) (Jenkins); Longitudinal Interval Follow-Up Evaluation (LIFE); Ferrans and Powers Quality of Life Index. NLM UID: 8908809. PMID: NLM32557829. NV - Malden, Massachusetts PB - Wiley-Blackwell PY - 2020 SP - 1721-1724 ST - RVAD implantation in a Fontan patient with protein-losing enteropathy as a bridge to transplant: Prêtre modification TI - RVAD implantation in a Fontan patient with protein-losing enteropathy as a bridge to transplant: Prêtre modification UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=144522203&site=ehost-live&scope=site VL - 35 ID - 5319 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 AN - 146102538. Language: English. Entry Date: In Process. Revision Date: 20201019. Publication Type: journal article. Journal Subset: Biomedical AU - Alsaied, Tarek AU - Allen, Kiona Y. AU - Anderson, Jeffrey B. AU - Anixt, Julia S. AU - Brown, David W. AU - Cetta, Frank AU - Cordina, Rachael AU - D'Udekem, Yves AU - Didier, Meghan AU - Ginde, Salil AU - Di Maria, Michael V. AU - Eversole, Michelle AU - Goldberg, David AU - Goldstein, Bryan H. AU - Hoffmann, Erin AU - Kovacs, Adrienne H. AU - Lannon, Carole AU - Lihn, Stacey AU - Lubert, Adam M. AU - Marino, Bradley S. DB - Ucinahl DO - 10.1017/S1047951120001869 DP - EBSCOhost IS - 8 N1 - Editorial Board Reviewed; Europe; Expert Peer Reviewed; Peer Reviewed; UK & Ireland. NLM UID: 9200019. PMID: NLM32635947. 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 - Cardiology in the Young TI - The Fontan outcomes network: first steps towards building a lifespan registry for individuals with Fontan circulation in the United States UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=146102538&site=ehost-live&scope=site VL - 30 ID - 5321 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 - 145105585. Language: English. Entry Date: In Process. Revision Date: 20200923. Publication Type: journal article. Journal Subset: Biomedical AU - Emamaullee, Juliet AU - Zaidi, Ali N. AU - Schiano, Thomas AU - Kahn, Jeffrey AU - Valentino, Pamela L. AU - Hofer, Ryan E. AU - Taner, Timucin AU - Wald, Joyce W. AU - Olthoff, Kim M. AU - Bucuvalas, John AU - Fischer, Ryan DB - Ucinahl DO - 10.1161/CIRCULATIONAHA.120.045597 DP - EBSCOhost IS - 6 N1 - Peer Reviewed; USA. Grant Information: R01 HL141857/HL/NHLBI NIH HHS/United States. NLM UID: 0147763. PMID: NLM32776846. PY - 2020 SN - 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=145105585&site=ehost-live&scope=site VL - 142 ID - 5322 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 - 141876006. Corporate Author: Western Canadian Complex Pediatric Therapies Follow‐up Program*. Language: English. Entry Date: 20200923. Revision Date: 20210111. Publication Type: journal article AU - Atallah, Joseph AU - Guerra, Gonzalo Garcia AU - Joffe, Ari R. AU - Bond, Gwen Y. AU - Islam, Sunjidatul AU - Ricci, M. Florencia AU - AlAklabi, Mohammed AU - Rebeyka, Ivan M. AU - Robertson, Charlene M. T. AU - Garcia Guerra, Gonzalo DB - Ucinahl DO - 10.1161/JAHA.119.013632 DP - EBSCOhost IS - 4 KW - Heart Surgery -- Methods Cardiopulmonary Bypass -- Mortality Child Behavior Hypoplastic Left Heart Syndrome -- Surgery Anastomosis, Surgical -- Adverse Effects Cardiopulmonary Bypass -- Adverse Effects Nervous System Palliative Care Anastomosis, Surgical -- Mortality Cognition Child Development Risk Assessment Hypoplastic Left Heart Syndrome -- Psychosocial Factors Risk Factors Treatment Outcomes Child Human Resource Databases Age Factors Hypoplastic Left Heart Syndrome -- Physiopathology Infant, Newborn Intelligence Time Factors Child, Preschool Infant Hypoplastic Left Heart Syndrome -- Mortality Validation Studies Comparative Studies Evaluation Research Multicenter Studies Clinical Assessment Tools N1 - research. Journal Subset: Biomedical; Europe; Peer Reviewed; UK & Ireland. Instrumentation: Functional Living Index: Cancer (FLIC) (Schipper et al). NLM UID: 101580524. PMID: NLM32067591. PY - 2020 SN - 2047-9980 SP - 1-15 ST - Survival, Neurocognitive, and Functional Outcomes After Completion of Staged Surgical Palliation in a Cohort of Patients With Hypoplastic Left Heart Syndrome T2 - Journal of the American Heart Association TI - Survival, Neurocognitive, and Functional Outcomes After Completion of Staged Surgical Palliation in a Cohort of Patients With Hypoplastic Left Heart Syndrome UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=141876006&site=ehost-live&scope=site VL - 9 ID - 5330 ER - TY - CASE A4 - Bucholz, Emily M. A2 - Sleeper, Lynn A. A2 - Sananes, Renee A2 - Brosig, Cheryl L. A2 - Goldberg, Caren S. A2 - Pasquali, Sara K. A2 - Newburger, Jane W. 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 DB - Ucinahl DO - 10.1016/j.jpeds.2020.09.066 DP - EBSCOhost J2 - Journal of Pediatrics N1 - Accession Number: 148141914. Language: English. Entry Date: In Process. Revision Date: 20210207. Publication Type: case study. Journal Subset: Biomedical; Peer Reviewed; USA. Grant Information: U24 HL135691/HL/NHLBI NIH HHS/United States. NLM UID: 0375410. PMID: NLM33031800. NV - New York, New York PB - Elsevier B.V. PY - 2021 SP - 289-289 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 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 UR - http://proxy.lib.umich.edu/login?url=http://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=148141914&site=ehost-live&scope=site VL - 229 ID - 5331 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. © 2021 John Wiley & Sons Ltd AD - University of Missouri-Kansas City, Kansas City, MO, United States Children’s Mercy Hospital, Kansas City, MO, United States AU - Elliott, M. AU - Erickson, L. AU - Russell, C. L. AU - Chrisman, M. AU - Gross Toalson, J. AU - Emerson, A. DB - UScopus DO - 10.1111/jan.14785 IS - 5 KW - adherence congenital heart disease home monitoring program infant mHealth nursing stress parent experience single ventricle interstage transition to home M3 - Article N1 - Export Date: 10 May 2021 PY - 2021 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 - Journal of Advanced Nursing TI - Defining a new normal: A qualitative exploration of the parent experience during the single ventricle congenital heart disease interstage period UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85100950234&doi=10.1111%2fjan.14785&partnerID=40&md5=144b7bc050198b871be52c66d069514f VL - 77 ID - 5859 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. © 2020 The American Association for Thoracic Surgery AD - Department of Cardiothoracic Surgery, Heart Center and Shanghai Institution of Pediatric Congenital Heart Diseases, Shanghai Children's Medical Center, National Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China Clinical Research Center, Shanghai Children's Medical Center, National Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China Department of Cardiothoracic Surgery, Wuhan Children's Hospital, Wuhan, Hubei, China Department of Cardiothoracic Surgery, Guangzhou Woman and Children's Hospital, Guangzhou, Guangdong, China Department of Cardiac Surgery, Beijing Children's Hospital, National Center for Children Health, Beijing, China Department of Cardiothoracic Surgery, Shenzhen Children's Hospital, Shenzhen, Guangdong, China Department of Cardiothoracic Surgery, Hunan Children's Hospital, Changsha, China Department of Cardiothoracic Surgery, Jiangxi Children's Hospital, Nanchang, Jiangxi, China Department of Cardiothoracic Surgery, Shanxi Children's Hospital, Taiyuan, Shanxi, China Department of Cardiothoracic Surgery, Yuying Children's Hospital, Wenzhou Medical University, Wenzhou, Zhejiang, China Department of Cardiac Surgery, 2nd Affiliated Hospital, Harbin Medical University, Harbin, Heilongjiang, China Heart Center, Hainan Women and Children Medical Center, Haikou, Hainan, China Department of Cardiothoracic Surgery, Xi'an Children's Hospital, Xi'an, Shaanxi, China Department of Cardiac Surgery, Yan'an Hospital, Kunming, Yunnan, 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. AU - National Association of Pediatric, Cardiology AU - Cardiac Surgery Working, Group DB - UScopus DO - 10.1016/j.jtcvs.2020.11.074 IS - 5 KW - congenital heart disease COVID-19 follow-up surgery M3 - Article N1 - Cited By :1 Export Date: 10 May 2021 PY - 2021 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.scopus.com/inward/record.uri?eid=2-s2.0-85098946587&doi=10.1016%2fj.jtcvs.2020.11.074&partnerID=40&md5=26e3d1354bcfb58bf7b588ffb1f39e0b VL - 161 ID - 5860 ER - TY - JOUR AD - Department of Emergency Medicine, Carolinas Medical Center, Blvd Charlotte, Blythe, NC 1001, United States Department of Radiology, Carolinas Medical Center, Blvd Charlotte, Blythe, NC 1001, United States AU - Potter, J. K. AU - Clemente, J. D. AU - Asimos, A. W. DB - UScopus DO - 10.1016/j.ajem.2020.11.055 M3 - Article N1 - Export Date: 10 May 2021 PY - 2021 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.scopus.com/inward/record.uri?eid=2-s2.0-85097797054&doi=10.1016%2fj.ajem.2020.11.055&partnerID=40&md5=6c2d1b7a820a6f47afb491e787d9e2e7 VL - 42 ID - 5861 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. © 2020 Elsevier Inc. AD - Department of Environmental and Occupational Health, Program in Public Health, Susan and Henry Samueli College of Health Sciences, University of California, Irvine, Irvine, United States Division of Epidemiology, Human Genetics, and Environmental Sciences, University of Texas School of Public Health Austin Regional Campus, Austin, TX, United States AU - Tang, I. W. AU - Langlois, P. H. AU - Vieira, V. M. C7 - 110511 DB - UScopus DO - 10.1016/j.envres.2020.110511 KW - Birth defects Exposure assessment Maternal health Unconventional natural gas developments M3 - Article N1 - Export Date: 10 May 2021 PY - 2021 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.scopus.com/inward/record.uri?eid=2-s2.0-85097057250&doi=10.1016%2fj.envres.2020.110511&partnerID=40&md5=eefc0c0d438bd0f262a8a8703be13064 VL - 194 ID - 5862 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. © 2020 AD - Pediatric Heart Disease & Adult Congenital Heart Disease Center, Showa University, 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 - UScopus DO - 10.1016/j.jjcc.2020.10.021 IS - 3 KW - Balloon dilatation Congenital heart disease Pulmonary artery debanding Stent M3 - Article N1 - Export Date: 10 May 2021 PY - 2021 SP - 307-312 ST - Percutaneous pulmonary artery debanding T2 - Journal of Cardiology TI - Percutaneous pulmonary artery debanding UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85097401228&doi=10.1016%2fj.jjcc.2020.10.021&partnerID=40&md5=683369aca14c3409f879166885622542 VL - 77 ID - 5863 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. © 2020 Elsevier Inc. AD - Department of Cardiology, Boston Children's Hospital, Boston, MA, United States Department of Pediatrics, Harvard Medical School, Boston, MA, United States Department of Psychology, The Hospital for Sick Children, Toronto, ON, Canada Department of Pediatrics, Medical College of Wisconsin and Herma Heart Institute, Children's Wisconsin, Milwaukee, WI, United States Department of Pediatrics, University of Michigan CS Mott Children's Hospital, Ann Arbor, MI, United States 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. DB - UScopus DO - 10.1016/j.jpeds.2020.09.066 KW - congenital heart disease hypoplastic left heart syndrome M3 - Article N1 - Export Date: 10 May 2021 PY - 2021 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 - Journal of Pediatrics 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85096389649&doi=10.1016%2fj.jpeds.2020.09.066&partnerID=40&md5=52805c8a9954d310366917a3fb75d546 VL - 229 ID - 5864 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. © 2021 The Authors. Brain and Behavior published by Wiley Periodicals LLC AD - UCLA School of Nursing, University of California Los Angeles, Los Angeles, CA, United States Division of Pediatric Cardiology, University of California Los Angeles, Los Angeles, CA, United States Division of Pediatric Cardiology, Children's Hospital Los Angeles, Los Angeles, CA, United States Departments of Anesthesiology, University of California Los Angeles, Los Angeles, CA, United States Radiological Sciences, University of California Los Angeles, Los Angeles, CA, United States Bioengineering, University of California Los Angeles, Los Angeles, CA, United States Brain Research Institute, University of California Los Angeles, Los Angeles, CA, United States 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. C7 - e01977 DB - UScopus DO - 10.1002/brb3.1977 IS - 2 KW - cognition congenital heart disease gray matter magnetic resonance imaging M3 - Article N1 - Export Date: 10 May 2021 PY - 2021 ST - Reduced hippocampal volumes and memory deficits in adolescents with single ventricle heart disease T2 - Brain and Behavior TI - Reduced hippocampal volumes and memory deficits in adolescents with single ventricle heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85099055629&doi=10.1002%2fbrb3.1977&partnerID=40&md5=a096f0ddeb8e2156d17621d6809dcd58 VL - 11 ID - 5865 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. Copyright © 2021 by the American Academy of Pediatrics AD - Department of Pediatrics, Section of Pediatric Cardiology, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, United States Department of Sociology, Rice University, Houston, TX, United States 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. C7 - e20201693 DB - UScopus DO - 10.1542/peds.2020-1693 IS - 2 M3 - Article N1 - Export Date: 10 May 2021 PY - 2021 ST - Mental health disorders in children with congenital heart disease T2 - Pediatrics TI - Mental health disorders in children with congenital heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85101241930&doi=10.1542%2fpeds.2020-1693&partnerID=40&md5=824902ffdc77f28df2c827a17d73cca1 VL - 147 ID - 5866 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. © 2020 Wiley Periodicals LLC AD - Department of Medicine, Division of Cardiology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, United States Department of Cardiology, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States Department of Medicine, Division of Rheumatology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, United States Department of Medicine, Division of Gastroenterology, Brigham and Women'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 - UScopus DO - 10.1111/jocs.15244 IS - 2 KW - adult congenital heart disease ascites congestive heart failure Fontan operation M3 - Article N1 - Export Date: 10 May 2021 PY - 2021 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.scopus.com/inward/record.uri?eid=2-s2.0-85097440515&doi=10.1111%2fjocs.15244&partnerID=40&md5=646ff11f70ec204aae14f0b794ff56ce VL - 36 ID - 5868 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. © 2020 AD - Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia Sydney Medical School, University of Sydney, Sydney, NSW, Australia Heart Research Institute, Sydney, NSW, Australia AU - Rehan, R. AU - Kotchetkova, I. AU - Cordina, R. AU - Celermajer, D. DB - UScopus DO - 10.1016/j.hlc.2020.05.114 IS - 2 KW - Adult congenital heart disease Arrhythmia Depression Survival M3 - Article N1 - Cited By :1 Export Date: 10 May 2021 PY - 2021 SP - 261-266 ST - Adult Congenital Heart Disease Survivors at Age 50 Years: Medical and Psychosocial Status T2 - Heart Lung and Circulation TI - Adult Congenital Heart Disease Survivors at Age 50 Years: Medical and Psychosocial Status UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85088796457&doi=10.1016%2fj.hlc.2020.05.114&partnerID=40&md5=9419599fc8c18973ef48ebcc30f76a8c VL - 30 ID - 5869 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. Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc. AD - Department of Trauma Surgery Department of Emergency Medicine, Eulji University HospitalDaejeon, South Korea AU - Mun, Y. S. AU - Sung, W. Y. DB - UScopus DO - 10.1097/MD.0000000000024299 IS - 2 M3 - Article N1 - Export Date: 10 May 2021 PY - 2021 SP - e24299 ST - A rare case report of renal vein pseudoaneurysm after blunt trauma T2 - Medicine TI - A rare case report of renal vein pseudoaneurysm after blunt trauma UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85100227959&doi=10.1097%2fMD.0000000000024299&partnerID=40&md5=d4ee36955f7310871588bab20481bdf6 VL - 100 ID - 5870 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. © 2021 The American Association for Thoracic Surgery AD - 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 Department of Paediatric Endocrinology and Diabetes, Monash Children's Hospital, Clayton, Australia Paediatric and Congenital Cardiac Service, Starship Children's Hospital, Auckland, New Zealand Florey Institute of Neuroscience and Mental Health, Parkville, Australia Department of Endocrinology, The Children's Hospital at Westmead, Westmead, Australia Cardiovascular, Renal & Endocrine Services, Royal Melbourne Hospital, Melbourne, Australia Heart Centre for Children, The Children's Hospital at Westmead, Sydney, Australia 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. AU - Mandelstam, S. AU - Munns, C. AU - Gentles, T. AU - Grigg, L. AU - Winlaw, D. AU - Yang, J. Y. M. AU - group, A. N. Z. Fontan Registry Research DB - UScopus DO - 10.1016/j.jtcvs.2020.12.102 KW - aspirin bone density Fontan single ventricle stroke warfarin M3 - Article N1 - Cited By :3 Export Date: 10 May 2021 PY - 2021 ST - Long-term outcomes of warfarin versus aspirin after Fontan surgery T2 - Journal of Thoracic and Cardiovascular Surgery TI - Long-term outcomes of warfarin versus aspirin after Fontan surgery UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85100560951&doi=10.1016%2fj.jtcvs.2020.12.102&partnerID=40&md5=e4752aa8d8964b949eb7d2827db85d95 ID - 5872 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. © 2021, The Author(s), under exclusive licence to Springer Science+Business Media, LLC part of Springer Nature. AD - Department of Pediatric Cardiology, Advocate Children’s Hospital, Oaklawn, IL, United States Department of Pediatric Cardiology, Carle BroMenn Medical Center, Normal, IL, United States Department of Pediatric Cardiology, Lurie Children’s Hospital, Chicago, IL, United States Advocate Aurora Research Institute, Chicago, IL, United States AU - Zaidi, S. J. AU - Ross, E. AU - Gandhi, R. AU - Li, Y. AU - Penk, J. DB - UScopus DO - 10.1007/s00246-021-02539-8 KW - Bidirectional Glenn Congenital heart disease Fontan Hypoplastic left heart Norwood Qualitative echo Stage II M3 - Article N1 - Export Date: 10 May 2021 PY - 2021 ST - Comparison of Quantitative Versus Qualitative Assessment of Single Right Ventricular Function for the Prediction of Midterm Outcomes T2 - Pediatric Cardiology TI - Comparison of Quantitative Versus Qualitative Assessment of Single Right Ventricular Function for the Prediction of Midterm Outcomes UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85100032399&doi=10.1007%2fs00246-021-02539-8&partnerID=40&md5=a6b16dc195d362f3988eb8d33a771ac0 ID - 5873 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. © 2020 Elsevier Inc. AD - Division of Congenital Heart Surgery, Department of Surgery, Herma Heart Institute, Medical College of Wisconsin, Milwaukee, WI, United States AU - Hraska, V. DB - UScopus DO - 10.1053/j.optechstcvs.2020.08.001 KW - Aortic stenosis balloon dilatation of aortic valve critical aortic stenosis open valvuloplasty M3 - Article N1 - Export Date: 10 May 2021 PY - 2021 ST - Aortic Valve Repair in Neonates T2 - Operative Techniques in Thoracic and Cardiovascular Surgery TI - Aortic Valve Repair in Neonates UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85101151541&doi=10.1053%2fj.optechstcvs.2020.08.001&partnerID=40&md5=e77b2204e8a84bfc0343a91e68111b06 ID - 5874 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. © 2020 Elsevier Inc. AD - Section of Neurology, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO, United States Section of Pulmonology, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO, United States Section of Gastroenterology, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO, United States Section of Cardiology, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO, United States Section of Anesthesiology, University of Colorado School of Medicine, Aurora, CO, United States 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. DB - UScopus DO - 10.1016/j.jpeds.2020.07.043 KW - cognition hypoplastic left heart multiorgan comorbidities neurodevelopment pediatric neuropsychology single ventricle M3 - Article N1 - Cited By :1 Export Date: 10 May 2021 PY - 2020 SP - 239-246 ST - Relationships between Physiologic and Neuropsychologic Functioning after Fontan T2 - Journal of Pediatrics TI - Relationships between Physiologic and Neuropsychologic Functioning after Fontan UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85090064234&doi=10.1016%2fj.jpeds.2020.07.043&partnerID=40&md5=22721f9ffbb0cd3f54c3616d24b317c7 VL - 227 ID - 5876 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. Copyright © 2020 by the American Academy of Pediatrics. AD - Department of Cardiology, Boston Children’s Hospital, Boston, MA, United States Department of Pediatrics, Harvard Medical School, Harvard University, Boston, MA, United States Department of Pediatrics, University of Michigan, C.S. Mott Children’s Hospital, Ann Arbor, MI, United States Division of Pediatric Cardiology, Department of Pediatrics, Columbia University Irving Medical Center, New York-Presbyterian Morgan Stanley Children’s Hospital, New York City, NY, United States Division of Pediatric Cardiac Surgery, Cardiac Center, Children’s Hospital of Pennsylvania, Philadelphia, PA, United States Department of Cardiology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, United States AU - Bucholz, E. M. AU - Sleeper, L. A. AU - Goldberg, C. S. AU - Pasquali, S. K. AU - Anderson, B. R. AU - William Gaynor, J. AU - Cnota, J. F. AU - Newburger, J. W. C7 - e20201240 DB - UScopus DO - 10.1542/peds.2020-020396 IS - 4 M3 - Article N1 - Cited By :2 Export Date: 10 May 2021 PY - 2020 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85092682374&doi=10.1542%2fpeds.2020-020396&partnerID=40&md5=e40b7a31f3121131f13d4c37259fd9eb VL - 146 ID - 5877 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. © 2020 Elsevier B.V. AD - Division of Psychology and Mental Health, The University of Manchester, United Kingdom Greater Manchester Mental Health NHS Foundation Trust, United Kingdom Leeds Trinity University, United Kingdom Alder Hey Children's Hospital NHS Trust, United Kingdom AU - Lumsden, M. R. AU - Smith, D. M. AU - Twigg, E. AU - Guerrero, R. AU - Wittkowski, A. C7 - 101297 DB - UScopus DO - 10.1016/j.ppedcard.2020.101297 KW - CHD Family Interpretative phenomenological analysis Interview Qualitative M3 - Article N1 - Export Date: 10 May 2021 PY - 2020 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.scopus.com/inward/record.uri?eid=2-s2.0-85092024540&doi=10.1016%2fj.ppedcard.2020.101297&partnerID=40&md5=db5e374f8af3492ef0453f0cbadf7add VL - 59 ID - 5878 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. © 2020 Elsevier Masson SAS AD - Paediatric and Congenital Cardiology Department, Clinical Investigation Centre, Arnaud-De-Villeneuve University Hospital, M3C Regional Reference Centre, Montpellier, 34295, France Paediatric Cardiology Department, hôpital Necker–Enfants malades, AP–HP, M3C National Reference Centre, Paris-Descartes University, Sorbonne Paris Cité, Paris, 75015, France Paediatric Cardiology and Rehabilitation Unit, St-Pierre Institute, Palavas-Les-Flots, 34250, France Public Health and Chronic Diseases Laboratory, centre for Studies and Research into Health Services and Quality of Life, Aix-Marseille University, Marseille, 13385, France Epidemiology Department, University Hospital, Clinical Investigation Centre, INSERM–CIC 1411, University of Montpellier, Montpellier, 34295, France PhyMedExp, CNRS, INSERM, University of Montpellier, Montpellier, 34295, 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 - UScopus DO - 10.1016/j.acvd.2020.05.022 IS - 12 KW - Cardiac disease Paediatrics Patient education Quality of life Vitamin K antagonists M3 - Article N1 - Cited By :1 Export Date: 10 May 2021 PY - 2020 SP - 811-820 ST - Corrélation entre la qualité de vie et le temps dans la zone thérapeutique cible chez les enfants sous anticoagulants et participant à un programme d’éducation thérapeutique de l'automesure de l'International Normalised Ratio 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.scopus.com/inward/record.uri?eid=2-s2.0-85095452435&doi=10.1016%2fj.acvd.2020.05.022&partnerID=40&md5=6e735055f1bcde4859883180e0d84564 VL - 113 ID - 5879 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. © 2020, The Author(s). AD - Department of Pediatrics III, Division of Pediatric Cardiology, Medical University of Innsbruck, Anichstraße 35, Innsbruck, 6020, Austria Center of Pediatric Cardiology and Congenital Heart Disease, Heart and Diabetes Center North Rhine-Westphalia, Ruhr-University of Bochum, Georgstraße 11, Bad Oeynhausen, 32545, Germany Department of Pediatrics I, Division of Gastroenterology and Hepatology, Medical University of Innsbruck, Anichstraße 35, Innsbruck, 6020, Austria Department of Pediatrics I, Division of Inherited Metabolic Disorders, Medical University of Innsbruck, Anichstraße 35, Innsbruck, 6020, Austria Biocrates Life Sciences AG, Eduard-Bodem-Gasse 8, Innsbruck, 6020, Austria 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. C7 - 8930 DB - UScopus DO - 10.1038/s41598-020-65852-x IS - 1 M3 - Article N1 - Cited By :2 Export Date: 10 May 2021 PY - 2020 ST - Targeted metabolomic analysis of serum amino acids in the adult Fontan patient with a dominant left ventricle T2 - Scientific Reports TI - Targeted metabolomic analysis of serum amino acids in the adult Fontan patient with a dominant left ventricle UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85085908798&doi=10.1038%2fs41598-020-65852-x&partnerID=40&md5=7d35eaee629331458bb07baa1703a8c1 VL - 10 ID - 5880 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. © 2020 Author(s). AD - Hospices Civils de Lyon, Hopital Femme Mere Enfant, Service d'Endocrinologie Pediatrique, Bron, France Universite Claude Bernard, Lyon 1, Lyon, France Hospices Civils de Lyon, Hopital Femme Mere Enfant, Service de Gynecologie-obstetrique, Bron, France Hospices Civils de Lyon, Hopital Femme Mere Enfant, Centre Pluridisciplinaire de Diagnostic Prenatal, Bron, France Hospices Civils de Lyon, Hopital Femme Mere Enfant, Service de Neonatologie, Bron, France Hopital Saint-Antoine, Laboratoire Commun de Biologie et Genetique Moleculaires, Paris, France Hospices Civils de Lyon, Hopital Femme Mere Enfant, Service de Cardiologie Pediatrique, Bron, France Hospices Civils de Lyon, Hopital Femme Mere Enfant, Service de Genetique, Bron, France AU - Perge, K. AU - Massoud, M. AU - Gauthier-Moulinier, H. AU - Lascols, O. AU - Pangaud, N. AU - Villanueva, C. AU - Pons, L. DB - UScopus DO - 10.1159/000509837 IS - 4 M3 - Article N1 - Export Date: 10 May 2021 PY - 2020 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.scopus.com/inward/record.uri?eid=2-s2.0-85090996003&doi=10.1159%2f000509837&partnerID=40&md5=4624c59620a7b038ee9be96b91d3e62d VL - 11 ID - 5883 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 mm3, p < 0.004; right, 3,162.1 ± 475.4 vs. 3,504.8 ± 465.9 mm3, 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. © 2020 Wiley Periodicals, Inc. AD - Department of Anesthesiology, David Geffen School of Medicine at UCLA, University of California Los Angeles, Los Angeles, CA, United States UCLA School of Nursing, University of California Los Angeles, Los Angeles, CA, United States Division of Pediatric Cardiology, University of California Los Angeles, Los Angeles, CA, United States Division of Pediatric Cardiology, Children's Hospital Los Angeles, Los Angeles, CA, United States Department of Radiological Sciences, University of California Los Angeles, Los Angeles, CA, United States Department of Bioengineering, University of California Los Angeles, Los Angeles, CA, United States Brain Research Institute, University of California Los Angeles, Los Angeles, CA, United States 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. DB - UScopus DO - 10.1002/jnr.24667 IS - 10 KW - 3D surface morphometry anxiety basal ganglia congenital heart disease depression magnetic resonance imaging M3 - Article N1 - Export Date: 10 May 2021 PY - 2020 SP - 1877-1888 ST - Caudate nuclei volume alterations and cognition and mood dysfunctions in adolescents with single ventricle heart disease T2 - Journal of Neuroscience Research TI - Caudate nuclei volume alterations and cognition and mood dysfunctions in adolescents with single ventricle heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85086342574&doi=10.1002%2fjnr.24667&partnerID=40&md5=795421e5bc6bf2f9114585d4b06822f2 VL - 98 ID - 5884 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. © 2019 The American Association for Thoracic Surgery AD - Department of Cardiology, Boston Children's Hospital, Boston, MA, United States Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, Mich, United States Arnold Palmer Hospital for Children, Orlando, Fla, United States Children's Hospital of Philadelphia, Philadelphia, Pa, United States Texas Children's Hospital, Baylor College of Medicine, Houston, Tex, 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. AU - National Pediatric Cardiology Quality Improvement, Collaborative DB - UScopus DO - 10.1016/j.jtcvs.2019.11.001 IS - 4 KW - cardiovascular surgery congenital heart disease mortality/survival transplant M3 - Article N1 - Cited By :5 Export Date: 10 May 2021 PY - 2020 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.scopus.com/inward/record.uri?eid=2-s2.0-85077635515&doi=10.1016%2fj.jtcvs.2019.11.001&partnerID=40&md5=0885dd492366b5b186bce46e05f939f2 VL - 160 ID - 5885 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. © 2020, Springer Science+Business Media, LLC, part of Springer Nature. AD - 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 AU - Kiran, V. S. AU - Choudhary, S. AU - Shaik, A. AU - Gadabanahalli, K. AU - Raj, V. AU - Bhat, V. DB - UScopus DO - 10.1007/s00246-020-02402-2 IS - 7 KW - Asplenia Heterotaxy Isomerism Polysplenia Situs ambiguus M3 - Article N1 - Export Date: 10 May 2021 PY - 2020 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 - Pediatric Cardiology TI - The Spectrum of Cardiac Anomalies Associated with Heterotaxy: A Single-Center Study of a Large Series Based on Computed Tomography UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85086565195&doi=10.1007%2fs00246-020-02402-2&partnerID=40&md5=78fa15d032c9e17698c9b1bcd38b5e2c VL - 41 ID - 5887 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 - Department of Plastic and Reconstructive Surgery, Queen Elizabeth Hospital, Birmingham, United Kingdom Department of Plastic and Reconstructive Surgery, Guy's and Saint Thomas' NHS Foundation Trust, London, United Kingdom AU - Kiely, A. L. AU - Cooper, L. R. L. AU - Greig, A. C7 - e236253 DB - UScopus DO - 10.1136/bcr-2020-236253 IS - 9 KW - orthopaedic and trauma surgery paediatric surgery plastic and reconstructive surgery trauma M3 - Article N1 - Export Date: 10 May 2021 PY - 2020 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.scopus.com/inward/record.uri?eid=2-s2.0-85091579079&doi=10.1136%2fbcr-2020-236253&partnerID=40&md5=cce589755edf82182e9cee0d278ac7df VL - 13 ID - 5888 ER - TY - JOUR AD - Division of Pediatric Cardiology, Children's Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, NY, United States AU - Paul, E. A. AU - Hsu, D. T. DB - UScopus DO - 10.1016/j.jpeds.2020.02.041 M3 - Article N1 - Export Date: 10 May 2021 PY - 2020 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.scopus.com/inward/record.uri?eid=2-s2.0-85089553906&doi=10.1016%2fj.jpeds.2020.02.041&partnerID=40&md5=0a49d2a92cc3fdee1bc991832ea4037e VL - 224 ID - 5889 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. © 2020 Elsevier Ltd AD - Wallace H. Coulter Department of Biomedical Engineering, Georgia Institute of Technology, Atlanta, GA, United States Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA, United States Pediatric Cardiology Division, Department of Pediatrics, Emory University School of Medicine, 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. C7 - 109917 DB - UScopus DO - 10.1016/j.jbiomech.2020.109917 KW - Fontan Mechanical circulatory support Right-sided support Single ventricle Ventricular assist device M3 - Article N1 - Cited By :1 Export Date: 10 May 2021 PY - 2020 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.scopus.com/inward/record.uri?eid=2-s2.0-85087901889&doi=10.1016%2fj.jbiomech.2020.109917&partnerID=40&md5=ae0dd627edeee36829089163ad56f7cd VL - 109 ID - 5890 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. © 2019 Mac Keith Press AD - Research Institute of the McGill University Health Centre, Montreal Children’s Hospital, Montreal, QC, Canada School of Physical and Occupational Therapy, Faculty of Medicine, McGill University, Montreal, QC, Canada Centre for Interdisciplinary Research in Rehabilitation of Greater Montreal, Montreal, QC, Canada Shriner’s Hospital for Children, Montreal, QC, Canada Occupational Therapy Program, School of Rehabilitation Science, University of Ottawa, Ottawa, ON, Canada Université Laval, Quebec City, QC, Canada Douglas Hospital, 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 - UScopus DO - 10.1111/dmcn.14422 IS - 8 M3 - Article N1 - Cited By :2 Export Date: 10 May 2021 PY - 2020 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.scopus.com/inward/record.uri?eid=2-s2.0-85076279677&doi=10.1111%2fdmcn.14422&partnerID=40&md5=e4b69dfbf85b5835aed0a57e9e50f3ef VL - 62 ID - 5891 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. © 2020, Springer Science+Business Media, LLC, part of Springer Nature. AD - Section of Cardiology, Children’s Mercy Hospital, University of Missouri-Kansas City School of Medicine, Kansas City, MO, United States 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, United States Center for Ethics, Children’s Mercy Hospital, University of Missouri-Kansas City School of Medicine, Kansas City, MO, United States Section of Cardiology, Baylor College of Medicine, Texas Children’s Hospital, Houston, TX, United States Section of Cardiology, Stanford University School of Medicine, Lucile Packard Children’s Hospital, Stanford, CA, United States AU - Swanson, T. M. AU - Patel, A. AU - Baxter, A. J. AU - Morris, S. A. AU - Maskatia, S. A. AU - Lantos, J. D. DB - UScopus DO - 10.1007/s00246-020-02367-2 IS - 6 KW - Comfort care Congenital heart disease Ethics M3 - Article N1 - Cited By :1 Export Date: 10 May 2021 PY - 2020 SP - 1160-1165 ST - Pediatric Cardiology Specialist’s Opinions Toward the Acceptability of Comfort Care for Congenital Heart Disease T2 - Pediatric Cardiology TI - Pediatric Cardiology Specialist’s Opinions Toward the Acceptability of Comfort Care for Congenital Heart Disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85085023122&doi=10.1007%2fs00246-020-02367-2&partnerID=40&md5=12a97e33d450aaa902497b8d09147bb9 VL - 41 ID - 5892 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. © 2020, Biomedical Engineering Society. AD - Wallace H. Coulter School of Biomedical Engineering, Georgia Institute of Technology, 313 Ferst Drive NW, Atlanta, GA 30332, United States School of Chemistry and Biochemistry, Georgia Institute of Technology, Atlanta, GA, United States Department of Cardiology, Children’s Hospital of Philadelphia, Philadelphia, PA, United States Department of Mathematics and Computer Science, Emory, 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 - UScopus DO - 10.1007/s10439-020-02527-8 IS - 8 KW - Congenital heart defects Non-Newtonian rheology Patient-specific modeling M3 - Article N1 - Cited By :2 Export Date: 10 May 2021 PY - 2020 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.scopus.com/inward/record.uri?eid=2-s2.0-85085067228&doi=10.1007%2fs10439-020-02527-8&partnerID=40&md5=385483f67a2db6fc1bb5fd3f99895325 VL - 48 ID - 5893 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. © Copyright © 2020 Luckman, Chokron, Michowiz, Belenky, Toledano, Zahavi and Goldenberg-Cohen. 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 AU - Luckman, J. AU - Chokron, S. AU - Michowiz, S. AU - Belenky, E. AU - Toledano, H. AU - Zahavi, A. AU - Goldenberg-Cohen, N. C7 - 617 DB - UScopus DO - 10.3389/fneur.2020.00617 KW - arterial stroke children cortical visual impairment eye exam ophthalmological complication M3 - Article N1 - Export Date: 10 May 2021 PY - 2020 ST - The Need to Look for Visual Deficit After Stroke in Children T2 - Frontiers in Neurology TI - The Need to Look for Visual Deficit After Stroke in Children UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85088442781&doi=10.3389%2ffneur.2020.00617&partnerID=40&md5=a29d693dcf218aa45e0d62b009e72123 VL - 11 ID - 5894 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. © 2020 Israel Medical Association. All rights reserved. AD - Department of Pediatrics, Shaare Zedek Medical Center, Jerusalem, Israel Department of Pediatric Emergency Medicine, Shaare Zedek Medical Center, Jerusalem, Israel Division of Pediatric Endocrinology, 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 - UScopus IS - 7 KW - Glucose Morbidity Pediatric emergency department (PED) Stress hyperglycemia (SH) M3 - Article N1 - Cited By :1 Export Date: 10 May 2021 PY - 2020 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.scopus.com/inward/record.uri?eid=2-s2.0-85088611622&partnerID=40&md5=0a2e5d2c068dc8bb43a500d92be80ff3 VL - 22 ID - 5895 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. © 2020 The Authors. Journal of Cardiac Surgery published by Wiley Periodicals LLC AD - Department of Pediatric Cardiology, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany Department of Pediatric Cardiac Surgery, Friedrich-AlexanderUniversity Erlangen-Nürnberg, Erlangen, Germany AU - Moosmann, J. AU - Dittrich, S. AU - Purbojo, A. AU - Cesnjevar, R. DB - UScopus DO - 10.1111/jocs.14658 IS - 7 KW - assist in Fontan congenital heart disease Fontan circulation protein-losing enteropathy right ventricular assist device M3 - Article N1 - Export Date: 10 May 2021 PY - 2020 SP - 1721-1724 ST - RVAD implantation in a Fontan patient with protein-losing enteropathy as a bridge to transplant: Prêtre modification T2 - Journal of Cardiac Surgery TI - RVAD implantation in a Fontan patient with protein-losing enteropathy as a bridge to transplant: Prêtre modification UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85087210944&doi=10.1111%2fjocs.14658&partnerID=40&md5=6962bebb756b8247ed87016b67674b5b VL - 35 ID - 5896 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, R2 = 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. © 2019 Elsevier Inc. AD - 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 1st Department of Surgery, Faculty of Medicine, University of Toyama, Toyama, Japan AU - Hiraiwa, A. AU - Ibuki, K. AU - Tanaka, T. AU - Hirono, K. AU - Miya, K. AU - Yoshimura, N. AU - Ichida, F. DB - UScopus DO - 10.1053/j.semtcvs.2019.10.017 IS - 2 KW - IQ levels Neurodevelopment School age Single ventricle physiology Special education services M3 - Article N1 - Cited By :1 Export Date: 10 May 2021 PY - 2020 SP - 302-310 ST - Toddler Neurodevelopmental Outcomes Are Associated With School-Age IQ in Children With Single Ventricle Physiology T2 - Seminars in Thoracic and Cardiovascular Surgery TI - Toddler Neurodevelopmental Outcomes Are Associated With School-Age IQ in Children With Single Ventricle Physiology UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85076508919&doi=10.1053%2fj.semtcvs.2019.10.017&partnerID=40&md5=9d8b4308e95dcaaf7ac9c2461aa0ecf6 VL - 32 ID - 5901 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. © The Author(s) 2020. Published by Cambridge University Press. AD - Division of Pediatric Cardiology, Department of Pediatrics, Medical University of South Carolina, Charleston, SC, United States Division of Cardiology, Department of Medicine, Medical University of South Carolina, Charleston, SC, United States AU - Gaydos, S. S. AU - Chowdhury, S. M. AU - Judd, R. N. AU - McHugh, K. E. DB - UScopus DO - 10.1017/S1047951120000682 IS - 5 KW - Congenital cardiology follow-up care transition M3 - Article N1 - Cited By :4 Export Date: 10 May 2021 PY - 2020 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.scopus.com/inward/record.uri?eid=2-s2.0-85083195809&doi=10.1017%2fS1047951120000682&partnerID=40&md5=f763dc4431250af7ad0ace499916422e VL - 30 ID - 5903 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. © 2020 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley. AD - Department of Pediatrics, University of Alberta, Edmonton, AB, Canada Glenrose Rehabilitation Hospital, Edmonton, AB, Canada Department of Medicine, University of Alberta, Edmonton, AB, Canada Department of Pediatrics and Child Health, University of Manitoba, Winnipeg, MB, Canada Department of Surgery, University of Alberta, Edmonton, AB, Canada AU - Atallah, J. AU - Garcia Guerra, G. AU - Joffe, A. R. AU - Bond, G. Y. AU - Islam, S. AU - Ricci, M. F. AU - AlAklabi, M. AU - Rebeyka, I. M. AU - Robertson, C. M. T. AU - for the Western Canadian Complex Pediatric Therapies Follow-up, Program C7 - e013632 DB - UScopus DO - 10.1161/JAHA.119.013632 IS - 4 KW - cognitive Fontan procedure Glenn procedure mortality neurodevelopment Norwood procedure single ventricle M3 - Article N1 - Export Date: 10 May 2021 PY - 2020 ST - Survival, Neurocognitive, and Functional Outcomes After Completion of Staged Surgical Palliation in a Cohort of Patients With Hypoplastic Left Heart Syndrome T2 - Journal of the American Heart Association TI - Survival, Neurocognitive, and Functional Outcomes After Completion of Staged Surgical Palliation in a Cohort of Patients With Hypoplastic Left Heart Syndrome UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85079690408&doi=10.1161%2fJAHA.119.013632&partnerID=40&md5=eede12d40e983a534e6eec7a2481fb30 VL - 9 ID - 5913 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. © 2020 Sri Maya, Eka Gunawijaya, N. P. Veny Kartika Yantie, I. G. A. Trisna Windiani. AD - Department of Child Health, Sondosia General Hospital, Bima, West Nusa Tenggara, Indonesia Department of Child Health, Faculty of Medicine, Udayana University, Denpasar, Indonesia AU - Maya, S. AU - Gunawijaya, E. AU - Yantie, N. P. V. K. AU - Windiani, I. G. A. T. DB - UScopus DO - 10.3889/oamjms.2020.4047 IS - B KW - Cognitive Congenital heart disease Quality of life M3 - Article N1 - Export Date: 10 May 2021 PY - 2020 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.scopus.com/inward/record.uri?eid=2-s2.0-85097368182&doi=10.3889%2foamjms.2020.4047&partnerID=40&md5=8598a153a862e7873883d99dbfda6632 VL - 8 ID - 5917 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. © The Author(s), 2020. Published by Cambridge University Press. AD - School of Medicine, Oregon Health and Science University, 707 SW Gaines St., Portland, OR 97239, United States Bridges Palliative Care Program, Doernbecher Children's Hospital, Portland, OR, United States Pediatric Cardiology, Doernbecher Children's Hospital, Portland, OR, United States AU - Lowenstein, S. AU - MacAuley, R. AU - Perko, K. AU - Ronai, C. DB - UScopus DO - 10.1017/S1047951120000128 IS - 3 KW - goals of care Hypoplastic left heart syndrome palliative care prenatal diagnosis single ventricle defect M3 - Article N1 - Cited By :1 Export Date: 10 May 2021 PY - 2020 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 - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85081680410&doi=10.1017%2fS1047951120000128&partnerID=40&md5=73483d29dfdb07e1c531ed5abec606c0 VL - 30 ID - 5919 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. © 2020 Oxford University Press. All rights reserved. AD - Division of Allergy and Immunology, Department of Pediatrics, Morsani College of Medicine, University of South Florida, Tampa, FL, United States Department of Biology, University of Tampa, Tampa, FL, United States Division of Cardiology, Johns Hopkins All Children's Hospital, St. Petersburg, United States Division of Allergy and Immunology, Johns Hopkins All Children's Hospital, St. Petersburg, FL, United States Massachusetts General Hospital for Children, Boston, MA, 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. C7 - ofaa076 DB - UScopus DO - 10.1093/OFID/OFAA076 IS - 5 KW - Heart transplant Immunodeficiency Parvovirus B19 Pediatric Subcutaneous immunoglobulin M3 - Article N1 - Export Date: 10 May 2021 PY - 2020 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.scopus.com/inward/record.uri?eid=2-s2.0-85101400266&doi=10.1093%2fOFID%2fOFAA076&partnerID=40&md5=bc0ef7efb1a13ab128e9fb6f78384422 VL - 7 ID - 5920 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. © 2019 The Authors AD - Department of Cardiology, Royal Melbourne Hospital, Melbourne, Vic, Australia Murdoch Children's Research Institute, Melbourne, Vic, Australia Department of Genomic Medicine, Royal Melbourne Hospital, Melbourne, Vic, Australia Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia Faculty of Medicine and Health, University of Sydney, Sydney, NSW, Australia Paediatric and Congenital Cardiac Service, Starship Children's Hospital, Auckland, New Zealand Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Vic, Australia Department of Paediatrics, University of Melbourne, Melbourne, Vic, Australia Heart Centre for Children, Sydney Children's Hospitals Network, Sydney, NSW, Australia Sydney Medical School, University of Sydney, Sydney, NSW, Australia Department of Cardiology, Flinders Medical Centre, Adelaide, SA, Australia Department of Medicine, University of Melbourne, Melbourne, Vic, Australia Department of Cardiology, Royal Children's Hospital, Melbourne, Vic, Australia Transition Support Service, Royal Children's Hospital, Melbourne, Vic, Australia Faculty of Medicine, University of Queensland, Brisbane, Qld, Australia, Australia Queensland Paediatric Cardiac Research, Children's Health Queensland, Brisbane, Qld, Australia Australian and New Zealand Fontan Advocacy CommitteeAustralia and New Zealand, New Zealand Department of Gastroenterology and Clinical Nutrition, Royal Children's Hospital, Melbourne, Vic, Australia Victorian Paediatric Palliative Care Program, Royal Children's Hospital, Melbourne, Vic, Australia Department of Pathology, University of Melbourne, Melbourne, Vic, Australia Discipline of Paediatrics, School of Women's and Children's Health, UNSW Medicine, University of New South Wales, Sydney, NSW, Australia Palliative Care, Royal Melbourne Hospital, Melbourne, Vic, Australia Department of Cardiothoracic Surgery, Alfred Health, Melbourne, Vic, Australia Department of Surgery, Monash University, Melbourne, Vic, Australia Department of Haematology, Royal Children's Hospital, Melbourne, Vic, Australia Heart Kids NZ, Auckland, New Zealand Allied Health, Royal Children's Hospital, Melbourne, Vic, Australia Department of Gastroenterology and Hepatology, Royal Melbourne Hospital, Melbourne, Vic, Australia Congenital Heart Alliance of Australia and New Zealand Registry Board, 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 - UScopus DO - 10.1016/j.hlc.2019.09.010 IS - 1 M3 - Article N1 - Cited By :11 Export Date: 10 May 2021 PY - 2020 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.scopus.com/inward/record.uri?eid=2-s2.0-85076457240&doi=10.1016%2fj.hlc.2019.09.010&partnerID=40&md5=66c50dff6d7a16b53c17656fd890b8e3 VL - 29 ID - 5922 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. © 2019, International Pediatric Research Foundation, Inc. AD - UCLA School of Nursing, University of California Los Angeles, Los Angeles, CA, United States Department of Anesthesiology, University of California Los Angeles, Los Angeles, CA, United States Division of Pediatric Cardiology, University of California Los Angeles, Los Angeles, CA, United States Division of Pediatric Cardiology, Children’s Hospital Los Angeles, Los Angeles, CA, United States Department of Radiological Sciences, University of California Los Angeles, Los Angeles, CA, United States Department of Bioengineering, University of California Los Angeles, Los Angeles, CA, United States Brain Research Institute, University of California Los Angeles, Los Angeles, CA, United States 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. DB - UScopus DO - 10.1038/s41390-019-0569-3 IS - 1 M3 - Article N1 - Cited By :6 Export Date: 10 May 2021 PY - 2020 SP - 169-175 ST - Reduced brain mammillary body volumes and memory deficits in adolescents who have undergone the Fontan procedure T2 - Pediatric Research TI - Reduced brain mammillary body volumes and memory deficits in adolescents who have undergone the Fontan procedure UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85073948865&doi=10.1038%2fs41390-019-0569-3&partnerID=40&md5=7af523ae0495bf4bc528c377041d0a8a VL - 87 ID - 5924 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. © The Author(s), 2020. Published by Cambridge University Press. AD - Johns Hopkins University School of Nursing, Baltimore, MD, United States Sue and Bill Gross School of Nursing, University of California, Irvine, CA, United States University of Texas Medical Branch School of Nursing, Galveston, TX, United States School of Nursing, University of California, Los Angeles, CA, United States Cardiothoracic Surgery, Children's Hospital, Los Angeles, CA, United States AU - Peterson, J. K. AU - Olshansky, E. F. AU - Guo, Y. AU - Evangelista, L. S. AU - Pike, N. A. DB - UScopus DO - 10.1017/S1047951120003790 KW - academic achievement Fontan procedure peer relationships qualitative research M3 - Article N1 - Export Date: 10 May 2021 PY - 2020 ST - Optimism despite profound uncertainty: School and social relationships in adolescents with single ventricle heart disease T2 - Cardiology in the Young TI - Optimism despite profound uncertainty: School and social relationships in adolescents with single ventricle heart disease UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85096007387&doi=10.1017%2fS1047951120003790&partnerID=40&md5=d2f199baf6c64eaf1a47ac587f61a9c1 ID - 5925 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. © 2020 The Author(s). Published by Cambridge University Press. AD - Department of Pediatrics, George Washington University School of Medicine, Washington, DC, United States Division of Biostatistics and Study Methodology, Children's National Hospital, Washington, DC, United States Division of Cardiology, Children's National Hospital, 111 Michigan Ave, NW, Washington, DC 20010, United States AU - Alkofair, B. AU - Alruwaili, A. AU - Gai, J. AU - Harahsheh, A. S. DB - UScopus DO - 10.1017/S1047951120001985 KW - congenital heart surgery functionally single ventricle anatomy health economics Post-Fontan protein-losing enteropathy resource utilisation M3 - Article N1 - Export Date: 10 May 2021 PY - 2020 ST - Impact of protein-losing enteropathy in children who underwent the Fontan operation T2 - Cardiology in the Young TI - Impact of protein-losing enteropathy in children who underwent the Fontan operation UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85092802316&doi=10.1017%2fS1047951120001985&partnerID=40&md5=159327e7d758fe30c5c00bee1f2e594e ID - 5927 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 (Z0) 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 Z0 of the legs and kidneys, resulting in greater systemic Z0 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. © 2020 the American Physiological Society 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 AU - Hauser, J. A. AU - Jones, A. AU - Pandya, B. AU - Taylor, A. M. AU - Muthurangu, V. DB - UScopus DO - 10.1152/AJPHEART.00500.2020 IS - 4 KW - Blood flow Fontan Magnetic resonance imagine Postprandial Vascular physiology M3 - Article N1 - Export Date: 10 May 2021 PY - 2020 SP - H808-H813 ST - Comprehensive MRI assessment of the cardiovascular responses to food ingestion in Fontan physiology T2 - American Journal of Physiology - Heart and Circulatory Physiology TI - Comprehensive MRI assessment of the cardiovascular responses to food ingestion in Fontan physiology UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85091469871&doi=10.1152%2fAJPHEART.00500.2020&partnerID=40&md5=08272de7e8b6e11247c306b4aa2e98d4 VL - 319 ID - 5929 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. © 2019 Author(s) (or their employer(s)) 2019. AD - Department of Pediatrics and Pediatric Cardiology, Kitasato University School of Medicine, Sagamihara, 350-1298, Japan Department of Pediatric Cardiology, Saitama Medical University, Kawagoe, Japan Department of Pediatrics, Hokkaido Ryoiku-en, Asahikawa, Japan AU - Saiki, H. AU - Kuwata, S. AU - Iwamoto, Y. AU - Ishido, H. AU - Taketazu, M. AU - Masutani, S. AU - Nishida, T. AU - Senzaki, H. DB - UScopus DO - 10.1136/heartjnl-2018-314183 IS - 16 KW - congenital heart disease congenital heart surgery Fontan physiology heart failure M3 - Article N1 - Cited By :6 Export Date: 10 May 2021 PY - 2019 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 UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85062345120&doi=10.1136%2fheartjnl-2018-314183&partnerID=40&md5=67811e06284208586b1966f6dc0c2eab VL - 105 ID - 5950 ER - TY - JOUR AU - Mellander, M. AU - Berntsson, L. AU - Nilsson, B. DB - UScopus DO - 10.1111/j.1651-2227.2007.00221.x IS - 2 M3 - Erratum N1 - Export Date: 10 May 2021 PY - 2007 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.scopus.com/inward/record.uri?eid=2-s2.0-33847182466&doi=10.1111%2fj.1651-2227.2007.00221.x&partnerID=40&md5=9450c7e0f961335c396431df1765781c VL - 96 ID - 6423 ER - TY - JOUR AB - Background People with a Fontan circulation experience a range of physical, psychosocial and neurodevelopmental challenges alongside, or caused by, their cardiac condition, with significant consequences for health-related quality of life (HRQOL). We meta-analyzed HRQOL outcomes reported by people with a Fontan circulation or their proxies and evaluated predictors of poorer HRQOL. Methods and Results Six electronic databases were searched for peer-reviewed, English-language articles published before March 2019. Standardized mean differences (SMD) were calculated using fixed and random-effects models. Fifty articles reporting on 29 unique studies capturing HRQOL outcomes for 2793 people with a Fontan circulation and 1437 parent-proxies were analyzed. HRQOL was lower in individuals with a Fontan circulation compared with healthy referents or normative samples (SMD, -0.92; 95% CI, -1.36 to -0.48; P<0.001). Lower scores were reported across all HRQOL domains, with the largest differences found for physical (SMD, -0.90; 95% CI, -1.13 to -0.67; P<0.001) and school/work functioning (SMD, -0.71; 95% CI, -0.90 to -0.52; P<0.001). Meta-regression analyses found no significant predictors of self-reported physical functioning, but older age at Fontan operation was associated with poorer emotional functioning (β=-0.124; P=0.004), and diagnosis of hypoplastic left heart was associated with poorer social functioning (β=-0.007; P=0.048). Sensitivity analyses showed use of the PedsQL Core Module was associated with lower HRQOL scores compared with the Short-Form Health Survey-36. Conclusions HRQOL outcomes for people with a Fontan circulation are lower than the general population. Optimal care acknowledges the lifelong impact of the Fontan circulation on HRQOL and offers targeted strategies to improve outcomes for this growing population. AD - Heart Centre for Children The Children's Hospital at Westmead Sydney New South Wales Australia Discipline of Paediatrics School of Women's and Children's Health University of New South Wales Medicine The University of New South Wales Sydney New South Wales Australia Heart Research Group Murdoch Children's Research Institute Melbourne Victoria Australia Department of Cardiac Surgery The Royal Children's Hospital Melbourne Victoria Australia Sydney Medical School The University of Sydney New South Wales Australia Department of Pediatrics University of Cincinnati College of Medicine Cincinnati Ohio United States Cincinnati Adult Congenital Heart Disease Program Heart Institute Cincinnati Children's Hospital Cincinnati Ohio United States Department of Cardiology Boston Children's Hospital Boston Massachusetts United States Harvard Medical School Boston Massachusetts United States Pain Management Research Institute Royal North Shore Hospital Sydney New South Wales Australia School of Psychology The University of Sydney New South Wales Australia Department of Cardiology Royal Prince Alfred Hospital Sydney New South Wales Australia Cincinnati Children's Center for Heart Disease and the Developing Mind Heart Institute and Division of Behavioral Medicine & Clinical Psychology Cincinnati Children's Hospital Cincinnati Ohio United States AU - Marshall, K. H. AU - D'Udekem, Y. AU - Sholler, G. F. AU - Opotowsky, A. R. AU - Costa, D. S. J. AU - Sharpe, L. AU - Celermajer, D. S. AU - Winlaw, D. S. AU - Newburger, J. W. AU - Kasparian, N. A. DB - Scopus DO - 10.1161/JAHA.119.014172 IS - 6 KW - chronic illness congenital heart disease Fontan circulation health‐related quality of life mental health psychological stress M3 - Article N1 - Export Date: 15 June 2020 PY - 2020 SP - e014172 ST - Health-Related Quality of Life in Children, Adolescents, and Adults With a Fontan Circulation: A Meta-Analysis T2 - Journal of the American Heart Association TI - Health-Related Quality of Life in Children, Adolescents, and Adults With a Fontan Circulation: A Meta-Analysis UR - https://www.scopus.com/inward/record.uri?eid=2-s2.0-85081994644&doi=10.1161%2fJAHA.119.014172&partnerID=40&md5=e343ac83e87245d118029b7d21235f91 VL - 9 ID - 6560 ER -