Multiphysiologic State Computational Fluid Dynamics Modeling for Planning Fontan With Interrupted Inferior Vena Cava.

Background: Single ventricle (SV) patients with interrupted inferior vena cava (iIVC) and azygos continuation are at high risk for unbalanced hepatic venous flow (HVF) distribution to the lungs after Fontan completion and subsequent pulmonary arteriovenous malformations (AVMs) formation.

Objectives: The aim of the study was to utilize computational fluid dynamics (CFD) analysis to avoid maldistribution of HVF to the lungs after Fontan surgery.

Methods: Four SV subjects with iIVC were prospectively studied with a 3-dimensional (3D) modeling workflow with digital 3D models created from segmented magnetic resonance images or computer tomography scans, virtual surgery, and CFD analysis over multiple physiologic states for the evaluation of operative plans to achieve balanced HVF to both lungs.

Factors associated with morbidity, mortality, and hemodynamic failure after biventricular conversion in borderline hypoplastic left hearts.

Objective: A subset of patients with borderline hypoplastic left heart may be candidates for single to biventricular conversion, but long-term morbidity and mortality persist. Prior studies have shown conflicting results regarding the association of preoperative diastolic dysfunction and outcome, and patient selection remains challenging.

Methods: Patients with borderline hypoplastic left heart undergoing biventricular conversion from 2005 to 2017 were included.

Myopericarditis and Cardiac Magnetic Resonance Imaging in a Young Girl.

Cardiac infection with is rarely diagnosed, especially in children, and corresponding cardiac magnetic resonance imaging (CMR) has not been reported. We present a probable case, a 9-year-old girl with myopericarditis, eosinophilia, positive serology, and CMR findings consistent with myocardial edema.

Parent and Physician Understanding of Prognosis in Hospitalized Children With Advanced Heart Disease.

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).

Value of Troponin Testing for Detection of Heart Disease in Previously Healthy Children.

Background Troponin levels are frequently obtained in pediatric patients, but the benefit remains unclear. Methods and Results This retrospective study included 1993 patients aged 0 to 21 years without history of cardiac disease in whom troponin levels were obtained during clinical evaluation of cardiac and noncardiac presentations. Troponin was elevated (≥0.

Delayed Presentation of Traumatic Pericardial Rupture: Diagnostic and Surgical Considerations for Treatment.

Traumatic pericardial rupture is a rare event with high mortality. We present the case of a 15-year-old boy who sustained thoracic and abdominal trauma secondary to motor vehicle collision, with a delayed diagnosis of traumatic pericardial rupture with cardiac herniation. Out of concern for torsion and hemodynamic collapse, surgical repair was advised.

Staged ventricular recruitment in patients with borderline ventricles and large ventricular septal defects.

Objectives: Patients with borderline ventricles and ventricular septal defects (VSDs) who have previously undergone single ventricle palliation might be candidates for staged ventricular recruitment with the ultimate goal of biventricular conversion. This study aimed to determine the effect of atrial septal defect (ASD) restriction without VSD closure on ventricular growth in patients with borderline right or left ventricles and VSDs.

Methods: Patients with borderline ventricles and VSD who underwent a staged ventricular recruitment procedure with strategies to increase blood flow through hypoplastic ventricle via ASD restriction without VSD closure after single ventricle palliation were retrospectively reviewed.

Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure.

Background: Multicenter longitudinal objective data for survival into adulthood of patients who have undergone Fontan procedures are lacking.

Objectives: This study sought to describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time.

Methods: Exercise testing, echocardiography, B-type natriuretic peptide, functional health assessment, and medical history abstraction were repeated 9.

Truncus arteriosus versus tetralogy of Fallot with pulmonary atresia.

Truncus arteriosus and tetralogy of Fallot with pulmonary atresia may be difficult to differentiate prenatally. We present a case that, on newborn echocardiography, angiography, and intraoperative inspection, shared features of both diagnoses.

De novo mutations in congenital heart disease with neurodevelopmental and other congenital anomalies.

Congenital heart disease (CHD) patients have an increased prevalence of extracardiac congenital anomalies (CAs) and risk of neurodevelopmental disabilities (NDDs). Exome sequencing of 1213 CHD parent-offspring trios identified an excess of protein-damaging de novo mutations, especially in genes highly expressed in the developing heart and brain. These mutations accounted for 20% of patients with CHD, NDD, and CA but only 2% of patients with isolated CHD.

Survival data and predictors of functional outcome an average of 15 years after the Fontan procedure: the pediatric heart network Fontan cohort.

Objective: Multicenter longitudinal outcome data for Fontan patients surviving into adulthood are lacking. The aim of this study was to better understand contemporary outcomes in Fontan survivors by collecting follow-up data in a previously well-characterized cohort.

Design: Baseline data from the Fontan Cross-Sectional Study (Fontan 1) were previously obtained in 546 Fontan survivors aged 11.

Transient elastography may identify Fontan patients with unfavorable hemodynamics and advanced hepatic fibrosis.

Background: Transient elastography (TE) offers a noninvasive correlate with the degree of hepatic fibrosis. However, factors other than fibrosis affect liver stiffness. We sought to determine whether hepatic congestion related to hemodynamics in Fontan circulation influences liver stiffness measurement (LSM) assessed by TE.

Stented bovine jugular vein graft (Melody valve) for surgical mitral valve replacement in infants and children.

Objective: The options for mitral valve replacement in children with irreparable mitral valve disease have been limited to fixed-diameter prostheses that do not accommodate for somatic growth. We have modified an externally stented bovine jugular vein graft (Melody valve) for implantation in this cohort. Because it is not a fixed-diameter prosthesis, we hypothesized that the valve can be expanded in the catheterization laboratory as the child grows.

The relationship of patient medical and laboratory characteristics to changes in functional health status in children and adolescents after the Fontan procedure.

Despite hypothesized concerns about deterioration beginning in adolescence, longitudinal data and associated factors regarding standardized assessment of physical functioning are not available for Fontan patients. Parents who participated in the Fontan Cross-Sectional Study completed the Child Health Questionnaire at 2 time points for 245 subjects ages 6-18 years. Associations between change in Physical Functioning Summary Score and baseline patient, medical, and laboratory characteristics (mean age 9.

De novo mutations in histone-modifying genes in congenital heart disease.

Congenital heart disease (CHD) is the most frequent birth defect, affecting 0.8% of live births. Many cases occur sporadically and impair reproductive fitness, suggesting a role for de novo mutations.

Cardiac performance and quality of life in patients who have undergone the Fontan procedure with and without prior superior cavopulmonary connection.

Background: A superior cavopulmonary connection is commonly performed before the Fontan procedure in patients with a functionally univentricular heart. Data are limited regarding associations between a prior superior cavopulmonary connection and functional and ventricular performance late after the Fontan procedure.

Methods: We compared characteristics of those with and without prior superior cavopulmonary connection among 546 subjects enrolled in the Pediatric Heart Network Fontan Cross-Sectional Study.

Outcome after repair of atrioventricular septal defect with tetralogy of Fallot.

Background: Long-term outcomes of repair of tetralogy of Fallot associated with complete atrioventricular septal defect are seldom reported. We report our survival and reintervention outcomes over a 29-year time period.

Methods: Between March 1979 and April 2008, 61 patients with the combined cardiac defect of atrioventricular septal defect and tetralogy of Fallot were surgically managed.

Late status of Fontan patients with persistent surgical fenestration.

Objectives: This study was undertaken to determine the effects of creating a systemic-to-pulmonary venous atrial-level communication (fenestration) at the time of the Fontan procedure on late outcomes.

Background: Fenestrations are frequently performed during Fontan procedures, but late consequences are not well described.

Methods: Patient characteristics were compared between those with and without surgical fenestration among 536 subjects (mean age 11.

Factors associated with serum brain natriuretic peptide levels after the Fontan procedure.

Objective: Although a useful marker of heart failure in adults, the utility of brain natriuretic peptide concentration (BNP) for children after the Fontan procedure is not well studied.

Design: BNP was measured in 510 patients who were 6-18 years old in the Pediatric Heart Network Fontan cross-sectional study at a median of 8.2 years after Fontan.

The Fontan patient: inconsistencies in medication therapy across seven pediatric heart network centers.

Patients who have undergone the Fontan procedure are at risk for thrombosis, ventricular dysfunction, and valve regurgitation, but data to guide the medical treatment and prevention of these adverse outcomes in this population are lacking. This analysis examined medication usage among Fontan patients by putative indication and by study center. The medical history and current medications of 546 Fontan subjects, ages 6-18 years, were assessed in a Pediatric Heart Network multicenter cross-sectional study.

Laboratory measures of exercise capacity and ventricular characteristics and function are weakly associated with functional health status after Fontan procedure.

Background: Patients after the Fontan procedure are at risk for suboptimal functional health status, and associations with laboratory measures are important for planning interventions and outcome measures for clinical trials.

Methods And Results: Parents completed the generic Child Health Questionnaire for 511 Fontan Cross-Sectional Study patients 6 to 18 years of age (61% male). Associations of Child Health Questionnaire Physical and Psychosocial Functioning Summary Scores (FSS) with standardized measurements from prospective exercise testing, echocardiography, magnetic resonance imaging, and measurement of brain natriuretic peptide were determined by regression analyses.

De novo copy number variants identify new genes and loci in isolated sporadic tetralogy of Fallot.

Tetralogy of Fallot (TOF), the most common severe congenital heart malformation, occurs sporadically, without other anomaly, and from unknown cause in 70% of cases. Through a genome-wide survey of 114 subjects with TOF and their unaffected parents, we identified 11 de novo copy number variants (CNVs) that were absent or extremely rare (<0.1%) in 2,265 controls.

Functional status, heart rate, and rhythm abnormalities in 521 Fontan patients 6 to 18 years of age.

Objectives: Our objective was to determine the relationship between functional outcome and abnormalities of heart rate and rhythm after the Fontan operation.

Methods: The National Heart, Lung, and Blood Institute Pediatric Heart Network conducted a cross-sectional analysis of patients who had undergone a Fontan procedure at the 7 network centers. Analysis was based on 521 patients with an electrocardiogram (n = 509) and/or bicycle exercise test (n = 404).

Contemporary outcomes after the Fontan procedure: a Pediatric Heart Network multicenter study.

Objectives: We characterized a large cohort of children who had a Fontan procedure, with measures of functional health status, ventricular size and function, exercise capacity, heart rhythm, and brain natriuretic peptide (BNP).

Background: The characteristics of contemporary Fontan survivors are not well described.

Methods: We enrolled 546 children (age 6 to 18 years, mean 11.

Functional state of patients with heterotaxy syndrome following the Fontan operation.

Background: Children born with heterotaxy syndromes have poorer outcomes compared with children born with comparable cardiac lesions requiring similar surgical palliation. Heterotaxy has been reported as a separate risk factor for mortality and increased morbidity in a series of Fontan operations reported from single centres. Little is known, however, about the functional state of surviving patients with heterotaxy following a Fontan operation.