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BACKGROUND Use of implantable cardiac defibrillators (ICDs) in children and patients with congenital heart disease is complicated by body size and anatomy. A variety of creative implantation techniques has been used empirically in these groups on an ad hoc basis. OBJECTIVE To rationalize ICD placement in special populations, we used subject-specific,(More)
BACKGROUND Pulmonary valve replacement (PVR) in repaired tetralogy of Fallot (TOF) reduces pulmonary regurgitation and decreases right ventricular (RV) dilation, but its long-term impact on ventricular tachycardia (VT) and mortality is unknown. This study aimed to determine the incidence of death and VT in TOF after PVR and to test the hypothesis that PVR(More)
BACKGROUND Tetralogy of Fallot is the most common form of congenital heart disease in implantable cardioverter-defibrillator (ICD) recipients, yet little is known about the value of ICDs in this patient population. METHODS AND RESULTS We conducted a multicenter cohort study in high-risk patients with Tetralogy of Fallot to determine actuarial rates of ICD(More)
INTRODUCTION The incidence of appropriate and inappropriate discharges, indicators of system failure, and clinical implications of implantable cardioverter defibrillator (ICD) therapy in children and young adults with heart disease is poorly defined. METHODS AND RESULTS In a retrospective study at a single medical center, a total of 90 ICD procedures were(More)
BACKGROUND Ebstein anomaly is a rare and heterogeneous congenital heart defect affecting the tricuspid valve and right ventricular (RV) myocardium. Few studies have analysed the electrocardiographic features of Ebstein anomaly and none has addressed correlations with disease severity. METHODS Patients with Ebstein anomaly who had undergone(More)
Although long-term survival after craniopharyngioma treatment is excellent in childhood and early adulthood, sudden deaths in two craniopharyngioma survivors with cardiac findings suggest a need to determine whether treated patients exhibit potential substrates for sudden cardiac death. We present a retrospective review of two index patients with cardiac(More)
BACKGROUND Myocardial fibrosis is a hallmark of hypertrophic cardiomyopathy (HCM) and a risk factor for ventricular arrhythmia. Fibrosis can be reflected in circulating matrix remodeling protein concentrations. We explored differences in circulating markers of extracellular matrix turnover between young HCM patients with versus without history of serious(More)
BACKGROUND In Fontan and atrial switch patients, transcatheter ablation is limited by difficult access to the pulmonary venous atrium. In recent years, transbaffle access (TBA) has been described, but limited data document its safety and utility. METHODS AND RESULTS All ablative electrophysiological study cases of this population performed between January(More)
INTRODUCTION Nonautomatic focal atrial tachycardia (NAFAT) has been characterized in adults with structurally normal hearts. This article characterizes NAFAT in a population of patients with complex congenital heart disease. METHODS AND RESULTS Electrophysiologic and electroanatomic mapping data and acute outcomes were reviewed in patients undergoing(More)
BACKGROUND Less is known about depression, anxiety and quality of life (QoL) in children and adolescents with pacemakers (PMs) and implantable cardioverter-defibrillators (ICDs) than is known in adults with these devices. METHODS A standardized psychiatric interview diagnosed anxiety/depressive disorders in a cross-sectional study. Self-report measures of(More)