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BACKGROUND The risks of sports participation for implantable cardioverter-defibrillator (ICD) patients are unknown. METHODS AND RESULTS Athletes with ICDs (age, 10-60 years) participating in organized (n=328) or high-risk (n=44) sports were recruited. Sports-related and clinical data were obtained by phone interview and medical records. Follow-up occurred(More)
BACKGROUND Mutations in transcription factor NKX2.5 cause congenital heart disease (CHD). We identified a CHD family with atrial septal defects (ASDs), atrioventricular block, ventricular noncompaction, syncope and sudden death. Our objective is to identify the disease-causing mutation in the CHD family. METHODS Direct DNA sequence analysis was used to(More)
Risks associated with radiofrequency ablation (RFA) have been reported to be increased in children < or =15 kg. We sought to compare the safety and efficacy of RFA in children <15 kg with those between 15.1 and 20 kg. Clinical, electrophysiologic, and RFA data for all patients < or =20 kg who underwent RFA for supraventricular tachycardia between January(More)
Atrioventricular nodal reentrant tachycardia (AVNRT), a common tachycardia in children, is routinely treated by catheter ablation using radiofrequency or cryothermal energy. Acute success rates of 95-97 % are reported for cryoablation, similar to those achieved with radiofrequency ablation (RFA). However, early studies reported higher recurrence rates after(More)
Timothy syndrome type 1 (TS-1) is a rare disorder that affects multiple organ systems and has a high incidence of sudden death due to profound QT prolongation and resultant ventricular arrhythmias. All previously described cases of TS-1 are the result of a missense mutation in exon 8A (p.G406R), an alternatively spliced variant of the L-type calcium channel(More)
OBJECTIVES We sought to assess the spectrum and outcome of young long QT syndrome (LQTS) patients, addressing treatment including device indications, risks, and benefits. BACKGROUND Long QT syndrome has a phenotype ranging from asymptomatic electrocardiogram (ECG) abnormalities to sudden death. Treatments include beta-blockers and device implantation in(More)
The diagnosis of congenital long-QT syndrome (LQTS) in the relatives (nonprobands) of index patients (probands) is increasing because of screening. This report documents the clinical courses and outcomes of nonproband pediatric patients with LQTS. All patients aged <18 years with LQTS were identified at 3 pediatric centers. Demographic data, personal and(More)
OBJECTIVES The purpose of this study was to demonstrate that adverse but reversible effects on AV conduction may be observed during cryoablation despite no evidence of deleterious effects evident during cryomapping. BACKGROUND Transcatheter cryoablation has recently been introduced for treatment of supraventricular tachycardia. Potential advantages(More)
Dilated cardiomyopathy (DCM) due to a primary supraventricular tachycardia not originating from the sinus node is not frequently seen in older children or adolescents. However, it is important to recognize this entity as a reversible cause of DCM to avoid costly and inappropriate treatments for these patients. We describe 7 patients who presented with DCM.(More)
BACKGROUND Magnetic resonance imaging (MRI) is a standard of care in evaluating many disease processes. Given concerns about device damage or movement, programming changes, lead heating, inappropriate pacing, and image artifact, MRI is contraindicated in pacemaker patients. Despite this, studies have demonstrated safety and efficacy of MRI in adults with(More)