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OBJECTIVES The purpose of this study was to compare the efficacy of beta-blockers in congenital long QT syndrome (LQTS). BACKGROUND Beta-blockers are the mainstay in managing LQTS. Studies comparing the efficacy of commonly used beta-blockers are lacking, and clinicians generally assume they are equally effective. METHODS Electrocardiographic and(More)
BACKGROUND Cardiac resynchronisation therapy (CRT) is increasingly used in children in a variety of anatomical and pathophysiological conditions, but published data are scarce. OBJECTIVE To record current practice and results of CRT in paediatric and congenital heart disease. DESIGN Retrospective multicentre European survey. SETTING Paediatric(More)
BACKGROUND Desmin-related myopathy is a clinically heterogenous group of disorders encompassing myopathies, cardiomyopathies, conduction disease, and combinations of these disorders. Mutations in the gene encoding desmin (DES), a major intermediate filament protein, can underlie this phenotype. OBJECTIVE The purpose of this study was to investigate the(More)
BACKGROUND Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmia syndrome associated with mutations in the cardiac ryanodine receptor gene (Ryr2) in the majority of patients. Previous studies of CPVT patients mainly involved probands, so current insight into disease penetrance, expression, genotype-phenotype correlations,(More)
In this part of a series on founder mutations in the Netherlands, we review a Dutch family carrying the SCN5a 1795insD mutation. We describe the advances in our understanding of the premature sudden cardiac deaths that have accompanied this family in the past centuries. The mutation carriers show a unique overlap of long-QT syndrome (type 3), Brugada(More)
The objective of this multicenter study was to evaluate psychological functioning and disease-related quality of life (DRQoL) in pediatric patients with an implantable cardioverter defibrillator (ICD) in The Netherlands. Thirty patients were investigated; the mean age was 16.3 years, and the mean duration of implantation was 3.6 years. To assess(More)
OBJECTIVE To connect the hepatic veins to the azygos venous system through a lateral thoracotomy, and without the use of extracorporeal circulation, so as to relieve arteriovenous fistulas after a previous Kawashima operation. METHODS Description of the operative technique by which the hepatic veins are anastomosed to the hepatic venous system. RESULTS(More)
Fetal tachyarrhythmias are a life-threatening condition complicating a small proportion of normal pregnancies. Despite major advances in the (intrauterine) pharmacologic treatment of these arrhythmias over the last years major uncertainties remain. Among these are controversies in the choice of agents in relation to arrhythmia type, and timing and duration(More)