منابع مشابه
Inherited Arrhythmias in Children
Sinus node dysfunction has been described as an inherited (autosomal recessive form) problem by Benson et al1. The cause seems to be certain specific mutations on the SCN5A gene. Patients with long QT type 3 (SCN5A mutation) have sinus bradycardia as part of the spectrum of abnormalities in this disease2. Atrial standstill is a rare condition where the atrium is silent i.e. there is no electric...
متن کاملMolecular Mechanisms of Inherited Arrhythmias
Inherited arrhythmias and conduction system diseases are known causes of sudden cardiac death and are responsible for significant mortality and morbidity in patients with congenital heart disease and electrical disorders. Knowledge derived from human genetics and studies in animal models have led to the discovery of multiple molecular defects responsible for arrhythmogenesis. This review summar...
متن کاملInherited sodium channelopathies: models for acquired arrhythmias?
VOLTAGE-GATED Na channels, transmembrane proteins that produce the ionic current responsible for the rapid upstroke of the cardiac action potential, are key elements required for rapid conduction through the myocardium and maintenance of the cardiac rhythm. As such, inherited mutations in SCN5A, the gene encoding the human cardiac Na channel (hH1; Fig. 1), are associated with a range of life-th...
متن کاملAtrial arrhythmias in inherited arrhythmogenic disorders
Atrial arrhythmias are being increasingly recognized in inherited arrhythmogenic disorders particularly in patients with Brugada syndrome and short QT syndrome. Atrial arrhythmias in inherited arrhythmogenic disorders have significant epidemiologic, clinical, and prognostic implications. There has been progress in the understanding of underlying genetic characteristics and the mechanistic link ...
متن کاملGenetics of Inherited Arrhythmias in Children
Over the past two decades, breakthroughs in basic science have revealed the genetic etiology for several inherited arrhythmias. Onset of arrhythmias often commences in childhood and adolescence. The aim of the article is to provide a succinct overview of the genetic background of diseases that may cause life threatening arrhythmias in children and provide a description of reported genotype-phen...
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ژورنال
عنوان ژورنال: Circulation
سال: 2007
ISSN: 0009-7322,1524-4539
DOI: 10.1161/circulationaha.107.711689