There is increasing evidence regarding the prevalence of genetic cardiomyopathies, for
which arrhythmias may be the first presentation. Ventricular and atrial arrhythmias …

After its recognition as a distinct clinical entity, Brugada syndrome is increasingly recognized
worldwide as an important cause of sudden cardiac death. Brugada syndrome exhibits …

Background: The right ventricular outflow tract (RVOT) is acknowledged to be responsible
for arrhythmogenesis in Brugada syndrome (BrS), but the pathophysiology remains …

Brugada syndrome (BrS), early repolarization syndrome (ERS), and idiopathic ventricular
fibrillation (iVF) have long been considered primary electrical disorders associated with …

Background—Brugada syndrome is a rare, autosomal-dominant, male-predominant form of
idiopathic ventricular fibrillation characterized by a right bundle-branch block and ST …

Myotonic dystrophy (DM) is caused by the expression of mutant RNAs containing expanded
CUG repeats that sequester muscleblind-like (MBNL) proteins, leading to alternative splicing …

Background—Mutations in SCN5A are identified in≈ 20% to 30% of probands affected by
Brugada syndrome (BrS). However, in familial studies, the relationship between SCN5A …

The generation of the mammalian heartbeat is a complex and vital function requiring
multiple and coordinated ionic channel activities. The functional role of low-voltage activated …

Since the identification of the first SCN5A mutation associated with long QT syndrome in
1995, several mutations in this gene for the α subunit of the cardiac sodium channel have …

First introduced as a new clinical entity in 1992, the Brugada syndrome is associated with a
relatively high risk of sudden death in young adults, and occasionally in children and infants …