Genetic basis of malignant channelopathies and ventricular fibrillation in the structurally normal heart

Abstract

Sudden cardiac death occurs in a minority of patients in the absence of structural or functional abnormalities. In this category, pure electrical heart diseases are responsible for a large number of these unexpected deaths. These conditions include the long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, short QT syndrome (collectively referred to as channelopathies) and idiopathic ventricular fibrillation. This article reviews the current molecular understanding of the electrical diseases of the heart associated with sudden cardiac death, and provides a summary of the causal genes and a flowchart with an overview of the genotype–phenotype correlation of the most common arrhythmia syndromes.