Channelopathies as Causes of Sudden Cardiac Death

Abstract

This article reviews the main clinical aspects of 3 channelopathies: the long QT syndrome, the catecholaminergic polymorphic ventricular tachycardia, and the Brugada syndrome. The text summarizes our views on clinical presentation and diagnosis, on risk stratification, and on therapy. Special attention is given to the progress in the understanding of the genetic bases and on the growing impact of genetics on therapy, which, at least in the case of long QT syndrome, now allows gene-specific management.

Keywords: Brugada syndrome; Catecholaminergic polymorphic ventricular tachycardia; Genetic testing; Ion channels; Left cardiac sympathetic denervation; Long QT syndrome; Ryanodine receptor.