Ventricular Arrhythmias in Inherited Channelopathies

The majority of sudden cardiac death (SCD) events are associated with structural heart disease; however, in 10% to 20% of SCDs, no cardiac struc­tural abnormalities are detectable. The lack of an apparent cause in many of those cases initially led to the classification as “sudden unexplained death syndrome” or “sudden infant death syndrome.” Many of these are caused by primary electrical disorders, including long QT syndrome, catecholaminergic polymorphic VT, Brugada syndrome, and short QT syn­drome, as well as cases identified as “idiopathic VF” when the underlying cause often remains unknown. Risk stratification, genetic testing, genetic counseling, lifestyle modifications, pharmacologic therapy, and defibrillator implantation are important considerations in the management of affected individuals.