Sudden Cardiac Death in Genetic Cardiomyopathies

Abstract

Sudden cardiac death (SCD) caused by ventricular arrhythmias is common in patients with genetic cardiomyopathies (CMs) including dilated CM, hypertrophic CM, and arrhythmogenic right ventricular CM (ARVC). Phenotypic features can identify individuals at high enough risk to warrant placement of an implantable cardioverter-defibrillator, although risk stratification schemes remain imperfect. Genetic testing is valuable for family cascade screening but with few exceptions (eg, LMNA mutations) do not identify higher risk for SCD. Although randomized trials are lacking, observational data suggest that ICDs can be beneficial. Vigorous exercise can exacerbate ARVC disease progression and increase likelihood of ventricular arrhythmias.

Keywords: Arrhythmogenic right ventricular cardiomyopathy; Dilated cardiomyopathy; Genetic cardiomyopathy; Hypertrophic cardiomyopathy.