Understanding arrhythmogenic right ventricular cardiomyopathy

Abstract

ABSTRACT Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiac disease characterized by fibrofatty replacement of myocardial tissue and is an important cause of ventricular dysrhythmias, ventricular dysfunction, and sudden cardiac death. The clinical course and genetics of this condition are highly variable, and definitive diagnosis can be challenging, despite published diagnostic criteria. Recognizing symptoms and risk factors for ventricular dysrhythmias is key to managing affected patients and family members. High-intensity and endurance exercise is widely known to increase disease expression and progression; however, a safe exercise regimen remains uncertain, and a personalized approach to management should be considered. This article reviews the incidence, pathophysiology, diagnostic criteria, and treatment considerations for ARVC.