The Other Side of Arrhythmogenic Right Ventricular Cardiomyopathy

Knowledge regarding arrhythmogenic right ventricular cardiomyopathy (ARVC) has increased dramatically since the publication of the original International Task Force diagnostic criteria in 1994. Based on symptomatic index cases and sudden cardiac death victims, the Task Force criteria emphasized manifestations observed only at the most severe end of the clinical spectrum of ARVC. It is now known that the phenotypic expression of the disease may vary considerably both in its severity and in the pattern of myocardial involvement. Recent familial studies have emphasized that, despite its name, the abnormalities of ARVC are not limited to the right ventricle. In recognition of the prevalence and clinical significance of left ventricular involvement in ARVC, a proposed modification to the original International Task Force criteria was recently published. We herein describe 3 cases of patients with ARVC who presented with left ventricular involvement as manifested by nonischemic pattern of delayed enhancement in the left ventricle.