Arrhythmogenic Right Ventricular Cardiomyopathy in a Young Athlete

Sudden death in young athletes is rare, with an incidence of 0.61/100,000 person-years in the United States (Circulation. 2009;119(8):1085-1092). Etiologies may be divided into cardiac and noncardiac. We present a case of arrhythmogenic right ventricular cardiomyopathy (ARVC) resulting in sudden death in a high school athlete.A 17-year-old adolescent boy with an unremarkable medical history suddenly collapsed while running. Autopsy revealed a 510-g heart with a markedly dilated right ventricle and multifocal areas of wall thinning and apparent fatty infiltration. The left ventricle demonstrated concentric hypertrophy. Coronary arteries were unremarkable, as were the cardiac valves. Microscopic examination revealed right ventricular myocyte loss with replacement by fibrofatty tissue. Remainder of the autopsy was negative except for mild chronic asthmatic changes. Serum and urine drug screens were negative. The cause of death was ARVC. The manner of death was natural.Arrhythmogenic right ventricular cardiomyopathy is an inherited cardiomyopathy with variable expressivity and incomplete penetrance often resulting in heart failure or sudden cardiac death. It is characterized by segmental and multifocal fibro-fatty replacement of the right ventricle free wall. Arrhythmogenic right ventricular cardiomyopathy accounts for 2.1% to 4.0% of total sudden deaths in young athletes and thus should be considered in similar cases (J Am Coll Cardiol. 2018;72(7):784-804).