Research Day

Arrhythmogenic Right Ventricular Cardiomyopathy In A Young Athlete

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INTRODUCTION: Sudden death in young athletes is rare, with an incidence of 0.61/100,000 person-years in the United States1. Etiologies may be divided into cardiac and non-cardiac, including but not limited to hypertrophic cardiomyopathy, coronary artery anomalies, valvulopathies, hyperthermia, sickle cell disease, and illicit drug use2.

PURPOSE: We present a case of arrhythmogenic right ventricular cardiomyopathy (ARVC) resulting in sudden death in a high school athlete.

MATERIALS: This case is selected from the files of one of the authors (JP).

CASE REPORT: A 17-year-old male with an unremarkable medical history suddenly collapsed while running at football practice. All resuscitative efforts failed. Autopsy revealed a 510g heart with a markedly dilated right ventricle, with 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 exam 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.

DISCUSSION/CONCLUSION: ARVC is an inherited cardiomyopathy with variable expressivity and incomplete penetrance often resulting in heart failure or sudden cardiac death3. It is characterized by segmental and multifocal fibro-fatty replacement of the right ventricle free wall - making early diagnosis challenging3. ARVC accounts for 2.1-4.0% of total sudden deaths in young athletes and thus should be considered as a possible cause in similar cases1,3.

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