The American Journal of Forensic Medicine and Pathology 22(1):19–22, 2001. ©2001 Lippincott Williams & Wilkins, Inc., Philadelphia 19 Right ventricular cardiomyopathy dysplasia, now a well- established clinical and morphologic entity, was first re- ported in the medical literature in 1982. The cases of sudden death of two young men are here reported, with macroscopic and histologic findings. The anatomical ex- planation of such death was a fibrotic, lipomatous, or fi- brolipomatous replacement and infiltration of the myo- cardium of the right ventricle. It is suggested that death due to right ventricular cardiomyopathy seems to be the result of electrical instability of right ventricular myo- cardium. There were no congenital malformations such as septal defect or valvular deformity. The subjects’ heart weights were normal. Signs of myocardial degeneration and necrosis with or without inflammatory infiltrates were not identified. Key Words: Sudden cardiac death—Right ventricular cardiomyopathy—Right ventricular dysplasia. Right ventricular cardiomyopathy is currently a definite cause of sudden, unexpected death, espe- cially in young people (1). The entity was reported first in 1982 by Marcus et al. (2), who described the findings in 24 cases, and next by Fontaine et al. (3). Nowadays, right ventricular cardiomyopathy has become a well-established clinical entity, character- ized by ventricular arrhythmias caused by electrical instability of the right ventricular myocardium (4,5). It is a genetically heterogeneous cardio- myopathy, which often causes sudden death in ju- veniles and athletes. The disease loci are mapped to 14q23–24(ARVD1), to 1q42–q43(ARVD2), to 14q12–q2 (ARVD3), and to 2q32(ARVD4) (6–8). Thiene et al. (9) indicated that this entity must be a more common cause of sudden cardiac death in young people than has previously been suspected. Although the excellent summary by Goodin et al. (10) emphasizes the rarity of right ventricular dys- plasia accounting for 1% of sudden cardiac deaths, it is essential to be aware of these rare anatomical conditions that may be implicated (11). More recent studies escalate this number to 2.5% of sudden deaths per year and a prevalence of 1 in 5,000 births (6). Therefore, it is important to know the pathologic states and attempt to explain the pathophysiology of this unusual disturbance in order to interpret the cause of sudden death of this kind. We report here the cases of sudden cardiac deaths of two young men in which right ventricular cardiomyopathy was revealed by postmortem examination. CASE REPORTS Case 1 A 29-year-old man had been asymptomatic and in excellent physical condition until he consulted a physician because of palpitations and sporadic pre- mature ventricular contractions. He took no med- ication because his electrocardiogram showed no Sudden Cardiac Death and Right Ventricular Dysplasia E. N. Michalodimitrakis, M.D. J.D., D. D.-A. Tsiftsis, M.D., A. M. Tsatsakis, Ph.D., and I. Stiakakis, M.D. Manuscript received March 3, 2000; accepted April 21, 2000. From the University of Crete, Department of Forensic Sci- ences, Crete, Greece. Address correspondence and reprint requests to E. N. Michalodimitrakis and Aristidis M. Tsatsakis, Department of Forensic Sciences, Medical School, University of Crete, Her- aklion 714 09, P.O. Box 1393, Crete, Greece.