2. Sudden Death by Arrhythmogenic Right Ventricular Dysplasia in a South American Transsexual?

Abstract

Arrhythmogenic right ventricular dysplasia (ARVD) is a rare, but increasingly diagnosed, anatomo-clinical entity, characterized by a regional or diffuse replacement of the myocardium of the right ventricle (RV) by adipose/fibroadipose tissue. We report a probable case of a sudden death by ARVD, initially misinterpreted by investigators as a violent death, in a 22-year-old, South American transsexual, HIV-positive, while attempting clandestine immigration from Switzerland to Italy. The demonstration of the causal connection between RVD, electrical instability of the RV and death may be puzzling, especially when circumstances of death are unclear. The still unclear nosological borders between normal adipose content, ‘near physiological’ lipomatosis and RVD, the uncertain topographic correlation between (fibro) adipose infiltration and electrical instability of the RV as well as the incidental finding of extensive fibro-fatty infiltration of the RV also in victims of violent deaths indicate the need to consider with caution — and always after the exclusion of other causes — the causal connection between RVD and sudden death.