ARRHYTHMOGENIC RIGHT-VENTRICULAR DYSPLASIA AS A CAUSE OF SUDDEN-DEATH

Arrhythmogenic right ventricular dysplasia (ARVD) is a poorly understo od and often underdiagnosed disorder of the right ventricle, character ized by replacement of myocardium by fibroadipose tissue, arrhythmic m anifestations, and sudden death. The disease occurs in families and is inherited as an autosomal dominant trait. This report describes five cases of ARVD identified by autopsy. In three of the cases, sudden dea th occurred in the young (16-28 years old) during or shortly after exe rcise. In another case, a 46-year-old man with no relevant medical his tory was found dead in his bathroom. In the last case, a 57-year-old w oman died from pulmonary thromboembolism. In none of the subjects had the disease been diagnosed or suspected before death. Only one (a 21-y ear-old man) had previous typical symptoms of the disease. Autopsy exa mination showed right ventricle dilation and, in four cases, cardiomeg aly. The right ventricular myocardium of all hearts was almost replace d by adipose tissue and to a variable degree by fibrous tissue, while the left ventricle myocardium demonstrated no, or only scattered, fibr o-fatty infiltration. Postmortem diagnosis of ARVD can be important in identifying possible affected family members in order to initiate tre atment.