Sudden death due to Arrhythmogenic Right Ventricular Dysplasia in young men. Report of two cases

Abstract

Right ventricular dysplasia is a distinct cardiomyopathy characterized by progressive replacement of right ventricular myocytes by fat cells. The disease is frequently associated with ventricular arrhythmias, with a variable clinical course and a constant threat of sudden death. We report two cases of arrhithmogenic dysplasia in young men, whose death was myocardial infarction due to tachyarrhythmias. The right ventricle showed interstitial sclerolipomatosis and chronic myocarditis. Ventricular septum presented dystrophic lesions of myocardial fibers, compression of adjacent myocardial fibers, pronounced stasis and sclerolipomatosis to. Left ventricle wall showed patchy interstitial sclerolipomatosis or fibrosis and interstitial edema. Coagulative necrosis was identified in the right ventricle wall in one case and in the left ventricle wall in the second case. As a special feature, in both cases, left ventricular involvement was observed. Since the disease is a cause of sudden death and has a vital prognosis, an early diagnosis is essential for preventing lethal arrhythmias.