Right Ventricular Cardiomyopathy and Sudden Death in Young People

TLDR

We performed postmortem examinations of 60 individuals under 35 who died suddenly in the Veneto Region between 1979 and 1986. We identified right ventricular cardiomyopathy in 12 cases, often undiagnosed before death, with two histologic patterns (lipomatous or fibrolipomatous transformation of the RV free wall) and evidence of exertional deaths, suggesting the condition may be a more common cause of sudden death in young people than previously recognized. (N Engl J Med 1988; 318:129–33.).

Abstract

From 1979 to 1986, we conducted postmortem studies of 60 persons under 35 years of age who had died suddenly in the Veneto Region of northeastern Italy. Unexpectedly, we found that 12 subjects — 7 males and 5 females ranging in age from 13 to 30 years — had morphologic features of right ventricular cardiomyopathy. This disorder had not been diagnosed or suspected before the subjects died. In five cases, sudden death was the first sign of disease; the remaining seven subjects had a history of palpitations, syncopal episodes, or both, and in five of those seven, ventricular arrhythmias had previously been recorded on electrocardiographic examination. Ten of the subjects had died during exertion. At autopsy, the subjects' heart weights were normal or moderately increased. Two main histologic patterns were identified — a lipomatous transformation or a fibrolipomatous transformation of the right ventricular free wall (6 cases each); in all cases, the left ventricle was substantially spared. Signs of myocardial degeneration and necrosis, with or without inflammatory infiltrates, were occasionally observed. These findings indicate that right ventricular cardiomyopathy, the cause of which is still unknown, may be more frequent than previously thought. At least in this area of Italy, it may represent an important cause of sudden death among young people. (N Engl J Med 1988; 318:129–33.)