ARRHYTHMOGENIC RIGHT-VENTRICULAR CARDIOMYOPATHY IN YOUNG VERSUS ADULT PATIENTS - SIMILARITIES AND DIFFERENCES

Objectives. This study was designed to evaluate and compare the patterns of arrhythmogenic right ventricular cardiomyopathy in young people and adults. Background. Few data are available on this cardiomyopathy in young people because clinical and morphologic findings considered pathognomonic are normally based on observations in adults. However, a familial occurrence with a probable genetic transmission led to the study of children and to early detection of this disease, in which sudden death has been reported even in young people. Methods. Seventeen young patients with arrhythmogenic right ventricular cardiomyopathy diagnosed at a mean age +/- SD of 14.9 +/- 4.9 years were studied. Clinical, electrocardiographic, echocardiographic, cineangiographic and biopsy findings,were compared with those of 19 adult patients whose condition was diagnosed at a mean age of 38.1 +/- 13.4 years. Results. Syncope occurred in 23.5% of the young patients but in none of the adults (odds ratio of familial sudden death 5.54, p 0.1). Ventricular couplets (adds ratio 16.0, p = 0.004) and subtricuspid bulging on echocardiography (odds ratio 5.95, p 0.036) were prevalent in the young group. Cineangiographic data in the two groups were similar, except that more hypokinetic areas were found in adults (odds ratio 4.44, p = 0.05). Morphometric analysis of biopsy sections showed a greater amount of fibrous tissue in the young patients (p = 0.04) and a prevalence of fatty tissue in the adults (odds ratio 12, p = 0.005). During an equivalent follow-up time (mean 7 years), two young patients died suddenly, and two had ventricular fibrillation in the absence of antiarrhythmic therapy. Conclusions. The pathognomonic criteria for the diagnosis of arrhythmogenic right ventricular cardiomyopathy in adults are also valid for young people. Sudden or aborted death occurred frequently in young untreated patients.